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Ідіопатичний мідь-асоційований цироз печінки

ORPHA:209919· ICD-10 K74.6· Idiopathic copper-associated cirrhosis

Визначення(English summary)

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.

Поширеність
Unknown
Успадкування
Unknown
Вік початку
Childhood