Idiopathic copper-associated cirrhosis

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Idiopathic copper-associated cirrhosis

Synonyms: Non-Wilsonian hepatic copper toxicosis of infancy and childhood

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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version May 2024

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Idiopathic copper-associated cirrhosis?

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