Wilson's disease, Kayser and Fleischer's sign and Walshe's treatment.
Wilson's disease is named after Kinnier Wilson (1878-1937), a famous British neurologist. It is an inherited condition, due to an excess of copper in the liver and brain. The mechanism is unknown, but the gene has been mapped to chromosome 13. The worldwide prevalence is about 30 per million. Patients present with liver disease and/or neuropsychiatric manifestations. Early diagnosis is crucial because there is an effective treatment. This treatment, usually with penicillamine, must be continued for life and without interruption. Liver transplantation may be lifesaving in patients with fulminant hepatitis, or with severe liver disease which is unresponsive to treatment.
McIntyre
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University Department of Medicine, Royal Free Hospital School of Medicine, London.
D002570:Ceruloplasmin / Q000378:metabolism; D003316:Corneal Diseases / Q000209:etiology; D004112:Dimercaprol / Q000627:therapeutic use; D006527:Hepatolenticular Degeneration / Q000188:drug therapy / Q000266:history*; D049673:History, 20th Century; D006801:Humans; D010859:Pigmentation Disorders / Q000209:etiology; D006113:United Kingdom
D004112:Dimercaprol; D002570:Ceruloplasmin