| ziekte van Caroli (aandoening) | | ziekte van Caroli | | syndroom van Caroli | | Aangeboren leverziekte waarbij de kleine galwegen in de lever uitrekken, zodat er gal in blijft staan, waardoor makkelijk ontstekingen en galstenen ontstaan. | | Caroli disease | | 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. |
| | Id | 717232005 | | Status | Primitive |
| PALGA thesaurus simple reference set for pathology |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | Q44.5 | | Term | Overige congenitale misvormingen van galwegen |
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| SNOMED CT to Orphanet simple map | 53035 |
| SNOMED CT to ICD-10 extended map | | Target | Q44.6 | | Rule | TRUE | | Advice | ALWAYS Q44.6 | | Correlation | SNOMED CT source code to target map code correlation not specified |
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