Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

Autosomal recessive polycystic kidney disease (ARPKD) is a severe monogenic disorder that occurs due to mutations in the PKHD1 gene. Congenital hepatic fibrosis (CHF) associated with ARPKD is characterized by the presence of hepatic cysts derived from dilated bile ducts and a robust, pericystic fibr...

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Bibliographic Details
Main Authors: Jiang, Lu, Fang, Pingping, Weemhoff, James L., Apte, Udayan, Pritchard, Michele T.
Format: Online
Language:English
Published: Hindawi Publishing Corporation 2016
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5116503/

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5116503/

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