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717232005: Caroli disease (disorder)


Status: current. Date: 31-Jul 2016

Descriptions:

Id Description Lang Type Status Case? Module
3308765010 Caroli disease (disorder) en Fully specified name Active Entire term case sensitive (core metadata concept) SNOMED CT core
3308766011 Caroli disease en Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT core
3308767019 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. en Definition Inactive Entire term case sensitive (core metadata concept) SNOMED CT core
3308768012 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. en Definition Inactive Entire term case sensitive (core metadata concept) SNOMED CT core
5401523014 Caroli disease (CD) is a rare congenital liver disease characterized by non-obstructive cystic dilatations of the intra-hepatic and rarely extra-hepatic bile ducts. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core
5401524015 Caroli disease (CD) is a rare congenital liver disease characterised by non-obstructive cystic dilatations of the intra-hepatic and rarely extra-hepatic bile ducts. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core


0 descendants.

Expanded Value Set


Outbound Relationships Type Target Active Characteristic Refinability Group Values
Caroli disease (disorder) Is a Congenital dilatation of lobar intrahepatic bile duct true Congenital syphilitic hepatomegaly Congenital syphilitic hepatomegaly
Caroli disease (disorder) Associated morphology Dilatation true Congenital syphilitic hepatomegaly Congenital syphilitic hepatomegaly 1
Caroli disease (disorder) Occurrence Congenital true Congenital syphilitic hepatomegaly Congenital syphilitic hepatomegaly 1
Caroli disease (disorder) Finding site Structure of wall of intrahepatic biliary tract true Congenital syphilitic hepatomegaly Congenital syphilitic hepatomegaly 1
Caroli disease (disorder) Pathological process (attribute) Pathological developmental process true Congenital syphilitic hepatomegaly Congenital syphilitic hepatomegaly 1

Inbound Relationships Type Active Source Characteristic Refinability Group

This concept is not in any reference sets

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