Idiopathic copper-associated cirrhosis
Idiopathic copper-associated cirrhosis
Definition
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.
Also known as non-Wilsonian hepatic copper toxicosis of infancy and childhood — per MONDO