Prepsa 2022

PREP ® Self-Assessment PREPSA 2022
American Academy of Pediatrics 1

Question 1
3-year-old boy is seen for a health supervision visit. His mother notes that some of his teeth have
developed areas that are whiter than others, similar to those shown in Item Q1 and asks what the
cause is. He does not complain of any pain. He eats a healthy diet, drinking 24 ounces of whole
cow milk per day in addition to tap water. His mother strictly limits his sugar intake. He does not
engage in non-nutritive sucking. He has not yet seen a dentist and he brushes his teeth twice a
week with a pea-sized amount of toothpaste. He takes no other medications or supplements. His
medical history is unremarkable as is his family history with no history of dental problems. On
physical examination, his vital signs and growth parameters are within normal limits. His
examination findings are normal, with the exception of the dental findings.
Item Q1
Of the following, the MOST likely cause of the dental findings for the boy in the vignette is
A. dental erosion
B. early childhood caries
C. fluorosis stains
D. inherited enamel defect

Correct Answer: B
The most likely cause of the findings for the boy in the vignette is early childhood caries (ECC),
given his poor oral hygiene (brushing only 2 times per week) and the pattern of discoloration that
follows the gum line. Dental erosion usually results from increased exposure to acid for which
this child has no risk factors. Fluorosis stains (Item C1A) are usually more scattered in location,
inherited enamel defects appear as white discoloration, but will affect all teeth and are relatively
rare.
Item C1A & Item C1F: Fluorosis categorized as (A) very mild, (B) mild, (C) moderate, and (D) severe.
Reprinted with permission from Lewis CW. Fluoride and dental caries prevention in children. Pediatr
Rev. 2014;35(1):6
Even before their child's first tooth erupts. Pediatricians should advise caregivers on appropriate
oral care. Caregivers should set a good example for their child by limiting their intake or
cariogenic substances such as sweets and tobacco, brushing their own teeth twice daily with
fluoridated toothpaste. Flossing once daily, and seeing a dentist semiannually. They should clean
their child is gums with a clean cloth, especially after any feeding, with naps or at night.
Pediatricians should advise caregivers against bottle-propping and putting the infant to bed with
a bottle. These activities promote prolonged exposure to carbohydrates which can lead to caries
development.

Tooth eruption usually occurs by 12 months of age. Delayed eruption has many causes, including
those listed Item C1B. After the first tooth erupts. Children should have their teeth brushed with
fluoridated toothpaste, using a rice grain-sized amount for children younger than 3 years and a
pea-sized amount for children aged 3 to 6 years (Item C1C). Fluoride varnish should be applied
semiannually. Either by the general pediatrician or by a pediatric dentist. Other age-based oral
health care guidelines are listed in Item C1D and the Bright Futures Oral Health Pocket
Guide.
Item C1B: Causes of Delayed eruption

• Prematurity
• Low birthweight
• Genetic abnormalities like amelogenesis imperfecta and regional odontodysplasia
• Nutritional deficiency
• Down’s syndrome
• Hypopituitarism
Item C1C: A rice-grain-size amount of 1,100-ppm fluoride toothpaste contains 0.055 mg of fluoride. A
pea-size amount of 1,100-ppm fluoride toothpaste contains 0.27 mg of fluoride.
Reprinted with permission from Lewis CW. Fluoride and dental caries prevention in children. Pediatr
Rev. 2014;35(1):10. (photographs courtesy of Katherine Lewis, PhD).

Item C1D: Oral health care guidelines
Minimize exposure to natural or refined sugars
in the infant’s mouth.
• Avoid frequent exposure to foods that can lead to dental caries.
• Hold the infant while feeding. Never prop a bottle (ie, use pillows or any other object to hold a
bottle in the infant’s mouth).
• Do not allow the infant to fall asleep with a bottle that contains milk, formula, juice, or other
sweetened liquid.
• Avoid dipping pacifiers in any sweetened liquid, sugars, or syrups
• For infants and children younger than 3 years, brush the teeth with a small smear (ie, no larger
than a grain of rice) of fluoride toothpaste twice a day (after breakfast and before bed). The child
should not spit out the toothpaste or rinse with water. The small amount of toothpaste that
remains in his mouth helps prevent dental caries.
Prevent the transmission of caries-causing bacteria from adult to infant:

– Practice good oral hygiene and seek oral health care.
– Do not share utensils, cups, spoons, or toothbrushes with the infant.
– Do not put the child’s pacifiers in their own mouths. Clean pacifiers with mild soap
– and water.
– Consult with an oral health professional about the use of xylitol gum or lozenges (if the adult’s
oral health is a concern). This gum may have a positive effect on oral health by decreasing the
bacterial load in an adult’s mouth
Fluoride supplementation (Item C1E) should be considered if the primary water source is
inadequately fluoridated. Fluorosis occurs when children are exposed to excess fluoride,
especially before 8 years of age. After this age, the risk for fluorosis is extremely low, because
all teeth, except the third molars, have completely mineralized. The vast majority or fluorosis in
the United States is either very mild or mild (Item C1F. panels A and B). Moderate and severe
fluorosis (Item G1F, panels C and D) and systemic fluorosis are rare in the United States but
more common in countries where natural fluoride levels in the water are higher.
Item C1E: Fluoride supplementation

Inadequate oral hygiene contributes to caries development, which can initially be asymptomatic
and appear as white discoloration. Without treatment, the caries may become browner and go
deeper into the tooth, causing pain, and if severe, lead to an abscess or facial cellulitis. Early
childhood caries refers to caries in children younger than 6 years and usually affects the upper
incisors, because these teeth are less protected by saliva. The lower teeth are generally spared
because they are better protected by sativa; canines ore also spared because they have smoother
surfaces that do not allow bacteria to accumulate. Caries in older children is more likely to be in
between teeth or affect the molars, which have more grooves.
Inadequate oral hygiene also contributes to periodontal disease. Allowing for plaque build-up
and gingival hyperplasia that easily bleeds and cap be painful. Periodontal hyperplasia can also
be infiltrative as in acute monocytic leukemia, idiopathic, or caused by medications (e.g.,
phenytoin. cyclosporine, o calcium channel blockers). In the latter case, the hyperplasia is not as
painful as prone to bleeding. Chronic gingivitis may progress to gingival recession leading to
loss of bone around the tooth, which may cause pain, sensitivity, and tooth loss. Gingivitis from
poor hygiene usually presents in adolescence or adulthood. Children With-severe gingivitis or
unexplained gingival hypertrophy should be evaluated for other systemic conditions listed in
Item C1G.
Item C1G gingival hypertrophy

– Hereditary: Hereditary gingival fibromatosis
– Storage diseases: GM1 gangliosidosis, I-cell disease
– Drug-induced: phenytoin, nifedipine, and cyclosporin A
– Infiltrative: Leukemia, particularly myelogenous leukemia,

Enamel defects not from caries appear was a rough, white, yellow, or brown discoloration on the
crown of the tooth. Defects can lead to sensitivity, tooth fracture, and caries. Risk factors include
trauma to the primary teeth. Nutritional deficiencies during pregnancy or childhood (especially
vitamins A, C, or D; calcium; or phosphate), and prematurity. Item C1H shows an example of a
congenital enamel defect.
Dental erosion is usually a result of extrinsic acids, such as orange juice, or intrinsic acids, such
as stomach acid Acidic drinks or bulimia usually affect anterior teeth. Acidic solid foods or
gastroesophageal reflux disease usually affect the molars. Item C1I demonstrates erosion on the
lingual aspect of the central incisors in a child with gastroesophageal reflux disease.
Item C1I gastroesophageal reflux disease lingual dental erosion
Children with special health care needs are at higher risk for oral health problems. For instance,
those with severe reflux may experience dental erosion. Children who require polypharmacy
may experience side effects such as dry mouth, which to caries formation. They may also be
uncooperative with daily oral hygiene practices. A dental home that specializes in children with
special needs is therefore crucial for these children.
A brief patient education video from the AAP (healthy children.org) regarding oral health in
children can be found at https://youtu.be/Av26wxBjmdq.

Oral Health Risk Assessment Tool
PREP Pearls
• Early childhood caries present in children younger than 6 years as white discoloration
along the gum line of upper incisors and may progress if untreated.
• Good oral hygiene can prevent most dental and periodontal disease, begins before the
first tooth erupts, and includes limiting the intake of cariogenic substances, appropriate
fluoride usage, flossing, and having a dental home.
• Children with special health care needs are especially prone to develop dental and
periodontal disease.
ABP Content Specifications

• Recognize the causes of delayed dental eruption
• Recognize the various clinical findings associated with dental and periodontal disease

Suggested Readings
• American Academy of Pediatrics, Oral health. https://www.aap.org/en-us/advocacy-and-
policy/aap-health-initiatives/Oral-Health/Paqes/Oral-Health.aspx.
• Dental problems. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. 1948-
1952.
• Council on Children with Disabilities, and Section on Oral Health Oral health care for
children with developmental disabilities. Pediatrics, 2013;131(3):614-619. Reaffirmed June
2018. doi: 10.1542/peds.2012-3650.
• Section on Oral Health, Maintaining and improving the oral health of young children.
Pediatrics 2014; 134(6):1224-1229. Reaffirmed January 2019. Doi 10.1542/peds.2014-2984
• Slayton RL. Oral health. In: Mclnerny TK, Adam HM, Campbell DE. DeWitt TG, Foy JM.
Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca.
IL: American Academy of Pediatrics, 2017:281-291. Pediatric care online

Question 2
A 7-year-old girl is brought to the emergency department for severe muscle cramping in her
hands and feet. The cramping has increased in frequency over the past several days and worsens
with physical activity. She has no significant past medical history. She has a temperature of
37°C, heart rate of 96 beats/min, blood pressure of 102/66 mm Hg, respiratory rate of 14
breaths/min, and oxygen saturation of 98% on room air. Her height and weight are at the 40th
percentile. While the blood pressure cuff is inflated, her hand and wrist muscles maintain
sustained contraction. When her facial nerve is lapped, the corner of her mouth twitches. The
remainder of her physical examination findings are unremarkable. An electrocardiogram shows a
prolonged QTc interval.
Laboratory results are shown:
Laboratory Test Result

Calcium 6.6 mg/dL (1.7 mmol/L)
Albumin 4.2 g/dL (42 g/L)
Phosphorus 8 mg/dL (2.6 mmol/L)
Sodium 140 mEq/L (140 mmol/L)
Potassium 4 mEq/L (4 mmol/L)
Blood urea nitrogen 17 mg/dL (6.1 mmol/L)
Creatinine 0.5 mg/dL (44.2 µmol/L)
Glucose 86 mg/dL (4.8 mmol/L)
Of the following, the MOST likely etiology of the girl's hypocalcemia is

A. Albright hereditary osteodystrophy
B. hypoparathyroidism
C. renal tubular acidosis
D. vitamin D deficiency

Correct Answer: B
The girl described in the vignette has hypocalcemia caused by hypoparathyroidism. Muscle
cramping of her hands and feet that worsens with physical activity, the Chvostek sign (tapping
on the facial nerve eliciting twitching of the muscles at the corner of the mouth), the Trousseau
sign (hand and wrist spasm with inflation of a blood pressure cuff), and the prolonged QTc
interval are all consistent with hypocalcemia. Other symptoms and signs of hypocalcemia
include circumoral and extremity numbness and tingling, laryngospasm, and seizure.
The girl's low calcium and high phosphorus levels are consistent with hypoparathyroidism, Her
parathyroid hormone (PTH) level, although not given in the vignette, would be low. Parathyroid
hormone acts to keep serum calcium levels normal. It releases calcium from bone and reabsorbs
calcium in the kidney. It also stimulates lα-hydroxylase in the kidney to form 1.25-
dihydroxyvitamin D, which increases absorption of calcium from the gastrointestinal tract.
Parathyroid hormone has a phosphorus-lowering effect by causing phosphorus excretion through
the kidney. Therefore, a low PTH level results in hypocalcemia and hyperphosphatemia.
Hypoparathyroidism may be caused by genetic disorders, autoimmune destruction of the

parathyroid glands, infiltrative disorders, or surgical trauma, such as after a total thyroidectomy.
Genetic disorders associated with hypoparathyroidism include 22q11.2 deletion syndrome and
activating mutations in the calcium-sensing receptor. For the girl in the vignette, the most likely
cause of her hypoparathyroidism is autoimmune destruction of the parathyroid glands or an
activating mutation in the calcium-sensing receptor.
Treatment of symptomatic hypocalcemia is with an intravenous infusion of calcium. In the long

term, treatment of hypoparathyroidism consists of oral calcium and calcitriol (1, 25-
dihydroxyvitamin D), the activated form of vitamin D. Calcitriol replacement is required because
of the lack of PTH action on lα-hydroxylase in the kidney to form 1,25-dihydroxyvitamin D.
Albright hereditary osteodystrophy (AHO) results from an inactivating mutation in the GNAS
gene. It is associated with pseudohypoparathyroidism
(Pseudohypoparathyroidism type la). Hypocalcemia and hyperphosphatemia are present, but
PTH levels are high because of PTH resistance. Associated features of AHO include short
stature, obesity, rounded face, brachydactyly, and mild cognitive impairment. Although the
laboratory findings (low calcium. high phosphorus) for the girl in the vignette could be
consistent with pseudohypoparathyroidism, she does not have any of the other associated
features of AHO. There are other forms of pseudohypoparathyroidism not associated with the
AHO Phenotype that can have a similar presentation as the girl in the vignette (e.g.,
pseudohypoparathyroidism type lb), but they are not included in the answer choices.
Renal disorders may result in hypocalcemia. The other laboratory results for the girl in the
vignette, however, are not consistent with renal tubular acidosis.
Vitamin D deficiency may result in hypocalcemia. However, phosphorus levels are typically low
in vitamin D deficiency. Parathyroid hormone levels are high in response to low calcium levels,
resulting in excess renal phosphorus excretion through the kidney.

PREP Pearls
• Laboratory findings consistent with hypoparathyroidism include a low parathyroid
hormone level, hypocalcemia, and hyperphosphatemia.
• Treatment of hypoparathyroidism consists of oral calcium and calcitriol (1,25-
dihydroxyvitamin D), the activated form of vitamin D.
• Signs and symptoms of hypocalcemia include muscle cramping of the hands and feet that
worsens with physical activity, the Chvostek sign (tapping on the facial nerve eliciting
twitching of the muscles at the corner of the mouth), the Trousseau sign (hand and wrist
spasm with inflation of a blood pressure cuff), and the prolonged QTc interval.

• Recognize the typical laboratory features associated with hypo- and hyperparathyroidism
Suggested Readings
• Mancilla E, Levine MA. Hypocalcemia, hypercalcemia, and hypercalciuria, In: Mclnerny
TK, Adam HM, Campbell DE, DeWitt TG, Foy RA, Karnat DM, eds. American
Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL:
American Academy of Pediatrics; 2017:2171-2180. Pediatric Care Online,
• Markowitz ME, Underland L. Gensure R. Parathyroid disorders. Pediatr Rev.
2016;37(12):524.535. doi: https://doi.orq/10.1542/pir.2015-0076.
• Underland L. Markowitz M, Censure R. Calcium, and phosphate hormones: vitamin D,
parathyroid hormone, and fibroblast growth factor 23 Pediatr Rev. 2020;41(1):3-11. doi:
https://doi.orq/10.1542/pir.2018-0065.
• Zhou P. Markowitz M. Hypocalcemia in infants and children. Pediatr Rev
2009;30(5):190-192. Doi: https://doi.orq/10.1542/pir.30-5-190

Question 3
A 2-year-old boy is brought to the emergency department after 5 days of vomiting and diarrhea,
His only oral intake over the past 2 days has been popsicles and small amounts of water. He has
a heart rate of 116 beats/min, respiratory rate of 22 breaths/min. and blood pressure of 90/56 mm
Hg. The boy is lethargic, his mucous membranes are dry, and his capillary refill time is more
than 2 seconds. Laboratory study results are shown:

Serum sodium 120 mEq/L (120 mmol/L)
Blood urea nitrogen 28 mg/dL (10 mmol/L)
Serum creatinine 0.5 mg/dL (44.2 µmol/L)
Serum glucose 90 mg/dL (5 mmol/L)
Of the following, the laboratory data that are MOST consistent with this child's condition are
A. high serum osmolality high urine osmolality, low urine sodium

B. low serum osmolality, high urine osmolality, high urine sodium
C. low serum osmolality, high urine osmolality, low urine sodium
D. low serum osmolality, low urine osmolality, low urine sodium

Correct Answer: C
The child in the vignette has hyponatremic dehydration caused by excessive sodium loss from
vomiting and diarrhea, exacerbated by attempted rehydration with hypotonic fluids (popsicles
and water). This condition results in low serum osmolality. His loss of sodium is extrarenal:
therefore, his urine sodium levels would be low and there would be water conservation by the
kidneys in response to dehydration, resulting in a high urine osmolality.
Serum sodium content is the largest determinant of serum osmolality, the other factors being
glucose and blood urea nitrogen. Hyponatremia is most often associated with hypo-osmolality.
Rarely, hyponatremia may occur with either high (hyperglycemia, mannitol infusion) or normal
(hyperlipidemia, hyperproteinemia) serum osmolality. Hyponatremia results from depletion of
body sodium more than total body water (total body deficit of sodium), .dilution of sodium by
increased body water (dilutional hyponatremia), or a combination of sodium depletion and water
retention. The etiopathogenesis of common disorders leading to hyponatremia is shown in Item
C3A.
Evaluation for the cause of hyponatremia includes a detailed history, clinical assessment of
dehydration or euvolemia, and laboratory tests including serum electrolytes, blood urea nitrogen,
creatinine, serum glucose, serum osmolality, urine osmolality, and urine sodium. In the setting of
hyponatremia, high urine osmolality (concentrated urine) is a result of decreased free water
excretion. This condition may occur as an appropriate response to dehydration (diarrhea,
vomiting), decreased extracellular fluid (nephrotic syndrome, congestive heart failure), or as an
inappropriate response in a euvolemic patient (syndrome of inappropriate antidiuretic hormone
[SIADH]). In the setting of hyponatremia, a low urine osmolality (<100 mEq/L) reflects normal
water excretion, as seen in patients with primary polydipsia.

The urine sodium level is low (<20 mEq/L) in children with extrarenal losses of sodium (e.g. ,
diarrhea, vomiting, burns) or increased proximal renal tubular reabsorption (e.g., nephrotic
syndrome. congestive heart failure). The urine sodium level is high (>40 mEq/L) in children with
diuretic use, salt-losing nephropathy, or SIADH. A simple algorithm for identifying the cause of
hyponatremia using urine osmolality is shown in Item C3B
Hyponatremia with high serum osmolality is seen with hyperglycemia, the serum glucose level
of the child in vignette is normal. Low serum osmolality. High urine osmolality, and high urine
sodium in association with euvolemia es seen with SIADH; the child in the vignette has clinical
evidence of dehydration. Low serum osmolality, low urine osmolality, and low urine sodium
may be seen with primary polydipsia: the child in the vignette lacks a history of excessive water
intake.
Hyponatremia or hypo-osmolality can present with headache, confusion, and seizure secondary
to cerebral edema that results from the shifting of fluids from the extracellular fluid compartment
into the brain cells. Hyponatremia should be corrected slowly, with a target sodium rise of not
more than 0.5 mEq/L per hour or 10 to 15 mEq/L in 24 hours. The rapid correction of chronic
hyponatremia may lead to central pontine myelinolysis, which may result from the shining of
water from brain cells to the extracellular fluid compartment.
PREP Pearls
• Hyponatremia results from a total body deficit of sodium, increased total body water
(dilutional hyponatremia), or a combination of both.
• Urine osmolality and urine sodium levels are helpful in differentiating various causes of
hyponatremia
• Rapid correction of hyponatremia may lead to central pontine myelinolysis

• Distinguish between dilutional hyponatremia and a total body deficit of sodium
• Recognize the clinical and laboratory abnormalities associated with hyponatremic
dehydration, and manage appropriately
• Plan the laboratory, evaluation of hyponatremia while considering the differential
diagnoses associated with the disorder
• Identify the various etiologies of hyponatremia
Suggested Readings
• Jain A. Body fluid composition. Pediatr Rev. 2015:36(4)141-152.doi:
https://doi.orq/10.1542/pir.36-4-141.
• Jones DP. Syndrome of inappropriate secretion of antidiuretic hormone and
hyponatremia. Pediatr Rev. 2018;39(1):27-35.dol https://doi.orq/10.1542/pir.2016-0165
• Mahajan P. Felt JR. Fluids, electrolytes, and acid-base composition. In: Mclnerny TK,
Adam HM. Campbell DE, DeWitt TG. Foy JM, Karnat DM, eds. American Academy of
Pediatrics Textbook of Pediamc Care. 2nd ed. Elk Grove Village, IL: American Academy
of Pediatrics; 2017:419-432, Pediatric care online

Question 4
A 6-year-old boy is being evaluated for a boil on his scalp. He was seen in an urgent care center
1 week ago and treated with amoxicillin-clavulanate but showed no improvement. The scalp
lesion is pruritic but not painful. He has not had a fever. On physical examination, there is a 2 x
2-cm area of hair loss, redness, exudative discharge, and bogginess on the parietal scalp (Item
Q4). He has shotty posterior cervical lymphadenopathy. The remainder of his physical
examination findings are normal.
Item Q4
Of the following, the BEST next step in this boy's management is to administer
A. cephalexin orally
B. clindamycin orally
C. griseofulvin orally
D. vancomycin Intravenously

Correct Answer: C
The boy in the vignette has a localized area of alopecia, redness, exudative discharge, and
bogginess consistent with a kerion, an acute local inflammatory response to a tinea capitis
infection. Griseofulvin a Benzofuran, is the systemic antifungal treatment of choice for kerion
and other noninflammatory tinea capitis infections of the scalp. Cephalexin, a first-generation
cephalosporin, would be appropriate if he had a confirmed or suspected bacterial skin or soft
tissue infection it has good coverage against methicillin-susceptible Staphylococcus aureus and
group A Streptococus, the 2 common gram-positive bacteria implicated in bacterial skin
Infections. Clindamycin offers the benefit of methicillin-resistant S aureus coverage. Intravenous
vancomycin is generally reserved for severe or extensive skin and son tissue bacterial infections.
The boy in the vignette was initially misdiagnosed with a bacterial scalp infection. His clinical
findings or redness, swelling, and discharge could be consistent with a bacterial skin infection.
However, the boy does not have any systemic signs of infection (e.g., fever, malaise), or local
pain or discomfort: he has localized alopecia and pruritus; the lesion is boggy and nontender, and
he did not respond to amoxicillin clavulanate. These features are suggestive of a kerion.
Tinea capitis is one or the most common and highly communicable fungal infections of the scalp
and hair shaft in children. The typical age at presentation is 3 to 9 years. It is more prevalent in
children living in crowded conditions. Most linea capitis infections are caused by Trichophyton
species dermatophyte, with Trichophyton tonsurans being the most common cause.
Microsporum is responsible for the remainder of tinea capitis infections and is transmitted by
pets.
Tinea capitis manifests along a spectrum of 5 clinical presentations:

1. Mild, with seborrhea and scaling, with or without hair loss (Item C4)
2. Pustular, with crusting, either localized or extensive
3. Black dots within areas of alopecia
4. A scaly annular patch of alopecia
5. Kerion. an Inflammatory mass
Item C4

Tinea capitis is primarily a clinical diagnosis. The presence or alopecia, scaling, pruritus, and
posterior cervical lymphadenopathy is highly suggestive of the diagnosis, if the diagnosis is
unclear, dermatoscopy with a lighted magnifier can be performed and may show corkscrew- or
comma-shaped hair. A potassium hydroxide (KOH) wet mount of hair or scale from the affected
area is a quick method to visualize hyphae. Fungal culture can establish the diagnosis.
Because tinea capitis is an infection of the root of the hair follicle, topical antifungals are
ineffective. Griseofulvin has traditionally been the systemic oral antifungal agent of choice. The
duration of therapy is 6 weeks. Other oral treatment options include terbinafine and fluconazole.
PREP Pearls
• Kerion is an inflammatory reaction to a scalp tinea Infection which presents as a large,
hairless, red, and boggy area over the scalp.
• Trichophyton is the most common cause of tinea capitis, followed by Microsporum
species.
• Tinea capitis is treated with a systemic oral antifungal agent such as griseofulvin,
terbinafine, or fluconazole.
MOCA-Peds Objective
• Evaluate and manage a child with a disorder of the scalp
ABP Content specifications

• Recognize the clinical findings associated with tinea capitis, and manage appropriately
• Understand the etiology and complications of Kerions
Suggested Reading
• American Academy of Pediatrics. Tinea capitis. In: Kimberlin DW, Barnett ED, Lynfield
R, Sawyer MH, eds. Red Book: 2021-2024 Report of the Committee on Infectious
Diseases. 32nd ed. Itasca, IL: American Academy of Pediatrics: 2021:755-759. Red Book
Online.
• Barnett NK. Alopecia and hair shaft anomalies. In:Mclnemy, TK, Adam HM, Campbell
DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of
Pediatric Care. American Academy of Pediatrics; 2017:1188-1194. Pediatric Care
Online.
• Gupta AK Imhof RL, Davis DMR, Tollefson MM. Fungal Skin Infections. Pediair Rev,
2017;38(1):8-22. doi: https://doi.orq/10.1542/pir.2015-0140.
• Zaraa I Hawilo A. Aounallah A, et al. Inflammatory tinea capitis: A 12-year, study and a
review of the literature. Afycoses, 2013;56:110-116. doi:10.1111/1439-
0507.2012.02219.x

Question 5
A 10-year-old child with obesity is seen in the offices discus laboratory results. Six months ago,
the child underwent screening for obesity complications. The results were notable for an elevated
alanine transaminase (ALT) level of 85 U/L and an elevated aspartate transaminase (AST) level
of 67 U/L with a normal lipid profile and hemoglobin A1C level. Nutritional and exercise
counseling were provided at that time to the child and family. At today's visit, the child's body
mass index (30 kg/m2), and ALT and AST Levels are unchanged. The child is otherwise healthy
and does not take any medications. The father has a history of hepatitis C infection, which was
successfully treated in the past year. The mother believes that she does not have hepatitis C
infection. There are multiple adult family members with obesity and type 2 diabetes mellitus.
Of the following, the REST next step in the management of this child is
A. initiation of metformin therapy
B. referral for bariatric Surgery
C. referral for polysomnography
D. testing for causes of chronic hepatitis

Correct Answer: D
The child in the vignette has obesity and persistently elevated liver enzymes (alanine
transaminase [ALT] and aspartate transaminase [AST)). Although non-alcoholic fatty liver
disease (NAFLD) is a common cause of elevated liver enzymes in children with obesity
(occurring in up to 30% of cases), other chronic hepatic diseases should be considered. This is
important, as the treatment plan will vary based on the cause. The child in this vignette
underwent further testing and was found to have chronic hepatitis C infection.
`Hepatitis' is a term used to describe hepatic injury; the differential diagnosis includes infectious,
autoimmune, metabolic, drug-induced, genetic, toxin/drug, and traumatic/ischemic causes (Item
C5A). Causes of acute hepatitis in children include infectious hepatitis (hepatitis A, B. C, D, E
and G; adenovirus; cytomegalovirus; Epstein-Barr virus, and others), metabolic disease,
autoimmune hepatitis, and drug-induced liver injury. Chronic hepatitis, defined as hepatitis
lasting longer than 3 to 6 months, is seen in children with infectious hepatitis (generally hepatitis
B and C), autoimmune hepatitis, primary sclerosing cholangitis, metabolic/genetic diseases
(including NAFLD), and drug-induced liver injury. Even without prolonged laboratory
abnormalities, examination findings consistent with cirrhosis and/or portal hypertension (e.g.,
splenomegaly, firm nodular liver edge, palmar erythema, and spider angiomas) should prompt
evaluation for causes of chronic hepatitis. Diagnostic testing for non-infectious causes of
hepatitis is outlined in Item C5B. Children with chronic hepatitis should be monitored for
complications, including malnutrition and failure to thrive, portal hypertension, gastrointestinal
bleeding, ascites, and encephalopathy.
Item C5A: Hepatitis differential diagnosis
Bariatric surgery, metformin, and polysomnography would not be appropriate management for
the child in the vignette. At the time of presentation, the cause of the child's elevated liver
enzymes was unknown. Additionally, neither bariatric surgery nor metformin is recommended to
treat NAFLD in children. While obstructive sleep apnea is associated with NAFLD In children, it
is unknown whether treatment of obstructive sleep apnea improves NAFLD outcomes.
Therefore, polysomnography would not be appropriate at this time.

Item C5B: Diagnostic testing for non-infectious causes of hepatitis
PREP Pearls
• Children with obesity and chronically elevated liver enzyme levels should be screened for
causes of chronic liver disease.
• Children with physical examination findings consistent with portal hypertension
(splenomegaly, firm/nodular liver edge, palmar erythema, and spider angiomas) should
undergo evaluation for causes of chronic hepatitis.
• Complications of chronic liver disease can include Malnutrition and sequelae of portal
hypertension (e.g., gastrointestinal bleeding, ascites, and encephalopathy).

• Identify the immediate and long-term complications of hepatitis
• Recognize the age-related clinical features associated with chronic hepatitis
• Plan the appropriate diagnostic evaluation of hepatitis

Suggested Readings
• Clemente MG. Schwarz K. Hepatitis: General principles, Pediatr Rev. 2011;32(8):333-
340. doi: 10.1542/pir.32-8-333,
• Hardikar W, Schwarz KB. Hepatitis. In: Mclnemy TK, Adam HM, Campbell DE, DeWitt
TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics, Textbook of Pediatric
Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2131-2144,
Pediatric Care Online.
• Vos MB et al, NASPGHAN Clinical practice guideline for the diagnosis and treatment of
nonalcoholic fatty liver disease in children: Recommendations from the Expert
Committee on NAFLD (ECON) and the North American Society of Pediatric
Gastroenterology, Hepatology, and Nutrition (NASPGHAN). J Pediatr Gastroenterol
Nutr. 2017:64(2):319-334.doi:10.1097/MPG 0000000000001482

Question 6
A 2-hour-old term neonate has decreased movement of her left arm in the newborn nursery. Her
mother's pregnancy was complicated by obesity and gestational diabetes. She was delivered
vaginally with vacuum assistance. Her Apgar scores were 8 and 9 at 1 and 5 minutes,
respectively. She weighs 4.6 kg. Physical examination findings are notable for a comfortable-
appearing, macrosomic neonate with left posterior cephalohematoma, no crepitus of the clavicle,
absent left Moro reflex, no movement against gravity of the left arm, positive left-hand grasp,
and active movement of the right arm and lower extremities, Sensation appears intact in all
extremities. The remainder of the physical examination findings are normal. The chest
radiograph is shown in Item Q6.
Of the following, the BEST next step in management for this neonate is
A. left arm immobilization
B. left arm muscle biopsy
C. nerve conduction studies
D. Physical therapy consultation

Correct Answer. D
Based on the chest radiography findings, the neonate in this vignette has a left humeral fracture
likely sustained during birth. She is not moving her left arm because of pain, not neurologic
injury. The next best step in management is immobilization of the left arm at 90 degrees.
Long bone fractures involving the humerus and femur are not rare findings and occur more often
with breech presentation. Additional risk factors and clinical features of humerus and femur
fractures in the neonate are listed in Item C6A. Fractures of the humerus heal quickly and rarely
require additional therapy. They should be evaluated by a pediatric orthopedist on an outpatient
basis.
Item C6A: risk factors and clinical features of humerus and femur fractures
The incidence of birth trauma has decreased over the past 40 years. In 1981, birth injury was the
sixth most common cause of neonatal death (23.8 deaths per 100,000 live births). In 1993, with
changes in obstetric practice and increased use of cesarean delivery rather than complicated
vaginal deliveries, mortality from birth injury decreased to 3.7 deaths per 100.000 live births.
Risk factors and commonly associated birth injuries are listed in Item C6B
Item C6B: Risk factors for birth injuries

Brachial plexus injury is caused by swelling of the nerve sheath surrounding cervical nerve roots
and is more commonly associated with a breech delivery. It typically is a transient Injury. The
clinical presentation varies based on the involved nerve roots. Neonates with brachial plexus
Injury should be referred for physical therapy to optimize range of motion and muscle strength.
Nerve conduction studies and a muscle biopsy are sometimes indicated to diagnose peripheral
Neuropathy or myopathy. This is unlikely given the localized findings of decreased left arm
movement seen in the neonate in the vignette.
PREP Pearls
• The incidence of birth trauma has decreased over the past 40 years likely because of
changes in obstetric care and an increase in operative deliveries compared with
complicated vaginal deliveries
• An asymmetric Moro reflex should be evaluated with chest radiography including humeri
to evaluate for fracture.
• Risk factors for birth trauma include macrosomia and instrument assisted delivery.

• Recognize situations that may Increase the risk of birth injuries
• Identify and manage the neurologic injuries that may occur at birth
Suggested Readings
• Akangire C, Carter B. Birth injuries in neonates. Pediatr Rev. 2016;37(11):451-462.
doi:10.1542/pir.2015-0125.
• Merhar SL, Thomas CW. Nervous system disorders. In : Kliegman RM, St. Geme JW.
Blum NJ, Shah SS, Tasker RC, Wilson KM. Nelson Textbook of Pediatrics. 21st ed.
Philadelphia, PA. Elsevier, 2020:913-925.
• Prazad PA, Rajpal MN, Mangurten HH, Puppala BL. Birth injuries. In Martin RJ,
Fanaroff AA, Walsh MC. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed.
Philadelphia. PA. Elsevier; 2020:458-488.

Question 7
The mother of a 16-year-old calls the office after noticing that one of the dietary supplements her
son is using has 'growth hormone factors' and "human growth hormone secretagogues" listed on
the product label. She reports that he is hoping to make his school's varsity football team next
year. He has started an intensive training program and uses multiple dietary supplements,
including protein powder and creatine. She became very concerned after researching _some of
the effects of growth hormone use, and finding that its use for performance enhancement is
illegal. She has not noticed any significant physical changes in him since starting this regimen
and is asking for advice.
Of the following, the BEST next step is to

A. A, advise her to schedule an office visit to evaluate for possible adverse effects
B. order laboratory evaluation to assess for the presence of exogenous growth hormone
C. reassure her that these supplements are safe for him to continue to use
D. warn her that muscle-building supplements have a significant risk of harmful
contaminants

Correct Answer: D
Dietary supplements are not subject to the same rigorous US Food and Drug Administration
(FDA) oversight as are foods and medications sold by US retailers. In particular, supplements
sold for the purpose of strength building have some of the highest rates of potentially harmful
contamination, and their use should be avoided in children and adolescents.
Adolescents often seek out dietary supplements to improve athletic performance or in pursuit of
a more muscular appearance. These most commonly include protein powders, weight gainers,'
and creatine, but also Include supplements that are marketed as having an effect on human
growth hormone (hGH). Human growth hormone is a large polypeptide that is broken down in
the stomach after oral ingestion, and therefore, oral supplements that claim to contain hGH
would not be expected to produce significant changes in hGH levels. In addition, a number of
supplements are promoted as hGH secretagogues or "boosters" that increase endogenous levels
of the hormone. These supplements often contain high levels of lysine, ornithine, and/or
arginine, which increase hGH levels when administered intravenously, but there is no evidence if
this effect after oral ingestion. These supplements would not produce a physiologic increase in
hGH that would be detectable during an office-based physical examination.
Recombinant hGH is available for therapeutic use in children and adolescents with documented
growth hormone deficiency and is administered subcutaneously. Physiologic replacement of
hGH in deficient Individuals results in increased height, lean body mass, bone density, and
aerobic exercise capacity. However, supraphysiologic doses of hGH do not exert the same
effects. Multiple studies to date have failed to demonstrate athletic performance enhancement
from hGH supplementation in athletes with normal levels of endogenous hGH. However, these
studies cannot ethically replicate the supraphysiologic doses taken by same athletes, and
therefore, it is possible that performance enhancement may occur with high doses of intravenous
or subcutaneous hGH. More recently, hGH supplementation has been purported to assist with
recovery after training or injury, but this has not been studied in the pediatric or young adult
population.
Current data on the prevalence of hGH abuse in adolescents are limited. A 2013 survey found
that 11% of high school students reported previous use of hGH. However, when locking at
results of these survey studies, it is important to recognize that much of this purported use of
hGH is likely ingestion of the dietary supplement categories as described before. For
comparison, almost 20% of male high school seniors report previous creatine use and 1% to 3%
report prior use of anabolic steroids, Polypharmacy is very common among users of appearance
and performance-enhancing substances, and their use appears to correlate with future substance
use and other risk-taking behaviors.
Recombinant hGH is a schedule III drug, and the 1990 Anabolic Steroids Control Act made its
distribution a felony punishable by up to 5 years in prison. Nontherapeutic use of hGH is
prohibited by most major professional sports leagues in the United States, as well as the National
Collegiate Athletic Association and the International Olympic Committee. Doping control
testing within sports consists primarily of looking at ratios of endogenous to exogenous isoforms
of hGH or looking at levels of downstream biomarkers, such as insulin like growth factor 1.
Routine laboratory testing would not be expected to identify potential exogenous use of hGH.
It is important that families realize that dietary supplements do not require FDA approval for
safety or efficacy before coming to market. The requirements for food and medications are very
different, and these products are often intermingled on retail shelves. One way to differentiate
between these categories is to look at the label. Foods contain labels that list 'Nutrition Facts,”
medications list 'Drug Facts,' and dietary supplements list 'Supplement Facts. '
Given the lack of regulatory oversight, consumers of supplements risk using products that
contain contaminants or unlabeled ingredients (including potential allergens), of conversely, do
not contain active ingredients as listed Several studies tested the content of protein supplements
and found that 8% to 20% of these products were contaminated with significant amounts of
heavy metals. An analysis of supplements obtained from US retailers in 2007 revealed that 25%
of them were contaminated with androgenic steroids, and 11% were contaminated with
stimulants. A 2015 investigation found that only 5 of 20 dietary supplement products
consistently contained the active ingredients listed.
PREP pearls
• Supplements sold as human growth hormone (hGH) secretagogues or “boosters” do not
produce changes in hGH levels
• Physiologic replacement of human growth hormone in deficient Increases muscle mass
and exercise capacity, but this does not occur with supraphysiologic supplementation in
individuals with normal levels
• Supplements sold for the purpose of strength building frequently contain potentially
harmful contaminants, and their use should be avoided in children and adolescents.

• Recognize and apply ethical principles involved in deciding when to use enhancement
therapies
• Recognize and apply ethical principles Involved in determining when the use of growth
hormone therapy is appropriate (e, g., in consultation with a pediatric endocrinologist)
• Know the indications for and adverse effects when student athletes ingest sports energy
drinks and protein supplements.
Suggested Readings
• Baumann GP. Growth hormone doping in sports: a critical review of use and detection
strategies. Endocr Rev. 2012:33(2):155-186. doi: 10.1210/er.2011-1035.
• LaBotz M, Griesemer BA; Council on Sports Medicine and Fitness, Use of performance-
enhancing substances. Pediatrics. 2016;138(1):e20161300; doi: 10.1542/peds.2016.1300.
• Siebert DM, Rao AL. The use and abuse of human growth hormone in sports, Sports
Health.2018;10(5):419-426. doi:10.1177/1941738118782688.

Question 8
An 8-year-old boy is brought to the Office for a health supervision visit. His past medial history
is significant for coarctation of the aorta that was surgically repaired when he was a neonate. He
recovered well post-operatively and has had no further medical issues He is active, plays sports,
and does well in school. Hrs parents voice no concerns today. The boy's growth trajectory is
appropriate. He is afebrile. He has a head rate of 80 beats/min, respiratory rate of 15 breaths/min.
blood Pressure in the right upper extremity of 120/80 mm Hg and in the right lower extremity of
95/55 mmHg, and oxygen saturation of 99% on room air. His physical examination findings are
otherwise unremarkable.
Of the following, the BEST next step in management is to

A. Initiate amlodipine
B. Initiate enalapril
C. order abdominal ultrasonography with Doppler
D. order echocardiography

Correct Answer: D
The boy in the vignette has a history of coarctation of the aorta that was repaired surgically as a
neonate. He feels well but has >20 mm Hg gradient of his blood pressure from the right upper
extremity to the right lower extremity. This finding causes concern for re-coarctation of the prior
surgical site. This child needs to see his pediatric cardiologist and undergo echocardiography to
evaluate the aortic arch end left ventricular function. Advanced Imaging. Computed tomography
angiogram, may also be indicated. Thereafter, the child may need surgical or cardiac
catheterization Intervention to relieve any significant narrowing.
It would be inappropriate to start a blood pressure-lowering agent, such as amlodipine or

enalapril, before this fixed obstruction ruled out. While it is possible that someone with an upper
to lower extremity blood pressure gradient has an abdominal coarctation of the aorta, it is less
likely than re-coarctation in a patient with a history of coarctation of the aorta, therefore
abdominal ultrasonography is not the correct choice in this situation.
Coarctation of the aorta refers to significant narrowing of the aorta at the aortic isthmus. This
condition warrants repair, either surgically or by cardiac catheterization procedure. Children with
coarctation are at risk for developing hypertension, even after successful repair. The reported
Incidence is variable but significant. The etiology of this hypertension may be re-coarctation, but
there may be another anatomical explanation. Children with a history of coarctation are also at
risk for 'masked" hypertension, in which the blood pressure is normal in the office but is found to
be elevated by ambulatory monitoring. For this reason, the 2017 Clinical Practice Guideline for
Screening and Management of High Blood Pressure in Children and Adolescents stales that
annual ambulatory blood pressure monitoring is necessary for all children with a history of
repaired coarctation of the aorta, be-ginning no later than 12 years post repair.
An important secondary cause of hypertension to consider, in addition to renal, endocrine, and

genetic abnormalities, is medication use. A thorough history review may reveal not only
prescription medication but over-the-counter medications, alternative therapies, dietary Products,
or recreational drugs that could be the etiology of new hypertension, some of the more common
drugs known to cause hypertension include: oral contraceptives, central nervous system
stimulants, corticosteroids, cold medications, ephedra, recreational stimulants (e.g. cocaine,
amphetamines) and anabolic steroids. In these cases, the hypertension is usually Mild and
reversible with discontinuation of the drug,
PREP Pearls
• Children with a history of repaired coarctation of the aorta are at risk for developing
hypertension rater in life; in some cases, this may be secondary to re-coarctation.
• Annual ambulatory blood pressure monitoring is necessary for all children with a history
of repaired coarctation of the aorta, beginning no later than 12 years post repair.
• A thorough history can reveal pharmacologic etiologies of hypertension; discontinuation
of the drug will typically reverse the Hypertension.

• Recognize the cardiovascular causes of hypertension
• Understand the effects of certain drugs on the development of hypertension in children of
various ages
• Plan the era chat management of hypertension in a patient with coarctation of the aorta
Suggested Readings
• Brady, TM. Hypertension., Pediatr Rev. 2012:33(12):541-552. 10.1542/pir.33-12-541.
• Chandar J, Messiah SE, Zilleruelo G, Lipshultz SE. High blood pressure. In: Mclnemy
TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American
Academy of Pediatrics, Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL:
American Academy of Pediatrics; 2017: 1429-1443, Pediatric Care Online.
• Flynn JT, Kelber DC, Baker-Smith CMn , Bloawdey D, Carroll children and adolescents.
Pediatncs, 2017; 140(3):e20171904. Doi:10.1542/peds.2017-1904
• Khoury M. M Khoury PR, Dolan LM, et al. Clinical implications oo the revised AAP
pediatrics hypertension guidelines 2018:142(2):e20180245 10.1542/peds.2018-0245

Question 9
A fully immunized, previously healthy, 3-year-old girl is being evaluated for viral upper
respiratory illness symptoms of 3 days' duration. She now has a fever of 39.4 C, cough, and
noisy breathing. Her parents have given her an antipyretic medication in the past 3 hours. On
physical examination, she has a temperature of 37.7 °C, respiratory rate of 35 breaths/min, and
oxygen saturation of 94% on room air. The girl seems comfortable and has mild intercostal
retractions but no nasal flaring, diffuse fine crackles and wheezing can be heard in all lung fields.
She is adequately hydrated. The parents are instructed to return if the girl does not improve in 48
to 72 hours or if she worsens at any time.
Of the following, the BEST additional management step for this girl is to
A. obtain a blood specimen for culture
B. obtain a 2-view chest radiograph
C. recommend symptomatic treatment of fever
D. treat with a macrolide antibiotic

Correct Answer: C
The girl in the vignette has a clinical diagnosis of pneumonia based on findings crackles and
wheezing on lung examination, tachypnea, and mildly decreased oxygen saturation. The primary
cause of pneumonia in preschool children is viral infection, and no additional evaluation or
antibiotic treatment is needed. The most appropriate approach for the girl in the vignette is
supportive treatment and monitoring clinical status. If she does not improve in 48 to 72 hours or
worsens at any time, further evaluation for a secondary bacterial infection or complicated
pneumonia is indicated. According to the Infectious Diseases Society of
America/pediatric Infectious Diseases Society guidelines for the management of community-
acquired pneumonia, chest radiograph or laboratory studies are not indicated for the initial
evaluation, and antibiotic treatment is not needed for uncomplicated pneumonia In this age
group.
If there were reason to suspect a bacterial pneumonia, amoxicillin would be the antibiotic of
choice rather than a macrolide. Indications for the use of antibiotics might include sudden onset
of high fever after several days of mild symptoms, focal rather than diffuse findings on chest
examination, signs of respiratory distress, or presence of another underlying condition making
bacterial pneumonia more likely.
If this girl were a fully immunized. School-aged child, a virus would still be the most likely
cause of community-acquired pneumonia, but bacterial infections, particularly with
Streptococcus pneumoniae, become of greater concern. Treatment with amoxicillin from the
time of Initial diagnosis of pneumonia is appropriate. Even in older children, the diagnosis of
pneumonia is made clinically in most cases and chest radiography, cultures and blood cell counts
are not indicated unless there is no response to the treatment in 48 to 72 hours. In school aged
children and adolescents, atypical organisms become more prevalent and should be considered in
the differential diagnosis and treatment planning. In this situation, a macrolide antibiotic would
be appropriate.
Although viral pathogens are the predominant cause of Pneumonia from 2 to 12 months of age,
bacterial infections are the most common source in the first 2 months after birth. Item C9
reviews the most likely causes of Pneumonia in children by age. 4
In making a clinical diagnosis of pneumonia, the most reliable Physical examination findings are
wheezing, retractions, and elevated respiratory rate. Chest radiography adds little to the physical
examination in uncomplicated pneumonia. Of the laboratory inflammatory biomarkers (while
blood cell count. absolute neutrophil count, C-reactive protein, and Procalcitonin), none has been
found useful in discriminating severe from nonsevere disease in children. Blood cultures may be
positive for bacterial pathogens in children with severe or complicated pneumonia, but they are
not likely to be positive in mild to moderate illness conducive to outpatient management.
A child who does not improve with supportive therapy, has a late-onset fever or persistence of
high fever for several days, or has hypoxia and signs of respiratory distress must be considered at
risk for complicated pneumonia with pleural effusion, empyema, necrotizing pneumonia, or a
resistant organism. In these children, it is appropriate to perform chest radiography and possibly
ultrasonography or computed tomography to evaluate for effusion or necrotizing pneumonia. A
blood culture and consideration for bronchoscopy with bronchoalveolar lavage for culture may
be appropriate in uncovering a specific organism and directing treatment in the most severely ill
children, especially when they do not respond to initial narrow-spectrum antibiotic treatment.
Item C9: Pneumonia in children by age
PREP Pearls
• Most pneumonia cases in preschool children are viral and need no intervention.
• The most reliable findings for making a clinical diagnosis of pneumonia in children are
wheezing, retractions, and respiratory rate.
• Bacterial pneumonia in Preschool and school-aged children is most often caused by
Streptococcus pneumoniae for which amoxicillin is the appropriate treatment.
ASP Content Specifications

• identify the major acute and chronic complications of pneumonia
• Recognize the clinical features of pneumonias of various etiologies and the associated
sequelae
• a Plan the appropriate diagnostic evaluation for pneumonias of various etiologies
• a Plan appropriate management of the different types of pneumonia

Suggested Readings
• Bradley JS, Byington CL, Shah SS, et al. Executive summary: the management of
community-acquired pneumonia in infants and children older than 3 months of age -
clinical practice guidelines by the Pediatric Infectious Diseases Society and the Infectious
Diseases Society of America. Clin infect Dis. 2011;53:617-630. doi: 10.1093/cid/cir625.
• Florin TA, Ambroggio L, Brokarnp C. et at. Biomarkers and disease severity in children
with community-acquired pneumonia. Pediatrics. 2020:145(6):e20193728. doi:
10.1542/peds.2019-3728.
• Florin TA, Ambroggio L. Brokarno C, et al. Reliability of examination findings in
suspected community-acquired pneumonia. Pediatrics 2017:140(3):e20170310. doi:
10.1542/peds.2017-0310.
• Light M. Pneumonia. In: Mclnemy TK, Adam HM, Campbell DE, DeWitt TG, Foy JM,
Kamat DM, eds. American Academy of Pediatrics, Textbook of Pediatric Care. 2nd ed.
Elk Grove Village, IL: American Academy of Pediatrics; 2017:2510-2522, Pediatric Care
Online.
• Upsett SC, Han M, Ambroggio L, et at Predictors of bacteremia in children hospitalized
with community-acquired pneumonia, Hosp Pediatr. 2019;9:770-778. doi:
10.152/hpeds.2019-0149.
• Messinger At, Kupfer 0. Hurst A,rarker S Management of pediatric community-acquired
bacterial pneumonia Pediatr Rev. 2017;38:394-407. doi: 10.1542/pir.2016-0183.

Question 10
A 10-year-old girl is accompanied by her single father for her annual hearth supervision visit.
The father is worried about puberty; he wants to know when to expect his daughters first period.
Maternal age of menarche is not known. He steps out of the room for the physical examination
and the girl says that her cousin, age 11 years, just got her first period she would like the
physician to tell her father that she should start wearing a teen bra like her girlfriends.
The girl's height is 142 cm (71st percentile), her weight is 33 kg (50th percentile), and her body
mass index is 18 kg/m2 (41st percentile) A review of her growth chart reveals that since age 3
years, she has consistently grown along the 75th percentile for height and 50th percentile for
weight She has breast tissue that is limited to the area underneath the areola of both breasts, and
she has a small amount of sparse fine pubic hair. The remainder of the physical examination
findings are unremarkable,
Of the following, the MOST accurate statement about this girl's puberty is that her menarche will
occur
A. after she attains the next sexual maturity rating for pubic hair
B. before her growth spurt
C. later than the average age of menarche
D. within the next 2 to 3 years

Correct Answer: D
The girl in the vignette has breast budding (thelarche), sexual maturity rating (SMR) 2, an early
sign of that signals the onset of puberty in girls. Typical/ menarche can be expected to occur
within the subsequent 2 to 3 years of the development thelarche. Most girls achieve menarche at
breast SMR 3 or 4. Many girls attain menarche at an age close to their mother’s age at menarche.
This girl with normal growth and onset of puberty is on track to achieve menarche around the
average age of menarche, about age 12½ years. However, for girls with a body mass index
(BMI) greater than 85%, menarche may occur at an earlier age than for normal-weight girls.
The low levels of estrogen that characterize early puberty also stimulate a growth spurt. Most
gains in linear growth and peak height velocity occur before menarche. Higher levels of estrogen
promote epiphyseal closure that usually limits height increases to 4 to 6 cm after menarche. An
increase in BMI is noted with an increasing percentage of adipose mass during puberty. Most of
the increase occurs after peak height velocity, with a redistribution of adiposity from the
abdomen to the breasts and hips.
The vaginal mucosa is very sensitive to the effects of estrogen. At the onset of puberty.
glycogen-producing epithelial cells increase, lactobacilli flora become more predominant, and
the pH of the vagina changes from neutral (prepubertal) to acidic (<4.5), Physiologic leukorrhea
that may go unnoticed typically occurs 6 to 12 months before menarche. Other physiologic
changes that commonly precede menarche include an increase in the size of the uterus, ovaries,
vagina, labia, and clitoris and thickening of the hymen.
The usual development of secondary sexual characteristics is breast budding first, which is
followed by early pubic hair. Breast budding (thelarche) is a reflection of gonadarche, the
production of estrogen by the ovaries. Pubic hair growth (Pubarche) is a result or adrenarche,
associated with a rise in adrenal androgens. Ovarian androgens also contribute to pubic and
axillary hair growth the development of axillary hair and body odor also is a manifestation of
adrenarche. Gonadarche and adrenarche are separate and independent physiologic events of
puberty in girls. However, some girls may have breast budding and early pubic hair that appear
concurrently. Pubertal development is typically completed within 4 years of the onset. The
sequence of development or secondary sexual characteristics for the girl is summarized in Item
C10
Item C10: Sequence of sexual development

PREP Pearls
• Menarche can be expected to occur within 2 to 3 years of breast budding (thelarche).
• Most gains in linear growth and peak height velocity occur before menarche. ABP
ABP Content Specification

• Understand the sequence of development of secondary sexual characteristics
• Understand the timing of menarche in female adolescents
• Recognize the physiologic changes that commonly precede menarche
Suggested Readings
• Bordini B, Rosenfield RL. Normal pubertal development: Part aspects of puberty. Pediatr
Rev. 2011.1;37(7):281-292. doi: 10.1542/pir.32-7-281.
• Carswell JM, Stafford DEJ. Normal physical growth and development. In: Neinstein LS,
Katzman DK, Callahan ST, Gordon CM, Joffe A, Rickert VI, eds. Neinsfein's Adolescent
and Young Adult Health Care: A Practical Guide. 6th ed. Philadelphia, PA: Wolters
Kluwer, 2016:28-37,
• Chulani VL, Gordon LP. Adolescent growth and development. Prim Care.
2014;41(3):465-487. doi: 100.1016/j.pop.2014.05.002.
• Kritzler RK, Long D, Plotnick L. Puberty: normal and abnormal. In: Mcinemy TK, Adam
HM, Campbell DE, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook
of Pediatric Care. 2nd ed. Itasca, IL American Academy of Pediatrics; 2017:1540-1545.
Pediatric Care Online.

Question 11
A 9-year-old, developmentally appropriate girl is evaluated for unusual movements, the
movements first appeared 2 years ago as forceful eye blinking which occurs at various times
throughout the day but is especially noticeable in the evening when watching television. These
movements self-resolved but re-emerged 6 months ago accompanied by a grimacing expression
and head twitch to the right. When asked the mother reports that the girl frequently sniffs and
clears her throat; these vocalizations have been attributed to seasonal allergies but continued
despite initiation of an antihistamine. Her mother believes that her daughter is unaware of these
movements and vocalizations, however the girl reports that it "feels good” when she does them.
Family history is notable for paternal attention-deficit/hyperactivity disorder. Her physical
examination findings are normal, with the exception of frequent movements and vocalizations
(Item Q11).
Of the following, the comorbid condition this girl is at HIGHEST risk for is
A. breath-holding spells
B. childhood absence epilepsy
C. migraine headache
D. obsessive-compulsive disorder

Correct Answer: D
The child in the vignette meets the diagnostic criteria for Tourette syndrome (TS), a lifelong
neurodevelopmental disorder characterized by motor and vocal tics. Children with Tourette
syndrome frequently experience learning disabilities and behavioral comorbidities. Including
attention-deficit/hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), and
mood and anxiety, disorders. While migraines, breath-holding spells, and childhood absence
epilepsy are all common childhood neurological conditions, they are not considered
comorbidities for children with TS.
Tics, brief stereotyped movements or vocalizations, are a common movement disorder in

childhood; they can be simple or complex and further classified into subtypes (Item C11A),
Item C11B shows an example of a complex motor tic. Tics most commonly emerge in school-
age children, may worsen during adolescence, and persist into adulthood. Several clinical
features distinguish tics from other paroxysmal movements, most notably that tics are:
suppressible, distractible, suggestible, and variable. Tics follow a waxing and waning course,
may evolve over time, and may be suppressed for a brief period (much like a sneeze). Children
younger than 10 years of age commonly appear unaware of the tic, while older children often
describe a premonitory urge. Tics can be provoked by stress or excitement. However, tics are
often noted during more relaxed times, such as when watching television or playing video
games, after having been 'held in' during the day. These clinical features help distinguish tics
from other hyperkinetic primary movement disorders (Item C11C).
Item C11A: Ticks classifications

Item C11C: Movement Disorders
Tourette syndrome represents a subset of tic disorders, characterized by motor and vocal tics that
occur for over 1 year and are often associated with comorbidities such as OCD and ADHD.
Genetic factors likely play a role in causing TS. Although no gene has yet been identified, there
is frequently a family history of tics or their common comorbidities. Children with TS should be
monitored for development of comorbid conditions to allow for prompt recognition and
treatment. While tics often wane in late adolescence and adulthood, psychiatric comorbidities
persist, and individuals may require ongoing clinical care. When discussing the diagnosis with
the family, it is important to define the disorder and discuss any preconceived beliefs about TS.
Providing educational resources to the family, such as those found on the Tourette Association of
America www.tourette.org website, may be useful,
Treatment of tics is symptomatic; early initiation of treatment is not believed to improve the
long-term prognosis Clinical observation is appropriate unless tics impact the child's activities or
become disruptive or painful. The American Academy of Neurology published guidelines for the
management of TS (Pringsheim et al) in 2019. Comprehensive behavioral intervention treatment
is considered first-line treatment if the child is motivated to participate in therapy.
Antipsychotics, α-agonists, or topiramate may be used for symptomatic treatment when tics
cause functional Impairment, Comorbidities, such as ADHD and OCD, should also be addressed
and treatment offered. While stimulants may exacerbate or unmask tics, they may be useful in
treating comorbid ADHD and do not need to be avoided.
PREP Pearls
• Tics are a common childhood movement disorder characterized by suppressible,
suggestible. Variable, and, distractible movements or vocalizations that can wax and
wane over time.
• Tourette syndrome, a lifelong neurodevelopmental disorder defined by the presence of
motor AND vocal tics over a year, is often associated with psychiatric comorbidities,
such attention-deficit/hyperactivity disorder and obsessive-compulsive disorder.
• Clinical observation and surveillance for common comorbidities is appropriate
management for most children with tics. If there is functional impairment, comprehensive
behavioral intervention treatment. α-agonists, antipsychotics, or topiramate can be used
for symptomatic relief.

• Recognize clinical findings associated with Tourette syndrome, and manage
appropriately
• Distinguish among the findings associated with various movement disorders, and manage
appropriately
• Identify the risk factors for various movement disorders
Suggested Readings
• Groth C. Mot Debes N. Rask CU. Lange T, Skov L. Course of Tourette syndrome and
comorbidities in a large prospective clinical study, J Am Acad Child Adolesc Psychiatry.
2017;56(4):304-312 doi: 10.1016/i.jaac.2017.01.010.
• King RA. Tics. In: Mclrierny TK, Adam HM, Campbell DE. DeWitt TG, Foy JM,Kamat
DM. eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk
Grove Village, IL: American Academy of Pediatrics; 2017:1644-1649.
• Kruer MC. Pediatric movement disorders. Pediatr Rev. 2015.36(4104-115. doi: .1 2,
542_12jr,L16-3-11
• Pringsheim T, Okun MS, Muller-Vahl K, et al. Practice guideline recommendations
summary: Treatment of tics in people with Tourette syndrome and chronic tic disorders,
Neurology. 2019:92(19):896-906. doi: 10.1212/WNL.0000000000007466.
• Zinner SH, Mink NJ. Movement disorders I: Tics and stereotypies, Pediatr Rev.
2010;31(6):223. doi:10.1542/pir.31-6-223.

Question 12
A recent study on the use of corticosteroids for the treatment of a newly described multisystem
inflammatory syndrome in children revealed that corticosteroids may confer a statistically
significant reduction in hospital length of stay. In addition, a mortality benefit was seen for
patients receiving corticosteroids, but the authors noted that the study was not powered to detect
a difference in mortality. The researchers wish to replicate the study with a specific focus on the
question of mortality and plan to increase the sample size so that if present, a statistically
significant difference in mortality can be detected between those receiving and not receiving
corticosteroids.
Of the following, the CHANGE in the study design will result in an increase in
A. effect size
B. power
C. random error
A. D significance level

Correct Answer: B
The power of a study relates to the ability to correctly accept or reject the null hypothesis. To
that end, a type 1 error occurs when the, investigator rejects the null hypothesis when it is in fact
true, and the type II error is failing to reject the null hypothesis (accepting the hypothesis) when
it Is in fact false. The probability of a type I error is typically known as alpha (α), while the
probability of a type II known as beta (β). The power a study is defined as (1-β). As the
probability of committing a type II error decreases, the power of the study increases. The
statistical power be thought of as the probability of accepting an alternative hypothesis, when the
alternative hypothesis is true. The higher the power the less likely it is that the investigators will
fail to detect a difference between the groups when a difference actually exists. A larger sample
size will result in a larger power (a lower likelihood of failing to detect a difference between
groups if one exists). None of the other choices are directly affected by the new sample size,
however effect size, random error, and significance level may affect the sample size required
new study.
When designing a clinical trial, the investigator must try to obtain a representative sample of
patients to study. Given that it is rarely feasible to study an entire population, a subset of
participants is therefore selected from the population such that inferences and assumptions can
be made about the population. The subset of study participants is known as the study sample.
Depending on the intended result, the sample size should be calculated before the start or any
clinical trial. The primary reason to determine the sample size is to avoid bias in interpreting
results. If the sample size is too small, the results may not be generalizable to the population
because it is likely that the sample does not represent the population. Also, with a very small
sample size, the investigator may not be able to detect differences between the study groups.
Sample size calculations depend on a number of factors including:

• Power of the study (set by the Investigator)
• Level of significance (set by the investigator)
• Expected effect size (set by the investigator)
• Standard deviation in the population (random error in the sample population)
The difference between the measured variable in the control group and that in the test group is
the effect size and can be expressed as an absolute difference in a measurement (such as 5 kg, or
15 mm Hg) or the relative difference (25% reduction) as a result of the Intervention. The smaller
the effect size, the larger is the sample size required to accurately detect the difference between
the 2 groups. Increasing the sample size has no effect on the effect size; however, decreasing the
effect size would require a larger sample size to detect differences between the groups.
Random error is reflected in the standard deviation of the sample. With larger standard
deviations, a larger sample size is required to ensure that the results of the study are valid. Larger
sample size does not have an impact on random error; however, the smaller the standard
deviation, the smaller is the sample size needed to detect a difference between groups.
The level of significance, often expressed as a P value, indicates the probability that the results
of the study were found by chance alone and not the intervention in question. When set at
P<0.05, the study design allows for a 5% probability that the result observed was because of
chance and not because of the study intervention. Level of significance is set according to the
Investigators' threshold to erroneously report a significant effect. A smaller level of significance
requires a larger sample size, but sample size does not affect the level of significance.
PREP Pearls
• Statistical power (1-β) is defined as the probability or rejecting the null hypothesis when
the null hypothesis is in fact false, or alternatively the probability of accepting an
alternative hypothesis when the alternative hypothesis is true.
• Sample size of study depends on the power set by the researcher, and a larger power
requires a larger sample size.
• Increasing sample size allows for the detection of smaller differences between study
groups.

• Understand how sample size may limit the ability to detect adverse events
• Understand how sample size affects the power of a study
Suggested readings
• Stokes L. Sample Size Calculation for a hypothesis test JAMA. 2014;312(2):180-181
doi:10.100/jama.2014.8295.
• van der Tweel l, Askie L, Vandermeer B, et al. Standard 4: determining adequate sample
sizes. Pediatrics. 2012; 129(suppl 3) S138-S145. doi: 10.1542/peds.2012-0055G.

Question 13
A 30-months-old girl is evaluated for loose stools. She started toilet training at 24 months of age
but has not yet achieved daytime continence, which has frustrated her parents. For the past
month, she has had 1 to 2 large, hard stools per Week. White stooling. She hides in the closet and
refuses to stool on her potty. Over the past week, she has had daily large-volume, stools. The girl
eats a regular diet appropriate for her age and drinks 40 ounces of 2% cow milk per day.
She has been growing and developing well. She appears well and in no distress.
Her abdomen is distended but nontender. There is a large amount of stool palpable in the left
lower quadrant. Rectal examination reveals normal-appearing anus with brown soiling present,
normal anal wink, and dilated rectal vault with palpable, hard stool.
Of the following the MOST effective recommendation to manage the patient's condition is to
A. decrease her milk intake
B. delay toilet training
C. increase her fiber intake
D. initiate a laxative

Correct Answer: D
The girl in the vignette has functional constipation and encopresis due to toilet training refusal
and stool withholding. The clinical picture is or a child who avoids passing large-volume stools
due to dyschezia (excessive straining), leading to hard bowel movements, increased discomfort,
and likely acquired megacolon. She has overflow incontinence with loose stools because she
does not sense a full rectum. The most effective recommendation for managing her constipation
and encopresis at this time is to start a laxative, in addition to education and behavior
modification. While the girl's milk intake is excessive (more than 32 ounces per day), decreasing
her milk intake alone Will not resolve her constipation and encopresis; the same is true for her
fiber intake. Delaying toilet training for a period of at least 3 months in the setting of refusal is
recommended (perhaps longer given her rectal stool burden and encopresis). However, delaying
toilet training will not treat her constipation and encopresis.
Educating families about fecal Incontinence due to constipation is on important first step,
because many families may associate toilet training refusal and soiling with laziness or
purposeful action. Initiating a bowel cleanout disimpaction with a laxative is the next step,
particularly for children who have a large rectal stool burden and overflow incontinence.
Polyethylene glycol, an osmotic laxative, at a dose of 1.0 to 1.5 g/kg/day for up to 3 days is
advised. If possible, rectal enemas should be discouraged. After cleanout, maintenance therapy
includes daily laxative administration and behavior modification. Polyethylene glycol is
recommended as first-line maintenance therapy starting at a dose 0.4 g/kg/day, titrated to achieve
daily soft stools for a minimum of 2 months. Prior to stopping medication, symptoms should be
resolved for at least 1 month. For children who are toilet training, medical therapy should not be
discontinued until successfully toilet trained. Behavior modification may include scheduled toilet
sitting for 5 to 10 minutes after meals, appropriate toilet positioning with feet securely planted on
a surface, and maintaining a diary of episodes of Incontinence and bowel movements to track
progress.
To avoid toilet training refusal, it is recommended that parents avoid parent-child conflict over
toileting, which may be counterproductive and potentially damaging, leading to development of
withholding behaviors, chronic constipation, and encopresis. Any stressors in the child's life
should be addressed, pressure on toilet training should be avoided, and the child should be the
one in charge of the process. Positive feedback systems, such as sticker charts may be helpful.
PREP Pearls
• Management of constipation and encopresis includes medication therapy (for
disimpaction and maintenance), family education, and behavior modification.
• First-line therapy for functional constipation is daily polyethylene glycol; this should
continue for at least 2 months and should not stop unless symptoms have resolved for at
least 1 month.
• To avoid toilet training refusal, it is recommended that parents avoid parent-child conflict
over the issue. Positive feedback systems may be helpful.

MOCA-Peds Objective
• Provide guidance regarding toilet training refusal.

• Recognize stool withholding during toilet training, and manage appropriate
• Plan the appropriate management of encopresis or various etiologies
• Recognize co-morbidities commonly associated with encopresis
• Distinguish between encopresis and delayed bowel training
• Understand the physiologic effects or stool retention.
• Recognize the clinical features associated with fecal overflow incontinence
Suggested Readings
• Toilet training guidelines: Clinicians—the role of the clinician in toilet training,
Pediatrics. 1999;103:1364-1366.
• Toilet training. Pediatr Rev doi:10/154/pir.20-7-240.
• Encopresis, American Academy of Pediatrics Textbook ot Pediatric Care 1995-2001.
• Toilet training. Zuckerman Parker Handbook of Developmental and Behavioral
Pediatrics for Primary Care. 4th ed. 2019:431.
• Toilet training methods, clinical interventions, and recommendations. Pediatrics. PMID:
10353954.

Question 14
A 16-year-old adolescent girl is seen for a health supervision visit. She was evaluated 1 month
ago at the emergency department for acute-onset chest pain; she was found to have a left-sided
spontaneous pneumothorax and underwent urgent needle decompression. She was discharged to
home after 4 days and recovered fully within a week. Today, the girl's height and weight are at
the 98th and 40th percentile, respectively. She has a long and narrow face, a high-arched palate,
malar hypoplasia, joint laxity, tong fingers, and asymmetry on forward bending. She wears
glasses for high myopia.
Of the following, the MOST common cause of morbidity and mortality in individuals affected by
this adolescent's condition is
A. aortic dilatation and dissection
B. Dural ectasia
C. hypertrophic cardiomyopathy
D. spontaneous pneumothorax

Correct Answer: A
The girl described in thea vignette has Marfan syndrome, a progressive tissue disorder with
multiorgan Involvement. Marfan syndrome is caused by a variant in the FBN1 gene and is
inherited in an autosomal dominant manner with phenotypic variability. The major organ
systems affected include the cardiovascular, skeletal, and ocular. Cardiovascular features which
are the most common cause of morbidity and modality, include aortic dilatation with risk for tear
and rupture, mitral valve prolapse and regurgitation, aortic valve insufficiency, tricuspid valve
prolapse, and enlargement of the proximal pulmonary artery. Hypertrophic cardiomyopathy is
not a clinical feature of Marfan syndrome. Skeletal features include a reduced upper-to-lower
body segment ratio, increased arm span to-height ratio, scoliosis, rib-cage asymmetry with
pectus carinatum and/or excavatum, and joint laxity. Ocular features include progressive high
myopia, and ectopia lentis associated with an increased risk of glaucoma, retinal detachment, and
early cataracts.
Baseline ophthalmological surveillance for children with Marfan syndrome includes annual slit
lamp examination for assessment of refraction and monitoring for evidence of lens subluxation,
glaucoma, or cataract. Cardiovascular surveillance includes annual echocardiography with aortic
root (Ao) measurements.
Cardiovascular management of children with Marfan syndrome is initiated at the time of

diagnosis of aortic dilatation with agents that decrease the hemodynamic stress on the aortic wall
such as ß-blockers or angiotensin receptor blockers. Surgical intervention for Ao dilatation in
infants is considered for an excessive rate of increase of Ao diameter or progressive and severe
aortic regurgitation. In addition to the above criteria, surgical intervention for older children is
considered when the Ao diameter is close to 5 cm. Pregnancy is considered high risk because of
the risk for rapid progression of Ao dilatation and subsequent dissection or rupture. The risk is
sustained during delivery and the immediate postpartum period and requires close monitoring
Dural ectasia refers to excessive stretching of the dural sac in the lumbosacral region. This
occurrence is usually asymptomatic; however, it can produce symptoms of low and proximal leg
pain, weakness, numbness, and genital pain secondary to bone erosion and nerve entrapment.
There is no effective therapy for symptomatic ductal ectasia
Spontaneous pneumothorax occurs as a result of lung bullae, which commonly occur in children
with Marfan syndrome. Positive-pressure ventilation should be avoided, when possible, in those
with multiple bullae. Recurrent pneumothorax can be managed by chemical or surgical
pleurodesis or surgical removal of the blebs.
PREP pearls
• Marfan syndrome is an autosomal dominant disorder with phenotypic variability.
• Cardiovascular, skeletal, and ocular are the major organ systems affected in Marian
syndrome.
• The Cardiovascular features are the most common cause of morbidity and mortality in
Marfan syndrome

• Recognize the clinical and laboratory findings associated with Marfan syndrome
Suggested Readings
• Marfan syndrome. Gene Reviews. Seattle, WA: University of Washington, Seattfe; 2017
• The Marian Foundation https://www.marfan.orq.
• Committee on Genetics. Health supervision for children with Marfan syndrome.
Pediatrics , doi: 10.1542/peds.2013-2063.

Question 15
A 4-year-old previously healthy girl is brought to the clinic with a 3-week history of right neck
swelling. The neck swelling has been gradually increasing in size and is associated with mild
pain. She has no history of fever, malaise, cough, loss of appetite, or weight loss, and no
exposure to pets, sick contacts, or recent international travel. The girl is up to date on her
immunizations. She was treated with a 10-day course of oral cephalexin for the neck mass
without any improvement. On physical examination, she is well appearing and normal vital
signs. The neck shows a swollen, indurated, nontender left anterior cervical adenitis measuring
approximately 3 cm. The skin overlying the mass has a violaceous hue (Item Q15). There is no
adenopathy in the axilla or inguinal region. The rest of the physical examination findings are
normal. A complete blood cell count is normal for age. Purified protein derivative skin test is
negative at 48 hours.
Item Q15
Of the following, the best next management step for this girl is
A. Azithromycin therapy
B. clindamycin therapy
C. incision and drainage of the lymph node
D. surgical excision of the lymph node

Correct Answer: D
The girl described in this vignette has a clinical picture suggestive of subacute unilateral cervical
adenitis because of nontuberculous mycobacteria (NTM) infection. The most appropriate next
step is to surgically remove the affected lymph node.
In children. Lymphadenopathy may be defined as acute (<3 weeks duration), subacute (3 to 6

weeks), or chronic (>6 weeks). Subacute or chronic cervical adenopathy is characterized by
gradual onset of lymph node enlargement which persists over a period of weeks to months. In
children an infectious disease is the most frequent cause of cervical adenopathy (Item C15).
Nontuberculous mycobacteria infection is a common cause of subacute lymphadenopathy in
children, followed by infection with Bartonella henselae (the etiologic agent of cat scratch
disease). Other possible infectious causes of subacute to chronic lymphadenopathy in children
include acquire toxoplasmosis, viral infections (e.g., Epstein-Barr virus, cytomegalovirus. human
immunodeficiency virus type 1), and tuberculosis (scrofula). Mycobacterium tuberculosis must
be considered in cases of chronic lymphadenopathy that are unresponsive to appropriate
antibiotic therapy especially when there are risk factors or tuberculosis.
Item C15: Causes of Pediatric Cervical Lymphadenopathy

• Infectious
– Reactive to viral antigens
• Acute: Rhinovirus, adenovirus, influenza, parainfluenza, respiratory syncytial virus,
others
– Subacute or chronic: Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus
bacterial
• Acute: Staphylococcus aureus, group A streptococcus
• Neonates: Group B streptococcus
• Rarely: Anaerobes
• Subacute or chronic: Bartonella
– Atypical mycobacterial and Mycobacterium tuberculosis
– Fungal
– Parasites
• Congenital neck mass
– Thyroglossal duct cyst, dermoid, branchial cleft, lymphovascular malformation,
hemangioma, ectopic thymus
• Malignancies
– Lymphoma
– Rhabdosarcoma
– Neuroblastoma
• Metastasis (especially from nasopharyngeal and thyroid cancer)
• Miscellaneous
– Kikuchi-Fujimoto disease
– Rosai-Dorfman disease
– Langerhans cell histiocytosis
– Kawasaki disease
– Castleman disease

The Mycobacterium avium complex (MAC) group of NTM (i.e., M avium and Mycobacterium
intracellular) are the most common species of NTM causing adenitis in the United States.
However, many other species have been reported to cause adenitis such as Mycobacterium
scrofulaceum, Mycobacterium haemophilum, and Mycobacterium kansasii.
Nontuberculous mycobacteria are ubiquitous in a wide range of environmental sources (such as

water, food, and soil), and the disease is acquired via exposure to environmental sources. Young
children ranging from 1 to 5 years of age are most frequently affected. Most infections caused by
NTM occur in previously healthy children. Infection with NTM can occur opportunistically in
individuals with compromised cell-mediated immunity (e.g., receipt of immunosuppressive
therapy, acquired immunodeficiency syndrome, genetic defects in interferon-γ receptors or
interleukin 12). Pulmonary disease caused by NTM can complicate bronchiectasis in patients
with cystic fibrosis.
Cervicofacial lymphadenitis is the most common manifestation of NTM infection in the pediatric
age group, followed by skin and soft tissue infection. Entry of NTM via oropharyngeal mucosa
can lead to cervical adenitis. Most frequently, the superior anterior cervical lymph or
submandibular nodes are affected, followed by posterior cervical and preauricular nodes. Lymph
node disease is usually unilateral with an indolent course over weeks to months and absence of
fever or other constitutional symptoms. The affected nodes are enlarged and firm in consistency,
generally nontender and may have a violaceous discoloration; fluctuance can be noted with
spontaneous drainage and fistula formation. Spontaneous regression may occur. Preauricular
suppurative infection can be complicated by facial nerve involvement
In a patient with NTM and chronic lymphadenopathy with absence of fever and other systemic
symptoms, a purified protein derivative (PPD) skin test should be considered as part of the initial
diagnostic workup. The result of the PPD skin test is usually negative but intermediate results
may be positive (6 to 14 mm induration). In contrast, adenitis caused by M tuberculosis is
common in older children and adolescents and often has associated symptoms of low grade
fever, malaise, and weight loss. The diagnosis of atypical mycobacterial adenitis is confirmed
with surgical excision of the lymph node, and isolation of the pathogen on mycobacterial
cultures. Histopathologic examination of the biopsy specimen demonstrates the presence or acid-
fast bacilli on tissue stains and caseating granulomas. Polymerase chain reaction testing of
excised lymph node tissue may be helpful in rapid detection of certain species of NTM. Imaging
modalities such as computed tomographic scanning may not be indicated in all cases of NTM
cervical adenitis, but is often considered if the differential diagnosis of the neck mass includes
lymphoma or congenital malformations such as branchial cyst.
The recommended treatment for NTM cervical adenitis is complete surgical excision of the
affected lymph node which results in a cure rate ranging from 80% to 96%. Local drainage after
excisional biopsy is reported in approximately 10% of cases. Antimicrobial therapy macrolide
class of antibiotics such as clarithromycin or azithromycin in combination with rifampin may be
an alternative treatment option in conjunction with incision and curettage when complete
excisional biopsy is not feasible (e.g., risk of facial nerve injury). Monotherapy is not
recommended because of the emergence of macrolide resistance to MAC and certain other
species of NTM.
Incision end drainage alone without complete excision of the affected nodal tissue is not
recommended in the treatment of NTM adenitis given lower cure rates and risk for development
of chronic fistula. For the girl in the vignette, lack of exposure to cats (or kittens) makes
infection with Bartonella henselae (cat-scratch disease) unlikely, therefore azithromycin therapy
would not be appropriate. The clinical picture of nontender subacute cervical adenitis in a child
without systemic symptoms is not suggestive of bacterial cervical adenitis (caused by
Staphylococcus aureus, group A Streptococcus) and therefore clindamycin would not be
indicated for treatment.
PREP Pearls
• Atypical Mycobacterium infection often presents with subacute to chronic, unilateral
cervical lymphadenopathy without systemic illness in previously healthy young children.
• The superior anterior cervical lymph nodes or submandibular nodes ate most frequently
affected in atypical mycobacterial Infection.
• The treatment of choice for atypical Mycobacterium cervical node infection is complete
surgical excision of the affected lymph node.

• Plan the appropriate management of the complications of nontuberculous mycobacteria
infection
• Recognize the major clinical features associated with a nontuberculous mycobacterial
infection in immunocompetent children
Suggested Readings
• American Academy of Pediatrics. Nontuberculous mycobacteria. Red Book: 2021-2024
Report of the Committee on Infectious Diseases. 32nd ed, 2021:841-821.
• Nontuberculous mycobacterial infections in children. Pediatr Rev. doi:10.1542/pir.2018-
0131
• Weinstock NA Smith I-P. Pediatric cervical lymphadenopathy. Pediatr Rev. doi:
10.1542/pir.2017-0249.

Question 16
A 17-year-old adolescent boy in foster care is seen for a health supervision visit. He has
attention-deficit/hyperactivity disorder, depression, and a learning disability. The boy’s
symptoms have been under poor control. He does not participate during sessions with his
therapist and does not take his prescribed medication regularly. Foster care ends in the state at
age 18 years, and there is concern that he will not be successful in addressing his mental health
needs or living independently when he is no longer in foster care. He states that he can take care
of himself.
Of the following, the core ethical principle MOST applicable to this situation is
A. assent
B. autonomy
C. beneficence
D. nonmaleficence

Correct Answer: B
The ethical principle of autonomy holds that a person has a right to make and act on their own
decisions, such as to decide what happens to their body. Autonomy is the most applicable core
ethical principle for this vignette. Adolescents are recognized to be developing autonomy and
may have the capacity to make decisions about their medical care. This 17-year-old adolescent is
approaching adulthood, and once he is 18 years old, will be considered to have autonomy and the
legal right to make decisions about his medical treatment. This right includes deciding that he
does not want a recommended treatment, such as therapy or medication.
Respect for autonomy means that physicians should accept the patient's decision to agree to or
decline treatment if the patient has the ability to understand the treatment and its risks, benefits,
and alternatives. Adults are generally considered to have the capacity to make medical decisions,
with the exception of those with intellectual disabilities. In pediatrics, parents typically make
medical decisions for their children. However, children in treatment discussions and their input
considered as appropriate for their developmental/cognitive level and for the specific situation.
Assent, or the child's agreement to receive a treatment, should be obtained whenever possible.
For children in foster care, issues surrounding consent can be complex, and health care decisions
may be made by individuals or entitles such as the child's parents, legal guardian, the court, the
child welfare agency, or the county social worker. Consent rules may be different for special
circumstances (e.g., emergency care. temporary custody) or for certain services (e.g., for mental
health, pregnancy-related care, sexual health care). Specific rules about medical decision making
are dependent on the foster care system involved.
Core ethical principles in medicine include autonomy, beneficence, and nonmaleficence. In

medicine, the ethical principle of beneficence means that the Physician should act for the
patient's well-being; the principle of nonmaleficence means that the physician should prevent or
minimize harm to the patient. Although the physician in this vignette may continue to
recommend therapy and medication to address this adolescent’s mental health conditions
(beneficence) and to prevent harm from lack of treatment (nonmaleficence), in the United States,
autonomy generally takes priority, such as when an adult decides to decline recommended
treatment. Assent is not one of the major ethical principles.
PREP Pearls
• Respect autonomy means that physicians should accept a patient’s decision to agree to
decline treatment if the patient has the ability to understand the treatment and its risks,
benefits, and alternatives.
• Children should be included in treatment discussions and their input considered as
appropriate for their developmental/cognitive level and for the specific situation. Assent,
the child's agreement to receive a treatment, be obtained whenever possible.
• For children in foster care, issues surrounding consent can be complex and health care
decisions may be made by individuals or entities such as the child's parents, legal
guardian, the court, the child welfare agency, or the county social worker.

• Recognize and apply ethical principles regarding children in foster care
Suggested Readings
• American Academy of Pediatrics; Committee on Bioethics. Informed consent in
decision-making in pediatric practice. Pediatrics doi: 10.1542/peds.2016-1494
• Ethics for the pediatrician: autonomy, beneficence. and rights; Pediatr Rev. doi:
10.1542/pir.31-6-252.
• Ethical and legal issues for the primary care physician. American Academy of Pediatrics
Textbook of Pediatric Care. 2nd ed:61-72
• American Academy of Pediatrics. Committee on Bioethics. Technical report Informed
consent in decision-making In pediatric practice. Pediatrics. doi: 10.1542/peds.2016-
1485

Question 17
A previously healthy, 2-year-old girl is brought to the clinic for evaluation of drainage from the
right ear for the past 8 weeks. She had cold symptoms just prior to the onset of ear drainage. The
girl has not had any fevers or systemic symptoms of infection. The family has not tried any
treatments. The girl currently appears well. Examination of the right ear reveals purulent
drainage in the ear canal from a hole in the middle of the tympanic membrane,
Of the following, the BEST treatment for this child is

A. cefepime intravenously
B. ceftriaxone intramuscularly
C. levofloxacin orally
D. ofloxacin ototopically

Correct Answer. D
The child in the vignette has chronic suppurative otitis media (CSOM), with greater than 6 weeks
of ear drainage from a perforation in her tympanic membrane. Chronic suppurative otitis media
is most common in children between 0 and 5 years of age. Risk factors include anatomical
malformations of the ear canal, immunodeficiency, and decreased access to medical care.
The bacteria most commonly identified in acute otitis media are Hemophilus influenzae
Streptococcus pneumoniae, and Moraxella catarrhalis. Bacteria that cause CSOM Include
Staphylococcus aureus (both methicillin-resistant and sensitive). Pseudomonas aeruginosa.
Proteus mirabilis, and anaerobic bacteria such as Pepto streptococus and Fusobacterium, Fungal
infections should be considered if the condition is not improving with antibiotic treatment.
Antimicrobial treatment for CSOM should always include Pseudomonas coverage. Treatment
can typically be achieved with ototopical agents such as ofloxacin, ideally administered daily in
the office after clearing debris if there is no improvement after 7 days of treatment, a culture of
the drainage should be obtained and referral to an otolaryngologist should be considered.
Complications of CSOM can include Conductive or sensorineural hearing loss; cerebral

subperiosteal, or extradural abscess formation; meningitis; mastoiditis; or lateral sinus
thrombosis.
In the case of the child in the vignette, an ototopical antibiotic is the first choice for treatment.
Oral levofloxacin would have appropriate coverage but is not recommended for young children.
Intramuscular ceftriaxone lacks Pseudomonas coverage. Intravenous cefepime would have
appropriate coverage but is not indicated in this child who has not failed topical antibiotic
treatment.
PREP Pearls
• Pseudomonas coverage should be included in the treatment of children with chronic
suppurative otitis media.
• Ototopical treatment. Including daily debris removal in the office, is considered first-line
therapy for children with chronic suppurative otitis media
• A culture should be obtained and referral to an otolaryngologist should be considered if
appropriate topical treatment does not resolve chronic suppurative Otitis media.

• Distinguish the pathogens associated with chronic suppurative otitis media from those of
acute otitis media
• Plan the appropriate management of chronic suppurative otitis media in patients of
various ages

Suggested Readings
• Chronic suppurative otitis media. Pediatr Rev. doi:10.1542/pir.20-8-277
• Otitis media: To treat, to refer, to do nothing a review for the practitioner. Pediatr Rev.
doi: 10.1542/pir.36-11-480.
• Otitis media. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed: 2452-
2457.

Question 18
A 14-year-old adolescent boy is brought to the office by his mother for evaluation after he
underwent computerized neuropsychologic testing at his school as a baseline for potential post-
concussion comparison. He performed very poorly on this test, and was advised by the school's
athletic trainer that he needs additional evaluation before he will be allowed to participate on his
school's freshman lacrosse team.
The boy had a concussion last year with complete resolution of symptoms and returned to his
previous level of academic performance. A subsequent preparticipation examination was
performed, and he was cleared for unrestricted sports participation. He reports no additional
injuries. His mother reports no change in personality or behavior and his teachers have not
reported any classroom difficulties. He has been involved in high-intensity cardiovascular
conditioning and plays for a travel lacrosse team without difficulty. He has attention
deficit/hyperactivity disorder (ADHD) that has been well controlled with atomoxetine 60 mg
daily. His neurologic examination findings are normal.
Of the following, the BEST next management step for this boy is to
A. change his ADHD medication regimen
B. clear him for noncontact sports only
C. clear him for unrestricted Sports participation
D. refer him to a neuropsychologist for testing

Correct Answer: C
Clearance (or return to participation in contact Sports after concussion requires
• Full symptom resolution
• No symptom recurrence with high-intensity physical activity
• Return to baseline neurocognitive function and academic performance
The boy in the vignette meets all of these criteria and can be cleared for full athletic
participation. Poor performance on a computerized neuropsychological test (CNT) in an
individual who otherwise meets these requirements should not be used as a sole criterion for
withholding clearance for return to sport.
Provider awareness and documentation of preinjury neurocognitive function are important for
post injury comparisons, and are more accurate than a CNT in detecting post-concussion
neurocognitive deficits. Preinjury function is best documented when an appropriate
preparticipation evaluation (PPE) is performed in the medical home. The current standard of care
for PPEs is reflected in forms from the American Academy of Pediatrics (AAP) at
https://www.aap.org/en/patient-care/preparticipation.physical-evaluation/
The Sideline Concussion Assessment Tool, 5th edition (SCAT5), is used by many providers for
post-concussion assessment of neurocognitive function. Some providers also perform SCAT5
during the PPE to ease postinjury determination of potential deficit, but routine use during PPE is
not currently recommended. The SCAT5 takes about 10 minutes to perform in the office and can
be found at:
Child-SCAT5 for 5-12 years of age
https://bjsm-bmj.com/content/bjsports/early/2017/04/28/bjsports-2017-097492childscat5.full.pdf
SCAT5 for 13 years of age and up:
https://bjsm-bmj.com/content/bjsports/early/2017/04/28/bjsports-2017-097492childscat5.full.pdf
Symptom resolution is a prime determinant for return-to-play decisions after concussion.

However, this relies on accurate, subjective reporting by young athletes who are often highly
motivated to return to their sport. Multiple studies have found that many young athletes do not
accurately report post-concussion symptoms. Computerized neuropsychological tests were
developed in an effort to provide an additional, and more objective, data point to help health care
providers with return-to-play decision-making. These tests are often perceived as de facto
“concussion tests” and as the final arbiter regarding return to play after receiving an injury.
However, this is inaccurate.
Current good standards for determining concussion diagnosis, resolution, and return to play are
based on appropriate evaluation by a provider knowledgeable in concussion assessment and
management. The AAP’s clinical report on sports related concussion
(https://pediatrics.aappublications.org/content/142/6/e20183074#sec-11) provides an overview or
concussion assessment and management for pediatric providers. Computerized
neuropsychological tests may be helpful in equivocal cases, or if there is concern about symptom
reporting. There are multiple reasons for poor performance on these tests (Item C18), and
insolated CNT result should not supersede clinical decision-making.
Use of CNTs in concussion management varies widely by school and region and most typically
includes either:
• Baseline CNTs and mandated post-concussion CNT testing before return to training and
sport
• Postinjury CNT testing without preinjury baselines, and use of normative data to aid
decision-making
Although many schools continue to use CNTs. a number of programs have recognized their
limited benefit (especially relative to cost) and are moving away from their use.
Athletes with ADHD frequently perform poorly on baseline CNT tests compared with
established general norms. These athletes may particularly benefit from obtaining baseline,
preinjury CNTs to ensure an accurate postinjury comparison in those settings in which CNTs are
routine. Changes in ADHD medication may be for problematic changes in behavior or academic
performance but are not indicated for isolated CNT results.
Limited clearance for noncontact sports activity or referral to a neuropsychologist may be helpful
for some athletes with persistent post-concussion symptoms or impairment, but would not be
indicated based solely on CNT results.
PREP Pearls
• Preinjury assessment of neurocognitive function is essential for detecting postconcussion
deficit.
• Neurocognitive function is best assessed in the medical home during health supervision
Visits and/or preparticipation examinations
• Clearance for return to participation in contact sports after concussion requires: full
symptom resolution, no symptom recurrence with high-intensity physical activity, and
return to baseline neurocognitive function and academic performance.
• Computerized neuropsychological test results should not be used to diagnose concussion
or as the sole criterion for return to sports.
MOCA-Peds Objective
• Evaluate and manage a child with postconcussion syndrome.

• Understand the importance of assessing and documenting neurocognitive function prior
to sports participation.

Suggested Readings
• American Academy of Pediatrics (AAP) at https://www.aap.org/en/patient-
care/preparticipation.physical-evaluation/
• Sport-related concussion in children and adolescents. Pediatric. doi:10.1542/peds.2018-
3074.
• Consensus statement on concussion 5th international conference on concussion in sport
held in 2016. BrJSportsMed/. doi: 10.1136/bisports-2017-097699.
• The sports preparticipation evaluation. Pediatr Rev. doi 10.1542/pir.2016-0216.

Question 19
A 1-month-old boy is seen at the clinic for difficulty feeding. He was born at 34 weeks’ gestation
and required a 2-week neonatal intensive care unit stay, mainly for feeding and growing. He
required 1 day of nasal continuous positive airway pressure. At birth, his newborn screen showed
a borderline T-cell receptor excision circle (TREC) assay result, and was therefore repeated.
The infant had been doing well at home, breastfeeding well ad lib, with normal bowel
movements and urine output, until this morning. His vital signs show a temperature of 37 °C,
heart rate of 150 beats/min, respiratory rate of 30 breaths/min and blood pressure of 92/50 mm
Hg He is awake, appears nontoxic and remains arousable.
While the infant is still in the office the pediatrician follows up on the results of the repeat
newborn screen, which are shown below
Laboratory test Result

TREC <125 TERCs/µl
(Newborn screen) (Low, abnormal)
The mother is advised to stop breastfeeding her infant,
Of the following, the BEST next step in the management of this infant is to
A. initiate prophylactic antimicrobial therapy
B. re-evaluate him in 1 month
C. refer urgently to an allergist/Immunologist
D. refer urgently to a hematologist/oncologist

Correct Answer: A
The infant in the vignette has an abnormal newborn screen result with a low T-cell receptor
excision circle (TREC) level. This result causes concern for primary immunodeficiency. T-cell
receptor excision circle levels may be borderline or have a false positive result in premature
infants. It therefore must be repeated until negative or the infant reaches term, 37 weeks. As this
child most likely has a primary immunodeficiency, protecting him from infection is of utmost
importance, He should be started on prophylactic antimicrobials immediately (trimethoprim
sulfamethoxazole and fluconazole). Treatment of infections with trimethoprim-sulfamethoxazole
is usually reserved for infants greater than 2 months of age, however, it may be used for
prophylaxis (mainly for Pneumocystis jirovecii pneumonia) in Infants 4 weeks of age.
The boy will require evaluation of lymphocyte subsets by flow cytometry and referral to an
allergist/immunologist in order to determine his exact diagnosis; however, because his condition
is stable this can occur at a non-urgent outpatient visit. He will also eventually, but not urgently,
need evaluation by the hematologist/oncologist and stem cell transplantation team to determine
whether stem cell transplantation is the treatment of choice for his primary immunodeficiency.
Breastfeeding is usually encouraged, even for infants with many primary immunodeficiencies, as
maternal antibodies can offer some protection; however, it is discouraged if the breast milk
contents may lead to infection. For example, in the setting of a neonate with severe combined
immunodeficiency, a cytomegalovirus (CMV) positive mother may transmit reactivated virus
through breast milk, which can lead to CMV viremia in the child. This transmission may have
serious consequences, especially in the setting of stem cell transplantation. Therefore, the mother
is advised to stop breastfeeding the child until further evaluation can be done.
The Child in this vignette has concerns for primary immunodeficiency and must be evaluated as
soon as possible. Re-evaluation in 1 month, without any current intervention, would be
inappropriate as he could succumb to an overwhelming infection during that time.
PREP Pearls
• Antimicrobial prophylaxis should be started urgently for an infant with a suspected
primary immunodeficiency.
• A low T-cell receptor excision circle (TREC) level on newborn Screening raises concern
for primary immunodeficiency and should be evaluated by flow cytometry for
lymphocyte subsets.
• The cytomegalovirus (CMV) positive mother of a neonate or infant with combined
immunodeficiency disorder can transmit reactivated virus through breast milk, which can
lead to CMV viremia in the child.
ABP Content Specifications(s)

• Recognize the clinical characteristics of cellular immunodeficiency in the first few
months after birth

Suggested Readings
• Immunodeficiency disorders. Pediatr Rev. 10.1542/pir.207-0308
• The role of breast-feeding in cytomegalovirus transmission and hematopoietic stem cell
transplant outcomes in infants with severe combined immunodeficiency. J Allergy Clin
Immunol Pract doi: 10.1016/j.jaip.2019.05.041
• Clinical features that identify children with primary immunodeficiency diseases.
Pediatrics. Doi: 10.1542/peds.2010-3680.
• Challenges of newborn severe combined immunodeficiency screening among premature
infants. Pediatrics. doi:10.1542/peds.2012-1921.

Question 20
A 2-year-old boy is seen in the clinic for evaluation of left eye pain and swelling. He is fussy and
his parents have noticed him touching his left eye through the day. There is no known history or
trauma. He is afebrile but has had nasal congestion and a cough for the past 2 weeks. His medical
history is noncontributory and his vaccinations are up to date. On physical examination, his
temperature is 36.5 °C and his vital signs are normal for age. He is scared and limited in his
cooperation, but his extraocular movements appear normal bilaterally. His visual acuity cannot
be assessed. His conjunctiva is cleat and without discharge. His left eyelid is swollen and
erythematous as shown In Item Q20
Of the following, the factor MOST to predispose this boy to develop this condition is
A. bacterial sinusitis
B. congenital glaucoma
C. Otitis media
D. pollen allergy

Correct Answer: A
The boy in the vignette has findings consistent with preseptal (or Periorbital) cellulitis, including
unilateral erythema and swelling of the anterior eyelid with associated eye pain. Preseptal
cellulitis is associated with sinusitis, dental infections, trauma to the skin (e.g., insect bite, atopic
dermatitis, impetigo), and ocular conditions such os hordeolum or dacryocystitis. It is not
associated with congenital glaucoma or otitis media, Pollen and Other environmental allergies
are associated with periorbital swelling and angioedema, but this is typically bilateral and not
with eye pain.
Preseptal cellulitis is a superficial infection of the eyelid anterior to the orbital septum. It must be
distinguished from orbital cellulitis, or infection of the fat and ocular muscles behind the orbital
septum. Orbital Cellulitis is less common but can lead to permanent vision loss, meningitis,
sepsis, and death. Clinical findings and predisposing factors for each condition are shown in
Item C20
Item C20 Preseptal cellulitis and Orbital Cellulitis

• PRESEPTAL CELLULITIS
• Localized infection of the eyelid or adjacent structure
• Conjunctivitis
• Hordeolum
• Dacryoadenitis
• Dacryocystitis
• Bacterial cellulitis (trauma)
• Hematogenous dissemination
• Bacteremic periorbital cellulitis
• Acute sinusitis
• Inflammatory edema
• ORBITAL CELLULITIS (POSTSEPTAL)
• Subperiosteal abscess
• Orbital abscess
• Orbital cellulitis
• Cavernous sinus thrombosis
• Hematogenous dissemination
• Endophthalmitis
• Traumatic inoculation
• Panophthalmitis
If preseptal and orbital cellulitis cannot be differentiated clinically, computed tomography of

orbits and sinuses can be used to aid in diagnosis. If the diagnosis remains unclear, patients
should be treated clinically as If they have orbital cellulitis because of the potential for severe
outcomes. Blood cultures are rarely positive in patients with preseptal or orbital cellulitis, and
culture material is almost never available at the site of infection. Therefore, antibiotic choices are
empiric and based on the most likely source of infection. Common treatments include
trimethoprim-sulfamethoxazole or clindamycin (for coverage of methicillin resistant
Staphylococcus aureus) plus amoxicillin. Augmentin, or a cephalosporin like cefdinir or
cefpodoxime (for coverage of Streptococcus pneumoniae. other streptococci, and anaerobic
organisms). Preseptal cellulitis be treated with oral end close follow-up, whereas patients with
orbital cellulitis typically require admission to the hospital for multidisciplinary care.
PREP Pearls
• Preseptal cellulitis is a superficial infection of the eyelid anterior to the orbital septum;
orbital cellulitis is infection the fat and ocular muscles behind the orbital septum and is
both more rare and more serious.
• Patients with both preseptal and orbital cellulitis have eyelid swelling end erythema with
associated pain; proptosis pain with eye movements, ophthalmoplegia, diplopia, visual
changes, and fever are suggestive of orbital cellulitis.

• Recognize pathogens commonly associated with periorbital (preseptal) cellulitis
• Plan the appropriate management or periorbital (preseptal) cellulitis
• Plan the appropriate diagnostic evaluation of periorbital (preseptal) cellulitis
Suggested Readings
• Differential diagnosis of the swollen red eyelid. Am Fam Physician.
https://www.aafp.org/afp/2015/0715/p106.html
• Periorbital and orbita cellulitis. Pediatr Rev. doi: 10.1542/pir.31-6-242
• Preseptal and orbital cellulitis. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed 2537-2543
• Paediatric orbital and periorbital infections. Curr Opin Ophthalmol doi:
10.1097/ICU.0000000000000589.

Question 21
A 2-hour-old male neonate born at 38 weeks' gestation is being evaluated. His mother is a 22-
year-old. Gravida 1 para 0 woman with a history of mild phenylketonuria and herpes Infection
treated with valacyclovir. No active genital lesions were noted at the time of delivery. He was
born vaginally with Apgar score of 8 and 9 at 1 and 5 minutes, respectively. His weight is 2.2 kg
(2nd percentile), length 46 cm (5th percentile), and head circumference 33 cm (10th percentile)
Physical examination reveals a well-appearing neonate with 2/6 systolic murmur
Of the following, the finding seen in this neonate are MOST likely caused by
A. abnormal maternal phenylalanine level
B. exposure to valacyclovir
C. herpes simplex infection
D. neonatal phenylalanine hydroxylase genotype

Correct Answer: A
Neonates born to mothers with phenylketonuria (PKU) oro at risk for complications related to
maternal phenylalanine levels. Neonates born to mothers with PKU are exposed to elevated
phenylalanine levels and may develop intrauterine growth restriction, microcephaly, structural
cardiac anomalies, and developmental delays. The risk of developing these complications is
proportional to maternal serum phenylalanine levels.
Phenylketonuria is caused by a defect in phenylalanine hydroxylase resulting in excess serum

phenylalanine. It is the most common inherited disease of metabolism related to aminoacid
metabolism with an incidence of 0.8 in 100.000 live births.
Elevated phenylalanine levels impair myelination and can result in profound neurologic
impairment. Management Includes dietary restriction of natural proteins supplemented with
phenylalanine-free amino acid mixtures. In the 1970s, it was thought that dietary restriction was
only required during childhood and adolescence. However, more recent data suggest that
restriction should continue through adulthood to optimize outcomes. Strict dietary adherence
during pregnancy improves neonatal outcomes.
Neonates with PKU are often asymptomatic in the neonatal period, with abnormal neurologic
findings developing over time. Phenylketonuria is routinely diagnosed on newborn screening
performed before hospital discharge. Ideally, neonates diagnosed with PKU should be followed
in a center with experience managing inherited diseases of metabolism. Strict adherence to these
dietary restrictions is challenging because bland taste.
In comparison to maternal PKU, phenylalanine hydroxylase deficiency in the fetus does not
cause intrauterine growth restriction, because in this situation maternal phenylalanine
hydroxylase would maintain normal serum levels of phenylalanine in the growing fetus. If
neonates with PKU do not have a phenylalanine-restricted diet, they develop neurologic
Impairment and poor growth. Neonatal PKU typically presents only after enteral feedings have
begun. Risk of microcephaly and growth restriction because of herpes infection is unlikely given
that this was not a primary herpes infection. Intrauterine exposure to valacyclovir is not
associated with growth restriction.
PREP Pearls
• Poorly controlled maternal phenylketonuria is associated with growth restriction,
congenital heart disease, and developmental delays.
• Neonates with phenylketonuria are often asymptomatic in the neonatal period and
develop abnormal neurologic findings over time.
• For Individuals with phenylketonuria, dietary restriction of phenylalanine is
recommended throughout life to optimize outcomes.

• Understand the risks for a neonate of a nonadherent mother with phenylketonuria

Suggested Readings
• Phenylketonuria. Pediatr Rev. dot:10.1542/pir.19-6-214.
• Screening for genetic-metabolic diseases. American Academy of Pediatrics Textbook of
Pediatric Care. 2nd ed 210-225.
• Phenylalanine. Nelson Textbook of Pediatrics 21st 695-739

Question 22
A 13-year-old boy with a history of urinary withholding presents to your office with left scrotal
pain and swelling that developed gradually over the last 3 to 4 days. He now complains of pain
with urination, as well as subjective fever. He denies nausea, vomiting, or abdominal pain. On
physical examination, his temperature is 38.5°C, heart rate is 70 beats/min, respiratory rate is 20
breaths/min, and blood pressure is 100/60 mm Hg. The left scrotal area is swollen and tender to
palpation. Pain is relieved with testicular elevation. There is a normal cremasteric reflex.
Urinalysis results are showing pyuria
Of the following, the MOST likely diagnosis is

A. epididymitis
B. Incarcerated inguinal hernia
C. testicular torsion
D. Varicocele

Correct Answer: A
The adolescent boy in this vignette has acute epididymitis. This diagnosis is supported by the
symptoms of dysuria, scrotal pain alleviated with elevation, normal cremasteric reflex, and
pyuria.
Inflammation of the epididymis (epididymitis) in children occurs most frequently in late

adolescence as an ascending Infection from the urethra through the vas deferens. Predisposing
factors in post pubertal boys include sexual activity, heavy exercise, and local trauma (bicycle or
motorcycle riding). Chlamydia trachomatis, Neisseria gonorrhoeae, and Escherichia coli are
common agents that cause epididymitis in sexually active adolescents. Epididymitis in
prepubertal boys is associated with structural anomalies of the urinary tract including ectopic
ureter, urethral obstruction, and dysfunctional voiding.
Epididymitis usually presents with acute or subacute (>24 hours) scrotal pain along with dysuria,
urinary frequency, urethral discharge, and fever. Nausea, vomiting, and a clear history of trauma
are uncommon presenting features. On physical examination, the scrotum may be red swollen
and tender. Elevation of the scrotum may alleviate the pain (Prehn sign). The testis is in a normal
vertical position, and a cremasteric reflex is present. Urinalaysis may show pyuria: a urine
culture should be obtained. Nucleic acid amplification testing for C trachomatis and N
gonorrhoeae in a urine specimen is recommended for sexually active post pubertal boys.
Epididymitis is usually a clinical diagnosis; however, when the diagnosis is unclear. Doppler
ultrasonograph may be performed to confirm increased flow to the epididymis.
Treatment or epididymitis includes analgesics, scrotal support, and rest. For sexually active
adolescents antibiotics (ceftriaxone plus doxycycline) are recommended to treat for chlamydia or
gonorrhea Infection. Quinolones may be used for epididymitis caused by enteric organisms
based on results of the urine culture. Trimethoprim-sulfamethoxazole or cephalexin treatment is
recommended for prepubertal boys and post pubertal boys who are not sexually active and who
have pyuria, a positive urine culture result, or an underlying risk factor for urinary tract infection;
antibiotic treatment is otherwise not required.
Testicular torsion is a serious condition that should be considered in the differential diagnosis of
epididymitis. Testicular torsion presents with the sudden onset (<12 hours) of severe scrotal pain
and swelling. There may be associated nausea and vomiting. The testis is tender, with a
horizontal lie and slight elevation because of twisting of the spermatic cord. The cremasteric
reflex is absent. Doppler ultrasonography should be performed urgently and will confirm
decreased flow to the testicle and twisting of the spermatic cord.
Incarcerated inguinal hernia, most often seen in infants, presents with a painful inguinal mass
that extends into the scrotum. Herniation of bowel or omentum may occur; bowel sounds may be
audible in the scrotum.
Varicocele is a dilation of the veins of the pampiniform plexus the spermatic cord. Varicocele,
most often seen in adolescent boys, presents as a painless, soft scrotal mass ("bag of worms"),
usually on the left side, which decreases in size when recumbent.

PREP Pearls
• Epididymitis usually presents with acute or subacute (>24 hours) scrotal pain along with
dysuria, urinary frequency, urethral discharge, and fever.
• In epididymitis elevation of the scrotum may alleviate pain (Prehn sign), the testis is in
normal vertical position, and the cremasteric reflex is present.
• Treatment of epididymitis includes analgesics, scrotal support, and rest. Antibiotics are
indicated for those who are sexually active and those with pyuria a positive urine culture
or an underlying risk factor for urinary tract infection.

• Recognize the clinical findings associated with epididymitis
• Identify common causes of epididymitis in patients of various ages
Suggested Readings
• Scrotal swelling and pain. American Academy Textbook of Pediatric Care. 2nd ed. Elk
Grove Village.; 2017:1571-1577..
• Update to CDC's treatment guidelines for gonococcal infection, 2020. 1-1916. DOI:
10.15585/mmwr.mm6950a6.
• The male genital system. Pediatr Rev. doi: 10.1542/pir.2017-0316.

Question 23
A 6-year-old boy with no past medical history is brought to the emergency department via
emergency medical services after seizure-like activity. For the past 4 days, he has had headache,
fever, cough, rhinorrhea, and intermittent nonbloody nonbilious vomiting. He has had no rashes,
oral lesions, neck pain, or diarrhea. The boy has been tolerating oral fluids at home, and he has
been given no medications. Today, he began to behave strangely, with incoherent speech,
difficulty Walking and lethargy. He had approximately 1 minute of generalized seizure activity
that self-resolved by the time emergency medical service personnel arrived, and his rectal
temperature was 39.6 °C. Acetaminophen was administered.
In the emergency department, the patient has a temperature of 37.8 °C, heart rate of 125
beats/min, respiratory rate of 16 beats/mi, and blood pressure of 108/70 mm Hg. He is awake and
alert, although he appears confused and agitated, with incoherent speech. His physical
examination reveals a clinically stable, fussy, uncooperative child with no focal neurologic
findings and clear nasal rhinorrhea. Results from a complete brood count, complete metabolic
panel and urinalysis are Unremarkable. An emergency computed tomography scan of the head is
normal. On re-evaluation, the patient’s mental status is unchanged.
Of the following, the BEST next step in the management of this child is to
A. administer an intravenous anti-epileptic medication
B. obtain brain magnetic resonance imaging with and without contrast
C. obtain emergency electroencephalography
D. perform a lumbar puncture with opening pressure measurement

Correct Answer: D
The boy in the vignette has acute encephalopathy. HIS symptoms in combination with a fever
make viral encephalitis (the most common cause of encephalitis) the most likely diagnosis.
Encephalitis (i.e., inflammation of brain parenchyma) may present with neurologic deficits,
abnormal body movement speech abnormalities, altered mental status, behavior and personality
changes seizures, and coma. Presentation can be acute, or there may be a subtle progression of
symptoms. Encephalitis is often accompanied by evidence of meningeal inflammation (i.e.,
meningoencephalitis) such as head and neck pain, nuchal rigidity, Kernig and Brudzinski signs,
nausea, and vomiting. Encephalitis may have bacterial or fungal infectious causes as well as
noninfectious causes such as autoimmune disease; up to 50% or cases may not have an
identifiable cause.
As in all cases of serious illness in children, an urgent assessment of the ABC's (airway.
breathing. and circulation) must be performed for children who show signs and symptoms
concerning for encephalitis, as well as a thorough neurologic examination, including an
assessment of the child’s ability to protect his or her airway. In severe cases, children may have
significant hemodynamic compromise and require resuscitation. In almost all cases, a brain
computed tomography scan should be obtained; this should be ordered before a lumbar puncture
is performed to look for a space-occupying lesion or evidence of increased intracranial pressure
when there are concerning signs or symptoms, or focal neurologic findings. A majority of
children with encephalitis will require nonurgent magnetic resonance imaging (MRI) as well
In children with encephalitis, cerebral spinal fluid (CSF) results commonly include a white blood
cell count of <500 cells/HPF, with a majority of cells being lymphocytes; normal glucose levels;
and normal or slightly elevated protein levels, Cerebrospinal fluid bacterial cultures must be
obtained. Cerebrospinal fluid and blood herpes simplex virus (HSV) and varicella zoster virus
polymerase chain reaction (PCR) tests should be performed. Depending on the clinical situation,
there may be an indication to test for specific viral agents (neonates have a high incidence of
HSV encephalitis) and for additional testing, such as for anti-NMDA (N-methyl-D-aspartate)
receptor antibodies.
Brain MRI should be performed to look for evidence of acute demyelinating encephalomyelitis
(ADEM), HSV encephalitis, or mass lesions, but this imaging is not as urgent as performance of
a lumbar puncture. Electroencephalography to identify the presence of nonconvulsive or
subclinical status epilepticus is important in the evaluation of any patient with encephalitis or
encephalopathy, however, this would not be prioritized over lumbar puncture. The vast majority
of children with encephalitis will have abnormal electroencephalography findings.
Management of encephalitis will vary based on the cause of the condition. Empiric antibiotics
are the standard of care pending 48-hour results-of CSF culture. Additionally, empiric acyclovir
should be administered in most cases until negative results are obtained on the HSV and varicella
zoster virus PCR tests. Because the child in the vignette is not displaying ongoing seizure
Activity, administration of an intravenous anti-epileptic medication is not necessary at this time,
although it may be needed if the patient has another seizure. If culture and PCR test results are
negative and treatable causes of encephalitis have been ruled out, the best management is
supportive care, including physical and occupational therapy as indicated by the child's clinical
condition. No significant benefit has been demonstrated from the use of glucocorticoids (the
preferred treatment in ADEM) plasma exchange, or immune modulator therapies in the treatment
of viral encephalitis.
The prognosis for children with encephalitis is dependent on the cause of the condition, clinical
presentation, and individual patient factors. The risk of mortality and persistent neurologic
consequences is significantly higher in cases of Eastern equine encephalitis and HSV
encephalitis than with other viral causes. Young age and evidence of seizures or encephalopathy
at the time of presentation are risk factors for persistent neurologic conditions. Consequences of
encephalitis may be seen in up to 50% of cases and may include:
• Learning disorders
• Intellectual disability
• Movement abnormality
• Sensory disturbance
• Gait abnormality
• Behavior disorders
• Personality change
PREP pearls
• Cerebrospinal fluid studies and neuroimaging should be performed in the majority of
cases or encephalitis to identify the cause and plan treatment
• Acute encephalitis is characterized by diffuse inflammation of the brain parenchyma;
signs and symptoms may include altered mental status, behavior, or personality changes,
motor or sensory deficits, speech or movement disorders, seizures, and coma.
• Treatment end prognosis for encephalitis depend on the Underlying cause, children may
experience persistent neurologic sequelae
MOCA-Peds Objective
• Recognize and plan initial evaluation of altered mental status

• Plan the appropriate diagnostic evaluation of encephalitis
• Plan the appropriate management encephalitis
• Recognize the clinical findings associated with encephalitis of various causes

Suggested Readings
• Encephalitis in the pediatric population. Pediatr Rev. doi:10.1542/pir.33-3-122.
• Etiology of acute childhood encephalitis at The hospital for Sick Children, Toronto,
1994-1995. Clin Infect Dis. Doi:10.1086/516301
• Encephalitis. Pediatr Rev. 2005, doi: 10.1542/pir.26-10-353
• Cerebrospinal fluid findings in aseptic versus bacterial meningitis.
doi:10.1542/peds.105.2.316.
• Meningoencephalitis. In: American Academy of Pediatrics Textbook of Pediatric Care,
2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2309-2315.

Question 24
A 4-month-old infant is seen for a health supervision visit. She does not track voices or respond
to loud noises. She failed both her newborn hearing screen and a followup audiology evaluation
in both ears. An otolaryngologist and audiologist confirmed bilateral sensorineural hearing loss.
Her father asks about devices that could help to improve her hearing.
Of the following, the MOST appropriate device to consider for this infant is/are
A. a brainstem implant
B. behind-the-ear hearing aids
C. bone-anchored hearing aids
D. cochlear implants

Correct Answer: B
The most appropriate device to consider for the infant in the vignette with sensorineural hearing
loss is behind-the-ear (BTE) hearing aids. Behind-the-ear hearing aids may be provided for
infants as young as age 2 months and are the first line intervention for infants with hearing loss.
Bone-anchored hearing aids are appropriate for children at least 5 years old with conductive
hearing loss. Brainstem implantation in children is considered only in special situations.
Cochlear implants may be considered if hearing aids are not effective; they ore approved by the
US Food and Drug Administration for children as young as 12 months of age.
Sensorineural hearing loss (SNHL) originates from problems with the cochlea or structures
proximal to the cochlea. Early identification is crucial to provide the rapid intervention needed to
maximize communication potential. Most SNHL in children is present at birth, with a genetic
cause responsible for about 50% of cases. Maternal infection (cytomegalovirus, toxoplasmosis,
rubella, and syphilis) may also cause congenital SNHL. In some cases, the Cause remains
unknown.
Sudden-onset SNHL and transient SNHL in previously healthy children are uncommon, and an
otolaryngologist should be involved in their care. These cases are usually caused by infection
and steroids can minimize hearing loss when used in addition to appropriate antibiotics and
antivirals. If sudden-onset SNHL is caused by trauma, emergency surgery may be indicated.
Other rare causes of sudden-onset SNHL include vascular events or fistulas. Sensorineural
hearing loss can result from exposure to medications including aminoglycosides, loop diuretics,
and cisplatin. With medications induced SNHL, the effect is dependent on the dose and duration
of treatment.
Most SNHL in children is permanent, making external devices such as hearing aids the mainstay
of intervention. Although children with very mild hearing loss may benefit from headphones or
earbuds, hearing aids to maintain auditory stimulation of the cerebral cortex are usually the first
device considered. Many types of hearing aids are available. Infants and young children usually
start with BTE hearing aids. Children with unilateral SNHL or bilateral mixed (SNHL and
conductive) hearing loss may use bone conduction hearing aids in which the device is worn on a
headband or attached to the skin with adhesive. Bone-anchored hearing aids are a type of bone
conduction hearing aid that is surgically attached to the mastoid bone. Children with unilateral
hearing loss may benefit from contralateral routing of signal hearing aids through which sound
from the affected ear is transmitted to the unaffected ear. Hearing aids may be paired with other
assistive technologies such as frequency modulation, hearing or induction loop, or infrared
systems, which transmit sounds sound via radio. Electromagnetic, or infrared waves respectively.
Cochlear implants (Item C24) should be considered in children aged 1 to 2 years with bilateral
profound SNHL and older children with severe to profound SNHL if hearing aids or other
amplification devices have not been effective. Children who are not strictly meet these criteria
may still receive cochlear implants after shared decision-making with a cochlear implant
surgeon.
Brainstem implantation in children is a relatively new procedure. This intervention may be

offered to children in whom cochlear Implants are not effective or whom are unable to receive a
cochlear implant (e.g., due to an unformed or malformed cochlea or defective cochlear
innervation).
Item C24: Cochlear implants
All children with SNHL should be referred as soon as possible to speech therapy services and
audiologic habilitation to learn how to listen, communicate, and use their device. They should
receive accommodations in school, following a 504 plan such as preferential seating, and in
public places in accordance with the American with Disabilities Act, use interpreters and
captioning. They should also be referred to an ophthalmologist to evaluate for associated eye
abnormalities, and electrocardiography should be considered to evaluate (or prolonged QT
syndrome (associated with some forms of congenital SNHL). Genetics referral may also be
indicated
PREP Pearls
• Behind-the-ear hearing aids should be offered as soon as possible to infants with
sensorineural hearing loss as young as 2 months; various other devices are available for
older children.
• Early identification of hearing loss is critical to maximize communication potential

• Understand the commonly used treatment for sensorineural hearing loss in children
• Understand the indications for the use of cochlear implants in children
Suggested Readings
• American Academy of Pediatrics Early hearing detection and intervention (EHDI).
https://www.aap.org/en/patient-care/early-hearing-detection-and-intervention/
• Pediatric hearing loss. Pediatr Rev. doi: 10.1542/pir.35-11-456.
• National Institute on Deafness and Other Communication Disorders. Hearing, ear
infections. and deafness. www.nidcd.nih.gov.
• Hearing loss. In: American Academy of Pediatric Textbook of Pediatric Care. 2nd ed.
2017:1408-1412.

Question 25
A 12 years old girl is seen for a preparticipation examination attend summer camp. Her medical
history is significant for Turner syndrome associated with a mosaic 45,X/46,XX karyotype and a
bicuspid aortic valve. She has been treated with recombinant human growth hormone since 6
years of age. The girl and her mother are concerned that she has not yet developed any signs of
puberty. The girl's mother and older sister had menarche at age 13 years, and her father had
typical pubertal timing. Her height is at the 2nd percentile and weight is at the 10th percentile.
Her ears are low and posteriorly rotated, and she has a high-arched palate. Her sexual maturity
rating is 1 for breast and pubic hair development, remainder of her physical examination findings
are unremarkable.

A. obtain gonadotropin levels
B. order pelvic ultrasonograph
C. provide reassurance that her development is normal
D. start a combined oral contraceptive pill

Correct Answer: A
The girl described in the vignette has Turner syndrome and no signs of puberty at age 12 years.
Although she does not yet meet the well-established definition of delayed puberty (lack of breast
development by age 13 years in girls), evaluation at this time is indicated, as most girls with
Turner syndrome develop primary ovarian failure (hypergonadotropic hypogonadism). Although
the risk of developing primary ovarian failure in girls with a mosaic karyotype is lower than in
those with a 45, X karyotype, the risk Is still high.
The best next step in this girl’s management is to obtain gonadotropin levels to evaluate for
hypergonadotropic hypogonadism. Elevated gonadotropin levels
(Luteinizing hormone (LH] and follicle-stimulating hormone [FSH)) would confirm
hypergonadotropic hypogonadism, and pubertal induction with low-dose estradiol would be
indicated. Guidelines recommend starting estradiol for pubertal induction at age 11 to 12 years in
girls with Turner syndrome after primary ovarian failure is confirmed. Therefore, obtaining
gonadotropin levels now would allow for pubertal induction at the recommended age if the levels
are elevated.
Guidelines recommend starting low and gradually increasing the estradiol dose over 2 to 3 years
to an adult replacement dose in order to mimic normal puberty. The transdermal route of
estradiol administration is preferred, as it is the most physiologic. Progesterone should be added
once breakthrough breeding occurs or after 2 years of estradiol.
Ordering pelvic ultrasonography is not the preferred response for the girl in the vignette, as this
would not confirm hypergonadotropic hypogonadism. Providing reassurance that her
development is normal is not the preferred response because of her high risk of primary ovarian
failure. Starting a combined Oral contraceptive pills is not the preferred response, as the estrogen
dose would be too high for pubertal induction, and hypergonadotropic hypogonadism should be
confirmed prior to starting any therapy.
Features of Turner syndrome displayed by the girl in the vignette include her bicuspid aortic
valve, short stature, low-set and posteriorly rotated ears, and high-arched palate. Bicuspid aortic
valve is the most common cardiac defect associated with Turner syndrome. Coarctation of the
aorta, partial anomalous pulmonary venous return, and aortic dilation or aneurysm can also
occur. Short stature is the most manifestation of Turner syndrome and may be the only
presenting feature. Turner syndrome should be considered in all girls with short stature
Recombinant human growth hormone is approved by the United States Food and Drug
Administration for the treatment of short stature associated with Turner syndrome.
Prenatally, a cystic hygroma may be present in girls with Turner syndrome.

Neonates may have a small birth size and lymphedema of the hands and feet. Other clinical
features Include recurrent acute otitis media, ptosis, epicanthal folds, neck webbing, low
posterior hairline, broad chest with wide-spaced nipples; multiple nevi, renal anomalies (e.g..
horseshoe kidney); and skeletal anomalies (scoliosis. shortened 4th metacarpals. high upper-to-
lower segment ratio). Intelligence is usually normal, but difficulties in math and visual-spatial
skills are common. Girls with Turner syndrome are at increased risk for developing autoimmune

thyroid disease and celiac disease. Current guidelines outline screening recommendations at
diagnosis and throughout life for those with Turner syndrome.
PREP Pearls
• Most girls with Turner syndrome develop primary ovarian failure (hypergonadotropic
hypogonadism).
• Clinical guidelines recommend starting low-dose transdermal estradiol for pubertal
Induction at age 11 to 12 years in girls with Turner syndrome after primary ovarian
failure is confirmed.
• Turner syndrome should be considered in all girls with short statute.
MOCA-Peds Objective
• Recognize and evaluate an adolescent with abnormal pubertal development

• Plan the appropriate clinical evaluation of a patient with primary gonadal dysgenesis
(Turner syndrome)
• Plan the appropriate diagnostic evaluation of a patient with primary gonadal dysgenesis
(Turner syndrome)
• Recognize the clinical features associated with primary gonadal dysgenesis (Turner
syndrome) In patients of various ages
Suggested Readings
• International Turner Syndrome Consensus Group. Clinical practice guidelines for the
care of girls and women with Turner syndrome: Eur J Endocrinol doi: 10.1530/EJE-17-
0430.
• Turner syndrome and Noonan syndrome. American Academy of Pediatrics Textbook of
Pediatric Care. 2nd ed :2732-2744..
• Turner syndrome. Pediatr Rev. doi: 10.1542/pir.29-7-219.
• Turner syndrome. Pediatr Rev. doi: 10.1542/pir.34-9-420.

Question 26
A 4-month-oid male infant born at 30 weeks’ gestation is admitted to the hospital in November
with respiratory distress. He has a 4-day history of copious rhinorrhea associated with cough and
a 2-day history of fever. He has reduced appetite. His urine output has been adequate. On
admission, he has a temperature of 40 °C, respiratory rate of 65 breaths/min, oxygen saturation
of 92% on room air, heart rate of 140 beats/min, and blood pressure of 92/60 mm Hg. His
physical examination findings are remarkable for subcostal retractions and bilateral rhonchi with
expiratory wheezing on lung auscultation. Laboratory data are shown:

White blood cell 21,000/µL (2.1 x 109/L)
Lymphocytes 70%
Neutrophils 30%
Hemoglobin 13 g/dL (130 g/L)
Platelet count 200 x 103/µL (200 x 109/L)
C-reactive protein 20 mg/dL (200 mg/L)
Blood culture results are pending. A chest radiograph shows bilateral hyperinflation, peri
bronchial thickening, and patchy opacification.
Of the following, the BEST next step in the management of this infant is to Initiate
A. ampicillin, intravenously
B. azithromycin, Orally
C. supportive care
D. vancomycin, intravenously

Correct Answer: C
The infant in the vignette has clinical viral bronchiolitis. Respiratory Syncytial virus (RSV) is the
most common cause of lower respiratory tract Infection in infants and young children. The 2014
clinical practice guidelines from the American Academy of Pediatrics (AAP) recommend
supportive care for viral bronchiolitis, including hydration and oxygen supplementation as
needed. Ampicillin to treat Streptococcus pneumoniae infection, vancomycin to treat
Staphylococcus/Methicillin resistant S aurous infection, or azithromycin to treat Mycoplasma
pneumoniae infection would be Inappropriate for this clinical scenario.
The current AAP guidelines do not recommend routine chest radiography for children with
bronchiolitis because the findings do not aid in management decisions and may increase the risk
of inappropriate antibiotic use. Chest radiography findings in RSV infection include
hyperinflation with hyperlucency of the lungs, flattened diaphragm, and peri bronchial
thickenings patchy areas of atelectasis may be present, more frequently in the right middle and
upper lobes. Areas of atelectasis may be large and misinterpreted as consolidation. Multiplex
polymerase chain reaction (PCR) testing can provide a definitive viral diagnosis for children
with symptoms of bronchiolitis; a specific RSV diagnosis provides objective data that helps to
limit unnecessary antibiotic use.
Antimicrobial resistance is one of the most important public health crises worldwide. The use of
antimicrobial agents is the most important single factor leading to tie development of resistance
in the United States, according to the Centers for Disease Control and Prevention (CDC)
estimate, more than 2 million people are infected with antimicrobial-resistant bacteria annually,
and at least 23,000 people die as a direct result of these infections. A concerning rise in antibiotic
resistance has been documented in the pediatric population for the following organisms:
Staphylococcus aureus, Enterococcus faecium, extended-spectrum β-lactamase (ESBL)-
producing Enterobacteriaceae, and carbapenem- resistant Enterobacteriaceae. In one study
(Barlam TF et al), a pooled antibiogram from 200 pediatric institutions revealed 50% methicillin-
resistant S aureus strains and 21% clindamycin resistance. Among Enterococcus faecium,
ampicillin resistance was 75%, vancomycin 55%, and linezolid 8%.
Antimicrobial resistance can be addressed through a multifaceted approach. The CDC has
provided core elements for antibiotic stewardship efforts in both inpatient settings and in the
pediatrician's office (Item C26). Among the specific interventions recommended are
implementation of prospective audit and feedback, prior approval processes, and utilization of
clinical guidelines. Reduction of unnecessary antibiotic use in suspected or confirmed viral
infections is a critical component of this effort
PREP Pearls
• Chest radiography is not recommended in cases of bronchiolitis. The typical findings of
hyperinflation, peribronchial thickening, and patchy opacification likely reflect atelectasis
and viral pneumonitis, which are not indications for antibiotic use.
• Unnecessary antibiotic use is the single most important factor responsible for antibiotic
resistance in the community.
• Antimicrobial stewardship efforts include interventions such as prospective audit and
feedback, prior approval, and implementation of clinical guidelines.

• Recognize the effects of excessive antibiotic usage on the development of antibiotic
resistance in the community
• Understand the diseases for which antibiotic therapy is inappropriate with regard to the
development of antimicrobial resistance
Suggested Readings
• American Academy of pediatrics. Actions to prevent or slow antimicrobial resistance. In:
Red Book: 2021-2024 Report of the Committee on Infectious Diseases. 32nd ed. 869-
870.
• Implementing an antibiotic stewardship program: guidelines by the Infectious Diseases
Society of America and the Society for Healthcare Epidemiology of America.
doi:10.1093/cid/ciw118
• Interventions to improve antibiotic Prescribing practices for hospital inpatients. Cochrane
Database doi:10.1002/14651858.CD003543.pub4 .
• Clinical practice guideline: The diagnosis, management, and prevention of bronchiolitis.
Pediatrics doi:10.1542/peds.2015-2862.

Question 27
A 2-day-old term neonate has a total serum bilirubin concentration of 7.6 mg/dl (130 µmol/L)
and conjugated serum bilirubin of 2.3 mg/dL (39.3 µmol/L). He is breastfeeding and bottle
feeding, and has had a 2% weight loss since birth. Physical examination findings are remarkable
for a head circumference of 32 cm. Ultrasonography of the abdomen shows a contracted
gallbladder with no with no evidence of biliary atresia.
Of the following, the MOST likely cause of this neonate’s findings is

A. breast milk jaundice
B. cytomegalovirus Infection
C. group B Streptococus infection
D. methylmalonic acidemia

Correct Answer: B
The neonate in the vignette has conjugated hyperbilirubinemia and microcephaly, the most likely
cause of these findings is congenital cytomegalovirus (CMV) infection.
Conjugated hyperbilirubinemia is defined by a conjugated serum bilirubin greater than 20% Of

the total serum bilirubin level. The differential diagnosis of conjugated hyperbilirubinemia is
broad and, in addition to CMV infection, includes hypothyroidism, hepatitis, Escherichia coli
infection, Alagille syndrome, billary atresia, galactosemia, choledochal cyst tyrosinemia, and α1-
antitrypsin deficiency. Because biliary atresia should be treated with the Kasai procedure within
the first 8 weeks after birth, the initial evaluation of conjugated hyperbilirubinemia must involve
abdominal ultrasonography to confirm the presence of bile ducts.
Breast milk jaundice is not associated with an elevated conjugated bilirubin level. Infection with
group B Streptococus does not typically present with elevated conjugated bilirubin level.
Although inherited diseases of metabolism are associated with an elevated conjugated bilirubin
level, methylmalonic acidemia is not among those.
Symptomatic infection with CMV affects 0.4% of neonates in the United States annually,
Neonates with symptomatic congenital CMV exhibit the clinical findings listed in Item C27.
Cytomegalovirus is the leading cause of sensorineural hearing loss (SNHL) among children in
the United States. Of note, half of children with hearing loss related to congenital CMV infection
pass their neonatal hearing screening and exhibit hearing loss in infancy or early childhood.
Fifty percent of women of childbearing age in the United States have a history of past infection
with CMV. Among seronegative women. 2% will develop primary CMV infection during their
pregnancy. The risk or perinatal transmission is 30% to 40% among those with primary Infection
compared with 1% to 2% for those with previous CMV infection. Maternal CMV Infection
during the first and second trimester is associated with a higher risk for neonatal complications.
Overall, 85% to 90% of neonates born to mothers positive for CMV will be asymptomatic.
Asymptomatic CMV infection without SNHL is not associated with long-term sequelae.
Children with symptomatic CMV Infection should be treated with Oral valganciclovir for a total
of 6 months to decrease the risk of SNHL. Infants who are critically ill or do not tolerate the oral
formulation may require Intravenous ganciclovir. Because Of the risk for neutropenia, absolute
neutrophil counts should be monitored weekly for the first 6 weeks, at 8 weeks, and then
monthly until completion of treatment.

Item C27
PREP Pearls
• Cytomegalovirus is the leading cause of sensorineural hearing loss among children in the
United States.
• Symptomatic cytomegalovirus Infection should be treated with oral ganciclovir for a total
of 6 months to decrease the risk of sensorineural hearing loss
• The risk of perinatal transmission of cytomegalovirus is 30% to 40% among women with
primary infection compared with 1% to 2% transmission for those with previous
infection.

• Recognize disorders associated with conjugated hyperbilirubinemia in neonates
• Plan the appropriate diagnostic evaluation of conjugated hyperbilirubinemia in a neonate
• Recognize metabolic diseases that can produce conjugated hyperbilirubinemia in
neonates
Suggested Readings
• Cytomegalovirus infection. Red Book: 2021-2024 Report of the Committee on infectious
Diseases. 32nd ed; 294-300.
• Cytomegalovirus. Nelson Textbook of Pediatrics 21st ed. Chap 282:1718-1723
• The newborn at risk for infection. American Textbook of Pediatric care 2nd ed, 899-909.
• Cytomegalovirus and Epstein Barr virus infections. Pediatr Rev. doi:10.1542/pir.2015-
0072

Question 28
A local middle school is looking to implement a dedicated strength training program for athletes
to reduce injuries and improve athletic performance. The school physician is asked to provide
guidance regarding optimizing injury prevention with this type of training program.
Of the following, the BEST recommendation is that this program should

A. avoid high intensity or heavy weight training
B. delay strength training programs until puberty
C. use exercises that focus on a single muscle group
D. use free weights rather than weight machines

Correct Answer D
Strength training programs can reduce the risk of sports-related acute and overuse injury by 66%,
and are effective at helping young athletes build strength and enhance athletic performance. Free
weights (e.g., dumbbells, kettlebells, and medicine balls) can be used by athletes of all sizes and
allow for individualization of movement patterns, progression of motor control, and strength, and
are preferred over weight machines for resistant training in young athletes. Weight machines are
generally sized for adults, cannot accommodate the smaller frames of many middle school
athletes, and do not confer the same injury prevention and performance benefits as free weights.
“Strength trainings” and “resistance training” are synonymous terms used to describe exercise
programs that are designed to build muscle strength and power. "Weight training” describes
programs that specifically use external weights to build strength, but strength can also be
enhanced by exercises that use elastic bands, body weight or other forms of resistance.
Resistance training programs in prepubertal children generally result in greater relative gains in
strength and motor performance compared with adolescents or adults. These changes in younger
children primarily result from enhanced neural efficiency and muscle recruitment, even without
the muscle hypertrophy associated with strength gains in postpubescent children. Children who
are ready for organized sports participation (often at 5 to 7 years of age) are generally able to
benefit from an appropriate strength training program. Young children often start by using body
weight for resistance activities that can be incorporated into play or games. Examples includes
jumps, single leg hops, wheelbarrow walking, bear crawls, and crab walks. Further ideas for
strength building in young children can be found at: https://activeforlife.com/activities/.
Previously, the open growth plates in skeletally immature athletes were presumed to be uniquely
susceptible to damage from heavy resistance training, however, these concerns appear to be
largely unfounded, and current evidence indicates that skeletally immature athletes can
participate in a high-Intensity strength-training program within the following parameters:
• Supervision by a knowledgeable adult
• Graduated progression from lighter to heavier loads and from simpler to more complex
movement patterns
• Emphasis on appropriate technique
• Adequate rest and recovery between sessions
Appropriate strength training programs seem to primarily reduce injury risk in young athletes by
facilitating development of proper movement patterns during sports. Exercises that focus on
integrating movements of multiple joints and muscle groups (e.g., squats, snatches) are more
effective at enhancing performance and reducing sports-related injury than are exercises that
focus on a single muscle group (e.g., bicep curls, leg extensions).
Practical guidance for the development of resistance training programs includes the following:
• Optimal benefits occur with 2 to 3 training sessions per week on nonconsecutive days
• Resistance training should begin at least 2 months before the start of the sports season
• Initial training should be with low resistance and 1 to 2 sets of 8 to 12 repetitions per
exercise to facilitate development of proper technique
• Resistance can be increased in increments of 5% to 10%
• Recommendations for further progression into higher intensity training can be found in
the American Academy of Pediatrics clinical report "Resistance Training for Children
and Adolescents.” https://pediatrics.aappublications.org/content/145/6/e20201011
PREP Pearls
• Resistance training programs reduce injury risk and enhance athletic performance in
young athletes.
• Prepubertal children build strength through enhanced neuromuscular function rather than
muscle hypertrophy.
• Children and adolescents can safety participate in high-intensity weight training programs
with appropriate supervision.

• Recognize the preventable causes of trauma in juvenile athletes and the physiology
associated with increased trauma risk
Suggested Readings
• Effects, and dose-response relationships of resistance training on physical performance in
youth athletes: a systemic review and metaanalysis. Br J Sports Med.
Doi:10.1136/bjsports-2015.095497.
• Resistance training for children and adolescents. Pediatrics. Doi:10.1542/peds.2020-1011
• Resistance training in youth Laying the foundation for injury prevention and physical
literacy. Sports Health. Doi:10.1177/1941738117704153

Question 29
A 10-year-old boy is brought to the office for evaluation of dizziness with activity and
generalized fatigue. Typically, he enjoys being active in gym class and playing on a selected
soccer team. Several weeks ago, he won the most valuable player award at a high-level soccer
tournament in New England, but he has not had the energy to participate for the past 2 weeks, He
is drinking well but eating less. He reports no cough, rhinorrhea, nausea, vomiting, or diarrhea.
On physical examination, he is afebrile and has a heart rate of 50 beats/min, respiratory rate of
20 breaths/min, blood pressure of 90/50 mm Hg, and oxygen saturation by pulse oximetry of
99% on room air. The remainder of his examination findings are normal. Electrocardiograph was
performed Item Q29, after which he is admitted to the hospital telemetry unit
Of the following, the BEST next step in management is to administer
A. amoxicillin-clavulanic acid, orally

B. ceftriaxone, intravenously
C. doxycycline, Orally
D. gentamicin, intravenously

Correct Answer: B
The child in the vignette has a new, symptomatic complete heart block. Additionally, he reports
recent travel to New England. While not diagnostic, this raises suspicion for Lyme carditis. He
should be admitted to the hospital for cardiac monitoring, given his symptomatic cardiac
condition. While undergoing evaluation for the cause of his heart block the boy should be started
on intravenous (IV) therapy for possible Lyme disease. While there are several appropriate
antibiotic choices to treat Lyme disease, including oral doxycycline and amoxicillin-clavulanic
acid, Red Book® recommends IV ceftriaxone for those admitted to the hospital. Gentamicin is
not an appropriate treatment for Lyme disease.
Pediatric dysrhythmias are categorized by their rate (too fast or too slow) and by their cardiac
chamber of origin (supraventricular or ventricular). The child in the vignette has complete heart
block; there is no electrical connection between the P waves and the ORS complexes, and his
rhythm is slow, a bradyarrhythmia. Other bradyarrhythmias include sick sinus syndrome or slow
junctional rhythm. Treatment is dictated by symptoms, which indicate the impact of the
dysrhythmia on cardiac output. Symptoms vary from asymptomatic to those warranting
pacemaker placement or temporary treatment with inotropic agent Infusions to augment
chronotropy.
Tachyarrhythmias are more commonly seen in children. Narrow complex tachyarrhythmias

originate from above the ventricle (e.g., supraventricular tachycardia [SVT]) and wide complex
from within the ventricle (e.g., ventricular tachycardia). They are additionally categorized as
having a regular vs irregular rhythm, determined by comparison of the length of the R-R
intervals.
The term “supraventricular tachycardia” is commonly used as a diagnosis although it is actually

a category of tachyarrhythmias. Dysrhythmias that fall into the category of SVT include
atrioventricular reciprocating tachycardia (AVRT), atrioventricular node reentrant tachycardia
(AVNRT), atrial flutter (Item C29A), atrial fibrillation, atrial tachycardia, and junctional
tachycardia. Atrioventricular reciprocating tachycardia and AVNRT are each commonly referred
to simply as SVT (Item C29B). Ventricular arrhythmias include ventricular tachycardia,
Torsade's de pointes (Item C29C), and ventricular fibrillation
The treatment of tachycardias is also dictated by the degree of hemodynamic instability. They
are treated with medications that slow down the heart rate or specific parts of the conduction
system in order to either restore sinus rhythm or at least slow down or “rate-control” the
abnormal rhythm. These medications include, but are not limited to ß-blockers, calcium channel
blockers, and amiodarone. Adenosine is frequently used to treat the forms commonly known as
SVT (AVRT& AVNRT). This medication results in a pause at the AV node, which breaks the
circuit of the abnormal rhythm and allows the conduction system to “reset” into sinus rhythm. In
cases of AVRT or AVNRT where cardiac output is significantly compromised, synchronized
cardioversion or defibrillation is necessary.

Item C29A: atrial flutter
Item C29B: SVT
Item C29C: torsade's de pointes
PREP Pearls
• Lyme carditis can impact the conduction system and may cause complete heart block.
• Bradyarrhythmias include head block, sick sinus syndrome, and slow junctional rhythm.
• Tachyarrhythmias include supraventricular tachycardias (e.g., atrioventricular
reciprocating tachycardia, atrioventricular node reentrant tachycardia, atrial flutter, atrial
fibrillation, atrial tachycardia, and Junctional ectopic tachycardia).
MOCA-Peds Objective
• Recognize the clinical manifestations or cardiac dysrhythmias.

• Plan the appropriate management of various cardiac dysrhythmias
• Recognize the clinical findings associated with various cardiac dysrhythmias
• Recognize the electrocardiographic characteristics of various cardiac dysrhythmias
Suggested Readings
• Red Book: 2021-2024 Report of the Committee on infectious Diseases. 32nd ed;482-489.
• Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric
population: Europace doi: 10.1093/europace/eut082
• Cardiac arrhythmias. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
1227-1235
• Heart rate and rhythm disorders. Pediatr Rev. 2017. doi: 10.1542/pir.2016-0119.

Question 30
A 4-year-old boy is brought for a health supervision visit. He has had a lifelong persistent cough
and intermittent wheezing. He becomes short of breath and has increased cough when running,
and cannot keep up with his peers while playing. He had surgical repair of a complete vascular
ring at 2 years of age. There is a strong family history of asthma.
On physical examination, he looks well, Oxygen saturation on pulse oximetry is 99% on room
air. Respiratory rate is 20 breaths/min. Heart sounds are normal, and breath sounds are clear to
tidal breathing. He has monophonic expiratory wheezing that is heard best centrally and
transmitted throughout the chest. There is no inspiratory stridor. There is no change after 4 puffs
of an albuterol inhaler with a holding chamber and mask. The boy is diagnosed with
tracheomalacia
Of the following, the boy's findings MOST consistent with this diagnosis include
A. exercise intolerance, family history of asthma, no response to albuterol
B. family history of asthma, history of vascular ring, normal oxygen saturation
C. history of vascular ring, central monophonic wheezing, exercise intolerance
D. No stridor, normal heart sounds, normal oxygen saturation

Correct Answer: C
The boy in the vignette has tracheomalacia after the repair of a complete vascular ring. The
history of a congenital anomaly producing compression of the trachea with either failure to
develop ring cartilages or injury to them, plus the monophonic wheezing and exercise intolerance
are characteristic findings or tracheomalacia. Although he has a family history of asthma and
may be at risk for the development of asthma in the future, the monophonic wheezing is
characteristic of a single point obstruction of the airway, rather than the polyphonic wheezing
with multiple sites of small airway obstruction heard in asthma. The failure to respond to
albuterol also suggests that his airway obstruction is not that of the small, muscular airways.
Because the problem is one of variable central airway obstruction, there is no small airway
component and ventilation/perfusion mismatch, resulting in no effect on oxygen saturation.
Cough that worsens with exercise and is severe enough to produce breathlessness with exercise
results from the poorly supported trachea collapsing on itself with increased work of breathing.
The cough may be more prevalent than wheezing, but both are characteristic of tracheomalacia
The presence of monophonic wheezing and lack of stridor localizes the point of obstruction for
the boy in the vignette to the intrathoracic airway. Extra thoracic airway obstruction, as is
characteristic of fixed or dynamic laryngeal anomalies (e.g., laryngeal web, laryngomalacia), is
associated with airway narrowing on inspiration and stridor during the inspiratory phase of
respiration. Intrathoracic obstruction of the central or peripheral airways is associated with
increased airflow obstruction on expiration and wheezing. This relationship is shown in Item
C30.
Item C30: Airway pressures during respiratory cycle. During inspiration, expansion of
the thorax creates negative pressure within the thoracic cavity and airways (black
arrows), allowing inflow of air (blue arrow). Thus, a weakness in the extrathoracic
airway (red arrow) is likely to be symptomatic during inspiration. During expiration,
compression of the thorax creates positive pressures within the thoracic cavity and
airways (black arrows), allowing outflow of air (blue arrows). Thus, a weakness in an
intrathoracic airway (red arrow) is likely to be symptomatic during expiration
Tracheomalacia and bronchomalacia are often misdiagnosed and treated as asthma because of
the finding of expiratory wheezing. Failure of first-line asthma therapy such as bronchodilators
should suggest the possibility of an alternative diagnosis. Hilar adenopathy, tracheal or
esophageal duplication cyst, or other source of extrinsic tracheal compression may cause a single
point of obstruction resulting in monophonic wheezing. Intrinsic tracheal lesions such as a
tracheal hemangioma or polyp would also be characterized by central expiratory monophonic
wheezing. Tracheoesophageal fistula is associated with persistent tracheomalacia after repair of
the primary anomaly.
It is important for the examining physician to conduct a careful physical examination noting the
site of adventitious sounds, the relationship to the respiratory cycle and the presence of a single
or variable/multiple tones. If there is a question of polyphonic wheezing or other suggestion of
asthma, a brief therapeutic trial of inhaled bronchodilator may be warranted if the child is
otherwise stable and not in distress. Although the current recommendation is to limit exposure to
radiation, a view radiograph of the chest would be the next most appropriate intervention. If a
hilar or mediastinal mass or compressing structure can be ruled out, then explanation and
observation may be the best approach. Contrasted computed tomography of the chest and/or
bronchoscopy may be required to rule out extrinsic compression of the trachea and to feel
comfortable that tracheomalacia is the cause of the wheezing. In most cases, a careful history and
physical examination, coupled with a normal chest radiograph and tack of response to albuterol,
may be all that is required to make the diagnosis of tracheomalacia and spare the child multiple
medical interventions
PREP Pearl
• Intrathoracic airway obstruction is the source for expiratory wheezing; extra thoracic
airway obstruction is the source for inspiratory stridor.
• Monophonic wheezing is characteristic of a single point of obstruction in the airway.
• Polyphonic or musical wheezing is characteristic of multiple points of variable
obstruction.
• Tracheomalacia may be an isolated congenital anomaly or may be secondary to an injury
to the ring cartilages of the trachea.

• Plan appropriate management for wheezing of various etiologies
• Plan the appropriate clinical and diagnostic evaluation of wheezing of various etiologies
Suggested Readings
• Airway obstruction. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed.2777-2786
• Differential diagnosis of wheezing in children. Pediatr Rev. doi:10.1542/pir.1-8-239
• Age related changes in childhood wheezing characteristics a whole population study.
Pediatr Pulmonol doi:10.1002/ppul.23783
• Laryngomalacia and tracheomalacia: Common dynamic airway lesions. Pediatr Rev. doi:
10.1542/pir.27-4-e33
• Pseudo-asthma: when cough, wheezing. and dyspnea are not asthma. Pediatrics: doi:
10.1542/peds.2007-0078

Question 31
A 16 years old adolescent boy is seen at the school-based health center and requests to be tested
for sexually transmitted infections (STIs). He and his male partner of 12 months, his first and
only sexual partner, asymptomatic and have an exclusive relationship. Neither of them has even
tested or treated for an STI. He participates in receptive anal sex and insertive oral sex. Condoms
are used during anal sex. After sex, he and his partner often vape marijuana. He does not drink
alcohol or use other substances. A review of systems and physical examination findings are
unremarkable. He agrees to HIV testing.
Of the following, the MOST appropriate additional screening for this adolescent should include a
A. rectal swab for gonorrhea, chlamydia, and trichomoniasis
B. serology test for herpes simplex Vitus
C. serology test for syphilis
D. urine sample for gonorrhea, chlamydia, and Mycoplasma genitalium

Correct Answer: C
The adolescent in the vignette should be screened for syphilis. The Centers for Disease Control
and Prevention (CDC) and the American Academy of Pediatrics (AAP) recommend that
adolescent men having sex with men (MSM) be screened for syphilis, chlamydia, and gonorrhea
at least annually. More frequent screening, as often as every 3 to 6 months, may be considered
for those at high risk.
Routine screening serology for herpes simplex virus (HSV) is not recommended. The CDC
recommends screening adolescent MSM for gonorrhea and chlamydia at anatomic sites
consistent with the individual’s sexual practices. Nucleic acid amplification tests (NAATs) of
urine and rectal swab are appropriate for chlamydia and gonorrhea. Patient self-collected rectal
swabs are acceptable. Pharyngeal swabs be tested with US Food and Drug administration (FDA)-
approved NAATs for gonorrhea. Screening for chlamydia of pharynx is not recommended;
however, the FDA has recently approved NAATs for pharyngeal chlamydia. Routine screening
of adolescent MSM for trichomoniasis and Mycoplasma genitalium is not recommended.
Decisions regarding the routine screening of asymptomatic adolescents for sexually transmitted
infections (STIs) are based on sexual behaviors/practices, risk factors, and prevalence rates for
populations or settings. Risk factors for the acquisition of STIs include the patient's and/or their
current and past partners' history of:
• Multiple/new/anonymous partners
• STIs
• Living with H V
• Anal sex
• Injection drug use
• Commercial sex, sex for drugs
• Incarceration
• Membership in a high-prevalence population such as MSM
• Health care in settings with high-prevalence rates such as correctional facilities, national
job training programs. STI clinics, and high school-based clinics.
Health care providers should be mindful that risk factors and sexual Practices can change over
time. The AAP’s Bright Futures guidance on STI screening is in Item C31.
Item C31: Sexually Transmitted Infections Screening

Prenatal Screening
Screening pregnant women for hepatitis B, HIV, syphilis, chlamydia, and gonorrhea can have direct health benefits
later for the child.
The USPSTF recommends that all pregnant women be screened for hepatitis B (Grade A), HIV (Grade A), and
syphilis (Grade A). Each of these infections requires urgent treatment of the newborn.
The USPSTF recommendation for chlamydia and gonorrhea screening of pregnant women is the same as
for nonpregnant women. The USPSTF recommends that females younger than 25 years and those engaging in high-
risk sexual behaviors be screened for chlamydia (Grade B) and gonorrhea (Grade B). Although the USPSTF does
not recommend routine screening for chlamydia in pregnant women who are older than 25 and not at increased
risk, it notes that individual circumstances may support screening.
The USPSTF has concluded that current evidence is insufficient to recommend for or against screening for
gonorrhea in pregnant women who are not at increased risk (I Statement).19
Screening Adolescents for Chlamydia trachomatis

Chlamydia is the most common STI in the United States, and many of those infected are asymptomatic.
In females, untreated chlamydial infection can lead to infertility. Furthermore, infants may develop serious illness if
chlamydial infection is acquired through vertical transmission. In adolescent and adult males, chlamydia rarely
leads to significant illness. Of course, infected adolescent and adult males can be important vectors for
transmission.
The USPSTF recommends screening for chlamydial infection in all sexually active, nonpregnant females
24 years and younger (Grade B).
The USPSTF has concluded that current evidence is insufficient to recommend for or against screening for
chlamydial infection in men (I Statement).19
The Periodicity Schedule calls for screening of adolescents for STIs according to the recommendations in
the current edition of the AAP Red Book: Report of the Committee on Infectious Diseases
Screening Adolescents for Neisseria gonorrhea

The USPSTF recommends screening for gonorrheal infection in all sexually active, nonpregnant females
24 years and younger (Grade B).
The USPSTF has concluded that current evidence is insufficient to recommend for or against screening
for gonorrheal infection in men (I Statement).19 Asymptomatic infection is less common in males than
in females.
Males who have sex with males or who have other STIs are at increased risk.
Screening Adolescents for Syphilis

The USPSTF strongly recommends that clinicians screen persons at increased risk for syphilis infection
(Grade A).
The USPSTF does not recommend routine screening of asymptomatic persons who are not at increased
risk for syphilis infection (Grade D)
Additional STI screening recommendations from the Centers for Disease Control and Prevention
include the following (Screening Recommendations Referenced in the 2015 STD Treatment
Guidelines and Original Recommendation Sources).
Human immunodeficiency virus: The CDC recommends universal screening at least once for all
individuals 13 to 64 years of age. The AAP recommends that all adolescents be offered HIV
screening at least once by age 16 to 18 years. HIV testing for all sexually active adolescents is
encouraged. Decisions for repeat HIV screening are driven by assessment of the risk for
acquisition.
Papanicolaou test: The American College of Obstetrics and Gynecology (ACOG) and the US
Preventive Services Task Force recommend initiating cervical cancer screening with a
Papanicolaou (Pap) smear at age 21 years with repeat testing every 3 years between ages 21 to
29 addition, ACOG allows an alternative option of primary human papilloma virus (HPV) testing
every 3 years for those aged 25 years and older. The American Cancer Society released
guidelines in 2020 for routine cervical cancer screening beginning at age 25 years, with repeat
testing every 5 years if screening incorporates HPV testing, or every 3 years with a Pap smear
atone. These recommendations are applicable to all individuals with a cervix.
For adolescent women having sex with men (WSM) and adolescent men having sex with women
(MSW), the following is recommended
Syphilis: There are no syphilis screening recommendations for MSW or WSM. However, health
care providers may decide to screen based on risk factors. Pregnant adolescents should be
screened.
Chlamydia and gonorrhea: Adolescent WSM should be screened at least annually with NAATs
from a vaginal swab (preferred) or urine sample (acceptable and convenient). Provider or patient
self-collected vaginal swabs are acceptable, Screening should be repeated sooner if there is a
new sexual partner and 3 months after treatment for chlamydial infection. It may be prudent to
screen MSW as well as WSM for chlamydia and gonorrhea, especially in high-prevalence
settings (e.g., school-based clinics) or in the presence of risk factors (e.g., multiple partners).
Herpes simplex virus, trichomoniasis, and Mycoplasma genitalium. Routine screening is not
recommended for MSW or WSM.
For adolescent women having sex with women (WSW): Data on the transmission of STIs among
WSW are limited. Screening should be based on a review of sexual practices. Oro genital sex
may be a risk factor for HSV. Digital Sex and use of penetrative sex items may be risk factors for
trichomoniasis.
For transgender adolescents: Transgender individuals should be offered STI screening that
reflects their sexual practices, behaviors, current genital anatomy, and population prevalence,
Repeat HIV testing should be considered. The prevalence of HIV approaches 30% for
transgender women.
Recommended STI prophylaxis after sexual assault includes the following

• Empiric treatment for chlamydia, gonorrhea, and trichomoniasis
• Hepatitis B vaccination for those unvaccinated, incompletely vaccinated, or vaccinated
without adequate immune response
• Human papillomavirus vaccination for those not previously or incompletely vaccinated
• Assessment of HIV risk and provides indicated, HIV postexposure prophylaxis within 72
hours of the assault
• Emergency contraception should be offered to all women

PREP Pearls
• Adolescent men having sex with men should be screened at least annually for HIV,
syphilis, chlamydia, and gonorrhea.
• Adolescent women having sex with men should be screened at least annually for
chlamydia and gonorrhea.
• Routine screening for chlamydia and gonorrhea is not recommended for men having sex
with women; screening may be offered to those with risk factors for infection.
• Routine cervical cancer screening should be offered to all individuals with a cervix
beginning at age 21 years (or at age 25 years as recommended by the American Cancer
Society).

• Plan an appropriate screening evaluation for sexually transmitted infections in various
adolescent populations
• Plan appropriate prophylaxis following possible exposure to sexually transmitted
Infection
• Understand the indications for a Papanicolaou test in female adolescents
Suggested Readings
• Centers for Disease Control and Prevention. Screening recommendations considerations
and referenced In the 2015 std treatment guidelines and original sources. www.cdc.gov
• AAP Committee on Chu Abuse and Neglect, Care of the adolescent after an acute sexual
assault Pediatrics. doi:10.1542/peds.2017-0958.
• Emerging issues in male adolescent sexual and reproductive health care. Pediatrics.
doi:10.1542/peds.2020-0627.
• Sexually transmitted infections. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed.2628-2651
• Office-based screening for sexually transmitted Infections in adolescents.
doi:10.1542/peds.2019-2056K.

Question 32
A 14 years old adolescent girl is brought to the emergency department 30 minutes after she
collapsed while walking with a friend. She is awake and able to follow commands but is not
speaking. Her pupils are equal, round, and reactive to light; extraocular movements are intact,
and she can close her eyes completely. Her smile is asymmetric. She is moving her left arm and
leg vigorously; there is minimal movement in the right arm and leg. She is unable to ambulate. A
head computed tomography scan is normal. The girl is admitted to the pediatric intensive care
unit for observation with a diagnosis of seizure with a post-ictal paralysis
Over the next 12 hours, the girl's hemiparesis remains unchanged. Her friend reports that the girl
collapsed while walking; she did not lose consciousness, had no abnormal movements, and was
unable to get up or speak.
Of the following, the BEST next diagnostic study to order in the management of this child is
A. brain magnetic resonance imaging
B. toxicology screening
C. transthoracic echocardiography
D. video electroencephalographic monitoring

Correct Answer: A
The adolescent in the vignette has an acute-onset focal neurological deficit. While the differential
diagnosis in this situation is extensive, the most time-sensitive diagnosis is acute ischemic stroke
(Item C32A). Stroke is an under recognized condition in children, occurring in 1 to 2/100,000
children. This condition is often not considered in the initial differential diagnosis of a child
acute focal neurological deficit, leading to delay in diagnosis and management. Consideration of
the diagnosis of stroke at the time of presentation is critical for prompt evaluation which includes
obtaining emergency neuro-imaging. The preferred study is resonance imaging (MRI) of the
brain and magnetic resonance angiography (MRA) of the head and neck, which can show
ischemic changes within minutes of the event. A computed tomography (CT) scan of the head
can be performed when urgent MRI is unavailable; however, CT results can be normal in the
setting of acute stroke, necessitating further imaging.
Item C32A differential diagnosis of pediatric stroke
The clinical presentation of acute stroke in children is similar to that of adults and dependent on
the vascular territory affected, Common symptoms include hemiparesis, speech/language
disturbances, vision changes, or ataxia. Children are more likely than adults to have non-
localizing features, such as altered mental status, headache, or seizures. Item C32B depicts
common symptoms of childhood stroke by vascular territory.
Item C32B common symptoms of childhood stroke by vascular territory
The pathophysiology of stroke is broadly divided into 2 categories: ischemic and hemorrhagic
(Item C32C). Ischemic stroke occurs when a blood vessel is occluded, resulting in infarction of
the supplied brain tissue. Hemorrhagic stroke occurs with spontaneous hemorrhage, either
intraparenchymal or in the subarachnoid space. The diagnostic approach to ischemic stroke in
children is shown in Item C32D.

Item C32C ischemic and hemorrhagic stroke




Item C32D The diagnostic approach to ischemic stroke in children is shown in
Approximately 50% of children with ischemic stroke and 90% with hemorrhagic stroke have an
identifiable risk factor. Common risk factors in children include sickle cell disease, moyamoya,
congenital heart disease, prothrombotic conditions, or concomitant infection (in particular, of the
head and neck). Evaluation to identify risk factors after a stroke can include trans-thoracic or
trans-esophageal echocardiography with bubble study, laboratory testing for thrombophilia, or
Holter monitor study; the choice of testing is dependent on the clinical presentation.
Acute and chronic management of stroke is tailored to the underlying mechanism; this is
summarized in the American Heart Association's recently updated guidelines: (Ferriero et al).
Acute management is focused on restoration of blood flow to the penumbra, the region of brain
tissue, which has not been irreversibly injured. This occurs through collateral circulation and
institution or neuroprotective measures that maximize cerebral perfusion and oxygenation while
minimizing metabolic demands. Secondary stroke prevention is aimed at addressing modifiable
risk factors, including the use of antithrombotic or anticoagulation as appropriate to minimize
risk of recurrence.

While toxicology screening and video electroencephalographic monitoring are useful diagnostic
tests in a child with neurologic findings their performance is not as urgent as the need to rule out
an acute ischemic stroke.
PREP Pearls
• Acute stroke is an under recognized, time-sensitive diagnosis that should be considered in
any child with an acute focal neurological deficit. Clinical symptoms include
hemiparesis, facial weakness, disturbances of language and speech. ataxia, or vision
changes
• Children with an acute stroke are more likely than adults to have non localizing
symptoms and signs such as altered mental status and seizures.
• Stroke risk factors include sickle cell disease, moyamoya and other vasculitides,
congenital heart disease, prothrombotic conditions, and infection (especially of the head
and neck).

• Understand the pathophysiology of childhood stroke
• Recognize the clinical findings associated with childhood stroke
Suggested Readings
• Stroke in neonates and children. Pediatr Rev. doi: 10.1542/pir.2016-0002.
• Management of stroke in neonates and children: A scientific statement from the
American Heart Association/American Stroke Association. Stroke. doi:
10.1161/str.0000000000000183.
• International Pediatric Stroke Study. https://internationalpediatricstroke.orq/ipss-research/
• Arterial ischemic stroke in children: Risk factors and etiologies. Current neurology and
neuroscience reports. doi:10.1007/s11910-013-0422-8

Question 33
A female neonate in the newborn nursery has a heart rate of 60 beats/min. She is in no acute
distress. Her heart has an irregular rate and rhythm with 2+ brachial and femoral pulses without
delay, her lungs are clear bilaterally, and there is no cyanosis or peripheral edema. There is a
diffuse rash characterized by elliptical erythematous, papulosquamous lesions with central
clearing, annular erythema, and a fine scale (Item Q33). Electrocardiography is performed.
Of the following, the finding MOST often associated with this neonate's condition is
A. First degree atrioventricular block
B. second degree atrioventricular block
C. sinus bradycardia
D. third-degree atrioventricular block

Correct Answer: D
The newborn in this vignette has neonatal systemic lupus erythematosus (NSLE). This
autoimmune condition occurs in 1% to 2% of infants with transplacental passage of maternal
anti-Ro/SSA or anti-La/SSB antibodies due to rheumatologic conditions, such as Sjögren
disease, systemic lupus erythematosus (SLE), mixed connective tissue disease, and rheumatoid
arthritis. However, half of all infants with NSLE are born to mothers with no identified
autoimmune condition.
The most common cardiac conduction abnormality in infants with NSLE is complete congenital
heart block (CHB) or third-degree atrioventricular block. This is due to SSA and SSB antibody-
mediated injury to fetal and neonatal cardiac conduction tissue. While Infants with NSLE may
develop first-, second-, or third-degree CHB, bradycardia most commonly occurs with third
degree CHB, and less commonly with second-degree CHB. The current American heart
Association recommendations for obstetric monitoring in cases of maternal SLE include weekly
fetal echocardiography surveillance, particularly from 16 to 26 weeks’ gestation when the risk
for CHB is greatest. Postnatally, a full 12-lead electrocardiogram should be performed on infants
born to mothers with SSA and SSB antibodies to evaluate for CHB. While most infants with
complete CHB in the setting of NSLE are asymptomatic, such as the infant in the vignette, a
subset may exhibit or develop signs or symptoms such as gallops, murmurs, variable first heart
sound intensity, and congestive heart failure (CHF) due to cardiomyopathy. Asymptomatic
infants require close follow-up. Those with signs and symptoms of cardiomyopathy and CHF
(e.g., fatigue, diaphoresis, rales on lung examination, peripheral edema, hepatomegaly, etc.) may
require pacemaker implantation.
Electrocardiographic findings Associated with NSLE:

Cardiac Electrocardiographic Findings Example Electrocardiogram
abnormality
Complete or Third Bradycardia Item C33A
Degree Regularly occurring P waves that lack
correlation with ORS complex
Second degree P wave blocked from initiating a QRS Item C33B
Type I (Mobitz I or complex
Wenckebach) Progressive prolongation of PR interval
followed by blocked P wave, or
dropped QRS complex
Second degree P wave blocked from initiating a QRS Item C33C
Type II (Mobitz II) complex
Intermittently non-conducted P waves
not preceded by prolongation of PR
interval and followed by shortening of
PR interval
Sinus bradycardia Bradycardia for age Item C33D
Normal PR intervals
Every P wave accompanied by a QRS
complex

Item C33A: Complete or Third Degree
Item C33B: Second degree Type I (Mobitz I or Wenckebach)
Item C33C: Second degree Type II (Mobitz II)
Item C33D: Sinus bradycardia
The clinical manifestations of NSLE are wide ranging (Item C33E). The classic NSLE rash is
characterized by elliptical erythematous papulosquamous lesions with central clearing, annular
erythema or dyspigmentation, and a fine scale, which worsens with exposure to ultraviolet light.
The rash becomes apparent during the first 3 months after birth an commonly spontaneously
resolves after maternal antibodies disappear at 6 to 8 months of age.

Item C33E: The clinical manifestations of NSLE

PREP Pearls
• The most common cardiac conduction abnormality associated with neonatal systemic
lupus erythematosus presenting with bradycardia is complete heart block, or third-degree
atrioventricular block.
• A full 12-lead electrocardiogram should be performed on neonates born to mothers with
SSA and SSB antibodies to evaluate for congenital heart block.
• Asymptomatic neonates with congenital heart block require close follow-up, those with
signs or symptoms of cardiomyopathy and congestive heart failure may require
pacemaker implantation.

• plan the appropriate management of systemic lupus erythematosus, including recognition
of drug-related complications
• plan the appropriate diagnostic evaluation of systemic lupus erythematosus, and interpret
the results appropriately
Suggested Readings
• Pediatric dysrhythmias. Pediatr Clin North Am. doi:10.1016/j.pcl.2005.10.004.
• Neonatal lupus and related autoimmune disorders of infants. NeoReviews.
Doi:10.1542/neo.9-5-e206
• Epidemiology, etiology, detection, and treatment of autoantibody-associated congenital
heart block in neonatal lupus. Curr Rheumatol Rep. doi: 10.1007/s11926-007-0003-4
• Systemic lupus erythematosus. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed. 2586-2592
• Pediatric systemic lupus erythematosus: More than a positive antinuclear antibody.
Pediatr Rev. doi:10.1542/pir.33-2-62.

Question 34
A 6-year-old is seen for a health supervision visit. He attends the first grade in a regular
education classroom and is doing well. He has multiple café au lait macules on his body, at least
6 of which are larger than 5 mm, and axillary freckling. A 3-generation pedigree is obtained
(Item Q34)
Of the following, the inheritance pattern for this boy's condition is

A. autosomal dominant with incomplete penetrance
B. autosomal dominant with variable expressivity
C. autosomal recessive
D. X-linked dominant

Correct Answer: B
The boy described in the vignette has neurofibromatosis type 1 (NFI)
Neurofibromatosis type 1 is a neurocutaneous disorder inherited in an autosomal dominant
manner with 100% penetrance. Characteristics of an include that multiple family members in
multiple generations are affected, males and females are equally affected, and the recurrence risk
is 50% with every pregnancy. Penetrance refers to the percentage of individuals who express
clinical features of the condition and carry a disease-causing change in the associated gene.
The clinical diagnosis of NF1 is made when at least 2 of the following features are present:
• Café au lait macules (CALs) (at least 6; larger than 5 mm prepubertal, larger than 15 mm
post pubertal)
• Axillary and/or inguinal freckling
• Cutaneous neurofibromas (at least 2)
• Iris Lisch nodules (at least 2)
• Plexiform neurofibroma (at feast 1)
• Optic glioma
• Osseous lesion (sphenoid dysplasia or tibial pseudoarthrosis)
• First-degree relative with NF1 as defined by the above criteria
Neurofibromatosis type 1 demonstrates both inter- and intrafamilial variability in expression. As

demonstrated in the pedigree (Item C34), the proband's clinical features are axillary/inguinal
freckling and CALs. The remainder of the affected family members have axillary/inguinal
freckling, CALs, and various additional features, including optic glioma in his sister, left forearm
plexiform neurofibroma in his brother, neurofibromas in his father, plexiform neurofibroma of
the right ankle in his paternal grandmother, and early-onset breast cancer in his paternal aunt.
Female patients with NF1 are at an increased risk of breast cancer. The National Cancer
Comprehensive Network recommends surveillance with annual mammograms starting at age 30
years.
Autosomal recessive inheritance is characterized by an affected child of unaffected carrier

parents. Consanguinity increases the risk of an autosomal recessive disorder. Mate-to-male
transmission in a pedigree rules out an X-Inked condition as the Y chromosome would pass to
the son from his father and the X chromosome would have to come from his mother.
PREP Pearls
• An autosomal dominant pedigree is characterized by multiple affected individuals in
multiple generations, males and females being equally affected and a recurrence risk of
50% with every pregnancy.
• An autosomal recessive pedigree is characterized by an affected child born to unaffected
carrier parents.
• The presence of male-to-male disease transmission rules out an X-linked disorder.


• Recognize the inheritance pattern associated with an autosomal dominant disorder with
variable expressivity
• Recognize the inheritance pattern associated with an autosomal dominant disorder with
incomplete penetrance
• Recognize the inheritance pattern associated with autosomal dominant disorders
Suggested Readings
• Neurocutaneous syndromes. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed. 2379-2396
• Dysmorphorogy. Pediatr Rev. doi:10.1542/pir.2018-0331
• Patterns of single-gene inheritance. In: Thompson and Thompson Genetics In Medicine.
8th ed. Philadelphia, PA: Elsevier; 2016.

Question 35
A 7 years old girl recently adopted from an orphanage in China is being seen for a health
supervision visit. The mother reports no concerns. No information is known about the child's
biological parents. The mother shares a record of the child’s immunization status before her
arrival in the United States, which indicates receipt of the Bacillus Calmette - Guerin vaccine and
a few other childhood vaccines (hepatitis B. measles-mumps-rubella). Het physical examination
findings are normal. Laboratory evaluation at the local health department shows a negative result
on the interferon-γ release assay and nonreactive serologic test for syphilis and HIV type I. A
complete blood cell count and white blood cell differential are normal. Two stool specimens
obtained on separate days are negative for ova and parasites.
Of the following, the BEST next infectious disease screening test for this child is
A. chest radiography
B. serologic testing for hepatitis B virus
C. serologic testing for Schistosoma species
D. serologic testing for Strongyloides species

Correct Answer: B
Each year, thousands of children are internationally adopted in the United States. Many of these
children have lived in orphanages before adoption, where they are an increased risk for
infectious diseases, such as tuberculosis, chronic viral hepatitis„ and endemic parasitic
infections. On arrival in the United States, specialized screening evaluation for infectious
diseases and other conditions is recommended for all international adoptees and refugee and
immigrant children. The American Academy of Pediatrics (AAP) has published guidance on the
medical evaluation of internationally adopted refugee and immigrants children (Item C35A). In
addition to recommendations for infectious disease screening, the AAP guidance also informs
clinicians about appropriate immunizations for vaccine-preventable diseases, cultural adjustment,
nutrition, growth and development, and psychosocial needs.
The recently adopted girl from an orphanage in China described in the vignette is asymptomatic
with normal physical examination findings. Screening for HIV type 1, tuberculosis, syphilis, and
intestinal parasites is negative. Hepatitis B virus (HBV) serologic screening is recommended for
all internationally adopted refugee and immigrant children, regardless of immunization status. In
many HBV-endemic countries, infant HBV vaccine is not included as part of the universal
childhood immunization program. The prevalence of chronic HBV infection is high among
people residing in many countries in Southeast Asia and other regions worldwide, especially in
the World Health Organization African region and Western Pacific region. Globally, an
estimated 257 million people are living with chronic HBV infection. Serologic screening for
tissue-invasive parasites such as Schistosoma and Strongyloides species is not recommended for
the child in the vignette given the absence of eosinophilia (defined as an absolute eosinophil
count >450 cells/µL). Routine chest radiography to evaluate for tuberculosis is not indicated in
this child with negative result on the interferon-γ release assay.
Screening for HBV Includes testing serum for hepatitis B surface antigen (HBsAg),
In addition, some experts recommend serologic testing for the presence of hepatitis B surface
antibody (anti-HBs) and hepatitis B core antibody (anti-HBc). The presence of HBsAg (or HBV
DNA or hepatitis B e antigen) in serum for at least 6 months with negative IgM antibody to
hepatitis B core antigen (IgM anti-HBc) indicates chronic HBV infection (Item C35B). The
presence of hepatitis B e antigen indicates high viral replication with associated increased risk of
HBV transmission. In individuals with resolved HBV infection, anti-HBs and total anti-HBc are
present. In individuals immunized with hepatitis B vaccine, antibody to HBsAg alone is present.
Children diagnosed with chronic HBV infection must be evaluated further with liver function
tests and referred to a specialist with expertise in evaluation and management of chronic viral
hepatitis (typically pediatric gastroenterologist or infectious diseases specialist). The local and
state health department must be notified of all children with positive HBsAg screening.

Item C35B: Typical serologic course of acute hepatitis B virus (HBV) infection with progression to
chronic HBV infection
PREP Pearls
• Hepatitis B virus infection is endemic in many regions of the globe, including Southeast
Asia, sub-Saharan Africa, and Western pacific region.
• Screening for chronic hepatitis B virus infection is recommended for all international
adoptees, refugees, and immigrants, regardless of their immunization status.
• Screening for hepatitis B virus infection includes testing serum for hepatitis B surface
antigen.

• Know how to obtain information about immunizations for patients and families traveling
to foreign countries
• Plan the appropriate infectious disease screening evaluation of an intonationally adopted
child
• Plan the appropriate immunizations for an intentionally adopted child
Suggested Readings
• Hepatitis B. Red Book: 2021-2024 Report of the Committee on infectious Diseases. 32nd
ed; 381-398.
• Medical evaluation for infectious diseases for intonationally adopted, refugee, and
immigrant children. Red Book: 2021-2024 Report of the Committee on infectious
Diseases. 32nd ed; 158-166.
• Council on community pediatrics, providing care for immigrant, migrant, and refugee
children. Pediatrics. doi:10.1542/peds.2013-1099
• Hepatitis At B, and C. Pediatr Rev. doi:10.1542/pir.2015-0075
• Committee on Early Childhood, Adoption, and Dependent care. Comprehensive health
evaluation of the newly adopted child. Pediatrics. doi:10.1542/peds.2011.2381.
Question 36
A 4-year-old boy is brought to the office by his mother who is concerned that her son is delayed
in his development. He speaks in 2- to 3-word phrases and sentences and can follow 2-step
commands. He can balance on each foot and hop. He has difficulty catching a ball. The boy can
draw 3-part person and tell his age and sex. He knows 1 color and can copy a circle but not a
square. He can undress independently; he requires assistance with dressing. His mother is
concerned that the boy may have similar challenges with learning as her brother, who received
special education services in school for intellectual disability.
Of the following, the area of development that correlates MOST closely with this mother's
concern is
A. adaptive
B. expressive language
C. fine motor
D. receptive language

Correct Answer: D
The boy in the vignette has delays in multiple areas of development. He is behind in his
expressive and receptive language, fine and gross motor skills, and adaptive skills. Receptive
language is an important early indicator of cognition, and of the choices given, most strongly
correlates with this mother's concern about possible intellectual disability.
Developmental delays are common and therefore, it is essential that primary care providers
formally screen for such delays, as recommended by the American Academy of Pediatrics.
Delays can be isolated and affect just one area of development (e.g., motor delay) or can affect
several developmental domains. Development can also be atypical, as in the child autism
spectrum disorder whose social and communication skills may be not only delayed but also
different (e.g., unusual tone of voice, phrasing, or use of language).
For a child younger than 5 years, the appropriate term for delay in multiple areas of development
is global developmental delay. Once a child is 5 years or older, cognition can be reasonably
measured. A diagnosis of intellectual disability (ID) can be made for a child who is 5 years or
older if the child is significantly below age expectations for both cognitive and adaptive
development. Cognition includes abilities such as planning, reasoning, problem-solving, and
learning. Adaptive skills include those involved in self-management and independent living (e.g.,
self-care, communication, navigating relationships, recreation, work, money management).
The first sign of ID in a young child can be a delay in language development. Language involves
using signals to represent end communicate ideas. Skills in language use, particularly
understanding language (i.e., receptive language), correlate with cognitive function. Expressive
language, fine motor, and adaptive skills are less strongly correlated with cognitive skills than
receptive language skills at the age of the boy in the vignette.
Approximately 85% of people with ID have mild ID whereas about 10% have moderate ID.
Those with mild ID typically exhibit problems in teaming and may eventually achieve academic
skills up to the 6th grade level. As adults they may need some intermittent support but can
potentially take care of themselves and live and work independently. Those with moderate ID
may reach the 2nd grade in academics; they typically have difficulty with understanding
concepts around time and money. As adults, people with moderate ID usually require prompting
for their self-care and supervision and support for living and work activities.
PREP Pearls
• For a child younger than 5 years, the appropriate term for delay in multiple areas of
development is global developmental delay. Once a child is 5 years or older, a diagnosis
of intellectual disability can be made if the child is significantly below age expectations
for both cognitive and adaptive
• The first sign of intellectual disability in a young child can be delay in language
development, particularly receptive language, as this correlates with cognitive function.
• Most people with intellectual disability (ID) have mild ID. Adults with mild ID may need
some intermittent support but can potentially take care of themselves and live and work
independently

• Recognize the age-related clinical findings associated with intellectual disabilities of
various etiologies
• Distinguish between isolated, global, and atypical developmental delay
• Distinguish between mild and moderate intellectual disabilities with regard to the
potential for and independence/vocational achievement
• Understand the correlation between language development and cognitive function
Suggested Readings
• Cognitive development and disorders. Developmental and Behavioral Pediatrics. 2nd ed.
2018:309-346
• Intellectual disability. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed. 2017 :2208-2216.
• Intellectual disabilities. Pediatr Rev. doi:10.1542/pir.2016-0116 .
• Developmental milestones: cognitive development Pediatr Rev. doi:10.1542/pir.31-9-364

Question 37
A 4-month-old infant is seen for a health supervision visit. She was born at 32 weeks' gestation
and discharged from the neonatal intensive care unit at 8 weeks of age. She received her 2-month
vaccination upon discharge. She is breastfeeding well and gaining weight appropriately. She was
hospitalized 3 weeks ago with intussusceptions reduced by an air-contrast enema. The infant
currently has no infectious symptoms. Her father has rheumatoid arthritis and is
immunosuppressed due to his treatment. The patient's mother is informed that the rotavirus
vaccine is contraindicated today.
Of the following, the CONTRAINDICATION in this situation is due to

A. breastfeeding
B. history of intussusception
C. history of prematurity
D. immunosuppressed parent

Correct Answer: B
Rotavirus vaccination is contraindicated in children with a history of intussusception, severe
combined immunodeficiency (SCID), or a severe allergic reaction to the vaccine or one of its
components. Questions are often raised regarding the safety of this vaccine in premature infants,
breastfeeding infants, or those with household member who is immunosuppressed or pregnant;
however, these are not contraindications.
The first rotavirus vaccine, RV5 (oral pentavalent human-bovine reassortant rotavirus), was
licensed in the United States in 2006 as a 3-dose series and the second vaccine, RV1 (oral human
attenuated monovalent rotavirus), in 2008 as a 2-dose series. Both vaccines are approved by the
centre for Disease Control Prevention. The RV1 prefilled tip contains latex and therefore this
form should not be given to infants with or at risk of latex allergy (e.g., bladder exstrophy or
spina bifida). Prior to the widespread use of these vaccines, rotavirus was the most common viral
cause of profuse, watery diarrhea that leads to severe dehydration especially in infants and young
children. The vaccine is 85% to 95% effective in preventing rotavirus infection that requires
hospitalization.
One concerning potential adverse effect of rotavirus vaccination is intussusception typically

occurring within 7 days of vaccine administration. The incidence of intussusception is highest in
Children under 1 year of age. While the increase in cases in children who receive the vaccine is
low (between 1 in 30,000 and 1 in 100,000) compared to unvaccinated peers, a prior case of
intussusception, which by itself has a recurrence risk of up to 15%, is a contraindication for
rotavirus vaccination.
The first dose in the rotavirus series may be given between 6 weeks and 14 weeks and 6 days of
age. If an infant has not received the first dose by 15 weeks and 0 days, the Infant should not
begin the series. At least a 4-week interval is required between doses, and the vaccine may not be
administered after 8 months and 0 days. Typically, RV5 is given at 2, 4, and 6 months of age, or
RV1 is given at 2 and 4 months of age. Infants born prematurely should follow the same
immunization schedule as full-term infants. Importantly, the rotavirus vaccine should not be
administered while an infant is still in the neonatal intensive care unit due to the risks associated
with viral shedding in the stool after the vaccination.
PREP Pearls
• The rotavirus vaccine series must be started by age 14 Weeks and 6 day. The rotavirus
vaccine may not be administered after age 8 months and 0 days
• Contraindications to rotavirus vaccination include:
o Anaphylaxis to the vaccine or components (i.e., latex in RV1)
o History of intussusception
o Severe combined immunodeficiency
• Infants are at slightly higher risk or intussusception for up to 7 days after receiving the
rotavirus vaccine.

• Know the recommendations, contraindications, and schedule for the rotavirus vaccine

Suggested Readings
• Rotavirus infections. Red Book: 2021-2024 Report of the Committee on infectious
Diseases. 32nd ed; 2021; 644-647.
• Centers for Disease Control and Prevention. Vaccines and preventable diseases: Routine
vaccine recommendations. www.cdc.gov

Question 38
A 5-year-old girl is seen for evaluation. Her mother says that the patient started kindergarten this
year; she has had difficulty in keeping up with her classmates and seems unusually tired and
irritable some days. The patient had been cared for at home only before starting kindergarten, so
her mother attributed the changes to being in a new environment. However, for the past week the
patient has been on a school break, but the fatigue and irritability have persisted.
The patient is thin and appears initially fearful but then acts friendly and smiles. Her vital signs
show a blood pressure of 105/55 mm Hg, heart rate of 120 beats/min, and respiratory rate of 25
breaths/min. Her weight is 14 kg, at the 3rd percentile. The girl’s conjunctiva and oral mucosa
are pale. Her lungs sound clear, and there is no organomegaly. Her neurologic examination-
shows a lack of deep tendon reflexes. The family eats a strict vegetarian diet. The girl was
breastfed for the first year of life, and she became an increasingly picky eater when she started
on solid foods. She stools without difficulty, usually once daily; the stools are sometimes loose.
Her mother has never noticed any blood in the stools.
Laboratory data are shown:

White blood cell count 6,300 /µL (6.3 x 109/L)
Hemoglobin 7.4 g/dL (74 g/L)
Mean corpuscular volume 95.8 fL
Neutrophils 2,400/µL (2.4 x 109/L)
Lymphocytes 2,200/µL (2.2 x109/L)
Monocytes 360/µL (0.36 x 109/L)
Reticulocytes 1.5%
solute reticulocyte count 75,000/µL (75 x 109/L)
Of the following, the BEST next step in the management of this child is
A. folate supplementation
B. a gluten-free diet
C. iron supplementation r
D. vitamin B12 supplementation

Correct Answer: D
The child in the vignette most likely has vitamin B12 deficiency. She has macrocytic anemias
(mean corpuscular volume [MCV]>80 fL), with pallor and tachycardia on physical examination.
Macrocytic anemia is most consistent with a folate or vitamin B12 deficiency. Her neurological
findings of loss of deep tendon reflexes makes vitamin B12 deficiency more likely. With a lack of
intake or decreased intake, vitamin B12 deficiency takes several years to develop. A strict
vegetarian diet with a lack of any animal fats, the most common source of vitamin B12,
significantly increases the risk. If her mother’s level of vitamin B12 were suboptimal, exclusive
breast milk as the patient's main source of nutrition during infancy increases the girl’s risk for
vitamin B12 deficiency. Due to her current neurologic findings, intramuscular vitamin B12
supplementation would be most beneficial and would rapidly increase the girl’s vitamin B12
level.
Fortification of common foods (e.g., bread and other grain products) with folic to prevent neural
tube defects, has decreased the incidence of folic acid deficiency in the United States. Neurologic
abnormalities associated with folate deficiency include loss of developmental milestones, a
finding not noted for this child.
The girl does not currently require iron supplementation. The fact that she is a “picky eater” may
raise concern for iron deficiency. The lack or microcytosis (MCV <80 fL) makes iron deficiency
less likely. If she had iron deficiency as a part of a mixed anemia, laboratory findings might
demonstrate both large and small red blood cells, resulting in an overall normal MCV. The
neurologic findings seen in children with iron deficiency usually affect the intelligence quotient
and cognition and cause a loss of deep tendon reflexes.
Malabsorption should be considered as a cause of this girl’s condition, especially with the
occasional loose stools and her low weight. Celiac disease can lead to both folate and vitamin
B12 deficiencies. However, restricting this child's is not appropriate before that diagnosis is
made. While considering the causes of intestinal malabsorption syndrome, oral or parenteral
replacement of vitamin B12 deficiency should be considered.
The girl's macrocytosis should raise concern about a bone marrow failure abnormality; however,
the other cell lines (white blood cell and platelet counts) are normal, which is reassuring.
PREP Pearls
• It is important to counsel vegetarian families on how to ensure adequate intake of vitamin
B12, especially mothers of breastfed infants.
• Vitamin B12 deficiency should be considered in children with nonspecific symptoms
(including generalized symptoms of fatigue, weakness, poor appetite, irritability), and
those with failure to thrive.
• Neurological findings of B12 deficiency may include hypotonia, ataxia, seizure activity,
and loss of deep tendon reflexes.

• Recognize the signs. symptoms, and causes of vitamin B12 deficiency, and manage
appropriately
• Recognize the signs, symptoms, and causes of folate deficiency, and manage
appropriately
Suggested Readings
• Vitamin excess and deficiency. Pediatr Rev. doi:10.1542/pir.2016-0068
• Lankowsky’s Manual of Pediatric Hematology and Oncology. 6th ed. Philadelphia, PA:
Elsevier:2016:85-101
• Anemia and pallor. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed.
2017 :1199-1208

Question 39
A 14-year-old adolescent girl is seen at the emergency department with dizziness. She has a 5-
week history of worsening abdominal pain, diarrhea, and rectal bleeding. She reports up to 7
watery, red bowel movements daily with urgency, nocturnal stooling, and tenesmus. She reports
weight loss, intermittent fevers, and mouth sores over the past 2 weeks. She is otherwise healthy
and does not take any medications. She appears tired and pale with a temperature of 39 °C,
tachycardia, a grade 2/6 systolic ejection murmur, mild, diffuse abdominal tenderness and
distention, and normal external rectal findings. A digital rectal examination reveals mucusy and
bloody stool
Laboratory data are show

Mean corpuscular volume 63 fL
Erythrocyte sedimentation rate 40 mm/h
Fecal lactoferrin Positive
Fecal calprotectin (reference range <50 µg/g) 890 µg/g
Fecal occult blood Positive
Of the following, the adolescent's MOST likely diagnosis is

A. celiac disease
B. Escherichia coli 0157:H7 infection
C. inflammatory bowel disease
D. juvenile polyp

Correct Answer: C
The adolescent in the vignette has severe ulcerative colitis. Findings consistent with colitis
include the presence of bloody diarrhea, urgency, nocturnal stooling, and tenesmus in the context
of elevated stool inflammatory markers (lactoferrin and calprotectin). The duration of symptoms
is consistent with a chronic process; most infectious etiologies would be expected to resolve after
10 to 14 days.
The differential diagnosis of bloody stool includes infectious causes (e.g., Salmonella, Shigella,
Campylobacter, and Yersinia species; Clostridioides difficile (formerly known as Clostridium
difficile), Escherichia coli 0157:H7, and Entameba histolytica), inflammatory bowel disease
(IBD), vascular abnormalities, Meckel diverticulum, colonic polyps, and hemorrhoids/fissures.
Initial evaluation of colitis should include testing for likely pathogens and, if negative in the
context of persistent symptoms, evaluation with endoscopy and colonoscopy for inflammatory
bowel disease. The adolescent in the vignette underwent testing for infectious colitis (negative)
and then endoscopy and colonoscopy, which demonstrated severe ulcerative pancolitis (Item
C39A). Her clinical presentation meets criteria for acute severe colitis based on the Pediatric
Ulcerative Colitis Activity Index (PUCAI) score. The PUCAI score is based on the presence of
abdominal pain, degree of rectal bleeding, stool consistency and frequency, presence of
nocturnal stooling, and degree of activity restriction due to symptoms. Complications of acute
severe colitis (ASC) include severe gastrointestinal hemorrhage, toxic megacolon, and
multiorgan failure. Recommended treatment for hospitalized patients with ASC due to ulcerative
colitis (Item 039B) includes intravenous steroids as well as biologic therapies (e.g., infliximab).
Colectomy should be discussed and surgical consultation obtained early in the hospitalization; it
May be recommended for patients with a lack of response 10 to 12 days after initiation of
treatment.
Item C39A: ulcerative pancolitis
Supportive care is essential. Nutritional status should be evaluated and monitored. If tolerated, a
regular diet may be prescribed; enteral or parenteral nutrition may be used if needed.
Hospitalized patients with IBD are at increased risk for venous thromboembolism, including
intracranial thrombosis and portal vein thrombosis. Venous thromboembolism prophylaxis with
subcutaneous low-molecular-weight heparin should be considered in addition to mobilization,
hydration, and avoidance/prompt removal of central venous catheters (including peripherally
inserted central catheters). Anemia should be treated with packed red blood cell transfusions to
attain a hemoglobin > 8 gm/dL (80g/L)
Item 039B : Recommended treatment ulcerative colitis
Celiac disease causes enteropathy and may result in chronic, nonbloody diarrhea. Hematochezia
is not associated with celiac disease. E coli 0157:H7 infection may result in colitis and anemia,
however it generally resolves in 6 to 10 days; a 5-week course of bloody diarrhea would not be
consistent with E coli 0157:H7 infection. A juvenile polyp could present with chronic
gastrointestinal bleeding; however, it is generally painless and would not result in symptoms
consistent with colitis (e.g., nocturnal stooling, urgency, and tenesmus).

PREP Pearls
• Signs and symptoms of colitis include bloody diarrhea, nocturnal stooling, urgency, and
tenesmus.
• Infectious causes of colitis include Salmonella, Shigella, Campylobacter, and Yersinia
species; Clostridioides difficile (formerly known as Clostridium difficile), Escherichia
coli 0157:H7, and Entameba histolytica
• Treatment of acute severe colitis in children with ulcerative colitis includes inflammatory
bowel disease (Intravenous steroids, biologic therapies) and supportive care (attention to
nutritional status, decreasing risk of venous thromboembolism, and treatment of anemia).
ABP content Specifications

• Recognize the clinical features associated with inflammatory bowel disease (e.g., Crohn
disease, ulcerative colitis)
• Plan appropriate management of severe colitis
• Plan the initial evaluation of inflammatory bowel disease
• Formulate a differential diagnosis of acute colitis
Suggested Readings
• Inflammatory bowel disease. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017:2199-2203.
• Management of acute severe colitis in children with ulcerative colitis in the biologic's era.
Pediatrics. Doi: 10.1542/peds.2015-1184
• Health supervision in the management of children and adolescents with IBD:
NASPGHAN recommendations. J Pediatr Gastroenterol Nutr. Doi:
10.1097/MPG.0b013e31825959b8
• Inflammatory bowel disease. Pediatr Rev. doi:10.1542/pir.2015-0110

Question 40
A 14-year-old adolescent boy is seen in the clinic for nasal congestion and bilateral nasal
drainage that has waxed and waned over the past 3 months. His mother reports that he “mouth
breaths” during the day and snores when he sleeps. He has been afebrile and reports no pain. On
physical examination, the boy is in no apparent distress and has age-appropriate vital signs. His
weight and height are at the 50th percentile for age, and his growth trajectories are appropriate.
He has audible mouth breathing and allergic shiners. His tonsils are 1+ in size, and he has cobble
stoning of the posterior oropharynx. Findings on otoscopic examination of his nasal passages are
shown Item Q40.
Of the following, the BEST next management step for this adolescent is
A. amoxicillin-clavulanate
B. diphenhydramine
C. fluticasone nasals spray
D. foreign body removal

Correct Answer: C
The adolescent in the vignette has a nasal polyp (Item C40). Treatment options for nasal polyps
include intranasal corticosteroids, such as fluticasone nasal spray, nasal saline rinses, or systemic
corticosteroids. If symptoms persist despite treatment, polyps can be surgically removed.
Nasal polyps result from chronic inflammation of the mucosa of the nares or paranasal sinuses.
Typical symptoms are similar to those or chronic rhinosinusitis, nasal obstruction, mucopurulent
discharge, decrease sense of smell, and sometimes facial pain or pressure. If the nasal passages is
clear and the child is sufficiently cooperative, polyps may be visualized with an otoscope tip
inserted into the nares. Polyps that are more posteriorly located may require nasal endoscopy for
visualization. Occasionally computed tomography is needed to determine the extent of
involvement and to assess for associated anatomic abnormalities.
Although nasal polyps are common in adults with chronic Rhinosinusitis, they are rare in
children younger than 10 years. Children with cystic fibrosis and primary ciliary dyskinesia have
a higher risk of developing nasal polyps; accordingly, these conditions must be considered in
children with nasal polyps.
The history and physical examination findings of the adolescent in the vignette are not
suggestive of a foreign body or infection. There is no role for antibiotics. Although
diphenhydramine may help to alleviate symptoms, this medication often causes somnolence and
is therefore not preferred. A nonsedating systemic antihistamine would be more appropriate.
PREP Pearls
• Nasal polyps can cause symptoms of nasal obstruction, mucopurulent discharge,
decreased sense of smell, and sometimes facial pain or pressure
• Although nasal polyps are common in adults with chronic rhinosinusitis, they are rare in
children under 10 years of age. Children with cystic fibrosis and primary ciliary
dyskinesia have a higher risk of developing nasal polyps; these conditions must be
considered in children with nasal polyps
• Nasal polyps can be treated with intranasal corticosteroids and nasal rinses or systemic
corticosteroids. If symptoms persist despite medical treatment, polyps can be surgically
removed
MOCA-Peds Objectives
• Evaluate and manage a patient with nasal polyps

• Recognize the clinical findings associated with nasal polyps in patients of various ages
• Plan the appropriate diagnostic evaluation of nasal polyps
• Identify conditions associated with nasal polyps in patients of various ages

Suggested Readings
• Primary ciliary dyskinesia. Pediatr Rev. doi:10.1542/pir.2016-0108.
• Chronic rhinosinusitis in children: pathophysiology, evaluation, and medical
management. Curr Allergy Asthma Rep. doi:10.1007/s11882-018-0792-8.
• Masses of the nose, nasal cavity, and nasopharynx in children. Radiographic. doi:
10.1148/rg.2017170064

Question 41
A 6-year-old girl is evaluated tor a 3-month history of recurrent headaches that increased in have
increased in frequency and severity. She reports that they occur 2 to 3 times per month, often in
the middle of the night, with associated nausea and vomiting. The girt is unable to describe the
quality of the headache. Her mother notes that her daughter usually wants to lie down in a quiet,
dark room when they occur. The headaches are responsive to appropriately dosed nonsteroidal
anti-inflammatory drugs. She reports no associated focal neurologic deficit, vision or speech
changes or preceding aura. No clear triggers have been identified. Valsalva maneuver does not
initiate headache. There is no family history of migraine headaches. Neurologic examination
findings, including fundoscopic examination, are normal.
Of the following, the BEST next step in this girl's management is

B. lumbar puncture
C. trial of cyproheptadine
D. trial of rizatriptan

Correct Answer: A
The child in the vignette's headaches could fit that of a primary headache disorder, such as
migraine without aura, however there are several red flags that warrant further evaluation for a
secondary headache. Causes of secondary headaches include pseudotumor cerebri syndrome,
brain tumor, febrile illness, hypertension, hydrocephalus, or intracranial infection. Differentiating
primary versus secondary headache conditions is an important part of the initial clinical
evaluation, as this determines the next steps in management (Item C41A). In a non-emergency
setting, due to the high level of image detail and lack of radiation exposure, brain magnetic
resonance imaging (MRI) is generally the appropriate next step in the diagnostic evaluation of
secondary headache.
Item C41A: Primary versus secondary headache
The clinical history plays a critical role in evaluation of pediatric headache, serving 3 purposes:
• To evaluate for red flags differentiating versus secondary headache
• If a primary headache disorder, to determine headache type based on clinical features
• If a primary headache disorder, to identify lifestyle triggers, which will help to shape the
management plan
Item C41B lists questions that should be asked when evaluating a patient with headaches.
Item C41B: Basic Headache Questions

1. When did you first begin having headache(s)?
2. What is the temporal pattern of your headaches?
- sudden onset of first lifetime headache
- episodic headaches, normal in between
- frequent nonprogressive headaches
- gradually worsening headaches
- a mixture of daily headache with episodic worsening
3. Where does your head hurt with your headaches?
4. What do your headaches feel like (throbbing, pounding, stabbing, squeezing, etc)?
5. What do you do when you get a headache?
6. How long do your headaches typically last?
7. With your headaches do you have:

- nausea
- vomiting
- photophobia
- phonophobia
- dizziness
- numbness
- weakness
- double vision
8. Do you get a warning sign or can you tell a headache is coming on?
9. Has a headache ever awoken you at night or is present first thing on awakening?
10. Have you ever had a seizure?
11. Do any activities, foods, or medications make your headaches worse
The mnemonic "SNOOPY” highlights red flags in the clinical presentation of headache that
warrant further investigation (Item C41C)
Item C41C: "SNOOPY” Headache red flags

• System signs and disorders/ Secondary risk factors
o awakening with headache or vomiting
o worsening of headache while straining
• Neurologic signs/symptoms:
o double vision;
o Presence of seizures;
o associated mood, mental status, or school performance change;
o neurocutaneous stigmata (café-au-lait macules, hypopigmented macules).
o
• Onset is sudden/abrupt
o recent onset (<6 months) with a steadily worsening pattern (frequency or intensity);
o early-morning
o explosive onset
• Papilledema/pulsatile/positional/ precipitated by exercise
• Younger than 3 years of age;
Most childhood headache disorders are primary headaches, often either migraine or tension
headache, which are both clinical diagnoses and do not require further testing in the absence of
red flags. The clinical time course can be a clue for differentiating primary versus secondary
headaches and headache subtype. Headache time course can be divided into 4 broad categories
(Item C41D): 1) acute, 2) acute recurrent (episodic), 3) chronic progressive, and 4) chronic
nonprogressive. Acute recurrent (episodic) and chronic nonprogressive headaches are most likely
to be primary headache disorders, while acute and chronic progressive headaches are more
concerning for secondary headache disorders and warrant further evaluation. All children
evaluated for headache should have a complete neurologic examination, including fundoscopic
examination; if abnormalities are identified, further diagnostic evaluation is required.

Item C41D: All headaches fit in this diagram. The severity of the headache is on the Y axis, and time
measured in days is on the X axis. (From Rothner AD. The evaluation of headaches in children and
adolescents. Semin Pediatr Neurol. 1995;2(2):109–119, with permission from Elsevier.)
For younger children, it can be challenging to obtain a complete history of headache features
because it can be difficult for them to describe. Close attention to the child's behavior during the
headache may provide important clues for associated symptoms with the headache. The child in
the vignette's headache history has features suggestive of migraine without aura: a headache that
impacts her activities, preference a quiet and dark room (photophobia and phonophobia),
associated nausea and vomiting, episodic pattern, and responsiveness to nonsteroidal anti-
inflammatory drugs. However, there are red flags in her history, including repeated occurrence of
headache in the middle of the night, lack of family history of migraine, young age, and
progressive frequency and severity.
Lumbar puncture can be a useful diagnostic tool when headaches are to be secondary to high
pressure (idiopathic intracranial hypertension), intracranial infection, or subarachnoid
hemorrhage. This procedure should only be performed after diagnostic imaging to rule out a
structural cause.
Empiric treatment for migraine with a daily preventative medication, such as cyproheptadine
may be part of this child's management plan once secondary causes have been ruled out. Her
headaches are currently responsive to nonsteroidal anti-inflammatory drugs, so there is no need
to change her abortive headache care to include rizatriptan.
PREP Pearls
• Pediatric headache disorders include primary headache conditions (migraine, tension,
chronic daily headache) and secondary headache conditions (pseudotumor cerebri
syndrome, brain tumor, hypertension, intracranial infection, or hydrocephalus).
• Clinical history and physical examination are critical for identifying potential red flags
suggesting a secondary headache disorder (SNOOPY mnemonic)
• In non-emergency situations, when red flags are identified by clinical evaluation of
headache, brain magnetic resonance imaging is the preferred neuroimaging modality due
to the high level of detail and tack of radiation exposure.
MOCA-Peds Objective
• Recognize and manage migraine variants in children.

• Understand the appropriate use of neuroimaging in the evaluation of headache
• Recognize the clinical findings associated with headaches of various etiologies
• Recognize elements of history associated with headaches ot various etiologies
Suggested Readings
• Occipital headaches and neuroimaging in children. Neurology.
Doi:10.1212/WNL.0000000000004186
• Headache. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. 1404-
1408
• Headache in children. Pediatr Rev. doi:10.1542/pir.2017-0012

Question 42
A 2-day-old female neonate is seen in the office for an initial visit. Her mother is 22 years old
and has a history of epilepsy-treated with levetiracetam. The neonate was born at home with the
assistance of a midwife. The delivery was uncomplicated, followed by administration of
intramuscular vitamin K and ophthalmic erythromycin. The neonate is breastfeeding and had 2
stools today. Physical examination findings are unremarkable. Her mother recalls no testing
being done after the birth. She has no paperwork from the delivery.
Of the following, the BEST screening test to perform now is

A. blood typing
B. complete blood cell count
C. levetiracetam measurement
D. pulse oximetry

Correct Answer: D
Newborn screening was developed to identify conditions which, when diagnosed and treated
early, could decrease long-term complications. Newborn screening is mandated by law for all
neonates born in the United State. Historically, newborn screening was developed to detect
inherited diseases of metabolism with analysis of blood taken from the neonate before hospital
discharge. With recognition of delayed language development with congenital hearing loss, a
hearing screen was added to newborn screening tests. In 2011, screening with pulse oximetry for
critical congenital heart disease was recommended. As home births increase in popularity
pediatric providers should ensure that neonates continue to be screened to optimize outcomes.
The hearing test, screen for inherited diseases of metabolism, and pulse oximetry test should be
conducted in a birth center or primary care provider's office. Thus, the neonate in the vignette
should undergo pulse oximetry screening.
Screening for critical congenital heart disease should be completed 24 hours after birth or 24
hours after the last respiratory support. If there is a more than 3% difference between the pre-
and post-ductal saturation values, the test should be repeated in 12 hours. Preductal saturation
should be checked on the right hand. Post-ductal saturation may be measured on either foot. If
the neonate fails the test again, the neonate should be referred for echocardiography. Pulse
oximetry does not detect all forms of critical congenital heart disease. In particular, coarctation
the aorta and tetralogy of Fallot are less likely to be detected with pulse oximetry screening.
Screening for inherited disease of metabolism varies from state to state. There is a recommended
list of conditions provided by the Health Resources and Service Administration (HRSA)
(https://mchb.hrsa.gov/maternal-child-health-initiatives/newborn.screening/process). Blood taken
from a heel stick placed onto filter paper between 24 and 48 hours after birth is analyzed using
tandem mass spectroscopy. In some states, a second test is performed by an outpatient primary
care provider. Of note, 90% of positive test results on newborn screening are false positives. For
neonates who have positive test results, detailed instructions regarding clinical outcomes and
follow-up testing can be found on ACTion (ACT) sheets which are maintained by HRSA
(https://www.acmg.net/ACMG/Medical-Genetics-Practice-
Resources/ACT_Sheets_and_Algorithms/ACMG/Medical-Genetics-Practice-
Resources/ACT_Sheets_and_Algorithms.aspx?hkey=9d6bce5a182e-42a6-84a5-b2d88240c508)
Premature infants are more likely to have false positive test results because of the administration
or parenteral nutrition and inaccurate reference ranges. Neonates with positive test results may
require referral to a center with experience treating inherited diseases of metabolism for further
management.
Hearing screening should be completed for all neonates using the otoacoustic emission test. This
should be done before hospital discharge or within the first month after birth for home births.
Any neonate who remains in the neonatal intensive care unit more than 5 days requires a
brainstem auditory evoked response. Neonates who do not pass the hearing screen should be
referred for outpatient audiology evaluation and tested for cytomegalovirus infection.
Neonates should be screened for physiologic jaundice with a serum bilirubin level. Blood typing
is not required. Because there is no concern for hemolysis, a complete blood cell count is not
needed. A levetiracetam level measurement is not indicated because the neonate does not have a
seizure disorder.
PREP Pearls
• Newborn screening consists of testing for inherited diseases of metabolism, critical
congenital heart disease and congenital hearing loss.
• Newborn screening is required but varies in scope from state to state.
• For neonates born outside the hospital, newborn screening should be completed in the
birth center or primary care provider’s office.

• Recognize that newborn screening identifies conditions that affect a child’s long-term
health or survival white recognizing that testing requirements vary from state to state
Suggested Readings
• Baby's first test. What is newborn screening? https://www.babysfirsttest.org
• Committee on fetus and newborn. Planned home birth. Pediatrics,
doi:10.1542/peds.2013-0575
• The current state of newborn screening in the United States. Pediatr Clin North Am. doi:
10.1016/j.pcl.2018.12.007.
• Immunizations. newborn screening, and blood tests. A Bright Futures Handbook
2010:137-146.
• The newborn infant. Nelson Textbook of Pediatrics 21st ed. 2020:867-876
• Screening for critical congenital heart disease in newborns. Circulation.
doi:10.1161/ClRCULATlONAHA.113.008522

Question 43
A 5-year-old boy is brought to the emergency department with decreased urine output for 1 day.
He has had diarrhea vomiting, and poor oral intake for 5 days, He has not received any
medications. The boy's temperature is 38.6 °C, heart rate is 130 beats/min, respiratory rate is 20
breaths/min, and blood pressure is 80/50 mm of Hg. He is lethargic, his mucous membranes are
dry, and the capillary refill is more than 3 seconds. His chest is clear to auscultation.

Blood
White blood cell count 15,200/µL (15.2 x 109/L)
Platelet count 300 x103 /µL (300 x 109/L)
Creatinine 2.5 mg/dL (221 µmol/L)
Urine
Specific gravity 1.015
Leukocyte esterase 1+
Nitrite Negative
Blood Trace
Protein 1+
Red blood cells 2-5/HPF
White blood cells 5-10/HPF
Casts Granular, 1-2/low-power field
Of the following, this boy's MOST likely diagnosis is

A. acute glomerulonephritis
B. acute interstitial nephritis
C. acute tubular necrosis
D. hemolytic uremic syndrome

Correct Answer: C
The boy in the vignette has acute kidney injury (AKI) as supported by a decrease in urine output
after an episode of diarrhea and vomiting, and elevated blood urea nitrogen and creatinine levels,
He most likely has acute tubular necrosis (ATN) secondary to hypovolemic shock (tachycardia.
hypotension, and prolonged capillary refill). The presence of minimal blood and protein on
urinalysis and of granular casts favor a diagnosis Of ATN.
Acute kidney injury is defined as a decrease in the glomerular filtration rate on the basis of an
elevated serum creatinine level or a decrease in urine output. Acute kidney injury is classified
into 3 types:
• Prerenal (decreased effective renal blood now)
• Renal (intrinsic renal damage)
• Postrenal (obstruction to the flow of urine)
Prerenal AKI, the most common type, is seen in children with gastroenteritis, sepsis, bleeding,
nephrotic syndrome, and liver disease. Renal AKI can be further divided into glomerular injury
(glomerulonephritis), tubular injury (ATN), interstitial injury (interstitial nephritis), and vascular
injury (hemolytic uremic syndrome). Postrenal AKI results from upper/lower urinary tract
obstruction or posterior urethral valves. The etiology of AKI in different age groups is shown in
Item C43.
Item C43: AKI etiology of in different age groups.
Acute tubular necrosis is the most common cause of renal Kl in hospitalized children. Acute
tubular necrosis results from damage to renal tubular cells by ischemia (e.g., shock, trauma,
sepsis, burns), exogenous toxins (e.g., nephrotoxic medications, contrast agents), or endogenous
toxins (e.g., myoglobinuria. hemoglobinuria). Urinalysis shows epithelial or muddy-brown
granular casts, mild pyuria, and low-grade proteinuria. In ATN, the urine may be dilute, with
specific gravity below 1.010, urine sodium level greater than 40 mEq/L, and fractional excretion
of sodium (FENa) greater than 2%. In contrast, prerenal AKI is characterized by negative
urinalysis result, urine specific gravity greater than 1.020 urine sodium level less than 10 mEq/L
and FENa less than 1%.
Acute tubular necrosis may be prevented by prompt reversal of the cause of ischemia or prerenal
AKI. When caused by nephrotoxic medications or toxin-mediated injury, ATN may be prevented
in children by maintaining appropriate hydration. Once ATN develops, treatment is primarily
supportive.
The common complications of severe AKI include metabolic abnormalities (e.g., hyperkalemia,
hyperphosphatemia, metabolic acidosis, hypocalcemia), uremia (e.g., encephalopathy,
pericarditis), hematologic abnormalities (e.g., anemia, platelet dysfunction), hypertension (e.g.,
volume overload or renin mediated), and upper gastrointestinal bleeding.
Acute glomerulonephritis is characterized by gross or microscopic hematuria, presence of red

blood cells casts, moderate to high-grade proteinuria, hypertension and azotemia. The child in
the vignette has hypotension and minimal proteinuria or hematuria. Acute interstitial nephritis is
usually caused by an allergic reaction to a drug, and is characterized by fever, rash, and
eosinophilia. Urinalysis may show white blood cells, urine eosinophils, red blood cells, and low-
grade proteinuria. Those with hemolytic uremic syndrome may have a history of preceding
diarrhea and the classic triad of hemolytic anemia, renal failure, and thrombocytopenia.
Hemolytic uremic syndrome is an unlikely diagnosis for the child in the vignette because of the
absence of anemia and thrombocytopenia.
PREP Pearls
• Acuta kidney injury is classified as prerenal (decreased effective renal blood flow), renal
(intrinsic renal damage), and postrenal (obstruction to the flow of urine)
• Acute tubular necrosis is the most common cause of acute kidney injury in hospitalized
children.
• Acute tubular necrosis results from ischemia or exogenous/endogenous toxins,
ABP content Specification

• Identify the etiology of acute renal failure in patients of various ages
• Recognize complications associated acute renal failure
Suggested Readings
• Oliguria and anuria. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed
2017:2423-2427
• Acute kidney injury. Pediatr Rev. doi: 10.1542/pir.35-1-30

Question 44
A 2-year-old girl is brought to the emergency department for difficulty breathing. The girl was
sitting on the floor playing when her mother heard her cough and gag. She appeared to be having
difficulty breathing and continued to cough. She had been afebrile and in her usual state of health
prior to this event. She has no previous medical history. On arrival to the emergency department,
her vital signs are a temperature of 37.6 °C, heart rate or 118 beats/min, respiratory rate of 38
breaths/min, and blood pressure of 108/76 mm Hg. She is a well-developed girl in moderate
respiratory distress, her lung examination reveals wheezing on the right side and decreased
breath sounds in the right lower lobe. She has no accessory muscle use. Her anteroposterior and
lateral chest radiographs are shown in Item Q44.
Of the following, this girl's MOST likely diagnosis is

A. acute asthma exacerbation
B. anaphylaxis
C. croup
D. foreign body aspiration

Correct Answer: D
The girl in the vignette has acute onset of difficulty breathing after being found on the gagging
and coughing. The most likely diagnosis is a foreign body aspiration. The girl does not have a
history of asthma or reactive airway disease and did not have any signs or symptoms of an upper
respiratory infection or fever prior to the onset of acute respiratory symptoms, making an acute
asthma exacerbation or croup less likely.
Signs and symptoms may occur suddenly in anaphylaxis, and this diagnosis should be considered
in any child who presents with acute-onset of difficulty breathing. Anaphylaxis is defined as an
acute reaction that involves 2 or more organ systems (e skin, gastrointestinal, cardiovascular, and
respiratory) after exposure to an allergen. The definition has recently been expanded to include
the isolated finding of hypotension after exposure to an allergen. Symptoms of anaphylaxis may
include rash, flushing, angioedema, pruritus, rhinorrhea, stridor, cough, wheeze, difficulty
breathing, abdominal pain, diarrhea, vomiting, dizziness, syncope, and hypotension. The girl in
the vignette has isolated respiratory findings, no evidence of involvement of another organ
system, and her blood pressure is normal for age, making anaphylaxis unlikely.
A high index of suspicion based on history and clinical findings is essential to diagnosing an
aspirated foreign body in a timely manner. The diagnosis requires a careful history and physical
examination. The history should include the child’s state of health prior to the development of
respiratory distress, acuity of onset, any witnessed choking event, and any coughing or gagging
prior to the onset respiratory symptoms. Foreign body aspiration should be suspected when there
is a history of choking in a child with recurrent pulmonary illnesses that do not respond as
expected to standard therapies. Physical examination findings can vary depending on the location
(Item C44A) and size of the aspirated foreign body, and the duration of time the foreign body
has been present.
Item C44A: Physical examination findings

The most commonly aspirated foreign bodies are food matter (e.g., peanuts, popcorn, hot dogs),
toys, and small household objects (e.g., coins, buttons).
Up to one-third of children with acutely aspirated foreign bodies have normal chest radiography
findings. The majority of aspirated foreign bodies are radiolucent. Radiographic signs suggestive
of aspiration include:
• Hyper expansion/air trapping on the affected side(s)
• Flattened hemidiaphragm on the affected side(s)
• Atelectasis
• Consolidation
• Pneumonia
• Mediastinal shift
• Pneumothorax
Anteroposterior and lateral chest radiographs taken during the expiratory phase with bilateral
decubitus chest radiographs may be helpful in making the diagnosis. Item C44B shows hyper
expansion due to air trapping on the side of the foreign body. The classic finding on bilateral
decubitus chest radiographs is lack of expected lung collapse, due to air trapping, on the side
with the foreign body when placed in the dependent position (Item C44C). The absence of
abnormal radiographic and/or physical examination findings does not exclude the diagnosis of
foreign body aspiration.
Fluoroscopy can be used to evaluate the movement of the diaphragms, mediastinum, and lungs
on inspiration and expiration, which can be altered in the case of foreign body aspiration. Rigid
bronchoscopy is the gold standard for the diagnosis of aspirated foreign bodies and is also used
for foreign body removal.
An algorithm for evaluation of an aspirated foreign body is shown in Item C44D.

Management of a child with an aspirated foreign body varies according to their signs and
symptoms. Complete obstruction of the airway is a life-threatening emergency. For children <1
year of age, chest thrust and back slaps with the child positioned head down should be performed
immediately. For children >1 year of age the Heimlich maneuver should be performed. Non-
obstructing airway foreign bodies are removed by rigid bronchoscopy under general anesthesia.
Open thoracotomy or lung resection may be required in the rare case that foreign body removal
by bronchoscopy is not successful. Antibiotics may be required in children who develop
obstructive or recurrent pneumonia or lung abscess.
Complications of an aspirated foreign body can include:

• Bronchiectasis
• Lung abscess
• Recurrent or persistent pneumonia
• Perforation of the bronchial tree
• Fistula formation and pneumothorax
• Sudden cardiac arrest and death (if complete obstruction of the upper airway occurs)
PREP Pearls
• Normal physical examination and radiographic findings do not exclude the diagnosis of
an airway foreign body.
• physical examination findings can vary depending on the location and size of an aspirated
foreign body and the duration of time it has been present
• Bronchoscopy is the gold standard for the diagnosis of aspirated foreign bodies and is
used for foreign body removal

• Plan the appropriate evaluation or suspected foreign body aspiration, and manage
appropriately
• Recognize the historical, clinical, and laboratory findings associated with foreign body
aspiration
• Recognize long-term complications associated with foreign body aspiration
Suggested Readings
• Ingested and aspirated foreign bodies. Pediatr Rev. doi:10.1542/pir.36-10-430.
• Foreign body ingestion and aspiration. Pediatr Rev. doi:10.1542/pir.30-8-295.
• Pediatric foreign body aspiration. Pediatr Rev. doi:10.1542/pir.21-3-86
• Foreign bodies of the ear. nose, airway and esophagus. American Academy of Pediatrics
Textbook of Pediatric Care. 2nd ed 2017:2021.2027.

Question 45
A 9-month-old male infant is brought for a health supervision visit. His mother is concerned that
since she returned to work, he cries a lot and only wants to be held. The boy cries for at least 1
hour when the mother leaves for work and is only pacified when his grandmother, who cares for
him during the day, has him watch television. He also cries for an hour when the mother returns
from work or if she is not in the same room. The crying resolves if he watches videos on her
smartphone. The mother states that co-sleeping is the only way to get him to fall and stay asleep.
She says she truly loves her son but feels tired and overwhelmed. Further questioning leads to no
suspicion of infant maltreatment. Responses to the survey of Well-being of Young Children are
shown in Item Q45. He has no previous or new medical problems. Physical examination shows
vital signs and growth parameters to be normal. He initially sits on the examination table calmly
and quietly but when approached to be examined, the infant cries and retreats to be picked up by
his mother. When his mother picks him up, she appears defeated, and he continues to cry and
struggle against her arms. He continues this until the examination is completed. The examination
findings are otherwise normal, with no signs of infant maltreatment.
Of the following, the MOST appropriate next step is to

1. reassure the mother that the infant's behavior is normal
2. refer both the mother and infant to supportive services
3. refer only the infant to early intervention services
4. refer only the mother to mental health services

Correct Answer: B
The history, physical examination, and Survey of Well-being of Young Children (SWYC)
screening tool for the mother and infant in the vignette all demonstrate concerning signs and
symptoms for emotional disturbances. They should both be referred to supportive services, such
as early intervention and mental health services. Reassurance alone is inappropriate given the
degree of distress reported and seen. Referring only the mother or the infant would also be
inadequate.
Infant mental health is shaped in part by how the infant’s temperament reacts with that of
caregivers. Infant temperament is shaped by prenatal factors such as maternal stress and
postnatal factors such as consistency and predictability provided by the caregiver(s). These
parenting skills are influenced by the caregiver's own mental health and experiences as a child.
Infants for whom fussiness, irritability, or crying is the primary concern should be assessed for
medical or traumatic causes especially maltreatment. Infants who are perceived to be very needy
are at greater risk for abuse and neglect. Pediatricians who suspect any maltreatment should
promptly address this with the caregiver and refer the caregiver/infant dyad to mental health
and/or child protective services as needed.
Observation of attachment patterns during the examination (Item C45) can shed light on the
infant's and caregiver’s temperament, mental health, and parenting styles. Insecure attachment,
as demonstrated by the infant in the vignette may be a sign of poor mental health on the part of
the caregiver and should prompt the pediatrician to screen these parents and provide appropriate
mental health referrals.
Screening tools such as the SWYC, Ages and Stages Questionnaire: Social-Emotional,
Edinburgh Postpartum Depression Scale, and Patient Health Questionnaire can highlight
concerns that caregivers may feel embarrassed about discussing openly. Creating an efficient
workflow in a busy office can allow time during the appointment to review or elicit more
detailed responses and shed light on unmet needs.
If the primary cause of the fussiness is a mismatch of temperament between the infant and the
caregiver, the pediatrician should validate the caregivers' frustrations and help caregivers explore
positive responses. They should help caregivers set realistic expectations of normal infant
behaviors, including hunger and sleeping cues. For example, excessive crying may be the result
of inadequate sleep: an infant who naps in a swing may actually lack the amount of quiet sleep
necessary for adequate rest. Separation anxiety may be another cause of fussiness with some
infants crying more when they feel safe. Explaining when this is normal and encouraging
consistency among caregivers can help ease transitions.

The pediatrician can also help families by (modified from Gleason):

1. Celebrating strengths: Identifying. praising, and encouraging current parenting skills that
are positive and constructive
2. Engaging the family: Validating caregivers concerns and finding small, achievable goals
upon which both the family and clinician can agree
3. Promoting safety: Identifying and addressing possible safety risks such as firearms,
medications, or unsafe conditions in the home
4. Addressing basic needs: Identifying food, housing, or financial insecurity referring to
appropriate community resources
5. Supporting caregivers' mental health: Discussing how caregivers are coping with
parenthood and encouraging them to seek help
6. Reframing behaviors: Explaining at their child is not being intentionally disruptive, but
expressing a need for emotional support
7. Encouraging consistency all caregivers: If possible. clinicians should try to communicate
with other caregivers to discuss the importance of maintaining a uniform set of positive
responses to disruptive behaviors
8. Encouraging positive interactions: Explaining the importance of spending time in positive
experiences such as play so that not all experiences seem negative
9. Encouraging healthy habits: Instituting a regular schedule with active interactions instead
of passive activities such as screen time
10. Monitoring over time: Continuing to ask how child-caregiver interactions are changing as
the child grows
11. Referring as appropriate to parenting programs, adult and child psychologists, and/or
early intervention
Prevention is key. Awareness of the great importance of the interrelationship between the
Infant's and caregiver’s temperament empowers the pediatrician to provide proactive preventive
anticipatory guidance to help families nurture themselves and allow for healthy relationships and
communities to grow
PREP Pearls
• Infants and parents who demonstrate emotional disturbances should both be referred to
early intervention and mental health services
• Observing attachment patterns and using screening tools can help clinicians develop an
individualized plan for families struggling with their infant’s temperament.
• Any concern for child maltreatment should be promptly addressed and those families
referred to child protective services.
ABP content Specifications(s)

• Understand the variations in temperament in infants and counsel parents appropriately
Suggested Readings
• Promoting healthy development. Bright Futures: Guidelines for Health Supervision of
Infants Children and adolescents 4th ed. 2017:77-113
• Promoting mental health Bright Futures: Guidelines for Health Supervision of Infants
Children and adolescents 4th ed; 2017:115-150
• Symptoms of emotional disturbance in young children. American Academy of Pediatrics
Textbook of Pediatric Care. 2nd ed; 2017;1627-1636.

Question 46
A 15-year-old adolescent girl is seen for a preparticipation examination. She has participated in
gymnastics for the past 8 years and has aspirations to compete at the state championship meet
this year. She has no significant past medical or family history and no concerns. A
comprehensive review of systems is unremarkable except that she has not yet achieved
menarche. She reports eating a healthy diet with 3 meals per day. She has a temperature of 37
°C, heart rate of 60 beats/min, and blood pressure of 102/64 mm Hg. Her weight is 36.8 kg (<5th
percentile), height is 149 cm (<5th percentile; z score is -2) and body mass index is 16.6 kg/m2
(7th percentile). She appears fit and muscular. Her sexual maturity rating is 3 for breast and pubic
hair development. The remainder of her physical examination findings are unremarkable. A bone
age radiograph is read as 12 years.

Laboratory Test Result Reference Range
Luteinizing hormone 1.2 mIU/mL (1.2 IU/L) (For SMR 3, 0.1-12 mIU/mL
Follicle-stimulating hormone 2.4 mIU/mL (2.4 IU/L) (For SMR 3, 1.5-12.8 mIU/mL)
Estradiol 25 pg/mL (91.8 pmol/L) (For SMR 3, 7-60 pg/mL)
Thyroid-stimulating hormone 0.7 mIU/L (0.5-4.8 mIU/L)
Free thyroxine (FT4) 0.9 ng/dL (11.6 pmol/L) (0.93-1.7 ng/dL)
Prolactin 8 ng/mL (8 µg/L) (3-24 ng/mL)
Hemoglobin 12.2 g/dL (122 g/L) (12-15.5 g/dL)
Comprehensive metabolic panel Normal for age
Erythrocyte sedimentation rate 3 mm/h
Tissue transglutaminase <1.2 U/mL <4 U/mL
Of following, the MOST likely cause of this adolescent's amenorrhea is

A. celiac disease
B. constitutional delay of puberty
C. hypothyroidism
D. negative energy balance

Correct Answer: D
The adolescent described in the vignette is an otherwise healthy athlete with primary
amenorrhea, the absence of menarche by age 15 years. The most likely cause of her primary
amenorrhea is negative energy balance. Her energy intake, which may be normal for age, is not
adequate to meet the increased demands of her physical activity. Her low body mass index (7th
percentile), low-normal hemoglobin level, and low free thyroxine (FT4) level are all consistent
with negative energy balance, The low FT4 level with normal thyroid-stimulating hormone level
is consistent with euthyroid sick syndrome, a physiologic adaptation to conserve energy. Her
gonadotropin and estradiol levels, although within the reference range for her sexual maturity
rating, are low for age. A luteinizing hormone level of 0.3 mIU/mL or greater and an estradiol
level of at least 20 pg/mL usually indicates the onset of puberty. The adolescent's low
gonadotropin and estradiol levels for age also represent a physiologic adaptation to negative
energy balance in this clinical context. Her Primary amenorrhea can thus be classified as
functional hypogonadotropic hypogonadism.
Factors contributing to negative energy balance in athletes include a high training load,
inadequate caloric intake, and psychological stress resulting from the drive or pressure to do
well. The inadequate caloric intake may or may not be intentional. Participants in activities such
as gymnastics and ballet, if there is a perceived advantage to a pre-pubertal or thin appearance,
are at risk for maintaining a negative energy balance. Treatment should focus on achieving a
positive energy balance, often by increasing caloric intake, as athletes may not want to decrease
training, and managing stress.
Delayed puberty is defined as lack of breast development in girls by age 13 years and lack of
testicular growth to 4 mL in volume (2.5 cm in length) by age 14 years in boys. Delayed puberty
in girls can also manifest as primary amenorrhea, the lack of menses by age 15 years.
Delayed puberty may be classified as normal variant constitutional delay, functional

hypogonadotropic hypogonadism, permanent hypogonadotropic hypogonadism, and
hypergonadotropic hypogonadism. Constitutional delay is the most common cause of delayed
puberty, especially in boys. Those with constitutional delay often preset with short stature and
are otherwise healthy. There is often a family history of constitutional delay ("late bloomers").
Bone age is delayed and catch-up growth is achieved with a growth spurt at a later age, it is a
diagnosis of exclusion. Constitutional delay of puberty is not the preferred response for the girl
in the vignette because her delayed puberty can be better explained by a negative energy balance.
Functional hypogonadotropic hypogonadism is the next most common cause of delayed puberty.
Causes include chronic systemic illness, eating disorders, and excessive exercise. Delayed
puberty caused by hypothyroidism and celiac disease would be classified as functional
hypogonadotropic hypogonadism. These are not the preferred responses for the girl in the
vignette given her unremarkable review of systems and lack of other signs or symptoms of
thyroid or gastrointestinal disease.
Permanent hypogonadotropic hypogonadism can be isolated, as in Kallmann syndrome, or

associated with other pituitary hormone deficiencies. Anosmia or hyposmia is a feature of
Kallmann syndrome.
Hypergonadotropic hypogonadism is the result of primary gonadal failure. Causes include

Turner syndrome, Klinefelter syndrome, and toxicity to the gonads from radiation or alkylating
agents as cancer therapies.
The evaluation of an adolescent with delayed puberty should include a comprehensive history
and physical examination focusing on indicators of potential causes of the delayed puberty. First-
line laboratory testing includes gonadotropin (luteinizing and follicle-stimulating hormones) and
estradiol (in girls) or testosterone (in boys) levels. Additional testing should be directed by the
history and physical examination findings. A bone age radiograph may be helpful especially if
short stature is present since it can predict catch-up growth. A delayed bon age, however, may be
present with any cause of delayed puberty. Bone age correlates better with pubertal status than
chronological age.
The gonadotropin levels provide important information for narrowing the differential diagnosis.
Low levels may be consistent with constitutional delay of puberty, functional hypogonadotropic
hypogonadism, or permanent hypogonadotropic hypogonadism. Levels are usually very low with
permanent hypogonadotropic hypogonadism. High gonadotropin levels signify
hypergonadotropic hypogonadism. Further testing may be done based on the initial gonadotropin
levels to narrow the differential diagnosis.
PREP Pearls
• Delayed puberty is defined as lack of breast development in girls by age 13 years or lack
of menses by age 15 years (primary amenorrhea) and lack of testicular growth to 4 mL in
volume (2.5 cm in length) by age 14 years in boys.
• Delayed puberty may be classified as normal variant constitutional delay, functional
hypogonadotropic hypogonadism, permanent hypogonadotropic hypogonadism, and
hypergonadotropic hypogonadism.
• Gonadotropin levels (luteinizing and follicle-stimulating hormones) provide important
information for narrowing the differential diagnosis of delayed puberty.

• Recognize the clinical features associated with a delay in sexual maturation of various
causes
• Understand the relationship between bone age and chronologic age
• Plan the appropriate evaluation of an adolescent boy or girl who has no signs of the onset
of puberty
• Identify the causes of delayed puberty
Suggested Readings
• Elite athletes and pubertal delay. Minerva Pediatr. doi:10.23736/S0026-4946.17.05044-7
• Delayed puberty. Pediatr Rev. doi:10.1542/pir.31-5-189
• Puberty: Normal and abnormal. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017:1540-1544.
• Pubertal Development. Pediatr Rev, doi:10.1542/pir.2015-0065

Question 47
A fully vaccinated, 5-year-old boy with a history of recurrent middle ear infection is admitted to
the hospital from the emergency department. He has had fevers, headaches, and abdominal pain
for 2 days, and his mother notes that he is lethargic. On arrival at the emergency department, his
temperature is 40 °C, respiratory rate is 20 breaths/min. heart rate is 110 beats/min, blood
pressure 90/55 mm Hg, and oxygen saturation is 98% on room air. A complete blood cell count
is obtained and a lumbar puncture performed.
Laboratory Test Results

White blood cell count 25.000/µL (25.0 x 109/L)
Neutrophils 80%
Lymphocytes 10%
Monocytes 10%
Cerebrospinal fluid (CSF) white blood cells 600 cells/µL
CSF red blood cells 20 cells/µL
CSF protein 95 mg/dL (reference range, 5-40 mg/dl)
CSF glucose 25 mg/dL (reference range, 40-80 mg/dL)
CSF Gram stain gram-positive cocci in chains
Abdominal ultrasonography findings show a normal spleen and liver.
On admission, it is noted that he had been treated for meningitis 6 months ago.
Cerebrospinal fluid culture at that time grew a nonencapsulated strain of Streptococcus
pneumoniae. Results of a comprehensive immunologic evaluation, including genetic testing, are
unremarkable. High-resolution computed tomography demonstrates inner ear dysplasia and a
defect in the cribriform plate. The boy's mother asks what can be done to prevent further
episodes of meningitis.
Of the following, the BEST management recommendation for this child is to administer
A. benzathine penicillin prophylaxis, monthly
B. Intravenous immunoglobulin, monthly
C. meningococcal vaccine ACWY and B
D. pneumococcal polysaccharide vaccine

Correct Answer: D
The child in the vignette has a congenital defect of the cribriform plate, which can predispose
him to recurrent meningitis. Recurrent bacterial meningitis can be associated with a congenital or
acquired disruption of skull integrity resulting in a cerebrospinal fluid leak. Congenital
abnormalities of the anterior fossa, temporal bone, and spinal defects, as well as fractures or a
neurosurgical procedure involving the nasal or otic bones place children at risk for recurrent
bacterial meningitis, Particularly from Streptococcus pneumoniae, Children at high risk for
invasive pneumococcal infection benefit from the administration of pneumococcal
Polysaccharide vaccine 23 valent, 8 weeks after their last conjugate pneumococcal
13 vaccine. Item C47 lists indications for the pneumococcal polysaccharide vaccine.
Recurrent meningococcal infection is associated with deficiency of complement membrane

attack complex (C5-C9), and this child has a normal spleen seen on ultrasound. Therefore, the
meningococcal vaccine ACWY and B is not indicated for this child. Monthly intravenous
immunoglobulin infusions can be beneficial for a child with an immunodeficient state. Penicillin

prophylaxis has not been shown to reduce the risk of recurrent meningitis and is associated with
development of antibiotic resistance.
Recurrent invasive pneumococcal disease occurs in up to 2.5% of patients who survive their
initial episode. Recurrence is higher in children younger than 5 years with sickle cell disease, and
children with HIV. The risk is also higher in children with hemoglobinopathies, asplenia,
nephrotic syndrome, and primary immune deficiencies, as well as those receiving
immunosuppressive therapy. Children with a cochlear implant are at increased risk of
pneumococcal meningitis.
Streptococcus pneumoniae is a gram-positive, catalase-negative, facultative anaerobic bacterium.

On microscopy, it appears as lancet-shaped diplococci in short chains. On blood agar, the
colonies are a hemolytic (partial) and can be identified presumptively from susceptibility to an
optochin test. S pneumoniae is initially acquired in the first few months of age, at a mean age of
6 months. Nasopharyngeal carriage varies based on age and geographic region. Factors
associated with higher carriage rates include age less than 2 years, being in childcare, crowding,
winter months, and parental smoking. Since the widespread use of pneumococcal 7-valent
conjugate (available in 2000) and pneumococcal 13-valent conjugate vaccine (available in 2010)
the carriage of vaccine serotypes has declined; in contrast, carriage of non-vaccine serotypes has
increased.
Clinical diseases caused by S pneumoniae include invasive infections such as meningitis,

bacteremia, severe/complicated pneumonia, orbital cellulitis, and noninvasive infections otitis
media, sinusitis, and conjunctivitis. Less frequently, S pneumoniae causes endocarditis,
pericarditis, peritonitis, skin and soft tissue infections, septic joint, and osteomyelitis.
Children with meningitis should be started on empiric vancomycin and a third-generation

cephalosporin (e.g., cefotaxime, ceftriaxone) until susceptibility data are available. Vancomycin
should be discontinued if the species identified is susceptible to ß-lactams (e.g., penicillins or
cephalosporins), Noninvasive infection (e.g., acute otitis media, sinusitis, or uncomplicated
community-acquired pneumonia) can be treated with oral amoxicillin.
PREP Pearls
• A congenital or acquired defect of the skull resulting in a cerebrospinal fluid leak
predisposes a child to recurrent pneumococci meningitis.
• Children at risk for invasive pneumococcal disease should receive pneumococcal
polysaccharide vaccine in addition to the routine conjugate pneumococcal vaccination
series
• Streptococcus pneumoniae is responsible for infections ranging from noninvasive
infections (e.g., acute otitis media, sinusitis, conjunctivitis, uncomplicated community-
acquired pneumonia) to invasive infections (e.g., meningitis, orbital cellulitis, bacteremia,
complicated pneumonia [empyema, necrotizing]).

• Recognize the clinical features associated with Streptococcus pneumoniae
• Understand the epidemiology of Streptococcus pneumoniae infection
• Plan appropriate management for a patient with Streptococcus pneumoniae infection
Suggested Readings
• Streptococcus pneumoniae pneumococcal infections. Red Book: 2021-2024 Report of the
Committee on infectious Diseases. 32nd ed; 2021:717-726.
• Streptococcus pneumonia. Principles and Practice of Pediatric Infectious Diseases. 5th
ed. 2018:737-746.
• Dynamics ot nasopharyngeal colonization by Streptococcus pneumoniae. Pediatr infect
Dis J. doi:10.1097/00006454-199907000-00016
• Pneumococcal Infections. Pediatr Rev, doi:10.1542/pir.35-7-299
• High rates of multiple antibiotic resistance in Streptococcus pneumoniae from healthy
children living in isolated rural communities: association with cephalosporin use and
intrafamilial transmission. Pediatrics. doi:10.1542/peds.108.4.856

Question 48
A 15-year-old female lacrosse player is seen in the office with a 4-week history of hip pain. She
reports gradual onset of pain in the anterior hip and medial thigh. Her pain was initially present
only during running. With continued training, it became constant throughout the day, and she
was limping at school. She rested for 2 weeks and the pain resolved, but recurred upon her return
to lacrosse practice. She reports no trauma or recent change in activity. She is a multisport athlete
who also plays soccer and basketball.
On physical examination, she has minimal pain to palpation over her anterior hip. Pain is
reproduced with the log roll test and passive flexion and internal rotation of the hip. The girl has
pain when jumping and hopping. Strength and neurovascular assessments are normal. She
reports mild pain with walking, but her gait is normal. Radiographs of the hip and pelvis are
normal.
Of the following, the BEST next management step for this adolescent is to
A. advise her to rest and start physical therapy
B. fit her with crutches and perform hip magnetic resonance imaging
C. perform radiography of the lumbar spine
D. start a nonsteroidal anti-inflammatory medication

Correct Answer: B
The history and physical examination findings for the adolescent in the vignette are consistent
with a femoral neck stress fracture. Femoral neck stress fracture is a “can’t miss” diagnosis, with
potentially devastating consequences if recognition and treatment are delayed. Radiography has
poor sensitivity for these injuries, and magnetic resonance imaging (MRI) is needed for
definitive diagnosis. Pain with ambulation is associated with risk for progression of the injury to
a complete fracture of the femoral neck. She should be advised to avoid weight-bearing activities
and use crutches until a definitive diagnosis is obtained.
Femoral neck stress fractures result from an accumulation of bony microtrauma, and are most
commonly seen in girls participating in running sports. These injuries typically result from a
combination of factors that should be explored during the history of any suspected stress fracture
(Item C48A).
Item C48A
Femoral neck stress fractures may occur on the medial or lateral side (Item C48B and Item
C48C), and should be considered in any young athlete with examination findings that suggest an
interarticular process, such as described in the adolescent in the vignette. Some of these findings
are shown in the video in Item C48D. Because of the shape of femoral neck, weight-bearing
activities compress the fracture with medial injuries and distract (or pull apart) the fracture with
lateral injuries.
Medial femoral neck fracture on coronal T1 image

Medial femoral neck stress fractures are much less likely to progress to complete hip fractures
compared with lateral injuries. Children with medial femoral neck stress fractures should
continue to crutch use until daily activities are pain-free. They may start non-weight-bearing
exercise (i.e., cycling or swimming) and physical therapy as soon as tolerated. When strength
and range of motion are symmetric on the injured and non-injured side, gradual return to low-
impact, and subsequently high-impact activity may occur as long as the child remains pain-free.
Magnetic resonance imaging findings can be useful in estimating time to return to sport. For
young healthy athletes, this time may vary from 12 to 28 weeks.
Those diagnosed with lateral femoral neck stress fractures should be kept strictly non-weight
bearing and be referred to an orthopedic surgeon for screw fixation. Early surgical intervention
is preferred for these injuries to reduce the risk for subsequent avascular necrosis of the femoral
head.
Physical therapy, rest, and a brief course of nonsteroidal anti-inflammatory medications are
appropriate initial treatments for many sport-related injuries.
However, in athletes with history and/or physical examination findings that suggests an
interarticular process in the hip, imaging to determine a definitive diagnosis is needed before
progressing with treatment.
Hip pain extending into the medial thigh may result from a lumbar radiculopathy of L1, L2, or
L3. For children who have back pain in addition to hip or leg pain or those whose symptoms are
not specifically provoked on hip examination, it would be reasonable to obtain lumbar
radiographs. The physical examination findings for the adolescent in the vignette strongly
suggest hip-related pathology, therefore, lumbar imaging is not indicated.
PREP Pearls
• Femoral neck stress fracture should be considered in female athletes with groin pain.
• Children with suspected femoral neck fractures should not bear weght on the hip
• Radiographs cannot “rule out” a stress fracture; magnetic resonance imaging is indicated
for a definitive diagnosis.
MOCA-Peds Objective
• Know the differential diagnosis of hip pain

• Formulate a differential diagnosis of a painful hip

Suggested Readings
• Stress fractures of the pelvis and legs in athletes: a review Sports Health,
doi:10.1177/1941738112467423
• Sports musculoskeletal injuries. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed, 2017: 2675-2686
• Hip and spine Injuries in the Young Athlete. Sports Medicine the Pediatric office. 2nd ed,
2018; 139-160
• Femoral neck stress fractures in sport: A current concept, Sports Med Int Open,
doi:10.1055/s-0043-103946

Question 49
A 9-month-old boy is seen for a routine health supervision visit. His parents report no concerns.
He is eating well, has appropriate weight gain, and is meeting appropriate developmental
milestones. His past medical history is significant for premature birth at 34 weeks' gestation and
a ventricular septal defect, status post repair. He has no residual cardiopulmonary disease. He
required only short-term nutritional support and did not require vascular access in the neonatal
intensive care unit. His family history is negative for early myocardial infarction, hypertension,
or renovascular disease. His physical examination findings are normal. The office does not
routinely measure blood pressure in very young children. The physician reviewing the discharge
summary for recommendations regarding blood pressure monitoring for this child.
Of the following, the BEST recommendation for this child is that he

A. does not require blood pressure measurement because he is younger than 1years of age
B. does not require blood pressure measurement given his benign family history
C. does require blood pressure measurement because he was born prematurely
D. does require blood pressure measurement because of his congenital heart disease

Correct Answer: D
The infant in the vignette’s history of congenital heart disease warrants routine blood pressure
monitoring at this young age.
The American Academy of Pediatrics Clinical Practice Guideline for Screening and
Management of High Blood Pressure in Children and Adolescents was last updated in 2017
(Flynn JT et al). The guideline recommends that children should have a blood pressure
measurement at each annual health supervision visit beginning at 3 years of age. Routine blood
pressure measurement should begin at an earlier age in patient populations with specific
conditions or circumstances including
• Obesity
• Renal disease
• Diabetes
• Aortic arch obstruction
• Those taking medications known to cause hypertension
• History of prematurity <32 weeks, small for gestational age, very low birth weight,
neonatal completion requiring intensive care or umbilical artery line
• Congenital heart disease (repaired or unrepaired)
• Recurrent urinary tract infections, hematuria, or proteinuria
• Renal disease or urologic malformations
• Family history of congenital renal disease
• Solid-organ transplant
• Malignancy or bone marrow transplant
• Treatment with drugs known to raise blood pressure
• Other systemic illnesses associated with hypertension (e.g., neurofibromatosis, tuberous
sclerosis, sickle cell disease)
• Evidence of increased intracranial pressure
In a child with an elevated blood pressure, it is important to take several measurements prior to
making an official diagnosis of hypertension. There can be a lot of variability between
measurements, even within the same office visit. For this reason, blood pressure measurements
should be repeated both within one office visit and several visits prior to making a diagnosis of
hypertension. The blood pressure measurements may be taken by oscillometric or auscultatory
methods. The right arm should be used unless there is known atypical aortic arch anatomy (i.e.,
aberrant right subclavian artery). An appropriately sized cuff should be used for each child,
which means that a pediatric office needs to have a range of sizes to accommodate neonates to
adolescents with obesity. The mid-arm circumference can help determine the correct cuff size.
The mid-arm circumference is measured at the midpoint between the acromion of the scapula
and olecranon of the elbow, when the shoulder is in the neutral position and the elbow is flexed
90 degrees. The cuff bladder should encircle 80% to 100% of the mid-arm circumference.

PREP pearls
• Routine blood pressure screening should be started at age 3 for routine health screening
or sooner in those with a condition that increases the risk for hypertension.
• Blood pressure measurement should be taken with the appropriately sized blood pressure
cuff.
• The cuff bladder should be the proper length to encircle 80% to 100% of the mid-arm
circumference, which is defined as the midpoint between the acromion of the scapula and
olecranon of the elbow, when the shoulder is in the neutral position and the elbow is
flexed 90 degrees.

• Understand when to screen for an increased blood pressure and how to interpret the
results
• Understand the appropriate technique, including appropriate cuff size, for measuring
blood pressure
Suggested Readings
• Clinical practice guideline screening and management of high blood pressure in children
and adolescents. Pediatrics, doi:10.1542/peds.2017-1904
• Clinical implications of the revised AAP pediatric hypertension guidelines. Pediatrics,
doi:10.1542/peds.2018-0245

Question 50
A 12-year-old, previously healthy, fully immunized girl is seen for a fever of up to 39 °C,
worsening nasal congestion, and frontal headache after a 10-day viral upper respiratory infection.
Her mother has treated the fever and headache symptomatically for 2 days without resolution.
On physical examination the patient appears mildly ill and has tenderness to deep palpation over
the frontal and maxillary areas of the face. There is thick mucoid discharge in the nasopharynx.
Her neck has full flexion without nuchal rigidity. Her lung fields are clear, and cardiovascular
examination findings are normal. The remainder of the physical examination findings are normal
Of the following, the MOST appropriate next intervention for this girl is to
A. begin amoxicillin-clavulanic acid treatment for 10 days
B. obtain computed tomography scans o the sinuses
C. perform a nasopharyngeal swab for viral antigen detection
D. refer to the emergency department for evaluation

Correct Answer: A
The girl in the vignette has acute sinusitis following a viral upper respiratory infection (URI).
This is the most common presentation for acute sinusitis in children. Acute or subacute sinusitis
should be considered for children who develop new fever and worsening congestion after initial
improvement, or whose viral URI symptoms do not resolve after 10 to 14 days, The American
Academy of Pediatrics clinical practice guidelines for diagnosis and management of acute
bacterial sinusitis recommend treatment with high-dose amoxicillin or amoxicillin-clavulanate as
the most appropriate intervention. In studies of cultures obtained via sinus puncture, the most
commonly recovered bacterial organisms are Streptococcus pneumoniae and Hemophilus
influenzae, both susceptible to amoxicillin-clavulanate. Although these studies were performed
many years ago, amoxicillin or amoxicillin-clavulanate remain the most appropriate antibiotic
treatment. For those who are allergic to amoxicillin, trimethoprim-sulfamethoxazole or a second-
generation cephalosporin are acceptable alternative.
The diagnosis of sinusitis is clinical, and plain radiography of the sinuses has no benefit in
making the diagnosis. Computed tomography (CT) should be reserved for recurrent or
complicated sinusitis that requires surgical intervention. Children with repeated episodes of acute
sinusitis may be considered for CT examination in the context of an otolaryngology consultation,
but not as part of the evaluation of an isolated episode. A nasopharyngeal swab for viral antigen
identification would not be helpful in determining treatment for this patient. Likewise, bacterial
culture of the nasopharyngeal discharge will be contaminated by oropharyngeal flora and does
not necessarily represent the flora of the sinuses. Cultures, other than at the time of surgery, are
not helpful in the management of uncomplicated upper respiratory infections.
Although the girl in the vignette has a headache and new onset of fever that has not responded to
symptomatic treatment, her examination findings are not suggestive of meningitis. Nothing in
her history or physical examination warrants an emergency department evaluation.
Other factors that predispose children to acute sinusitis include allergic rhinitis, ciliary
dyskinesia, cystic fibrosis, and long-term smoke exposure. All of these are associated with
episodes of mucosal swelling, poor drainage of sinus ostia, mucus stasis, and promotion of
microbial growth. Children with these predispositions are likely to have repeated episodes of
acute sinusitis or even chronic sinus inflammation. Rarely, local factors such as trauma, tumors,
or occult foreign body may underlie repeated episodes of sinusitis. Complications of acute
sinusitis include orbital or intracranial extension of infection with the development of orbital
cellulitis, meningitis, or central nervous system abscess. Direct bone invasion by the infection
can lead to maxillary or frontal osteitis.
Adjunctive therapies for relief of nasal congestion and promotion of sinus drainage have not
been consistently beneficial in children with acute sinusitis. Systemic antihistamines and/or
decongestants, topical decongestants and topical nasal steroids are frequently prescribed, but
without convincing relief. Large-volume saline lavage of the nasal passage (4 to g oz per
treatment), a staple of treatment by otolaryngologist, can help with mechanical removal of
secretions, reduction in postnasal drainage, and improvement in nasal congestion

PREP Pearls
• Acute bacterial sinusitis is diagnosed clinically, and imaging studies are not needed.
• Amoxicillin-clavulanic acid is the treatment of choice for acute sinusitis.
• Viral upper respiratory tract infection and other conditions promoting nasal congestion
and obstruction of the sinus ostia are the most frequent predisposing conditions for acute
sinusitis.

• Plan the appropriate management of acute sinusitis
• Recognize complications associated with acute sinusitis
• Plan the appropriate diagnostic evaluation of acute sinusitis while recognizing the
limitations of some modalities
Suggested Readings
• Acute bacterial sinusitis in children. Pediatr Rev, doi:10.1542/pir.34-10-429
• National trends in visit rates and antibiotic prescribing for children with acute sinusitis.
Pediatrics, doi: 10.1542/peds.2010-1340
• Evidence for the diagnosis and treatment of acute uncomplicated sinusitis in children:
Pediatrics, doi:10.1542/peds.2013-1072.
• Sinusitis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. 2017;
2652-2655
• Clinical practice guideline diagnosis and management of acute bacterial aged 1 to 18
years. Pediatrics, doi:10.1542/peds.2013-1071

Question 51
The parents of a 14-year-old adolescent girl are concerned about changes in their daughter's
behavior. Over the past 6 months she has been spending more time with a small group of “music
friends” from school. The entire family is very involved in competitive soccer. The girl's father
often officiates as a referee at games. The family participates in many evening practices and
weekends at tournaments, and enjoys the camaraderie and friendship of the other soccer families.
The girl's parents are worried because they do not know her new friends or their parents. The
girl seems preoccupied with text messages and talks to her new friends while alone in her
bedroom. She has developed an interest in trendy clothes and hair styles. Recently she insisted
on auditioning for a role in a school musical rather than play in a soccer tournament. Her
academic performance continues to be excellent.
The girl’s examination findings are unremarkable. During the private portion of the health
supervision visit the girl says she still likes soccer; however, her “music friends” are 'funnier”
and share her interest in theater. She is interested in dating and has not been sexually active. She
has no history of substance use. Standardized screens for depressions and anxiety are negative.
Of the following, the MOST appropriate statement to include in a discussion with parents about
their daughter is that
A. her need for conformity with a chosen peer group will continue to intensify over the next
few years
B. her peer group participation fosters the development of autonomy and self-identity
C. she needs clear, consistent, and non-negotiable rules for social activities
D. she should be prohibited from associating with this new group of friends

Correct Answer: B
Peer group participation fosters the development of adolescent autonomy and self-identity.
Separation from the family may evoke parental feelings of loss and rejection. Parents may fear
that the prominence of a peer group in their adolescent’s life will erode internalized family
values. They may also fear that their adolescent will be vulnerable to the negative effects of peer
pressure and the consequences of risky behaviors. Parental angst may be alleviated by
recognition of the positive role of peer groups in the psychosocial development of their child.
Peer group participation provides adolescents with space and time to be independent. Peer
relationships provide opportunities for adolescents to develop critical social skills for adult life
such as communication, conflict resolution, and decision-making. Peer pressure can have a
positive impact on the development of new skills or support expression of talents and attributes
that enhance an adolescent's self-esteem.
An authoritative (balanced) parenting style is associated with better behavioral outcomes than
authoritarian parenting styles. Authoritarian parents set clear, consistent rules and consequences
that are steadfast with no opportunity for the adolescent to discuss or negotiate modifications. In
contrast, authoritative parents set clear, consistent rules and consequences that are
developmentally appropriate while allowing for discussion and negotiation. Authoritative parents
listen and engage the adolescent in the process of assessing choices and making decisions,
thereby equipping the adolescent with skills to respond to peer pressure. They support peer group
participation while effectively monitoring for safety. Although parents may feel they have a
diminished role in their adolescent's daily life, well established family values and family
connectedness will continue to exert substantial influence on adolescent behavior.
Adolescent peer group affiliations include large groups (crowds) such as the “popular group”,
“athletes/jocks”, “academics”, “deviants”, “hybrids”, (socializing with >1group), and “loners”
(no crowd affiliation). These generalized groups have been linked to differing risky behaviors.
Knowing with which group an adolescent aligns may help inform the likelihood of risky
behaviors; presumptions and overgeneralization should be avoided. Learning about an
adolescent’s identification with a smaller subgroup that has unique characteristics may provide a
better understanding of an adolescent’s friendships, special interests, behaviors, and risks.
Smaller group affiliations, as exists for the adolescent in the vignette, reflect interests and values
shared by the adolescent and the group. These smaller subgroups are characterized by having a
leader(s) and tiers (ranks) of members. The pressure to conform is highest in these smaller
subgroups or cliques.
The need for conformity with peers is strongest during early adolescence and wanes during later
adolescents and young adulthood. Lack of affiliation with a peer group should raise concern for
psychosocial problems. Typically, a change in peer group affiliation is gradual. A sudden change
in peer group or severing of former good friendships is a red flag for a psychosocial problem
such as depression, anxiety, mood disorder, bullying, or high-risk behaviors such as substance
abuse and risky sexual behaviors. During early adolescence, peer group are predominantly same
sex, whereas in midadolescence, a mixed-sex peer group evolves, with a heightened interest in
dating. Later adolescence and young adulthood are characterized by mature interpersonal and
reciprocal friendships and dating relationships. The importance of the peer group is diminished
as relationships with parents often regain prominence in adulthood.
PREP pearls
• Peer group participation facilitate autonomy and fosters development of an adolescent's
self-identity.
• Peer relationships provide opportunities to develop life skills.
• The need for conformity with peer groups is strongest during early adolescence and
wanes during later adolescence and young adulthood

• Recognize the importance of a peer group in a young adolescent's separation from the
family
• Identify typical characteristics of a young adolescent's peer group while understanding
the influence of that group on behaviors
• Understand the importance of accepting an adolescent's separation from the family, and
the role re-adjustments that may be required
Suggested Readings
• Friendship and peers. Reaching Teens. 2nd ed. American Academy of Pediatrics;
2020.113-124
• Promoting the health of adolescents. American Academy of Pediatrics Textbook of
Pediatric Care 2nd ed.187-190
• Counseling parents of adolescents. American Academy of Pediatrics Textbook of
Pediatric Care 2nd ed. 1149-1151.
• Adolescent psychosocial, social, and cognitive development. Pediatr Rev. doi:
10.1542/pir.34-8-354

Question 52
A 7-year-old boy is brought to the emergency department for evaluation of a seizure. His mother
heard a thud in his room at 6 AM and found him lying on the floor next to his bed. She noted that
he had generalized shaking for 3 minutes, after which he was very tired and confused. In the
emergency department, he is groggy but arousable. His neurologic examination findings are
normal. A computed tomography scan of the head as well as results from a complete blood cell
count, comprehensive metabolic panel, and serum and urine toxicology are all normal. On
further questioning, the boy recalls several self-resolving episodes or waking in the middle of the
night unable to talk, with associated drooling and facial twitching.
Of the following, the boy's MOST likely diagnosis is

A. benign rolandic epilepsy
B. childhood absence epilepsy
C. juvenile myoclonic epilepsy
D. Panayiotopoulos syndrome

Correct Answer: A
In this vignette, the boy’s clinical presentation is most consistent with benign rolandic epilepsy
(BRE), also known as benign childhood epilepsy centrotemporal spikes. Benign rolandic
epilepsy is a common childhood focal motor epilepsy syndrome, typically presenting between
the ages of 5 and 10 years. The syndrome is characterized by infrequent brief focal motor
seizures that occur predominantly in sleep. Due to the neuro anatomic location of the rolandic
region near the central sulcus, the clinical presentation of seizure is typically described as facial
and arm twitching, speech arrest with accompanying oropharyngeal guttural sounds, and
hypersalivation with preservation of consciousness. Generalized tonic-clonic seizures may occur
in a subset of patients. Seizures spontaneously remit in adolescence, usually by 16 years of age.
The diagnosis of BRE is confirmed by electroencephalography demonstrating biphasic

independently bilateral centrotemporal spikes on a normal background, often identified in light
sleep. Neuroimaging findings are normal. Anti-epileptic medication is offered when seizures are
frequent; initiation requires shared decision-making with the family regarding potential adverse
effects of medication balanced against seizure frequency, the impact on the child, and parental
concern. Common antiepileptic medications options include oxcarbazepine and levetiracetam.
Medication-resistant epilepsy can occur in a small subset of the population with BRE that evolve
into an encephalopathy, such as Landau-Kleffner syndrome, or continuous spike and slow wave
during sleep encephalopathy.
Childhood absence epilepsy is an idiopathic generalized epilepsy that occurs in school-age

children and is characterized by absence seizures (brief lapses of consciousness) that last 10 to
15 seconds and multiple times in a day. Electroencephalography demonstrates a classic pattern of
3 Hz spike and slow wave discharges on a normal background, provoked by hyperventilation.
Generalized tonic-clonic seizures can occur in a subset of children. Anti-epileptic medications
include ethosuximide, valproic acid, and lamotrigine. The likelihood of seizure remission is
favorable in children with onset at a younger age.
Juvenile myoclonic epilepsy is an idiopathic generalized epilepsy that occurs in late school-age
and adolescent children and is characterized by a triad of 1) myoclonic seizures in early morning,
2) generalized tonic-clonic seizures, and 3) absence seizures. Myoclonic seizures often precede
the development of generalized tonic-clonic seizures but may not be recognized as abnormal by
the patient or family until a generalized tonic-clonic seizure occurs. Seizures may be precipitated
by alcohol, stress, and sleep deprivation. Unlike childhood absence epilepsy and BRE, juvenile
myoclonic epilepsy persists into adulthood.
Panayiotopoulos syndrome is a childhood focal epilepsy that typically presents between 1 and 4
years of age. It is characterized by infrequent autonomic seizures. Seizure semiology may
include ictal vomiting with preservation of consciousness, mydriasis, pallor, and cyanosis;
seizures often occur out of sleep. It is common for seizures to be prolonged, evolving into status
epilepticus with hemi clonic or clonic movements; symptoms are frequently mistaken for
encephalitis on initial presentation. The condition is self-limiting, with a majority of children
outgrowing it within few years of onset.

PREP Pearls
• Benign rolandic epilepsy is a common childhood focal epilepsy with seizures
characterized by preservation of consciousness, speech arrest, hypersalivation, and facial
and arm clonic movements. Some children will have generalized tonic-clonic seizures
• Electroencephalography in benign rolandic epilepsy demonstrates biphasic independently
bilateral centrotemporal spikes on a normal background. Often identified in light sleep.
• Benign rolandic epilepsy is typically self-limited, with the majority of children
outgrowing the condition in adolescence.

• Recognize the clinical findings associated with rolandic epilepsy, and manage
appropriately
Suggested Readings
• Seizure disorders. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.
2599-2617.
• Pediatric seizures. Pediatr Rev. doi:10.1542/pir.34-8-333.

Question 53
A 5-month-old infant is brought to the emergency department with a 6-day history of poor
feeding, emesis, and diarrhea. The mother reports that the infant was previously healthy and had
a normal health supervision visit at 4 months’ age. Over the past 5 days, the infant has had a
progressive increase of watery diarrhea, up to 10 episodes per day, and emesis after nearly every
feeding. On physical examination, the infant appears lethargic, limp, and pale. The anterior
fontanelle is sunken, and there are no tears in spite of a weak, soft cry. The oral mucosa is dry,
lips are cracked, and tongue is membrane-coated and dry. The neck is supple and lungs are clear
with no rales. Sl and S2 are normal with no murmur; the heart rate is 195 beats/min; and the
pulse is thready and palpable, the abdomen is scaphoid, and doughy skin which tents on
examination. The infant is placed on full monitors and despite 2 attempts-by experienced staff,
vascular access cannot be established.
Of the following, the BEST next management step for this infant is to
A. perform arterial puncture for arterial blood gas and electrolyte evaluation
B. place a femoral central venous catheter and bolus isotonic saline
C. place a tibial intraosseous catheter and bolus isotonic saline
D. start chest compressions and cardiopulmonary resuscitation

Correct Answer: C
The infant in the vignette is demonstrating signs and symptoms of hypovolemic shock. The
history provided by the mother is consistent with significant gastrointestinal losses, and the
prolonged duration of illness has resulted in a severely dehydrated infant. End-organ dysfunction
as evidenced by lethargy is ominous sign, and given the severe tachycardia, represents pre-arrest
condition. As such, the immediate priority is to restore intravascular circulating volume and
prevent further deterioration. After failure to rapidly establish intravascular access, the boy in
this vignette should undergo placement of an intraosseous catheter and receive a bolus of
isotonic saline.
Severe dehydration, particularly in those with signs of hypovolemic shock, is treated with rapid
instillation of intravenous fluids until the patient is stabilized and circulating blood volume is
restored. Initial treatment should include 20 mL/kg of isotonic crystalloid (either normal saline or
lactated Ringer solution) by rapid intravenous infusion over 5 to 10 minutes. Other colloid fluids
such as 5% albumin or hydroxyethyl starch are not indicated. Infusions should be repeated with
frequent reassessment of clinical status Including the patient's vital signs, pulse strength,
capillary refill time, mental status, and urine output. In severe dehydration, a total bolus volume
of up to 60 mL/kg of fluid may be required within the first hour. After hemodynamic
stabilization, electrolyte measurement including assessment of serum blood glucose should be
performed because electrolyte derangements and hypoglycemia may need to be addressed
urgently. Hypernatremia, hyponatremia, and hypoglycemia often complicate severe dehydration.
Following guidelines for resuscitation of patients in shock, vascular access is a requisite first step
resuscitation and restoration of intravascular volume. The placement of a peripheral large-bore
intravenous catheter is the preferred method of fluid resuscitation for patients in shock. The
patient in the vignette does not require chest compressions at this time. In addition, although
laboratory evaluations will be required, vascular access should be established before an arterial
puncture is made for laboratory studies. A central venous catheter can be placed urgently, but it
is not the preferred method of administering bolus intravenous fluids during resuscitation; a
central venous catheter requires specific expertise and equipment, and more time to place
compared with other intravascular devices. An intraosseous catheter is recommended when
immediate vascular access is required, and intravenous access may cause undue delay or has
been unsuccessful.
Intraosseous access can be obtained in any large bone with an expected marrow cavity and the
intraosseous space will stay patent even in patients with severe hypovolemic shock. In children,
the sites of placement most often include the proximal tibia, distal femur, and proximal humerus.
Once inserted into the marrow cavity, fluid infusion or medication administration can begin.
Also, a bone marrow aspirate can be used for basic laboratory tests including serum
concentration of electrolytes, dextrose, and blood counts. Finally, the venous, plexus of long
bones drain directly into the central circulation at a rate comparable to central venous access.

PREP Pearls
• In a patient with hypovolemic shock caused by fluid loss, the immediate priority is to
restore intravascular circulating volume and prevent further deterioration.
• After failure to rapidly establish intravenous access with a large bore intravascular
catheter in a patient with hypovolemic shock, the placement of an intraosseous catheter,
and infusion of isotonic saline is indicated.
• Initial treatment of hypovolemic shock from fluid loss is 20 mL/kg of isotonic crystalloid
(either normal saline lactated Ringer solution) by rapid intravenous or intraosseous
infusion over 5 to 10 minutes. In severe dehydration, a total infusion volume of up to 60
mL/kg of fluid may be required within the first hour.

• Recognize the clinical signs of shock due to fluid loss, and manage appropriately
Suggested Readings
• Shock. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed. 2976-2984.
• College of Critical Care Medicine clinical practice parameters for hemodynamic support
of pediatric and neonatal septic shock. Crit Care Med.
doi:10.1097/CCM.0000000000002425
• Septic shock and sepsis-ass organ dysfunction in children. Pediatr crit care Med.
doi:10.1097/CCM.0000000000002198
• Shock. Pediatr Rev. doi:10.1542/pir.31-8-311

Question 54
A 2-year-old boy is brought to the urgent care center for evaluation. He has had a fever, pain
with swallowing or neck movement, and decreased oral intake over the past few days. Today, his
breathing quickened, and his voice became muffled. His temperature is 38.8 °C, heart rate is 130
beats/min, respiratory rate is 45 breaths/min. and oxygen saturation is 96% on room air. He is ill
appearing. He has respiratory stridor and subcostal retractions. He holds his head midline and
refuse, to move his neck or open his mouth widely, so his oropharynx is not well visualized. A
lateral neck radiograph is obtained (Item Q54).
Of the following, the MOST likely pathogen causing this boy’s condition is
A. Moraxella catarrhalis
B. Pseudomonas aeruginosa
C. Streptococcus pneumoniae
D. Streptococcus pyogenes

Correct Answer: D
The boy in the vignette has a retropharyngeal abscess (RPA), a deep infection within the
potential space between the posterior pharyngeal wall and the prevertebral fascia that extends
from the skull base to the posterior mediastinum. Retropharyngeal abscesses are often
polymicrobial. The predominant pathogens are Streptococcus pyogenes (group A Streptococcus);
Staphylococcus aureus; and respiratory anaerobic bacteria, such as Fusobacterium, Pepto
Streptococcus and Prevotella species. Moraxella catarrhalis, Pseudomonas aeruginosa and
Streptococcus pneumoniae are not associated with RPAs.
An RPA typically follows an upper respiratory infection and most often occurs in children ages 2
to 4 years. Children with RPA seen with fever, dysphagia, odynophagia, drooling, neck
stiffness/torticollis, change in voice quality, respiratory distress (e.g., stridor and/or tachypnea),
and trismus. Physical examination may be limited due to pain but is often notable for edema of
the posterior oropharynx and tender, anterior cervical lymphadenopathy. Children with
respiratory compromise due to airway obstruction may appear ill and anxious, be leaning toward
("sniffing position'), and have tachypnea, retractions, and stridor. These children warrant
immediate evaluation by specialists for possible endotracheal intubation and/or surgical
intervention.
The diagnosis of RPA can be made on the basis of history and physical examination. However,
diagnostic imaging may be useful if there is a question about the diagnosis, lack of improvement
after intravenous antibiotics for 48 to 72 hours, or for perioperative management. For patients
without respiratory compromise, the first imaging modality is typically a lateral neck radiograph,
which may reveal edema of the prevertebral soft tissues (Item C54). Contrast-enhanced
computed tomography is the modality of choice for differentiating cellulitis, phlegmon, and
abscess, as well as for operative planning purposes.
Item C54: retropharyngeal abscess
For children without respiratory compromise, it is reasonable to initiate empiric, broad-spectrum,

intravenous antibiotic therapy, such as clindamycin, a second-generation cephalosporin, or a β-
lactamase penicillin, for 24 to 48 hours before determining whether surgical intervention is
necessary. Approximately 60% or infections resolve with solely medical management. For

children who do not respond to this empiric treatment, to cover for methicillin-resistant S aureus,
intravenous Vancomycin or linezolid may be added.
PREP Pearls
• Children with retropharyngeal abscess often have fever, dysphagia, odynophagia,
drooling, neck stiffness/torticollis, change in voice quality, respiratory distress (stridor
and/or tachypnea), and trismus.
• A lateral neck radiograph showing an edematous prevertebral space is suggestive of a
retropharyngeal abscess.
• Retropharyngeal abscesses are often polymicrobial; the predominant pathogens are group
A Streptococcus, Staphylococcus aureus, and respiratory anaerobes.

• Recognize the clinical findings associated with retropharyngeal abscess
• Identify the pathogens commonly associated with retropharyngeal abcess
• Plan the appropriate diagnostic evaluation of retropharyngeal abscess
• Plan the appropriate management o retropharyngeal abscess
Suggested Readings
• A clinical approach to tonsillitis, tonsillar hypertrophy, and peritonsillar and
retropharyngeal abscesses. Pediatr Rev. doi:10.1542/pir.2016-0072.
• Microbiology and management of peritonsillar retropharyngeal, and parapharyngeal
abscesses. J Oral Maxillofacial Surge doi: 10.1016/j.joms.2003.12.043.
• Airway obstruction. Peritonsillar abscess and retropharyngeal abscess, American
Academy of Pediatrics Textbook of Pediatric Care 2nd ed. 2782.
• Retropharyngeal Abscess. Pediatr Rev. doi: .10.1542/pir.27-6-e45.

Question 55
A 17-year-old male college student is evaluated in the emergency department for a 1 day history
of fever, headache, and vomiting. He was well until 3 days ago when he developed malaise, loss
of appetite, and painful swelling of his left jaw and cheek area. His immunizations are up to date.
Two of his college roommates recently developed a similar illness, one of whom also developed
orchitis. On physical examination, he is febrile (38 ºC); his other vital signs are normal. He is
alert and interactive, but has neck stiffness and pain with neck flexion. He has left parotid
swelling that is mildly tender to palpation. The overlying skip is not warm or red. The remainder
of his physical examination findings are unremarkable. Cerebrospinal fluid analysis shows 460
white blood cells (35% neutrophils, 60% lymphocytes, and 5% monocytes), a glucose level of 48
mg/dL (2.7 mmol/L), and a protein level of 65 mg/dL (0.65 g/L), Gram stain of the cerebrospinal
fluid shows no organisms. He is admitted to the hospital
Of the following, the MOST appropriate isolation precaution for this adolescent is
A. airborne
B. airborne
C. contact
D. droplet

Correct Answer: D
The adolescent in vignette has aseptic meningitis and parotitis due to mumps. Mumps is an
acute, communicable, vaccine-preventable infection in children caused by the mumps virus.
Mumps is characterized by unilateral or bilateral parotid gland swelling; common complications
of mumps include aseptic meningitis and orchitis. Mumps is spread via contact with infected
respiratory droplet secretions and saliva. Individuals with mumps contagious from 1 to 2 days
before the onset of parotitis to several days after. Therefore, droplet precaution is the
recommended choice isolation for this adolescent. In the hospital setting, other transmission-
based precautions (e.g., airborne, droplet, contact) may be indicated based on the causative
organism (Item C55).
Isolation precautions are aimed to protect patients, health care personnel, and visitors by
reducing the transmission of microorganisms in the health care environment. Isolation
procedures are based-on the route of pathogen transmission. Isolation precautions must be
implemented empirically and always include standard (universal) precautions. The major
transmission-based categories of isolation precautions include contact, droplet and airborne.
More than one category of isolation may be utilized in the clinical setting. Precautions are
generally discontinued when patients are no longer contagious, but policies for multidrug
resistant organisms may vary. Isolation procedure guidelines are summarized by the
Centers for Disease Control and Prevention
https://www.cdc.gov/infectioncontrol/pdf/guidelines/isolation-guideline-H.pdf
Standard precautions are used to prevent pathogen transmission via contact with non-intact skin,
membranes, or anybody fluid. Standard precautions are applied to all patients in health care
settings when there is potential for exposure to blood and body fluids. Standard precautions
include the following components: hand hygiene, personal protective equipment (PPE) (e.g.,
gloves, gowns, mask, eye protection), sterile instruments and devices, sharps safety, clean and
disinfected environmental surfaces, proper handling of textiles and laundry, safe injection
practices, patient placement (isolation precautions), and respiratory hygiene/cough etiquette.
Contact precautions are used to minimize spread of organisms by direct or indirect contact with
the patient or the environment. In hospital-acquired infection, contact transmission is the most
frequently implicated. Infections caused by common microorganisms that require contact
precautions are multidrug resistant bacteria herpes simplex virus, respiratory syncytial virus.
parainfluenza virus, scabies, enteroviruses, Shigella species, and Staphylococcus aureus
(cutaneous infection or draining abscesses). A single patient room is preferred, but if unavailable,
children infected with the same organism may be placed in a cohort if more than 3 to 6 feet
distance is maintained between beds. Gloves and gowns are recommended for all patients’
interactions and contact with environmental surfaces. Hand hygiene is recommended prior to and
after removal of gloves.
Droplet precautions are used to prevent transmission of organisms that spread by respiratory or
mucous membrane contact. Infections caused by common microorganisms that require droplet
precautions are influenza, pertussis, parvovirus B19 (before the onset of rash), Neisseria
meningitidis (invasive), rhinovirus, mumps, and adenovirus (pneumonia). A single patient room
is preferred, but if unavailable, children infected with the same organism may be placed in a
cohort if more than 3 to 6 feet distance is maintained between beds. A mask is recommended
before entering the room. Patients must wear masks when transported out of the room. Hand
hygiene is recommended prior to and after removal of PPE.
Airborne precautions are used to prevent transmission of organisms that spread by droplet nuclei
(≤5 µm in diameter) that can remain suspended in air for long periods, allowing the organism to
be transmitted over distances greater than 1 m. Infections caused by common microorganisms
that require airborne precautions are measles, varicella, and Mycobacterium tuberculosis (TB). A
single patient room is recommended with the door closed at all times and with special air
handling and ventilation. Patients should be placed in a negative air pressure room with 6 to 12
air changes per hour (air exhausted to outside); if air is recirculated, it must be processed using
high-efficiency particulate air (HEPA) filtration. For patients with suspected (or proven)
contagious TB, N95 masks (fit-tested) are recommended for health care providers before entry
into the patient room.
For certain microorganisms that have multiple transmission routes, combined transmission-based
precautions are recommended. For example, contact, droplet. and airborne precautions are
recommended for health care providers before entering the room of patients with suspected or
confirmed COVID-19 (SARS-CoV-2), In addition, in the setting of aerosol-generating
procedures (e.g., intubation, bronchoscopy) involving patients with COVID-19 (SARS-CoV-2),
fitted respiratory masks (e.g., N95 respirators) should be used along with other appropriate PPE
gloves, a gown, eye protection).
PREP Pearls
• Isolation precautions protect patients, health care personnel, and visitors by reducing the
transmission of microorganisms in the health care environment and are based on the route
of pathogen transmission.
• Isolation precautions must be implemented empirically and always include standard
precautions; the major transmission-based categories of isolation precautions include
contact, droplet, and airborne.
• Droplet precautions are used to prevent transmission of organisms that spread by close
respiratory or mucous membrane contact.

• Understand the appropriate use of universal, airborne, droplet, and contact precautions

Suggested Readings
• American Academy of Pediatrics. Infection prevention and control for hospitalized
children. Red Report of the Committee on Infectious Diseases 32nd ed. American
Academy of Pediatrics; 2021:133-141
• Centers for Disease Control and Prevention. 2007 Guideline for Isolation Precautions:
Preventing Transmission of Infectious Agents in Healthcare Settings. Recommendations
of the Healthcare Infection Control Practices Advisory Committee.
https://www.cdc.gov/infectioncontrol/pdf/guidelines/isolation-guidelines-H.pdf
• Centers for disease Control and Prevention. Guideline for hand hygiene in healthcare
settings. Recommendations for the Healthcare Infection Control Practices Advisory
Committee and the HICPAC/SHEA/APIC/IDSA Hand Hygiene Task Force. MMWR
https://www.cdc.gov/mmwr/preview/mmwrhtml/rr5116a1.htm

Question 56
The following neonates are being evaluated in the neonatal intensive care unit
Room 1: A 6-day old, full-term female neonate with marked hypotonia, poor suck and swallow,
and failure to gain weight requiring placement of nasogastric tube.
Room 2: A 3-day old male neonate with ventricular septal defect, hypotonia, and dysmorphic
features including: Up-slanted palpebral fissures, midface hypoplasia, epicanthal folds, bilateral
single transverse palmar creases, and increased gap between the first and second toes.
Room 3: A 5-day old male neonate with atrial septal defect, meningocele, right clubfoot, and
postaxial polydactyly.
Room 4: A 1-day-old, critically ill small-for-gestational-age, female neonate with tetralogy of

Fallot, cleft palate, and hypocalcemia
Of the following, the BEST genetic test to reveal the diagnosis for each neonate is
Response Neonate in Room Neonate in Room Neonate in Room Neonate in Room
Choice 1 2 3 4
A Chromosomal chromosomal fluorescent In situ methylation
analysis microarray hybridization for analysis for the
the critical region disorder
associated with the
disorder
B chromosomal fluorescent in situ methylation chromosome
microarray hybridization for analysis for the analysis
associated with the
disorder
C fluorescent in situ methylation chromosome chromosomal
hybridization for analysis for the analysis microarray
associated with
the disorder
D methylation chromosome chromosomal fluorescent in situ
analysis for the analysis microarray hybridization for
disorder the critical region
associated with the
disorder

Correct Answer: D
The best genetic test for a given clinical scenario depends on the suspected diagnosis. Common
testing modalities (Item C56) include chromosome analysis, fluorescent in situ hybridization
(FISH), chromosomal microarray (CMA), methylation analysis, and whole exome sequencing.
Item C56 Genetic testing modalities

1. G banded (conventional) karyotype
2. Fluorescent in situ hybridisation (FISH)
3. Molecular karyotype (eg single-nucleotide polymorphism [SNP] microarray)
4. Sanger sequencing
5. Polymerase chain reaction (PCR)
6. Multiplex ligation-dependent probe amplification (MLPA)
7. DNA methylation studies of specific chromosome region
The neonate in room 1 has Prader-Willi syndrome (PWS), which is characterized by marked
hypotonia and poor suck and swallow at birth. Most neonates with PWS require placement of a
feeding tube due to feeding difficulties. Later, hyperphagia develops, resulting in excessive
weight gain if food intake is not strictly controlled Global developmental delay, hypogonadism,
and behavioral issues are also common. Early diagnosis of PWS is critical. Associated with
improved health and developmental outcomes, early diagnosis allows for appropriate nutritional
management, early developmental intervention, anticipatory guidance, and treatment with
recombinant human growth hormone. Growth hormone therapy is associated with improved
muscle tone, motor function, and body composition (Increased lean body mass, decreased fat
mass); it can be started in infancy.
Prader-Willi syndrome is caused by the lack of expression of paternally inherited genes in the
imprinted region of chromosome 15 at 15q11.2-q13. The diagnosis is made when methylation
analysis of this Prader-Willi critical region identifies only the maternally inherited methylation
pattern. Methylation analysis detects >99% of those with PWS but does not identify the genetic
mechanism. A CMA or FISH for the Prader-Willi critical region can then be used to detect PWS
caused by a paternally inherited deletion of 15q11.2-q13 (70% of cases). They will not detect
PWS caused by maternal uniparental disomy (UPD) of 15q11.2-q13 (approximately 25% of
eases) or an imprinting defect and therefore are not first-line tests. A CMA that includes single
nucleotide polymorphism (SNP) analysis can detect some cases of maternal UPD.
The neonate in room 2 has typical features of trisomy 21 (Down syndrome), which is diagnosed
with a chromosome analysis (karyotype) demonstrating an extra Chromosome 21. Chromosome
analysis can also detect a Robertsonian translocation (i.e., an entire acrocentric chromosome
attaches to another at the centromere), for which parental testing would be recommended to
estimate recurrence risk. While CMA would demonstrate the extra chromosome 21 and the
diagnosis, it does not differentiate whether the extra chromosome is free standing or translocated.
FISH for chromosome 21 can provide a rapid diagnosis of trisomy 21 but would need to be
followed up with a karyotype to rule out a Robertsonian translocation.
The neonate in room 3 has multiple congenital anomalies that are not suggestive of a specific
genetic syndrome. According to the American College of Medical Genetics Practice Guidelines,
the first-line diagnostic test in this situation is a CMA to look for deletion/duplication(s) of
genomic content across all chromosomes. Since a specific disorder is not suspected, FISH and
methylation analysis are not appropriate.
The neonate in room 4 has 22q11.2 deletion syndrome, also known as DiGeorge syndrome or
Velocardiofacial syndrome. Common clinical features include a congenital heart defect (e.g.,
tetralogy of Fallot, ventricular septal defect, and truncus arteriosus), cleft palate, vascular ring,
hearing loss, hypocalcemia, failure to thrive, immunological dysfunction, and developmental
delay. Approximately 95% of cases can be detected by a FISH probe for the DiGeorge critical
region. While CMA can also be used for the diagnosis, FISH has a rapid turnaround time of 48
hours which may be important in the care of a critically ill neonate who has clinical features
suggestive of 22q11.2 deletion.
PREP Pearls
• Common genetic testing modalities include chromosome analysis, fluorescent in situ
hybridization, chromosomal microarray, methylation analysis, and whole exome
sequencing.
• Chromosome analysis the recommended first-line test for suspected aneuploidies such as
trisomy 13, trisomy 18, and trisomy 21.
• Chromosomal microarray analysis is the recommended first-line test for multiple
congenital anomalies not suggestive of a specific syndrome, autism spectrum disorder,
and unexplained non-syndromic developmental delay.

• Understand the role of karyotyping in postnatal diagnosis
• Understand the role of fluorescence in situ hybridization studies in postnatal diagnosis
• Understand the role of microarray testing in postnatal diagnosis
Suggested Readings
• AAP Committee on Genetics. Overview of genetic testing. Saul RA, ed. Medical
Genetics in Pediatric Practice. American Academy of pediatrics 2017
• Prader-Willi syndrome. Gene Reviews (Internet], Seattle. WA: University of
Washington: 2014.
• Professional Practice and Guidelines Committee. Array-based technology and
recommendations for utilization in medical genetics practice for detection of
chromosomal abnormalities. Genet Med doi:10.1097/GIM.0b013e3181f8baad
• 22q11.2 Deletion syndrome. Gene Reviews [Internet]. Seattle, WA: University of
Washington, 2020.

Question 57
A 7-year-old boy is brought to the office by his mother for learning concerns. The boy has
received early intervention services for delays in language and motor development. Due to
increasing difficulties keeping up with his peers, he received a recent psychoeducational
assessment which identified mild intellectual disability. The boy's medical history is otherwise
noncontributory. He is a social and outgoing child with a wide smile. He has a broad forehead,
full cheeks, and a short nose (Item Q57). He is shorter than expected for parental height. No one
else in their family has had problems with learning. His mother would like to know why her son
has had such difficulties with development.
Item Q57: the patient in the vignette
Of the following, the BEST next step in diagnosis for this boy is to order
A. Chromosomal microarray
B. FMR1 DNA test
C. high-resolution karyotype
D. testing for the MECP2 mutation

Correct Answer: A
Chromosomal microarray is typically ordered as a first step in the medical evaluation of a child
with intellectual disability (ID). The boy in the vignette has dysmorphic features and behaviors
suspicious for Williams syndrome, which is caused by deletion at 7q11.23 and would be detected
on chromosomal microarray. Alternatively, if the clinical features of Williams syndrome are
recognized, fluorescence in situ hybridization (FISH) using a probe targeted to 7q11.23 can be
ordered instead of microarray to detect this deletion.
Genetics must be considered when evaluating for causes of ID. Hundreds of genetic syndromes
are associated with IDs. These include Chromosomal disorders, contiguous gene deletions, and
single-gene deletions. The most common genetic cause of ID is Down syndrome, whereas the
most common genetic inherited cause of ID is fragile X. Inheritance patterns of ID include X-
linked, autosomal dominant, and autosomal recessive. Genetic causes, inheritance, and
characteristic features of some of the more common genetic conditions associated with ID are
listed in Item C57.
Item C57
In addition to chromosomal microarray, further genetic testing can be helpful for identifying
other causes of ID. Testing using FISH can detect specific conditions, including Williams
syndrome. The FMR1 DNA test specifically looks for fragile X syndrome by examining the
number of CGG trinucleotide repeats in the FMR1 gene. A karyotype will detect chromosomal
conditions such as Down syndrome. Testing for the MECP2 mutation will identify Rett
syndrome.

PREP pearls
• The medical workup for intellectual disability typically begins with a chromosomal
microarray.
• Williams syndrome, which is caused by a deletion at 7q11.23, can be detected with
fluorescence in situ hybridization using a probe targeted to 7q11.23 with chromosomal
microarray.
• The most common genetic cause of intellectual disability is Down syndrome whereas the
most common inherited cause of intellectual disability is fragile X.
• Inheritance patterns of intellectual disability include X-linked, autosomal dominant, and
autosomal recessive.

• Identify common chromosomal causes of intellectual disabilities
• Identify common inheritance patterns of intellectual disabilities
Suggested Readings
• Cognitive development and disorders, Developmental and Behavioral pediatrics. 2nd ed.:
309-346
• Intellectual disability. American Academy of pediatrics Textbook of Pediatric Care 2nd :
2208-2216.
• Intellectual disabilities. Pediatr Rev doi:10.1542/pir.2016-0116

Question 58
A previously healthy, 6-month-old, male infant is admitted to the pediatric intensive care unit for
respiratory failure secondary to respiratory syncytial virus. He is intubated and supported with a
ventilator.
Of the following, the BEST modality to support this infant's nutrition is

A. gastrostomy feeding
B. nasogastric feeding
C. nasojejunal feeding
D. parenteral nutrition

Correct Answer: C
The infant in the vignette is unable to feed from a bottle due to respiratory failure. The safest
way to feed this infant is through a nasojejunal tube. The aspiration risk while intubated for
respiratory failure is too high with nasogastric tube feeding. Because the infant is likely to
improve within a few days to weeks, placement of a gastrostomy tube is not necessary. A
gastrostomy tube would be indicated if the infant were expected to require tube feeding for >3
months. Providing nutrition through the stomach or jejunum is preferred over intravenous
nutrition because lower risk of adverse reactions and better cost-effectiveness.
Nutrition is crucial for children's growth, development, and healing. Multiple factors contribute
to a child's inability to consume adequate fluid and calories orally. A few examples include
neurological impairment leading to poor or unsafe oral skills, aspiration, critical illness, severe
anxiety, and anorexia nervosa. In these cases, a feeding tube is appropriate. Some children are
unable to meet increased caloric needs due to conditions such as congenital cardiac defects or
severe burns. Others have malabsorption due to conditions such as inflammatory bowel disease
or gut syndrome. If the illness is likely to persist and the need for support will be chronic, a
surgically placed gastrostomy tube should be considered. Enteral feedings, when possible, are
best since they are less expensive; stimulate motility, decrease the risk of bacteria overgrowth,
which can lead to translocation of bacteria into the peritoneal cavity and blood stream; and do
not require a central line that confers associated risks such as thrombus and bacteremia.
Gastric feedings have several advantages. They may be provided at a continuous rate or as bolus
feeding, a defined volume of formula administered either by a pump or gravity through a syringe
over a short period of time, after which the tube disconnected. Gastric feedings can be used in
conjunction with bottle feeding in infants. The infant begins feeding with a bottle, and the
portion of the required volume not consumed by the infant is administered via the gastric tube.
The gastric acid stimulated by gastric feedings decreases the risk of infection. Jejunal feedings
can be used if the child experiences nausea and vomiting, had poor gastric motility or has a high
risk of aspiration. Jejunal feedings are administered via a pump at a continuous rate.
Family members or others caring for children fed via a tube should understand potential
malfunctions including dislodgement or clogging of the tube. Some medications or incorrect
mixing of formula can lead to tube clogging. Caregivers should know how to perform or access
care urgently for tube replacement.
There are a variety of formulas available to meet each child's needs. Formulas with higher
osmolality especially when greater than physiologic levels, can have an osmotic effect in the
intestines, resulting in an influx of water; the benefits must be considered in concert with the risk
of diarrhea and abdominal cramping. Hydrolyzed amino acid-based formulas are indicated for
children with cow milk protein or soy intolerance. Formulas with additional, fiber are helpful for
children with constipation, a common concern among those who are not mobile.

PREP Pearls
• Enteral feeding is preferred over parenteral nutrition whenever possible.
• Short-term nutritional support (1 to 3 months) should be provided with nasogastric or
nasojejunal feedings; chronic nutritional support can be accomplished with a gastrostomy
or jejunostomy tube.
• Children at high risk of aspiration benefit from jejunal feedings.

• Judge the advantages of enteral nutrition over parenteral nutrition
• Understand the indications for providing enteral nutritional support
Suggested Readings
• Enteral feeding. Pediatr Rev. doi:10.1542/pir.29-3-105
• Enteral nutrition in pediatric patients. Pediatr Gastroentero Hepatoi Nut. 2018;
doi:105223/pghn.201821.1.12

Question 59
A 7-year-old girl with sickle cell disease, hemoglobin S β°-thalassemia, is seen in the emergency
department for difficulty breathing. She has had upper respiratory symptoms for the past 4 days
and developed a fever today. She had been eating and drinking well until today, when she had an
episode of post tussive emesis and developed abdominal pain. She takes hydroxyurea as
prescribed. Her vital signs include a temperature of 39.4 °C, heart rate of 150 beats/min,
respiratory rate of 35 breaths/min, blood pressure of 106/61 mm Hg, and room air oxygen
saturation by pulse oximetry of 91%. She is awake and alert. She has moist mucous membranes
conjunctival pallor, intercostal and suprasternal retractions, and decreased breath sounds on the
left side. There is a normal sinus rhythm and no wheezing. Her abdomen is soft and distended;
the spleen is palpated 5 cm below the left costal margin.
Chest radiography demonstrates a left lower lobe infiltrate. Laboratory data are shown:

Platelet count 130 x 103/µL (130 109/L)
Reticulocytes 13.6%
Absolute reticulocyte count 416 x 103/µL (416 x 109/L)
Results from a blood culture are pending.
Of the following, the BEST next step in the management of this child is to administer
A. a 10 mL/kg packed red blood cell transfusion
B. a 20 mL/kg normal saline bolus
C. ceftriaxone and azithromycin
D. oxygen via high-flow nasal cannula

Correct Answer: C
The child in the vignette has a form of sickle cell disease, an inherited group of hemolytic
anemias caused by mutations in chromosome 11 in the hemoglobin β-chain. The most common
form, hemoglobin S, results from a β (p.Glu6Val) substitution. The abnormal hemoglobin causes
hemoglobin polymerization that ultimately results in red cell sickling, which leads to hemolysis
and red blood cell rigidity resulting in vaso-occlusion. This causes crises such as severe recurrent
pain episodes, acute splenic sequestration, acute chest syndrome, and organ damage. Newborn
screening for sickle cell disease began in the United States in the 1980s and was included in all
50 states by 2006.
The term “sickle cell anemia” is reserved for individuals with hemoglobin SS and hemoglobin S
β°-thalassemia, like the child in the vignette. Due to functional asplenia resulting from recurrent
splenic infarction, individuals living with sickle cell disease are at increased risk of infection.
There is usually atrophy of the spleen by around age 5 years, however, with the use of disease-
modifying therapy such as hydroxyurea and blood transfusions, this may be delayed.
Hydroxyurea use leads to an increase in fetal hemoglobin, causing an increase in the mean
corpuscular volume (MCV). Acute splenic sequestration crisis remains a risk until atrophy
occurs. Individuals with other genotypes of sickle cell disease, such as hemoglobin SC or
hemoglobin S β+-thalassemia are at risk for acute splenic sequestration crises throughout their
lifetime as they do not progress to complete atrophy of their spleen.
The child in the vignette has several acute sickle cell complications, including fever, acute chest
syndrome (new pulmonary infiltrate on chest radiograph with respiratory symptoms, fever, or
chest pain); and acute splenic sequestration crisis (enlargement of the spleen with a drop in the
hemoglobin level from baseline, and thrombocytopenia).
For a child with fever and functional asplenia, obtaining a blood culture followed by prompt
administration of a broad-spectrum antibiotic such as ceftriaxone is critical to avoid progression
to overwhelming sepsis. Because this child has acute chest syndrome, a macrolide such as
azithromycin should also be administered for coverage of atypical bacteria such as Chlamydia or
Mycoplasma pneumoniae.
For this child with both acute splenic sequestration crisis and hypoxia in the setting acute chest
syndrome, a packed red blood cell transfusion is warranted. However, the volume of blood to
administer should be carefully calculated.
Administration of a large volume may lead to cardiac output failure or cause hyperviscosity,
which may precipitate a stroke. In the setting of an acute splenic sequestration crisis, the blood
cells are trapped in the spleen: they are released back into circulation once the event is reversed.
Therefore, transfusions administered in small aliquots of 3-5 ml/kg; with monitoring of the
complete blood count and frequent spleen checks to ensure it is not enlarging.
Administration of a normal saline bolus is not appropriate for the child in the vignette as there
are no findings of dehydration on physical examination. If rehydration were necessary, a small
fluid bolus should be given followed by reassessment and administration of another fluid bolus,
if needed. It is important to note that fluid overload could worsen acute chest syndrome. Also,
too much fluid will dilute the already low hemoglobin, decreasing oxygen carrying capacity in
already hypoxic patient.
Due to hypoxia, oxygen therapy is indicated for this child. However, it can initially be
administered via normal-flow nasal cannula. It is possible that eventually high-flay nasal cannula
or even BIPAP (bilevel positive airway pressure) may be required.
PREP Pearls
• Acute splenic sequestration crisis may occur in older children with sickle cell anemia;
there may be delayed splenic atrophy as a result of disease-modifying therapy (e.g.,
hydroxyurea and blood transfusions).
• For a child with sickle cell disease requiring a packed red blood transfusion, the
underlying cause of anemia and goal of transfusion should be considered when
calculating the volume to administer.
• Fluid boluses in children with sickle cell disease should be done carefully, and only if
clinically warranted, to minimize risk of fluid overload or worsened hypoxia or anemia.

• Recognize the clinical findings associated with sickle cell disease in children of various
ages
• Understand that sickle cell disease can be diagnosed at birth
Suggested Readings
• Hemoglobinopathies and sickle cell disease. American Academy of Pediatrics Textbook
of Pediatric Care 2nd ed.:2117-2125
• Sickle cell disease. Pediatr Rev. doi:10.1542/pir.33-5-195
• Acquired and congenital hemolytic anemia Pediatr Rev. doi: 10.1542/pir.2015-0053
• Sickle cell disease N Engl J Med. doi: 10.1056/NEJMra1510865
• Sickle cell disease. Lancet. doi:10.1016/S0140-6736(17)30193-9
• Management of sickle cell disease: Summary of the 2014 evidence-based report by expert
panel members. JAMA. Doi:10.1001/jama.2014.10517

Question 60
A 3-year-old girl is brought to the clinic by her foster parents. Child protective services removed
the girl from her parents' custody because of allegations of physical abuse toward her infant
brother. The girl’s foster parents are concerned that she will have difficulty forming appropriate
emotional connections.
Of the following, the factor MOST likely to be associated with the outcome of concern is a
history of
A. physical abuse when she was an infant
B. psychological abuse of the girl by her parents
C. witnessing violence between her parents
D. witnessing physical abuse of her brother

Correct Answer: B
The foster parents of the girl in the vignette are concerned about her developing an attachment
disorder as a result of her past experience. Although all of the response choices (history of
physical abuse as an infant, witnessing violence between parents or child abuse of a sibling, and
experiencing psychological abuse) can be associated with negative psychological and emotional
outcomes in children, psychological abuse is the most highly associated with development of an
attachment disorder.
Psychological abuse is a common form of child maltreatment. True rates of psychological abuse
are unknown, as this type of maltreatment can be difficult to identify and is underreported to
child protective services. Although psychological abuse can occur as an isolated form of
maltreatment, it can also be foundational to other forms of maltreatment such as neglect or
physical or sexual abuse. Psychological abuse is defined as caregiver behaviors (verbal or
nonverbal, active or passive, with or without intent to harm) that negatively affect a child's
cognitive, social, emotional, and/or physical development. Types of psychologically abusive
behaviors are outlined in Item C60. At times, differentiating psychological abuse from poor
parenting skills can be difficult, in part because of varying societal and cultural norms.
Item C60: Types of Psychologically Abusive Behaviors by Caregivers

Spurning • Belittling, denigrating, or other rejecting
• Ridiculing for showing normal emotions
• Singling out or humiliating in public
Terrorizing • Placing in unpredictable/chaotic circumstances
• Placing in recognizably dangerous situations
• Having rigid/unrealistic expectations accompanied by threats if
not met
• Threatening/perpetrating violence against child or child’s
loved ones/objects
Isolating • Confining within environment
• Restricting social interactions in community
Exploiting/Corruptin • Modeling, permitting, or encouraging antisocial or
g developmentally inappropriate behavior
• Restricting/undermining psychological autonomy
• Restricting/interfering with cognitive development
Denying emotional • Being detached or uninvolved; interacting only when
responsiveness necessary
• Providing little or no warmth, nurturing, praise during any
developmental period in childhood
Mental • Limiting a child’s access to necessary health care because of
health/medical/educ reasons other than inadequate resources
ational neglect
• Refusing to provide for serious emotional/behavioral, physical
health, or educational needs
Children who experience psychological abuse have higher rates of attachment disorders,
disruptive behaviors, developmental and educational delays, and mental health problems. The
long-term effects of psychological abuse experienced in infancy and toddlerhood can be
especially profound. The causes of psychological abuse are complex. The researchers Kimber
and MacMillan write that causes of psychological abuse are “best understood within what is
referred to as the ecological model, which considers the balance of risk and protective factors at
the individual, family, community, and societal levels”. The experience of multiple, chronic
stressors is associated with psychological abuse. Screening for the presence of these stressors
(especially family conflict, adult mental health problems, and parental substance abuse) may help
clinicians identify children at risk for psychological abuse and other forms of maltreatment.
Strategies to prevent psychological abuse include universal interventions to promote optimal

parenting, targeted interventions for those at risk, and programs to maximize protective factors in
individuals, families, and communities.
PREP Pearls
• Psychological abuse is defined as caregiver behaviors (verbal or nonverbal, active or
passive, with or without intent to harm) that negatively affect a child’s cognitive, social,
emotional, and/or physical development. Examples Include terrorizing, isolating,
belittling, or denying emotional responsiveness.
• Children who experience psychological abuse have higher rates of attachment disorders,
disruptive behaviors, developmental and educational delays, and mental health problems.
MOCA-Peds Objective
• Understand the role of the pediatrician in prevention of child abuse and neglect.

• Recognize the history, signs, and symptoms indicative of psychological
• Understand the behavioral and emotional consequences of psychological abuse
Suggested Readings
• American Academy Pediatrics Textbook of Pediatric care 2017:2905-2918.
• Committee on Child Abuse and Neglect; American Academy of Child and Adolescent
Psychiatry. Psychological maltreatment Pediatrics doi: 10.1542/peds.2012-1552

Question 61
A 7 years old previously healthy child is seen at the emergency with a 12-hour history of
worsening right lower quadrant abdominal pain and vomiting. Since the onset of symptoms, the
child has not wanted to eat anything and has only been able to drink small amounts of juice. The
patient appears comfortable and has a temperature of 37.3 °C and a heart rate of 98 beats/min.
There is mild right lower quadrant tenderness both with deep palpation of the right lower
extension of the right hip.

White blood cell count 18,000/µL (18 x 109/L)
Neutrophils 85%
Potassium 4.8 mEq/L (4.8 mmol/L)
Chloride 104 mEq/L (104 mmol/L)
Bicarbonate 15 mEq/L (15 mmol/L)
Intravenous fluids are started. Pediatric services (ultrasonography and surgery) are not locally
available.
Of the following, the BEST next step in management of this child is to
A. admit to the local hospital for rehydration and observation
B. discharge to home if tolerating oral rehydration therapy
C. order computerized tomography scan of the abdomen
D. transfer to a facility with resources for pediatric surgical care

Correct Answer: D
The child in this vignette has signs and symptoms suggestive of acute appendicitis and needs
further evaluation at a facility with capability for both pediatric and pediatric radiology and
pediatric surgery services.
History, physical examination, laboratory results, and radiographic findings can all be supportive
tools to determine the need for an appendectomy.
Symptoms that are concerning for acute appendicitis:

• Initial periumbilical abdominal pain with migration to the right lower quadrant (RLQ)
over a 24-hour period
• Anorexia
• Nausea/vomiting
• Fever
Physical examination findings that are concerning for acute appendicitis:

• RLQ tenderness
• Guarding
• Rebound tenderness in the RLQ
• Rovsing sign (referred pain in the RLQ with left lower quadrant palpation)
• Obturator sign (RLQ pain with internal rotation of the right leg)
• Psoas sign (RLQ pain with extension of the right hip)
Laboratory data, including elevated white blood cell count, neutrophil count, and C-reactive
protein level, may raise suspicion for acute appendicitis when paired with concerning signs and
symptoms. Scoring systems, including the Pediatric Appendicitis Score (PAS) and the Pediatric
Appendicitis Risk Calculator (pARC), may be used to help predict the likelihood of appendicitis
in a pediatric patient, guiding usage of surgical consultation and additional imaging. Pediatric
patients with classic findings of appendicitis should undergo immediate surgical consultation.
Patients with many concerning features (moderate or intermediate risk) may warrant surgical
consultation and/or additional imaging. Patients with moderate/intermediate or high risk for
appendicitis should be evaluated at a center with pediatric capability for radiography and surgical
services. Patients with low-risk features may warrant observation or even discharge to home with
clear instructions for when to return.
Imaging may be indicated for children with moderate or intermediate risk of appendicitis
imaging based on the available clinical information. Ultrasonography should be the initial
imaging test performed in children where history, examination, and laboratory findings are
suspicious, but not classic, for appendicitis. Ultrasonography is safe and cost effective, with
accuracy similar to computed tomography when performed by sonographers and radiologists
experienced in pediatric radiology. Computed tomography, because of concerns for radiation
exposure and subsequent increased risk for cancer, should be reserved for children with unclear
ultrasonography results or those suspected abscess. The use of magnetic resonance imaging,
considered safe and accurate, is limited by availability and cost. Magnetic resonance imaging
may be an alternative to computed tomography when an ultrasound is unclear.

Because this child has concerning signs (history of migrating pain), examination findings (RLQ
tenderness), and laboratory results (elevated white blood cell count), the risk for appendicitis is
moderate/intermediate. Thus, discharging to home or remaining at an institution without
pediatric capable radiology and surgery would not be appropriate in this situation.
PREP Pearls
• Symptoms concerning for acute appendicitis include: periumbilical abdominal pain with
migration to the right lower quadrant over 24 hours, anorexia, nausea/vomiting, and
fever.
• Children with signs, symptoms, and laboratory results that cause concern for acute
appendicitis should be evaluated in a facility with appropriate resources for pediatric
surgical care.
• Ultrasonography is the preferred radiographic modality for assessing appendicitis when
imaging is warranted.
MOCA-Peds Objective
• Know the differential diagnosis of acute abdominal pain

• Recognize the clinical features associated with appendicitis
• Plan the appropriate diagnostic evaluation when appendicitis is suspected
Suggested Readings
• Trends in the diagnosis management or appendicitis. Pediatr Rev. doi:10.1542/pir.2015-
0021
• Pediatric appendicitis. Surg Clin North Am, doi:10.1016/j.suc.2016.08.009
• Appendicitis. American Academy of Pediatrics Pediatric Cam 2nd ed. 2796-2799.

Question 62
A 3-day old neonate is being evaluated for jaundice. He has been breast feeding well and has had
6 wet diapers and 3 stools today. Physical examination is significant for a 3% weight loss from
birth weight, scleral icterus, and diffuse jaundice. The remainder of the physical examination
findings are normal. Maternal blood type is AB positive. The neonate's blood type is A positive.
A transcutaneous bilirubin level obtained before discharge from hospital was 8.6 mg/dL (147
µmol/L) at 28 hours after birth.
Laboratory data from today are shown:

Total serum bilirubin 15 mg/dL (256.6 µmol/L)
Conjugated bilirubin 2.3 mg/dL (39.3 µmol/L)
Of the following, the MOST likely of this neonate finding is

A. anti-B immunoglobulin antibodies
B. deficiency of glucose-6-phosphate dehydrogenase
C. inadequate breast milk intake
D. inhibition of bilirubin uridine diphosphate glucuronyltransferase

Correct Answer: B
The most etiology of this neonate's elevated bilirubin level and anemia is deficiency of glucose-
6-phosphate dehydrogenase (G6PD). Glucose-6-phosphate dehydrogenase is required for the
synthesis of nicotinamide adenine dinucleotide phosphate (NADPH), which converts glutathione
to a reduced state. Glutathione has an antioxidant effect that stabilizes red blood cells. Without
this protection, red blood cell lysis and bilirubin production are increased. Individuals with
G6PD deficiency may have elevated bilirubin levels in the neonatal period as well as with stress,
intake of fava beans, ingestion of sulfa-containing medications, and infection. Medications
associated with acute hemolysis in G6PD deficiency include sulfa-containing antimicrobials and
aspirin. The degree of hemolysis varies, depending on the specific G6PD mutation, quantity of
medication ingested, and the type of medication ingested.
Breast milk jaundice is thought to result from inhibition of bilirubin uridine diphosphate
glucuronosyltransferase (BUGT). The enzyme BUGT is required for conjugation of bilirubin
into water-soluble form that can be excreted in urine, therefore BUGT deficiency results in
decreased bilirubin excretion. The activity of BUGT increases during the first month after birth.
Breast milk jaundice peaks after the first week of age and may persist for as long as 10 weeks
after birth. Because this neonate presented in the first week after birth, breast milk jaundice
caused BUGT inhibition is a less likely diagnosis.
Physiologic jaundice starts in the first 2 to 4 days after birth and peaks between 4 and 5 days
after birth. It results from increased red blood cell turnover and decreased BUGT activity. In
addition, reduced intestinal bacteria that digest bilirubin to urobilinoids may contribute to
elevated serum bilirubin levels.
Breastfeeding jaundice occurs in the first week after birth and has some overlap with physiologic
jaundice. Breastfeeding jaundice occurs when neonates are not taking adequate amounts of
breast milk. Enterohepatic circulation is increased and bilirubin elimination through stool is
decreased. Breastfeeding jaundice can be treated with on-demand feeding and rooming-in to
facilitate breastfeeding. If there is weight loss and concern for dehydration, supplementation with
formula can be considered. For this neonate with only 3% weight loss, decreased oral intake is
unlikely to be the cause of his elevated bilirubin level.
Hemolytic disease of the fetus and newborn (HDFN) is characterized by maternal antibodies
against red blood cell antigens that cause hemolysis. Typically, HDFN occurs in neonates with
blood types A or B who are born to mothers with type O blood. Minor red blood cell antigens,
such as Kell. C or E, can rarely cause HDFN. Although typically more severe with subsequent
pregnancies, HDFN can present during the first pregnancy because antibodies develop from
exposure to carbohydrate moieties. Most women produce IgM antibodies against the A and B
antigen which do not cross the placenta. In 1% to 10% of instances of ABO incompatibility, IgG
transfer from the mother to the neonate causes hemolysis. Hemolytic disease of the fetus and
newborn that is caused by Rh incompatibility is severe; it is less commonly seen because of
administration of rho (D) immune globulin during pregnancy. There is no ABO incompatibility
between the mother and neonate in the vignette.

PREP Pearls
• In glucose-6-phosphate dehydrogenase deficiency, reduced glutathione is not available to
stabilize red blood cells during times of stress, leading to hemolysis and elevated
bilirubin levels
• Breast milk jaundice peaks after the first week of age; it results from inhibition of
bilirubin uridine diphosphate glucuronosyltransferase.
• Hemolytic disease of the fetus and newborn typically occurs in neonates with type A or B
blood born to mothers with type O blood.

• Understand the mechanism of breast-milk jaundice and manage appropriately
• Plan the appropriate diagnostic evaluation of jaundice in a full-term infant
• Understand the differences between physiologic jaundice in pre-term and full-term
infants
• Recognize the association between breast-feeding and physiologic jaundice in the
neonatal period
Suggested Readings
• Enzymatic defects. Nelson Textbook of Pediatrics 21st ed. 2553-2562.
• Blood disorders. Nelson Textbook of Pediatrics 21st ed. 961-974.
• Jaundice. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed. 1473-
1480.
• Jaundice: Newborn to age 2 months. Pediatr Rev. doi: 1542/pin2015-0132

Question 63
A 12-month-old girl is seen in the office after a hospital admission for fever and vomiting. She
was diagnosed with her first febrile urinary tract infection. The urine culture grew Escherichia
coli > 100,000 colony-forming units/ml.
Susceptibility results are shown

Antimicrobial Minimum Interpretation
inhibitory
concentration
Ampicillin/sulbactam 16/8 Moderately susceptible
Ampicillin >16 Resistant
Aztreonam ≤2 Susceptible
Cefazolin 4 Susceptible
Cefepime ≤1 Susceptible
Cefoxitin 8 Susceptible
Ceftriaxone ≤1 Susceptible
Ertapenem ≤0.25 Susceptible
Gentamycin ≤2 Susceptible
Meropenem ≤0.5 Susceptible
Nitrofurantoin ≤16 Susceptible
Piperacillin/tazobactam ≤2/4 Susceptible
Tobramycin ≤2 Susceptible
Trimethoprim/sulfamethoxazole >2/38 Resistant
She received intravenous ceftriaxone for 2 days in the hospital and oral cefixime at home for 8
days. She has no known drug allergies. Renal ultrasonography shows moderate right
hydronephrosis. Avoiding cystourethrogram shows right grade IV vesicoureteral reflux.
Of the MOST appropriate prophylactic antibiotic for this girl is

A. Amoxicillin
B. Cefixime
C. Nitrofurantoin
D. Trimethoprim-sulfamethoxazole

Correct Answer: D
The girl in the vignette was treated for a febrile Escherichia coli urinary tract infection (UTI).
Imaging revealed right hydronephrosis and grade IV vesicoureteral reflux (VUR). Antibiotic
prophylaxis is indicated because she is not toilet-trained and her VUR is at grade III or higher.
Trimethoprim-sulfamethoxazole (TMP/SMX) is preferred for prophylaxis because of its
bactericidal action. After the urine is made sterile with treatment doses of third-generation
cephalosporins, TMP/SMX would be effective in preventing future infection for this girl despite
her recent E coli infection showing resistance to TMP/SMX.
Vesicoureteral reflux is defined as the retrograde passage of urine from the bladder to the upper
urinary tract. Vesicoureteral reflux is a common urologic problem in children that occurs as a
result of incomplete closure of the ureterovesical junction during voiding (primary VUR) or as a
result of abnormally high bladder pressures (secondary VUR). Vesicoureteral reflux can be
asymptomatic (typically diagnosed during evaluation of a neonate with antenatal
hydronephrosis) or may present with a febrile UTI. Voiding cystourethrography (VCUG) is the
best test to confirm the presence and degree of VUR. The International Reflux study group
classifies VUR into 5 grades as shown in Item C63.
Item C63: VUR grades
Vesicoureteral reflux is managed with either watchful waiting or antibiotic prophylaxis based on
clinical criteria. Associated bladder/bowel dysfunction, if present, should also be addressed.
Watchful waiting may be considered for grade I and II VUR. Antibiotic prophylaxis is indicated
for children with low-grade VUR with recurrent UTI, grade III or higher VUR, or non-toilet-
trained children with any grade of VUR. Prophylactic antibiotics sterilize the urine and decrease
the risk of recurrent UTI. The antibiotic is preferably given once daily at bedtime. Prophylactic
Antibiotic dosing is generally one-fourth to one-half of the daily treatment dose. Commonly used
agents include TMP-SMX, trimethoprim alone, or nitrofurantoin. Trimethoprim-
sulfamethoxazole is preferred for prophylaxis because of its bactericidal action.
Amoxicillin prophylaxis is recommended for infants younger than 2 months, because both
TMP/SMX and nitrofurantoin interfere with hepatic conjugation and increase the risk of
hyperbilirubinemia. Cefixime, a third-generation cephalosporin, is used to treat UTI but not for
prophylaxis. Nitrofurantoin is bacteriostatic and indicated as prophylaxis for children with sulfa
allergy and those having breakthrough infection with TMP/SMX. Cephalexin is used for children
with breakthrough infections with TMP/SMX or nitrofurantoin prophylaxis. Cephalexin is not
the preferred choice for UTI prophylaxis because of the risk of developing resistance.
Persistent VUR may cause loss of renal parenchyma, and potentially lead to renal scarring and
reflux nephropathy. Children with reflux nephropathy are at risk of developing proteinuria and/or
hypertension. Bilateral reflux nephropathy can also lead to chronic kidney disease, which can
progress to end-stage renal disease requiring dialysis and renal transplantation.
Surgical correction is advised for children with persistent VUR who have breakthrough
infections with antibiotic prophylaxis or those with high-grade VUR due to increased risk of
renal scarring. One option is ambulatory cystoscopic injection of periureteral bulking agent
(deflux procedure). Another surgical option is ureteric implantation which is more invasive, but
has a higher success rate.
PREP Pearls
• Antibiotic prophylaxis decreases the risk of recurrent urinary tract infection (UTI) in
children with vesicoureteral reflux (VUR); prophylaxis is indicated for children with low-
grade VUR with recurrent UTI, grade III or higher VUR, or non-toilet-trained children
with any grade of VUR.
• Trimethoprim-sulfamethoxazole (TMP/SMX) is preferred for prophylaxis in children
with vesicoureteral reflux because of its bactericidal action, despite a first urinary tract
infection with an organism showing resistance to TMP/SMX.
• Amoxicillin is recommended for infants younger than 2 months when prophylaxis is
indicated to prevent urinary tract infection.
MOCA-Peds Objective
• Evaluate and manage infants and children with acute urinary tract infection

• Plan the appropriate prophylaxis for urinary tract infection
• Recognize the clinical findings associated with urinary tract infection in children of
various ages
• Recognize the clinical findings associated with reflux nephropathy
Suggested Readings
• Urinary tract infections Pediatr Rev. doi: 10.1542/pir.2017-0007
• Urinary tract Infections in children: knowledge updates and a salute to the future. Pediatr
Rev. doi: 10.1542/pir.36-4-153
• Obstructive uropathy and vesicoureteral reflux. American Academy of Pediatrics
Textbook of Pediatric Care. 2nd; 2406-2415.

Question 64
A 7-year-old boy was bitten on the hand by a stray dog in his neighborhood yesterday. His
parents immediately washed the hand with soap and water. Today the area is red, swollen, and
painful with range of motion. He has not had a fever. The dog was captured by animal control
officers; it is acting normally and remains in their custody. The child is up to date with his
immunizations and has an allergy to penicillin.
Of the following, the MOST appropriate therapy at this time is

A. amoxicillin-clavulanate orally
B. clindamycin and trimethoprim-sulfamethoxazole orally
C. doxycycline intravenously
D. rabies vaccine and rabies immunoglobulin

Correct Answer: B
The boy in the vignette has developed cellulitis after sustaining a dog bite. Wound infections
after animal bite such as this are polymicrobial. The most common pathogens in dog, cat, or
mammal bites are Pasteurella multocida, Staphylococcus, Streptococcus, Capnocytophaga,
Moraxella, Fusobacterium and Bacteroides species. The oral drug of choice for treatment of
animal bites is amoxicillin-clavulanate. However, this boy has an allergy to penicillin; the most
appropriate alterative treatment for infection caused by dog bite is clindamycin plus
trimethoprim-sulfamethoxazole. A broad-spectrum cephalosporin plus clindamycin is another
acceptable oral alterative. Although doxycycline can be used to treat cellulitis caused by a dog
bite, the child in this vignette is less than 8 years old and therefore treatment with doxycycline is
contraindicated due the risk of dental enamel hypoplasia and yellow tooth discoloration.
Therefore, treatment with intravenous doxycycline would not be appropriate for this child.
The decision to initiate post-exposure prophylaxis for rabies requires careful consideration of
multiple factors, including the type of animal, whether or not the animal is displaying signs of
rabies, and the ability to observe the animal for 10 days. In the case of bats, postexposure
prophylaxis should be given for any bite, scratch, or mucous membrane exposure if the animal
cannot be collected and tested. In the case of dogs, cats, and ferrets, the animal should be
quarantined for 10 days. It the quarantined animal remains healthy; rabies treatment is not
necessary. If the animal becomes ill or develops signs of rabies, the animal should be euthanized
humanely and the brain examined for evidence of rabies. If the animal is infected, the child
should be immediately vaccinated. The dog that bit the boy in the vignette is acting normally and
is in the custody of animal control, where it can be carefully observed for signs of rabies.
Therefore, initiation of rabies vaccination and immunoglobulin is not necessary for the boy at
this time. As with bats, other carnivores, including foxes, raccoons, and skunks, should be
considered rabid unless proven negative by laboratory testing.
The most common complication of bite wounds is wound infection. The proper management of
bite wounds starts with a thorough history and physical examination. Important history elements
include: the type of animal, animal behaviour at the time of the bite, the vaccination status of the
animal, and when the bite occurred. Physical examination should include evaluation and
exploration of the wound, including assessment for involvement of deep structures. If the wound
crosses over a joint, the joint should be meticulously evaluated. Radiography or computed
tomography imaging may be appropriate to evaluate occult injuries. The wound should be
cleaned with soap and copious irrigation with 1 to 2 liters of normal saline or sterile water. High-
pressure irrigation should be avoided in puncture wounds. The wound should be debrided as
necessary. If the wound displays signs of infection, it is advisable to obtain wound cultures. The
need for tetanus immunization and/or rabies prophylaxis must be determined. Any bite wound
treated on an outpatient basis should be reevaluated within 48 hours.

PREP Pearls
• Amoxicillin-clavulanate is the drug of choice for treatment of dog, cat, or other mammal
bites. For children with penicillin allergy, oral alternatives are extended-spectrum
cephalosporin plus clindamycin or trimethoprim-sulfamethoxazole plus clindamycin.
• Rabies prophylaxis is not required for common household animals if the animal is acting
normally and can be observed for 10 days for signs or symptoms of rabies. In the case of
bats, postexposure prophylaxis should be given for any bite, scratch, or mucous
membrane exposure if the animal cannot be collected and tested
• Appropriate wound evaluation and care is essential in the management of animal bites
and wounds.

• Plan the appropriate antimicrobial management of a dog or cat bite
• Understand the appropriate steps to take with regard to an animal that has bitten a patient
Suggested Readings
• Human and animal bites. Pediatr Rev. 2018; doi: 10.1542/pir.2017-0212.
• Animal and human bites. In: American Academy of Pediatrics Textbook of Pediatric
Care; 2017:1717-1719.
• Pasteurella multocida. Pediatr Rev. doi: 10.1542/pir.2017-0178.

Question 65
A 5-year-old boy is brought by his father fora health supervision visit. He excitedly states that
his aunt, a sports shooter, has invited him to a sleepover that night and take him to watch her
perform in a shooting exhibition the next day.
Of the following, the MOST important advice to give this boy's father is that he should
A. ask his aunt to remove all firearms before sleepover
B. ask how firearms are stored at his aunt’s house
C. not allow him to sleepover at his aunt’s house
D. teach him how to handle a firearm safely

Correct answer: B
The father in the vignette should ask how his aunt's stores firearms. If the firearms are stored
safely and his father is comfortable, then the boy may be allowed to stay at his aunt’s house.
Asking his aunt to remove all firearms before the sleepover is impractical and potentially
damaging to the relationship. Not allowing him to stay over at all will not lead to open
conversation and may also harm their relationship. A 5-year-old is not able to learn how to
handle a firearm safely and reliably.
Firearm safety education should be performed at every health supervision visit and at any visit
during which safety concerns arise. Item C65 lists sample questions and anticipatory guidance
points from Bright Futures Guidelines. The best way to prevent firearm-related injuries is to
make firearms unavailable in homes and communities. Short of that, safe storage practices are
protective against firearm related injuries. Firearms should be stored unloaded in a locked box
with ammunition stored in a separate location in its own locked box with the keys or
combination kept inaccessible to children.
Item C65 firearm safety sample questions and anticipatory guidance

• Sample Questions
o Do you keep firearms at home?
o Are they unloaded and locked?
o Is the ammunition locked and stored separately?
o Are there firearms in the homes where you visit, such as the homes of grandparents,
other relatives, or friends?
• Anticipatory Guidance
o Homicide and suicide and unintentional firearm injuries are more common in homes that
have firearms.
o The best way to keep your child safe from injury or death from firearms is to never have a
firearm in the home.
o If it is necessary to keep a firearm in your home or if the homes of people you visit have
firearms, they should be stored unloaded and locked, with the ammunition locked
separately from the firearm.
o Make sure the firearm is stored safely before your baby starts crawling and exploring
your home.
Having firearms at home increases the risk of fatal suicide. All adolescents and their families,
especially those with mood disorder, substance abuse, or a history of suicide attempts, should be
asked about access to firearms in and outside the home and counseled on access. Pediatricians
should meet privately with adolescents to ask and answer questions honestly and provide
counseling confidentially.
Gun safety and injury prevention strategies can be accessed at

https://www.aap.orq/en/patient-care/gun-safety-and-injuy-prevention/
The AAP also has produced a 2 minute educational vide on firearm safety
http://youtube.com/watch?v=hzQqMo2-ar8

PREP Pearls
• Pediatricians should ask families at every health supervision visit about access to and
storage of firearms outside and inside the home.
• Access to firearms increases the risk of a fatal suicide.
• Short of making firearms unavailable, safe storage is protective against firearm-related
injuries.

• Counsel parents and adolescents regarding firearm safety in the home
Suggested Readings
• Council on Injury, Violence, and Poison Prevention Executive Committee, Firearm-
related injuries affecting the pediatric population. Pediatrics doi: 10.1542/peds.2012-2481
• Preventing gun injuries in children Pediatr Rev. doi: 10.1542/pir.36-2-43
• Bright Futures: 4th ed. 2017

Question 66
A 15-year-old adolescent girl is seen for completion of paperwork for an overnight school trip.
She has no significant medical history and takes no medication. Her review of systems is
significant for weight gain of 9 kg over the past 2 years and regular menses. Menarche was at
age 12 years and menses have occurred every 2 to 3 months since that time. She does not eat
breakfast, eat lunch at school, snacks when she comes from school, and eats dinner at home. She
does not exercise regularly. Her family history is significant for hypothyroidism in her mother
and diabetes in her father and paternal grandmother. Vital signs show a heart rate of 96 beats/min
and blood pressure of 126/84 mmHg. Her weight is 92 kg (98th percentile) Height is 168 cm (80th
percentile) and body mass index is 32.6 kg/m2 (98th percentile. She has acanthosis nigricans over
the nape of her neck and fresh-colored striae over her abdomen. Her physical examination
findings are otherwise unremarkable.
Of the following, the best test to order is a

A. 24-hour urine free cortisol
B. Fasting insulin level
C. Hemoglobin A1c level
D. TSH level

Correct answer: C
The adolescent in the vignette has obesity (body mass index [BMI] ≥95th percentile for age and
gender) and risk factors for type 2 diabetes. The best test to order is a hemoglobin Alc level to
screen for prediabetes and type 2 diabetes.
The American Diabetes Association recommends screening for prediabetes and type 2 diabetes
in at-risk children and adolescents starting at age 10 years or after pubertal onset whichever is
earlier. Screening is recommended in those with overweight status (BMI ≥85th percentile) or
obesity (BMI ≥95th percentile) and at least 1 of the following risk factors:
1. Maternal diabetes, including gestational diabetes while pregnant with the individual
2. Family history of type 2 diabetes in a first- or second-degree relative
3. Signs of or conditions associated with insulin resistance such as acanthosis nigricans,
hypertension, dyslipidemia polycystic ovary syndrome, or small for gestational-age birth
weight.
For the girl in the vignette, risk factors include her family history of type 2 diabetes in a first
(father) and second-degree (paternal grandmother) relative, and acanthosis nigricans. Her
irregular menses are suggestive of polycystic ovary syndrome, also a risk factor.
Screening options for prediabetes and diabetes include obtaining one of the following
1. Fasting plasma glucose (≥100 mg/dL is impaired fasting glucose, ≥125 mg/dL is
diagnostic for diabetes)
2. 2-hour plasma glucose during a 75-g oral glucose tolerance test (≥140 mg/dL is impaired
glucose tolerance, ≥200 mg/dL is diagnostic for diabetes)
3. Hemoglobin A1c (≥5.7% is classified as prediabetes. ≥6.5% is diagnostic for diabetes)
Unless there is unequivocal hyperglycemia, a second abnormal test meeting the diagnostic
criteria for diabetes is needed to make the diagnosis. If the diagnosis of diabetes is made in a
child or adolescent suspected of having type 2 diabetes obtaining a panel of pancreatic
autoantibodies is recommended to rule out type 1 diabetes.
The constellation of risk factors for type 2 diabetes, along with risk factors for cardiovascular
disease, contribute to metabolic syndrome. Components of metabolic syndrome include obesity
glucose intolerance, hypertension, and dyslipidemia. Obesity and insulin resistance play key
roles in the pathogenesis of type 2 diabetes incorporates both insulin resistance and relative
pancreatic β-cell failure.
An Endocrine Society Pediatric Obesity Guideline recommends that children and adolescents
with a BMI ≥85th percentile be screened for complications and comorbidities of obesity. These
complications include glucose intolerance, dyslipidemia, hypertension, non-alcoholic fatty liver
disease, polycystic ovary syndrome (in females), obstructive sleep apnea, and psychiatric
comorbidities.
The guideline also recommends against measuring insulin levels as part of an evaluation for
obesity due to lack of diagnostic value. The guideline further recommends against routine testing
for endocrine etiologies of obesity without other signs and symptoms. The girl in the vignette has
no signs or symptoms of Cushing syndrome or hypothyroidism, so measurement of 24-hour
urine free cortisol and thyroid stimulating hormone levels is not indicated. Her flesh-colored
striae are a common consequence of weight gain. Striae form Cushing syndrome are thick and
purple or red colored
PREP Pearls
• Screening for prediabetes and type 2 diabetes is recommended in children and
adolescents with a body mass index ≥85th percentile and at least 1 other risk factor
(family history or evidence of insulin resistance)
• Screening options for prediabetes and diabetes include obtaining levels for fasting plasma
glucose, 2-hour plasma glucose during a 75-g oral glucose tolerance test, or hemoglobin
A1c.
• Components of metabolic syndrome include obesity, glucose intolerance, hypertension,
and dyslipidemia.

• Understand the epidemiology of and risk factors associated with type 2 diabetes
• Plan an appropriate screening evaluation for a patient in whom type 2 diabetes is
suspected
Suggested Readings
• Children and adolescents: Standards of medical care in diabetes 2021.American Diabetes
Association.S180-S199. 10.2337/dc21.S013.
• Classification and diagnosis of diabetes: Standards of medical care in diabetes-—2021.
American Diabetes Association. Diabetes Care. S15-S33. 10.2337/dc21.S002
• Type 2 diabetes mellitus in children and adolescents. Pediatr Rev doi:10.1542/pir.34-12-
541
• Diabetes mellitus. In: American Academy of Pediatrics Textbook of Pediatric care.
2017:1982-1962,
• Pediatric obesity-assessment treatment, and prevention: doi:
https://doi.orq/10.1210/jc.2016-2573.
• Metabolic syndrome in children and adolescents. doi: 10.1542/pir.2014-0095

Question 67
A 7 years old boy is being evaluated for “white spots” on his face that developed 6 weeks ago.
He has no associated symptoms and has been in good health. Physical examination findings are
notable only for the lesions shown in Item Q67.
Of the following, the most appropriate treatment is

A. Clotrimazole topically
B. Emollient topically
C. Triamcinolone topically
D. Ultraviolet B phototherapy

Correct Answer: B
The boy in the vignette has hypopigmented scaling macules on his face. The borders of the
lesions are not sharply demarcated; rather, there is a gradual transition from normal to abnormal
pigmentation. These features are most consistent with pityriasis alba, and treatment with an
emollient is appropriate. Some clinicians treat with a low potency topical corticosteroid (e.g.,
hydrocortisone 1% or 2.5%) for 7 days, but using a mid-potency preparation like triamcinolone
is not advisable because of the potential for skin atrophy.
Pityriasis alba is a form of post inflammatory hypopigmentation that often occurs in children
who have a history of atopic dermatitis. Lesions commonly involve the face but may occur on
the trunk and extremities. Pityriasis alba often becomes apparent after sun exposure because
normal skin tans but affected areas do not. The diagnosis is made clinically based on the
characteristic appearance of lesions. Regardless of the treatment selected, the patient and family
should be counseled that the return of normal pigmentation takes months. In addition, they
should be instructed to treat new lesions (i.e., pink, scaling macules) with a low-potency topical
corticosteroids to prevent new areas of hypopigmentation.
Tinea versicolor, a fungal infection caused by Malassezia species, is not commonly seen in
children. Findings include hypopigmentation and/or hyperpigmented macules with well-defined
borders (Item C67A) that occasionally involve the face. Localized infection may be treated with
clotrimazole.
Item C67A: Tinea versicolor involving the face. There are well-defined hypopigmented
macules. Courtesy of D. Krowchuk
The lesions of vitiligo are depigmenting macules or patches with well-defined borders (Item
C67B); scaling is not present. First line treatment is usually a topical corticosteroid or
calcineurin inhibitor. Other options include narrowband ultraviolet B phototherapy,
Photochemotherapy using psoralen and ultraviolet A, and excimer laser

Item C67B: In vitiligo, lesions are well-defined depigmented macules or patches. In this
patient, some areas have begun repigmenting. Courtesy of D. Krowchuk
PREP Pearls
• Pityriasis alba is characterized by hypopigmented macules that may have associated
scale. The borders are indistinct, with a gradual transition normal to abnormal
pigmentation.
• Pityriasis alba is a form of post inflammatory hypopigmentation that often occurs in
children who have a history of atopic dermatitis.
• Treatment of pityriasis alba is with an emollient or a short course (7 days) of low-potency
topical corticosteroid
MOCA-Ped Objective
• Evaluate and manage a child with a disorder of the scalp.

• Recognize the clinical manifestations of vitiligo
• Recognize the clinical features of pityriasis alba
Suggested Readings
• American Academy of Pediatrics Section on Dermatology. Pityriasis alba Pediatric
Dematology: A Quick Reference Guide. 4th ed. Itasca, IL: American Academy of
Pediatrics; 2020:429431
• A practical approach to the diagnosis and treatment of vitiligo in children. Pediatrics.
10.1542/peds.2015-4126
• Atopic dermatitis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed.
Itasca, IL: American Academy of Pediatrics; 2017:1754-1758. Pediatric care Online.

Question 68
A neonate born prematurely to a 35-year-old primigravida mother due to maternal preeclampsia,
is seen in the neonatal intensive care unit. The mother recently emigrated from Southeast Asia
and did not receive prenatal care in United States. The results of maternal hepatitis B virus
screening are pending. The mother’s laboratory findings include an elevated alanine
aminotransferase level of 1,500 U/L and aspartate aminotransferase of 900 U/L. the neonate’s
weight is 1,500 g (3 lb., 5 oz); physical examination findings are normal with no hepatomegaly.
Of the following, the BEST next step in the management of this neonate is to administer
A. Hepatitis B vaccine within 24 hours of birth
B. Hepatitis B vaccine +/- immunoglobulin is indicated when maternal hepatitis results are
available
C. Hepatitis B vaccine now and immunoglobulin within 7 days
D. Hepatitis B vaccine now and immunoglobulin within 12 hours of birth

Correct Answer: D
For the neonate in the vignette, the Centers for Disease Control and Prevention recommends
administration of hepatitis B vaccine at birth and hepatitis B immunoglobulin (HBIG) within 12
hours (Item C68). The mother's hepatitis B virus (HBV) infection status unknown. She recently
emigrated from Southeast Asia, an endemic region for HBV infection, her liver enzyme levels
are elevated, and the neonate's birth weight is 1,500 g (<2,000 g). This infant should continue the
vaccine series beginning at 1 to 2 months of age based on mother's hepatitis B surface antigen
(HBsAg) result (i.e., administer 3 additional hepatitis B vaccine doses with single-antigen
vaccine at ages 1, 2 to 3, and 6 months or a hepatitis B-containing combination vaccine at ages
2.4, and 6 months).
Item C68: Administration of the birth dose of hepatitis B vaccine by maternal HBsAg status
Hepatitis B virus is an enveloped virus with a partially double-stranded DNA. Southeast Asia,
China, and Africa are endemic regions. Hepatitis B virus is primarily transmitted through blood
or other body fluids. Perinatal transmission typically occurs from maternal blood exposure
during childbirth. All pregnant women should be routinely screened for the presence of HBsAg.
Hepatitis B surface antigen is detectable during acute and chronic infection (acute if <6 months
and chronic if >6 months). In a self-limited hepatitis B infection, HBsAg disappears followed by
the appearance of anti-HBs (hepatitis B surface antibody). The interval between the
disappearance of HBsAg and appearance of anti-HBs called the “window period”. During this
time frame, the only marker of acute infection is IgM anti-HBc (hepatitis B core antibody),
which suggests acute infection. However, IgM anti-HBG is usually not present in infants who are
infected perinatally. People with chronic HBV infection have circulating HBsAg and total anti-
HBc. People with resolved infection have anti-HBs and total anti-HBc. People immunized with
hepatitis B vaccine have anti-HBs alone. Passively transferred maternal anti-HBc is detectable
for as long as 24 months among infants born to HBsAg-positive women.
In the United States, universal immunization programs to eradicate perinatal HBV transmission
have been implemented. The core elements include universal HBV immunization beginning at
birth and postexposure prophylaxis with HBIG in neonates born to mothers with a positive
HBsAg or unknown HBV status.
Hepatitis B vaccine is more than 90% effective in preventing infection. Immunization with HBV
vaccine is recommended for all infants, children, and adolescents through 18 years of age. It is
administered as a series or 3 doses (0.5 mL/dose) on a 0-, 1-, and 6 months schedule (0-, 2-, and
6- months is acceptable with combination vaccines)
Hepatitis B immunoglobulin prophylaxis is recommended for neonates born to mothers with

positive HBsAg or unknown HBV status. In the absence of post exposure HBV prophylaxis, the
risk of acquiring HBV from the infected mother 70% to 90% for infants born to mothers who are
HBsAg and HBeAg positive. HBV acquisition risk is 5% to 20% for infants born to HBsAg
positive but HBeAg negative mothers. HBV immunoprophylaxis is most effective if
administered within 12 hours of birth. Data on its effectiveness when administered between 25
hours and 7 days after birth is limited. Breastfeeding should not be delayed after the infant is
immunized.
HBsAg and Anti-HBs should be checked in infants born to HBsAg-positive mothers 1 to 2

months after the last vaccine dose (test ≥9 months of age).
PREP Pearls
• All neonates (birth weight≥ 2000 g) should receive hepatitis B vaccination within 24
hours of birth.
• Neonates born to mothers with positive hepatitis B surface antigen or unknown status
should receive hepatitis B vaccination and immunoglobulin within 12 hours when birth
weight is <2,000 g or within 7 days for those weighing ≥2,000 g.
• Infants born to hepatitis B surface antigen-positive mothers should have post-
immunization testing for hepatitis B surface antigen and anti-HBs (hepatitis B surface
antibody) performed 1 to 2 months after the last vaccine dose (not before 9 months of
age).

• Know the indications and schedule for hepatitis B vaccine in patients of various ages,
including those born prematurely
• Understand the importance of follow-up screening evaluations for hepatitis B virus
infection
• Recognize the clinical features associated with hepatitis B virus infection

Suggested Readings
• American Academy of Pediatrics, Committee on Infectious Diseases and Committee on
Fetus and Newborn Elimination of perinatal hepatitis B: Providing the first vaccine dose
within 24 hours of birth. Pediatrics doi:10.1542/peds.2017-1870
• American Academy of Pediatrics. Hepatitis B. In: Red Book: 2021-2024 32nd ed.
2021:381.398. Red Book Online.
• Centers for Disease Control and Prevention. A comprehensive immunization strategy to
eliminate transmission of hepatitis B virus infection in the United States:
Recommendations or the Advisory Committee on Immunization Practices (ACIP).
PMID: 17159833
• Hepatitis A, B, and C Pediatr Rev. 2016; 101542/pir.2015-0075
• Update: shortened interval for post vaccination serologic testing of infant born to
hepatitis B-infected mothers.
https://www.cdc.gov/mmwr/preview/mmwrhtml/mm6439a6.htm

Question 69
A 15-year-old adolescent girl is seen for a routine health supervision visit. During a recent soccer
practice, her coach expressed concern that she looks “knock-kneed” when running, jumping, or
walking. The adolescent reports occasionally peripatellar pain but is currently pain-free. She is
teased about her running and is self-conscious about her gait. She is otherwise health with
normal growth and development. Lower extremity examination findings include 80 degrees of
pain-free hip internal rotation and standing alignment as shown in Item Q69. The reminder of
her physical examination findings are normal
Of the following, the BEST next management step for this adolescent is
A. Hip radiography
B. Orthopedic consultation
C. Patellar bracing
D. Reassurance

Correct Answer: D
The adolescent in the vignette has femoral anteversion. The best next management step for her is
reassurance. Femoral version describes the relationship between the head and shaft of the femur.
In femoral anteversion (i.e., medial femoral torsion), the femoral shaft is internally rotated
relative to the femoral head. Older children with femoral anteversion may have knee pain and
concern about the impact of anteversion on their appearance and sports performance. Although
femoral anteversion in in children older than 10 to 12 years of age is likely to persist through
adulthood, families can be reassured that there is no association with impaired function,
diminished athletic performance, or increased risk for hip osteoarthritis in adulthood.
Femoral alignment evolves throughout childhood. Femoral anteversion is about 40 degrees in

newborns as a result of intrauterine positioning. In about 80% of children the angle regresses to
the normal adult value of 10 to 15 degrees by 8 to 10 years of age. Children with internal hip
rotation greater than 70 degrees have increased femoral anteversion, with severity classified as:
• Mild: 70-80 degrees
• Moderate: 80-90 degrees
• Severe: >90 degrees
Femoral anteversion is the most common cause of in-toeing in children older than 3 years,
whereas internal tibial torsion is the most common cause in children younger than 3 years. It is
often noted when a caregiver expresses concern about clumsiness or an awkward gait. Physical
examination should include:
• Visual inspection:
o Children with femoral anteversion will have "squinting patellae” with the patellae
angled medially (Item C69A). Sometimes, this finding is best seen with the child
standing (Item C69B) or examined from a superior view (Item C69C).
o Children with internal tibial torsion will have patellae that are forward facing and
a foot that points toward the midline.
• Range of motion testing: Assessment of hip internal and external range of motion (Item
C69D and Item C69E).
• Gait mild findings noted on examination with the child standing may become more
prominent walking and running.
Item C69A, Item C69B, Item C69C

Item C69D and Item C69E
Hip radiography should be performed if the child has significant knee pain, hip pain, or restricted
hip range of motion, but would not be indicated for adolescent in the vignette.
Families are often reassured to learn that an adult gait pattern is typically not established until 6
to 8 years of age, and that most cases of in toeing spontaneously resolve. Nonoperative
treatments, including bracing and orthopedic footwear or inserts, have not been shown to alter
the natural history of femoral anteversion; they are no longer recommended. Controversy exists
regarding the impact of “W” sitting on femoral head remodeling, but current evidence suggests
that W sitting does not affect the natural history of femoral anteversion.
Orthopedic consultation is rarely needed in children without underlying medical conditions (e.g.,
cerebral palsy, collagen vascular disorders). Surgery may be indicated for severe femoral
anteversion that persists and creates significant fractional impairment. In-toeing may have
greater impact on children and adolescents with conditions affecting strength or coordination;
these children may benefit from physical therapy. Although bony alignment cannot be changed
via nonsurgical measures, changes in dynamic alignment can occur with rehabilitation. Poor hip
strength does not cause femoral anteversion, but ensuring adequate strength may reduce the
mechanical strain across the patellofemoral joint and improve knee pain. Examples of core
exercises can be found
http://www.healthychildren.org/English/healthy-living/fitness/Pages/Core-Exercises-Guidelines-
and-Examples.aspx
Neuromuscular training (i.e., training movement patterns in addition to strength) with a physical
therapist can decrease knee pain, but a knee brace alone would not be expected to result in
significant improvement.
PREP Pearls
• Femoral anteversion is the most common cause of in-toeing in children order than 3 years
whereas internal tibial torsion is the most common cause in children younger than 3
years.
• Femoral anteversion is not associated with impaired function, diminished athletic
performance, or increased risk for hip osteoarthritis in adulthood.
• Femoral anteversion may contribute to anterior knee pain in older children and
adolescents; knee pain is usually successfully addressed with appropriate rehabilitation.

• Recognize the clinical findings associated with tibial torsion
• Plan the appropriate clinical and diagnostic evaluation of femoral anteversion
• Understand the natural history of femoral anteversion
Suggested Readings
• Rotational and angular deformities: General treatment guidelines, Pediatric Orthopaedics
and sport Injuries. American Academy of Pediatrics; 2010: 199-212.
• Lower extremity disorders in children and adolescents. Pediatr Rev doi:10.1542/pir.30-
8-287.
• Foot and leg problems. American Academy of Pediatrics Text book of Pediatric Care.
2017:1366-1378.

Question 70
After a serious safety event that involved a 10-fold overdose of a narcotic, a physician is asked to
join an interprofessional team to identify potential safety interventions to decrease the risk of
medication overdoses. After reviewing this particular case and others in the hospital system, the
team presents their recommendation for an intervention that will have a long-lasting impact.
Of the following, the BEST recommendation is to implement a (n)

A. computer order entry with hard stops to prevent ordering and administering doses of
high-risk drugs outside the recommended range
B. double check system requiring that all high-risk drugs be checked by 2 nurses prior to
administration
C. online education program for nurses and providers about ordering and administering
high-risk drugs
D. policy limiting who can administer and order specific high-risk drugs

Correct Answer: A
The patient in the vignette received 10-fold overdose of a narcotic after which an
interprofessional team met to discuss strategies for error prevention. While all of the choices
offered are strategies used in error prevention, creating hard stops with forced function in an
electronic ordering system in order to provide doses in recommended ranges has been shown to
be a more beneficial strategy for error prevention (Alotaibi YK,Federico F)
Preventable adverse drug events are one type or adverse event. Adverse drug events cause
patient harm and death and cost the health care system an estimated $42 billion a year. The
pediatric population is especially vulnerable, given the dosing-by-weight system and the age
specific physiologic and developmental variation
The literature demonstrates that several initiatives have made a positive impact on preventing
adverse events. Computer order entry alone has not had a significant impact, but when coupled
with a clinical decision support system, a reduction in error has been seen. Clinical decision
support systems can link a provider, in real time to a formulary, an order set or a clinical
guideline. Alerts (a forcing function) can be designed to prevent medication overdoses or the
ordering of a medication listed as an allergy. Other technologic aids that have been shown to be
beneficial include electronic handoff tools, barcode-scanning systems for patient identifications,
smart pumps to deliver infusions in approved ranges, automated medication dispensing
technology, and patient electronic portals.
PREP Pearls
• Adverse drug events lead to patient harm including death, as well as a loss of billions of
dollars each year.
• Initiatives that have a significant impact on preventing adverse drug events include
computer order entry coupled with clinical decision support system, electronic handoff
tools, barcode scanning for medications, and smart infusion pumps.
MOCA-Peds Objective
• Recognize the factors that relate to risk of error or reduced patient safety
• Recognize the common causes of adverse events in pediatric patients
• Understand the contribution of adverse events to the morbidity and pediatric patients
• Understand the contribution of adverse events to the cost of medical
Suggested readings
• The impact of health information technology on patient safety. Saud Med J doi:
10.15537/smj.2017.12.20631.
• Preventive adverse drug events among inpatients: A systematic review. Pediatrics doi:
10.1542/peds.2018-0805.
• Patient safety and quality improvement: Medical errors and adverse events. Pediatr Rev.
doi:10.1542/pir.31-4-151
• Adverse events in hospitalized pediatric patients. Pediatrics. Doi:10.1542/peds.2017-
3360

Question 71
A 12-year-old girl has had a chronic dry cough for 6 months. She also notes recent onset of
shortness of breath with exercise. A therapeutic trial of inhaled albuterol has not changed her
cough or exercise symptoms. Spirometry shows forced vital capacity (FVC) to be 79% of that
predicted for age and height, forced expiratory volume in 1 second (FEV1) 85% predicted, and
the FEV1/FVC ratio 0.94 (Item Q71). Restrictive lung disease is suspected.
Of the following, the BEST next study to confirm the suspected diagnosis for this girl is
A. carbon monoxide diffusing capacity
B. fraction of exhaled nitric oxide
C. lung volumes with body plethysmography
D. methacholine inhalation challenge test

Correct Answer: C
The girl in the vignette has a mild restrictive lung disease process with decreased forced vital
capacity (FVC) but normal forced expiratory volume in 1 second (FEV1) and FEV1/FVC ratio.
Measurement of total lung capacity (TLC) will confirm a restrictive process. Lung volumes can
be measured with plethysmography (body box) or nitrogen or helium dilution techniques. In
spirometry, FVC is used as a marker for TLC, assuming that the compartments or lung volume
are normally distributed. Functional residual capacity (FRC), residual volume (RV) and RV/TLC
ratio are also helpful in further defining restrictive and obstructive lung disease. The
relationships of compartments within TLC can be seen in Item C71A and the patterns predicted
by spirometry and lung volume results in Item C71B.
Item C71A: TLC compartments
Item C71B: spirometry and lung volume

Forced vital capacity and TLC are the best measures of loss or restriction of lung volume. The
FEV1 value and the FEV1/FVC ratio are the best measures of airflow obstruction. There are
many historic normative data sets against which a patient's pulmonary function results are
compared, and predicted values are standardized for age and height. Most of these normative sets
represent white populations. In general, the best dataset to use most closely mimics the
characteristics of the population from which the subject comes. Most recently, however, the
accepted standard dataset has come from the Global Lung Initiative For children, there is no
difference by sex, but for adults, the normative data differs by sex as well as age and height.
Generally, 80% or greater of the predicted values is considered to be a normal result for FVC and
FEV1. Based on the 2007 National Institutes of Health Guidelines for the Diagnosis and
Management of Asthma the FEV1/FVC ratio should be greater than 0.85 for children of age 17
years or less. This was not addressed in the 2020 update of the NIH guidelines and has not
changed from the original recommendation. Forced expiratory now between 25% and 75% of
FVC (FEF 25/75) is a marker of airflow in smaller, more peripheral airways, this is a more
variable function and the lower limit of normal is 65% of the predicted value for age and height.
If airflow obstruction is severe enough to produce air trapped in peripheral airways, measured
residual volume will be elevated. Total lung capacity is the sum of RV and vital capacity; thus,
an elevated RV/TLC ratio is indicative of air trapping. Vital capacity will be reduced because
RV is elevated, but TLC does not change. For the girl in the vignette, lung compartments are
normally distributed and TLC is reduced with a normal RV/TLC ratio, indicative of a restrictive
process (Item C71C)
Item C71C
Carbone monoxide diffusing capacity represents the ease with which gases are transported across
alveolar membranes. It is reduced in diseases involving inflammation or thickening of the
alveolar membrane, including sickle cell disease, radiation therapy and cancer chemotherapy,
collagen vascular diseases and also reduced in young adults with a history of prematurity.
Carbon monoxide diffusing capacity is also affected by hemoglobin concentration, capillary
volume, and reaction rates between carbon monoxide and hemoglobin.

The fraction of exhaled nitric oxide (FeNO) is a measure of eosinophilic airway inflammation. It
is sometimes helpful in the diagnosis and management of asthma, as well as in differentiation of
asthma from non-eosinophilic processes. It is used as an adjunct measurement in children of age
5 years or older and adolescents when other parameters do not provide a clear picture or asthma
diagnosis, or as a maker for asthma control. Elevated FeNO is consistent with eosinophilic
inflammation and poor asthma control; normal FeNO is consistent with good control of known
asthma or with an alternative diagnosis rather than eosinophilic asthma. Exhaled nitric oxide is
reduced in ciliary dyskinesia and in cystic fibrosis, both neutrophilic inflammatory processes. It
is not a measure of lung or of airflow and does not indicate restriction or obstruction. Updated
asthma management guidelines (2020) from the National Heart, Lung, and Blood Institute
discuss the use of FeNO in the diagnosis of asthma.
A methacholine challenge test is used to measure the presence and severity of away
hyperreactivity. Methacholine, a cholinergic agonist, is inhaled in increasing concentrations, with
spirometry performed at timed intervals after each dose to evaluate changes in airflow. A
positive test result is defined as a decrease in FEV1 by 20% from pre-challenge baseline at a
concentration of less than 4 mg/ml methacholine. The severity of airway reactivity is defined by
the dose/concentration at which the 20% decline in FEV1 occurs. Lower dose indicates greater
reactivity or more severely hyperresponsive airways.
Office spirometry is often available and most children 6 years or older can do reliable testing
with good coaching but obtaining reproducible results may be challenging. However, testing
beyond spirometry should be done in a dedicated pulmonary function laboratory having technical
staff skilled with pediatric patients. The tests other than spirometry described here require a great
deal of skill on the part of the testing staff to get usable results, particularly in the pediatric
population and sometimes even in adolescent and adult populations.
PREP Pearls
• Decreased forced vital capacity may represent restrictive lung disease, which can be
corroborated by measurement of total lung capacity.
• When lungs are normally distributed, forced vital capacity is a reasonable marker for total
lung capacity.
• Forced expiratory volume in 1 second (FEV1) and ratio of FEV1 to forced vital capacity
are measures of airflow and when reduced indicate obstruction

• Understand the basic terminology and purpose of various pulmonary function tests
Suggested Readings
• Pulmonary function testing. In: Light MJ ed. Pediatric Pulmonology 2011:89-113
• Office spirometry: Indications and interpretation. Am fam Phys. 2020;101:362- 368.
https://www.aafp.org/afp/2020/0315/p362.html.
• Pulmonary function testing in pediatric practice, Pediatr Rev. doi:10.1542/pir.15-10-403
• National Heart Lung and Blood Institute. 2020 Focused Updates to the Asthma
Management Guide. 2020. https://www.nhlbi.nih.gov
• Spirometry Pediatr Rev. doi:10.1542/pir.33-10-469
Question 72
During a sports preparation evaluation of a 17-years-old adolescent boy reports that he has been
vaping. He requests help to quit. He began vaping nicotine 1 year ago at weekend parties with
his friends. He now vapes multiple times every day at school and at home using electronic
cigarette device that looks like a computer flash drive. He has episodes of irritability and
shakiness and if he cannot vape, he will smoke a cigarette. He has tried gradual reduction and
“cold turkey” methods that resulted in only 3 to 4 days of abstinence beyond his quit date. He
does not smoke or vape marijuana. He is otherwise health and has no history of alcohol or other
substance use, depression, or anxiety. He has no problem at school, has a part-time job, and plans
to attend college. His parents do not know about his e-cigarette use. His physical examination
findings are unremarkable.
Of the following, the MOST appropriate pharmacologic management to recommend for this
patient is
A. Aripiprazole
B. Citalopram
C. Fluoxetine
D. Nicotine replacement therapy

Correct Answer: D
The adolescent in this vignette has experienced withdrawal symptoms consistent with a nicotine
addiction and like many adolescents who use electronic cigarettes; he has progressed to the use
of tobacco products. Nicotine replacement therapy (NRT) may be considered as part of a
cessation plan for this otherwise healthy motivated adolescent. The other choices would not be
considered without a co-occurring psychiatric condition.
The use of NRT for the management of nicotine withdrawal is based on treatment of adults; its
use is not approved by the US Food and Drug Administration younger than 18 years. The risks
and benefits for its use must be weighed for each adolescent patient. In general, the safety profile
of NRT and the risks of continued exposure to e-cigarettes and/or other tobacco products
supports its use in adolescents, albeit in the absence of efficacious clinical trials. Nicotine
replacement therapy is available in both short acting (lozenges and gum) and long-acting (patch)
forms: instructions, adverse effects, and advantages are reviewed in Item C72A and dosing
guidelines are reviewed in Item C72B. A combination of long- and short-acting forms may be
useful for the management of withdrawal symptoms. Although available over the counter for
adults, the patch, lozenges, and gum require a prescription for use by children younger than 18
years.
Item C72A, Item C72B Nicotine replacement therapy

Nicotine Transdermal Dosage:
Patch • 21mg, 14mg, 7mg
(OTC for 18+ Use Instructions:
Rx for <18) • Apply patch to clean skin, change patch every 24 hours
• 8-10 week treatment regimen:
Cost: o Use first dose for 6 weeks, then “step down” to lower dose
Over-the-counter retail cost o Use lower dose for 2 weeks, then “step down” to lowest dose for
ranges from $25-$70 for 28 2 more weeks
patches. • See package for full details
Side Effects:
Out-of-pocket prescription • Skin Irritation, sleep disturbance
costs will vary by insurance Advantages:
plan. • Sustained blood levels of nicotine, compliance is relatively easy
Nicotine Gum Dosage:
(OTC for 18+ • 4mg, 2mg
Rx for <18) Use Instructions:
• “Chew and park” method:
Cost: o Place the gum in your mouth and chew until you feel a tingling
Over-the-counter retail cost sensation
ranges from $17-$50 for 100 o Stop chewing and “park” the gum between cheek and gums
pieces of gum. o After about a minute, start chewing again, until you feel a
tingling sensation
o Stop chewing and “park” the gum again
Out-of-pocket prescription o Repeating for about 30 minutes
costs will vary by insurance • 12-week treatment regimen:
plan. o Chew 1 piece every 1-2 hours for first 6 weeks
o Chew 1 piece every 2-4 hours for 3 additional weeks
o Chew 1 piece every 4-8 hours for 3 additional weeks

• See package for full details
Side Effects:
• Jaw soreness, mouth irritation, indigestion, nausea, hiccups
Advantages:
• Flexible dosing, rapid delivery of nicotine into blood stream
Nicotine Lozenge Dosage:
(OTC for 18+ • 4mg, 2mg
Rx for <18) Use Instructions:
• Dissolving method:
o Place lozenge in your mouth, occasionally moving from side-to-
Cost: side
Over-the-counter retail cost o Allow lozenge to slowly dissolve, do not chew or swallow the
ranges from $15-$50 for 100 lozenge
lozenges. o Do not use more than 1 lozenge at a time
• 12-week treatment regimen:
o Use 1 lozenge every 1-2 hours for first 6 weeks
Out-of-pocket prescription o Use 1 lozenge every 2-4 hours for 3 additional weeks
costs will vary by insurance o Use 1 lozenge every 4-8 hours for 3 additional weeks
plan. • See package for full details
Side Effects:
• Oral irritation, nausea, hiccups
Advantages:
• Flexible dosing, rapid delivery of nicotine into blood stream, no
chewing (discrete)
Cessation management involves the use of motivational strategies and a quit plan with or without
pharmaceutical intervention. The components of a quit plan include a firm quit date;
identification of triggers; withdrawal management; behavioral and social support services; self-
care strategies; and discussion of relapses. Any co-occurring psychiatric and behavioral
conditions and/or concomitant use of marijuana and other drugs separately or as additives to
vaping solutions should be addressed. For adolescents, the quit plan should also include a
discussion about parental engagement. The American Academy of Pediatrics, Julius B.
Richmond center of Excellence website, has tools and resources, including linkages to online
support services from the National Institutes of Health (SmokefreeTeen.gov) and Truth initiative
(This is Quitting).
Inhaled nicotine is rapidly absorbed into the systemic circulation, causing an acute increase in
heart rate, respiratory rate, and blood pressure. Highly addictive, nicotine reaches the brain
within seconds, stimulating the dopamine system. Users may transiently experience a decreased
perception of pain and anxiety; heightened levels of alertness and motivation; and positive sense
of pleasure, relaxation, and mood. Adolescents are particularly vulnerable to the properties of
nicotine; dependency and tolerance can occur soon after initiation among adolescent users even
with infrequent low dose use. Adolescent users of e-cigarettes are at risk for subsequent and dual
use of tobacco products and other substances of abuse. The susceptibility of the adolescent brain
to the toxicity of nicotine, particularly the late maturing prefrontal cortex, negatively affects
cognitive development (e.g., processing of information and attention), self-regulation, and mood.
Maternal tobacco smoke during pregnancy has been strongly associated with sudden infant death

syndrome and small-for-gestational age infants. These infants also are at risk for
neurodevelopmental effects and later addiction to nicotine and other substances of abuse.
Tobacco use and vaping are associated with other multisystemic physiologic effects. The long-
term burden of disease associated with tobacco use is well established and includes lung cancer
as well as other cancers, pulmonary and cardiovascular disease, diabetes and other autoimmune
disease, eye disease, and erectile dysfunction. It is the other constituents not nicotine that have
primarily been implicated as a cause of cancer. Nicotine probably has a role as an oncogenic
promoter. Nicotine-induced changes in the endothelium and smooth muscle of blood vessels and
in lipolysis are linked to risks for premature coronary heart disease, vascular disease, and
peripheral vascular disease, and stroke. Nicotine has a role in osteoporosis and bone fractures;
Crohn disease; and progression of renal disease. Vaping has been with associated exacerbation of
asthma and acute presentations of severe lung injury affecting youth and adults.
Behavioral consequences of tobacco and e-cigarette use among adolescents are related to
nicotine addiction and its toxic effects on the maturing brain. Withdrawal symptoms include
craving, anxiety, irritability, blunted feelings of pleasure, decreased ability to concentrate,
shakiness, and insomnia that may have a negative effect on activities of daily life and
relationships with others. Withdrawal may compound problems for adolescents who already
have behavioral issues such as poor attention, anger management, and school performance.
Smoking or vaping at school may result in disciplinary action or suspension. The negative effects
of nicotine use on cognition and mood may compound the challenges of self-control,
impulsivity, and decision making inherent to adolescence. Risky behaviors associated with
tobacco and e-cigarette use, including violence, truancy, 4 of more sexual partners and lack of
condom use, alcohol use, and other drug use are increased among dual users.
PREP pearls
• Off-label nicotine replacement therapy may be considered for adolescents younger than
18 years who have nicotine addiction.
• In adolescents. nicotine use is associated with negative effect on cognition and mood
• Electronic cigarette use often precedes use of other tobacco products and marijuana.

• Identify the major physiologic consequences associated with electronic cigarettes
• Recognize the major behavioral consequences of tobacco use/abuse

Suggested Readings
• American Academy of Pediatrics. Nicotine replacement therapy and adolescent patients:
https://downloads.aap.org/aap/pdf/nrt_and_adolescents_pediatrician_guidance_factsheet.
pdf
• New understanding of health hazards of electronic cigarettes and vaping. Pediatr Rev.
2020;41(3):152-154. doi:10.1542/pir.2019-0269
• Public health considerations for adolescent initiation of electronic cigarettes. Pediatrics.
doi: 10.1542/peds.2019-2056E
• Section on Tobacco Control. E-cigarettes and similar devices. doi: 10.1542/peds.2018-
3652.
• Committee on Substance Use and Prevention. Nicotine and tobacco as substances of
abuse in children and adolescents. Pediatrics doi: 10.1542/peds.2016-3436.

Question 73
A 4-month-old infant born with myelomeningocele is seen for health supervision visit. The
infant underwent an uncomplicated repair 3 days after birth. Brain magnetic resonance imaging
in the neonatal period demonstrates a Chiari II malformation with accompanying
ventriculomegaly. Ventricular size has been stable on serial head ultrasonography. Anticipatory
guidance regarding symptoms caused by Chiari II malformation is provided.
Of the following, the sign and symptom MOST likely to occur with the condition identified on
this child’s brain magnetic resonance imaging is
A. Constipation
B. Scoliosis
C. Swallowing difficulties
D. Urinary retention

Correct Answer: C
Among the response choices, the sign or symptom most likely to occur in a child with Chiari II
malformation is swallowing difficulties.
Myelomeningocele is a complex multisystem congenital malformation due to incomplete closure

of the posterior neural tube, resulting in an exposed meningeal sac that contains a portion of the
spinal cord and nerves. This primary defect leads to secondary defects in brain development,
including Chiari II malformation and hydrocephalus. Additional associated brain anomalies may
include agenesis of the corpus callosum, polymicrogyria, heterotopia, or structural micro-
anomalies that can be associated with later development of learning difficulties and attention
deficit/hyperactivity disorder.
Chiari II malformation, the most common malformation accompanying myelomeningocele,

occurs in the majority of children with myelomeningocele. Decreased fourth ventricular pressure
leads to displacement of the medulla and cerebellum with subsequent development of a smaller
posterior fossa that is unable to accommodate for the growth of those structures. Children may be
asymptomatic or develop symptoms related to brainstem and proximal spinal cord compression,
which can include swallowing difficulties, stridor, hoarse voice, disordered breathing, choking,
breath-holding spells, sudden death (Item C73A). Hydrocephalus is also common and may
present with rapidly expanding head circumference, bulging fontanelle, restricted upward gaze
(sun-setting eyes), VI nerve palsies, or lethargy (Item C73B). Treatment is surgical and, if
hydrocephalus is present, includes placement of a ventriculo-peritoneal shunt, which can help
ameliorate brainstem compression, symptoms of high pressure, or craniovertebral
decompression.

Myelomeningocele results in damage directly to the spinal cord and nerves. Clinical presentation
and functional outcome is dependent on the location of the spinal cord defect, below which there
is impaired motor and sensory function. Three functional categories have been proposed,
subdividing patients based upon defect location and mobility (Item C73C). Virtually all children
experience a neurogenic bowel and bladder, resulting in constipation and urinary retention
secondary to affected innervation from the myelomeningocele. Vertebral abnormalities,
including scoliosis are common and are caused by muscle action imbalance on the joints in the
setting of diminished sensation and movement. A Comprehensive list of accompanying systemic
effects of myelomeningocele are found in Item C73D.
Item C73C: Functional Levels in Children Born With Myelomeningocele

Level Implications for mobility
High lumbar Can walk for short distances using long leg (high) braces. By early adolescence,
thoracic most use wheelchair for mobility.
Low lumbar Can walk with short leg braces and forearm crutches.
High sacral Can walk with a gluteal lurch using braces to stabilize the ankle and foot. Walking
ability usually is retained through adolescence.
Multidisciplinary care anchored by a primary care medical home is critical in the care of children
with myelomeningocele. After initial surgical closure, management requires multidisciplinary
care to address the multi-system effects of the myelomeningocele.
Prevention of myelomeningocele remains an important focus. Folic acid supplementation is

recommended for women of child-bearing age; evidence has demonstrated that this reduces the
risk of neural tube defects. Early prenatal diagnosis (before 25 weeks’ gestation) of
myelomeningocele provides the opportunity for fetal repair of the defect, if desired by the
family.

Item C73D systemic effects of myelomeningocele

PREP Pearls
• Myelomeningocele is a neural tube defect resulting in protrusion of the spinal cord and
nerves in an exposed meningeal sac. Impaired motor and sensory function is found below
the level of the defect.
• The majority of children with meningomyelocele have an associated Chiari II
malformation and accompanied hydrocephalus. Signs and symptoms of a Chiari II
malformation include swallowing difficulties, breath-holding spells, apnea, stridor,
hoarse voice, or choking.
• Management of the child with myelomeningocele requires a multidisciplinary team
anchored by the primary care medical home to provide comprehensive coordinated care
that addresses the condition's multisystem effects.

• Recognize the clinical manifestations of and complications associated with spinal
dysraphism, and manage appropriately
• Recognize other abnormalities commonly associated with myelomeningocele
Suggested Readings
• A randomized trial or prenatal versus postnatal repair of myelomeningocele. N Engl J
Med doi:10.1056/nejmoa1014379.
• Council on Children with Disabilities. Providing a primary care medical home for
children and youth with spina bifida. Pediatrics doi: 10.1542/peds.2011-2219.
• Spina bifida. American Academy of Pediatric Textbook of Pediatric Care. 2nd ed.
2017:2655-2633
• Myelomeningocele. Pediatr Rev. doi: 10.1542/pir.31-11-443

Question 74
An 8-month-old infant with chronic otitis media is taken to the operating room for
tympanostomy tube placement. His history is notable for hypotonia of unknown etiology and
neonatal seizures, for which he takes phenobarbital. He undergoes rapid-sequence intubation
with succinylcholine and etomidate, and his anesthesia is maintained with sevoflurane without
incident. In the post anesthesia care unit, immediately after the procedure, he becomes acutely
tachycardic and tachypneic and develops total body muscle rigidity. His vital signs include a
temperature of 38.5 °C, heart rate of 175 beats/min, respiratory rate of 55 breaths/min, and
pressure of 85/45 mm Hg. End-tidal nasal capnography reveals a PCO2 of 65 mmHg, which is
increasing in spite of increased respiratory rate and effort. He is arousable and irritable, and has a
tightly clenched mouth and rigid extremities. The remainder of his examination findings are
unremarkable.
Of the following, the MOST important medication to administer to this Infant is

A. calcium
B. dantrolene
C. phenobarbital
D. sodium bicarbonate

Correct Answer: B
The hyperthermia, tachycardia, tachypnea, increased carbon dioxide production, and muscle
rigidity demonstrated by the infant in the vignette are suggestive of malignant hyperthermia, and
dantrolene should be administered immediately. Malignant hyperthermia is a genetic disorder
affecting skeletal muscle and is associated with defects in ryanodine receptor. Nearly 100
individual mutations have been identified in the ryanodine receptor type 1 (RYR-1) gene. Of
these 25 to 35 are related to the development of malignant hyperthermia.
Genetically susceptible individuals who receive halogenated inhalational anesthesia such as

halothane, sevoflurane, or isoflurane or those who receive neuromuscular blockade with
succinylcholine are at increased risk of developing malignant hyperthermia. Those with certain
skeletal myopathies are also at a higher risk for the development or malignant hyperthermia.
Known associations include central core disease (a rare, autosomal dominant nonprogressive
myopathy), hypokalemic periodic paralysis, and King-Denborough syndrome.
Clinical symptoms and associated findings of malignant hyperthermia include hypercapnia,

masseter muscle and/or generalized muscle rigidity, metabolic and respiratory acidosis,
hyperkalemia, and hyperthermia. The hypermetabolic state reduced by excessive influx of
sarcoplasmic calcium results in sustained skeletal muscular contractions and depletion of
adenosine triphosphate. If untreated, the risk death is high.
Dantrolene is the treatment for malignant hyperthermia; the mechanism of action is through
direct binding to RYR-1, thus interfering with muscle contraction by inhibiting calcium ion
release from the sarcoplasmic reticulum. Calcium may stabilize the myocardium in patients with
acute hyperkalemia, but it will not address the underlying process. Sodium bicarbonate is used to
treat metabolic acidosis, hyperkalemia, and rhabdomyolysis; however, these conditions are all
secondary to the primary problem of malignant hyperthermia, and thus sodium bicarbonate is not
The best choice. Although malignant hyperthermia may result in tonic muscle contractions that
may resemble seizure, the constellation of signs, including hypercarbia and hyperpyrexia shortly
after anesthesia with sevoflurane, make malignant hyperthermia much more likely in the infant
in the vignette, and therefore phenobarbital would not be indicated.
PREP Pearls
• The hypermetabolic syndrome of malignant hyperthermia includes hypercarbia,
hyperthermia, tachycardia, mixed metabolic and respiratory acidosis, muscle rigidity,
rhabdomyolysis, and if left untreated, can lead to death.
• Dantrolene the treatment of choice for patients suspected of having malignant
hyperthermia.
• Patients who have certain skeletal myopathies are at an increased risk for malignant
hyperthermia.

• Identify the conditions associated with malignant hyperthermia

Suggested Readings
• Postoperative Care. American Academy of Pediatric Textbook of Pediatric Care. 2nd ed.
2017:521-534
• Core myopathies and malignant hyperthermia susceptibility: A review. Paediatr Ahaesth.
Doi:10.1111/pan.12175.
• Recognition of impending systemic failure, Pediatr Rev. doi: 10.1542/pir.2016-0102.
• Malignant hyperthermia in children: An analysis of the North America Malignant
hyperthermia in children. Anesth Analg. Doi:10.1213/ANE.0b013e3182a8fad0.

Question 75
A 12-year-old boy is seen as a new patient. He has a history of cystic fibrosis but has not taken
any medications for the past 3 months due to parental job loss, which led to insurance lapse and
homelessness. His parents did not bring records to the visit today; the boy’s concerns are fatigue,
chronic “oily” and foul-smelling stools, tingling extremities, and difficulty walking. He is a
febrile, his body mass index is at the 3rd percentile heart rate is 115 beets/min, respiratory rate is
25 breaths/min, and oxygen saturation is 94% on room air. He is small for his age and pale but
appears well. His conjunctivae are pale. He has scattered wheezes through all lung fields and
poor aeration. His liver edge is palpable 3 cm below the costal margin. Neurologic examination
reveals ataxia, hyporeflexia, and loss of proprioception.
Of the following, the MOST appropriate vitamin therapy for the boy is
A. Alpha tocopherol (vitamin E)
B. Ascorbic acid (Vitamin C)
C. Cyanocobalamin (vitamin B12)
D. Thiamine (Vitamin B1)

Correct Answer: A
The boy in the vignette has untreated cystic fibrosis complicated by pancreatic insufficiency.
Pancreatic insufficiency results in malabsorption, steatorrhea, and failure to thrive. Affected
children will have fat and fat-soluble vitamin malabsorption including vitamins A (retinol), D
(cholecalciferol), E (alpha tocopherol) and K (phytonadione). The boy in the vignette has
developed vitamin E deficiency, as evidenced by of neuropathy. His pallor may be due to
hemolytic anemia, another complication of vitamin E deficiency; laboratory analysis should be
performed to confirm this suspicion. In children with cystic fibrosis, deficiencies of water-
soluble vitamins (e.g., vitamin B12 [cyanocobalamin], vitamin C [ascorbic acid] and vitamin B1
[thiamine]) are uncommon and generally occur with severe malnutrition associated with reduced
dietary intake of these vitamins.
Children with cystic fibrosis should be carefully monitored to ensure sufficient energy intake,
optimize pancreatic enzyme dosing, and ensure adequate fat-soluble vitamins supplementation.
The energy requirement for individuals with cystic fibrosis can be up to 150% that of unaffected
children; this requirement is dependent on genotype, age, and state of health. Fat and fat-soluble
vitamin malabsorption is the most common nutritional problem seen in children with cystic
fibrosis; depending on individual losses, the recommended daily intake of these vitamins ranges
from 2 to 20 times that which is recommended for unaffected children. Given the poor clinical
outcomes associated with malnutrition in children with cystic fibrosis. Adherence to pancreatic
enzyme replacement and vitamin therapy are important objectives in overall care.
PREP Pearls
• Fat and fat-soluble vitamin malabsorption is the most common nutritional problem in
children with cystic fibrosis.
• In children with cystic fibrosis, pancreatic insufficiency results in fat and fat-soluble
vitamin malabsorption, including vitamins A (retinol), D (cholecalciferol), E (alpha
tocopherol) and K (phytonadione).
• Children with cystic fibrosis should be carefully monitored to ensure sufficient energy
intake, optimize pancreatic enzyme dosing, and ensure adequate fat-soluble vitamin
supplementation.

• Recognize the signs, symptoms, and causes of vitamin E deficiency and manage
appropriately
Suggested Readings
• Nutritional consideration in pediatric chronic disease. Pediatr Rev. doi:10-1542/pir.2016-
0030
• Vitamin excess and deficiency. Pediatr Rev. doi:10-1542/pir.2016-0068
• Cystic fibrosis. Pediatr Rev. doi:10-1542/pir.35-5-194

Question 76
A 2 years old boy is seen for a health supervision visit. His mother reports episodes of otitis
media over the past year, which required the placement of myringotomy tubes. He has had 10 to
12 episodes of upper respiratory tract infection over his lifetime. He has course fascial features,
macrocephaly, contractures of hands, a gibbus deformity, and corneal clouding. His liver is
palpable 3 cm below the right costal margin. His height is 2.5 standard deviations below the
mean.
Of the following, the diagnosis that is most consistent with the boy’s clinical features is
A. Fabry disease
B. Hunter syndrome
C. Hurler Syndrome
D. Pompe disease

Correct answer: C
The clinical disorder seen in the child described in the vignette is Hurler syndrome, also known
as mucopolysaccharidosis type I (MPS I), a lysosomal storage disorder. Affected children have
completely normal appearance at birth. There is gradual progression of the disease over time.
Deficiency of the enzyme α-L-iduronidase leads to storage of 2 glycosaminoglycans (GAGS),
dermatan and heparan sulfate, in tissues and organs. Mucopolysaccharidosis type I is inherited in
an autosomal recessive manner.
Clinical features lie on a spectrum, with considerable overlap between the 3 forms; the classical
form is known as Hurler syndrome, the intermediate form as Hurler-Scheie syndrome, and the
mildest form as Scheie syndrome. Affected patients are described as having either the severe or
the attenuated form of MPS I. Clinical features in the severe form may include umbilical or
inguinal hernia, coarse facial features (Item C76A), global developmental delay, progressive
intellectual disability, hearing loss, corneal clouding, and hepatosplenomegaly. Skeletal
manifestations include thoracolumbar kyphosis (gibbus deformity), progressive skeletal
dysplasia (dysostosis multiplex), progressive arthropathy leading to joint contractures (Item
C76B), and short stature due to poor linear growth. Cardiovascular manifestations include
progressive thickening of the valve leaflets, left ventricular hypertrophy, systemic or pulmonary
hypertension, aortic root dilatation, and coronary artery disease. Affected children experience
recurrent upper respiratory tract infections in first year of life. In the classic form, mortality is
typically caused by cardiorespiratory compromise in the first decade of life. In the attenuated
forms, mentation in school-age children can range from normal intellect to learning disabilities.
Their onset of clinical features is typically between 3 and 10 years of age.

Item C76A Age of onset of the main signs and symptoms in different mucopolysaccharidosis (MPS) types

Item C76B: dysostosis multiplex
Hematopoietic stem cell transplantation (HSCT) is the standard of care for severe MPS I; this
procedure is typically performed before 30 months of age. Hematopoietic stem cell
transplantation decreases facial coarseness, improves hearing, prevents progression of cardiac
complications, and improves hepatosplenomegaly. However, there is no improvement in cardiac
valvular or skeletal manifestations. In children who show no signs of developmental delay prior
to HSCT; the progression of cognitive decline is slowed. In children with significant cognitive
delay prior to HSCT, there is no improvement in development or cognition. Enzyme replacement
therapy (ERT) is recommended for children with attenuated forms of MPS I or those over 30
months of age with severe MPS I who were not treated with HSCT. Enzyme replacement therapy
does not cross the blood-brain barrier; it is in effective in patients experiencing the progressive
cognitive decline. Early diagnosis and initiation of treatment changes the long-term outcome of
the disease. Testing for MPS I has been added to the newborn screen panel in many states in the
United States.
Fabry disease is an X-linked lysosomal and storage disorder characterized by periodic pain
crises, angiokeratomas, corneal and lenticular opacities, stroke, left ventricular hypertrophy,
sweating abnormalities, and renal disorder progressing to end-stage renal disease. Enzyme
replacement therapy with recombinant or gene activated human α-galactosidase an enzyme is the
standard of treatment for Fabry disease.
Hunter syndrome (MPS II) is an X-linked recessive disorder. It causes accumulation of GAGs
dermatan and heparan sulfate due to a deficiency of the enzyme iduronate sulfatase. Hunter
syndrome has significant clinical overlap with Hurler syndrome. Differentiating features of
Hunter syndrome include better cognitive outcome and the absence of corneal clouding.
Treatment with ERT with idursulfase is the standard of care.
Pompe disease is a lysosomal storage disorder caused by a deficiency of the enzyme acid α-
glucosidase. It is inherited in an autosomal recessive manner. Pompe disease is classified as
infantile onset (IOPD) and late onset (LOPD). Clinical features of IOPD include marked
hypotonia, failure to thrive, and hypertrophic cardiomyopathy with the first year of life. Clinical
features of LOPD include proximal muscle weakness with respiratory insufficiency; there is no
cardiac involvement. Treatment with ERT with a glucosidase alfa is the standard of care.
Newborn screening for Pompe and Fabry disease has been implemented in a few US states.
PREP Pearls
• Mucopolysaccharidoses are lysosomal storage disorders characterized by accumulation of
glycosaminoglycans in various tissues and organs.
• Mucopolysaccharidosis type I (Hurter syndrome) is inherited in an autosomal recessive
manner; mucopolysaccharidoses type II (Hunter syndrome) is inherited in an X-Linked
recessive manner.
• Hurler and Hunter syndromes have similar clinical features. Children with Hunter
syndrome do not have corneal clouding and have a better cognitive outcome

• Recognize the laboratory features associated with mucopolysaccharidoses
• Recognize the signs and symptoms of the mucopolysaccharidoses including Hurler
syndrome.
Suggested Readings
• Mucopolysaccharidosis Type l. Gene Reviews Seattle, Of Washington, Seattle; 2016.
• Mucopolysaccharidoses: Early diagnostic signs in infants and children. Ital J of
Pediatrics, doi: 10.1186/s13052-018-0550-5
• Mucopolysaccharidosis Type l. Diagnostics. Doi:10.3390/diagnostics10030161
• Mucopolysaccharidosis Type l treatment guidelines. Pediatrics. Doi10.1542/peds.2008-
0416
• In born error of metabolism. Pediatr rev. doi:10.1542/pir.2014-0122

Question 77
A previously healthy, 22-month-old girl is brought to the emergency department in January with
a 3-day history of high fever, runny nose, cough, and decreased feeding. On physical
examination, she has a temperature of 39.1 °C, heart rate of 141 beats/min, respiratory rate of 48
breaths/min, blood pressure of 117/68 mmHg, and oxygen saturation respiratory of 90% in room
air. She is tachypneic with nasal flaring and mild subcostal retractions. On lung examination, she
has equal breath sounds both sides with bilateral scattered wheezes and no crackles. A rapid
antigen assay from a nasopharyngeal specimen is negative for respiratory syncytial virus and
influenza A. A nasopharyngeal specimen has been submitted to test for community respiratory
viruses using a molecular assay. Chest radiography shows increased perihilar cuffing with no
focal infiltrate. Following oxygen supplementation, oxygen saturation increases to 92% and
improvement in respiratory distress noted. She is started on intravenous fluids and admitted to
the hospital for observation.
Of the following, in addition to supportive care, the BEST next management step for this girl is
to give
A. Azithromycin
B. Ceftriaxone
C. Nebulized albuterol
D. Oseltamivir

Correct Answer: D
The clinical presentation of the child described in the vignette is consistent with a diagnosis of
bronchiolitis caused by influenza. Given her age (< 2 years), she may develop influenza-related
complicated disease, therefore, starling empiric oral oseltamivir therapy is the best next
management step. Higher rates of hospital admission and complicated clinical course because of
influenza infection are observed among young children or those with preexisting chronic disease
(e.g., congenital heart disease, diabetes, chronic neurologic or neurodevelopmental disorders,
underlying immunodeficiency disorder, or receipt of immunosuppressive therapy). Antecedent
infection with influenza can result in secondary bacterial pneumonia due to Staphylococcus
aurous or Streptococus However, initiation of antibiotics (ceftriaxone or azithromycin) would
not be appropriate in the child described in the vignette, given the low likelihood of secondary
bacterial pneumonia based on clinical presentation (mild respiratory distress, no crackles, and
chest radiograph showing only increased perihilar cuffing with no focal infiltrate). Nebulized
albuterol would not be indicated in viral bronchiolitis because adverse effects of β-agonist
bronchodilators such as albuterol outweigh possible benefits.
Influenza is a common seasonal respiratory disease with the highest incidence seen among
school-aged children. Influenza activity peaks during the winter months (January to March) in
temperate climates, but cases may occur at any time from November through May.
Hospitalization rates because of influenza are substantially higher in younger children (aged <2
years) compared with older children. Deaths related to influenza have been reported among
previously healthy children and those with chronic underling illness. During the 2019 to 2020
influenza season, over 182 influenza-associated pediatric deaths were reported in the United
States.
Influenza viruses are orthomyxoviruses. Influenza virus types A and B cause epidemic disease in
humans whereas type C disease causes mild illness. Influenza A virus can also cause sporadic
pandemic disease in humans. Influenza A virus subtypes (i.e., H1N1 and H3N2) are determined
by the antigenic characteristics of 2 surface glycoproteins known as hemagglutinin (H) and
neuraminidase (N). Seasonal epidemics of influenza occur due to antigenic variation conferred
by the continuous point mutations in the H and N genes of both influenza A and B viruses (e.g.,
antigenic drift), Key determinants of immunity include antibodies to hemagglutinin and other
influenza antigens.
Human to human transmission of influenza viruses occurs via large particle droplets by coughing
or sneezing or by direct contact with respiratory secretions or contaminated surfaces. The
incubation period ranges from 1 to 4 days. Virus shedding in children may occur 24 hours before
developing symptoms resulting in transmission to others. Virus shedding typically ceases in
nasal secretions by 7 days in infected patients but may be longer in young children and immune
suppressed hosts.
Clinical manifestations of influenza may vary with age. Uncomplicated influenza disease in
children is characterized by an abrupt onset of high fever associated with myalgia followed by
nonproductive cough, pharyngitis, and rhinitis; other associated symptoms may include vomiting
and diarrhea. In some children, influenza may present with fever with minimal other symptoms.
Influenza can cause croup or bronchiolitis (as illustrated in the vignette) in young children.
Infants may exhibit sepsis-like symptoms. Symptoms of uncomplicated influenza disease
commonly resolve in 3 to 7 days. In addition to viral pneumonia, influenza illness may be
complicated by secondary bacterial pneumonia, otitis media, and sinusitis.
Influenza virus can be detected with rapid antigen detection testing of nasopharyngeal
specimens, but fate-negative results may occur because of the relatively low sensitivity of these
tests. Highly sensitive and specific reverse transcriptase polymerase chain reaction is
increasingly being used to detect the virus at most medical center.
Antiviral therapy for influenza must be given early for all hospitalized children with presumed
influenza or those with complicated disease, regardless of influenza immunization status or
duration of onset of illness (as demonstrated by the girl in the vignette). Although antiviral
therapy is most beneficial when administered within 48 hours of illness onset, treatment must be
considered for hospitalized patients with complicated progressive and severe disease. Antiviral
treatment must also be considered for children with presumed influenza illness who reside in
households with high-risk contacts (underlying chronic medical illness, infants aged <6 months).
Antiviral therapy should not be withheld in high-risk patients (pending laboratory confirmation)
presenting with a clinical illness consistent with influenza.
The antiviral agents approved for the treatment and prophylaxis for influenza virus A and B are
neuraminidase inhibitors (oseltamivir, zanamivir, peramivir) (Item C77). The antiviral agent of
choice is oral oseltamivir. Oseltamivir is approved by the US Food and Drug Administration in
the pediatric age group (2 weeks of age and older) Oseltamivir also has been used to treat term
and preterm infants with influenza because of the potential benefits of the drug outweigh the risk
of toxicity. Common adverse effects with oseltamivir are nausea and vomiting.
Item C77: Antiviral Drugs for Influenza

Immunization on an annual basis is the most effective strategy for influenza control and is
indicated in all individuals aged 6 months and older.
PREP Pearls
• Children younger than 2 years or who have underlying chronic medical disease are at
high risk for hospitalization and complications from influenza infection.
• Antiviral therapy for influenza must be given early for all hospitalized children with
presumed influenza or those with complicated disease, regardless of immunization status
or duration of illness onset.
• Although antiviral therapy is most beneficial when administered within 48 hours of
illness onset, treatment must be considered for hospitalized patients with complicated
progressive and severe disease.
• Annual vaccination is the primary prevention strategy for influenza and it is indicated in
all individuals aged 6 months and older.
ABP content specifications

• Plan the appropriate diagnostic evaluation of influenza virus infection
• Understand the changing antigen composition of the influenza vaccine (and the
importance of its annual administration to children with chronic vaccine diseases)
• Know the indications, contraindications, and schedule for the inactivated and live
attenuated influenza vaccines
• Understand the epidemiology of the influenza virus
• Recognize the clinical features associated with influenza virus infection
Suggested Readings
• Academy of Pediatrics. Committee on Infectious Diseases. Recommendations for
Prevention and Control of Influenza in Children, 2020-2021. Pediatrics. 2020; doi:
10.1542/peds.2020-024588
• Influenza. Redbook: 2021-2024 Report of the Committee on Infectious Diseases. 32nd
ed; 2021:447-456
• Centers for Disease Control and Prevention. Influenza antiviral medications.
https://www.cdc.gov/flu/professionals/antivirals/index.htm
• Prevention and control of seasonal Influenza with vaccines: recommendations of the
Advisory Committee on Infectious Diseases (ACIP) 2017-2018

Question 78
During a health supervision visit for their child, the parents share that they in the process of
adopting a 15-month-old boy. They are excited to add to their family and would like guidance in
preparing for this child’s arrival. The parents ask when they should tell their child that he is
adopted.
Of the following, the MOST appropriate time is when the child

A. asks about his birth
B. can understand what adoption means
C. is at least 5 years old
D. Joins the family

Correct Answer: D
Experts in adoption generally agree that children should be informed of their status earl, such as
upon joining their adoptive family. It can be problematic to wait until the child is older, asks
about his birth, or can understand what adoption means. Early disclosure can help adoptive
parents become comfortable talking about adoption in their family, prevent this information from
being revealed by people other than the adoptive parents and result in less distress to the child.
Although a young child, such as the one in this vignette, may not understand what adoption
means, informing the child early in a developmentally appropriate manner ensures that there is
no time when the child did not know that he or she was adopted.
About 2% to 2.5% of children in the United States are adopted. Primary care providers have an
important role in supporting families and children before and after adoption. Before adoption, the
pediatrician can guide families in obtaining pertinent information about the birth parents, family
history, and the child's history pf placement(s), education, health, and adverse childhood
experiences. This information is important for identifying and addressing the child’s physical,
developmental and mental health needs at the time of adoption, as the child grows, and develops.
Soon after adoption, over 1 or multiple visits, the pediatrician should conduct a thorough
evaluation of the child's health. This evaluation includes review of records and known history,
assessment of health risks, a complete physical examination, age-appropriate screenings, and
clinically indicated diagnostic testing. Diagnoses should be ascertained and confirmed,
developmental status should be determined and referrals to relevant specialists and connections
to needed services should be made. Screening for infections (e.g., tuberculosis, intestinal
parasites), vision/hearing problems, developmental delays, and general health status is
particularly important for children adopted from other countries with consideration of risks
relevant to the specific country.
With close followup after adoption, the pediatrician can also support the family as the parents
and their child adjust to each other. Children may have problems with sleep, feeding, or
elimination. They may cry, throw tantrums, withdraw, or act out. The pediatrician can help the
family understand what to expect and can provide guidance on positive ways to interact with
their child. Pediatricians can also help guide the family as the children move through different
ages and developmental ages. They can support parents in addressing questions their child might
have about their birth parents. When a child is of different racial or ethnic identity from the
adoptive family, the pediatrician can encourage parents to educate themselves and their child
about their child's heritage and to integrate and celebrate this heritage. Positive role models of
the adopted child’s racial or ethnic background can be helpful in the child's identity
development.

PREP Pearls
• Children should be informed of their adoptive status early, such as upon joining their
adoptive family.
• Screening for infections (e.g., tuberculosis, intestinal parasites), vision/hearing problems,
developmental delays, and general health status is particularly important for children
adopted from other countries with consideration of risks relevant to the specific country.
• When a child is of a different racial or ethnic identity from the adoptive family, the
pediatrician can encourage parents to educate themselves and their child about their
child's heritage and to integrate and celebrate this heritage.

• Understand the pediatrician's role in the adoption process
• Understand the psychosocial issues surrounding adoption
Suggested Readings
• Council on Foster Care, Adoption, and Kinship Care. Pediatric guidance in supporting
families of children who an adopted, kinship care. Pediatrics. doi:10.1542/peds.2020-
034629
• Council on Foster Care, Adoption, and Kinship Comprehensive health evaluation of the
newly adopted child. Pediatrics. doi: 10.1542/peds.2019-0657
• Zuckerman Parker Handbook of Developmental and Behavioral Pediatrics for Primary
care 4th ed. 113-117.
• Adoption. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed. 589-601.

Question 79
An infant is brought to the office for her 2-month health supervision visit. The mother is
concerned because the infant stools every 4 days. The stool is soft, without any blood or mucus.
There is no significant straining with passage of stool. The infant breastfeeds for 10 minutes
every 2 to 3 hours. Her weight has been tracking at the 40th percentile for her age since birth.
Of the following, the BEST next step in the management of this child is
A. milk elimination diet
B. mineral oil daily, orally
C. polyethylene glycol daily, orally
D. reassurance

Correct Answer: D
Exclusively breastfed infants often stool only once every 3 to 4 days. The infant in the vignette
stools appears normal, she is feeding well, and her weight gain is good, therefore, reassurance is
appropriate in this situation. The infant has no blood or mucus in her stools, which would be
suggestive of colitis, therefore milk elimination would not be beneficial. Mineral oil or
polyethylene glycol given orally can be used to treat constipation; however, this infant does not
have hard or difficult to-pass stools. Mineral Oil is also associated with lipid pneumonitis and
should be avoided in infants under 1 year of age.
Exclusive breastfeeding has many benefits for both mother and infant. There are a number of
barriers to successful breastfeeding that can be addressed during routine care visits; consultation
with a certified lactation specialist may also be helpful. Early on, breastfeeding can lead to
painful nipples, which typically improve within 7 to 10 days. Factors that can worsen nipple pain
include prolonged feeding (>30 minutes per feed), poor latch, or an anterior tongue-tie.
Encouraging the baby to feed vigorously by keeping the infant awake during the feeding will
help the infant adequately feed within 30 minutes. When the infant latches, the infant's mouth
should be around the entire nipple and at least a portion of the areola, with the nose and chin to
the breast and the lips flared out in a "fish lips" position. If the infant has an anterior tongue-tie
that limits lateral and anterior movement of the tongue, treatment with a frenulectomy should be
considered.
As the infant grows and develops, new breastfeeding concerns may arise. Once teeth erupt,
biting can become problematic. Typically, infants will bite once they are full or if offered the
breast to nurse when not hungry. Ending the breastfeeding session immediately usually helps the
infant learn not to bite. As their visual acuity improves and infants become more engaged with
and curious about their environment (around 4 to 5 months of age), they may not complete
feeding sessions, leading to increased frequency of feedings and waking at night. Feeding in a
quiet place can decrease distractions. Infants become more efficient feeders over time; the
transfer of milk may only require 5 to 10 minutes. Reassuring parents that the infant is extracting
sufficient nutrition in this time is important, as long as weight gain remains adequate. If milk
supply does become problematic, increasing the frequency of feeding sessions and/or pumping is
helpful along with ensuring adequate maternal caloric and fluid intake. Oral contraceptive pills,
previous breast surgery and scar tissue, as well as postpartum complications such as blood loss or
hypertension, can negatively impact the milk supply.
Several prescription medications, as well as drugs and alcohol, can be transferred to the baby
through breast milk. Chemotherapy drugs, oral retinoids, iodine, and lithium are contraindicated
during breastfeeding. There are several online and print resources available to help mothers
understand which medications are safe to take while breastfeeding. Alcohol is expressed in
breast milk at about the same concentration as the mother’s blood alcohol level; it is
recommended to avoid alcohol while nursing. If the mother chooses to consume alcohol while
breastfeeding, it is recommended to limit to 1 serving of an alcohol and wait for at least 2 hours
to pump or nurse. Repeated exposure to alcohol should be avoided. Nicotine may cause an infant
to be jittery and may interfere with milk supply and letdown. Secondhand smoke increases the
risk of sudden infant death syndrome. Research regarding marijuana is lacking; current
recommendations are that it should be avoided.
Breastfed infants typically drink no more than 3 to 4 oz per feed. They typically feed more
frequently than their formula-fed peers and will regulate the volume they take in Encouraging
feeding every 2 hours initially will help establish breastfeeding. Breastfed infants typically stool
with every feeding at first, then less frequently; about one-third will decrease to 1 stool every 3
to 4 days or as infrequently as 12 to 14 days.
Colitis in breastfed infants most often results from a reaction to cow milk protein in the mother’s
milk from dairy-products she has consumed; this can lead to bloody stools. Elimination of dairy
from the mother's diet will typically resolve the colitis; if symptoms persist, progressive
elimination of foods from the mother's diet may be recommended; first soy, then other common
allergens such as egg, nut, wheat, and corn can be eliminated one at a time.
PREP Pearls
• Breastfed infants initially stool with every feeding, then stooling frequency decreases.
• Colitis in breastfed infants is most commonly due to maternally ingested cow milk
protein.
• Anticipatory guidance regarding common barriers to breastfeeding will improve the
chance of breastfeeding
ABP contents specifications

• Understand factors that could interfere with breast-feeding
• Recognize the effects of maternal ingestion of drugs on breast-fed infants
• Understand the significance or colitis in a breast-red infant
• Know the normal pattern of feeding and stool frequency in formula-fed infants
• Know the normal pattern of feeding and stool frequency in breast fed infants
Suggested Readings
• Alcohol and breast milk. Healthy Children.org
https://www.healthychildren.org/English/ages-stages/baby/breastfeeding/pages/Alcohol-
breast-milk.aspx
• The pediatrician’s role in encouraging exclusive breastfeeding. Pediatr Rev.
doi:10.1542/pir.2016-0109
• Breastfeeding the essential principles. Pediatr Rev. doi:10.1542/pir.27-11-409
• Breast feeding the newborn. American Academy of Pediatrics Textbook of Pediatric Care
2nd ed. 749-795

Question 80
A 2-year-old boy is evaluated for a rash, which his mother noted last night while she was giving
him a bath. He is otherwise well. His mother reports that he had an episode of epistaxis 2 days
ago, that lasted 7 minutes. The boy occasionally has episodes of epistaxis that typically last 3 to
5 minutes so she was not concerned enough to bring him in at that time. She also reports that he
has bruise on his forehead due to hitting his head on the dining table last night. The boy is “picky
eater” but has not had any recent change in his appetite. He consumes 30 oz of cow milk per day.
Review of his chart reveals that his mother called 2 weeks ago for advice because the boy had
fever and upper respiratory symptoms.
The boy’s vital signs are normal for age. His physical examination reveals a pleasant, interactive
boy who is well nourished, well developed, and in no acute distress. His lungs are clear to
auscultation, his heart rhythm is normal, and there is no palpable hepatosplenomegaly or
lymphadenopathy. Examination of his skin reveals a generalized, erythematous, pinpoint, non-
blanching rash; bruises on his buttocks and bilateral shins; and 2 x 2 cm bruise on his forehead.
There are no focal neurological findings.

White blood cell count 7,800/µL (7.8x 109/L)
Neutrophils 40%
Lymphocytes 55%
Monocytes 5%
Hemoglobin 9.7 g/dL (97g/L)
Platelet count 3x103/µL (3x 109/L)
Mean platelet count 12 fL
Prothrombin time 12s
Activated partial thromboplastin time 36s
Of the following, the BEST next step in this boy's management is to

A. anti-D immunoglobulin
B. intravenous immunoglobulin
C. no treatment; observation
D. platelet transfusion

Correct Answer: B
Based on the history, physical examination findings, and laboratory results, the child in the
vignette most likely has a diagnosis of acute idiopathic thrombocytopenic purpura (ITP). This
diagnosis is supported by the low platelet count with large (increased mean platelet volume,
mean corpuscular volume) platelets, normal white blood cell count and differential, and the fact
that the child is otherwise well with no lymphadenopathy or hepatosplenomegaly. This diagnosis
is one of exclusion.
Idiopathic thrombocytopenic purpura is an immune phenomenon. In most cases, there is a

history of recent viral illness. Autoantibodies created toward a glycoprotein on platelets lead to
their destruction and clearance, mainly by the spleen and also the liver. Though platelet antibody
testing is available, a negative antibody does not exclude the diagnosis.
In the United States, treatment is usually offered when the platelet count is less than 10 x 103/µL
(10 x 109/1), especially in young individuals or those with higher risk of bleeding. This is due to
the risk of spontaneous, unprovoked bleeding at such low platelet counts, for this child, who is at
risk for age-appropriate frequent falls and hit his head and whose history includes recurrent
epistaxis, treatment is warranted. For an older child with significant history of bleeding,
observation can be an option.
There are a few first-line treatment options for acute ITP, including intravenous
immunoglobulins (IVIG), anti-D immunoglobulin, and oral or intravenous steroids. Mode of
response to each treatment method varies with IVIG and anti-D immunoglobulins having the
earliest response, then steroids. For an infant who developmentally has a significant risk of head
injury, it would be advantageous to administer a therapy that would raise the platelet count
quickly and thereby decrease the bleeding risk. Intravenous immunoglobulin and anti-D
immunoglobulin are the best choices to achieve a rapid response. For this child, IVIG is the
preferred treatment choice. It may increase the platelet count rapidly, and its response can remain
for several weeks. Due to his mild microcytic anemia (low mean corpuscular volume) which is
most likely due to iron deficiency anemia as a result of his poor diet and occasional epistaxis,
anti-D Immunoglobulin would not be an appropriate option for the child in the vignette, as the
treatment mechanism of action can lead to worsening anemia.
This child with both anemia and thrombocytopenia, steroids should be avoided as first-line due
to the remote possibility of leukemia presenting with a normal white blood count. Additionally,
steroids have a longer response time. If the child in the vignette were older and more able to
avoid any significant injury such as head injury and showed only an isolated abnormality in the
platelet count (totally normal white blood cell count and hemoglobin level), steroids would be an
appropriate treatment option and may be administered as an outpatient.
Because ITP is an immune phenomenon, the autoantibodies could destroy any platelet
administered; therefore, platelet transfusions are reserved for life-threatening bleeding episodes.

PREP Pearls
• Acute idiopathic thrombocytopenic purpura is typically a diagnosis of exclusion.
• First-line treatment options for acute idiopathic thrombocytopenic purpura include
intravenous immunoglobulin, anti-D immunoglobulin, and oral or intravenous steroids.
• Age, developmental stage, and bleeding signs and symptoms should be considered when
choosing a treatment choice for acute idiopathic thrombocytopenic purpura.

• Recognize the clinical and laboratory findings associated with immune thrombocytopenia
purpura and manage appropriately
• Recognize complications associated with immune thrombocytopenia purpura
Suggested Readings
• Thrombocytopenia during childhood: What the pediatrician needs to know. Pediatr Rev.
doi:10.1542/pir.26-11-401
• Idiopathic Thrombocytopenic. Pediatr Rev. doi: 10.1542/pir.21-3-95
• Platelet disorders. Pediatr Rev. doi: 10.1542/pir.2018-0359.

Question 81
A previously healthy, 1-year-old girl is screened for lead exposure with a venous sample. She
lives in a community with a high density of homes built before 1950. She has had normal
growth and development. The laboratory result is 6 µg/dL (0.29 µmol/L). Nutritional assessment
and counseling about potential environmental exposures are provided.
Of the following, the MOS appropriate next step is

A. abdominal radiography and iron studies
B. reassurance; this is a normal result
C. repeat testing in 1 to 3 months
D. repeat testing in year

Correct Answer: C
Although a lead level of 6 µg/dL (0.29 µmol/L) was previously considered to be normal,
evidence confirms detrimental developmental and health outcomes for children with even low
levels of lead exposure. Beginning in 2012, the centers for Disease Control and Prevention
(CDC) have used 5 µg/dL (0.24 µmol/L) as the reference value to identify children with elevated
lead levels. This value is determined based on the 97.5th percentile of the National health and
Nutritional Examination Survey (NHANES) blood lead levels for children 1 to 5 years of age in
the 2007-2008 and 2009-2010 data sets and will be reassessed by the CDC regularly as new
NHANES data are available.
Children are at particularly high risk for lead poisoning for several reasons: an increased
permeability of the blood-brain barrier, a greater prevalence of iron deficiency (which promotes
absorption of lead in the gastrointestinal tract), and a higher exposure to lead-containing dust
because of crawling and hand-to-mouth behaviors. Although the incidence of lead poisoning in
the United States has fallen over the past few decades, disparities exist with refugee children,
children in urban environments, and children in foster care having higher blood lead levels.
Exposures occur through either ingestion or inhalation, with common sources including chips of
paint or dust, containers used to store or serve food or beverages, lead-soldered plumbing,
automobile emissions and byproducts from industrial production.
It is clear that no level of lead exposure should be considered “safe”. There is incidence that
measurable blood lead levels less than 5 µg/dL (<0.24 µmol/L) are with associated with lower
academic achievement and lower IQ scores, increased incidence of attention deficit hyperactivity
disorder, and potentially also delayed puberty and impaired renal function. Additionally, levels
less than 10 µg/dL (<0.48 µmol/L) are associated with delayed puberty, reduced growth, and
hearing impairment.
The CDC has clear recommendations for evaluation and treatment of children with elevated
blood lead levels (Item C81).
https://www.cdc.gov/nceh/lead/acclpp/final_document_030712.pdf
The child in the vignette, with a blood lead level of 6 µg/dL (<0.29 µmol/L), should have a
nutritional assessment, be counseled about potential environmental exposures, and have her lead
level retested in 1 to 3 months. Her level is not normal and waiting for 1 year to retest is
inappropriate. Unless otherwise indicated, radiography and iron studies are unnecessary.
PREP Pearls
• Although no measurable blood lead level is safe in children, levels greater than 5 µg/dL
(<0.24 µmol/L) are considered elevated.
• Elevated blood lead levels are associated with impaired neurocognitive development,
pubertal delay, reduced growth, and decreased renal function
• Children are particularly high-risk lead poisoning for several reasons: an increased
permeability of the blood-brain barrier, a greater prevalence of iron deficiency (which
promotes absorption of lead in the gastrointestinal tract), and higher exposure to lead-
containing dust because of crawling and hand-to-mouth behaviors.


• Understand the outcomes associated with lead poisoning
• Recognize the multiple sources of exposure to lead
• Plan appropriate management of an increased blood lead concentration
Suggested Readings
• Centers for Disease control Prevention, Low Level Lead Exposure Harms children
https://www.cdc.gov/nceh/lead/acclpp/final_document_030712.pdf
• Lead poisoning: Basics and new developments. Pediatr Rev. 2010;31(10):399-406.
• Council on Environmental Health. Prevention of childhood lead toxicity. Pediatrics.
doi:10.1542/peds.2016-1493
• Lead poisoning. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.
2242-2245.

Question 82
A 17-year-old adolescent patient is evaluated in the office for recurrent episodes of nonbilious,
nonbloody emesis accompanied by nausea and abdominal pain. The patient is otherwise healthy.
The patient reports 4 episodes since the symptoms began 8 months ago: the episodes last
between 24 to 48 hours. There are no reported triggers, and frequent hot showers relief some of
the symptoms. There has been an emergency department evaluation for the most recent episode.
Between episodes, the patient is well. The patient reports increased stress at school while trying
to prepare for college as well as a 2-year history of frequent marijuana use. There is a strong
family history of migraine headaches. Physical examination findings, including growth
parameters, are normal.
Laboratory data from the emergency department visit are shown:

Glucose 90 mg/dL (5 mmol/L)
Lipase 60 U/L
Alanine aminotransferase 32 U/L
Aspartate aminotransferase 38 U/L
Abdominal radiography and ultrasonography findings from the emergency department visit are
normal.
Of the following, the BEST therapeutic option for this adolescent is
A. acupuncture
B. cessation of marijuana use
C. cyproheptadine
D. propanolol

Correct Answer: B
The adolescent in the vignette has a history of cyclical vomiting and regular cannabis use, and
his symptoms improve with prolonged hot bathing. It is most likely that this adolescent is
experiencing cannabinoid hyperemesis syndrome (CHS), and cessation of cannabis use is the
treatment of choice.
Causes of vomiting are reviewed in Item C82. The initial evaluation for cyclical vomiting
should be tailored based on the child’s history and physical examination findings. Causes of
cyclical vomiting in adolescents include abdominal migraine, CHS, cyclic vomiting syndrome
(CVS), eating disorder, superior mesenteric syndrome, and ureteropelvic junction obstruction.
Thus, laboratory and imaging evaluation could include upper gastrointestinal series and
abdominal ultrasonography. For an infant with cyclical vomiting, because of the increased
likelihood of inborn errors of metabolism and adrenal insufficiency, laboratory and imaging
evaluation should be performed when symptomatic (and before intravenous fluids) and should
include: electrolytes, glucose, urine ketones, lactate, ammonia, serum amino acids, urine organic
acids, upper gastrointestinal series, and abdominal ultrasonograph.
Cyclic vomiting a migraine variant, is a functional gastrointestinal disorder defined 2 or more

episodes of intense nausea and vomiting that lasts hours to days over a 6-month period, separated
by a return to baseline health between episodes. The presentation of each episode is similar for
the individual patient and cannot be attributed to another medical condition. Treatment for CVS
includes management of the acute episode (abortive strategies) and prophylactic therapy.
Abortive strategies include supportive care (e.g., quiet, dark environment maintaining hydration),
and, if necessary, antiemetic therapies or triptans. Prophylactic therapies include stress
management, avoidance of fasting, and elimination of trigger foods (if relevant). Medical
management is indicated if episodes result in multiple hospitalizations or school absences, or are
occurring more than every 1 to 2 months. First-tine therapy for children older than 5 years of age
is amitriptyline; cyproheptadine is recommended for children 5 years of age and younger.
Prophylaxis with propranolol is recommended as second-line therapy for all ages.
Complementary therapies, including acupuncture or cognitive behavioral therapy, may be
considered.
The presentation of CHS is similar CVS with the exception of the history of prolonged cannabis
usage. The use of excessive, prolonged hot baths or showers for symptom relief is more
commonly associated with CHS but can be seen people with CVS who do not report cannabis
use. Diagnosis of CHS is confirmed when abstinence from cannabis use results in resolution of
symptoms (which may take months, or up to the time in which 3 episodes would typically
occur). Abortive and prophylactic CVS therapies may be used while working with the patient to
abstain from cannabis use.
Acupuncture could be an effective therapy for some patients with CVS. However, it would not
be as effective of a treatment for this adolescent as cessation of cannabis use. Neither
cyproheptadine nor propranolol would be appropriate first-line treatment strategies for cyclical
vomiting in this adolescent.


PREP Pearls
• Cannabinoid hyperemesis syndrome should be considered in individuals with a history of
prolonged cannabis use and cyclical vomiting.
• The most effective treatment for cannabinoid hyperemesis syndrome is cessation of
cannabis use.
MOCA-Peds Objective
• Recognize and manage migraine variants in children

• Plan the evaluation of recurrent cyclic vomiting
Suggested Readings
• North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition
consensus statement on the diagnosis and management of cyclic vomiting syndrome. J
Pediatr Gastroentero/ Nutr. doi: 10 IQ97/MPG Ob013e338173ed39.
• Gastroesophageal reflux disease. Cyclic vomiting syndrome. American Academy of
Pediatrics Textbook of Pediatric Care 2nd ed. 2069.
• Childhood functional gastrointestinal disorders. Gastroenterology.
Doi:10.1053/j.gastro.2005.08.063
• Vomiting in children. Pediatr Rev. doi:10.1542/pir.2017-0053
• Role of chronic cannabis use on cyclic vomiting syndromes cannabinoid hyperemesis
syndrome. Neurogastroenterol Motil. Doi:10.1111/nmo.13606

Question 83
A 1-week-old female neonate is being seen after discharge from the neonatal intensive care unit.
She was born at 41 weeks' gestation and had meconium aspiration syndrome. She was supported
with continuous positive airway pressure for 3 days and received 48 hours of ampicillin and
gentamicin. Her blood culture results were negative. Since discharge 2 days ago, she has been
breast and bottle feeding. Her weight is 2% below birth weight. Her vital signs are a respiratory
rate of 65 breaths/min, heart rate of 130 beats/min, temperature of 37.3 C, and blood pressure of
65/42 mm Hg. She is interactive and has clear breath sounds with comfortable tachypnea. The
remainder of her physical examination findings are unremarkable.
Of the following, the BEST next step in management is

A. antibiotic therapy
B. β-agonist therapy
C. observation
D. oral diuretic therapy

Correct Answer C
Meconium-stained amniotic fluid occurs in 10% to 15% of births and is associated with post-
term gestation. Only 5% of neonates born with meconium-stained amniotic fluid will develop
meconium aspiration syndrome (MAS). A fetus in distress may pass meconium before birth into
the amniotic fluid. Presence of meconium in the lung can alter pulmonary vasculature and result
in persistent pulmonary hypertension the newborns. Meconium also acts via a ball-valve
mechanism obstructing small airways with distal alveolar collapse; chest radiography shows
patchy areas of atelectasis and hyperinflation. Most neonates with MAS who require supportive
care recover without long-term complications. However, neonates with MAS may remain
tachypneic for days to weeks. Management of tachypnea should be supportive.
Over the past 20 years, the incidence of MAS has decreased, largely because of changes in
obstetric practice. Pregnant women undergo labor induction and before post-term gestation, thus
decreasing the risk of MAS. Concurrently, the management approach for neonates with
meconium-stained amniotic fluid in the delivery room has evolved. Neonates born with
meconium-stained amniotic fluid now treated the same way as neonates born with clear amniotic
fluid.
For an otherwise well neonate with tachypnea caused by MAS, there is no role for antibiotic
therapy. Although a few neonates with MAS will develop reactive airway disease (RAD), there
is no evidence to suggest that this neonate has RAD, and so β-agonist therapy is not indicated.
Oral diuretic therapy would not be helpful for this neonate, because her tachypnea is not related
to fluid overload.
PREP Pearls
• Meconium aspiration syndrome is seen most often in post-term gestation neonates.
• Meconium aspiration syndrome may be associated with persistent hypertension.
• Neonates with meconium aspiration syndrome may remain tachypneic days to weeks.

• Recognize the clinical features associated with a neonate who has persisted pulmonary
hypertension following meconium aspiration
• Distinguish between persistent pulmonary hypertension with meconium aspiration and
cyanotic congenital heart disease in a neonate
Suggested Readings
• Respiratory tract disorders. Nelson Textbook of Pediatrics. 21st ed. 929-949.
• Meconium aspiration. Pediatr Rev. doi:10.1542/pir.29-6-212.
• Respiratory distress and breathing disorders in the newborn. Meconium aspiration
syndrome. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed. 867-887.

Question 84
A 9-year-old boy is brought to the clinic for evaluation of cola-colored urine for 2 days. He
reports no dysuria, flank pain, fever, diarrhea, vomiting, sore pain, or joint pain. He was
diagnosed with impetigo 4 weeks ago, and the lesions healed a treatment with antibiotic ointment
The boy has a heart rate of 88 beats/min respiratory rate of 14 breaths/min, and blood pressure of
122/78 mm Hg. His chest is clear to auscultation. He has mild facial puffiness and pitting edema
of his lower extremities. The remainder of his physical examination findings are unremarkable.
Laboratory data are shown

Blood
Ant streptolysin O <200 IU
Urine
Nitrite Negative
Blood 3+
Protein 2+
Red blood cells >100/HPF
Red blood cell casts 1-2/low power field
Of the following, the MOST likely additional laboratory finding for this boy is a
A. high antinuclear antibody titer

B. high serum immunoglobulin A level
C. low complement C3 level
D. low complement C4 level

Correct Answer: C
The boy in the vignette most likely has poststreptococcal glomerulonephritis (PSGN). His prior
history of a skin infection, gross hematuria, hypertension, azotemia, edema, and proteinuria favor
this diagnosis. The laboratory finding of a low complement C3 and a normal C4 further supports
the diagnosis of PSGN.
Poststreptococcal glomerulonephritis is the most common childhood glomerulonephritis,

typically presenting between 4 and 12 years of age. The symptoms and signs of acute
glomerulonephritis (hematuria, hypertension, azotemia, and edema) usually present 1 to 2 weeks
after a streptococcal sore throat or 2 to 6 weeks after a streptococcal skin infection. Children with
PSGN may have minimal microscopic hematuria or a rapidly progressive glomerulonephritis.
Severe acute-onset hypertension leading to headache, visual disturbances, and encephalopathy
can sometimes be seen. Salt and water retention in PSGN leads to generalized edema, and in
severe cases, can cause pulmonary edema and congestive heart failure.
An elevated ant streptolysin O (ASO) titer in PSGN supports a preceding Streptococcal

pharyngitis. The ASO titer may not be elevated in streptococcal skin infection because
streptolysin may be bound to the lipids in the skin. In such cases, antideoxyribonuclease B levels
may be checked to confirm prior streptococcal infection. The serum complement 3 (C3) is the
most important test to differentiate PSGN from other forms of acute glomerulonephritis (Item
C84). The C3 level is usually low and C4 level is normal in children with PSGN.

Immunoglobulin A (IgA) nephritis is a less likely diagnosis for the boy in the vignette because
he has a prior history of skin infection. Furthermore, measuring serum IgA has limited diagnostic
value in IgA nephritis because the levels may be normal. Antinuclear antibody is a nonspecific
inflammatory marker commonly in systemic lupus erythematosus, which is an unlikely diagnosis
for the child vignette.
The management of PSGN includes restriction of fluids and dietary sodium intake to prevent
complications of hypertension and fluid overload. Furosemide may be considered for initial
management of hypertension and edema in children with PSGN. Calcium channel blockers are
the preferred agent to treat persistent hypertension. The complications of acute kidney injury are
managed symptomatically and may rarely require dialysis.
Poststreptococcal glomerulonephritis is a self-limiting illness with a good prognosis. Typically,

the gross hematuria resolves in 1 to 3 weeks; hypertension, azotemia, and proteinuria resolve in 3
to 4 weeks; low C3 levels normalize in 8 to 12 weeks; and microscopic hematuria may persist for
1 year. If low C3 levels persist beyond 12 weeks, a renal biopsy may be indicated to confirm a
diagnosis of C3 glomerulopathy or membranoproliferative glomerulonephritis.
PREP Pearls
• Poststreptococcal glomerulonephritis usually presents 1 to 2 weeks after a streptococcal
sore throat or 2 to 6 weeks after a streptococcal skin infection
• Complement C3 level usually and complement C4 level is normal in children with post
streptococcal glomerulonephritis.
• Poststreptococcal glomerulonephritis is a self-limited illness. Typically, the gross
hematuria resolves in 1 to 3 weeks; hypertension, azotemia, and proteinuria resolve in 3
to 4 weeks; low C3 levels normalize in 8 to 12 weeks and microscopic hematuria may
persist for 1 year.

• Understand the natural history of acute Poststreptococcal glomerulonephritis
• Plan the appropriate diagnostic evaluation of acute post-streptococcal glomerulonephritis,
with attention to the timing of resolution of abnormal findings
• Plan the initial management of acute post streptococcal glomerulonephritis
Suggested Readings
• Acute poststreptococcal glomerulonephritis. The most common acute glomerulonephritis.
Pediatr Rev. doi: 10.1542/pir.36-1-3
• Nephritis American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.:2358-
2367.
• Hematuria and proteinuria in children. Pediatr Rev. doi:10.1542/pir.2017-0300

Question 85
A 15-year-old adolescent boy is brought to the emergency department via ambulance after being
found in a nearby neighborhood. A passerby called 911; he became concerned when he saw that
the boy was agitated, confused, and appeared to be hallucinating. On arrival to the emergency
department, the adolescent appears disoriented and reports having double vision. His parents
state that over the past month he has been argumentative and has had erratic behavior. He has
reported seeing objects and colors that are not there, which has made parents concerned that he is
hallucinating. The boy has a new group of friends at school, and his grades have dropped
significantly over the past 3 months. He has no history of fever or recent illness. He was seen by
his pediatrician 4 months ago for a health supervision visit, at which time everything appeared to
be 'completely normal.”
He has a temperature of 37.5 C, heart rate of 123 beats/min, respiratory rate of 14 breaths/min,
and blood pressure of 115/75 mm Hg. He is awake and alert. He appears confused and agitated,
with disorientated and slurred speech. He occasionally laughs uncontrollably and points to the
corner of the room. His physical examination reveals flushed skin, excessive salivation,
horizontal nystagmus with normal-sized, reactive, equal pupils, and an occasionally irregular
cardiac rhythm.
Of the following, the adolescent's condition is MOST likely due to

A. acetaminophen overdose
B. inhalant abuse
C. marijuana Intoxication
D. opiate overdose

Correct Answer: B
The adolescent in the vignette is exhibiting sign and symptoms of intoxication caused by inhalant
use, Manifestations of inhalant abuse include double vision, nystagmus, excessive salivation,
flushed skin, dysphoria, delusions, slurred speech, and hallucinations. Acute cardiotoxicity and
dysrhythmias are the most common cause of death resulting from inhalant abuse. Chronic abuse
of inhalants can lead to cardiomyopathy, leukoencephalopathy, neuropathy, and severe
neurotoxic sequelae. Acid-base imbalance and electrolyte abnormalities may occur, especially
with toluene-based products (e.g., spray paints). The initial evaluation of a child suspected of
intoxication due inhalant abuse includes electrocardiography to assess for arrhythmias, arterial or
venous blood gas analysis, and a basic metabolic panel. A urine drug screen hay be considered to
evaluate for polysubstance abuse.
Children with acetaminophen overdose may initially be asymptomatic with progression of

symptoms to include nausea, vomiting, malaise, and fatigue in the first 24 hours. As
acetaminophen poisoning progresses, children may develop worsened vomiting, right upper
quadrant abdominal pain, tachycardia, hypotension and decreased urinary output. In the late
stages of acetaminophen toxicity, children will develop fulminant liver failure, coagulopathy,
hypoglycemia, and hepatic encephalopathy; eventually, death from multi-organ failure may
occur.
Intoxication from marijuana use may include increased blood pressure and respiratory rate,
increased appetite, euphoria and conjunctival injection, Classic signs of opiate overdose include
altered mental status, miosis, decreased respiratory rate or depressed respiratory status,
hypothermia, hypotension, and bradycardia.
Inhalants are an often under recognized drug of abuse; they are widely popular especially in the
teenage population due to their easily accessible, commonly available, inexpensive, and easily
hidden. Commonly inhaled products include spray paint and hairspray), volatile solvents (e.g.,
paint thinners and glues), and gases (e.g., propane tanks and butane). “Huffers” of paint may
have important findings on physical examination of acutely intoxicated child. Metallic-colored
paint, gold or silver, are particularly popular due to a high solvent content. There is no specific
antidote for inhalant toxicity. Treatment is supportive, including stabilization of the airway,
breathing, and circulation (e.g., dysrhythmias).
PREP Pearls
• Signs and symptoms of acute inhalant intoxication may include euphoria, hallucinations,
nystagmus, skin flushing, slurred speech, excessive salivation, and altered mental status.
• Acute cardiotoxicity and dysrhythmias are the most common cause of death from
inhalant abuse.
• Inhalants are easily accessible, widely available, inexpensive, and easily hidden, making
them a popular drug of choice, particularly in the adolescent population.

• Recognize the clinical findings and risks associated with an acute inhalant overdose, and
manage appropriately
• Understand the variety of substances used as inhalants
• Identify the major physiologic consequences associated with inhalant use/abuse
Suggested Readings
• Clinical review of Inhalants. Am J Addict. doi: 10.1080/105504901750160529.
• Poisoning. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed. 2924-
2950
• Inhalant use and Inhalant use disorders In the United States. Addict Sci Clin Pract.
PMID: 2200.3419
• Common substances of abuse. Pediatr Rev. doi:10.1542/pir.2017-0267

Question 86
A 17-year-old adolescent boy with poorly controlled asthma and obesity seen for a health
supervision visit. He and his mother have heard of a new dieting program that claims to help
individuals lose 10% of their weight within 6 weeks without exercising. They ask if this would
be a viable option for him. He leads a sedentary lifestyle, often skips breakfast and lunch and has
been non-compliant with asthma medications. On physical examination, his weight is at the 95th
percentile, his height is at the 50th percentile and his body mass index is at 96th percentile for age.
His vital signs and physical examination findings are otherwise normal, with a sexual maturity
rating of 4.
Of the following, the MOST appropriate advice for this family that the boy should
A. First have measurement of vitamin and levels to personalize his diet
B. Increase his physical activity while maintaining a well-balanced diet
C. start the new diet in order to improve both asthma and his obesity
D. wait until he has achieved his full height potential before starting the diet

Correct Answer: B
The adolescent in the vignette should increase his physical activity while maintaining a well-
balanced diet. A well-balanced diet will provide all the nutrients needed in appropriate
proportions to maintain a healthy weight. Including other healthy habits such as exercising is a
crucial part of weight management. Targeted screening for vitamin and mineral deficiency may
be warranted, but would be complementary to other lifestyle changes. A dieting regimen alone
will not improve his asthma. He does not need to wait to reach his full height potential before
starting the diet.
Families trying to lose weight may focus primarily on diet changes to achieve goals. These
include fad diets that become popular for a short period but have not been studied rigorously, are
not sustainable, do not improve long-term health, and may be nutritionally deficient. Younger
children in particular should be advised against fad diets, because they do not provide the
appropriate nutrition or energy requirements needed for optimal growth
Low carbohydrate diets are classic examples of fad diets. They purport to decrease weight by
suppressing hunger from ketosis, but this claim lacks substantial evidence. These diets often
limit dairy, fruit, and vegetable intake which may lead to deficiencies in calcium; folate; iron;
magnesium; potassium; thiamin; vitamins A, B12 and E; and fiber. They may also be higher in
protein, which is harmful for children who have renal disease.
Some diets designed for legitimate medical conditions are used as fad diets outside of medical
supervision. These diets are not without risks. For example, ketogenic diets used to control
medically refractory epilepsy may lead to problems such as poor growth, hyperlipidemia, iron-
deficiency anemia, and kidney stones. Gluten free diets, used to manage celiac disease, gluten
sensitivity and wheat allergy may actually lead to weight gain and cause gastrointestinal
complaints and vitamin deficiency among other issues. These diets should therefore be used
specific, diagnosed medical conditions and only under the supervision of a nutritionists,
specialists, and primary Care providers.
A plethora of other fad diets are also available including liquid diets, fasting diets, and food-
specific diets (e.g., grapefruit or cabbage). These diets have not been studied adequately in
children and should not be attempted. Families may, however try fad diets on their own without
informing their medical providers. Pediatricians should proactively ask families about their food
choice, any supplements, or nontraditional medications.
Fad diets may also claim to produce results within a limited time frame. This may lead into
families' desires fora quick fix, especially those struggle with other chronic medical conditions
such as the boy in the vignette, but these claims belie the chronic and complex nature of obesity.
Unsuccessful attempts at dieting may lead to disordered eating and exacerbate any underlying
problems with mental health or family dynamics.
Weight management should have a multidisciplinary approach within a medical home, involving
the entire family, primary care physician, nutritionist, and mental health providers to set small,
achievable goals toward sustaining lifelong healthy practices. The American Academy of

Pediatrics Institute for Healthy Childhood Weight (https://ihcw.aap.org) provides resources for
families and providers, as well as a management algorithm
https://ihcw.aap.org/resources/Documents/algorithm_brightfuture_032819.pdf
PREP Pearls
• Diet changes alone are inadequate for weight management; a multidisciplinary approach
including exercise should be used.
• Fad diets may result in nutrient deficiency, growth problems, and disordered eating,
exacerbating any underlying problems in mental health or family dynamics.
• Pediatricians should proactively ask families about their food choices, supplements, or
nontraditional medications.

• Recognize the possible adverse effects of 'fad" weight loss diets
Suggested Readings
• Fad diets. Pediatric Nutrition, 6th ed. 391-404.
• Fad dieting and weight loss in children. Pediatr Rev. doi:10.1542/pir.2016-0009

Question 87
A group pediatric practice is planning a quality improvement project to implement
comprehensive healthy lifestyle counseling for children with obesity. Prior to starting the project,
the group would like to determine the prevalence of obesity in the patient population.
Of the following, the BEST data source for the most accurate determination is
A. body mass index percentile calculated from height and weight measurements
B. documentation of obesity in the text of the clinic note
C. International Classification of Diseases, 10th revision (ICD-10) codes for obesity
D. the known prevalence of childhood obesity in the United States

Correct Answer: A
The best data source to determine the prevalence of obesity in this group practice's patient
population is body mass index percentile calculated from height and weight measurements. The
primary data are the height and weight measurements. Body mass index percentiles for age and
sex can be calculated (or have been calculated by the electronic medical record), and the
prevalence of those with a body mass greater than or equal to the 95th percentile for age and sex
can be determined. This method will give the most accurate prevalence of obesity for the group's
patient population.
Documentation of obesity in the text of the clinic note or by International classification of

Diseases, 10th Revision (ICD-10) codes can estimate obesity prevalence. However, obesity may
not be recognized and/or documented when it is present. Thus, these data sources will likely
underestimate the true prevalence of obesity. The known prevalence of childhood obesity in the
United States may not reflect the prevalence of the group practice’s patient population.
The best data source for a given study is that which best answers the question being asked.
Primary data are collected specifically for a research study. Primary data collection is often
prospective but may be retrospective. Although the height and weight measurements in the
vignette were not initially collected for research purposes, this data can be collected
retrospectively to calculate body mass index percentiles and determine true prevalence of obesity
for those who have accurate height and weight measurements available. Primary data collection
be time consuming and expensive.
Secondary data are existing data that were not collected for a specific research purpose.
Examples in clinical research include information in the medical record collected for clinical
care purposes, diagnostic and billing codes, and clinical registries. Using secondary data is
usually less time consuming and expensive than collecting primary data, but data quality can be
compromised. Thus, data quality and limitations for the specific research purpose should be
carefully considered when using secondary data.
PREP Pearls
• The best data source for a given study is that which best answers the question being
asked.
• Primary data are collected specifically for a research study. Primary data collection can
be time consuming and expensive.
• Secondary data are existing data that were not collected for a specific research purpose.
Data quality and limitations for the specific research purpose should be carefully
considered when using secondary data.

• Assess how the data source (e.g., diaries, billing data, and discharge diagnostic code) may
affect study results

Suggested Readings
• Selection of a data sources developing a protocol for observational comparative
Effectiveness Research: A User's Guide. Agency for Healthcare Research and Quality
• Research and statistics: Study design and data sources. Pediat Rev. doi: 104542/pir.34-8-
371.
• Fundamentals of research data and variables: the devil is in the details. Anesth Analg.
doi: 10.123/ANE.0000000000002370

Question 88
A 14-year-old adolescent girl is seen for a health supervision visit. Her mother has questions
about possible scoliosis. She is otherwise healthy and has had normal growth and development,
with menarche occurring 2 years ago. She has no family of scoliosis. A scoliometer measurement
is 10 degrees with an Adams forward bend test (See procedure demonstrated in Item Q88). Her
physical examination findings are otherwise unremarkable. Standing radiography of the spine
reveals a wedge vertebra at T11 with a Cobb angle of 15 degrees and a Risser score of 4.
A. initiate thoracolumbar bracing
B. obtain orthopedic consultation
C. perform flexion and extension radiography
D. perform renal ultrasonography

Correct Answer: D
The adolescent in the vignette has scoliosis caused by a vertebral malformation (T11 wedge
vertebra), and is considered to have “congenital scoliosis,” even though her curve was not
detected until adolescence. Because congenital scoliosis is associated with genitourinary
anomalies, renal ultrasonography should be performed.
Vertebral malformations can be classified as:

• Failures of formation: Wedge vertebra and hemivertebra are caused by partial or
complete lack of development of half of the involved vertebral body (Item C88A)
• Failures of segmentation: Vertebrae do not property separate during development.
Bilateral segmentation defects produce block vertebrae. Unilateral segmentation defects
result in bony bars that tether one side of the vertebral bodies and produce scoliosis that
progresses 5 to 6 degrees per year (Item C88B)
• Failures or mixed type: Variable combinations of the 2 malformations described above.
Scoliosis caused by a unilateral bar with a contralateral hemivertebrae may progress
between 5 and 10 degrees per year.
Item C88A & Item C88B
Children with suspected congenital scoliosis should undergo anteroposterior and lateral
radiography of the entire spine that includes all cervical, thoracic, and lumbar vertebrae. In
infants, these radiographs can be obtained while supine; older children should be standing.
The progression of congenital scoliosis is highly variable. Affected children should be monitored
closely during periods of rapid growth. Depending on the type of malformation and pattern of
progression, radiography should be performed every 3 to 12 months while the child is still
growing. Congenital scoliosis is not typically associated with spinal instability; therefore,
radiography in flexion and extension is not indicated.
The goal of scoliosis treatment is to prevent the development of a curve that impairs function or
progresses into adulthood. Bracing does not correct the structural curve in congenital scoliosis
and is not an effective treatment. Children with curves that approach 20 degrees who are still
growing should be referred to an orthopedic surgeon for monitoring and potential surgical
planning. Surgical correction is typically considered in skeletally immature children when curve
progression is greater than 10 degrees per year, or when curves approach 40 degrees. Curves
greater than 50 degrees in skeletally mature individuals will typically progress through adulthood
and warrant surgical correction. Skeletal maturity is assessed by Risser assessment of
ossification or ossification of the iliac crest apophyses on anteroposterior radiographs. The Risser
score ranges from 1 to 5 with a score of 5 indicative of being fully mature. A Risser score or 4 or
5 indicates limited remaining linear growth and is associated with a low risk for curve
progression. The adolescent in the vignette is approaching skeletal maturity and has a
radiographic curve of only 15 degrees; therefore, orthopedic consultation is not necessary.
The spine and the urogenital, cardiovascular, and musculoskeletal systems are formed
concurrently during early embryologic development; more than half of the children with
congenital scoliosis have associated anomaly. Genitourinary defects occur in 25% to 39% of
children with congenital scoliosis, and renal ultrasonography is universally recommended. Up to
40% of affected children have spinal cord abnormalities and 12% have cardiac defects. Some
experts recommend echocardiography and spine magnetic resonance imaging based on clinical
findings, while others recommend these studies in all children with congenital scoliosis
PREP Pearls
• Congenital scoliosis results from structural vertebral malformations and cannot be
corrected by bracing
• Children with congenital scoliosis and curves of 20 degrees or greater should be referred
to an orthopedic surgeon.
• Renal ultrasonography should be performed for all children with congenital scoliosis.
• Recognize common congenital anomalies.

• Recognize conditions commonly associated with congenital scoliosis
Suggested Reading
• Spinal deformities. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.
2663-2675.
• Congenital scoliosis. Eur Spine J. 463. doi:10.1007/s00586-003-0555-6 .
• Congenital scoliosis. J Am Acad Orthop Surg. doi: 10.5435/00124635-200407000-
00007.
• Spinal deformities. In: Pediatric orthopaedics and Sports Injuries. American Academy of
Pediatrics: 2010:111-139

Question 89
A 13-year-old adolescent boy is seen in the emergency department for right lower quadrant
abdominal pain and diarrhea of 2 days' duration. He has had 5 nonbloody loose stools. He has
not had any fever. One week ago, the family visited a poultry farm. No other travel was reported.
The adolescent's history is significant for acute lymphoblastic leukemia treated with
chemotherapy at age 3 years. His disease has been in remission since then, he is fully
immunized, and he is otherwise healthy. His vital signs include a temperature of 37.5 °C, heart
rate of 90 beats/min, respiratory rate of 15 breaths/min, and blood pressure of 105/60 mm Hg.
Physical examination findings are significant only for increased bowel sounds and mild right
lower quadrant abdominal tenderness. Abdominal ultrasonography shows a normal appendix. A
stool multiplex polymerase chain reaction panel detects Campylobacter Jejuni.
Of the following, the BEST next management step for this boy is
A. azithromycin orally for 3 days
B. levofloxacin orally for 5 days
C. reassurance and supportive care
D. stool culture for confirmation

Correct Answer: C
The boy in the vignette has acute gastroenteritis caused by Campylobacter jejuni. The best next
management step for him is supportive care. The boy has mild symptoms of abdominal pain and
non-bloody diarrhea, does not appear dehydrated and currently does not have any underlying
immune deficiency or chronic condition that predispose him to severe disease. In this scenario,
the mainstay of management is adequate replenishment of fluid losses and correction of any
electrolyte imbalance. Antibiotics are not indicated in otherwise healthy children with mild to
moderate symptoms. Use of stool polymerase chain reaction assays (PCR) is rapidly expanding.
It provides a faster result and is more sensitive compared with culture. The drawback is detection
of nonviable DNA end lack of antibiotic susceptibility data. For the boy in the vignette a stool
culture is not needed.
Campylobacter are gram-negative, comma shaped bacteria. The 2 most common species
responsible for gastroenteritis are C jejuni and Campylobacter coli. The incubation period is 2 to
5 days. Campylobacter spreads through consumption of raw or undercooked poultry,
unpasteurized milk, or contaminated water and through contact with infected animals.
Abdominal pain, diarrhea, and vomiting are the most common presenting symptoms of
Campylobacter infection in children. Bloody diarrhea is present in half of affected children.
Symptom severity is often mild to moderate and the infection is usually self-limiting.
Campylobacter gastroenteritis can mimic other gastrointestinal illnesses. The clinical

presentation in infants can mimic intussusception, with vomiting and abdominal pain without
fever. In older children, like the boy in the vignette, ileocecal inflammation can cause right lower
quadrant pain that mimics appendicitis. Imaging studies can help differentiate Campylobacter
gastroenteritis from other illnesses.
Antibiotics can be considered for a child with Campylobacter gastroenteritis with high fever,
bloody diarrhea, worsening symptoms, symptoms tasting longer than 7 days, or a compromised
immune system. Antibiotic treatment shortens the duration of illness by 1 to 3 days. When
indicated, the antibiotic treatment choices include azithromycin for 1 to 3 days and erythromycin
for 5 days. A quinolone, such as levofloxacin, is an acceptable option for definitive treatment
when susceptibilities are available. However, empiric treatment with a quinolone is discouraged
given the high incidence of resistance to Campylobacter species (22% to 35%).
Campylobacter species have been the most commonly identified causes of foodborne infections
since 2013 according to the Food Borne Diseases Active Surveillance Network (Food Net). In
the United States, in 2018, the incidence of Campylobacter infection was 19.5 per 100,000
people, followed by Salmonella (18.3 per 100,000), Shiga toxin Escherichia coli (5.9 per
100,000), Shigella (4.9 per 100,000), Vibrio (1.1 per 100,000), Yersinia (0.9), Cyclospora (0.7
per 100,000), and Listeria (0.3 per 100,000)

PREP Pearls
• Campylobacter species are the most common causes of acute gastroenteritis in the United
States. Common presenting symptoms include vomiting, abdominal pain, and diarrhea,
which may or may not be bloody
• Supportive care with attention to hydration is the mainstay of management in mild to
moderate Campylobacter infections in otherwise healthy children.
• Antibiotic treatment (e.g., azithromycin) can be considered for severe Campylobacter
infections or in an immunocompromised child
MOCA-Peds Objective
• Understand the diagnosis and management of infectious diarrhea.

• Plan appropriate management for a patient with Campylobacter infection
• Recognize the clinic features associated with a Campylobacter infection
• Understand the epidemiology of Campylobacter infections
Suggested Readings
• Campylobacter Infections. Red Book: 2021-2024 Report of the Committee on Infectious
Diseases. 32nd ed. 243-245.
• Campylobacter infections in children. Pediatr Rev. doi: 10.15242/pir.2017-0285.
• Infectious Diseases society of America clinical practice guidelines for the diagnosis and
management of infectious diarrhea. Clin Infect Dis. doi: 10.1093/cid/cix669
• Preliminary Incidence and trends of infections with pathogens transmitted commonly
through food-Foodborn Diseases Active Surveillance Network. Doi:
10.15585/mmwr.mm6816a2
• Diarrhea and steatorrhea, American Academy of Pediatrics Textbook of Pediatric Care
2nd ed. 2194-2199

Question 90
A 9-year-old boy is brought to the clinic for evaluation of sore throat, nasal congestion, cough,
fever, and itchy, red eyes that began 2 days ago. He was treated for “strep throat” 2 weeks ago
and says that his throat feels the same now. Review of his chart confirms a positive group A
Streptococcus rapid antigen detection test and a prescription for a 10-day course of penicillin.
The boy and his parents confirm that he took the antibiotic as prescribed and are worried that he
has a resistant infection. On physical examination today, the boy is mildly ill appearing. His
temperature is 38.1 °C and his other vital signs are appropriate for age. He has bilateral
conjunctival injection and yellow-white nasal discharge. His posterior oropharynx is
erythematous with scant tonsillar exudates bilaterally. He has shotty anterior and posterior
cervical lymphadenopathy. His lungs are clear to auscultation bilaterally.
Of the following, the BEST next management step for this boy is
A. a first-generation cephalosporin prescription
B. otolaryngology referral for tonsillectomy
C. repeat group A Streptococcus rapid antigen detection test
D. Supportive care with a nonsteroidal anti-inflammatory drug or acetaminophen and fluids

Correct Answer: D
Despite the recent diagnosis of group A streptococcal (GAS) pharyngitis, the boy’s current
symptoms are consistent with a viral upper respiratory infection. Adenovirus, Influenza,
parainfluenza, rhinovirus, and coronaviruses are all possible causes of this symptoms. Given the
presence of viral symptoms, it is not appropriate to treat him with an antibiotic or to repeat the
group A Streptococcus rapid antigen detection test. Tonsillectomy is rarely indicated for
recurrent GAS infections, but would not be appropriate for this patient. Supportive care with
nonsteroidal drugs (NSAIDs) or acetaminophen for pain and careful attention to hydration is the
best next step for this patient.
Group A streptococcal pharyngitis is common, accounting for 15% to 30% of cases of

pharyngitis in children between the ages of 5 and 15 years. The incidence peaks in winter and
early spring, and the infection is spread through oral and respiratory droplets. The clinical
presentation of GAS pharyngitis overlaps with that of viral pharyngitis, but the presence of
cough, rhinorrhea, ocular symptoms, diarrhea, and/or oropharyngeal vesicles suggest a viral
etiology, whereas scarlatiniform rash, palatal petechiae, tender anterior cervical
lymphadenopathy, pharyngeal exudates and/or abdominal pain are more highly associated with
GAS pharyngitis. Up to 25% of children in high prevalence areas may be asymptomatic carriers
of GAS; it is therefore important to test only patients with symptoms suggestive of GAS
pharyngitis rather than viral pharyngitis.
Group A streptococcal pharyngitis can be diagnosed with a rapid antigen detection test or a
throat culture. Antigen testing has false negatives; culture is considered the gold standard and
should be ordered if the rapid antigen test is negative. Antimicrobial treatment of GAS
pharyngitis decreases the length and severity of illness, prevents acute rheumatic fever, and
reduces the risk of local suppurative complications like peritonsillar abscess. A 10-day course of
penicillin (or amoxicillin because of its superior palatability in suspension form) is the preferred
treatment. Group A streptococcal pharyngitis is almost universally sensitive to penicillin.
Children who are allergic to penicillin can be treated with a first-generation cephalosporin (if no
history of anaphylaxis), macrolide, or clindamycin.
PREP Pearls
• The clinical presentation of group, A streptococcal (GAS) pharyngitis ovelaps with that
of viral pharyngitis, but the presence of cough, rhinorrhea, ocular symptoms, diarrhea,
and/or oropharyngeal vesicles suggest a viral etiology.
• Whereas scarlatiniform rash, palatal petechiae, tender anterior cervical lymphadenopathy,
pharyngeal exudates, and/or abdominal pain are more highly associated with GAS
pharyngitis.
• Group A streptococcal pharyngitis can be diagnosed with a rapid antigen detection test or
a throat culture. Antigen testing has false negatives; culture is considered the gold
standard and should be ordered if the rapid antigen test resul is negative.
• penicillin is the preferred antibiotic for the treatment of group A streptococcal pharyngitis
MOCA-Peds Objective
• Plan the management of a child with recurrent or persistent infection.
• Recognize complication of antibiotic overuse.

• Plan the appropriate diagnostic evaluation of tonsillitis/pharyngitis
• Formulate a differential diagnosis of exudative tonsillitis/pharyngitis
• Plan the appropriate management of tonsillitis/pharyngitis, including when culture results
remain positive following initial therapy
Suggested Readings
• Group A streptococcal infections. Red Book: 2021-2024 Report of the Committee on
Infectious Diseases. 32nd ed :694-706.
• A clinical approach to tonsillitis, tonsillar hypertrophy, and peritonsillar and
retropharyngeal abscesses. Pediatr Rev. doi: 10.1542/pir.2016-0072.
• Clinical practice guideline for the diagnosis and management of group A streptococcal
pharyngitis: 2012 update by the Infectious Diseases Society of America. Clin Infect Dis.
Doi:10.1093/cid/cis629
• Pharyngitis and tonsillitis. American Academy of Pediatrics Textbook of Pediatric Care
2nd ed.:2498-2502.

Question 91
A 5-year-old boy is seen in the office for a health supervision visit. His past medical history is
significant for an atrioventricular septal defect that was repaired at 6 months of age; there is
residual left atrioventricular valve regurgitation that is being monitored by his pediatric
cardiologist. His activity and diet have been normal. His mother has no concerns. The boy's
physical examination findings are normal aside from his murmur, which is unchanged from prior
visits. At the most recent cardiologist visit, his heart function was depressed and boy was started
new medication; the mother cannot remember its name.
Of the following, the MOST likely medication prescribed for this child is
A. atenolol
B. carvedilol
C. enalapril
D. losartan

Correct Answer: C
The boy in the vignette has evidence of heart failure. Among the options presented, the most
likely medication prescribed for this child by the cardiologist is enalapril. The boy has repaired
congenital heart disease and has developed ventricular systolic dysfunction. There are many
conditions leading to heart failure (Item C91A) and multiple clinical presentations (Item C91B)
that vary by age. Children with heart failure may have nausea, vomiting, abdominal pain,
decreased oral intake, fatigue, irritability and increased work or breathing. Physical examination
findings may include tachycardia, tachypnea, diaphoresis, increased work of breathing, and
hepatomegaly. Testing that may help guide the diagnosis and management of heart failure
include electrocardiography, echocardiography, chest radiography, and a brain natriuretic peptide
level. Management of heart failure in children stems from adult data translated into pediatric
practice (Kirk R et al). Treatment is dictated by the stage of heart failure (Item C91C), as
classified by the health care team.
Item C91A: conditions leading to heart failure
Item C91B: clinical presentations of heart failure

The boy in the vignette, who is asymptomatic with decreased systolic function, would be
classified as being in stage B, Clinical guidelines recommend starting an angiotensin-converting
enzyme (ACE) inhibitor, such as enalapril, for patients in stage B. While ß-blockers (carvedilol)
and angiotensin receptor blockers (losartan) have a place in heart failure management, they
would not be the first-line agent in this situation. Atenolol is a ß-blocker typically used for
hypertension or arrhythmia management, but not heart failure specifically. The recommended
approach to the outpatient management of pediatric heart failure is outlined in Item C91D. As
their disease severity advances children may require inotropic support (e.g., intravenous
epinephrine or milrinone), respiratory support, and even progress to the need for mechanical
circulatory support and heart transplantation.
Item C91D: heart failure management

PREP Pearls
• The etiology of new-onset pediatric heart failure varies by age at presentation.
• Clinical manifestations of heart failure in children may include gastrointestinal
complaints, fatigue, irritability, and decreased oral intake.
• Outpatient management of pediatric heart failure is dependent on the stage of
presentation; medications may include angiotensin-converting enzyme inhibitors. ß-
blockers, angiotensin receptor blockers, diuretics, and digoxin.
ABP Content-Specifications
• Plan the appropriate initial diagnostic evaluation of congestive heart failure in children of
various ages
• Plan the appropriate initial management of congestive heart failure in children of various
ages
Suggested Readings
• The International Society and Lung Transplantation Guidelines for the management of
pediatric failure: executive summary. J Heart Lung Transplant.
Doi:10.1016/jhealun.2014.06.002
• Heart failure in infants and children. Pediatr Rev doi: 10.1542/pir.31-1-4
• Cardiogenic shock beyond the neonatal period. Clinical Pediatric Emergency Medicine.
doi:10.1016/j.cpem.2018.12.004
• Congestive heart failure in children. Pediatr Rev. doi: 10.1542/pir.2016-0168
• Heart failure. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.:
2868-2878.

Question 92
A 10-year-old girl with moderately severe asthma requiring an inhaled corticosteroid/long acting
ß-agonist plus leukotriene receptor antagonist daily is brought for evaluation. She has a strong
family history of allergies and chronic nasal congestion and rhinorrhea in addition to her asthma.
Allergy skin testing shows positive results for house dust mites, tree pollen, grass pollen, and
ragweed. In addition to a multimodal approach to environmental control, the allergy consultant
recommends subcutaneous allergen immunotherapy. The child's parents wish to know if they
should pursue this course.
Of the following, the STRONGEST evidence for this therapy is a

A. decreased need for rescue medication
B. decreased use of inhaled corticosteroids
C. improved exercise capacity
D. improved lung function

Correct Answer: B
Allergen immunotherapy is a viable adjunctive treatment for children and adults allergic asthma.
Many studies have evaluated different aspects of this benefit, with variable outcomes. The best
evidence is that allergen immunotherapy allows for decreased use of inhaled corticosteroids and
other controller therapy. Other benefits have been improved quality of life and asthma symptom
scores, though the measurement tools used in studies have not been consistent and some have not
been validated. Allergen immunotherapy has not produced a difference in the use albuterol or
other rescue medication, in frequency of exacerbations or hospitalizations, or in measured lung
function, particularly the forced expiratory volume in 1 second. The benefits of immunotherapy
in exercise tolerance have not been formally evaluated.
Many children with asthma have coexisting atopy. One can make assumptions about potential
allergens in the environment after taking a careful history, including perennial and seasonal
exposures. Recommendations for allergen avoidance can be made generically, but are often met
with resistance, especially when the suggestions relate to family pets or treasured family
pastimes. Avoidance of allergens when reasonable and use of daily controller medications have
been the cornerstone of asthma therapy. When these alone are not adequate for the maintenance
of asthma control specific testing for suspected allergens may be useful. This may involve either
percutaneous prick/scratch tests with allergens to elicit a local wheal and flare response
indicative of focal mast cell release in the skin, or measurement of circulating immunoglobulin E
(IgE) antibodies with in vitro testing. In both cases, positive test results need to be correlated
with symptoms and exposures and may help shape recommendations more specifically.
Consideration for allergen-specific immunotherapy is appropriate when avoidance and a modest
amount of medication do not provide adequate control.
Guidelines for initiating immunotherapy include demonstration of allergen-specific IgE

antibodies (including either allergy skin tests or in vitro tests such as the radioallergosorbent test)
and correlation of symptoms with exposure to those allergens. Consideration of the risk-to-
benefit ratio also should be part of the decision to pursue immunotherapy in a given patient.
Risks include the time needed away from other life events to receive the injections on a regular
basis, local and systemic reactions to the antigens, and failure to improve with treatment.
Benefits in addition to the potential for decreased need for daily medications include subjective
assessment of improvement and improvement in clinical symptom scores. Studies measuring the
use of systemic corticosteroids and hospital or emergency department visits have been
inconclusive or contradictory. Studies have demonstrated that preemptive use of allergen
immunotherapy may prevent the onset of asthma in children with atopy who are predisposed to
asthma.

PREP Pearls
• Testing for specific allergens may be helpful in directing avoidance measures for children
with allergic asthma
• Allergen immunotherapy is indicated for children with allergic asthma and symptoms on
exposure to demonstrated allergens
• Allergen immunotherapy may help decrease the need for daily controller therapies in
children with allergic asthma.

• Understand the indications and limitations of immunotherapy, and manage associated
side effects
• Recognize the frequency of positive immediate-type allergic skin tests in school-age
children who have asthma
Suggested Readings
• Injection allergen immunotherapy for asthma. Cochrane Database Syst Rev. doi:
10.1002/14651858.CD001186.pub2.
• Allergen immunotherapy: A practice parameter third update. J Allergy Clin Immunol
doi: 10.1016/j.jacf.2010.09.034.
• The PAT investigator group. Specific immunotherapy has long-term preventive effect of
seasonal and perennial asthma: 10-year follow-up on the PAT study. Allergy.
Doi:10.1111/j.1398-9995.2007.01451.x
• Allergen-specific immunotherapy in the treatment of pediatric asthma: A systematic
review. Pediatrics. doi:10.1542/peds.2017-3833
• Allergy testing and immunotherapy. Pediatr Rev. doi:10.1542/pir.2018-0126

Question 93
The chair of the school improvement committee has invited the high school health center staff to
a meeting to discuss a plan to address school violence. Most of the physical fights at the school
have been between male students. However, this semester there have been physical fights among
girls as well as concerning acts of aggression between students and teachers. Recently, both a
teacher and a student required urgent medical care for injuries sustained during a fight.
Of the following, the intervention MOST likely to be successful in addressing this problem is (a)
A. cognitive behavioral therapy for students with mental health disorders
B. mentorship program that targets male student with a record of school suspension
C. school-based peer mediation program
D. universal school-based violence prevention program

Correct Answer: D
The vignette describes a school situation in which acts of violence affect multiple groups of
individuals within the school community. The intervention most likely to be successful in
addressing this problem is a universal school-based violence prevention program.
Youth violence involves acts of aggression perpetrated by youth that harm or threaten others.
The acts of aggression may be physical, with or without a weapon, verbal, or cyber-based, and
may be impulsive (emotional) or premeditated (planned). School violence occurs at or en route
to or from school or a school-sponsored event. The perpetrator, victim, and witness (bystander)
all suffer consequences that may include physical injuries, anxiety, depression, substance use,
future perpetration, school dropout, gang involvement, incarceration, homicide, and suicide.
Universal school-based violence prevention programs are characterized by a comprehensive

(multicomponent) approach that incorporates a school-wide violence prevention curriculum and
the development of prosocial, communication, problem-solving, anger management, and
nonviolent conflict resolution skills. These school-based prevention programs also called social-
emotional learning approaches, have been shown to be effective in reducing youth violence
across all school levels, prekindergarten through high school. Cognitive behavioral therapy may
reduce aggression in youth with some mental health disorders, and robust mentorship programs
have been shown to reduce violent behavior. However, universal school-based programs are
more likely to be successful in preventing youth violence than a single-component intervention
that targets a select group of at-risk youth. The success of universal comprehensive programs is
dependent on content, consistency of messages, and fidelity of delivery.
Hoot-based peer mediation programs and authoritarian military style “boot camps” have not been
shown to reduce violence and may have detrimental effects.
Other programs that have been shown to reduce youth violence include early home childhood
home visitation, preschool enrichment with family engagement, parenting skill and relationship
programs, and after-school programs, Model programs have been identified and a summary of
strategies and programs to prevent youth violence is listed (See Item C93 from David-Ferdon C,
Vivolo-Kanter AM, Dahlberg, et al. A Comprehensive Technical Package for the Prevention of
Youth Violence and Associated Risk Behaviors. Atlanta. GA: National Center for Injury
Prevention and Control, Centers for Disease Control and Prevention.)
PREP pearls
• Universal school-based violence prevention programs are more likely to be successful
than interventions that target an individual group of at-risk youth
• Nonviolent conflict resolution skills are an integral component of universal school-based
violence prevention programs.
• Youth perpetrators, victims, and witnesses of violence can all experience consequences
such as physical injury, anxiety, depression, substance use, future perpetration, school
dropout, gang involvement, incarceration, and suicide.

• Identify the various rotes of adolescents with regard to school violence
• Understand age-appropriate non-violent strategies for conflict resolution in adolescence
• Recognize and apply ethical principles regarding violence in society
Suggested Readings
• Violence and aggressive behavior. Pediatr Rev. doi: 10.1542/pir.2016-0062.
• Comprehensive Technical Package for the Prevention of Youth Violence and Associated
Risk Behaviors. National Center for Injury Prevention and Control, Centers for Disease
Control and Prevention. https://www.cdc.gov/violenceprevention/pdf/yv-
technicalpackage.pdf
• Violence. Neinstein’s Adolescent and Young Adult Health Care: A Practical Guide. 6th
ed: 604-609.
• Violence prevention. American Academy of Pediatrics Textbook of Pediatric Care 2nd
ed.:315 321.
• The community guide: Violence Prevention Focused on Children and Youth.
www.thecommunityguide.org/violence

Question 94
A 7-year-old girl is seen in the emergency department for an abnormal gait and change in speech
for the past 2 days. Her family recently returned from a camping trip in rural Pennsylvania. On
physical examination, the girl is drowsy but arousable. She has bilateral ptosis, her speech is
dysarthric, and she is drooling. Lower extremity strength is 4/5 bilaterally at the ankles and
knees, and she has diminished grip strength. She is areflexic in all 4 extremities, and her toes are
unresponsive to plantar stimulation. She has difficulty maintaining an upright seated position.
Sensation is intact to light touch, pinprick, and temperature. No sensory level is identified.
Finger-to-nose testing is normal. She can ambulate with significant assistance with a wide based
ataxic gate.
Of the following, the BEST next step in the evaluation of this child is
A. Brain magnetic resonance imaging
B. Nerve conduction studies
C. Skin examination
D. Toxicology screening

Correct Answer: C
The child in the vignette has a rapid progressive ascending paralysis. The clinical presentation is
consistent with several neurological conditions including Guillain-Barre syndrome, early
transverse myelitis, botulism, tick paralysis, or encephalitis. In children with a history of outdoor
exposure, such as camping in a tick endemic area, a thorough skin examination is a critical
component of the initial diagnostic evaluation and management. If the tick is identified on
examination, removal can result in neurological improvement within an hour and full recovery
within a few days.
Tick paralysis causes a rapid, ascending paralysis, which can involve the bulbar, cranial nerves,
and respiratory muscles. An unsteady ataxic gait can precede the paralysis. Associated cranial
neuropathies can include pupillary dysfunction, ptosis, bilateral facial weakness, and extraocular
muscle weakness. Sensory domains, and bowel and bladder function are preserved. The
presentation and severity vary with duration of symptoms. The neurological symptoms are
secondary to a neurotoxin secreted by the female tick, which blocks acetylcholine release. In
North America, the Rocky Mountain wood tick (Dermacentor andersoni) and American dog tick
(Dermacentor variabilis) are the most common vectors. The appearance and regional
distribution of ticks can be found at
https://www.cdc.gov/ticks/geographic_distribution.html
Diagnosis is dependent on identification of the tick.
The differential diagnosis of rapidly progressive ascending paralysis is broad. If skin

examination fails to identify a tick in the appropriate clinical situation, or there is no identified
risk factor for tick exposure, evaluation should be pursued for alternate diagnosis. Brain and
spine magnetic resonance imaging can be useful for evaluation for early transverse myelitis; the
typical presentation includes bilateral motor and sensory deficits and often bowel and bladder
dysfunction. Nerve conduction studies will show a reduced compound motor action potential in
tick paralysis, which recovers following tick removal, but this test is not needed for confirmation
of the diagnosis. Toxicology screen results would be negative in tick paralysis but can rule out a
toxidrome. Cerebrospinal fluid results are normal in tick paralysis, but testing can be useful in
the diagnosis of infectious and inflammatory etiology.
PREP Pearls
• Tick paralysis presents with a rapidly progressive ascending paralysis, ataxic gait, and
bulbar and cranial nerve dysfunction. The presentation is similar to Guillain-Barré
syndrome.
• The presence of a tick on skin examination is supportive of the diagnosis of tick
paralysis.
• In cases of tick paralysis, there can be improvement in weakness as soon as 1hour
following tick removal, with full recovery within a few days.

• Recognize the clinical findings associated with tick paralysis

Suggested Readings
• Centers for Disease Control and Prevention. Ticks. https://www.cdc.gov/ticks/index.html
• Tick paralysis: A diagnosis not to miss. Neurology doi:11212/WNL.0000000000000206.
• Progressive Weakness in a Previously Healthy 4-year-old Boy. Pediatr Rev
doi:10.1542/pir.2016-0141

Question 95
A 14-year-old adolescent boy with a history of chronic lung disease, heart failure, and chronic
renal insufficiency is admitted to the pediatric intensive care unit for exacerbation of his heart
failure. He has an extensive medical history and confirmed severe allergic reactions to morphine,
vancomycin, and sulfamethoxazole-trimethoprim. He has a heart rate of 100 beats/min,
respiratory rate of 20 breaths/min, blood Pressure of 145/85 mm Hg, and oxygen saturation of
95% on 3 L/min of oxygen. Physical examination findings are significant for signs of plethora
with facial and eyelid edema, diffuse fine crackles in his lungs, and an S3 gallop. Chest
radiography shows cardiomegaly and diffuse interstitial edema with small bilateral pleural
effusions. He is given a medication with his condition, and within 30 minutes develops diffuse
urticaria, wheezing, hypotension, and respiratory distress
Of the following, the medication that was MOST likely administered is

A. Digoxin
B. Enalapril
C. Furosemide
D. Spironolactone

Correct Answer: C
The adolescent in the vignette is experiencing a hypersensitivity reaction and anaphylaxis after
the administration of a medication aimed at addressing acute-on-chronic heart failure. Given his
known allergies, of the response choices, furosemide is the most likely drug to have elicited the
allergic reaction; furosemide is a sulfonamide and can induce hypersensitivity reactions in
individuals with known allergy to sulfa medications.
Digoxin is a digitalis glycoside used to treat congestive heart failure. Allergy to digoxin is rarely
reported in the medical literature. Enalapril is an angiotensin converting enzyme inhibitor used to
treat hypertension and congestive heart failure. Although enalapril can cause serious side effects,
the most often described serious effect is enalapril-related angioedema. Spironolactone is an
aldosterone receptor antagonist that promotes water and sodium excretion with reduced
potassium loss compared with other diuretics. Adverse effect; of spironolactone include central
nervous system disturbances and gastrointestinal upset.
Diuretics work by inhibiting sodium reabsorption in specific areas of the renal tubules, resulting
in an increase in both sodium and free water excretion in the urine. Furosemide is a loop diuretic
commonly used to reduce intravascular volume and peripheral edema in children with congestive
heart failure. Furosemide inhibits sodium reabsorption by acting on the thick ascending limb of
the loop of Henle, resulting in increased sodium excretion and free water loss.
Four major types of adverse reactions are associated with furosemide therapy:
• Excessive diuresis leading to intravascular water loss and extracellular fluid volume
contraction. Contraction alkalosis with hypochloremia and high serum bicarbonate may
occur. Prerenal azotemia and acute kidney injury may develop as a result of
overaggressive diuresis and low systemic perfusion.
• Electrolyte abnormalities including hypokalemia, hypomagnesemia, hypocalcemia, and
hyponatremia.
• Hypersensitivity reaction.
• Ototoxicity: Loop diuretics, specifically furosemide and ethacrynic acid, can lead to
permanent deafness. Ototoxicity from loop diuretics is a function of both peak serum
drug concentration and rate of medication infusion.
PREP Pearls
• furosemide is a sulfonamide; it should be avoided in patients with sulfa allergy
• Loop diuretic therapy can result in clinically significant electrolyte abnormalities
including hypokalemia, hypomagnesemia, hypocalcemia, and hyponatremia.
• Loop diuretic induced ototoxicity is a function of serum concentration and rate of drug
administration.

• Recognize the adverse effects associated with diuretic therapy

Suggested Readings
• Common Diuretics Used in the Preterm and Term Infant: What’s Changed?.
NeoReviews. Doi:10.1542/neo.13-7-e410
• Diuretic Therapy. N Engl J Med. Doi:10.1056NEJM199808063390607
• Diuretics in Pediatrics. Paediatr Drugs. Doi:10.2165/00148581-200608040-00004

Question 96
A 13-month-old previously healthy boy is evaluated at an urgent care center for a 5 day history
of fever and frequent loose, nonbloody stools (up to 10 per day). He normally stools 2 times per
day. He has been fussy and his oral intake has diminished; his mother offers him an oral
electrolyte solution frequently. His mother is uncertain about his urine output because all of his
diapers contain loose stool.
The boy has a temperature of 38.5° C, heart rate of 150 beats/min, respiratory rate of 52
breaths/min, blood pressure of 90/58 mm Hg, and capillary refill time of 2 sec.
The boy is nontoxic in appearance and is interactive. His mucus membranes are moist.
Abdominal examination reveals hyperactive bowel sounds; there is no hepatosplenomegaly or
tenderness.
The boy was seen by his pediatrician 3 days ago for similar concerns. A stool culture from that
visit is growing colonies suspicious for Salmonella, blood and urine cultures are negative. His
mother would like to know when he may return to daycare.
Of the following, the BEST response to the mother's question is

A. after completion of a full antibiotic course
B. after a negative stool culture result is obtained
C. once he is completely asymptomatic
D. When stools are <5 per day and stay contained in the diaper

Correct Answer: D
The boy in the vignette has nontyphoidal Salmonella (NTS) gastroenteritis. He may return to
daycare when his stools are fewer than 5 per day and stay contained in the diaper. Nontyphoidal
Salmonella is the most common cause worldwide of bacterial gastroenteritis. The highest
incidence of NTS is in children under the age of 5 years. Clinical presentations of NTS include
asymptomatic carriage the gastrointestinal tract, gastroenteritis, urinary tract infection,
bacteremia, meningitis, brain abscess, and osteomyelitis. Most commonly, NTS presents in
immunocompetent hosts as gastroenteritis with fever, abdominal cramps, and diarrhea, with or
without blood. Invasive NTS is more commonly seen in infants and immunocompromised
children.
The American Academy of Pediatrics Red Book® recommends antimicrobial therapy (which has
unproven benefit) only for patients at risk for invasive disease, including infants younger than 3
months of age, and people with chronic gastrointestinal tract disease, malignant neoplasms,
hemoglobinopathies, HIV infection, or other immunosuppressive illnesses or therapies. If
antimicrobial therapy is initiated in patients in the United States with presumed or proven NTS
gastroenteritis, a blood culture should be obtained prior to antibiotic administration and an initial
dose of ceftriaxone should be given. The patient who does not appear ill or have evidence of
disseminated infection can be discharged with oral azithromycin pending blood culture results.”
The boy in the vignette is well appearing, well hydrated, and at low risk for invasive disease.
Therefore, he can be discharged home with strict return precautions and adherence to hygiene
practices. The Red Book ® provides the following guidance for returning to child care. When
NTS serovars are identified in a symptomatic child care attendee or staff member with
enterocolitis:
• Older children and staff members do not need to be excluded unless they are
symptomatic.
• Stool cultures are not required for asymptomatic contacts.
• Children or staff members with NTS enterocolitis do not require negative stool culture
results.
• Children can return to child care facilities if stools are contained in the diaper or when
toilet-trained children no longer have accidents using the toilet, and stool frequency is no
more than 2 stools above that child's normal frequency for the time the child is in the
program, even if the stools remain loose; and
• Antimicrobial therapy is not recommended for people with asymptomatic NTS infection
or uncomplicated diarrhea or for people who are contacts of an infected person.

PREP Pearls
• Nontyphoidal Salmonella is the most common cause worldwide of bacterial
gastroenteritis; the highest incidence is in children under the age of 5 years
• Antimicrobial therapy for nontyphoidal Salmonella is reserved for infants younger than 3
months of age and those with a chronic gastrointestinal disease or immunocompromised
condition.
• Children with nontyphoidal Salmonella can return to daycare when stools are contained
in the diaper or when toilet-trained children no longer have accidents using the toilet, and
stool frequency is no more than 2 stools above that child’s normal frequency, even if the
stools remain loose.
MOCA-Peds Objective

• Recommend appropriate measures to prevent transmission of pathogens to child-care
center attendees and their families
• Understanding which illnesses require and do not require exclusion from child-care
center attendance
Suggested Readings
• Managing Infectious Diseases in Child care and Schools: A Quick Reference Guide. 4th
ed. 2016.
• Salmonella infections. Red Book: 2021-2024 Report of the Committee on Infectious
Diseases. 32nd ed :655-662.
• Infectious diseases in early education and child care programs. Pediatr Rev. doi:
10.1542/pir.35-5-182.
• Salmonella infections in brief. Pediatr Rev. doi: 10.1542/pir.2017-0198.

Question 97
A 3-day-old term male neonate is being evaluated in the emergency department for hypotonia
and decreased feeding. The neonate was discharged to home yesterday after an uneventful
delivery and postnatal period. He is hypotonic and difficult to arouse. His vital signs include a
blood pressure of 68/40 mmHg, temperature of 37.3 C, heart rate of 170 beats/min, and
respiratory rate of 80 breaths/min.

Blood urea nitrogen 16 mg/dL (5.7 mmo/L)
Ammonia 35 µg/dL (25 µmol/L)
Carbon dioxide 12 mEq/L (12 mmol/L)
Lactate 41.4 mg/dL (4.6 mmol/L)
Urine ketones Negative
Anion gap 20 mEq/L (20 mmol/L)
white blood cell count 8,000/µL (8 x 109/L)
C-reactive protein 0.2 mg/dL (2 mg/L)
Of the following, the neonate's MOST likely diagnosis is

A. fatty acid oxidation disorder
B. glycogen storage disease
C. organic acidemia
D. urea cycle defect

Correct Answer: A
The neonate described in the vignette is seen for acid for hypoketotic hypoglycemia with high
anion gap acidosis, indicating a fatty acid oxidation disorder (FAO). Laboratory findings
differentiating inborn error of metabolism disorders are outlined in Item C97. Common FAO
disorders include medium-chain acyl-CoA dehydrogenase deficiency, very long-chain acyl-CoA
dehydrogenase deficiency, carnitine palmitoyl transferase I and II deficiency, and carnitine
transporter defects. Plasma acylcarnitine and carnitine profiles are used to distinguish between
the different FAO disorders. Acute treatment includes administration of intravenous 10%
dextrose with electrolytes to maintain normoglycemia and prevent catabolism, and intravenous
carnitine. Long-term management includes a low-fat diet, avoidance of prolonged fasting,
supplementation with extra calories when sick, and daily carnitine administration. Annual
echocardiography should be performed to monitor for cardiomyopathy.
Item C97: Laboratory findings differentiating inborn error of metabolism disorders

• Complete blood count with differential
• Urinalysis
• Blood gases
• Serum electrolytes
• Blood glucose
• Plasma ammonia
• Urine reducing substances
• Urine ketones if acidosis or hypoglycemia present
• Plasma and urine amino acids, quantitative
• Urine organic acids
• Plasma lactate
Glycogen storage disorders (GSD) are a group of conditions with clinical features that depend on
the site of abnormal glycogen metabolism. Glycogen metabolism occurs in the liver, muscle,
heart, and brain. Abnormal glycogen metabolism in the liver presents with hepatomegaly and/or
hypoglycemia. Common disorders are GSDI, GSDIII, and GSDXI. Patients are not typically
seen in the neonatal period. The earliest presentation is typically around 3 to 6 months of age
with seizures, lethargy, failure to thrive, developmental delay with ketotic hypoglycemia, and
hepatomegaly. Treatment includes frequent feedings to maintain normoglycemia and prevent
secondary metabolic derangements. Continuous overnight feedings may be administered via
nasogastric or gastric tube. Uncooked cornstarch can be administered overnight via bolus
feedings every 4 to 5 hours. Abnormal glycogen metabolism in the skeletal muscle is typically
seen in young adulthood with exercise intolerance (GSDV/McArdle disease). Those with skeletal
muscle and cardiac involvement (GSD II/Pompe disease) are seen in the neonatal period with
cardiomyopathy and hypotonia. Brain glycogenosis present with neurodegeneration (Lafora
disease).
Organic acidemia presents in the neonatal period with hyperammonemia and high anion gap
acidosis in the presence of urine ketones. Common organic acid disorders include isovaleric
aciduria, methylmalonic aciduria, propionic aciduria, and maple syrup urine disease. The specific
diagnosis is distinguished by the metabolic abnormalities seen on plasma amino acid, urine
organic acid, and acylcarnitine profile testing. Intervention during an acute decompensation

event includes: stopping protein intake, administration of high-rate intravenous glucose (and, if
additional calories are needed, intravenous fat emulsion), intravenous carnitine, and
hemodialysis for hyperammonemia. Long-term management includes a low-protein diet with
supplementation of essential amino acids, and carnitine
Urea cycle defects present in the neonatal period with hyperammonemia and respiratory
alkalosis. Common urea cycle defects include ornithine transcarbamylase deficiency,
citrullinemia and argininosuccinic aciduria. Plasma amino acid, urine organic acid and urine
erotic testing is used to distinguish between these conditions. Long-term management includes a
low-protein diet with supplementation of essential amino acids and utilization nitrogen scavenger
medications, such as sodium phenylacetate and sodium phenylbutyrate
PREP Pearls
• Inborn errors of metabolism can be distinguished during an acute neonatal
decompensation event based on laboratory results.
• Hypoketotic hypoglycemia is a hallmark of fatty acid oxidation disorders.

• Recognize the laboratory features associated with disorders of fatty acid and carnitine
metabolism
• Recognize the clinical features associated with disorders of fatty acid and carnitine
metabolism
Suggested Readings
• Inborn errors of metabolism in infancy. A guide to diagnosis. Pediatrics doi:
10.1542/peds.102.6.e69.
• Metabolic disorders beyond the newborn period. Special Considerations in the
biochemical evaluation of inborn errors of metabolism. American Academy of Pediatrics
Textbook of Pediatric Care 2nd ed. 2328-2329.
• Inborn Errors of Metabolism.15. Pediatr Rev. doi:10.1542/pir.2014-0122

Question 98
A neonate with a cleft lip is evaluated in the newborn nursery. The cleft extends through the
uvula lip but does not extend up to the nose. The neonate has an intact palate and normal uvula
with appropriate suck reflex. The remainder of the physical examination findings are normal.
The family asks what methods of feeding may safely attempted.
Of the following, the BEST response is that the neonate

A. can attempt to breastfeed with assistance from a lactation consultant
B. can only attempt bottle feeding and only with specialty bottle
C. can only attempt bottle feeding and may use a standard bottle
D. cannot attempt oral feedings and will require nasogastric feedings

Correct Answer: A
The neonate in the vignette can attempt to breastfeed with assistance from a lactation consultant.
Some neonates with a cleft lip, but intact palate, can be successful at breastfeeding or standard
bottle feeding. Affected neonates should be evaluated shortly after delivery by a lactation
specialist to assess their ability to feed and help parents be successful. Cleft lip alone should not
cause the neonate to cough or choke with feedings; if this occurs, further evaluation is necessary.
Neonates with cleft palate often require assistance with feedings given their inability to make
adequate seal required to express milk from the breast or typical bottle. Tere are a number of
options to help with bottle feedings for these neonates (see Lewis CW, Jacob LS, Lehmann CU,
section on oral Health). Holding the infant upright to avoid milk spilling out of the nose,
increased frequency of burping given the volume of swallowed air, and limiting feedings to 30
minutes to avoid fatigue can improve feeding and weight gain.
A Thorough physical examination of all neonates in the newborn nursery is important both to
evaluate for cleft lip and palate as well as associated abnormalities. A subtle finding such as bifid
uvula can be associated with a submucous cleft palate. One condition associated with cleft palate
is Pierre Robin sequence. Affected children have a small, recessed jaw (i.e., micrognathia),
posterior tongue (i.e., glossoptosis), and a U-shaped cleft palate (Item C98). These neonates can
experience airway obstruction shortly after birth or in the weeks after birth. Placing the neonate
prone can help position the tongue anteriorly and improve the obstruction. If respiratory
difficulties continue, additional support, such as intubation may be required. There are nearly
300 genetic syndromes that can be associated with cleft palate including single gene mutations
(e.g., TBX22, MSX1), chromosomal abnormalities (e.g., trisomy 13, Velocardiofacial
syndrome/22q11.2 deletion syndrome) and teratogens (e.g., anticonvulsants).
Item C98: U-shaped cleft palate
Children with a cleft palate require multidisciplinary team health care throughout their life
including care by a nutritionist, geneticist, otolaryngologist, oral surgeon, plastic surgeon, speech
therapist, dentist, orthodontist, audiologist, social worker, and psychologist. Ideally, the family
will meet with the cleft team within the first week of life to establish care, evaluate for additional
anomalies or a known syndrome, and establish adequate nutrition for optimal growth.

Surgical correction of a cleft lip and/or palate varies based on the infants other medical needs,
weight gain, and severity of the defect. Typically, a cleft tip can be repaired between 2 to 6
months of age. Palate repair often takes place between 9 to 18 months of age; repair before 7
months of age is associated with increased risk of midface hypoplasia. A maxillary bone graft to
fill the alveolar cleft is often needed between 6 to 8 years of age.
Children with cleft palate should establish care with a dentist comfortable pediatric care by the
age of 1 year. Affected children are at increased risk of dental caries due to enamel hypoplasia,
increased retention of food particles due to structural anomalies, tight scars which make adequate
brushing and flossing difficult, and the need for oral devices that increase bacterial colonization.
Twice daily brushing with a fluoride toothpaste should be encouraged along with drinking
fluoridated water and fluoride application twice yearly beginning once the first tooth erupts.
Cleft palate is commonly associated with abnormal anatomy of the Eustachian tubes, leading to
frequent middle ear effusions. Affected children often experience acute otitis media prior to 1
year or age and may progress to chronic otitis media and possible hearing loss if not treated.
Hearing loss can further impact the child's speech. It is common practice for myringotomy tubes
to be placed at the time of palate repair. All children with cleft palate require frequent evaluation
by an audiologist.
Velopharyngeal insufficiency occurs in about 10% to 20% of individuals with a cleft palate,
including those with a submucosal cleft. The soft palate is unable to form an adequate seal,
allowing air to escape through the nose, which can cause nasal sounding (hyper nasal) speech.
Sounds most affected are s, z, sh, f, p, b. Surgical correction is required, followed by therapy
with an experienced speech therapist.
PREP Pearls
• Children with a cleft lip and intact palate may attempt to breastfeed.
• Children with cleft lip and palate require a multidisciplinary medical team including
nutritionist, geneticist, otolaryngologist, oral surgeon, plastic surgeon, speech therapist,
dentist, orthodontist, audiologist, social worker, and psychologist.
• Children with cleft palate will require several surgeries, frequent dental and audiological
examinations, and therapies to develop normal speech.

• Plan the appropriate management of a cleft palate in patients of various ages
• Recognize the clinical findings associated with mandibular abnomatities, and manage
appropriately
• Understand the general concept of velopharyngeal insufficiency
• Recognize conditions commonly associated with cleft palate
• Recognize the clinical findings associated with cleft palate, including submucous cleft
and ear sequelae of poor eustachian tube function

Suggested Reading
• Cleft lip and palate. Pediatr Rev. doi:10.1542/pir.35-5-177.
• Section on Oral health. The primary care pediatrician and the care of children with cleft
lip and/or cleft palate. Pediatrics. doi:10.1542/peds.2017-0628
• Cleft lip and cleft palate. American Academy of Pediatrics Textbook of Pediatric Care
2nd ed. 1848-1858.
• Cleft palate. Pediatr Rev. doi:10.1542/pir.30-6-230.

Question 99
A 26-month-old boy is being evaluated for concerns about speech and language. His mother says
that he speaks using single words and has a total vocabulary of about 20 words. She is also
concerned about his behaviors. She states that he does not look at her or looks away, even when
she holds his face in her hands. He has prolonged tantrums, particularly when he does not get
what he wants. The boy is particular about what he eats and gets upset with loud and sudden
sounds. His favorite activity is to line up his blocks and cars. The boy’s father is not as
concerned about the boy’s language and development as he has a personal of “not talking until
preschool”. The father is concerned that the boy tantrums told “no” and that this has made it
difficult when they are out in public.
Of the following, the BEST next management step is referral to a (n)

A. audiologist
B. behavior therapist
C. developmental psychologist
D. occupational therapist

Correct Answer: C
The boy in the vignette has not only a significant delay in his language development but also
atypical social development, repetitive behaviors, and sensitivity to sensory input. This
constellation of symptoms is concerning for autism spectrum disorder (ASD), and the best next
management step is to seek further evaluation of his development. This evaluation could be done
by a specialist experienced in developmental assessment such as a developmental psychologist,
developmental behavioral pediatrician, or neurologist.
Autism spectrum disorder is diagnosed when deficits in social communication and interaction are
seen in combination with restricted and repetitive behaviors. The social deficits include problems
with social and emotional reciprocity and can present as lack of engagement or shared interest in
activities with others (joint attention). Both verbal and nonverbal communication can be affected.
The child with ASD may have delayed language development; difficulty with starting,
maintaining or finishing conversations; and/or poor use and understanding of eye contact, facial
expressions, gestures, and body language. Speech can be unusual in tone, inflection, or rhythm.
Challenges with interpersonal relationships are common. The child with ASD may use scripted
or repetitive speech (i.e., echolalia) or may have repetitive or stereotyped movements such as
hand-flapping or rocking. Play can also be repetitive (e.g., lining things up). The child may have
unusual interests, or their interests may be intense and narrow. The restricted behaviors may
involve insistence on maintaining sameness that results in difficulty tolerating change
particularly when it is unexpected. Transitions can be difficult. When their expectations or
desires are not met, children with ASD may respond with tantrums, self-injurious behaviors, or
aggression. Sensory-seeking or sensory-avoidant behaviors around inputs such as sound, touch,
and smells may also be see.
Young children with ASD typically come to attention due to concerns about their speech and
language development. However, not all children with speech and language delay have ASD.
Those with isolated speech and language delay have age-appropriate nonverbal communication
and play skills. They exhibit normal interest in engaging socially with others. When children first
learn to speak, they may repeat back or “echo” what they hear; this developmentally appropriate
echolalia should resolve as children develop their language skills. Echolalia that persists and is
used without communicative intent should prompt further assessment for possible autism as
should language delay that is seen with behavioral rigidity, repetitive behaviors, sensory issues,
or social difficulties.
Any child who is seen with significant language delay should receive an audiological
evaluation. However, given the accompanying behaviors, a developmental evaluation is more
likely to identify ASD as the source of this language delay and behaviors. A behavior therapist,
particularly one trained in applied behavioral analysis therapy, would be an appropriate
professional to assist this child and family once the child's diagnosis and developmental status
have been established. An occupational therapist could be helpful in addressing this child's picky
eating and sensory issues but would not provide the developmental evaluation needed to
establish the child's diagnosis.

PREP Pearls
• Language delay accompanied by behavioral rigidity, repetitive behaviors, sensory issues,
or social difficulties should prompt further assessment for autism.
• When children first learn to speak, they may repeat back or “echo” what they hear; this
developmentally appropriate echolalia should resolve as children develop their language
skills.
• When their expectations or desires are not met, children with autism spectrum disorder
may respond with tantrums, self-injurious behaviors, or aggression

• Identify the clinical findings, including developmental parameters, associated with autism
spectrum disorders
• Distinguish findings associated with autism spectrum disorder from those of isolated
speech and language delay
Suggested Readings
• Autism spectrum disorders. Zuckerman Parker Handbook of Developmental and
Behavioral Pediatrics for Primary Care 4th ed, 140-146
• The clinician's guide to autism. Pediatr Rev. doi: 10.1542/pir.35-2-62.
• Council on Children with Disabilities. Identification, evaluation and management of
children with autism spectrum disorder. Pediatrics. Doi:10.1542/peds.2019-3447

Question 100
A 6-month-old boy is seen as a new patient for health supervision visit. His parents report that he
has been overall healthy and meeting all his milestones. He started eating solid foods 1 week ago
and does not appear to have any allergic reactions. He occasionally has increased drooling and
decreased oral intake, which his parents have attributed to teething. They report 1 event of a
swollen finger and an occasional diaper rash that usually resolves after a few weeks. He currently
has a bump in his diaper area that does not seem to be resolving.
The boy was born full term via normal spontaneous vaginal delivery. He weighed 3.4 kg and was
discharged home after 2 days. His mother is healthy. Other than occasional mouth sores, his
father reports that he is healthy as well.
The boy is in no apparent distress and is comfortable in his mother's arms. He has a temperature
of 38.1 °C. blood pressure of 100/50 mm Hg, heart rate of 110 beats/min, respiratory rate of 30
breaths/min, and weight of 8 kg. His physical examination reveals moist mucous membranes, a
clear oropharynx, clear lung sounds, normal Sl/S2, no tachycardia or murmur, a soft abdomen,
and no hepatosplenomegaly. There is no palpable lymphadenopathy in the cervical, axillary, or
inguinal chains. His genitourinary examination reveals a sexual maturity rating stage 1 male with
descended testes bilaterally. There is a 1 x 1-cm, raised papule in the perianal region with very
mild erythema, which seems tender to touch. There are no focal neurologic deficits.
Results from a complete blood count are shown:

Hemoglobin 10.5 g/dL (105g/L)
Mean corpuscular volume 92.5 fL
Neutrophils 1%
Lymphocytes 80%
Monocytes 19%
Reticulocytes 1.3%
Absolute reticulocyte count 63,000/µL (63 x 109/L)
Of the following, the most appropriate next step in this boy’s management is
A. Intravenous antibiotics
B. Oral antibiotics
C. Reassessment in 1 week
D. Topical antibacterial ointment

Correct Answer: A
This 6-month-old infant has febrile neutropenia. Neutropenia can be divided into mild, absolute
neutrophil count (ANC) from 1.0 x 103/µL (1,000/µL) to 1.5 x 103/µL (1.500/µL); moderate,
ANC from 0.5 x 103/µL (500/µL) to 1.0 x 103/µL (1,000/µL); and severe ANC less than 0.5 x
103/µL (500/µL). The ANC is calculated using the total percentage of neutrophils and bands
multiplied by the total white blood cell count. A febrile illness in the setting of severe
neutropenia, as seen in the boy in the vignette, requires prompt medical attention. Immediate
administration of intravenous antibiotics, after obtaining a blood culture, is of the utmost
importance to prevent an overwhelming bacterial infection and sepsis. In the case of the child,
there is concern for infection in his perianal area, making intravenous antibiotic therapy of even
greater importance. Oral antibiotics are not recommended in this setting. Due to the risk of
bacterial dissemination, it is important to avoid anything per rectum, including digital rectal
examination, rectal temperatures, and rectal medications.
Though a topical antibiotic ointment can be used in addition to intravenous antibiotics, treating
with only a topical antibiotic ointment in the setting of such severe neutropenia and fever could
lead to a poor outcome.
Reassessment of this febrile neutropenic patient with an active infection at a later date is not
appropriate, as he could develop an overwhelming infection during that time frame.
The cause of this infant's neutropenia will require further investigation. The fact that the other
blood cell lines (hemoglobin and platelets) are normal and that the child appears well is
reassuring. His age raises concern for congenital neutropenia.
There are several causes of isolated neutropenia. Two that are similar in presentation but differ
greatly in their treatment and prognosis are severe congenital neutropenia (SCN) and cyclic
neutropenia. Both may present within the first year of life with chronic infection, often
mucocutaneous or respiratory (e.g., pneumonia, sinusitis). Serial complete blood counts may
help differentiate the 2 conditions. A pattern of recurrent neutropenia approximately every 21
days, will be identified in cyclic neutropenia. Both conditions may have an autosomal dominant
inheritance and can carry a mutation in the neutrophil elastase gene, ELANE. Additional
mutations have been identified for SCN.
The use of recombinant human granulocyte colony-stimulating factor (G-CSF), which rapidly
increases the release of neutrophils into the circulation from the bone marrow, has decreased the
severity and frequency of infections in children with both SCN and cyclic neutropenia. However,
individuals with SCN may require more definitive therapy such as stem cell transplantation, as
some may later develop myelodysplastic syndrome or leukemia. It is therefore important to
investigate the bone marrow of children with SCN at diagnosis, then annually or when there is a
clinical change, such as new cytopenias. Bone marrow investigation is not required in children
with cyclic neutropenia.
For the infant in the vignette, due to the severity of his neutropenia and the presence of an
infection (the area of cellulitis in his diaper region), G-CSF should be administered. It is
important to note that due to the severity of the neutropenia, the usual signs of cellulitis (e.g.,
swelling, warmth, erythema, pus) may not be visible, as neutrophils are required to mount that
response.
PREP Pearls
• Febrile neutropenia requires prompt investigation and administration of intravenous
antibiotics.
• It is important to diagnose severe congenital neutropenia versus cyclic neutropenia, as the
treatment and prognosis can differ significantly.
• It is appropriate to use recombinant human granulocyte colony-stimulating factor in the
setting of febrile neutropenia for both severe congenital neutropenia and cyclic
neutropenia.
MOCA-Peds Objective
• Recognize the presentation of primary disorders of phagocytic number and/or function

• Recognize the variable presentation of congenital neutropenia, and manage appropriately
• Recognize clinical findings associated with neutropenia
• Plan the appropriate laboratory evaluation of neutropenia, and interpret the results
Suggested Readings
• Date DC. How I manage children with neutropenia. Br J Haematol. Doi:
10.1111/bjh.14677
• How to approach neutropenia in childhood. Pediatr Rev. DOI: 10.1542/pir.34-4-173

Question 101
A 16-year-old adolescent boy is being evaluated for a lesion on his back. He reports that the
lesion appeared 5 or 6 weeks ago as a "small red bump" and has grown since then. He has no
history of trauma and no associated symptoms. His physical examination findings are
unremarkable with the exception of a lesion on the upper right side of his back (Item Q101).

A. Advise the adolescent that the lesion will resolve without intervention
B. Apply mupirocin and reevaluate in 4 weeks
C. Cauterize the lesion with silver nitrate
D. Refer the adolescent to a dermatologist for excision of the lesion

Correct Answer: D
The adolescent in this vignette has an enlarging red nodule adjacent to a hyperpigmented plaque
(Item C101A). These findings are concerning for melanoma (the nodule represents an
amelanotic lesion), and prompt referral to a dermatologist for excision is indicated. For a skin
lesion exhibiting a course that is atypical for a benign lesion (i.e., is “EFG”: elevated, firm, and
growing progressively for > 1month), providing reassurance that the lesion will resolve without
intervention, applying a topical antibiotic, or cauterizing with silver nitrate would not be
appropriate.
Only 0.5% of new cases of malignant melanoma occur in individuals younger than 20 years.
Several studies have documented that melanomas in about 60% of children and 40% of
adolescents do not meet the conventional ABCD criteria (asymmetry, border irregularity, color
variegation, diameter >6 mm). Item C101B shows a melanoma that does meet these
conventional criteria. In addition, in children and adolescents, most melanomas (67%) appear de
novo, not in a preexisting congenital or acquired melanocytic nevus. In both children and
adolescents, recent evolution was reported for nearly all lesions, and has been added as an "E' to
the ABCD criteria).
Item C101 B: a melanoma that does meet these conventional criteria
The ABCDE criteria to assist in the diagnosis of melanoma in children and adolescents include:
• A = Amelanotic. Lesions lack visible pigment (Item C101A). Most amelanotic
melanomas (70%) will appear pink or red (and may mimic a pyogenic granuloma, Item
C101C), but some may be skin colored and resemble warts.
• B = Bleeding, bump, Lesions often are papules or nodules, not flat, that may ulcerate or
bleed.
• C = Color uniformity.
• D = De novo, any diameter. Lesions often do not arise in preexisting melanocytic nevi
and may measure 6 mm or more in diameter.
• E = Evolution (recent).
Item C101C: amelanotic melanomas

The physical findings most often exhibited by children and adolescents with melanoma are
summarized in Item C101D.
PREP Pearls
• Melanomas in children often do not exhibit the conventional ABCD detection criteria
(asymmetry, border irregularity, color variegation, diameter >6mm).
• Pediatric melanomas are often pink or red, papular or nodular, and have uniform color
and smooth borders.
• If a skin lesion id "EFG" (elevated, firm, and growing progressively >1 month), the
possibility of amelanotic or nodular melanoma should be considered

• Recognize the clinical findings associated with melanoma
Suggested Readings
• Pediatric solid tumors in children and adolescents: An overview. Pediatr Rev. doi:
10.1542/pir.2017-0268.
• Pediatric melanoma: Results of a large cohort Study and proposal for modified ABCD
detection criteria for children. J Am Acad Dermatol: doi: 10.1016/j.jaad.2012.12.953
• National Cancer Institute, Surveillance. Epidemiology, and End Results Program. Cancer
Stat Facts: Melanoma of the Skin. https://seer.cancer.gov/statfacts/html/melan.html
• Childhood melanoma: An increasingly important health problem in the USA. Curr Opin
Pediatr. doi:10.1097/MOP.0000000000000082

Question 102
The mother of twin 10-year-old boys calls to discuss family dynamics. She explains that one
twin, Bernardo, excels at school, teams quickly, and completes his homework without conflict.
His brother, Jonah, has struggled academically; he has difficulty paying attention in class and
often forgets to turn in his homework assignments. The boys' father coaches their soccer team.
Whereas Bernardo “hates” soccer and complains about attending practices and games, Jonah
loves soccer, scores goals during most games, and has strong friendships with other on the team.
Family conflict around soccer has escalated. Their mother explains that “competition” between
the boys has worsened recently; she has witnessed them “making fun of” each other more often
and on several occasions, they have had arguments that escalate to pushing and shoving.
Of the following, the BEST advice to provide the boys’ mother is to

A. allow the children to resolve physical conflicts independently
B. encourage each child to explore his individual interests
C. require both children to play on soccer team
D. require both children to quit soccer team

Correct Answer: B
The twin boys in the vignette, like most siblings, have Individual strengths and interests and are
experiencing sibling rivalry. The most appropriate advice to provide for this situation is to
encourage each child to explore his individual interests. It would not be appropriate to require
both to play soccer or to quit soccer; neither strategy would diminish rivalry between brothers.
Although parents should allow siblings to resolve nonphysical conflict without interfering,
physical violence should be stopped
Although rivalry is a normal part of most sibling relationships, parents and caregiver can work to
minimize and manage conflict between children. Sibling bullying is an often underappreciated
and underreported phenomenon that is associated with the same negative outcomes as bullying
by a no sibling, including mental health and decreased self-confidence. Although sibling rivalry
may be considered a normal part of child development, sibling bullying is not.
Rather than striving to treat each child in exactly the same way, parents should respond to the
individual needs of each of their children. Parents should avoid commenting on differences
between siblings in front of them; children may assign value (positive or negative) to some of
these differences. Parents should avoid scolding or disciplining children in front of their siblings
when possible.
If children are of similar developmental abilities, parents should let children navigate their
arguments without adult interference, as long as the situation remains safe and nonviolent. This
strategy, allows children to learn skills of negotiation, compromise, and acceptance that will be
important in other relationships.
PREP Pearls
• If children are of similar developmental abilities, parents should let them navigate their
arguments without adult interference, as long as the situation remains safe and
nonviolent. This strategy allows children to learn skills or negotiation, compromise, and
acceptance that will be important in other relationships.
• Sibling bullying is associated with the same negative outcomes as bullying by a no
sibling, including mental health problems and decreased self-confidence. Although
sibling rivalry may be considered a normal part of child development, sibling bullying is
not.

• Provide appropriate anticipatory guidance and counseling with regard to sibling rivalry
Suggested Readings
• Sibling rivalry. 2015. https://www.healthychildren.org/English/family-life/family-
dynamics/pages/siblings-rivalry.aspx
• Sibling bullying and risk of depression anxiety, and self-harm: a prospective cohort study
doi:10.1542/peds.2014-0832
• Siblings without Rivalry. How to help your child live together so you can live too. New
York, 1999

Question 103
A 1-day-old neonate born to a 24-year-old mother with varicella (chickenpox) is being evaluated
the newborn nursery. Her mother developed pruritic vesicular skin lesions 4 days before
delivery. A swab from a skin lesion was positive for varicella zoster virus on polymerase chain
reaction testing. Maternal rapid plasma reagin and HIV antigen-antibody tests were nonreactive.
The neonate was born at term via spontaneous vaginal delivery. After delivery, Apgar scores
were 8 and 9 at 1 and 5 minutes, respectively. Her birth weight was 3.1 kg. The neonate's
physical examination findings are normal.
Of the following, the BEST next management step for this neonate is administration of
A. acyclovir
B. supportive care
C. varicella vaccine
D. varicella zoster immune globulin

Correct Answer: D
The best next management step for the neonate in the vignette is administration of varicella-
zoster immune globulin (VZIG). This asymptomatic neonate is at risk for severe neonatal
varicella infection because her mother was diagnosed with varicella between 5 days before and 2
days after delivery. Neonatal varicella occurs when the mother develops infection around the
time of delivery (perinatal transmission), with the risk to the neonate being greatest when
maternal varicella infection occurs 5 days before through 2 days after delivery. In this setting,
neonatal disease usually manifests between 5 and 10 days after birth and can be life-threatening
(e.g., severe pneumonia, disseminated disease) with a case fatality rate of approximately 20%.
As postexposure prophylaxis, administration of VZIG to high-risk susceptible neonates prevents

symptomatic infection in approximately 50% of cases and decreases disease severity in those
neonates who develop varicella infection. Varicella-zoster immune globulin should be
administered to neonates with high-risk perinatal exposure as soon as possible. Varicella-zoster
immunoglobulin is maximally effective when given within 96 hours but may be given up to 10
days after exposure.
If VZIG is not available, administration of immune globulin intravenous (IGIV) is an alternative

postexposure prophylaxis management strategy to prevent neonatal disease. The American
Academy of Pediatrics recommendations for management of exposures to varicella-zoster (VZV)
are summarized in Item C103. If VZIG or IGIV is not available, some experts recommend oral
acyclovir as preemptive therapy (80 mg/kg per day in 4 divided doses). Valacyclovir is only
recommended as postexposure prophylaxis for high-risk exposed infants older than 3 months.
Varicella vaccine is licensed for use in children 12 months and older for primary prevention and
postexposure prophylaxis.
Intravenous acyclovir is recommended for treatment of neonatal varicella disease. Suppotive care
alone may suffice for asymptomatic, term neonates with exposure to varicella during the
postnatal period, including infants born to mothers who develop varicella 2 days after delivery.
Some experts recommend administration Of VZIG to infants exposed to VZV in the first 2
weeks after birth whose mothers lack varicella immunity.
Airborne and contact isolation precautions should be in place when caring for mothers with
varicella and their exposed neonates. The infant should be separated from the mother until all
maternal lesions are crusted, even after the infant receives VZIG prophylaxis. Although direct
breastfeeding is not recommended during this period of isolation, breast milk can be expressed
and fed to the infant.
Varicella (chickenpox) is a vaccine-preventable communicable disease caused by VZV.

Varicella can cause a wide spectrum of clinical manifestation ranging from mild infection to
severe, complicated disease especially in immunocompromised hosts, pregnant women, and
newborns. The timing of maternal varicella infection during pregnancy determines the disease
severity in neonates. Approximately 25% to 35% of women infected during pregnancy transmit
VZY to their neonates.


Congenital varicella occurs in 2% of neonates whose mothers develop infection during the first
20 weeks of gestation. Congenital varicella syndrome is characterized by severe abnormalities
involving the central nervous system (microcephaly, cortical atrophy, seizures), eyes
(chorioretinitis), skin (scarred skin lesions along a dermatome), and skeleton (limb hypoplasia).
The fatality rate of congenital varicella is approximately 30%.
PREP Pearls
• Neonatal varicella occurs when mothers develop infection around the time of delivery
(perinatal transmission)
• Maternal varicella infection 5days before through 2 days after delivery confers the
greatest risk of neonatal varicella
• Varicella-zoster immune globulin (VZIG) administration as postexposure prophylaxis to
high-risk susceptible neonates prevents symptomatic infection in approximately 50% of
cases; if VZIG is not available, administration of immune globulin intravenously is an
alternative prophylactic treatment strategy to prevent neonatal disease.

• Plan the appropriate management of an immunocompromised patient exposed to varicella
• Plan appropriate antiviral therapy for normal and immunocompromised patients who
have varicella-zoster virus infection
Suggested Readings
• Varicella-zoster virus infections. Red Book: 2021-2024 Report of the Committee on
Infectious Diseases. 32nd ed.831-842.
• Varicella-zoster infections. Pediatr Rev. doi: 10.1542/pir2017-0242
• Chickenpox. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.1835-
1846.

Question 104
An 18-month-old boy about is brought to the office for health supervision visit. His mother is
concerned about his resistance to going to sleep and picky eating. The boy’s medical history is
not significant. His growth, development, and physical examination findings are within normal
for his age. His mother is pregnant, and worried about how she is going to manage her family's
needs when her second child is born. Neither parent is employed, and the family is having a
difficult time making sure they have a stable place to live and food to eat.
Of the following, the MOST appropriate referral to address this family's needs is
A. an early intervention program
B. a home visiting program
C. a parent support group
D. the women, infant, and children program

Correct Answer: B
The family in the vignette, consisting of expectant parents and a young child, is struggling with
multiple needs and could benefit from help with their parenting skills food insecurity, and
housing insecurity. A home visiting program can provide an array of services such as family
support, counseling, case management, and parenting training, to address the challenges faced by
this at-risk family.
The Maternal, Infant, and Early Childhood Home Visiting Program (MIECHV) was established
to implement home visiting models and has been successful in improving outcomes for parents
and their children. Participants in these evidence-based programs have improved physical and
mental health outcomes; children also demonstrate improved development. The MIECHV-
funded programs serve at-risk expectant parents and families with children up through
kindergarten entry. They work to improve family health and relationships, economic self-
sufficiency, development, and school readiness by providing various services such as
management, educational programs, a family support services. Services may include parenting
training, counseling, connection to medical services, screening parental mental health issues, and
screening children for developmental behavioral concerns. Home visits may be conducted by
social workers, childhood educators, or other professionals. Healthy Families America, Healthy
Beginnings, Nurse-Family Partnership, and Early Head Start-Home Based Option are examples
of evidence-based home visiting programs.
Other types of programs are available to support mothers and families in the prenatal and
perinatal periods and families with young infants. Low-cost health care and services can be
accessed at many government-funded health centers. The Special Supplemental Nutrition
Program for Women, Infants, and Children (WIC) is a federal assistance program providing
healthy food, nutrition education, monitoring. Early Head Start is a federally funded early
childhood education program for children younger than 3 years and pregnant women with low
income. Early Head Start provides community-based services for family support, mental health,
and child development; services include prenatal and adult education, State early intervention
programs provide in-home or community developmental services for children from birth to age 3
years who demonstrate significant delays in development. Families can call the national 211
helpline for information on resources in their community that address food. Housing,
employment, and health care needs.
The 18-month-old boy in the vignette does not have developmental delays that would qualify
him for early intervention program. Although this family could benefit from access to
supplemental healthy food from the WIC program and from connecting with others in a parent
Support group, of the response choices, a home visiting program would best provide the range of
services and support needed by this disadvantaged family.

PREP Pearls
• Federally funded home visiting programs serve at-risk expectant parents and families
with children up through kindergarten entry. They work to improve family health and
relationships, economic self-sufficiency, child development, and school readiness.
• Participants in high-quality evidence-based home visiting programs have improved
physical and mental health outcomes; children also demonstrate improved development.
• Families can call the national 211 helpline for information on resources in their
community that address food, housing, employment, and health care needs.

• Identify resources for maternal/familial support during the prenatal and perinatal periods
and early infancy
Suggested Readings
• Overview of the federal home visiting program. Pediatrics doi:10.1542/peds.2013-1021C
• Effectiveness of home visiting in improving child health and reducing child maltreatment
Pediatrics. doi:10.1542/peds.2013-1021G
• Committee on Early Childhood, Adoption, and Dependent Care. The Pediatrician's role
in family support and family support programs. Pediatrics. doi: 10.1542/peds.2011-2664
• Early childhood home visiting. Pediatrics. Doi: 10.1542/peds.2017-2150
• Promoting the Health of young children. American Academy of Pediatrics Textbook of
Pediatric Care 2nd ed.:171-179.

Question 105
A 4-month-old boy is seen for a health supervision visit. His mother states that he smiles and
laughs a lot, makes many noises, and seems to have conversations with himself. When he cries,
he easily soothes with singing. He turns his head to voices and responds appropriately to visual
cues. He has not yet begun to roll, does not reach for toys, and has poor neck control. He was
born at full term. On physical examination, his vital signs and growth parameters are normal. His
head lags when pulled into a sitting position. He does not prop himself up when placed prone. He
does not reach for objects. The remainder of his examination findings are normal.
Of the following, the BEST description of the boy's development is that he has delay in
A. global development
B. language
C. motor development
D. social-emotional development

Correct Answer: C
The boy in the vignette whose head lags when sitting, does not prop himself up when prone, and
does not reach for objects has delayed motor development. His language development is normal
because he laughs, vocalizes, and orients to voice. His social-emotional development is normal
because he smiles spontaneously and soothes to voice. He does not have global developmental
delay.
Infant development is categorized in the domains of motor, cognitive, social-emotional, and

language. A 4-month-old should be able to roll over from front to back, sit with truncal support,
have no head lag, prop himself on his wrists when reach for objects, smile spontaneously, laugh,
and vocalize. Developmental milestones for children of various ages can be found in
“Developmental Milestones* by Scharf et al. The Centers for Disease Control and Prevention
has produced videos of normal infant development
(http://www.cdc.gov/ncbddd/actearly/milestones/milestones-in-action.html) as well as an app for
mobile devices, children's' books, and pamphlets
http://www.cdc.gov/ncbddd/actearly/index.html
for promoting vigilance in monitoring development. Knowledge of what is normal allows for
prompt recognition of any delays in development, which is critical for early diagnosis and
management.
Absence of certain skills should prompt early referral. Item C105A lists red flags for infants up
to 6 months of age. Additional red flags include parental insensitivity, emotional lability,
substance use and depression. Pediatricians should screen for parental red flags at every health
supervision visit.
Infants born prematurely, including those born as late as 36 6/7 weeks' gestation, are at higher
risk for developmental delay. These infants should be assessed based on corrected gestational
age, rather than chronological age, until at least 24 months' chronological age. After this age, any
delays caused by prematurity are usually minimal. Risk factors for developmental delay for these
infants are listed in Item C105B. These infants are also at higher risk for hearing and visual
impairments, which could adversely affect development and may therefore require more frequent
hearing screening and ophthalmology evaluations.
Item C105B: Risk factors for developmental delay in preterm infants

Item C105A: Developmental red flags
PREP Pearls
• An infant with a developmental age of 4 months should be able to roll from front to back,
sit with truncal support, have no head lag, prop himself on his wrists when prone, reach
for objects, smile spontaneously, laugh, and vocalize.
• Parental insensitivity, emotional lability, substance use, and depression are distinct red
nags that negatively affect infant development and shoutd be screened for at every health
supervision visit.
• Infants born prematurely are at higher risk for developmental delay, as well as for hearing
and visual impairments that may adversely affect development

• Evaluate the cognitive and behavioral developmental progress/status of an infant at 4
months of age, including recognition of abnormalities
• Evaluate the motor developmental progress/status of an infant at 4 months age, including
recognition of abnormalities

Suggested Readings
• Infancy visits. Bright Futures; Guidelines for Health Supervision of Infants, Children, and
Adolescents. 4th ed. 2017:303-500.
• Developmental milestones. Pediatr Rev. doi:10.1542/pir.2014-0103.

Question 106
A 5-year-old boy is seen in the emergency department after he fell from the monkey bars on the
playground just prior to arrival. He reports severe pain in his left forearm, he rates his pain as a
7/10 on a pain rating scale that uses faces. Physical examination reveals an anxious child, crying
in pain, with an obvious deformity of his left forearm. His left forearm and hand are
neurovascularly intact. There are no other boy's mother witnessed the fall and states that there
was no loss of consciousness.
Of the following, the BEST next step in the management of this child is to administer
A. codeine orally
B. ibuprofen orally
C. midazolam intravenously
D. morphine intravenously

Correct Answer: D
The boy in the vignette has acute severe pain due to a forearm fracture. A short-acting opiate
such as intravenous morphine would be the preferred medication to treat the pain. Intravenous
benzodiazepines, such as midazolam do not have pain-blocking properties but may be an
appropriate adjuvant to treat anxiety in children experiencing acute pain. Administration of a
nonsteroidal anti-inflammatory drug such as ibuprofen may be appropriate in conjunction with a
short-acting opioid but would not be appropriate monotherapy in a child experiencing acute,
severe pain. Codeine is not recommended for the treatment of pain in children due to variable
metabolism that may result in overdosing or undertreatment. Up to 15% of people poorly
metabolize codeine. Oral hydrocodone and oxycodone are better metabolized into the active
form of morphine.
Appropriate pain assessment with self-reporting or a validated pediatric pain scales essential to
proper pain management. Limited communication skills in children can hinder the evaluation of
pain and often lead to underestimation and therefore undertreatment of their pain. There are
multiple tools available for assessing pain in children including pain scales using faces; the
Neonatal Infant Pain Scale (NIPS); and the Face, Legs, Activity, Cry, Consolability (FLACC)
Scale. The World Health Organization recommends that mild pain be treated with
acetaminophen or nonsteroidal anti-inflammatory medications. Moderate to severe pain should
be treated with acetaminophen or a nonsteroidal anti-inflammatory medication with the addition
of an opiate medication. Side effects of opioid analgesics may include nausea, vomiting, itching,
urticaria, or cardiovascular and respiratory depression. Severe cardiovascular or respiratory
effects may be reversed with naloxone.
Pain management in children should include both pharmacological and nonpharmacological

interventions. Nonpharmacological techniques such as distraction, relaxation exercises,
coaching, comfort positioning, and biofeedback are good adjuvants to pharmaceutical
interventions. Children undergoing pain management require frequent reassessment to monitor
their need for additional doses of medication and for adverse reactions.
PREP Pearls
• Acetaminophen or a nonsteroidal anti-inflammatory medication alone can be used in
children to treat mild pain.
• Acetaminophen or nonsteroidal anti-inflammatory medications plus an opiate should be
used for children to treat acute moderate to severe pain.
• Pain management should include both pharmacological and nonpharmacological
interventions.

• understand the risks associated with the use of narcotics for pain management
• understand the appropriate use of pharmacologic pain management modalities
• Understand the appropriate use of nonpharmacologic pain management modalities

Suggested Readings
• The assessment and management of acute pain in infants, children, and adolescents.
Pediatrics. doi:10.1542/peds.108.3.793
• Relief of pain and anxiety in pediatric patients in emergency medicine systems,
Pediatrics. Doi:10.1542/peds.2012-2536.
• WHO guidelines on the pharmacological treatment of persisting pain in children with
medical illnesses. http://www.ncbi.nlm.gov/books/NBK138354
• What’s new in the management of pain in children. Pediatr Rev. doi:10.1542/pir.24-10-
337

Question 107
A 16-month-old girl is evaluated in the office for a 9-month history of worsening vomiting. She
has nonbilious, nonbloody emesis that occurs after nearly every meal, although she tends to
vomit less often when given liquids (she prefers whole milk or water) or dissolvable foods like
butter crackers and puffs. Noodles, bites of meats, and fruits (like bananas and strawberries)
almost always result in emesis. The girl was born at term. She was breastfed, with intermittent
regurgitation noted prior to the introduction of solids. She has been growing and developing
normally. Her physical examination findings are normal. For her age, her weight is at the 12th
percentile, length is at the 15th percentile, and weight-length is at the 25th percentile.
Of the following, the BEST next step in management is

A. dietary elimination of cow milk protein
B. esophagography
C. reassurance
D. trial of proton-pump inhibitor therapy

Correct Answer: B
The child in this vignette has late-onset (older than 6 months of age) vomiting with persistent
symptoms. Her difficulty associated with eating solids is suggestive of dysphagia, and therefore
causes of esophageal dysfunction, including congenital esophageal stricture and vascular ring,
should be considered. Esophagography is the test of choice to delineate esophageal anatomy.
This patent's esophagography identified a congenital esophageal stricture (Item C107A).
Item C107A: esophagography
Gastroesophageal reflux (GER), the passage of stomach contents into the esophagus, is
extremely common in infants and can vary in presentation from mild spit-up to significant
emesis. Gastroesophageal reflux, which does not require treatment, typically peaks at age 3 to 4
months with significant decreases noted in infants aged 6 to 7 months, and resolution of
symptoms by age 12 months in 95% of infants.
Gastroesophageal reflux disease (GERD) is defined as the presence of GER with concerning
signs and symptoms (including poor weight gain. significant irritability, difficulty feeding).
Treatment may be recommended for GERD depending on the severity of symptoms, which can
include: avoiding overfeeding, thickening feedings, trial of elimination of cow milk protein, or a
trial of acid suppression therapy. Concerning "alarm" signs and/or symptoms include weight
loss, late onset of symptoms (in children older 6 months of age), symptoms without improvement
by 12 months of age, or the warning signs listed in Item C107B these children should be
evaluated for other causes of vomiting. The differential diagnosis for vomiting in infants and
young children is broad (Item C107C), and further testing should be tailored to individual
child’s history and physical examination findings.
Item C107B: Warning Signs in the Vomiting Infant

Bilious vomiting
• Gastrointestinal bleeding: hematemesis, hematochezia
• Consistent forceful vomiting
• Onset of vomiting after 6 months of life
• Failure to thrive
• Diarrhea
• Constipation
• Fever
• Lethargy
• Hepatosplenomegaly
• Bulging fontanelle
• Macrocephaly, microcephaly
• Seizures
• Abdominal tenderness, distention
• Documented or suspected genetic/metabolic disorders (eg, trisomy 21)
The child in this vignette has vomiting associated with concerning symptoms (e.g., difficulty
swallowing, onset of symptoms at greater than 6 months of age, and symptoms without
improvement by 12 months of age), thus further evaluation is warranted (i.e., esophagography).
Reassurance, elimination or dietary cow milk protein, or a trial of proton-pump inhibitor would
not be recommended as initial management steps for this child given her concerning features.
Item C107C: The differential diagnosis for vomiting in infants and young children

PREP Pearls
• Gastroesophageal reflux in infants is common, peaking at 3 to 4 months of age; 95% of
infants experience resolution of symptoms by age 12 months.
• Concerning "alarm" signs and/or symptoms in a child who appears to have
gastroesophageal reflux include weight loss, late onset of symptoms (in children older
than 6 months of age), or symptoms without improvement by 12 months of age.
• Children with signs and symptoms of gastroesophageal reflux disease without
improvement by 12 months of age or with warning signs should undergo further
evaluation.
MOCA-Peds Objective
• Recognize the clinical features of gastroesophageal reflux (including the symptoms
involving other organ systems).
ABP Content Specifications (s)

• Recognize the structural anomalies that interfere with normal esophageal function
• Recognize the significance of regurgitation in infants
Suggested Readings
• Pediatric esophageal disorders: Diagnosis and treatment of reflux and eosinophilic
esophagitis. Pediatr Rev. doi: 10.1542/pir.2017-0266
• Gastroesophageal reflux disease. American Academy of Pediatrics Textbook of Pediatric
Care 2nd ed.:2063-2075.
• Pediatric gastroesophageal reflux clinical practice guidelines: Joint recommendations of
the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition
and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J
Pediatr Gastroenterol Nut. doi:10.1097/MPG.0000000000001889.

Question 108
A female newborn is rooming in with her mother on the maternity ward. The mother is
concerned about her child's coloring, which she states appears blue. She reports that the neonate
otherwise seems fine, and she has no other concerns. The neonate the neonate appears
appropriate for gestational age, is in no distress, and has symmetric bluish purple discoloration of
her extremities (Item Q108A, Item Q108B). Her examination findings are otherwise normal.
Of the following, the BEST next step in management of this neonate is

A. chest radiography
B. complete blood count
C. echocardiography
D. reassurance

Correct Answer: D
The neonate in the vignette has acrocyanosis of the hands and feet. This is a benign finding in
newborns. Reassurance is all that is needed. No other abnormality is noted on examination;
therefore, no other studies are warranted.
Acrocyanosis is a symmetrical bluish-purple discoloration the hands, feet, and, in infants,

sometimes the perioral area. This should not include the lips or tongue. It can commonly be seen
after bathing, feeding, or in a cold room.
Acrocyanosis can be seen outside of the neonatal period. Primary acrocyanosis refers to benign
acrocyanosis, which has no comorbid diagnoses. Secondary acrocyanosis can be an adverse
effect of medications, including psychostimulants (such as those used in the treatment of
attention-deficit disorder). More rarely, it can be associated with anorexia nervosa, neoplasms,
postural tachycardia syndrome, or infection. Treatment recommendations should be targeted at
the underlying disease process.
Central cyanosis includes a bluish discoloration of the skin, lips, and tongue. It may be seen in
patients who have decreased oxygen saturation, typically for pulmonary or cardiac reasons.
Pulmonary causes may include disease processes that result in ventilation to perfusion mismatch,
such as pulmonary edema or pneumonia. Cardiac etiologies include those that have an overall
decrease in pulmonary blood flow and or complete mixing, such as transposition of the great
arteries, truncus arteriosus, pulmonary atresia, critical pulmonary stenosis. Epstein anomaly, and
hypoplastic left heart syndrome.
PREP Pearls
• Primary acrocyanosis is a benign disorder with bluish-purple discoloration of the hands
and feet, and, in infants, the perioral area.
• Secondary acrocyanosis may be associated with medications, neoplasms, postural
tachycardia syndrome, anorexia nervosa, and infections.
• Central cyanosis is a bluish-purple skin discoloration that includes the lips and tongue
and is associated with either pulmonary or cardiac processes that result in hypoxia.

• Distinguish between central cyanosis and acrocyanosis
Suggested Readings
• Hand and foot color change: Diagnosis and management. Pediatr Rev.
doi:10.1542/pir.2016-0234
• Congenital heart disease. Pediatr Rev. doi: 10.1542/pir.2017-0032
• Cyanosis. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.; 2017:
1252-1259.

Question 109
A 12-year-old boy with asthma that has been well managed with low-dose daily inhaled
corticosteroids is brought to the office by his mother because he is needing to use his rescue
inhaler more frequently than previously-up to twice daily on a regular basis. Although he reports
that he has not missed any doses or his inhaled corticosteroids, a review of his medication refill
history includes no better than 50% adherence with his medication. On physical examination, he
is comfortable at rest, with no increased work of breathing or use of accessory muscles. Air entry
is normal but expiration is slightly prolonged, and there is a suggestion of end-expiratory
wheeze. The remainder of the examination findings are normal.
Of the following, the BEST next intervention is to

A. add a leukotriene receptor antagonist
B. change controller to combined inhaled steroid/long-acting ß-agonist
C. obtain spirometry and lung volumes
D. Problem solve with the patient regarding adherence

Correct Answer: D
Nonadherence with or improper use of medications is the most common cause of failure of
asthma treatment. Before changing the regimen or adding additional medications, exploring the
likelihood of and reasons for nonadherence and developing a treatment plan that is agreeable to
all parties is the most appropriate. Even if the patient adheres to the treatment regimen,
inappropriate timing or methods of drug administration may be contributing to poor outcomes.
For a child with asthma such as the boy in the vignette who uses a metered dose inhaler (MDI),
reviewing spacer/holding chamber adherence and technique and ensuring that the inhaler is being
refilled when the counted number of doses has been dispensed is paramount. Many patients will
use a pressurized MDI until it “feels empty" or no spray emits, forgetting or not knowing that
there are more actuations for the canister than there are guaranteed doses of medication. Even
dry powder inhalers with an obvious dose counter can be misused in this way.
Addition of a leukotriene receptor antagonist (e.g., montelukast) or change to a combined inhaled

corticosteroid/long-acting ß-agonist MDI may be beneficial if asthma is still poorly controlled
when the child adherent and using medications properly. The US Federal Drug Administration
implemented a black boxed warning regarding serious neuropsychiatric events that may include
suicidal thoughts or actions in patients taking montelukast. Risks and benefits must be carefully
considered when prescribing or continuing this medication. Measuring spirometry would help to
confirm airflow obstruction as a manifestation of poor asthma control and confirm the status
suspected from history. Measurement of lung volumes might show elevated residual volume as
an indication of air trapping, but it would not be immediately helpful in resolving the poor
asthma control.
The corner stone of asthma therapy is educating the patient and family regarding
pathophysiology, triggers, recognition of changes in status, and appropriate intervention. Use of
an asthma action plan will allow self-directed intervention for anticipated triggers, symptoms, or
changes in documented airflow. Patient/parent understanding of the role of controller and
reliever medications and identification of which specific drugs fill each role is important. This
information can be outlined in the asthma action plan. For some, but not all patients, use of
objective measurements such as peak expiratory flow rate or home spirometry may allow early
deployment of asthma action plan escalation, particularly in those who are poor perceivers of the
severity of airflow obstruction. However, these objective measures are very effort dependent and
cannot be reliably performed by very young and some school-aged children and adolescents.
PREP Pearls
• Medication nonadherence is the primary cause of asthma treatment failure,
• Patient and parent education about asthma is the cornerstone of therapy.
• Use of an asthma action plan can help patients with self-directed therapy for
exacerbations.

• plan appropriate outpatient management of a patient with asthma (e.g., self. assessment,
education, pulmonary function testing, drug therapy, asthma action plans)

Suggested Readings
• Clinical tools to assess asthma control in children. Pediatrics. doi:10.1542/peds.2016-
3438
• Pediatric asthma in a nutshell. Pediatr Rev. doi:10.1542/pir.35-7-287
• Asthma. Pediatr Rev. doi:10.1542/pir.2018-0282
• Communication during pediatric asthma visits and self-reported asthma medication
adherence. Pediatrics. doi:10.1542/peds.2012-0913.
• Family chaos and asthma control. Pediatrics. Doi:10.1542/peds.2018-2758.

Question 110
A 5-year-old boy is seen in the office to establish care. His family emigrated from Indonesia 6
months ago. He has a good appetite and eats a variety of foods including fruits and vegetables.
His mother reports that he often played outdoors barefoot in rural Indonesia. His vital signs are
normal. His weight is at the 25th percentile and height is at the 50th percentile for age. Physical
examination findings are normal except for mild conjunctival pallor.
Laboratory results are shown

Neutrophils 55%
Lymphocytes 25%
Monocytes 5%
Eosinophils 15%
Stool culture No growth of bacteria
Stool for ova and parasite Eggs present, 60 x 40 µm, oval,
thin-shelled (Item Q110)
Item Q110
Of the following, the boy is MOST likely infected with

A. Enterobius vermicularis
B. Giardia duodenalis
C. Necator americanus
D. Taenia saginata

Correct Answer: C
The boy in the vignette has microcytic anemia and peripheral eosinophilia. These findings and
his history of barefoot outdoor play in rural Indonesia are highly suggestive of a Necator
americanus (hookworm) infection, which is transmitted through soil in endemic regions. Necator
americanus is prevalent in North America, Central Africa, parts of India, South Pacific Islands.
And Indonesia. Ancylostoma duodenale, another important hookworm, is in the Mediterranean
region, Northern Asia, and areas of South America
The diagnosis of hookworm is made by visualization of its eggs on microscopy. The eggs are 57-
76 µm x 35-47 µm in size, have an oval or ellipsoidal shape, and have a thin shell (Item
C110A). Treatment choices for hookworm include albendazole, mebendazole, and pyrantel
pamoate.
Item C110A
Although most affected individuals are asymptomatic, hookworm infection can result in
moderate to severe hypochromic microcytic anemia. Chronic infestation in children can cause
growth delay. Eosinophilia can be a sign of parasitic infection. The eosinophilia associated with
hookworm infection has been attributed to persistent attachment of adult worms to the intestinal
mucosa (Item C110B). The period from skin exposure to development of non-cutaneous
symptoms is 4 to 12 weeks.
Item C110B
Enterobius vermicularis (pinworm) presents with intense anal pruritus. The diagnosis made on
microscopic visualization of its eggs obtained with cellophane tape pressed against the perianal
region. Autoinfection occurs after scratching the perianal region and transferring the eggs to the
mouth. Person-to-person transmission occurs when handling contaminated food, clothing, or bed
linens.
Giardia duodenalis (formerly Giardia lamblia and Giardia intestinalis) is a protozoan that
causes giardiasis. Diarrhea is the most common symptom, occurring in 90% of affected
individuals. It can be waterborne, foodborne, or transmitted through the fecal-oral route. Giardia
exists in 2 forms: cysts and trophozoites. The diagnosis is made with stool microscopy detecting
cysts and trophozoites, a nucleic acid amplification test, or an antigen essay.

Taeniasis is caused by 2 species: Taenia saginata, the beef tapeworm, and Taenia solium the
pork tapeworm. Humans are infected by eating raw or undercooked meat containing cysticerci.
Common symptoms of Taenia saginata infection include nausea, vomiting, and epigastric pain.
Peripheral eosinophilia can also be observed. The diagnosis is made on observation of eggs of
proglottids in the stool. Taenia species eggs are round, double-walled, and 30-40 µm in size.
PREP Pearls
• Hookworm infections (Necator americanus, Ancylostoma duodenale) are transmitted
through soil, often during barefoot outdoor activities in endemic regions.
• Hookworm infections can cause normochromic normocytic anemia and peripheral
eosinophilia
• The diagnosis of hookworm is made by visualization of eggs on microscopy. Eggs are
57-76 µm x 35-47 µm in size, have an oval or ellipsoidal shape, and have a thin shell

• Recognize the clinical features associate with Necator americanus infestation
• Understand the epidemiology of Necator americanus
Suggested Readings
• Parasitic infections. American Academy of Pediatrics Textbook of Pediatric Care 2nd
ed.; 2017:2194-2199.
• Hookworm infections (Ancylostoma duodenale and Necator americanus). Red Book:
2021-2024 Report of the Committee on Infectious Diseases. 32nd ed; 421-422
• The Global Economic and Health Burden of Human Hookworm Infection. PLoS Negl
Trop Dis. Doi:10.1371/journal.pntd.0004922
• Intestinal nematodes. Principles and Practice of Pediatric Infectious Diseases. 5th
2018.1373-1381.
• Worm infections in children. Pediatr Rev. doi:10.1542/pir.36-8-341

Question 111
A 15-year-old adolescent boy is brought to the office for a health supervision visit. He has a
history of mild intermittent asthma. He is not receiving any medications. The boy’s mother has a
history of high blood pressure and diabetes. The boy’s weight is 100 kg (>95th percentile), height
is 170 cm (50th percentile), and body mass index is 34.6 kg/m2 (>95th percentile). His heart rate
is 80 beats/min, respiratory rate is 15 breaths/min, and blood pressure is 140/86 mm Hg using an
oscillometric device. A repeat blood pressure with the auscultatory method is 136/82 mm Hg.
Physical examination shows dark, velvety skin discoloration on the back of his neck and axilla.
He has no abdominal bruit. Neurologic examination findings are unremarkable.
Laboratory results reveal normal serum electrolytes, renal function, and thyroid function.
Hemoglobin A1c is 6%. Results of urinalysis with microscopy are normal.
Of the following, this boy’s MOST likely diagnosis is

A. Cushing syndrome
B. essential hypertension
C. pheochromocytoma
D. prehypertension

Correct Answer: B
The adolescent boy in the vignette has stage 1 essential hypertension. The features supporting the
diagnosis of essential hypertension are obesity (body mass index >95th percentile). family
history of hypertension, presence or acanthosis nigricans elevated hemoglobin A1c, and an
unremarkable laboratory evaluation for causes of hypertension.
The American Academy of Pediatrics clinical practice guidelines on pediatric hypertension were
updated in 2017. Hypertension is defined as an average systolic and/or diastolic blood pressure
greater than or equal to the 95th percentile for age, sex, and height. Key changes in the 2017
guidelines include:
• The use of normative blood pressure tables based on children with normal weight: they
do not include values for children who are overweight or obese.
• The criteria for stage 1 and stage 2 hypertension were revised.
• The term “prehypertension” was replaced with "elevated blood pressure.”
• For children 13 years or older, the staging scheme was made consistent with the 2017
American Heart Association and American College of Cardiology adult hypertension
guidelines. The updated definition and stages of hypertension are shown in Item C111A.
Item C111A: updated definition and stages of hypertension
The boy in the vignette had a repeat auscultatory blood pressure reading of 136/82 mm Hg,
which classifies him as having stage 1 hypertension. A systolic blood pressure reading between
120 and 129 mm Hg, with a diastolic reading of less than 80 mm Hg would classify him as
having elevated blood pressure.
Hypertension in children can be classified as primary or essential (no identifiable cause) or

secondary (identifiable underlying cause). Primary hypertension is usually seen in older children
and adolescents who are overweight or have obesity, have a positive family history of primary
hypertension, and are asymptomatic. In contrast, secondary hypertension is more likely in
younger children (age <6 years), may present with diastolic hypertension, and may have signs
and symptoms of an underlying cause. The common secondary causes of hypertension include
renal conditions (e.g., glomerulonephritis, reflux nephropathy, congenital urologic disorders),

renovascular conditions (e.g., renal artery stenosis), cardiac conditions (e.g., coarctation of
aorta), endocrine conditions (e.g., hyperthyroidism. Cushing syndrome, pheochromocytoma),
medications (e.g., corticosteroids, stimulants, decongestants), and other conditions (e g.,
neuroblastoma, tuberous sclerosis). A detailed history, physical examination, laboratory testing,
and imaging are helpful in differentiating primary from secondary causes of hypertension (Item
C111B).
Item C111B: Hypertension evaluation

In Cushing syndrome, hypertension occurs secondary to excessive cortisol levels because of
medications or a tumor. The classic features of Cushing syndrome include moon facies, truncal
obesity, abdominal striae, and easy bruising. None of which are reported for the adolescent in the
vignette. Pheochromocytoma, a catecholamine-secreting tumor, is an unlikely diagnosis in this
case because of the absence of episodic headache, tachycardia, and sweating.
Management of primary and secondary hypertension includes nonpharmacologic (i.e., lifestyle

modifications) and pharmacologic interventions. Lifestyle modifications include weight
reduction for children with overweight/obesity; regular aerobic exercise; dietary changes (low
salt, low fat, high intake of fruits and vegetables); and avoidance of energy drinks, caffeine, and
smoking. Pharmacologic intervention is indicated for stage 2 hypertension, symptomatic
children, presence of end-organ damage (e.g., left ventricular hypertrophy) and stage 1
hypertension not responsive to lifestyle modification. The initial pharmacologic intervention
options for primary hypertension include an angiotensin-converting enzyme inhibitor,
angiotensin receptor blocker, calcium channel blocker, or thiazide diuretic. The choice of
antihypertensive in secondary hypertension will vary depending on the underlying cause. The
goal is to reduce the systolic and diastolic blood pressure below 120/80 mm Hg for adolescents
and below the 90th percentile blood pressure for children younger than 13 years.
PREP Pearls
• Primary hypertension is usually seen in older children and adolescents who are
overweight or have obesity, have a positive family history of primary hypertension, and
are asymptomatic.
• Secondary hypertension is more likely in younger children (<6 years), may present with
diastolic hypertension, and may have signs and symptoms of an underlying cause.
• Pharmacologic intervention is indicated for stage 2 hypertension, symptomatic children,
presence of end-organ damage (e.g., left ventricular hypertrophy), and stage 1
hypertension not responsive to lifestyle modification.

• Plan the appropriate management Of essential hypertension
• Formulate a differential diagnosis of essential hypertension
• Understand the effects of certain drugs on the development of hypertension in children of
various ages
Suggested Readings
• High blood pressure. American Academy of Pediatrics Textbook of Pediatric Care 2nd
ed.; 2017:1429-1443. Pediatric Online.
• Clinical practice guideline for screening and management of high blood pressure in
children and adolescents. Pediatrics. doi: 10.1542/peds.2017-1904.
• Hypertension in children and adolescents. Pediatr Rev: Doi: 10.1542/pir.2016-0106.

Question 112
A 14-year-old right-handed baseball pitcher is seen in the office for a 3-month history of right
shoulder pain. HIS pain started gradually, initially occurring only when throwing, then became
constant throughout the day. The pain is located throughout his shoulder and upper arm. Shortly
before the onset of pain, his throwing velocity and accuracy decreased, and he began working
with a pitching coach to address his technique.
His pain resolved after cessation of throwing for several weeks, but rapidly returned when he
resumed throwing. He is using ice and ibuprofen to manage his pain, and is asymptomatic at rest.
He reports no paresthesias or weakness in his arm or hand.
On physical examination, there is diffuse tenderness to palpation throughout the right shoulder
and upper arm. His pain is reproduced with internal and external rotation of the shoulder, and he
has an internal rotation deficit. Anteroposterior, scapular "Y,” and axillary radiographs of the
right shoulder were obtained. Anteroposterior view and a comparison view of the left are shown
in Item Q112
A. perform magnetic resonance imaging of his shoulder
B. recommend a gradual return to throwing
C. recommend he discontinue throwing for 3 months
D. refer him to an orthopedic surgeon

Correct Answer: C
The shoulder radiographs of the adolescent in the vignette show delayed closure of the right
proximal humeral physis compared with the left. This asymmetry represents a stress fracture
through the growth plate on the right, known as "little league shoulder”, “proximal humeral
epiphysitis”, “proximal humeral epiphysiolysis”. Initial treatment is a period of rest to allow for
bony healing, which typically includes cessation of throwing for about 3 months. A gradual
return throwing is not appropriate at this time.
Little league shoulder is an overuse injury seen most commonly in baseball pitchers, but can
occur in any throwing athlete. It occurs less commonly in gymnasts or those who play tennis or
other racquet sports. Throwing volume and velocity are the greatest risk factors, particularly
among athletes who participate year-round.
Affected children and adolescents commonly report a decrease in throwing velocity or

performance before the onset of pain, and will often increase their training to compensate.
Tenderness to palpation usually is seen over the proximal humerus. Pain may be elicited with all
range-of-motion testing, but is typically most severe with internal and external rotation. Strength
testing may also elicit pain.
Radiographic evaluation should include anteroposterior, scapular Y, and axillary views. Given
the intrinsic irregularities of the humeral physis, it is helpful to perform contralateral
anteroposterior radiography to enhance detection of significant widening or delayed closure on
the involved side.
The throwing motion in skeletally immature children results in significant external rotation stress
across the proximal humeral physis. There is a constant balance between the microtrauma
associated with the throwing motion and the need for recovery that allows the bones and soft
tissues of the shoulder to strengthen and adapt. Appropriate adaptations to this repeated stress
result in a remodeling of the proximal humerus that increases external rotation and decreases
internal rotation, This process is known as humeral retroversion and is considered non
pathological. Humeral retroversion may confer a competitive advantage for the throwing athlete,
but the long-term impact on shoulder function and injury is not clear.
Athletes should be reminded that training results in microtrauma and breakdown of bone and soft
tissues, and that strength and performance improve only when the body adapts and heals after
each training session. When recovery is inadequate microtrauma effects accumulate and injury
occurs. In adults and skeletally mature throwing athletes, this injury most commonly affects the
rotator cuff. In younger throwers, the proximal humeral physis is the "weak link' and widens in
response to cumulative rotary stress.
Injuries typically occur because of some combination of the following:

• Throwing motions that create excessive force across the shoulder. Excessive force
generation may occur with large volumes of throwing at high velocity (often seen in
pitchers who are using a radar gun to monitor their performance), or with suboptimal
technique.

• Inadequate rest and recovery: Recommended rest periods are based on age and number of
throws. Guidance from USA Baseball can be found at htps://www.mlb.com/pitch-
smart/pitching-guidelines. It is important to emphasize that rest days are specifically for
recovery, and not for additional throwing or upper body training. Overhead throwing
should be limited to 8 months per year, with break intervals lasting at least 2 consecutive
months to allow for adequate recovery and to minimize injury risk. Injury risk is
markedly increased in throwers participating on multiple teams simultaneously, and
current recommendations are to limit participation to 1 team per season
Throwing is a complicated movement pattern. Force is ideally generated by the powerful lower
extremity muscles and transmitted through the torso (i.e., core) to the throwing arm. Young
throwers often have difficulty with this force transmission, and will typically try to generate
power with their arm. Poor technique is a risk factor for significant shoulder and elbow injury.
Rehabilitation should focus on developing an effective pattern of power generation and
transmission to avoid recurrent injury.
Magnetic resonance imaging (MRI) is not needed for the adolescent in the vignette because the
clinical diagnosis is straightforward. Shoulder MRI is indicated when the pain is acute in onset,
or if there is significant pain at rest that does not improve within several weeks.
Little league shoulder typically represents a season-ending injury. Management is medical;

orthopedic consultation is not indicated, Aspects of treatment include:
• Placing the affected arm in a sling for several days if there is significant
discomfort at rest or pain with activities of daily living.
• Physical therapy, or an appropriate rehabilitation program with a certified
athletic trainer, if there is no pain at rest. Rehabilitation is initially focused on
the remainder of the throwing kinetic chain (i.e., core/hips) rather than on the
shoulder.
• Rehabilitation goals: Full and pain-free range of motion of the shoulder; full
strength through the entire throwing kinetic chain: and demonstration of
appropriate movement patterns to support correct throwing technique.
• Gradual return to throwing after rehabilitation goats are met, typically about 3
months after treatment is started. Structured progression should occur under
the guidance of a rehabilitation professional or coach.
• Follow-up radiography is not needed. Clinical healing precedes radiographic
healing. Follow-up radiography should be performed only if symptom
improvement does not follow the expected course.
PREP Pearls
• Little league shoulder (proximal humeral epiphysitis) is a stress injury to the proximal
humeral physis and typically requires 3 months of rest to allow for healing.
• Throwing technique is often faulty in children and adolescents who develop little league
shoulder and shoulder and should be addressed before returning to play.

• Recognize the clinical findings associated with growth plate fractures and injuries
• Recognize the clinical findings associated with occult fractures that may or may not
affect gait in patients of various ages
Suggested Readings
• American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.; 2017:2675-2685.
• Chronic shoulder problems in the young athlete. Care of the Young Athlete. 2nd ed.
2010; 369-375.
• Sport specialization and overuse injuries in adolescent throwing athletes: A narrative
review. J Athl Train. doi: 10.4085/1062-6050-333-18

Question 113
An 11-year-old girl is seen with a 1-month history of fatigue, decreased appetite, and dizziness
whenever she stands up. She has a history of autoimmune polyglandular syndrome type 1 with
associated recurrent oral and esophageal Candida infections and hypoparathyroidism. Her
hypoparathyroidism is well controlled with oral calcium carbonate and calcitriol. She has not had
a recent illness, got adequate sleep, and had no abdominal pain, vomiting, or change in stool
pattern. Her vital signs show a temperature of 37 C, heart rate of 106 beats/min, and blood
pressure of 94/48 mm Hg. She has lost 2.3 kg since her last health supervision visit 3 months
ago. Her skin appears diffusely hyperpigmented. The remainder of her physical examination
findings are unremarkable. Laboratory data are shown:
Morning cortisol level 1.5 µg/dL (41.2 nmol/L) (reference range, 6.2-19.4 µg/dL)
serum sodium 130 mEq/L (130 mmol/L) (reference range, 135-147 mEq/L)
Plasma glucose 68 mg/dL (3.8 mmol/L)
Of the following, the additional serum laboratory test results that are MOST likely to be found
for this girl are
Adrenocorticotropic hormone Potassium

(Reference range, 7.2-63.3 pg/mL) (Reference range, 3.5-5.2 mmol/L)
A 4 pg/mL (0.9 pmol/L) 2.8 mEq/L (2.8 mmol/L)
B 4 pg/mL (0.9 pmol/L) 6.5 mEq/L (6.5 mmol/L)
C 2,560 pg/mL (563 pmol/L) 2.8 mEq/L (2.8 mmol/L)
D 2,560 pg/mL (563 pmol/L) 6.5 mEq/L (6.5 mmol/L)

Correct Answer: D
The girl described in the vignette with signs and symptoms of fatigue, decreased appetite, weight
loss, tachycardia, hyperpigmentation, and lower blood pressure has primary adrenal insufficiency
(Addison disease) associated with autoimmune polyglandular syndrome type 1 (APS-1).
Addison disease is caused by damage to the adrenal cortex, leading to decreased production of
glucocorticoid (cortisol) and mineralocorticoid (aldosterone). In the United States, autoimmunity
is the most common cause of Addison disease.
There are several distinguishing features of primary (i.e., a problem with the adrenal gland itself)
and secondary (i.e., a pituitary or hypothalamus problem) adrenal insufficiency. In primary
adrenal insufficiency, there is a deficiency of both glucocorticoid (cortisol) and
mineralocorticoid (aldosterone). Adrenocorticotropic hormone (ACTH) levels are high in
response to low cortisol levels, and plasma renin activity is high in response to low aldosterone
levels. Serum sodium levels are low due to both glucocorticoid and mineralocorticoid deficiency.
Serum potassium levels are high due to the mineralocorticoid deficiency. Mineralocorticoid
deficiency manifests as signs of dehydration. Skin hyperpigmentation occurs due to the high
ACTH levels.
In secondary adrenal insufficiency, there is a deficiency of ACTH, leading to low levels of

glucocorticoid (cortisol). Mineralocorticoid production is normal because the renin-angiotensin-
aldosterone system remains intact. Serum sodium levels can be low due to glucocorticoid
deficiency, but serum potassium levels are not affected due to normal mineralocorticoid
production. Skin hyperpigmentation does not occur because ACTH levels are not high.
Because the girl in the vignette has primary adrenal insufficiency, high levels of both and
potassium levels are expected. Other laboratory findings consistent with primary adrenal
insufficiency include hypoglycemia, metabolic acidosis, and a high eosinophil count.
Autoimmune polyglandular syndrome type 1 (APS-I), also known as autoimmune

polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), is a rare autosomal recessive
condition caused by pathogenic changes in the AIRE gene (autoimmune regulator). The classic
triad consists of chronic mucocutaneous candidiasis, hypoparathyroidism, and primary adrenal
insufficiency. Autoimmunity in multiple other organs also occurs. Autoimmune adrenal
insufficiency can occur as isolated condition or in conjunction with other autoimmune
conditions.
If adrenal insufficiency is suspected, a bedside blood glucose test should be performed given the
high risk for hypoglycemia. Serum electrolytes, glucose, cortisol, and ACTH levels should be
obtained.
The initial treatment of an adrenal crisis consists of restoration of intravascular fluid volume and
correction of hypoglycemia. Five percent dextrose-containing normal saline at 20 mL/kg may be
given if hypoglycemia is present. Subsequently, a stress dose of hydrocortisone at 100 mg/m2
should be given intravenously and continued either as a continuous infusion or at 100 mg/m2/day
divided every 6 hours until clinical status is improved. High-dose hydrocortisone has both
glucocorticoid and mineralocorticoid activity.
Long-term treatment of primary adrenal insufficiency consists of replacement doses of
hydrocortisone (8 mg/m2/day), a glucocorticoid, and fludrocortisone (0.1 mg daily), a
mineralocorticoid. Families should also be provided with instructions on stress dose
hydrocortisone for fever and other moderate stress (double or triple the daily dose divided every
8 hours), a medical alert tag, a prescription for an intramuscular injectable form of
hydrocortisone to have at home in case of an emergency adrenal crisis and an emergency letter
with instructions on stress dose steroids.
PREP Pearls
• Common symptoms of adrenal insufficiency include fatigue, decreased appetite, and
weight loss. Signs of dehydration. Hypotension, and hyperpigmentation may be present
with primary adrenal insufficiency.
• In primary adrenal insufficiency, there is a problem with adrenal gland production of both
glucocorticoid (cortisol) and mineralocorticoid (aldosterone)
• If adrenal insufficiency is suspected, a bedside blood glucose test should be performed
given the high risk for hypoglycemia. Serum electrolytes, glucose, cortisol, and
adrenocorticotropic hormone levels should be obtained

• Recognize the clinical and laboratory manifestations of adrenal insufficiency
• Plan the appropriate management of Addison disease, including an adrenal crisis
associated with the disorder
• Recognize the clinical features associated with Addison disease
• Plan the appropriate diagnostic evaluation for Addison disease
Suggested Readings
• Adrenal insufficiency. Pediatr Rev. doi: 10.1542/pir.36-3-92.
• Diagnosis and treatment of primary adrenal insufficiency. J Clin Endocrinol Metab. doi:
10.1210/jc.2015-1710
• Adrenal dysfunction. American Academy of Pediatrics Textbook of Pediatric Care 2nd
ed.; 2017: 1692-1701.

Question 114
A 1-day-old male neonate born at term gestation is being evaluated for jerky movements. The
maternal history is notable for preeclampsia and group B streptococcus-negative status. The
neonate exhibits sustained rhythmic movement of the upper and lower extremities. The
remainder of the physical examination findings are unremarkable. A bedside glucose level is 65
mg/dL (3.6 mmol/L). Serum electrolyte levels are normal. Echocardiography shows a patent
foramen ovale and branch pulmonary artery stenosis. Brain magnetic resonance imaging is
shown in Item Q114 .
Of the following, the BEST test to obtain in the evaluation of this neonate is a
A. C-reactive protein
B. platelet count
C. protein C level
D. serum calcium level

Correct Answer: C
The clinical presentation and magnetic resonance imaging (MRI) findings for the neonate in the
vignette are consistent with an ischemic perinatal stroke (IPS). Among the response choices,
protein C level is the best test to obtain in the evaluation of this neonate. Ischemic perinatal
stroke occurs in 1 of 1,600 to 5,000 live births with a slight male predominance. The risk of
stroke for children is highest in the perinatal period after birth. The majority of IPS are ischemic
(80%); the left middle cerebral artery is the most commonly affected blood vessel. The
remainder are caused by cerebral sino-venous thrombosis and hemorrhage. Ischemic perinatal
stroke may manifest in the neonatal period as seizures in the first 3 days after birth. These
neonates often feed poorly and may have increased or decreased muscle tone. Alternatively, an
infant with IPS may exhibit early hand preference or delayed motor development during infancy.
For an infant who does not have acute neurologic injury, MRI findings suggestive of IPS are
presumed to have occurred in the first month after birth.
The etiology of IPS is unclear; it is likely a combination of maternal, placental, and neonatal
factors (Item C114A). A battery of laboratory tests should be performed in neonates diagnosed
with IPS, to include a protein C level (Item C114B). Age specific reference ranges should be
used when interpreting coagulation factor levers. Infants should also be evaluated for congenital
heart disease and thrombus in the heart.
Item C114A: The etiology of IPS

• Maternal factors:
• Infection
• Thrombophilia, including antiphospholipid antibodies
• Preeclampsia
• Cocaine use
• Smoking
• History of infertility
• Fetal/infant factors:
• Infection
• Thrombophilia
• Congenital heart disease
• Need for resuscitation or low Apgar score at 5 minutes
• Arterial injury during delivery from mechanical forces on the
• head and neck
• Placental factors:
• Infarction
• Abruption
• Insufficiency
• Chorioamnionitis
Item C114B: laboratory tests for neonates with IPS

• Plasma/Protein Based
o Activated protein C resistance
o Protein C activity/antigen
o Free and total protein S antigen
o Antithrombin activity/antigen
o Lipoprotein (a)
o Fasting homocysteine
o Lupus anticoagulant/antiphospholipid antibodies
o Fibrinogen (Clauss)
o Plasminogen
o Factor VIIIC
• DNA Based
o Factor V G1691A
o Prothrombin G20210A
The distribution of changes on MRI would not be explained by an abnormal C-reactive protein
level. Although meningitis may present with seizure activity, these ischemic changes would not
be expected. Hypocalcemia may precipitate neonatal seizures. However, a low serum calcium
level would not explain the MRI findings. Thrombocytopenia alone without coagulopathy is
unlikely to result in ischemic changes on MRI in a vascular distribution.
Head ultrasonography (HUS) should be performed to evaluate neonates suspected of having IPS
However, HUS may not identify lesions in all anatomic locations and may not be sensitive
enough to identify a recent injury. Ideally, brain MRI with T2-weighted images should be
obtained for diagnosis.
Treatment for IPS is supportive, including stabilization of cardiorespiratory status. Seizures

should be treated. There is no recommendation for routine anticoagulation therapy for IPS.
However, if there is evidence of thrombus propagation, anticoagulation may be considered. Risk
factors for recurrent IPS include congenital heart disease, thrombotic disorders, and vascular
disorders. Long term, 50% to 75% with IPS will have neurologic impairment; IPS Is the leading
cause of congenital hemiparesis.
PREP Pearls
• Ischemic perinatal stroke is the leading cause congenital hemiplegia.
• Infants with ischemic perinatal stroke should be evaluated for thrombotic disorders and
congenital heart disease
• The left middle cerebral artery is the most commonly affected blood vessel in the
ischemic perinatal stroke

• Recognize the clinical and laboratory features associated with intracranial hemorrhage in
a neonate, and manage appropriately
• Recognize clinical findings associated with intracranial bleeding, and manage
appropriately

Suggested Readings
• Stroke in neonates and children. Pediatr Rev. doi: 10.1542/pir.2016-0002
• Pediatric stroke. Nelson Textbook of Pediatrics 21st ed. 2020:3209-3218.
• A Scientific Statement from the American Heart Association American Stroke
Association. Stroke. 2019; doi:10.1161/STR.0000000000000183
• The newborn with hematologic abnormalities. American Academy of Pediatrics
Textbook of Pediatric Care 2nd ed.; 2017:909-917
• Ischemic perinatal stroke: Summary of a workshop sponsored by the National Institute of
Child Health and Human Development and the National Institute of Neurological
Disorders and Stroke. Pediatrics. doi:10.1542/peds.2007-0336

Question 115
An 18-month-old girl is evaluated for a 4-day history of bilateral otalgia. She has been a febrile
with good oral intake and urine output. She started daycare at 12 months of age, and since then
has had frequent ear infections. At her last visit, she was diagnosed with otitis media with
effusion, which was present for over 3 months. The appearance of her tympanic membrane on
physical examination today is seen in Item Q115. The findings of her hearing screening are
abnormal.
The physician and the patient's mother discuss criteria for tympanostomy tube insertion, and the
patient is referred to an otolaryngologist.
Of the following, the BEST indication for the child to undergo this procedure is bilateral otitis
media
A. 3 months or longer
B. 3 months or longer in addition to conductive hearing loss
C. 6 months or longer
D. 6 months or longer in addition to conductive hearing loss

Correct Answer: B
The child the vignette has chronic otitis media with effusion (OME) that has lasted at least 3
months, a retraction pocket of her tympanic membrane (Item C115), and hearing abnormalities.
She should be referred to an otolaryngologist for evaluation potential tympanostomy tube
insertion. The decision to perform tympanostomy tube placement should be made through shared
decision-making with an otolaryngologist, the child's guardian, and the pediatrician.
Tympanostomy tube insertion is recommended in cases for which middle ear ventilation would
be beneficial.
Indications for tympanostomy tube insertion include:

• Bilateral OME that lasts 3 months or longer with conductive hearing loss
• Bilateral or unilateral OME that lasts at least 3 months together with risk factors for
speech. Language, or learning problems (e g., craniofacial anomalies,
neurodevelopmental disabilities, etc.)
• Bilateral or unilateral OME that lasts 3 months or longer with tympanic membrane or
middle ear damage
• Recurrent acute otitis media (AOM) with OME at the time of evaluation
Tympanostomy tube insertion is not recommended for children with OME that lasts fewer than 3
months or for recurrent AOM without the presence of OME at the time of evaluation. Children
with prolonged OME who do not have conductive hearing loss or other symptorns should be
monitored every 3 months until their OME resolves, or until they develop an indication for
tympanostomy tube insertion.
PREP Pearls
• Tympanostomy tubes are indicated for children with otitis media with effusion that lasts
longer than 3 months and is accompanied by hearing loss, damage to the middle ear or
tympanic membrane, or risk factors for speech, language or learning delays.
• Children with prolonged otitis media with effusion who do not have conductive hearing
loss or other symptoms should be monitored every 3 months until their otitis media with
effusion resolves, or until they develop an indication for tympanostomy tube insertion.

• Understand the indications for tympanostomy tube insertion
Suggested Readings
• Otitis Media: To Treat, To Refer, To Do Nothing: A Review for the Practitioner. Pediatr
Rev. doi: 10.1542/pir.36-11-480
• Clinical practice guideline: Tympanostomy tubes in children. Otolaryngology Head Neck
Surg. doi:10.1177/0194599813487302
• Clinical practice guideline: Otitis media with effusion (update). Otolaryngology Head
Neck Surg. doi:10.1177/0194599815623467.
• Otitis media and otitis externa Management of otitis media with effusion In: American
Academy of Pediatrics Textbook of Pediatric Care 2nd ed. 2017:2455.

Question 116
A 13-year-old adolescent girl is being seen for behavioral concerns. Her parents report that for
the past 2 months, their daughter has been spending most of her time in her room, sleeping or
playing video games. She has to be reminded to come out for meals and eats little when she does.
She is not interested in seeing her friends or joining in family activities. The adolescent states
that she has been having a hard time at school. She has not been getting along with her
classmates, and her grades have been poor. She states that she cannot concentrate on her studies
and admits to crying often and feeling sad most days.
Of the following, the MOST appropriate treatment for this adolescent is

A. Bupropion
B. Escitalopram
C. Nortriptyline
D. Paroxetine

Correct Answer: B
Of the options listed, escitalopram is the most appropriate choice for treating depression in the
adolescent in the vignette. Escitalopram is a selective Serotonin reuptake inhibitor (SSRI), and
along with fluoxetine, is 1 of the only 2 medications approved by the United States Food and
Drug Administration (FDA) for treatment of depression in adolescents.
Major depressive disorder (MDD) is diagnosed when the patient has persistent sadness or
irritability and anhedonia (lack of enjoyment) for a minimum of 2 weeks resulting in impairment
in functioning. Major depressive disorder is accompanied by change in appetite or weight, sleep,
activity level, or concentration; feelings of worthlessness or guilt; fatigue or loss of energy; or
suicidal ideation. Depression occurs in about 12% of adolescents by age 17 years with an
approximately 2:1 ratio of females to males.
After confirming the diagnosis of depression and establishing the adolescent's safety, a treatment
plan should be developed with the patient and family. Evidence-based treatment for depression
consists of psychotherapy (e.g., cognitive behavioral therapy, interpersonal therapy), medication,
or both. Individual or group psychotherapy should be considered for treating depression of any
severity. Medication should be considered for moderate to severe depression and when there is
insufficient response to psychotherapy for mild depression. The combination of psychotherapy
and medication is typically more effective than either alone.
The FDA has approved 2 medications for treatment of depression in adolescents-fluoxetine and
escitalopram. Both of these medications are SSRIs, the primary medications used to treat
depression in pediatrics. Common adverse effects of SSRIs include headaches, gastrointestinal
upset, and sleep disturbance. Selective serotonin reuptake inhibitors can also cause behavioral
activation (e.g., disinhibition, restlessness, impulsive behaviors) or mood disturbance and can
possibly activate mania in those at risk for bipolar disorder. Because of concern about a possible
increase in suicidal ideation or behaviors, the FDA issued a black box warning in 2004. Close
monitoring for these potential adverse effects is prudent and immediate evaluation should occur
for increased suicidality. Studies have demonstrated that as SSRI prescriptions decrease, rates of
suicide increase.
Paroxetine is a SSRI but is not FDA approved for treating depression in adolescents. In addition
to the previously noted adverse effects of SSRIs, paroxetine has mild anticholinergic side effects
(e.g., nausea, constipation, dry mouth).
Compared with other SSRIs, discontinuation of paroxetine is more likely to be accompanied by

withdrawal symptoms (e.g., dizziness, sweating, nausea), which are also more likely to be
severe. Tricyclic medications such as nortriptyline are effective in adults but not in children.
They can potentially cause significant cardiac (e.g., arrhythmias) and central nervous system
(e.g., seizure) problems. Bupropion is a norepinephrine dopamine reuptake inhibitor FDA-
approved to treat depression but not yet sufficiently studied in children. Adverse effects of
bupropion include constipation, tremor, headache, and insomnia. Seizures can rarely occur.

PREP Pearls
• Escitalopram and fluoxetine are selective serotonin reuptake inhibitors, and are the only 2
medications approved by the United States Food and Drug Administration for treatment
of depression in adolescents.
• Major depressive disorder is diagnosed when the patient has persistent sadness or
irritability and anhedonia (lack of enjoyment) for a minimum of 2 weeks, resulting in
impairment in functioning.
• Selective serotonin reuptake inhibitors can cause behavioral activation (e.g.,
disinhibition, restlessness, impulsive behaviors) or mood disturbance and can possibly
activate mania in those at risk for bipolar disorder.
MOCA-Peds Objective
• Develop a management plan for a patient with an abnormal depression screening.

• Understand the risks associated with the use of various antidepressant drugs
Suggested Readings
• Depression. Zuckerman Parker Handbook of Developmental and Behavioral Pediatrics
for Primary Care. 4th ed. 2019:203-206.
• Guidelines for adolescent depression in primary care (GLAD-PC): Part II—treatment and
ongoing management. Pediatrics. doi:10.1542/peds.2017-4082
• Depression and suicide in children and adolescents. Pediatr Rev.Doi: 10.1542/pir.36-7-
299
• Depression, American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.; 2017;
1259-1266.

Question 117
A 6-month-old infant born at full term is seen for a health supervision visit. The infant was last
seen at 2 weeks of age and was subsequently lost to follow-up. His mother reports that he is
“spitting up a lot" and often “looks cross-eyed.” Over the past 3 days he has been very sleepy
and difficult to arouse. On physical examination, the infant is irritable. His head circumference is
47 (Item Q117A). His anterior fontanelle is wide and firm with splayed sutures. He has a
persistent downward gaze (Item Q117B), mild hypertonia in all 4 extremities, and diffuse
hyperreflexia. The remainder of his physical examination findings are unremarkable.
(Item Q117A) & (Item Q117B)
Of the following, the BEST next step in this infant's management is

A. frequent follow-up for serial head circumference measurements
B. outpatient head ultrasonography
C. outpatient pediatric neurosurgery evaluation
D. urgent neurosurgical consultation in the emergency department

Correct Answer: D
The infant in the vignette is exhibiting signs and symptoms of increased intracranial pressure
including emesis, irritability, lethargy, macrocephaly, splayed sutures with a firm fontanelle, and
sun-setting eyes. Urgent evaluation is required including neuroimaging and neurosurgical
consultation. This infant has progressive lethargy and requires expedited evaluation and
neurosurgical care. Outpatient consultation and evaluation would not be appropriate.
Pediatric hydrocephalus presents with signs and symptoms of increased intracranial pressure,
which vary by age. In neonates, apnea may be a presenting sign, along with increasing head
circumference, bulging fontanelle, and splayed sutures. Lethargy and irritability may be present.
Older infants may have vomiting, delayed closure of the fontanelle, and sun-setting eyes
(Parinaud syndrome); the increased intracranial pressure causes an upward gaze palsy resulting
in downward deviation of the eyes. Older children, after the sutures and fontanelles have closed,
will often have a more acute presentation due to the skull's diminished ability to accommodate
the building pressure. Signs and symptoms suggestive of increased intracranial pressure are
headache, lethargy, change in vision (e.g., blurry and/or dotted vision), papilledema, and bilateral
cranial nerve 6 palsies (with resulting inability to abduct the eyes).
Hydrocephalus develops when the balance of cerebrospinal fluid (CSF) production, flow, and
absorption is disrupted (Item C117). Normally, CSF is produced in the choroid plexus, which
lines the ventricles. It then flows through the ventricular system and out through the foramina of
Luschka and foramen of Magendie to circulate in the subarachnoid space cushioning the brain
and spinal cord. Cerebrospinal fluid absorption occurs at the arachnoid granulations and adjacent
to the superior sagittal sinus.
Item C117: Conditions Associated With CSF disruption
Disruption at any point in the system can result in hydrocephalus, which is broadly categorized
into 2 types: communicating and noncommunicating. Communicating hydrocephalus occurs
when abnormal CSF absorption results in a build-up of fluid throughout the system.
Noncommunicating (i.e., obstructive) hydrocephalus occurs when there is a blockage of flow at
any point in the system. Congenital hydrocephalus is often caused by cerebral aqueduct stenosis,
leading to dilation of the lateral and third ventricles; this may be noted on prenatal
ultrasonography. Other congenital etiologies include: intraventricular hemorrhage, Chiari
malformation, Dandy-Walker malformation, neural tube defects, and genetic conditions (e.g., X-
linked hydrocephalus). Acquired hydrocephalus can occur secondary to brain tumors,
intracranial cysts, hemorrhage, or infections.
Serial head circumference measurement is a crucial component of infant health supervision.

Crossing percentiles should warrant investigation. For the infant in the vignette, serial head
circumference measurements already demonstrate crossing percentiles. With the accompanying
clinical features, continued observation with serial measurements is not appropriate. Neuro-
imaging is required to evaluate ventricular size and for accompanying pathology. For infants
with open fontanelles head ultrasonography can provide a quick evaluation of ventricular size
without radiation exposure or need for sedation. Head computed tomography or brain magnetic
resonance imaging can be used in the acute or subacute setting.
Hydrocephalus is treated with CSF diversion either with a ventriculoperitoneal shunt or

endoscopic third ventriculostomy. Lifelong monitoring for efficacy and complications is
required. Ventriculoperitoneal shunt complications include obstruction, malfunction, and
infection. Serial monitoring of head circumference is a cornerstone of long-term care, along with
counseling of the patient and family on common signs and symptoms of shunt malfunction.
PREP Pearls
• Hydrocephalus presents with signs and symptoms of increased intracranial pressure
including irritability, lethargy, vomiting, headache, papilledema, sunsetting eyes, cranial
nerve 6 palsies, firm and bulging fontanelle and splayed sutures.
• Serial head circumference measurements help to both detect increased intracranial
pressure and monitor patients with hydrocephalus long term.
• In an acute symptomatic infant or child with hydrocephalus, urgent neuroimaging and
neurosurgical consultation are required.

• Understand the risk factors for hydrocephalus
• Recognize the clinical findings associated with shunt malfunction or infection in a patient
with hydrocephalus and manage appropriately
• Recognize the clinical findings associated with hydrocephalus
Suggested Readings
• Hydrocephalus. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.
2017:2162-2167.
• Pediatric hydrocephalus: Current state of diagnosis and treatment. Pediatr Rev.
doi:10.1542/pir.2015-0134

Question 118
A 15-year-old adolescent boy is seen for a routine health supervision visit. His father is
concerned about worsening behaviors. The adolescent seems more angry than his brother and his
peers. He becomes agitated easily; has gotten into physical altercation with other students at
school, often yells at his mother, and frequently his older brother, inflicting a bloody nose on
several occasions.
Of the following, the neurobiological change MOST likely associated with this adolescent’s
behaviors is a (n)
A. decreased serotonin level
B. decreased vasopressin level
C. increased γ-aminobutyric acid level
D. increased oxytocin level

Correct Answer: A
The boy in the vignette most likely has a decreased brain serotonin level. The adolescent is
displaying aggressive behaviors, defined as behaviors that harm or inflict pain upon others
Cultural norms define pathologic aggression versus normal variants. In general, pathologic
aggressive behavior is chronic and more severe frequent compared to behaviors displayed by the
perpetrator’s peers.
Aggressive behavior has multiple environmental and biological causes. Neuropeptide and
neurohormonal changes that contribute include decreased serotonin and oxytocin levels,
decreased γ-aminobutyric acid (GABA) receptor activation, and increased dopamine and
vasopressin levels. The direction of change for the response choices other than the opposite that
of their association with aggressive behavior. Understanding these neurobiological changes
allows for targeted therapies. For example, benzodiazepines activate the GABA receptor and
valproate slows the breakdown of GABA.
Environmental factors associated with aggressive behavior can begin in utero and include
exposure to cocaine, methamphetamine, alcohol, and tobacco. Throughout infancy and childhood
strong connections are important in behavior development. Unhealthy relationships and lack of
strong connections result in changes in brain physiology and biochemistry; and development of
poor coping strategies.
Environmental risk factors include socioeconomic status, decreased parental education, younger
maternal age, single-parent families, greater number of siblings, and family discord. The Kaiser
adverse childhood events study identified a relationship between diseases in adulthood and these
and similar stressors. This information is available on the Centers for Disease Control and
Prevention website
https://www.cdc.gov/violenceprevention/aces/about.html
Furthermore, parenting styles can both contribute to and be affected by aggressive behavior.
Inconsistent parenting with a reactive authoritative parenting style leads to increased aggression
in children. For example, a child is told they may not have candy. In response, the child has a
tantrum and the parent becomes frustrated and shouts. Ultimately the parent gives the child the
candy to stop the tantrum. The parent's response re-enforced the child's undesired behavior and
modeled additional undesired behavior (shouting). The child learns that a tantrum gets them what
they want and that shouting is acceptable behavior.
Rejection by peers can increase aggressive behaviors in older children, which leads to more
rejection by peers. Peer relationships that Increase the risk of aggressive behaviors include gang
involvement and having peers with antisocial or delinquent tendencies. Protective factors include
a strong sense of belonging and strong relationships. Enrolling children in extracurricular
activities to strengthen their connections may be helpful.
Children and adolescents with mental health disorders, especially those manic or bipolar
disorder, have an increased risk of aggression, especially when combined with substance use.
Other disorders associated with an increased frequency of aggressive behavior include
oppositional defiant disorder, conduct disorder, and attention-deficit/hyperactivity disorder.
Given the environmental contributions to aggression, it is not surprising that successful treatment
includes the child's family and community. Improving the child’s and family members' social
skills, frustration tolerance, and problem-solving skills is helpful. Effective programs involve
community organizations, such as schools, child protective services, and law enforcement.
Programs such as the strategic Home Intervention and Early Leadership Development (SHIELD)
use law enforcement to identify children exposed to violence in the home, appropriately respond
to violent behavior from these children as they become adolescents, and enroll these children in
the program leading to community support and protective factors to decrease future aggressive
behavior
(https://www.ncjrs.gov/pdffiles1/ojjdp/184579.pdf)
PREP Pearls
• Stress and lack of emotional connections in early childhood contribute to aggressive
behavior
• Changes in neurobiological factors in the brain contribute to aggressive behaviors. These
changes may include decreased serotonin, oxytocin, and γ-aminobutyric acid (GABA)
levels, and increased vasopressin and dopamine levels.
• Treatment of aggressive behavior should include the child (e.g., developing healthy
coping and problem-solving skills), the family (e.g., healthy parenting skills), and the
community (e.g., school involvement).

• Recognize the various environmental and biological contributors to the development and
maintenance of aggressive behaviors
• Plan the appropriate management of aggressive or intimidating (bullying) behavior in
patients of various ages. including those who are victims or such behavior
• Differentiate the findings associated with aggressive behavior from those of normal
variants
Suggested Readings
• Violence and aggressive behavior. Pediatr Rev. doi:10.1542/pir.2016-062
• Centers for Disease Control and Prevention. Violence prevention.
https://www.cdc.gov/voiolenceprevention/aces/about.html
• Behavior-problems during early childhood in children with prenatal methamphetamine
exposure. Pediatrics. Doi:10.1542/peds.2019-0270.
• Maladaptive aggression: with a focus on impulsive aggression in children and
adolescents. J Child Adolesc Psychophamacol. Doi:10.1089/cap.2019.0039
• Disruptive behavior and aggression. American Academy of Pediatrics Textbook of
Pediatric Care 2nd ed.; 2017:1282-1289.

Question 119
A previously healthy, 4-year-old girl is seen in the office for evaluation; this is her third visit in
the past 2 months. She was initially seen 2 months ago when she had a minor fall while playing
soccer. Radiography demonstrated a hairline fracture of the right ulna, for which the arm was
splinted for 3 weeks. One month ago, while riding her tricycle, she reported severe left knee
pain. There was no witnessed fall or injury. Radiography findings of the knee were normal. Due
to persistent pain, she was evaluated by an orthopedic surgeon, who obtained additional images
for a fracture. Her extremity was placed in a walking cast for 3 weeks after which she was seen
by a physical medicine and rehabilitation physician, who prescribed gabapentin and physical
therapy.
She brought to the office today with severe right knee pain and refusal to walk.
On physical examination, the girl is afebrile, heart rate is 124 beats/min, respiratory rate is 34
breaths/min, blood pressure is 115/83 mm Hg, and oxygen saturation is 97% on room air via
pulse oximetry. Her height is 99 cm and weight is 22 kg; her weight was 28 kg 2 months ago.
She is awake, alert, and in no apparent distress. Physical examination reveals bilateral clear lung
sounds, normal S1 and S2 sinus rhythm, and no palpable cervical, axillary, or inguinal
lymphadenopathy. Her abdomen is soft and nontender, with no hepatosplenomegaly or masses.
She has limited range of motion of her right knee, which is tender to palpation. The remainder of
her examination findings are normal.
Laboratory data are show

White blood cell counts 7,800/µL (7.8 x 109/L)
Mean corpuscular Volume 76.8 L
Neutrophils 17%
Lymphocytes 65%
Monocytes 7%
Other (blasts) % 11%
Calcium 13.8 mg/dL (3.5 mmol/L)
Uric acid 4.2 mg/dL (0.25 mmol/L)
(reference range, 3.4-8.8
mg/dL; 0.2-0.52 mmol/L

Of the following, the BEST next step this child's management is administration of
A. Calcitonin
B. oxycodone
C. prednisone
D. rasburicase

Correct Answer: B
The child in the vignette most likely has leukemia, as suggested by the finding of blasts on the
complete blood count. She is having pain with refusal to ambulate; therefore, management of her
pain is of utmost importance. She is currently otherwise clinically stable and can await further
interventions. Of the response choices, oxycodone is the only one that can offer pain relief.
Acute leukemia is the most common childhood cancer. It results from over proliferation of
immature white blood cells, known as blasts. The blasts crowd the bone marrow and can lead to
a decrease in production of normal blood cells, which can lead to pancytopenia. Children with
unexplained persistent fever, pain (e.g., back and extremities), pallor, fatigue, petechiae, or
bruising should be evaluated for leukemia. Children with pathologic fractures may also merit
investigation for leukemia.
Evaluation of a child with suspected leukemia should include a complete blood count with
peripheral smear review, complete metabolic panel, magnesium level phosphorus level, bone
marrow aspirate and biopsy, and flow cytometry to identify white blood cell surface markers in
the peripheral blood or bone marrow. Lumbar puncture should be performed and the
cerebrospinal fluid examined for leukemic cells. For boys, the testes should be evaluated for any
painless mass at the time of diagnosis. The type of leukemia is defined by the predominant type
of immature white blood cell. The 2 main forms are acute lymphoblastic leukemia (ALL) and
acute myelogenous leukemia, both of which have sub-types. It is important to appropriately
diagnose the type and risk category of leukemia in order to determine the best curative treatment.
Due to collaborative research efforts and care models, childhood leukemia now has a high cure
rate (Item C119A). For patients with ALL, children age >1 year and <10 years with a presenting
white blood cell count <50,000/µL (50 x 109/L) are considered standard risk and have a very
good prognosis. Item C119B presents the risk stratification for childhood ALL.
Item C119A: childhood leukemia survival rate

Item C119B: ALL risk stratification
Once a specific diagnosis of leukemia is made, treatment with appropriate combination

chemotherapy is initiated. These medications can have immediate and long-term adverse effects,
which must be discussed with the family prior to treatment initiation. Treatment also includes
hydration, pain control, allopurinol or rasburicase (treatment for hyperuricemia from tumor
lysis), antiemetics, and blood products (i.e., red blood cell or platelet transfusions). Intrathecal
chemotherapy is given, even if blasts are not visualized in the cerebrospinal fluid. In order to
prevent relapse in that site. The testes are also a site for potential relapse and should be examined
routinely for any painless mass. Boys with ALL are usually treated for longer than girls because
of this risk.
Leukemia mainly occurs by random chance. It is important that parents understand this to avoid
feelings of responsibility and guilt. However, some syndromes have a cancer predisposition
which may lead to a higher risk developing leukemia (e.g., trisomy 21, Klinefelter syndrome,
Fanconi anemia, and Bloom syndrome)
Calcitonin is not required at this time to treat this child's hypercalcemia. She does not have any
acute signs or symptoms of hypercalcemia and her elevated calcium level may respond to
volume expansion alone.
Prednisone is a standard medication used in the treatment of ALL. Generally, it should be

administered as part of a treatment protocol that usually begins after the diagnosis is confirmed.
Occasionally, if there is an oncologic emergency such as a mediastinal mass causing respiratory
compromise, steroids may be prescribed by the oncologist prior to initiating the full treatment
protocol. Administration of prednisone prior to obtaining a baseline white blood cell count in a
child suspected to having leukemia could increase the risk stratification.

Rasburicase is an enzyme used to treat severe hyperuricemia in the setting of acute leukemia.
Rarely, rasburicase may cause red cell hemolysis in an individual with glucose-6-phosphate
dehydrogenase deficiency, The uric acid level is not elevated in the child in the vignette;
therefore, she can receive allopurinol instead.
PREP Pearls
• Children with unexplained persistent fever, severe persistent pain (often of the back or
extremities), pallor. Fatigue, petechiae, or bruising, should be evaluated for leukemia.
• Pathologic fractures may warrant investigation for leukemia.
• Children with trisomy 21, Klinefelter syndrome, Fanconi anemia, and Bloom syndrome
have an increased risk of developing leukemia.
MOCA-Peds Objective
• Recognize the Signs and symptoms of cancers involving the bone.

• Understand that management of leukemia is dependent on its type
• Understand the outcome associated with treated acute lymphoblastic leukemia
• Identify disorders associated with an increased risk of leukemia
• Recognize clinical findings associated with leukemia, including sites of relapse
Suggested Readings
• Childhood leukemia. Pediatr Rev. doi:10.1542/pir.31-6-234
• Leukemia in children. Pediatr Rev. doi:10.1542/pir.2018-0192
• Leukemias. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed. 2253-
2269

Question 120
The mother of a 14-year-old adolescent boy is worried about her son's recent behavior. After
school he and 2 friends were recently seen vaping in a garage. She is disappointed and worried
he may be susceptible to peer pressure. The boy and his mother live with his maternal
grandmother in a low socioeconomic neighborhood. There is no household exposure to tobacco
products. He attends the local middle school where he has a good attendance and conduct record.
He gets good grades and enjoys reading and playing video games. He has no chronic health
conditions. Although his puberty was slower to start than for many of his friends. He is now
experiencing a “growth spurt."
Of the following, the factor that BEST explains this boy's recent risky behavior is
A. lack of ability to understand consequences

B. late onset of puberty
C. living in a lower socioeconomic neighborhood
D. the pursuit of pleasure or excitement

Correct Answer: D
Risk taking may be associated with positive adolescent development by promoting independence
and autonomy. However, risk taking that is associated with harm to the adolescent and/or others
is termed “risky behavior” and accounts for much of the morbidity and mortality associated with
the adolescent years. In general, adolescents have a decreased ability to anticipate danger and a
decreased physiologic response to fear. Contextual factors such as living in a low socioeconomic
neighborhood, especially a neighborhood with few opportunities and services, may be associated
with an increased likelihood risky behavior. However, the driving factor for adolescent risk
taking is the pursuit of excitement and pleasure.
Brain development continues during adolescence with changes in brain structure and function
that extends into young adult years. A decrease in gray matter volume is associated with
remodeling (pruning) of neuroconnections. The prelimbic and limbic area of the brain that is
responsible for emotions and striving for pleasure is activated in early puberty. The prefrontal
associated with executive functioning, cognitive control, self-awareness, and self-regulation is
the last area of the brain to mature. This “maturation gap” in brain development fosters a
susceptibility in adolescents to the lure or appeal of pleasure and excitement (reward-related
behavior) that facilitates risk taking.
Adolescents, particularly younger adolescents, may be concrete thinkers with limited capacity to
think abstractly. They can assess risks versus benefits and understand consequences. However,
lack of knowledge, especially during initiation into the use of novel or rediscovered drugs, sexual
activity, and fads (e.g., cinnamon and laundry pod challenges) can be a factor for adolescent
risky behavior. Knowledge of dangers may not be enough to deter risky behavior. Despite being
aware of potential harms and capable of rational decisions, adolescent behavior, nevertheless,
may reflect the “feel good” choice. The pursuit of thrills and pleasure weighs heavily in their
decision-making. Without the balancing benefit of a mature prefrontal cortex, adolescents' ability
to self-regulate is challenged and the propensity for risky behaviors is increased, most notably
during emotional situations.
Contextual factors modulate the adolescent’s vulnerability for reward-related behavior. Peer
pressure has long been observed to be a very influential factor in risk taking. Adolescents seek
the pleasure of peer approval and avoid the distress of social exclusion/rejection by peers. The
actual presence of a group of adolescents is an additional independent factor for risk taking and
increases the likelihood of risky behaviors. Other contextual factors that increase risky behaviors
include individual factors (e.g., attention-deficit disorders, mental health problems), authoritarian
or permissive parenting, and family and community norms that condone or tolerate risky
behaviors. However, having positive role models, living in communities with opportunities and
social services, and school connectedness are examples of protective factors. Adolescents who
exhibit 1 risky behavior are at risk for other risky behaviors. Early onset of puberty rather than a
late onset has been associated with risky behaviors (e.g., risky sexual behaviors and
delinquency).

PREP Pearls
• The pursuit of thrills and pleasure has a substantial influence on adolescent decision-
making and risk taking
• The maturation of the prefrontal cortex associated with self-regulation extends into early
adult years.
• Risky behaviors among adolescents increase in the presence of peers.

• Recognize factors associated with risk-taking in adolescents
•
Suggested Readings
• Cornell University Bronfenbrenner Center for Translational Research-ACT for Youth
Adolescent development toolkit: Risk taking.
https://actforyouth.net/adolescence/toolkit/risk.cfm
• Adolescent decision-making and risk taking, Child Psychology: A Handbook of
Contemporary issues. 3rd ed. 2016;263-284.
• The teen brain. In: Ginsburg KR ed. Reaching Teens. 2nd ed. 2020:107-112.

Question 121
As part of a quality improvement initiative, an interprofessional team from the Department or
Pediatrics identified a series of medication-prescribing errors isolated to a single group of
hospital-based physicians. The improvement team wants to engage the hospital medicine group
in their effort to reduce the frequency of medication errors and improve the culture of safety in
the organization. The desired outcome is a reduced number of prescribing errors that result in
patient harm for each individual pediatrician in the group.
Of the following, the course of action MOST likely to achieve this goal is to
A. make contract renewal dependent on individual physician performance
B. mandate quarterly medication safety educational sessions
C. provide financial bonus incentives to the physicians
D. provide quarterly data and performance reminders to individual physicians

Correct Answer: D
The course of action most likely to improve the culture of safety and reduce ham from the
medication errors described in the vignette is to provide quarterly data and performance
reminders to individual physicians. A culture of safety requires an organization to have shared
values and attitudes centered on a commitment to continuous efforts to minimize harm. It is thus
important that all members of the health care team become engaged in initiatives that target
improved patient safety and reduced harm, and assume a shared responsibility for improving the
culture of safety.
The most compelling and successful method of engaging clinicians in quality improvement
endeavors is to provide them with specific data and frequent reminders to promote process
change. Financial incentives may temporarily encourage participation or change, but generally
do not lead to long-standing behavior change. Educational endeavors, whether voluntary or
mandatory, are the least effective strategies in changing behavior. Ultimatums regarding
employment status are unlikely to improve the culture of safety and are not part of a positive
safety culture because they violate the nature of a “just culture.”
In the simplest of terms, a culture of safety unifies an organization’s attitudes and behaviors and
encourages all aspects of the health care system to focus on reducing harm. A number of specific
characteristics of organizational culture contribute to and promote a safety culture, these
practices and policies can affect clinician and staff behavior. The Joint Commission and the
Institute for Healthcare Improvement have identified a number of key attributes required to
promote a culture of safety including;
• Transparency. accountability, and mutual respect
• Making safety the first priority for all clinicians and caregivers
• Identifying activities that undermine a culture of safety as unacceptable
• Encouraging staff to identify hazardous conditions and near-miss situations before a
patient is harmed
• Encouraging error reporting and addressing system failures that contribute to patient
safety events
• Creating a blame-free environment where the focus of improvement is on the system and
not the individuals involved
All members of the health care team have a role in creating a culture of safety. Physicians play
an important role in the planning and development of safe systems of care because of their direct
patient interaction. Hospital-based endeavors that do not engage all members of the health care
team are certain to fail to achieve their optimal end point. To improve patient safety and the
safety culture of an institution leaders need to appraise their organization's current safety culture
and promote evidence-based strategies for implementing quality improvement initiatives.
Medical errors are a major problem in hospitalized patient, often leading to unintended patient
harm. There are significant costs beyond the direct harm to patients, including financial costs to
the health care system, patient, and family members, as well as personal and emotional costs to
patients, their families, and the caregivers who may have been involved. Medication errors are
the most frequent reported medical errors. Medication errors present a unique and significant
problem for children because of formulary constraints, liquid medication dosing, lack of unit
dosing, compounding, and lack of patient participation in the medication administration process.
To reduce medical errors, a culture of safety is required to ensure that all, including those that do
not result in harm, are identified and that appropriate system-based mitigation strategies are put
in place to prevent latent errors from becoming realized.
PREP Pearls
• The most compelling and successful method of engaging clinicians in quality
improvement endeavors is to regularly provide them with specific data and frequent
reminders to promote process change.
• A culture of safety unifies an organization's attitudes and behaviors and encourages the
entire health care system to focus on reducing harm
• Medication errors are the most frequently reported medical errors; medication errors
present a unique and significant problem for children.
MOCA-Peds Objective
• Recognize the factors that relate to risk of error or reduced patient safety.

• Promote effective team functioning in the prevention of medical error
• Understand the importance of assessment and redesign of health-care processes before
error occurs
• Understand the importance of leadership in creating a culture of safety in the health-care
system
• Apply knowledge of human factors in the design of systems and processes promoting
patient safety
Suggested Readings
• Core principles of quality improvement and patient safety. Pediatr Rev.
doi:10.1542/pir.2015-0091
• A comprehensive patient safety program can significantly reduce preventable harm,
associated costs, and hospital mortality. J Pediatr. Doi:10.1016/j.jpeds.2013.06.031
• Council on Quality Improvement and Patient Safety, Committee on Hospital Care.
Principles or pediatric patient safety: reducing harm due to medical care. Pediatrics. doi:
10.1542/peds.2018-3649.
• Minimizing medication errors in children. Arch Dis Child.
Doi:10.1136/adc.2007.116442.

Question 122
6-year-old girl is seen in the office for evaluation of a rash on her trunk. On physical
examination, she is well appearing with age-appropriate vital signs. Her skin examination reveals
an erythematous, welt-demarcated plaque with an overlying silvery scale (Item Q122).
Of the following, children with this condition are at an INCREASED risk of developing
A. growth failure
B. hearing loss
C. infertility
D. metabolic syndrome

Correct Answer: D
The girl in the vignette has psoriasis, a systemic, immune-mediated condition typically
characterized by well-demarcated, erythematous plaques with adherent silvery-white scale.
Patients with psoriasis are at increased risk of developing metabolic syndrome. They are not at
increased risk for growth failure, hearing loss, or infertility.
Common areas of involvement in children with psoriasis include the face, scalp, intertriginous
skin, and the diaper area. Psoriasis of the scalp can sometimes be difficult to distinguish from
seborrheic dermatitis. Whereas seborrheic dermatitis typically remains within the hairline,
psoriasis often extends to the upper neck, pre or postauricular skin, or the forehead. The most
common subtypes of psoriasis in pediatric patients are chronic plaque psoriasis (erythematous
plaques with overlying scale) and guttate psoriasis (acute eruption of small, round, erythematous
'drop-like' scaly papules, Item C122).
Item C122: guttate psoriasis
Psoriasis is associated with an increased risk for several comorbidities, including obesity,
metabolic syndrome, arthritis, psychiatric disorders including anxiety and depression, and less
commonly, Crohn disease, uveitis, and rheumatoid arthritis. Importantly, the most strongly
associated comorbidity is obesity. Although psoriasis is a small independent risk factor for the
hyperlipidemia, hypercholesterolemia, hypertension, and insulin resistance seen in metabolic
syndrome, obesity is a much stronger contributor.
Treatment of mild to moderate psoriasis (affecting of the body surface area) can usually be
topical, with corticosteroids, calcineurin inhibitors, vitamin D analogs, or a combination of these
agents. Moderate to severe psoriasis (affecting of the body surface area or not responsive to
topical treatments) can be treated with systemic therapy with methotrexate or biologic agents or
with narrowband ultraviolet B phototherapy.
PREP Pearls
• Psoriasis is associated with an increased risk for obesity, metabolic syndrome, arthritis,
psychiatric disorders including anxiety and depression, and less commonly, Crohn
disease, uveitis, and rheumatoid arthritis.
• The most common subtypes of psoriasis in children are chronic plaque psoriasis
(erythematous plaques with overlying silvery-white scale) and guttate psoriasis (acute
eruption of small, round, erythematous 'drop-like' scaly papules).
• Evaluate and manage a child with a disorder of the scalp.

• Recognize the clinical findings associated with psoriasis
Suggested Readings
• Joint American Academy of Dermatology-National Psoriasis Foundation guidelines of
care for the management and treatment of psoriasis in pediatric patients. J Am Acad
Dermatol. doi: 10.1016/j.jaad.2019.08.049.
• Psoriasis. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.;
2017:2543-2551.
• Association of psoriasis with comorbidity development in children with psoriasis. JAMA
Dermatol. Doi:10.1001/jamadermatol.2017.5417

Question 123
A 1-day-old mate infant born to a 21-year-old mother with chronic hepatitis C virus infection is
being evaluated in the newborn nursery. The neonate was born at term via spontaneous vaginal
delivery. His mother used injection drugs during pregnancy and did not receive prenatal care.
Maternal rapid plasma reagin and human immunodeficiency virus antigen-antibody tests are
nonreactive. The neonate's physical examination findings are normal.
Of the following, the BEST hepatitis C virus screening test to perform in this neonate's
evaluation is
A. antibody at 12 months of age
B. antibody at 18 months of age
C. polymerase chain reaction at 10 days of age
D. polymerase chain reaction at 2 weeks of age

Correct Answer: B
The best next screening test to assess this neonate's perinatal exposure to hepatitis C virus (HCV)
infection is HCV antibody at 18 months of age. Hepatitis C virus is a single-stranded, plus-sense
ribonucleic acid (RNA) virus from the Flaviviridae family. The diagnosis of HCV infection can
be made with serologic testing (i.e., presence of anti-HCV immunoglobulin G antibody) or real-
time RNA polymerase chain reaction (PCR) assay. Current American Academy of Pediatrics
guidelines recommend serologic testing of children with perinatal exposure to HCV at 18 months
of age because the risk of perinatal transmission is low (5% to 6%), asymptomatic infection can
persist for years, and currently no antiviral agents are available for HCV-infected children
younger than 2 years. Serologic diagnosis before age 18 months is problematic because of the
persistence of maternal anti-HCV antibodies passively transferred across the placenta. Although
RNA PCR can detect HCV in the early months of age. The viremia may be transient and clear
spontaneously.
Approximately 20% of perinatally HCV-infected cases resolve by early childhood, False-

positive and false-negative PCR results may also be seen. Testing for HCV using RNA PCR
assay at age 1 to 2 months is an option for infants when parents request earlier testing or if there
are concerns about follow-up at age 18 months.
Detection of anti-HCV IgG antibody in children aged 18 months warrants further evaluation with
quantitative HCV RNA PCR testing. Chronic HCV infection is defined by the persistence of
HCV RNA in blood for a minimum of 6 months. Consultation with a pediatric infectious disease
or gastroenterology specialist is recommended for children diagnosed with chronic HCV
infection. Children with perinatally acquired HCV infection are usually asymptomatic for several
years and have slow disease progression. Chronic disease occurs in about 30% of these children.
Severe liver disease rarely occurs during childhood, Children with other chronic conditions (i.e.,
HIV, thalassemia syndromes) are at increased risk for disease progression.
Hepatitis C virus is a bloodborne infection that remains a major public health problem in the
United States; an estimated 3.5 million individuals live with chronic HCV infection. The
incidence of acute HCV infection increased significantly in the past decade from an estimated
11,800 cases in 2010 to 44,700 in 2017. Young adults between 20 and 39 years of age, especially
in rural areas with associated injection drug use and exposure to opioids, have experienced the
highest rates of HCV infection. Transmission occurs through contact with contaminated blood or
body fluids in the setting of injection drug use, blood transfusion, transplantation (before 1992),
and perinatally.
Because stringent blood product screening has virtually eliminated transfusion associated
hepatitis in the United States, perinatal transmission of HCV has emerged as the most common
mode of pediatric HCV infection. In recent years, rates of HCV infection among women of
childbearing age have significantly increased. In the United States, the prevalence of HCV
infection among pregnant women is estimated to be 1% to 2.5%. The risk of perinatal
transmission of HCV is estimated at 5% to Approximately 40,000 infants are born each year to
mothers with chronic HCV, leading to approximately 2,700 to 4,000 new perinatal HCV
infections. In April 2020, the Centers for Disease Control and Prevention issued new guidance

recommending HCV screening for all pregnant women during each pregnancy, except in areas
with HCV prevalence below 0.1 %.
The precise timing of mother-to-infant transmission of HCV is unclear but likely occurs during
intrauterine of intrapartum periods, similar to perinatal HIV infection. Risk factors for perinatal
transmission of HCV, include high levels of maternal viremia, prolonged rupture of membranes,
lacerations of the vagina, and use of internal fetal monitoring. The mode of delivery and
breastfeeding do not affect the risk of transmission. However, in the presence of cracked or
bleeding nipples, infected mothers should discontinue breastfeeding until the nipples have
healed.
No antiviral therapy is currently available for young children with chronic HCV infection, but
ongoing clinical trials offer promise for pediatric curative therapy similar to that for adults. Oral
direct-acting antiviral therapy is available for children aged 12 years and older with chronic HCV
infection.
PREP Pearls
• Perinatal transmission is the most common cause of pediatric hepatitis C virus infection.
• American Academy of Pediatrics guidelines recommend serologic testing for infants with
perinatal exposure to hepatitis C virus at 18 months of age.
• Infants with perinatal hepatitis C virus infection are usually asymptomatic for several
years with slow disease progression.

• Understand the importance of follow-up screening evaluations for complications of
hepatitis C virus infection
• Understand the possible long-term outcomes of patients who have hepatitis C virus
infections
Suggested Readings
• Hepatitis. Red Book: 2021-2024 Report of the Committee on Infectious Diseases. 32nd
ed 399-403.
• Hepatitis A, B, and C. Pediatr Rev. doi:10.1542/pir.2015-0075
• Hepatitis A, American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.; 2017:
2131-2143.
• Hepatitis C testing among perinatally exposed infants. Pediatrics. doi:10.1542/peds.2019-
2482
• North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition,
NASPGHAN practice guidelines: Diagnosis and management of hepatitis C infection in
infants, children, and adolescents. J Pediatr Gastroenterol Nutr. doi:
10.1097/MPG.0b013e318258328d.

Question 124
An otherwise healthy, 8-year-old girl is seen in the clinic for a second episode of cola-colored
urine, which occurred this morning. She reports having a few episodes of pain with exercise and
stiffening or muscles during her school gym class over the past year. Each time, the pain
improved after a brief period of rest and hydration, and she was able to continue her gym class.
The first episode of cola-colored urine occurred 1 month ago after a long, hot day spent at the
county fair. Her urine color normalized and the pain subsided after drinking 3 bottles of apple
juice and resting. She recently joined a soccer team; last night after practice she complained of
severe pain in her legs and arms. On physical examination she has tenderness in her calf and
thigh muscles. Vitals signs are normal for age. Her family history is noncontributory.

Creatine kinase 20,000 U/L
Urine ketenes Negative
Urine myoglobin 3+
Of the following, the MOST likely etiology of this girl's condition is

A. glycogen storage disease I
B. glycogen storage disease II
C. glycogen storage disease IV
D. glycogen storage disease V

Correct Answer: D
The girl in the vignette has glycogen storage disease (GSD) type V, also known as McArdle
disease. Glycogen storage disease V is the most common disorder of muscle glycogenosis. The
primary clinical facture is exercise intolerance, presenting with fatigue, pain, and cramps in the
exercising muscle. Moderate exercise is usually fairly well tolerated. Symptoms are increased
during sustained aerobic (e.g., stair climbing, jogging) of isometric exercise (e.g., carrying
weights). Onset usually occurs in childhood, but recognition is frequently delayed, as pain and
fatigue can be overlooked.
The majority of children with GSD V have minimal to no symptoms and no limitations of their
day-to-day activities. Approximately 50% of affected individuals have recurrent episodes of
myoglobinuria and 25% have fixed muscle weakness. Episodes of rhabdomyolysis can be
triggered by high-intensity dynamic exercises (e.g., soccer). Myoglobinuria places the child at
risk of renal failure; adequate hydration is critical in these situations, and children at risk should
be closely monitored. Rarely, affected children can have difficulty with mastication, dysphagia,
and oral motor function. A classic feature of GSD V is the second-wind phenomenon, where test
after a brief period of exercise-induced myalgia can improve exercise tolerance. This increased
tolerance after rest is due to the muscles' ability to metabolize free glucose and fatty acids in the
bloodstream as exercise progresses. Some middle-aged adults report worsening of their
symptoms accompanied by muscle wasting.
Glycogen storage disease V is inherited in an autosomal recessive manner. Diagnosis is made by

genetic analysis with identification of 2 disease-causing changes in the PYGM gene.
Management of children with GSD V includes:

• Obtaining a baseline creatine kinase level
• Physical examination with assessment of muscle strength/weakness
• Simple healthy lifestyle interventions
• A diet rich in complex carbohydrates
• Maintenance of good hydration
• Exercise
• Avoid: handgrip, weightlifting, dynamic (e.g., ball games), lengthening muscle
contractions (e.g., jumps), intense dynamic aerobic (e.g., running, strenuous
swimming)
• Encourage: daily moderate-intensity aerobic training (e.g., walking, bicycling) to
increase cardiorespiratory fitness: glucose-containing fluids prior to exercise to
avoid rhabdomyolysis.
• Avoidance of general anesthetics-muscle damage may occur with the use of muscle
relaxants or inhaled anesthetics
Glycogen storage disease I presents in the first few months of life with failure to thrive, seizures,
developmental delay, and severe hypoglycemic episodes. Clinical features in an untreated patient
can include hepatomegaly, lactic acidosis, hyperuricemia, hyperlipidemia, hypertriglyceridemia,
and sometimes seizures from hypoglycemia.

Glycogen storage disease II, also known as Pompe disease, is a glycogen and lysosomal storage
disorder classified as either infantile-onset (IOPD) or late-onset (LOPD), Clinical features of
IOPD include marked hypotonia, failure to thrive, and hypertrophic cardiomyopathy within the
first year of life. Clinical features of LOPD include proximal muscle weakness with respiratory
insufficiency; there is no cardiac involvement.
Glycogen storage disease IV includes several subtypes affecting liver, muscle, and other tissues
to varying extents. Subtypes include: classic progressive hepatic, nonprogressive hepatic, the rare
childhood neuromuscular form, as well as lethal forms (perinatal and congenital neuromuscular
subtypes).
The presenting features of the child in the vignette are not consistent with GSD I, II or IV.
PREP Pearls
• Glycogen storage disease type V Is the most common disorder of muscle glycogenosis.
• A classic feature of glycogen storage disease type V is the second-wind phenomenon,
where rest after a brief period of exercise-induced myalgia can improve exercise
tolerance.
• Encouragement of good hydration with glucose-containing fluids prior to exercise can
help prevent rhabdomyolysis.

• Recognize the clinical features associated with glycogen storage disease
• Plan the appropriate immediate and long-term management of glycogen storage disease,
while considering the long-term prognosis
Suggested Readings
• Glycogen storage disease type V, Gene Reviews. University of Washington, Seattle,
2019.
• Inborn errors of metabolism (metabolic disorders). Pediatr Rev. doi: 10.1542/pir.2014-
0122.
• Specific congenital metabolic diseases. American Academy of Pediatrics Textbook of

Question 125
A 2-year-old boy is seen for a health supervision visit. He has profound sensorineural hearing
loss and will be receiving a cochlear implant in a few month. He is up to date on the routine
vaccination schedule.
Of the following, the MOST appropriate vaccine to administer today is

A. Hemophilus influenza type b
B. meningococcal serogroup B
C. pneumococcal conjugate
D. pneumococcal polysaccharide

Correct Answer: D
The boy in the vignette should receive the pneumococcal polysaccharide vaccine because a
cochlear implant places him at increased risk for meningitis caused by Streptococcus
pneumoniae. Compared with other children, those with cochlear implants are not at increased
risk of meningitis due to Neisseria meningitidis, so he does not need to receive this vaccine
outside of the routine schedule. He does not need additional doses of pneumococcal conjugate or
the Hemophilus influenzae type B vaccines because he has completed the routine series.
Streptococcus pneumoniae is a gram-positive, catalase-negative diplococcus organism with more

than 90 serotypes. Serotype classification is based on the particular polysaccharide that
encapsulates the organism. S pneumoniae can cause infections such as otitis media, pneumonia,
meningitis, endocarditis, osteomyelitis, and septicemia. Routine vaccination has significantly
reduced the rates of colonization and infection with S pneumoniae.
Two pneumococcal vaccinations are available in the United States: a conjugated vaccine and an
unconjugated polysaccharide vaccine. The conjugated vaccine contains purified S pneumoniae
capsular polysaccharide conjugated to a diphtheria toxin protein variant. Conjugation of the
polysaccharide antigen to the protein carrier improves the antibody response to the vaccine.
Pneumococcal conjugate vaccine 13 (PCV13) protects against serotypes 1, 3, 4, 5, 6B, 7F, 9V,
14, 19A, 19F and 23F. The minimum age at which PCV13 can be given is 6 weeks; it should be
administered to all children as a 4-dose series at 2, 4, 6, and 12 to 15 months of age. Premature
infants should receive PCV13 according to their chronologic ages as long as they are medically
stable.
The purified polysaccharide vaccine (PPSV23) protects against the 23 serotypes 1, 2, 3, 4, 5, 6B,
7F, 8, 9N, 9V, 10A, 11A, 12F, 14, 15B, 17F, 18C, 19A, 19F, 20, 22F, 23F, and 33F. The
minimum age at which PPSV23 can be given is 2 years because it does not elicit adequate
immunogenicity in children younger than 2 years. The PPSV23 and PCV13 vaccines should not
be administered at the same time. The PPSV23 vaccine should be given at least 8 weeks after
any previous PCV13 dose, because antibody concentrations are greater when given in this order.
The PPSV23 vaccine is indicated for children with the high-risk conditions listed in Item C125.
For some conditions, 2 doses are recommended, which should be administered at least 5 years
apart. In the event that the second dose is administered sooner, no additional doses need to be
given; administration of more than 2 doses of PPSV23 is not recommended because of antibody
hypo responsiveness.
Children who will undergo planned solid organ transplantation splenectomy, cochlear
implantation, or administration of immunocompromising therapy should complete their
pneumococcal vaccination series at least 2 weeks before the procedure, if possible.
Catch-up guidance for incompletely immunized children is published by the Centers for Disease
Control and Prevention annually. After 59 months of age, healthy children who have not been
completely vaccinated with PCV13 do not need additional doses.

Pneumococcal vaccines should not be given at the same time as the quadrivalent meningococcal
conjugate vaccine MenACWY-D because of interference with immunogenicity to the
pneumococcal vaccine. Instead, they should be administered 4 weeks apart, or the MenACWY-
CRM vaccine should be given.
Contraindications to administration of pneumococcal vaccines include severe allergic reaction to

any vaccine component or a current moderate or severe illness.
PREP Pearls
• Children 2 years of age and older scheduled to receive cochlear implants should receive a
single dose of pneumococcal polysaccharide vaccine (PPSV23), ideally at least 2 weeks
before implantation.
• The pneumococcal polysaccharide vaccine is indicated in children with high risk
conditions; it should be administered at least 8 weeks after any previous pneumococcal
conjugate (PCV13) vaccine.
• Pneumococcal vaccines should not be given at the same time as the quadrivalent
meningococcal conjugate vaccine MenACWY-D because of interference with
immunogenicity to the pneumococcal vaccine.

• Understand the composition of conjugated and unconjugated pneumococcal vaccines are
multivalent
• Differentiate between appropriate use of conjugated versus unconjugated pneumococcal
vaccine
• Know the indications and schedule for the pneumococcal vaccines
Suggested Readings
• Streptococcus pneumoniae pneumococcal infections. Red Book: 2021-2024 Report of the
Committee on Infectious Diseases. 32nd ed :717-726.
• Centers for Disease Control and Prevention. Vaccines and immunizations.
https://www.cdc.qov/vaccines/
• Pneumococcal disease, Epidemiology and Prevention of Vaccine-Preventable Diseases.
13th ed 2015.

Question 126
An 11-year-old girl is brought to the office after falling off her skateboard. She was wearing a
helmet at the time of the fall. She did not lose consciousness or hit her head. The girl has been
acting appropriately since the fall. She has a large abrasion on her leg with debris in it, and her
parents are concerned about the wound developing an infection. On physical examination, she
has a 3 x 3-cm, deep abrasion of her left lower anterior leg with a small amount of visible dirt
and rocks embedded in the tissue. There is no active bleeding from the wound. The remainder of
her physical examination findings are normal. Review of her medical record reveals that she
received all of her childhood immunizations as recommended, and her last tetanus immunization
was 2 years ago. She has no known drug allergies.
Of the following, the MOST appropriate next step in this girl's management is
A. a 7-day course of oral clindamycin
B. analgesia, visible foreign body removal, and wound irrigation
C. administration of tetanus vaccine
D. wound irrigation and application of vitamin E ointment

Correct Answer: B
The child in the vignette has a deep soft tissue abrasion. The most important next step in this
child’s management is to remove any visible debris and thoroughly clean the wound by
irrigation, while ensuring that adequate analgesia is provided. Removal of foreign body particles
from the wound is essential to prevent wound infection and permanent pigmentation (e.g.,
traumatic tattooing).
Prophylactic antibiotics have not been shown to decrease the rate of wound infection in this type
of scenario and are not routinely recommended. Prophylactic antibiotics may be considered for
children with grossly contaminated wounds, bite wounds, or an underlying immunodeficiency.
The antibiotic of choice for children who meet criteria for wound infection prophylaxis is
cephalexin or, in the case of animal or human bites, amoxicillin-clavulanate (for Pasteurella
multocida coverage). Clindamycin would not be an appropriate first-line drug for wound
infection prophylaxis for the child in this vignette. Vitamin E preparations should not be used on
wounds in children due to an unacceptable rate of hypersensitivity reactions. Tetanus
immunization status should be assessed in all children with soft tissue injuries and wounds. For
children with contaminated, puncture, animal bite, crushing, avulsion, burn, or frost bite wounds,
tetanus immunization should be administered if it has been greater than 5 years since their last
tetanus immunization. The child in the vignette received a tetanus vaccine 2 years ago, so tetanus
immunization is not indicated.
Wound management must take into consideration host factors, wound factors, and plans for
follow-up care. Host factors include the child's immunization status, medical history, allergies,
and wound healing history (e.g., keloid formation). Conditions such-as developmental delay,
collagen-vascular disease, immunocompromised status, or malnutrition may inhibit the child's
ability to heal properly. Wound factors include the size and depth of the wound, wound location,
circulatory status, motor and sensory function, presence of foreign bodies or other contaminants,
and involvement of underlying structures (e.g., nerves, tendons, blood vessels, joints, bones).
Analgesia and anesthesia for wound assessment and management can be obtained through oral or
intravenous analgesics, topical or local infiltration of anesthetics such as lidocaine, or, when
necessary, the use of chemical sedation. Irrigation and removal of foreign bodies and devitalized
tissue are the most important steps to prevent wound infection. Irrigation should be performed
using tap water or sterile saline. Tap water has been shown to be as effective as sterile saline and
does not increase infection risk. Irrigation using 5-8 psi of pressure is sufficient to remove
bacteria and debris from most wounds. This can be achieved using an 18 to 20 gauge plastic
catheter tip attached to a 30-mL syringe. Splatter from irrigation can be minimized using
commercially available shield devices. Wounds heal best in a moist environment; topical
antibiotic ointments or petroleum jelly may be used with an occlusive or semi-occlusive
dressing. Re-epithelialization occurs within 24 hours; the wound should be kept covered for 24
hours prior to removing the dressing and reapplying ointment and a new dressing. All wounds
should be reassessed within 48 hours to evaluate for healing and signs of infection. A splint
should be applied if the wound occurs over an area of movement, such as a joint, to prevent
dehiscence. Parents should be counseled to avoid sun exposure and use sunscreen over the
wound after healing to prevent hyperpigmentation of the scar.

If the wound is grossly contaminated, even with thorough cleansing, it may not be possible to
remove all foreign bodies. It is essential to discuss the possibility of retained foreign bodies and
infection with parents. The potential for adverse cosmetic outcomes as well as appropriate
wound care techniques to prevent infection should also be discussed.
PREP Pearls
• Wound healing is dependent on both host and wound factors.
• Wound cleansing involves removal of visible debris and thorough irrigation of the wound
with normal saline or tap water using 5-8 psi of pressure. Adequate analgesia and/or
anesthesia should be provided
• Prophylactic antibiotics are not recommended for most clean wounds.

• Understand the principles of wound cleansing
Suggested Readings
• Wound management. Pediatr Rev. doi:10.1542/pir.36-5-207.
• Wounds. Pediatr Rev. doi:10.1542/pir.31-8-326.
• Wound irrigation in children: Saline solution or tap water?. Annals of Emerg Med.
Doi:10.1067/mem.2003.137

Question 127
A 3-year-old with severe traumatic head injury develops acute respiratory failure after requiring
intubation and mechanical ventilation. Initial arterial blood gas immediately after intubation
reveals acute respiratory acidosis with pH 7.12, blood PCO2 75 mm Hg, PO2 100 mm Hg,
bicarbonate 24 mEq/L (24 mmol/L), and base excess 0. Over the ensuing months, the child
shows no neurologic improvement, undergoes tracheostomy tube placement, receives long-term
mechanical ventilation, and has developed a chronic respiratory acidosis.
Of the following, compared with the arterial blood gas obtained on admission, the NEW arterial
blood gas would have a
A. Lower (more negative) base excess
B. higher bicarbonate
C. higher PCO2
D. lower pH

Correct Answer: B
Maintenance of normal acid-base homeostasis is essential for nearly all enzymatic functions of
the body. Acid-base abnormalities are primarily driven by derangements in serum acids, bases,
and electrolytes (metabolic component) or in ventilation and clearance or dissolved carbon
dioxide (respiratory component). Patients may have acute or chronic acid-base abnormalities,
which are reflected in characteristic serum electrolyte findings and arterial blood gas parameters.
The patient in the vignette was found to have acute respiratory acidosis. Respiratory acidosis
occurs when there is a decrease in alveolar ventilation and thus an increased concentration of
carbon dioxide. In acute respiratory acidosis, changes in PCO2 ate inversely proportional to
changes pH and can otherwise be calculated by the following formula:
• For every 10 mm Hg increase in PCO2, the pH is expected to decrease by ~0.08 units
• For every 10 mm Hg decrease in PCO2, the pH is expected to increase by ~0.08 units
For patients with more chronic acid-base abnormalities, compensatory mechanisms are required
to restore electrochemical neutrality (Item C127).
Item C127
In patients with primary respiratory acid-base disorders, metabolic compensation is required to

ensure normal homeostasis and maintenance of a neutral pH. A primary respiratory acidosis is
initially offset by intracellular buffers, followed by increased acid excretion by the kidneys. The
metabolic compensation (compensatory metabolic alkalosis) occurs by increasing renal excretion
of hydrogen ion (H+). In a chronic respiratory acidosis, acid excretion is promoted by the kidneys
through the generation of ammonium chloride, which then enhances bicarbonate retention, and
thus secondary bicarbonate buffering. Although the renal response to respiratory acidosis begins
within several hours of onset, maximal bicarbonate buffering occurs over 3 to 5 days. The serum
pH will return to near normal in spite of the fact that patients with chronic respiratory failure will
continue to have elevated PCO2.
Conversely, for patients with chronic respiratory alkalosis, the increased pH results in decreased
renal excretion of H+ and results in elevated chloride levels to maintain electroneutrality. Thus,
the renal compensatory mechanism for a primary respiratory alkalosis is a compensatory
metabolic acidosis through hyperchloremia.
The patient in the vignette has developed chronic respiratory acidosis and as such, a metabolic
alkalosis is required to restore and maintain normal pH. The base excess would be expected to be
more positive than the first arterial blood gas measurement and the serum bicarbonate will be
higher. Although PCO2 will remain elevated in chronic respiratory acidosis, because the child is
ventilated, the PCO2 should be lower than the initial values. Finally, pH should be higher (less
acidotic) compared to the first blood gas measurement as a result of the renal compensation.
PREP Pearls
• Compensation for primary acid-base disorders is opposite to the primary disturbance:
metabolic compensation for a primary respiratory and respiratory compensation for a
primary metabolic disturbance.
• Chronic compensation for respiratory acidosis requires the development of metabolic
alkalosis to normalize pH and electrochemical derangements.
• Compensation for respiratory acidosis is driven by renal mechanisms that result in
excretion of hydrogen ions and retention of serum bicarbonate.

• Identify the renal compensatory changes associated with primary respiratory acidosis
• Identify the renal compensatory changes associated with primary respiratory alkalosis
Suggested Readings
• A clinical approach to Paediatric acid-base disorders. Postgrad Med J.
doi:10.1136/postgradmedj-2011-130191.
• Fluids, electrolytes, and acid-base composition. American Academy of Pediatrics
Textbook of Pediatric Care 2nd ed.; 2017:419-433.
• Acid-base disorders. Pediatr Rev. doi:10.1542/pir.32-6-240.

Question 128
A 15-year-old adolescent girl with cystic fibrosis and exocrine pancreatic insufficiency is seen in
the clinic for a health supervision visit. She has been following her prescribed pulmonary
treatment regimen and cysti fibrosis transmembrane conductance receptor (CFTR) modulator
therapy, and is doing well from a respiratory stand point. However, she has developed oily loose
stools and has lost 6.8 kg over the past several months. For several years prior to this visit, she
had maintained a body mass index at the 50th percentile for age. The girl admits that she is not
happy with her physical appearance and has been purposefully missing doses of pancreatic
enzyme replacement therapy in order to help with weight loss. Her physical examination findings
are notable for a body mass index at the 25% for age, and abdominal distension. The physician
concerned about the long-term risks if she continues to avoid taking these medications.
Of the following, based on the adolescent’s history and findings, she is at GREATEST risk for
the development of
A. Celiac disease
B. fibrosing colonopathy
C. hypervitaminosis A
D. vitamin D deficiency

Correct Answer: D
The adolescent in the vignette has exocrine pancreatic insufficiency (EPI) as a result of cystic
fibrosis (CF). Because she is not taking her pancreatic enzyme replacement therapy (PERT) as
recommended, she is experiencing symptoms of fat malabsorption (e.g., oily, loose stools
[steatorrhea], abdominal pain, and distention) and is at risk for malnutrition, particularly fat-
soluble vitamin (A D, E, and K) deficiency.
Exocrine pancreatic insufficiency occurs when pancreatic enzymes (which aid in carbohydrate,
protein. and fat digestion) are not present in adequate amounts in the small intestine to facilitate
normal macronutrient digestion. Because there are digestive enzymes for carbohydrate and
protein but not for fats, in other areas of the gastrointestinal tract fat digestion is particularly
compromised in the presence of EPI. Causes of EPI include CF (the most common cause of EPI
in children), chronic pancreatitis, Schwachman-Diamond syndrome, Johanson-Blizzard
syndrome, and Pearson marrow pancreas syndrome. Celiac disease and duodenal Crohn disease
can also result in EPI because of inadequate stimulation of pancreatic secretion due to enterocyte
damage; in these cases, once the underlying disease is treated pancreatic secretion normalizes
and EPI resolves.
The most common test used to diagnose pancreatic insufficiency is a fecal elastase-1 level.
Elastase-l produced by pancreas is not degraded by intestinal proteases and is not impacted by
the use of PERT. A low (<100 µg/g) elastase-l level correlates with EPI; elastase levels from
100-200 µg/g are indeterminate; levels >200 µg/g are associated with pancreatic sufficiency.
Watery stools may result in a falsely low elastase-1 level.
Exocrine pancreatic insufficiency is treated with PERT, which consists of encapsulated (most
commonly enteric-coated) pancreatic enzymes designed for delayed release. Pancreatic enzyme
replacement therapy should be administered with the first bite of any meal of snack and should
be re-administered if eating a meal lasting longer than 30 to 60 minutes, attempting to mimic
what would be normal pancreatic enzyme secretion. The goal of PERT is to improve fat
malabsorption and, therefore, improve overall nutritional status. The use of PERT in children
with CF improves anthropometrics and fat-soluble vitamin absorption.
White celiac disease can present with EPI due to insufficient pancreatic stimulation and resulting
low pancreatic enzyme secretion, there is no increased risk for the development of celiac disease
with poor PERT adherence. Fibrosing colonopathy was a complication seen with very high doses
of PERT, which is not the current standard of CF care, and would not be a complication of poor
PERT adherence. Fat-soluble vitamin (A. D, E, K) deficiency may be seen in patients with
untreated EPI; hypervitaminosis A is not a complication of poor PERT adherence.
PREP Pearls
• Signs and symptoms of exocrine pancreatic insufficiency include steatorrhea, abdominal
bloating and cramping, fat-soluble vitamin deficiency, and weight loss/poor weight gain.
• Treatment of exocrine pancreatic insufficiency includes pancreatic enzyme replacement
therapy (PERT).
• Cystic fibrosis is the most common cause of exocrine pancreatic insufficiency in children


• Plan the appropriate management of pancreatic exocrine insufficiency
• Recognize the clinical features associated with pancreatic insufficiency
• Understand the etiology of pancreatic insufficiency
Suggested Readings
• Exocrine pancreatic insufficiency. Pediatr Rev. doi:10.1542/pir.2015-0084.
• Practical guide to exocrine pancreatic insufficiency: Breaking the myths. BMC Medicine.
2017. Doi:10.1186/s12916-017-0783-y
• ESPGHAN and NASPGHAN Report on the Assessment of Exocrine Pancreatic Function
and Pancreatitis in Children. J Pediatr Gastroenterol Nutr. 2015;
https://www.naspghan.orq

Question 129
A 7-month-old female infant with the skin lesion shown in Item Q129 is seen for routine follow-
up. Her growth and development have been normal. Magnetic resonance imaging of the brain at
3 months of age showed leptomeningeal angiomatosis.
Of the following, the MOST appropriate screening evaluation for this infant is
A. computed tomography of the lungs
B. echocardiography
C. intraocular pressure measurement
D. magnetic resonance imaging of the abdomen

Correct Answer: C
The infant in the vignette has Sturge-Weber syndrome with a facial port wine stain (PWS) and
leptomeningeal angiomatosis. This diagnosis has an associated risk of abnormal intraocular
vascular development. Therefore, the most appropriate screening evaluation for this infant is an
intraocular pressure measurement for glaucoma.
Port wine stain is a capillary malformation characterized by dilated capillaries in the superficial
or dermis skin layer. It is the most common vascular malformation of the skin. Unlike
hemangiomas, PWS is not proliferative. The appearance of PWS may change over time, but
lesions do not grow in size. Port wine stain manifests as red, pink, or purple macules with sharp
borders most often found on the face and neck. Port wine stain must be distinguished from nevus
simplex, a superficial vascular malformation with no associated abnormalities that typically
resolves by age 1 year.
Port wine stain most often occurs in isolation but can be associated with a number of syndromes,
including:
• Sturge-Weber
• Parkes-Weber
• Klippel-Trenaunay
• Proteus
• Servelle-Martorell
• CLOVES (congenital lipomatous overgrowth, vascular malformation, epidermal nevi and
scoliosis/skeletal/spinal anomalies)
Infants with PWS on the face should be evaluated for Sturge-Weber syndrome, which is
characterized by at least 2 of the following:
• Facial PWS, most often on the forehead and upper eyelid
• Leptomeningeal angiomatosis
• Abnormal intraocular vascular development
Sturge-Weber syndrome is associated with several conditions requiring monitoring and/or

additional evaluation (Item Cl29).
Item Cl29: Sturge-Weber syndrome

• Leptomeningeal venous angiomatosis
• Seizures
• Stroke like episodes
• Intellectual disability
• Glaucoma
• Hypopituitarism
• Hypothyroidism
Historically, the distribution of PWS in the pattern of the trigeminal nerve branches was thought
to correlate with the risk of leptomeningeal angiomatosis. However, the presence of PWS in this
pattern does not accurately predict leptomeningeal angiomatosis.

Computed tomography of the lungs is recommended for adult women with tuberous sclerosis
complex to diagnose pulmonary lymphangioleiomyomatosis. Echocardiography should be
completed in individuals with tuberous sclerosis complex to identify cardiac rhabdomyomas.
Abdominal magnetic resonance imaging should be used to screen for renal angiomyolipoma and
renal cysts in those with tuberous sclerosis complex.
PREP Pearls
• Port wine stain is a capillary malformation, most often found on the face and neck; it may
be associated with a genetic syndrome.
• Sturge-Weber syndrome is diagnosed by the presence of at least 2 of the following: facial
port wine stain, leptomeningeal angiomatosis, and elevated intraocular pressure.
• Children with Sturge-Weber syndrome should undergo ophthalmologic evaluation for
elevated intraocular pressure.
MOCA-Peds Objective
• Identify and manage cutaneous vascular lesions of infancy.

• Plan the appropriate management of a port wine stain
• Recognize the importance of the distribution of a port wine stain
• Recognize the clinical findings associated with Sturge-Weber syndrome
Suggested Readings
• Neurocutaneous syndromes. American Academy of Pediatrics Textbook of Pediatric Care
2nd ed.; 2017:2379-2396.
• Visual diagnosis: Newborn with a facial vascular birthmark. Pediatr Rev.
doi:10.1542/pir.36-9-e30
• Neurocutaneous disorders in children. Pediatr Rev. doi:10.1542/pir.2015-0118.

Question 130
A 14-year-old adolescent boy is seen for a health supervision visit. His growth and development
have been normal, and he plays basketball for his high school team. His mother reports that she
would like him to use a mouth guard during basketball, but he is very resistant. His team does
not require mouthguards during practice or play. He has no history of dental or facial injury, and
no dental-related concerns.
She is concerned about the potential cost.
Of the following, the BEST advice for this patient and his mother is that
A. dental consultation should be obtained to fabricate upper and lower mouthguards
B. moldable “boil and bites” mouthguards can effectively prevent most dental injuries in
sports
C. mouthguards are not generally recommended for young basketball players
D. prefabricated stock mouthguards are sufficient for most young athletes

Correct Answer: B
Dental injuries are common in high school basketball players, and mouthguards can reduce risk
by 80% to 90%. Therefore, the athlete and his mother in this vignette should be counseled on
appropriate mouthguard use. For young athletes without orthodontics or significant
malocclusions, a moldable *boil and bite” mouthguard is a cost-effective way to prevent dental
injury and is the best recommendation for this patient.
Dental injuries are common in high school sports and have been reported in up to 25% or soccer
players, 50% of basketball players, and 75% of wrestlers. These injuries are usually caused by
hits from balls of equipment or from direct prayer-to-player contact. Properly fit mouthguards
are very effective at minimizing this injury risk, Item C130A lists sports in which mouthguard
use is recommended by the American Dental Association, this list is far more inclusive than
recommendations made by the National Federation of High Schools and national sports
governing bodies. It is important to recognize that sports-related organizations often receive
sufficient pushback when mandating use of protective gear, and their decisions may not reflect
best medical practice. When mouthguards are mandated, clear and white mouthguards are often
prohibited because they are difficult to see and interfere with the ability of officials to ensure
compliance. Categories of commonly used mouthguards are shown in Item C130B.
Item C130A: Mouthguard recommended sports

acrobats, basketball, boxing, field hockey, football, gymnastics, handball, ice hockey, lacrosse, martial
arts, racquetball, roller hockey, rugby, shot putting, skateboarding, skiing, skydiving, soccer, squash,
surfing, volleyball, water polo, weightlifting, and wrestling
Item C130B: Categories of commonly used mouthguards
The thickness of the mouthguard material is the key factor in injury prevention, but there is a
trade-off between protection and comfort. The first International Sports Dentistry Workshop
recommended that mouthguards for sports should be 3-mm thick on the labial and occlusal
surfaces, and 2-mm thick on the palate side, and should fully cover teeth from the central incisors
back to the first molar. The mouthguard should feel comfortable and secure without the need for
muscle activation to maintain position. During the fitting process, some mouthguards may thin
up to 70% to 90%, which decreases their efficacy. Mouthguards should be cleaned with a
toothbrush and cool water after use and stored in a sturdy and well ventilated container, Boil and
bite mouth guards will thin with use and should generally be replaced at the end of each
competitive season.
Fifty percent to 90% of dental injuries involve the maxillary incisors (i.e., front teeth). The
normal human overbite of 2 to 3 mm makes these teeth more vulnerable and protects the lower
teeth. Mouthguards in athletes with a normal overbite typically need to cover the upper teeth
only; however, those with an underbite (class 3 malocclusion) need to consider guards for the
lower mandibular teeth as well. There are relatively few mandibular (lower) mouthguards
available “off the shelf” and comfort, fit, and compliance are often problematic. These
mouthguards often require custom fabrication by a dental professional. Custom mouthguards are
also indicated for athletes with other significant malalignments, and orthodontic or other
hardware often precludes the use of commercially available mouth-formed mouthguards.
PREP Pearl
• Mouthguards are recommended for most young athletes participating in contact collision
sports.
• “Boil and bite” moldable mouthguards can effectively reduce dental injury risk in young
athletes.
• Custom mouthguards are indicated for athletes with orthodontia, an underbite, or other
dental malocclusion.

• Recognize the indications for the use of a mouth guard during sports activities
Suggested Readings
• American Dental Association. Oral health topics: Mouthguards.
https://www.ada.org/en/member-center/oral-health-topics/mouthguards
• National Athletic Trainers' Association position statement; preventing and managing
sport-related dental and oral injuries. J Athl Train. doi:10.4085/1062-6050-51.8.01
• Dental problems in athletes. Curr Sports Med Rep. doi:10.1249/JSR.0000000000000114.
• Effectiveness of mouthguards for the prevention of orofacial injuries and concussions in
sports: Systematic review and meta-analysis. Sports Med. Doi 10.1007/s40279-019-
01121-w
• Mouthguards and their use in sports: report of the 1st International Sports Dentistry
Workshop. Dent traumatol. Doi:10.1111/edt.12375

Question 131
A 4-year-old boy is brought to the emergency department for poor oral intake and decreased
urine output for 1 day. He has had watery stools with blood and vomiting for 3 days. One week
ago, he visited a farmhouse where he consumed unpasteurized apple cider. His heart rate is 110
beats/min, respiratory rate is 18 breaths/min, and blood pressure is 120/72 mm Hg. His
conjunctivae are pale, mucous membranes are moist, and he has mild facial edema. His chest is
clear to auscultation. The remainder of his physical examination findings are unremarkable.

Blood
Peripheral smear Schistocytes present
Prothrombin time 10 s (reference range 9.4-11.7 s)
Activated partial 27 s (reference range, 23-33 s)
thromboplastin time
Urine
Nitrite Negative
Blood 2+
Protein 1+
Of the following, the MOST likely diagnosis for this boy is

A. disseminated intravascular coagulation
B. hemolytic uremic syndrome
C. purpura
D. prerenal acute kidney injury

Correct Answer: B
The boy in the vignette has hemolytic uremic syndrome (HUS). He has a history of bloody
diarrhea after consumption of unpasteurized apple cider, a potential source of Shiga toxin-
producing Escherichia coli (STEC) infection. The classic triad of microangiopathic
thrombocytopenia, hemolytic anemia (presence of schistocytes on peripheral blood smear),
thrombocytopenia, and acute kidney injury favors a diagnosis of HUS.
Hemolytic uremic syndrome most commonly occurs after an infection with STEC. Potential
sources of STEC exposure include consumption of undercooked ground beef, unpasteurized
cider, or food contaminated with fecal material; swimming in contaminated lakes or pools; and
contact with farm animals. Escherichia coli O157:H7 is the most common serotype associated
with HUS. Shigella dysenteriae and pneumococcal infections can also result in HUS. Typical
HUS results from diarrheal illness or other infection. Mutations in complement or coagulation
pathway genes or autoantibodies to complement factors can cause atypical HUS.
Children commonly exhibit abdominal pain, vomiting, and bloody diarrhea before showing signs
of HUS. Hemolytic uremic syndrome is defined by a triad of microangiopathic hemolytic
anemia, thrombocytopenia, and acute kidney injury. Hemolytic anemia is characterized by low
hemoglobin levels, a negative finding on antiglobulin (Coombs) test, and a large number of
schistocytes seen on a peripheral blood smear. Other features of hemolysis include elevation of
indirect bilirubin and lactate dehydrogenase levels, and reduction in serum haptoglobin levels.
Thrombocytopenia results from formation of microthrombi. Renal manifestations include acute

kidney injury, hematuria-proteinuria, and hypertension. Hemolytic uremic syndrome can also
affect the central nervous system (e.g., altered mental status, seizure), gastrointestinal tract (e.g.,
hemorrhagic colitis, perforation), heart (e.g., myocarditis), pancreas (e.g., transient diabetes
mellitus), and liver (e.g., elevated liver enzymes, hepatomegaly).
Treatment of STEC-HUS is supportive. Children with severe anemia (hemoglobin < 6g/dL
[60g/L]) may require multiple red blood cell transfusions, Platelet transfusion is indicated only if
there is active bleeding. Acute kidney injury is managed by maintaining an euvolemic state.
Dehydration, if present, should be corrected with volume repletion. However, fluid restriction
may be required because oliguria develops later in the course of illness. Hypertension managed
using fluid restriction and calcium channel blockers. Renal replacement therapy (i.e., dialysis)
may be indicated for managing symptoms of severe acute kidney injury (e.g., hyperkalemia.
fluid overload, severe metabolic acidosis, and uremia). Administration of eculizumab
(complement factor 5 inhibitor) may be considered for a child with neurologic involvement.
Disseminated intravascular coagulation (DIC) is characterized by abnormal coagulation studies

and an elevated D-dimer level. However, for the boy in the vignette, DIC is an unlikely diagnosis
because he has a normal prothrombin and activated thromboplastin time. Henoch-Schonlein
purpura is a systemic vasculitis characterized by palpable purpura, arthralgia, gastrointestinal
manifestations, and varying degree of renal involvement. Anemia and thrombocytopenia, as
reported for the child in the vignette, are not presenting features in a child with Henoch-
Schönlein purpura. Prerenal acute kidney injury may be seen in children with diarrhea and
vomiting, but the boy in the vignette has no clinical features of dehydration. His elevated blood
pressure and the presence of hematuria and proteinuria suggest a renal (not prerenal) cause of
acute kidney injury.
PREP Pearls
• Hemolytic uremic syndrome is characterized by a triad of microangiopathic hemolytic
anemia, thrombocytopenia, and acute kidney injury.
• Hemolytic uremic syndrome most commonly occurs after infection with Shiga toxin-
producing Escherichia coli.
• Treatment Of hemolytic uremic syndrome related to Shiga toxin is supportive
MOCA-Peds Objective
• Understand the diagnosis and management of infectious diarrhea,

• Plan the appropriate initial management of hemolytic-uremic syndrome
• Recognize the clinical and laboratory findings associated with hemolytic-uremic
syndrome
• Recognize the clinical and laboratory findings associated with Escherichia coli infection
in children of various ages, including its association with hemolytic-uremic syndrome
Suggested Readings
• Hemolytic-uremic syndrome. American Academy of Pediatrics Textbook of Pediatric
Care 2nd ed.; 2017:2125-2127.
• Hemolytic uremic syndrome. Pediatr Rev, doi:10.1542/pir.2018-0346

Question 132
A 6-month-old male infant is evaluated in the emergency department in July for a nonpruritic
rash on the trunk and extremities (Item Q132). The initial onset was 3 days ago with associated
fever. His temperature is 38.3º C, heart rate is 110 beats/min, respiratory rate is 25 breaths/min,
and blood pressure is 95/60 mm Hg. The oropharyngeal examination findings are normal.
Data from a complete blood count are shown:

Platelet 450 x 103/µL (450 109/L)
The infant has a history of atopic dermatitis since 2 months of age. His fully vaccinated 6-year-
old sister was diagnosed with a virus 1 week ago and doing well.
Of the following, the MOST likely diagnosis for this child is

A. eczema coxsackium infection
B. hand-foot-and-mouth disease
C. primary herpes simplex virus infection
D. primary varicella infection

Correct Answer: A
The infant in the vignette has eczema coxsackium. While usually a clinical diagnosis, an
enterovirus polymerase chain reaction (PCR) or culture of the skin can provide confirmation.
Eczema coxsackium is an enteroviral rash seen in infants and children with underlying atopic
dermatitis. It is characterized by vesicles, bullae, and/or erosive lesions of skin affected by atopic
dermatitis (Item C132A). The appearance is similar to eczema herpeticum caused by herpes
simplex virus. Herpes simplex virus is a less likely cause for this child's condition; eczema
coxsackium caused by coxsackievirus A6 generally results in a less painful rash, and affected
children typically have a milder and shorter course of illness than those with herpes simplex
virus infection. In primary uncomplicated varicella, the rash is pruritic with lesions at different
stages of evolution.
Item C132A
Eczema coxsackium differs from the classic clinical presentation of hand-foot-and-mouth disease
(HFMD). The eczema coxsackium rash is generally more widespread, consists of vesicles and
erosions, and includes more than 10% of the body surface area without oral involvement. In
contrast, the HFMD rash typically consists of vesicles and involves the palms and soles along
with oral lesions. Herpangina, caused by coxsackie viruses, primarily affects the oral mucosa and
presents with painful vesicles in the mouth along with fever and sore throat. It is most frequently
seen in children 3 to 10 years of age.
Enteroviruses (EVs) belong to the Picornaviridae family. The conventional classification of

human EVs grouped them as polio- and nonpolio-EVs. The nonpolio EVs include echoviruses,
coxsackie viruses, and other numbered EVs. The newer classification is based on molecular
serotyping.
Enterovirus infections peak in the summer months. Most infections are seen in summer and fall
in the temperate northern hemisphere. The virus circulates throughout the year in tropical
regions. Enteroviruses are among the most common causes of community viral outbreaks
encountered by pediatricians and a frequent cause of aseptic meningitis during the summer
months. Enteroviruses are highly contagious and spread through fecal-oral and respiratory
secretions. Approximately half of EV infections are asymptomatic. Symptomatic infections

present as a spectrum; from a minor illness such as a common cold to fulminant sepsis and
meningitis (Item C132B).
The average incubation period for EVs is 3 to 6 days. Respiratory tract shedding is limited to 1 to
3 weeks whereas fecal shedding can persist longer, 2 to 8 weeks after primary infection.
Enterovirus 71, the cause of hemorrhagic conjunctivitis, is transmitted via fingers, fomites, and
tears. Infants, especially those in diapers, are highly infectious vehicles of EV transmission.
After entry into the host, viral replication occurs in the upper respiratory tract and distal small
bowel. This viral replication in the submucosal lymphoid tissue results in brief primary viremia
that distributes virus to reticuloendothelial tissue in distant lymph nodes, liver, spleen, and bone
marrow. Additional replication in these organs results in continued secondary viremia and
dissemination of virus to organs such as the central nervous system, heart, and skin.
Item C132B
Polymerase chain reaction is the most sensitive (86%) test for identification in the cerebrospinal
fluid and respiratory tract secretions. Enterovirus serology is of limited use in acute infections
because it requires acute and convalescent titers, there is cross reactivity among different
serotypes, and there is a lack of IgM assays.

There is no specific treatment for EV infections. The primary management is supportive care.
Transmission can be reduced with simple infection control precautions such as hand washing and
careful disposal of soiled diapers.
PREP Pearls
• Eczema coxsackium is an atypical skin rash in children with atopic dermatitis
characterized by vesicles and erosions within areas of eczema.
• Herpangina presents with painful vesicles of the oral mucosa, fever, and sore throat most
commonly children 3 to 10 years of age.
• Hand-foot-and-mouth disease manifests as a vesicular skin rash on the hands and feet
along with oral vesicles
• Enteroviruses are the most common cause of aseptic meningitis during the summer
months

• Plan appropriate laboratory evaluation for enterovirus infection
• Understand the epidemiology of the enteroviruses
• Recognize the clinical features associated with echo- and coxsackievirus infection in
patients of various ages
Suggested Readings
• Eczema coxsackium. Arch Dis child, doi: 10.1136/archdischild-2014-307336
• Eczema coxsackium caused by coxsackievirus A6. Pediatr Dermatol,
doi:10.1111/pde.12874
• Eczema coxsackium and unusual cutaneous findings in an enterovirus outbreak.
Pediatrics, doi: 10.1542/peds.2012-3175
• Hand-foot-and-mouth disease: A new look at a classic viral rash. Curr Opin Pediatr, doi:
10.1097/MOP.0000000000000246
• Enterovirus infections. Pediatr Rev. doi:10.1542/pir.2016-0103

Question 133
A 13-year-old, previously healthy, adolescent boy is having his annual hearth supervision visit.
He and his parents are concerned about his puberty. They have noticed that the boy's growth and
physical maturation are slower than those of his soccer teammates. He has a twin sister who has
had a growth spurt and is 5’5’ tall. Her menses recently began at about the same age as her
mother’s menarche. The father recalls that his own pubertal development began slightly later
than that of his peers. Historically the boy's height and weight have consistently tracked just
below the 50th percentile since age 3 years. A year ago, the boy had no pubic hair and the
testicles were noted to be 4 mL in volume and were in the scrotum, which was reddened and
rugated.
At today's visit, the boy says that he looks forward to an increase in height and strength
associated with puberty.
On physical examination, his height is 155 cm (42nd percentile), weight is 44 kg (42nd

percentile), body mass index is 18 kg/m2 (48th percentile), and blood pressure is 112/74 mm Hg.
He has grown 7 cm in the last year. He has no acne or gynecomastia. A small amount of axillary
hair is noted, and the remainder of the general examination findings are unremarkable. The
genital examination shows straight pubic hair at the base of the penis and testicles of 10-mL
volume.
Of the following, the BEST next step in the evaluation of this boy is a
A. bone age measurement
B. karyotype
C. measurement of gonadotropin levels
D. return visit in 6 to 12 months

Correct Answer: D
One year ago, at age 12 years, the boy in the vignette had testicles that were 4 ml in volume,
which is consistent with a sexual maturity rating genital 2 (SMN G2) that indicated onset of
puberty (gonadarche). His onset of puberty is well within the normal range of 9 to 14 years.
At today's visit 1 year later, the boy in the vignette has further enlargement of testicles, to SMR
G3, and development of early pubic hair (PH) growth SMN PH2 (adrenarche). He also has had
an increase in linear height growth. These findings are consistent with the usual sequence and
progression of male puberty. The best next step for the evaluation of this boy is a return visit in 6
to 12 months to assess his pubertal progression. The expectation is that he will have continued
progression of testicular and pubic hair maturation. Axillary hair growth and acne accompany
adrenarche. Growth in the length followed by an increase in breadth of the phallus is observed
during SMN G3 and G4. He should experience a sustained growth spurt with a peak height
velocity during SMN G3 to G4. The mean peak height velocity for boys is 9.5 cm/year. Peak
strength increases as the height velocity declines closer to the end of puberty. Deepening of the
voice also is a later pubertal event that occurs during SMR G4. Time for completion of puberty
for boys is 3 years, with the range being 2 to 5 years. The sequence of pubertal events is
summarized in Item C133. Physiologic gynecomastia is common during SMR G3 and is
typically associated with less than 4 cm diameter of palpable breast tissue and a duration of less
than 18 months.
Distinguishing normal from abnormal sexual development for boys requires the recognition of
delayed pubertal onset, lack of expected progression of pubertal events, incongruent pubertal
events, or virilization that is early, rapid, or poor. Delayed puberty in boys is defined as the
failure of testicular enlargement to achieve SMN G2 by age 14 years. A bone age, karyotype,
and measurement of gonadotropin levels may be indicated for the evaluation of delayed or
abnormal puberty and would not be needed for the boy in the vignette.
The most common cause of delayed puberty in boys is constitutional delay of puberty and
growth. Both gonadarche and adrenarche are delayed. Typically, there is a family history, and an
affected boy experiences a relatively slower rate of prepubertal linear growth. The prepubertal
height usually is below the 25th percentile for age; otherwise, the history and physical
examination findings are unremarkable. The evaluation may include a bone age that is expected
to be delayed followed by subsequent observation of his growth and pubertal development.
Functional causes of delayed puberty or lack of expected progression include chronic diseases
such as inflammatory bowel disease, diabetes mellitus and other autoimmune disorders, cystic
fibrosis, sickle cell disease, and eating disorders.
Other less common causes of delayed onset of puberty include gonadal failure, hypothalamic and
pituitary pathology, and genetic disorders. Klinefelter Syndrome would be suspected in a boy
who has small, firm testicles that have had limited enlargement after SMR G2, normal pubic hair
growth, decreased upper to lower body ratio (long legs), and gynecomastia. Boys with Kallmann
syndrome have adrenarche but no gonadarche. They also have anosmia or hyposmia.
Isolated early or premature adrenarche in boys requires periodic reevaluation and may be benign.
Peripheral precocious puberty can occur from congenital adrenal hyperplasia, adrenal tumors,
and pharmaceutical or herbal exposure to androgens. Central precocious puberty, though
uncommon in boys, is frequently associated with central nervous system pathology such as
tumors, malformations, and trauma.
Item C133: The sequence of pubertal events

PREP Pearls
• Testicular enlargement of 4 mL in volume indicates the onset of puberty
• For boys, peak height velocity occurs during sexual maturity rating genital 3 to
MOCA-Peds Objective
• Recognize and evaluate an adolescent with abnormal pubertal development

• Understand the sequence of development or secondary sexual characteristics in boys
• Distinguish normal from abnormal sexual development in males
Suggested Readinqs
• Normal pubertal development part II: Clinical aspects of puberty. Pediatr Rev,
doi:10.1542/pir.32-7-281
• Normal physical growth and development. Neinstein’s Adolescent and Young Adult
Heath Care: A Practical Guide 6th ed. 2016;28-37
• Adolescent growth and development. Prim Care, doi:10.1016/j.pop.2014.05.002
• Puberty: Normal and abnormal. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017:1540-1545.

Question 134
A 16-year-old adolescent boy is seen for a health supervision visit accompanied by his mother.
During the visit, he shares that he has developed significant anxiety, contributing to a decline in
school performance and self-esteem. His mother has encouraged him to attend cognitive
behavioral therapy sessions, but he has been reluctant to go because he does not feel comfortable
“talking with a stranger”. When interviewed without his mother present, he shares that his
anxiety has affected his friendships, and he cannot sleep because he spends hours at night
worrying about different things. He reports no substance abuse, or suicidal or homicidal ideation.
Of the following, the BEST next step in this child's care is to

A. ask him about his concerns regarding cognitive-behavioral therapy
B. discuss with his mother the different management approaches to anxiety treatment
C. prescribe a selective serotonin reuptake inhibitor and review adverse effects with his
mother
D. refer the adolescent to cognitive behavioral therapy with his mother’s consent

Correct Answer: A
Adolescents and children old enough to provide assent should be included in a developmentally
appropriate manner in discussions involving their medical care. Initiating a conversation with an
adolescent to understand their perspective on recommended treatment, in this case cognitive
behavioral therapy, is an important first step in partnering with the child and their family in
making care decisions. The adolescent in the vignette should be included in conversations with
his mother on the use of medications and anxiety management. Referral for cognitive behavioral
therapy without his assent, or further exploration of his prior refusal, undermines the adolescent's
ability to be an active participant in his own care.
Shared medical decision-making is grounded in the concept of informed consent, which has both
legal and ethical components. The core ethical principles of informed consent include:
autonomy, respect for the decisions one makes regarding one's own life; beneficence, the duty to
act for the benefit of the patient; and justice, fairness in all medical decision-making. From a
legal standpoint, the process of informed consent incorporates 3 duties (Item C134A).
Item C134A: Elements of Informed Consent for Medical Decision-Making

• disclosure of information to patients and their surrogates
• assessment of patient and surrogate understanding of the information and their capacity for
medical decision-making
• obtaining informed consent before treatments and interventions
In adult patients with capacity for medical decision-making, informed consent takes place
directly between the physician and patient. In pediatric practice, medical decision-making
involves the physician, parent or legal guardian, and child. Shared decision-making is a dynamic
and collaborative process that allows a child, their family, and physicians to make decisions
together, taking into account evidence based data along with the child's/family's values, beliefs
and goals. Informed consent and medical decision making do not take place in isolation, but
rather are influenced by family dynamics, culture, religion, and ethnicity, which can all impact
the process. The goal is to determine the most beneficial care for the child. While pediatric
patients are generally unable to provide consent, with few exceptions, shared decision-making
enables children to participate in the informed consent process in a developmentally appropriate
manner.
Around 7 years of age, children transition in their cognitive development from preoperational to
a concrete operational stage, signaling an ability to participate in the informed consent process
and offer assent in shared medical decision-making. Key elements of the assent process by a
pediatric patient are summarized in Item C134B. Inclusion of children in the medical decision-
making process fosters their growth and development of autonomy. As developmental
maturation continues the child or adolescent will grow in their ability to participate. This
longitudinal process reflects ongoing cognitive development. The final stage, formal operational,
is reached by mid-adolescence and allows for conceptualizing more abstract or complex
problems.
Item C134B Practical Aspects of Assent by Pediatric Patients for Medical Decision-Making
• Help the patient achieve a developmentally appropriate awareness of the nature of his or her
condition
• Tell the patient what he or she can expect with tests and treatments
• Make a clinical assessment of the patient’s understanding of the situation and the factors
influencing
• how he or she is responding (including whether there is inappropriate pressure to accept testing
or therapy)
• Solicit an expression of the patient’s willingness to accept the proposed care
There are several important additional considerations for adolescents in medical decision-
making. Adolescents are able to provide independent consent in all states for health care related
to sexual activity and in many states for care related to mental health and substance abuse. While
consent by the adolescent can occur in these clinical scenarios, the ability to maintain
confidentiality regarding the care may vary by state. Adolescent parents are able to provide
informed consent for the care of their own children, despite being unable to do so for themselves
unless they have emancipated minor status; thus, creating an interesting paradox in informed
consent models.
PREP Pearls
• Shared decision-making is a dynamic and collaborative process that allows a child, their
family, and physicians to make decisions together, taking into account evidence-based
data along with the family's values, beliefs, and goals.
• Starting at age 7 years, most children are able to participate in a developmentally
appropriate manner in shared decision-making. This will evolve as their cognitive
development advances, allowing increasing participation in discussions and decision-
making.
• The informed consent process is rooted in ethical principles of autonomy, beneficence,
and justice. Assent, or ability to agree with medical decision making, is an important
component of the informed consent process in pediatrics.
MOCA-Peds Objective
• Recognize and apply ethical principles involved in informed consent/assent.

• Understand when it is appropriate to have a minor involved in making decisions about his
or her medical care
• Recognize end apply ethical principles involved in the patient-parent-Pediatrician
relationship regarding minors as decision-makers
Suggested Readings
• AAP Committee on Bioethics. Informed consent in decision-making in pediatric practice.
Pediatrics, doi:10.1542/peds.2016-1484
• Medical decision-making in children and adolescents; Developmental and neuroscientific
aspects. BMC Pediatr, doi:10.1186/s12887-017-0869-x
• Shared decision-making in pediatric practice: A systemic review and metanalysis. Acad
Pediatr, doi:10.1016/j.acap.2015.03.011
• Shared decision-making in pediatric practice: A board review, Pediatrics.
doi:10.1542/peds.2018-0516B
Question 135
Forty-eight hours after birth, a neonate born at 35 weeks' gestation is undergoing a car seat
challenge prior to discharge home from the newborn nursery. During the 90-minute observation
with cardiorespiratory monitoring, he experiences a sustained 20-second oxygen desaturation to
88% in room air, requiring stimulation. His weight is 2,400 g, heart rate is 140 beats/min,
respiratory rate is 50 breaths/min, and pre and post-ductal oxygen saturations are 98% and 98%
on room air, respectively. His physical examination findings are otherwise unremarkable.

A. discharge him home in a car bed
B. discharge him home in his upright car seat
C. rechallenge him in a car bed
D. rechallenge him in his upright car seat

Correct Answer: C
The 2017 American Academy of Pediatrics (AAP) Guidelines for perinatal care recommend that
preterm infants be screened with an infant car seat challenge prior to discharge from the hospital.
This process involves monitoring the neonate/infant for bradycardia, apnea, or oxygen
desaturation for 90 to 120 minutes while in an infant car seat (their own is preferred). Risk
factors for cardiorespiratory events while restrained in a semi-reclined car seat include
prematurity; low birth weight, hypotonia (e.g., Down syndrome), micrognathia (e.g., Pierre
Robin sequence), and congenital heart disease.
If an infant experiences a sustained desaturation during a car seat challenge, the best next step is
to rechallenge the neonate in a car bed. While the AAP does not specify the definition of a
“failed” car seat challenge, widely recognized criteria include: apnea as cessation of respiratory
effort for ≥20 seconds, bradycardia as a heart rate of ≤80 beats per minute, and a lower limit of
oxygen saturation in room air via pulse oximetry as 90% or 93% for 10 or 20 seconds or longer.
Neonates/infants who continue to “fail” the car seat challenge should not be discharged to home
until investigation for cardiopulmonary abnormalities is performed. Of note, the car seat
challenge is controversial, as little evidence currently exists demonstrating an association long-
term neurodevelopmental outcomes and hypoxemic events noted during a car seat challenge.
Further research is needed to determine the predictive value of the car seat challenge for
neurodevelopment in neonates/infants at higher risk for cardiorespiratory events.
PREP Pearls
• Risk factors for cardiorespiratory events (e.g., apnea. bradycardia, and oxygen
desaturation) while restrained in a semi-reclined car seat include prematurity, low birth
weight, hypotonia (e.g., Down syndrome), (e.g., Pierre Robin sequence), and congenital
heart disease.
• For the infant car seat challenge, apnea is widely recognized as cessation of respiratory
effort for ≥20 seconds, bradycardia as ≤80 beats per minute, desaturation as oxygen
saturation below 90% or 93% for 10 or 20 seconds or longer.
• Neonates who continue to “fail” the car seat challenge should not be discharged until
investigation for cardiopulmonary abnormalities is performed

• Recommend appropriate car restraint systems, including car seats, based the age and
weight of the child. including those appropriate for premature infants
Suggested Readings
• Care of the newborn. American Academy of Pediatrics Guidelines for Perinatal Care, 8th
ed. 2017:347-402
• Safe Transportation of preterm and low birth weight infants at hospital discharge.
Pediatrics doi:10.1542/peds.2009-0559
• Council on Injury, Violence and Poison Prevention, Car seat safety. Pediatrics,
doi:10.1542/peds.2019-1703.

Question 136
An 11-year-old girl is brought to the emergency department because she “looks pale”. She
reports occasional fatigue, but no syncope, dizziness, or difficulty breathing with exertion. The
girl had menarche at age 10 years and reports heavy bleeding for the first few days of each
menstrual period. Her vital signs are normal for age except for a heart rate of 110 beats/min. She
appears well but pale. Her physical examination is notable for pallor her nail beds and mucous
membranes, and a 1/6 systolic flow murmur. She has no rashes or hepatosplenomegaly. Physical
examination findings are otherwise unremarkable. Her hemoglobin level is 5.9 g/dL (59 g/L).
Admission to the hospital for further evaluation of her anemia and treatment with a blood
transfusion are planned. Upon hearing this plan of care, both parents state that they refuse any
blood transfusion because they are Jehovah's Witnesses and blood transfusions are not permitted
under their religious beliefs

A. consult the institutional ethics committee and child protective services to file for
immediate medical custody of the girl
B. explain the medical necessity of the treatment and that withholding medically necessary
treatment from a child is prohibited by law
C. invoke emergency informed consent and give the girl a blood transfusion against her
parents' wishes
D. reassure the patents that because of their religious beliefs, under no circumstances will
the girl receive a blood transfusion

Correct Answer: B
Under the principle of ethical autonomy, adults have the freedom to make decisions that reflect
their faith and religious values, even at the expense of their medical well-being. They may refuse
life-saving therapies and interventions provided they have the mental capacity and are provided
with all the information needed to make an informed decision. However, parents may not refuse
care on behalf of their minor children if such refusal endangers the well-being of the child. Thus,
given that child in the vignette has significant and symptomatic anemia, the physician should
explain that the law does not allow for a medically necessary transfusion to be withheld from the
minor child.
The American Academy of Pediatrics statement on religious objections to medical care notes, in
accordance with state and federal statutes, “children, regardless of parental religious beliefs,
deserve effective treatment when such treatment is not overly burdensome and is likely to
prevent substantial ham, serious disability or death.” Although clinicians must support parental
autonomy and take into account the religious and cultural perspectives of families, they must
balance those needs with the need to protect children from serious and imminent harm. In some
cases, failure to provide or consent to medically necessary care may constitute abuse or neglect.
Recognizing religious and cultural beliefs as important factors affecting parental medical
decision-making is necessary for physicians to provide appropriate cross-cultural medical care.
Cross-cultural respect in medical decision-making is imperative for clinicians to uphold the
principles of ethical autonomy. However, conflict may arise between the medical
recommendations of the care team and the beliefs and desires of parents of minor children.
Ethical principles may be invoked when there are familial religious or cultural-based refusals,
such as the refusal of blood transfusions by patients who practice the Jehovah's Witnesses faith,
or refusal of chemotherapy for childhood cancers by families who are Christian Scientists. When
a situation is not life-threatening, the care team can often negotiate an appropriate alternative
with the family while ensuring that the safety of the child is maintained, as with medically
recommended childhood vaccinations. If the child in the vignette had a modest anemia, rather
than symptomatic anemia, the physician may have suggested iron therapy and erythropoietin as
medically reasonable alternatives to transfusion therapy.
In certain situations, parental consent is not required for the treatment of pediatric patients.
Consent may be implied when children have life-threatening conditions that require emergency
care and a parent or legal guardian is not available. In these situations, emergency implied
consent can be invoked. The definition of a life-threatening condition may at times be subjective,
and ideas of what constitutes a true emergency may differ between clinicians and parents.
However, care should not be delayed when interventions to prevent significant harm are
medically necessary regardless of the ability to locate a parent or legal guardian and obtain
consent from them.
Although ethics consultation can be used to assist clinicians with difficult decision-making
around ethical principles, there is no indication for filing a report with child protective services
of to seek immediate medical custodianship before attempting to work with the family. In
addition, emergency informed consent is reserved for life-threatening situations in which the
parents or legal guardians are unable to provide informed consent; in this case, the parents are
readily available. Finally, given the clear medical indications for blood transfusion, it would be
inappropriate to falsely reassure the parents that under no circumstances will the patient receive a
blood transfusion.
PREP Pearls
• Cross-cultural respect in medical decision-making is imperative for clinicians to uphold
the principles of ethical autonomy.
• Under current US law, parents may not refuse care on behalf of their minor children if
such a refusal endangers the well-being of the child.
• Consent may be implied when children have lite-threatening conditions that require
emergency care and a parent or legal guardian is not available.
MOCA-Peds Objective
• Understand the role of the pediatrician in prevention of child abuse and neglect.
• Recognize and apply ethical principles involved in informed consent/assent.

• Recognize and apply ethical principles involved in professionalism and institutional
ethics relative to cross-cultural issues
• Recognize and apply ethical principles regarding institutional ethics committees
Suggested Readings
• A case study in cross-cultural health care and ethis. J Hospice Palliative Nurs,
doi:10.1097/NJH.0000000000000529
• Committee on Bioethics Policy statement: Religious objections to medical care.
Pediatrics. doi:10.1542/peds.99.2.279
• Informed consent in decision-making in pediatric practice. Pediatrics.
doi:10.1542/peds.2016-1485

Question 137
A 6-month-old boy is seen in the emergency department with fever of up to 38.8 ºC rhinorrhea,
cough, tachypnea, and wheezing. He was born at term after an uncomplicated pregnancy and has
been exclusively breastfed. He started daycare 2 weeks ago when his mother returned to work as
a school teacher. The mother reports the onset of symptoms 3 days ago, with worsening of cough
and breathing difficulty today. He is still able to nurse, but not as vigorously as she expects. He
continues to have the expected number of wet diapers.
On physical examination, he is alert but clings to his mother. He appears adequately hydrated,
with tears in his eyes, capillary refill is 2 to 3 seconds, and skin turgor is normal. His heart rate is
130 beats/min respiratory rate is 40 breaths/min; room air oxygen saturation is 94% on pulse
oximetry. He has mild intercostal retractions no suprasternal retraction or nasal flaring. There is
diffuse expiratory wheezing and mildly prolonged expiration.
Of the following, the MOST appropriate management approach for this child is
A. chest radiography, albuterol nebulizer treatment, oral amoxicillin treatment
B. ensuring adequate hydration, counseling family on hand hygiene, providing follow-up
instructions
C. nasopharyngeal swab for viral antigens, 3% saline nebulizer treatment ensuring adequate
hydration
D. Oral prednisolone. oral amoxicillin, counseling family on adequate hydration, hand
hygiene

Correct Answer: B
The infant in the vignette has acute bronchiolitis, characterized by fever and acute respiratory
symptoms including cough and wheezing in a child younger than 2 years. The appropriate
intervention is supportive treatment and monitoring, according to the 2014 American Academy
of Pediatrics (AAP) guidelines for diagnosis, management, and prevention of bronchiolitis.
Bronchiolitis is a clinical diagnosis requiring no imaging or laboratory studies in the
uncomplicated case. This child, who was not premature, is more than 12 weeks old, has no
underlying heart or respiratory disease to put him at risk for poor outcome, represents the most
common presentation of bronchiolitis, and is at low risk for complications. Oxygen saturation
greater than 90% when awake, respiratory rate less than 50 breaths/min, mild but not severe
retractions, adequate hydration, and ability to feed by mouth all indicate that it is safe at this time
to treat him as an outpatient if the family is reliable and close follow-up is possible to monitor
respiratory distress and hydration status. Item C137 shows features of assessment of severity of
bronchiolitis. Infants and toddlers with mild disease can be appropriately treated at home; those
with moderate symptoms should be considered for hospitalization, and those with severe
symptoms should be hospitalized. Counseling of this family should include instructions for good
hand hygiene and tobacco smoke avoidance, as well as signs of concern regarding hydration and
respiratory distress.
Item C137: Bronchiolitis assessment features
Chest radiography is not helpful in the diagnosis or management or uncomplicated acute

bronchiolitis, nor are any other laboratory studies needed. The virus most often implicated is
respiratory syncytial virus, but detection or this or any of the other respiratory viruses (human
rhinovirus and human metapneumovirus are the next most common) does not change the
management or course of the illness.
There has been a great deal of controversy about the use of bronchodilators, inhaled hypertonic
saline with or without epinephrine, and oral or intravenous steroids. None of the appropriately
conducted studies has demonstrated a benefit with these therapies in the outpatient setting
Children with poor hydration and inability to meet feeding needs, oxygen saturation less than
90% when awake or 88% when asleep, respiratory rate greater than 70 breaths/min, or physical
signs of moderate to severe respiratory distress should be admitted to the hospital for supportive
care. Data suggest that hypertonic saline inhalation is effective in hospitalized children with
bronchiolitis and this is supported in the AAP guidelines. Antibiotic treatment of bronchiolitis is
not necessary without a clear indication of concurrent bacterial infection.
Multiple guidelines from other organizations generally agree with the AAP guidelines, but not
uniformly. In a review of 32 different published guidelines for bronchiolitis. Kirolos et al found
complete concurrence only on the lack of need imaging and laboratory studies (other than one
guideline that recommended blood gas analysis). There was much less agreement on
pharmacologic treatment, with complete against the use of montelukast, antibiotics,
corticosteroids, and antiviral agents. There was no agreement among the different guidelines
regarding the use of inhaled bronchodilators, hypertonic saline, or nebulized epinephrine. Only
one of the guidelines recommended routine use of chest physical therapy in the management of
bronchiolitis, and most did not address the issues of hand hygiene or isolation procedures.
PREP Pearls
• Bronchiolitis is diagnosed clinically in children younger than 2 years without underlying
predispositions who exhibit the typical constellation of signs and symptoms including
fever, rhinorrhea, cough, wheezing, and tachypnea.
• Multiple viruses may be implicated in bronchiolitis including respiratory syncytial virus
(RSV), human rhinovirus, human metapneumovirus; RSV is the most common.
• The management of bronchiolitis is supportive. There is no indication for antibiotics,
bronchodilators, or corticosteroids.

• Recognize the clinical findings associated with bronchiolitis
• Plan the appropriate management of bronchiolitis
Suggested Readings
• Reducing albuterol use in children with bronchiolitis. Pediatrics, doi:10.1542/peds.2019-
0306
• Bronchiolitis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:1797-1803
• A systematic review of clinical practice guidelines for the diagnosis and management of
bronchiolitis. J Infect Dis, doi:10.1093/infdis/jiz600
• Bronchiolitis: From practice guideline to clinical practice. Emerg Med Chin North Am,
doi:10.1016/j.emc.2017.12.006
• Bronchiolitis. Pediatr Rev, doi:10.1542/pir.2018-0260

Question 138
An infant is seen for a 4-week health supervision visit. She has been gaining weight
appropriately and breastfeeding well every 1 to 3 hours. She seems to respond when her parents
talk to her and looks at their faces. At times she crosses her eyes. She cries when she hears a loud
noise.
The girl's vital signs are normal for age. She is able to turn her head side to side while lying on
her stomach and can hold her chin up off the table. She opens her hands at rest and pulls them to
her mouth to self-soothe. The girl's eyes turn medially briefly when looking at the physician. She
began crying while the physician was talking with her parents and quickly calmed when held by
her mother.
Of the following, the MOST accurate description of this infant's developmental milestones is that
she is
A. delayed in fine motor skills
B. delayed in gross motor skills
C. delayed in social skills
D. developmentally normal for age

Correct Answer: D
The infant in the vignette is a developmentally normal, 4-week-old girl. In the newborn period
there should be some wakeful time, fixation on parents' faces, and calmness while being held. By
4 weeks, an infant’s social skills include the ability to self-soothe through methods such as
sucking on a hand or a thumb. They begin visually tracking parents or other faces, but may
experience eye-crossing for periods of time. At this age, infants show interest in objects that are
8 to 12 inches away, especially those that are black and white.
Newborns should respond to visual and auditory stimuli. A hearing screen should be completed
on newborns prior to discharge from the nursery. At 4 weeks of age the infant's language skills
should include making short vowel sounds, quieting to a parent's voice, and startling to loud or
unexpected sounds. A difference in cry based on needs such as feeling hungry, hurt, lonely
emerge at this age. They respond to their parent's voice and cry or grimace when upset. By 3 to 5
days after birth the neonate should be developing the ability to move the head side to side when
lying prone. By 4 weeks of age, gross motor skills include the ability to hold the chin up while
prone, and the arms and legs begin to move together. This is a great age to encourage “tummy
time” several times daily. Regarding fine motor skills, in the newborn period the hands are
clenched at rest and grasp objects placed in the hand. At 4 weeks of age, the infant should begin
to hold the hands slightly open while at rest.
At the 4-week health supervision visit, anticipatory guidance includes avoiding screen time; and
for bed or naptime, placing the infant in the crib or bassinet on their back while slightly awake,
and encouraging parents to consider the use of a pacifier. Discussion should include what to do
when an infant is crying including soothing methods such as stroking the infant's head,
swaddling, gently rocking, or making soothing sounds. It is important to remind parents to never
shake an infant; if a parent break is needed, they can place a fussy infant in a safe environment,
such a crib
PREP Pearls
• Newborns should respond to their parent's voices.
• Four-week-old infants track their parent's face and look at objects 8 to 12 inches away
• Brief intermittent, eye-crossing is normal at 4 weeks of age.

• Evaluate the developmental progress/status of a neonate through the first four weeks after
birth
Suggested Readings
• Bright Futures: Guidelines for Health Supervision of Infants Children and adolescents 4th
ed; 2017:26-30
• www.healthychildren.org. Developmental milestones: 1 month.
https://www.healthychildren.org/English/ages-stages/baby/Pages/Developmental-
Milestones-1-Month.aspx

Question 139
A 16-year-old adolescent boy is seen in the emergency department with progressive exertional
shortness of breath, orthopnea, and persistent cough. He has constant sternal chest pain that
radiates to his back. His symptoms began 2 weeks ago with a fever and cough. At that time, he
was diagnosed with pneumonia and an asthma exacerbation. He completed 10-day Course of
antibiotics and appropriate asthma management; however, his respiratory symptoms have
continued to worsen. The adolescent slept in an upright chair overnight due to respiratory
discomfort; when he woke up this morning his face looked a bit swollen. He reports a 10-1b
weight loss over the past month. There has been no change in his diet. He has had to change his
sweat-soaked T-shirt during the night several times recently. He has not been able to participate
in usual sports lately due to his symptoms.
His vital signs include a temperature of 38.40 º C, heart rate of 140 beats/min, respiratory rate of
30 breaths/min, blood pressure of 140/90 mm Hg, and oxygen saturation of 93% on room air by
pulse oximetry. He is awake and alert, well nourished, and in apparent respiratory distress. His
face and neck look swollen. His lung sounds are decreased on the right side; the left side is clear
with good aeration. He has some intercostal retractions, tenderness to palpation of the sternum,
and a normal Sl S2 sinus rhythm. The boy is unable to lie flat on his back for an abdominal
examination due to difficulty breathing. There is no palpable cervical, axillary, or inguinal
lymphadenopathy. He is at sexual maturity rating 4 with testes descended bilaterally and no
testicular masses.
Chest radiography shows a widened mediastinum and near complete opacification of the right
hemithorax with shifting of the heart, mediastinum, and trachea to the left (Item Q139)
Of the following, the BEST next step in management of this patient is

A. admission to the pediatric floor
B. admission to the pediatric intensive care unit
C. chest and abdomen magnetic resonance imaging
D. surgical consultation to obtain biopsy

Correct Answer: B
The adolescent in the vignette is in significant respiratory distress with radiologic evidence or
pneumoniae, effusion, and tracheal deviation, and clinical findings suggestive of superior vena
cava compression (facial edema). His signs and symptoms are likely due to a mediastinal mass.
This is a medical emergency. The best next step in the boy’s management is to support his
breathing and avoid respiratory compromise. Any additional investigations and intervention
should take place after his condition is stabilized.
Admission to a regular pediatric floor would be inappropriate. He requires intensive care-level

respiratory support, such as a high-now nasal cannula, and his condition raises concern that he
may be progressing toward the need for intubation.
The adolescents head and neck swelling are suggestive of superior vena cava (SVC) syndrome,
which can result from compression of the SVC by a mediastinal mass or a thrombus. Computed
tomography or magnetic resonance imaging are the best imaging modalities to make this
diagnosis. However, the boy is in significant respiratory distress and currently unable to lay flat.
It is of utmost importance to support his respiratory status first, and obtain additional imaging
when it is clinically safe to do so. Once SVC syndrome is confirmed, anticoagulation must be
started (which must be held prior to any biopsy or invasive procedure).
In the setting of a mediastinal mass with concern for lymphoma or leukemia, the pediatric
oncologist may recommend steroid administration in order to shrink the mass and improve the
child's respiratory status. Biopsy of the mass should be performed prior to starting steroids, if
possible or within 72 hours of starting steroid treatment.
PREP Pearls
• A mediastinal mass may cause significant respiratory compromise and must be treated as
a medical emergency.
• Superior vena cava syndrome is a medical emergency that may occur in the setting of a
mediastinal mass; computed tomography or magnetic resonance imaging are the imaging
modalities of choice to make the diagnosis when the child's condition is stable.
• Once a diagnosis of superior ena cava syndrome is confirmed, anticoagulation should be-
started.

• Recognize the clinical findings associated with a chest mass
• Recognize the need for immediate evaluation of a child with a chest mass who is at risk
of acute-respiratory failure
Suggested Readings
• Pediatric solid tumors of infancy: Pediatr Rev. doi:10.1542/pir.2017-0057
• Pediatric lymphoma. Pediatr Rev. doi:10.1542/pir.2016-0152

Question 140
A 3-week-old neonate bom at 35 weeks' gestation is admitted to the hospital with a 2-day history
of fever and decreased oral intake. In the emergency department, his vital signs reveal a
temperature of 39 º C, heart rate of 150 beats/min, blood pressure of 52/36 mm Hg, and oxygen
saturation of 92% in room air. He appears lethargic and has a capillary refill time lasting 2 to 3
seconds. The remainder of the neonate’s physical examination findings are unremarkable.
Treatment is initiated with intravenous cefepime and acyclovir. Within 6 hours, his blood culture
is reported positive for Streptococcus agalactiae.
Of the following, the MOST likely diagnosis for this is

A. meningitis
B. necrotizing enterocolitis
C. pneumonia
D. urinary tract infection

Correct Answer: A
The neonate in the vignette has meningitis caused by late-onset group B Streptococcus (GBS)
infection (Streptococcus agalactiae). Late-onset GBS infection presents after the first week of
life. The neonate's physical examination findings support the diagnosis of meningitis.
Infection with GBS is a significant cause of morbidity and mortality among neonates in the
United States. Universal screening of pregnant women for GBS between 35 and 37 weeks of
gestation with intrapartum prophylaxis for mothers colonized with GBS has significantly
decreased the rate of early-onset disease (Item C140A). However, the rate of late-onset disease
has remained constant. Early-onset GBS infection is thought to result from perinatal exposure to
maternal GBS colonization. The origins of late-onset GBS infection are less clear, but may be
related to breast milk feedings, environmental exposures, or maternal colonization. Premature
birth is a risk factor for both early- and late-onset GBS Infection.
Item C140A: Incidence of early- and late-onset, invasive group B streptococcal (GBS) disease–active
bacterial core surveillance areas, 1990–2008, and activities for prevention of GBS disease. AAP,
American Academy of Pediatrics; ACOG, American College of Obstetricians and Gynecologists.
(Adapted from Jordan HT, Farley MM, Craig A, et al: Revisiting the need for vaccine prevention of late-
onset neonatal group B streptococcal disease, Pediatr Infect Dis J 27:1057–1064, 2008.)
The clinical presentation of early- and late-onset GBS infection varies. Asymptomatic
bacteremia may be seen in both. Associated findings in early- and late-onset GBS are listed in
Item C140B.
Item C140B
Suspected rate-onset GBS should be treated empirically with ampicillin (or cefotaxime) and an
aminoglycoside. Antibiotic coverage should be narrowed to penicillin G once GBS infection is
confirmed. Bacteremia should be treated for 10 days. Uncomplicated meningitis should be
treated for a minimum of 14 days. Soft tissue infection, osteomyelitis, and septic arthritis may
require treatment for 3 to 4 weeks. Group B Streptococcus meningitis is associated with long-
term complications such as cerebral palsy, cortical blindness, and teaming disabilities.
For a late preterm neonate, necrotizing enterocolitis would be an unusual condition without
findings suggestive of abdominal pathology, such as tenderness on palpation. If the neonate in
the vignette had presented in the first week after birth, GBS Pneumonia would have been the
most likely diagnosis, but not in the case of late-onset GBS. Group B Streptococus infection
rarely causes urinary tract infection.
PREP Pearls
• Late-onset group B Streptococcus infection presents between 7 and 89 days after birth.
• Meningitis is commonly seen in neonates and infants with late-onset group B
Streptococcus
• Premature birth is a risk for early- and late-onset group B Streptococcus infection.

• Recognize the major clinical features associated with group B Streptococcus infection,
and manage appropriately
• Plan the appropriate management of an infant born to a mother with a positive culture for
group B Streptococcus
• Understand the epidemiology of Streptococcus agalactiae
Suggested Readings
• Group B streptococcal infections. Red Book: 2021-2024 Report of the Committee on
infectious Diseases. 32nd ed; 2021:707-712.
• Group B Streptococcus, Nelson Textbook of Pediatrics 21st ed., 2020:1450-1455
• Committee on Fetus and Newborn; Committee on infectious Diseases. Management of
infants at risk for group B 2019: doi: 10.1542/peds.2019-1881
• Group B streptococcal infections. Pediatr Rev. doi:10.1542/pir.2016-0127

Question 141
An 8-year-old boy is brought to the emergency department with a 2-day history of headache and
nausea. He has no diarrhea or vomiting. He fell 5 days ago while riding his bicycle without a
helmet; he had no loss of consciousness. On physical examination, the boy's heart rate is 80
beats/min, respiratory rate is 16 breaths/min, and blood pressure is 110/70 mm Hg, He is sleepy,
his mucous membranes are moist, and his capillary refill time is less than 2 seconds. The
remainder of his physical examination findings are normal.

Blood
Blood urea nitrogen 14 mg/dl (5 mmol/L)
Serum osmolality 255 mOsm/kg
Urine
Leukocyte esterase Negative
Nitrite Negative
Blood Negative
Protein Negative
Urine osmolality 450 mOsm/kg
Urine sodium 55 mEq/L (55 mmol/L)
(reference range, 20-40 mEq/L)
Of the following, the boy’s MOST likely diagnosis is

A. Central diabetes insipidus
B. Hyponatremic dehydration
C. Psychogenic polydipsia
D. Syndrome inappropriate secretion of antidiuretic hormone

Correct Answer: D
The boy in the vignette has Syndrome of inappropriate secretion of antidiuretic hormone
(SIADH). He has a history of head trauma and on physical examination is euvolemic. His serum
sodium level, low serum osmolality, inappropriately high urine osmolality, and high urine
sodium level favor a diagnosis of SIADH.
The syndrome of inappropriate secretion of antidiuretic hormone is characterized by excess

secretion of antidiuretic hormone leading to impaired water excretion. This excess antidiuretic
hormone results in concentrated urine and reduced urine volume, leading to water retention and
hyponatremia. Common causes of SIADH in children are shown in Item C141A and include
traumatic brain injury, meningitis pneumonia, and some medications.
Item C141A: Common causes of SIADH in children
Signs and symptoms of SIADH are dependent on the rate of development and severity of the
hyponatremia. Early symptoms include nausea and malaise. A severe and acute worsening of
hyponatremia presents with headaches, lethargy, and seizures. Children with chronic
hyponatremia may remain asymptomatic. Children with SIADH are euvolemic, therefore, an
assessment of volume status should be performed to include examination of the mucous
membranes, skin turgor, and capillary refill, as well as measurement of weight and blood
pressure.
Laboratory testing in children with SIADH shows:

• Hyponatremia (serum sodium <135 mEq/L [135 mmol/L])
• Hypo-osmolality (serum osmolality <280 mOsm/kg)
• Inappropriately concentrated urine (urine osmolality >100 mOsm/kg)
• High urine sodium concentration (>40 mEq/L [40 mmol/L])

Blood urea nitrogen, serum creatinine, blood glucose, and serum cortisol levels, as well as
thyroid function tests, are normal in SIADH.
Management of SIADH includes treatment of the underlying cause (e.g., meningitis, pneumonia,
removal of the offending drug) and correction of the hyponatremia. Fluid restriction is the
primary treatment. Additional therapy with sodium supplementation (oral or intravenous) and/or
loop diuretics (e.g., furosemide) may be indicated.
The fluid and electrolyte abnormalities seen in the other response choices are not seen in the
child in the vignette. Central diabetes insipidus presents with polyuria, polydipsia,
hypernatremia, and dilute urine. Hyponatremic dehydration is important to consider in the
differential diagnosis of SIADH because hyponatremia and concentrated urine are seen in both
conditions. Hyponatremic dehydration is a state of volume depletion with low urine sodium level
(<10 mEq/L); blood urea nitrogen and creatinine levels may be high. Psychogenic polydipsia
presents with a history of excessive water intake, polyuria, hyponatremia, dilute urine (urine
osmolality <100 mOsm/kg), and low urine sodium level (<10mEq/L). Item C141B displays the
findings in each of these conditions.
Item C141B: hyponatremia
PREP Pearls
• Common causes of syndrome of inappropriate secretion of antidiuretic hormone in
children include traumatic brain injury, meningitis, pneumonia, and some medications
• Children with syndrome of inappropriate secretion of antidiuretic hormone are euvolemic
and have hyponatremia, hypo-osmolality, inappropriately concentrated urine, and a high
urine sodium concentration.
• The primary treatment for syndrome of inappropriate secretion of antidiuretic hormone
secretion is fluid restriction.

MOCA-Peds. Objective
• Diagnose and manage acute head injury.

• Differentiate SIADH from hyponatremic dehydration
• Recognize disease conditions and medications associated with SIADH
• Recognize the role of head trauma in the development of SIADH
• Recognize the clinical and laboratory features associated with SIADH, manage
appropriately
Suggested Readings
• Syndrome of inappropriate secretion of antidiuretic hormone and hyponatremia. Pediatr
Rev, doi:10.1542/pir.2016-0165
• Fluids, electrolytes, and acid-base composition. American Academy of Pediatrics
Textbook of Pediatric Care. 2nd ed; 2017:419-433
• Hyponatremia. Pediatr Rev, doi:10.1542/pir.34-9-417

Question 142
A 4-year-old girl is seen in the clinic for a routine health supervision visit. Her mother notes that
in some photographs the girl seems to have a "lazy eye." Her medical history is uncomplicated,
and she has had normal growth and development. On physical examination, the girl's left eye
appears to be slightly medially deviated, and the corneal light reflex on the left is off center. Her
red light reflexes are normal. Strabismus is suspected.
Of the following, the examination finding that BEST supports the girl's suspected diagnosis is
when her
A. left eye is covered, her right eye moves laterally
B. left eye is covered, her right eye moves medially
C. right eye is covered. her left eye moves laterally
D. right eye is covered, her left eye moves medially

Correct Answer: C
The parent of the girl in the vignette is concerned about a "lazy eye" and the appearance of the
medial deviation of her left eye at rest. This finding could be caused by strabismus (i.e., eye
misalignment) or pseudo strabismus (a properly aligned eye that appears misaligned, often
because of a wide nasal bridge or prominent epicanthal folds). It is important to differentiate
these diagnoses. Children with strabismus must be referred to ophthalmology for evaluation and
treatment to optimize their visual outcome, whereas children with pseudo strabismus do not
additional evaluation or treatment.
The examination finding that best supports a diagnosis of strabismus (manifest esotropia of the
left eye) for this girl is that when her right (fixating) eye is coveted, her left eye moves laterally.
Because her left eye was inwardly deviated at rest, it would not be expected to move further
medially when the right eye is covered. In addition, her fixating (right) eye would not be
expected to move either laterally or medially when the left (affected) eye is coveted.
Strabismus is classified as:

• Latent (“phoria”): Not present under normal conditions; only revealed with maneuvers
such as the cover-uncover test
o Esophoria (medial misalignment)
o Exophoria (lateral misalignment)
• Manifest (“tropia”): Constantly present
o Esotropia (medial misalignment)
o Exotropia (lateral misalignment)
Manifest strabismus can result in amblyopia, a loss of visual acuity caused by a disruption of the
cortical visual pathway between the eye and brain in early childhood. Other causes of amblyopia
include congenital cataracts, retinoblastoma, anisometropia, and high refractive error. Amblyopia
can be prevented or treated when the presence or risk factor is promptly in young children.
Treatment after age 7 to 9 years is rarely successful.
Several physical examination maneuvers help identify strabismus. If findings are abnormal,
referral for ophthalmologic evaluation is indicated:
• Corneal light reflex test: Direct a penlight at the child while the child is looking straight
ahead. If eye alignment is normal, the light reflects symmetrically from the center of each
pupil.
• Cover-uncover test: Have the child fixate on an object such as a toy or your nose, and
observe the positioning of each eye at rest. Cover and then uncover each eye in sequence,
while observing the movements of each eye.
When children have strabismus, eye misalignment causes incongruent visual input to the brain
(i.e., “double vision”); the brain learns to “ignore” input from the affected eye. The unaffected
eye will routinely fixate on objects. When the fixated (unaffected) eye is covered, the misaligned
eye will adjust to then fixate on the object of focus (laterally if it was medially misaligned or
medially if it was laterally deviated). This examination finding is indicative of manifest
strabismus. Latent strabismus can be identified by observing the movement of an affected eye as
the cover is removed; the affected eye “drifts” to its natural position when covered, and then
moves to reestablish fusion with the fixated (unaffected) eye when the eye is uncovered (Item
C142A).
Item C142A: Screening tests for abnormal alignment. (A through D) The cover test. (A) On simple
observation, one eye (the tested eye, which is the patient's left eye in this illustration) appears to deviate.
(B) The uncovered (tested) eye is observed for movement into alignment as the other eye is covered (in
this illustration, an exotropia is exposed). This movement into alignment can come from any direction and
is generally reproducible on repeat examinations. (C) When the opposite eye is uncovered, the tested eye
is observed to return to its original location. (D) When the test eye is the normal eye (which is the
patient's right eye in this illustration), it does not move when the opposite eye is covered or uncovered. (E
through G) The cover-uncover test. (E) The tested eye (which is the patient's left eye in this illustration) is
covered. On simple observation, before it is covered, the eye appears to be in alignment. (F) The tested
eye is covered for a few seconds. (G) As the cover is rapidly removed, the tested eye is observed for
movement back into alignment (in this illustration, an exophoria is exposed).

The corneal light reflex test and cover-uncover test should be part of routine vision screening for
infants and young children (Item C142B).
Item C142B: (A) Normal corneal light reflex in normally aligned eyes. (B) Pseudostrabismus. The child
appears to be converging his left eye toward his nose because of the enlarged epicanthal folds; however,
note that the light reflex is symmetric in each eye. (C) True strabismus. Note the light reflex is central in
the fixating (the child's right) eye, but is displaced in the nonfixating (the child's left) eye.

In a corneal light reflex test, the child's attention is attracted to a target (a light or a brightly colored
object), while a light is directed at the child's eyes. (A) In normally aligned eyes, the light reflex will be in
the center of each pupil. (B) Corneal light reflex in esotropia. (C) Corneal light reflex in exotropia. (D)
Corneal light reflex in hypertropia.
PREP Pearls
• Conditions that disrupt the ocular-cortical pathway (e.g., manifest strabismus, congenital
cataracts, retinoblastoma, anisometropia. or high refractive error) can lead to amblyopia if
not identified and treated promptly.
• The corneal light reflex and cover-uncover tests should be performed as part of routine
vision screening to identify strabismus.

• Understand which conditions can be detected by periodic ophthalmoscopic examinations
• Plan the appropriate evaluation of vision in patients of various ages
• Understand the importance of vision screening, including in newborn infants
Suggested Readings
• Childhood eye examination, Am Fam Physician.
https://www.aafp.org/afp/2013/0815/p241.html
• Ophthalmology. Atlas or Pediatric Physical Diagnosis. 5th ed. 2007; 713-754.
• Pediatric vision screening. Pediatr Rev, doi:10.1542/pir.2016-0191
• Amblyopia and strabismus. American Academy of Pediatrics Textbook of Pediatric Care.
2nd ed. 2017; 1710-1716

Question 143
A previously healthy, 16-year-old adolescent girl is brought to the emergency department with
fever, rash, vomiting, and confusion over the past 3 hours. She has no history of sick contacts,
recent travel, tick or mosquito bites, trauma, or drug use. She had not received any childhood
and adolescent immunization because of philosophical reasons. On physical examination she is
critically ill with a temperature of 39.6 º C, heart rate of 174 beats/min, respiratory rate of 20
breaths/min, and blood pressure of 74/47 mm Hg. Examination of the skin shows a non-
blanching rash on her face, trunk, and extremities (Item Q143A and Item Q143B). Her hands
and feet are cool, with a capillary refill time or 4 seconds. She is difficult to arouse but moves all
her extremities on painful stimuli with occasional eye opening. The rest of the physical

Serum sodium 128 mEq/L (128 mmol/L)
Cerebrospinal fluid 111
White blood cell count 7/µL
Lymphocytes 96%
Monocytes 4%
Red blood cell count 440/µL
Protein 5 mg/dL
Glucose concentration 65 mg/dL (3,6 mmol/L)
Gram staining of CSF did not reveal any organisms. Blood and cerebrospinal fluid culture
specimens are obtained, and empiric antimicrobial therapy is initiated.
Of the following, the MOST likely pathogen causing this girls illness is
A. Hemophilus influenzae type b
B. herpes simplex virus
C. Neisseria meningitidis
D. Rickettsia rickettsii

Correct Answer: C
The clinical presentation of the febrile, ill-appearing adolescent girl described in the vignette
with a rapid onset of a petechial or purpuric rash and progressive worsening course is suggestive
of invasive-meningococcal infection. Rocky Mountain spotted fever (RMSF) must be included in
the differential diagnosis in this febrile, ill appearing adolescent with a petechial/purpuric rash.
However, the petechial rash in RMSF typically occurs several days after onset of a febrile
illness. Other common invasive infections to consider in this setting include bacterial sepsis
caused by group A Streptococcus, Staphylococcus aureus, and Streptococcus pneumoniae.
Hemophilus influenzae type b is a leading cause of bacteremia and meningitis among
unvaccinated children younger than 5 years, with a peak incidence seen from 6 to 18 months of
age. In immunocompetent older children and adolescent, herpes simplex virus (HSV) can
manifest as encephalitis; however, sepsis syndrome caused by HSV is more common in neonates
as part of disseminated Infection.
Neisseria meningitidis is a predominant cause of sepsis and bacterial meningitis in adolescents

and young adults. The peak age-specific incidence of infection is reported in infants followed by
adolescents and young adults aged 16 to 21 years and adults aged 65 years or older. Although
sporadic infections account for 98% of invasive meningococcal disease, outbreaks and large
epidemics can occur.
Neisseriae meningitidis is a gram-negative diplococcus classified into 13 serogroups depending

on the capsular type. In the United States, vaccine-preventable serogroups D, C, Y, or W account
for approximately 90% of cases in adolescents and young adults whereas serogroup B accounts
for 66% of cases in infants and children younger than 60 months. Neisseria meningitidis is a
strictly human microorganism and highly communicable. Person-to-person transmission occurs
via droplets secreted from the upper respiratory tract. Risk factors for increased colonization
rates include crowded settings, smoking exposure, and associated upper respiratory tract
infection. Fewer than 1% of carriers become symptomatic.
Most individuals who develop invasive meningococcal disease are previously healthy without
recognized risk factors. Individuals complement deficiencies (e.g., C5-C9, properdin, factor H,
or factor D) and functional or anatomic asplenia (2 to 54 years of age) experience higher rates of
invasive and recurrent disease.
The incubation period for invasive meningococcal disease ranges from 1 to 10 days but most
cases occur within 4 days of exposure. Invasive syndromes include sepsis with or without
meningitis, pneumonia, and septic arthritis. Meningitis is the most frequent manifestation (50%
of cases). During the early stage of the disease, symptoms and signs of meningococcal disease
(such as fever, malaise, vomiting) can be nonspecific and difficult to distinguish from common
viral infections. Meningococcemia (35% to 40% of cases) is characterized by a rash (macular or
maculopapular rash which progresses rapidly to petechiae or purpura within hours), shock,
disseminated intravascular coagulation, vascular damage, and multiorgan failure. The clinical
course of meningococcal sepsis is rapidly progressive with high fatality rates (10% to 15%)
despite treatment. Deaths can occur within 24 hours of symptom onset. Among survivors of
meningococcal sepsis and meningitis, 10% to 11% suffer from sequelae such as hearing loss,
motor and cognitive disabilities, blindness, or extremity amputations and scarring.
Confirmation of invasive meningococcal disease is based on a clinically compatible presentation
with isolation of the pathogen in culture from a sterile site (e.g., blood, cerebrospinal fluid, skin
scraping of petechial or purpuric rash) or detection of bacterial nucleic acid in sterile body fluid
site using multiplex polymerase chain reaction. Treatment with intravenous ceftriaxone or
cefotaxime should be rapidly initiated for suspected meningoccal infection. The recommended
duration of antibiotic therapy for meningoccal sepsis and meningitis is 5 to 7 days. Hospitalized
patients with suspected meningococcal disease should be isolated using droplet precautions until
24 hours after starting appropriate antibiotic therapy.
Post-exposure chemoprophylaxis with rifampin in children and ceftriaxone or ciprofloxacin in

adults is recommended within 24 hours of exposure to prevent the development of secondary
cases among high-risk close contacts, irrespective of their vaccination status (Item C143A and
Item C143B).
Item C143A and Item C143B
Invasive meningococcal disease is a vaccine-preventable disease. Routine immunization with

quadrivalent meningococcal conjugate vaccine (MCV4) is recommended for adolescents 11
through 12 years of age followed by a booster dose at 16 years of age. Adolescents who receive
the first dose at age 13 through 15 years should receive a booster dose at age 16 through 18

years. A booster dose is not recommended for adolescents who receive their first dose of
meningococcal conjugate at or after 16 years of age. Administration of serogroup B
meningococcal vaccines is a consideration for immunocompetent individuals aged 16 through 23
years.
The MCV4 vaccine is recommended for all children 2 months of age and older and adults who
are at increased risk for invasive meningococcal disease. In addition, serogroup B meningococcal
vaccine is recommended for people 10 years of age and older who are at increased risk for
meningococcal disease and in the setting of serogroup B meningococcal disease outbreak.
PREP Pearls
• Meningococcemia must be included in the differential diagnosis in any febrile, ill-
appearing child or adolescent with a petechial or purpuric rash.
• Most individuals who develop invasive meningococcal disease are previously healthy
without recognized risk factors.
• The clinical course of meningococcal sepsis is rapidly progressive with high fatality rates
despite treatment.

• Plan appropriate prophylaxis for individuals exposed to Neisseria meningitidis
• Understand the epidemiology of Neisseria meningitidis
• Understand which patients are at increased risk of invasive and recurrent meningococcal
disease (e.g., asplenia, terminal complement component)
• Plan appropriate management for a patient with meningococcal disease
• Understand the significance of purpura in a febrile child
• Recognize the major clinical features associated with Neisseria meningitidis infection
Suggested Readings
• American Academy of Pediatrics. Committee on infectious Diseases. Updated
recommendations on the use of meningococcal vaccines. Pediatrics.
doi:10.1542/peds.2014-1383
• Meningococcal infections. Red Book: 2021-2024 Report of the Committee on infectious
Diseases. 32nd ed; 2021:519-531 .
• Meningococcemia. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2899-2905
• Use of serogroup B meningococcal vaccines in adolescents and young adults:
recommendations of the Advisory Committee on Immunization Practices, 2015,
doi:1015585/mmwr.mm6441a3
• Meningococcal disease. Pediatr rev, doi:10.1542/pir.2016-0131

Question 144
A 3-year-old boy is brought to the emergency department for lack of urine output for 12 hours.
He has had diarrhea and vomiting for 3 days and decreased oral intake for 1 day. On physical
examination, the boy’s temperature is 37.5 º C, heart rate is 140 beats/min, respiratory rate is 20
breaths/min and blood pressure is 82/40 mm Hg. He is lethargic. His mucous membranes are dry,
skin turgor is reduced, and refill time is greater than 3 seconds, The remainder of his physical
Of the following, the MOST appropriate intravenous fluid to administer to this boy is
A. 0.45% normal saline, 20 mL/kg
B. 0.9% normal saline, 20 ml/kg
C. 5% albumin, 10 ml/kg
D. Ringer lactate, 10 mL/kg

Correct Answer: B
The boy in the vignette has severe dehydration and oliguria secondary to gastroenteritis. The
most appropriate initial intravenous fluid to administer is 20 ml/Kg of 0.9% normal saline to
quickly restore intravascular volume.
Oliguria or decreased urine output is a common sign of acute kidney injury. Oliguria is defined
as urine output less than 0.5 mL/kg per hour for 24 hours in an infant or less than 500 mL/day in
older children and adults. Oliguria results from a decrease in intravascular volume or
hypotension (prerenal), intrinsic renal injury (renal), or obstruction to the flow of urine
(postrenal). The most common cause of oliguria in children is prerenal acute kidney injury
secondary to dehydration.
The first step in the treatment of prerenal oliguria is to restore intravascular volume. Intravenous
fluid therapy is determined after a detailed assessment of volume status using body weight, skin
turgor, capillary refill, and the presence or absence of hypotension, tachycardia, labored
respiration, and peripheral edema. An initial 20 mL/Kg fluid bolus of isotonic crystalloid fluid
(e.g., 0.9% normal saline or Ringer lactate) should be given rapidly in the case of severe
hypovolemia. Normal saline is commonly used; however, Ringer lactate may be preferred in
septic shock. A 20 mL/kg bolus of isotonic fluid may be repeated 2 or 3 times, depending on
improvement in vital signs and urine output. Additional fluid boluses should be avoided if
peripheral or pulmonary edema develops. A smaller fluid bolus (10 mL/kg) may be given over a
longer period (e.g., 60 minutes) for prerenal oliguria with underlying cardiac-disease and for
those at risk for fluid overload.
Volume status should be reassessed after administration of each fluid bolus. Vasopressors are
indicated if hypotension is present after adequate fluid resuscitation. A trial of furosemide is
indicated if oliguria persists after blood pressure returns to normal. Intravenous fluids can be
restricted to insensible fluid losses (400-500 mL/m2 per day) along with replacement of each
milliliter of urine output to maintain euvolemia in cases of persistent oliguria.
Ringer lactate is an isotonic fluid that would appropriate to administer to the boy in the vignette.
However, because he has no features of fluid overload, 10 to ml/kg would be inadequate. Normal
saline 0.45% is a hypotonic fluid and is not recommended for fluid resuscitations. Albumin 5%
is a colloid that may be used when there is no improvement with a crystalloid bolus and in
children with low serum albumin; it is not indicated for initial fluid resuscitation for the boy in
the vignette.
PREP Pearls
• The initial step in treatment of prerenal oliguria is to restore intravascular volume.
• An isotonic crystalloid fluid bolus (e.g., 0.9% normal saline or Ringer lactate) should be
given at 20 mL/kg rapidly in cases of severe hypovolemia.
• A 10 mL/kg isotonic fluid bolus may be given slowly for prerenal oliguria with
underlying cardiac disease and for those at risk for fluid overload.

• Plan the management of fluid depletion in a patient with acute renal failure
• Understand the changing fluid requirements in a patient who has severe oliguria
Suggested Readings
• Oliguria and anuria. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed; 2017; 2423-2427
• Dehydration: isonatremic, hyponatremic, and hypernatremic recognition and
management. Pediatr Rev, doi: 10.1542/pir.36-7-274
• Acute kidney injury. Pediatr Rev, doi:10.1542/pir.35-1-30

Question 145
A 36-day-old infant is seen in the emergency department after an episode of bloody emesis. The
infant was born full term without any complications. She is breastfed and gaining weight
appropriately. She was afebrile and in good health prior to this event. Since birth, she has had
frequent spit-ups after feeding, but never any blood in her emesis previously. Today, her emesis
was noted to be milk colored with streaks of red and what appeared to be small blood clots. On
arrival to the emergency department, the infant has a temperature of 37.9 º C, heart rate of 146
beats/min, respiratory rate of 38 breaths/mint and blood pressure of 90/68 mm Hg. Physical
examination reveals a well-developed infant who is awake and alert. Her abdomen is soft,
nontender, and nondistended without hepatomegaly or splenomegaly, the remainder of her
examination findings are unremarkable. A bedside gastric occult blood test on aspirated stomach
content confirms the presence of brood.
Of the following, the MOST appropriate next step in this infant's evaluation is
A. Apt-Downey test on aspirated stomach contents
B. supine and left lateral decubitus radiograph of the abdomen
C. ultrasonography of the abdomen
D. upper gastrointestinal tract radiography with contrast

Correct Answer: A
The most appropriate next step in the evaluation of the infant in the vignette is to perform an
Apt-Downey test on aspirated stomach contents. The infant has evidence of upper
gastrointestinal (GI) bleeding. She is clinically stable with vital signs. Many substances such as
food, medications, or dyes can mimic hematemesis. Therefore, the first step in evaluation of red
or pink emesis is to determine if blood is actually present with a test that uses the peroxidase
activity of hemoglobin to catalyze a color change on a test card. Once the presence of blood
confirmed positive, the most appropriate next step is to determine if the source of the blood is the
infant or her mother. Maternal blood may be swallowed during the birthing process or when
breastfeeding if the mother as bleeding nipples. These sources can be distinguished by the Apt-
Downey test, which detects fetal hemoglobin. A positive Apt-Downey test result indicates that
the source of is the neonate/infant, a negative test indicates maternal origin. The Apt-Downey
test can be used on infants up to age 8 weeks; thereafter the reliability of the test decreases as the
proportion of fetal hemoglobin decreases.
If the Apt-Downey test performed on this infant's stomach contents reveals a negative result, no
further evaluation or treatment is indicated. Therefore, abdominal ultrasonography, abdominal
radiography, or upper GI radiography with contrast is not indicated prior to determining whether
the source of the blood is fetal or maternal. If the Apt-Downey test result is positive, further
evaluation is warranted.
Upper GI bleeding is define as the presence of bleeding proximal to the ligament of Treitz; this
classically presents with hematemesis. The blood color may be bright red (indicating fresh blood,
brisk bleeding, or active bleeding) or coffee-ground (indicating slower bleeding, or older,
oxidized blood products). The initial evaluation of a child with upper GI bleeding should include
assessment of cardiovascular stability and initiation of resuscitation, if indicated. Item C145 lists
the causes of upper GI bleeding in various age groups.
The evaluation for sources of upper GI bleeding may include laboratory tests, imaging studies,
and endoscopy. Laboratory evaluation includes a complete blood count to evaluate for evidence
or infection, anemia, or platelet dysfunction; liver function tests; and coagulation studies. A type
and screen should be obtained in case the need for emergency blood transfusion arises.
Abdominal radiography may be used to assess for foreign body, congenital abnormalities,
pneumatosis intestinalis, and evidence of bowel obstruction or perforation. Abdominal
ultrasonography may be performed to identify liver disease or evidence of portal hypertension
that may be contributing to esophageal variceal formation. Upper endoscopy can both identify
and treat the source of upper GI bleeding. Other diagnostic testing may be indicated based on the
clinical presentation and suspicion for specific sources of upper GI bleeding: this may include
capsule endoscopy, computed tomography, or radionucleotide imaging.

Item C145: causes of upper GI bleeding in various age groups.
PREP Pearls
• For neonates and infants (up to age 8 weeks) with hematemesis, the Apt-Downey test
differentiates whether the source of the blood is the mother or the neonate/infant.
• Common causes of upper gastrointestinal bleeding in children vary by age group.
• Evaluation of upper gastrointestinal bleeding depends on the severity and suspected
source of bleeding; this may include laboratory testing, imaging studies (e.g., abdominal
ultrasonography, abdominal radiography, or computed tomography) and upper
endoscopy.

• Recognize the clinical features associated with upper gastrointestinal bleeding
• Formulate a differential diagnosis for coffee-ground material in vomitus
• Plan the appropriate evaluation of upper gastrointestinal bleeding
• Formulate an age-appropriate differential diagnosis for vomiting bright red blood
• Formulate a differential diagnosis for vomitus that tests positive for occult blood
Suggested Readings
• Gastrointestinal hemorrhage. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed;2017; 1543-1550
• Gastrointestinal bleeding in infants and children. Pediatr Rev, doi:10.1542/pir.29-2-39
• Gastrointestinal bleeding. Pediatr Rev, doi: 10.1542/pir.35-6-243

Question 146
A neonate born at 29 weeks’ gestation is admitted to the neonatal intensive care unit. His birth
weight is 1,150 g, Maternal hepatitis B surface antigen is positive. He appropriately received
hepatitis B immune globulin.
Of the following, the BEST time for this neonate to receive the first dose of the hepatitis B
vaccine is
A. at 24 hours of age
B. at 1 month of age
C. at the time of discharge
D. within 12 hours of birth

Correct Answer: D
The neonate in the vignette should receive the hepatitis B vaccine within 12 hours of birth to
protect against prenatal transmission, because his mother's hepatitis B surface antigen is positive.
Delaying vaccination longer than 12 hours greatly increases the risk of hepatitis B infection.
Administration of hepatitis B vaccine at 24 hours of age is appropriate for infants born weighing
at least 2 kg whose mothers test negative for hepatitis B surface antigen. Hepatitis B vaccine
administration at 1 month of age, or at the time of hospital discharge, is appropriate for infants
born weighing less than 2 kg whose mothers have a negative hepatitis B surface antigen.
Premature infants are at increased risk for morbidity and mortality from vaccine-preventable
disease. Timely vaccination for this population is, therefore, especially critical.
Vaccination should be administered when a preterm neonate or infant is medically stable (Item
C164A) and, in general, should follow published schedules according to their chronologic age
rather than corrected age. Doses of each individual vaccine should not be split or reduced. The
availability of injection sites, however, may be limited. In this case, vaccines may be given at
different times, with some experts suggesting a 2-week interval to allow any local reactions to be
identified more easily.
Item C164A: medically stable infant:

The one who does not require ongoing
management for serious infection; metabolic disease; or acute renal, cardiovascular, neurologic, or
respiratory tract illness and who demonstrates a clinical course of sustained recovery and a pattern of
steady growth
Some preterm neonates or infants may experience apnea, bradycardia, and desaturation events
with vaccination, but these events are transient and not associated with long-term effects.
Vaccination schedules should not be altered solely on this basis.
The vaccination schedule for preterm neonates and infants should be adjusted for 2 vaccines,
rotavirus, and hepatitis B. The first dose of live rotavirus vaccine, usually given at 2 months of
age, should not be given while the infant is still hospitalized because of the risk of transmission
to other high-risk infants. This dose should be given when the infant is to be discharged and
between the ages of 6 weeks and 14 6/7 weeks. If the infant requires rehospitalization within 2 to
3 weeks of receiving the vaccine, physicians may consider placing the infant under contact
precautions.
The hepatitis B vaccine schedule should also be adjusted for preterm neonates and infants, If
maternal serology is negative, the first dose of hepatitis B vaccine is usually given within 24
hours of birth to infants weighing at least 2 kg. However, the vaccine exhibits decreased
immunogenicity in neonates and infants born weighing less than 2 kg. These infants should
receive their first dose at the time of hospital discharge or at 30 days of age chronologically,
whichever is earlier. Additional guidelines for hepatitis B vaccination according to birth weight
and maternal hepatitis B status are listed in Item C146B.

Item C146B: Hepatitis B Vaccine Schedules for Infants by Maternal Hepatitis B Surface Antigen
(HBsAg) Status and Birth Weight

Although not a vaccine, pediatricians should be aware that palivizumab, the monoclonal
antibody against respiratory syncytial virus (RSV), should be considered for administration every
month for 5 months starting at the onset of RSV season to infants who were born before 29
weeks' gestation and younger than 1 year when the RSV season starts, as well as to infants with
certain types of heart defects or chronic lung diseases. Specific eligibility criteria are outlined in
the American Academy Of Pediatrics Red Book: 2021-2024 Report of the Committee on
Infectious Diseases.
In addition to timely vaccination of preterm infants, their caregivers and household contacts
should also receive appropriate vaccinations, especially against influenza and pertussis, to
provide additional protection for the child.
PREP Pearls
• Hepatitis B vaccine should be administered within 12 hours of birth to all neonates,
including those weighing less than 2 kg born to mothers with a positive hepatitis B
surface antigen.
• Vaccines for rotavirus and hepatitis B are the only vaccines that require adjustment in the
administration schedule for preterm infants.
• All caregivers and household contacts of preterm infants should receive appropriate
vaccinations, especially against influenza and pertussis

• Plan an immunization schedule for an infant bom prematurely
Suggested Readings
• Immunization In preterm and low birth weight infants. Red Book: 2021-2024 Report of
the Committee on infectious Diseases. 32nd ed; 67-68.
• Respiratory syncytial virus. Red Book: 2021-2024 Report of the Committee on infectious
Diseases. 32nd ed; 628-635
• Continuing care of the infant after transfer from neonatal intensive care. American
Academy of Pediatrics Textbook of Pediatric Care. 2nd ed; 2017:1018-1049
• Discharge planning for the high-risk newborn requiring intensive care. American
Academy of Pediatrics Textbook of Pediatric Care. 2nd ed; 2017:1050-1067.

Question 147
A previously healthy, 8-year-old girl is evaluated for a 2-month history of headache, nausea, and
worsening vision. Additionally, her fluid intake has increased significantly, and she wakes up
multiple times at night to urinate. Vital signs reveal a temperature of 37 º C, blood pressure of
100/62 mm Hg, and heart rate of 104 beats/min. Her weight has been tracking at the 50th
percentile, and her height has decreased from the 75th to the 25th percentile over the past year.
Her visual acuity is 20/100 bilaterally. Physical examination findings are significant for bilateral
peripheral visual field deficits and are otherwise unremarkable. Brain magnetic resonance
imaging shows a 4-cm, mostly cystic, suprasellar mass consistent with a craniopharyngioma.

Laboratory test Result Reference Range
Insulin like growth factor 1 26 ng/mL (3.4 nmol/L) 70-225 ng/mL (9.2-29.5 nmol/L)
Thyroid-stimulating 2.5 uIU/ml 0.6-4.8 uIU/ml
hormone
Free thyroxine 1.3 ng/dL (16.7 pmol/L) 0.9-1.7 ng/dL (11.6-21.9 pmol/L)
AM cortisol 12 µg/dl (331.1 nmol/L) 6.2-19.4 µg/dl (171.1-535.2 nmol/L)
Of the following, the MOST likely additional laboratory results for this girl include
Response Serum Sodium (ref Serum osmolality (ref Urine osmolality (ref
choice range, 135-145 mEq/L) range, 285-295 mOsm/kg) range, 50-1,200
mOsm/kg)
A 130 270 80
B 130 270 1,000
C 150 312 80
D 150 312 1,000

Correct Answer: C
The girl in the vignette has craniopharyngioma that is causing symptoms due to increased
intracranial pressure (e.g., headache, nausea), compression of the optic chiasm (e.g., vision
problems, visual field deficits), and pituitary gland dysfunction specifically growth hormone
deficiency (e.g., declining height percentiles, insulin-like growth factor 1 level), and diabetes
insipidus (e.g., polyuria, polydipsia). Central diabetes insipidus occurs when there is a deficiency
of antidiuretic hormone from the posterior pituitary gland and results in the kidney's inability to
reabsorb free water. Polyuria ensues, and if the child is not able to compensate with adequate
free water intake, hypernatremic dehydration develops.
Hypernatremia, a high serum osmolality, and low urine osmolality are consistent with diabetes
insipidus and are the most likely additional laboratory results for the girl in the vignette. Other
laboratory findings consistent with dehydration may be seen with diabetes insipidus. The
diagnosis is made if the serum osmolality is > 300 mOsm/kg when the urine osmolality is <300
mOsm/kg. A water deprivation test may be required to make the diagnosis. Diabetes insipidus
can be differentiated from other causes of hypernatremic dehydration by the presence of dilute
urine (i.e., low urine osmolality). Hyponatremia, high serum osmolality, and high urine
osmolality (i.e., concentrated urine) are consistent with hypernatremic dehydration with a normal
antidiuretic hormone response.
Hyponatremia, a low serum osmolality, and low urine osmolality are consistent with primary
polydipsia. Hyponatremia, a low serum osmolality, and high urine osmolality are consistent with
the syndrome of inappropriate diuretic hormone.
Craniopharyngioma is the most common suprasellar tumor seen in children. It is a benign

neoplasm originating from the remnants of the Rathke pouch that can cause significant problems
due to its location and mass effect. Common presenting symptoms are those of increased
intracranial pressure (e.g., headache, nausea, vomiting), mass effect on the optic chiasm (e.g.,
vision problems, visual field deficits), and pituitary gland dysfunction. On neuroimaging, cysts
and calcifications are often present, with the latter better visualized on tomography scan.
Treatment is associated with significant morbidity, specifically panhypopituitarism, diabetes
insipidus, hypothalamic hyperphagia and vision loss.
Many children have at least 1 pituitary hormone deficiency at the time of diagnosis of
craniopharyngioma, and the majority have multiple pituitary hormone deficiencies after
resection. Growth hormone is the most common pituitary hormone deficiency at the time of
diagnosis followed by gonadotropin deficiency, then deficiencies of antidiuretic hormone,
thyroid-stimulating hormone (TSH), and adrenocorticotropic hormone (ACTH). Growth
problems (most commonly), pubertal delay or arrest, diabetes insipidus, and weight gain may be
present at the time of diagnosis. The girl in the vignette has evidence of growth hormone
deficiency in addition to her diabetes insipidus but does not have TSH or ACTH (i.e., normal
morning cortisol level) deficiency. She is too young to detect gonadotropin deficiency.

PREP Pearls
• Craniopharyngioma commonly presents with symptoms of increased intracranial pressure
(e.g., headache, nausea, vomiting), mass effect on the optic chiasm (e.g., vision problems,
visual field deficits), and pituitary gland dysfunction (e.g., growth problems. delayed or
halted puberty, diabetes insipidus, weight gain).
• Diabetes insipidus is characterized by polyuria. Polydipsia, hypernatremia,
hyperosmolality, and dilute urine.
• Diagnostic criteria for diabetes insipidus include a serum osmolality of >300 mOsm/kg
when the urine osmolality is <300 mOsm/kg. A water deprivation test may be required to
make the diagnosis

• Recognize the clinical and laboratory features associated with diabetes insipidus
• Recognize the clinical features associated with pituitary disorders caused by
craniopharyngioma
• Recognize the clinical and laboratory features associated with hypopituitarism
Suggested Readings
• Neuroendocrine disorders in pediatric craniopharyngioma patients. J Clin Med.
doi:10.3390/jcm4030389
• Polyuria. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:1528-1533.
• Evaluation and initial management of hypopituitarism. Pediatr Rev,
doi:10.1542/pir.2015-0081
• Brain tumors. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed ;
2017:1792-1797.
• Diabetes insipidus. Pediatr Rev doi:10.1542/pir.2018-0337

Question 148
13-year-old adolescent girl is being evaluated for behavioral concerns. Her parents have had
increasing difficulty with their daughter's bursts or energy and activity. She frequently works on
ambitious projects, and her latest is a plan to create and sell clothing for pets. She has spent her
birthday money and allowance on supplies and has asked her parents and relatives for more
money. She stays up late to sew clothes and she seems to function on little sleep. At times, she
suddenly becomes angry and aggressive. She talks so quickly that it is difficult to follow her
train of thought, but she appears confident that she will be so successful that she could quit
school early and that her parents could stop working.
Of the following, the associated feature MOST consistent with this adolescent's condition is
A. a diagnosis of autism at age 3 years
B. a relative diagnosed with the suspected condition
C. consistent oppositional behavior toward her teachers
D. fewer sexual feelings than her peers

Correct Answer: B
The adolescent in the vignette has goal-driven activity, grandiosity, decreased need for sleep,
rapid speech, and periods of out-of-control irritability consistent with a diagnosis of bipolar
disorder. Bipolar disorder is highly heritable and, therefore, a family history of bipolar disorder
is most supportive of the diagnosis in a pediatric patient.
Bipolar disorder affects about 1% to 3% of children and adolescents. It is a mood disorder that is
diagnosed clinically and characterized by abnormal and fluctuating mood. An episode of
euphoria and out-of-control irritability lasting at least 1 week with severe impairment (mania) is
required for making a diagnosis of bipolar I disorder; these symptoms lasting at least 4 days
without severe impairment (hypomania) are needed for a diagnosis of bipolar II disorder.
Episodes of mania or hypomania can alternate with episodes of depression in some patients,
whereas other patients may have manic or depressive episodes with features of the other mood
(i.e., mania with depressive symptoms or depression with manic symptoms). Children and
adolescents tend to have “mixed” states and to cycle mote quickly through these moods than do
adults. Additional symptoms of bipolar disorder include distractibility, grandiosity, decreased
need for sleep, rapid speech, flight of ideas, and increased goal-directed activity. These behaviors
are beyond those expected for the child's developmental age and are more likely to indicate
bipolar disorder when hallucinations are also present.
Genetic and environmental factors are involved in the development of bipolar disorder in
children ard adolescents. Heritability is estimated to be over 80%; having a first-degree relative
with bipolar disorder is a significant risk factor. Neuroimaging studies in those with polar
disorder have shown abnormalities in the limbic structures of the brain as well as involvement of
the frontotemporal and front striatal areas. Trauma, family discord, and low socioeconomic
status are environmental contributors to poor prognosis.
Hypersexuality not hyposexuality, is more commonly seen in bipolar disorder. Although children
and adolescents with autism spectrum disorder can also have bipolar disorder, there is a higher
co-occurrence of bipolar disorder with conditions such as attention-deficit/hyperactivity disorder
and oppositional defiant disorder. Oppositional behaviors are not a core feature of bipolar
disorder.
PREP Pearls
• An episode of mania (euphoria and out-of-control irritability) lasting at least 1 week is
required for a diagnosis of bipolar disorder.
• Children and adolescents tend to have “mixed” states of mania and depression and to
cycle more quickly through these moods than do adults.
• Having a first-degree relative with bipolar disorder is a significant risk factor for
developing bipolar disorder,

• Recognize the various environmental and biological contributors to the development of
bipolar disorder in children and adolescents
• Recognize the clinical findings associated with bipolar disorder in children and
adolescents
Suggested Readings
• Bipolar disorder in children. Pediatr Ann, doi:10.3928/19382359-20160920-01
• Mood and affect disorders. Pediatr rev, doi:10.1542/pir.36-2-52
• Bipolar disorder in children. Zuckerman Parker Handbook of Developmental and
Rehavioral Pediatrics for Primary Care. 4th ed; 2019:152-156.

Question 149
A 1-month-old girl is evaluated at the emergency department for seizure-like activity that lasted
for 5 minutes. The patient was bom full term after an uneventful pregnancy and delivery, and
was discharged home 2 days after birth without postnatal complications. Her physical
examination shows 4 hypomelanotic maculae on the trunk (Item Q149A). The mother reports a
personal history of early-onset seizures, learning disability, and multiple bilateral renal
angiomyolipoma's. Multiple erythematous papules are present on the mother's face (Item Q149B
and Item Q149C). Magnetic resonance imaging of the mother's brain demonstrated
subependymal nodules.
Of the following, the BEST next screening test recommended for this infant is
A. computerized tomography of the chest
B. echocardiography
C. liver ultrasonography
D. thyroid ultrasonography

Correct Answer: B
The disorder seen in the family described in the vignette is tuberous sclerosis complex (TSC), a
hereditary tumor predisposition syndrome. Of the response choices, the best next screening test
for this infant would be echocardiography to look for a cardiac rhabdomyoma, which may be
seen at birth children with TSC. The mother’s history of early-onset seizures, learning disability,
and bilateral multiple renal angiomyolipomas support this diagnosis. The erythematous papules
on the mother’s face are angiofibroma's; these can become-disfiguring. Tuberous sclerosis
complex has autosomal dominant inheritance with phenotypic variability, even within the same
family. Item C149 highlights the clinical features of TSC based on the organ system involved;
features are grouped into major and minor criteria. Diagnosis of TSC can be made in the
presence of 2 major clinical features, or 1 major and 2 minor clinical features, or with the
identification of a disease-causing variant in either the TSC1 or TSC2 gene.
Item C149: clinical features of TSC
Seizure control in TSC can be a challenge, often requiring multiple anti-epileptic medications
and, at times, surgery to remove the epileptic focus. Treatment for enlarging subependymal giant
cell astrocytoma consists of mammalian target of rapamycin (mTOR) inhibitors (e.g., sirolimus
or everolimus) prior to attempting surgery. Topical mTOR inhibitors have shown good control
for facial angiofibromas and are generally well tolerated. Renal angiomyolipomas are treated
with an mTOR inhibitor, and with selective embolization as second-line therapy. MTOR
inhibitors are also used for treatment of lymphangioleiomyomatosis. In TSC, cardiac
rhabdomyoma may be seen on a prenatal ultrasound. The natural history of cardiac
rhabdomyoma in TSC is gradual involution over time. Management is observation with serial
echocardiography. Surgery may be required for those with outflow tract obstruction.
For the girl in the vignette with TSC, ultrasonography of the liver would not be indicated as
hepatic tumors are not increased in TSC. Although a computerized tomography of the chest
might show cardiac rhabdomyoma, the need for sedation and the radiation exposure would make
this study less appropriate than an echocardiogram for diagnosis and follow-up of rhabdomyoma.
Thyroid ultrasonography is not indicated in children with TSC.
Although tumor predisposition syndromes are rare, it is important for the pediatrician to
recognize these and perform appropriate screening.
Beckwith-Wiedemann syndrome is an overgrowth disorder characterized by macrosomia,

macroglossia, body asymmetry, abdominal wall defects, and increased risk for tumors.
Recommended tumor screening for children with Beckwith-Wiedemann syndrome includes a
serum α-fetoprotein level (the tumor marker for hepatoblastoma) every 3 to 4 months until age 4
years, and abdominal (looking for embryonal tumors) every 3 to 4 months until age of 8 years
Evaluation by hepatic ultrasonography alone would not be an appropriate study with Beckwith-
Wiedemann syndrome.
Abdomen and pelvic ultrasonography every 3 to 4 months is the recommended surveillance test
for individuals with Li-Fraumeni syndrome to look for adrenocortical carcinoma and soft tissue
sarcomas; annual brain, and whole-body magnetic resonance imaging are recommended to look
for soft tissue and bone sarcoma. Li-Fraumeni syndrome is a hereditary cancer predisposition
syndrome caused by pathologic variants in the tumor suppressor gene TP53. The condition
predisposes the individual to a multitude of childhood, and adult-onset cancers, including but not
limited to adrenocortical carcinoma, embryonal rhabdomyosarcoma, soft-tissue sarcoma, acute
lymphocytic leukemia, and brain tumors.
Thyroid ultrasonography, to look fog medullary thyroid cancer (MTC), is the recommended
surveillance screening test for children with multiple endocrine neoplasia type 2 (MEN2). MEN2
is caused by a pathogenic variant in the RET proto-oncogene. MEN2A has an increased risk for
MTC, pheochromocytoma, or parathyroid adenoma, MEN2B is characterized by lip and tongue
mucosal neuromas, marfanoid habitus, and has an increased risk of MTC.
Neurofibromatosis is a neurocutaneous tumor predisposition syndrome caused by a pathogenic

variant in the NF1 gene. Recommended pediatric surveillance for NF1 is an annual dilated eye
examination looking for changes seen in optic nerve glioma imaging studies are performed based
on symptoms of neurological compromise such as decreased vision. Pain in an extremity or an
enlarging cutaneous mass would be indications for imaging to look for malignant changes in a
plexiform neurofibroma.

PREP Pearls
• Tuberous sclerosis complex is a tumor predisposition syndrome with involvement of
multiple organ systems.
• Tuberous sclerosis complex has autosomal dominant inheritance with phenotypic
variability.
• The natural history of cardiac rhabdomyoma in tuberous sclerosis complex is gradual
involution over time.

• Recognize the clinical findings associated with tuberous sclerosis, and manage
appropriately
Suggested Readings
• Presentation and Diagnosis of Tuberous Sclerosis Complex in Infants. Pediatrics,
doi:10.1542/peds.2016-4040
Care. 2nd ed; 2017.2379-2396
• Neurocutaneous disorders in children. Pediatr Rev. doi:10.1542/pir.2015-0118
• Imaging of cancer predisposition syndromes in children. Radiographic,
doi:10.1148/rg.311105099
• Gene Reviews. University of Washington, 2020.
https://www.ncbi.nlm.nih.gov/books/NBK1220

Question 150
A 15-year-old adolescent girl is seen for a health supervision visit. She is preparing to return to
competitive judo after reconstruction of her anterior cruciate ligament. After her surgery, she
gained 5 kg. She reports losing 1.5 kg over the past week with diet and exercise. The adolescent's
weight today is 50 kg (50th percentile). Her weight and height previously tracked at the 25th
percentile. She is hoping to compete at a weight of 44 kg in several months.
Of the following, the BEST advice to give this adolescent is that

A. her goal is attainable if she continues with her current dietary and exercise regimen
B. her rate of weight loss is associated with excessive loss of lean muscle
C. she should have her body fat percentage calculated to determine her optimal weight
D. she should minimize her carbohydrate intake to decrease fluid retention

Correct Answer: B
The adolescent in the vignette has intentionally lost 3% of her body weight (1 .5 kg) in 1 week.
A healthy rate of weight loss for adolescent is no greater than 1.5 % of body weight per week or
about 0.75 kg (1.6 lbs.) per week for this adolescent. Higher rates of weight loss are often the
result of fluid loss and are associated with a reduction in lean muscle mass and strength.
Therefore, continuing her current rate of weight loss is not recommended.
There are currently no accepted methods for calculating optimal body weight or composition for
adolescent sport performance, the range of optimal body weight for sport performance in a given
individual is based on measures of performance and health, rather than body composition.
Minimum allowable weights are determined for high school wrestlers based on body
composition measurements: 7% body fat for boys and 12% for girls. These minimums serve to
prevent wrestlers from competing at excessively low weights that are not compatible with
normal growth and development. The minimum allowable body fat percentage should not be
considered a goal for athletes in weight-classified sports.
Carbohydrates are an important fuel source for high-intensity training. Adequate carbohydrate
intake minimizes the catabolism of muscle and lean tissue. However, each gram of intramuscular
glycogen is stored with about 3 g of water. As glycogen is depleted with vigorous physical
activity, marked fluid loss resulting in weight loss. This weight returns rapidly when
carbohydrates are repleted after a workout. The initial weight loss associated with low
carbohydrate diets is primarily the loss of water rather than fat, Young, competitive athletes
should consume 6 to 10 g of carbohydrate per kilogram of body weight daily to fuel high-
intensity training. Lower intake leads to loss of lean muscular tissue and is not compatible with
sustainable weight loss or maintenance of athletic performance.
PREP Pearls
• Weight loss of more than 1.5% of body weight per week is associated with significant
loss of fluid and lean tissue.
• Carbohydrates are an important source of fuel for high-intensity exercise. Athletes
require adequate carbohydrate intake for peak performance.
• Initial weight loss associated with carbohydrate restriction is the result of water loss, and
will rapidly reverse when healthy eating patterns are restored.

• Understand the role of fluids in weight control for athletes
• Recognize inappropriate weight-loss regimens for athletes who participate sports with
weight categories
Suggested Readings
• Council on Sports Medicine and Fitness. Promotion of healthy weight-control practices in
young athletes. Pediatrics, doi:10.1542/peds.2017-1871
Sports nutrition. Pediatric Nutrition Handbook 8th ed; 2020; 321-366.

Question 151
A 4-year-old girl is brought to the clinic with a 3-day history of fever and right facial swelling.
She has no history of rhinorrhea or cough. She has odynophagia, tooth sensitivity to cold and hot
on her right side, and decreased oral intake, but she is drinking juice well and has normal urine
output. She has no allergies, and her vaccinations are up to date. Physical examination reveals a
well-appearing girl with a temperature of 38.5 º C; tender, right maxillary soft tissue edema;
boggy edema in the right preauricular area; no tenderness on manipulation of the right helix;
clear external auditory canals and normal tympanic membranes: a 4-mm erythematous pustule on
the upper right gingiva, a normal oropharynx; and a 2.5 x 2.5 cm, tender right submandibular
lymph node.
Of the following, the MOST appropriate treatment for this girl’s infection is
A. amoxicillin-clavulanate
B. azithromycin
C. cefdinir
D. trimethoprim-sulfamethoxazole

Correct Answer: A
The girl in the vignette has right facial swelling arising from a right upper molar dental abscess, a
type of soft tissue odontogenic infection. Signs and symptoms supporting the diagnosis of dental
abscess include fever, hot and cold sensitivity, right upper maxillary edema, the collection of
purulent fluid on the right upper gingiva, right submandibular lymphadenopathy, and lack of
upper respiratory symptoms or abnormal findings on otologic and pharyngeal examination.
Furthermore, this girl is well appearing and tolerating oral intake well. For her soft tissue
odontogenic infection, the treatment of choice is amoxicillin-clavulanate.
Odontogenic infections are polymicrobial, consisting of oral anaerobic gram-negative rods and
gram-positive organisms, including streptococcal species. The predominant organisms include
Actinomyces, Bacteroides, Fusobacterium, Peptococcus, Pepto streptococcus, and Prevotella.
Ultimately, the girl will require evaluation by a dentist and possible drainage of the abscess or
extraction of the tooth, but empiric oral antibiotic therapy should be started immediately with
amoxicillin-clavulanate. Azithromycin may be an acceptable alternative for the penicillin-
allergic child, but it is not the treatment of choice: clindamycin may be a better alternative in the
child with penicillin allergy. Cefdinir and trimethoprim-sulfamethoxazole have poor activity
against anaerobic organisms.
Dental caries are a risk factor for developing odontogenic infections. Dental decay is one of the
most common chronic diseases of childhood. Those living in poverty are disproportionately
affected. Behavioral risk factors for dental decay include extended use of a bottle/sippy cup,
sleeping with a bottle, frequent snacks with sugar, sugar-sweetened beverages, and sugary
medications. Early transmission of bacteria associated with dental caries from the primary care
giver to the child is a major risk factor. Other risk factors include certain diseases (cystic fibrosis,
diabetes mellitus, and Sjögren syndrome), medications limiting salivary flow, and prematurity
(because of enamel defects).
Once teeth emerge, often as early as 6 months, they are colonized by cariogenic bacteria. In the
setting of repeated carbohydrate exposure, these bacteria cause demineralization resulting in
dental decay. Exposure to fluoride as soon as teeth erupt may prevent, halt, or even reverse
carious lesions. Therefore, as recommended in the American Academy of Pediatrics Clinical
Report on Fluoride use, the parents of the girl in the vignette should brush her teeth twice per day
with a pea-sized amount fluoride-containing toothpaste. Parental supervision is recommended
until a child masters the toothbrushing technique, typically at 8 years of age. Brushing with
fluoridated toothpaste should begin with first tooth eruption; non-fluoridated toothpaste is not
recommended. The girl should visit her dentist regularly for dental supervision.
PREP Pearl
• For soft tissue odontogenic infections, the oral antibiotic of choice is amoxicillin-
clavulanate.
• Dental decay is one of the most common chronic diseases of childhood; those living in
poverty are disproportionately affected
• Brushing with fluoridated toothpaste should begin with the first tooth eruption, non-
fluoridated toothpaste is not recommended.

MOCA-Peds Objective
• Counsel families about healthy beverage consumption in early childhood.

• Recognize the clinical findings associated with caries in patients of various ages
• Recognize the various clinical findings associated with dental and periodontal disease
Suggested Readings
• Maintaining and improving the oral health of young children. Pediatrics,
doi:10.1542/peds.2014-2984
• Preventing dental caries in children < 5 years: systematic review updating USPSTF
recommendation. Pediatrics, doi:10.1542/peds.2013-1469
• Section on Oral Health. Fluoride use in caries prevention in the primary care setting.
Pediatrics, doi:10.1542/peds.2020.034637
• Fluoride and dental caries prevention in children. Pediatr Rev, doi:10.1542/pir.35-1-3
• Rev.
• Consultation with the specialist: Dental care. Pediatr Rev, doi:10.1542/pir.22-1-13

Question 152
A neonate is born via spontaneous vaginal delivery to a woman with painful genital ulcers.
Maternal membranes were ruptured for 18 hours, and fetal heart tracings were reassuring. A
genital lesion swab is sent for herpes simplex virus (HSV) polymerase chain reaction testing,
along with maternal serology (IgG and IgM) for HSV-1 and HSV-2. The neonate is well
appearing. His temperature is 36.5 º C, respiratory rate is 45 breaths/min, heart rate is 110
beats/min, and oxygen saturation is 99% on room air. He weighs 5 lb., 6 oz. The remainder of his
physical examination findings are unremarkable.
Of the following, the BEST next management step for this neonate is to obtain
A. herpes simplex virus IgG at 24 hours after birth
B. herpes simplex virus surface culture at 24 hours after birth
C. herpes simplex virus surface culture now
D. herpes simplex virus surface cultures when a rash appears

Correct Answer: B
The neonate in the vignette was born to a mother with active herpes simplex (HSV) genital
lesions. The neonate is asymptomatic and has normal physical examination findings. In this case,
neonatal surface HSV cultures and a blood specimen for HSV DNA polymerase chain reaction
(PCR) testing should be obtained at 24 hours after birth. Detection of HSV at 24 hours after birth
most likely reflects neonatal viral replication rather than contamination from maternal lesions.
Detection of HSV IgG reflects placental transfer of maternal antibodies rather than neonatal
infection. It would be inappropriate to wait until a rash appears to send a skin HSV culture.
Herpes simplex is a DNA virus that can establish latency after primary infection. Two distinct
HSV types, 1 and 2, exist. Historically, HSV-1 was predominantly associated with oral infection
and HSV-2 was mainly associated with genital mucocutaneous infection. However, HSV-1
increasingly causes genital infections. In the United States, neonatal HSV infections occur in 1 in
2,000 to 3,000 live births; infection is rare despite HSV being a common genital infection in
adults. Symptomatic neonatal HSV infection occurs within the third to fourth week after birth
and is classified as:
• Skin-eye-mouth disease (45%)
• Central nervous system disease with or without skin involvement (30%)
• Disseminated disease (25%)
Maternal factors affect the risk of neonatal infection. Maternal primary infection confers a 57%
risk of neonatal infection, compared with a 2% risk with recurrent maternal infection. A history
of prior HSV genital infections should be sought during pregnancy. Maternal testing should
include HSV PCR testing or culture of a sample from a lesion and HSV serology (IgG and IgM).
Management of an asymptomatic neonate potentially exposed to HSV is challenging. Signs of

neonatal infection may not be apparent until the second to fourth week after birth. An
asymptomatic neonate with negative 24-hour HSV surface cultures and blood PCR testing can be
safely sent home provided other parameters for discharge are met. An asymptomatic neonate
with a surface culture positive for HSV should undergo lumbar puncture; a cerebrospinal fluid
(CSF) specimen should be sent for cell count, protein and glucose levels, and HSV PCR.
Treatment should be initiated with intravenous acyclovir 60 mg/kg per day divided every 8
hours. If the results of CSF evaluation are normal and the neonate remains asymptomatic,
treatment should continue for 10 days. This scenario assumes that the neonate acquired HSV
infection but did not develop HSV disease.
The clinical manifestations, diagnosis, and treatment of pediatric HSV infection are detailed in
Item C152. The nature and severity of HSV infection depends on the route of transmission and
the immune status of the host. The severity of illness also depends on primary versus recurrent
infection.
Diagnostic testing options for suspected HSV infection include culture, PCR, and serology (IgG,
IgM). HSV PCR testing is the diagnostic test of choice. PCR has a faster turnaround time (1 to 2
hours) than culture (1 to 3 days). HSV culture and PCR testing can be performed on a swab of a
skin vesicle (rupture the vesicle roof and rub the base), CSF fluid, and oral or genital ulcers.
HSV PCR testing can also be performed on serum. Most children's hospitals have in-house HSV
PCR testing available either independently or as part of multiplex meningitis/encephalitis panel.
Antibodies to HSV-1 and -2 begin to develop 1 to 2 weeks after infection; 95% are seropositive
by 12 weeks. Type-specific HSV-2 antibodies are helpful in diagnosing recent genital infection.
The presence of HSV-1 IgG does not differentiate past genital infection from oral infection.
Item C152: The clinical manifestations, diagnosis, and treatment of pediatric HSV infection
PREP Pearls
• An asymptomatic neonate born to a woman with active genital herpes simplex virus
lesions should have surface cultures sent at 24 hours after birth
• In older children, the type and severity of herpes simplex virus infection depends on the
route of transmission and immune status. A variety of clinical manifestations occurs.
• Herpes simplex virus (HSV) polymerase chain reaction is the diagnostic test of choice for
suspected HSV infection.
APP Content Specifications(S)

• Recognize the clinical features associated with herpes simplex virus infection in children
of various ages
• Plan the appropriate diagnostic evaluation for herpes simplex virus infection
• Plan the appropriate management of herpes simplex virus infection in children of various
ages, taking into account appropriate timing of therapy
Suggested Readings
• Herpes simplex. Red Book: 2021-2024 Report of the Committee on Infectious Diseases.
32nd ed; 2021:407-416.
• Herpes simplex. Pediatr Rev, doi:10.1542/pir.30-4-119
• Committee on Infectious Diseases, Committee on Fetus and Newborn, Guidance on
management of asymptomatic neonates born to women with active genital herpes lesions.
Pediatrics, doi:10.1542/peds.2012-3216
• Vital infections in the nursery. Common problems in the newborn nursery. An Evidence
and Case Based Guide. Springer. 2019:81-87

Question 153
A neonate who appears "dusky” is evaluated in the special care nursery. The neonate is vigorous
and without distress. The oral mucosa, hands, and feet have a bluish-purple color. Vital signs
include a heart rate of 145 beats/min, respiratory rate of 50 breaths/min, blood pressure in the
right upper extremity of 75/45 mm Hg, and a room air oxygen saturation, measured by a pulse
oximeter located on the right hand, of 65%. When the pulse oximeter is placed on the left lower
extremity, it measures an oxygen saturation of 80%.
Of the following, this child's MOST likely diagnosis is

A. Epstein anomaly
B. hypoplastic left heart syndrome
C. transposition of the great arteries
D. tricuspid atresia

Correct Answer: C
The neonate in the vignette is hypoxic. To evaluate his condition, it is important to obtain pre-
and post-ductal oxygen saturation levels. The preductal oxygen saturation is measured in the
right upper extremity; the post ductal oxygen saturation is typically measured in either lower
extremity. The oxygen saturation levels in the pre- and post-ductal extremities should either be
equal, or the post ductal extremity should be lower than the preductal extremity. The post ductal
oxygen saturation would be lower if the ductus arteriosus is shunting right to left (i.e., pulmonary
artery to aorta), as seen in pulmonary arterial hypertension or coarctation of the aorta. The
neonate in the vignette demonstrates “reverse differential cyanosis,” where the oxygen saturation
levels are unequal but in the reverse of the order expected. This can only happen in cases of
dextro-transposition of the great vessels with arch obstruction or pulmonary hypertension. The
other response choices would not demonstrate reverse differential cyanosis.
Transposition is the second-most common form of cyanotic congenital heart disease. It typically
presents in the first 1 to 2 weeks after birth, if not prenatally diagnosed. A failure in the normal
septation of the common trunk into 2 great vessels causes the aorta to be positioned anteriorly,
arising off of the right ventricle while the pulmonary artery arises off of the left ventricle; this is
referred to as ventriculoarterial discordance. This results in deoxygenated blood returning from
the body to the right atrium, then into the right ventricle and back out to the body. Similarly, the
oxygenated blood runs in a parallel circuit. Without a site for mixing, oxygenated blood cannot
be delivered to the body and deoxygenated blood to the lungs. In fetal life, the patent foramen
ovale and patent ductus arteriosus allow for this vital mixing. Postnatally, a prostaglandin
infusion to keep the ductus arteriosus open and sometimes an atrial septostomy are required to
maintain this critically important mixing.
Associated lesions found in children with transposition of the great arteries (Item C153),
include ventricular septal defect, left ventricular outflow tract obstruction, atrioventricular valve
abnormalities, and/or coronary artery abnormalities. Children not diagnosed prenatally may
present with cyanosis, poor feeding, murmur, and/or tachypnea. Children with dextro-
transposition of the great arteries are typically treated with an arterial switch operation, which
involves detaching the great arteries from the current ventricle and attaching them to the other
ventricle. In addition, the coronary arteries are removed from the original aorta and replaced onto
the neo-aorta (former pulmonary artery).

PREP Pearls
• Pre- and post-ductal oxygen saturation levels should be measured in neonates with
cyanosis.
• Reverse differential cyanosis, in which the post ductal oxygen saturation level is higher
than the preductal level, is seen in post dextro-transposition of the great arteries with
either pulmonary hypertension or coarctation of the aorta.
• Dextro-transposition of the great arteries refers to the aorta arising anteriorly from the
right ventricle and the pulmonary artery arising posteriorly from the left ventricle.
Surgical correction via an arterial switch procedure is warranted.
MOCA-Peds Objective
• Understand the differential diagnosis of tachypnea in an infant.
• Evaluate a neonate with respiratory distress.

• Recognize the clinical findings of transposition of the great arteries
Suggested Readings
• Congenital and acquired heart. American Academy of Pediatrics Textbook of Pediatric
Care 2nd ed; 2017:1883-1917.
• Congenital heart disease. Pediatr Rev, doi:10.1542/pir.2017-0032

Question 154
A 17-year-old adolescent boy is seen in the office for a routine health supervision visit. He is a
childhood cancer survivor who was treated for acute myeloid leukemia when he was 4 years old.
He reports that over the past week he has had a cough. He has been afebrile and has not had
rhinorrhea or other associated upper respiratory symptoms. He reports being unable to complete
his usual daily activities, including exercise, due to shortness of breath and fatigue.
The boy's vital signs include a temperature of 37 º C, heart rate of 100 beats/min, respiratory rate
of 25 breaths/min, blood pressure of 130/180 mm Hg. and oxygen saturation of 96% in room air
by pulse oximetry. He looks tired but is in no acute distress. Physical examination reveals
crackles in the lower lung fields, a regular heart rate with no murmur, palpable hepatomegaly,
and pitting edema of his bilateral lower extremities up to his shins. There is no palpable
lymphadenopathy.
Of the following, the chemotherapeutic agent that is the MOST likely cause of the boy's
condition is
A. Cytarabine
B. Cyclophosphamide
C. Daunorubicin
D. etoposide

Correct Answer: C
The child in the vignette is a childhood cancer survivor. His signs and symptoms are concerning
for congestive heart failure. Among the response choices, the most likely to cause this delayed
adverse effect is daunorubicin. An anthracycline, daunorubicin is known to cause
cardiomyopathy as a result of free radical damage. Dexrazoxane, a cardioprotective free radical
scavenger, is routinely administered with anthracycline treatment, the cardiotoxicity of
anthracyclines is cumulative; it is therefore important to know the total amount a patient has
received in order to advise and monitor accordingly.
The tong-term adverse effects of etoposide, a topoisomerase II inhibitor, include acute myeloid
leukemia (AML) and myelodysplastic syndrome.
Cyclophosphamide and cytarabine are alkylating agents with delayed effects including gonadal
failure, bladder cancer, and treatment-related AML and myelodysplastic syndrome.
Cyclophosphamide is not routinely used for AML therapy.
As the survival rate for childhood cancer continues to improve, the impact of long-term toxicity
of treatments more significant. Current protocols address this issue, with clear efforts to
administer effective curative therapies while decreasing long-term adverse effects. Item Cl54
lists some known side effects of chemotherapy and necessary monitoring.
It is important to follow childhood cancer survivors long term to monitor for and educate about
these effects. This is best accomplished in a multidisciplinary clinic setting.


PREP Pearls
• Childhood cancer survivors are at risk for several delayed treatment effects (e.g.,
cardiomyopathy or treatment-related acute myeloid leukemia and myelodysplastic
syndrome) and must be monitored closely for several years post treatment.
• The free radical cardiotoxicity of anthracyclines can be lessened by dexrazoxane, a free
radical scavenger.


• Recognize the long-term risks associated with immunosuppressive drug
Suggested Readings
• Early and often: The need for comprehensive discussion of treatment-induced cancer late
effects. Pediatrics, doi:10.1542/peds.2020-0498.
• The late effects of childhood cancer therapy. Pediatrics, doi:10.1542/peds.2006-2826.
• Late effects of childhood cancer and its treatment. Pediatr Clin Noth Am, doi:
10.1016/j.pcl.2014.09.017
• Late effects in survivors of childhood cancer. Pediatr Rev, doi:10.1542/pir.27-7-257

Question 155
A 14-year-old adolescent girl is seen for a health supervision visit. Her mother, who experienced
menarche at age 13 years, is concerned that her daughter has never had a period. The girl had
breast budding at age 11 years. She has no history of chronic illnesses or hospitalizations. The
girl states that she "eats healthy” and exercises every day. Her mother reports that her daughter
has stopped eating some of her favorite foods and has become a vegetarian. In private, the girl
reveals that she wants to lose weight and increase her exercise efforts because her "stomach
pooches out” and her “thighs are too big.” She is not concerned about her lack of periods. She
reports no binge eating, purging behaviors, or substance use. A review of systems is
unremarkable; she has no history of gastrointestinal symptoms, fatigue, cold intolerance, or
syncope. She just completed her first semester of high school with excellent grades and has a
role as a dancer in the school musical.
Physical examination reveals a temperature of 36.6 º C, heart rate of 64 beats/min, and blood
pressure of 105/70 mm Hg with normal orthostatic measurements. Her height is 160 cm (50th
percentile), weight is 45 kg (25th percentile), and body mass index is 18 kg/m2 (25th percentile).
Review of her growth from age 7 to 13 years reveals a height trajectory along the 50th percentile
and body mass index trajectory along the 75th percentile. Her sexual maturity rating of 2 for
breast and 3 for pubic hair development is unchanged from 1 year ago. She has hypoactive bowel
sounds and dry skin, and her pelvic bones appear prominent. The remainder of her physical
examination findings are unremarkable.
Laboratory evaluation reveals a normal complete blood cell count, comprehensive metabolic
profile, thyroid-stimulating hormone, and free thyroxine level. Serum levels of estradiol,
luteinizing hormone, and follicle-stimulating level are low for age. Electrocardiography reveals
normal sinus rhythm, with a heart rate of 65 beats/min, a QTc duration of 0.380 seconds, and
normal voltage. The diagnosis is discussed with the girl and her mother.
Of the following, the BEST next management step for this girl is to
A. arrange for hospitalization in an adolescent unit
B. initiate treatment with fluoxetine
C. prescribe a detailed 3,000-calorie meal plan
D. refer for family-based therapy

Correct Answer: D
The girl in the vignette fulfills the Diagnostic and Statistical Manual of Mental Disorders, Fifth
Edition (DSM-5) diagnostic criteria for anorexia nervosa (AN):
• Restricted energy intake manifested by a fall in her body mass index trajectory from the
75th to the 25th percentile
• Eating and exercise behaviors that interfere with weight gain and pubertal progression
• Disturbed body image; she wants to lose weight and exercise to change the
shape/appearance of her stomach and thighs
Lack of binge eating or purging for this girl is consistent with restricting-type AN. The best next
management step is to refer her for family-based therapy (FBT). She does not meet the criteria
for hospital admission (Item Cl55A). Fluoxetine is approved by the United States Food and
Drug Administration (FDA) for the treatment of bulimia it is not a first-line intervention for AN.
Treatment of AN requires a multifaceted approach. Prescribing a detailed meal plan to increase
calories would not alone be sufficient to address this patient's AN.
Item C155A: Criteria for Hospital Admission for Patients with Eating Disorders
Heart rate <50 beats per minute while awake
• Heart rate <45 beats per minute while asleep
• Systolic pressure <90 mm Hg
• Temperature <35.6°C (96°F)
• Prolonged QTc or other arrhythmia
• Orthostatic changes in blood pressure (>10 mm Hg)
• Orthostatic changes in pulse (>20 beats per minute)
• Syncope
• Electrolyte abnormalities
• Esophageal tears or hematemesis
• Intractable vomiting
• Suicide risk
• Weight <75% of expected body weight or body fat <10%
• Ongoing weight loss despite intensive management
• Acute weight loss and food refusal
• Failure to respond to outpatient treatment
Common behaviors observed in adolescents with restrictive AN include “picky” eating, food
eliminations, adopting a vegetarian diet, preoccupation with reading food labels, food rituals,
overinvestment in meal preparations, frequent weighing, avoidance of social eating, and social
isolation.
Clinical findings associated with AN include delayed or stalled puberty, menstrual irregularities,
and amenorrhea. Low serum levels of estradiol, luteinizing hormone, and follicle-stimulating
hormone indicate suppression of the hypothalamic-pituitary-gonadal (HPC) axis (functional
hypogonadotropic hypogonadism). Low bone mineral density and an increased risk of fractures
are consequences of AN and also associated with suppression of the HPG axis. Consequences of
ongoing undernutrition include psychomotor slowing of movement or speech and disordered
cognition, with difficulties in concentration and memory and decline in academic performance.

Like the girl in the vignette, it is common for patients with AN to have a paucity of physical
findings. Item C155B lists potential physical examination findings.
Item C155B: physical examination findings
Outpatient treatment options for AN include FBT, cognitive behavioral therapy

(CBT), and dialectical behavioral therapy (DBT). Phase I of FBT puts the parent in control of
food choices and meal preparation to facilitate nutritional rehabilitation and healthy eating. Phase
II gradually transitions control back to the patient. Phase III addresses associated psychosocial
issues. Family-based therapy has shown promise for the treatment of AN among younger
adolescents with a short duration of illness. Cognitive behavioral therapy, an individual therapy,
provides insight into behaviors to foster healthy change. Dialectical behavioral therapy uses CBT
with strategies for emotional regulation.
There is no FDA-approved medication for the treatment of AN. Adolescents with symptoms or
comorbid diagnoses of depression, anxiety, or obsessive compulsive disorder may benefit from
antidepressant or psychotropic medication in conjunction with psychotherapy.
The DSM-5 diagnostic criteria for bulimia include recurrent episodes of binge eating and
compensatory behaviors to prevent weight gain that occurs on average once a week or more, for
3 months, Binge eating is defined as a large amount of food eaten in a discrete time while feeling
unable to stop. Compensatory behaviors include Purging (e.g., self-induced vomiting and misuse
of diuretics, laxatives, thyroxine, amphetamines, diet pills), fasting, and exercise.
Clinical findings of bulimia, when present, are typically a consequence of purging behaviors.
Symptoms suggestive of vomiting include sore throat, heartburn, and chest pain. Pharyngeal
abrasions, erosion of dental enamel, dental calluses on the dorsum of hand or fingers (Russell
sign), and parotid gland enlargement may be present, Weakness, dizziness, or syncope with
hypotension and orthostatic changes may occur in association with dehydration. Electrolyte
abnormalities, a consequence of purging, are an indication for hospitalization (Item C155A).
These abnormalities may include hypokalemia, metabolic alkalosis, hypochloremia (associated
with vomiting), or hyperchloremia (associated with laxatives). Hypokalemia can precipitate
cardiac arrhythmias, and patients may complain of fatigue and muscle weakness. Menstrual
irregularities are common among girls with bulimia and vomiting. An esophageal tear (Mallory-
Weiss) and pneumothorax are uncommon complications of forceful vomiting.
Although outpatient CBT is considered the most effective therapeutic intervention for bulimia,
FBT and DBT may also be useful. Fluoxetine, tricyclic antidepressants, topiramate have also
shown benefit.
PREP Pearls
• Diagnostic criteria for anorexia nervosa include a low weight in the context of the
adolescent's pubertal maturation and growth trajectory, disturbed body image, and
abnormal behaviors surrounding eating and exercise.
• Family-based therapy should be considered for the management of anorexia nervosa,
particularly for younger patients.
• Electrolyte abnormalities, a consequence of purging, are an indication for hospitalization,

• Plan the appropriate management of bulimia
• Plan the appropriate management of anorexia nervosa
• Recognize the clinical findings associated with bulimia and the criteria for diagnosis
• Recognize the clinical findings associated with anorexia nervosa and the criteria for
diagnosis
Suggested Readings
• The role of the adolescent health provider and nutritionist in family based therapy.
Adolesc Med. 2018;29(2):359-374•
• Anorexia Nervosa. N Engl Med, doi:10.1056/NEJMcp.1803175
• Eating disorders. Reaching Teens. 2nd ed. 2020:485;488A488H
• Eating disorders. Pediatr Rev, doi:10.1542/pir.2015-0180
• Anorexia nervosa, bulimia nervosa, and other eating disorders. American Academy of
Pediatrics Textbook of Pediatric Care 2nd ed.2017, 1719-1727

Question 156
A 15-year-old adolescent boy was ejected from an all-terrain vehicle while riding without a
helmet and sustained multiorgan trauma, including open skull fracture and severe traumatic brain
injury. His Glasgow Coma Score on presentation was 3, and hemodynamic lability required
vasopressor infusions to maintain adequate blood pressure. Despite receiving no sedatives or
paralytics, after 48 hours of aggressive care in the pediatric intensive care unit, his
hemodynamics have stabilized, but he has no respiratory effort above the ventilator, no motor
response to stimulation, and his pupils measure 8 mm bilaterally and are nonreactive to light. His
electrolytes are within normal ranges, and he is normothermic.
Of the following, the BEST test after a neurologic examination in determining whether the
patient has met initial criteria for brain death is
A. apnea testing
B. electroencephalography
C. radionucleotide cerebral blood flow study
D. transcranial Doppler ultrasonography

Correct Answer: A
An apnea test is the next step in assessing the patient in the vignette. To declare brain death the
physician must document:
1. Absent cortical function: No spontaneous movement or response to voice or painful
stimuli
2. Absent brainstem function: Absent pupillary response with fixed and dilated pupils,
absent corneal reflex, cough or gag, and absent oculocephalic (doll's eyes) and
oculovestibular (cold calorics) reflexes
3. Apnea test: No spontaneous respiratory effort with a documented PCO2 above 60 mm Hg
and greater than 20 mm Hg change above baseline PCO2
The Uniform Brain Death Act Of 1978 aimed to clarify legal ambiguity surrounding the
determination of death and established that the “irreversible cessation of all functioning of the
brain, including the brain stem” is death. In 1987, guidelines for the determination of brain death
in children were published cooperatively and in 2011 were revised and again promulgated by the
Society of Care Medicine, the American Academy of Pediatrics, and the Child Neurology
Society.
The diagnosis of brain death requires confirmation of irreversible loss of all functions of the
brain, including the brainstem. In clinical practice, the requisite findings in brain death are
persistent coma of a known cause in the absence of metabolic derangements or pharmacologic
confounders, absence of brainstem reflexes, and apnea. Reversible conditions that can interfere
with the neurologic examination, such as hypothermia, electrolyte derangements, or medication
effects, must be excluded before brain death testing is performed for the clinical determination of
brain death to be valid. A patient determined to be brain dead is legally and clinically dead. The
diagnosis of brain death allows patients to be considered for organ donation.
For brain death to be determined in children, 2 separate examinations by different Physicians are
required, with an interval of 24 hours for term newborns (>37 week’s gestational age) up to age
30 days, and 12 hours for children who are between 30 days and 18 years of age. The interval
can be shortened with the use of ancillary testing. It is important to note that the determination of
brain death is based on clinical examination findings, and ancillary testing is not required
provided all aspects of the clinical brain death can be performed, including the apnea test.
Specifically, ancillary testing is indicated when apnea testing cannot be safely performed or
completed, all components of the neurologic examination cannot be performed, the neurologic
examination results are uncertain, or a medication effect interferes with accurate clinical
neurologic examination. Regardless of whether ancillary testing is used, a second neurologic
examination is required for the determination of brain death.
In children, the accepted ancillary testing available includes electroencephalography (EEG) and
radionuclide cerebral blood flow study. Brain death standards for EEG have been established by
the American Electroencephalographic Society. In addition, low to mid therapeutic barbiturate
levels do not preclude the use of an ancillary test for aiding in the determination of brain death.
Cerebral study standards established by the Society of Nuclear Medicine and Molecular Imaging
and the American College of Radiology may be used in the presence of high-dose barbiturate

therapy. Transcranial Doppler ultrasonography has not been validated as an ancillary study for
children and is not recommended for ancillary brain death testing in children.
PREP Pearls
• Brain death is a clinical diagnosis that requires a complete neurologic examination and
the documentation of Absent cerebral function, including absent brainstem reflexes.
• Ancillary studies may be required for the determination of brain death if a full clinical
brain death examination, including the apnea test, cannot be performed.
• Ancillary studies are not required for the declaration of brain death if 2 complete brain
death examinations can be performed adequately.
APP Content Specifications

• Understand the criteria for brain death and the role of neurodiagnostic studies in making
that determination
Suggested Readings
• Pediatric brain death. Pediatr Rev, doi:10.1542/pir.23-6-222
• Society of Critical Care Medicine, section on critical care and section on Neurology of
American Academy of Pediatrics; Child Neurology society. Clinical report-guidelines for
the determination of brain death in infants and children: an update of the 1987 task force
recommendations. Pediatrics, doi:10.1542/peds.2011-1511

Question 157
A 14-year-old adolescent girl is seen in the emergency department for worsening asthma over the
past 2 days. She used a full inhaler of albuterol in the past week and 2 inhalers in the past month,
usually taking 4 to 8 puffs per dose. She has not refilled her asthma controller medication for
several months.
On physical examination, the girl is afebrile, has a respiratory rate of 42 breaths/min, heart rate
of 135 beats/min, blood pressure of 135/70 mm Hg, and oxygen saturation of 92% in room air.
She answers questions in short, clipped phrases and uses intercostal and neck muscles to support
her respiration. Her breath sounds are diminished throughout with a prolonged expiratory phase
and inspiratory: expiratory ratio of 1:3. Biphasic wheezing is noted. Her heart sounds are distant.
The remainder of her physical examination findings are unremarkable.
After 3 treatments of nebulized albuterol 2.5 mg with ipratropium 0.5 mg over 1 hour and oral
prednisone 60 mg, her oxygen saturation is 92% on 2 L/min of oxygen via nasal cannula. Her
physical examination findings are unchanged except that she appears more comfortable after
starting the oxygen.
A. administer magnesium sulfate intravenously
B. begin an intravenous infusion of terbutaline
C. give an additional dose of prednisone 60 mg orally
D. prepare for rapid intubation and ventilator management

Correct Answer: A
Of the response choices, administration of intravenous magnesium sulfate is the best next
management step. The adolescent in the vignette is experiencing an asthma exacerbation with
signs of severe airway obstruction, including an inspiratory: expiratory ratio of 1:3 increased
work of breathing; biphasic wheezing and oxygen need. Signs of progressive obstruction in acute
asthma include an increasingly prolonged expiratory phase of respiration; transition from
expiratory wheezing to biphasic wheezing to no wheezing: and progressively increased work of
breathing. Respiratory fatigue can occur as severe obstruction progresses. The adolescent in the
vignette is responding poorly to bronchodilators and is at risk of progression to respiratory
failure. It is appropriate that she be treated in the pediatric intensive care unit (PICU), though the
level of intervention that can be provided outside the PICU varies from institution to institution.
Administration of continuously inhaled albuterol at 0.5 mg/kg per hour (maximum 15 mg/h)
might be considered as a next step in management. However, this is rarely initiated in the
emergency department (ED) because of the level of monitoring required. If continuously inhaled
albuterol and/or intravenous magnesium sulfate do not provide an adequate response, initiation
of a continuous terbutaline infusion may be considered, but would not be appropriate for ED
management.
If endotracheal intubation and ventilator management are required, the child should be admitted
to a PICU. In most circumstances, a trial of noninvasive ventilator support would be appropriate
before intubations in some institutions; noninvasive ventilation is performed in the ED and is
well tolerated, but has not been shown to decrease hospital admissions for severe asthma.
Continuing systemic corticosteroids, either oral or intravenous, is appropriate, but would not
precede treatment with magnesium sulfate.
Initial management of an acute asthma exacerbation includes early oral or intravenous

corticosteroids and aggressive use of inhaled albuterol and ipratropium. Opinions on the use of
dexamethasone versus prednisone in the ED for children and adolescents who can be discharged
from the hospital differ. Both medications are equally effective, but a recent cost-effectiveness
study found that a 2-dose course of dexamethasone, particularly when the second dose is
dispensed from ED at discharge, is less expensive and more likely to be completed than a 5-day
course of prednisone. Some have also suggested using inhaled magnesium in the ED or using
intravenous magnesium sulfate as part of first-line ED therapy. Recent studies have shown that
magnesium administered via either mode is beneficial as an adjunctive bronchodilator to
albuterol, but neither is associated with a decrease in the need for hospitalization. Several models
predicting hospitalization based on ED presentation or treatment of acute asthma have been
proposed. One model found that oxygen saturation <94% and prolonged expiration with an
inspiratory: expiratory ratio greater than 1:1 at presentation to the ED was the best predictor.
Second-level treatment of acute asthma exacerbation includes intravenous magnesium sulfate

and additional adrenergic agonist (e.g., albuterol) administered in frequent intermittent doses or
via continuous inhalation. Oxygen supplementation should be provided to keep peripheral
saturation levels above 90%. Item C157 outlines the stepwise management of severe asthma.
The order of implementation within and between treatment steps varies, based on local practices.

Item C157: A Stepwise Approach to the Management of Status Asthmaticus
• Step 1: Supportive care and prompt inhaled β-agonists and anticholinergics plus systemic
corticosteroids
• Step 2: Intravenous magnesium sulfate and/or intravenous aminophylline
• Step 3: Sedation and noninvasive or invasive ventilation. Inhaled anesthetics, bronchoscopy, and
extracorporeal life support may be needed for the most severe cases
Continuously inhaled albuterol at a high dose (15 mg/h) or intravenous terbutaline may be
associated with signs and symptoms of adrenergic agonist toxicity, including tachycardia;
tremor; decreased diastolic blood pressure; decreased serum potassium, magnesium, and
phosphorus levels; and hyperglycemia. Inhaled 70:30 helium/oxygen therapy and intravenous
ketamine are additional measures available for use in the PICU, as well as noninvasive
ventilation or intubation and ventilator management.
PREP Pearls
• Inhaled bronchodilators and oral corticosteroids are the first steps in the management of
an acute asthma exacerbation,
• Second-level treatments for an acute asthma exacerbation include intravenous
magnesium sulfate, frequent albuterol inhalation, and supplemental oxygen.
• Prolonged expiration and biphasic wheezing, or a quiet chest with increased work of
breathing, are signs of severe obstruction in asthma.
• Signs of adrenergic agonist (continuously inhaled albuterol of intravenous terbutaline)
toxicity include tachycardia, tremor, decreased diastolic blood pressure, electrolyte
abnormalities, and hyperglycemia.

• Provide appropriate treatment for a patient who has an acute exacerbation of asthma,
including asthma that is not responsive to adrenergic agonist therapy
• Recognize the signs of severe obstruction during an acute exacerbation of asthma
• Recognize the clinical features associated with toxicity to in a patient with an acute
exacerbation of asthma
Suggested Readings
• Development and internal validation of a pediatric acute asthma prediction rule for
hospitalization. J Allergy Clin Immunol Pract, doi:10.1016/j.jaip.2014.09.017
• Dexamethasone for acute asthma exacerbations in children: a meta-analysis. Pediatrics.
doi:10.1542/peds.2013-2273
• Asthma. doi: Pediatr Rev, doi:10.1542/pir.2018-0282
• Severe acute asthma (status Asthmaticus). American Academy of Pediatrics Textbook of
Pediatric Care 2nd ed; 2017:2971-2976.
• Effect of nebulized magnesium vs placebo added to albuterol on hospitalization among
children with refractory acute asthma treated in the emergency department. JAMA,
doi:10.1001/jama2020.19839

Question 158
A 13-year-old adolescent boy is brought to the emergency department 20 minutes he fell off his
skateboard, knocking out his front tooth. He has no other injuries. His friend picked up the tooth
and immediately placed it in a container of milk. In the emergency department, the tooth is
cleaned and returned to place. Upon discharge to home, he is prescribed an antibiotic after it is
determined that he has no medication allergies.
Of the following, the MOST appropriate antibiotic choice for this patient is
A. Azithromycin
B. Cefdinir
C. Clindamycin
D. Doxycycline

Correct Answer: D
Tooth avulsion is defined as the displacement of an entire tooth including the root. When this
occurrence involves a permanent tooth, it is important to attempt to preserve the tooth for
reimplantation. Immediately after the injury, if the tooth can be found, it should be cleaned
briefly (less than 10 seconds) with clean water. Ideally, prior to transporting the child for
emergency care, the tooth should be replaced the socket or held in the mouth between the molars
and the cheek (if the child is old enough that there is no risk of choking). If this measure is not
possible or safe, the tooth can be transported in an alkaline liquid such milk. Transporting a tooth
dry or in water should be avoided; a tooth exposed to air for 60 minutes or transported in a non-
alkaline medium is not viable.
Upon arrival to the emergency department, if tooth was already replaced into the socket, it
should be left in place. The area can be flushed with saline and lacerations repaired. If the tooth
has not been replaced, the tooth and socket should be gently cleaned with a saline wash via
syringe. Then, with gentle pressure, the tooth can be replaced into the socket.
After the tooth is replaced, the child or parent should perform twice-daily chlorhexidine (0.1%)
solution rinses for 1 week. Prophylactic antibiotics are recommended. Doxycycline twice daily
for 7 days is the recommended antibiotic for children. Amoxicillin or penicillin are alternative
antibiotics that may be used. Azithromycin, cefdinir, and clindamycin are not recommended as
first-line choices for prophylaxis in this situation.
PREP Pearl
• Pending emergency dental care. an avulsed tooth should be briefly rinsed in water, then
replaced into the socket or held in the mouth between the molars and the cheek (if the
child is old enough that there is no risk of choking)
• If unable to be replaced or held in the mouth during transport for emergency dental care,
an avulsed tooth should be transported in an alkaline medium, such as milk.
• Prophylactic antibiotics (doxycycline or amoxicillin) should be given after an avulsed
tooth is replaced.

• Plan the appropriate management of an avulsed tooth
Suggested Readings
• International Association of Dental Traumatology guidelines for the management of
traumatic dental injuries: 2. Avulsion of permanent teeth. Dent Traumatol,
doi:10.1111/j.1600-9657.2012.01125.x
• Dental Trauma Guide. Avulsion. https://dentaltraumaguide.orq/free-dental-
guides/permanent-teeth/avulsion/.
• The knocked out permanent instructions on pediatric dentistry practice websites;
pediatrics, http://pediatrics.aappublications.org/content/142/1_meetingabstract/427
• Oral Health. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.;
2017:281-291

Question 159
A 16-year-old adolescent patient is seen in the clinic for a follow-up visit for a recent diagnosis
of celiac disease. The adolescent had a 3-month history of bloating, diffuse abdominal pain,
diarrhea, and weight loss, and was diagnosed with Celia disease after confirmatory laboratory
testing and esophagogastroduodenoscopy. The adolescent received appropriate nutrition
education and reports good adherence to the gluten-free diet, however, when drinking milk and
eating ice cream, cottage cheese, or yogurt, the cramping and diarrhea seem to worsen. The
adolescent notes that, prior to 3 months ago, ingestion these foods did not cause any increase in
symptoms.
Of the following, the condition MOST likely to explain these symptoms is

A. Campylobacteriosis
B. Cow milk protein allergy
C. Primary lactase deficiency
D. Secondary lactose intolerance

Correct Answer: D
The adolescent in this vignette has newly diagnosed celiac disease, a small intestinal
enteropathy, resulting in villous atrophy in the presence of gluten ingestion. The adolescent
reports increased symptoms with consumption of foods high in lactose, which is consistent with
secondary lactose intolerance. When the small intestine is injured and villi are atrophied, lactase-
containing cells are temporarily lost. Treatment of the underlying inflammatory condition will
result in recovery of the villi and lactase-containing cells. Other causes of small intestinal
enteropathy include infection (particularly viruses and parasites), inflammatory bowel disease
(e.g., Crohn disease), and other less common immune-related enteropathies.
Lactose, a disaccharide composed of monosaccharides galactose and glucose, is present in milk

products. Metabolism of lactose occurs when lactase enzyme, located in the tips of small
intestinal villi, digests lactose to galactose and glucose, which then are directly absorbed through
the intestinal epithelial cells. When malabsorption of lactose occurs, undigested lactose passes to
the colon and fermentation occurs, with subsequent development of bloating, cramping, diarrhea,
and flatulence.
Lactose intolerance, the development of symptoms after lactose ingestion, may occur in several
scenarios. Congenital lactase deficiency, diagnosed in infancy, is rare. Primary lactase deficiency
occurs in children and adults due to decreasing lactase enzyme in small intestine. Secondary
lactase deficiency occurs with small intestinal enteropathy and resolves when the underlying
condition is treated.
Lactose intolerance can be diagnosed with a good clinical history and documented improvement
in symptoms with removal of lactose-containing foods from the diet. Lactose breath testing
(measurement of expired breath hydrogen levels over 2 to 3 hours after ingestion of a standard
lactose dose), interpreted by a pediatric gastroenterologist, is another method to diagnose lactose
intolerance. Finally, small intestinal biopsies taken during endoscopy performed while looking
for other causes of concerning gastrointestinal symptoms can provide information regarding
lactase concentration.
Management of lactose intolerance includes avoidance of lactose-containing products (Item

C159A and Item Cl59B) and/or use of oral lactase replacement therapy. Some children and
adults, depending on the concentration of lactase present, may be able to ingest smaller amounts
of lactose-containing foods without symptoms. Foods high in lactose, including milk or ice
cream, may cause more symptoms than lower lactose-containing foods like aged cheeses.
Yogurt, although generally considered to be a higher lactose-containing food, may be easier to
tolerate due to naturally occurring bacteria (and therefore partial digestion lactose).
Cow milk protein allergy is a cause of gastrointestinal symptoms, but it is due to an allergy
(either IgE-mediated or non-IgE mediated) and would not be expected to develop with newly
diagnosed celiac disease. Campylobacteriosis, a bacterial infection that can be seen with
ingestion of raw, unpasteurized milk, may be accompanied by bloody diarrhea and would be
expected to last up to 7 days. Primary lactase deficiency is less likely in this patient who did not
have symptoms prior to presentation of celiac disease.

Item C159A: lactose-containing products
Item Cl59B: Hidden Sources of Lactose

• Bread and other baked goods
• Processed breakfast cereals
• Mixes for pancakes, biscuits, and cookies
• Instant potatoes, soups, and breakfast drinks
• Margarine
• Nonkosher lunchmeats
• Salad dressings
• Candies and other snacks
PREP Pearls
• Lactose intolerance occurs when children and adults experience gastrointestinal
symptoms (generally abdominal pain, diarrhea, flatulence, bloating) after lactose
ingestion.
• Secondary lactose intolerance occurs in the setting of another underlying small intestinal
enteropathy and resolves when the underlying condition is treated.
• Treatment of lactose intolerance includes avoidance of high lactose-containing products
and/or use of oral lactase replacement therapy.

• Understand the role of lactose intolerance in the development of chronic abdominal pain
• Differentiate milk protein allergy from lactose intolerance
• Recognize the gastrointestinal causes of secondary lactose intolerance
• Plan appropriate management of lactose intolerance, taking into consideration the
mechanisms causing the disorder
Suggested Readings
• Lactose intolerance in infants, children, and adolescents. Pediatrics,
doi:10.1542/peds.2006-1721
• Lactose intolerance. Asia Pac J Clin Nutr, doi:10.6133/apjcn.2015.24.s1.02
• Milk and health. N Engl J Med, doi:10.1056/NEJMra1903547

Question 160
A 9-year-old girl is being evaluated for paroxysmal episodes. She describes episodes of altered
awareness over the past 3 months, first noted at school. Each event starts with a feeling of
anxiety and restlessness, then a repetitive smacking of her lips and picking at her clothes,
followed by loss of consciousness. She may wander or move her legs as though she is riding a
bicycle. She appears anxious and agitated throughout the episode but is unresponsive to all
external stimuli. Each episode lasts 1 to 2 minutes and self-resolves. She quickly returns to her
neurologic baseline. The girl has no prior history of paroxysmal episodes and no family history
of seizures. Her neurological examination findings are normal.
Of the following, the BEST next step in this girl's management is

B. electroencephalography
C. psychological evaluation
D. urine toxicology screen

Correct Answer: B
The girl in the vignette is experiencing stereotyped, self-resolving paroxysmal events
characterized by altered awareness and automatisms, without clear provocation. Her presentation
is most suggestive of a complex partial (focal-onset impaired awareness) seizure.
Electroencephalography to evaluate for the presence of epileptiform discharges is the best next
step in this girl's management.
Seizures are broadly categorized as generalized or focal. Generalized characterized by bilateral

brain dysfunction at onset, with impaired consciousness occurring as a rule. Focal seizures are
classified by the preservation or loss of consciousness; previously termed simple partial and
complex partial seizures, respectively. Focal seizures are caused by abnormal electrical
discharges from a specific focus in the brain, the location of which determines the clinical
features seen. Focal aware seizures (previously termed simple partial seizures) are often
recognized by the patient as an aura; they can manifest as motor movements or with nonmotor
symptoms (e.g., cognitive, behavioral, sensory, olfactory, visual). Focal impaired awareness
seizures often include automatisms (e.g., mouth movements, picking motion), and, if the frontal
lobes are involved, more complex motor movements such as bicycling of the legs. Focal seizures
can evolve into a generalized event, termed secondary generalized seizures, if the electrical
discharges propagate to involve bilateral brain networks (Item Cl60A).
Item Cl60A: Seizures classifications
When evaluating a first-time event that is concerning for a seizure, a stepwise approach to the
clinical workup and management helps the provider accurately characterize, classify, and make a
diagnosis (Item C160B). The first question to consider is whether the event was a seizure, or if a
seizure mimic may better explain the event. If determined to be a seizure, the next consideration
is whether the event occurred in a provoked setting (e.g., illness, fever, medication/drugs or

significant metabolic derangements). If unprovoked, the seizure should be categorized as
described above.
Item C160B: seizure stepwise approach
Epilepsy is diagnosed using both clinical information (e.g., recurrent seizures) and
electroencephalography (EEG) data (e.g., interictal abnormalities). If a diagnosis of epilepsy is
established, the next step is to determine the type and if a known epilepsy syndrome is present;
this information can help guide management. An underlying etiology should be sought
(structural, autoimmune, genetic, unknown) with second- and third-tier diagnostic studies and
comorbid conditions screened for and managed. A graphic description is shown in Item Cl60C.
Electroencephalography is the initial diagnostic study of choice for a child with a suspected first
time seizure, to evaluate for an underlying seizure tendency. Brain magnetic resonance imaging
is often performed as a second-tine study in focal seizures to evaluate for structural abnormalities
such as focal cortical dysplasia, focal gliosis, or cortical malformations. While urine toxicology
screens are often performed in children presenting with seizure in an acute setting to determine if
there was a provoking nature to the event, EEG is the more definitive diagnostic evaluation to
guide next management steps. Psychological evaluation may be beneficial if paroxysmal
nonepileptic events are strongly suspected or if anxiety and/or depression, two common epilepsy
comorbidities, are present.

Item Cl60C: Epilepsies classification Framework
PREP Pearls
• Focal seizures are caused by abnormal electrical discharges arising from a specific brain
focus, the location of which determines the clinical features seen. They are further
subdivided into focal aware and focal unaware seizures.
• Electroencephalography is the diagnostic study of choice in the evaluation of a first time
seizure to evaluate for underlying seizure tendency (presence of interictal discharges,
background abnormalities) and aid in classification of an epilepsy type/syndrome and
management decisions.
MOCA-Peds Objective
• Recognize and plan initial evaluation of altered mental status.

• Recognize the clinical findings associated with complex partial seizures, and manage
appropriately
Suggested Readings
• Seizures in children. Pediatr Rev, doi:10.1542/pir.2019-0134
• Seizure disorders. American Academy of Pediatrics Textbook of Pediatric Care 2nd ed.
2599-2617
• ILAE classification of the epilepsies: Position paper of the ILAE Commission for
Classification and Terminology. Epilepsia, doi:10.1111/epi.13709

Question 161
A 3-day-old neonate born at 40 weeks' gestation via vacuum-assisted vagina delivery is seen in
the office for a health supervision visit. His physical examination findings are significant for an
asymmetric Moro reflex, active movement against gravity of his left wrist and elbow, and a left
palmar grasp. No movement is noted at his left shoulder. The remainder of his physical
examination findings are normal
Of the following, the MOST likely mechanism explaining this neonate's condition is
A. Avulsion of the cervical nerve roots
B. Congenital myopathy
C. Peripheral neuropathy
D. Stretching of nerves

Correct Answer: D
The neonate in the vignette has a brachial plexus injury (BPI), which results from stretching of
the complex of cervical nerve roots innervating the shoulder, arm, and hand. Brachial plexus
injury is common, with an incidence ranging from 0.4 to 4.6 per 1,000 live births. It occurs most
often when traction is placed on the neonate's neck to aid in the delivery. Risk factors include
shoulder dystocia, macrosomia, maternal delivery of a prior infant with a BPI, maternal diabetes,
maternal obesity, and prolonged labor.
Most commonly, BPI occurs because of disruption of nerve conduction without a change in
neural architecture. This injury, which results from stretching of the nerves or swelling of the
nerve sheath, heals over time with full return of function in most cases. Brachial plexus injury
may also be caused by injury to axons or nerves. When BPI is caused by injury to axons or
nerves, recovery is variable.
The location of the injury determines the clinical findings (Item C161). Injury to cervical nerve
roots C5 to T1 may be accompanied by ipsilateral Horner syndrome, which manifests as miosis,
ptosis, and anhidrosis, hemi diaphragmatic paralysis, and winging of the scapula.
Item C161: Narakas Classification

Group Characteristics Roots
Group I Paralysis of deltoid and biceps. C5-C6
(Duchenne-Erb's Palsy) Intact wrist and digital flexion/extension.
Group II Paralysis of deltoid, biceps, and wrist and digital C5-C7
(Intermediate Paralysis) extension.
Intact wrist and digital flexion.
Group III (Total Brachial Flail extremity without Horner's syndrome C5-T1
Plexus Palsy)
Group IV (Total Brachial Flail extremity with Horner's syndrome C5-T1
Plexus Palsy with Horner's
syndrome)
Initial evaluation of BPI should include a thorough physical examination and chest radiography
to evaluate for fractures of the clavicle and humerus. Neonates should be followed closely with
serial examinations to ensure recovery of function. Those with persistent or severe injury should
be evaluated by a pediatric neurologist, pediatric orthopedist, physical therapist, and/or
occupational therapist. Parents should be taught gentle massage and passive range-of-motion
exercises to aid in recovery. Because BPI is associated with plagiocephaly and torticollis,
providers should stress the importance of tummy time. Recovery is generally better from injury
which causes deficits in the upper arm compared with the lower arm. Residual deficits occur in
20% to 30% of neonates with BPI.
Both congenital myopathy and peripheral neuropathy cause generalized symptoms, not the
isolated findings seen in this neonate. Avulsion of the cervical nerve roots is a rare cause of BPI.

PREP Pearls
• Risk factors for brachial plexus injury include shoulder dystocia, macrosomia, maternal
delivery of a prior infant with a brachial plexus injury, maternal diabetes, maternal
obesity, and prolonged labor.
• Residual deficits occur in up to 20% to 30% of neonates with a brachial plexus injury
• Initial evaluation of brachial plexus injury should include a thorough physical
examination and chest radiography to evaluate for fractures of the clavicle and humerus.

• Recognize the clinical findings associated with brachial plexus injuries, and manage
appropriately
• Recognize the clinical features in an infant whose delivery was complicated by shoulder
dystocia
• Understand the prognosis associated with brachial plexus injuries
Suggested Readings
• Neonatal brachial plexus injury. Pediatr Rev, doi:10.1542/pir.2018-0113
• The newborn with neurologic findings. American Academy of Pediatrics Textbook of
• Nervous system disorders. Nelson Textbook of Pediatrics 21st ed, 2020:913-925

Question 162
A 5-month-old male infant is seen in the emergency department for refusal to feed, a weak cry,
and constipation for the past 2 days. The infant was born at full term via spontaneous vaginal
delivery. The perinatal and postnatal periods were uneventful. He has been breastfed since birth.
His physical examination findings were normal, with appropriate weight gain at his 4-month
health supervision visit. On physical examination today, he is afebrile with normal vital signs.
He is alert but has a weak cry and sucking reflex. He has notable head lag, bilateral ptosis, and
dilated pupils with a sluggish reaction to light. Neurologic examination reveals reduced
movement of all 4 extremities, hypotonia, and decreased stretch reflexes. He responds
appropriately to pain. The remainder of his examination findings are normal.
Of the following, the test MOST likely to confirm the cause of this infant's symptoms is
A. Brain magnetic resonance imaging
B. Cerebrospinal fluid evaluation
C. Ophthalmologic examination
D. Stool studies

Correct Answer: D
The infant in the vignette, with hypotonia, poor head control, weak cry and suck reflex, and a
history of constipation, has a clinical picture suggestive of infant botulism. Detection of the
botulinum toxin on a stool specimen confirms this diagnosis. His clinical presentation is not
consistent with bacterial meningitis (alert with normal vital signs) or cerebrovascular accident
(absence of unilateral weakness and seizure). Therefore, cerebrospinal fluid studies and brain
magnetic resonance imaging would not reveal the infant's diagnosis. Ophthalmologic evaluation
could be helpful if there were cranial nerve palsies complicating infant botulism, but would not
confirm the diagnosis.
Botulism is a neuroparalytic illness caused by a heat-labile neurotoxin secreted by Clostridium

botulinum, a gram-positive, spore-forming, anaerobic microorganism. There are many forms of
botulism including infant, foodborne, and wound botulism.
Infant botulism is the most commonly reported form in the United States. From 2011 to 2015, an
annual average of 125 laboratory-confirmed cases were reported in the United States with a
median age of 16 weeks at presentation. The highest incidence is reported in Pennsylvania,
Delaware, New Jersey, California, and Utah. Infant botulism occurs from ingestion of C
botulinum spores. The source of the spores is usually not identified; known sources include the
environment (e.g., dust, soil) and certain foods (e.g., honey). Infants younger than 12 months of
age should not be given honey because of the known risk. Ingested spores colonize, germinate,
and replicate in the colon, where they produce a neurotoxin that enters the circulation and causes
presynaptic blockage, inhibiting the release of acetylcholine and resulting in flaccid paralysis.
The incubation period for infant botulism ranges from 3 to 30 days after ingestion of spores. The
clinical spectrum is wide, ranging from mild to severe disease characterized by cranial nerve
palsy followed by progressive symmetric descending flaccid paralysis. Common symptoms
include constipation, poor feeding, and weak cry and suck. Fever is typically absent. On physical
examination, affected infants alert but have an expressionless face, ptosis, head lag, decreased
gag and suck reflexes, sluggish pupillary reaction, and generalized hypotonia. Deep tendon
reflexes are decreased later in the disease course. Respiratory failure may result from poor
respiratory effort. Because the neurotoxin does not cross the blood-brain barrier, the sensorium is
normal. Autonomic dysfunction (i.e., labile blood pressures, and heart rate) may accompany the
neurologic abnormalities. Infant botulism in infants younger than 2 months can present with
acute apneic spells or sudden infant death syndrome.
The diagnosis of infant botulism warrants a high degree of clinical suspicion. Infants are often
admitted for evaluation of sepsis, failure to thrive, or dehydration. The differential diagnosis
includes sepsis, meningitis, hypothyroidism, toxin ingestion, and neurologic diseases (e.g., spinal
muscular atrophy, myasthenia gravis, Guillain-Barré syndrome, and cerebrovascular accident).
A definitive diagnosis of infant botulism is made with the detection of botulinum toxin on a stool
specimen or enema fluid. C botulinum may also be isolated from the stool (or enema fluid) using
enriched selective media. Early in the disease course, botulinum toxin may be detected in serum.
Electromyography may demonstrate an incremental response of muscle action potentials on
high-frequency nerve stimulation (20-50 Hz).
When the clinical suspicion of infant botulism is high, treatment should not be delayed while
awaiting laboratory results. Treatment includes the administration of human botulism immune
globulin intravenous (BIG-IV) and supportive care. This human-derived botulinum antitoxin
neutralizes the neurotoxin and halts the progression of neuromuscular blockade. In a controlled
trial, treatment for infant botulism with BIG-IV was safe and resulted in decreased length of
mean hospital stay, intensive care unit stay, and days on mechanical ventilation. Botulism
Immune globulin intravenous is available from the California Department of Public Health (24-
hour contact number: 510-231-7600: www.infantbotulism.orq).
Antibiotics have no role in the management of infant botulism unless there is evidence of
secondary bacterial infection complicating the illness (such as aspiration pneumonia).
Aminoglycosides are contraindicated in suspected cases of infant botulism or sepsis as they can
worsen paralysis. Cases of botulism must be reported to the local and state public health
department.
PREP Pearls
• The clinical features of infant botulism include constipation, poor feeding, cranial nerve
palsy, head lag, and progressive symmetric descending flaccid paralysis.
• The laboratory diagnosis of infant botulism is made by detection of the botulinum toxin
on a stool or enema fluid specimen.
• Treatment of infant botulism includes the administration of human botulism immune
globulin intravenous (BIG-IV) and supportive care. Botulism immune globulin
intravenous is available from the California Department of public Health (24-hour
contact number: 510-231-7600; www.infantbotulism.org

• Plan appropriate management for a patient with botulism
• Plan appropriate diagnostic evaluation of botulism
• Recognize the clinical features associated with botulism
Suggested Reading
• Botulism and infant botulism (Clostridium botulinum). Red Book: 2021-2024 Report of
the Committee on Infectious Diseases. 32nd ed; 2021:266-268.
• Honey pacifier use among an indigent pediatric population. Pediatrics,
doi:10.1542/peds.2012-3835
• Botulism. Pediatr Rev, doi:10.1542/pir.2015-0018.
• Fatigue and weakness. American Academy of Pediatrics Textbook of Pediatric Care 2nd
ed.; 2017:1345-1351.

Question 163
A 24-month-old typically developing girl is seen for a routine health supervision visit. She can
jump, kick a ball, and throw a ball overhand.
Of the following, she is MOST likely to also be able to

A. balance on 1 foot for 3 seconds
B. catch a ball with stiff arms
C. walk down stairs while holding the rail
D. walk upstairs with alternating feet

Correct Answer: C
The 2-year-old, typically developing girl in the vignette is most likely able to walk down stairs
while holding a handrail. Two-year-old children typically walk down stairs by placing both feet
on each step, rather than alternating their feet. The other response choices (balance on l foot for 3
seconds, catch a ball with stiff arms, and walk upstairs with alternating feet) are gross motor
milestones typically seen in 3 year old. Developmental milestones typically seen in 2-year-old
children are shown in Item C163.
Item C163: Developmental milestones typically seen in 2-year-old children
Developmental surveillance should occur at all health supervision visits. Children should be
screened for developmental delays with a validated tool at the 9-month, 18-month, and 24- or 30-
month visits, or any time there are concerns for atypical development.
PREP Pearls
• The typical 24-month-old can jump, kick a ball, throw a ball overhand, and walk down
stairs holding the rail, placing both feet on each step.
• Developmental surveillance should occur at all health supervision visits.
• Children should be screened for developmental delays with a validated tool at the 9-
month, 18-month, and or 30-month visits, or any time there are concerns for atypical
development.
MOCA-Peds Objective
• Evaluate the developmental status of a child at 24 months of age

• Evaluate the cognitive and behavioral developmental progress/status of a child at 24
months of age, including recognition of abnormalities
• Evaluate the motor developmental progress/status of a child at 24 month of age
Suggested Readings
• Developmental milestones: Motor development. Pediatr Rev, doi:10.1542/pir.31-7-267
• Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents.
4th ed. 2017.
• Council on Children with Disabilities, Section on Developmental and Behavioral
Pediatrics. Promoting optimal development Identifying infants and young children with
developmental disorders through developmental surveillance and screening. Pediatrics,
doi:10.1542/peds.2019-3449

Question 164
An unsupervised 2-year-old boy is found by his parents in the family garage near an open bottle
of an unknown liquid. He is minimally responsive and is transported to the emergency
department by ambulance. On physical examination, he has labored respiratory effort with
diffuse wheezing and is somnolent. An arterial blood gas measurement with electrolytes is
obtained, and the data are shown:

pH 6.83
PCO2 47 mmHg
PO2 100 mm Hg
Of the following, the substance MOST likely ingested by the boy is

A. ethanol (grain alcohol)
B. isopropyl alcohol (rubbing alcohol)
C. kerosene (lamp oil)
D. paint thinner (mineral spirits)

Correct Answer: A
The laboratory data for the boy in the vignette reveal a metabolic acidosis. In addition, a
significant increase is seen in the anion gap, which is 30 mEq/L. Of the answer choices, the
ingestion most likely to cause an increased anion gap metabolic acidosis is ethanol. Ethanol is
the main form of alcohol in grain alcohol, which is used in common libations such as beer, wine,
vodka, gin, moonshine, and other spirits. Although the ingestion of ethanol itself does not
necessarily generate a metabolic acidosis, ethanol can cause an increase in both lactic acid and
ketoacids production and thus produce an anion gap acidosis as a result of the byproducts of
metabolism. Ethanol also causes reduced hepatic gluconeogenesis, decreased insulin secretion,
increased lipolysis, impaired fatty acid oxidation, and subsequent ketogenesis, leading to an
elevated anion gap metabolic acidosis.
The anion gap is a function of the electroneutrality of the blood; the total number of serum
cations must necessarily equal the total number of anions. The normal anion gap value for
otherwise healthy children is 12 ± 2 mEq/L. A high anion gap indicates the presence of more
anions than cations. When bicarbonate (or other buffer) is exhausted to correct the acid-base
balance, the anion gap increases. In contrast, processes in which bicarbonate is lost in the stool or
urine result in the loss of electronegative bicarbonate, and electroneutrality is recovered by the
retention of chloride. When chloride is retained, a normal anion gap acidosis may be present.
Calculation of the anion gap relies on measurement of serum electrolytes and is made by
subtracting the major extracellular anions from the cations. In its simplest form, the anion gap is
calculated using the following equation:
Anion Gap = (Na+ +K+) — (Cl- + HCO3-)
An increased anion gab metabolic acidosis has a limited number of causes. The mnemonic
"MUDPILES' helps to identify the common causes of an anion gap metabolic acidosis.
• Methanol
• Uremia
• Diabetic ketoacidosis
• Paraldehyde
• Iron/isoniazid
• Lactic acid
• Ethanol/ethylene glycol
• Salicylates
Although no longer commercially manufactured in the United States, paraldehyde is an

antiepileptic that was used until the mid-1970s and was also indicated for the treatment of
delirium tremens, an often fatal alcohol withdrawal syndrome. Paraldehyde was also used as a
sedative, which was attractive because it did not induce respiratory depression. Methanol and
ethylene glycol are known to cause an anion gap metabolic acidosis. Methanol is derived from
wood and is a common ingredient in windshield washer fluid. Ethylene glycol is used in
automobile antifreeze. Methanol and ethylene glycol are both primary alcohols that are oxidized
to carboxylic acids. The toxicity or these acid metabolites can cause severe complications,
including blindness (methanol) and renal failure (ethylene glycol).
Ingestion of isopropyl alcohol (rubbing alcohol) does not cause an elevated anion gap metabolic
acidosis. Isopropyl alcohol is a secondary alcohol that is metabolized to a ketone rather than an
aldehyde. Ketones cannot be oxidized to carboxylic acids and thus lead to only limited acidemia.
Kerosene and paint thinner are hydrocarbons which, when ingested, inflict systemic toxicity with
effects generally limited to central nervous system depression, seizures, and/or ventricular
arrhythmias caused by myocardial sensitization. Hydrocarbon aspirations, however, can cause
life-threatening acute lung injury and hypoxia. Hydrocarbon ingestion itself is not a common
cause of metabolic acidosis.
PREP Pearls
• A wide anion gap metabolic acidosis has a limited number of causes.
• The anion gap is easily calculated as follows: Anion Gap = (Na+ +K+) - (Cl- + HCO3-)
• Isopropyl alcohol ingestion does not cause an anion gap metabolic acidosis.
APP Content Specifications(s)

• Identify the arterial blood gas abnormalities associated with an acid-base imbalance
• Recognize the clinical and laboratory features associated with metabolic acidosis, and
• Identify factors contributing to metabolic acidosis
• Formulate a differential diagnosis of acidosis associated with various anion gap values
Suggested Readings
• Physiological approach to assess acid-base disturbances. N Engl J Med,
doi:10.1056/NEJMra1003327
• Acid-base disorders. Pediatr Rev, doi:10.1542/pir.2015-0093
• Toxic alcohol ingestions: clinical features, diagnosis. and management. J Am Soc
Nephrol, doi:10.2215/CJN.03220807

Question 165
A 4-year-old girl is brought to the emergency department by her parents with a rash and facial
swelling. Her mother reports that they were at a family cookout when the girl was stung on the
right arm by a bee. Within minutes she developed swelling, pain, and a raised, diffuse, red/ itchy
rash. Her mother noted swelling of her lips and eyes. Soon after, the girl developed difficulty
breathing and had nonbloody, nonbilious emesis once. No medications were given prior to her
arrival. Her vital signs are a temperature of 37.6 º C, heart rate of 130 beats/min, respiratory rate
34 breaths/min, and blood pressure of 105/72 mm Hg. Her weight is 22 kg. She appears anxious
and has clear rhinorrhea; periorbital edema; tachypnea with expiratory wheezes bilaterally;
tachycardia; and multiple raised, erythematous lesions on her arms and trunk.
Of the following, the BEST next step in the management of this child is administration of
A. corticosteroid intravenously
B. diphenhydramine, famotidine, and a corticosteroid orally
C. epinephrine intramuscularly
D. isotonic crystalloid bolus 20 mL/kg intravenously

Correct Answer: C
The girl in the vignette has anaphylaxis from a bee sting (Hymenoptera family). Acute allergic
reactions can range from mild to severe. Anaphylaxis is the sudden, life-threatening, IgE-
mediated, systemic reaction to an allergen, which may be known or unknown. Anaphylaxis has
been defined as an acute reaction that involves 2 or more organ systems (e.g., skin,
gastrointestinal, cardiovascular, respiratory) after exposure to an allergen. The definition has
been expanded to include the isolated finding of hypotension after exposure to an allergen.
Symptoms of anaphylaxis may include rash, flushing, pruritus, rhinorrhea, stridor, cough,
wheeze, difficulty breathing, abdominal pain, diarrhea, vomiting, dizziness, syncope, and
hypotension. Approximately 10% of children with anaphylaxis do not have cutaneous
involvement.
The most common trigger of allergic reactions is food, particularly nuts and seafood, followed by
medications, stinging insects, and latex. The onset of symptoms is usually rapid, typically
occurring within minutes to a few hours after an exposure, but symptoms may be delayed up to
24 hours. Approximately 20% of children will experience a biphasic latent anaphylactic reaction
with recurrence of symptoms 4 to 24 hours after the initial reaction.
First-line treatment of anaphylaxis is the immediate administration of 0.01 mg/kg of 1:1,000

concentration of intramuscular epinephrine. Intramuscular administration is preferred over
subcutaneous due to more efficient absorption, Delayed administration of epinephrine has been
associated with fatal outcomes. Repeat intramuscular epinephrine may be required if there is an
inadequate response or in children with biphasic reactions. Adjuvant therapies include
corticosteroids, H1 receptor antagonists such as diphenhydramine, H2 receptor antagonists such
as famotidine, inhaled ß-agonists such as albuterol, and administration of intravenous isotonic
crystalloid fluids. The girl in the vignette requires immediate treatment with epinephrine. The
administration of oral diphenhydramine, famotidine, and oral or intravenous corticosteroids may
be appropriate for use in this case but should be initiated only after epinephrine is given. The girl
has a normal blood pressure and is not displaying signs of shock, so administration of an
intravenous fluid bolus would not be the appropriate next step in management.
Epinephrine is the mainstay of treatment for anaphylaxis; adjuvant therapies such as

corticosteroids, histamine blockers, and inhaled ß-agonists should not be used in its place. It is
important for children with a history of anaphylaxis to be prescribed an epinephrine auto-injector
and their families educated on its use. The correct dose of auto-injector epinephrine is 0.15 mg
for children less than 30 kg and 0.3 mg for children greater than or equal to 30 kg. Long-term
management should include identification of the allergen when possible, and family education
regarding avoidance. All caregivers, including extended family members, schools, and workers,
should receive education on emergency food avoidance plan epinephrine auto-injector use, and
an allergy and anaphylaxis emergency plan (found at
https://www.healthychildren.org/SiteCollectionDocuments/AAP_allergy_and_Anaphylaxis_Eme
rgency_Plan.pdf)
Children with mild to moderate allergic reactions with no signs or symptoms of anaphylaxis,
including reactions to stinging insects, may be safely managed at home by application of a cold

compress, administration of oral or antihistamines and topical corticosteroids, and
implementation of supportive symptomatic care.
PREP Pearls
• Anaphylaxis is the IgE-mediated, acute, life-threatening reaction to an allergen.
• The treatment for acute anaphylaxis is 0.01 mg/kg of 1:1,000 concentration of
epinephrine. The correct dose of auto-injector epinephrine is 0.15 mg for children less
than 30 kg and 0.3 mg for children greater than or equal to 30 kg. The preferred route is
Intramuscular.
• Adjuvant therapy for anaphylaxis may include corticosteroids, H1 and H2 receptor
antagonists, inhaled ß-agonists, and intravenous fluid replacement
APP Content
• Recognize the clinical findings associated with life-threatening reactions to Hymenoptera
stings, and manage appropriately
• Recognize the signs and symptoms of anaphylaxis, and manage appropriately
• Recognize the reactions to insect stings that require no further management in patients
younger than 16 years of age
• Instruct families regarding the acute management of sting anaphylaxis at home
Suggested Readings
• Insect Sting Anaphylaxis. Immunol Allergy Clin North Am,
doi:10.1016/j.iac.2007.03.008
• Anaphylaxis, urticaria and Angioedema. Pediatr Rev, doi:10.1542/pir.34-6-247
• Improving anaphylaxis care: The Impact of a clinical pathway. Pediatrics,
doi:10.1542/peds.2017-1616
• Anaphylaxis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:2791-2799
• Anaphylaxis. Pediatr Rev, doi:10.1542/pir.29-8-255

Question 166
A 16-year-old adolescent boy is evaluated in the clinic for a 2-month history of worsening
epigastric abdominal pain. The pain is described as severe, occurs after meals, and is
accompanied by nausea. A few weeks ago, he vomited dark brown, granular material, but has not
had any further vomiting. He reports no change in stool color or pattern; he has a soft, brown
stool daily. Antacids have been used intermittently, with some resultant improvement in pain.
The adolescent reports a poor appetite and a 15-1b weight loss since his symptoms began. He has
a several-year history of frequent headaches, treated with ibuprofen. He reports no other over
the-counter or recreational drug use. Physical examination findings are significant for a body
mass index 20.5 kg/m2 (50th percentile for age, decreased from 85th percentile for age) and
epigastric tenderness with palpation, without guarding.
Of the following the BEST treatment for this adolescent's condition is

A. a gluten-free diet
B. Helicobacter pylori eradication treatment
C. pancreatic enzyme replacement therapy
D. proton pump inhibitor therapy

Correct Answer: D
The adolescent in the vignette has a history consistent with peptic ulcer disease (e.g., severe,
postprandial abdominal pain, history of hematemesis, weight loss, with epigastric tenderness),
and acid-suppressing treatment with proton pump (PPI) therapy should be initiated. Chronic use
of ibuprofen, a nonsteroidal anti-inflammatory drug (NSAID), increases the risk for development
of peptic ulcer disease (PUD) in this adolescent. Gastroenterology referral, and consideration of
endoscopic evaluation, is also indicated.
Peptic ulcer disease, part of the spectrum of acid-related disorders (which also includes gastritis),
is rare in children. It is identified in 2% to 5% of children undergoing endoscopy, and in up to
4.4 per 100,000 hospitalized children, peptic ulcers can be caused by infection (e.g.,
Helicobacter pylori, cytomegalovirus, Epstein-Bart virus, fungi, and parasites), medications
(most commonly corticosteroids and NSAIDs), stress or trauma, immune-related gastritis (e.g.,
inflammatory bowel disease), and hypersecretory states (e.g., Zollinger-Ellison syndrome).
Signs and symptoms of PUD vary by age. Younger children often have nonspecific symptoms
including irritability, difficulty feeding, regurgitation and/or vomiting, or poor weight
gain/weight loss. Older children may report epigastric pain, regurgitation and/or vomiting,
nausea, and weight loss. The presence of upper gastrointestinal bleeding (e.g., hematemesis,
melena, or brisk hematochezia) should raise immediate concern for POD and result in urgent
gastroenterology consultation.
The management of PUD is dependent on the severity of symptoms. Endoscopy is indicated in

children with signs of upper gastrointestinal bleeding, both as a diagnostic and therapeutic
intervention. Therapeutic endoscopy is indicated to treat actively bleeding ulcers, with
maneuvers including thermal coagulation, endoclip application, hemostatic spray, and
epinephrine injection. Endoscopy also allows for collection of biopsy specimens, thus evaluating
the cause of the peptic ulcer. Medications, including PPIs, histamine-2 receptor antagonists
(H2RAs), and surface-coating agents (sucralfate) can be used to treat peptic ulcers (Item C166)
Children with suspicion for PUD but milder symptoms may be empirically treated with
medications (e.g., PPI, H2RAs, sucralfate). Endoscopy should be considered for children with
refractory or recurrent symptoms, or if the clinical impression is concerning for a non-acid
related disorder (e.g., inflammatory bowel disease)
Helicobacter pylori eradication treatment is not indicated without proven H pylori infection. A
gluten-free diet may be helpful in celiac disease, but would not improve outcomes in peptic ulcer
disease. Pancreatic enzyme replacement therapy would be beneficial in treating exocrine
pancreatic insufficiency; however, it would not be used for treatment of peptic ulcer disease.

PREP Pearls
• Signs and symptoms that cause concern for peptic ulcer disease in children include
epigastric abdominal pain, vomiting, hematemesis, weight loss, poor appetite, and
nausea.
• Endoscopy is indicated in children with bleeding due to peptic ulcer disease, refractory
peptic ulcer disease symptoms, or recurrent peptic ulcer disease symptoms.
• Medical treatment of peptic ulcer disease can include use of proton pump inhibitors,
histamine-2 receptor antagonists, and surface-coating agents (sucralfate).

• Plan the appropriate evaluation of suspected ulcer disease not caused by Helicobacter
pylori
• Identify the risk factors associated with ulcer disease (other than that caused by
Helicobacter pylori)
• Plan the appropriate management of ulcer disease not caused by Helicobacter pylori
infection
• Recognize the clinical features associated with acid-peptic disorder in a patient with-
recurrent abdominal pain
Suggested Reading
• Gastrointestinal hemorrhage. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017: 1379-1386,
• Gastritis, gastropathy, and ulcer disease. Pediatric Gastrointestinal and liver Disease;
2015; 294-308.
• Pediatric gastritis, gastropathy, and peptic ulcer disease. Pediatr Rev,
doi:10.1542/pir.2017-0234

Question 167
An 8-year-old girl is being evaluated for a rash on her hand that developed 2 months ago. It
began as a "bump' and has become larger, forming a ring. Her parents applied an over-the-
counter antifungal cream without improvement. Her physical examination findings are
remarkable only for a skin-colored annulus with an elevated border overlying the 4th
metacarpophalangeal joint of her left hand (Item Q167). The border is firm to palpation.
Item Q167: Annulus on the left hand of the girl in this vignette. Courtesy of D. Krowchuk
Of the following, the most appropriate treatment is

A. Clotrimazole topically
B. griseofulvin orally
C. hydrocortisone 1 % topically
D. no intervention

Correct Answer: D
The girl in this vignette has a skin-colored ting (i.e., annulus) with an elevated border and central
clearings. The lesion has been treated unsuccessfully with a topical antifungal agent. Although
the physical findings are reminiscent of tinea corporis (Item Cl67A), the absence of erythema
and scale supports a diagnosis of granuloma annulare (GA). The lesions of GA resolve
spontaneously within 2 to 4 years; no, intervention is required. Clotrimazole is effective in the
management of tinea corporis, and griseofulvin would be indicated in resistant infections or
infections characterized by large or numerous lesions. An emollient and topical corticosteroid
would be useful in managing nummular eczema.
Item Cl67A: tinea corporis
Granuloma annulare is a relatively common disorder with an unknown cause. It usually occurs in
school-aged children and affects girls more often than boys. Although an association with
diabetes mellitus has been suggested in adults, this is not the case in children. Granuloma
annulare is characterized by papules that form a ring; occasionally the ring is incomplete (Item
C167B). Lesions may occur at anybody site but commonly involve areas that are prone to trauma
like the dorsa of the hands or feet. The lesions of GA may be skin-colored but often are
erythematous (Item Cl67B) or Violaceous (Item C167C). Most individuals have a single lesion,
but multiple lesions may occur (Item Cl 67C). Because the pathology of GA occurs in the
dermis, lesions have a “deeper” or “firmer” feel than the border of a tinea corporis lesion. A
subcutaneous form of GA is characterized by multiple subcutaneous nodules with normal
overlying skin. The diagnosis of GA is made clinically and may be confirmed with a skin biopsy,
although this is rarely required.

Item C167B: At times, the ring of granuloma annulare may be incomplete. In this patient, the
ring is slightly erythematous. Courtesy of D. Krowchuk
Item C167C: Lesions of granuloma annulare often are violaceous in color. Some individuals
develop multiple lesions. Courtesy of D. Krowchuk
Granuloma annulare is often confused with tinea corporis. It may also mimic nummular eczema
(Item Cl67D). However, nummular eczema does not exhibit border elevation or central clearing.
In addition, a crust is present in nummular eczema. Subcutaneous GA may mimic a soft-tissue
malignancy, rheumatoid nodules, or sarcoidosis.

Item C167D: Nummular eczema is characterized by oval or round lesions that form crust
(arrows). Reprinted with permission from Mancini AJ, Krowchuk DP, eds. Pediatric
Dermatology. A Quick Reference Guide.3rd ed. Elk Grove Village, IL; American Academy of
Pediatrics: 2016, page 34
PREP Pearls
• Granuloma annulare lesions consist of papules that form a ring that may mimic tinea
corporis.
• Granuloma annulare may be differentiated from tinea corporis by the absence of scale,
firmness of the borders and tendency of the lesions to have a violaceous color
• Granuloma annulare lesions resolve spontaneously within 2 to 4 intervention is required.

• Differentiate the clinical findings of tinea corporis from those of granuloma annulare, and
Suggested Readings
• Granuloma annulare. Pediatric Dermatology: A Reference Guide. 4th; 2020:497-499.
• Rash. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed; 2017:1545-
1553
• Granuloma annulare. Clinical and histologic variants, epidemiology, and genetics. J Am
Acad Dermatol. doi:10.1016/j.jaad.2015.03.055

Question 168
A 17-year-old adolescent girl was prescribed the wrong oral contraceptive pill. She is sexually
active with 1 male partner. She has not experienced any side effects.
Of the following, disclosure of this error is BEST made to the adolescent and
A. her parents
B. her partner
C. no one else
D. the pharmacist

Correct Answer: C
The error described in the vignette should be disclosed to the adolescent because she has
autonomy and control over her reproductive health care decisions. If the error occurred in
relation to a different facet of her medical care, her parents would need to be informed about the
error. The pharmacist and her sexual partner do not require provider disclosure.
Medical errors occur frequently in all areas of medicine as highlighted by the 1999 Institute Of
Medicine Report, To err is human. Prevention of medical errors requires a systematic approach
focusing on systems-level changes rather than targeting individual providers. In all clinical
settings there are multiple layers of protection against medical errors.
Disclosure of medical errors has become standard clinical practice. However, it is encouraged,
not mandated. In several states, legislation protects clinicians who discuss medical errors from
future litigation related to the disclosure. In pediatric Practice, families should be informed of
medical errors irrespective of whether the event affected the patient. Disclosure should occur as
soon as possible after the event and involve the primary parties. It is not necessary to provide an
explanation for why the error occurred. Instead, clinicians should provide an apology and assure
families that the error will be investigated to prevent future occurrences.
There is ongoing discussion about whether pediatric patients should be included in the disclosure
of medical errors. Similar to discussions of autonomy for adolescents, the decision to involve
pediatric patients depends on the situation and the patient’s capacity and decision-making ability.
Advocates of disclosure to children suggest this involvement will increase agency and
willingness to follow care plans. Others argue these events can be distressing to the child.
PREP Pearls
• Prevention of medical error should focus on systems issues.
• Medical errors should be disclosed as soon as possible after the event and involve the
primary parties.
• The decision to involve pediatric patients in disclosure of medical errors depends on the
situation and the patient's capacity and decision-making ability.
MOCA-Peds Objective

• Recognize the relative role of systems and individuals in producing medical error and
harm
Suggested Readings
• Ethics for the pediatrician: autonomy, beneficence, and rights. Pediatr Rev, doi:
10.1542/pir.31-6-252
• Disclosure of medical errors. Pediatr Rev, doi: 10.1542/pir.2018-0228

Question 169
A full-term female neonate is seen in the neonatal intensive care unit for lumbar
myelomeningocele. The pregnancy was otherwise uneventful. The remainder of the physical
examination findings are normal. Family history is noncontributory.
Of the following, the MOST likely inheritance pattern for the neonate's examination
A. autosomal
B. mitochondrial
C. Multifactorial
D. sex-linked

Correct Answer: C
The neonate in the vignette has a neural tube defect (NTD). Neural tube defects exhibit a
multifactorial inheritance pattern, which involves the interaction of gene(s) contributed from
both sides of the family and the environment. Common multifactorial conditions seen in children
include NTDs, cleft lip and palate, and pyloric stenosis.
Neural tube disorders are among the most common birth defects seen in 1 to 2 per
1,000 live births. Neural tube defects result from abnormal embryonic development of the central
nervous system. Defects can occur anywhere along the spine or in the brain; the most common
types are spina bifida and anencephaly. Neural tube defects are categorized as open, where there
is an external outpouching and/or exposure of neural tissue (e.g., anencephaly,
myelomeningocele) or closed where there is an epithelial covering without neural tissue
exposure (e.g., meningocele).
The majority of NTDs can be diagnosed prenatally by ultrasonography performed after 15

weeks’ gestation, with findings such as abnormal skull shape (i.e., lemon sign), abnormal
cerebellum shape (i.e., banana sign), hydrocephalus, or the presence of neural tissue in a sac.
Open defects can be identified through detection of α-fetoprotein on maternal serum screening
during the second trimester. Open defects are often associated with other anomalies, most
commonly renal, cardiovascular, musculoskeletal, or oral clefts, and generally have worse
neurological outcomes in comparison to closed defects.
Genetic causes of NTDs are not well understood. Known genetic etiologies are identified in < 10
% of cases and include chromosomal abnormalities such as trisomy 13, trisomy 18, triploidy, and
some single gene defects (e.g., those in the folate metabolism pathway). The majority of cases
exhibit a sporadic isolated non-syndromic pattern of inheritance. The prevalence of NTDs
females is 5 times that in males. The recurrence risk for a couple to have another child with NTD
is 3% to 5%. Modifiable risk factors include maternal diabetes, maternal obesity, maternal
hyperthermia, drugs (particularly valproate), inadequate maternal nutritional status (e.g., low
folate. zinc, or vitamin B12 level), alcohol use, and smoking.
Folic acid supplementation has been shown to reduce the risk of NTDs. Because the process of
neural tube closure in the human embryo occurs between days 17 to 28 after fertilization, the
defect has usually occurred before the mother is aware of the pregnancy. Hence, to help prevent
NTDs, the Centers for Disease Control and Prevention recommends intake of 400 µg of folic
acid each day for all women of reproductive age, in addition to a diet including folate-containing
foods. In 1992, the US and Drug Administration (FDA) required folate fortification of all
enriched cereal grain products to help ensure adequate folate intake in this age group. For
women with a previous NTD-affected pregnancy, 4,000 µg of folate daily 1 month prior to
becoming pregnant and during the first 3 months of pregnancy is recommended.
Autosomal and sex-linked in inheritance patterns can be traced through families, with clearly
defined disease-causing changes in a single gene in an autosome (chromosome 1 through 22) or
a sex chromosome (X or Y). Mitochondrial inheritance involves the mitochondrial genome
inherited from the egg cell. Hence defects in mitochondrial DNA have a maternal inheritance

pattern; an affected mother will pass the mutation to all her offsprings, and none can be passed
from affected father. The neonate in the vignette does not exhibit these inheritance patterns.
PREP Pearls
• Multifactorial inheritance involves the interaction of gene(s) contributed both sides of the
family and the environment.
• Common multifactorial conditions seen in children include neural tube defects cleft lip
and palate, and pyloric stenosis.
• Folic acid supplementation has been shown to reduce the risk of neural tube defect.
Intake of 400 µg of folic acid each day, in addition to a diet including folate-containing
foods, is recommended for all women of reproductive age,

• Understand the role of multifactorial inheritance in genetic disorders
Suggested Readings
• Overview on neural tube defects: From development to physical characteristics. Birth
Detects Res, doi:10.1002/bdr2.1380
• Neural tube defects. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed; 2017:2374-2379
• Centers for Disease Control and Prevention. Recommendations: Women & folic acid.
https://www.cdc.gov/ncbddd/folicacid/recommendations.html
• Folic acid and primary prevention of neural tube defects: A review, Reprod Toxicol,
doi:10.1016/j.reprotox.2018.05.004

Question 170
A 15-year-old adolescent boy is brought to the emergency department with a 2 day history of
fever; sores in his mouth and on his genitalia; red, irritated eyes, and a diffuse rash, including on
his palms and soles. Approximately 1 week ago, he completed a course of trimethoprim-
sulfamethoxazole for cellulitis on his right leg, which has since resolved. Physical examination
reveals an ill-appearing adolescent with a temperature of 39.1 ºC, respiratory rate of 22
breaths/min, heart rate 100 beats/min, and blood pressure of 115/70 mm Hg. His conjunctivae
are erythematous; his lips are dry, peeling, and bleeding with erosions (Item 170A); he has
several erosions in his oropharynx. There are multiple 5-mm, soft, nontender, bilateral
submandibular and cervical lymph nodes; and scattered erythematous annular vesicular lesions
on his palms (Item Q170B) and soles and progressing up his extremities. There is a 2-cm
circumferential area of desquamation around his urethral meatus (Item Q170C).
Of the following, the BEST treatment for this boy’s condition is

A. Corticosteroids
B. Immune globulin
C. Plasmapheresis
D. Supportive care

Correct Answer: D
Of the choices provided, supportive care is the best treatment option for this child. The boy
described in the vignette has Stevens-Johnson syndrome (SJS), an uncommon, severe
mucocutaneous reaction that can be caused by infectious agents or medications (Item C170),
such as trimethoprim-sulfamethoxazole (TMP-SMX), administered 4 days to 4 weeks prior to
symptom onset. Clinical manifestations include a prodrome of fever, malaise, and upper
respiratory symptoms. This is followed by the development of mucosal lesions affecting the
eyes, mouth, and genitalia and diffuse cutaneous lesions (frequently target-shaped macules) that
are often tender and may progress to vesicles and bullae, The acute phase of SJS may last 8 to 12
days. Reepithelialization may last for several weeks, during which skin may desquamate and
nails may shed. Severe cases of SJS may result in extensive fluid loss due to eroded skin,
electrolyte abnormalities, hypovolemic shock, acute respiratory distress syndrome, and
multiorgan failure; in such cases, there is a high risk for bacterial infection and septic shock.
Item C170: Causes of SJS

• Medications (most common)
o Antibiotics
o mainly sulfonamides less often penicillins, clindamycin, and quinolones
o anticonvulsants (eg, phenobarbital, phenytoin, carbamazepine, and lamotrigine)
o oxicam-type NSAIDs
o allopurinol
• Infections,
o particularly Mycoplasma pneumoniae
o viruses (Coxsackie, enteroviruses, influenza, mumps, hepatitis)
o bacteria (Group A Streptococcus, rickettsia, diphtheria, brucella, tularemia, typhoid, and
Lymphogranuloma venereum)
o fungi (coccidia, histoplasma)
o protozoa (malaria and trichomonas).
The standard of care for SJS is discontinuation of the offending medication and supportive care,
such as electrolyte and fluid management, nutritional support, pain control, wound care, and
prevention and management of complications. While several medications have been used in
clinical practice to manage SJS, there are no established therapies. The efficacy of several
adjuvant therapies including corticosteroids, immune globulin, and plasmapheresis, have not
been evaluated with randomized controlled trials and are not standard of care. Furthermore, there
is controversy regarding the use of corticosteroids in SIS given the theoretical risk of sepsis, and
the use of immune globulin in SJS in light of potential hematologic renal, and thrombotic
complications. Plasmapheresis has not been shown to improve mortality rates length of stay, or
time to re-epithelialization in children with SJS.

PREP Pearls
• Stevens-Johnson syndrome is an uncommon, severe mucocutaneous reaction that may be
caused by infectious agents or medication, such as sulfonamides.
• Clinical manifestations of Stevens-Johnson syndrome include a prodrome of fever,
malaise, and upper respiratory symptoms; this is followed by the development of mucosal
lesions affecting the eyes, mouth, and genitalia and diffuse target-shaped cutaneous
lesions that may progress to vesicles and bullae.
• The standard of care for Stevens-Johnson syndrome is discontinuation of the offending
medication and supportive therapy.

• Recognize the clinical features associated with a drug allergy or hypersensitivity, and
Suggested Readings
• Drug eruptions, erythema multiforme, Steven Johnson syndrome. American Academy of
Pediatrics Textbook of Pediatric Care. 2nd ed; 2017:1984-1992.
• Systematic review of treatments for Stevens Johnson syndrome and toxic epidermal
necrolysis using the SCORTEN score as a tool for evaluating mortality, Ther Adv Drug
Saf, doi:10.1177/2042098611404094
• Cutaneous reactions to drugs in children. Pediatrics, doi:10.1542/peds.2005-2321
• Stevens-Johnson syndrome and toxic epidermal necrolysis: associations and outcomes,
and pathobiology: thirty years of progress but still much to be done. J Invest Dermatol.
doi:10.1016/j.jid.2017.01.003

Question 171
A 7-year-old boy is being evaluated for anal pruritus that wakes him up at night. Six months ago,
he was diagnosed with a pinworm infection after he returned from a summer camp. He was
treated with 2 doses of albendazole 2 weeks apart, and his symptoms completely resolved. His 3
asymptomatic older siblings were not treated. No one else at home has symptoms at this time. A
cellulose tape test detects the eggs shown (Item Q171)
Item Q171: Microscopic findings for the girl described in the vignette. (A, B) Enterobius egg(s).
(C) Enterobius eggs on cellulose tape prep. Courtesy of the Centers for Disease Control and
Prevention
Of the following, the BEST next management step is

A. albendazole for all household members
B. albendazole for the affected child
C. ivermectin for all household members
D. no treatment recommended

Correct Answer: A
The boy in the vignette has anal pruritus caused by a pinworm (Enterobius vermicularis)
infection. Reinfection from pinworm is very common and can be difficult to eradicate. Because
of high transmission rates among family members, treatment of reinfection should include an
antiparasitic agent for the affected child along with all household members. Treatment options
include 2 doses of 1 of the following medications administered orally 2 weeks apart:
1) Pyrantel pamoate (11 mg/kg/dose); available over the counter
2) Albendazole (400 mg/dose)
3) Mebendazole (100 mg/dose)
Single-dose treatments are effective against adult worms. A repeat dose 2 weeks later is
recommended for eradication of eggs and hatching larvae. Ivermectin is an option for the
treatment of E vermicularis but is not recommended as the first-line agent.
Enterobius vermicularis is a white, thread-tike roundworm that lives in the cecum and appendix
(Item C171). The life cycle begins with deposition of eggs by the gravid adult female worm in
the perianal folds. Scratching of the perianal region and transfer of eggs to the mouth via
contaminated hands is responsible for autoinfection. After ingestion, the eggs hatch and release
larva into the small intestine. Adult worms establish infection in the cecum and appendix. Gravid
worms migrate through the rectum and lay eggs on the perianal skin, typically at nighttime. The
duration from ingestion of infected eggs to migration of the adult female to the perianal region is
to 2 months.
Item C171: Enterobius vermicularis
Intense anal pruritus is the most common presenting symptom in children with pinworms;
however, one-third of affected individuals are asymptomatic. Urethritis and vaginitis can occur
in girls because of migration of adult gravid females to the urogenital organs. The diagnosis of E
vermicularis infection is confirmed with a cellophane tape preparation. The adhesive side of the
tape is pressed onto the perianal region in the morning when the child wakes up. The tape is then
examined under a microscope; the eggs are 50 x 25 µm in size and appear flat On 1 side, giving a
bean-shaped appearance. A single test can detect 50% of infestations, 3 tests can detect 90%, and
5 tests detect 100%.

PREP Pearls
• Pinworm infestation is a common cause of anal pruritus in children.
• Reinfection and autoinfection with pinworms is common, particularly when there is a
high parasite burden, in childcare settings, and among family members
• Management of pinworm reinfection includes repeat treatment with the same agent and
treatment of all household members.

• Recognize the clinical features associated with Enterobius vermicularis infestation
• Understand the epidemiology of Enterobius vermicularis
Suggested Readings
• Pinworm infections (Enterobius vermicularis). Red Book: 2021-2024 Report of the
Committee on infectious Diseases. 32nd ed; 2021:589-590.
• Intestinal nematodes. Principles and Practice of Pediatric Infectious Diseases 5th ed.
2018; 1373-1381
• Worm infections in children, Pediatr Rev. doi:10.1542/pir.36-8-341
• The diagnosis and treatment of pinworm infection. Dtsch Arztebl Int.
doi:10.3238/arztebl.2019.0213
• Pinworm infestations. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed; 2017:2509-1194.

Question 172
A 12-year-old boy is seen in the office for bilateral knee pain. He reports that the pain began
several weeks after starting basketball practice, and he has had no specific injuries. The pain is
located just below his patella. It initially occurred primarily after activity, but now occurs
throughout the day. Ibuprofen provides temporary relief. He has not experienced any swelling or
redness. The boy is otherwise healthy with normal growth and development.
On physical examination, he has focal tenderness over the inferior pole of both patellae and
patellar tendon, but none over the tibial tuberosities. Mild pain is elicited with lateral patellar
movement, without guarding or apprehension. He has pain with resisted knee extension. He has
full lower extremity strength and range of motion. No knee ligamentous laxity is noted. His gait
is unremarkable. The remainder of the boy's physical examination findings are normal.

A. distal patellar apophysitis
B. Osgood-Schlatter disease
C. patellar instability
D. patellofemoral syndrome

Correct Answer: A
The boy in the vignette has distal patellar apophysitis, also known as Sinding-Larsen-Johansson
syndrome (SLJS), a traction apophysitis of the patellar tendon on the growth plate at the inferior
pore of the patella. Atraumatic pain over the inferior pole of the patella in a skeletally immature
child or adolescent is consistent with SLJS. Sinding-Larsen-Johansson syndrome is an overuse
injury caused by repeated high-intensity activation of the extensor mechanism of the knee, such
as running or jumping.
The pain associated with SLJS is typically gradual in onset, located over the aspect of the
inferior patella, and often occurs after an increase in running and/or jumping activity. Physical
examination findings may include pain, tenderness, and focal swelling over the inferior patellar
apophysis (Item C172A). A true knee effusion indicates intra-articular pathology and warrants
additional evaluation. The pain may be reproduced with active or resisted knee extension. Gait
should be evaluated for a limp or malalignment that may predispose a child to SLJS.
Item C172A: Sinding-Larsen-Johansson syndrome
Other causes of anterior knee pain include:

• Osgood-Schlatter disease
o More common than SLJS
o Presents as focal pain over the tibial tuberosity
o Osgood-Schlatter and SLJS represent similar processes at opposite ends of the
patellar tendon. Forces generated by the tendon create a stress injury across the
cartilaginous growth plates.
• Patellofemoral syndrome ("Joggers knees”):
o Presents with gradual onset of diffuse anterior pain after a period increased
activity
• Patellar tendinitis ("Jumper's knee"):
o Presents with gradual onset of pain along the patellar tendon after a period of
increased high-intensity running and/or jumping
• Patellar instability:
o Often presents after an episode of patellar subluxation or dislocation

• Lateral patellar instability is most common and often results in significant medial
peripatellar pain and joint effusion.
• Recurrent atraumatic episodes of patellar instability may occur when there is generalized
ligamentous laxity.
Sinding-Larsen-Johansson syndrome is a clinical diagnosis. Knee radiography (including

anteroposterior, lateral, patellar, and tunnel views) should be considered in those with a history
of local trauma, severe swelling, or lack of improvement after several weeks of treatment. In
cases of acute trauma with severe pain and swelling over the inferior patellar pole, radiographs
should be obtained to assess for a patellar sleeve fracture (Item C172B). This fracture results in
superior displacement of the patella (patella alta) and displacement of the inferior patellar physis.
Patellar sleeve fractures warrant surgical consultation.
Item C172B Patellar sleeve fractures
When evaluating anterior knee pain, it can be helpful to think of the knee as a “hinge” and the
tendons that attach to the knee as “ropes." These structures work best when the “hinge* is well-
aligned, and the “ropes” are able to pull in a straight line. Malalignments that create angular or
rotational forces at the knee create added strain across the joint and tendon, predisposing children
to anterior knee pain. Angular malalignments include genu valgum ("knock knees”) or varum
("bow legs”). Rotary forces are generated by increases in femoral anteversion ("squinting
patellae;" (Item C172C) and overpronation of the foot. Although nonsurgical treatment cannot
change static bone alignment, physical therapy can change movement patterns to help decrease
the mechanical strain generated by activity.
Item C172C: squinting patellae

Treatment for anterior such as that caused by SLJS and Osgood-Schlatter disease includes:
• Minimizing discomfort with local application of ice for 10 to 15 minutes per session 3 to
4 times per day, especially after activity, and over-the-counter analgesics.
• Relative rest and avoidance of pain-generating activities (e.g., running, jumping,
hiking/prolonged walking, especially on hills). Activities that do not create pain during or
after participation should be encouraged or continued. Although bicycling is generally
well-tolerated, cycling up hills or against heavy resistance can provoke symptoms.
• Use of a counterforce brace (Item C172D) in those with SLJS, Osgood-Schlatter, or
patellar tendinitis, if there is symptom relief.
• Use of crutches and/or a knee immobilizer for several days for those with a significant
limp or pain with daily activity. Children requiring these devices should be monitored
closely and undergo imaging studies if they do not show rapid improvement.
• Physical therapy or a therapeutic exercise regimen. Compliance with a good home
exercise program is important for rapid recovery and to minimize recurrence risk.
Children and adolescents can progress to full activity when:

• The knee demonstrates return to full and pain-free range of motion and strength.
• Gait mechanics with walking and running are normalized.
• Sport-specific activity has been reintroduced.
Although SLJS may create recurrent episodes of pain during high-intensity activity, families can
be reassured that the vast majority completely resolve after physeal plate closure.
PREP Pearls
• Distal patellar apophysitis (Sinding-Larsen-Johansson syndrome) is an overuse
apophysitis that causes focal pain over the inferior pore of the patella
• Distal patellar apophysitis is a clinical diagnosis: radiography is to rule out other
pathology.

• Distal patellar apophysitis typically improves with rest and conservative measures and
permanently resolves with physeal closure.

• Identify the etiology of Osgood-Schlatter disease
• Recognize the clinical findings associated with Osgood-Schlatter disease, and manage
appropriately
Suggested Reading
• Overuse injuries of the knee. Care of the Young Athlete. 2nd ed; 2010:409-430.
• sports injuries. Pediatr Rev, doi:10.1542/pir.2018-0221
• Knee conditions. Pediatr Rev. doi: 10.1542/pir.35-9-359
• Sports musculoskeletal injuries. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017; 2675-5686

Question 173
A 9-year-old boy is seen in the office for a health supervision visit. He and his mother have no
concerns. He is doing well in school. He is planning to play in a fall soccer league, and his
mother requests completion of a sports participation clearance form. His vital signs are normal
for age. He appears well. Auscultation reveals clear breath sounds throughout, a normal S1,
physiologically split S2, and a 2/6 low-pitch, vibratory systolic murmur located at the left lower
sternal border that does not radiate and is louder when supine than when sitting up (Item Q173).
The remainder of his physical examination findings are unremarkable.
Of the following, the BEST next step in this child's management is

A. Echocardiography
B. electrocardiography
C. reassurance
D. referral to pediatric cardiology

Correct Answer: C
The boy in the vignette has a murmur that is described as having a(n):
• Intensity of 2 out of 6
• Timing during systole
• Low pitch
• Vibratory quality
• Location at the left sternal border without radiation
• Volume that is louder when supine
These characteristics are typical of an innocent murmur and do not warrant further evaluation.
Therefore, reassurance only is recommended for this child.
Innocent murmurs are common during childhood and are one of the most common reasons for
referral to a pediatric cardiologist. A thorough history and an understanding of the typical
auscultatory findings are needed to distinguish innocent murmurs from those that are pathologic.
History taken should include maternal, neonatal, and childhood. It is important to inquire about
the maternal age, infectious history (e.g., TORCH; Toxoplasmosis, Other agents, Rubella,
Cytomegalovirus, and Herpes simplex), and the presence of diabetes mellitus during pregnancy.
Questions that address evidence of child having increased metabolic demand or pulmonary
congestion can be revealing. For infants, issues such as feeding difficulties, sweating with
feedings, cyanosis, cyanosis with feedings, failure to thrive, tachypnea, or increased work of
breathing are highly relevant. In older children, questions should address any change in activity
or energy level, inability to keep up with peers, chest pain or syncope on exertion, and
respiratory complaints.
On physical examination, it is important to note the child’s general appearance, dysmorphic

features, or increased respiratory effort. Vital signs should include heart rate (looking for
tachycardia), blood pressure, upper to lower limb blood pressure gradients, pulse oximetry, and
in neonates, pre- and post-ductal saturation. Palpation of pulses and the abdomen for
hepatomegaly can be helpful in discovering cardiac pathology. Palpation over the heart is
important, looking for a hyperdynamic precordium or a thrill. Finally, the heart should be
auscultated in a systematic manner, listening to the 4 general areas: aortic, pulmonary, tricuspid,
mitral valve as well as areas to which murmurs often radiate such as the neck, axillae and back.
In each area, the quality of S1, and S2 should be noted as well as any additional cardiac sounds
such as clicks, rubs, or gallops. S2 should be studied for splitting caused by delayed closure of
the pulmonary valve relative to the aortic during inspiration. A fixed split S2 is indicative of an
atrial level shunt.
Any murmurs noted should be described based on the following characteristics
Timing: Murmurs occur in systole, diastole, or are continuous. Most innocent murmurs occur in
systole, unless the murmur is pansystolic/holosystolic, as seen with a ventricular septal defect. A
continuous murmur can be associated with a venous hum (benign), a patent ductus arteriosus, or
other pathologic findings. A diastolic murmur is pathologic until proven otherwise and warrants
further evaluation.
Intensity: Intensity refers to the loudness of the murmur and is determined by the relative
loudness of the murmur in regards to S1 and S2. Grade 1 is softer than S1/S2, grade 2 is the
same intensity, grade 3 is-louder, grade 4 implies the presence of a thrill, grade 5 is loud enough
to hear with a stethoscope barely on the chest, and grade 6 is loud enough to hear with the
stethoscope barely off the chest.
Location: The locations of interest are over the aortic, pulmonic, tricuspid, and mitral valves.
Quality: Murmur quality can be described as harsh, blowing, musical, squeaky, or vibratory.
Innocent murmurs generally have a vibratory or musical quality.
Pitch: Pitch can be described as low, medium, or high. Innocent murmurs tend to be low pitched.
High-pitched murmurs are often pathologic.
Radiation: It is important-to note radiation of a murmur to the neckt, axilla, or back.
Response to maneuvers: Maneuvers that impact preload or afterload, and thereby change the
flow in and around the heart, can change the intensity of innocent and some pathologic murmurs
that do not change with maneuvers are more likely to be pathologic.
Item C173A is an example of the pansystolic murmur of a small ventricular septal defect.
Item C173B is an example of the decrescendo diastolic murmur of aortic regurgitation, heard
immediately after S2 and persisting almost throughout diastole.
If a pathologic murmur is suspected, it is important to refer the patient to a pediatric cardiologist.
PREP Pearls
• Innocent murmurs are very common in children and are one of the most common reasons
for referral to a pediatric cardiologist.
• A thorough history addressing any symptoms that occur when increased demand is
placed on the heart (e.g., feeding for a neonate, gym class for a school-aged child) is
important in the evaluation of a murmur.
• Any murmur should be carefully auscultated and the liming, location, intensity, quality,
pitch, radiation, and response to maneuvers should be noted.
MOCA-Peds Objective
• Evaluate and manage an infant or child with a new heart murmur.

• Plan the appropriate evaluation of an innocent murmur, and manage appropriately

Suggested Readings
• Innocent murmurs. Circulation. doi:10.1161/01.CIR.0000153388.41229.CB
• Evaluation and management of heart murmurs in children. Am Fam Physician.
http://unmfm.pbworks.com/f/Heart%20Murmurs%20in%20Children.pdf
• Heart murmurs. Pediatr Rev. doi:10.1542/pir.28-4-e19
• Heart murmurs. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed;2017:1412-1417.

Question 174
A previously healthy, 16-year-old adolescent girl is seen in the school health clinic for a 2-week
history of mild persistent lower abdominal pain. Today, while at school, the pain worsened and is
now accompanied by nausea. She bad an episode of vomiting 2 hours ago, and reports that she
wants to go home. She has no respiratory symptoms, diarrhea, dysuria, urinary frequency,
hematuria, or vaginal discharge. She has no sick contacts. She has taken no medication for the
pain. Her menstrual periods typically are very regular, with 4 to 5 days of flow occurring once
per month. During the past month, she has had intermittent spotting and her last period, 1 week
ago, lasted 7 days. She has had 3 sequential male partners and no female partners. Her sexual
practices include vaginal and receptive oral sex. She and her current partner of 4 months have
used condoms inconsistently.
Six months ago, the girl evaluated for vaginal discharge and treated for bacterial vaginosis; urine
nucleic amplification tests for chlamydia and gonorrhea were negative. At that visit, she declined
alternative contraception options and HIV testing.
On physical examination, she appears to be hydrated and in mild distress. Her vital signs are
normal. Palpation of the abdomen elicits diffuse lower abdominal pain with mild guarding but no
rebound tenderness. She has no flank pain. The remainder of her physical examination findings
are unremarkable. A urine pregnancy test result is negative.
Of the following, the BEST next step in this adolescent’s evaluation is

A. Admission to the hospital
B. supportive treatment
C. transvaginal ultrasonography
D. pelvic examination

Correct Answer: D
The girl in the vignette has a history and physical examination findings that suggestive of pelvic
inflammatory disease (PID). Lower abdominal pain in sexually active adolescent girl without
another apparent cause is an indication pelvic examination that includes a bimanual and
speculum examination. A bimanual examination provides the opportunity to elicit cervical
motion and uterine and/or adnexal pain. Such pain would meet the minimum 2015 Centers for
Disease Control and Prevention (CDC) criteria for the diagnosis of PIP and would indicate
initiation of empiric treatment.
Among sexually active girls the minimum criteria for diagnosis of PID requires:
• Pelvic or lower abdominal pain without another apparent cause OR
• Pain present on pelvic examination
o Cervical motion or
o Uterine tenderness or
o Adnexal tenderness
The specificity for a diagnosis of PID is increased with evidence of cervicitis. A speculum
examination allows for visualization of the cervix and evaluation for cervicitis. The presence ef
mucopurulent discharge from the cervical os, yellow green mucus on a swab of the os, cervical
friability, or an increased number of white blood cells on a wet mount preparation from a
cervical or vaginal swab indicates cervicitis.
Cervical or vaginal swab samples should be tested for chlamydia and gonorrhea using a nucleic
acid amplification test. Although positive results support a diagnosis of PID, negative results do
not exclude the diagnosis. The diagnosis of PID requires a high index of suspicion and timely
evaluation. Delay in diagnosis and associated with an increased risk for complications and
sequelae.
For the mildly ill adolescent girl in the vignette, admission to the hospital without information
from a pelvic examination is premature. Supportive treatment, with evaluation in 48 hours, may
delay diagnosis and treatment of PID, with the potential for negative consequences, Although
transvaginal ultrasonography can confirm diagnosis of acute PID, it is not routinely
recommended tor suspected PID and could delay diagnosis and treatment. Ultrasonography may
be considered when there is notable lateralization of adnexal pain, the presence of an adnexal
mass, concern for an ectopic pregnancy, or for those requiring hospitalization.
The adolescent in the vignette has symptoms associated with acute (symptoms for ≤30 days)
PID. These symptoms include a history of lower abdominal pain that is persistent, bilateral, and
diffuse; intermenstrual spotting: prolonged menstrual bleeding; and nausea and vomiting. Acute
PID may also be associated with a history of vaginal discharge, dyspareunia, dysmenorrhea,
postcoital spotting/bleeding, heavy menstrual bleeding, dysuria; and urinary frequency. The
severity of symptoms ranges from subtle to severe. Risk factors for PID include age (higher risk
in adolescents and young adults), cervical ectropion, low condom usage, previous sexually
transmitted infection, multiple sexual partners, patient or partner with chlamydia or gonorrhea,
vaginal douching, and 3 weeks or less after the placement of an intrauterine device.

Physical examination findings associated with PID often are limited to diffuse lower
abdominal/pelvic tenderness on direct palpation, with or without rebound tenderness or guarding,
Thus, a pelvic examination is very important for the diagnosis of PID. The presence of fever
greater than 38.3 °C increases the specificity for PID but is not a common associated finding.
Findings of fever, tachycardia, and pain that worsens with ambulation may indicate more severe
PID. Right upper quadrant pain, a relatively uncommon findings suggestive of perihepatitis, a
complication of PID (Fitz-Hugh-Curtis syndrome). Mildly elevated liver function test levels may
accompany perihepatitis.
Indications for hospitalization for acute PID include:

• Need to exclude a surgical emergency
• Evaluation/treatment for tubo-ovarian abscess
• Severe illness with systemic symptoms
• HIV infection
• Pregnancy
• Inability to tolerate oral medications
• Lack of clinical improvement after 48 to 72 hours of outpatient treatment
• Lack of a support structure to facilitate treatment for adolescents
Treatment guidelines for PID are summarized in Item C174. Hospitalization allows for the
institution of parenteral therapy and further evaluation for complications (e.g., tubo-ovarian
abcess) or alternative diagnoses. Laparoscopy and endometrial biopsy can establish a definitive
diagnosis; however, these are rarely indicated. Individuals who test positive for chlamydia or
gonorrhea should be retested 3 months after PID treatment. Adolescent girls with PID should be
tested for HIV and testing for syphilis should be considered.
Short-term complications of PID are a consequence of ascending infection and include

salpingitis, tubo-ovarian abscess, perihepatitis, and peritonitis. Long-term sequelae, a
consequence of scarring that can follow even one mild or subclinical clinical presentation of PID
include ectopic pregnancy, infertility, and chronic pelvic pain.

Item C174: Treatment guidelines for PID

PREP Pearls
• In a sexually active adolescent girl with persistent lower abdominal/pelvic pain, a
diagnosis of pelvic inflammatory disease should be considered and is an indication for a
pelvic examination.
• Hospitalization is indicated for lack of clinical improvement 48 to 72 hours after
initiation of outpatient treatment for pelvic inflammatory disease.
• Long-term sequelae of pelvic inflammatory disease include ectopic pregnancy infertility,
and chronic pelvic pain.

• Plan the appropriate management of pelvic inflammatory disease
• Understand the complications associated with pelvic inflammatory disease
• Recognize the clinical and laboratory findings associated with pelvic inflammatory
disease, and manage appropriately
Suggested Readings
• Pelvic Inflammatory disease. N Engl J Med. doi:10.1056/NEJMra.1411426
Care. 2nd ed; 2017:2628-2652
• Sexually transmitted infections Part 2: Discharge syndromes and pelvic inflammatory
disease. Pediatr Rev, doi:10.1542/pir.2019-0078
• Pelvic inflammatory disease. Neinstein’s Adolescent and Young Adult Medicine. 6th ed.
2016;486-489.
• Centers for Disease Control and Prevention. Sexually transmitted diseases treatment
guideline. 2015; 1-137

Question 175
A previously healthy, 3-week-old girl, born at term is admitted to the pediatric ward for fever of
38.9 °C. The neonate has been sleepy and not feeding well since the day prior to admission. Her
temperature 37.7 º C (after 1 dose of acetaminophen), heart rate is 180 beats/min, respiratory rate
is 40 breaths/min, and oxygen saturation by pulse oximetry is 97% in room air. Her anterior
fontanelle is bulging, capillary refill time is 4 seconds, and she is sleepy but arousable. Cardiac
and respiratory examination findings are normal.
A complete evaluation for sepsis is initiated, and appropriate antibiotic therapy is administered.
Results of a complete blood count and basic metabolic panel are normal. Bacterial culture from
the cerebrospinal fluid is positive the following day for Streptococcus pneumoniae. After 72
hours, blood and urine culture results are negative.
Of the following, this child should MOST likely be evaluated for

A. congenital heart disease
B. hearing loss
C. immunodeficiency
D. vesicoureteral reflux

Correct Answer: B
The neonate in the vignette has bacterial meningitis from Streptococcus pneumoniae. Up to 35%
of children with meningitis from S pneumoniae will develop hearing loss. All children with
bacterial meningitis should undergo evaluation for hearing loss at the time of diagnosis with
repeat evaluation at 4 to 8 weeks, 6 months, and 12 months after discharge.
This neonate does not have signs or symptoms suggestive of congenital heart disease. Evaluation
for an immunodeficiency is not indicated for a neonate with pneumoccal meningitis. An older
infant with meningitis resulting from bacteria against which they have been vaccinated may
require further evaluation. The neonate's evaluation did not demonstrate a urinary tract infection,
and therefore she does not require evaluation for vesicoureteral reflux.
Congenital hearing loss that is moderate, severe, or profound occurs in 3 out of every 1,000
births. By the age of 18 years, 17 out of 1,000 children will have some level of permanent
hearing loss. Hearing is categorized based on the decibel level a person is able to hear. Item
C175 shows definitions of normal hearing and hearing loss as well as typical noises at the same
level. Children with mild to severe hearing loss can be fitted for hearing-amplification devices
beginning as young as 3 months of age.
Item C175: normal hearing and hearing loss
For the best functional outcome, hearing loss should be identified in all infants by age 3 months,
according to the Universal Hearing Screening Program guidelines. Children with hearing loss
often coo and babble like other infants initially, but then lose these skills after 6 to 9 months of
age. They do not continue to develop speech such as saying “mama” or “dada” at the typical age
of 12 to 15 months. Affected infants often do not look at their caregivers when spoken to and are
less likely to make eye contact. Gross motor function can also be delayed, such as sitting or
walking. Based on this and other evidence, the Universal Hearing screening Program
recommends that interventions for infants with hearing loss should begin by 6 months of age,
and the child should be established in a medical home.

Two types of screening tests for hearing are commonly used in the newborn nursery. Automated
auditory brainstem response testing measures the auditory nerve and brainstem response to
sounds by using electrodes. Otoacoustic emission (OAE) testing measures the sound emitted
from the cochlea in response to a projected sound. Results of OAE testing are reported as present
or absent. In addition to hearing loss, an absent OAE result can be caused by occlusion of the
external auditory canal or fluid in the middle ear. If the results of a hearing screening are
abnormal, an automated auditory brainstem response test should be completed within 3 months,
to assess the severity of hearing loss; this often requires sedation in infants.
For a developmentally appropriate child, by 6 to 9 months of age behavioral testing can be

performed, during which an audiologist monitors the child's behavior while a tone is played in
one or both ears. By 2 years of age, play audio testing can be conducted. Finally, routine testing
can be completed between 4 to 5 years of age.
Hearing loss can be caused by problems in the external auditory canal, middle ear, inner ear, or
auditory nerve. Hearing loss resulting from a condition of the external auditory canal or middle
ear is defined as “conductive hearing loss.” Hearing loss resulting from a condition involving or
proximal to the cochlea is known as sensorineural hearing loss (SNHL). At times, both auditory
regions contribute, and the resulting condition is defined as mixed hearing loss.
Obstruction of the outer ear with cerumen or a foreign body, or malformations of the external ear
such as stenosis or atresia (often seen with craniofacial abnormalities), can interfere with
hearing. The middle ear consists of the tympanic membrane, middle ear bones or ossicles
(malleus, incus, stapes), and the oval window. Examples of middle ear conditions that may result
in conductive hearing loss include effusions (such as in acute otitis media), because the middle
ear bones require air to transmit sound; a large perforation in the tympanic membrane; or
abnormally shaped or ossified ossicles. Hearing loss due to destruction, trauma, or abnormal
development of any or all of the 3 ossicles can be treated surgically with an ossicle prosthetic
device. Effusion is a common cause of conductive hearing loss, especially after episodes of acute
otitis media; 40% of children have an effusion persisting 1 month after treatment, 20% at 2
months, and 10% at 3 months. Most often, the hearing loss is transient; however, if an effusion
persists for 3 months, or if there are symptoms of hearing loss, formal hearing testing, and
treatment of the effusion with surgical placement of tympanostomy tubes should be considered.
Sensorineural hearing loss has numerous causes, including infections (e.g., bacterial meningitis),
medications, genetic conditions, trauma, and other medical conditions. Cytomegalovirus (CMV)
is the most common cause of acquired SNHL, with 50% of symptomatic infants and 10% to 15%
of asymptomatic infants experiencing hearing loss. Diagnosis of congenital CMV requires
testing prior to 3 weeks of age. Ototoxic medications include aminoglycosides, loop diuretics,
and cisplatin. Genetic causes of SNHL include Alport syndrome, neurofibromatosis type 2, and
Stickler syndrome. Trauma, especially of the temporal bone, can lead to SNHL. In addition, the
risk of SNHL is increased for neonates with elevated bilirubin levels requiring exchange
transfusion, those requiring ventilator support for more than 10 days, those with Apgar scores of
less than 6 at 10 minutes after birth, those with a birthweight of less than 1.500 g, or those with a
family history of congenital or early hearing loss. Children with profound SNHL may benefit

from a cochlear implant. It is important to note that children with bilateral SNHL have an
increased risk of QT prolongation and should undergo electrocardiography screening.
PREP Pearls
• All infants should have a hearing screen prior to 1 month of age with the goal of
identification of hearing loss by 3 months of age, and treatment beginning by age 6
months.
• Hearing loss is a common complication of bacterial meningitis. All affected children
should have an audiologic evaluation.
• Hearing loss can delay childhood development in several domains

• Understand the indications for and limitations of standard audiology tests (including
acoustic emissions, tympanometry, auditory brainstem response, and behavioral
audiometry) and be able to interpret their results
• Understand how hearing toss is categorized by audiometric testing
• Plan the age-appropriate initial and follow-up evaluation of hearing loss of various
etiologies
• Understand the importance of a screening examination for hearing
Suggested Readings
• American Academy of Pediatrics. Newborn hearing screening FAQs.
https://www.healthychildren.org
• Hearing. Performing Preventive Services: A Bright Futures Handbook; 2010:129-126.
• Pediatric hearing loss. Pediatr Rev. doi:10.1542/pir.35-11-456
• Hearing loss. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:1408-1411

Question 176
A 2-yeat-old boy is seen in the emergency department with right knee pain and swelling. He is
now having some difficulty with ambulation. There is no known of trauma, however, he was
playing at the park 2 days prior to the onset of symptoms.
His temperature is 37.8 º C. His right knee is slightly warm, swollen, ecchymotic, non-
erythematous, and has limited range of motion, His left knee appears normal. The circumference
of the right knee is 23 cm; the left is 22 cm. He limps with ambulation. There are bruises noted
on his right proximal tibia and buttocks. The remainder of the examination findings are
unremarkable.
Prothrombin time 11.7 s
Activated partial thromboplastin time >200 s
Factor VIII assay 1.1%
Factor IX assay 67.3%
Of the following, the BEST next step in this child's management is to

A. administer empiric antibiotics
B. administer factor concentrate
C. administer fresh frozen plasma
D. obtain knee magnetic resonance imaging

Correct Answer: B
The child in the vignette has severe factor VIII deficiency, hemophilia A, as confirmed with the
laboratory findings. His current signs and symptoms are most likely due to an injury to the right
knee, resulting in bleeding into the joint as evidenced by the pain, swelling, increase in knee
circumference, and inability to ambulate. This complication, known as hemarthrosis, should be
treated as soon as possible with factor concentrate administration. Repeated bleeding into this
may lead to the development of a target joint, a join at risk for deformity, limited range of
motion, and decreased strength. Prophylactic factor concentrate administration helps prevent
this.
Factor VIII deficiency, one of the most common congenital coagulation factor deficiencies, is
inherited in an X-linked recessive pattern. Therefore, all males are affected. Females, who are
carriers, may also have significant bleeding (e.g., menorrhagia), It is classified as mild (i.e.,
factor VIII level 6% to 30%); moderate (i.e., factor VIII level 1% to 5%); or severe (i.e., factor
VIII level <1%).
A bleeding workup is warranted when a child has either repeated and/or unexplained bleeding.
Screening for a bleeding disorder warrants a detailed history and physical examination. This will
help guide the initial laboratory evaluation, which should include a complete blood with a
peripheral blood smear review, prothrombin time (PT) with mixing studies, activated partial
thromboplastin time (aPTT) with mixing studies, prothrombin time, fibrinogen level and von
Willebrand antigen and activity levels. Factor levels may be obtained after determining which is
prolonged, the PT or aPTT. It should be noted that PT and aPTT may be normal in factor XIII
deficiency, though the child can experience severe bleeding. Administration of fresh frozen
plasma, which contains several clotting factors, is reserved for situations such as severe bleeding
when the factor deficiency is unknown. It is not recommended in a known factor deficiency
where an isolated factor concentrate product is available.
While imaging is sometimes helpful in diagnosing hemarthrosis, it is usually a clinical diagnosis

based on the history and physical examination findings. If imaging is required, it should be
obtained after factor concentrate is administered. Additionally, a child 2 years of age would
likely require sedation for magnetic resonance imaging, which would result in additional delay in
treatment. However, if available, point-of-care ultrasonography may be used to identify
hemarthrosis.
Though the child in the vignette has a slight elevation in temperature, white blood cell count, and
platelet count, a septic joint is a less likely cause of his current condition given his diagnosis of
hemophilia. Inflammation from the blood in his joint explains these laboratory findings and
slight increase in temperature. There is therefore no need to administer empiric antibiotics.

PREP Pearls
• Factor VIII deficiency is one of the most common congenital bleeding disorders.
• A child should be evaluated for a bleeding disorder when there is a significant history of
bleeding/bruising, especially unexplained.
• Factor XIII deficiency may be diagnosed when there is significant bleeding in the setting
of a normal prothrombin time and activated partial thromboplastin time.
MOCA-Peds Objective
• Recognize common X-linked genetic disorders.
• Understand the inherited patterns of various blood disorders.

• Plan the general therapeutic approach for a patient with a bleeding diatheses
• Plan the appropriate diagnostic evaluation for increased bruising
• Recognize clinical findings associated with congenital coagulation factor deficiency
• Plan an appropriate screening evaluation for a coagulation disorder
Suggested Readings
• Coagulation disorders. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed; 2017:1858-1868.
• Coagulation disorders. Pediatr Rev. doi:10.1542/pir.24-3-83
• Pediatric coagulation disorders. Pediatr Rev. doi: 10.1542/pir.2015-0062
• Hemophilia: Pediatr Rev, doi:10.1542/pir.34-7-289

Question 177
A 15-year-old adolescent boy was seen 1 week ago in the emergency department for 8 days of
headache, fever, and sinus pain. He was diagnosed with acute bacterial sinusitis, treated with
antipyretics and antibiotics, and discharged home with outpatient follow-up. Today he returns to
the emergency department after developing vomiting, lethargy, and confusion. On physical
examination, he is febrile and mildly tachycardic. He is sleeping and arouses briefly to
stimulation before falling back to sleep. The adolescent is only oriented to self; he is unable to
identify his location or the date. He does not consistently follow commands. His neurological
examination is notable for bilateral papilledema and diminished strength in his left arm. Brain
magnetic resonance imaging is ordered, and results are shown in Item Q177.
Item C177: Brain T1 axial magnetic resonance image with contrast. Courtesy of D. Morita
Of the following, the BEST next step in this adolescent's management is to

A. consult a neurosurgeon
B. initiate intravenous antibiotics
C. obtain blood cultures
D. perform a lumbar puncture

Correct Answer: C
The adolescent in the vignette has developed brain abscess, a rare complication of bacterial
sinusitis. Prior to initiating empiric antibiotic therapy, blood cultures should be obtained to
attempt to identify the causative pathogen. Empiric antibiotics indicated after blood cultures are
obtained. Lumbar puncture is not indicated, as it is often low yield and carries risk due to
potential mass effect with increased intracranial pressure. Consultation with neurosurgery for
aspiration and drainage is important for diagnosis and management, but blood cultures should be
obtained and empiric antibiotics initiated first.
A brain abscess is a focal area of infection within the brain parenchyma that progresses from an
area of inflammation, termed cerebritis, to a central core of necrotic tissue and pus encapsulated
in a thick rim. A brain infection can occur due to:
• Contiguous spread from a local site of infection (e.g., otitis media. Mastoiditis. sinusitis)
• Hematogenous spread from a distant site of infection (e.g., infective endocarditis)
• Penetrating head trauma or neurosurgical procedures
The overall incidence of brain abscess has declined, with fewer cases occurring secondary to
contiguous spread in the setting of local infections. However, immunocompromised children and
adolescents are at higher risk than the general population. Causative organisms are related to the
mechanism of infection. Local infections are most often due Streptococcus species, anaerobic
bacteria, or microaerophilic bacteria. Abscesses secondary to head trauma and/or neurosurgical
procedures are more commonly due to Staphylococcus aureus, coagulase-negative
staphylococci, or gram-negative bacilli. Up to 25% of infections are polymicrobial. Empiric
antibiotic therapy based on the most likely causative organism(s) should be initiated without
delay after obtaining blood cultures.
The classic triad of symptoms is fever, headache, and focal neurological deficits; however, only
20% of children and adolescents present with all three. Seizures can also occur. Unlike in
meningitis, which evolves rapidly, papilledema can be seen in cases of brain abscesses large
enough to cause increased intracranial pressure. When an abscess is suspected, head computed
tomography or brain magnetic resonance imaging with contrast should be obtained. On imaging,
an abscess appears as focal edema with a rim of contrast enhancement. Management should
involve a multidisciplinary team including critical care, neurosurgery, infectious disease, and
neurology specialists. Neurosurgical intervention is both diagnostic and therapeutic.
Complications of brain abscess may include hydrocephalus due to the compression of ventricular
system, ventriculitis due to rupture of the abscess into the ventricles, seizures, and status
epilepticus. There is a high risk of increased intracranial pressure with the potential for
herniation. The majority of those affected survive without neurologic sequelae.

PREP Pearls
• Brain abscess is an uncommon central nervous system infection that can be caused by
direct extension of an infection (e.g., sinusitis). hematogenous spread, or as a
complication of head trauma or a neurosurgical procedure
• Head computed tomography or brain magnetic resonance imaging with contrast should
be obtained if a brain abscess is suspected. Lumbar puncture is not indicated, as it is often
low yield and carries risk due to potential mass effect with increased intracranial
pressure.
• Empiric antibiotic therapy for a brain abscess should be initiated after obtaining blood
cultures, without delay for neurosurgical intervention.

• Plan the appropriate diagnostic evaluation of a brain abscess
• Plan the appropriate management of a brain abscess
• Understand the risk factors associated with a brain abscess
• Recognize the clinical findings associated with brain abscess
Suggested Readings
• Clinical characteristics and outcome of brain abscess: Systematic review and meta-
analysis. Neurology doi:10.1212/WNL.0000000000000172
• Brain abscess. N Engl J Med, doi:10.1056/NEJMra1301635
• Brain abscess. Pediatr Rev, doi: 10.1542/pir.2017-0147

Question 178
17-year-old adolescent boy is seen as a new patient to establish primary care, He a history of
recurrent otitis media and sinusitis, chronic cough, and clinically diagnosed pneumonia at least
once each year. He has had negative results when evaluated for immunodeficiency, and a normal
sweat chloride level; he has no demonstrable environmental allergies, He does not smoke
tobacco or marijuana and does not vape. As a term newborn he had respiratory distress requiring
oxygen supplementation for 1 Week but no sequelae. There is no family history of allergy or
immunodeficiency.
The adolescent is well grown and appears healthy. Tympanic membranes are scarred and dull;
nasal mucosa is edematous with polypoid material in the right antrum. There is no tenderness to
deep palpation of the maxillary sinuses, but mucopurulent postnasal drainage is visible.
Significant findings on chest examination include scattered inspiratory crackles in the midlung
fields bilaterally. but no expiratory wheezing. Normal heart sounds are auscultated on the left.
The liver edge is palpable at the right costal margin and abdominal examination findings are
normal. No digital clubbing is noted.
After an otolaryngology consultation, electron microscopy of respiratory epithelium

demonstrates absent outer dynein arms in more than 50% of cilia examined.
Of the following, the condition MOST likely to affect this adolescent's reproductive health is
A. bilateral absence of the vas deferens
B. deficient spermatogenesis
C. hypomotile sperm
D. predilection for testicular torsion

Correct Answer: C
The adolescent in the vignette has primary ciliary dyskinesia (PCD), an autosomal recessive
condition characterized by recurrent respiratory infections, male infertility, and situs inversus.
Flagellar dyskinesis causes hypomotile sperm, resulting in the male infertility associated with
PCD. The adolescent in the vignette does not have situs inversus, which is present in about 50%
of those with PCD.
Bilateral absence of the vas deferens is the cause of male infertility in cystic fibrosis, a diagnosis
that should be considered with the findings in the adolescent in the vignette. However, his
normal sweat chloride level makes cystic fibrosis a less likely diagnosis. Primary ciliary
dyskinesia is not associated with structural abnormalities of the vas deferens. A few male
patients with ciliary dyskinesia have low sperm counts. There is no propensity for testicular
torsion in PCD or in any of the other conditions in the differential diagnosis for PCD.
The inheritance of PCD is autosomal recessive, but a specific genotype is often difficult to
demonstrate because of the genetic heterogeneity of the condition. However,
genotype/phenotype relationships continue to emerge as more is discovered about the genetics of
RCD.
Biopsy of ciliated epithelium with electron microscopy of cilia ultrastructure has been the
primary diagnostic test for PCD. However, abnormal ciliary ultrastructure is not required for the
diagnosis; a high index of suspicion with normal ultrastructure should lead to additional
diagnostic testing. Structural abnormalities may include absence of either outer or inner dynein
arms, or defects in the central microtubules of cilia. Any of these structural defects may lead to
poor or inconsistent motility of individual cilia and poorly coordinated motion of the epithelial
cilia. It is this inadequate ciliary motion that underlies recurrent respiratory infections, because
both mucus and microbes are poorly cleared from respiratory structures. Because of the recurrent
respiratory infections, cystic fibrosis and immunodeficiency are also in the differential diagnosis
for PCD.
Poor airway clearance may contribute to respiratory distress in newborns affected with PCD. In a
retrospective case-control study, 46 term infants later found to PCD were matched with 46
infants having respiratory distress and requiring chest radiography. The presence of situs
inversus, pulmonary lobar collapse, or oxygen need for more than 2 days had 87% sensitivity
and 95% specificity for the later diagnosis of PCD. Unexplained respiratory distress in a term
neonate should concern for PCD.
Additional testing that may support a diagnosis of PCD includes measurement of the expired
nasal nitric oxide (NO) level. A measure of eosinophil activity, nasal NO is elevated in poorly
controlled asthma. Nasal NO is very low in PCD, but also in acute infections and in patients with
cystic fibrosis; thus, nasal NO measurement is supportive but not diagnostic. Measurement of
functional ciliary beat waveform with high-speed video microscopy can provide confirmation of
PCD but is not an appropriate single diagnostic test, is difficult to perform and requires a great
deal of experience to avoid false-positive and false-negative results. Immunofluorescence using
antibodies to dynein arm proteins can detect abnormal structural proteins in the cilia.
The best diagnostic approach for PCD uses a panel of tests including:
• Genetic analysis
• Nasal NO measurement
• Structural examination of respiratory cilia via electron microscopy
• Immunofluorescence for abnormal dynein arm proteins
• Functional cilia waveform analysis where available
Because much of this testing is very specialized and the diagnosis may be difficult to establish,
evaluations should be performed in centers with expertise in PCD.
PREP Pearls
• Primary ciliary dyskinesia is characterized by recurrent respiratory infections, male
infertility, and situs inversus.
• Mate infertility in primary ciliary dyskinesia is the result of poor sperm motility caused
by abnormal flagellar motion.
• Otherwise, unexplained neonatal respiratory distress should raise suspicion for primary
ciliary dyskinesia.

• Recognize disorders associated with primary ciliary dyskinesia
• Plan the appropriate diagnostic evaluation of primary ciliary dyskinesia
Suggested Readings
• Primary ciliary dyskinesia. Pediatr Rev, doi:10.1542/pir.2016-0108
• Primary ciliary dyskinesia in genomics age. Lancet Respir Med. doi:10.1016/S2213-
2600(19)30374-1
• Primary ciliary dyskinesia and neonatal respiratory distress. Pediatrics,
doi:10.1542/peds.2014-0808
• Primary ciliary dyskinesia. Am J Resp Crit Care Med, doi:10.1164/rccm.1982P3
• Genetic Disorders of Mucociliary Clearance Consortium. Diagnosis, monitoring and
treatment of primary dyskinesia: PCD Foundation consensus recommendations based on
state of the art review. Pediatr pulmonal, doi:10.1002/ppul.23304

Question 179
A 4-year-old boy recently adopted from an orphanage in Myanmar is being seen a routine health
supervision visit. The parents do not report any complaints. He arrived in the United States
approximately 2 weeks earlier. One week earlier, he was evaluated at the local health department
with complaints of low-grade fever of 3 days' duration with associated malaise, loss of appetite.
and emesis. Hepatitis serologic testing performed at the local health department is positive for
hepatitis A virus (HAV)-specific total antibody (IgG plus IgM) with a positive serum IgM
antibody to HAV. He received immunization against HAV. The boy lives with his adoptive
parents and 12-month-old sister, all of whom are asymptomatic. The sister has not received her
vaccination against HAV.
On physical examination, the boy is afebrile and well appearing with normal vital signs. No
scleral icterus is noted. His abdomen is soft and nontender, with no hepatosplenomegaly. The
rest of the physical examination findings are normal. Laboratory evaluation is notable for
elevation of liver enzymes but normal level of serum bilirubin. Supportive care is recommended
for the child with viral hepatitis.
Of the following, the BEST next management step for the postexposure prophylaxis of the 12-
month-old sister is administration of
A. HAV immune globulin
B. HAV immune globulin and vaccine
C. hepatitis A vaccine
D. interferon α

Correct Answer: C
The adopted child described in the vignette is recovering from acute viral hepatitis caused by
hepatitis A virus (HAV). The best next management step for the 12-month-old exposed sibling is
administration of the hepatitis A vaccine as postexposure immunoprophylaxis (Item C179A).
The hepatitis A vaccine is licensed for use in people aged 12 months through 40 years and is
recommended for postexposure immunoprophylaxis of HAV among unimmunized individuals
within this age range. Administration of immune globulin (Ig) is recommended for postexposure
immunoprophylaxis of HAV for exposed infants younger than 12 months. No antiviral therapy is
available for prophylaxis or treatment of HAV.
Item C179A: Recommendations for Postexposure Prophylaxis of Hepatitis A Virus (HAV)
Hepatitis A virus, which leads to a vaccine-preventable communicable disease, is a major cause

of acute viral hepatitis worldwide and results in significant morbidity. The disease is endemic in
many regions of the world including sub-Saharan Africa, Asia, Eastern Europe, Western Pacific,
and Central and South America. Hepatitis A virus is transmitted from person to person via the
fecal-oral route after close contact with an infected individual. Transmission is more likely in
settings with poor sanitation, lack of availability of clean water and food, and overcrowding,
especially in underserved communities in low and middle-income countries.
Risk factors for HAV infection in the United States include close contact with infected
individuals, an international adoptee, a child who attends daycare, and someone who has traveled
internationally. Individuals with injection drug use and men who have sex with men are also at
high risk for acquisition of HAV infection. Young children are often asymptomatic when they
become infected with HAV, but often shed the virus in stools for prolonged periods and play a
crucial role in the transmission to others. Community outbreaks of HAV infection have been
reported especially involving children attending child care centers. Individuals are most
infectious during the first 2 weeks, before onset of jaundice. The incubation period of HAV
infection ranges from 15 to 50 days (average 28 days).
The clinical presentation of HAV infection in younger children is different compared with older
children and adults. The majority (70%) of children younger than 6 years with HAV infection
remain asymptomatic (without jaundice): In contrast, asymptomatic disease occurs in only 30%
of adolescents and adults. Hepatitis A virus illness is characterized by fever, malaise, loss of
appetite, jaundice, vomiting, and abdominal pain, similar to hepatitis caused by other viruses.
The disease is typically self-limited, but may result in serious complications including fulminant
liver failure and death. Hepatitis A virus does not result in chronic infection.
Available serologic tests for diagnosis of HAV infection include measurement of HAV-specific
total antibody (i.e., IgG plus IgM), IgG-only anti-HAV, and IgM only anti-HAV. Serologic
testing for HAV is part of the acute hepatitis serology panels at most hospitals and reference
laboratories. A single positive total antibody or positive IgG-only anti-HAV is not indicative of
infection. The diagnosis of current or recent HAV infection is made based on serologic evidence
of the presence of serum IgM anti-HAV. In contrast, serologic evidence of positive total anti-
HAV (i.e., IgM plus IgG) with a negative serum IgM anti-HAV assay indicates immunity from
past infection or receipt of the hepatitis A vaccination. Treatment of HAV infection is
supportive. No antiviral agents are currently available to treat HAV infection.
Prevention measures against HAV include routine immunization of all children 12 to 23 months
of age with the hepatitis A vaccine. Hepatitis A vaccines, available in the United States since
1995, are highly effective in disease prevention. Routine universal childhood hepatitis A
vaccination has resulted in a significant decrease in the rate of pediatric infection with HAV.
Hepatitis A vaccination is also recommended for individuals at increased risk of HAV infection
or its complications including people traveling internationally to countries with high or
intermediate endemicity (Item C179B), close contacts of newly arriving international adoptees
men who have sex with men, users of injection and non-injection drugs, patients with clotting
factor disorders, people with chronic liver disease, and individuals at risk of occupational
exposure (e.g., handlers of nonhuman primates and laboratory researchers working with HAV).

Item C179B: Recommendations for Preexposure Prophylaxis of Hepatitis A Virus (HAV) for Travelers to
Countries With High or Intermediate Endemic Rates of Hepatitis A
PREP Pearls
• Hepatitis A vaccine is recommended for postexposure immunoprophylaxis of hepatitis A
virus in unimmunized individuals aged 12 months to 40 years; monovalent hepatitis A
vaccine recommended for postexposure immunoprophylaxis
• Administration of immune globulin is recommended for postexposure
immunoprophylaxis of hepatitis A virus for exposed infants younger than 12 months.
• A key pediatric hepatitis A virus infection prevention strategy is routine immunization of
all children 12 to 23 months of age with the hepatitis A vaccine

• Recognize the clinical features associated with hepatitis A virus infection in children of
various ages plan the appropriate diagnostic evaluation of hepatitis A virus infection
• Know the indications and schedule for hepatitis A vaccine
• Understand the epidemiology of the hepatitis A virus

Suggested Readings
• Prevention of hepatitis A through active or passive immunization: recommendations of
the Advisory Committee on Immunization Practices (ACIP). MMWR
• Hepatitis A. Red Book: 2021-2024 Report of the Committee on infectious Diseases. 32nd
ed; 2021;373-380
• Hepatitis A B, and C. Pediatr Rev, doi:10.1542/pir.2015-0075
• Update: Recommendations of the Advisory Committee on Immunization Practices for
use of hepatitis A vaccine for postexposure prophylaxis and for preexposure prophylaxis
for international. travel. MMWR Morb Mortl Wkly Rep, doi:10.15585/mmwr.mm6743a5

Question 180
A 16-month-old boy is brought to the emergency department for trouble breathing. His mother
states that he was well until approximately 48 hours ago when congestion and wet cough began.
On physical examination, the boy has a heart rate of 160 beats/min, respiratory rate of 60
breaths/min, oxygen saturation room of 88% on room air, and blood pressure of 98/55 mm Hg.
He is in moderate respiratory distress with Intercostal and subcostal retractions. Lung
examination reveals coarse rhonchi and scant wheezing with good aeration throughout. Although
somnolent, the boy interacts with his mother appropriately and cries when a blood specimen is
obtained via arterial puncture. The remainder of his physical examination findings are
unremarkable. Arterial blood gas reveals a pH of 7.30 PCO2 of 55 mm Hg, and PO2 of 60 mm
Hg.
Of the following, the BEST next management step for this boy is to initiate
A. bilevel positive airway pressure ventilation
B. Intubation and mechanical ventilation
C. low-flow nasal cannula oxygen
D. non-rebreather face mask oxygen

Correct Answer: A
The boy in the vignette has acute respiratory failure and is in immediate need of supplemental
respiratory support. Given that the boy has evidence of hypoxia (low PO2 and oxygen saturation)
and hypercapnia (elevated PCO2 and respiratory acidosis), management must address both acute
hypoxic and hypercapnic respiratory failure simultaneously. Non-invasive positive-pressure
ventilation (NIPPV) for acute respiratory failure in children is associated with rapid clinical
improvement and a decreased need for intubation, thus, a trial of NIPPV, with bilevel positive
pressure (BiPAP) would be the next best step in management. A nasal or full-face mask is used
to administer BiPAP and allows for independent adjustment of inspiratory positive airway
pressure (iPAP) and expiratory positive airway pressure (ePAP). Inspiratory positive airway
pressure promotes lung inflation during inspiration, leading to alveolar recruitment, and ePAP
prevents end-expiratory alveolar collapse with the application of continuous end-expiratory
pressure.
Although oxygenation may improve with low-flow nasal cannula or non-rebreather face mask
oxygen, neither will provide adequate respiratory support to address the hypercapnic component
of acute respiratory failure. Intubation and mechanical ventilation may be required for children
with acute respiratory failure and for those who fall a trial of NIPPV. Attempting NIPPV is
warranted for children who have stable hemodynamics and are without severe respiratory or
metabolic acidosis, altered mental status, or signs of shock.
Acute respiratory failure occurs when the respiratory system fails to provide adequate
oxygenation, is unable to eliminate appropriate levels of carbon dioxide, or both. Oxygen
delivery is a function of oxygen content and cardiac output. It requires adequate oxygenation of
the arterial blood through oxygen diffusion across the alveolar membrane into the pulmonary
venous system. Ventilation is the physiologic function of eliminating carbon dioxide from the
systemic venous circulation as it returns to the pulmonary vascular bed through the pulmonary
arteries. Respiratory failure can be a function of impaired oxygenation, ventilation, or both.
The Pathophysiology of acute respiratory failure in children involves an imbalance of the normal
ratio of alveolar ventilation (V) and pulmonary blood flow (Q), or impairment of oxygen
diffusion across the alveolar-capillary membrane. An imbalance in ventilation and perfusion
(V/Q mismatch) is the most common cause of pediatric respiratory failure. During normal gas
exchange, lung perfusion and ventilation are nearly matched. However, some areas of the lungs
do not have equal amounts of ventilation and perfusion as a result of normal physiologic changes
in pulmonary blood flow. During normal breathing, the lung apices tend to be under perfused,
whereas the bases are over perfused. Similarly, the V/Q ratio may increase when the alveoli are
well ventilated but are not well perfused. In the extreme, areas of the lung receiving adequate
perfusion but limited ventilation are considered shunts because the blood flowing through the
capillaries does not participate in adequate gas exchange. Conversely, areas of the lung that
appropriately ventilated without adequate perfusion are considered dead space because none of
the alveolar gas is diffusing into the pulmonary venous blood, nor is carbon dioxide being
eliminated.
The clinical signs of acute respiratory failure depend on the underlying pulmonary condition and
the severity of hypoxemia and/or hypercapnia. Young children demonstrate increased work of
breathing with tachypnea, nasal flaring, and retractions. Infants may have grunting with
respiratory effort in an attempt to maintain positive end-expiratory pressure and functional
residual capacity, and to decrease atelectasis. Respiratory distress accompanied by cyanosis or
pallor, disorientation, significant tachycardia, agitation, and lactic acidosis signals end-organ
oxygenation impairment and may herald impending respiratory arrest. Hypercapnia may cause
flushing, somnolence or confusion, and altered mental status. Contrary to popular belief, infants
and young children do not develop respiratory muscle fatigue, or “tire out,” from excessive work
of breathing from acute respiratory failure; however, they may develop increased somnolence if
their minute ventilation is unable to adequately clear carbon dioxide resulting in severe
hypercarbia.
The most common cause of cardiopulmonary arrest in children is acute respiratory failure. The
initial step in the management of pediatric respiratory failure is rapid assessment of airway
patency, breathing effort and effectiveness, and clinical signs of adequate cardiac output. This
enables determination of the appropriate interventions required to maintain adequate respiratory
status and avert deterioration in cardiopulmonary function. Although NIPPV is often first-line
therapy for acute respiratory failure, intubation and mechanical ventilation should be considered
when a child cannot maintain a patent and protected airway, or if deteriorating mental status or
respiratory effort results in an inability to maintain airway patency and normal gas exchange. If
NIPPV fails to reverse symptom severity (particularly tachypnea, tachycardia, and work of
breathing) after 1 to 2 hours, escalation to invasive ventilation may be warranted.
PREP Pearls
• Acute respiratory failure is caused by any clinical condition that impairs the pulmonary
system and may result in hypoxia, hypercarbia, or both.
• The most common cause ot cardiac arrest in children is an acute respiratory process; thus,
reversal of acute respiratory failure is critical in preventing cardiac arrest in children
• A trial of noninvasive positive pressure ventilation for acute respiratory failure may be
warranted when there is hemodynamic stability and no clear contraindication.

• Recognize the clinical and laboratory manifestations associated with respiratory failure of
various etiologies
• Recognize the clinical features of acute respiratory distress syndrome, including
associated sequelae
• Plan appropriate management for respiratory failure of various etiologies

Suggested Readings
• What are the current indications for noninvasive ventilation in children?. Curr Opin
Anaesthesiol, doi:10.1097/ACO.0b013e328339507b
• Noninvasive ventilation in pediatric intensive care. Curr Opin Pediatr,
doi:10.1097/MOP.0b013e328360dbdf
• CPAP and high-flow nasal cannula oxygen in bronchiolitis. Chest, doi:10.1378/chest.14-
1589
• Respiratory failure. Pediatr Rev, doi:10.1542/pir.35-11-476

Question 181
A 6-month-old infant is brought to the emergency department for diarrhaea and vomiting for the
past 4 days. He has been refusing to drink formula for a day. He has no fever, abdominal
distension, or blood in the stool. His heart rate is 130 beats/min, respiratory rate is 18
breaths/min, and blood pressure is 82/48 mmHg. He is lethargic and has a shrill cry when
aroused. His mucus membranes are dry and his capillary refill time is 3 seconds. His skin has a
doughy feel. The reminder of his physician examination findings are normal

Blood
Creatinine 0.4 mg/dL (35.4 µmmol/L)
Urine
Nitrite Negative
Blood Negative
Protein Negative
Of the following, the BEST next step in this Infant's management is to

A. administer a 0.45% sodium chloride bolus
B. administer intravenous vasopressin
C. correct his serum sodium over 24 hours
D. replace his free water deficit

Correct Answer: D
The infant in the vignette has hypernatremic dehydration secondary to acute gastroenteritis. The
history (diarrhea, vomiting, refusal to drink), signs of dehydration (tachycardia, prolonged
capillary refill, dry mucous membrane, doughy skin, high-pitched cry), and laboratory results
showing hypernatremia and high urine specific gravity favor this diagnosis. Intravenous fluid
therapy to correct hypernatremic dehydration should include replacement of the free water
deficit, electrolyte deficits, ongoing losses, and provision of maintenance fluids.
A child with hypernatremic dehydration has water loss that exceeds sodium loss, resulting in a
serum sodium level greater than 150 mEq/L (>150 mmol/L). Hypernatremic dehydration is
commonly seen in infants because of poor feeding and inadequate water intake after a diarrheal
illness (e.g., rotavirus). Hypernatremia (Hyperosmolality) leads to a shift in fluids from the
intracellular fluid compartment to the extracellular fluid compartment. Thus, in the early stages
of hypernatremic dehydration, children appear less hemodynamically compromised, causing
caregivers to delay seeking medical attention.
Infants with hypernatremic dehydration need close monitoring, free water deficit replacement,
and slow correction of their serum sodium level. A 20 mL/kg bolus of 0.9% normal saline should
be administered to children with hemodynamic compromise, just as is done for children with
hyponatremic and isonatremic dehydration. Free water deficit can be calculated using the
following formula
4 mL x weight (in kg) x (observed serum sodium — desired serum sodium)
An example of fluid replacement calculations, including the free water deficit and electrolyte
requirements, for a child with hypernatremic dehydration is shown in Item C181.
Serum sodium levels should initially be monitored every 4 to 6 hours in hypernatremic

dehydration. Infants with a serum sodium level greater than 160 mEq/L (160 mmol/L) should
undergo correction over a period of 48 to 72 hours. The target drop in the serum sodium level is
less than 0.5 mEq/L (<0.5 mmol/L) per hour or 10-12 mEq/L (10-12 mmol/L) per day. Rapid
correction of hypernatremia lead to cerebral edema and seizure.
The differential diagnosis of hypernatremia includes diabetes insipidus, which is associated with
polyuria and dilute urine. Diabetes insipidus is an unlikely diagnosis for the infant in the vignette
as he has concentrated urine with hypernatremia. Thus, administration of vasopressin is not
indicated.
PREP Pearls
• Hypernatremic dehydration occurs when water losses exceed sodium losses
• Hypernatremic dehydration correction includes replacement of the deficit, electrolyte
deficits, ongoing losses, in addition to provision of maintenance fluids.
• The target drop in the serum sodium level in hypernatremic dehydration should be less
than 0,5 mEq/L (<0.5 mmol/L) per hour or 10-12 mEq/L (10-12 mmol/L) per day, Rapid
correction of hypernatremia can lead to cerebral edema and seizure.

• Differentiate diabetes insipidus from other causes of hypernatremic dehydration
• Recognize the clinical and laboratory abnormalities associated with hypernatremic
dehydration, and manage appropriately
Suggested Readings
• Dehydration. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:2805-2813
• Dehydration: isonatremic, hyponatremic, and hypernatremic recognition and
management. Pediatr Rev, doi:10.1542/pir.36-7-274

Question 182
A 9-year-old boy is brought to the office for behavioral concerns. His parents recently met with
his school's principal because he has been talking back to his teachers and getting into physical
fights with his peers. At home, he throws toys and slam doors when he is not allowed to do what
he wants. He refuses to do his chores and homework. The boy's parents state that his behaviors
have been difficult to manage since he was young. They are concerned that the behaviors will
Escalate. He has an older cousin who has been in trouble with the law and is in a gang.
Of the following, the BEST next management step is to

A. prescribe a stimulant medication
B. recommend parent management training
C. recommend social skills training
D. request an individualize education program

Correct Answer: B
For the child in the vignette with defiant, argumentative, and aggressive behaviors, parent
management training is the best next step to address these behaviors. With this training, parents
are taught strategies to encourage and reinforce desired behaviors and techniques to decrease
unwanted behaviors.
Antisocial behaviors in children are those that violate the rights of others or the rules of society
(e.g., aggression toward others, property destruction, fire-setting, deceit, theft, truancy). The
child/adolescent with angry moods, argumentative behavior, and vindictiveness may meet
criteria for oppositional defiant disorder. The child/adolescent whose antisocial behaviors are
severe and persistent may meet criteria for conduct disorder.
When evaluating the child/adolescent with antisocial behaviors, information from parents,
teachers, and other adults should be collected to characterize the aggressive and disruptive
behaviors (e.g., quality, severity, frequency, timing, persistence), identify triggers for the
behaviors, determine how people respond to the behavior, and establish their impact on school,
home and social functioning. These behaviors should be considered in the context of the child’s
development. The child's perception of their situation should be ascertained. Children and
adolescents can provide important information about their home and school environment,
relationship with those affected by their behaviors, functioning, and mood. Standardized rating
scales can be helpful in identifying conditions that present with or coexist with antisocial
behaviors. These conditions include attention, deficit/hyperactivity disorder, oppositional defiant
disorder, conduct disorder, anxiety, and depression.
Psychosocial interventions such as individual therapy, parent training, and multimodal treatments
are first-line approaches for treating the child or adolescent with antisocial behaviors. Cognitive
behavioral therapy and training in problem solving, anger management, and conflict resolution
can help the child directly. The parent can benefit from training in behavioral management and
parent-child communication and interaction. Multisystemic therapy incorporates cognitive
behavioral therapy, behavior modification training, and family therapy. Addressing coexisting
mental health conditions with the appropriate therapy or medication can decrease aggression and
disruptive behaviors in children or adolescents with antisocial behaviors. Children and
adolescents with more severe or impairing behaviors should be connected to mental health
services. Potential providers of the specific therapies could include psychologists, marriage
family therapists, licensed clinical social workers, psychiatrists, and developmental behavioral
pediatricians. There are several evidence-based programs to help parents and/or teachers address
oppositional behaviors in children.
The child in the vignette should be evaluated for attention-deficit/hyperactivity disorder,

oppositional defiant disorder, conduct disorder, anxiety, and depression. If diagnosed with
attention-deficit/hyperactivity disorder, a stimulant medication would be an option for treatment.
A child with significant behavioral/mental health concerns may qualify for an individualized
education program under the category of emotional disturbance; this could be considered once a
diagnosis is established. Social skills training may be helpful for addressing problems with
understanding how to interact with peers; it would not address the difficulties with defiance
toward authority figures like teachers and parents.
PREP Pearls
• Psychosocial interventions such as individual therapy, parent training, and multimodal
treatments are first-line approaches for treating the child or adolescent with antisocial
behaviors.
• In parent management training, parents are taught strategies to encourage and reinforce
desired behaviors and techniques to decrease unwanted behaviors.
• Conditions that may present with or coexist with antisocial behaviors include attention-
deficit/hyperactivity disorder, oppositional defiant disorder, conduct disorder, anxiety,
and depression.

• Plan the appropriate management of antisocial behavior/delinquency
• Plan the appropriate evaluation of antisocial behavior/delinquency
Suggested Readings
• Conduct disorder and callous-unemotional traits in youth. N Engl J Med, doi:
10.1056/NEJMra1315612
• Early identification and treatment of antisocial behavior. Pediatr Clin North Am, doi:
10.1016/j.pcl.2016.06.008
• Disruptive behavior and aggression. American Academy of Pediatrics Textbook of
Pediatric Care. 2nd ed: 2017:1282-1289.
• Aggressive behavior in children and adolescents. Pediatr Rev, doi:10.1542/pir.32-8-325

Question 183
A 2-month-old infant is seen for a health supervision visit. Since her last visit, her father has
noticed a red skin lesion she had at birth has grown in size and become more raised and rubbery.
It does not bleed and is not painful. She has no other medical problems. Her vital signs and
growth parameters are normal. Physical examination findings are normal except for the
described lesion
Item Q183A
Item Q183B
Item Q183C
Item Q183D

Of the following, the lesion is BEST represented in
A. Item Q183A
B. Item Q183B
C. Item Q183C
D. Item Q183D

Correct Answer: D
The girl in the vignette has an infantile hemangioma (IH), best represented in Item C183A.
Item C183A
Infantile hemangiomas are benign vascular tumors characterized by rapid proliferation of blood
vessels beginning in the first few weeks after birth. This proliferation is usually completed by
about 5 months of age. During proliferation, IHs become more raised and rubbery, and may
ulcerate. Slow Involution follows, which may continue until about 4 years of age. Involution
usually begins centrally, often first noted as central pallor, and when complete. Telangiectasia,
discoloration, redundant skin, or scars may remain. A subset of IHs, abortive IHS (Item C183B)
do not proliferate and appear as telangiectatic macules.
Item C183B: abortive IHS
The other response choices represent benign vascular tumors that may be confused for IHs. Item
C183C shows a congenital hemangioma, which may exhibit telangiectasia and surrounding
pallor. Congenital hemangiomas are fully formed at birth and do not continue to grow. Item
Cl83D is a pyogenic granuloma, which tends to bleed easily and is more pedunculated. Item
Cl83E is a tufted angioma, a rare tumor that is more plaque-like and may exhibit excess sweat or
hair.

Item C183C congenital hemangioma
Item Cl83D: pyogenic granuloma
Item Cl83E: tufted angioma

Infantile hemangiomas are classified based on depth: superficial, deep, or combined (Item
C183F, panels A, B, and C respectively) and distribution pattern: localized segmental (Item
C183G), multifocal (Item Cl83H), or indeterminate. Classification informs prognosis and
morbidity. Superficial IHs usually have a shorter proliferation phase than deeper IHs. Deeper IHs
may impinge on internal structures. Segmental IHs may indicate a syndrome, for example,
PHACE (posterior fossa, anomalies, hemangioma on the face, arterial anomalies, cardiac
anomalies, and eye anomalies) and LUMBAR (lower body IH and other cutaneous defects,
urogenital anomalies and ulceration, myelopathy, bony deformities, anorectal malformations,
and arterial anomalies, and renal anomalies) syndromes. Multifocal IHs may be associated with
hepatic involvement; if more than 5 cutaneous IHs are present, liver ultrasonography should be
performed.
Item C183F,G,H: Classification of Infantile Hemangioma

• PATTERN
o Focal
o Multifocal
o Segmental
o Indeterminate
• DIFFERENT TYPES
o Superficial
o Deep
o Mixed (superficial + deep)
o Reticular/abortive/minimal growth
o Others
• ASSOCIATION WITH OTHER LESIONS
o PHACES syndrome
▪ Posterior fossa malformations
▪ Hemangioma
▪ Arterial anomalies
▪ Cardiovascular anomalies
▪ Eye anomalies
▪ Sternal clefting or supraumbilical raphe
o LUMBAR (SACRAL, PELVIS) syndrome
▪ Lower body hemangioma
▪ Urogenital anomalies
▪ Ulceration
▪ Myelopathy
▪ Bony deformities
▪ Anorectal malformations
▪ Arterial anomalies
▪ Renal anomalies
Although most IHs do not require intervention, those with complications do. Ulceration is the
most common complication and can lead to bleeding, infection, pain, and scarring. Management
of ulceration is listed in Item C183I.

Item C183I: Treatment Options in the Management of Ulcerated IH
Early referral is indicated for IHs In certain locations:

• Perioral: May interfere with feeding because of both the IH itself and its ulceration.
• Airway: Usually present as worsening biphasic stridor and barky cough in the first 6
months after birth. These require early otolaryngology referral. It is important to note that
half of all children with IHs involving the airway will have cutaneous IHs and
approximately half of those who have cutaneous IHs in the “beard” distribution (Item
Cl83J) will have an airway IH
• Orbital or periocular: Require early ophthalmology referral to mitigate and detect
amblyopia, strabismus, and refractive errors because of the mechanical obstruction
casued by IH and, if visual field is obstructed, the lack of stimulation of the visual cortex.
• Nose (and other cosmetically significant areas): May require earlier surgical resection.
• Perineum: Prone to ulceration. Surgical resection is complicated in this area; aggressive
medical therapy is the treatment of choice.
• Hepatic: Diffuse hepatic His, while rare, are extremely serious. Complications may
include consumptive hypothyroidism because of type 3 iodothyronine deiodinase
production, hepatomegaly, abdominal compartment syndrome, renal vein compression,
and compromise of inferior vena cava flow. Rarely, large IHs or multiple hepatic IHs
may result in high output congestive heart failure because of shunting.
High-risk IHs (Item C183K ) require systemic therapy. Therapy should be initiated as early as
possible in the proliferation phase for maximal effect. Oral propranolol is first-line therapy
because of its high safety profile. Some experts recommend additional screening and monitoring
procedures when initiating propanolol in high risk infants (Item C183L). Providers should be
aware of propranolol contraindications and complications (Item C183M), especially
hypoglycemia; it is important to take it with food and temporarily decrease or stop the
propranolol in the event of an acute illness that compromises oral intake. Propranolol therapy is
continued until the IH would have been expected to naturally begin involution and is tapered
gradually. Rebound may occur if the IH is large or has a longer proliferation phase and in this
case, propranolol can be restarted.
Item C183K :High-risk IHs
Item C183L: additional screening and monitoring when initiating propanolol

• Bradycardia and hypotension are known to accompany propranolol-associated β-receptor
blockade, both tend to be mild and asymptomatic in children treated for IHs who have no
preexisting cardiac comorbidities
o In-office intermittent heart rate and blood pressure monitoring for 2 hours after the first
dose of propranolol or for increasing the dose for infants 5 weeks’ adjusted gestational
age or older
o Monitoring for those who are younger or for those with other comorbidities should be
individualized and may require brief hospitalization for medication initiation. These
recommendations may change over time as more information becomes available now that
the medication is in widespread use
• ECG screening only
o in infants with a baseline heart rate below normal for age
o in infants with a family history of congenital heart conditions or arrhythmias or with a
maternal history of connective tissue disease
o when there is a history of arrhythmia or one is auscultated during examination

Item C183M: propranolol contraindications and complications
If propranolol therapy is contraindicated or the response is inadequate, systemic steroids, topical

timolol, or intralesional steroids may be considered. Adverse effects of systemic steroids are
usually transient, but children should be monitored closely and dosing reduced or aborted if not
tolerated. Systemic absorption of topical or intralesional therapies may occur if given in large
doses or the lesion is in an unfavorable location (e.g., mucosal surfaces).
Surgical resection, when indicated, is usually performed around the end of the involution phase
and may include removal of scar tissue or reconstruction. Resection during the proliferation
phase is an option if medical therapies are unsuccessful or contraindicated, the location is
favorable, future surgery would be simplified if required, and final scarring would be
comparable.
Pulsed-dye laser therapy may be used for situations in which cosmetics are important, although
scarring or hypopigmentation may rarely result.
PREP Pearls
• Infantile hemangiomas are characterized by a proliferation phase in the first few months
after birth, causing them to become more raised and rubbery without bleeding or pain,
which is followed by a period of involution.
• High-risk infantile hemangiomas require early and aggressive management by a
multidisciplinary team.
• Most infantile hemangiomas do not require treatment, but if treatment is indicated, oral
propranolol is the first-line therapy.
MOCA-Ped Objective
• Identify and manage cutaneous vascular lesions of infancy.

• Recognize hemangioma and manage appropriately
Suggested Readings
• Hemangiomas. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed:
2017:2110-2116.
• Diagnosis and management of infantile hemangioma, Pediatrics, doi:10.1542/peds.2015-
2485
• Clinical practice guideline for the management ot infantile hemangiomas. Pediatrics,
doi:10.1542/peds.2018-3475

Question 184
An 18-month-old girl is evaluated in the emergency department for 2 days of nonbilious,
nonbloody emesis. She had 2 other discrete, similar episodes in the past 6 months. With each
episode, the girl experiences fatigue and decreased appetite. During one of the previous episodes,
laboratory studies were notable for an elevated lipase (722 U/L). She is otherwise healthy and
does not take any medications. Her weight-for-length has decreased from the 75th percentile at
age 12 months to the 50th percentile at age 18 months. Her physical examination findings are
notable for a soft abdomen with epigastric tenderness. Laboratory data are shown:

Lipase 1,321 U/L
Alanine transferase 51 U/L
Total bilirubin <0.5 mg/dL (<8.6 µmol/L)
Albumin 0.042 g/dL (0.42 g/L)
Vitamin D, 25-hydroxyvitamin 18 ng/mL (44.9 nmol/L) (reference range. 30-100 ng/mL;
D 74.9-249.6 nmol/L)
Pancreatic Elastase 30 µg/g (reference range. >200 µg/g)
Abdominal ultrasonography demonstrates pancreatic calcifications and a normal appearing liver

and gallbladder.
Of the following, the girl's MOST likely diagnosis is

A. acute pancreatitis
B. acute recurrent pancreatitis
C. chronic pancreatitis
D. isolated hyperlipasemia

Correct Answer: C
The girl in the vignette has findings most consistent with chronic pancreatitis, these include
abdominal ultrasonography demonstrating pancreatic calcifications and evidence of exocrine
pancreatic insufficiency, as shown by a low elastase level and fat-soluble vitamin (vitamin D)
deficiency.
Acute pancreatitis (AP) is diagnosed when at least 2 of 3 criteria are met:

• Abdominal pain consistent with AP (e.g., acute onset and/or epigastric pain but can be
vague, particularly in younger children)
• Serum amylase and/or serum lipase levels >3 times the upper limit of normal
• Imaging findings consistent with pancreatitis (e.g., pancreatic edema, pancreatic necrosis,
pancreatic abscess, pancreatic pseudocyst, pancreatic hemorrhage)
Acute recurrent pancreatitis (ARP) is diagnosed following at least 2 episodes of AP and 1 of the
following:
• Complete resolution of pain (lasting longer than 1 month) between AP episodes
• Complete normalization of pancreatic enzyme measurements (amylase, lipase) between
AP episodes
Chronic pancreatitis (CP) is diagnosed with imaging consistent with CP (e.g., pancreatic
calcifications, pancreatic duct strictures and/or dilations) AND at least 1 of the following criteria:
• Abdominal pain consistent with pancreatitis
• Evidence of exocrine pancreatic insufficiency
• Evidence of endocrine pancreatic insufficiency
A diagnosis or CP can also be made by pancreatic biopsy

The differential diagnosis for causes of pancreatitis (AP, ARP, and CP) is broad (Item C184).
Risk factors for the development of ARP or CP Include:

• Genetic mutations
o Cystic fibrosis transmembrane conductance regulator (CFTR). PRSS1, SPINK1,
CTRC,CPA1
• Anatomic/Obstruction
o Pancreas divisum. annular pancreas
• Toxic/metabolic disorders
o Hyperlipidemia, medications (e.g., valproic acid, 6-mercaptopurine)
• Autoimmune disease
o Autoimmune pancreatitis, primary sclerosing cholangitis

Item C184: The differential diagnosis for causes of pancreatitis (AP, ARP, and CP)

Initial laboratory testing to identify the cause of ARP of CP should include:
• Serum lipase and/or amylase
• Complete blood count
• Glucose
• Electrolytes
• Albumin
• Calcium
• Liver function tests
• Fecal pancreatic elastase
• Fat-soluble vitamin levels (A, E, 25-hydroxyvitamin D)
• Prothrombin time or international normalized ratio
Diagnostic imaging should include abdominal ultrasonography and magnetic resonance imaging
cholangiopancreatography
Identifying children with CP is important, as it is an irreversible process that can result in

nutritional deficiencies (particularly fat soluble vitamin deficiencies), development of
pancreatogenic diabetes mellitus, and chronic pain. Monitoring for these sequelae is important.
Smoking, alcohol use, and obesity have been shown in adult patients to worsen CP prognosis:
thus, anticipatory guidance around these risks should be provided.
The girl in the vignette meets criteria for chronic pancreatitis (with imaging consistent with CP
and exocrine pancreatic insufficiency). A diagnosis of AP ARP would not be appropriate in this
situation given the findings of CP. Isolated hyperlipasemia (isolated lipase elevation) can occur
in individuals without evidence of pancreatitis, and has been reported in extra-pancreatic
diseases including esophagitis, and malignancy.
PREP Pearls
• Risk factors for the development of acute recurrent pancreatitis and chronic pancreatitis
include genetic mutations. Toxic/metabolic disorders, anatomic causes, and autoimmune
diseases.
• Chronic pancreatitis is diagnosed when imaging findings are consistent with chronic
pancreatitis; and there is at least 1 of the following. 1) abdominal pain consistent with
pancreatitis, 2) exocrine pancreatic insufficiency, 3) endocrine pancreatic insufficiency
• Complications of chronic pancreatitis include nutritional deficiencies, development of
pancreatogenic diabetes mellitus, and chronic pain

• Recognize the clinical and laboratory features associated with pancreatitis
• Formulate a differential diagnosis for a patient who has chronic pancreatitis

Suggested Readings
• Medical management ot chronic pancreatitis in children: J Pediatr Gastroenterol nutr,
doi:10.1097/MPG.0000000000003001
• Definitions of pediatric pancreatitis and survey of present clinical practices. J Pediatr
Gastroenterol Nutr, doi:10.1097/MPG.0b013e031824f1516
• Pancreatitis. Pediatr Rev, doi: 10.1542/pir.2018-0252.
• Pancreatitis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:2453-2466.

Question 185
A 6-year-old girl is hospitalized after a house fire, in which she sustained second- and third-
degree burns to her upper extremities, face, torso, and legs that cover 38% of the total body
surface area. She did not sustain any significant inhalation injury. On the day of admission to the
hospital, the child develops a fever and tachycardia. She has a temperature of 38.6 º C, heart rate
of 143 beats/min, respiratory rate or 22 breaths/min, and blood pressure of 115/75 mm Hg. The
diagnosis of a burn wound infection is considered.
Of the following, the finding that would be MOST consistent with this diagnosis is
A. 105 bacteria per gram of tissue from a burn wound biopsy
B. erythema and tenderness of the skin surrounding the burned tissue
C. a positive bacterial culture from a swab of the center of the burn
D. a temperature ≥38.5 º C

Correct Answer: A
The child in the vignette has signs and symptoms of increased metabolic demand, which may
easily be confused with infection in a child who has sustained a burn. The gold standard finding
used to diagnose a burn wound infection is the presence of 105 bacteria per gram of tissue from a
burn wound biopsy or the isolation of fungus or yeast on a culture. Burn wounds are often
contaminated with skin flora. Thus, a positive bacterial culture from the wound that does not
meet this criterion does not indicate infection. Due to their hyperdynamic state, the severely
burned child often develops fever, tachycardia, and tachypnea in the absence of infection; the
presence of these signs does not necessarily indicate the burn is infected. Erythematous, tender
skin around burned tissue is often due to the injury itself and does not necessarily indicate
infection. In burn patients, it is imperative that providers distinguish signs and symptoms of
infection or sepsis from the hypermetabolic state.
Skin plays an essential role in infection prevention. Normal skin provides a natural protection
from bacteria. A child who has been burned is more susceptible to both wound infection and
generalized infection, including sepsis. Infection may be difficult to recognize in a burn patient,
as they frequently have elevated temperatures, tachycardia, and tachypnea because of a
continuous release of inflammatory mediators due to the burn itself. In addition to a positive burn
wound biopsy demonstrating the presence of 105 bacteria per gram of tissue, common physical
examination findings associated with burn infection include:
• Rapid change in the appearance of the wound
• Loss of previously viable tissue or skin graft
• Conversion to deeper tissue injury (partial to full thickness)
• Increased pain, discoloration, purulent exudate edema, swelling, drainage, or malodor
from the burn wound
The most common organisms that cause burn wound infections are Staphylococcus spp and
Pseudomonas. For the first 5 days after injury, the most common organisms to cause infection
are gram-positive bacteria such as Staphylococcus aureus and Enterococcus spp. Beyond the
fifth day, the most common organisms include gram-negative bacteria such as Pseudomonas
aeruginosa and Escherichia coli. Pseudomonas infection is often associated with bluish-green
drainage and a sweet smell. Less commonly, burns can be infected by fungi such as Candida,
Aspergillus, or Mucor species or viruses; the most common viral infection is herpes simplex
virus type 1 (HSV-1).
Burns lessen the skin's ability to prevent infection. Patients with >20% of total body surface area
involved are at greater risk for burn wound infection and sepsis. Other infections that may occur
in children with a burn injury include sepsis, catheter-related infections, urinary tract infections,
and pneumonia.
Oral or intravenous prophylactic antibiotics are not recommended to prevent infections in burn
patients. Recommended steps to reduce risk or prevent burn wound infection include thorough
cleaning and debridement of the burn wounds, antibiotics (e.g., bacitracin ointment and silver
sulfadiazine cream), and the use of commercially available impregnated burn dressings (e.g.,
bismuth-impregnated petroleum gauze).

Burns are a leading cause of traumatic death in children, the majority occurring in the home. The
most common causes of burns in children include flame injury, scald, direct contact with hot
objects, and non-accidental trauma. Burns in the home largely preventable therefore, counseling
on burn injury prevention is an essential part of the health supervision visit. Item C185 suggest
items to include during counseling. It is particularly important to counsel parents to ensure that
their hot water heater is set so that the temperature of the water does not exceed 120 °F at the
faucet.
Item C185: Selected Elements of Burn Prevention Counseling

• Do not let cooking appliance electric cords hang off a counter.
• Do not leave hot beverages or foods unattended or near the edge of a table where a curious child
can reach them.
• Keep hot beverages away from children and do not have a child sit in your lap while you drink a
hot beverage.
• Teach older children how to safely remove hot food from a microwave or stove top.
• Minimize use of front burners on stoves so curious children cannot pull down pots of hot liquid.
• When carrying hot foods in the kitchen, ensure children are not in the path of movement.
• Test bathwater and shower temperature with your hand for 30 seconds.
• Never leave children unattended in the bath or shower.
• Adjust water heater so that the hottest temperature at the faucet is <120°F.
• Avoid leaving unattended pots on a stove.
• Keep children away from fireplace and wood stove doors with appropriate guards.
• Install smoke detectors on every floor of a dwelling and test them monthly. They are ideally hard-
wired with battery backup.
• Batteries should be replaced at least annually on a schedule.
• Practice home fire drills and make sure children know how to exit the house and where to meet
outdoors.
• Keep fire extinguishers in the kitchen, in the furnace room, and near a fireplace.
• Teach children to exit the house low to the floor if there is smoke in a room.
• Obtain a safety ladder if your home has a second story.
• Teach children who live in tall buildings not to use the elevator during a fire.
• Teach your children to "stop, drop, and roll" if clothing is on fire.
• Avoid smoking indoors.
• Minimize storage of flammable liquids and keep them away from areas where children can play
and away from any ignition sources.
• Minimize use of extension cords.
• Keep matches and lighters where they will not be available to children.
• Avoid use of fireworks.
PREP Pearls
• Significant bums result in a hypermetabolic state caused by the continuous release of
inflammatory mediators. This leads to elevated temperature and increased heart and
respiratory rates that may be difficult to distinguish from infection or sepsis.
• Diagnosis of burn wound infection requires demonstration of more than 105 bacteria per
gram of tissue obtained by biopsy of the burn site.
• Counseling on burn injury prevention is an essential part of health supervision visits.

• Counsel parents regarding prevention Of burns (e.g., matches, electrical burns, fireworks,
hot water heater settings)
• Recognize the major infections seen in patients with burn injuries
Suggested Readings
• Initial assessment and management of thermal burn injuries in children. Pediatr Rev,
doi:10.1542/pir.34-9-395
• Burns. Pediatr Rev, doi:10.1542/pir.25-12-411
• Burn care for children. Pediatr Rev. doi:10.1542/pir.2016-0179
• Thermal injuries. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017;2821-2828

Question 186
An 8-year-old boy is being evaluated for persistent neck pain and torticollis 5 days after a failed
front flip on his backyard trampoline. He was initially brought to a local urgent clinic where
cervical spine radiographs were reported as normal. At that time, he was prescribed a
nonsteroidal anti-inflammatory drug and provided with a soft collar to wear for comfort. His
mother reports no significant change in his symptoms since then. He has no neurologic
symptoms, including changes in gait or bowel and bladder function. He was previously healthy
with normal growth and development.
On physical examination, the boy holds his neck in left lateral flexion with his chin rotated to the
right (i.e., “cock-robin” position). He is able to actively rotate to the right, but is not able to
actively rotate to the left past midline. Findings of a full neurologic examination are normal.
Of the following, the BEST next step in this boy’s care is to

A. perform cervical spine computed tomography
B. perform cervical spine magnetic resonance imaging
C. refer him for neurosurgical evaluation
D. refer him for physical therapy

Correct Answer: A
This boy's history and physical examination findings most strongly suggest atlantoaxial rotary
subluxation (AARS). Given his lack of improvement with initial conservative management, thin-
slice dynamic computed tomography (CT) of his cervical spine is the most appropriate next step.
This study will provide a definitive diagnosis, which is needed before proceeding with physical
therapy or additional treatment modalities. Radiography is often problematic; because of
difficulties with patient positioning, the interpretation is often compromised. Therefore, some
experts recommend proceeding directly to CT scanning without prior radiography in these cases.
Atlantoaxial rotary subluxation, a subluxation of the facet joints between C1 and C2 should be
suspected in any child with new-onset painful torticollis. Peak incidence is between 6 and 8 years
of age, and is attributed to the relative ligamentous laxity in this age group. Atlantoaxial rotary
subluxation can be seen after trauma; in patients with Down syndrome, rheumatologic disease, or
other congenital or acquired abnormalities of the upper cervical spine; and rarely after an upper
respiratory infection, tonsillectomy, or other surgery involving the upper airways.
Early recognition of AARS is important for minimizing the risk of long-term sequelae. History
and physical examination should focus on identifying signs or symptoms that may indicate a
myelopathy or spinal cord involvement (Item C186). During the evaluation, torticollis of
muscular origin can be distinguished by spasm of the sternocleidomastoid on the side
contralateral to chin rotation. In patients with AARS, sternocleidomastoid spasm occurs
ipsilaterally. A child with any evidence of myelopathy should be referred for neurosurgical
consultation and possible magnetic resonance imaging (MRI). Although MRI shows superior
visualization of the spinal cord and soft tissue without radiation exposure, it typically does not
demonstrate sufficient detail of bony anatomy to diagnose AARS. Computed tomography is
imaging modality of choice in the absence of myelopathy or focal neurologic deficit.
Atlantoaxial rotary subluxation will typically resolve within 1 to 2 weeks with conservative
management. Which may include rest, soft collar, nonsteroidal anti-inflammatory drugs,
stretching exercises, and/or physical therapy. Prolonged persistence of this subluxation makes
definitive treatment more difficult, and these patients should be closely followed to ensure
resolution. Children with symptoms that persist despite these measures, or those with delayed
presentation, referred to a neurosurgeon or pediatric orthopedic surgeon for initiation of traction
therapy. Surgical reduction and fixation is indicated children who do not respond to traction
therapy, have neurologic deficits, or have recurrent episodes of AARS
PREP Pearls
• Atlantoaxial rotary subluxation (AARS) should be considered in any child who has a new
onset of painful torticollis. Torticollis of muscular origin can be distinguished by spasm
of the sternocleidomastoid on the side contralateral to chin rotation whereas in patients
with AARS, sternocleidomastoid spasm occurs ipsilaterally.
• Dynamic cervical computed tomography scanning is the study of choice to evaluate for
possible atlantoaxial rotary subluxation.
• Any child with myelopathic or focal neurologic findings on examination should be
referred for neurosurgical evaluation.

• Understand the association between atlantoaxial instability and potential neurologic
complications
Suggested Readings
• Atlantoaxial rotatory subluxation: a review for the pediatric emergency physician. Pediatr
Emerg care, doi:10.1097/PEC.0000000000000817
• Atlantoaxial rotatory subluxation in children. Pediatr Emery Care, doi:
10.1097/PEC.0000000000001023
• Atlantoaxial rotary subluxation or fixation. Pediatric Orthopaedics and Sports Injuries;
2010;169-175

Question 187
A 4-year-old, previously healthy boy who is fully immunized (including with the bacillus
Calmette-Guérin vaccine) is seen for a routine health supervision visit. He was born in India and
emigrated to the United States at 1 year of age. His mother states that the boy's grandfather, who
is visiting from India, was recently diagnosed with reactivation tuberculosis. The grandfather
arrived 2 months ago and has had a cough and weight loss. The boy has not had fever or cough
and has been gaining weight appropriately. His physical examination findings are normal.
Anteroposterior and lateral chest radiographs obtained on the day of the visit are normal.
Of the following, the BEST next management step for this boy is to order a/an
A. interferon-γ release assay, and start isoniazid if positive
B. interferon-γ release assay, and start isoniazid immediately
C. sputum for acid-fast bacillus smear and mycobacterial culture
D. tuberculin skin test, and start isoniazid if positive

Correct Answer: B
This boy in the vignette has a close contact (grandfather) with symptomatic pulmonary
tuberculosis disease who recently arrived from a tuberculosis-endemic region. Symptoms of
reactivation tuberculosis are insidious and may be present for weeks or months before the
diagnosis is made. All household members, including this boy, should undergo evaluation for
tuberculosis infection. The boy is asymptomatic and has normal chest radiograph findings.
Because the boy has received the bacillus Calmette-Guérin (BCG) vaccine and his age is
younger than 5 years, the best next step in management is to order an interferon-γ release assay
(IGRA) and start isoniazid immediately.
Asymptomatic child ren with suspected tuberculosis infection or disease should undergo
evaluation with either a tuberculin skip test (TST) or IGRA, based on age and history of BCG
vaccination. The boy was born in India, where the BCG vaccine is administered at birth or as
soon as possible before age 1 year. Receiving the BCG vaccine soon after birth can result in
false-positive TST up to 1 years the of age, with reactivity of less than 10 mm. The IGRA result
is not affected by BCG vaccination.
Children younger than 5 years and those who are immunocompromised are at high risk of
progression from tuberculosis infection to disease. Children who are at high risk, when exposed
to tuberculosis, should receive prophylaxis, even if the result of the initial tuberculosis test (TST
or IGRA) is negative. A repeat TST or IGRA should be performed 8 to 10 weeks after the last
exposure to the infected person. If the result of the repeat test is negative, prophylaxis can be
discontinued. Microbiologic evaluation with acid-fast bacillus smears and culture should be
performed in symptomatic children and those with radiographic findings consistent with active
tuberculosis. Options for prophylaxis include isoniazid for 9 months, rifampin for 4 months, or
isoniazid-rifapentine weekly treatment for 3 months.
Tuberculosis is a communicable disease that affects 1 in 3 individuals worldwide. It is caused by

Mycobacterium tuberculosis complex, which includes M tuberculosis, Mycobacterium bovis,
Mycobacterium africanum (rare in the United States), and a few other species. Transmission of
tuberculosis is via the airborne route. Small droplet nuclei, 1 to 5 µm in diameter, are released
when a person with tuberculosis disease coughs, sneezes, shouts, or sings. Children almost
always acquire tuberculosis from an adult close contact.
After exposure to tuberculosis, there are 3 possible outcomes:

• The immunocompetent child eliminates M tuberculosis with innate and adaptive immune
responses
• The child develops latent infection; the immune system/granuloma is able to control but
not eliminate M tuberculosis
• The child develops active primary tuberculosis; the adaptive immune system is unable to
control M tuberculosis. Primary tuberculosis is the least common outcome, occurring
most often in children younger than 5 years and immunocompromised hosts. More than
90% of affected children develop active disease within 12 months of exposure.
Active tuberculosis can also occur because of reactivation of disease. After a period of latency,
usually in the setting of immunosuppression or immune senescence, granuloma is unable to
control proliferating M tuberculosis. The lifetime risk of reactivation is approximately 5% to
10% in those with latent tuberculosis. In children, reactivation is not seen until late childhood or
adolescence.
The 2 available diagnostic tests for tuberculosis are the TST and the IGRA. Both tests assess host
immune response to tuberculosis and cannot distinguish latent tuberculosis infection from active
disease. The choice of test should be based on age, setting, availability, and cost. The TST is a
trans dermally injected purified protein derivative that stimulates a delayed-type hypersensitivity
response to tuberculosis antigen. Induration within 48 to 72 hours indicates a current or prior
tuberculosis infection. There exists significant interobserver reliability with TST placement and
interpretation. False positives are seen with prior BCG vaccination and nontuberculous
Mycobacterium (NTM) infections. The IGRA test measures interferon-γ production after in vitro
stimulation of T cells with M tuberculosis antigens. False positives caused by the BCG
vaccination or prior NTM infection are less likely because the IGRA does not include antigens
shared by BCG or common NTM. The American Academy of Pediatrics has expanded the
recommendation for IGRA testing in children as young as 2 years of age if they received the
BCG vaccine or are unlikely to return between 48 and 72 hours for a TST reading. An algorithm
for latent tuberculosis testing is shown in Item C187.
Children who are symptomatic with fever, cough, hemoptysis, or weight toss, or have chest
radiography findings consistent with active tuberculosis should undergo further microbiologic
evaluation. Acid-fast bacillus smears and mycobacterial cultures should be obtained using the
most convenient and feasible method, with either induced sputum or gastric lavage.

Item C187: An algorithm for latent tuberculosis testing
PREP Pearls
• Children younger than 5 Years and immunocompromised hosts are at high risk
progression to active disease after exposure to a person with tuberculosis
• Interferon-γ release assays to diagnose tuberculosis can be used in children as young as 2
years with a history of bacillus Calmette-Guérin vaccination
• Children younger than 5 years who are exposed to tuberculosis should receive isoniazid
prophylaxis even if the result of the initial tuberculosis test (tuberculin skin test or
interferon-γ release assay) result is negative.

• Recognize the major clinical features associated with Mycobacterium tuberculosis
infection
• Understand the epidemiology of Mycobacterium tuberculosis
• Understand the diagnostic tests useful in the evaluation of tuberculosis (both latent and
active)
Suggested Readings
• Tuberculosis. Red Book: 2021-2024 Report of the Committee on infectious Diseases.
32nd ed; 2021:786-813.
• Mycobacterium tuberculosis. Principles and Practice of Pediatric Infectious Diseases. 5th
ed. 2018:790-806.
• Clinical manifestations of tuberculosis in children. Paediar Respir Rev,
doi:10.1016/j.prrv.2007.04.008
• Tuberculosis in children, Pediatr Rev, doi:10.1542/pir.2018-0093
• Tuberculosis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:2718-2732.

Question 188
A 3-year-old boy is being evaluated for a 5-day history of painful “diaper rash”. His mother
reports that he has pain with stooling wiping. He is otherwise well, and his medical history is
unremarkable. The boy is afebrile. The only physical examination finding of note is the
appearance of his perianal skin (Item Q188)
Of the following, the MOST appropriate treatment is

A. cephalexin orally
B. clotrimazole topically
C. hydrocortisone topically
D. nystatin orally

Correct Answer: A
The boy in the vignette has a painful rash around his anus that is intensely erythematous and has
a well-defined border; there is maceration and some exudate. These findings are typical of
perianal bacterial dermatitis (formerly termed perianal streptococcal dermatitis or perianal
cellulitis). Treatment with oral cephalexin is appropriate. Other common forms of diaper rash
(e.g., irritant contact, candidiasis, seborrheic dermatitis, psoriasis) are not limited to the perianal
area and, therefore, treatment with topical hydrocortisone or clotrimazole or oral nystatin is not
warranted.
Perianal bacterial dermatitis is a superficial cellulitis caused by Streptococcus pyogenes or

Staphylococcus aureus. The peak incidence is between 3 and 4 of age, and boys are affected
more often than girls. Most affected children are well but some may have concomitant
streptococcal pharyngitis. Other family members may be affected, especially there has been co-
bathing with the affected child.
Perianal bacterial dermatitis is characterized by intense perianal erythema with a well-defined

border. Pruritus or discomfort is usually present, and a foul odor may be noted. Children may
experience pain with defecation, stool withholding, or passage of blood-tinged stools.
Occasionally, affected children have associated balanoposthitis or vulvovaginitis. Less
commonly, the neck folds, axillae, or inguinal creases may be involved as part of streptococcal
intertrigo (Item C188)
The diagnosis of perianal bacterial dermatitis is usually made clinically but may be confirmed
with bacterial skin culture. If a culture is performed, it is prudent to inform the laboratory or the
suspected diagnosis because routine processing of perianal swabs may involve the use of
inhibitors to the growth of S pyogenes.
Perianal bacterial dermatitis may be treated with a 14-day course of oral penicillin or
amoxicillin. However, because S aurous appears to be an increasingly common cause, some
clinicians prefer oral cephalexin or another antibiotic that possesses anti-staphyloccoccal activity
based on local resistance patterns or the results of bacterial culture and sensitivity. An oral
macrolide is an acceptable alternative for children with a penicillin allergy. Rarely, there is
recurrence of infection, which may necessitate a repeat course of antibiotics.

PREP Pearls
• Perianal bacterial dermatitis is characterized by intense, well-defined perianal erythema,
often with maceration and exudate. Pruritus or discomfort is usually present.
• Perianal bacterial dermatitis may be caused by Streptococcus pyogenes or Staphylococcus
aureus.

• Plan the appropriate management of cellulitis of the skin of various etiologies
Suggested Readings
• Perianal bacterial dermatitis. Pediatric Dermatology: A Quick Reference Guide 4th ed.
2020:193-196.
• Bacterial skin infections. American Academy of Pediatrics Textbook of Pediatric Care.
2nd ed; 2017:1786-1792.
• Practice guidelines for the diagnosis and management of skin and soft tissue infections.
Clin Infect Dis, doi:10.1093/cid/ciu296

Question 189
A 4-year-old girl is brought to the clinic for routine health supervision visit. She can hop on one
foot 2 to 3 times but cannot jump backward; throw a ball overhand; cut a straight line with
scissors but not circle; string small beads together; vocalize when she is sad, scared, happy or
angry; follow 3-step commands: and tell stories but without a clear beginning, middle, and end.
Of the following, this child most likely has developmental DELAY in

A. Fine motor skills
B. Gross motor skills
C. language skills
D. social-emotional skills

Correct Answer: A
The domains of development and their relevant skills are described in Item C189A. The girl in
the vignette is 4 years of age. At this age, expected fine motor skills include copying a square,
tying single knots, cutting a 5-inch circle, using tongs to transfer, writing part of the first name,
imitating a gate with cubes, and drawing a 4- to 6-part person.
Item C189A: domains of development
The child in the vignette can cut a straight line but is not yet cutting a circle; she possibly has
delayed fine motor skills. This would need further evaluation, as a perceived delay in this area
may sometimes be due to lack of exposure. Her other developmental domains are appropriate for
age. Item Cl89B and Item Cl89C list the gross motor, fine motor, self-help, problem-solving,
social-emotional, receptive language, and expressive language developmental milestones
expected for 3- and 4-year-old children and 5- and 6-year old children, respectively.


PREP Pearls
• The 5 domain of development include fine motors, gross motor, language, cognitive and
social-emotional.
• At 4 years of age, expected fine motor skills include copying a square, tying a single
knots, cutting a 5-inch circle, using tongs to transfer, writing part of the first name,
imitating a gate with cubes, and drawing a 4- to 6-part person

• Evaluate the cognitive and behavioral developmental progress/status of a child at 4 years
of age
• Evaluate the motor developmental progress/status of a child at 4 years of age
Suggested Readings
• The preschool years. Nelson Textbook of Pediatrics 21st ed.
• Developmental milestones. Pediatr Rev, doi:10.1542/pir.2014-0103

Question 190
A 16-year-old adolescent boy is seen in the emergency department for chest pain, He describes
the pain as sharp and points to his lower sternum and to the left. His pain improves while sitting
still. He is active in school sports but has not been playing recently due to an upper respiratory
infection that seems to be improving. He is afebrile, and his vital signs are a heart rate of 110
beats/min, respiratory rate of 18 breaths/min, blood pressure of 100/65 mm Hg, and oxygen
saturation of 99% in room air, His heart and lung sounds are normal on auscultation. The pain is
reproducible with deep inspiration. The boy is notably uncomfortable when he lies down. The
remainder of his examination findings are unremarkable. A chest radiograph is normal. An
electrocardiogram is obtained (Item Q190). Laboratory data are significant for an elevated C-
reactive protein level, erythrocyte sedimentation rate, and troponin level.
Item Q190: adolescent ECG
Of the following, the BEST next step in this adolescent's management is

A. Cardiac catheterization
B. Cardiac magnetic resonance imaging
C. Chest computed tomography
D. Echocardiography

Correct Answer: D
The adolescent in the vignette has sharp, substernal chest pain, which is relieved by and sitting
and exacerbated when supine, in the setting of a recent upper respiratory infection. He has
elevated inflammatory markers and troponin level (due to myocardial cell injury). This
presentation is most consistent with acute pericarditis. An echocardiogram must be obtained to
assess cardiac function and any involvement of the myocardium. The other answer choices are
studies that may ultimately be needed but are not part of the typical initial evaluation of
suspected pericarditis.
Pericarditis is the most common disease that impacts the pericardium. The true incidence is
unknown as many cases are likely not brought to medical attention, but it is not uncommon.
There is a higher incidence in adolescent boys. In Europe and North America, the most common
etiology pericarditis is viral or post-viral, while in other parts of the world it is commonly
associated with tuberculosis. Pericarditis can also result from cardiac surgery, autoimmune
disease, and malignancy or associated radiation.
The underlying pathophysiology of pericarditis is inflammation of the pericardium that may or

may not be associated with pericardial effusion and/or inflammation of the myocardium the
myocardium (myocarditis). In the setting of pericardial effusion, the fluid volume can be
sufficient enough to result in tamponade physiology. Cardiac tamponade presents as cardiogenic
shock secondary to the heart being unable to fill sufficiently for adequate cardiac output. Pulsus
paradoxus and neck vein distention can also be seen. Other examination findings consistent with
pericarditis include tachycardia, a rub, and a feeling of apprehension or discomfort upon lying
down. It is quite common for those with pericarditis to have completely normal physical
examination findings.
Diagnostic Workup for suspected pericarditis includes laboratory evaluation, chest radiography,
electrocardiography, and echocardiography. Laboratory evaluation may reveal elevation of acute
phase reactant levels, including white blood cell count, sedimentation rate, and C-reactive
protein. About one-third of patients with pericarditis will have an elevated troponin level
secondary to inflammation of the underlying myocardium (i.e., myocarditis). Chest radiography
is generally normal but may demonstrate cardiomegaly of a mediastinal mass.
Electrocardiography may reveal diffuse ST segment elevation in the precordial leads and PR
interval depression (Item C190). It is important to differentiate this pattern from early
repolarization, a benign finding, and coronary ischemia. With coronary ischemia, the
ST segment changes should follow a coronary distribution (i.e., the left anterior descending
[LAD] coronary artery is anatomically anterior, and so the anterior ECG leads will show signs of
ischemia in a LAD myocardial infarction) as compared to pericarditis when these changes should
be diffuse. An echocardiogram obtained to assess myocardial function and look for pericardial
effusion.
Nonsteroidal anti-inflammatory drugs are the mainstay of therapy for pericarditis. There is some
data that suggests colchicine can be helpful to minimize recurrence. Corticosteroids have been
shown to increase the risk of recurrence. Those with significant pericardial fluid should undergo
pericardiocentesis and the fluid can be sent for infectious, inflammatory, or neoplastic
evaluation. For children with pericarditis, activity restrictions are needed for several months.
Item C190: ECG Changes in Acute Pericarditis
PREP Pearls
• Chest pain caused by pericarditis is sharp, substernal, exacerbated with deep inspiration
and laying down, and relieved by sitting up.
• The diagnosis of pericarditis made by history, physical examination, and
electrocardiographic findings of diffuse ST segment elevation and PR segment
depression.
• Treatment of pericarditis includes nonsteroidal anti-inflammatory drugs and activity
restriction.

• Recognize the clinical findings associated with pericarditis and plan appropriate initial
management
• Understand the natural history of pericarditis
• Plan the appropriate diagnostic evaluation of pericarditis
Suggested Readings
• In Brief. Pericarditis. Pediatr Rev, doi:10.1542/pir.31-2-83
• Management of acute and recurrent pericarditis. J Am Coll Cadiol,
doi:10.1016/j.jacc.2019.11.021
• Congenital and acquired heart. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed;2017;1883-1917
• Acute myocarditis and pericarditis in children. Pediatr Rev, doi:10.1542/pir.2018-0044

Question 191
The mother of a 13-year-old adolescent boy is concerned about a rumor that some students his
middle school have been using alcohol. She has discussed the rumor with her son, and he assured
her that he has never used alcohol, tobacco, marijuana, or any other drug, and he does not have
friends who use drugs. The mother has discussed with her son the use of drugs in their
neighborhood and used the rumor as an opportunity to re-emphasize her anti-substance abuse
stance. She is particularly worried because the boys have a history of problems associated with
alcohol use. The boy’s parents divorced when he was 18 months old and the father has
subsequently not been involved in his son's life. The boy has no siblings. The mother is
employed as a secretary for a construction firm and has no history of substance use. The boy has
had no behavioral problems, got good grades. and attended an after-school program associated
with his school. He enjoys playing on a community soccer team, and his mother enjoys
accompanying him to weekly practices and games. The importance of the mothers consistent and
clear anti-substance use message is affirmed, and continued involvement in her son's
extracurricular activities is encouraged.
Of the following, the BEST additional advice for this mother is for her to
A. discuss rules in the context of safety and responsibility
B. enroll her son in a faith-based school system
C. find a community-based mentor for her son
D. keep her son's schedule full with adult-directed activities

Correct Answer: A
The boy in the vignette has several risk factors for substance use that include a family history of
substance use os well as living in a neighborhood and attending a school where students have
been reported to use substances. His mother has appropriately delivered a consistent and clear
anti-substance use message. She has made arrangements for after-school supervision and has
been engaged in son's soccer activities. These are examples of parenting efforts that promote
family and social connectedness, protective factors against substance use/abuse.
Discussion with the mother should support an authoritative (balanced) parenting style.
Authoritative parents listen and engage the adolescent in the process of assessing choices and
making decisions. Behavioral expectations are clear and consistently communicated with
appropriate consequences and effective monitoring. Rule setting is best discussed in the context
of safety and responsibility. An authoritative parenting style is associated with better behavioral
outcomes lower risk for adolescent substance use/abuse than permissive or authoritarian
patenting styles. Authoritarian (high control) parents may have clear rules; however, they are
unlikely to be open to questioning of their parental authority. This type parenting promotes “do
as I say because I say” and does not foster adolescent decision-making.
The influence of peer pressure in promoting adolescent risk taking should be considered when
setting rules. Having friends who are involved in substance use increases the risk of substance
use/abuse. The likelihood of substance use is further increased by being in the presence of other
substance-using adolescents.
School connectedness is a protective factor against substance use. The characteristics of the
school environment rather than the type of school (e.g., public faith based, private, or magnet)
are important in promoting school connectedness. School connectedness can be promoted by:
• Establishing and enacting positive policies for conflict resolution and the safety of all
students, including underrepresented minorities
• Offering opportunities and recognition for diverse talents/skills
• Providing services that address the social, academic, and health and mental health care
needs of the student body
Having a community mentor, whether informal or part of a formal program, may or may not be
associated with improved behavioral outcomes including substance use. There is wide variation
in the components and oversight of mentorship programs. The effectiveness of mentorship has
been mixed and appears to wane over time. Advice about the potential benefits of a mentor is
best accompanied with information about reputable mentorship programs.
Extracurricular activities, particularly those that promote self-esteem, can also decrease the risk
of substance use. However, a full schedule of adult directed activities may be a form of control
reflective of en authoritarian style parenting. Time for self-directed activities provides
opportunities exploration of one’s identity and for problem solving and decision-making. Other
individual, family, and community factors protective against substance use/abuse include good
social skills, high self-esteem, spirituality, communities with a low tolerance and limited access
to drugs, and strategic/effective prevention programs.

The boy in the vignette did not have in utero exposure to maternal substance use. However,
prenatal exposure is associated with an increased risk for substance use and addiction. He also
gets good grades. Academic failure in late elementary school has been associated with an
increased risk for early substance use. The earlier the age for initiation of substance use, the
greater is the risk of developing a substance use disorder.
PREP Pearls
• Authoritative (balanced) parenting style is associated with better behavioral outcomes
including lower risk of adolescent substance use/abuse.
• Rules for adolescents should be established in the context of safety, responsibility, and
peer pressure.
• Key protective factors against substance abuse include authoritative parenting style,
individual attributes (e.g., high self-esteem, good social/problem solving skills,
spirituality), school connectedness. communities with low tolerance for and limited
access to drugs, and effective prevention programs.

• Recognize the influence of peer groups on substance use/abuse
• Identify factors protective against substance use/abuse
• Recognize the association between early academic failure and substance use/abuse
Suggested Readings
• Substance use disorders. American Academy of Pediatrics Textbook of Pediatric Care.
2nd ed;2016; 2690-2694
• Promoting balanced parenting: Warmth, boundaries, and effective monitoring. Reaching
teens. 2nd ed; 2020:417-424
• Evidence based prevention for adolescent substance use. Child Adolesc Psychiatric Clin
North Am, doi:10.1016/j.chc.2016.03.001
• Adolescents and substance use. Reaching Teens. 2nd ed, 2020:489-504.
• Long-term effects of the communities that care trial on substance use, antisocial behavior,
and violence through age 21years. Am J Public Health. doi:10.2105/AJPH.2018.304320
• University of Washington Center for Communities that Care.
https://www.communitiesthatcare.net

Question 192
A previously healthy, fully vaccinated, 2-year-old boy is seen at an urgent care for a painful,
erythematous eye. His mother noticed redness around his left eye last night before bed; this
morning the eye was redder, moto swollen, and had become painful. He has had congestion over
the past 5 days. There is no history of trauma.
On physical examination. the boy has a temperature of 37.9 º C, heart rate of 120 beats/min,
respiratory rate of 25 breaths/min, and blood pressure of 95/60 mm Hg. His left conjunctiva and
eyelid are erythematous and swollen. He appears to have pain when opening his eye. There is no
proptosis, extraocular movements are intact, and the pupil is normally responsive. The right eye
appears normal and has a normal pupillary reflex. He has thick, green nasal secretions. The
remainder of his examination findings are normal.

Platelet count 500 x 103 /µL (500 x 109/L)
C-reactive protein 45 mg/dL (450 mg/L)
Of the following, the BEST medication to administer to this child is oral

A. amoxicillin-clavulanic acid
B. cephalexin
C. diphenhydramine
D. methylprednisolone

Correct Answer: A
In this vignette, the child's history and physical examination findings are with consistent with
complicated rhinosinusitis with periorbital cellulitis. He should be treated oral antibiotic that
covers Streptococcus pneumoniae and Hemophilus influenzae. Cephalexin does not have
adequate coverage. If there were bilateral eye swelling without other infectious signs or
symptoms, an allergic reaction should be considered and might be treated with medications such
as diphenhydramine or methylprednisolone.
Red, painful eyes are a common complaint, and the differential diagnosis is wide. Ocular causes
of eye pain and redness include foreign body, corneal abrasion, cellulitis, conjunctivitis, trauma.
and uveitis. If the history suggests a foreign body or other corneal trauma, evaluation for a
corneal abrasion should be performed using fluorescein and a blue light or, if available, slit-lamp
examination. Corneal abrasions can become ulcerated if not treated.
In the case of cellulitis around the eye, it is important to determine whether the infection is
periorbital or orbital. Periorbital cellulitis, which is more commonly seem, involves the soft
tissue surrounding the eye. It can be treated with oral antibiotics if the child is otherwise well
appearing. Orbital cellulitis requires hospitalization and urgent consultation with otolaryngology
and/or ophthalmology; it can lead to vision loss if not addressed quickly. Clinical findings
suggestive of orbital involvement include proptosis, blurred vision, ophthalmoplegia, and
conjunctival chemosis. Computerized tomography can distinguish between these diagnoses if the
clinical examination findings cannot.
Systemic causes eye pain and redness should be considered, especially when findings are
bilateral. The differential diagnosis in such cases includes juvenile idiopathic arthritis, Kawasaki
disease, and other vasculitides, Stevens-Johnson syndrome, vitamin A deficiency, and viral
exanthems. Many inflammatory diseases will cause eye redness and pain without drainage (e.g.,
juvenile idiopathic arthritis and Kawasaki disease). Commonly used medications that can lead to
eye irritation include antihistamines, anticholinergics, decongestants, and opioids.
PREP Pearls
• Symptoms and signs suggestive of orbital cellulitis in a child with a red. painful eye may
include proptosis, blurred vision, ophthalmoplegia, or conjunctival chemosis.
• Orbital cellulitis requires hospitalization and urgent consultation with ophthalmology
and/or otolaryngology
• Systemic causes should be considered when a child has bilateral eye pain and redness

• Plan the appropriate clinical evaluation of a painful erythematous eye

Suggested Readings
• Eye pain in children. Pediatr Rev. doi:10.1542/pir.2015-0096
• Periorbital and orbital cellulitis. Pediatr Rev. doi:10.1542/pir.31-6-242
• Preseptal and orbital cellulitis. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017:2537-2543

Question 193
A 3-year-old girl is brought to the clinic for evaluation of vaginal “itching.” Her parents have
observed the girl putting her stuffed animals between her legs while lying face down and moving
back and forth on top or it while she is in bed before falling asleep or after waking up. She is
otherwise healthy, and her growth and development are normal. she attends preschool, and her
teachers report similar behaviors during nap time. physical examination findings are normal.
Of the following, the next BEST step in management of this child is to

A. evaluate the child for sexual abuse
B. reassure the child’s parents about this behavior
C. recommend disciplining the child to limit these episodes
D. treat the child for vulvovaginitis

Correct Answer: B
Masturbation, or self-exploration, is a common childhood behavior and a normal part of
development. It involves the self-stimulation of genitalia and may be concerning to parents.
Masturbation in prepubescent children frequently presents as: stereotyped posturing of the legs
and/or mechanical pressure on the suprapubic or perineal area associated with grunting, facial
flushing and sweating, and irregular breathing. The episodes may be variable in duration and
frequency, cease with distraction, and cause no alteration of consciousness.
These episodes are often mistaken for movement disorders, seizures, abdominal pain, or vaginal
pain. The parents in the vignette should be reassured that her behavior is normal and can be
ignored when it occurs only in a private setting. They should guide her to understand that
masturbation should be limited to private settings. Parents should not ignore sexual behaviors
that occur in public settings, as it will reinforce the belief that masturbation is acceptable in
public. Parents should avoid punishing the child for normal sexual behaviors. Behavioral
modification and redirection may help limit these behaviors to private settings.
Based on developmental level and age, children progress through appropriate sexual behaviors.
Masturbation may develop in infancy and increase through early childhood, peaking at year 5
and then decreasing as a child understands and adapts in social norms. Private masturbation
continues through adolescence when it may increase particularly in boys. It is imperative for
pediatricians to differentiate between normal and abnormal sexual behaviors (Item C193),
particularly because sexual behaviors may be a red flag for physical and/or sexual abuse or
neglect. According to the American Pediatrics Clinical Report on evaluating “developmentally
inappropriate, intrusive, or abusive” (Kellogg ND). The girl in the vignette is not displaying
uncommon or rarely normal sexual behaviors, so she does not need an evaluation for sexual
abuse.
Item C193: Examples of Sexual Behaviors in Children 2 to 6 Years of Age

PREP Pearls
• Masturbation, or self-stimulation of the genitalia, is a common childhood behavior and a
normal part of development.
• Behavioral modification and redirection may help limit normal sexual behaviors to
private settings
• Parents should avoid punishing the child for normal sexual behaviors,

• Understand the natural history of self-exploration and masturbation
Suggested Readings
• The evaluation of sexual behaviors in children. Pediatrics, doi:10.1542/peds.2009-1692
• Practical approach to childhood masturbation- a review. Eur J Pediatrics,
doi:10.1007/s00431-008-0766-2
• Masturbation. Pediatr Rev, doi:10.1542/pir33-4-190

Question 194
A full-term newborn is seen in the nursery for dysmorphic facial features, The pregnancy and
delivery were uneventful. Physical examination shows a hypotonic, alert male with normal vital
signs. Dysmorphic features include a depressed nasal bridge, up slanted palpebral fissures,
epicanthal folds, excess nuchal fold, bilateral single transverse palmar crease, and an increased
gap between first and second toes bilaterally. Echocardiography shows an atrioventricular canal.
Of the following, the BEST test to confirm the neonate's diagnosis is

A. chromosome analysis
B. chromosome microarray analysis
C. single gene analysis
D. whole exome sequencing

Correct Answer: A
The neonate described in the vignette has Down syndrome or trisomy 21. It is the most common
chromosomal disorder, with an incidence of 1 in 800 births across the world. Chromosome
analysis is the best test to confirm the diagnosis for any aneuploidy, including trisomy 21.
Clinical features of trisomy 21 are seen in Item C194.
Item C194: Clinical features of trisomy 21
The majority (~95%) of cases of trisomy 21 are due to a free-standing extra chromosome 21,
primarily resulting from maternal meiotic nondisjunction (95% cases). Recurrence risk (RR) for
parents to have another child with a free-standing extra chromosome 21 would be 1% above the
age-adjusted risk of the mother at the time of a subsequent pregnancy. In around 3% to 4% of
cases, the extra chromosome 21 arises because of an unbalanced translocation between
chromosome 21 and another acrocentric chromosome (13, 14, 15, 21, or 22) resulting in 2
normal copies of chromosome 21 and one extra copy translocated onto another chromosome. In
the case of a translocation, it is important to obtain parental chromosome analysis to determine if
either of them has a balanced translocation. If neither parent carries a balanced translocation, the
RR is the same as that of a free-standing chromosome 21 trisomy. If either parent carries a
balanced translocation, the RR would be dependent on the sex of the parent: 10% to 15% if the
mother is the carrier versus 2% to 5% if the father is the carrier. A chromosome analysis for the

infant not only confirms the diagnosis, it also provides information regarding the recurrence risk
estimate for the parents.
Evaluation of a neonate diagnosed with trisomy 21 includes 2-dimensional echocardiography,

ophthalmological evaluation, free thyroxine and thyroid-stimulating hormone levels, and hearing
evaluation. The American academy of Pediatrics has published life-long and age-dependent
screening guidelines for children with trisomy 21.
A chromosomal microarray is not the appropriate test for diagnosis of trisomy 21 as it cannot
detect chromosomal rearrangement, such as translocation. Single gene analysis is used to test for
known single gene disorders such as cystic fibrosis. Whole exome sequencing is used to
sequence and analyze regions of all genes. Single gene analysis and whole exome sequencing
would not be able to test for and detect an entire extra chromosome as seen in trisomy 21.
PREP Pearls
• Chromosome analysis is the appropriate test to diagnose trisomy 21 and other
aneuploidies.
• Trisomy 21 most often results from a free-standing extra chromosome 21 due to maternal
meiotic nondisjunction.
• Trisomy 21 may result from an unbalanced translocation; this may be inherited or occur
spontaneously.

• Recognize the clinical features associated with trisomy 21
• Plan the diagnostic evaluation of a patient with trisomy 21
Suggested Readings
• Down syndrome. N Engl J Med, doi:10.1056/NEJMra1706537
• Down syndrome: Managing the child and family. American Academy of Pediatrics
• Health supervision for children with Down syndrome. Pediatrics, doi:10.1542/peds.2011-
1605
• Practice guidelines for communicating a prenatal or postnatal diagnosis of Down
syndrome. J Genet Couns, doi:10.1007/s10897-011-9375-8

Question 195
A 5-year-old boy has an intensely pruritic rash on his lower legs and feet that has been present
for several weeks. It has waxed and waned but has never completely disappeared. He has some
areas of excoriation and hyperpigmentation where old lesions have healed (Item Q195). They
seem to come in crops of new lesions every few days. He has been playing outside without
shoes in his yard, which has both sand and grass. He takes no medications, has no known
allergies, and is otherwise well. There is no family history of allergy or significant systemic
disease, He will begin kindergarten in the fall but is not currently in school or daycare.
Of the following, the MOST likely etiology for this boy’s rash is
A. contact dermatitis from grassy
B. hypersensitivity response to insect bites
C. subclinical infection with group A Streptococcus
D. systemic viral infection

Correct Answer: B
The boy in the vignette has papular urticaria, a pruritic and often chronic skin eruption related to
hypersensitivity to insect bites, most often fleas, mosquitoes, or bedbugs. The lesions are papular
and sometimes bullous; they appear in various stages of eruption and resolution over many days
or weeks. Lesions are often bilateral, symmetric, and grouped in patches on exposed skin. The
groin, palms, and soles are usually spared.
Predisposed children have hypersensitivity to insect bites in several stages that evolve over
months or years:
• Induction of hypersensitivity with acquisition of early isolated insect bites; then bites
with only delayed skin reactions are seen
• immediate bite reactions followed by delayed reactions
• immediate reactions only
• No reactivity
The second and third stages are the most symptomatic. The dermatologists Hernandez and Cohen
defined a set of principles to guide the clinical diagnosis of papular urticaria using the mnemonic
SCRATCH (Item C195A).
The first step in management of papular urticaria is recognition of the etiology, followed by
symptomatic treatment with topical corticosteroids and/or oral antihistamines. Often a “3-P”
approach is helpful:
• Prevention of bites by wearing protective clothing and using insect repellants
• Pruritus control
• Patience, understanding that the problem will be recurrent but will eventually self-resolve
Contact dermatitis most often manifests on exposed skin. It is typically unilateral or asymmetric.
Lesions tend to be linear and streaked rather than grouped, fairly uniform in evolution, with an
erythematous base and superimposed papules and crusting (Item C195B). Contact dermatitis
caused by rubber or elastic may appear at the waist or bands of clothing. Nickel dermatitis
commonly occurs under clothing snaps or belt buckles.

Item C195B: Contact dermatitis
Systemic infections with group A Streptococcus or viral infections may present with urticaria
(Item C195C). Urticaria can take many forms and be localized or diffuse.
Item C195C: Urticaria associated with acute group A beta-hemolytic streptococci infection
Individual lesions may be serpiginous and cover large areas or may be small and uniform.
Urticaria is usually intensely pruritic; treatment is most often symptomatic with antihistamines
There are many causes of acute urticaria, including adverse drug or food reactions, contact
reactions, and infections. Viral illnesses are more commonly associated with nonpruritic macular
or papular eruptions on the trunk known as viral exanthems. These requires no treatment; they
are often confused with allergic responses when antibiotics are used to treat viral infections.
Chronic urticaria, occurring and persisting for 6 weeks or more, is most often idiopathic.

PREP Pearls
• Papular urticaria is a hypersensitivity reaction to insect bites
• Papular urticaria occurs on exposed areas; it is usually symmetrically distributed as
groups or lesions in various stages of acuity or resolution.
• The treatment or papular urticaria is symptomatic; it will self-resolve over days to weeks

• Know the cause of papular urticaria
• Recognize the clinical manifestations of papular urticaria
Suggested Readings
• Insect bite-induced hypersensitivity and the SCRATCH principles a new approach to
papular urticaria. Pediatrics, doi:10.1542/peds.2005-2550
• Anaphylaxis, urticaria and angioedema. Pediatr Rev, doi:10.1542/pir.34-6-247
• Insect bites and infestations. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2016:2203-2207

Question 196
A 2-year-old boy is evaluated in the office for nose bleeds that occurred about once a week over
the past several months, each lasting about 10 minutes. He had an upper respiratory illness 2
weeks ago. His medical history is remarkable for 4 episodes of acute otitis media and an episode
of pneumonia. On physical examination he has petechial rash on his chest, and flexural eczema.
The reminder of the physical examination findings are unremarkable. After reviewing his
laboratory results, the child is referred to a hematologist.

Hemoglobin 11.0 g/dl (110 g/L)
Platelet count 75 x 103 /µL (75 x 109/L)
Mean platelet volume 6 fL
Prothrombin time 12 s
Partial thromboplastin time 36 s
Of the following, the BEST next step in the boy's evaluation is referral to a(an)
A. Allergist/Immunologist
B. infectious disease specialist
C. nephrologist
D. Otolaryngologist

Correct Answer: A
The child in the vignette has bleeding (petechiae, epistaxis), moderate thrombocytopenia
(50,000-100,000 / µL) with small platelets (mean platelet volume 6 fl.), and eczema. He has had
multiple episodes of acute otitis media and an episode of pneumonia. The triad of
thrombocytopenia, eczema (atopic dermatitis), infections suggest that he has Wiskott-Aldrich
syndrome (WAS), an X-linked recessive disorder. This triad is seen in around one-third of
patients with WAS. The diagnosis should be confirmed with genetic testing.
Children with WAS should have a thorough interrogation of their immune system by an
immunologist. The immune deficiency associated with WAS is of varying severity, with an
increased susceptibility to viral, bacterial, and fungal infections. The treatment of WAS ranges
from supportive care to hematopoietic stem cell transplantation. While the boy in the vignette’s
recent history of upper respiratory symptoms makes immune thrombocytopenia a diagnostic
consideration, he does not have any large platelets. Temporary viral suppression should also be
included in the differential diagnosis; however, platelets are expected to be of normal size that
setting.
Despite the boy's history of recurrent acute otitis media, there is no immediate indication for
evaluation by otolaryngology. There is no immediate role for a nephrologist in the evaluation of
a child with WAS. An infectious disease specialist is not needed at this time, as there is no
evidence of an inability to clear any of his infections.
PREP Pearls
• The triad of thrombocytopenia, eczema (atopic dermatitis), and infections suggests a
diagnosis of Wiskott-Aldrich syndrome, an X-linked recessive disorders.
• Wiskott-Aldrich syndrome treatment requires a multidisciplinary team including an
immunologist, hematologist, and transplant team.
• Immune thrombocytopenic purpura is a diagnosis of exclusion for a child birth with a
recent viral illness and thrombocytopenia; large platelets are a hallmark of the condition
MOCA-Peds Objective

• Recognize the clinical findings associated with Wiskott-Aldrich syndrome
Suggested Readings
• Wiskott-Aldrich syndrome: Diagnosis, current management, and emerging treatments.
Appl Clin Genet, doi:102147/TACG.S58444
• Petechiae and purpura. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed; 2017:1525-1528
• A multi-institutional survey on Wiskott-Aldrich syndrome. J Pediatr, doi:10.1016/s0022-
3476(05)82002-5

Question 197
A 16-year-old adolescent girl is seen in the emergency department for a headache. She has a 4-
year history of migraine headaches. She says this headache is different, describing it as bilateral
persistent, pounding pain that wraps around her head; it is worse in the morning and improves
over the course of the day. Over the past week she has woken up twice in the middle of the night
because of the pain and vomited. Appropriately dosed nonsteroidal anti-inflammatory
medications provide temporary relief. She also describes blurry vision that fluctuates throughout
the day irrespective of the headache. Her physical examination findings are significant for
bilateral papilledema with restricted visual fields and reduced acuity. The remainder of her
examination findings are unremarkable. Magnetic resonance imaging of the brain and magnetic
resonance venography findings are normal.
Of the following. the BEST next step in this girl’s management is

A. administration of intravenous fluids and a nonsteroidal anti-inflammatory drug
B. administration of a triptan and an anti-emetic medication
C. lumbar puncture with opening pressure measurement
D. urgent referral to ophthalmologist

Correct Answer: C
The adolescent in the vignette has a headache accompanied by signs and symptoms suggestive of
increased intracranial pressure (ICP). Her history has several red flags including early morning
headache, a change from her baseline headache pattern, positional worsening, and acute vision
changes. Of the response choices, lumbar puncture with opening pressure measurement is the
best next step her management.
Fundoscopic examination is critical in the evaluation of headache, especially when red flags are
present in the history. Swelling of the optic disc (papilledema) with accompanying changes in
visual acuity and visual fiefs warrants urgent evaluation with neuroimaging to evaluate for a
structural (e.g., space occupying lesion, hydrocephalus) or vascular etiology (venous sinus
thrombosis) of increased ICP. If neuroimaging fails to identify a cause, lumbar puncture with
opening pressure measurement should be performed to evaluate for primary intracranial
hypertension. This procedure can have both a diagnostic and therapeutic benefit. Although
intravenous fluids and pain medications may provide temporary relief, lumbar puncture is
needed for diagnosis and will likely provide similar relief. Ophthalmology consultation is
indicated to follow the papilledema over time.
Pediatric intracranial hypertension is classified into 2 subtypes: primary and secondary. Primary
intracranial hypertension (previously termed idiopathic intracranial hypertension or pseudotumor
cerebri) is defined as increased ICP without a clear cause. Risk factors include obesity, post
pubertal status in females, and polycystic ovarian syndrome. Secondary intracranial hypertension
refers to increased ICP with an identifiable cause. Secondary causes include intracranial
pathology, an underlying medical condition, or medication (Item C197A).
Item C197A: Causes of Increased Intracranial Pressure in Children

• HEAD TRAUMA
• Cerebral edema
• Intracerebral hemorrhage
• Extracerebral hemorrhage (subdural, epidural)
• VASCULAR CAUSES
• Arterial or venous infarctions
• Intracerebral hemorrhage
• Dural sinus thrombosis
• Subarachnoid hemorrhage
• Vascular anomalies (vein of Galen malformation,
• arteriovenous malformations)
• NEOPLASTIC CAUSES
• Primary brain tumors
• Metastatic (intracerebral, meningeal infiltration)
• Hydrocephalus (congenital or acquired,
• communicating or noncommunicating)
• Pseudotumor cerebri (benign intracranial
• hypertension)
• Central nervous system infections
• Meningitis (bacterial, fungal, mycobacterial)
• Encephalitis (focal or diffuse)
• Abscess
• METABOLIC CAUSES
• Inborn errors of metabolism (hyperammonemia)
• Hepatic encephalopathy
• Diabetic ketoacidosis
• Renal failure
• Reye syndrome
• Status epilepticus
• Hypoxic-ischemic encephalopathy
• Fluid-electrolyte abnormalities (hyponatremia,
• hypernatremia)
• STRUCTURAL CAUSES
• Craniosynostosis
The clinical presentation of intracranial hypertension is variable. Papilledema may be identified

on routine ophthalmological examination in asymptomatic children. Headache, usually described
as constant and worse in the morning. is the most common symptom and may be exacerbated by
Valsalva maneuvers, lying flat, or bending over. Vision symptoms include transient visual
obscurations, diplopia if cranial nerve VI palsies are present, and pulsatile tinnitus.
Careful examination of the eye is critical for the diagnosis of intracranial hypertension.
Fundoscopy (Item C197B) should be performed to evaluate for the presence of papilledema.
which is usually bilateral as noted in Item C197C. Unilateral papilledema warrants further
evaluation for alternate causes (such as optic neuritis). Visual field testing (Item C197D) should
be performed to evaluate for an enlarged blind spot or restricted visual fields. Visual acuity
testing should be performed; acuity is usually preserved unless severe edema is present.
Extraocular movements should be tested (Item C197E), in particular looking for cranial nerve
VI palsies (inability to abduct the eye), which can cause diplopia.
Diagnostic workup is aimed at determining if the increased ICP is primary or secondary. A

careful past medical and medication history (including over the counter medications) should be
taken. Magnetic resonance imaging of the brain and magnetic resonance venography should be
performed to evaluate for structural and vascular pathology. If craniosynostosis is suspected,
head computed tomography with 3D reconstruction is recommended.
If imaging is normal and no contraindications are present, lumbar puncture with opening
pressure measurement should be performed to determine the intracranial pressure. Normative
values for cerebrospinal fluid (CSF) opening pressure can vary. One pediatric cohort study
reported a mean opening pressure of 19.8 cm H2O with a 90th percentile value of 28 cm H2O in a
healthy cohort. Obesity, demyelination, and sedation may influence these values. In the setting of
intracranial hypertension, however, the opening pressure values are unequivocally high.
The treatment of intracranial hypertension includes removing the causative agent(s) if present
and medically appropriate to do so. Acetazolamide, the first-line medication used to lower ICP,
decreases CSF production. Furosemide and topiramate can be used for those unable to tolerate
acetazolamide. In severe or rapidly progressing cases, optic nerve fenestration or CSF diversion
with shunt can be performed.

Item C197B Item C197C: Optic nerve edema stages. Left to right. First row: Normal (0), stage 1. Second row:
stage 2, stage 3 (note cotton wool spot with hemorrhage at 5 o’clock). Third row: stage 4 (note hemorrhages 1-2
and 6-8 o’clock, retinal striae due to severity of edema), stage 5
Item C197D: enlarged blind spot

Item C197E: Left lateral rectus palsy as demonstrated by the patient’s inability to abduct her left eye
PREP Pearls
• Papilledema is a sign of increased intracranial pressure, can be identified on routine
ophthalmologic examination or in children presenting with headaches, pulsatile tinnitus,
or vision changes
• When increased intracranial pressure is suspected, neuroimaging should be performed.
including venous vascular imaging, followed by a lumbar puncture with opening pressure
measurement if imaging is normal
• Treatment of intracranial hypertension includes removing any inciting agent, and
administering a medication that decreases CSF production such as acetzolamide

• Recognize the clinical findings associated with papilledema
Suggested Readings
• Reference Range for cerebrospinal fluid opening pressure. N Engl J Med,
doi:10.1056/NEJMc1004957
• Pediatric intracranial hypertension. Pediatr Neurol, doi:
10.1016/j.pediatrneurol.2016.08.010
• Lumbar puncture. N Engl J Med, doi:10.1056/NEJMvcm054952
• Increased Intracranial pressure. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed. 2888-2894

Question 198
A 9-month-old infant is being evaluated for “spots” on her skin. A few spots were present at
birth, and the number has been increasing. She is otherwise healthy. The girl is the only child.
Her mother is unaware of family members with similar spots. Physical examination findings are
remarkable only for the lesions shown in Item 0198A and Item 198B
Of the following, the BEST next step in management for this infant is
A. magnetic resonance imaging of the brain
B. no further evaluation
C. referral for developmental assessment
D. referral for ophthalmology examination

Correct Answer: D
The infant in the vignette has multiple café-au-lait macules (CALMS). The presence of 6 or
more macules measuring >5 mm in a child is one of the diagnostic criteria for neurofibromatosis
type 1 (NF1). An ophthalmology examination is warranted to evaluate for iris hamartomas
(Lisch nodules) and optic glioma. If present, these findings would meet criteria for diagnosis of
NF1. If she does have NF1, the girl would be at increased risk for central nervous system tumors;
however, imaging is not indicated in the absence of concerning symptoms or signs. While
learning disabilities are common in children, who have NF1 referral to a developmental
specialist is not warranted at this age without specific developmental concerns.
Neurofibromatosis type 1 is a multisystem disease that primarily affects the skin and central
nervous system. It is inherited in an autosomal dominant fashion, but half of all cases result from
a de novo mutation in NF1. Two or more of the following criteria are required to make the
diagnosis:
• Six or more CALMs measuring >5 mm in a prepubertal individual or 15 mm in a post
pubertal individual
o Multiple CALMs are present on nearly all individuals with NF1; they often are
present at birth and increase in number during the first several years of childhood
o Frequently the first sign of NF1
o Most children with 6 or more café-au-lait macules will ultimately be diagnosed
with NF1
• Axillary or inguinal freckling often appears in children aged 5 years or older (Item
C198A)
• Optic glioma occurs in approximately 15% of children, usually before age 6 years; it may
cause reduced visual acuity or proptosis.
• Two or more iris hamartomas (Lisch nodules, Item C198B)
o Detected on slit-lamp examination
o Present in 25% of patients with NF1 by age 5 years, 50% by age 10 years, and
95% by age 20 years
• Two or more neurofibromas (Item C198C) or 1 or more plexiform neurofibroma (Item
C198D)
o Neurofibromas are common in adults but rare before late childhood.
o Plexiform neurofibromas occur in about 50% of individuals with NF1
• Characteristic osseous lesion (e.g., sphenoid wing dysplasia, anterolateral bowing of the
tibia and fibula) (Item C198E)
• First degree relative who has NF1
Other important clinical features of NF1 include:

• Neurocognitive deficit:
• The most commonly reported complications of NF1
• Include specific learning disabilities, attention-deficit/hyperactivity disorder,
autism spectrum disorder, and behavioral difficulties
• Musculoskeletal abnormalities:
• Increased risk for scoliosis
• Hypertension:

• Common in NF1
• May develop at any age
• Usually, primary.
• May result from renal artery stenosis or coarctation of the aorta
Many disorders are characterized by multiple CALMs but rarely are these confused with NF1,
Three disorders that may mimic NF1 include.
• Legius syndrome: dominant inheritance, multiple CALMs, axillary freckling, and
macrocephaly, without other signs of NF1
• Familial CALMs: without other signs or NF1
• McCune-Albright syndrome: CALMs have more jagged borders and are unilateral;
fibrous dysplasia of the bone; precocious puberty in girls; and other endocrine disorders.
If a child has 6 or more CALMs without other features of NF1, an annual ophthalmology
evaluation and monitoring for the appearance of the other features of NF1 are indicated. If a
child meets 2 or more diagnostic criteria (i.e., meets the definition of NF1) then the following are
indicated: medical genetics consultation, annual pediatric ophthalmology evaluation, regular
developmental assessment and regular blood pressure monitoring.
Item C198A: Axillary freckling
Item C198B Lisch nodules

Item C198C: Neurofibroma
Item C198D: plexiform neurofibroma
Item C198E: Characteristic osseous lesion (anterolateral bowing of the tibia and fibula)

PREP Pearls
• Six or more CALMs measuring >5 mm in a prepubertal individual or 15 mm in a post
pubertal individual are among the diagnostic criteria of NF1
• Most children who have more than 6 café-au-lait macules will ultimately be diagnosed as
having NF1

• Recognize the clinical findings associated with neurofibromatosis in patients with various
ages
• Know that six or more café-au-lait spots > 0.5 cm in diameter suggests the diagnosis of
neurofibromatosis
Suggested Readings
Care. 2nd ed; 2017; 2379-2396.
• Neurofibromatosis I. Gene Reviews [Internet].
• Neurocutaneous disorders in children. Pediatr Rev, doi:10.1542/pir.2015-0118
• Health supervision for children with neurofibromatosis type I. Pediatrics,
doi:10.1542/peds.2019-0660
• Neurofibromatosis type I revisited. Pediatrics, doi:10.1542/peds.2007-3204

Question 199
A previously healthy, 2-year-old boy is being evaluated in November for an abrupt onset of
barking cough, voice hoarseness, and respiratory distress. Two days ago, he had developed
rhinorrhea. His immunization are up to date. On physical examination his temperature is 38 °C,
heart rate is 141 beats/min, respiratory rate 24 breaths/min, and oxygen saturation 94% in room
air. Examination of the respiratory tract reveals yellowish nasal drainage, inspiratory stridor,
mild suprasternal retractions but no cyanosis. The stridor worsens when he is upset. The
reminder of the physical examination findings are normal.
Of the following, the MOST likely pathogen causing this child’s illness is
A. Influenza virus
B. parainfluenza virus
C. respiratory syncytial virus
D. staphylococcus aureus

Correct Answer: B
The abrupt onset or barking cough and inspiratory stridor associated hoarseness of voice, low-
grade fever, and rhinorrhea in the nontoxic-appearing boy described in the vignette is highly
suggestive of croup (laryngotracheitis). Croup is a common inflammatory illness of the
subglottis in infants and preschool-aged children. Croup affects 3% to 5% of children during the
second year of age, with a peak incidence noted from age 6 months through 3 years. Croup is
more common in boys than in girls.
Parainfluenza virus (PIV) is the most common cause of croup and is implicated in 75% of cases
during seasonal PIV epidemics. Human PIVs are RNA viruses belonging to the Paramyxoviridae
family, and are classified into 4 distinct serotypes (PIV type 1, 2, 3, and 4), each with unique
age- and season-specific cyclic outbreak patterns in temperate climates. The most common
serotype is PIV type 1, and can cause large biennial mid-autumn outbreaks of croup.
Parainfluenza virus type 2 exhibits a sporadic seasonal pattern whereas PIV type 3 infections
peak during spring and summer.
Seasonal PIV infection causes 4% of all respiratory infections and 6% to 11% of hospital
admissions for lower respiratory tract infections in children aged less than 5 years. Among
children aged 2 to 6 years, croup caused by PIV type 1 is the primary reason for hospital
admission. Coinfection with other respiratory viruses is common among young children (rates up
to 40%).
Transmission of PIV occurs via direct contact with infected respiratory tract secretions via
droplets and fomite exposure. Most children acquire primary infections with multiple PIV types
by 5 years of age. The immunity from PIV infection in childhood is transient: reinfection is
common with PIV serotypes at any age and usually causes a mild illness limited to the upper
respiratory tract.
In addition to croup, PIV can cause bronchiolitis and pneumonia in young infants. An influenza-
like illness has also been associated with PIV. Upper respiratory tract infection caused by PIV is
complicated by otitis media in 30% to 50% of cases. Other unusual manifestations include
parotitis, aseptic meningitis, and encephalitis, Infection with PIV may aggravate symptoms of
asthma or chronic lung disease. In immunocompromised hosts (e.g., hematopoietic stem cell
transplant recipients). PIVs can cause severe lower respiratory tract infection complicated by
respiratory failure, disseminated disease, and death.
Croup can also be caused by other community respiratory viruses such as influenza A and B,
respiratory syncytial virus, human metapneumovirus, adenovirus, measles, coronavirus and
rhinovirus. Mycoplasma pneumoniae has also been rarely implicated. Children with croup-like
symptoms caused by influenza illness have high-grade fever. Secondary bacterial tracheitis
caused by Staphylococcus aureus occur after croup, but children often are toxic appearing with
higher temperature and severe respiratory distress.
The differential diagnosis of croup includes bacterial tracheitis, retropharyngeal or

parapharyngeal abscess, epiglottitis, foreign body aspiration, or spasmodic croup. Epiglottitis
caused by hemophilus influenza type b is very rare because of routine childhood immunization.
Children with spasmodic croup usually well-appearing without fever or preceding upper
respiratory tract complaints. The illness is transient, with a history of recurrent illness.
The diagnosis of croup is clinical, based primarily on history and physical examination. Neck
radiography may show evidence of subglottic narrowing of the trachea (steeple sign) (Item
C199). Laboratory studies ate rarefy helpful in the evaluation of croup. Reverse-transcriptase
multiplex polymerase chain reaction detect PIV and community respiratory viruses in
nasopharyngeal specimens.
Item C199: Radiograph of the posteroanterior neck of a child with viral croup, showing narrowing in the subglottic
area.
The main stay of treatment for croup is administration of oral, nebulized, or parenteral
corticosteroids. A single dose of oral dexamethasone is beneficial for children with mild croup in
the outpatient setting. Therapeutic options for children with moderate to severe croup include
dexamethasone (oral or intramuscular), nebulized epinephrine, and nebulized budesonide. In
most patients with croup, symptoms improve within 48 hours. Antibiotics are not recommended
for viral croup. No specific antiviral treatment, prophylaxis, or vaccine exists for PIV infection.
PREP Pearls
• Croup (laryngotracheitis) is the most notable clinical manifestation of human
parainfluenza virus. followed by bronchiolitis and pneumonia
• The diagnosis of croup is clinical, based on history and physical examination, abrupt
onset of seal-like barking cough and inspiratory stridor associated with hoarseness of
voice, low-grade fever. and rhinorrhea
• The mainstay of treatment for croup is administration of oral, nebulized, or parenteral
corticosteroids.

• Recognize the clinical features associated with parainfluenza virus infection
• Understand the epidemiology of parainfluenza virus
Suggested Readings
• Parainfluenza viral infections. Red Book: 2021-2024 Report of the Committee on
infectious Diseases. 32nd ed; 2021:555-556
• Croup (acute laryngotracheobronchitis). American Academy of Pediatrics Textbook of
Pediatric Care. 2nd ed; 2016; 2799-2804.
• Cold weather viruses. Pediatr Rev. doi:10.1542/pir.2018-0237

Question 200
A full-term neonate is delivered via scheduled repeat cesarean section. After warming, drying,
and stimulation, his heart rate is 80 beats/min and respiratory effort is poor. A pulse oximeter is
applied to his right hand, and positive-pressure ventilation is initiated. The neonate's chest is not
moving despite an adequate seal, suctioning of the mouth and nose, and open airway. The peak
pressure is increased from 20-25 cm H2O, and adequate chest rise is achieved.
Of the following, the MOST likely reason this neonate required resuscitation is
A. atelectatic alveoli and collapsed airways
B. failed activation of alveolar Na-K ATPase channels
C. lack of thoracic compression during vaginal delivery
D. surfactant deficiency

Correct Answer: A
The neonate in the vignette displayed poor respiratory effort and had a heart rate less than 100
beats/min after delivery, requiring resuscitation with positive pressure ventilation (PPV). This
neonate most likely required resuscitation to overcome the resistance of atelectatic alveoli and
collapsed airways.
Thoracic compression during vaginal delivery is associated with minimal clearance of alveolar
fluid; therefore, a cesarean delivery would not explain this neonate’s condition. Labor activates
epithelial Na-K ATPase channels, which contribute to the clearance of fluid from the alveoli.
This process may not occur in scheduled cesarean deliveries, often leading to transient tachypnea
of the newborn. However, the lack of activation of the Na-K ATPase channels does not explain
the need for PPV in the delivery room. Surfactant deficiency is present in many premature
neonates because of inadequate production of surfactant from type II pneumocytes. It is unlikely
to explain the resuscitation needs of this full-term neonate.
The initial steps of neonatal resuscitation are warming, drying, and stimulating the neonate. For
most neonates, these maneuvers are sufficient to initiate spontaneous respiration with adequate
chest rise and improvement in heart rate. Term neonates who display good tone and are breathing
or crying do not need additional interventions. For neonates who do not respond to these initial
resuscitation steps or have a heart rate less than 100 beats/min, PPV should be initiated. The
facemask should be positioned to cover the nose and mouth with a tight seal. The neck should be
slightly extended to prevent collapse of the hypopharynx. Care should be taken to not overextend
the neck; which may also impede airflow through the trachea. The initial positive pressure used
should be 20 cm H2O. If the heart rate does not improve in response to PPV, the pneumonic MR
SOPA, from the Neonatal Resuscitation Program, is helpful to identify potential problems:
• M—adjust the mask
• R—reposition head to open the airway
• S—suction the mouth and nose
• O—open the mouth and tilt the jaw forward
• P—increase the pressure
• A—consider en alternative airway
PREP Pearls
• For most neonates, the initial steps of warming, drying, and stimulation are sufficient to
initiate spontaneous respiration with adequate chest rise and improvement in heart rate.
• Positive-pressure ventilation should be initiated for neonates with a heart rate less than
100 beats/min or labored respiration.
• The pneumonic MR SOPA (M—adjust mask, R—reposition the head to open the airway,
S—suction the mouth and nose, O—open the mouth and tilt the Jaw forward, P—
increase the pressure, A—consider an alternative airway) can be helpful to troubleshoot
ineffective positive-pressure ventilation.
MOCA-Peds Objective

• Understand the respiratory pattern in newborn infants, recognizing that increased
pressure may be required for the first breath
Suggested Readings
• Neonatal resuscitation. Pediatr Rev, doi:10.1542/pir.2018-0203
• Assessment and stabilization at delivery. American Academy of Pediatrics Textbook of
Pediatric Care. 2nd ed; 2017;987-1001.
• Part 13: neonatal resuscitation. Circulation, doi:10.1161/CIR.0000000000000267

Question 201
A 3-year-old boy is brought to the office by his foster mother. He was placed in her care 2 weeks
ago due to parental neglect. His foster mother states that she knows little about the circumstances
of the boy’s removal from his home; she was informed that his medical examination findings at
that time were normal. At the end of today's visit, a 1 month follow-up visit is planned. The
foster mother asks how often the boy should be brought to the pediatrician for routine follow-up
visits.
Of the following, the MOST appropriate interval is every

A. 3 months
B. 4 months
C. 6 months
D. 12 months

Correct Answer: C
The American Academy of Pediatrics recommends pediatrician visits every 6 months for
children in foster care between 24 months and 21 years of age, such as the child in the vignette.
Children are placed into foster care through the child welfare system in cases of abuse and/or
neglect. Most are placed with relatives (kinship care) or a nonrelative family member. Some are
placed in residential or group care. About 7% of foster children are infants; under a third each are
1 to 5 years, 6 to 12 years, and 13 to 20 years of age.
The mission of the child welfare system is to ensure the safety and well-being of children. A
caseworker is assigned to the family and works toward their safe reunification. At the same time,
the caseworker looks for an alternative permanent placement for the child(ren) should
reunification be unsuccessful. About 20% to 25% Children in foster care are eventually adopted.
Pediatricians should consider children in foster care to have special health care needs. These
children are more likely to have developmental and educational challenges. mental health issues,
and psychosocial problems. Many have had toxic stress resulting from adverse childhood
experiences such as exposure to violence, parental substance use, and/or parental mental illness.
One-third have a chronic medical condition such as obesity or asthma.
Children entering foster care should be seen by a pediatrician within 72 hours for a complete
physical examination and within 1 month of placement for assessment of the child's health care
needs. Children in foster care may have multiple placements. As a result of these transitions,
their needs may not have been fully identified addressed, or treated in previous settings. It is
important to review all available records; use validated tools to screen for developmental and
mental health conditions; and arrange for relevant referrals, medications, equipment, and
services.
The American Academy of Pediatrics recommends that regular visits for children in foster care
occur monthly until age 6 months, every 3 months from age 6 to 24 months, and then every 6
months until age 21 years. These visits help pediatricians monitor the child's physical and
psychological health/coordinate care, and connect children to relevant specialists and services.
Pediatricians can provide a strong medical home, supporting these children by communicating
and collaborating with other members of the care team. Pediatricians can provide guidance and
education as appropriate to the situation, to the birth parents, foster parents, and caseworker
regarding the child's behavioral and mental health needs. These needs may increase during times
of transition (e.g., placement or visitation changes).
The state child welfare agency is required to develop a transition plan to prepare adolescents in
foster care for the transition to independent living. Housing, employment, education, and health
are areas addressed in this plan. Depending on the state, foster care extends to 18 or 21 years of
age. Pediatricians can monitor the adolescent's progress toward independence and can assist with
the transition to adult medical and mental health providers. Pediatricians can educate adolescents
on their specific health issues and how to access medical and mental health care.

PREP Pearls
• Children entering foster care should be evaluated by a pediatrician within 72 hours for a
complete physical examination and within 1 month of placement for assessment of the
child's health care needs.
• The American Academy of Pediatrics recommends that regular visits for children in
foster care occur monthly until age 6 months, every 3 months age 6 to 24 months, and
then every 6 months until age 21 years.
• The state child welfare agency is required to develop a transition plan to prepare
adolescents in foster care for the transition to independent living. Depending on the state,
foster care extends to 18 or 21 years of age,
ABP Content
• Plan the appropriate evaluation of children of various ages who are in the foster care
system, and manage appropriately
• Understand the basic functions of the child welfare and foster care systems and the
pediatrician’s role in that system
• Recognize the needs of youth aging out of the foster care system, and manage
appropriately
Suggested Readings
• Hearth care issues for children and adolescents in foster care and kinship care. Pediatrics.
doi:10.1542/peds.2015-2655
• Pediatrician guidance in supporting families of children who are adopted, fostered, or in
kinship care, Pediatrics. doi:10.1542/peds.2020-034629
• Foster care. Pediatr Rev. doi:10.1542/pir.2016-0076
• Children in foster or kinship care. American Academy of Pediatrics Textbook of
Pediatric Care. 2nd ed; 2017:605-620
• Foster Care. Zuckerman Parker Handbook of Developmental and Behavioral Pediatrics
for Primary Care. 4th. 2019;251-255.

Question 202
A previously healthy, 7-year-old girl is evaluated for a 1-day history of lower abdominal pain
and burning with urination. She has had increased urinary frequency and hard bowel movements
for the past 3 days. She has had no fever, vomiting, or diarrhea. The girl was toilet trained at age
3 years. She has a history of waiting for too long to urinate and occasionally has urinary
accidents at school. The girl’s temperature is 37 ºC, heart rate is 80 beats/min, respiratory rate is
14 breaths/min. and blood pressure is 100/60 mmHg. On physical examination her abdomen is
soft and nondistended with mild suprapubic tenderness. There is no costovertebral angle
tenderness. The remainder of her physical examination findings, including her external genital
examination, are normal.
Results of a spot urinalysis with microscopy are shown
Urine Test Result

pH 6.0
Nitrite Positive
Blood 1+
Protein Negative
Bacteria Present
Of the following, the girl's MOST likely diagnosis is

A. acute cystitis
B. acute pyelonephritis
C. acute vulvovaginitis
D. asymptomatic bacteriuria

Correct Answer: A
The girl in the vignette has acute cystitis resulting from dysfunctional voiding of urine. Her
symptoms (dysuria, lower abdominal pain, urinary frequency), signs (suprapubic tenderness),
associated risk factors (dysfunctional voiding, constipation), and abnormal urinalysis results
(presence of nitrites. white blood cells, and bacteria) favor this diagnosis.
Cystitis, commonly caused by an acute bacterial infection, is inflammation localized to the

bladder. Risk factors for cute cystitis include female sex, lack of circumcision in boys, sexual
activity, dysfunctional voiding, constipation, neurogenic bladder, bladder stones, indwelling
catheters, anatomic abnormalities (e.g., posterior urethral valves, vesicoureteral reflux), and
underlying medical conditions (e.g., diabetes mellitus, immunosuppression). Acute cystitis is
most commonly caused by Escherichia coli and other gram-negative organisms. Less commonly.
acute cystitis is caused by a virus (e.g., adenovirus) or fungi (e.g., Candida species).
Pseudomonas aeruginosa and Candida albicans infections commonly seen with catheter-
associated cystitis.
Acute cystitis presents with lower urinary tract symptoms including dysuria. urinary frequency,
urgency, hematuria, urinary incontinence. and lower abdominal pain. Children with acute cystitis
usually do not have systemic symptoms of fever, vomiting, or flank pain. Adenovirus cystitis is
associated with gross hematuria with clots, and there may be a history of a recent upper
respiratory infection. Physical examination may show suprapubic tenderness. A tuft of hair,
lipoma, or pigmentation at the lower spine suggests a neurogenic bladder. Genital examination
may show phimosis or labial adhesions predisposing a child to acute cystitis.
Urinalysis with microscopy in acute bacterial cystitis typically shows pyuria (i.e., positive
leukocyte esterase and white blood cells) and bacteriuria (i.e., presence of nitrites and bacteria).
Urine culture confirms the presence of a single bacteria ≥100,000 colony-forming units
(CFU)/mL from a clean catch sample or ≥50,000 CFU/mL on a sample obtained via bladder
catheterization.
Acute bacterial cystitis is treated with empiric oral antibiotics (e.g., cefixime,
cefuroxime, trimethoprim-sulfamethoxazole. or amoxicillin-clavulanic acid) pending urine
culture results. The choice of empiric oral antibiotic depends on local patterns of susceptibility
and organisms identified during any prior cystitis events; the antibiotic may be modified once
sensitivities are known. The total duration of therapy should be 3 to 7 days. Correctible risk
factors, such as dysfunctional voiding or constipation, should be addressed to prevent recurrence
of cystitis.
Acute pyelonephritis, an infection of the upper urinary tract, is a less likely diagnosis for the girl
in the vignette; this infection presents with fever and flank pain. Other signs and symptoms
associated with acute pyelonephritis include chills, vomiting, poor oral intake, and flank
tenderness, none of which is present in the girl in the vignette. Acute vulvovaginitis usually
presents with vaginal discharge, erythema, and pruritus in the genital area along with dysuria and
thus is an unlikely diagnosis for the girl in the vignette. Children with asymptomatic bacteriuria
are without any symptoms, often do not have leukocytes in the urine, and have bacteria on urine

culture. The organisms causing asymptomatic bacteriuria are usually of low virulence and
protect against overgrowth of pathogenic bacteria, so treatment with antibiotics is not indicated
PREP Pearls
• Signs and symptoms of acute cystitis may include dysuria, urinary frequency, urgency,
hematuria, urinary incontinence, and lower abdominal pain.
• Acute cystitis is not associated with systemic symptoms of fever, vomiting, or flank pain.
• Acute cystitis should be treated with oral antibiotics for 3 to 7 days.
MOCA-Peds Objective
• Evaluate and manage infants and children with acute urinary tract infection.
• Recognize and manage labial adhesions.

• Plan the appropriate clinical evaluation of cystitis
• Recognize the clinical and laboratory findings associated with cystitis plan the
appropriate initial and long-term management of cystitis
Suggested Readings
• Urinary tract infections in children: knowledge updates and a salute to the future. Pediatr
Rev. doi:10.1542/pir.2017-0007
• Urinary tract infections in children. Pediatr Rev. doi:10.1542/pir.36-4-153
• Urinary tract infections. American Academy of Pediatrics Textbook of Pediatric Care.
2nd ed; 2017; 2748-2757.

Question 203
A 6-year-old girl is being evaluated for a 4-month history of a perineal rash accompanied by
intermittent pruritus and discomfort, painful urination and defecation, and constipation. She is
otherwise healthy. She has an unremarkable medical history and normal growth at the 25th
percentile for height and weight. Physical examination findings are normal except for the
perineum (Item Q203)
Of the following, the child’s MOST likely diagnosis is

A. Irritant contact dermatitis
B. lichen sclerosus et atrophicus
C. sexual abuse
D. vulvovaginitis

Correct Answer: B
The girl in the vignette has well-defined areas of hypopigmentation and skin atrophy involving
the medial labia majora and the perianal region. The affected skin has a shiny appearance, and an
area of ecchymosis is present. These physical findings and their distribution are characteristic of
lichen sclerosus et atrophicus, also known as lichen sclerosis. Although child sexual abuse may
produce bruising, hypopigmentation and atrophy would not be present. Both irritant contact
dermatitis and vulvovaginitis may cause post inflammatory hypopigmentation, but the abnormal
pigmentation would not be so well demarcated, and bruising would be absent.
Lichen sclerosis is an uncommon chronic inflammatory disease of unknown cause that usually
affects the anogenital region. It occurs most often in adult women, but 5% to 15% of cases occur
in children, almost exclusively girls younger than 7 years. Lichen sclerosis begins as small pink
or white papules that coalesce to form plaques. Ultimately, the lesions become atrophic and
appear as shiny, ivory-colored areas of hypopigmentation in a figure-of-8 or hourglass
distribution surrounding the vulva, perineum, and anus (Item C203). The skin may be wrinkled,
and blisters (sometimes hemorrhagic) may occur. Patients may experience dysuria, painful
defecation, constipation, or bleeding. In boys, involvement usually is limited to the foreskin,
causing it to become sclerotic and difficult to retract.
Item C203
There is no cure for lichen sclerosis, but it often involutes at or before puberty. First, treatment is
usually a medium to high potency topical steroid. Many clinicians initiate therapy with an
ultrapotent (group 1) agent (e.g., clobetasol propionate or betamethasone dipropionate), tapering
the frequency of application or potency as the condition improves. Once control is achieved, the
topical steroid can be withdrawn, and maintenance therapy is initiated with topical calcineurin
inhibitor (e tacrolimus or pimecrolimus).
PREP pearls
• Lichen sclerosis is primarily a disease of adult women; 5% to 15% of cases occur in
children, almost exclusively girls younger than 7 years of age.
• Typical lichen sclerosis lesions are shiny, wrinkled, ivory-colored atrophic patches
located in a figure-of-8 or hourglass distribution surrounding the vulva, perineum, and
anus. Blisters may occur.
• Lichen sclerosis lesions may be confused with those of child sexual abuse: however,
lesions of abuse would not appear hypopigmented or atrophic.

• Recognize the clinical findings associated with lichen sclerosis, and manage
appropriately
Suggested Readings
• Lichen sclerosus et atrophicus (LSA). Pediatric Dermatology: A Quick Reference Guide.
4th ed. 2020;777-782
• Gynecologic examination of the prepubertal girl. Pediatr Rev. doi:10.1542/pir.35-3-97
• Pediatric lichen sclerosus: a review of the epidemiology and treatment options. Pediatr
Dermatol, doi:10.1111/pde.12615

Question 204
A 5-year-old boy brought to the emergency department after being hit on the head with a tennis
suture racquet. He has 3-cm superficial laceration on his eyebrow that will require suture repair.
The reminder of his physical examination findings are unremarkable. Procedural sedation is
planned with goals of providing sedation facilitate the laceration repair as well as pain control
during suturing.
Of the following, the BEST medication to use for this boy's procedure is
A. Ketamine
B. Midazolam
C. Pentobarbital
D. propofol

Correct Answer: A
The best medication to use for the boy in the vignette is ketamine, given its analgesic, sedative,
and amnestic properties. Midazolam, pentobarbital and propofol do not have intrinsic analgesic
effects. Common medications used for pediatric procedural sedation and their properties are
detailed in Item C204.
The provision of safe and effective sedation and analgesia is an important skill for pediatricians
practicing in an acute care setting. After an acute injury children are prone to anxiety and benefit
from adequate sedation and analgesia, particularly to facilitate diagnostic or painful procedures.
It is not acceptable to withhold sedation or analgesia, especially for painful procedures,
regardless of the duration or complexity of the procedure involved. As such, barring
contraindications, sedation and analgesia should be offered for children undergoing painful or
otherwise potentially distressing procedures.
Procedural sedation is defined as the delivery of sedating or dissociative medications to produce

a state of depressed consciousness, with or without analgesics. Analgesia, which is distinctly
different from sedation, is defined as the loss of sensation to painful stimuli. Although some
analgesic medications may induce sedation, pure analgesia has no effect on mental status of level
of alertness.
Anxiolysis reduces patient anxiety or apprehension without a concomitant reduction in the level
of consciousness or mental state. Sedation occurs along a continuum ranging from minimal, in
which patients can freely respond to verbal commands, to moderate, in which cognition is
impaired, to deep, in which the patient cannot be easily aroused without repeated or painful
stimuli.
Ketamine is a dissociative anesthetic. Its effects are mediated primarily by noncompetitive

antagonism at the N-methyl-D-aspartate receptor and also agonism at the µ- and κ-opioid
receptors. Ketamine is ideal for procedural sedation because it has analgesic, amnestic and
sedative properties without loss of protective airway or hemodynamic reflexes. An important
adverse effect of ketamine is the emergence phenomenon, which can manifest as disturbing
hallucinations in older children and adults; it is often mitigated by the use of low dose
midazolam
Midazolam is a short-acting benzodiazepine. Benzodiazepines modulate the γ-aminobutyric acid

(GABA) receptors. They have no analgesic activity but are anxiolytic, amnestic, and hypnotic,
and act as skeletal muscle relaxant. Benzodiazepines are a good option for moderate sedation
because their effects can be reversed with flumazenil. Because midazolam has a rapid onset and
short duration of action, it is frequently used when short-term sedation is required; the
anterograde amnesia can be a desired side effect. However, with no analgesic properties,
midazolam should not be used alone for painful procedures.
Pentobarbital is an ultra-short-acting barbiturate useful for sedation before short diagnostic

imaging procedure. Adverse reactions include those similar to all central nervous system (CNS)
depressants, such as hypoxia caused by respiratory drive depression. The cardiovascular effects
include a decrease in systemic vascular resistance with a compensatory increase in heart rate.
Barbiturates have no intrinsic analgesic activity and thus should not be used alone for procedural
sedation when analgesia is required
Propofol is effective for short-term sedation. but its use is often restricted to the operating room
or intensive care unit. Propofol is an intravenous general anesthetic that works by increasing
GABA-mediated inhibitory tone in the CNS. propofol has no analgesic properties and is often
very painful upon injection. propofol can profoundly depress respiratory drive. It can cause
hypoventilation and even transient apnea, and should only be administered by well-trained
providers with appropriate advanced airway skills.
Procedural sedation requires skilled and well-trained practitioners whose sole attention is on
providing sedation. Intravenous access may be preferred for administering sedatives. but
regardless of administration route. equipment to secure venous access must be available should
resuscitation be required, Given the risk of hypoventilation and hypoxemia during sedation,
airway equipment including bag-valve masks with an oxygen supply, along with either face-
masks or nasal cannula oxygen, should be immediately available. Monitoring of vital signs,
including pressure, pulse, and respirations, is standard; these should be documented at frequent
and regular intervals. In addition, continuous pulse oximetry is used to monitor oxygenation and
capnography is used to monitor ventilation; hypercapnia often precedes oxygen desaturation as a
result of inadequate ventilation.
PREP Pearls
• Procedural sedation is defined as the delivery of sedating or dissociative medications to
produce a state of depressed consciousness; sedation occurs along a continuum ranging
from minimal to moderate to deep.
• Procedural sedation requires skilled and well-trained practitioners, equipment, and
adequate provisions or patient monitoring.
• Ketamine is an ideal medication for procedural sedation because it has analgesic,
amnestic, and sedative properties.
• Diagnose and manage acute head injury.

• Understand the appropriate use of sedative analgesia
• Understand the appropriate use of minimal sedation (anxiolytic)
Suggested Readings
• Pediatric sedation management. Pediatr Rev. doi:10.1542/pir.2014-0116
• Risk factors for adverse events in emergency department procedural sedation for
children, JAMA Pediatr, doi:10.1001/jamapediatrics.2017.2135
• Guidelines for Monitoring and Management of Pediatric Patients Before, During, and
After Sedation for Diagnostic and Therapeutic Procedures„ Pediatrics,
doi:10.1542/peds.2019-1000
• Incidence and nature ot adverse events during pediatric sedation/anesthesia for
procedures outside the operating room: report from the Pediatric Sedation Research
Consortium. Pediatrics, doi:10.1542/peds.2006-0313

Question 205
An 18-month-old boy who has been fully vaccinated is seen for fever, ear pain, and eye drainage.
For the past 2 days, he has had fever up to 38.5 ºC, a red right eye, purulent drainage from his
right eye, congestion, rhinorrhea, and dry cough, and has been tugging at his right ear. He has no
other medical problems. On physical examination, his temperature is 38.3 ºC, heart rate is 120
beats/min, respiratory rate is 32 breaths/min, blood pressure is 90/40 mm Hg and oxygen
saturation is 99% in room air. His right tympanic membrane is bulging with purulent fluid, and
his right conjunctiva is injected with a mild amount purulent drainage. His nasal turbinates are
edematous with clear rhinorrhea and his throat is injected. The remainder or his physical
Of the following, the pathogen that is MOST likely this boy's symptoms is
A. group A ß-hemolytic Streptococcus
B. Moraxella catarrhalis
C. Non typeable Hemophilus influenzae
D. Streptococcus pneumoniae

Correct Answer: C
The boy in the vignette has both bacterial conjunctivitis and acute otitis media (AOM), The most
likely causative organism in this case is nontypeable Hemophilus influenzae. Group A ß-
hemolytic Streptococus does not typically cause bacterial conjunctivitis. Moraxella catarrhalis
and Streptococcus pneumoniae may cause both conjunctivitis and AOM. but they are less
common.
Acute otitis media is usually preceded by a viral upper respiratory tract (URI) infection. The URI
leads to edema and increased fluid production. which contribute to eustachian tube dysfunction.
Fluid and bacteria are retained behind the tympanic membrane, causing AOM. Infants and young
children are especially prone to AOM because their eustachian tubes are oriented more
horizontally than in older children and are thus unable to drain as well. As the viral infection
resolves, edema also resolves and the eustachian tube can then drain properly.
Acute otitis media is generally self-limiting but the fluid retained behind the tympanic membrane
may take several months to resolve. Sometimes, the increased pressure behind the tympanic
membrane may result in spontaneous rupture. Rarely, AOM may lead to serious complications
such as mastoiditis intracranial abscesses, and lateral sinus thrombosis.
Children with AOM usually exhibit symptoms of URI as well as ear pain. Depending on the
child's age, signs and symptoms of ear pain may be as vague as sleep disruption or fussiness or
as specific as pointing to the affected ear(s) and complaining of decreased hearing. On physical
examination, the tympanic membrane will be bulging, with evidence of inflammation and
purulent fluid, as shown in Item C205.
Item C205
The most common organisms isolated from middle-ear fluid in children with AOM are
nontypeable H influenzae including those producing ß-lactamase, M catarrhalis, and the S
pneumoniae serotypes not included in the pneumococcal vaccine. Less commonly isolated
organisms include respiratory syncytial virus, influenza, parainfluenza virus, group A ß-
hemolytic Streptococcus, Staphylococcus aureus, and Mycoplasma pneumoniae. Neonates may
have AOM caused by gram-negative bacilli.

PREP Pearls
• Nontypeable Hemophilus influenzae is the most common cause of acute otitis media with
concurrent bacterial conjunctivitis.
• Since the introduction of the pneumococcal vaccine, Hemophilus influenzae and
Moraxella catarrhalis have become the most commonly isolated organisms in children
with acute otitis media
• Understand the differential diagnosis, evaluation, and management of otorrhea in children

• Recognize pathogens commonly associated with acute otitis media in patients of various
ages
Suggested Readings
• Otitis Media: To Treat, To Refer, To Do Nothing: A Review for the Practitioner. Pediatr
Rev. doi: 10.1542/pir.36-11-480
• Otitis media and otitis externa. American Academy of Pediatrics Textbook of Pediatric
Care 2nd ed. 2017:2452-2457

Question 206
A 10-year-old girl is seen as a first-time patient for a health supervision visit. She has no
significant past medical history and is up to date on her immunizations. She is active in dance
and does well in school. There are no known heritable diseases in the family. Both parents and
all grandparents are healthy. Her physical examination findings are normal.
Of the following the MOST appropriate test to obtain for this girl is (a)
A. fasting lipid profile
B. non-fasting lipid profile
C. nothing; no routine screening
D. total cholesterol level

Correct Answer: B
The girl in the vignette has no identifiable risk factors for cardiovascular disease. In this case, the
American Academy of Pediatrics (AAP) recommends universal screening for dyslipidemia at
age 9 to 11 years and again after age 16 years with a lipid profile that can be obtained in the non-
fasted state.
A non-high density lipoprotein (non-HDL) cholesterol level is accurate in the nonfasted state and
is as strong a predictor of atherosclerosis as any other lipoprotein. These factors plus the
increased compliance and convenience of obtaining a sample in a non-fasted state makes this
method the test of choice. In the non-fasted state, a non-HDL cholesterol level can be calculated
as follows: non-HDL cholesterol equals total cholesterol minus HDL cholesterol. If the non-
HDL cholesterol level is ≥145 mg/dL (3.76 mmol/L), then a fasting lipid profile should be
obtained.
Relying on family history to detect cardiovascular risk factors and determine who should be
screened for dyslipidemia has not been demonstrated to be very effective. Therefore, universal
screening is recommended by the AAP between 9 to 11 years of age and again after age 16
years. Lipoprotein levels fluctuate with age. They reach a steady state around age 10 years, prior
to decreasing at puberty.
Those with risk factors for cardiovascular disease, including a positive family history, undergo
screening starting at age 2 years with a fasting lipid profile. These risk factors include:
• Obesity
• Hypertension
• Diabetes conditions or medications associated with secondary dyslipidemia (Item
C206A)
• Family history of dyslipidemia (total cholesterol> 240 mg/dL [6.22 mmol/L])
• Family history of infarction; angina: coronary artery bypass, graft, or stent; angioplasty;
or sudden cardiac death in a parent, grandparent, aunt, or uncle at <55 years for males or
<65 years for females
Item C206B shows acceptable and elevated lipid cut-off values for children and adolescents.
Item C206C lists the major lipid disorders in children and adolescents.
Initial therapy for hyperlipidemia is focused on lifestyle modification, including nutrition and
exercise. If lipid levels do not reach acceptable levers in response to these changes, medications
may be prescribed in order to lower triglyceride or lipoprotein levels. Treatment of underlying
conditions that increase cardiovascular risk, such as hypertension is also very important.
Item C206A: Causes of Secondary Hypercholesterolemia in Children

• EXOGENOUS
• Drugs: corticosteroids, isotretinoin (Accutane),
• thiazides, anticonvulsants, beta blockers, anabolic
• steroids, certain oral contraceptives
• Alcohol
• Obesity
• ENDOCRINE AND METABOLIC
• Hypothyroidism
• Diabetes mellitus
• Lipodystrophy
• Pregnancy
• Idiopathic hypercalcemia
• STORAGE DISEASES
• Glycogen storage diseases
• Sphingolipidoses
• OBSTRUCTIVE LIVER DISEASES
• Biliary atresia
• Biliary cirrhosis
• Alagille syndrome
• CHRONIC RENAL DISEASES
• Nephrotic syndrome
• OTHERS
• Anorexia nervosa
• Progeria
• Collagen disease
• Klinefelter syndrome
Item C206B: shows acceptable and elevated lipid cut-off values for children and adolescents

Item C206C: major lipid disorders in children and adolescents
PREP Pearls
• The American Academy of Pediatrics recommends universal screening for dyslipidemia
between ages 9 to 11 years and again at age 16 years with a lipid profile that can be
obtained in the non-fasted state.
• Children with risk factors for cardiovascular disease, including a positive family history,
should undergo screening starting at age 2 years with a fasting lipid Profile.
• Risk factors for atherosclerosis include primary or secondary dyslipidemias, a family
history of cardiovascular risk, obesity, and hypertension.

• Understand the importance of cholesterol/lipid screening examinations
• Recognize the clinical features associated with hypercholesterolemia/hyperlipidemia, and
evaluate appropriately
Suggested Readings
• Lipid abnormalities American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:2269-2283
• Update on screening, etiology, and treatment of dyslipidemia in children. J Clin
Endocrinol Metab, doi:10.1210.jc.2013-3860
• Expert panel on integrated guidelines for cardiovascular health and risk reduction in
children and adolescents. Pediatrics. doi:10.1542/peds.2009-2107C
• Highlights of the report of the expert panel on blood cholesterol levels in children and
adolescents. Pediatrics. https://pediatrics.aappublications.org/content/89/3/495
• Hyperlipidemia. Pediatr Rev. doi:10.1542/pir.2019-0053

Question 207
A 14-year-old adolescent girl with a history of obesity, autism, and asthma is seen in the
emergency department for acute onset of right-sided scapular pain and vomiting. Her symptoms
began about 2 hours ago, shortly after eating a fast food dinner, Het only medication is albuterol,
which she requires infrequently. The girl is minimally verbal. Her temperature is 40 ºC. The
ability to examine her is limited by her significant autism, but she appears to have a firm
abdomen with voluntary guarding.
Of the following. the MOST likely cause of the girl's pain is

A. cholecystitis
B. nephrolithiasis
C. ovarian torsion
D. septic arthritis of the shoulder

Correct Answer: A
Based on her history and limited physical examination findings. the girl's pain is most likely due
to cholecystitis. Abdominal pain may present as visceral, somatoparietal, or referred. The child
in the vignette developed pain after a fast food meal and has fever, scapular pain, and abdominal
tenderness. Her right scapular pain is likely referred abdominal pain due to inflammation of the
gallbladder (i.e., cholecystitis).
Visceral pain occurs with stretching of the internal organs, as their pain receptor are located in
the muscles and mucosa. Visceral pain is often described as poorly localized or diffuse pain that
is aching and/or cramping in nature. Somatoparietal pain occurs when the peritoneal pain
receptors are stimulated, resulting in sharp, severe, localized pain that can improve with
relaxation of the peritoneum. Referred pain is the result of convergent neural inputs, resulting in
pain perceived in cutaneous dermatomes sharing the same spinal cord level. For example, injury
or inflammation of the gallbladder, proximal small bowel, ot right diaphragm can manifest as
right-sided scapular pain. Similarly, causes of pain outside the abdomen can manifest as
abdominal pain (e.g., pneumonia or diabetic ketoacidosis).
Referred pain can provide a diagnostic challenge, particularly in young children or individuals
with communication difficulties, such as the girl in the vignette. Item 207A describes common
causes and location of pain referred from the abdomen. Item 207B describes causes of extra-
abdominal pain referred to the abdomen.
Item 207A & Item 207B: Referred Abdominal pain

Nephrolithiasis can present as lower back or flank pain, but would not be expected to cause
scapular pain. Ovarian torsion would be associated with lower abdominal pain and would not
cause scapular pain. Septic arthritis of the shoulder could present with shoulder pain and fever;
however, it would not result in abnormal abdominal examination findings.
PREP Pearls
• Pain caused by a gastrointestinal etiology may present as pain outside of the
gastrointestinal tract; this type or pain is known as referred abdominal pain
• When assessing abdominal pain, it is important to consider a broad differential diagnosis,
including pain referred to the abdomen from extra-abdominal locations.
MOCA-Peds Objective
• Know the differential diagnosis of acute abdominal pain.

• Understand sources of referred abdominal pain and their associated pain patterns
Suggested Readings
• Referred abdominal pain. Pediatr Rev, doi:10.1542/pir.2019-0221
• Abdominal pain. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed;2017;1181-1188

Question 208
A 3-year-old girl is brought to the office for a health supervision visit. She has a history of
neuroblastoma status post chemotherapy; the last dose was given 3 months ago, and she is
currently in remission. She did not receive any vaccines after 6 months of age. Per her last
oncology consult note, she has evidence of restored immunocompetence. She is due for varicella,
measles-mumps-rubella, hepatitis A, and influenza vaccines.
Of the following, the BEST next step in this girl’s management is to administer
A. all 4 vaccines
B. hepatitis A and influenza vaccines only
C. hepatitis A vaccine only
D. no vaccines

Correct Answer: A
The girl in the vignette has a history of neuroblastoma that is in remission. and it has been 3
months since her last chemotherapy treatment. She should receive both live and inactivated
vaccines at this time.
Inactivated-virus vaccines (e.g., hepatitis A and influenza) may be given to children with cancer
who are receiving chemotherapy. However. unless there is documentation of a protective
antibody level, inactivated vaccines given during chemotherapy should not be considered valid
doses.
Immunization with live-virus vaccines is contraindicated for children with cancer while
receiving chemotherapy. Current recommendations are to vaccinate children who have cancer
with live vaccines, including measles-mumps-rubella (MMR) and varicella, when they are in
remission, it has been at least 3 months after chemotherapy, and there is evidence of
immunocompetence. Because the child in the vignette meets these criteria, she should receive all
4 vaccines at her health supervision visit. For children with cancer who received live-virus
vaccines prior to chemotherapy. vaccine titers should be checked and if absent, readministered.
PREP Pearls
• Children who have cancer should be immunized with live vaccines when in remission, at
least 3 months after chemotherapy, and with evidence of immunocompetence.
• Inactivated-virus vaccines may be given to children with cancer who are receiving
chemotherapy; however, unless there is documentation of a protective antibody level,
inactivated vaccines given during chemotherapy should not be considered valid doses,
• For children with cancer who received live-virus vaccines prior to chemotherapy. vaccine
titers should be checked, and if absent, readministered.

• Know the indications, contraindications, limitations, and schedule for varicella zoster
vaccine. including after exposure
Suggested Readings
• Varicella-zoster virus infections. Red Book: 2021-2024 Report of the Committee on
infectious Diseases. 32nd ed; 831-842
• Varicella-zoster virus infections. Pediatr Rev. doi:10.1542/pir.29-1-5
• Preventing infections in children with cancer. Pediatr Rev. doi:10.1542/pir.2015-0059
• Prevention of varicella: recommendations of the Advisory Committee on Immunization
Practices (ACIP).
• Clinical Practice Guideline for Vaccination of the Immunocompromised Host. Clin Infect
Dis, doi:10.1093/cid/cit684

Question 209
A 15-year-old adolescent boy with Marfan syndrome is seen for a routine health supervision
visit. He played golf on his school's team last year and is hoping to continue this year. However;
over the past several months, he has had continued joint pain and fatigue that interfere with his
golfing performance. Cardiac reevaluation several weeks ago demonstrated no evidence of aortic
root dilatation and normal valve function. He takes propranolol and has not had a recent dose
change. His parents are very concerned about his fatigue and pain during and after rounds of
golf.
Of the following, the BEST next step for this adolescent is to

A. begin an aerobic training program
B. discontinue golf participation
C. start physical therapy
D. stop his ß-blocking medication

Correct Answer: C
Children and adolescents with Marfan syndrome often experience fatigue and joint pain with
physical activity. A carefully designed program to improve strength and aerobic capacity can
help improve function and endurance in these individuals. He should seek guidance from a
physical therapist to help design a safe and appropriate therapeutic exercise program that should
ideally become part of an ongoing fitness routine. Beginning an aerobic training program without
the guidance of a physical therapist would not be recommended for the adolescent in the
vignette.
Individuals with Marfan syndrome are at increased risk for injury and sudden death with high-
intensity activity or competitive sport participation. Therefore, delineating the appropriate
amount, intensity, and types of activity is important to allow these children and adolescents to
glean the benefits of sports and exercise while minimizing risk.
Sports are classified as having static dynamic components, as shown in Item C209. High
dynamic sports result in rates and cardiac output and are often perceived as “aerobic” ot “cardio”
training. High static sports often create maximal voluntary muscle contractions and result in high
blood pressure afterload, Activities with high dynamic and/or high static cardiac demands place
individuals with Marian syndrome at risk for progression of aortic dilation and cardiac
dysfunction. A consensus statement from the American Heart Association and American College
of Cardiology makes the following recommendations for the care and activity of athletes with
Marfan syndrome:
• Evaluation of aortic root dimensions should be performed every 6 to 12 months
• May consider participation in class IA or IIA sports if they do NOT have any of the
following:
o Aortic root dilation
o Ventricular dysfunction
o Moderate or severe mitral regurgitation
o Family history of aortic dissection at an aortic diameter of < 50 mm
• Avoid participation in contact/collision sports
A β-blocking medication may be used to reduce the progression of aortic dilation in children
with Marfan syndrome. These medications typically reduce capacity for higher intensity exercise
and sport, and fatigue is common. Patients and families should be counseled on potential
reductions in activity tolerance and energy levels with β-blocker use. Individuals taking these
medications are advised to only exercise up to maximum heart rate of 100 beats/min. Athletic
activity can be modified to keep exertion under this maximum. Golfers with Marfan syndrome
should use a cart or caddy to carry their golf bags, and should consider using a cart on hilly or
challenging terrain. If significant fatigue persists, changes in dose or formulation considered
before discontinuing ß-blocking medication.
Although most literature regarding the risk of sport participation with Marfan syndrome focuses
on cardiovascular concerns, there is also risk for ocular injury. Athletes with Marfan syndrome
should be counseled on the use of protective eyewear and avoidance of combat-type sports that
place athletes at undue risk for eye injury. In addition, the ligamentous laxity seen in Marfan
syndrome may lead to joint pain, particularly with high impact or repetitive activity. An
appropriate low intensity strengthening program may enhance muscular stabilization of loose
joints during activity. This program should be designed by a physical therapist or other licenced
professional familiar with the medical needs of children and adolescents with Marfan syndrome.
Item C209: Static dynamic sports classification
Appropriate participation in physical activity and sports has physical, psychological, and social
benefits for children and adolescents with Marfan syndrome. Goll is a medically appropriate
sport for the adolescent in the vignette, and efforts should be made to allow him to continue to
participate safely and comfortably before recommending that he give up this sport.
PREP Pearls
• Children and adolescents with Marfan syndrome may participate in sports with low
dynamic and static cardiovascular demands.
• Children and adolescents with Marfan syndrome should be encouraged to participate in
healthy levels of appropriate physical activity.

• Recognize the implications for sports participation in a patient with Marfan Syndrome
Suggested Readings
• Eligibility and disqualification recommendations for competitive athletes with
cardiovascular abnormalities: Task Force 7; aortic diseases, including Marfan syndrome:
a scientific statement from the American Heart Association and American College of
Cardiology. J Am coll Cardiol, doi:10.1016/j.jacc.2015.09.039
• Health Supervision for children with Marfan syndrome. Pediatrics,
doi:10.1542/peds.2013-2063
Question 210
An 8-year-old boy is brought to the office in August with a 1-week history of left knee swelling
not associated with fever (Item Q210A). He is able to walk without limping but has pain
associated with running. His vital signs include a temperature of 37.3 º C, heart rate of 90
beats/min, respiratory rate of 20 breaths/min, and blood pressure of 110/72 mm Hg. He had a
febrile illness with rash (Item Q210B) 8 weeks ago while visiting family in eastern Long Island,
New York. At that time, he was evaluated at a local urgent care center and was treated with oral
cephalexin for presumptive diagnosis of cellulitis.
Of the following, the MOST appropriate management of this boy's condition is

A. amoxicillin for 21 days
B. doxycycline for 21 days
C. doxycycline for 28 days
D. vancomycin for 28 days

Correct Answer: C
The boy in the vignette has Lyme arthritis, which is a late manifestation of Lyme disease. It is
observed in children who are not adequately treated during the early stages of infection. Children
with Lyme arthritis may be successfully treated with a 28-day course of oral antibiotics.
Amoxicillin is first-line treatment for children under 8 years of age and doxycycline for children
at or above 8 years of age. Oral cefuroxime may be used for children who are unable to tolerate
amoxicillin. In a child with a documented hypersensitivity reaction to penicillin. a 7-day course
of azithromycin can be used. Because late Lyme arthritis is inflammatory in nature, nonsteroidal
anti-inflammatory drugs, such as ibuprofen or naproxen, can be used in addition to antibiotics
treatment for faster resolution of swelling. A second course of oral antibiotics can be considered
for children who do not respond completely or relapse after 28 days of treatment. Children who
experienced worsening symptoms can be treated with a 14 to 28 day course of parenteral
ceftriaxone.
Lyme disease is caused by a spirochete, Borrelia burgdorferi, and is the most tick-borne
infection in the United States. Human infections result from the bite of an infected Ixodes
scapularis tick (deer tick), which are active in summer months. The majority of cases are
transmitted by baby (nymph) ticks. Although adult ticks often feed on deer, these animals do not
become infected, Deer are nevertheless important in transporting ticks and maintaining the tick
populations. White-footed mice are the principal reservoirs for B burgdorferi, in 2018 in the
United States, 14 states accounted for over 95% of confirmed Lyme disease cases. These states
included Connecticut, Delaware, Maine. Maryland, Minnesota, New Hampshire, New Jersey,
New York, Pennsylvania, Rhode Island, Vermont, Virginia, Virginia, and Wisconsin. Lyme
disease most commonly in forested regions.
The clinical manifestation of Lyme disease in children can be broadly classified into a) early
localized, b) early disseminated, c) and late Lyme disease (Item C210) late Lyme disease in
children manifests as an inflammatory arthritis, commonly mono or pauci-articular, affecting
large joints such as the knee. Lyme disease was first recognized in 1977 as Lyme arthritis; a
cluster of children in Connecticut were seen with arthritis and were initially thought to have
juvenile idiopathic arthritis. Lyme arthritis can occur with or without a history of erythema
migrant, typically seen in early Lyme disease. In comparison to children with pyogenic arthritis,
children with Lyme arthritis have swelling disproportionate to pain; do not have high fevers; are
otherwise well appearing; and have lower serum white blood cell count, neutrophilia, and
inflammatory markers.
The diagnosis of Lyme disease is made based on results of clinical findings coupled with a
plausible geographic exposure. Serological assays aid in diagnosis. The standard approach is 2-
tier testing, enzyme linked-immunosorbent assay (ELISA) or immunofluorescent assay (IFA)
followed by a Western blot. This sequence increases test specificity. False positives may occur
due to cross-reactivity with other spirochete species, human oral flora, other acute infections, or
certain autoimmune conditions, Serological assays should not be performed in children without
signs and symptoms suggestive of Lyme disease and without plausible geographic exposure. In
cases of Lyme arthritis, the initial serological assay typically demonstrates an elevated antibody
level with detection of ≥5 IgG bands on the Western blot. Synovial fluid cell count typically is
>2,000-50,000 white blood cells/µL. The joint aspirate of a pyogenic/septic joint has a white
blood cell typically >50,000 /µL. The presence of B burgdorferi DNA in the synovial fluid
assists in the diagnosis.
Item C210: The clinical manifestation of Lyme disease
PREP Pearls
• Lyme disease, caused by the spirochete Borrelia burgdorferi, is the most common tick-
borne infection in the United States. Clinical manifestations in children include early
localized, early disseminated, and late Lyme disease
• Lyme arthritis should be suspected in children with large joint swelling who reside in or
have visited a Lyme disease-endemic region. Serological assays along with synovial fluid
analysis can support the clinical diagnosis.
• The initial treatment for Lyme arthritis is oral antibiotics for 28 days, amoxicillin < 8
years of age, doxycycline ≥8 years of age.

• Recognize the clinical features associated with Borrelia burgdorferi infection
• Plan the appropriate laboratory evaluation for Borrelia burgdorferi infection
• Understand the epidemiology of Borrelia burgdorferi
• Plan appropriate management fora patient with Borrelia burgdorferi infection
Suggested Readings
• Lyme disease. Red Book: 2021-2024 Report of the Committee on infectious Diseases.
32nd ed;2021; 482-489
• Pediatric Lyme Disease Study Group. Lyme disease in children in southeastern
Connecticut. N Engl J Med, doi:10.1056/NEJM199610243351703
• Empiric antibiotic treatment of erythema migrans-like skin lesions as a function of
geography: A clinical and cost effectiveness modeling study. Vector Borne Zoonotic Dis,
doi:10.1089/vbz.2013.1365
• Borrelia burgdorferi (Lyme disease), Pediatr Rev, doi:10.1542/pir.35-12-500
• The clinical assessment, treatment, and prevention of Lyme disease, human granulocytic
anaplasmosis and babesiosis: Clinical practice guidelines by the infectious disease society
of America. Clin Infect Dis, doi:10.1086/508667

Question 211
A neonate born at term develops respiratory distress in the first hour after birth. His mother had
minimal prenatal care and arrived at the emergency department in active labor. The neonate's
Apgar scores were 5 and 8 at 1 at 5 minutes, respectively. His birth weight was 2,950 g. After
delivery, the neonate developed tachycardia, tachypnea, nasal flaring, and intercostal retraction
requiring intubation and mechanical ventilation.
On physical examination, the neonate is a afebrile. He has diminished breath sounds in the lateral
and bases of his lung fields with fair aeration centrally and no adventitious breath sounds. The
remainder of his physical examination findings are unremarkable.
A point-of-care blood glucose level is 75 mg/dL (4.2 mmol/L). Chest radiography (Item Q211)
shows bilateral pleural effusions; the left is greater than the right. Echocardiography reveals
normal cardiac anatomy and function, Chest ultrasonography demonstrates nonloculated pleural
effusions bilaterally.
Diagnostic and therapeutic thoracentesis is performed. Analysis of the pleural fluid reveals the
following:
Pleural fluid test Result

Appearance Thin, pale, milky
Pleural: serum protein ratio 0.8
Pleural: serum LDH ratio 0.7
White blood cells 12,000 /µL (12 x 109/L), 85% lymphocytes
pH 7.1

Of the following, the MOST likely cause of this neonate's pleural effusion is
A. decreased colloid oncotic pressure
B. decreased pulmonary lymphatic drainage
C. increased pulmonary capillary permeability
D. increased pulmonary vascular hydrostatic pressure

Correct Answer: B
The neonate in the vignette had respiratory distress and abnormal lung examination and chest
radiography findings. He was evaluated for cardiac and pulmonary parenchymal sources of his
chest opacification and was found to have only an exudative effusion. His likely diagnosis is
congenital chylothorax, the most common cause of neonatal pleural effusion. Chylothorax results
from interruption of pleural lymphatic drainage and is characterized by an exudative effusion
with high levels of chylomicrons, triglycerides, and lymphocytes (>80% of the total white blood
cell [WBC] count in the pleural fluid). Chylous effusions typically appear thin and milky, as
opposed to empyema, which appear cloudy. Triglycerides and chylomicrons in the fluid are the
source or the characteristic milky appearance. Chylous effusions may be caused by thoracic duct
trauma (e.g., during surgery), thoracic duct obstruction, or lymphangiectasia, or may be
idiopathic. Most cases of congenital chylothorax are idiopathic.
Pleural effusions are designated as transudates or exudates using the ratios of serum and pleural
fluid protein and lactate dehydrogenase levels, as well as glucose levels and pH (Item C211A).
In addition, a WBC count greater than 1,000/µL is characteristic of an exudate. The elevated
pleural: serum protein ratio (>0.5) and lactic dehydrogenase ratio (>0.6) as well as low pH (<7.3)
and elevated WBC (>1,000/ µL [1 x 109/L], >80% lymphocytes) define this neonate's effusion as
an exudate.
Item C211A: Exudative and Transudative Effusions
Pleural effusions result from an imbalance between hydrostatic and oncotic pressures in the
pleural space. Normally, a very small amount of hypotonic fluid is seen in the pleural space. with
a glucose concentration similar to that of serum and a protein level of approximately 1.5 g/dL
(15 g/L). Pleural fluid can accumulate when pathologic processes increases the transfer of fluid
from the vascular space or pulmonary parenchyma into the pleural space, or interfere with
resorption in to the lymphatic and interstitial spaces. Examples of physiologic processes
responsible for pleural effusion are outlined in Item C211B.
Item C211B: physiologic processes for pleural effusion
In the normal physiologic state, pleural fluid is hypotonic, with a pH of 7.6, glucose concentrations
similar to those in serum, and protein concentrations of approximately 1.5 g/dL (15 g/L). Pleural fluid is
filtered at the parietal pleural level, and reabsorption occurs via parietal pleural lymphatics. Movement
between the vascular and pleural spaces is described by the Starling principle. To prevent excessive
accumulation of pleural fluid, the flow rate in pleural lymphatics increases in response to increased
pleural fluid filtration. Opposing hydrostatic and oncotic pressures act on the visceral and parietal
pleural membranes to prevent passage of pleural fluid from the parietal capillaries. A pleural effusion
forms when this balance is disrupted and fluid accumulates in the pleural space due to an underlying
pathologic process.
Increased hydrostatic pressure (CHF), a decrease in colloid osmotic pressure (hypoalbuminemia), an

increase in capillary permeability (pneumonia), increased intrapleural negative pressure (atelectasis),
and decreased lymphatic drainage may all lead to pleural effusions. Blood may cause a pleural effusion
related to trauma (hemothorax), and chyle may accumulate in the pleural space (chylothorax) due to
disruption or obstruction of the thoracic duct. In addition, pleural inflammation from disease processes
causes an influx of immune cells, leading to activation of the coagulation cascade and fibrin deposition.
This contributes to the increased permeability of adjacent capillaries and further accumulation of pleural
fluid
Neonatal pleural effusions are rare. Nonchylous effusions in neonates may be associated with
hydrops fetalis, congenital heart disease, trisomy 21, and Noonan syndrome
The neonate in the vignette does not have pulmonary parenchymal findings on physical
examination or radiography findings suggestive of pneumonia, which could cause increased
pulmonary capillary permeability. Cardiac anatomy and function for this neonate were normal on
echocardiography, ruling out congestive heart failure or other causes of increased pulmonary
vascular hydrostatic pressure. Pleural effusion occurs in neonates with hydrops fetalis and other
conditions associated with low serum protein levels because of decreased colloid oncotic
pressure. The neonate in the vignette does not have clinical findings of hydrops or
hypoproteinemia.
The most common causa of pleural effusion in children is community-acquired pneumonia

(CAP). An effusion occurs in 2% to 12% of all cases of CAP and up to 28% of children
hospitalized for CAP. About 3% of effusions in hospitalized children are empyema,
characterized by purulence, elevated protein levels. low glucose levels and low pH. Causes of
pleural effusions in children are described in Item C211C.

Item C211B: Causes of Pleural Effusions in Children
PREP Pearls
• Chylothorax is the most common congenital pleural effusion.
• Pleural effusion result from an imbalance between hydrostatic and oncotic pressures in
the pleural space
• Exudative pleural effusions have elevated protein and LDH levels and WBC counts
compared to serum levels; transudates have lower protein and LDH levels than serum and
white blood cell counts less than 1,000/ µL (1 x 109/L).
MOCA-Peds Objective

• Understand the etiology of pleural fluid accumulation

Suggested Readings
• Pleural effusions and pneumothoraces. Pediatr Rev, doi:10.1542/pir.2016-0088
• The use of Light’s criteria in hospitalized children with pleural effusion of unknown
etiology. Pediatr Pulmonol, doi:10.1002/ppul.24065
• Useful clinical biological markers in diagnosis of pleural in children. Paediatric Resp
Rev, doi:10.1016/s1526-0542(04)90039-5
• Pleural effusion (nonbacterial). Pediatric Pulmonology. 2011:559-569
• Chylothorax: diagnosis and management in children. Paediatr Respie Rev,
doi:10.1016/j.prrv.2009.06.008

Question 212
An 8-year-old boy is seen in the office for persistent fever. His mother has been taking his
temperature with a thermometer, and it has ranged between 38.5-39.6 ºC every day for the past
14 days. The boy has complained of fatigue and myalgias but has had no other specific
complains. He has no past medical history and reports no recent travel. The boy has a
temperature of 38.9 ºC, heart rate of 115 beats/min, respiratory rate of 18 breaths/min, and blood
pressure of 105/74 mm Hg. A thorough history does not elicit any additional pertinent
information. Physical examination reveals a well-developed boy who is awake, alert, and
tachycardic. The remainder of his examination findings are unremarkable. The child was seen in
an urgent center 4 days ago, at which time a complete blood count, complete metabolic panel,
and rapid strep and viral testing results were unremarkable.
Of the following, the boy's MOST likely diagnosis is

A. fever of unknown origin
B. fever without a source
C. pneumonia
D. urinary tract infection

Correct Answer: A
Of the response choices, the boy in the vignette's history, laboratory results, and physical
examination findings are most consistent with fever of unknown origin (FUO). Fever of
unknown origin is diagnosed when a child has a prolonged fever without apparent cause after a
thorough history and physical examination, and basic laboratory evaluations, fail to reveal a
specific diagnosis. The diagnosis FUO can be difficult to make. There is varying opinion among
experts regarding duration of fever required to consider the diagnosis of FUO, ranging from 8 to
21 days. The American Academy Textbook of Pediatric Care states the Current acceptable
definition of FUO is “a daily rectal temperature of greater than 38.3 ºC (101 ºF), lasting for at
least 2 weeks, the cause of which has not been determined by simple diagnostic tests, including a
complete history and thorough examinations” (van der Jaqt). Other sources have a similar
definition but only require 8 days of daily fever.
The child In the vignette has had fever for 14 days without an obvious source by history or
physical examination, and his basic laboratory results are normal; therefore, he meets the criteria
for the diagnosis of FUO. It is important to differentiate between fever of unknown origin (FUO)
and fever without a source (FWS) in children. These diagnoses are differentiated based on
duration of fever. Fever without source has a fever duration of less than 14 days without a readily
identifiable cause and often requires further evaluation that may include laboratory testing and
imaging studies, Fever without a source can progress to FUO if the fever continues for ≥14 days
and no cause is identified during the initial evaluation. Other than fever, the boy in the vignette
does not have any signs or symptoms suggestive of pneumonia or urinary tract infection, making
these diagnoses less likely.
A thorough history and full physical examination are vital in the diagnosis of FUO, as this will
guide the evaluation, laboratory testing, and imaging studies chosen to help elucidate the cause
of fever. The elements of the history must include documented fever with an accurate
thermometer, detailed description of the fever pattern, growth patterns, travel history, and animal
or other infectious source exposures, with particular consideration of infectious diseases that may
be endemic to the particular area. The history should also include medications, environmental
factors, and family and social history.
Although infection is the most common cause of FUO in children, there are both infectious and
noninfectious. Item C212A lists common etiologies of FUO in children. Due to the broad
differential of FUO in children. many infectious disease experts recommend a tiered diagnostic
evaluation process that is individualized based on the history and physical examination findings.
An example of a tiered approach to FUO in children can be found in Item C212B

Item C212A: Causes of pediatric fever of unknown origin

Item C212B: tiered approach to FUO
PREP Pearls
• The American Academy Textbook of Pediatric Care defines fever of unknown origin as
“a daily rectal temperature of greater than 38.3 ºC (101 ºF), lasting for at least 2 weeks,
the cause of which has not been determined by simple diagnostic tests, including a
complete history and thorough examinations”.
• The differential diagnosis of fever of unknown origin is broad and may include infectious
diseases, malignancies, autoimmune or inflammatory diseases, periodic fever syndromes,
and iatrogenic causes, as well factitious fever.
• The evaluation of fever of unknown origin in children should be individualized to the
patient based on history and physical examination findings and may include a tiered
diagnostic approach to identify the source.

• Formulate a differential diagnosis of fever without localizing signs in patients of various
ages
• Plan the appropriate evaluation and management of fever without source in patients of
various ages

Suggested Readings
• Pediatric fever of unknown origin. Pediatr Rev, doi:10.1542/pir.36-9-380
• Fever of unknown origin: Where science meets art. Pediatr Rev, doi:10.1542/pir.36-9-
378
• Fever of unknown origin. American Academy of Pediatrics Textbook of Pediatric Care.
2nd ed; 2017:1524-1528.

Question 213
An 18-month-old girl is seen for right ear drainage. She is up to date on her immunizations and
has a history significant for several episodes of acute otitis media. The girl has had a runny nose
and cough for 1 week and developed fever 3 days ago. She was quite fussy overnight and awoke
this morning with yellow-red material on her bedding and a crust on her right ear. on physical
examination, she is ill appearing but in no distress. Her temperature is 39 º C. Her left ear has a
normal external ear canal and no pain with manipulation of the tragus pinna; the tympanic
membrane is obscured by thick cerumen. Her right ear has and purulent drainage in the canal
which obscures the tympanic membrane. Her pinna and tragus can be moved during evaluation
without a notable increase in the girl’s discomfort. She has nasal drainage.
Of the following, the MOST appropriate initial treatment for this girl is
A. oral amoxicillin
B. otic benzocaine drops
C. otic ciprofloxacin drops
D. watchful waiting

Correct Answer: A
The girl in the vignette has otorrhea; her clinical history and physical examination findings
suggest acute otitis medica (AOM) with tympanic membrane (TM) rupture as the underlying
etiology. Although otic antibiotic drops are appropriate for treating purulent otorrhea in children
with tympanostomy tubes, oral rather than otic antibiotics are recommended for children with
AOM and TM perforation because the patency of the TM rupture cannot be ensured, and
antibiotic administration topically as otic drops may not reach the middle ear. Amoxicillin (high
dose, 90 mg/kg per day) is the preferred initial treatment for AOM. Benzocaine drops should not
be used with TM perforations, and can cause contact dermatitis of the ear canal. Watchful
waiting is not appropriate in this situation because the girl has severe symptoms.
Otorrhea is a common pediatric complaint with various causes. The most common otorrhea
include otitis externa, foreign body in the external ear canal, and AOM with TM rupture.
Chronic suppurative otitis media is less common and is a complication of untreated AOM, which
leads to a perforation of the TM and chronic middle ear drainage. Contact dermatitis of the
external ear canal, bullous myringitis, and cholesteatoma can also cause otorrhea. In children
with tympanostomy tubes, otorrhea is quite commonly seen with middle ear infection or
inflammation. Life-threatening but rare causes of otorrhea include cerebrospinal fluid leak
(typically in the setting of trauma), neoplasm (Langerhans cell histiocytosis), and necrotizing
(also known as malignant) otitis externa (a condition in which bacterial otitis externa
spreads to surrounding soft tissue, bony, and cartilaginous structures). The cause of otorrhea can
typically be elucidated by a thorough history and physical examination.
PREP Pearls
• The most common cause of otorrhea include otitis externa, foreign body in the external
canal, and acute otitis media with tympanic membrane rupture.
• Life-threatening but rare causes of otorrhea include cerebrospinal fluid leak (typically in
the setting of trauma), neoplasm (Langerhans cen histiocytosis), necrotizing (also known
as malignant) otitis externa
• Although otic antibiotic drops are appropriate for treating purulent otorrhea in children
with tympanostomy tubes. oral rather than otic antibiotics are recommended for children
with acute otitis media and tympanic membrane perforation because the patency of the
tympanic membrane rupture cannot be ensured and antibiotics may therefore not reach
the middle ear.
• Understand the differential diagnosis, evaluation, and management of otorrhea in
children.

• Understand the significance of otorrhea

Suggested Readings
• The diagnosis and management of acute otitis media. Pediatrics. doi:10.1542/peds.2012-
3488
• Otitis media: To treat, to refer, to do nothing: A review for the practitioner. Pediatr Rev.
doi:10.1542/pir.36-11-480
• Otitis media and otitis externa. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017; 2452-2457

Question 214
An 11-year-old boy is having a preparticipation sport evaluation to play on his middle school
basketball team. His father has questions about the boy’s growth spurt, which is occurring at a
younger age than many of his teammates. The was born at full term with a weight of 3.5 kg and
length of 50 cm, both appropriate for gestational age. His father and paternal uncle are 5'10 tall.
Both experienced growth spurts around the same time as their male peers. His mother is 5'2 tall
and had menarche at age 11 years. The boy has no siblings.
According to the boy’s medical record, he started puberty at age 9 years with a sexual maturity
rating of 2 for genital development and 1 for pubic hair. His previous height and weight
measurements tracked along the 40th and 30th percentiles, respectively.
Today his height is 136 cm (60th percentile) weight is 34 kg (30th percentile). He has grown 11
cm in the past year. He has mild comedonal acne and no gynecomastia. He has axillary hair and
a sexual maturity rating of 3 for genital development and pubic hair. The remainder of his
physical examination findings are unremarkable.
Of the following, the MOST likely expectation is that the boy will have a
A. final adult height that exceeds his adjusted midparental height
B. height growth spurt that will end later than that of his peers
C. peak in muscles strength that parallels his peak height velocity
D. Peak height velocity that is greater than that or his peers

Correct Answer: D
The boy in the vignette had earlier than average pubertal onset at age 9 years and is now
experiencing an earlier than average growth spurt at age 11 years. Boys with relatively early
maturation have greater peak height velocities (PHVs) than their later maturing peers.
Transiently, they may achieve a greater height percentile than expected. However, an earlier
initiation of a growth spurt is followed by an earlier cessation of height growth. Consequently,
the final adult height for some boys with early maturation may be shorter than prepubertal
estimates of final adult height. The boy in the vignette would not be expected to achieve a height
substantially greater than his adjusted midparental height target. Gain in muscle strength for boys
during puberty lags behind increases in height; peak muscle strength occurs after PHV.
For boys, the normal onset of puberty, indicated by an increase in testicular volume to 4 ml or
more (or a length of 2.5 cm), occurs between the ages of 9 and 14 years. An average PHV of 9.5
cm/year (range, 6-12 cm/year) is attained by boys with a sexual maturity rating (SMR) of 3 to 4.
around age 13.5 years. Height growth then declines until cessation around age 17 years. The
sequence of pubertal events and PHV for boys is summarized in Item C214A.
For girls, thelarche indicates the onset of puberty. Breast budding (breast SMR 2) is an early sign
of estrogenization and occurs between the ages of 8 and 13 years. At breast SMR 3 and about 6
months to 1 year before menarche, girls achieve a PHV of 8.3 cm/year (range 5-10 cm/year).
Higher levels of estrogen that accompany menarche promote epiphyseal closure with height
growth limited to an average of 7 cm after menarche. Average age of menarche is 12.5 years
(range, 9-15 years), Similar to boys, girls who experience a relatively early menarche may have a
greater PHV than later-maturing girls and may attain a shorter final adult height than predicted
by prepubertal growth. The sequence of pubertal events and PHV for girls is summarized in Item
C214B.
Genetic potential is the primary determinant of final adult height and is reflected by parental
heights. Calculation of the midparental height adjusted for sex provides a target range of
expected final adult height. The adjusted midparental height calculation for boys is:
(mother's height in cm + 13 cm + father’s height in cm) divided by 2
Target height is the adjusted midparental height plus or minus 2 standard deviations. Target
height for the boy in the vignette is 174 ± 8.5 cm.
The adjusted midparental height calculation for girls is

(Father’s height in cm - 13 cm + the mother's height in cm) divided by 2

Item C214A & Item C214B: Relationship of key pubertal stages to pubertal growth velocity. Top panel: After a slight
deceleration in growth, female puberty begins with breast development. Pubic hair development soon follows, and linear growth
accelerates. Girls achieve peak height velocity approximately 1 year before menarche. Note that the current estimates for
attainment of pubertal milestones in normal-weight girls (Table 1) are about 0.25 years (pubic hair stage 3) to 0.5 years (breast
stage 2) earlier than these 1985 estimates from Tanner and Davies (1985). (11) Bottom panel: After a slight deceleration in
growth, male puberty begins with testicular enlargement to genital stage 2 (volume >3 mL). Pubic hair development soon
follows, and linear growth accelerates. Boys achieve peak height velocity before entering genital stage 5 (testis volume 12 mL).
Note that the current estimates for pubic hair stage 3 milestones (Table 1) are 0.5 year earlier thanthese 1985 estimates.
Modified from Bock and Rosenfield (18) and Tanner and Davies. (11)

PREP Pearls
• A relatively early onset of puberty and height growth spurt is associated with attainment
of higher peak height velocities compared to those who mature later.
• Adolescents with earlier onset of puberty may transiently have a height percentile higher
than expected by prepubertal estimates. followed by an earlier cessation of growth, and
shorter adult stature than predicted
• Girls typically enter puberty about 1 year earlier than boys and initiate a growth spurt
about 2 years ahead of boys; most of their height growth occurs by menarche.
MOCA-Peds Objective
• Recognize and evaluate an adolescent with abnormal pubertal development.

• Understand the relationship between the timing of onset of puberty and final adult height
• Understand the timing, duration, and normal range of peak height velocity in male and
female adolescents
Suggested Readings
• Normal pubertal development: Part II. Clinical aspects of puberty. Pediatr Rev,
doi:10.1542/pir.32-7-281
• Normal physical growth and development. Neinstein's Adolescent and Young Adult
Health Care. A Practical Guide. 6th ed,2016;28-37
• Adolescent growth and development. Prim Care, doi:10.1016/j.pop.2014.05.002
• Puberty: normal and abnormal. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2016:1540-1545.

Question 215
A 12-year-old boy is seen for a health supervision visit. He has been well overall for the past few
months. He has had 3 episodes of bacterial pneumonia in his lifetime. He has also been treated
for several skin infections thought to have been precipitated by insect bites. His physical
examination findings and growth are normal

Hemoglobin 11.5 g/dL(115 g/L)
Platelet count 255 x 103 /µL(255 109/L)
Neutrophils 980/µL (0.98 x 109/L)
Direct antiglobulin (Coombs) IgG Positive
Anti-granulocyte antibody Positive
Serum IgG level 450 mg/dL (reference range, 550-1,440 mg/dL)
Of the following, the BEST next step in evaluation of this child is referral to a(n)
A. Dermatologist
B. Immunologist
C. infectious disease specialist
D. pulmonologist

Correct Answer: B
The child in the vignette has a history of multiple sinopulmonary and skin infections in addition
to cytopenia’s. His laboratory findings show evidence an autoimmune process; there is an
antibody toward red blood cells (i.e., direct antiglobulin [Coombs] IgG) and an antibody toward
neutrophils (i.e., anti-granulocyte antibody). The combination of autoimmune cytopenia with
frequent infections should raise concern for an immune deficiency syndrome. The low IgG level
is consistent with a diagnosis of common variable immune deficiency; however, the boy should
undergo evaluation by an immunologist to determine the cause of his immune deficiency.
Among the antibody deficiencies to consider in children with recurrent infections are disease and
immunoglobulin A deficiency, X-linked agammaglobulinemia (Bruton disease) is caused by a
mutation In Bruton’s tyrosine kinase, which results in a B-cell defect leading to a decrease in
immunoglobulin production and increased susceptibility to infections. Due to protection from
maternal antibodies, clinical manifestations often do not present before 3 months of age. Bruton
disease can be treated with immunoglobulin replacement therapy and prophylactic
antimicrobials. IgA deficiency is a humoral immunodeficiency, which can present with recurrent
infections and autoimmune disorders.
Although this boy has a history of recurrent skin infections, he does not currently have any skin
examination findings that would require a dermatologic evaluation.
An infectious disease evaluation might be warranted if the child had evidence of an active or
difficult-to-treat infection. However, that is not reported in this case.
Evaluation by a pulmonologist may be warranted if the child currently has abnormal pulmonary
findings on examination (e.g., rales, decreased breath sounds) or concerning pulmonary
symptoms (e.g., exertional dyspnea).
PREP Pearls
• Children with recurrent infections should be evaluated by an immunologist for potential
immune deficiency syndrome.
• Some immune deficiency syndromes are associated with autoimmune cytopenia's.
MOCA-Peds Objective

• Recognize the clinical characteristics of antibody deficiency syndromes after 4 to 6
months or age

Suggested Readings
• Management of primary antibody deficiency syndromes. Ann Allergy Asthma Immunol,
doi:10.1016/j.anai.2016.08.016
• Immunodeficiency disorders. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017:1553-1559.
• Lanzkowsky's Manual of Pediatric Hematology and Oncology. 6th ed. 2016;215-216.

Question 216
A 6-year-old girl is brought to the emergency department for a fracture of the right femur
without a history of trauma. She reports that she has experienced recurrent bone pain in that
region for several months. She has significant hepatosplenomegaly. No coarsening facial features
are noted. A complete blood cell count shows mild thrombocytopenia, anemia, and leukopenia.
You suspect a malignancy. A bone marrow examination reveals lipid-engorged macrophages,
known as foam cells, with positive periodic acid-Schiff staining (Item Q215). A skeletal survey
shows diffuse osteopenia and focal lytic lesions in varying regions.
Item Q215
Of the following, the MOST likely diagnosis for this girl is

A. Gaucher disease
B. Fabry disease
C. Hurler syndrome
D. Pompe disease

Correct Answer: A
Clinical features of type 1 Gaucher disease include hepatomegaly, splenomegaly, pancytopenia,
and skeletal disease. Bone marrow shows the characteristic storage cells of Gaucher disease.
Diagnosis via bone marrow aspiration and biopsy has been largely replaced by measurement of
the glucocerebrosidase enzymatic level in peripheral blood leukocytes.
Children with Gaucher disease may have delayed growth and menarche. Medullary infarction of
long bones leads to excruciating pain (bone crises). Complications include aseptic necrosis of the
femoral head and spontaneous fractures. Bone marrow infiltration with Gaucher cells is seen as
under tubulation in the distal femur, also called the Erlenmeyer flask deformity. Pulmonary
involvement may include interstitial lung disease, lobar consolidation, and pulmonary arterial
hypertensin. Treatment includes enzyme replacement therapy or substrate reduction therapy for
type 1 and some type 3 patients.
Fabry disease is an X-linked disorder of sphingolipid metabolism characterized by periodic pain

crises, angiokeratomas, corneal and lenticular opacities, stroke, left ventricular hypertrophy,
sweating abnormalities, and renal disorder progressing to end-stage renal disease.
Hurler syndrome, also known as mucopolysaccharidosis type l, is a progressive lysosomal

storage disorder. Clinical features include coarse facies, recurrent upper respiratory infection.
Umbilical hernia. hepatosplenomegaly, joint contractures, short stature, gibbus deformity, and
corneal clouding.
Pompe disease is a glycogen and lysosomal storage disorder inherited in an autosomal recessive
manner. Late-onset Pompe disease is classified as infantile onset and late onset. Clinical features
of infantile-onset Pompe disease include marked hypotonia, failure to thrive, and hypertrophic
cardiomyopathy within the first year of life. Clinical features of late-onset Pompe disease include
proximal muscle weakness with respiratory insufficiency; there is no cardiac involvement.
PREP Pearls
• Gaucher disease presents with bone crises, pancytopenia, and hepatosplenomegaly.
• Bone marrow infiltration by Gaucher cells produces the characteristic Erlenmeyer flask
deformity of distal femur.

• Recognize the clinical features associated with Gaucher and other lipid storage diseases
• Plan the appropriate immediate and long-term management of lipid storage disease,
including Gaucher disease, while considering the long-term prognosis
Suggested Readings
• Metabolic disorders beyond the newborn period. American Academy of Pediatrics
Textbook of Pediatric Care. 2nd ed;2017; 2015-2031
• Gabriele-de Vries syndrome. Gene Reviews [Internet]
• Inborn errors of metabolism (metabolic disorders). Pediatr Rev, doi:10.1542/pir.2014-
0122

Question 217
A 5-year-old boy who recently entered foster care is brought to the clinic by hid foster mother.
The boy was removed from his biological parents’ home for safety concerns due to exposure to
intimate partner violence. A medical student working in the clinic asks about risk factors for
intimate partner violence.
Of the following, the MOST accurate response is

A. gender equity
B. high level of education
C. older age
D. Poverty

Correct Answer: D
Intimate partner violence (IPV) is defined by the Centers for Disease Control and Prevention as
violence (physical, psychological, or sexual) occurring between people currently or previously in
an intimate relationship. Intimate partner violence is more likely to occur in the context of
economic stress or poverty among young individuals and those with a low education level, and
when societal norms support gender inequity. Additional contributors to IPV include exposure to
parental domestic violence and a history of child abuse.
Intimate partner violence affects approximately 1 in 3 women. Males, and adolescents or any
gender, can also be victims. Multiple visits for sexual health testing or somatic complaints should
raise concern for possible IPV. Consequences of IPV for the victim include sexually transmitted
infections, unplanned pregnancy, anxiety, depression, and posttraumatic stress disorder.
Women are more likely to experience IPV when pregnant. Miscarriage, preterm labor, and low-
birthweight infants are more common in pregnant women exposed to IPV. Neonatal death is also
more likely.
Children’s exposure to IPV may include witnessing violence, overhearing it, or seeing its effects
(e.g., injuries property damage). Children may be injured when present during an IPV episode.
Children exposed to IPV may exhibit internalizing behaviors (e.g., withdrawal, depression,
anxiety, school avoidance) and/or externalizing behaviors (e.g., aggression, defiance). They may
complain about headache or stomachaches. Sleep and eating may be disrupted. Children may
have difficulties with peer relationships and academics.
Intimate partner violence and child maltreatment frequently co-occur, often with IPV preceding
the child maltreatment. Intimate partner violence and child abuse/neglect are more likely to co-
occur when poverty, substance use, or depression are present. Adults who grew up with a parent
victim of IPV are more likely to have been physically, emotionally, or sexually abused than
adults who were not exposed to parental IPV. Adverse childhood experiences, including IPV
exposure, are associated with poorer adult health (e.g., obesity, depression, suicide attempts).
Pediatricians should be alert to the possibility of IPV and how it can affect the current and future
health of their patients and families.
PREP Pearls
• Intimate partner violence is more likely to occur in the context of economic stress or
poverty, among young individuals and those with a low education level, and when
societal norms support gender inequity.
• children exposed to intimate partner violence may exhibit internalizing behaviors (e.g.,
withdrawal, depression, anxiety, school avoidance) and/or externalizing behaviors (e.g.,
aggression, defiance).
• Intimate partner violence and child maltreatment frequently co-occur, often with intimate
partner violence preceding the child maltreatment.

• Understand the effects of societal violence on children
• Identify the important precipitants of intimate partner violence
• Recognize common characteristics that may indicate intimate partner violence, and the
effects of such violence on children
Suggested Readings
• Intimate partner violence. Pediatr Rev, doi:10.1542/pir.31-4-145
• Children exposed to adverse childhood experiences. American Academy of Pediatrics
• Intimate partner violence in the adolescent. Pediatr Rev, doi:10.1542/pir.2018-0053
• Intimate partner Violence: the role of the pediatrician. Pediatrics, doi:10.1542/peds.2010-
0451

Question 218
A 15-year-old adolescent boy is being evaluated for a rash on his face, which appeared several
weeks ago. The affected area is flaky and at times itchy. He also reports flaking of his scalp. The
boy is well in other respects and has an unremarkable medical history. His physical examination
findings are normal except for the alar creases, which reveal mild erythema, hypopigmentation,
and scaling.
Of the following, the MOST likely diagnosis is

A. atopic dermatitis
B. periorificial dermatitis
C. seborrheic dermatitis
D. tinea corporis

Correct Answer: C
The adolescent in the vignette has erythema, scaling, and hypopigmentation of the alar creases,
findings typical of seborrheic dermatitis in adolescents and young adults. Atopic dermatitis may
involve the face, but lesions are usually concentrated around the eyes and mouth with sparing of
the perinasal region. The absence of a history of atopic dermatitis also argues against this
diagnosis. Periorificial dermatitis may involve the perinasal area but is rarely limited to this
region, and lesions are erythematous papules or pustules, often with background erythema (Item
C218). Although the adolescent’s lesions are scaling, there is no border elevation or central
clearing as would be expected in tinea corporis (i.e., tinea faciei when located on the face).
Item C218
Seborrheic dermatitis is a chronic and relapsing inflammatory disorder that affects areas in which
sebaceous glands are concentrated. It is especially common in adolescents and young adults in
whom sebaceous gland activity is greatest. Although the cause is not fully understood, it may be
the result of an inflammatory response to the yeasts of the genus Malassezia. Typical findings
are scaling of the scalp (i.e., dandruff) or scaling and erythema of the eyebrows, eyelids, glabella,
alar of retro auricular creases, beard or sideburn areas, or ear canals.
For skin affected by seborrheic dermatitis, treatment is with a low-potency topical corticosteroid
(e.g., hydrocortisone 1% or 2.5 %) or an agent active against yeast (e.g., clotrimazole,
miconazole nitrate, or ketoconazole) applied twice daily as needed. Scalp involvement is treated
with anti-seborrheic shampoo containing pyrithione zinc, selenium sulfide, or ketoconazole. If
the scalp and face are involved some shampoo should be allowed to contact affected areas and
then rinsed. If signs of scalp inflammation are present (e.g., erythema or erosions), a topical
corticosteroid solution (e.g., fluocinolone acetonide 0.1%) may be applied at bed time.

PREP Pearls
• In adolescents and young adults, seborrheic dermatitis affects the scalp eyebrows,
glabella, alar or retro auricular creases, beard or sidebum areas or ear canals
• seborrheic dermatitis involving the scalp results in scaling (i.e., dandruff), skin lesions are
erythematous scaling macules or patches
• For skin affected with seborrheic dermatitis. treatment is with a low potency topical
corticosteroid (e.g., hydrocortisone 1% ot 2.5%) or an agent active against yeast (e.g.,
clotrimazole, miconazole nitrate, or ketoconazole) applied twice daily as needed
• Evaluate and manage a child with a disorder of the scalp

• Recognize the clinical findings associated with seborrheic dermatitis. and manage
appropriately
Suggested Readings
• Seborrheic dermatitis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed; 2017; 2597-2599
• Seborrheic dermatitis. Pediatric Dermatology: A Quick Reference Guide. 4th ed. 2020;
363-368
• Seborrhea. Pediatr Rev, doi:10.1542/pir.2017-0215
• Topical antifungals for Seborrheic dermatitis. Cochrane Database Syst Rev.
doi:10.1002/14651858.CDC008138.pub3

Question 219
A 3-month-old girl is evaluated in the emergency department for vomiting several times over the
past 24 hours. She has not had any diarrhea, fever, or congestion. She has appeared sleepier than
usual and is not feeding well. Her 3 older and mother are all healthy, as is her mother’s boyfriend
who lives in the same home. The girl was born at 33 weeks’ gestation. She had an uneventful
neonatal intensive care unit stay, initially requiring assistance with feedings and supplemental
oxygen but was taking all feedings by bottle and no longer requiring supplemental oxygen by the
time of discharge at 3 weeks of age.
On physical examination, the infant is sleepy and difficult to arouse. Her pupils are equal, round,
and reactive to light, oral mucosa is moist, heart rate is regular with no murmur, lung sounds are
clear with no increased work of breathing, and her abdomen is soft and nontender with no
palpable masses.
Of the following, the MOST appropriate test to order is

B. head computed tomography
C. supine abdominal radiography
D. upper gastrointestinal series

Correct Answer: B
The infant in the vignette, with vomiting end altered mental status, has signs and symptoms that
cause concern for abusive head trauma (AHT). In this situation. Head computed tomography
(CT) is the best imaging modality. This infant's risk factors for AHT include age, prematurity,
and having a nonrelated adult living in the home. While she may require brain magnetic
resonance imaging at some point, a CT scan should be obtained urgently to evaluate for
intracranial bleeding. Neither abdominal radiography or an upper gastrointestinal series is
indicated at this time, because the infant has a reassuring abdominal examination.
One in 8 children will experience child abuse by age 18 years; 17% of cases will consist of
physical abuse. Infants and toddlers are at the greatest risk of serious or fatal injuries related to
physical abuse. Especially for nonverbal children, medical professionals must have a high index
of suspicion for abuse independent of a family’s culture or socioeconomic background.
Abusive head trauma should be considered in infants with inappropriate sleepiness or fussiness;
emesis, especially without diarrhea; apnea; or seizures, Infants suspected of having AHT should
have an urgent head CT performed. If a CT scan demonstrates intracranial hemorrhage, urgent
neurosurgery and ophthalmology consultations are required. Physical examination findings
concerning for physical abuse include bruises, especially behind the ears, or on the abdomen,
neck, or on multiple planes of the body: any bruising in infants not yet mobile, such as those
under 4 months of age; and oral injuries, such as a torn frenulum. Pictures or a body diagram
should be used to document lesions; documentation should include the shape, size, color, and
location. Many children with a serious abuse event had been previously noted by a medical
professional to have bruising. Any concerns for abuse should be reported to social services.
A skeletal survey should be obtained for children less than 2 years of age found to have oral
injuries, concerning bruises, or a fracture that is not reasonably explained. if there is continued
uncertainty, repeat imaging should be obtained after 2 weeks, as healing fractures may become
apparent at that time. Laboratory evaluation should focus on causes of increased bruising or
fractures.
A child with a fracture that is not consistent with the history provided should undergo laboratory
evaluation including calcium, magnesium, phosphorus, alkaline phosphatase, complete blood
count, complete metabolic panel, amylase, lipase, international normalized ratio (INR), protime
and prothrombin time. If there is evidence of decreased bone density or an elevated alkaline
phosphatase level, 25 hydroxyvitamin D and parathyroid hormone levels should be obtained. If
there is an elevation in the aspartate aminotransferase, (AST), alanine aminotransferase (ALT),
amylase or lipase levels, abdominal CT should be obtained to evaluate for intra-abdominal
injuries.
Thorough individual interviews with all caregivers should be conducted, inquiring about when
the patient was last well, when the symptoms began, and how the symptoms progressed.
Information regarding how the patient appeared when changing from the supervision of one
caregiver to another, available photographs, and additional witnesses are all helpful. Document
all persons living in the home, including their ages, relationship to the patient, as well as
information regarding previous involvement with child protective services, drug or alcohol use,
and mental health concerns. Past medical history obtained should include diet, growth, and any
bleeding concerns such as bleeding after circumcision.
Family history inquiry should include bleeding abnormalities, metabolic abnormalities, or bone
disease such as osteogenesis imperfecta. If there is a family history of bleeding abnormalities, the
child's laboratory evaluation should include von Willebrand antigen and activity, factors VIII and
IX levels, and platelet function assay; a hematology consultation should be obtained. A family
history of metabolic or bony abnormalities should prompt genetics consultation.
The Adverse Childhood Event (ACE) study examines the long-term impact of child abuse. This
study demonstrated that children with exposure to interpartner violence, a parent who is
incarcerated, a parent with drug or alcohol abuse or mental health concerns are at increased risk
for obesity, hypertension, cancer, substance abuse, and mental health disorders. Currently, there
is extensive work seeking to identify resiliency factors and to understand how these factors
modify these outcomes.
PREP Pearls
• Head computed tomography is the imaging modality of choice when there is concern for
abusive head trauma.
• Findings that cause concern for physical abuse include bruises. especially behind the ears,
on the abdomen, on the neck or on multiple planes of the body; bruising in infants not yet
mobile; and oral injuries.
• Children with exposure to interpartner violence, a parent who is incarcerated, a parent
with drug or alcohol abuse or mental health concerns are at increased risk for obesity.
Hypertension, cancer, substance abuse, and mental health disorders.
• Understand the role of pediatrician in prevention of child abuse and neglect
• Diagnose and manage acute head injury

• Plan the appropriate evaluation and management of suspected child abuse
• understand the behavioral and emotional consequences of child physical abuse
Suggested Readings
• Center for Disease Control and Prevention. About the CDC-Kaiser ACE study.
https://www.cdc.gov/violenceprevention/aces/about.html
• Community poverty and child abuse fatalities in the United States. Pediatrics.
doi:10.1542/peds.2016-1616
• Physical abuse of children. Pediatr Rev. doi:10.1542/pir.2015-0012

Question 220
A 14-year-old adolescent girl is seen in the emergency department for a 4-week history of cough,
headache, muscle aches, fatigue, and intermittent fever. Two weeks ago, she was treated with a
10-day course of amoxicillin after chest radiography showed a lower lobe pulmonary infiltrate.
Despite treatment, her cough has worsened and she has developed night sweats and left chest
pain. Her immunizations are up to date. She has no history of ill contacts, exposure to animals, or
international travel. She resides in Phoenix, Arizona, and visits the Grand Canyon frequently.
On physical examination, the adolescent has a temperature of 38º C, heart rate of 81 beats/min,
respiratory rate of 18 breaths/min, and oxygen saturation of 94% in room air. Breath sounds are
decreased over the left lower lung field. Repeat chest radiography demonstrate a persistent
opacity in the left lower lung, small pleural effusion, and left paratracheal and hilar lymph node
enlargement. Her white blood cell count is 16,100/µL (16.1 x 109/L) with 60% neutrophils, 30%
lymphocytes, 7% monocytes, and 3% eosinophils. An interferon-γ release assay is negative
Of the following, the BEST next step to establish this adolescent’s diagnosis is to perform a
A. Bronchoalveolar lavage fluid culture
B. Pleural fluid polymerase chain reaction
C. Serologic examination
D. Urine antigen assay

Correct Answer: C
The adolescent girl in the vignette, who resides in Phoenix. Arizona, has complicate community-
acquired pneumonia that did not respond to antibiotic therapy. This presentation is suggestive of
coccidioidomycosis. Coccidioidomycosis (i.e., San Joaquin fever) is a systemic fungal infection
caused by the endemic dimorphic fungus, Coccidioides. The diagnosis should be established
serologically
Coccidioides species are present in arid soil in California, Arizona, and other areas of the
southwestern United States. The disease is also prevalent in areas of Mexico and Central and
South America. The 2 Coccidioides species include Coccidioides immitis (primarily found in
California) and Coccidioides posadasii (found in Arizona, Utah, New Mexico, and Texas). The
life cycle of Coccidioides species consists of a free-living mycelial phase (found in soil) and a
spherule phase (found in infected host tissues). The spherules contain numerous endospores that
are released on spherule rupture, resulting in the formation of new spherules in the infected
tissue.
Primary pulmonary infection occurs via inhalation in dry and dusty endemic environments after
soil-disruptive activities. Previously healthy individuals can become infected. However,
approximately 17% of affected individuals have an underlying immunodeficiency disorder, and
30% to 50% of these develop disseminated (extrapulmonary) disease.
The incubation period of coccidioidomycosis ranges from 1 to 3 weeks. Asymptomatic infection

is common. Symptoms occur in approximately 40% of infected individuals, manifesting as
community-acquired pneumonia with cough, fatigue, malaise, arthralgia, and fever. Symptoms
are often mild and self-limited. Pneumonia may be complicated by progressive disease (e.g.,
cavitation, lung abscess, empyema, nodules, bronchiectasis) with a prolonged course ranging
from weeks to months. Disseminated disease by hematogenous spread after pulmonary infection
and may involve the skin (e.g., ulcers, subcutaneous abscess), skeletal system (e.g.,
osteomyelitis), and central nervous system (e.g., meningitis).
If clinical suspicion of coccidioidomycosis is high, specialty consultation may be warranted to

assist with diagnostic evaluation and test interpretation. Laboratory diagnosis is made by
detecting anti-coccidioidal antibodies in serum. Serologic methods include enzyme
immunoassay, immunodiffusion test, and complement fixation (CF). Serologic tests may have
negative results in early disease. In primary infection, enzyme immunoassays and
immunodiffusion tests can detect IgM antibodies in 90% of cases by 2 to 3 weeks of illness, but
these wane by 5 months.
Although the antibody level does not correlate with disease severity, an elevated CF titer of 1:32
or higher may suggest disseminated disease. Quantitative complement-fixing antibody assays are
helpful in monitoring clinical response to therapy. Anticoccidioidal CF antibody can be detected
in the cerebrospinal fluid in the setting of intracranial infection.
Polymerase chain reaction assays to detect Coccidioides species in the respiratory tract are not
recommended as stand-alone diagnostic tests. Antigen assay of urine and other specimens (e.g.,
blood, bronchoalveolar lavage fluid) may detect Coccidioides species end are often positive in
disseminated disease. However, antigen tests are not widely available and are limited by cross-
reactivity with other endemic fungal species (e.g., Blastomyces and Histoplasma species),
Isolation of Coccidioides species on respiratory tract, tissue biopsy, or cerebrospinal fluid
cultures establishes a definitive diagnosis and should be considered if serologic findings are
negative. Histopathologic examination may detect endospore-containing spherules.
The differential diagnosis of pulmonary coccidioidomycosis includes other endemic fungal

infections (e.g., blastomycosis, histoplasmosis), tuberculosis, bacterial pneumonia, and
noninfectious disorders (such as sarcoidosis). Early diagnosis may be challenging because of
negative serologic assays and nonspecific symptoms.
Children with uncomplicated coccidioidomycosis pneumonia often improve without antifungal

therapy; clinical evaluation, serologic tests, and chest radiography are recommended at follow-up
to ensure resolution. Treatment with fluconazole for 3 months to 1 year is recommended for
severe pulmonary disease (e.g., consolidation of more than half of 1 lung or areas of both lungs,
> 10% weight loss, severe chest pain, duration of symptom >2 months). Intravenous
amphotericin B may be required for critically ill patients (e.g., diffuse pneumonitis,
extrapulmonary disease) followed by step-down therapy with fluconazole. In children with
disseminated disease (e.g., bilateral reticulonodular or miliary pattern on imaging) or diffuse
pulmonary disease, therapy with fluconazole is recommended for at least 1 year. Given the high
risk of relapse, some experts recommend continued treatment for many years. Central nervous
system disease requires lifelong therapy with oral fluconazole.
PREP Pearls
• Coccidioidomycosis (also known as San Joaquin fever) is a systemic fungal infection
caused by the endemic dimorphic Coccidioides species.
• Detection of anticoccidioidal antibodies with serologic examination is the first-line
diagnostic test for coccidioidomycosis.
• uncomplicated pneumonia caused by coccidiomycosis often improves without antifungal
therapy; fluconazole therapy for 3 months to 1 year or more is recommended for
treatment of severe, pulmonary coccidioidomycosis and disseminated disease

• Understand the epidemiology of Coccidioides
• Plan the diagnostic evaluation of a suspected Coccidioides infection, and manage
appropriately
• Recognize the clinical features associated with Coccidioides infection

Suggested Readings
• Fungal infections (systemic). American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017:2036-2063
• Coccidioidomycosis. Red Book: 2021-2024 Report of the Committee on infectious
Diseases. 32nd ed; 277-279
• Centers for Disease Control and Prevention. Increase in reported coccidioidomycosis;
https://www.cdc.gov/mmwr/preview/mmwrhtml/mm6212a1.htm
• Coccidioidomycosis: a case series from a California pediatric infectious diseases clinic.
Pediatr Infect Dis J, doi:10.1097/INF.0000000000002069
• Coccidioidomycosis. Pediatr Rev. doi:10.1542/pir.36-4-181

Question 221
A 5-year-old girl is separated from her mother during a routine health supervision visit while the
staff performs screening audiometry. She begins screaming and kicks the nursing assistant.
Of the following, the BEST response to this situation is to

A. allow the girl to use a handheld device for distraction
B. discuss the girl's response with her mother
C. distract the girl with a book white completing the test
D. reschedule the hearing screening for the next month

Correct Answer: B
The girl in the vignette is having an abnormal reaction to being separated from her mother. This
response should be discussed with her mother as it may indicate a history of adverse childhood
experiences (ACEs). All children should be screened for ACEs at every health supervision visit.
Any child who screens positive for ACEs should be referred to community resources.
Recognizing a potential history of traumatic events is key to providing trauma-informed care.
These children are at increased risk for developmental delays and mental health concerns.
Distracting the girl with a book or handheld device may help complete the hearing screening.
However, these techniques probably will not help in understanding her response. Rescheduling
the screening test to a later date also avoids dealing with the issue at hand. It represents a missed
opportunity to understand the girl's reaction and facilitate the development of healthy coping
skills.
Childhood exposure to trauma affects short and long-term health outcomes. In 1998, researchers
showed a direct correlation between the number of self-reported ACEs and adult-onset heart
disease, cancer, skeletal fractures, chronic lung disease, and liver disease. In this study, ACEs
were defined as:
• Abuse: Physical, sexual, or emotional
• Neglect: Physical or emotional
• Household challenges: Incarceration. mental illness, substance dependence, parental
separation or divorce, intimate partner violence
In theory, repeated traumatic exposures without mitigating protective factors alter the body’s
response to stress. Individuals with long-term exposure to adverse events develop “toxic stress,”
which ultimately can change epigenetic signatures, structure and function, and lead to unhealthy
coping behaviors
Children who have had traumatic exposures may need to train their bodies to react to challenges
in new ways. The first step in supporting those exposed to repeated trauma is acknowledging the
stressful events and resultant response. Parents may benefit from learning positive coping
strategies. A parent taking a moment to breathe when dinner spills on the floor can be a powerful
example for children struggling with stressful situations. Ideally, providers should avoid
retraumatizing children and families in clinical settings.
Healthcare providers caring for children and families exposed to toxic stress may identify similar
effects in themselves, such as sleeplessness, hypervigilance, minimizing, and guilt. This
phenomenon has been termed “secondary traumatic stress”. As much as possible, providers
should balance caseloads to share the care of families with toxic stress as well as find
opportunities to process these challenging clinical scenarios.
The American Academy of Pediatrics published report provides advice for the
provision of Trauma-Informed Care in a variety of settings (see Forkey H, Szilagyi M, Kelly ET,
Duffee J).

PREP Pearls
• Repeated exposure to childhood trauma is directly correlated with short and long-term
health outcomes such as ischemic heart disease, chronic lung disease, cancer, and liver
failure.
• Adverse childhood events may be defined as abuse (physical, sexual, or emotional),
neglect (physical or emotional), or household challenges (incarceration, mental illness,
substance dependence, parental separation or divorce and intimate partner violence).
• Providers should avoid retraumatizing patients and families in the health care by using a
trauma-informed care approach.

• Understand the effect of socioeconomic stressors on family dynamics
• Recognize the family dynamics associated with separation anxiety
Suggested Readings
• Relationship of childhood abuse and household dysfunction to many of the leading
causes of death in adults: the adverse childhood experiences (ACE) study. Am J Prev
Med, doi:10.1016/S0749-3797(98)00017-8
• Trauma-informed care. Pediatrics. doi:10.1542/peds.2021-052580
• Children exposed to adverse childhood experiences. American Academy of Pediatrics
Textbook of Pediatric Care. 2nd ed; 2017:569-575.
• Early childhood adversity, toxic stress. and the role of the pediatrician: Translating
developmental science into lifelong health. Pediatrics. doi:10.1542/peds.2016-2595
• Adverse childhood experiences, outcomes, and interventions, Pediatr Clin North Am,
doi:10.1016/j.pcl.2019.12.001
• Implementing a trauma informed approach in pediatric health care networks. JAMA
Pediatr, doi:10.1001/jamapediatrics.2015.2206

Question 222
A 15-month-old, full term, developmentally normal child is brought to the emergency
department after having a first time seizure. The girl was in her usual state of health until she
became un responsive while playing, stiffened, and had rhythmic shaking of her extremities. Her
eyes were open and staring straight ahead. The episode lasted 8 minutes before self-resolving.
Immediately afterward she was sleepy and warm to touch. There is no family history of epilepsy
and no known sick contacts, although the girl attends a daycare center. Her immunizations are up
to date. On physical examination, the girl's temperature is 39 º C, heart rate is 110 beats/min,
respiratory rate is 28 breaths/min, blood pressure is 95/58 mm Hg, and oxygen saturation is
100% in room air. She is initially sleepy but easily arousable, and shortly after arrival begins to
play with her stuffed animal. Her right tympanic membrane is erythematous, bulging, and
opacified. The remainder of her examination findings are normal. An antipyretic is administered.
Of the following, the BEST next step in this girl's management is to

A. administer intravenous lorazepam
B. administer an oral antibiotic
C. obtain a computed tomography scan of the head
D. order routine electroencephalography

Correct Answer: B
The girl in the vignette had a simple febrile seizure in the setting of acute otitis media. In the
absence of additional infectious or seizure risk factors, the best next step in this girl's
management is to treat the source of the fever, acute otitis media with an oral antibiotic.
Febrile seizures are common, occurring in 3% to 5% of all children. They are classified into 2
categories: simple and complex. For febrile seizure to be designated as simple, it must meet all of
these 5 criteria;
• Occur in a child between 6 months through 5 years of age
• Occur in a developmentally normal child
• Be generalized with no focal features
• Last less than 15 minutes
• Occur once in a 24-hour period
If all criteria are not met, the seizure is classified as complex. Appropriate classification aids in
planning the diagnostic evaluation and management of the child seen with a febrile seizure.
Management of simple febrile seizures is aimed at identifying and treating the source of fever.
Febrile seizures can occur with any illness that causes fever, although there is an increased
incidence with Shigella, Salmonella and roseola infections. Often, the seizure occurs with fever
rise, with the first recorded temperature occurring after the seizure. It is unknown if the seizure is
triggered by rate of temperature rise or the peak temperature.
In a neurologically normal child with a simple febrile seizure, routine electroencephalography

and neuroimaging are not recommended. Laboratory evaluation should be focused on the cause
of the fever rather than the seizure. If signs or symptoms of meningitis or encephalitis are
present, the seizure cannot be classified as a simple febrile seizure, and a lumbar puncture should
be performed
Complex febrile seizure management may differ depending on the reason the seizure was
categorized as complex. Identifying and treating the source of fever remains a key component of
management. However, the clinical presentation and individual child's neurological history may
support diagnostic evaluations related to the seizure itself, and can include
electroencephalography, neuroimaging, or genetic evaluation. Administration of seizures of
lorazepam should be considered for ongoing seizure activity or clusters of seizures without
return to baseline.
The risk of developing epilepsy is only slightly higher for children with simple febrile seizure
than those in the general population. Recurrence is seen in approximately one-third of children
with simple febrile seizures; it is more likely when the first seizure occurs before age 12 months.
Overall prognosis is excellent, with the exception that children will outgrow the seizure tendency
by 6 years of age. Treatment with anti-epileptic medications has not been shown to improve
long-term prognosis

PREP Pearls
• Simple febrile seizures are generalized seizures lasting less than 15 minutes, occurring
once in 24 hours, in developmentally normal children between the ages of 6 months
through 5 years. A seizure is complex if any one of these criteria is not met.
• Management of simple febrile seizures is aimed at identifying the cause of the fever.
Electroencephalography and neuroimaging are not routinely recommended
• Recurrence of simple febrile seizure is more likely when the first event occurs in a child
younger than age 12 months. The lifetime risk of epilepsy in children with simple febrile
seizure is similar to that of the general population

• Understand the difference between simple and complex febrile seizures
• Plan the appropriate evaluation and management of a febrile seizure
• Understand the risk factors associated with febrile seizures
Suggested Readings
• Seizure disorders. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed
2017; 2599-2617
• Seizures in children. Pediatr Rev, doi:10.1542/pir.2019-0134
• Febrile seizures: clinical practice guideline for the long term management of the child
with simple febrile seizures. Pediatrics. doi:10.1542/peds.2008-0939
• Febrile seizures: Guideline for the neurodiagnostic evaluation of the child with a simple
febrile seizure. Pediatrics. doi:10.1542/peds.2010-3318

Question 223
A 15-year-old adolescent boy has recurrent episodes of sneezing, runny nose, itchy eyes and
nose, and cough after cutting the grass. He has treated himself with over the-counter
antihistamines with some symptom relief, but he still wakes up each morning with nasal
congestion and sneezing. He has a strong family history of seasonal allergic rhinitis. Physical
examination reveals infraorbital edema, pale and swollen nasal mucosa with a slight bluish
appearance, and thin rhinorrhea. The posterior pharyngeal wall has a cobblestone appearance but
no visible postnasal drainage. His palpebral conjunctivae also have a cobblestone appearance.
The remainder of his physical examination findings are unremarkable.
Of the following, the pathophysiologic mechanism MOST likely responsible for this adolescent’s
findings is
A. immunoglobulin E antibody bound to eosinophils
B. immunoglobulin G antibody bound to nasal mucosal cells
C. neutrophilic infiltrate in the respiratory submucosa
D. release of histamine and leukotrienes from nasal mast cells

Correct Answer: D
The boy in the vignette has seasonal allergic rhinitis characterized by paroxysmal episodes of
sneezing, itching, and rhinorrhea after exposure to pollens to which he is sensitized. The
mechanism of allergic rhinitis involves antigen binding to antigen-specific Immunoglobulin E
(IgE) on mucosal mast cells and basophils. Bridging of 2 IgE antibodies by antigen leads to a
cascade of immunologic reactions, which release preformed mediators from mast cell granules
(histamine, tryptase) and generate leukotrienes and other inflammatory mediators from the mast
cell membrane. The secondary mediators include chemotaxins for both eosinophils and
neutrophils, but the typical allergic reaction is characterized by intense eosinophilic infiltration
of the involved area.
Eosinophils are summoned to an area of allergic inflammation, but there is no specific IgE bound
to eosinophil receptors. Immunoglobulin G antibodies are associated with the immune response
to infectious agents rather than to allergens. Neutrophilic infiltrates are more characteristic of
inflammation related to infection and nonallergic causes of rhinitis than they are of allergy,
though there are chemotaxins for neutrophils released as part of the IgE-mediated allergic
reaction.
Both seasonal and perennial allergic rhinitis are managed by identifying and avoiding allergen
triggers, as well as medications to suppress symptoms or the process leading to them. The most
effective treatment for allergic rhinitis in adolescents and adults is the use of daily topical nasal
steroids. Second-line therapy a topical nasal antihistamine, which is preferable to oral
antihistamine in this age group. Systemic corticosteroids are effective, but tong-term adverse
effects outweigh the benefits, thus they are not recommended as part of standard treatment. Oral
leukotriene antagonists have been used for both seasonal and perennial allergic rhinitis, however,
recently the US Food and Drug Administration applied a black box warning to this medication
because of concern about adverse psychiatric effects. They should only be prescribed for those
with allergic rhinitis who an inadequate response or intolerance to other approved therapies.
Differentiation of allergies from nonallergic rhinitis may be difficult. Demonstration of

sensitization to allergens suspected in symptom causation is the major tool for identifying
allergic rhinitis. Testing may be accomplished in vitro by identifying IgE antibodies, or with
percutaneous skin testing with specific antigens to elicit localized wheal and flare response
(produced by release of histamine from skin mast cells sensitized with specific IgE antibody).
However, sensitivity to allergens does not necessarily indicate that they are responsible for
symptoms, and lack of allergen identification does not fully rule out allergic rhinitis. In general.
eosinophilic infiltration in the mucosa is pathognomonic of allergic rhinitis; however there exists
a form of nonallergic rhinitis with eosinophilia, in which local eosinophils are seen on biopsy of
nasal mucosa. but no allergens can be identified as for symptoms. Nonallergic rhinitis is more
likely to be perennial, without significant seasonal variation, and characterized by nasal
obstruction rather than itching and rhinorrhea.
Recurrent acute sinusitis and, in younger children, recurrent otitis media are common
complications of allergic rhinitis. Even if allergies are not identified, allergic rhinitis should be
suspected in patients with recurrent episodes of sinusitis and otitis. Nasal polyps, though most
characteristic of cystic fibrosis, can also be associated with allergic rhinitis.
PREP Pearls
• Allergic rhinitis is characterized by sneezing, rhinorrhea, and nasal/eye/throat itching
after exposure to allergens to which the child has been sensitized.
• Topical nasal corticosteroid sprays are the preferred treatment for seasonal or perennial
allergic rhinitis
• In a child with recurrent sinusitis or otitis media, the diagnosis of allergic rhinitis should
be considered

• Understand the association between allergic rhinitis and sinusitis and/or otitis
• Recognize the common characteristics of allergic rhinitis, and manage appropriately
• Differentiate the historical and clinical findings of allergic rhinitis from those of
nonallergic rhinitis
Suggested Readings
• Treatment of seasonal allergic rhinitis: an evidence-based focused 2017 guideline update.
Ann Allergy Asthma Immunol. doi:10.1016/j.anai.2017.08.012
• Update on allergic rhinitis. Pediatr Rev, doi:10.1542/pir.26-8-284
• allergic rhinitis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017; 1701-1705
• Natural course and comorbidities of allergic and nonallergic rhinitis in children. Allergy
Clin Immunol, doi:10.1016/j.jaci.2011.09.036

Question 224
A 5-year-old boy with a history of renal dysplasia is seen for a health supervision visit. He has a
history of drinking water every few hours during the day and bed wetting every night. He is a
picky eater. The boy’s weight is 15 kg (<5th percentile) and height is 100 cm (<5th percentile).
His heart rate is 80 beats/min. respiratory rate is 16 breaths/min, and blood pressure is 100/60
mm Hg. The remainder of his physical examination findings are normal.

Blood
Urine
Nitrite Negative
Blood Negative
Protein Negative
Of the following, the MOST likely additional laboratory finding for this boy is a
A. high parathyroid hormone level
B. high serum calcium level
C. low alkaline phosphatase level
D. low serum phosphorus level

Correct Answer: A
The boy in the vignette has evidence of chronic kidney disease (CKD) secondary to renal
dysplasia. His history and findings of polydipsia, nocturnal enuresis, an inability to concentrate
urine, growth failure, uremia, anemia. and acidosis are consistent with a diagnosis of CKD. He is
also at risk for metabolic bone disease (MBD) characterized by a high parathyroid hormone
level.
The diagnosis of CKD is based on the Kidney Disease: Improving Global Outcomes (KDIGO)
2012 guidelines that require 1 of the following:
• Kidney damage for 3 months or longer, with or without a change in glomerular filtration
rate (GFR), as evidenced by structural or functional abnormalities of the kidney,
including proteinuria, tubular-interstitial injury, or abnormalities in renal imaging or
pathology
• Glomerular filtration rate less than 60 mL/min per 1,73 m2 for more than 3 months
regardless of whether other CKD markers are present
In children, the GFR is estimated using the modified Schwartz formula.

0.413 x height (cm) + serum creatinine (mg/dL)
Chronic kidney disease is classified into 5 stages (Item C224A). The boy in the vignette has
stage 3 CKD based on a GFR calculation of 34 mL/min per 1.73 m2.
Item C224A
The clinical presentation of CKD depends on the stage and underlying renal disorder. Mild CKD
(stages 1-2) may be asymptomatic. Moderate to severe CKD (stages 3-5) may present with
polyuria, polydipsia, enuresis, fatigue, anorexia, vomiting and growth failure. The various
complications observed in advanced CKD are shown in Item C224B.

Item C224B: Complications observed in advanced CKD

Metabolic bone disease, previously known as renal osteodystrophy, Is a common complication of

CKD and can result in growth failure and skeletal deformities. The inability to excrete
phosphorus and synthesize active 1,25-dihydroxyvitamin D because of decreased kidney
function leads to CKD-MBD. The low 1,25 hydroxyvitamin D level causes impaired absorption
of calcium from the intestine, resulting in a relatively low serum calcium level. The parathyroid
glands are then stimulated by the relative hypocalcemia, causing secondary hyperparathyroidism.
In the early stages of CKD, calcium and phosphorus levels may remain normal, but the
parathyroid hormone level increases as a compensatory mechanism to maintain a normal serum
calcium revel. As CKD advances, there is a high serum phosphorus level, low serum calcium
level, low 1 ,25-dihydroxyvitamin D level, and a secondary increase in parathyroid hormone
level. Serum alkaline phosphatase level, a marker of bone turnover, is elevated.

The treatment of CKD-MBD includes normalizing the serum phosphorus levels via dietary
restriction and administration of a phosphate binder (e.g., calcium carbonate), controlling
hyperparathyroidism with an active vitamin D (1.25 dihydroxyvitamin D) analog (e.g.,
calcitriol), and supplementing dietary vitamin D to normalize the 25-hydroxyvitamin D level.
PREP Pearls
• Moderate to severe chronic kidney disease may present with polyuria, polydipsia,
enuresis, fatigue, anorexia, vomiting. and growth failure.
• Metabolic bone disease, a common complication of chronic kidney disease, can result in
growth failure and skeletal deformities
• A high serum phosphorus level, low serum calcium level, and secondary increase in
parathyroid hormone level are seen in metabolic bone disease caused by chronic kidney
disease.

• Recognize laboratory abnormalities associated with chronic kidney disease
• Plan the appropriate initial management of end-stage kidney disease
• Recognize complications associated with chronic kidney disease
Suggested Readings
• KDIGO 2012 clinical practice guideline for the evaluation and management of chronic
kidney disease. Kidney Int Suppl, https://kdigo.org/wp-
content/uploads/2017/02/KDIGO_2012_CKD_GL.pdf
• Chronic kidney disease in children and adolescents. Pediatr Rev. doi:10.1542/pir.35-1-16

Question 225
A 9-day-old infant is seen for a 1-day history of red eyes and eye discharge, He was born at full
term via an uncomplicated spontaneous vaginal delivery to a mother who had limited prenatal
care. His mother refused administration of erythromycin ointment at delivery. The family has a
cat at home, and his sister was recently diagnosed with viral conjunctivitis. He has no other
symptoms. On physical examination, his growth parameters and vital signs are normal. He has
bilateral eyelid edema, purulent eye drainage, and conjunctival injection. The remainder of his
Of the following, the MOST likely cause of this neonate’s condition is

A. adenovirus infection
B. chlamydia trachomatis infection
C. neisseria gonorrhea infection
D. nasolacrimal duct obstruction

Correct Answer: B
Chlamydia trachomatis infection is the most likely cause of this neonate's purulent
conjunctivitis, The onset of signs and symptoms in the first week after birth and the purulent
nature of his conjunctivitis support this diagnosis. Neisseria gonorrhoeae conjunctivitis usually
presents earlier than C trachomatis conjunctivitis if erythromycin is not given at birth. Allergic
and adenovirus conjunctivitis are likely to manifest in this age. Nasolacrimal duct obstruction
does not produce conjunctival injection.
Neonates with purulent conjunctivitis warrant early evaluation because of the risk of systemic
infection. Chlamydia trachomatis is the most common cause of bacterial conjunctivitis in
neonates up to 4 weeks of age, followed by Streptococcus pneumoniae and nontypeable
Hemophilus influenzae. Half of neonates with C trachomatis conjunctivitis develop extraocular
infections, including pulmonary (pneumonitis), genital tract, and nasopharyngeal Infections.
Neisseria gonorrhea is a very rare cause of neonatal bacterial conjunctivitis in developed

countries. It presents early (in the first week after birth if prophylactic medication was not given
at birth) and progresses rapidly; complications include corneal perforation, septicemia, and
meningitis.
Herpes simplex virus (HSV) may also cause conjunctivitis in neonates. Skin findings may not be
present. Neonates with HSV conjunctivitis should be evaluated for disseminated HSV infection:
Herpes simplex virus may also cause conjunctivitis in older children and is usually accompanied
by a nearby rash. Because the cornea is often involved, an ophthalmologist should be consulted
urgently.
Conjunctivitis in older children is usually infectious or allergic and may be accompanied by

eyelid edema and chemosis. Signs and symptoms differentiating common causes of
conjunctivitis are listed in Item C225A. Viral infection is the most common cause of
conjunctivitis; adenovirus is the most frequently identified viral organism. Affected children may
have associated viral signs and symptoms such as fever, pharyngitis, rhinorrhea, or preauricular
lymphadenopathy. Viral conjunctivitis usually worsens during the first week of illness before
resolving in the following week. It may be accompanied by subconjunctival hemorrhages.
Complications are uncommon; they include scarring and corneal subepithelial infiltrates, for
which an ophthalmologist should be consulted.

Other viral causes of conjunctivitis include coxsackievirus and enterovirus, which may also
cause subconjunctival hemorrhage. Molluscum contagiosum of the eyelid may result in chronic
conjunctivitis that can persist for weeks after the original lesion has resolved. Measles and
varicella zoster virus are uncommon causes of conjunctivitis because of widespread vaccination.
Bacterial conjunctivitis in older children is caused by the same organisms that cause acute otitis
media, as well as staphylococcus aureus and staphylococcus epidermidis. Some cases may
include concurrent acute otitis media infection.
Bacterial conjunctivitis in older children may also be caused by N gonorrhoeae or C trachomatis

In such cases, the possibility of sexual abuse must be considered. Neisseria gonorrhea eye
infections progress rapidly and may lead to perforations. Children infected with N gonorrhoeae
may experience extraocular complications including pelvic inflammatory disease and
Chlamydia trachomatis conjunctivitis is the most common infectious cause of blindness in the
world. Manifestations include watery discharge and follicles on both conjunctiva (Item C225B)
and inner eyelid. These follicles then scar resulting in conjunctival pits that cause the eyelid to
fold inwards, allowing the eyelashes to scratch the cornea, ultimately leading to blindness.

Item C225B
Allergic conjunctivitis is almost always bilateral at the onset and may be accompanied by
extraocular symptoms such as allergic rhinitis or atopic dermatitis. Symptoms tend to occur
immediately after exposure to the allergen. Children with vernal conjunctivitis may exhibit
papillae of the inner eyelid (Item C225C) and ulceration (Item C225D), the latter of which
requires ophthalmology consultation.
Item C225C
Item C225D

Contact lenses, medications, and irritative chemicals may also cause conjunctivitis, usually
temporally related to the insult.
Conjunctivitis may also be a sign of other systemic diseases, such as cat-scratch disease,
Kawasaki disease, Stevens-Johnson syndrome, or graft-vs-host disease. In these cases, the
conjunctivitis is usually accompanied by extraocular manifestations of the respective diseases.
Ophthalmology consultation should be considered for children who experience light sensitivity,
pain, blurred vision, or persistent symptoms for more than 2 weeks.
PREP Pearls
• Neonates with bacterial conjunctivitis are most likely infected with Chlamydia
trachomatis and should be assessed for systemic infection.
• Conjunctivitis in older children is usually viral and self-limiting; complications are
uncommon, but may warrant ophthalmology evaluation
• Conjunctivitis may be 1 symptom of a systemic disease; diagnosis is aided by associated
extraocular signs.

• Recognize the clinical findings associated with conjunctivitis in patients of various ages
• Recognize the association between conjunctivitis and systemic disease
Suggested Readings
• Red eye/pink eye: American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:1559-1566.
• Conjunctivitis. Pediatr Rev. doi:10.1542/pir.31-5-196
• Conjunctivitis Preferred Practice Patterns. Ophthalmology, doi:
10.1016/j.ophtha.2018.10.020

Question 226
A previously healthy, 11-year-old boy is brought to the emergency department for nausea and
vomiting for the past day. He has had polyuria and polydipsia for the past 2 weeks. On physical
examination, the boy's temperature is 37 ºC, heart rate is 110 beats/min, blood pressure is 108/64
mmHg, respiratory rate of 26 breaths/min, and oxygen saturation is 98% on room air. His weight
is 28 kg is down 4 kg from his health supervision visit 2 months ago. He appears tired but is
interactive, taking deep breaths. and has a fruity smell to his breath. His capillary refill time is 3
seconds. The reminder of his physical examination findings are unremarkable.

Blood
Venous pH 7.1
Urine
Ketones Large
Of the following, the BEST next step in this boy’s management is to administer intravenously
A. 0.9% sodium chloride, 10 mL/kg bolus, over 1 hour
B. 0.9% sodium chloride with potassium, at twice maintenance rate
C. regular insulin, 0.1 unit/kg bolus
D. regular insulin, 0.1 unit/kg per hour

Correct Answer: A
The boy in the vignette has moderate diabetic ketoacidosis (DKA) due to a new onset diabetes
mellitus. He meets criteria for DKA with his hyperglycemia (glucose >200 mg/dL), acidosis
(venous pH <7.3 or bicarbonate <15 mmol/L), and ketonuria (ketonemia or moderate to large
ketonuria). He also has clinical and biochemical evidence of volume depletion. The best next
step in management is to administer a 10 mL/kg bolus of 0.9% normal saline intravenously over
1 hour.
Current guidelines recommend volume resuscitation with isotonic saline as the first step in the
management of DKA. The role of initial fluid therapy, specifically greater volume and more
rapid administration, in the development of cerebral edema remains unclear. Therefore,
guidelines recommend more conservative volumes for the initial resuscitation: 10 mL/kg over 30
to 60 minutes for those with evidence of volume depletion and 20 mL/kg as quickly as possible
for those in shock. These boluses should be repeated until adequate perfusion is achieved.
An infusion of 0.9% sodium chloride with potassium at twice maintenance rate would be
appropriate for subsequent fluid management. The estimated fluid deficit should be calculated
and replaced evenly, along with maintenance requirements, over 24 to 48 hours. This fluid
should have a tonicity of 0.45% to 0.9% (sodium chloride) and contain 40 mEq/L of potassium
given the total body potassium depletion that occurs in DKA. Twice maintenance is a reasonable
rate for subsequent fluid management
A continuous infusion of regular insulin at 0.1 unit/kg per hour (guidelines recommend 0.05-0.1
unit/kg per hour) is indicated after at least 1 hour of fluid therapy. Earlier administration of
insulin is associated with a higher risk or cerebral edema. Insulin is required to correct the
acidosis and clear the ketones. The hyperglycemia, however, inevitably corrects before the
acidosis. Therefore, until the acidosis corrects, additional dextrose should be administered rather
than decreasing the insulin infusion rate.
An initial bolus of intravenous insulin should not be given. It is unnecessary, may increase the
risk of cerebral edema, and can worsen hypokalemia. Furthermore, unless severe hyperkalemia
or severe acidosis (venous pH <6.9) is present, sodium bicarbonate should not be given because
it is associated with an increased risk of cerebral edema
Cerebral edema is the major complications of DKA. While symptomatic cerebral edema is rare,
it is associated with significant morbidity and mortality. The etiology of cerebral edema remains
unclear, but risk factors include severe dehydration and acidosis at presentation, bicarbonate
administration, high volumes of fluid given in the first 4 hours, an insulin administration in the
first hour of fluid treatment. A proposed mechanism is dehydration and cerebral hypoperfusion
causing brain injury that worsens during DKA treatment. However, the role of specific treatment
factors has not been clearly elucidated.
Diabetic ketoacidosis can occur as the initial presentation of diabetes mellitus or in those with
existing diabetes. The most common cause of recurrent DKA is insulin omission. Common
symptoms of DKA include polyuria, polydipsia, fatigue, weight loss, vomiting, abdominal pain,
and rapid breathing. Symptoms can mimic gastroenteritis, viral syndromes (e.g., influenza), an
acute abdomen, pneumonia, or asthma. Thus, a high index of suspicion for diabetes is necessary
to avoid delayed diagnosis.
PREP Pearls
• Ahigh index of suspicion for diabetes mellitus and diabetic ketoacidosis is necessary to
avoid delayed diagnosis; symptoms can mimic gastroenteritis, viral syndromes, an acute
abdomen, pneumonia, or asthma.
• The first step in the management of diabetic ketoacidosis is volume resuscitation with
isotonic saline 10 mL/kg over 30 to 60 minutes for those with evidence of volume
depletion and 20 mL/kg as quickly as possible for those in shock
• Treatment of diabetic ketoacidosis with a continuous infusion of regular insulin at 0.05-
0.1 unit/kg per hour is indicated after at least 1 hour or fluid therapy.

• Recognize the complications associated with diabetic ketoacidosis
• Plan the appropriate management of diabetic ketoacidosis
• Recognize the risks associated with fluid and electrolyte therapy in patient with diabetic
ketoacidosis
• Recognize the clinical and laboratory features with diabetic ketoacidosis
• Understand the causes of recurrent diabetic ketoacidosis
Suggested Readings
• Diabetic ketoacidosis. Pediatr Rev, doi:10.1542/pir.2018-0231
• Diabetic ketoacidosis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed. 2017;2813-2819
• Diabetic ketoacidosis and the hyperglycemic hyperosmolar state. Pediatr Diabetes,
doi:10.1111/pedi.12701

Question 227
A 3-year-old boy is seen for a 14-day history of intermittent, watery diarrhea without blood or
mucus. The stools smell foul and appear greasy. He also has low-grade fever, anorexia, weight
loss, and occasional bouts of abdominal pain and emesis. He attends daycare. He has not been
exposed to pets or traveled internationally. No other family members have had diarrhea. The
boy's temperature is 37 º, and his remaining vital signs are normal. His physical examination
findings are significant only of mild dehydration and abdominal distension.
Of the following, the MOST likely cause of this boy’s illness is

A. Campylobacter jejuni
B. enterotoxigenic Escherichia coli
C. giardia duodenalis
D. norovirus

Correct Answer: C
The boy in the vignette, with a history of prolonged diarrhea associated with weight loss, low
grade fever, and abdominal distention has a clinical picture suggestive of giardiasis. Giardiasis is
caused by Giardia Duodenalis (formerly Giardia lamblia and Giardia intestinalis). a flagellated
protozoan that infects the small intestine and the biliary tract. Giardia cysts are the infective form
of the parasite, they are viable in the environment for prolonged periods. Human-to-human
infection occurs via fecal-oral transmission through consumption of contaminated water or food
products, or exposure to animals in petting zoos or farms. The infection cycle begins with
ingestion of cysts. Excystation in the small intestine results in the replicating trophozoites. which
attach to the small intestinal epithelium, causing inflammation, flattening of villi, and chronic
diarrhea due to malabsorption. Encystation then occurs in the large intestine, resulting in release
of infective cysts in the stool.
Common risk factors for giardiasis include daycare center attendance, foreign travel, exposure to
animals, and sexual transmission (i.e., men having sex with men). Outbreaks of giardiasis can
occur in children attending daycare centers. Community-wide outbreaks due to contaminated
drinking or recreational water have been reported. Children under age of 5 years and adults aged
45 to 49 years have a higher incidence of giardiasis. The disease is more common during the late
summer and early fall months in the United States.
The incubation period of giardiasis ranges from 1 to 3 weeks. Infection can be asymptomatic.
Symptomatic infection may manifest as acute, watery diarrhea or chronic diarrhea with
malnutrition. Compared with adults, symptomatic disease is more common in children. Giardia-
associated diarrheal illness is accompanied by nausea, vomiting. abdominal cramps, malaise, loss
of appetite, bloating, and mild elevations in temperature. The stools are typically explosive, foul-
smelling, and greasy in nature: the presence of blood is rare. Malnutrition due to malabsorption
is a frequent occurrence in protracted illness. Intestinal lactase deficiency may complicate the
course of illness. Immunocompromised individuals (e g., humoral immunodeficiencies, acquired
immune deficiency syndrome) may develop chronic giardiasis. Giardiasis is also more prevalent
in individuals with cystic fibrosis. Rare extraintestinal manifestations of giardiasis may include
antritis, biliary tract disease or chronic pancreatitis.
The diagnosis of giardiasis is made by detection of Giardia antigen by enzyme immunoassay

(EIA) or direct fluorescence antibody (DFA) assay testing of stool samples. The EIA assay is
highly sensitive (95%) and specific (98% to 100%). The of giardiasis by ova and parasite
detection on stool microscopy is being replaced by these antigen-based assays and polymerase
chain reaction testing. In addition to Giardia antigen, both EIA and DFA testing of stool samples
can Cryptosporidium species. Most health care settings in the United states also have access to
multiplex polymerase chain reaction tests, which can detect a variety of intestinal pathogens on a
stool sample including giardia duodenalis.
Given the self-limited nature of giardiasis, antiparasitic therapy may not be indicated. Indications
for treatment include acute or chronic diarrhea associated with failure to thrive, malabsorption
syndrome, extraintestinal disease; or infection in immunocompromised hosts. Oral antiparasitic
treatment options are dependent on age. These include: tinidazole (single dose) for children aged
>3 years, nitazoxanide (3 day course) for children aged 1 year or older, and metronidazole (5 to 7
day course). Symptoms may recur after treatment due to factors such as poor adherence,
reinfection, drug resistance, lactose intolerance, or underlying immune defect.
Infection control measures for hospitalized children with giardiasis include contact precautions.
Meticulous hand hygiene by staff and children, particularly white changing diapers, helps
prevent outbreaks of giardiasis in child care centers. In child care-associated outbreaks, treatment
is recommended for all symptomatic individuals. Infected individuals should not attend the
center until asymptomatic and based on the recommendation of the local public health
department.
Diarrhea caused by Campylobacter species is usually bloody and the result of ingestion of
contaminated food. While watery diarrhea is associated with enterotoxigenic Escherichia coli
infection, it primarily affects infants residing in resource-limited countries and is a common
cause of travelers’ diarrhea in all age groups. Gastroenteritis due to norovirus is characterized by
sudden onset of emesis followed by watery diarrhea, nausea, and abdominal pain; the illness lasts
for 1 to 5
PREP Pearls
• Giardiasis is caused by Giardia duodenalis, a flagellated protozoan that infects the small
intestine and the biliary tract; human infection occurs through fecal. transmission by
ingestion of contaminated water or food products and exposure to animals.
• Common risk factors for giardiasis include daycare center attendance, travel, exposure to
animals, and sexual transmission (i.e., men having sex with men),
• Giardiasis is characterized by chronic diarrhea associated with nausea, vomiting, low-
grade fever, anorexia, and weight loss; the stools are typically explosive, foul-smelling
and greasy in nature.
MOCA-Peds Objective

• Understand the epidemiology of Giardia duodenalis
• Plan the appropriate diagnostic evaluation when Giardia duodenalis infestation is
suspected
• Recognize the clinical features associated with Giardia duodenalis infestation, and
Suggested Readings
• Giardia duodenalis infections. Red Book: 2021-2024 Report of the Committee on
• Giardiasis. BMJ. Doi:10.1136/bmj.i5369
• Giardiasis surveillance-United States. MMWR Surveill Summ
• Giardiasis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. 2017;
2094-2097

Question 228
A 2-month-old boy is seen for his health supervision visit. He was diagnosed with
phenylketonuria through the newborn screening program. Last month the parents met with
the metabolic geneticist and were educated and counseled on the diagnosis. They were provided
with special formula and feeding instructions based on the protein content. In the clinic today,
the patient looks healthy and is growing well. The parents seem overwhelmed with the recent
diagnosis and ask until what age their child will need to be on a special diet.
Of the following, the MOST accurate response is

A. 2 years
B. 5 years
C. 15 years
D. lifelong

Correct Answer: D
Phenylketonuria (PKU) is a disorder of amino acid metabolism due to a deficiency in the liver
enzyme phenylalanine hydroxylase (PAH), This deficiency leads to accumulation of the
substrate phenylalanine (Phe) and reduction in the tyrosine (Tyr).
Sequelae of untreated PKU Include:

• Progressive, irreversible, neurological impairment (brain magnetic resonance imaging
shows progressive white matter disease)
• Severe intellectual disability (IQ ≤50)
• Seizures
• Behavioral issues (e.g., aggression, tantrums, stereotypy, anxiety, social withdrawal)
• Pyramidal signs (e.g., involuntary movements)
• Musty body odor (resulting from excretion of phenylacetic acid)
• Skin: decreased hair pigmentation (due to low levels of tyrosine and subsequent low
melanin)
• Eczema
Adults are usually in a group home setting and are unable to carry out activities of daily living.
Infants born to mothers with poorly controlled PKU may have intrauterine growth restriction,
microcephaly, congenital cardiac defect, limb malformation, or brain malformation.
Phenylketonuria was one of the first disorders included in the United States newborn
Screening program and is the biggest success story attributable to the program. The biochemical
markers for PKU on newborn screening are an elevated Phe level, low Tyr level, and a high Phe-
to-Tyr ratio, Phenylalanine hydroxylase deficiency can be detected in virtually 100% of cases
based on these markers. Confirmatory testing involves measurement of plasma amino acid levels
and identification of 2 disease-causing changes in the PAH gene.
Management of PKU:
• Treatment should be initiated as soon as possible after birth with a low-protein diet,
including a Phe-free medical formula, under the guidance of an experienced metabolic
dietician
• Include Phe ingested through breast milk and formula when calculating total dietary
intake
• Target blood Phe level is 120-360 µmol/L (2-6 mg/dL) throughout the lifespan of the
individual
• Blood Phe level should be checked weekly until age 1 year, then every other week until
age 13 years, and then monthly thereafter
• Phe should be monitored closely during times of increased anabolism: infancy,
childhood, and pregnancy
• Tetrahydrobiopterin (BH14) is a cofactor of the enzymatic reaction converting Phe to
Tyr. Some patients achieve better control of their Phe level when this cofactor is added to
their diet as an adjuvant therapy.
• Maintain blood Tyr in the normal range

• Monitor and supplement other nutrients as needed, such as vitamin D, iron, vitamin B,
and calcium
• Avoid aspartame, an artificial sweetener that contains a high amount of Phe
Previously it was thought that a protein-restricted, Phe-free diet could be stopped when an
affected individual reached adulthood; later studies showed that this resulted in mental health
deterioration and cognitive decline. Hence, lifelong dietary management is recommended.
Pegvaliase is an injectable recombinant form of phenylalanine ammonia lyase, a Phe-
metabolizing enzyme approved in the United States in 2018 for use in adult PKU patients with
high Phe levels who have discontinued existing treatment options. Blood Phe levels were found
to be reduced by approximately 50% to 70%.
PREP Pearls
• Phenylketonuria leads to high blood phenylalanine and low tyrosine levels. These are
used as markers for the newborn screen.
• Dietary management with low-protein, phenylalanine-free formula should be initiated as
soon as possible after birth under the guidance of an experienced metabolic dietician.
• Blood phenylalanine levels should be maintained between 120-360 µmol/L(2-6 mg/dL)
throughout the life of the patient.

• Recognize the clinical features associated with phenylketonuria
• Understand the natural history of treated and untreated phenylketonuria
• Understand the long-term prognosis for patients who have phenylketonuria including the
importance of dietary adherence
Suggested Readings
• Phenylalanine hydroxylase deficiency. Gene Reviews.
• Inborn errors of metabolism (metabolic disorders), Pediatr Rev. doi:10.1542/pir.2014-
0122
• Specific congenital metabolic diseases. American Academy of Pediatrics Textbook of
Pediatric Care. 2nd ed. 2017;938-962

Question 229
A 14-year-old adolescent girl is evaluated in the emergency department for a fever, intermittent
rash, and joint pain. Her fever, with a maximum temperature of 39.4 º C, started 4 days ago. Her
pink-colored rash comes and goes over the trunk and extremities. She has pain in her hips and
shoulders and has difficulty ambulating.
The girl responds appropriately to questions. On physical examination, temperature is 38.9 ºC,
heart rate is 110 beats/min, respiratory rate is 20 breaths/min, and oxygen saturation is 98% on
room air. She appears uncomfortable. There is a faint pink rash over her thighs and chest, and
swelling over her knees. She has pain when standing and walking. Her liver is felt 3 cm below
the costal margin, there are no masses, and her spleen is not palpable.

White blood cell count 10,000/µL (10 x 109/L)
Platelet count 400 x 103 /µL (400 109/L)
C-reactive protein 20 mg/dl (200 mg/L)
Ferritin 1,000 ng/ml (1,000 µg/L) (reference range,
15-77 ng/ml)
Alanine aminotransferase 47 U/L
Aspartate aminotransferase 51 U/L
D-dimer 0.4 µg/mL (2.2 nmol/L) reference range
<0.5 µg/mL
Of the following, this child's MOST likely diagnosis is

A. juvenile idiopathic arthritis
B. leukemia
C. macrophage activation syndrome
D. neuroblastoma

Correct Answer: A
The girl in the vignette has systemic juvenile idiopathic arthritis (JIA) based on her fever, joint
findings, elevated inflammatory markers, and rash. Leukemia is unlikely given her normal
complete blood count results; this could be ruled out with a bone marrow aspirate and biopsy.
Her normal white blood cell count; normal alanine aminotransferase, aspartate aminotransferase,
and D-dimer levels; and ferritin level <5,000 ng/mL do not meet the criteria for the diagnosis of
macrophage activation syndrome. However, as this can develop overtime, these tests should be
repeated if her signs and symptoms do not improve with treatment. Fever, fatigue, and swelling
of the lower extremities secondary to a mass pressing on the blood vessels or lymphatic vessels
can occur with neuroblastoma, but the lack of abdominal mass or other signs or symptoms
suggestive of this diagnosis such as ptosis, bruising around the eyes, or lymphadenopathy make
this less likely.
Juvenile idiopathic arthritis is a clinical diagnosis. It has a 14/100,000 incidence and 113/100,000
prevalence in the United States. Juvenile idiopathic arthritis can be classified into subgroups
including oligoarticular, polyarticular, and systemic forms.
Oligoarticular JIA involves <5 joints; diagnosis requires the presence of symptoms for at least 6
months. (Some children will develop symptoms in additional joints later in life.) It is most
common in girls aged 2 to 4 years. Symptoms can be difficult to identify at first. There may be
difficulty walking, limping, or fussiness early in the morning or after longer periods of inactivity.
Symptoms generally improve throughout the day with activity. The knees are the most
commonly affected joints. It is important to obtain an antinuclear antibody (ANA) test. which
will be positive in about 70% of affected children: these children are at increased risk of uveitis
and require ophthalmology screening every 3 months. Results of other laboratory tests such as
complete blood count, C-reactive protein level, and erythrocyte sedimentation rate are often
normal. Children with oligoarticular JIA generally have a good prognosis.
Polyarticular JIA involves > 4 joints within the first 6 months of symptoms. This subgroup is
further divided into rheumatoid factor (RF) positive or negative forms. A positive RF should be
repeated after 3 months, as this me be transient finding. Rheumatoid factor is positive in
approximately 10% of children with JIA. Polyarticular JIA has bimodal peak, most commonly
affecting children 1 to 3 years of age and adolescents.
Systemic JIA has a more acute onset and does not require 6 months of symptoms for diagnosis.
Signs and symptoms include fevers, a rash which is typically pink to salmon colored and
transient, arthritis/arthralgias, and sometimes hepatomegaly. The differential diagnosis includes
leukemia, lymphoma, neuroblastoma, and Ewing sarcoma; these conditions should be excluded
with laboratory evaluation, imaging studies, and bone marrow biopsy as appropriate.
A complication occurring in up to one-third of children with systemic JIA is macrophage

activation syndrome, a secondary form of hemophagocytic lymph histiocytosis. Characteristic
signs and symptoms include persistent fever, lethargy, seizures, and a rash that is fixed rather
than transient. Laboratory findings include leukopenia, thrombocytopenia; transaminitis; and
elevated D-dimer, ferritin (>5,000 ng/mL) and triglyceride levels. Hemophagocytosis can often

be found on bone marrow aspirate and biopsy. These children should be treated promptly as they
are at significant risk for severe illness or even death.
PREP Pearls
• Systemic juvenile idiopathic arthritis is a diagnosis based on clinical findings and
exclusion of alternative diagnoses.
• Among children with juvenile idiopathic arthritis, approximately 70% are antinuclear
antibody positive and approximately 10% are rheumatoid factor positive.
• Children with juvenile idiopathic arthritis are at increased risk of uveitis, especially those
who are antinuclear antibody positive.
• Recognize the clinical features of juvenile idiopathic arthritis

• Recognize the laboratory findings associated with juvenile rheumatoid (idiopathic)
arthritis and its complications
• Recognize the clinical findings associated with the various types of juvenile rheumatoid
(idiopathic) arthritis
Suggested Readings
• Juvenile idiopathic arthritis changing times, changing terms. changing treatments. Pediatr
Rev, doi:10.1542/pir.2016-0148
• Juvenile idiopathic arthritis. American Academy of Pediatrics Textbook of Pediatric
Care. 2nd ed; 2017:2578-2586

Question 230
A 13-year-old, previously healthy boy was seen in the office 2 days ago for a 3-day history of
severe sore throat and fever. His physical examination findings at that time were significant for a
temperature of 38.5 ºC, exudative tonsillitis, and bacterial tender cervical lymphadenopathy. A
rapid streptococcal antigen test result was positive and he was treated with oral amoxicillin.
Today, he feels much better.
Laboratory results from 2 days ago are shown.

Neutrophils 85%
Lymphocytes 11%
Monocytes 4%
Atypical lymphocytes 1%
Rapid streptococcal antigen Positive
Heterophile antibody spot test for Negative
infectious mononucleosis
Throat culture Group A streptococcus
Epstein-Barr virus Serology Positive
On further inquiry, the boy’s mother recalls that 3 years ago he had a week-long febrile illness
with tonsillitis, hepatosplenomegaly, fatigue.
Of the following, this boy’s serology MOST likely shows

Viral Capsid Viral Capsid Early Nuclear
Antigen IgG Antigen IgM Antigen Antigen
A Positive Positive Positive Negative
B Positive Negative Negative Negative
C Positive Negative Negative Positive
D Negative Negative Negative Negative

Correct Answer: C
The boy in the vignette has acute group A Streptococcus pharyngitis with typical symptoms and
signs (e.g., sore throat, fever, tender cervical lymphadenopathy) and laboratory evidence of
group A Streptococcus infection. A negative heterophile antibody spot test for infectious
mononucleosis and positive Epstein Barr virus (EBV) serology are not unexpected in this
adolescent with a history suggestive acute infectious mononucleosis 3 years ago. The serologic
pattern consistent with a past EBV infection is a positive viral capsid antigen (VCA), negative
VCA IgM, negative early antigen, and positive nuclear antigen.
The EBV serologic profile consists of 4 antibodies (VCA IgG, VCA IgM, early antigen, and
nuclear antigen). The pattern antibody response can help distinguish acute or recent from past
infection.
In most infectious diseases, IgG elevation is regarded as an indication of past infection.

However, the EBV VCA IgG rises early in the course of acute infection and stays elevated for
life; it is therefore not helpful in differentiating acute from past infection. The presence of high
titers of EBV nuclear antigen (EBNA) usually excludes an active/acute infection and would be
an expected finding for the child in the vignette. Findings of a positive VCA IgM with negative
nuclear antigen results suggest an active or recent infection. The absence of VCA IgG excludes a
previous history of EBV infection; however, occasionally in the early stages of an acute
infection, there is a delay in the rise of VCA IgG (by 1 to 2 weeks).
The heterophile antibody spot test for infectious mononucleosis detects heterophile IgM that is
produced by EBV-infected B cells but is not specific to EBV. It is a rapid test commonly used in
office and emergency department settings. The heterophile test is positive-in 80% to 90% of
affected children and adolescents during 2 weeks of illness. However, it is only 50% sensitive in
children younger than 4 years. Item C230A reviews the findings in an acute, past, and no EBV.
Reactivation is uncommon in immunocompetent children and is unreliable in diagnosing
reactivation.
Item C230A
Epstein-Barr virus, a human herpesvirus 4, is one of the most common childhood infections.
Epstein-Barr virus antibodies are present in 70% to 90% of children younger than 5 years from
low socioeconomic groups and 40% to 50% of children from high socioeconomic groups.
Transmission is through oral contact with saliva. The incubation period ranges from 2 to 6
weeks. Epstein-Barr virus causes a spectrum of clinical manifestations, of which infectious
mononucleosis is the most common. Infectious mononucleosis is characterized by fever,
exudative pharyngitis, hepatosplenomegaly, and an increased proportion of atypical lymphocytes
in the peripheral blood smear. The boy in the vignette has a normal percentage of atypical
lymphocytes. Epstein Barr virus infection in infants and young children may be asymptomatic or
atypical, presenting with otitis media, a brief febrile illness, an upper respiratory tract infection.
A morbilliform rash (Item C230B) may develop in children with acute EBV infection treated
with amoxicillin, ampicillin, or other penicillin.
Item C230B
Hematologic complications of EBV infection such splenic rupture, thrombocytopenia, hemolytic

anemia, and hemophagocytic lymph histiocytosis are rare. Primary EBV infection can have
associated central nervous system manifestations such as aseptic meningitis, transverse myelitis,
and Guillain-Barré syndrome. Epstein-Barr virus associated lymphoproliferative disorders can be
seen in immunocompromised children, particularly those with human immunodeficiency virus
infection or after transplantations. In the rare but severe disorder, X-linked lymphoproliferative
syndrome, an exaggerated response to EBV occurs in individuals with an abnormality in the
lymphocyte signaling pathway (SH2D1A gene mutation).
PREP Pearls
• Epstein-Bart virus, a human herpesvirus 4, is the most common cause of infectious
mononucleosis in children and adolescents. It is associated with rare complications such
as splenic rupture and hemophagocytic lymph histiocytosis.
• A serologic profile of antibodies can help differentiate an acute primary Epstein-Barr
virus infection from past infection (viral capsid antigen IgG, viral capsid antigen IgM,
early antigen, and nuclear antigen).
• The presence of Epstein-Barr virus nuclear antigen (EBNA) excludes acute primary
infection; antibodies against EBNA develop several weeks to months after an acute
infection.

• Recognize the potential complications of Epstein-Barr virus infection in normal and
immunocompromised children of various ages
• Assess the results of laboratory evaluation of a patient in whom Epstein-Bart virus
infection is suspected, including the ability to distinguish between acute and past
infection
• Identify the clinical features associated with Epstein-Batt virus infection in normal and
immunocompromised children of various ages
• Understand the significance of a rash following ampicillin therapy in patients with
infectious mononucleosis
Suggested Readings
• Epstein-Batt virus infection. Red Book: 2021-2024 Report of the Committee on
infectious Diseases. 32nd ed; 2021:318-321
• Epstein-Barr virus. Feigin and Cherry's Textbook of Pediatric Infectious Diseases. 7th ed;
2014:1992-2016.
• Epstein-Barr virus. Pediatr Rev, doi:10.1542/pir.32-9-375
• Epstein-Barr virus (infectious mononucleosis and lymphoproliferative disorders).
Principles and Practice of Pediatric Infectious Diseases. 2018;1088-1095
• Infectious mononucleosis and other Epstein-Barr Viral infections. American Academy of
Pediatrics Textbook of Pediatric Care. 2nd ed; 2017:2194-2199

Question 231
A 4-year-old girl is evaluated in the emergency department for severe headache and 2 episodes
of emesis at home. The parents administered a weight-appropriate dose of acetaminophen prior
to arrival without any improvement in her headache. The girl has a 3-week history of intermittent
headaches. There is no history of head trauma, vision changes, fever, neck pain, or gait change.
She has not had any recent illness and was in her normal state of health prior to the onset of
headaches. She was born prematurely at 29 weeks' gestation, requiring a 6-week stay in the
neonatal intensive care unit, and has mild, intermittent asthma.
The patient appears well developed and in moderate discomfort. Ocular/fundoscopic and
neurologic examination findings are normal. Vital signs include a temperature 37 º C, heart rate
of 115 beats/min, respiratory rate of 22 breaths/min, blood pressure of 187/105 mm Hg, and
oxygen saturation of 97% on room air. Brain computed tomography results are normal. Shortly
after the child returns to the emergency department after her scan she has a generalized tonic-
clonic seizure that lasts approximately 4 minutes.
Of the following, the MOST appropriate next step in this child's treatment is
A. hydralazine intravenously
B. labetalol orally
C. nifedipine orally
D. sodium nitroprusside intravenously

Correct Answer: D
The child in the vignette is experiencing a hypertensive emergency that would be best treated
with sodium nitroprusside, intravenously. Hypertension in children is defined as the systolic or
diastolic blood pressure above the 95 percentiles for age, height, and sex. Hypertensive crisis can
be classified as either a hypertensive urgency or hypertensive emergency. A hypertensive
emergency exists when the blood pressure is significantly elevated for age and there is evidence
of end-organ damage, usually involving the central nervous, renal, or cardiovascular systems.
This child’s seizure is evidence of end-organ damage. A hypertensive urgency exists with a
severely elevated blood pressure for age without evidence of end organ damage. If a
hypertensive urgency is left untreated, however, it will result in end-organ damage.
Sodium nitroprusside, given intravenously is a very potent vasodilator (arterial and venous) with
instantaneous onset of action with infusion that can be titrated as a continuous infusion. Sodium
nitroprusside has very little effect on cardiac output and does not cause reflex tachycardia.
Hydralazine is a less potent arteriolar vasodilator than sodium nitroprusside. Its onset of action is
30 minutes and it can cause reflex tachycardia, which makes it a less desirable choice to treat this
child's hypertensive emergency.
Labetalol, an α1 and non-selective ß-adrenergic blocker, can be used in hypertensive

emergencies. However, it has a delayed onset of action of 5 to 10 minutes and is contraindicated
in patients with asthma, heart block, and heart failure. The child in the vignette has a history of
asthma, therefore labetalol should not be given as first-line anti-hypertensive medication.
Additionally, intravenous, not oral, labetalol would be the preferred route of administration in a
child with a hypertensive emergency.
Nifedipine is a calcium channel blocker that reduces peripheral vascular resistance.

Its onset of action is 30 minutes, making it less desirable to treat hypertension in a child with
symptoms of acute end-organ damage. Of note, nifedipine is also contraindicated in any patient
with intracerebral hemorrhage.
Hypertension and hypertensive crisis in children are usually secondary to an endocrine, renal, or
cardiovascular disorder, although primary hypertension in children is becoming more common
due to childhood obesity. Item C231 lists common causes of hypertensive emergencies by age
group.
In the case of a hypertensive emergency the goal of treatment is to lower blood pressure without
delay, but gradually. A sudden, dramatic decrease in blood pressure can lead to neurologic
complications such as intracranial hemorrhage. Blood pressure should be lowered by no more
than 25% over the first 8 hours of therapy and then gradually normalized over the next 24 to 48
hours

Item C231: Causes of Hypertensive Emergencies Based on Age
PREP Pearls
• Hypertensive emergency is distinguished from hypertensive urgency by the presence of
acute end-organ dysfunction.
• Sodium nitroprusside is a potent arteriolar and venous vasodilator with instantaneous
onset of action that is easily titratable as a continuous infusion; it is a good choice for the
treatment of a hypertensive emergency.
• Treatment of a hypertensive emergency should lower the blood pressure by 25% or less
over the first 8 hours and then gradually normalize the blood pressure over the next 24 to
48 hours.

• Recognize the signs and symptoms of a renal hypertensive emergency, and manage
appropriately
Suggested Readings
• The emergency management of severe hypertension. Pediatr Nephrol,
doi:10.1007/s004670050787
• Diagnosis, epidemiology, and management of hypertension in children. Pediatrics doi:
10.1542/peds.2015-3616
• Hypertensive emergencies. American Academy of Pediatrics Textbook of Pediatric Care.
2nd ed; 2017:2878-2881
• Hypertensive emergencies. Lancet, doi:10.1016/S0140-6736(00)02539-3
• Hypertension in children and adolescents. Pediatr Rev, doi:10.1542/pir.2016-0106

Question 232
16-year-old adolescent girl is evaluated in the office for left knee pain that started a couple of
days ago. She has no history of trauma or known injury. The pain improves with rest and is
exacerbated by walking. She had right knee pain a few days prior that improved with ibuprofen.
She has otherwise been well aside from an urgent care visit for an upper respiratory infection
with a sore throat a few weeks ago that has respiratory since resolved. On physical examination,
her heart rate is 120 beats/min, respiratory rate is 15 breaths/min, blood pressure is 100/50 mm
Hg, and oxygen saturation by pulse oximetry is 97% in room air. Her auscultatory examination
demonstrates bilaterally clear breath sounds and a normal S1, physiologically space split S2 with
2/4 blowing diastolic murmur noted best at the second intercostal space with radiation to the
right upper sternal border. This murmur has not been noted before. Her left knee is painful with
range of motion, red, and slightly swollen. She has a pink rash on her back with a clear center
that blanches on palpation. The girl is up to date on immunizations and has no allergies.
Of the following, after resolution of the acute symptoms, the BEST long-term medication to treat
this adolescent's condition is
A. intramuscular ceftriaxone monthly
B. intramuscular penicillin G monthly
C. oral amoxicillin daily
D. oral azithromycin daily

Correct Answer: B
The adolescent in the vignette has polyarthritis, carditis, and a red rash with central clearing after
a recent sore throat. She has a new murmur that is consistent with aortic regurgitation. Her
history and physical examination findings are Consistent with acute rheumatic fever (ARF) for
which she should receive long-term treatment with intramuscular penicillin G. There is no role
for ceftriaxone, amoxicillin, or azithromycin in the long-term chemoprophylaxis of rheumatic
fever.
Acute rheumatic fever presents 2 to 4 weeks after a group A streptococcus pharyngitis or skin
infection. The Jones' Criteria for the diagnosis acute rheumatic fever were revised in 2015, with
the addition of echocardiographic data. The diagnosis is made when there is evidence of
preceding streptococcal infection and either 2 major manifestations or l major manifestation as
well as 2 minor manifestations are present and is dependent upon the risk of disease within the
population. Clinical manifestations that lead to a diagnosis are listed in (Item C232A). More
detail is available in Gewitz MH et al. The adolescent in the vignette is from a low-risk
population and has 3 major criteria.
Item C232A

Upon presentation, a child with suspected ARF should undergo laboratory evaluation including:
• Rapid strep antigen
• Throat culture
• Anti-DNAse B titer
• Ant streptolysin O titer
• Inflammatory markers (erythrocyte sedimentation rate and/or C-reactive protein level)
• Electrocardiography (to assess PR and Other conduction intervals)
The arthritis in rheumatic fever is usually relieved with nonsteroidal antiinflammatory

medications. Some patients will have symptoms of heart failure which may require treatment
with diuretics and/or afterload reduction and possibly admission to the hospital. Eradication of
the streptococcal infection is needed, followed by long-term secondary chemoprophylaxis (Item
C232B). Typically, monthly injections of penicillin G benzathine aro required for 5 to 10 years
depending on the presence or absence of carditis and residual cardiac disease.

PREP Pearls
• The revised Jones Criteria lists the major and minor criteria required for the diagnosis of
acute rheumatic fever from populations at low or moderate to high risk. Diagnosis is
made with either 2 major, 1 major and 2 minor. or 3 major criteria
• Evidence of prior group A Streptococcus infection is required for diagnosis of acute
rheumatic fever, unless chorea is present.
• After acute treatment of acute rheumatic fever is completed, long-term chemoprophylaxis
is needed to prevent recurrence; Penicillin G benzathine intramuscular monthly.
penicillin V orally twice daily. or sulfadiazine or sulfisoxazole orally once daily are
recommended.

• Understand the natural history of rheumatic fever
• Recognize the clinical findings with rheumatic fever, including major and minor criteria
Suggested Readings
• Group A streptococcal infections. Red Book: 2021-2024 Report of the Committee on
• Rheumatic fever. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:2572-2578
• Revision of Jones Criteria for diagnosis of acute rheumatic fever in the era of Doppler
echocardiography. AHA Scientific Statement. Circulation, doi:
10.1161/CIR.0000000000000205
• Group A acute rheumatic fever and rheumatic heart disease: Epidemiology and clinical
considerations. Curr Treat Options Cardiovasc Med, doi:10.1007/s11936-017-0513-y

Question 233
An adolescent girl is seen for a health supervision visit. Her 15th birthday is tomorrow. She has
no significant medical history and is healthy. She has received all of her routine childhood
vaccines with the exception of the human papillomavirus (HPV) vaccine. Although she and her
parents are amenable to the vaccine, she has a softball game today and would like to delay the
immunization until her next visit because she is worried that the injection site will be sore.
Of the following, the MOST accurate statement regarding this adolescent’s immunization
schedule is that she will need
A. 2 doses if the first dose is given today and 3 doses if she delays
B. 2 doses, regardless of the timing of the firs dose
C. 3 doses of the quadrivalent vaccine or 2 doses of the 9-valent Vaccine
D. 3 doses, regardless of the timing of the first dose

Correct Answer: A
The adolescent in the vignette would need to receive 2 doses of the human papillomavirus (HPV)
vaccine if she starts the vaccine series today, but would need 3 doses if she delays the vaccine
until her 15th birthday or after. Two doses are required if the vaccine series is started before the
15th birthday and 3 doses if it started after the 15th birthday, regardless of the HPV vaccine type
(i.e., quadrivalent ot 9-valent).
Human papilloma viruses are double-stranded DNA viruses that can cause asymptomatic and
self-resolving infection or can lead to warts on the skin or mucus membranes. There are
numerous types; types 6 and 11 cause condylomata acuminata but are not associated with
malignancy, and types 16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 58, 59, 66, and 69 are known as
“high-risk” types that can cause cervical cell changes and are precursors to cervical, anogenital,
and oral cancers. Types 16 and 18 are associated with 70% of cancer cases.
The quadrivalent HPV vaccine (4vHPV [types 6, 1 1, 16, and 18]) was licensed in the States in
2006 for females aged 9 to 26 years and in 2009 for males aged 9 to 26 years. Although it is still
licensed it is no longer available in the United States and was replaced by the 9-valent vaccine
(9vHPV [types 6, 11, 16, 18, 31, 45, 52, 58]) in 2014. The American academy of Pediatrics
recommends that the HPV be started between the ages of 9 and 12 years and the Advisory
Committee immunization Practices of the Centers for Disease Control and Prevention
recommends that the vaccine series begin at age 11 to 12 years, with the option to begin as early
as age 9 years. Individuals with immediate hypersensitivity reactions to one of the vaccine
components (including severe allergy to yeast) should not receive the vaccine.
Studies have demonstrated that antibody titers in 9- to 14-year-olds after 2 doses of the vaccine
(given 6 to 12 months apart) are equivalent to titers in 16- to 24-year-olds after doses of the
vaccine. Accordingly, vaccine recommendations are based on age; If the vaccine series is started
before the 15th birthday, 2 doses are recommended, with the 2nd dose at least 5 months after the
first (ideally 6 to 12 months). If the vaccine series is started on or after the 15th birthday, 3 doses
are recommended, with the 2nd dose given 1 to 2 months after the first and the 3rd dose at least 6
months after the first. Immunocompromised adolescents and young adults should receive a 3-
dose series, even if they are younger than 15 years at the first dose. If a patient begins the
vaccine series with the quadrivalent vaccine he or she can finish with the 9-valent vaccine
without alterations to the immunization schedule.
Although HPV vaccines have shown efficacy in reducing the incidence of anogenital warts and
of precancer cervical and anogenital cell changes, they do not treat existing infection or prevent
pre-existing HPV infection from progressing to symptomatic disease. The HPV vaccine must
therefore be administered before exposure to the virus through sexual contact. Long-term studies
are needed to examine the duration of antibody protection.

PREP Pearls
• Human papillomavirus types 6 and 11 are leading causes of condylomata acuminata
(genital warts), and types 16 and 18 are the most commonly implicated in cervical and
anogenital cancers
• A 2-dose series of human papilloma virus vaccines is indicated if the first dose is given
before the 15th birthday; if given on or after the 15th birthday, a 3- dose series is needed.
ABP Contort Specifications(s)

• Know the recommendations, limitations, and schedule for the human papillomavirus
vaccine
Suggested Readings
• Human papillomaviruses. Red Book: 2021-2024 Report of the Committee on infectious
Diseases. 32nd ed; 2021:440-446
• Use of a 2-dose schedule for human papillomavirus vaccination. MMWR Morb Mortal
Wkly Rep. doi:10.15585/mmwr.mm6549a5
• Advisory Committee on Immunization Practices Recommended Immunization Schedule
for Children and Adolescents Aged 18 Years or Younger - United States, 2021. MMWR
Morb Mortal Wkly Rep. doi:10.15585/mmwr.mm7006a1

Question 234
A 6-year-boy is evaluated in the office for a 4-year history of difficulty stooling and fecal
incontinence. His symptoms began at around 18 months of age with stooling frequently, often
requiring glycerin suppositories to stool. At 12 years of age, he was started on daily polyethylene
glycol 3350 powder, which he has used consistently since that time and is able to stool daily. His
stools occur in the evening and are described as “explosive” liquid stool accompanied by
significant flatulence. He often has episodes of fecal incontinence at school. Attempts at
decreasing his use of polyethylene glycol 3350 have resulted in infrequent stooling and increased
abdominal cramping. The boy has never had large diameter stools. He has other medical history,
and there was no stooling difficulty as a breastfed infant. Physical examination reveals a well-
appearing child in no distress. Growth parameters are normal (weight 75th percentile for age,
height 90th percentile for age, body mass index 45th percentile for age). His abdomen is soft but
distended. On rectal examination, there is a normal anal wink, a normally positioned anus, a
somewhat tight anal sphincter tone, and absence of stool in the rectal vault. His neurologic
Abdominal radiography is shown in Item Q234
Of the following, the BEST next step in this boy’s management is

A. disimpaction with polyethylene glycol 3350
B. fiber supplementation
C. pelvic floor physical therapy
D. rectal suction biopsy

Correct Answer D
The boy in the vignette has symptoms (e.g., infrequent stooling and diarrhea despite chronic
laxative therapy) and signs (e.g., abdominal distension, abnormal anal sphincter tone, and empty
rectal vault on digital rectal examination) suggestive of Hirschsprung disease (HSD). Of the
responses, the best next management step is a diagnostic evaluation for HSD with a rectal
suction busy.
Hirschsprung disease occurs due to incomplete migration of ganglion cell precursors from the
neural crest along the gastrointestinal tract, most commonly (75% to 80% of cases), resulting in
the absence of ganglion cells in the rectosigmoid colon. Less commonly, the aganglionic
segment can begin more proximally, even resulting in complete aganglionosis of the colon (with
rare extension into the small intestine). While HSD can be associated with other disorders (e.g.,
Down syndrome, Waardenburg syndrome, Laurence-Moon-Bardet-Biedl syndrome, Smith-
Lemli-Opitz syndrome) and nonsyndromic anomalies (e.g., hydrocephalus, ventricular septal
defects, renal anomalies, imperforate anus, Meckel diverticulum, colonic polyposis,
cryptorchidism), 70% of cases occur as an isolated defect.
Although, HSD presents most commonly during infancy, it is important to note that older
children may also be diagnosed with HSD. Neonates and infants with HSD may be otherwise
well, with a history of delayed (>48 hours) passage of meconium, or may be ill appearing with
abdominal distention and bilious emesis. Older children with HSD commonly describe
longstanding and refractory constipation, often requiring rectal stimulation (i.e., suppositories or
enemas) for defecation, and intermittent abdominal distention. Examination findings of
abdominal distention, tight anal sphincter tone. empty rectal vault, and/or expulsion of stool after
digital rectal examination should raise suspicion for HSD.
The gold standard test for diagnosis of HSD is rectal suction biopsy (performed by a pediatric
gastroenterologist or pediatric surgeon) or a full-thickness rectal biopsy (performed by a
pediatric surgeon) demonstrating the absence of ganglion cells in the rectal submucosa. Contrast
imaging (i.e., unprepped contrast enema) may support the diagnosis if a transition zone is
visualized at the junction of the aganglionic segment and proximally dilated bowel. Notably,
other causes of proctitis (e.g., cow milk protein proctitis) can cause a similar appearance, and it is
possible for a child with biopsy-proven HSD to have a normal appearing contrast enema. Thus,
the contrast enema alone should not be used to diagnose or exclude HSD. A contrast enema may
be useful to define the affected area for the purposes of surgical planning. Anorectal manometry
(performed by a pediatric gastroenterologist experienced with manometric testing) may be used
to screen for HSD; a normal study demonstrating rectal distention and resultant relaxation of the
internal anal sphincter excludes HSD. False positives, however, can occur, and should be
followed by a rectal biopsy containing submucosa. Anorectal manometry is more commonly
performed when ultra-short segment HSD is clinically suspected in children with a normal
contrast enema and insufficient rectal suction biopsy results.
Treatment of HSD involves surgical resection of the aganglionic segment with anastomosis of
the normal proximal bowel to the distal rectum or anal canal. This procedure may require
creation of a temporary colostomy if the proximal bowel is significantly dilated. Complications
of HSD include HSD- associated enterocolitis and postoperative constipation and/or
incontinence. Enterocolitis may occur prior to and/or after resection of the aganglionic segment
and presents with abdominal distention, diarrhea, vomiting, and fever. Rapid onset of
dehydration and sepsis may ensue. Treatment includes intravenous fluids and antibiotics, holding
oral/enteral feedings, end rectal irrigation.
Disimpaction with polyethylene glycol 3350 would not be indicated in this scenario, as there is
no evidence (either radiographically or by digital rectal examination) of rectal stool impaction,
Fiber supplementation and/or pelvic floor physical therapy would be helpful for patients with
functional constipation and encopresis; however, neither would be the best next option for this
patient with signs and symptoms concerning for HSD.
PREP Pearls
• Hirschsprung disease most often presents in infancy, but may be diagnosed older children
with refractory constipation
• Alarm signs suggestive of Hirschsprung disease include: history of delayed passage of
meconium, bilious emesis, and abdominal distention (particularly in neonates and
infants). dependance on suppositories or enemas, and those not responding to laxative
therapy
• Complications of Hirschsprung disease include Hirschsprung disease- associated
enterocolitis, constipation. and fecal incontinence

• Plan the appropriate diagnostic evaluation in a patient in whom Hirschsprung disease is
suspected
• Recognize the clinical features associated with Hirschsprung disease
• Identify complications associated with Hirschsprung disease
Suggested Readings
• Hirschsprung disease. Pediatr Rev, doi:10.1542/pir.27-8-e56
• Hirschsprung disease. Curr Opin Pediatr, doi:10.1097/MOP.0b013e328360c2a0
• Surgical emergencies of the chest and abdomen in the newborn. American Academy of
Pediatrics Textbook of Pediatric Care. 2nd ed. 2017; 968-987

Question 235
An 8-year-old boy is seen for concerns about learning and development. He was recently
diagnosed via psychoeducational evaluation as having moderate intellectual disability. A
chromosomal microarray did not identify any genetic abnormalities, and the fragile X test result
was negative. His parents are concerned about the possibility of a condition that was present at
birth and ask if there are any other laboratory tests that could be conducted. Testing for an inborn
error of metabolism is considered.
Of the following, the sign or symptom MOST consistent with this type of condition is
A. aggression
B. developmental regression
C. repetitive behaviors
D. stereotypies

Correct Answer: B
The more severe the intellectual disability, the higher is the likelihood of identifying a cause.
However, only 0% to 5% of children with intellectual disability are found to have an inborn error
of metabolism. Results are more likely to be positive in patients with developmental regression,
atypical odor, failure to thrive, or neurologic signs or symptoms such as abnormal tone, ataxia, or
seizures. Aggression, repetitive behaviors, and stereotypies are not suggestive of metabolic
disorder. Metabolic testing can include plasma amino acids, acylcarnitine profile, and total
homocysteine; and urine organic acids, oligosaccharides, and glycosaminoglycans. Causes of
intellectual disability present or occurring around the time of birth are shown in Item C235.
Item C235: Causes of Intellectual Disability

• Genetic syndromes
o Chromosomal disorders, eg, Down syndrome
o Contiguous gene deletions, eg, Williams syndrome, Angelman syndrome
o Single-gene deletions, eg, fragile X syndrome, Rett syndrome
• Environmental causes
o Alcohol and other teratogens
o Prenatal infections
o Early childhood central nervous system infections
o Traumatic brain injury
• Central nervous system disorders/malformations
• Inborn errors of metabolism
• Nutritional (eg, severe malnutrition, chronic iron deficiency)
• Not known
Some genetic conditions that are associated with intellectual disability may be recognized at
birth (e.g., Down syndrome), but others may not be diagnosed until later in childhood when
additional features of the condition become apparent (e.g., fragile X syndrome, Rett syndrome,
Angelman syndrome)
Metabolic causes of intellectual disabilities include inborn errors of metabolism such as

phenylketonuria. Newborn metabolic screening programs can identity potentially treatable
conditions, and long-term outcomes are improved when these inborn errors of metabolism are
identified and treated early. However, the specific conditions included in newborn metabolic
screening panels vary by state.
PREP Pearls
• Symptoms and signs suggestive of inborn errors of metabolism include developmental
regression, abnormal tone, ataxia, seizures, organomegaly, atypical odor, and failure to
thrive.
• The more severe the intellectual disability, the higher the likelihood of identifying a
cause.
• Metabolic testing can include plasma amino acids, acylcarnitine profile, and total
homocysteine; and urine organic acids, oligosaccharides, and glycosaminoglycans

• Identify common metabolic causes of intellectual disabilities
• Identify the prenatal and perinatal causes of intellectual disabilities including factors
associated with family history
Suggested Readings
• Intellectual disability: Evaluation end management. Zuckerman Parker Handbook of
Developmental and Behavioral Pediatrics for Primary Care. 4th. 2019;:300-306.
• Cognitive development and disorders. Developmental and Behavioral Pediatrics. 2nd ed;
2018:309-346
• Intellectual disability. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed 2017:2208-2216
• Intellectual disabilities. Pediatr Rev. doi: 10.1542/pir.2016-0116
• Inborn errors of metabolism (metabolic disorders). Pediatr Rev. doi:10.1542/pir.2014-
0122

Question 236
A 6-year-old boy is seen for a health supervision visit. He is attending first grade in a regular
classroom and is doing well. His parents are expecting a baby next month. A family history is
obtained (Item Q236), Review of the pedigree shows cystic fibrosis in the boy’s great
grandparent, advanced paternal and maternal age at conception, European ancestry, and parental
consanguinity (first cousins). His parents are concerned about the risk of cystic fibrosis in their
unborn child
Of the following, the MOST significant risk factor for cystic fibrosis is
A. advanced maternal age at conception
B. advanced paternal age at conception
C. parental consanguinity
D. parental ethnic background

Correct Answer: C
Cystic fibrosis (CF) is a multiorgan disease resulting from changes in the CF transmembrane
conductance regulator (CFR) gene. An autosomal recessive condition, CF occurs when 2
disease-carrying changes in the same gene are inherited in an affected child from unaffected
carrier parents. Consanguinity increases the risk of an autosomal recessive condition, as the same
disease-carrying change in the gene is more likely to be present in related parents who can then
pass it on to their child.
Advanced maternal age (≥35 years) at conception increases the risk of aneuploidies as trisomy
21 (Down syndrome), trisomy 13 (Patau syndrome), and trisomy 18
(Edwards syndrome) due to increased risk of meiotic nondisjunction. Advanced paternal age
(>40 years) at conception increases the risk of de novo mutation in the sperm for conditions such
as achondroplasia. Neither advanced maternal or paternal age alters the risk of an autosomal
recessive condition.
Parental ethnic background does affect the risk of an autosomal recessive condition, but not to
the same degree as does parental consanguinity. For example, the carrier frequency for
pathogenic CFTR variants in people of northern European descent is 1:28. Hence, the risk for a
couple of European ancestry to have a child with CF would be:
l/28 (risk father is a carrier) X 1/28 (risk mother is a carrier) X 1/4 (risk to pass on the disease
allele to the child) = 0.0003
In the vignette the great-grandfather had CF, hence his children are obligate carriers of CF. They
in turn are at 50% risk of passing on the disease-causing change in the CFTR-gene to the boy's
parents. In that case, the couple's risk to have a child with CF would be:
½ x ½ x ¼ =1/16 = 0.06
If one parent is at-risk of being a carrier by inheriting the change from the family, and the other
is at population risk. the risk for the unborn child would be calculated as
½ x 1/28 x ¼ =1/224 = 0.004
PREP Pearls
• An autosomal recessive condition occurs when 2 disease-causing changes are inherited in
an affected child from unaffected carrier parents.
• Parental consanguinity increases the risk for an autosomal recessive condition.

• Recognize the inheritance pattern associated with an autosomal recessive disorder

Suggested Readings
• Mutation Research/Reviews in Mutation Research. doi: 10.1016/j.mrrev.2020.108320
• Patterns of single-gene inheritance. Thompson and Thompson Genetics in Medicine. 8th
2016.
• Gene Reviews [Internet)
• Statement on guidance for genetic counseling in advanced paternal age. Genet Med.
doi:10.1097/GIM.0b013e318176fabb

Question 237
A 2-year-old boy is seen in the emergency department for 3-day history of fever; decreased oral
intake; nonbloody, nonbilious emesis with feedings; and rash. He has no upper respiratory
symptoms.
He has a temperature of 39.8 °C and tachycardia. The boy appears nontoxic and has scleral
icterus, pale conjunctivae, and dry mucous membrane. His capillary refill is less than 3 seconds,
abdomen is soft and distended with hepatosplenomegaly, and there is bilateral < 1-cm cervical
lymphadenopathy. There is a faint, lacy, macular; erythematous rash on the bilateral lower
extremities. He has no focal neurologic deficit.

Ferritin 308 ng/mL (308 µg/L)
Triglycerides 396 mg/dL (4.5 mmol/L)
Fibrinogen 137 mg/dL (1.4 g/L)
Soluble CD25 (Soluble IL-2 14,970 U/mL
receptor) reference range, 398-
1940 U/mL
Aspartate amino transferase 110 U/L
Alanine amino transferase 220 U/L
The boy is admitted to the hospital for further evaluation and management.
Of the following, the BEST next step in the diagnostic evaluation of this child is
A. liver biopsy
B. lumbar puncture
C. lymph node biopsy
D. skin rash biopsy

Correct Answer: B
The child in the vignette has sign, symptoms, and laboratory findings that meet the criteria for a
diagnosis of hemophagocytic lymph histiocytosis (HLH), a hyperinflammatory disorder It can be
primary or familial (e.g., underlying mutation or genetic disorder) or secondary (e.g., in response
to underlying infection or malignancy). Both forms result in an overactivation of the immune
system.
Criteria for diagnosis of HLH include:

• An identified genetic mutation OR
• Five of the following 8 criteria:
o Fever
o Splenomegaly
o Cytopenias in 2 out of the 3 blood cell lines
o Hypertriglyceridemia and/or hypofibrinogenemia
o Hemophagocytosis in the spleen, bone marrow, or lymph node without evidence
of malignancy
o Low or absent natural killer (NK) cell activity
o Elevated ferritin level
o Elevated soluble CD25 (IL-receptor) level
The child in the vignette with meets 6 of the diagnostic criteria

Once the child is diagnosed with HLH, additional investigations must be performed including:
• Genetic testing for primary HLH
• Evaluation for infectious causes of secondary HLH
• Bone marrow aspirate and biopsy to rule out malignancy and lock for the classic
Hemophagocytosis macrophage (Item C237)
• Lumbar puncture (to determine if intrathecal therapy is indicated)
• Brain imaging to assess for central nervous system disease
Item C237
Liver biopsy findings could support the diagnosis; however, this child meets diagnostic criteria
without liver biopsy
Biopsy of enlarged lymph node may assist in the diagnosis however, biopsy of this child’s lymph
nodes will be difficult and not helpful. Biopsy of this child’s skin rash will not add any valuable
information.
The treatment of HLH involves systemic therapy including steroids and etoposide with
intrathecal treatment, if indicated. For primary HLH, the goal is to include a clinical and
laboratory remission followed by hematopoietic stem cell transplantation to achieve curative
therapy. Emapalumab, an anti-interferon γ antibody, is the first FDA-approved medication for
the treatment of progressive or refractory primary HLH, or in individuals who cannot tolerate
standard therapy. Anakinra, a recombinant interleukin 1 receptor agonist, and ruxolitinib a Janus-
associated kinase (JAK) inhibitor, have also shown success in the treatment of HLH. For
secondary HLH, there should also be treatment directed toward any identified trigger (e.g.,
rituximab for Epstein-Barr virus -induced HLH).
PREP Pearls
• hemophagocytic lymph histiocytosis should be considered in children with organomegaly
and pancytopenia
• Once the diagnosis of hemophagocytic lymph histiocytosis is made, it is important to
perform cerebrospinal fluid sampling and brain imaging to assess for central nervous
system involvement.
• Recognize the presentation of primary disorders of phagocytic number and/or function

• Recognize the clinical findings associated with histiocytosis syndromes of childhood
Suggested Readings
• The use of anakinra in the treatment of secondary hemophagocytic lymph histiocytosis,
Pediatr Blood Cancer, doi:10.1002/pbc.28581
• Diagnostic and therapeutic guidelines for hemophagocytic lymph histiocytosis. Pediatr
Blood Cancer. doi:10.1002/pbc.21039
• Hemophagocytic syndromes-an update. Blood Rev. doi:10.1016/j.blre.2014.03.002
• Ruxolitinib in alleviating the cytokine storm of hemophagocytic lymph histiocytosis.
Pediatrics. doi:10.1542/peds.2019-1301
• Neonatal hemophagocytic lymph histiocytosis. Neoreviews. doi:10.1542/neo.20-6-e316

Question 238
A 16-year-old adolescent girl is seen for a vaginal discharge that began 3 weeks ago at the end of
her last menstrual period. The discharge is yellow and is not associated with itching, burning,
swelling, foul odor, or dysuria. This week on 2 separate occasions she experienced pain with
attempts at vaginal sex. She and her partner agreed to forego sex until she could be evaluated at
the clinic. She has not noticed any genital sores or ulcers. She and her male sexual partner are
infrequent users of condoms. Her last screen for chlamydia and gonorrhea 6 months ago was
negative. She has had 2 previous male sex partners. There is no history of dating violence or
sexually transmitted infections. A point-of-care urine pregnancy test is negative.
Of the following, the BEST next step in this girl's evaluation is

A. pelvic examination
B. pelvic ultrasonography
C. quantitative serum
D. urine test for chlamydia

Correct Answer: A
The gid in the vignette has dyspareunia (pain with vaginal intercourse), which is an indication for
a pelvic examination. The decision to perform a pelvic examination should be based on the
clinical presentation and likelihood that the examination will substantially inform decision-
making and treatment. In sexually active girls, unexplained lower abdominal pain or pelvic pain
(including dyspareunia) suggests a diagnosis of pelvic inflammatory disease (PID). The
minimum criteria for the diagnosis and empiric treatment of PID are pain on cervical motion or
uterine or adnexal tenderness elicited during the bimanual pelvic examination.
Other indications to perform a pelvic examination in an adolescent girl include pregnancy,

suspicion of a foreign body, known or suspected trauma (including sexual abuse), evaluation of
unexplained vaginal bleeding, inability to insert a tampon, and placement of an intrauterine
device. A pelvic examination may also help in the evaluation of dysmenorrhea unresponsive to
usual interventions or delayed menarche.
Although pelvic ultrasonography may be useful in the evaluation of pelvic anatomy and
pathology, it is not preferred next step for the girl in the vignette because she had dyspareunia in
addition to vaginal discharge. Likewise, sending a urine sample to be tested for chlamydia and
gonorrhea should not preempt a pelvic examination. Failure to perform a pelvic examination can
delay the diagnosis and treatment of PID and increase the risk for complications (e.g., tubo-
ovarian abscess) and sequelae (e.g., infertility, ectopic pregnancy, chronic pelvic pain). Pelvic
ultrasonography and a quantitative ß-human chorionic gonadotropin level would be indicated if
ectopic pregnancy were suspected.
The pelvic examination has 3 components: external genital inspection, bimanual, and speculum
examination. It should progress in that order, starting with the least invasive. A rectoabdominal
examination may be of some value for an adolescent who does not give consent for a pelvic
examination, put it would not be the preferred method of evaluation.
Routine pelvic examinations are not necessary before initiating cervical cancer screening. The
American College of Obstetrics and Gynecology (ACOG) and the US Preventive Services Task
Force recommend initiating screening with a Papanicolaou (Pap) smear at age 21 years with
repeat screening every 3 years between ages 21 and 29 years. In addition, ACOG allows an
alternative option of primary human papillomavirus (HPV) testing every 3 years for those aged
25 years and older. The American Cancer Society recommends initiating screening at age 25
years and offers a preferred option for primary HPV testing every 5 years. Alterative options
include cotesting every 5 years or a Pap test every 3 years.
Urine or vaginal swab specimens; including self-collected swabs, for the diagnosis of chlamydia,
gonorrhea, and trichomoniasis by nucleic acid amplification testing have decreased the need to
perform a pelvic examination for the evaluation of an uncomplicated presentation of a vaginal
discharge

PREP Pearls
• The decision to perform a pelvic examination should be based on the clinical presentation
and likelihood that the examination will inform decision-making/treatment.
• Indications for a pelvic examination for an adolescent girl include assessment for pelvic
inflammatory disease (e.g., lower abdominal/pelvic pain, dyspareunia) and insertion of an
intrauterine device
• Routine pelvic examination, Papanicolaou smears, and human papillomavirus screenings
are not recommended for adolescents and young women less than 21 to 25 years of age.

• understand the indications for a pelvic examination in an adolescent girl
Suggested Readings
• The utility of and indications for routine pelvic examination. Obstet Gynecol,
doi:10.1097/AOG.0000000000002895
Care. 2nd ed. 2017:2628-2651.
• A practical overview of managing adolescent gynecologic conditions in the pediatric
office. Pediatr Rev, doi:10.1542/pir.35-9-371
• Sexually transmitted infections part 2: discharge syndromes and pelvic inflammatory
disease. Pediatr Rev, doi:10.1542/pir.2019-0078

Question 239
A 13-year-old adolescent boy seen for a routine health supervision visit. He is concerned about
his small size. The boy is healthy, has no significant past medial history, and has a negative
review of systems. His mother is 5 feet, 5 inches tall, and had menarche at 12 years of age. His
father is 5 feet, 9 inches tall, and continued to grow in height during college. The boy's vital
signs are normal. His growth chart is shown in Item Q239. His body mass index is 17 kg/m2
(24th Percentile). He has a sexual maturity rating of 2 for pubic hair and genital development.
The remainder of his physical examination findings are unremarkable. A bone age radiograph is
read as 11 years.

A. constitutional delay
B. familial short stature
C. growth hormone deficiency
D. insufficient caloric intake

Correct Answer: A
The boy in the vignette has constitutional delay of growth and puberty. He is healthy, has no
evidence of an underlying growth disorder or systemic disease, has a family history of delayed
puberty in his father (completed linear growth in college), and has a delayed bone age that
matches his pubertal stage. Although he is in early puberty, he has not yet started his pubertal
growth spurt, which occurs later in puberty in boys. His height is becoming increasingly
discrepant from his peers because he continues to grow at a normal prepubertal height velocity (5
cm/year) while many his peers are growing at pubertal height velocities. His pubertal growth
spurt will start later and will grow for a longer time.
Genetic target height potential can be estimated as follows:

For boys: [mother's height + father's height + 5 inches (13 cm)]/2
For girls: [mother's height + father's height -5 inches (13 cm)]/2
Target height range is often determined by this number ± 1-2 inches (5 cm).
The boy's target height range is 69.5 +/- 1-2 inches, encompassing the 25th to 75th percentile for
an adult male. If his current height is plotted for his bone age (11 years) versus his chronological
age (13 years). it falls within his target height range percentile. Using published tables, his
predicted adult height based on his current height and bone age is 68.5 inches, which is
consistent with his target height range.
The boy in the vignette does not have familial short stature because his current height percentile
is below that expected for his target height range. In familial short stature, height percentile is
appropriate for the target height range, and bone age is concordant with chronological age.
The boy does not have growth hormone deficiency because he has a normal height velocity.
Children with growth hormone deficiency and short stature due to hypothyroidism have
abnormally low height velocities. There is no indication of inadequate caloric intake given his
consistent weight gain and normal body mass index.
Constitutional delay is the most common cause of short stature and delayed puberty in children,
especially in boys. These children are otherwise healthy. A family history of constitutional delay
is common. Bone age is delayed, is consistent with pubertal stage, and predicts catch-up growth.
Management of constitutional delay consists of reassurance regarding future pubertal

development and catch-up growth. In addition to clinical observation. constitutional delay,
however, can cause significant psychological stress. Referral to pediatric endocrinologist for
short course of sex steroid therapy is a treatment option once a boy is 14 years old, or a girl is 13
years old, and has no or minimal pubertal development. The goal of sex steroid therapy is to
facilitate pubertal progression and promote earlier initiation of the pubertal growth spurt. There
is no significant effect on final height.

PREP Pearls
• constitutional delay is the most common cause of short stature and delayed puberty in
children and is considered a normal variant
• children with constitutional delay are otherwise healthy, have a delayed bone age, and
often have a family history of delayed puberty
• Management of constitutional delay consists of reassurance regarding future catch-up
growth and pubertal development and clinical observation.

• Evaluate constitutional growth delay by growth chart evaluation
• Understand the natural history of constitutional growth delay
• Plan an appropriate diagnostic evaluation to differentiate constitutional growth delay and
other conditions causing growth delay
• Understand the natural history of genetic (familial) short stature
• Distinguish among constitutional short stature, genetic (familial) short stature, and
growth hormone or thyroid deficiencies by growth chart evaluation.
Suggested Readings
• Disorders of growth and statute. Pediatr Rev. doi:10.1542/pir.2016-0178
• Short stature. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed.
2017:1585-1589.
• A general pediatric approach to evaluating a short child. Pediatr Rev.
https://pubmed.ncbi.nlm.nih.gov/16264029/

Question 240
An 8-year-old girl with sickle cell disease is admitted to the pediatric intensive care unit with
fever, headache, and altered mental status. She is rapidly intubated for airway protection due to
her declining mental status. A head computed tomography scan obtained after she is stabilized is
normal. A lumbar puncture is performed, cerebrospinal fluid (CSF) analysis reveals:

CSF glucose 15 mg/dL
CSF protein 250 mg/dL
CSF white blood cell count 1,700 cells/µL; neutrophil predominance
CSF red blood cell count 4 cells/µL
Serum glucose 90 mg/dL (5 mmol/L)
Empiric antibiotic therapy at meningitic doses is initiated. Cerebrospinal fluid cultures grow
Streptococcus pneumoniae. One week later, the girl remains intubated and unresponsive. Her
neurological examination is limited by sedation but is significant for diminished movement in
her left arm and leg in response to stimulation. Brain magnetic resonance imaging with and
without contrast demonstrates multifocal areas of diffusion restriction and diffuse meningeal
enhancement with normal venography.
In discussing their daughter's clinical condition, her parents ask about additional complications
of meningitis that might occur in the near future.
Of the following, during the acute phase of illness, this child is at HIGHEST risk for
A. hearing loss
B. hydrocephalus
C. seizures
D. spasticity

Correct Answer: C
Of the response choices, in the acute phase of her illness the girl in the vignette is most at risk for
developing symptomatic seizures related to interval development of ischemic injury (indicated
by multifocal areas of diffusion restriction noted on the brain magnetic resonance imaging
study). The magnetic resonance imaging result demonstrate enlarged ventricles, making
hydrocephalus less likely for this child. Hearing loss and spasticity are potential complications
requiring ongoing followup, but they are less of a risk than seizures during her acute illness.
Bacterial meningitis is a neurological emergency requiring rapid stabilization, evaluation, and

administration of antibiotic therapy. Meningitis should be considered in children with fever,
lethargy, headache (often with photophobia, nausea/vomiting), confusion, and irritability. Nuchal
rigidity, or the inability to passively flex the neck is not uniformly present and can be difficult to
elicit in comatose patients; its absence cannot be used to rule out the diagnosis of meningitis.
The diagnostic evaluation for suspected meningitis includes serum complete blood cell count
with differential, blood culture, inflammatory marker levels, serum electrolyte and blood glucose
levels, coagulation studies, and lactate level. A lumbar puncture (LP) should be performed and
cerebrospinal fluid sent for analysis including cell count with differential, glucose level, protein
level, Gram stain, and herpes simplex virus polymerase chain reaction and culture. For children
with focal findings, papilledema, recent trauma, history of hydrocephalus, or coma,
neuroimaging should be obtained prior to LP to exclude contraindications to the procedure. If LP
cannot be performed promptly, or the patient's condition is rapidly deteriorating, empiric
antibiotic therapy should be initiated immediately and LP performed when feasible. An
algorithm for the diagnostic evaluation of a child suspected to have bacterial meningitis is shown
in Item C240.
In the acute setting, close monitoring for complications requires ongoing bedside examination,
including neurological assessment. Increased intracranial pressure can occur secondary to
meningeal infection and inflammation, or it can be the result of an acute complication such as
hydrocephalus, brain abscess, subdural empyema, or intracranial hemorrhage. Monitoring for
signs and symptoms of increased intracranial pressure should include close attention to vital sign
trends (for early detection of evolving evidence of Cushing triad), serial fundoscopic
examination, serial pupillary and extraocular movement examination, and, in younger children,
daily head circumference measurements. Development of focal deficits should prompt
neuroimaging to evaluate for interval development of stroke, hemorrhage, brain abscess, or
subdural empyema.
Seizures are a common complication of bacterial meningitis and can occur at presentation.
Generalized seizures occur in 20% to 25% of children with bacterial meningitis early in the
disease course. Later development of seizures (>48-72 hours after initiation of antibiotic therapy)
or focal seizures should raise concern for interval development of additional injury such as
parenchymal ischemia, hemorrhage, abscess, or subdural empyema.
Subdural effusions are seen in up to one-third of cases of bacterial meningitis. Effusions are
often asymptomatic or minimally symptomatic and do not require intervention. Subdural

empyema should be suspected if the child is febrile or develops new neurological deficits such as
focal abnormalities or seizures. Unlike effusions, empyema usually required drainage.
Item C240: Algorithm for suspected meningitis. BUN: blood urea nitrogen, CNS: central
nervous system, CRP: C-reactive protein, CSF: cerebrospinal fluid, CT: computed tomography, DIC: disseminated
intravascular coagulation, ICP: intracranial pressure, LP: lumbar puncture. Adapted from Mann and Jackson and
Tunkel et al

The long-term morbidity and mortality of bacterial meningitis is significant; pneumococcal
meningitis carries a particularly high rate in comparison causative organisms, even with optimal
treatment. Long-term neurological sequelae are common, occurring in up to 50% of survivors of
pneumococcal meningitis. Long-term sequelae of meningitis may include spastic paresis, ataxia,
aphasia, and cognitive impairment. Sensorineural hearing loss may develop in the first few days
of the disease course and can persist after with a range of severity and varying degrees of
improvement. Seizures can persist after discharge, although for many patients this resolves with
time.
Routine use of pneumococcal vaccination has reduced the overall incidence of invasive
pneumococcal disease. Children with certain underlying conditions, such as functional asplenia
in sickle disease, are at higher risk for invasive pneumococcal infections and therefore should
receive additional vaccinations as recommended by Centers for Disease Control and Prevention
guidelines.
PREP Pearls
• Rapid recognition and treatment is critical in the care of a patient with bacterial
meningitis. Ongoing serial examinations and monitoring is crucial for rapid detection and
treatment of complications.
• Common acute complications of bacterial meningitis include increased intracranial
pressure, hydrocephalus, seizures, stroke, hemorrhage, and subdural empyema.
• Long-term neurological sequalae of bacterial meningitis are common, especially
pneumococcal meningitis; these include spastic paresis, seizures, cognitive impairment,
and sensorineural hearing loss.

• Recognize the acute and long-term complications associated with meningitis
Suggested Readings
• Streptococcus pneumonia (pneumococcal). Red Book: 2021-2024 Report of the
Committee on infectious Diseases. 32nd ed; 2021:713-716
• Effect of pneumococcal conjugate vaccine on pneumococcal meningitis. N Eng J Med.
doi:10.1056/NEJMoa0800836
• Pneumococcal infections. Pediatr Rev, doi:10.1542/pir.35-7-299
• Meningitis. Pediatr Rev, doi:10.1542/pir.36-12-514
• Meningitis. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017:2294-2309

Question 241
An infant with normal growth and development is seen in the clinic for a health supervision visit.
She is initially fearful and looks to her mother when the examiner enters the room. With time,
she grows calm, smiles, and laughs when the examiner plays peek-a-boo with her. After
squirming in her mother’s arms, she is placed standing on the ground. In response, she holds
onto the seat of a chair and takes a few steps. She turns when her mother says her name during
the visit and makes a variety of vocalizations using vowel and consonant sounds as well as
shrieks and “growls.”
Of the following, this infant's fine motor abilities MOST likely include
A. grasping a cube with her thumb and fingertips
B. scribbling linear marking with a crayon
C. self-feeding soft foods with a spoon
D. stacking 2 small blocks

Correct Answer: A
The infant described in the vignette is a typically developing 9-month-old infant. At this age, fine
motor skills include the ability to grasp a cube with the thumb and fingertips ('radial-digital
grasp'), and bang 2 objects together. An inferior pincer grasp of a small object like dry cereal
typically develops around 9 to 10 months of age as well. Children typically learn to hold a
crayon around 12 months of age and scribble at 14 to 15 months of age. Spoon feeding (with
spilling) is typically attained by 15 months of age. Children may begin stacking cubes around 12
months of age.
Developmental milestones of the typical 9-month-old are shown in Item C241A.
Item C241B shows the typical timeline for grasp development. Infants are usually able to pick
up a cube using a radial-palmar grasp at 7 months and a radial-digital cube grasp at 8 months.
The typical infant can rake a pellet at 7 months and progresses to an inferior pincer grasp by 10
months and a fine pincer grasp by 12 months.
Item C241B: Development of pincer grasp. Illustrations from the Erhardt developmental prehension. In Erhardt RP.
Developmental Hand Dysfunction: Theory Assessment, Treatment. 2nd ed. San Antonio, Tex: Therapy Skill
Builders; 1994. Reprinted with permission
PREP Pearls
• The typical 9-month-old infant can pull to a stand and may begin to cruise, grasp a cube
with a radial-digital grip and bang 2 objects together, say ‘mamas’ nonspecific and
imitate sounds: to name and enjoy games; and recognize familiar people visually and
follow when someone points
• Infants are usually able to pick up a cube using a radial-palmar grasp at 7 months and a
radial-digital cube grasp at 8 months. The typical infant can rake pellet at 7 months and
progresses to an inferior pincer grasp by 10 months

• Evaluate the cognitive and behavioral developmental progress/status of an infant at 9
months of age
• Evaluate the motor developmental progress/status of an infant at 9 months of age,
including recognition of abnormalities
Suggested Readings
• Developmental milestones: Motor development. Pediatr Rev, doi:10.1542/pir.31-7-267
• Bright Futures: Guidelines for Health supervision of infants, Children, and Adolescents.
4th ed; 2017.
• Identifying Infants and young children with developmental disorders through
developmental surveillance and screening. Pediatrics, doi:10.1542/peds.2019-3449
• Developmental milestones. Pediatr Rev, doi:10.1542/pir.2014-0103

Question 242
The family of a newborn in the nursery wishes for a specific physician to perform a circumcision
before discharge from the hospital today. Currently 6 male infants are awaiting discharge from
the hospital. To ensure that the procedure is performed on the correct infant, the physician opts to
follow the guidance of the Joint Commission National Patient Safety Goal regarding patient
identification.

A. ask the father to point out the infant
B. compare a photo of the infant to the patients
C. review the name card attached to the bassinet
D. verify the patient ID band with 2 distinct identifiers

Correct Answer: D
In 2002. The Joint Commission promulgated the National Patient Safety Goals (NPSGs)
program to help accredited health care organizations address specific high-risk practices that
have a negative impact on patient safety. The NPSGs are developed by an interdisciplinary panel
of experts in patient safety including nurses, physicians, pharmacist, risk managers, and other
professionals with frontline clinical experience in mitigating patient safety issues. Updated
annually, the NPSGs are disseminated to health care organizations accredited by the Joint
Commission, and are an integral component of the accreditation standards. High-profile NPSGs
have included improvement initiatives such as reducing wrong-site surgery and hospital-acquired
infections including catheter-associated urinary tract infections (CAUTI) and central line-
associated bloodstream infections (CLABSI), and safe medication prescribing practices.
The Joint Commission “Universal Protocol” establishes and mandates steps for preventing
wrong-site, wrong-procedure, and wrong-patient incidents. In the vignette, the clinician
performing the circumcision must appropriately follow the universal *time out,” which requires
the clinician to identify the correct patient, indicate the correct procedure to be done, and if
indicated, the correct site, before performing an invasive procedure. Acceptable patient
identifiers include the patient's full name, an assigned identification number such a medical
number number, patient telephone number, or other person-specific identifier. In adults, patients
are often asked to verbally confirm their identity by stating their name while the clinician verifies
the name against the medical record or patient ID band.
However, young children and newborns are at higher risk misidentification because of their
inability to speak and lack of distinguishable features. In addition to wrong procedures
misidentification has resulted in medication administration errors and feeding a mother's
expressed breast milk to the wrong infant. The Joint commission NPSG standard requires that
clinicians use at least 2 patient identifiers administering medications, blood, and blood
components, and before tests or procedures. With respect to newborn infants, methods to prevent
patient misidentification include distinct naming systems using the mother's first and last names
and the newborn's sex (e.g., “Johnson, Jane Girl” or “Jones, Janet Boy”). In addition, infants
should undergo routine and standard placement of identification bands with both name and
medical record number. The parent's identification, photo, or card attached to the bassinet alone
cannot serve as adequate preprocedural patient identification.
PREP Pearls
• Clinicians should use 2 unique patient identifiers to prevent misidentification.
• The Joint Commission promulgates annual National Patient Safety Goats to reduce
patient harm and improve patient safety.
• Neonates and young children are at high risk for misidentification because of their
inability to communicate.
MOCA-Peds Objective
APP Content Specifications

• Recognize the use of National Patient Safety Goals to improve patient safety
Suggested Readings
• Principles of pediatric Patient safety: Reducing harm due to medical care. Pediatrics
doi:10.1542/peds.2018-3649
• patient safety and quality improvement: Terminology. Pediatr Rev. doi:10.1542/pir.36-9-
403
• The joint commission national patient safety goals.
https://www.jointcommission.org/standards/national-patient-safety-goals/

Question 243
A previously healthy, 15-year-old adolescent girl is brought to the clinic by her mother for
concern about weight loss. Review of records reveals that the girl lost 4.5 kg since her last health
supervision visit 2 months ago. The girl reports no changes in diet or physical activity level,
abdominal pain, nausea, vomiting, or recent illness. Review of systems is significant for trouble
falling asleep, more frequent urination (every 2 to 3 hours per day), and more frequent stooling
(2 to 3 times per day) for the past few months. Her appetite is good. The girl is afebrile. Her
weight is at the 10th percentile, height is at the 60th percentile, and body mass index is at the 3rd
percentile. Her heart rate is 120 beats/min and blood pressure is 136/88 mm Hg, Her neck
appears full. and she has a fine tremor when her hands are outstretched. The remainder of her
physical examination findings ate unremarkable.
Of the following, the test MOST likely to reveal this adolescent's diagnosis is a
A. complete blood count with differential
B. plasma metanephrine panel
C. thyroid function test panel
D. tissue transglutaminase antibody level

Correct Answer: C
The adolescent in the vignette has hyperthyroidism. The test most likely to reveal her diagnosis
is a thyroid function test panel. This panel would show a low thyroid stimulating hormone (TSH)
level and elevated free thyroxine (FT4) level.
The adolescent’s hyperthyroidism is causing her weight loss, trouble sleeping, frequent urination
and stooling, tachycardia, hypertension, and tremor. Her neck fullness is due to enlarged thyroid
gland. Other common symptoms and signs of hyperthyroidism include fatigue, increased
appetite, decreased exercise tolerance, heat tolerance, difficulty concentrating, menstrual
irregularities, and prominent deep tendon reflexes. Hyperthyroidism should be considered in
children and adolescents with persistent sinus tachycardia. In adults, it can cause atrial
fibrillation.
Further evaluation is indicated to determine the specific cause of hyperthyroidism for the
adolescent in the vignette, most likely Graves’ disease. Graves' disease is the most common
cause of hyperthyroidism in children and adolescents. It is an autoimmune disorder caused by
functional antibodies that stimulate the thyrotropin (TSH) receptor. Thyroid hormone production
by the thyroid gland is increased, and the gland enlarges in size. Graves eye disease, which
manifests as proptosis, can help distinguish Graves' disease clinically from other causes of
hyperthyroidism.
Other causes of hyperthyroidism include Hashitoxicosis, an autonomously functioning thyroid

nodule, ingestion of exogenous thyroid hormone, and elevated levels of human chorionic
gonadotropin (hCG). Hashitoxicosis is an initial hyperthyroid phase of Hashimoto thyroiditis that
is not commonly detected. It is due the release of stored thyroid hormone at the time of the initial
autoimmune insult to the thyroid gland. Once the stored thyroid hormone is depleted,
hypothyroidism usually ensues. Because TSH and hCG have identical α-subunits (their β-
subunits give them their specificity), hCG has some cross-reactivity at the TSH receptor.
In a child or adolescent with suspected hyperthyroidism, measurement of TSH, FT4, and total
triiodothyronine (T3) or free triiodothyronine (FT3) levels is indicated. The Presence of
thyrotropin receptor antibodies confirms a diagnosis of Graves' disease. Thyroid peroxidase and
anti-thyroglobulin antibodies are associated with Hashimoto thyroiditis. Measurement of an hCG
level is indicated in reproductive age females. Thyroglobulin is a protein made in the thyroid
gland. Thyroglobulin level measurement can be helpful if ingestion of exogenous thyroid
hormone is suspected and would be low. Levels are high in conditions associated with excessive
thyroid hormone production by the thyroid gland and in conditions associated with thyroid gland
tissue destruction.
A nuclear medicine thyroid uptake and scan can be helpful in determining the etiology of
hyperthyroidism when laboratory evaluation is not revealing. Diffusely increased uptake ot
radiolabeled iodine into the thyroid gland is seen in Graves’ disease, as the gland is metabolically
active. Decreased uptake is seen in conditions where hyperthyroidism is due to the release of
stored thyroid hormone or ingestion of exogenous thyroid hormone. In the case of an
autonomously functioning nodule, the scan will show concentrated uptake in the area of the

nodule and decreased uptake in the rest of the thyroid gland. Thyroid ultrasonography can
identify thyroid nodules and evaluate the size and consistency of the thyroid gland.
Although a complete blood count with differential may show abnormalities when
hyperthyroidism is present (low white blood cell count, anemia), and is indicated before starting
anti-thyroid medication (anti-thyroid medication can cause white blood cell suppression), it
would not reveal the diagnosis for the adolescent in the vignette. A tissue transglutaminase
antibody level would be indicated if celiac disease was suspected. Although weight loss and
more frequent stooling can be symptoms of celiac disease, the adolescent’s other findings are
more consistent with hyperthyroidism. A plasma metanephrine panel is indicated if a
pheochromocytoma is suspected. Although many symptoms of catecholamine excess overlap
with those of hyperthyroidism. pheochromocytoma is very rare, and the girl's neck fullness
(enlarged thyroid gland) makes hyperthyroidism more likely.
PREP Pearls
• Hyperthyroidism should be considered in children and adolescents with persistent sinus
tachycardia.
• Graves’ disease is the most common cause of hyperthyroidism in children and
adolescents.
• In a child or adolescent with hyperthyroidism, the presence of thyrotropin receptor
antibodies confirms a diagnosis of Graves' disease.
• Plan the initial evaluation of a child with thyroid enlargement.

Recognize the role of hyperthyroidism in persistent sinus tachycardia
Suggested Readings
• Hyperthyroidism. Lancet, doi:10.1016/S0140-6736(16)00278-6
• Hyperthyroidism. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed;
2017; 2167-2171
• Hyperthyroidism In Children. Pediatr Rev. doi:10.1542/pir.36-6-239

Question 244
A 9-year-old girl is seen for diarrhea. Three days ago, she started to have nonbloody, nonbilious
emesis and nonbloody diarrhea. The vomiting stopped yesterday. but her diarrhea has continued.
She has remained afebrile and has maintained good hydration with an appropriate oral
rehydration solution. She has no other symptoms. Before this illness, she had been struggling tp
find a good maintenance regimen for chronic constipation but she has no other medical
problems, The girl's vital signs and physical examination findings are normal. Her weight is
tracking at the 50th percentile. Her father asks what kind of diet he should give her at this time.
Of the following, the BEST advice to give this girl's father is to

A. begin a diet of bananas, rice, applesauce, and toast
B. begin a diet with twice the daily recommended intake of fiber
C. resume a healthy, age appropriate, well-balanced diet
D. withhold solid food until all symptoms have resolved

Correct Answer: C
The girl in the vignette has acute gastroenteritis (AGE) and is well-hydrated. She should resume
a healthy, age-appropriate, well-balanced diet. Children with AGE who resume a normal healthy
diet early in the course of the illness have improved nutrition compared with those who delay
refeeding. The other response choices would ameliorate neither her current condition nor her
chronic constipation. A restricted diet is nutritionally inferior to a well-balanced diet and will not
speed up her recovery. A diet with twice the daily recommended intake of fiber is not
recommended for children with constipation. Current evidence does not demonstrate
improvement of constipation with increased fiber intake.
Although many families believe gut rest to be beneficial for AGE, studies demonstrate no
difference between early refeeding versus later feeding in safety efficacy, episodes of vomiting
and diarrhea, or need for intravenous fluids. Full-strength formula may be given and lactose-
containing foods need not be avoided. Feeding may need to be delayed in children with moderate
or severe dehydration until rehydration is complete.
If gastroenteritis symptoms persist, clinicians should consider noninfectious etiologies including

dietary factors (e.g., lactose intolerance or celiac disease), which can be investigated with the aid
of a food diary. Under the guidance of dieticians, children with identified food restrictions may
safely find alternatives to preserve nutrition for growth.
Various gastrointestinal disorders require specific and sometimes complex dietary considerations
(Item C244). A multidisciplinary team may be necessary in some cases.
Item C244

PREP Pearls
• Children with acute gastroenteritis should be given a healthy, well-balanced, age-
appropriate diet early in their illness; if moderately or severely dehydrated, this may need
to be delayed pending rehydration,
• Certain gastrointestinal disorders require complex dietary management necessitating the
guidance of a multidisciplinary team.
• Current evidence does not demonstrate improvement of constipation with increased fiber
intake.

• Understand the importance of early refeeding in a child with gastroenteritis
• Plan dietary management for a patient with a gastrointestinal disorder
Suggested Readings
• Oral therapy for acute diarrhea. Pediatric Nutrition. 8th ed; 2019.815-827
• Clinical practice guidelines for the diagnosis and management of infectious diarrhea. Clin
Infect Dis, doi:10.1093/cid/cix669
• Diarrhea and steatorrhea, American Academy of Pediatrics Textbook of Pediatric Care.
2nd ed: 2017:1267-1281.

Question 245
A 14-year-old adolescent boy is evaluated in the emergency department for nausea, abdominal
pain, and multiple subsequent vomiting episodes. His symptoms developed after he locked
himself in his bathroom for several hours after arguing with his mother. He has been arguing
frequently with his parents. He is failing multiple classes in school, and his behavior at home
during the past 3 months has been sullen, argumentative, and withdrawn. The patient appears
diaphoretic end agitated, and reports that he "can't hear.' His temperature is 37.8 ºC, heart rate is
135 beats/min, respiratory rate is 30 breaths/min, and blood pressure is 115/75 mm Hg

Basic metabolic panel
Creatinine 0.9 mg/dL (79,6 µmol/L)
Arterial blood gas
pH 7.47
pCO2 13 mm Hg
pO2 195 mm Hg
HCO3 10 mmol/L
Base deficit -12.2 mmol/L
Lactic acid 0.9 mmol/L
Of the following, the substance that was MOST likely ingested by this adolescent is
A. acetaminophen
B. aspirin
C. isopropyl alcohol
D. an opiate

Correct Answer: B
The adolescent in the vignette has signs and symptoms consistent with a salicylate overdose.
Salicylates are found in a number of common household medications including aspirin, oil of
wintergreen, analgesic ointments, over-the-counter medications, and topical wart removers (e.g.,
salicylic acid). Findings of salicylate toxicity include nausea, vomiting, abdominal pain, diarrhea,
diaphoresis, tachypnea, tachycardia, impaired hearing, tinnitus, altered mental status (e.g.,
confusion or coma), pulmonary edema, and hypovolemic shock. The classic acid-base
derangement found in salicylate overdose is a mixed respiratory alkalosis and metabolic acidosis.
Respiratory alkalosis results from tachypnea caused by direct stimulation of the respiratory
center. An elevated anion gap metabolic acidosis results from the salicylate’s uncoupling of the
Krebs cycle and resultant anaerobic metabolism. This acidosis can be further worsened by
hypovolemia from vomiting, insensible fluid loss through sweating and increased respirations,
and osmotic diuresis.
Acetaminophen overdose may initially be asymptomatic. Progression of symptoms in the first 24

hours includes nausea, vomiting, malaise, and fatigue. As their condition progresses, children
may develop worsening vomiting, right upper quadrant abdominal pain, tachycardia,
hypotension, and urinary output. In the late stages of acetaminophen toxicity, children develop
fulminant liver failure, coagulopathy, hypoglycemia, and hepatic encephalopathy; eventually,
death from multi-organ failure may occur.
Isopropyl alcohol is found in many common household items including hand sanitizer, solvents,
disinfectants and “rubbing alcohol”. Isopropyl alcohol is metabolized to acetone and results in
ketosis without metabolic acidosis or an elevated anion gap. Common signs and symptoms of
Isopropyl alcohol ingestion include inebriation, altered mental status (e.g., lethargy, coma).
hypotension. and tachycardia. Hearing changes are not associated with isopropyl alcohol
ingestion.
Characteristic signs of opiate overdose include altered mental status, miosis, decreased
respiratory rate or depressed respiratory status, hypothermia, hypotension, and bradycardia,
Although toxic ingestions occur in all age ranges, the pediatric population has a bimodal
distribution, with the highest incidence in children less than 6 years of age and adolescents. Age
is an important consideration in evaluating potentially toxic ingestions in children. Toddlers and
young children are very curious about their environment; an affinity for exploration with their
mouths coupled with newly increased mobility and dexterity puts them at increased risk for
ingestion. Adolescents often demonstrate risk-taking behavior and impulsiveness, or may suffer
from a substance abuse disorder, mental disorders, depression, or suicidality. These factors may
contribute to substance ingestion in an attempt to get 'high' or self-harm. Item C245
demonstrates the characteristics of childhood and adolescent ingestions.

Toxic exposure should be considered in any child with acute alteration in mental status or
seizures. multiorgan dysfunction, cardiovascular or respiratory collapse, or metabolic
derangements including acidosis. A focused, careful history can reveal key facts associated with
substance ingestion and should include.
• Pertinent 'on-scene' data provided by family member, friend, or member of the
emergency medical service team
• Substances to which the child may have been exposed/had access (including a
comprehensive list of all medications taken by any household member)
• Location of ingestion (garage, bathroom. kitchen, out-of-home)
• Timing of ingestion
• Amount of ingestion
• Form of substance ingested (liquid, pill, patch)
• Route of ingestion (oral, intravenous, inhaled, dermal, rectal)
• Immediate vs extended-release formulation (if applicable)
• Symptoms and complaints (even if not currently present)
• Any treatment administered prior to arrival for care

It is often helpful to have a friend or family member return to the place of the suspected ingestion
and perform a thorough search of all potential products, including pill bottles and common
household items (e.g., chemicals, cleaners, bathroom products).
Cornerstones of care for the acutely poisoned pediatric patient include assessment and
stabilization of airway, breathing, circulation and disability in conjunction with decontamination,
evaluation for evidence of a particular toxidrome, and administration of antidote if indicated. The
poison control center should be contacted at 1-800-222-1222.
PREP Pearls
• Findings of salicylate toxicity may include nausea, vomiting, abdominal pain, changes in
hearing, tinnitus, diaphoresis, tachypnea, tachycardia, and altered mental status coupled
with a mixed respiratory alkalosis and metabolic acidosis on serum blood gas analysis.
• Age is an important factor in determining the type and amount of substance ingested as
well as the time to presentation after ingestion has occurred.
• A focused history should be obtained for any child with suspected or confirmed
ingestion; this should include the substance, timing, amount, form, and route of ingestion.
The poison control center should be contacted at 1-800-2221222.

• Understand the effects of a patient's age when exposed to a toxic substance environment
• Understand how to obtain a history of exposure to toxic substances in the environment
Suggested Readings
• Poisoning. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed; 2017;
2924-2950
• Toxic ingestions: initial management. Pediatr Rev, doi:10.1542/pir.2017-0119
• The poisoned pediatric patient. Pediatr Rev, doi:10.1542/pir.2016-0130

Question 246
A 15-month-old female infant is seen in the emergency department for vomiting and diarrhea.
She was in her usual state of good health until 12 hours ago, when she began to experience
nonbilious, nonbloody emesis. Over the past 4 hours, she developed watery, nonbloody diarrhea.
She has a poor appetite and has vomited after trying small amounts of commercially available
oral rehydration solution. She is making fewer wet diapers than usual. Her older sibling has had
similar, but milder symptoms over the past 2 days. On physical examination, the girl's
temperature is 38.5 ºC. She appears tired and irritable but consolable. Her mucous membranes
are dry. She is slightly tachycardic with normal respiratory effort. Her abdomen is soft, mildly
distended, and nontender. Her extremities feel cool but are not mottled. Attempts at rehydration
in the emergency department with small amounts of oral rehydration solution are unsuccessful,
resulting in vomiting.
Of the following, the medication MOST likely to reduce this child's likelihood of hospitalization
is
A. loperamide
B. metronidazole
C. ondansetron
D. promethazine

Correct Answer: C
The infant in the vignette has history and physical examination findings consistent with acute
gastroenteritis (AGE) with vomiting, diarrhea, anorexia, and fever in the context of a sick
contact. She meets criteria for mild to moderate dehydration (Item C246A). A one-time dose of
Ondansetron has been shown decrease the need fluid therapy and hospitalization in children in e
emergency department with acute gastroenteritis.
The first step in treating a child with vomiting is to assess hydration status and quickly evaluate
for surgical or life-threatening causes of vomiting. Dehydration, if present, should be
appropriately corrected.
Vomiting is a common symptom seen in children. It can be a sign of intestinal or extra-intestinal

disease; activation of mechanical, blood-borne toxin, motion/vestibular response, or emotional
response pathways can result in vomiting (Item C246B). When appropriate, pharmacologic
management may be considered when targeted to treat the underlying mechanism.

The mechanical pathway for vomiting is activated with intestinal stretch (e.g., AGE), and/or by
the presence of intestinal toxins (e.g., food poisoning). Because this results in activation of 5-
hydroxytryptamine (5-HT) and neurokinin receptors, administration of a 5-HT receptor
antagonist (e.g., ondansetron. granisetron, ginger) to treat vomiting may be appropriate (Item
C246C).
Blood borne toxins (e.g., cisplatin) induce vomiting via dopamine and 5-HT receptor activation;
thus, administration of a 5-HT receptor antagonist or a dopamine receptor antagonist (e.g.,
prochlorperazine, metoclopramide) could be recommended. Vomiting related to activation of the
motion/vestibular pathway (e.g., riding in a car) involves muscarinic and histamine receptors;
thus, administration of a muscarinic (e.g., scopolamine) and/or histamine (e.g.,
diphenhydramine) receptor antagonist could be considered.
Promethazine is an antiemetic medication that targets H1 (histamine)-receptors activated in the

vestibular pathway and its use in children under age 2 years is contraindicated; therefore, it
should not be administered to the child in the vignette. Loperamide, an antidiarrheal medication,
is not indicated for the treatment of acute gastroenteritis. Metronidazole is indicated for the

management of Clostridioides difficile (Clostridium difficile) colitis. amoebic colitis, and severe
giardiasis. but would not be helpful to treat this child.
Item C246C
PREP Pearls
• The differential diagnosis for vomiting is broad and includes intestinal and extraintestinal
causes.
• Understanding the underlying mechanism is important when considering pharmacologic
treatment of vomiting
• A one-time dose of ondansetron has been shown to decrease the need for intravenous
fluid therapy and hospitalization in children in the emergency department with acute
gastroenteritis

• Recognize the association of vomiting with a systemic illness
• Understand the role of serotonin receptor antagonists in the prevention and treatment of
vomiting
• Recognize the role of vomiting in the clinical presentation of acute gastroenteritis
Suggested Readings
• Acute gastroenteritis. Pediatr Rev. doi:10.1542/pir.33-11-487
• Evidence-based guidelines for the management of acute gastroenteritis in children in
Europe: update 2014. J Pediatr Gastroenterol Nutr,
doi:10.1097/MPG.0000000000000375
• Comparison of Recommendations in Clinical Practice Guidelines for Acute
Gastroenteritis in Children. J Pediatr Gastroenterol Nutr,
doi:10.1097/MPG.0000000000001133
• Vomiting In children. American Academy of Pediatrics Textbook of Pediatric Care. 2nd
ed. 2017:1662-1665

Question 247
A 15-year-old adolescent girl is brought to the clinic for a health supervision visit after her
parents leave the room, she confides that she is sexually active with her 16-year-old boyfriend,
does not use condoms, and would like testing for pregnancy and sexually transmitted infections.
Her parents do not know that she is sexually active.

A. encourage her to discuss her sexual activity with her parents
B. explain that this testing requires parental consent
C. inform the adolescent's parents of her request for testing
D. perform the test only after the adolescent discusses it with her parents

Correct Answer: A
The adolescent in the vignette should be encouraged to discuss her sexual activity with her
parents; however, testing should not be withheld until a discussion occurs. Confidentiality is a
core ethical tenet of pediatricians, particularly as it pertains to adolescent patients. It is important
for pediatricians to be aware of their states' confidentiality laws, as they vary from state to state.
However, in all situations involving adolescents confiding intimate health details, creating a
trusting environment is essential to adolescent health care. Without confidentiality protection,
adolescents may forgo care. As much as possible, respect for adolescent confidentiality should
be maintained, except in circumstances in which the patient poses a danger to herself or to
others.
While adolescents are developing autonomy and adult decision-making capacity, they may
engage in risk-taking behaviors (e.g., unprotected sex) that may lead to lifelong consequences.
Therefore, the adolescent health supervision visit represents a critical time for pediatricians to
screen for and counsel on these risky behaviors. Furthermore, pediatricians should encourage
adolescents to maintain open lines of honest communication with their parents, particularly as it
relates to intimate health concerns.
Providing adolescents testing for pregnancy and sexually transmitted infections does not require
parental consent. Pediatricians should neither wait to provide testing nor breach confidentiality
by informing parents of the adolescent’s request. While the adolescent in the vignette should be
encouraged to discuss her sexual activity with her parents, testing should not be withheld until
this discussion occurs.
PREP Pearls
• As much as possible, respect for adolescent confidentiality should be maintained, except
in circumstances in which the patient poses a danger to himself/herself or to others.
• It is important for pediatricians to be aware of their states' confidentiality laws as they
vary from state to state.
• Pediatricians should encourage adolescents to maintain open lines of honest
communication with their parents, particularly as it relates to intimate health concerns.

• Recognize and apply ethical principles involved in the patient-parent-pediatrician
relationship regarding issues of confidentiality

Suggested Readings
• Unique needs of the adolescent. Pediatrics. doi:10.1542/peds.2019-3150
• Ensuring access to care and reporting sexual activity and abuse. J Adolesc Health.
https://doi.org/10.1016/j.jadohealth.2004.09.001
• Confidentiality in adolescent health care. Obstet Gynecol,
doi:10.1097/AOG.0000000000003770
• Confidential health care for adolescents: Position paper for the society for adolescent
medicine. J Adolesc Health. PMID: 15298005
• Challenges of Health care delivery to adolescents. American Academy of Pediatrics
Textbook of Pediatric Care. 2nd ed. 2017: 1135-1141
• The Center for Adolescent Health & the Law. Policy Compendium on Confidential
Health Services for Adolescents 2nd ed. https://www.cahl.org/policy-compendium-2nd-
2005

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PREP ® Self-Assessment PREPSA 2022

American Academy of Pediatrics 1

American Academy of Pediatrics 2

American Academy of Pediatrics 3

Item C1B: Causes of Delayed eruption

American Academy of Pediatrics 4

Prevent the transmission of caries-causing bacteria from adult to infant:

Item C1E: Fluoride supplementation

American Academy of Pediatrics 5

Item C1G gingival hypertrophy

American Academy of Pediatrics 6

Item C1I gastroesophageal reflux disease lingual dental erosion

American Academy of Pediatrics 7

ABP Content Specifications

American Academy of Pediatrics 8

American Academy of Pediatrics 9

Laboratory results are shown:

Laboratory Test Result

Of the following, the MOST likely etiology of the girl's hypocalcemia is

American Academy of Pediatrics 10

Hypoparathyroidism may be caused by genetic disorders, autoimmune destruction of the

Treatment of symptomatic hypocalcemia is with an intravenous infusion of calcium. In the long

American Academy of Pediatrics 11

ABP Content Specifications

American Academy of Pediatrics 12

Laboratory Test Result

A. high serum osmolality high urine osmolality, low urine sodium

American Academy of Pediatrics 13

American Academy of Pediatrics 14

ABP Content Specifications

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Tinea capitis manifests along a spectrum of 5 clinical presentations:

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ABP Content specifications

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Item C5A: Hepatitis differential diagnosis

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ABP Content specifications

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Item C6B: Risk factors for birth injuries

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ABP Content Specifications

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Of the following, the BEST next step is to

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ABP Content specifications

American Academy of Pediatrics 29

Of the following, the BEST next step in management is to

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It would be inappropriate to start a blood pressure-lowering agent, such as amlodipine or

An important secondary cause of hypertension to consider, in addition to renal, endocrine, and

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Item C9: Pneumonia in children by age