DB93.20

ICD-11 MMS 2026-01

Hereditary North American Indian childhood cirrhosis

Description

Hereditary North American Indian childhood cirrhosis is a severe autosomal recessive intrahepatic cholestasis that has only been described in aboriginal children from northwestern Quebec. Manifesting first as transient neonatal jaundice, the disease evolves into periportal fibrosis and cirrhosis during a period ranging from childhood to adolescence.

Index terms

  • Hereditary North American 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

  • DB93.21 – Idiopathic copper-associated cirrhosis

Foundation entity

Hereditary North American Indian childhood cirrhosis – http://id.who.int/icd/entity/1992710077

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