Abstract：
Fibropolycystic liver diseases (FLDs) make up a rare spectrum of heritable hepatobiliary diseases resulting from congenital ductal plate malformations (DPMs) due to the dysfunction of proteins expressed on the primary cilia of cholangiocytes. The embryonic development of the ductal plate is key to understanding this spectrum of diseases. In particular, DPMs can result in various degrees of intrahepatic duct involvement and a wide spectrum of cholangiopathies, including congenital hepatic fibrosis, Caroli disease, polycystic liver disease, and Von Meyenberg complexes. The most common clinical manifestations of FLDs are portal hypertension, cholestasis, cholangitis, and (in rare cases) cholangiocarcinoma. This article reviews recent updates in the pathophysiology, imaging, and clinical management of FLDs.
摘要：
纤维多囊性肝病（FLD）构成了罕见的遗传性肝胆疾病，这是由于先天性导管板畸形（DPM）所致，这是由于胆管细胞初级纤毛上表达的蛋白质功能障碍所致。导管板的胚胎发育是理解这种疾病谱的关键。特别是,DPM可导致不同程度的肝内导管受累和广泛的胆管病变，包括先天性肝纤维化,Caroli病,多囊肝病,和冯·梅恩伯格情结。FLDs最常见的临床表现是门静脉高压,胆汁淤积,胆管炎,和（在极少数情况下）胆管癌。本文回顾了病理生理学的最新进展，成像,和FLD的临床管理。
