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Updated: Oct 29, 2026

Isolation of Neonatal Extrahepatic Cholangiocytes
Published on: June 5, 2014
Genetic and Familial Forms of Pediatric Intrahepatic Cholestasis
Maria Amendola1, Lara Berklite2, Simon Horslen1
1Division of Gastroenterology, Hepatology and Nutrition, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
Abstract:
This review explores the expanding landscape of genetic and familial forms of pediatric intrahepatic cholestasis-noting a pivot from the labeling of discrete progressive familial intrahepatic cholestasis disorders to a classification system based on mechanistic understandings of the specific genetic and molecular etiologies that lead to the wide-ranging spectrum of cholestatic phenotypes. This review suggests a novel pediatric intrahepatic cholestasis classification-by the mechanism of etiology-and reviews new and emerging advancements in the management of cholestasis and progression of liver disease.
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