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idiopathic copper-associated cirrhosis

Disease Summary
Associated Targets ()

Mondo Description 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.
Mondo Term and Equivalent IDs
MONDO:0016204:  idiopathic copper-associated cirrhosis
Orphanet:209919: 
SCTID:715864007: