2024年8月16日 · Caroli disease is a rare, congenital condition characterized by segmental dilation of large, intrahepatic bile ducts. Caroli disease belongs to a group of congenital disorders known as fibropolycystic liver diseases, which originate from ductal plate malformations during embryological development and are associated with various liver cysts.

2025年2月12日 · Caroli disease is associated with mutations in the PKHD1 gene, which is responsible for producing a protein that is essential for kidney and bile duct development during fetal growth. These mutations lead to the characteristic cystic dilation of the intrahepatic bile ducts seen in the disease.

2024年12月16日 · Pathologically, Caroli disease and Caroli syndrome belong to the spectrum of fibropolycystic liver disease which results from in utero malformation of the ductal plate 4. There is a high association with fibrocystic anomalies of the kidneys which share the same genetic defect (PKHD1 gene, chromosome region 6p21) 5.

Caroli disease (CD) is a rare congenital disorder characterized by segmental dilatation of the intrahepatic bile ducts. The simple CD is rare, with the majority of patients complicated by congenital liver fibrosis. Current treatments for CD mostly target complications but do not prevent disease progression.

2011年7月6日 · Caroli's disease is a developmental anomaly, and the most viable theory explaining its pathogenesis is that it is related to ductal plate malformation at different levels of the intrahepatic biliary tree [5].

2024年8月16日 · Caroli disease is a rare, congenital condition characterized by segmental dilation of large, intrahepatic bile ducts. Caroli disease belongs to a group of congenital disorders known as fibropolycystic liver diseases, which originate from ductal plate malformations during embryological development an …

Congenital hepatic fibrosis (CHF), Caroli disease and Caroli syndrome are three congenital conditions of abnormal embryonal biliary system remodeling, associated with a spectrum of macroscopic and microscopic cystic lesions of the liver.

Caroli’s disease is a rare congenital condition chara-cterized by non-obstructive saccular or fusiform dilatation of larger intrahepatic bile ducts. Cholangitis, liver cirrhosis, and cholangiocarcinoma are its potential complications.

Chin Med J (Engl). 2021 Oct 26;134 (23):2844-2846. doi: 10.1097/CM9.0000000000001827. 1 Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.

Caroli disease is a rare autosomal recessive disorder characterized by multiple segmental communicating dilatations of the intrahepatic bile ducts affecting all or part of the liver that is often associated with renal disorders.