Hepatic, pancreatic and renal manifestations of a ciliopathy  

作　　者：Sreelakshmi Kotha Philip Berry 

机构地区：[1]Department of Gastroenterology,Guy’s and St Thomas’Foundation Trust,London,UK

出　　处：《Hepatobiliary & Pancreatic Diseases International》2021年第4期394-395,共2页国际肝胆胰疾病杂志（英文版）

摘　　要：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.

关 键 词：HEPATIC kidney DEGENERATION 

分 类 号：R575[医药卫生—消化系统] R576[医药卫生—内科学] R692[医药卫生—临床医学]