Crigler-Najjar disease in pregnancy.

Abstract

Crigler-Najjar 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. However, the disease is expressed in two forms, one of which is fatal. The maternal total bilirubin (mostly unconjugated) was 8.5 mg/dL in the first trimester, fell to 5.0 mg/dL in the second, and rose again to 8.8 mg/dL at term. The infant was jaundiced at birth, with umbilical cord total bilirubin at 7.6 mg/dL. The jaundice resolved without treatment, and no sequelae of hyperbilirubinemia were present. Crigler-Najjar disease type II seems to pose no unique maternal risk during pregnancy. The fetus seems to be resistant to elevated maternal unconjugated bilirubin, but the neonate may required therapy for hyperbilirubinemia.