DB93.21

ICD-11 MMS 2026-01

Idiopathic copper-associated cirrhosis

Description

Idiopathic copper-associated cirrhosis is a rare copper-overload liver disease characterised by a rapidly progressive liver cirrhosis from the first few years of life leading to hepatic insufficiency and harbouring a specific pathological aspect: pericellular fibrosis, inflammatory infiltration, hepatocyte necrosis, absence of steatosis, poor regeneration and histochemical copper staining.

Index terms

  • Idiopathic copper-associated cirrhosis
  • Non-Wilsonian hepatic copper toxicosis of infancy and childhood
  • Tyrolean infantile cirrhosis
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  • Idiopathic copper toxicosis
  • Indian childhood cirrhosis

Exclusions from above levels

  • 1E51 – Chronic viral hepatitis with cirrhosis
  • DB92 – Non-alcoholic fatty liver disease
  • DB92.1 – Non-alcoholic steatohepatitis
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  • DB94.10 – Alcoholic hepatitis with cirrhosis
  • DB94.2 – Alcoholic liver fibrosis
  • DB94.3 – Alcoholic cirrhosis of liver without hepatitis
  • DB95.5 – Drug-induced or toxic liver disease with fibrosis or cirrhosis of liver
  • DB98.8 – Cardiac cirrhosis
  • LB20.00 – Congenital hepatic fibrosis
  • ME10.1 – Unspecified jaundice
  • 01 – Certain infectious or parasitic diseases
  • 02 – Neoplasms
  • 05 – Endocrine, nutritional or metabolic diseases
  • 06 – Mental, behavioural or neurodevelopmental disorders
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • DB93.20 – Hereditary North American Indian childhood cirrhosis

Foundation entity

Idiopathic copper-associated cirrhosis – http://id.who.int/icd/entity/1692504835

Allowed postcoordination axes

Part of: DB93.2: Certain specified fibrosis or cirrhosis of liver › DB93: Hepatic fibrosis or cirrhosis › 13: Diseases of the digestive system