Indirectly directed hyperbilirubinemia


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Indirectly directed hyperbilirubinemia

  1. 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. 2. Indirect hyperbilirubinemia at birth Is it all physiological? Dr. Smita Malhotra Apollo Centre for Advanced Pediatrics Indraprastha Apollo Hospitals, Delhi
  3. 3. Female Qatar Product of consanguineous marriage 27 months
  4. 4. Jaundice since D2 of life
  5. 5. Total Bilirubin 25 mg% Indirect Bilirubin 23.2 mg%
  6. 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. 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. 8. Phototherapy Multiple exchange transfusions Mutation analysis in Europe at 4 months ? Criggler Najjar Syndrome type 1
  9. 9. Phototherapy for 18 hours/day Bilirubin 16-18 mg/dl Home phototherapy
  10. 10. LDLT at 27 months LT advised at IAH at 1 year of age
  11. 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. 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. 13. BERA – Normal MRI brain Altered signal intensity in putamen Pediatric neurologist Cleared for LRLT Neurological improvement likely
  14. 14. Pre transplant evaluation Vaccination Nutritional status
  15. 15. Complete haemogram Cultures Coagulation studies KFT Thyroid function tests Urine P/Cr ratio
  16. 16. Viral markers CXR ECG ECHO Dental clearance ENT clearance
  17. 17. USG doppler Normal span of liver and spleen Normal flow pattern in PV/HA/HV CT angiography abdomen
  18. 18. Segment II and III graft from the left lobe Donor- Father Recovered well Discharged 21 days after liver transplantation
  19. 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. 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. 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. 22. High-performance liquid chromatography of bile Tissue enzyme assay of a liver biopsy sample Mutation studies
  23. 23. Treatment Phototherapy Exchange transfusions LT- only definitive Rx Future options Hepatocyte transplant Gene therapy