ICD·index Chapters ENESDE
DB93.21 category

Idiopathic copper-associated cirrhosis

Definition

Idiopathic copper-associated cirrhosis is a rare copper-overload liver disease characterised by a rapidly progressive liver cirrhosis from the first few years of life leading to hepatic insufficiency and harbouring a specific pathological aspect: pericellular fibrosis, inflammatory infiltration, hepatocyte necrosis, absence of steatosis, poor regeneration and histochemical copper staining.

Also indexed as

Idiopathic copper toxicosisIdiopathic copper-associated cirrhosisIndian childhood cirrhosisNon-Wilsonian hepatic copper toxicosis of infancy and childhoodTyrolean infantile cirrhosis

Nearby in Certain specified fibrosis or cirrhosis of liver