5C64.00

ICD-11 MMS 2026-01

Wilson disease

Description

Wilson disease is an autosomal recessive disorder of copper metabolism characterised by the toxic accumulation of copper, mainly in the liver and central nervous system that may present as hepatic, neurologic or psychiatric forms.

Index terms

  • Wilson disease
  • Copper retention
  • Copper storage disease
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  • Hepatocerebral degeneration
  • Kinnier-Wilson disease
  • Neurohepatic degeneration
  • Progressive hepatolenticular degeneration
  • Westphal-Strumpell Syndrome
  • Wilson's syndrome
  • lenticular degenerative disease
  • lenticular syndrome

Exclusions from above levels

  • 5B57 – Vitamin D deficiency
  • 5B5K – dietary mineral deficiency
  • http://id.who.int/icd/entity/962023213 – Disorders of the parathyroids or parathyroid hormone system
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  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C64.0Y – Other specified disorders of copper metabolism
  • 5C64.0Z – Disorders of copper metabolism, unspecified

Foundation entity

Wilson disease – http://id.who.int/icd/entity/468161208

Allowed postcoordination axes

Part of: 5C64.0: Disorders of copper metabolism › 5C64: Disorders of mineral absorption or transport › 05: Endocrine, nutritional or metabolic diseases