717232005: ziekte van Caroli (aandoening)

SNOMED CT Concept\Clinical finding (finding)\...

\Viscus structure finding (finding)\Abdominal organ finding\Liver finding\Disease of liver\Congenital anomaly of liver\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)

\Digestive system finding (finding)\Finding of biliary tract\Disorder of biliary tract\Congenital anomaly of biliary tract\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Finding of biliary tract\Disorder of biliary tract\Cholangiectasis\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Liver finding\Disease of liver\Congenital anomaly of liver\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive organ (disorder)\Disorder of liver and/or biliary tract\Congenital abnormality of liver and/or biliary tract (disorder)\Congenital anomaly of biliary tract\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive organ (disorder)\Disorder of liver and/or biliary tract\Congenital abnormality of liver and/or biliary tract (disorder)\Congenital anomaly of liver\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive organ (disorder)\Disorder of liver and/or biliary tract\Disease of liver\Congenital anomaly of liver\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive organ (disorder)\Disorder of liver and/or biliary tract\Disorder of biliary tract\Congenital anomaly of biliary tract\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive organ (disorder)\Disorder of liver and/or biliary tract\Disorder of biliary tract\Cholangiectasis\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive organ (disorder)\Congenital anomaly of digestive organ\Congenital abnormality of liver and/or biliary tract (disorder)\Congenital anomaly of biliary tract\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive organ (disorder)\Congenital anomaly of digestive organ\Congenital abnormality of liver and/or biliary tract (disorder)\Congenital anomaly of liver\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive system specific to fetus OR newborn\Congenital anomaly of digestive system\Congenital anomaly of digestive organ\Congenital abnormality of liver and/or biliary tract (disorder)\Congenital anomaly of biliary tract\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)
\Digestive system finding (finding)\Disorder of digestive system (disorder)\Disorder of digestive system specific to fetus OR newborn\Congenital anomaly of digestive system\Congenital anomaly of digestive organ\Congenital abnormality of liver and/or biliary tract (disorder)\Congenital anomaly of liver\Congenital dilatation of lobar intrahepatic bile duct\Caroli disease (disorder)

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jul 2016. Module: SNOMED CT core

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

698361000146113 ziekte van Caroli (aandoening) nl Fully specified name Active Only initial character case insensitive (core metadata concept) SNOMED CT Netherlands NRC maintained module (core metadata concept)

698371000146119 ziekte van Caroli nl Synonym (core metadata concept) Active Only initial character case insensitive (core metadata concept) SNOMED CT Netherlands NRC maintained module (core metadata concept)

12341931000146114 syndroom van Caroli nl Synonym (core metadata concept) Active Only initial character case insensitive (core metadata concept) SNOMED CT Netherlands NRC maintained module (core metadata concept)

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 Active 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 Active Entire term case sensitive (core metadata concept) SNOMED CT core

13087081000146118 Aangeboren leverziekte waarbij de kleine galwegen in de lever uitrekken, zodat er gal in blijft staan, waardoor makkelijk ontstekingen en galstenen ontstaan. nl Definition Active Entire term case sensitive (core metadata concept) SNOMED CT Netherlands NRC maintained module (core metadata concept)

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	Inferred relationship	Some
Caroli disease (disorder)	Associated morphology	congenitale dilatatie (afwijkende morfologie)	false	Inferred relationship	Some	1
Caroli disease (disorder)	Occurrence	Congenital	true	Inferred relationship	Some	1
Caroli disease (disorder)	Finding site	Intrahepatic biliary tract structure	false	Inferred relationship	Some	1
Caroli disease (disorder)	Pathological process (attribute)	Pathological developmental process	true	Inferred relationship	Some	1
Caroli disease (disorder)	Associated morphology	Dilatation	true	Inferred relationship	Some	1
Caroli disease (disorder)	Finding site	Structure of wall of intrahepatic biliary tract	true	Inferred relationship	Some	1

Inbound Relationships

Type

Active

Source

Characteristic

Refinability

Group

Reference Sets

Dutch pathology simple reference set (foundation metadata concept)

Back to Start

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
698361000146113	ziekte van Caroli (aandoening)	nl	Fully specified name	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT Netherlands NRC maintained module (core metadata concept)
698371000146119	ziekte van Caroli	nl	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT Netherlands NRC maintained module (core metadata concept)
12341931000146114	syndroom van Caroli	nl	Synonym (core metadata concept)	Active	Only initial character case insensitive (core metadata concept)	SNOMED CT Netherlands NRC maintained module (core metadata concept)
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	Active	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	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
13087081000146118	Aangeboren leverziekte waarbij de kleine galwegen in de lever uitrekken, zodat er gal in blijft staan, waardoor makkelijk ontstekingen en galstenen ontstaan.	nl	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT Netherlands NRC maintained module (core metadata concept)