Abstract
Crigler–Najjar (CN) syndrome is a congenital inborn error of hepatic bilirubin metabolism caused by the deficiency of bilirubin uridinediphosphate glucuronosyltransferase activity. The 2 types of CN can be distinguished based on their response to phenobarbital treatment. CN type I patients have profound unconjugated hyperbilirubinemia that does not respond to phenobarbital; they are at permanent risk for life-threatening bilirubin encephalopathy. Treatment of CN-1 disease relies on daily prolonged phototherapy (10–12 h/day). We report on a pregnancy in a CN-1 mother treated with intensive phototherapy and semimonthly albumin infusions. The mother gave birth to a healthy baby. Intensive management of pregnancy in CN-1 patients may result in a successful pregnancy.
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