HIPOGLIKEMIA PADA BAYI

(Pembahasan Kasus)

Dr. Aditiawati SpA(K)

UKK Endokrinologi Anak-IDAI
22 Januari 2021
Bayi, laki2, 11 hari : Hipoglikemia persisten

• Lahir aterm 38 minggu dari ibu G2P1A0 Critical sampling 7/8 8/8
di RS X
• BL 4165 gram, PB 52 cm, LK 36 cm
GDS (mg/dL) 80-100 29
• Lahir SC , BSC 1 kali, AS 9/10
Insulin <2 44,1
(uIU/mL)
• Faktor risiko ibu (-), riwayat DM pada ibu (-)
Kortisol 3,7-19,4 3,5 8,8
• Satu jam pasca lahir dilakukan pemeriksaan (ug/dL)
GDS 35 mg/dL >> diberikan SF ACTH 6,7
(pg/mL)
• Cek ulang 30 menit hasil low selanjutnya
diberikan infus Dx dengan GIR 10 > rawat
• Pemeriksaan fisis : dalam batas normal

• Selama perawatan di Unit Perinatologi RS X:

Demam (-) distres napas (-) muntah (-)

• Tidak ada riwayat serupa dalam keluarga

 BBL 4165 Gr SKRINING HIPOGLIKEMIA
1 BMK
Asimptomatik
PADA BBL
( History & Pem. Fisik)
1. Asidosis Perinatal
2. Hipotermia
1. Premature 3. Suspek/klinis sepsis
2. KMK/SGA
4. Cyanosis
3. BMK
4. Bayi dengan ibu DM- 5. Apnea
PE/Eclampsia - 6. Kesadaran turun
Hipertensi 7. Kejang
5. Bayi dengan midline 8. Hipotonia
defect-sindroma
9. Lethargi
6. Rx Keluarga
hipoglikemia 10.High pich cry
11.Bayi dgn polisitemia
De Leon, D.D, Thornton, P, Stanley, C.A, Sperling, M.A. 2021. Sperling Pediatric Endocrinology. Philadelphia: Elsevier
 GDS 35
2 mg/dL
HIPOGLIKEMIA

Time line 0-4 hours 4-24 hours 24-48 hours > 48 hours
AAP Asymptomatic screneed neonate the first-4 hours,
( American maintain blood glucose >40 mg/dL
Academic of If symptomatic-treat if blood glucose is< 40 mg/dL
pediatric) Asymptomatic screneed neonate the 4-24 hours,
maintain blood glucose >45 mg/dL

PES First 48 hours: A blood glucose > 60 mg/dL is

(Pediatric maintain blood glucose >50 mg/dL. recommended after 48 hours of
Endocrine Infants who are unable to maintain a blood glucose level> 50mg/dL in the first life. Infants at risk of persistent
Society) 48 hours of life may be at risk for disorder causing persistent hypoglycemia hypoglycemia syndrome à
challenge 0f 6-8 hours
maintenance blood glucose >70
Source : Recommendations from the Pediatric Endocrine Society for Evaluation mg/dL
and Management of Persistent Hypoglycemia in Neonates, Infants, and Children
, Paul S. Thornton, MB, BCh1, Charles A. Stanley, MD2, Diva D. De Leon ,ea, The
Journal of Pediatrics, Vol 167 No2, August 2015
 Tatalaksana SEGERA : mencegah komplikasi defek
neurologi

HIPOGLIKEMI à KEGAWATAN J Clin Res Pediatr ic Endocrinol

1. infus glukosa (GIR 6-8)mg/kg/m sd 2017;9 (Suppl 2):69-87
GIR 12 mg/kg/mt
2. injeksi glukagon 0,5-1 mg im/sc
3. frequent feeding

66 bayi hipoglikemi, umur 1-22

hari, 72% recurrent, USG 72,5%
Clin Med Insights Ped
normal, MRI Abnormal 55%,
2019:13
Conclusions:
The pattern of bilateral occipital
cortical injury is the most
common abnormality for neonatal
hypoglycemia.
 Infus Kontinyu Dextrose IV
GIR 6 mg/kg/menit

3 Pantau setiap jam sd euglikemia

Selanjutnay 4-6 jam HATI2
DENGAN
BOLUS
GD > 50 mg/dL GD < 50 mg/dL DEXTROSE

Stabil dlm 24 jam & 2 Naikkan GIR 2

pemeriksaan x GD > 50 mg/dL mg/kg/menit

Turunkan GIR 2 mg/kg/mnt tiap 6 Bila GIR > 12 mg/kg/menit

jam dan pantau GD tiap 6 jam

EVALUASI
Stop IV bila GIR sdh 2 mg/kg/mnt HIPOGLIKEMIA
dan bayi stabil PERSISTEN

Critical Sample :
AGD, keton, insulin,C-
Stop cek GD bila GD normal 2x ber
turut turut dan minum oral penuh peptida, kortisol, GH, laktat,
Asam lemak bebas
 GIR= GLUCOSE INFUSION RATE
• Konsentrasi glukosa neonatus berhub erat dengan kecepatan infus glukosa.
• GIR : istilah yg menunjukkan seberapa banyak glukosa dalam miligram (mg) per
kilogram berat badan neonatus per menitnya ( mg/kg/menit)
• Rate Normal : <8mg/kg/min
• Maximum konsentrasi infus glucose di perifer 10%
à bila konsentrasi >10% à Risiko “cutaneous and subcutaneous burns “bila terjadi
extravasation
à perlu akses Central
 4 RUJUK - KONSUL

EVALUASI
Bila GIR > 12 mg/kg/menit HIPOGLIKEMIA
Hipoglikemia ber ulang > 48 jam
PERSISTEN

HASIL KASUS INI :

Critical sampling di 7/8 8/8
Critical Sample :
RS X
AGD, keton, insulin,C-
GDS (mg/dL) 80-100 29 peptida, kortisol, GH, laktat,
Insulin <2 44,1 Asam lemak bebas
(uIU/mL)
Kortisol 3,7-19,4 3,5 8,8
(ug/dL)
Keton 0,1 < 0.6
mml/l Kelainan Hormonal ??
LAKTAT 0,7-2.5 1,2 Atau
PH 7.35-4.45
7.47 Kelainan metabolik (inborn error of
21-28 metabolism)??
HCO3 17,5
 EVALUASI

5 HIPOGLIKEMIA
PERSISTEN
 ETIOLOGI HIPOGLIKEMIA
NEONATUS-BAYI
TRANSIEN PERMANEN
Adaptasi yang lambat HIPERINSULINISME

KMK/SGA Sind Beckwith-Wiedermann PERINATAL STRESS

HIPERINSULINEMIA INDUCED
Sepsis, Asfiksia, MEN 1 KONGENITAL HYPERINSULINEMI
A
Hipotermia
Kebutuhan pasca natal Inborn error of metabolism
é TSH*
CORTISOL MONOGENIK (12 gen),
Bayi ibu DM GH ABCC8,KCNJ11,
GSD I,II,VI GLUD1,GCK,HADH1,UCP2,MCT1,
Infus Dextrose saat ibu PITUITARY HORMON DEFISIENSI HNF4A,HNF1A,HK1,PGM1,
intrapartum
PMM2
Toksemia Kelainan proses gluconeogenesis Sind. Beckwith Wiedemann
Sind Kabuki
Sind Turner
Poliseitemia Defek oksidasi asam lemak & ketogenesis

Tokolitik Galaktosemia, fruktosa intolerance

Genetic testing Result Summary
Genetic diagnostic of autosomal resessiv
congenital hyperinsulinism, ABCC8

Mutations in key genes which are involved in the regulation of insulin

secretion from pancreatic β-cells underlie the molecular basis of CHI.
Until recently mutations in only 12 different genes (ABCC8, KCNJ11,
GLUD1, GCK, HADH, SLC16A1, HNF4A, HNF1A, HK1, PGM1 and PMM2)
that lead to dysregulated secretion of insulin had been described

KATP channels are located in the β-cell membrane and transduce the
metabolic signals generated by glucose metabolism to regulate insulin
secretion . The KATP channel complex is composed of four outer,
sulphonlyurea receptor 1 (SUR1) subunits that are encoded by the ATP
Binding Cassette Subfamily C Member 8 (ABCC8) gene and the four
inner, pore-making, Kir6.2 channel proteins, encoded by the Potassium
Voltage-Gated Channel Subfamily J Member 11 (KCNJ11). Both these
genes are located on chrosome 11p15.1. The SUR1 component
regulates the activity of the Kir6.2 proteins and functions as the binding
site for the KATP channel opener (diazoxide) and sulphonylureas. The
inner Kir6.2 protein forms a pore allowing potassium influx across the
β-cell membrane. A change in the ratio of ATP to ADP causes closure of
the KATP channel and triggers depolarisation of the cell membrane,
activating the voltagegated calcium channels. This in turn causes insulin
release through exocytosis.
Mutations in the genes encoding K channel proteins
SUR1 and Kir6.2 ATP

ABCC8- KCNJ11
(2019)
 Tata
Tatalaksana Selanjutnya & jangka panjang

1. Diazoxide-oral *
2. Chlorthiazide-oral * DIFUS /
FOKAL
3. Nifedipine-oral * *Infus Kontinyu
4. Octreotide sc * Dextrose
5. Glucagon sc-im *Frequent feeding
6. Corticosteroid F DOPA-
PET/CT
7. Growth Hormon SCAN
8. Rapamycin
9. Octreotide LAR (deep sc Bila tak
responsif
surgery
 Diazoxide Nifedipin
Membuka Menghambat Ca-channels
Channels KATP & blocker dari membran β -sel
menghambat
sekresi insulin

+
Chlorothiazide,
prevensi
retensi cairan,
bersinergis dgn
Diazoxide pada
KATP channel Octriotide
dan Aktivasi SSTR2 &
menghambat menghambat
sekresi insulin mobilisasi Ca
dan
asetylcholin,
Glukagon :
menurunkan
menstimulasi
sintesis-sekresi
glycogenolisis &
insulin
gluconeogenesis
 Bagaimana follow up selanjutnya
6 ??
Minggu Minggu Minggu Minggu
I II III IV

BB = 4735 ONS 1 kcal/mL BB = 5460 Hipertriko BB = 5800

DPL normal
gram q3h gram sis gram
Na, K, Cl, Ca,
P, TSH Diazoxide- Muntah 2
Critical
Mg normal 1,934 sampling unresponsive Genetic
GDS
FT4 hyperinsulinis test
pH447,489
1,19 m
Keton 0,1 SGOT
Pemantauan klinis, balans
Laktat 3 26
diuresis, dan efek 1 Terapi lini 3
Amoniak SGPT MRI
samping obat 2 22
74,8 Kontra
GH 1,37 2 Genetic Demam, s
Kortisol 4,4 test marker
infeksi
3 Imaging meningkat,
Antibiotik +

Start Start Hidrokortison

Diazoxide 1
Start Octreotide
SC
 Minggu V Minggu VI Minggu VII Min ggu VIII

ONS 1 kcal/mL q2h BB = 6070 SGOT 123 Muntah BB = 6200

Kultur: S. gram gram
+ cornstarch perbaikan
aureus SGPT 124
Klinis baik Bilirubin Raw
USG abdomen
Pemantauan klinis, balans Antibiotik normal
normal at
diuresis, dan efek stop
samping obat USG kepala Jala
normal n

Turun GIR
bertahap Wean
Hidrokortison STOP IVFD Dx
 MRI Abdomen Kontras
08/09/20
• Tidak tampak lesi nodular, penyangatan
fokal, maupun restriksi difusi patologis.
• Pankreas bentuk dan ukuran normal,
tidak tampak lesi dengan intensitas,
penyangatan, maupun restriksi difusi
patologis. Duktus pankreatikus tidak
melebar.

Kesimpulan:
• Tidak tampak lesi fokal patologis pada pankreas.
• Tidak tampak kelainan pada organ abdominal atas lainnya yang
tervisualisasi.
 PROGNOSIS
§ Prognosis umumnya baik BILA
hipoglikemi didiagnosis CEPAT dan
ditataksana CEPAT DAN TEPAT
§ Evaluasi hasil jangka panjang à untuk
melihat disability intelectual, defek
neurologis,termasuk epilepsi dan
mikrosefali.
§ Motoric defisit 58,6%, speech delay
26,98%, defek kognitif 15.8%, sosial
emosional problem 9,48%. Tak ada
korelasi outcome dan latar belakang
genetik

Demirbilek H and Hussain K. Congenital Hyperinsulinism: Diagnosis and Treatment Update J Clin Res Pediatr Endocrinol 2017;9(Suppl
2):69-87
TERIMA
KASIH