Sinha, RDalal, SSodhi, K2026-03-232026-03-232015https://hdl.handle.net/20.500.14572/5360R Sinha Department of Pediatrics, Military Hospital Jodhpu S Dalal Department of Paediatrics, AFMC Pune K Sodhi Department of Paediatrics, Command Hospital, ChandigarhAbstract: Gilbert syndrome characterized by jaundice with intermittent elevations of indirect bilirubin, in the absence of haemolysis or underlying liver disease, has both autosomal dominant and recessive inheritance. Crigler-Najjar syndrome type II (CNS2) is a hereditary disorder of bilirubin metabolism characterized by unconjugated hyperbilirubinemia due to reduced and inducible activity of hepatic bilirubin glucuronosyltransferase (GT). We report 20 children between age 5 to 15 years with unconjugated hyperbilirubenemia who were given seven days of oral phenobarbitone (5mg/kg/day) and decrease in level of bilirubin was noted. There was only 30-40% reduction of bilirubin in Crigler Najjar Syndrome Type 2 compared to Gilberts Syndrome in which bilirubin level normalised. This case series highlights the importance of simple test to differentiate these two conditions. This test is also very helpful in a place where enzyme level and mutational study cannot be done.en-USGilbertsCrigler NajjarhyperbilirubinemiaArticle