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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Detalles Bibliográficos
Autores principales: Jiang, Lu, Fang, Pingping, Weemhoff, James L., Apte, Udayan, Pritchard, Michele T.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2016
Materias:
Review Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5116503/
https://www.ncbi.nlm.nih.gov/pubmed/27891514
http://dx.doi.org/10.1155/2016/4918798

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5116503/
https://www.ncbi.nlm.nih.gov/pubmed/27891514
http://dx.doi.org/10.1155/2016/4918798

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