Clinical features 412

Diagnosis 413

Management 414

Indications for liver transplantation 415

• Wilson's disease (WD) is an autosomal disorder of copper metabolism. The gene ATP7B codes for a copper carrier which both exports copper from hepatocyte to bile, and enables caeruloplasmin synthesis.

• WD occurs worldwide with reported incidences of 5–30 per million population. WD may present with almost any variety of liver disease in the age group 3–12 years, with psychiatric and/or neurological disease in adolescence or young adults, with combined hepatic and neurological problems, or less commonly with haemolysis or arthritis....