Crigler-Najjar syndrome is a rare autosomal recessive condition caused by complete (type I) or incomplete deficiency (type II) of hepatic microsomal enzyme uridine diphosphate-glucuronosyltransferase (UDPGT) activity. It is characterized by congenital unconjugated hyperbilirubinemia1-2. It is caused by mutations in the UGT1A1 gene which codes for the enzyme uridine diphosphate glucoronosyl transferase- 1, required for the conjugation and further excretion of bilirubin from the body.

Objectives: Our objective in report this case is to know the outome of pregnancy in patient with crigler-Najjar syndrome type 2.

Case: A 25 year old primigravida woman was admitted to our hospital with history of amenorrhoea for 9 months and history of jaundice since childhood. This patienthad been hospitalized two times in the medicine ward at 17 weeks and 33 weeks of gestation. Patient was diagnosed as crigler-Najjar syndrome type 2 at 33 weeks of gestation. Her total bilirubin level in pregnancy was in range of 10-20mg/dl. Her jaundice was persistent, though the severity fluctuated from time to time, increasing during periods of stress and pregnancy. Phenobarbitone treatment caused acute fall in bilirubin level. Pregnancy outcome was normal delivery of healthy newborn. Her newborn had mild indirect hyperbilirubinemia on day 3, did not require any treatment and his postnatal followup uptil 4 months  showed normal growth and development.

Conclusion: Crigler-Najjar syndrome type 2 disease, a rare cause of maternal unconjugated hyperbilirubinemia in pregnancy, poses no threat to the mother, and the elevated bilirubin levels do not seem harmful to the fetus.

Keywords: Crigler Najjar type 2, Pregnancy, Phenobarbitone

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