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

doi:10.1053/j.gastro.2012.09.056

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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 detection of mutations in PKHD1. METHODS: Patients with ARPKD and congenital hepatic fibrosis were evaluated at the...

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Identifiers

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

Genes

PKHD1

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