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Article

Treatment of the Crigler–Najjar Syndrome Type I with Hepatocyte Transplantation

14 May 1998

Abstract excerpt

Crigler–Najjar syndrome type I is a recessively inherited disorder characterized by severe unconjugated hyperbilirubinemia beginning at birth. The syndrome results from an absence of hepatic uridine diphosphoglucuronate (UDP) glucuronosyltransferase activity, which is essential for the conjugation and excretion of bilirubin. Because of the accumulation of unconjugated bilirubin in plasma, patients are at risk for...

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PMID: 9580649
DOI: 10.1056/nejm199805143382004

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