Open AccessCongenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease
Author(s) -
Wen Jessica
Publication year - 2011
Publication title -
clinical and translational science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.303
H-Index - 44
eISSN - 1752-8062
pISSN - 1752-8054
DOI - 10.1111/j.1752-8062.2011.00306.x
Subject(s) - congenital hepatic fibrosis , autosomal recessive polycystic kidney disease , medicine , cystic fibrosis , pathology , polycystic kidney disease , pathogenesis , kidney , disease , fibrosis , polycystic kidney , gastroenterology , cyst , cirrhosis , portal hypertension
Autosomal recessive polycystic kidney disease (ARPKD) is a developmental disorder that mainly affects the kidneys and the biliary tract. Affected patients often have massively enlarged cystic kidneys as well as congenital hepatic fibrosis (CHF) characterized by dilated bile ducts and associated peribiliary fibrosis. This review will examine what is known about ARPKD‐associated liver disease and will highlight areas of ongoing research into its pathogenesis and potential treatment. Clin Trans Sci 2011; Volume 4: 460–465