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 http://www.orphadata.org. Data version April 2024
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Idiopathic copper-associated cirrhosis?
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