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Article

Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease.

Journal of pediatric gastroenterology and nutrition - 1 Jan 2012

O'Brien Kevin, Font-Montgomery Esperanza, Lukose Linda, Bryant Joy, Piwnica-Worms Katie, Edwards Hailey, Riney Lauren, Garcia Angelica, Daryanani Kailash, Choyke Peter, Mohan Parvathi, Heller Theo, Gahl William A, Gunay-Aygun Meral

Abstract excerpt

OBJECTIVES: Autosomal dominant (ADPKD) and recessive (ARPKD) polycystic kidney diseases are the most common hepatorenal fibrocystic diseases (ciliopathies). Characteristics of liver disease of these disorders are quite different. All of the patients with ARPKD have congenital hepatic fibrosis (CHF) often complicated by portal hypertension. In contrast, typical liver involvement in ADPKD is polycystic liver...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Hypertension, Portal

Share to Liver Cirrhosis

Share to Polycystic Kidney, Autosomal Dominant

Share to Receptors, Cell Surface

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Source

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Identifiers

PMID: 21694639
DOI: 10.1097/MPG.0b013e318228330c
PMCID: PMC8366680

Genes

PKD1
PKHD1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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