Indirectly directed hyperbilirubinemia

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  • 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
  • 20. Criggler Najjar syndrome Rare (I in 1,000,000) and severe AR disorder UGT1A1 mutation Hereditary nonhemolytic unconjugated jaundice Inborn error of liver metabolism
  • 21. 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
  • 22. High-performance liquid chromatography of bile Tissue enzyme assay of a liver biopsy sample Mutation studies
  • 23. Treatment Phototherapy Exchange transfusions LT- only definitive Rx Future options Hepatocyte transplant Gene therapy