" /> Wilson disease - CISMeF





ICD-11 code : 5C64.00;

Preferred Label : Wilson disease;

ICD-11 definition : 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.;

ICD-11 synonym : Kinnier-Wilson disease; lenticular degenerative disease; Wilson's syndrome; Westphal-Strumpell Syndrome; lenticular syndrome; Progressive hepatolenticular degeneration; Copper storage disease; Neurohepatic degeneration; Copper retention; Hepatocerebral degeneration;

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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.

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06/07/2025


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