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Idiopathic copper-associated cirrhosis

Disease 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.

ORPHA:209919

Classification level: Disorder
  • Synonym(s):
    • Non-Wilsonian hepatic copper toxicosis of infancy and childhood
  • Prevalence: Unknown
  • Inheritance: Unknown 
  • Age of onset: Childhood
  • ICD-10: K74.6
  • ICD-11: DB93.21
  • OMIM: 215600
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

  A summary on this disease is available in Deutsch (2012) Español (2012) Italiano (2012) Nederlands (2012)

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Further information on this disease

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The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.