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Article

Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease.

Gastroenterology - 1 Jan 2013

Gunay-Aygun Meral, Font-Montgomery Esperanza, Lukose Linda, Tuchman Gerstein Maya, Piwnica-Worms Katie, Choyke Peter, Daryanani Kailash T, Turkbey Baris, Fischer Roxanne, Bernardini Isa, Sincan Murat, Zhao Xiongce, Sandler Netanya G, Roque Annelys, Douek Daniel C, Graf Jennifer, Huizing Marjan, Bryant Joy C, Mohan Parvathi, Gahl William A, Heller Theo

Abstract excerpt

BACKGROUND & AIMS: Autosomal recessive polycystic kidney disease (ARPKD), the most common ciliopathy of childhood, is characterized by congenital hepatic fibrosis and progressive cystic degeneration of kidneys. We aimed to describe congenital hepatic fibrosis in patients with ARPKD, confirmed by...

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Source

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Identifiers

PMID: 23041322
DOI: 10.1053/j.gastro.2012.09.056
PMCID: PMC4162098

Genes

PKHD1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-23041322-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 4 Sept 2026.

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