" /> Idiopathic copper-associated cirrhosis - CISMeF





ICD-11 code : DB93.21;

Preferred Label : Idiopathic copper-associated cirrhosis;

ICD-11 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.;

ICD-11 synonym : Non-Wilsonian hepatic copper toxicosis of infancy and childhood; Copper excess;

ICD-11 inclusion : Idiopathic copper toxicosis; Tyrolean infantile cirrhosis; Indian childhood cirrhosis;

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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.

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04/05/2025


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