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Indirectly directed hyperbilirubinemia
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Indirectly directed hyperbilirubinemia






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Indirectly directed hyperbilirubinemia Indirectly directed hyperbilirubinemia Presentation Transcript

  • 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
  • Indirect hyperbilirubinemia at birth Is it all physiological? Dr. Smita Malhotra Apollo Centre for Advanced Pediatrics Indraprastha Apollo Hospitals, Delhi
  • Female Qatar Product of consanguineous marriage 27 months
  • Jaundice since D2 of life
  • Total Bilirubin 25 mg% Indirect Bilirubin 23.2 mg%
  • 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
  • No hematoma No blood group incompatibility No evidence of sepsis No evidence of hemolysis CBC/PS/Retic’s/G6PD/DCT LFT normal TFT normal
  • Phototherapy Multiple exchange transfusions Mutation analysis in Europe at 4 months ? Criggler Najjar Syndrome type 1
  • Phototherapy for 18 hours/day Bilirubin 16-18 mg/dl Home phototherapy
  • LDLT at 27 months LT advised at IAH at 1 year of age
  • At admission Weight 14.8 kg Deeply icteric No organomegaly No ascites Unsteady gait LL power 4/5 Ankle jerk weak Plantars flexor
  • 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
  • BERA – Normal MRI brain Altered signal intensity in putamen Pediatric neurologist Cleared for LRLT Neurological improvement likely
  • Pre transplant evaluation Vaccination Nutritional status
  • Complete haemogram Cultures Coagulation studies KFT Thyroid function tests Urine P/Cr ratio
  • Viral markers CXR ECG ECHO Dental clearance ENT clearance
  • USG doppler Normal span of liver and spleen Normal flow pattern in PV/HA/HV CT angiography abdomen
  • Segment II and III graft from the left lobe Donor- Father Recovered well Discharged 21 days after liver transplantation
  • Post transplant 5 years post transplant doing well with normal liver function and a serum bilirubin of 0.8 mg/dL Neurological status- normal
  • Criggler Najjar syndrome Rare (I in 1,000,000) and severe AR disorder UGT1A1 mutation Hereditary nonhemolytic unconjugated jaundice Inborn error of liver metabolism
  • 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
  • High-performance liquid chromatography of bile Tissue enzyme assay of a liver biopsy sample Mutation studies
  • Treatment Phototherapy Exchange transfusions LT- only definitive Rx Future options Hepatocyte transplant Gene therapy