Article
Hepatic Parenchymal Injury in Crigler-Najjar Type I.
Journal of pediatric gastroenterology and nutrition - 1 Apr 2018
Mitchell Ellen, Ranganathan Sarangarajan, McKiernan Patrick, Squires Robert H, Strauss Kevin, Soltys Kyle, Mazariegos George, Squires James E
Abstract excerpt
BACKGROUND: Crigler-Najjar syndrome type I (CNI) arises from biallelic variants of UGT1A1 that abrogate uridine diphosphate glucuronosyltransferase (UGT1A1) activity resulting in unconjugated hyperbilirubinemia. Historically, liver parenchyma in CNI was considered structurally and histologically normal. Recent review of CNI liver explants revealed fibrosis. Our aim was to investigate the association between...
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