Hepatic,pancreatic and renal manifestations of a ciliopathy

摘要

cqvip:Hepatorenal fibrocystic diseases are inherited disorders,characterized by developmental abnormalities and fibrocystic degeneration of the portobiliary system and kidneys^([1]).They are part of a larger group of disorders referred to as"ciliopathies"which affect 1 in 1000 people.Polycystic kidney disease is the most common kidney manifestation,while others include nephronophthisis,glomerulocystic disease and cystic dysplasia.Hepatic manifesta-tions include Caroli’s disease,congenital hepatic fibrosis and poly-cystic liver disease.