Congenital hepatic fibrosis in autosomal recessive polycystic kidney disease.
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.
Division of Gastroenterology, Hepatology & Nutrition, The Children's Hospital of Philadelphia, Pennsylvania, USA. wenj@email.chop.edu
Jessica
J
Wen
D000818:Animals; D002118:Calcium / Q000378:metabolism; D016767:Caroli Disease / Q000175:diagnosis; D000242:Cyclic AMP / Q000378:metabolism; D004195:Disease Models, Animal; D005091:Exons; D006801:Humans; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000150:complications / Q000151:congenital*; D051379:Mice; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications* / Q000235:genetics; D051381:Rats; D017207:Rats, Sprague-Dawley; D057170:Translational Research, Biomedical
D000242:Cyclic AMP; D002118:Calcium