1. Indirect Hyperbilirubinemia at birth –
Is it all physiological?
Presenter:
Smita Malhotra - Apollo Hospital, New Delhi
Moderator:
Pediatric Gastroenterologist , Apollo Hospital, New Delhi
Anupam Sibal
Pediatric Gastroenterologist, SGPGI, Lucknow
Girish Gupte
Pediatric Hepatologist, Birmingham Children’s Hospital, UK
Panelists
Alka Jadhav
Pediatric Gastroenterologist, LTMGH, Sion, Mumbai

2. Indirect hyperbilirubinemia at birth
Is it all physiological?
Dr. Smita Malhotra
Apollo Centre for Advanced Pediatrics
Indraprastha Apollo Hospitals, Delhi

3. Female
Qatar
Product of consanguineous marriage
27 months

4. Jaundice since D2 of life

5. Total Bilirubin 25 mg%
Indirect Bilirubin 23.2 mg%

6. High Indirect Bilirubin
Direct coomb’s test
Positive
Coomb’s test
negative
Increased Hb/HaematocritDelayed
cord clamping
Maternal foetal or twin – twin transfusion
Isoimmunization Rh
incompatibility
ABO incompatibility
Minor group incompatibility
Normal / Low – Hb/Hct.
RBC Morphology abnormal
High reticulocyte countABO
incompatibility
G6PD deficiency
α – thalassaemia
Spherocytosis / Elliptocytosis
Disseminated intravascular
coagulation (D.I.C.)
RBC – normalReticulocyte count
– normalExtravascular blood Cephalhaematoma, Bruises
Increased entero – hepatic
circulation
Pyloric stenosis
Intestinal obstruction

7. No hematoma
No blood group incompatibility
No evidence of sepsis
No evidence of hemolysis
CBC/PS/Retic’s/G6PD/DCT
LFT normal
TFT normal

8. Phototherapy
Multiple exchange transfusions
Mutation analysis in Europe at 4 months
? Criggler Najjar Syndrome type 1

9. Phototherapy for 18 hours/day
Bilirubin 16-18 mg/dl
Home phototherapy

10. LDLT at 27 months
LT advised at IAH at 1 year of age

11. At admission
Weight 14.8 kg
Deeply icteric
No organomegaly
No ascites
Unsteady gait
LL power 4/5
Ankle jerk weak
Plantars flexor

12. Indirect hyperbilirubinemia
Total bilirubin 29.31 mg/dL, direct 0.53
AST 41 U/l
ALT 46 U/l
Alkaline phosphatase 218
Prothrombin time 12.4 sec

13. BERA – Normal
MRI brain
Altered signal intensity in putamen
Pediatric neurologist
Cleared for LRLT
Neurological improvement likely

14. Pre transplant evaluation
Vaccination
Nutritional status

15. Complete haemogram
Cultures
Coagulation studies
KFT
Thyroid function tests
Urine P/Cr ratio

16. Viral markers
CXR
ECG
ECHO
Dental clearance
ENT clearance

17. USG doppler
Normal span of liver and spleen
Normal flow pattern in PV/HA/HV
CT angiography abdomen

18. Segment II and III graft from the left lobe
Donor- Father
Recovered well
Discharged 21 days after liver transplantation

19. Post transplant
5 years post transplant doing well with
normal liver function and a serum bilirubin of
0.8 mg/dL
Neurological status- normal

23. Criggler Najjar syndrome
Rare (I in 1,000,000) and severe AR disorder
UGT1A1 mutation
Hereditary nonhemolytic unconjugated
jaundice
Inborn error of liver metabolism

24. Type 1
Complete absence of UGT activity
Bil 15-50 mg/dl
Risk for kernicterus
Type 2
Partial enzyme activity
Inducible with phenobarbitone
Lower bilirubin levels

25. High-performance liquid chromatography of
bile
Tissue enzyme assay of a liver biopsy sample
Mutation studies

26. Treatment
Phototherapy
Exchange transfusions
LT- only definitive Rx
Future options
Hepatocyte transplant
Gene therapy