Genetic and Familial Forms of Pediatric Intrahepatic Cholestasis.

Authors

Maria Amendola, Lara Berklite, Simon Horslen, James E Squires

Published in

Clinics in liver disease. Volume 30. Issue 4. Pages 835-861. Epub Sep 01, 2026.

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.

PMID:
42946520
Bibliographic data and abstract were imported from PubMed on 28 Oct 2026.

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