idiopathic copper-associated cirrhosis
Go to external page http://purl.obolibrary.org/obo/MONDO_0016204
Idiopathic copper-associated cirrhosis is a rare copper-overload liver disease characterized by a rapidly progressive liver cirrhosis from the first few years of life leading to hepatic insufficiency and harboring a specific pathological aspect: pericellular fibrosis, inflammatory infiltration, hepatocyte necrosis, absence of steatosis, poor regeneration and histochemical copper staining. [ Orphanet:209919 ]
Synonyms: non-Wilsonian hepatic copper toxicosis of infancy and childhood
Term information
- UMLS:C4274853 (MONDO:equivalentTo)
- GARD:17106 (MONDO:GARD)
- Orphanet:209919 (MONDO:equivalentTo)
- SCTID:715864007 (MONDO:equivalentTo)
- MEDGEN:907284 (MONDO:equivalentTo)
- icd11.foundation:1692504835 (Orphanet:209919)
ordo_disorder, gard_rare, rare, nord_rare, orphanet_rare
http://identifiers.org/medgen/907284
http://linkedlifedata.com/resource/umls/id/C4274853
http://www.orpha.net/ORDO/Orphanet_209919
http://identifiers.org/snomedct/715864007
Term relations
- liver disorder
- idiopathic disease
- has characteristic some rare
- disease has location some liver parenchyma