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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG - 01 GUIDELINES ON MANAGEMENT OF THE INFANT WHO IS AT RISK FOR EARLY ONSET NEONATAL SEPSIS Prevention of Early Onset Sepsis (EOS) in term and preterm infants who are at risk for, i.e. whose mothers have an indication for intrapartum antibiotic prophylaxis (IAP) or have chorioamnionitis.

PURPOSE:

APPLICABLE BY: All pediatricians DATE: Created: May 9, 2005 Revised: September 15, 2009 Prevention of Perinatal Group B Streptococcal Disease. Revised Guidelines from CDC. MMWR Morb Mortal Wkly Rep 2002; 51(RR-11): 1.

REFERENCE:

GUIDELINES: I. INDICATIONS OF INTRAPARTUM ANTIBIOTIC PROPHYLAXIS: These indicate the infants who are at risk for EOS. 1. Pregnant women with a positive screening culture, for GBS, from either vagina or rectum unless a planned cesarean delivery is performed in the absence of labor or membrane rupture. 2. Pregnant women who gave birth to a previous infant with invasive GBS disease. 3. Pregnant women with documented GBS bacteriuria during the current pregnancy. 4. Pregnant women whose culture status is unknown (culture not performed or results not available) and who have any of the following: Delivery at < 37 weeks of gestation. Amniotic membrane rupture for 18 hours. Intrapartum temperature 38C.

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II. MANAGEMENT OF THE INFANT WHOSE MOTHER HAS RECEIVED IAP: A. At any gestational age: If the infant is ill-appearing or sepsis is otherwise strongly suspected, a full diagnostic evaluation including a CBC with differential, a blood culture, and a lumbar puncture (unless the clinical status dictates otherwise) should be done and empiric antibiotic treatment initiated using ampicillin and gentamicin until laboratory results are known. A chest radiograph should be obtained if respiratory symptoms are present. B. Infants born at < 35 weeks of gestation: If the infant is healthy-appearing and IAP with penicillin, ampicillin, or cefazolin was administered to the mother at least 4 hours prior to delivery, a CBC with diff. and blood culture should be obtained and the infant observed without antibiotic treatment for at least 48 hrs. Empiric antibiotics are initiated if: There is a clinical suspicion for sepsis. A change occurs in clinical status. The blood culture yields GBS. IAP administered < 4 hrs prior to delivery. C. Infants born at 35 weeks of gestation: If the infant is healthy-appearing and IAP with penicillin, ampicillin, or cefazolin was administered to the mother at less than 4 hours prior to delivery, a CBC with diff. and blood culture should be obtained and the infant observed without antibiotic treatment for at least 48 hrs. Empiric antibiotics are initiated if: A change occurs in clinical status. The blood culture yields GBS. If the infant is healthy-appearing and IAP with penicillin, ampicillin, or cefazolin was administered to the mother at least 4 hours prior to delivery, the infant does not need further evaluation but should be observed in the hospital without antibiotic treatment for at least 48 hours.

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Prevention of Early Onset Sepsis (NCG-1 addendum)


Positive Screening Culture for GBS GBS Bacteriuria During Current Pregnancy Previous Infant with Invasive GBS Disease Unknown GBS Status with any of: Delivery at < 37 wk ROM 18 hrs Intrapartum Temp. 38C Chorioamnionitis

Intrapartum Antibiotic Prophylaxis IAP


Signs of Neonatal Sepsis?
No Yes

Maternal Antibiotic Therapy

Mother received IAP 4 hrs prior to delivery?


Yes No

GA < 35 wk
Yes No

GA < 35 wk
Yes No

No Evaluation No Therapy Observe for 48 hrs

Limited evaluation: CBC with Differential Blood Culture Observe 48hrs Full Septic Screen and Start I.V. Antibiotics if: Change in clinical status Blood culture yields GBS

Full Septic Screen: CBC with Differential Blood Culture Lumbar Puncture Chest X-ray, if indicated Start I.V. Antibiotics

Adapted from: Prevention of Perinatal Group B Streptococcal Disease. Revised Guidelines from CDC. MMWR Morb Mortal Wkly Rep 2002; 51(RR-11): 1. Prepared by: Dr. Ayman Abou Mehrem Reviewed by: Dr. Sameer Al-Abdi Approved by: Dr. Hesham Al-Girim
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NCG-01 The Infant at Risk of Early Onset Neonatal Sepsis

Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG - 02 GUIDELINES ON IMMUNIZATION OF PRETERM & LOW BIRTH WEIGHT INFANTS To ensure that proper vaccines are given at the proper time for preterm and low birth weight infants.

PURPOSE:

APPLICABLE BY: All pediatricians DATE: Created: May 22, 2005 Revised: September 15, 2009 1- Saudi Basic Vaccination Schedule 2009. 2- Immunization of Preterm and Low Birth Weight Infants, Clinical Report from American Academy of Pediatrics, PEDIATRICS Vol. 112 No. 1 July 2003 pages 193-198.

REFERENCE:

GUIDELINES: I. GENERAL CONSIDERATIONS 1. Timing Medically stable premature and low birth weight infants should receive all routinely recommended childhood vaccines at the same chronological age as recommended for full term infants. Infants with birth weight < 2000 g may require modification of the timing of hepatitis B immunoprophylaxis depending on maternal HBsAg status. 2. Dosage Use same vaccine dosages given to full term infants. 3. Vaccine Administration The anterolateral thigh is the site of choice for intramuscular vaccines. The choice of needle length is made on the basis of the available muscle of the infant and may be less than the standard length used for full term infants.

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II. HEPATITIS B: TABLE 1: Hepatitis B Immunoprophylaxis Scheme for PT and LBW Infants* Maternal Status

Infant 2000 g

Infant <2000 g

HBsAg positive

Hepatitis B vaccine + HBIG (within 12 h of birth) Immunize with total of 4 vaccine doses at 0, 2, 4, and 6 mo of chronologic age Check anti-HBs and HBsAg at 915 mo of age

Hepatitis B vaccine + HBIG (within 12 h of birth) Immunize with total of 4 vaccine doses at 0, 2, 4, and 6 mo of chronologic age Check anti-HBs and HBsAg at 915 mo of age

If infant is HBsAg and anti-HBs If infant is HBsAg and anti-HBs negative, negative, reimmunize with 3 reimmunize with 3 doses at 2-mo intervals doses at 2-mo intervals and and retest retest HBsAg status unknown Hepatitis B vaccine (by 12 h) + HBIG (within 7 days) if mother tests HBsAg positive Test mother for HBsAg immediately HBsAg negative Hepatitis B vaccine at birth Hepatitis B vaccine + HBIG (by 12 h)

Test mother for HBsAg immediately and if results are unavailable within 12 h, give infant HBIG Hepatitis B vaccine dose 1 at 30 days of chronologic age if medically stable, or at hospital discharge if before 30 days of chronologic age Immunize with total of 4 doses at 1, 2, 4, and 6 mo of chronologic age

Immunize with total of 4 doses at 0, 2, 4, and 6 mo of chronologic age

May give hepatitis B-containing May give hepatitis B-containing combination vaccine beginning combination vaccine beginning at 68 wk at 68 wk of chronologic age of chronologic age Follow-up anti-HBs and HBsAg Follow-up anti-HBs and HBsAg testing testing not needed not needed Extremes of gestational age and birth weight no longer a consideration for timing of HBV doses.

Some experts prefer to perform serologic testing 1 to 3 months after completion of the primary series.

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III. BCG (Bacillus Calmette-Guerin): BCG vaccine should be given intradermally in the left arm to stable preterm infants when they become 2000 g. IV. V. VI. VII. DTP (Diphtheria, Tetanus, and Pertussis vaccine) Hib (Haemophilus influenzae vaccine) PCV (Pneumococcal Conjugated Vaccine) IPV (Injectable Polio Vaccine)

All medically stable premature and LBW infants should begin routine childhood
immunization with full doses of DTP, Hib, PCV, and IPV at 2 months of chronological age regardless of gestational age or birth weight. Hospitalized extremely LBW infants born at < 31 weeks gestation should be observed for 72 hrs after receiving pertussis vaccine for significant apnea. If IPV is not available, give OPV at the time of discharge.

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: PURPOSE: NCG-03 TETANUS PROPHYLAXIS FOR NEWBORNS To prevent neonatal tetanus

APPLICABLE BY: All pediatricians DATE: May 2005, Updated September 2009

GUIDELINES: 1. If umbilical cord is cleanly cut in Emergency Room: Routine admission for observation in postnatal ward. If umbilical cord is cut with a non-sterile instrument: Administer tetanus immunoglobulin - 250 units IM Give tetanus toxoid - 0.5 ml IM Consider antibiotics penicillin G is the antibiotic of choice

2.

NCG-03-Tetanus Prophylaxis for Newborns

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG - 04 GUIDELINES ON SCREENING PREMATURE INFANTS FOR PREMATURITY (ROP) EXAMINATION RETINOPATHY OF OF

PURPOSE:

Early detection of Retinopathy of Prematurity in premature infants who are at risk

APPLICABLE BY: Neonatologists, Pediatricians working in Neonatology Service, and Ophthalmologists DATE: Created: May 15, 2005 Updated: September 2009 See page 4

REFERENCES:

GUIDELINES: I. INTRODUCTION: Retinopathy of Prematurity (ROP) is the disease of the developing retina. It occurs mainly in premature babies and leads to significant and devastating sequela, namely blindness. The aim is to reduce the incidence of the disease. Scheduled screening examination for babies at risk is implemented for early detection of the disease and early intervention attempting to spare vision.

II.

MEASURES TO REDUCE INCIDENCE OF ROP:


1. 2. 3.

Strict management of O2 delivery and monitoring.(1) Using breastmilk for feeding.(2) Minimizing blood transfusions.(3)

III. INFANTS SHOULD BE SCREENED: (4)


1. 2.

Infants with birth weight 1500 g. Infants with gestational age 32 weeks.

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3.

Selected infants with a birth weight between 1500 and 2000 g or gestational age of more than 32 weeks with an unstable clinical course, including those requiring cardiorespiratory support and who are believed by their attending pediatrician or neonatologist to be at high risk.

IV. SCREENING PROCEDURE:


1.

Pupil dilatation using Tropicamide 0.5 %, and Phenylephrine 2.5 % eye drops, 1 drop of each solution in each eye every 10 minutes for 3 times, 30-45 minutes before fundoscopy. Apply pressure to the lacrimal sac during and for 2 minutes after instillation to minimize systemic absorption.(13) Binocular indirect ophthalmoscopy (fundoscopy), which should be carried out by an ophthalmologist who has sufficient knowledge and experience to enable accurate identification of the location and sequential retinal changes of ROP.(4)

2.

3.

V.

TIMING OF THE EXAMINATION: (4) Examination should be performed at 4 weeks of chronological age or at 31 weeks of postmenstrual age, which ever is the later, and as determined by the attending neonatologist, or pediatrician. (Postmenstrual age is defined as gestational age at birth plus chronological age.) TABLE 1: Timing of First Eye Examination Based on Gestational Age at Birth Age at Initial Examination, wk Postmenstrual Chronological 31 9 31 8 31 7 31 6 31 5 31 4 32 4 33 4 34 4 35 4 36 4

Gestational Age at Birth, wk 22a 23a 24 25 26 27 28 29 30 31b 32b

Shown is a schedule for detecting prethreshold ROP with 99% confidence, usually well before any required treatment. This guideline should be considered tentative rather than evidence-based for infants with a gestational age of 22 to 23 weeks because of the small number of survivors in these gestational-age categories.
b a

If necessary.
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NCG-04 Retinopathy of Prematurity

VI. PRECAUTIONS DURING PROCEDURE: (7-13)


1. 2.

Feeding should be withheld during and for 4 hours following procedure. Observe for systemic anticholinergic effects: tachycardia, fever, flushing, dry mucous membranes, urinary retention, etc.

VII. DOCUMENTATION OF THE OPHTHALMIC EXAMINATION: (4) The ophthalmologist should determine the severity of the disease (using International Classification of Retinopathy of Prematurity, ICROP) and identify the location (by zone) and extent (by clock hours).

VIII. FOLLOW-UP PLANING: (4)


1.

Scheduling of follow-up examinations is determined by the finding at the first examination using ICROP. One-week or less follow-up: Stage 1 or 2 ROP: zone I Stage 3 ROP: zone II

2.

3. One- to two-week follow-up: Immature vascularization: zone Ino ROP Stage 2 ROP: zone II Regressing ROP: zone I 4. Two- to three-week follow-up: Immature vascularization: zone IIno ROP Stage 1 or 2 ROP: zone III Regressing ROP: zone III

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5. The presence of plus disease (defined as dilation and tortuosity of the posterior retinal blood vessels, as defined by a standard photograph) in zones I or II suggests that peripheral ablation, rather than observation, is appropriate.

IX. REFERENCES:
1. Chow LC, Wright KW, Sola A; CSMC Oxygen Administration Study Group. Can changes in clinical practice decrease the incidence of severe retinopathy of prematurity in very low birth weight infants? Pediatrics. 2003 Feb;111(2):339-45. Hylander MA, Strobino DM, Pezzullo JC, Dhanireddy R. Association of human milk feedings with a reduction in retinopathy of prematurity among very low birthweight infants. J Perinatol. 2001 Sep;21(6):356-62. Seiberth V, Linderkamp O. Risk factors in retinopathy of prematurity. a multivariate statistical analysis. Ophthalmologica. 2000;214(2):131-5. Section on Ophthalmology American Academy of Pediatrics; American Academy of Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus. Screening examination of premature infants for retinopathy of prematurity. Pediatrics. 2006 Feb;117(2):572-6. Erratum in: Pediatrics. 2006 Sep;118(3):1324. Binkhathlan AA, Almahmoud LA, Saleh MJ, Srungeri S. Retinopathy of prematurity in Saudi Arabia: incidence, risk factors, and the applicability of current screening criteria. Br J Ophthalmol. 2008 Feb;92(2):167-9. Larsson E, Holmstrm G. Screening for retinopathy of prematurity: evaluation and modification of guidelines. Br J Ophthalmol. 2002 Dec;86(12):1399-402. Hermansen MC, Sullivan LS. Feeding intolerance following ophthalmologic examination. Am J Dis Child. 1985 Apr;139(4):367-8. Hermansen MC, Hasan S. Abolition of feeding intolerance following ophthalmologic examination of neonates. J Pediatr Ophthalmol Strabismus. 1985 Nov-Dec;22(6):256-7. Bonthala S, Sparks JW, Musgrove KH, Berseth CL. Mydriatics slow gastric emptying in preterm infants. J Pediatr. 2000 Sep;137(3):327-30.

2.

3. 4.

5.

6. 7. 8. 9.

10. Lim DL, Batilando M, Rajadurai VS. Transient paralytic ileus following the use of cyclopentolate-phenylephrine eye drops during screening for retinopathy of prematurity. J Paediatr Child Health. 2003 May-Jun;39(4):318-20. 11. Sarici SU, Yurdakk M, Unal S. Acute gastric dilatation complicating the use of mydriatics in a preterm newborn. Pediatr Radiol. 2001 Aug;31(8):581-3. 12. Nair AK, Pai MG, da Costa DE, Khusaiby SM. Necrotising enterocolitis following ophthalmological examination in preterm neonates. Indian Pediatr. 2000 Apr;37(4):41721. 13. Young and Mangum, Neofax, 20th ed., 2007, page 243.

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: PURPOSE: NCG - 05 GUIDELINES ON BLOOD TRANSFUSION IN NEONATES To provide proper approach for anemia in newborn

APPLICABLE BY: All pediatricians DATE: REFERENCE: May 2005, Revised September 2009 Please see last page

A.

Introduction:
RBC transfusion in preterm or sick term infants is essential in the management of their clinical conditions to prevent the effects of anemia but can carry several risks.

B.

Prevention:
delayed clamping of the umbilical cord, restricting blood sampling; using recombinant human erythropoietin to stimulate erythropoiesis; using iron supplementation or vitamins to minimize the severity of anemia; using appropriately collected and stored multipack RBC units; using appropriately screened and handled RBCs from regular or designated donors; collecting and transfusing umbilical cord blood (autologous blood transfusion).

C.

Target hemoglobin or hematocrit Target hemoglobin and hematocrit, values


below which a RBC transfusion is needed, have been used universally as clinical indicators for RBC transfusion in neonates Clinical practice progressed from using high trigger levels (hematocrit of 40 percent referred to as a "liberal" approach) to lower levels (a "restrictive approach")

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D.

Recommendations for red blood cell (RBC) Transfusions:


RBC transfusions should be considered in newborn infants in the following specific clinical situations: 1. Hypovolemic shock associated with acute blood loss 2. For infants on supplemental oxygen who are not requiring mechanical ventilation, transfusions can be considered if the hematocrit is 20 percent (hemoglobin 7 gms/dL) and one or more of the following conditions is present: 2.1 2.2 2.3 2.4 2.5 2.6 24 hours of tachycardia (heart rate >180 beats per minute) or tachypnea (RR >60 breaths per minute) Doubling of the oxygen requirement from the previous 48 hours Serum lactate 2.5 mEq/L or an acute metabolic acidosis (pH <7.20) Weight gain <10 grams/kg/day over the previous 4 days while receiving 120 kcal/kg/day If the infant will undergo major surgery within 72 hours reticulocyte count <100,000 cells/L (<2 percent).

3. High threshold is considered in the first 2 weeks in sick or ventilated babies. Otherwise, low threshold is recommended as shown in table 1.

Table I. Hemoglobin threshold levels (g/L) triggering RBC transfusion Low threshold Age in days Blood sampling Capillary Central Capillary Central Capillary Central Respiratory support 115 104 100 90 85 77 No respiratory support 100 90 85 77 75 68 High threshold Respiratory support 135 122 120 109 100 90 No respiratory support 120 109 100 90 85 77

1-7 8-14 15

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E.

References: 1.
Kirpalani, H, Whyte, RK, Andersen, C. The Premature Infants in Need of Transfusion (PINT) study: a randomized, controlled trial of a restrictive (low) versus liberal (high) transfusion threshold for extremely low birth weight infants. J Pediatr 2006; 149:301. Bell, EF, Strauss, RG, Widness, JA. Randomized Trial of liberal versus restrictive guidelines for red blood cell transfusion in preterm infants. Pediatrics 2005; 115:1685. Fetus and Newborn Committee, Canadian Paediatric Society (CPS) Paediatrics & Child Health 2002;7(8):553-8 Reference No. FN02-02 British Journal of Haematology, 124, 433453Transfusion guidelines for neonates and older children When to transfuse preterm babies, Arch Dis Child Fetal Neonatal Ed. 2008 November Neurodevelopmental Outcome of Extremely Low Birth Weight Infants Randomly Assigned to Restrictive or Liberal Hemoglobin Thresholds for Blood Transfusion, PEDIATRICS Vol. 123 No. 1 January 2009, pp. 207-213 Red blood cell transfusions in the newborn up-to-date 2008

2.

3. 4. 5. 6.

7.

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Kingdom of Saudi Arabia National Guard Health Affairs


King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG - 06 PREVENTION AND MANAGEMENT OF HYPOGLYCAEMIA OF THE NEWBORN HYPOGLYCAEMIA IS LEADING AND TREATABLE CAUSE OF MORBIDITY AND MORTALITY IN NEWBORN SO EARLY RECOGNITION IS NECESSARY TO ENSURE APPROPRIATE THERAPY AND PREVENTION OF FATALITIES. ALL PEDIATRICIANS May 2005, September 2009 See page

PURPOSE:

APPLICABLE BY: DATE: REFERENCE:

I.

DEFINITION: 1. Plasma Glucose < 2.6 mmoL/liter in term or preterm infant.

II.

SIGNS AND SYMPTOMS OF NEONATAL HYPOGLYCEMIA 1. Hypothermia 2. Hypoactive 3. Lethargy 4. Poor feeding 5. Jitterness 6. Apnea 7. Tachycardia 8. Sweating 9. Vomiting 10. Pallor 11. Cyanosis 12. Heart failure

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III.

RISK FACTOR: 1. 2. 3. 4. 5. 6. 7. 8. Small for gestational age Large for gestational age Preterm Post term Polycythemic Infant of diabetic mother Inborn error metabolism Endocrine disorder

IV.

PREVENTION: 1. If the baby is unwell, check the glucose stat and treat any underlying condition. If Reflo is < 2.6 mmol/L in an unwell baby, consider IV treatment (Algorithm 1) 2. If the baby is at risk (SGA, LGA, IDM, or preterm): a. Check glucose at 1-2 hours and every 3-6 hours (before feeds) as long as infant remains well, until feeds are established and glucose > or = to 2.6 mmol/liter. b. If reflo <1.8 mmoL/liter at 2 hours or age or < 2.0 mmoL/Liter at subsequent checks, consider IV treatment c. If reflo is 1.8 2.0 mmoL/liter at subsequent checks, refeed and recheck glucose in 1 hour. d. If still remains < 2.6 mmoL/liter despite feeding, consider IV treatment. e. If rises to > or = to 2.6 mmoL/liter after feeding, routine care.

V.

SYMPTOMATIC HYPOGLYCEMIA: 1. If baby has system suggestive of Hypoglycaemia, do bedside gluco check 2. I.V. glucose bolus a. In the presence of convulsion (4ml/kg) of 10% glucose b. Symptom other than seizures (2ml/kg) of 10% glucose 3. Then give continuous infusion of glucose at rate of 80 ml/kg/day 10% dextrose (5.5 mg/kg/min) 4. Check glucose 30 minutes after any change and adjust therapy (up to 100 ml/kg/day and/or 12.5% dextrose) in order to maintain glucose level > or = to 2.6mmol/liter. If still blood glucose < 2.6 mmol/liter, do investigation, endocrine consultation and pharmacological intervention are indicated. May start weaning I.V. 12 hours after stable blood glucose is established. Continued breastfeeding is encouraged. 5. If IV catheter cannot be inserted glucagon can be given to infant with adequate glycogen stores, the dose is 0.2 mg/kg (S.C., I.M.) not to exceed 1mg per dose.

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VI.

PERSISTENT HYPOGLYCOMA: Hypoglycoma persists or recurs over a period of > 7 days. 1. Continue administration of IV glucose and increase rate to 16-20 mg/kg/min by increasing the concentration from 10% DW to 20% DW. 2. Investigations: a. If blood glucose is less than < 2.6mmol/Liter, draw blood sample for laboratory studies which include: serum glucose, ketones, free fatty acids, lactate, alamine, UA, C-peptide insuline, GH, cortisol, glucagons, T4, TSH, urine collection for catecholamines, organic acids, and specific reducing substances. 3. Other Medications: a. If the infant requires more than 12 mg/kg/min. - Hydrocortisone 5mg/kg/day IV or orally 6-8 hours divided doses or prednisolone 2mg/kg. b. If hypoglycoma persist, you can use: Diazoxide 2-5 mg/kg/dose orally Q8 hours. Octreotide (long-acting somatostatin, the starting dose is 2-10 mcg/kg/day (SC) divided every 6-8 hours, you can increase the dose up to 40 mcg/kg/day. c. Glucagon: 0.2 mg/kg. maximum 1 mg/dose can be repeated in 30 minutes.

VII.

REFERENCES: 1. 2. 3. 4. 5. Neonatal Hypoglycemia Guideline of Canadian Pediatric Society Neonatology, Tricia Lacy Gomella 5th Ed. 2007. Manual of Neonatal Care, 200 by Lippin Colt Williamson, Wilkins) Up to date Neonatal hypoglycemia Nelson 2008

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Algorithm for Management of Babies at Risk Prevention and Management of Hypoglycaemia in at Risk Neonates Small for gestational age (BW, 10th centile Preterm , 37 weeks Infants of Diabetic Mothers Hypoxia/ Ischaemia LGA babaies (BW .90th centile) Hypothermia Infection Severe Rhesus Disease Polycythaemia

Feed early, 1 hr to prevent hypoglycaemia Feeds well and clinically well Does not feed well. Check Reflo 1-2 hr of

Check Reflo prior to feed at 3 hours

If <2.6 mmol/L

>2.6 mmol/L NOT feeding well

>2.6 mmol/L

< 2.6 mmol/

Attempt a feed either at breast or give EBM / formula by cup or tube

Review in 1-hour attempt to feed and repeat Post feed Reflo at 1 hour

Repeat Reflo 1 hr post feed

If still poor feeding or Reflo <2.6mmol/L

Satisfactory feeding continue 3 hrly feeds and prefeed Reflo until 3 consecutive BM are >2.6mmol/L

<2.6 mmol/L

Contact Pediatrician

Venous glucose

blood

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: PURPOSE: NCG-07 GUIDELINES FOR THE USE OF PALIVIZUMAB To provide guidelines and recommendations for the use of Palivizumab for the prevention of respiratory syncytial virus infections. This policy applies to all Pediatric Physicians and all licensed nursing staff in Pediatric Department September 2009

APPLICABLE BY:

DATE: REFERENCE:

I.

INTRODUCTION: RSV is estimated to cause 90% of all bronchiolitis 40 % of those hospitalized have an underlying condition such as prematurity or chronic lung disease Palivizumab(synagis) has been approved as a prophylaxis for RSV and has been shown to reduce hospitalization for RSV

II.

POLICY/PROCEDURE: A. Eligibility Criteria for Prophylaxis of High-Risk Infants and Young Children: 1. Infants with CLD: Palivizumab prophylaxis may be considered for infants and children younger than 24 months of age with CLD who receive medical therapy (supplemental oxygen, bronchodilator, diuretic or chronic corticosteroid therapy) for CLD within 6 months before the start of the RSV season. These infants and young children should receive a maximum of 5 doses. Patients with the most severe CLD who continue to require medical therapy may benefit from prophylaxis during a second RSV season 2. Infants born before 32 weeks gestation (31 weeks, 6 days or less):

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Infants in this category may benefit from RSV prophylaxis, even if they do not have CLD. For these infants, major risk factors to consider include gestational age and chronologic age at the start of the RSV season. Infants born at 28 weeks of gestation or earlier may benefit from prophylaxis during the RSV season, whenever that occurs during the first 12 months of life. Infants born at 29 to 32 weeks (31 weeks, 6 days or less) of gestation may benefit most from prophylaxis up to 6 months of age. Administration should continue throughout the season and not stop when the infant reaches either 6 months or 12 months of age. A maximum of 5 monthly doses is recommended for infants in this category 3. Infants born at 32 to less than 35 weeks gestation (defined as 32 weeks, 0 days through 34 weeks, 6 days): Prophylaxis may be considered for infants from 32 through less than 35 weeks gestation (defined as 32 weeks, 0 days through 34 weeks, 6 days) who are born less than 3 months before the onset or during the RSV season and for whom at least 1 of the 2 following risk factors is present: 3.1 3.2 The infant attends child care, defined as a home or facility where care is provided for any number of infants or toddlers in the child care facility; or One or more siblings or other children younger than 5 years live permanently in the same household.

4. Infants with congenital abnormalities of the airway or neuromuscular disease: Infants and young children in this category should receive a maximum of 5 doses of palivizumab during the first year of life 5. Infants and children with CHD: 5.1 5.2 Children who are 24 months of age or younger with hemodynamically significant cyanotic or acyanotic CHD. Immunoprophylaxis include: 5.2.1 Infants who are receiving medication to control congestive heart failure; 5.2.2 Infants with moderate to severe pulmonary hypertension; and 5.2.3 Infants with cyanotic heart disease 5.3 The following groups of infants with CHD are NOT at increased risk of RSV and generally should not receive immunoprophylaxis: 5.3.1 Infants and children with hemodynamically insignificant heart disease (e.g. secundum atrial septal defect, small ventricular septal

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5.3.2 5.3.3

Infants with lesions adequately corrected by surgery, unless they continue to require medication for congestive heart failure; and Infants with mild cardiomyopathy who are not receiving medical therapy for the condition.

6. Immunocompromised children Specific recommendations for immunocompromised children cannot be made, infants and young children with severe immunodeficiency (e.g., severe combined immunodeficiency or advanced acquired immunodeficiency syndrome) may benefit from prophylaxis. 7. Patient with Trisomy 21 may benefit from prophylaxis. B. Special situations 1. If an infant or child who is receiving palivizumab immunoprophylaxis experiences a breakthrough RSV infection, monthly prophylaxis should continue until a maximum number of 3 doses has been administered to infants in the 32 weeks, 0 days through 34 weeks, 6 days gestational age group or until a maximum of 5 doses has been administered to infants with CHD, CLD, or preterm birth before 32 weeks gestation. This recommendation is based on the observation that high-risk infants may be hospitalized more than once in the same season with RSV lower respiratory tract disease and the fact that more than one RSV strain often co-circulates in a community. 2. Hospitalized infants who qualify for prophylaxis during the RSV season should receive the first dose of palivizumab 48 to 72 hours before discharge or promptly after discharge. 3. Infants who have begun palivizumab prophylaxis earlier in the season and are hospitalized on the date when the next monthly dose is due, should receive that dose as scheduled while they remain in the hospital. 4. RSV is known to be transmitted in the hospital setting and to cause serious disease in high-risk infants. Among hospitalized infants, the major means to reduce RSV transmission is strict observance of infection control practices, including prompt 5. Initiation of precautions for RSV-infected infants 6. If an RSV outbreak occurs in a high-risk unit (e.g. pediatric or neonatal intensive care unit or stem cell transplantation unit), primary emphasis should be placed on proper infection control practices, especially hand hygiene. No data exist to support palivizumab use in controlling outbreaks of health careassociated disease, and palivizumab use is not recommended for this purpose. 7. Palivizumab does not interfere with response to vaccines.

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C. Administration 1. Dose: By intramuscular injection, 15 mg/kg in the anterolateral aspect of the thigh. It comes in vials of 100 mg, and opened vials must be used within 6 hours. 2. Frequency: monthly (must have the vaccine ideally within 2 days of the monthly date) until the end of the RSV season. The Pharmacy Department should be notified when each dose is required. 3. When to start: The inevitability of the RSV season is predictable. Substantial variation in the timing of community outbreaks of RSV disease from year to year exists within and between communities in the same year, even in the same region, but usually beginning in November or December. In each September the timing of starting palivizumab will be decided after discussion between pediatric infectious disease consultant, neonatologist, pulmonologist. D. Maximum Number of Monthly Doses of Palivizumab for Respiratory Syncytial Virus

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E. Table: Maximum number of palivizumab doses for RSV prophylaxis of preterm infants without CLD, based on birth date, gestational age and presence of risk factors (shown for areas beginning prophylaxis on November 1st)a
Month of birth Maximum number of doses for Season Beginning November 1 < 28 weeks, 6 days 29 weeks, 0 days 32 weeks, 0 days gestation and < 12 through 31 weeks, 6 through 34 weeks, 6 months of age at days of gestation days and with risk start of season and < 6 months old factorb at start of season 0d 0e 5c

November 1 March 31 of previous RSV season April May June July August September October November December January February March
a

5 5 5 5 5 5 5 5 4 3 2 1

0d 5 5 5 5 5 5 5 4 3 2 1

0e 0e 0e 0e 1f 2f 3f 3f 3f 3f 2f 1f

If infant is discharged from the hospital during RSV season, fewer doses may be required Risk factors: infant attends childcare or has sibling younger than 5 years old c Some of these infants may have received 1 or more doses of palivizumab in the previous RSV season if discharged from the hospital during that season; if so, they still qualify for up to 5 doses during their second RSV season d Zero doses because infant will be older than 6 months of age at start of RSV season e Zero doses because infant will be older than 90 days of age at start of RSV season f On the basis of the age of patients at the time of discharge from the hospital, fewer doses may be required, because these infants will receive 1 dose every 30 days until the infant is 90 days of age
b

F. Cautions Contraindications and side-effects 1. Cautions: palivizumab is for intramuscular use only. As with any intramuscular injection, palivizumab should be given with caution to patients withthrombocytopenia or any coagulation disorders 2. Contra-indications: hypersensitivity to humanised monoclonal antibodies. 3. Side-effects: fever ,injection site reactions, Rash nervousness; less frequently diarrhea, vomiting, , rhinitis, cough, wheeze, pain, drowsiness, ALT increase, rarely apnea, hypersensitivity reactions (including, very rarely, anaphylaxis)

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III.

REFERENCES

1. American Academy of Pediatrics: Committee on Infectious DiseasesPolicy Statement 2009. 2. Saudi Task Force Committee for RSV Prophylaxis: Al-Alaiyan S.; MajeedSaidanM.A.; Al-Dabbagh M.; Al-Abdi Sameer.Y.

3. Revised Indications for the Use of Palivizumab and Respiratory Syncytial


Virus Immune Globulin Intravenous for the Prevention of Respiratory Syncytial Virus Infections Pediatrics 2003; 112; 1442- 1446

4. Neofax 2009 5. U.S. Food and Drug Administration (FDA)

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Recommendations for Use of Palivizumab for Prevention of Respiratory Syncytial Virus Infections

Chronic Lung Disease of Prematurity

.< 24 months of age with CLD who receive medical therapy (supplemental oxygen, bronchodilator, diuretic or chronic corticosteroid therapy) for CLD within 6 months before the start of the RSV season.

YES

NO

YES
prophylaxis on: November 1st to march maximum 5 doses

With/without

< 28 weeks, 6 days gestation and < 12 months of age at start of season

29 weeks, 0 days through 31 weeks, 6 days of gestation and < 6 months old at start of season

32 weeks, 0 days through 34 weeks, 6 days and with risk factor


(Infant attends childcare or has sibling younger than 5 years old)

Prophylaxis on: November 1st to march Maximum 5 doses

Prophylaxis on: November 1st to march Maximum 5 doses

Prophylaxis on: November 1st to march Maximum 3doses

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Recommendations for Use of Palivizumab for Prevention of Respiratory Syncytial Virus Infections

Congenital Heart Disease age <2years

Hemodynamically significant cyanotic or acyanotic CHD : Infants who are receiving medication to control congestive heart failure; Infants with moderate to severe pulmonary hypertension; and Infants with cyanotic heart disease

Hemodynamically insignificant heart disease: Secundum atrial septal defect, small ventricular septal defect, pulmonic stenosis, uncomplicated aortic stenosis, mild coarctation of the aorta, and patent ductus arteriosus Infants with lesions adequately corrected by surgery, unless they continue to require medication for congestive heart failure; and Infants with mild cardiomyopathy who are not receiving medical therapy for the condition.

Prophylaxis on: November 1st to march Maximum 5 doses

No Immunoprophylaxis

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: PURPOSE: NCG - 08 ENTERAL FEEDING AND SUPPLEMENTATION IN NICU TO ENSURE THAT ALL NEONATES RECEIVE OPTIMAL NUTRITION & ADEQUATE GROWTH & DEVELOPMENT

APPLICABLE BY: ALL NICU HEALTH CARE PROVIDERS DATE: 1st UPDATE REFERENCES: October 2005 September 2009 Page 5-6

Introduction (Sep.2009): The incidence of definite necrotizing enterocolitis (NEC) was dropped in our NICU from 13% to 5% after starting this guideline on October 2005.
Key Infant Feeding Terms Term Definition Ad libitum feeding Demand feeding Scheduled feeding Feeding is begun on infant cues of hunger and ended on infant cues of satisfaction of hunger, regardless of the time or volume taken. Feeding is begun on infant cues of hunger and ended when total ordered feeding volume has been taken. Feedings are begun on a timed or scheduled basis without regard to infant status. Infants are awakened from sleep to feed. Feeding is begun and ended on specified infant cues of hunger and volume of feeding is prescribed by providers, not determined by the infant. Hunger is assessed on a scheduled basis (i.e. every 3 hours). If the infant is sleeping, reassessment is done once more, 30 minutes later. If the infant remains asleep at that time, feeding is provided by gavage. If the infant displays hunger cues at either assessment, feeding is provided and ended when total volume is taken.

Semidemand or modified ad libitum

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INITIATION AND PROGRESSION OF ENTERAL FEEDING IN NICU

Birth Weight (grams) 750 751-1000 1001 -1250 1251-1500 1501-2000 >2000g / < 35 weeks

Initial Volume & Frequency of Feeding 1 cc Q 6H * 24H 1 cc Q 4H * 24H 1 cc Q 2H * 24H 1 cc Q 4H * 24H 1 cc Q 2H * 24H 1 cc Q 2H * 24H 2 cc Q 2H * 24H 2 cc Q 3H * 24H 3-5 cc Q3H * 24H 5 cc Q 3H

Rate & Volume of Feeding Increment 0.5 1 cc Q 24H 0.5 - 1 cc Q 12H 1 cc Q 12H 2 cc Q 12H 2-3 cc Q 12H 3 cc Q 6H

Demand/Semi-demand Feeding for Healthy Preterm Infants (32-34 weeks):


According to the last Cochrane review (2006), there are insufficient data at present to guide clinical practice. However, there is evidence indicates that preterm infants are ready to self-regulate feeding at 32 to 35 weeks PCA. So if these preterm infants are found ready for this type of feeding then these steps should be follow: Prescription of a total daily volume of milk. Follow the guideline for feeding advancement. Feed infants when they are awake. Limit oral feedings to 2030 minutes. Pre- and post-feeding weighing for assessment of milk volume that was feed in case of direct breast feeding.

VLBW baby (< 1500 grams)


When possible the Gavage bolus feed should be initiated on day 2 or 3 of life. But wait until breast milk is available. Breast milk is the ideal milk to be used, waiting for EBM may delay trophic feeding, and however EBM usually takes 45 days to come in. Domperidone OR metaclopromide can be given to the mother to ease lactation. After this period (up to five days) you may use full strength premature formula and hence (SC20) is not available, (SC24) can be used. Noteworthy, last Cochrane review showed that the electric or foot powered pump may be superior to the hand expression and simultaneous pumping takes less time than sequential pumping. Fortification of human milk (HMF) is recommended for these babies when full feed is established and HMF should be continued until infant weigh is 2000 grams. After discharge, these babies need higher calories in the first 6 to 12 months. This can be achieved by different ways: 1. Alternate breast milk feeding with a high calorie formula. 2. If baby is exclusively bottle-feeding use (22 Kcal/oz) formula such Neosure or S26 low birth weight formula.

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3. Add MCT oil / polycose. 4. Concentrated the stander formula. Use of Human Milk fortifier ( HMF) EBM must be older than 14 days as preterm breast milk has a protein of > 1.6 g/100cc which persists for the first 2 to 4 weeks of lactation. Mixing HMF with preterm breast milk may cause excessive protein intake. Fortification should continue until weight of 2000 g is achieved. Infants with a birth weight of < 1000 g and/or have BPD, and Infants below the 3rd percentile for corrected GA may benefit from continued feeding with HMF past a weight of 2000 g. Bone profile assessment will be done within 1 week of adding the HMF. Ongoing and/or more frequent assessment will be done based on individual needs.

Sufficient EBM Birth Weight (grams) < 1500 Age of EBM < 14 days old > 14 days old AND baby is full feed Clinically Well Unfortified EBM EBM + HMF Individual assessment for fortified EBM Unfortified EBM > 2000 Medically Compromised Unfortified EBM EBM + HMF EBM+HM

1500 2000

Individual assessment for fortified EBM

Insufficient EBM: Birth weight (grams) < 1500 Feeding Use available EBM (+HMF) Rest as Full strength SC24 Use available EBM ( HMF) Rest as Full strength SC24, may be changed to SC20 if baby does not require higher energy Use available EBM + Full strength SC 20

1500-2000 > 2000

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Vitamin Supplementation All vitamin fortification will start gradually when full enteral feeding is tolerated and will be given till 1st birthday except Folic acid until 40 weeks CGA. Regarding Vitamin D If intake of Ca & Po is adequate, there seems no justification in giving more than 400-600 IU / day. Breast Milk Breast milk + 2 packets HMF/100cc 400 IU Vit.D, 1500 IU Vit.A, 30mg Vit.C 200 IU Vit.D, 750 IU Vit.A, 15 mg Vit.C 200 IU Vit.D, 750 IU Vit.A, 15 mg Vit.C 400 IU Vit.D, 1500 IU Vit.A, 30mg Vit.C

Breast milk + 4 packets HMF/100cc

Breast milk + 5 packets HMF/100cc

Unfortified Breast milk Birth weight < 2000 g

Formula Special Care 20/24 Kcal/oz formula High calorie Formula & Birth weight < 2000 g Vit.D 200 IU Vit.A 750 IU Vit.C 15 mg Vit.D 200 IU Vit.A 750 IU Vit.C 15 mg Vit.D 400 IU Vit.A 1500 IU Vit.C 30mg

Regular formula & Birth weight < 2000 g

Folic Acid 50-100 microgram / day for VLBW (< 1500 grams) until 40 weeks postconceptional age. Iron Supplementation for Preterm infants ( : The current recommendation is to begin supplementation between 4 and 8 weeks of age or when full feed is reached, irrespective of gestational age or birth weight. At least 2 to 4 mg/kg / day of elemental iron are necessary to prevent iron deficiency in non-transfused VLBW infants. Most organizations recommend supplementation until 12 to 15 months of age. Ferrous sulfate is preferred over the others because it is used commonly for supplementation as well as for fortifying formula, inexpensive, widely available and can be administered once daily. It is recommended that other additives/supplements are not initiated on the same day. Although there are no specific recommendations for screening for iron deficiency anemia in preterm infants, it is considered prudent to screen them at 2-4 months.
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Dose of Elemental Iron Supplements:


Enteral iron intake recommendations for preterm infants in stable clinical condition Recommended Supplementation Organization Population and Dose (mg/kg/ d) Initiation Duration Infants on human milk: 2.0 Infants 1 month 12 months Committee on Nutrition, on formula milk: 1.0 During American Academy of Pediatrics Nutrition Committee, 68 weeks 12 months corrected age Birth weight R 1000 g: 2.03.0 Canadian Pediatric Society Birth weight < 1000 g: 3.04.0 Additional Considerations Only iron-fortified formulas should be used in formula-fed preterm infants. A formula containing 12 mg/L of iron may be used to meet the iron requirements of infants with birth weight R 1000 g. Additional oral iron supplementation is necessary for formula-fed infants with birth weight < 1000 g. A formula containing 1013 mg/L of iron is required to meet the total iron requirement without supplementation. Delay oral iron supplementation until erythrocyte transfusions have ceased.

Committee on Nutrition of the Preterm Infant, European Society of Pediatric Gastroenterology and Nutrition

Infants on human milk: 2.02.5 (maximum, 15 mg/d) Infants on formula milk: 2.02.5 (maximum, 15 mg/d) from all sources

No later than 8 weeks

1215 months

References: Intensive care of the fetus and neonate By Alan R. Spitzer, 2nd Edition, 2005. Robertons Textbook of Neonatology By Janet M. Rennie, 4th Edition, 2005. Manual of neonatal care By John P. Cloherty et al, 5th Edition, 2004. Joan R. Smith et al, early enteral feeding for VLBW infant: the development and impact of a research-based guideline. Neonatal Network, 24: 4, Jul-Aug 2005. 5. Feeding Guideline of Mont Sinai Hospital, Canada, Toronto. 6. Feeding Guideline of Sunnybrook & Womens College Health Sciences Center, Canada, Toronto. 7. Feeding Guideline for Hospital of sick children (Sickkids), Canada, Toronto. 8. Feeding Guideline of Foothills Hospital, Canada, Calgary. 9. Becker GE et al. Methods of milk expression for lactating women. Cochrane Database Syst Rev. 2008 Oct 8 ;( 4):CD006170. m 10. M C Backstr et al, Randomized controlled trial of vitamin D supplementation on bone density and biochemical indices in preterm infants. Arch Dis Child Fetal Neonatal Ed 1999; 80:F161-F166 (May) 11. Fuller NJ et al, Plasma folate levels in preterm infants, with and without a 1 mg daily folate supplement. Eur J Pediatr. 1992 Jan; 151(1): 48-50. 12. Ek J et al, Plasma and red cell folate values and folate requirements in formula-fed premature infants, Eur J Pediatr. 1984 Jun; 142(2): 78-82. 13. Strelling MK, Diagnosis and management of folate deficiency in low birth weight infants, Arch Dis Child. 1979 Apr; 54(4): 271-7. 14. Worthington-White DA et al. Premature infants require additional folate and vitamin B-12 to reduce the severity of the anemia of prematurity. American Journal of Clinical Nutrition. 60(6): 930-5, 1994 Dec. 15. Rao R, Georgieff MK. Iron therapy for preterm infants. Clin Perinatol. 2009 Mar; 36(1):27-42. 16. Sankar MJ, Saxena R, Mani K, Agarwal R, Deorari AK, Paul VK. Early iron supplementation in very low birth weight infants--a randomized controlled trial. Acta Paediatr. 2009 Jun; 98(6):953-8.An integrated review of the literature on demand feedings for preterm infants. Adv Neonatal Care. 2004 Aug;4(4):216-25. Review. 1. 2. 3. 4.

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17. Tosh K, McGuire W. Ad libitum or demand/semi-demand feeding versus scheduled interval feeding for preterm infants. Cochrane Database Syst Rev. 2006 Jul 19; 3:CD005255. 18. McCain GC. An evidence-based guideline for introducing oral feeding to healthy preterm infants. Neonatal Netw. 2003 Sep-Oct; 22(5):45-50. 19. Nye C. Transitioning premature infants from gavage to breast. Neonatal Netw. 2008 Jan-Feb; 27(1):7-13. Review.

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: PURPOSE: NCG - 09 PARENTERAL NUTRITION TO ENSURE THAT ALL NEONATES RECEIVE OPTIMAL NUTRITION & ADEQUATE GROWTH & DEVELOPMENT

APPLICABLE BY: All NICU Health Care Providers DATE: 1st UPDATE REFERENCE: October 2005 September 2009 See page 4

GUIDELINES: Hard or soft standardized TPN protocols are found to be better than individualized prescription. Also photo-protection of TPN and lipid sounds prudent. When to start? 1500 grams: start TPN as early as possible and Lipid within first 24 hours. 1501-2000 grams: If infant will be NPO 3 days, start TPN & lipid @1- 2 days. 2000 grams: If infant will be NPO 5 days, start TPN & lipid @ 2 -3days.

Carbohydrate Source: Dextrose 3.4kcal/g Carbohydrate Initial dose Average daily increase Usual maximum dose mg/kg/min 4-8 As tolerated * 10-14

*(Reflomat 8 mmol/dl, No glucosuria, No lactic acidosis and No CO2 retention). Normal reflomat: 2.6-8 mmol/dl.
NCG-09 Parenteral Nutrition 1/5

Protein- Amino Acids : Source: Different brand names (e.g.: Vaminolact). Protein Initial dose * Average daily increase Usual maximum dose ** 1.5-2.5 0.5-1.0 3 g/kg/d

4kcal/g

*To ensure that amino acids are being used for anabolism and not as energy source, emphasis should be on starting energy at > 30 kcal/kg/d and increasing as per tolerance. ** (In 1500 gram baby you can give up to 3.5 g/kg/d parentally but DO NOT exceed 3.8 g/kg/d of protein from both parental and enteral sources) Lipid : Source: Intralipid 20%. 2 kcal/ml Birth Weight Lipid Initial dose Average daily increase *Usual maximum dose < 1000 g 0.5-1.0 0.5 3 > 1000 g 1.0 0.5-1.0 3

* If baby has poor growth: The dose can be increased up to 3.5 g/k/d if tolerated and if energy intake does not exceed 120 kacl/kg/d. (Robertons) (Keep Triglyceride 2.5 mmol/dl)

Calcium and phosphorus : Parenteral requirements: mmol/kg/d (mg/kg/d)


Calcium Phosphorous Keep Ca/Po ratio 1.3-1.7 1.5-2.25 (40-90) 1.5-2.25 (40-70)

Trace element
In parenteral nutrition, the trace elements that are incorporated into the solution include zinc, copper, manganese, chromium, and selenium. The two that are contraindicated in cholestasis are copper and manganese. Copper and manganese are excreted primarily via the biliary tract; so the intake of these two elements should be discontinued if the infant develops cholestatic liver disease in our hospital the trace elements solution had no selenium.
Suggested Doses

Zinc:
Copper: Selenium: Iodine: Manganese:

150-400 mcg/kg/day.
16-20 mcg/kg/day 1.2-3 mcg/kg/day 1 mcg/kg/day 10-20 mcg/kg/day

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For premature baby ADD selenium For cholestasis: Do not add Copper and manganese For renal failure: Do not add chromium and selenium For Baby who need brain MRI Do not add manganese

Heparin dose for central linens is 0.5 unit/kg/hours and use the following formula to speed your calculation: Heparin (unit/ml) = Weight (kg)* 0.5 / Rate of TPN
Weaning and Discontinuation of Parenteral Nutrition (PN) A proper balance of macronutrient should be maintained when moving from Parenteral to enteral nutrition. Infant should be weaned from both the TPN and Lipid simultaneously as enteral feeds increase. Once enteral feeds are being tolerated well and provide 120 cc/kg/d of TFI, PN can be discontinued and replaced by IV Dextrose Suggested Weaning Prescription TPN solution is going to be weaned by rate rather than concentration. When TPN is at maximum concentration and feeding is advancing the physician will write same TPN and might change lipid dose in the TPN sheet, and the physician should write in order sheet (wean TPN to balance or make-up TFI with TPN) .The nurses should run the rate of Lipid as ordered in TPN sheet and the nurse should run TPN at a rate to make up the TFI regardless what is the rate of TPN in TPN sheet. Suggested Monitoring schedule for PN Variables to be monitored Suggested frequency per WEEK

Initial Period * Later Period ** Weight 7 7 HC 1 1 Electrolyte 2-4 1 Creatinine 1-2 1 Glucose 1-2/ day 2 Triglyceride 2-4 1 Calcium/ phosphorus 2-4 1 Magnesium With electrolyte With electrolyte LFT As needed 1 Acid base status 2 1 Urine Glucose Twice per day Once per day **Initial period: refers to the time during metabolic instability and/or before full intake is achieved. **Later Period: Refers to the time when the infant is metabolically stable.

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References: Intensive care of the fetus and neonate By Alan R. Spitzer, 2nd Edition, 2005. Robertons Textbook of Neonatology By Janet M. Rennie, 4th Edition, 2005. Manual of neonatal care By John P. Cloherty et al, 5th Edition, 2004. Feeding Guideline of Mont Sinai Hospital, Canada, Toronto. Feeding Guideline of Sunnybrook & Womens College Health Sciences Center, Canada, Toronto. 6. Feeding Guideline for Hospital of sick children (Sickkids), Canada, Toronto. 7. Feeding Guideline of Foothills Hospital, Canada, Calgary. 8. Peter J. Aggett ,Trace elements of micropremie, Clin Perinatol. 2000 Mar;27(1):11929, vi 9. Shah PS et al. A randomized, controlled trial of heparin versus placebo infusion to prolong the usability of peripherally placed percutaneous central venous catheters (PCVCs) in neonates: the HIP (Heparin Infusion for PCVC) study. Pediatrics.2007 Jan;119(1):e284-91. 10. Skouroliakou M et al. Computer assisted total parenteral nutrition for pre-term and sick term neonates. Pharm World Sci. 2005 Aug; 27(4):305-10. 11. Skouroliakou M et al. Comparison of two types of TPN prescription methods in preterm neonates. Pharm World Sci.2009 Apr; 31(2):202-8. Epub 2009 Jan 24. 12. Parish A, Bhatia J. Early aggressive nutrition for the premature infant. Neonatology. 2008; 94(3):211-4. Epub 2008 Oct 2. 13. Bassiouny MR et al. A randomized controlled trial on parenteral nutrition, oxidative stress, and chronic lung diseases in preterm infants. J Pediatr Gastroenterol Nutr. 2009 Mar; 48(3):363-9. 14. Khashu M et al. Photoprotection of parenteral nutrition enhances advancement of minimal enteral nutrition in preterm infants. Semin Perinatol. 2006 Jun; 30(3):139-45. 15. Khashu M. Impact of shielding parenteral nutrition from light on routine monitoring of blood glucose and triglyceride levels in preterm neonates. Arch Dis Child Fetal Neonatal Ed.2009 Mar;94(2):F111-5. Epub 2008 Jul 23. 1. 2. 3. 4. 5.

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: PURPOSE: NCG - 10 NON-OLIGURIC HYPERKALEMIA Standardized management of Hyperkalemia

APPLICABLE BY: NICU health care provider DATE: 1st UPDATE REFERENCE: December 2005 September 2009 see page 4

Introduction
Hyperkalemia (plasma potassium level > 6.5 mmol/L is observed in 30 to 60% of extremely low birthweight (ELBW) infants early in life. It had been demonstrated that serum K+ values > 7 mmol/l were associated with electrocardiographic disturbances (prolonged atrioventricular and ventricular conduction), whereas serum K+ values less than 7 mmol/l were associated with normal conduction. Cardiac arrhythmias have been reported to occur in up to 60% of hyperkalemia premature Infants. These data indicate that non-oliguric hyperkalemia of the premature infant is an emergency situation requiring prompt therapy, as soon as serum K+ reaches 7 mmol/L.

Prevention
1. All parenteral fluids for VLBW infant should be K+ free fluids in the first 24 hrs of life. Afterward K+ can be added according to the baby general condition and serum K+ level. 2. It is suggested that nonoliguric hyperkalemia in ELBW infants may be attenuated by maintaining the iCa level within the normal range by prophylactic Ca administration early in life. Therefore keep iCa in normal range (1-1.4 mmol/L) by starting daily Ca maintenance requirement in the first hours of life.

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Therapeutic regimens
Therapy should be initiated if a serum K+ 7 mmol/L is confirmed in a free flow non-hemolysed arterial or venous blood sample. The other indication for starting active treatment is Electrocardiographic (except peaked T wave) changes in the presence of a serum K+ below 7 mmol/l. First step is to make sure no K+ is running in any IVF Second is to assess the homodynamic stability of the patient and ECG lead 2 strip monitoring.

A. Counteracting arrythmogenicity of hyperkalemia


The electrocardiographic pattern of hyperkalemia may be amplified by a decreased plasma calcium concentration, and calcium ions can counteract the effects of hyperkalemia at the myocardial level and pH.

Ca gluconate
In the absence of hypocalcemia, it remains unclear whether Ca gluconate should be administered to ELBW infants with hyperkalemia whenever hyperkalemia is recognized or only when electrocardiographic abnormalities occur.

Indication
1. Serum k+ level 8 mmol/L. 2. ECG abnormality apart from peaked T wave. 3. Hypocalcemia (iCa < 0.9 mmol/L).

Dose 10% Ca gluconate: 0.5-1 ml /kg IV over 10 min. Ca chloride: 0.2 ml / kg IV over 10 min. Caution: Stop Ca infusion if the heart rate drops by 20 beat/min. B. Redistribution of potassium
THE MAIN LINE OF TREATMENT WILL BE EITHER SALBUTAMOL IV INFUSION OR INSULIN / GLUCOSE OR BOTH TOGETHER IN RESISTANT SEVER CASES. CHOOSING EITHER WAY IS LEFT FOR PHYSICIAN DISCRETION.

1. Insulin plus glucose


Doses: (0.2) 0.040.5 U /kg /hr plus (0.5) 0.21.7 g of glucose/kg /hr (In addition to the running glucose maintenance)

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Adjust dose according to serum K+ and blood glucose levels.

Practical point: Use two separate solutions as follows:


1. D50%W to give glucose dose 0.5 g /kg /hr = 1 ml/kg/hr 2. 25 units of Regular insulin in 25 ml of D5%W to give the required Insulin Dose : 0.2U /kg /hr

Monitoring:
Serum K+ level and glucose every 1-4 hrs Start tapering the infusion (Insulin + glucose) if serum K+ 5mmol/L and monitor serum k+ level closely. Prolonged and gradual tapering is recommended to prevent rebound hypoglycemia.

2. Salbutamol Inhaler/ Nebulizer:


HENCE THE AVAILABILITY OF SALBUTAMOL NEBULIZATION / INHALER IN OUR NICU, YOU MIGHT USE THIS ROUTE UNTIL YOU GET THE PRIMARY LINE OF THE TREATMENT FROM THE PHARMACY.

Dose: 150 mcg (0.03 ml) / kg, Maximum 400 mcg / dose, as Neb/ MDI every 2 hrs.
IN CASE OF NEBULIZATION: ADD THE SALBUTAMOL TO 1-2 ML OF NORMAL SALINE.

3. Salbutamol IV continuous Infusion:


Dose: 0.2-0.4 microgram /kg/min

Monitoring: Serum K+ levels every 1-4 hrs. Discontinue treatment if serum K+ 5 mmol/L and monitor serum k+ levels closely. Caution: Do not start salbutamol if heart rate > 180 beat per min.
ONE OF RECOGNIZED SIDE EFFECT OF SALBUTAMOL THERAPY IS A (PARADOXICAL EFFECT) SLIGHT INCREMENT IN SERUM K+ LEVEL IN THE FIRST 3-10 MINUTES AFTER STARTING THE TREATMENT.

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4. Sodium bicarbonate
In neonates, there are no studies on the effects of sodium bicarbonate therapy on non-oliguric hyperkalemia. In view of the potential risks of intravenous sodium bicarbonate administration to cause intracranial hemorrhage in premature infants, and other negative effects (e.g. decreases in intracellular pH and myocardial performance), sodium bicarbonate should not be given to infants without profound metabolic acidosis.

C. Measures to remove potassium from the body


1. Diuretics:
There are no studies on the effects of diuretics on non-oliguric hyperkalemia of the premature infant.

2. Ion exchange resins:


Exchange resins are ineffective and dangerous in the treatment of non-oliguric hyperkalemia of the premature infant.

3. Exchange transfusion:
Hyperkalemia in premature infants has been treated successfully by exchange transfusion (volumes of 100200 ml/kg). However this invasive therapy was used as a last resort after failure of treatment of hyperkalemia.

4. Peritoneal dialysis:
Requires some time for preparation, and the resultant delay may be problematic.

References:

1. Mildenberger E, Versmold HT. Pathogenesis and therapy of non-oliguric hyperkalaemia of the premature infant. Eur J Pediatr. 2002 Aug; 161(8):415-22. Epub 2002 Jul 3. Review. 2. Singh BS, et al. Efficacy of albuterol inhalation in treatment of hyperkalemia in premature neonates. J Pediatr. 2002 Jul; 141(1):16-20. 3. Helfrich E, et al. Salbutamol for hyperkalaemia in children. Acta Paediatr. 2001 Nov; 90(11): 1213-6. Review 4. Iijima S, Prophylactic calcium administration for hyperkalemia in extremely low birthweight infants. Am J Perinatol. 2005 May; 22(4):211-6. 5. Grammatikopoulos T, et al. Benefits and risks of calcium resonium therapy in hyperkalaemic preterm infants. Acta Paediatr. 2003; 92(1):118-20 6. The HSC Handbook of Pediatrics (Toronto, Canada), 10th Edition, 2003. 7. The Harriet Lane Handbook, 17th Edition, 2005. 8. Vemgal P, Ohlsson A. Interventions for non-oliguric hyperkalaemia in preterm neonates. Cochrane Database Syst Rev. 2007 Jan 24; (1):CD005257.

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: PURPOSE: NCG - 11 PAIN MANAGEMENT IN NEONATE TO PROVIDE COMFORT DURING PAINFUL PROCEDURES

APPLICABLE BY: All NICU Health Care Providers DATE: 1st UPDATE REFERENCE: October 2006 October 2009 See pages 10-11

1. Introduction:
It is unethical to cause pain needlessly to a neonate. Moreover, prolonged pain can have detrimental effects on the neonate, especially in preterm. In particular pain and stress have been associated with an increased incidence of intraventricaular hemorrhage (IVH) and/or periventricular leukomalcia (PVL). Increased exposure to repetitive pain in the neonate period may result in abnormal behavior response to pain later in childhood. Practical guidelines for pain management can be based on two goals: to minimize the intensity, duration and physiological cost of the pain experience and to maximize the baby's ability to cope with and recover from the painful experience. Health care providers are ultimately aimed at securing the baby's well being, which includes the alleviation of pain. They can create a pleasant, safe and painless environment and thus promote the baby's growth and development, as well as helping the baby to cope with illness and pain.

2. Guidelines
2.1. The Environment of Pain Management To make informed decisions in pain management, nurses need to have adequate skills to identify pain and proper tools to assess pain neonates. Tested pain instruments are necessary for systematic pain assessment and nurses should be involved in their development. The Premature Infant Pain Profile (PIPP) and the Neonatal Infant Pain Scale (NIPS) are perhaps the best-known tools. The PIPP is the pain tool assessment that is going to be used in our unit (see appendix 1). Nurses with inadequate knowledge about pain may fail in the task of pain assessment and therefore underestimate the pain experienced by the patient. 2.2. The Baby's Preparation for a Procedure Before a procedure or examination, the baby should be prepared for it. It is important to respect the dignity of a small premature baby, and the procedure should be started by letting the baby know what is going to happen next. A good way of doing this is by waking baby, by
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talking to him or her in a gentle voice, by stroking, caressing and touching the baby, who can then wake up feeling safe. The presence of parents and the primary nurse, and giving the baby a pacifier (dummy) before a nursing procedure or examination may help to create a sense of security. 2.3. Pain Alleviation During the Procedure Performing a procedure or examination in a technically and professionally competent manner can help reduce and shorten a baby's pain experience. Appropriate instruments like using an automated heel lancet instead of a manual lancet and efficient actions also help to perform the procedure less painfully. The presence of the parents and stroking, caressing and talking to the baby can also help the child to feel more secure in a painful situation. In addition, many simple non-pharmacological interventions like: Breast feeding or breast milk, non-nutritive sucking and a pacifier in the baby's mouth, application of massage, bundling, flexion, skin to skin, soothing vocalization, eye contact, etc. reduces pain reactions. After the procedure, the presence of the parents or primary nurse can help to restore the baby's sense of security. The baby should not be left alone with the pain. Babies that are neglected after heel lancing have been shown to be restless long after the procedure. It is important to document the pain alleviation methods used and their effectiveness each time a procedure or examination is performed. 2.4 Specific Painful Procedures NB: If it is not otherwise indicated, all the suggested steps should be applied together whenever possible and there is no contraindication. 2.4.1 Venipuncture - This is a preferred method for blood sampling as it is less painful and requires less re-sampling. Steps 2.1 through 2.3 Non-pharmacological interventions (like application of massage, bundling, non-nutritive sucking, flexion, skin to skin, soothing vocalization, eye contact, a pacifier in the baby's mouth, etc.) Oral Sucrose (see appendix 2) EMLA cream (see appendix 4)

2.4.2 Heel Lancing Steps 2.1 through 2.3 Non-pharmacological interventions Use Automated heel lancet like (BD QuikHeel lancet) when ever possible instead of a manual lancet Leg massage Oral Sucrose (appendix 2)

2.4.3 Peripheral Arterial & Venous Punctures/Insertion Steps 2.1 through 2.3
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Non-pharmacological interventions Oral Sucrose (appendix 2) EMLA cream (appendix 4)

2.4.4 PCVC Line Placement Non-pharmacological interventions Oral Sucrose (see appendix 2) OR IV Fentanyl or IV morphine in ventilated baby and IV accesses is available. (see appendix 3) EMLA cream ( appendix 4)

2.4.5 Intramuscular Injections Steps 2.1throug 2.3 Non-pharmacological Oral Sucrose ( see appendix 2)

2.4.6 Immunization Steps 2.1throug 2.3 Non-pharmacological interventions Oral Sucrose (appendix 2) Acetaminophen 30 minutes prior to immunization and q6h for 24 hours thereafter

2.4.7 Lumbar Puncture Non-pharmacological interventions Oral Sucrose (appendix 2) And / OR EMLA cream (appendix 4)

2.4.8 Ophthalmic Examination e.g. ROP screening Non-pharmacological interventions Oral Sucrose is still can be not routinely recommended for eye examination (see appendix 2)

2.4.9 Nasogastric or Orogastric Tube Insertion Non-pharmacological interventions Oral Sucrose (appendix 2)

2.4.10 Endotracheal suctioning Non-pharmacological

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2.4.11 Endotracheal intubation See appendix 5 2.4.12 Chest tube A. Insertion: Fentanyl in ventilated baby And/ OR Lidocaine Infiltration B. In Situ Fentanyl Or Morphine Infusion in ventilated baby OR Acetaminophen C. Removal Oral sucrose (appendix 2) Acetaminophen OR Small IV opioid In ventilated baby 2.4.13 Necrotizing enterocolitis (stage 2 or 3) Fentanyl 1-3 micrograms/kg SLOW IV push over two minutes followed by a continuous infusion at 2 micrograms/kg/hour for 24 hours. Thereafter adjust dose according to PIPP score. 2.3.14 Circumcision DPNB (Dorsal Penile Nerve Block) is the most effective methods and the preferred intervention Ring block OR Oral Sucrose (appendix 2) OR EMLA cream (appendix 4) Combined analgesia is advisable. Plus Steps 2.1 through 2.3 And Non- pharmacological interventions if it is feasible. Acetaminophen may be administered post circumcision as per physician's order 2.3.15 Post Operative Care According to the type of surgery, the homodynamic stability of the patient and PIPP Score, Acetaminophen and / Or infusion / boluses of opioid can be used.

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Appendix 1
Indicator

Premature Infant Pain Profile (PIPP) (For Term and Preterm Babies)
Points 0 1 32 - 35 + 6days quiet/awake eyes open no facial movements 5-14 BPM increase 2.5 - 4.9% decrease minimum (10-39% of time) minimum (10-39% of time) minimum (10-39% of time) 2 28 - 31+ 6days active/sleep eyes closed facial movements 15-24 BPM increase 5.0 - 7.4% decrease Moderate (40-69% of time) moderate (40-69% of time moderate (40-69% of time) 3 < 28 weeks quiet/sleep eyes closed no facial movements 25 BPM increase 7.5% decrease maximum (70% of time) maximum ( 70% of time maximum (70% of time) 36 weeks active/awake eyes open facial movements 0-4 BPM increase 0 to 2.4% decrease none ( 9% of time)

Gestational age ( weeks) Behavioral state

Heart rate Max. Oxygen saturation Min. Brow bulge

Eye squeeze

none ( 9% of time)

Nasolabial furrow

none ( 9% of time)

Total Score

Scoring Method for the PIPP 1. Familiarize yourself with each indicator and how it is to be scored by looking at the measure. 2. Score gestational age (from the chart) before you begin. 3. Score behavioral state by observing the infant for 15 s immediately before the event. 4. Record baseline heart rate and oxygen saturation. 5. Observe the infant for 30 s immediately following the event. You will have to look back and forth from the monitor to the baby's face. Score physiologic and facial action changes seen during that time and record immediately following the observation period. 6. Calculate the final score.

For all age groups:


Total scores of ( 6) generally indicated minimal or no pain Scores (>12) reflected moderate to severe pain. However, absolute scores must be interpreted with caution, as the pattern of how total scores change between pain and non-pain situations is most important in interpreting the individual infant's pain response. Guidelines for Intervention
Pain score 6 minimal or no pain >12 moderate to severe pain Guidelines for Intervention Non Pharmacologic Non Pharmacologic Pharmacologic: -Narcotic intermittent bolus -Consider narcotic drip

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Appendix 2

Oral Sucrose

Mechanism of action: The effects of sucrose are thought to be mediated by both endogenous opioids and non-opioid systems like effects On-nutritive sucking. Concentration: Sucrose 24% Different concentrations had been used in the studies but 24% was used more often than the other concentrations in the studies. Preparation: SweetEase as a ready made single use container OR Prepared in the pharmacy as a multidose solution in a 90 ml bottle (45 cc Ora Sweet and 45 cc sterile water) that should be refrigerated in between use.

Dose: The effective doses had been used ranged from 0.05-to-2ml of 24% oral sucrose. Suggestive dose: Term: 0.5-1 ml per dose .Total of 4 ml per 24 hrs Preterm: 0.5 ml per dose .Total of 2 ml per 24 hrs

Administration: To be administered to the anterior surface of the tongue two minutes prior to procedure. Indications: Can be used Term and preterm babies 32 weeks gestation for the fooling painful procedures 1. Heel lancing 2. Venipuncture 3. Peripheral Arterial & Venous Punctures/Insertion. 4. PCVC Line Placement 5. Intramuscular injections 6. Immunization 7. Lumbar Puncture 8. Nasogastric or Orogastric Tube Insertion 9. Chest tube Removal 10. Circumcision Regarding ROP screening, Oral sucrose can not be routinely recommended in because the analgesic effect of sucrose is not well established for ROP screening. Contraindications: 1. Premature baby < 32 weeks ( possible poor neurodevelopment outcome) 2. Critically ill infants 3. NPO status 4. Patient at risk of NEC. 5. Pre-Op sedated patient 6. Hyperglycemia Side effects: 1. Has a relatively high osmolarity and may lead to diarrhea with frequent use 2. Theoretically might increase risk of NEC. 3. Hyperglycemia
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4. Transient desaturation and chocking

Appendix 3
Fentanyl should be used as a substitute of morphine for patients who are: 1. Hypotensive 2. On inotropes 3. Have NEC 4. Those in whom a 20 minute onset of action time is too long. Bolus dose of Fentanyl: 1-3 micrograms/kg SLOW IV push over two minutes just prior to the procedure. Continuos Fentanyl infusion (post bolus): Start with 2 micrograms/kg/hour for 24 hours. Thereafter adjust dose according PIPP score.

Appendix 4

EMLA Cream (Eutectic Mixture of Lidocaine and Prilocaine)

It is a topical anesthetic cream and made up of equal parts of Lidocaine and Prilocaine. Dose: 0.5- 1 gram (0.5-1 ml) applied under an occlusive dressing for 60 minutes before the procedure Indications: A. Strong indication Circumcision B. Moderate indication Lumbar puncture in 34 weeks neonates C. Weak indications: 1. Venipuncture in term neonates 2. Peripheral arterial line in term neonates 3. Percutaneous venous catheter (PVC) insertion in 26 weeks neonates Side effects: 1. Methahemoglobinemia: may occur 1.25-18 hrs after administration and the treatment are by 100% oxygen and/or Methylene blue as anti-dote is. 2. Transient skin reaction like pallor and redness

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Appendix 5

(Adopted from Women's college Hospital, Toronto, Canada)

PROTOCOL FOR USE OF PREMEDICATION FOR NEONATAL ENDOTRACHEAL INTUBATION INDICATION Premedication may be administered for elective or semi-elective endotracheal intubation at the discretion of the NICU team. It is not intended for use in urgent intubations. The use of premedication for endotracheal intubation in neonates has been shown to decrease the number of intubation attempts and the time to intubation. There is no evidence demonstrating a consistent effect of premedication on physiological markers of pain. The insertion of an IV for the sole purpose of administration of premedication for intubation is at the discretion of the NICU team. If the team elects to insert an intravenous line, it is recommended that no more than two attempts be allowed before abandoning this objective. CONTRAINDICATIONS Atropine and succinylcholine should NOT be used in patients with current or familial history of myopathy (e.g. Duchennes muscular dystrophy), skeletal muscle disease, dystrophic neuromuscular disease, or malignant hyperthermia. Atropine and succinylcholine should not be used in patients with current or recent history of arrhythmias, sustained tachycardia (> 200 beats per minute in the previous 24 hours), or hyperkalemia (K > 6.5 non-hemolyzed or 8.0 hemolyzed in the previous 24 hours). DOSAGE AND ADMINISTRATION If none of the above contraindications are applicable, the following drug and doses should be administered in chronological order as indicated below. If atropine and succinylcholine are contraindicated, Fentanyl may be administered alone. It is imperative that the timelines indicated be followed in order to allow the appropriate time for the drugs to act prior to the onset of the procedure. 1. Atropine 20 micrograms/kg IV push 10-15 minutes prior to intubation (no additional doses) 2. Fentanyl 3 micrograms/kg SLOW IV push over two minutes just prior to intubation (an additional dose of 2 micrograms/kg SLOW IV push over two minutes may be given two minutes after the initial dose of Fentanyl if the baby still appears unsettled) 3. Succinylcholine 2 mg/kg IV push immediately prior to intubation (dose may be repeated after 1-2 minutes to a maximum of 4 mg/kg) RATIONALE FOR SELECTION OF DRUGS Pain management Fentanyl has a rapid onset of action and a short duration of action (30 60 minutes). The onset of action of morphine is 20 minutes and the duration is 3-6 hours. In observation of clinical practice, most healthcare providers do not wait 20 minutes post administration of morphine to begin the intubation and therefore the potential benefits of analgesia for intubation are not present at the time the procedure is performed. The long half-life of the morphine is often associated with a need for increased ventilatory support for a prolonged period of time. Chest wall rigidity reported to be associated with fentanyl is linked to high serum levels as a result of a rapid IV push of a high dose. Doses of 3-5
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micrograms/kg/day (diluted in an appropriate volume to allow a SLOW IV push) and administered over 2-4 minutes have not been associated with chest wall rigidity. Muscle Relaxation Succinylcholine is a depolarizing neuromuscular blocker. It acts similarly to acetylcholine, producing depolarization at the neuromuscular junction resulting in periods of repetitive excitation (fasciculations) followed by block of neuromuscular transmission and flaccid skeletal muscle paralysis. Depolarization cannot be reversed. Nondepolarizing neuromuscular blocking agents competitively antagonize cholinergic receptors at the neuromuscular junction and as such are associated with fewer side effects than depolarizing agents. Agonists may be used to reverse the effects of non-depolarizing neuromuscular blocking agents. However, none of the safer non-depolarizing neuromuscular blocking agents with a rapid onset of action and a short duration of action have been studied in neonatal endotracheal intubation. Atropine has a rapid onset of action and is used to counteract the bradycardic effects of succinylcholine.

Important Considerations with the Use of Succinylcholine 1. Cardiac arrest Although the incidence of sudden hyperkalemic induced arrhythmias and subsequent cardiac arrest is extremely rare with succinylcholine, the onset is rapid (within minutes of drug administration), and the mortality rare is high (50-60%). Monitoring for arrhythmias post succinylcholine administration is imperative. Should they occur, immediately institute standard hyperkalemic management. Begin with sodium bicarbonate and calcium. Insulin and glucose therapy should also be started but they will take longer to affect potassium levels. Inotropic support may also be needed. If treatment is not instituted immediately, a rapid progression to ventricular fibrillation and asystole will likely occur. 2. Malignant Hyperthermia The incidence of malignant hyperthermia is extremely rare. Malignant hyperthermia is an autosomal dominantly inherited condition characterized by a sudden, rapid rise in body temperature and associated with signs of generalized hypermetabolism such as hyperkaelemia, tachycardia, tachypnea, cyanosis and often muscle rigidity. If suspected, treatment with dantrolene should be instituted immediately. Begin treatment with 1 mg/kg rapid IV push. The dose should be repeated to a maximum cumulative dose of 10 mg/kg IV (administered as a continuous infusion) until the symptoms subside. The effective dose to reverse the crisis is directly proportional to the time delay in initiating treatment, the dose of the drug and the individuals susceptibility to malignant hyperthermia.

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REFERENCES
1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. Oei J, Hari R, Butha T, Lui K. Facilitation of neonatal nasotracheal intubation with premedication: a randomized controlled trial. J Paediatr Child Health. 2002 Apr; 38(2): 146-150. Bhutada A, Sahni R, Rastogi S, Wung JT. Randomized controlled trial of thiopental for intubation in neonates. Arch Dis Child Fetal Neonatal Ed. 2000 Jan; 82(1): F34-37. Pokela ML, Koivisto M. Physiological changes, plasma beta-endorphin and cortisol responses to tracheal intubation in neonates. Acta Paediatr. 1994 Feb; 83: 151-156. Khammash HM, OBrien K, Dunn MS. Blunting of hypertensive response to endotracheal intubation in neonates by premedication. Paediatr Res 1993; 33(4): 218A. Guinsburg R, Kopleman BI, Anand KJS, et al. Physiological, hormonal, and behavioral responses to a single fentanyl dose in intubated and ventilated preterm neonates. J Pediatr 1998;132:954-9. Orsini AJ, Leef KH, Costarino A, et al. Routine use of fentanyl infusions for pain and stress reduction in infants with respiratory distress syndrome. J Pediatr 1996;129:140-5. Vaughn PR, Townsend SF, Thilo EH, et al. Comparison of continuous infusion of fentanyl to bolus dosing in neonates after surgery. J Pediatr Surgery 1996;31:1616-23. Jacqz-Aigrain E, Burtin P. Clinical pharmacokinetics of sedatives in neonates. Clin Pharmacokinetic 1996;31;423-43. Arnold JH, Truog RD. Sedation in neonatal and pediatric intensive care. J Intensive Care Med 1992;7:24460. Roth B, Schlunder C, Houben F, et al. Analgesia and sedation in neonatal intensive care using fentanyl by continuous infusion. Dev Pharmacol Ther 1991;17:121-7. Taketomo CK, Hodding JH, Kraus DM. Pediatric Dosage Handbook. 5th edition. 1998. Lexi-Comp Inc. Hudson, Ohio. Bevan DR. Succinylcholine. Can J Anaesth 1994;41:465-8. Morell RC, Berman JM, Royster RI, et al. Revised label regarding use of succinylcholine in children and adolescents: I Anesthesiology 1994;80:242. Badgwell JM, Hall SC, Lockhart C. Revised label regarding use of succinylcholine in children and adolescents: II. Anesthesiology 1994;80:243-5. Goudsouzian NG. Recent changes in the package insert for succinylcholine chloride: should this drug be contraindicated for routine use in children and adolescents? (Summary of the discussions of the anesthetic and life support advisory meeting of the food and drug administration, FDA Building, Rockville, MD, June 9, 1994). Anesth Analg 1995;80:207-8. Parker SF, Bailey A, Drake AF. Infant hyperkalemic arrest after succinylcholine. Anesth Analg 1995;80:206-7. Rosenberg H, Gronert GA. Intractable cardiac arrest in children given succinylcholine. Anesthesiology 1992;77:1054. Sullivan M, Thompson WK, Hill GD. Succinylcholine-induced cardiac arrest in children with undiagnosed myopathy. Can J Anaesth 1994;41:497-501. Shah V, Ohlsson A. Venepuncture versus heel lance for blood sampling in term neonates.Cochrane Database Syst Rev. 2004 Oct 18;(4). Leef KH. Related Articles, Evidence-based review of oral sucrose administration to decrease the pain response in newborn infants. Neonatal Netw. 2006 Jul-Aug;25(4):275-84. Boyle EM, et al.Sucrose and non-nutritive sucking for the relief of pain in screening for retinopathy of prematurity: a randomised controlled trial.Arch Dis Child Fetal Neonatal Ed. 2006 May;91(3):F166-8. Shah PS, Aliwalas LI, Shah V. Breastfeeding or breast milk for procedural pain in neonates.Cochrane Database Syst Rev. 2006 Jul 19;3. Thompson DG. Utilizing an oral sucrose solution to minimize neonatal pain. J Spec Pediatr Nurs. 2005 JanMar;10(1):3-10. Morash D, Fowler K.An evidence-based approach to changing practice: using sucrose for infant analgesia.J Pediatr Nurs. 2004 Oct;19(5):366-70. Stevens B, Yamada J, Ohlsson A.Sucrose for analgesia in newborn infants undergoing painful procedures.Cochrane Database Syst Rev. 2004;(3). Johnston CC, et al, Routine sucrose analgesia during the first week of life in neonates younger than 31 weeks' postconceptional age. Pediatrics. 2002 Sep;110(3):523-8. Stevens, et al. Volume 12(1), Premature Infant Pain Profile: Development and Initial Validation .March 1996, pp 13-22 Stevens B, et al. Premature Infant Pain Profile: development and initial validation.Clin J Pain. 1996 Mar;12(1):13-22. Ballantyne M, et al.Validation of the premature infant pain profile in the clinical setting.Clin J Pain. 1999 Dec;15(4):297-303. Taddio A.Pain management for neonatal circumcision. Paediatr Drugs. 2001;3(2):101-11.

16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30.

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31. Weise KL, Nahata MC. EMLA for painful procedures in infants. J Pediatr Health Care. 2005 JanFeb;19(1):42-7. 32. Boyle EM, Freer Y, Khan-Orakzai Z, Watkinson M, Wright E, Ainsworth JR, McIntosh N. Related Articles, Links 33. Sucrose and non-nutritive sucking for the relief of pain in screening for retinopathy of prematurity: a randomized controlled trial. Arch Dis Child Fetal Neonatal Ed. 2006 May;91(3):F166-8. 34. Elserafy FA et al. Oral sucrose and a pacifier for pain relief during simple procedures in

preterm infants: a randomized controlled trial. Ann Saudi Med. 2009 May-Jun;29(3):1848. 35. Lago P et al. Guidelines for procedural pain in the newborn. Acta Paediatr. 2009 Jun; 98(6):932-9.

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG - 12 ASSESSMENT AND MANAGEMENT OF ANTENATAL HYDRONEPHROSIS Diagnosis and management and follow-up of antenatally diagnosed hydronephrosis

PURPOSE:

APPLICABLE BY: All pediatricians DATE: REFERENCE: September 2007; Updated October 2009 see page 6

1. Introduction:
Antenatal hydronephrosis is the most common renal anomaly that is detected by antenatal ultrasound (0.65% of pregnancies)

2. Guidelines:

Prenatally:
Antenatal ultrasound should be done at 16-20 week of gestational age Repeat at 30 week of gestational age There are several criteria to stage fetal hydronephrosis depending on the anterior posterior diameter (APD) of the pelvis, one of them measures the APD after 20 weeks of age: 1. Grade 1: pelvic APD of 1cm and no caliectasis (Resolves in 50% of patients) 2. Grade 2: Pelvic APD of 1 to 1.5 cm and no caliectasis (Resolves in 36% of patients) 3. Grade 3: Pelvic APD is greater than 1.5 cm and slight caliectasis (Resolves in 16% of patients) 4. Grade 4: Pelvic APD is greater than 1.5 cm and moderate caliectasis (Resolves in 3% of patients) 5. Grade 5: Pelvic APD is greater than 1.5 cm and severe caliectasis and cortical atrophy (cortex < 2mm thick).

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Prenatal interventions:
Those fetus with severe bilateral hydronephrosis thought secondary to PVUJ, bladder aspiration can be taken for urinary protein and electrolytes (albumin, 2-microglobulin, sodium, chloride, and osmolarity), and used as marker to predict the presence of renal deterioration or injury secondary to obstructive uropathy. Prognostic features for good renal function include fetal urinary: Sodium level less than100 mMol/L, chloride level less than 90 mMol/L, and an osmolarity of less than 219mOsm/kg. Normal urinary markers in the male fetus with suspected PVUJ (progressive oligohydroaminous, severe bilateral hydroureteronephrosis, thickened bladder) may indicate that fetal intervention to bypass the obstructed urinary tract is appropriate.

Prognosis:
It has been showed that if the APD of the fetal renal pelvis measuring > 6mm at less than 20 wks, > 8mm at 20 to 30 wks, and > 10mm at greater than 30 wks was associated with persistent postnatal renal abnormality.

Postnatally:
Classification:
According to of the pelvis (APD) 1. Normal: 0 4 mm 2. Mild: 5 9 mm 3. Moderate: 10-14 mm 4. Severe: 15 mm and more

Postnatal ultrasound:
Should be done for all babies diagnosed to have hydronephrosis prenatally regardless of the degree (after 48 hours of age and within the first week).

MCUG:
Should be done for all babies diagnosed to have hydronephrosis postnatally regardless of the degree . Timing: If the postnatal hydronephrosis is mild to moderate and the parents are compliant: Arrange for MCUG at the age of 4 weeks If postnatal hydronephrosis is severe or if there are signs of infravecicular obstruction (posterior urethral valve obstruction), or solitary kidney, or unilateral cystic kidney, or noncomplaint parents: Do MCUG before discharging the baby.

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MAG3:
Should be done for all babies diagnosed to have moderate to severe hydronephrosis Postnatally, after excluding VUR by MCUG Timing: after the result of MCUG If there is VUR do DMSA

Prophylactic antibiotics:
1. If the postnatal ultrasound is normal: no need for antibiotic & repeat US after 34 months. 2. If there is VUR: give until resolved 3. If MCUG normal and MAG3 showed no obstruction: Based on postnatal ultrasound A. Mild: give for 6 months and repeat US, if unchanged discontinue and repeat US in one year B. Moderate and severe: give until one year or reduction in diameter to < 9 mm 4. In severe obstructive hydronephrosis

Antibiotics that can be used:


You might chose on of the following drugs and if at all possible to be given at bedtime. Amoxicilline: 10 15 mg \ kg once daily (preferable) Cephalexin: 2 3 mg \kg once daily Nitrofurantoin:1 -2 mg \ kg once daily after the first month Trimethoprim: 2 mg \ kg once daily after the second month (if there is no G6PD deficiency)

Follow up:
1. Postnatal ultrasound normal: no need for MCUG. Repease US after 3-4 months If the US at the age of 4 months is still normal: no need for farther follow up. If there is any pelvic dilatation even if mild, start antibiotics and do MCUG 2. Postnatal ultrasound (mild hydronephrosis, or calceal dilatation) and MCUG normal (at the age of 4 weeks): Repeat US at 6 months of age: If unchanged discontinue and repeat US in one year If hydronephrosis progresses do MAG3 3. Postnatal ultrasound (moderate to severe hydronephrosis) with no VUR: A. If MAG3 showed non-obstructive (the time required for clearance of 50% of the accumulated radionuclide (t) is less than 10 minutes), do US at 6 months of age and one year, carefully measuring the pelvis and calyxes and the renal parenchyma. Continue the antibiotics until 1 year OR reduction of dilatation <9mm. B. If MAG3 is uncertain (t between 10 and 20 minutes), repeat US after 3 months

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C. If MAG3 showed obstructive (t more than 20 minutes) pattern refer to urologist or if not possible repeat US after 4 to 6 weeks D. If any situation of B and C and the differential kidney function decreases, especially more than 10%( 40% or less) and the pelvic diameter or calyx in the ultrasound increase, surgery must be considered (pyeloplasty) Note: all patient coming for MCUG, MAG3, DTPA, or DMSA should be admitted as a day case in pediatric department

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Prenatally diagnosed hydronephrosis

Renal US after 48 hr

if normal

if there is hydronephrosis

Repeat US at the age of 3 4 months (if repeated US IS normal, no need for further follow-up) Do MCUG: for timing see note below If there is Moderate to severe hydronephrosis Without VUR Mild hydronephrosis and MCUG normal at age of 4 weeks
REPEAT US AT 6 MONTHS: IF HYDRONEPHROSIS PROGRESSES DO MAG3 IF UNCHANGED DISCONTINUE

Do MAG3

if MAG3 showed non obstructive : US at 6 months and 1year In Moderate-Severe hydronephrosis: Continue antibiotics until 1 year OR Reduction of dilatation <9mm

if MAG3 is uncertain : Antibiotics Repeat US after 3 months

if MAG3 showed obstructive pattern: Antibiotics In severe obstructive hydronephrosis Refer to urologist and if not possible Repeat US after 4 to 6 weeks

Note: If postnatal hydronephrosis is severe or if there are signs of infravecicular obstruction (posterior urethral valve obstruction), or solitary kidney, or unilateral cystic kidney or non-compliant parents: Do MSUG before

discharging the baby otherwise arrange it at the age of 4 weeks.


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References:
1. SickKids hospital \ antenatal hydronephrosis-postnatal management\ \nephrologic clinic June 2004. 2. Cuban medical literature\prenatal hydronephrosis: a proposal for postnatal study& follow-up\ Medicc Review. http://www.medicc.org/publications/medicc_review/0605/cuban-medicalliterature.html 3. Aksu N, et al: postnatal management of infants with antenatally detected hydronephrosis. Pediatr Nephrol. 2005 Sep; 20(9): 1253-9. Epub 2005 Jul 16. 4. Woodward M, Frank: postnatal management of antenatal hydronephrosis. Postnatal management of antenatal hydronephrosis. BJU Int. 2002 Jan; 89(2):149-56. Review. 5. Lee RS, et al: antenatal hydronephrosis as a predictor of postnatal outcome: A Metaanalysis. Pediatrics. 2006 Aug; 118(2): 586-93. 6. Assessment and Management of Fetal Hydronephrosis Kennedy Neoreviews.2002; 3: 214-219 7. De Bruyn R, Gordon I : postnatal investigation of fetal renal disease. Postnatal investigation of fetal renal disease. Prenat Diagn. 2001 Nov;21(11):984-91. Review. 8. Roth JA, Diamond DA: Prenatal hydronephrosis. Curr Opin Pediatr. 2001 Apr;13(2):138-41. Review. 9. Shokeir AA, Nijman RJ: Antenatal hydronephrosis: changing concept in diagnosis and subsequent management BJU Int. 2000 May; 85(8): 987-94. Review. 10. Belarmino JM, Kogan BA: Management of neonatal hydronephrosis. Early Hum Dev. 2006 Jan; 82(1): 9-14. Epub 2006 Jan 19. Review. 11. Pates JA, Dashe JS : Prenatal diagnosis and management of hydronephrosis. Early Hum Dev. 2006 Jan;82(1):3-8. Epub 2005 Dec 27. Review. 12. Fefer S , Ellsworth P : Prenatal hydronephrosis. Pediatr Clin North Am.2006 Jun; 53(3): 429-47, vii.

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: PURPOSE: NCG -13 INTUBATION AND EXTUBATION FOR NEONATES 1- Organization of airway management procedures and minimizing the associated morbidity and mortality. 2- Minimizing extubation failure.

APPLICABLE BY: All Pediatricians DATE: REFERENCE: March 15, 2008, Revised September 2009 1- Neonatal Resuscitation Textbook, American Heart Association and American Academy of Pediatrics, 5th Edition, 2006. 2- Intensive Care of the Fetus & Neonate By Alan R. Spitzer, 2nd Edition, 2005. 3- Robertons Textbook of Neonatology, Janet M. Rennie, 4th Edition, 2005. 4- Assisted Ventilation of the Neonate, Jay P. Goldsmith & Edward H. Karotkin, 4th Edition, 2003. 5- Sinha SK, Donn SM. Weaning from assisted ventilation: art or science? Arch Dis Child Fetal Neonatal Ed. 2000 Jul; 83(1): F6470. 6- Departmental Clinical Guidelines, NCG -13, Sameer Al-Abdi, 2006.

I. INTRODUCTION:
The airway management and ventilation is a corner stone in the stabilization and resuscitation of newborns, which necessitates constant exposure, and active participation in the neonatal resuscitation. The Positive Pressure Ventilation (PPV) can be provided by different methods: 1. Bag and mask (Ambu-Bag). 2. Laryngeal Mask Airway (LMA). 3. Endotracheal Tube (ETT), which is the ultimate and the decisive airyway management. All what you should know about the above-mentioned methods as well as monitoring the effectiveness and successfulness of your intubation and ventilation are well covered in the NRP textbook (refer to the last edition).

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II. INTUBATION GUIDELINES:


A. The LMA (Size1) and Carbon dioxide detector are available and they should be checked in along with other resuscitation stuff. B. Carbon dioxide detector should be used to verify and monitor the correct ETT placement whenever you questions ETT position or the patient does not show improvement. C. All the clinical and ancillary signs of correct ETT position have some limitation, which might bestow fake assurance, therefore always deem changing the ETT and re-intubation if there is no clinical improvement after appropriate resuscitation and rolling out the other cofounders. D. Premeditation drugs: which are listed in Appendix 5 of the NCG-13 (Pain Management in Neonate) should only be given during regular working hours and at the discretion of the MRP outside this period. After intubation, X-ray should verify the ETT position prior to the commencement of sedation and/or muscle relaxation. E. Difficult Intubation: After two unsuccessful intubation attempts, help from another close at hand colleague(s) should be sought and the Assistant/Associate Consultant and MRP should be informed immediately. Until you get help, calm down and do one or both of the followings: 1. Efficient and appropriate (Ambu-Bag). 2. Laryngeal Mask Airway insertion (LMA).

III. EXTUBATION GUIDELINES:


A. The decision of extubation should be made well in advance. B. All extubations should be done before 12:00 Hours, however late extubation may be allowed only after discussed with MRP, on call team, nurses and respiratory therapist(s). C. Extubation from conventional mechanical ventilation (CMV) is reasonable if ALL the following criteria are achieved: 1. Low ventilatory settings: Fractional inspired oxygen of < 0.4 and Low rate ( 20 breaths/minute) and Low peak inspiratory pressure ( 15 cm H 2 O) 2. Acceptable blood gas: individualized according to the patients gestational age, postnatal age, and disease.

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3. Good respiratory effort. 4. Hemodynamic stability: acceptable blood pressure for age without inotrops. 5. Minimal or no sedation. 6. Chest x-ray (optional): to confirm resolution of pathology that indicated mechanical ventilation.

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG -14 NEONATAL SCREENING FOR GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD) DEFICIENCY 1- Early diagnosis of G-6-PD deficient babies. 2- Anticipating complications of G-6-PD deficiency to facilitate early intervention. 3- Providing the parents with appropriate instructions to avoid later presentation with acute hemolytic attacks.

PURPOSE:

APPLICABLE BY: All Pediatricians All Nurses in Labor and Delivery, Obstetrics (Ward 1), Neonatal Care Departments ER, Ward 4 and PICU DATE: Original Date 20 April 2008 Revised 24 June 2008; 03 August 2008, September 2009 See page 4

REFERENCE:

I. INTRODUCTION:
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency is the most common human enzyme defect, being present in more than 400 million people worldwide. It had been shown that G6PD deficiency occurs in all provinces of Saudi Arabia, though at a variable frequency. In the Eastern Province, both male (20.6-23.25%) and females (8.8-12.5%) have the highest frequency of G6PD deficiency. G6PD-Mediterranean which associated with severe clinical manifestations is the most frequently encountered deficient variant in all areas of Saudi Arabia.1,2,3. In 2007, the G6PD deficiency was diagnosed in 10% of neonates whom were admitted to the Neonatology Service at King Abdulaziz Hospital, Al-Ahsa. The incidence of neonatal hyperbilirubinemia has constantly been shown to be several folds greater in G6PD deficient populations than in the G6PD normal population. Hyperbilirubinemia occurs within hours postnatally in those neonates and bilirubin rises faster than in normal neonates.4,5 In 1999 a study from ALHASA demonstrated that 51% of the G6PD-deficient infants had serum bilirubin levels exceeding 340 mol/L and 16% needed exchange transfusion.3 A recent large Canadian population based study demonstrated that bilirubin level 325 mol/L associates with developmental delay, attention-deficit disorder, and autism.6 Moreover many of recently reported cases of

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kernicterus have been found to be G6PD deficient. Moreover, G6PD deficiency might be a risk factor for necrotizing enterocolitis (NEC).7 Neonatal screening for this condition is important and with necessary precaution, enzyme deficient infants are less likely to develop severe hemolysis and subsequent kernicterus. Therefore, World Health Organization recommends screening all newborns on cord blood sample in populations with a prevalence of G6PD deficiency of 3 to 5 % in males.8 Fluorescent spot test is the recommended for screening test for G6PD deficiency with, sensitivity of 98.2% and the specificity 99-100% in hemizygous male or homozygous female.9 However, this test is not that reliable in heterozygote females with sensitivity only of 32% and specificity of 99% so in such cases a quantitative test is recommended.10

II. GUIDELINES:
1. All babies born in King Abdulaziz Hospital, Al-Ahsa should have G6PD screening test done after birth from umbilical cord blood. 2. If the result of G6PD screening test is reported as DEFICIENT, the concerned nurse should do Total Serum Bilirubin (TSB) level as soon as possible with CBC, blood film and from the babys own blood this time. 3. The in-charge nurse will inform the pediatrician about the results of G6PD and TSB once both are available. 4. The pediatrician should inform the mother about the result as soon as possible, give her the appropriate instructions, the related pamphlet, should answer all the parents questions, and document that in the babys chart. 5. If the G6PD screening from cord blood was not sent, then the G6PD screen will be sent from newborns as clinically indicated. 6. If the result of G6PD screening test is reported as NORMAL, the pediatrician should inform the parents during regular postnatal round or at discharge, which is earlier, and document that in the babys chart. 7. Quantitative G6PD test is to be sent, should the normal result of G6PD screening test needs to be confirmed.

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Algorithm summarizing the guidelines


G6PD Screening Test From Cord Blood

NORMAL

DEFICIENT

The pediatrician should inform the parents during regular round or at discharge, which is earlier

The nurse will send TSB, CBC, Blood film

Send Quantitative G6PD test if the result of G6PD screening test needs to be confirmed.

The nurse should Inform the pediatrician about G6PD and TSB results

The pediatrician should inform the mother about the result as soon as possible with appropriate instructions

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REFERENCES:
1-Nasserullah Z. et al. Neonatal screening for sickle cell disease, glucose-6-phosphate dehydrogenase deficiency and a-thalassemia in Qatif and Al Hasa. Ann Saudi Med.1998 Jul-Aug; 18(4): 289-92. 2-A.K. Al-Ali, Common G6PD Variant from Saudi Population and its prevalence, Ann Saudi Med 1996; 16(6): 654-656. 3-Al-Omran A. et al. Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice in Al-Hofuf area. Ann Saudi Med. 1999 Mar-Apr; 19(2): 156-8. 4-1Kaplan M et al. Onset of jaundice in glucose-6-phosphate dehydrogenase-deficient neonates. Pediatrics 2001Oct;108(4):9565-Murki S, Dutta S. Onset of bilirubin rise in G6PD deficient neonates prenatal or postnatal.Indian Pediatr.2005Oct;42(10):1053. 6-Jangaard KA et al. Outcomes in a population of healthy term and near-term infants with serum bilirubin levels of >or=325 micromol/L (>or=19 mg/dL) who were born in Nova Scotia, Canada, between 1994 and 2000.Pediatrics.2008Jul;122(1):119-24. 7-Schutzman DL, Porat R. Glucose-6-phosphate dehydrogenase deficiency: another risk factor for necrotizing enterocolitis? J Pediatr.2007 Oct; 151(4): 435-7. 8-WHO WORKING GROUP. GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY. BULL WORLD HEALTH ORGAN 1989;67:601-11. 9-Meissner PE et al. Diagnosis of red cell G6PD deficiency in rural Burkina Faso: comparison of a rapid fluorescent enzyme test on filter paper with polymerase chain reaction based genotyping. Br J Haematol. 2005 Nov;131(3):395-9. 10-Ainoon O et al. Semiquantitative screening test for G6PD deficiency detects severe deficiency but misses a substantial proportion of partially-deficient females. Southeast Asian J Trop Med Public Health. 2003 Jun;34(2):405-14

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG-15 GUIDELINES FOR NEWBORNS WITH DISORDER OF SEX DEVELOPMENT DSD (AMBIGUOUS GENITALIA) Management of a baby born with ambiguous genitalia

PURPOSE:

APPLICABLE BY: DATE: REFERENCE:

All Pediatrician October 2009 See last page

A. INTRODUCTION
DSD is a very sensitive and complicated issue to handle, please inform the MRP on duty as early as possible. Newborns who present with DSD must be considered medical emergencies due to the life-threatening issues that present for some cases and the social implications in all cases. A work-up should be started immediately in the attempt to obtain a precise diagnosis as early as possible Professionals who have experience with DSDs (including neonatologist, who is usually the initial MRP, pediatric endocrinologists, pediatric surgeon/urologists, psychiatrists, psychologists, geneticists) should explain the clinical situation to parents of affected infants and when possible, of the underlying cause of DSD, it is important for these professionals to explain what is known about the long-term medical, surgical and psychosexual experience of others affected by DSDs, and to acknowledge when such information is unavailable; in addition, to the importance of detecting life-threatening conditions that may co-exist. Early diagnosis of the etiology underlying genital ambiguity can aid families and physicians in deciding upon an optimal sex of rearing for affected newborns.

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B. FACTORS TO BE CONSIDERED INCLUDE:


The degree of genital ambiguity at birth The ability to produce and respond to androgens Predictions of long-term sexual function and satisfaction with sex of rearing. Fertility potential.

In the absence of either testosterone secretion or 5-reductase, the fetal external genitalia continue to develop along female lines. Specifically, without DHT the labioscrotal and urethral folds form the labia majora and minora, respectively. The genital tubercle develops into a clitoris and the urogenital sinus gives rise to the urethral opening and anterior portion of the vagina. The posterior portion of the vagina develops from the Mllerian ducts in the absence of MIS. In 46XX DSD: female sex of rearing is preferred as most cases will develop the potential for fertility despite masculinization of the external genitalia. Additionally, much evidence indicates that development of female gender identity is typical in those cases. In 46XY DSD: the appearance of the genitalia at birth plays a stronger role in decisions regarding sex assignment. Female sex of rearing is preferred in complete DSDs, particularly CAIS or Swyer syndrome. Male sex of rearing is recommended for cases of congenital micropenis and 5-reductase deficiency. For clinical purposes, DSDs in newborns are classified according to karyotype: 1. DSD with a 46XY karyotype (formerly referred to as male pseudohermaphroditism, undervirilization of an XY male and undermasculinization of an XY male. 46XY DSDs can result from: (a) Gonadal dysgenesis (b) Testosterone biosynthetic defects (c) 5-reductase deficiency, or (d) Abnormal androgen receptor activity. 2. DSD with a 46XX karyotype (formerly referred to as female pseudohermaphroditism or masculinization of an XX female). 3. DSD with an unusual karyotype such as mosaicism (45XO/46XY or 46XX/46XY), transposition of genes (46XX, SRY+) or chromosome deletions.

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C. WORK-UP OF INFANTS BORN WITH AMBIGUOUS GENITALIA:


The etiology underlying genital ambiguity in newborns impacts several aspects of management including recommendations for sex of rearing. 1. Clinical Evaluation: A comprehensive assessment of an affected newborn is very important, as ambiguous genitalia can occur in conjunction with several other congenital malformations. It is necessary to look for signs of dehydration with vomiting and diarrhea, as these are symptoms of a salt-losing crisis. A careful examination of the external genitalia must also be performed. A genital exam must include a measure of stretched phallic length, evaluation of the quality of the corpora, and inspection of the labia, labio-scrotal folds or scrotum. The position of the urethral opening (and vaginal opening if applicable) should be documented, as well as the presence and location of palpable gonads.

2. Status of body hydration: U&Es and glucose levels should be monitored daily as cortisol deficiency can manifest as hypoglycemia in newborns affected by CAH. Body weight should also be monitored as excessive weight loss may indicate pathological dehydration.

3. Hormone studies: Gonadotropins (LH, FSH) Androgens and androgen precursors (17-hydroxypregnenolone, 17hydroxyprogesterone, androstenedione, testosterone, dihydrotestosterone) Adrenal steroids (cortisol, aldosterone, and their precursors) and Mllerian inhibiting substance (MIS).

The following schedule for hormone studies is suggested: Day 1: obtain a karyotype and SRY gene. In addition to U&Es, glucose, cortisol, Day 2 - 4: of life, measure plasma androstenedione, testosterone and dihydrotestosterone. These androgens must be measured from a single blood sample so that the ratios of androstenedione/testosterone and testosterone/DHT can be calculated. Also measure plasma 17hydroxyprogesterone.
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NCG-15-Newborn with Ambiguous Genitalia

Day 7 of life measure plasma MIS and obtain white cells for DNA studies such as androgen receptor gene mutations. And you may need to repeat androstenedione, testosterone, DHT, and 17-hydroxyprogesterone measures.

4. Imaging Studies: Ultra sound scan: can be helpful in identifying both the type and extent of internal sex organ development. (Kidney, ureters, and bladder) that occur in individuals affected by DSD. An MRI: may be better able to identify Mllerian structures (uterus, fallopian tubes, upper portion of the vagina) than a sonogram, and can also be useful for localizing the gonads. A genitogram is also needed for visualization of the urinary tract, and to determine its position in relation to the vagina or vagino-utricular pouch, when present. It is helpful to establish the presence and size of the vagina or utricular pouch. Information obtained from a genitogram is particularly useful for surgical construction of the genitalia when needed or elected.

5. Sex of Rearing: Whether or not the etiology of the genital ambiguity can be determined, a sex of rearing must be elected. Ideally the sex of rearing for newborns who present with ambiguous genitalia should be decided within the first 3-4 weeks of life. Sex assignment or reassignment later in childhood is difficult for both the child and the childs family. The final choice of sex assignment should be made by parents after receiving recommendations and education from the medical team The role of the team is to inform parents about the process of sex determination and differentiation, and the specific abnormality that affects their child. As knowledge concerning the long-term outcome of people affected by DSD is obtained, this too must be shared with parents.

The following recommendations for assigning a sex of newborns with ambiguous genitalia: 1. Sex Assignment for Newborns Affected by 46XX DSDs: Masculinization due to 21-hydroxylase deficiency does not impair the development of the ovaries or the Mllerian ducts. Fertility rates in women affected by 21-hydroxylase dehydroxylase deficiency is reduced compared to their unaffected siblings. For this reason, female sex of rearing is recommended for even the most severely masculinized 46XX newborns affected by 21-hydroxylase deficiency.

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2. Sex Assignment for Newborns Affected by 46XY DSDs: It is clear that the majority of genes on the X chromosome do not influence sex development and differentiation. Concerning the Y chromosome, only the SRY gene contributes to testicular formation. In fact, most of the genes required for normal sex development and differentiation are found on the autosomes. As such, a 46XY chromosomal complement does not necessarily dictate female sex of rearing in newborns affected by 46XY DSDs. Newborns presenting with a 46,XY chromosome complement and normalappearing, female external genitalia due to complete androgen insensitivity syndrome (CAIS), complete gonadal dysgenesis (Swyer syndrome) or other complete abnormality of testosterone biosynthesis function successfully when assigned a female sex of rearing. Female assignment in such cases is widely accepted by patients throughout their lives despite challenges that they experience regarding sexual dysfunction and infertility. A second category of 46XY DSD in which a particular sex of rearing is optimal is in cases of micropenis without hypospadias. While individuals with congenital micropenis can accept either a male or female sex of rearing, female assignment is complicated by feminizing surgery of the genitalia. In contrast, individuals reared male require no genital surgery and may attain an adult penile length within or close to the normal range following testosterone treatment. Thus, we recommend male sex of rearing for newborns with congenital micropenis. Another category of 46XY DSD in which male rearing is preferable is in newborns affected by 5-reductase-2 or 17-hydroxysteroid dehydrogenase deficiency. At puberty affected individuals secrete normal male levels of testosterone and thus masculinize. In both conditions gender role frequently changes toward male in patients reared female. Additionally, future fertility potential exists for newborns raised male. The most difficult 46XY DSD patients, in terms of recommending a sex of rearing, are those newborns with ambiguous external genitalia including a small phallus and perineo-scrotal hypospadias. In those cases genital surgeries are often required regardless of sex of rearing; therefore, consideration of factors beyond genital appearance must be evaluated when considering male or female rearing. Regarding satisfaction with sex of rearing, either male or female assignment is possible. In all cases it must be understood that DSDs result in congenital malformations of the sex organs and in many instances in malformations of other body systems as well, at this time, much can be done to
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NCG-15-Newborn with Ambiguous Genitalia

D. Abbreviations:
DSD: disorder of sex development DHT: dihydrotestosterone CAIS: complete androgen insensitivity syndrome MIS: Mllerian Inhibitory Substance DHT: Dihydrotestosterone

E. References:
1. Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on management of intersex disorders. Arch Dis Child. Apr 2006. 2. Joel Hutcheson, Howard M Snyder; Ambiguous Genitalia and Intersexuality. emedicine 2006. 3. Wilhelm D, Palmer S, Koopman P. Sex Determination and Gonadal Development in Mammals. Physiol Rev; 2007. 87:1-28 4. Rodolfo Rey,Nathalie Josso, endotext.org website, Chapter 7. Sexual Differentiation, April. 2007 5. Nelson 18th edition

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG-16 GUIDELINES FOR MANAGEMENT OF NEONATAL HYPOCALCEMIA To investigate and treat neonatal hypocalcemia

PURPOSE:

APPLICABLE BY: All pediatricians DATE: REFERENCE: October 2009 see last page

Background:
Neonatal hypocalcaemia can be asymptomatic discovered as incidental finding or present as, irritability, lethargy, dry skin, coarse hair, laryngeal/ bronchospasm, tetany, apnea, seizure or severe life threatening event. Hypocalcaemia is common in the first few days of life, particularly following birth asphyxia or RDS. Late onset neonatal hypocalcaemia occurs 4 10 days after birth may be secondary to vitamin D deficiency, Hypoparathyroidism, or hypomagnesaemia. It can be associated with seizures. Chronic hypocalcaemia is tolerated well than acute onset hypocalcaemia.

Definition:
Pre-Term infant: Term infant: < 1.75 mmol/L < 1.9 mmol/L

Initial investigations:
1. Calcium, ionized calcium, phosphate, alk phos, magnesium, albumin. Corrected calcium = Plasma Ca2 + 0.02 ([albumin] - 40) 2. U&Es, plasma creatinine 3. Serum vitamin D levels 4. Serum PTH 5. Maternal bone profile and vitamin D level

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Second line investigations if clinically indicated:


6. DNA analysis e.g., for Vitamin D resistance syndromes, DiGeorge syndrome 7. Chest X-ray to look for thymus. Skeletal survey 8. Echocardiogram in DiGeorge syndrome 9. Renal USS as baseline 10. PTH infusion test

Management:
Intravenous access: Ensure adequate intravenous access in a large vein. Intravenous calcium is very corrosive and can cause serious burns if it extravasates. Calcium Gluconate 10% (1 - 2 ml/kg = 0.225 0.45 mmol/kg = 9 18 mg/kg elemental calcium = 100 200 mg/kg of calcium gluconate) given over 10 minutes with ECG monitor, and repeated as necessary whilst symptoms persist. Intravenous maintenance dose of Calcium Gluconate 10% is 300 500 mg/kg over 24 hours adjusted to response, use oral route as soon as possible. Oral calcium maintenance therapy: 200 800mg/kg/day of calcium in 4 divided doses adjusted to response. To prevent rebound hypocalcaemia reduce the dose gradually over 3 days. Mild or asymptomatic hypocalcaemia: you could start treatment with oral calcium. 200 800mg/kg/day in 4 divided doses, orally; adjusted to response. If there is evidence of vitamin D deficiency, start treatment with oral vitamin D3 1000 3000 IU/day. Other wise give vitamin D3 daily requirement of 400 IU/day. DO NOT USE routinely1-alpha-hydroxyl-cholecalciferol (alpha-calcidol) until an appropriate definitive diagnosis has been made.

References:
1. Lorraine Fitzpatrick, Fredrick singer. Diagnosis and treatment of hypocalcaemia. Endotext.org chapter 7. 2002. 2. BNF 2008 3. Nelson textbook of Pediatrics 18th edition 2008 4. Micromedex Healthcare series

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Kingdom of Saudi Arabia National Guard Health Affairs King Abdulaziz Hospital Al Ahsa

PEDIATRIC DEPARTMENT CLINICAL GUIDELINES


NUMBER: TITLE: NCG-17 GUIDELINES FOR MANAGEMENT OF NEONATAL HYPERCALCEMIA To investigate and treat neonatal hypercalcemia

PURPOSE:

APPLICABLE BY: All pediatricians DATE: REFERENCE: October 2009 See the last page

Consider these causes of hypercalcaemia: 1. Primary hyperparathyroidism 2. Familiar hypocalciuric hypercalcaemia (familial benign hypercalcaemia) 3. Neonatal hypercalcaemia secondary to maternal hypocalcaemia/ vitamin D deficiency 4. Pseudohypoparathyroidism 5. Idiopathic infantile hypercalcemia 6. Williams syndrome 7. Osteogenisis imperfecta 8. Infantile hypophosphatesia and breast milk induced rickets of prematurity 9. Resolution phase of subcutaneous fat necrosis 10. Bartters syndrome 11. fractures Investigation: 1. Serum calcium, ionized calcium, phosphate, alk phos, magnesium, albumin 2. U&Es, plasma creatinine 3. Serum vitamin D level 4. Serum PTH 5. 24 hours collection for urinary calcium 6. Urine calcium: creatinine ratio 7. Renal ultrasound scans to exclude nephrocalcinosis
NCG-17-Neonatal Hypercalcemia Guidelines Page 1 of 3

8. Consider the following if clinically indicated: Skeletal survey, DNA analysis e.g. for elastin gene deletions in Williamss syndrome, tubular resorption of phosphate. PTH infusion Test: (measure urinary cAMP and phosphate) in Pseudohypoparathyroidism Type I there is inadequate rise in cAMP levels in and plasma after PTH stimulation and no rise in urinary phosphate. In Pseudohypoparathyroidism Type II the cAMP in urine elevated before and after stimulation and no rise in urinary phosphate (end-organ resistance) Management: In severe hypercalcaemia (plasma Calcium > 3.0mmol/l or symptomatic), the following manoeuvres can be used to reduce the serum calcium: 1. Hyperhydration: Usually intravenously. Normal saline at 3 litres/m2/day. Effect)

urine PTH

(Immediate

2. Forced diuresis: Usually using high doses of Furosemide (2mg/kg/dose) or equivalent loop diuretics. Diuretics should only be given when hyperhydration has been achieved as they may otherwise exacerbate volume depletion. (Effect: Immediate/24hrs). 3. Glucocorticoids: Prednisolone 2mg/kg/day. (Effect: 4-10 days). 4. Bisphosphonates: Pamidronate infusion (0.5mg/kg/dose, given intravenously over 4 hours in 50 ml of normal saline) one dose daily for 2-3 days (check Ca level before next dose is given) depending upon the response of serum calcium. (Effect: 24-72 hours). 5. Calcitonin: Given at a dose of 2-8 units/kg iv/sc/im at 6-12 hourly intervals depending upon the response. Be aware as it can cause anaphylactic reaction. (Immediate Effect) 6. Dialysis: In the rare event of hypercalcaemia in association with acute renal dialysis can be used. (Immediate Effect) failure,

References: 1. Ilene A Claudius, Oved F, Jon Nakamoto, Pisit P, hypercalcaemia. Pediatric Emedicine. Jul, 2008. 2. The Metabolic & Molecular Bases of Inherited Disease Charles R. Scriver et al, 7th ed. McGraw Hill Inc. pg.3103-3104. 3. Nelsons text book of Pediatrics 18th edition. 2008

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