Diseases

Caroli Disease

caroli disease

Entity Type: Diseases
Relation Groups: 3
Relation Preview: 24

Basic Information

Grouped by core information, identifiers, and descriptions.

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Core Information

Disease Name: Caroli Disease
Standard Disease Name: caroli disease
MeSH Tree: No data
ICD-10: No data

Identifiers

DO ID: No data
MeSH ID: D016767
OMIM ID: No data
UMLS ID: C0162510
HPO ID: No data

Description and Extensions

Description: SNOMEDCT_US_2016_09_01:A rare congenital disorder characterized by multifocal, segmental dilatation of the large intrahepatic bile ducts. It may present at any age and predominantly affects females. Less than 250 cases have been described worldwide. Caroli disease is characterized by bile ductal ectasia without other apparent hepatic abnormalities. It presents with recurrent bacterial cholangitis, biliary stones causing biliary pain or episodes of pancreatitis. The more common variant of this disease, named Caroli syndrome, is characterized by dilatations of the large bile duct associated with congenital hepatic fibrosis. The etiology of Caroli disease is unknown and its occurrence is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner.|SNOMEDCT_US_2016_09_01:A rare congenital disorder characterised by multifocal, segmental dilatation of the large intrahepatic bile ducts. It may present at any age and predominantly affects females. Less than 250 cases have been described worldwide. Caroli disease is characterised by bile ductal ectasia without other apparent hepatic abnormalities. It presents with recurrent bacterial cholangitis, biliary stones causing biliary pain or episodes of pancreatitis. The more common variant of this disease, named Caroli syndrome, is characterised by dilatations of the large bile duct associated with congenital hepatic fibrosis. The aetiology of Caroli disease is unknown and its occurrence is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner.|NCI2016_02D:A rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts. It is associated with cholangitis and the formation of stones.|MSH2017_2016_08_12:Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.

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