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Caroli syndrome

MONDO:0018808

A rare genetic hepatic disease characterized by multiple segmental cystic dilatations of both central and smaller peripheral bile ducts associated with congenital hepatic fibrosis. Age of symptom onset is variable, as is disease progression. Patients present with recurrent cholangitis, hepatolithiasis, and cholecystolithiasis. Portal hypertension may appear later in the disease course, and the risk of developing cholangiocarcinoma is increased significantly. The syndrome is often associated with autosomal recessive polycystic kidney disease.

9 clinical trials for this condition and its sub-types.

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Broader categories

Disease (618) Hereditary disease (172) Digestive system disorder (141) Syndromic disease (24) Biliary tract disorder (16) Human disease (14) Hepatobiliary disorder (7) Disease of genetic or genomic mechanism (2) Disease by body system or component (0) Disease by etiologic mechanism (0)
Trials to join now! 3 Not yet recruiting 2 Not yet finished but already full! 1 Completed 3
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