I. HERBERT SCHEINBERG, M.D.; IRMIN STERNLIEB, M.D.
Wilson's disease is the outcome of the interaction of an inherited defect and the environment. The inherited defect is an inability to synthesize a normal amount of the normal plasma copper-protein, ceruloplasmin.1 This is the consequence of the autosomal inheritance of one abnormal gene from each parent of the patient.2 The environmental factor is copper,3 and the interaction of inheritance and environment appears to result in the retention of abnormally large amounts of dietary copper.4, 5, 6 This copper is deposited in several organs, and seems ultimately to produce pathologic effects, most prominently in the brain, where degeneration, though
SCHEINBERG IH, STERNLIEB I. ENVIRONMENTAL TREATMENT OF A HEREDITARY ILLNESS: WILSON'S DISEASE12. Ann Intern Med. ;53:1151–1161. doi: 10.7326/0003-4819-53-6-1151
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Published: Ann Intern Med. 1960;53(6):1151-1161.
Encephalopathy, Gastroenterology/Hepatology, Liver Disease, Neurology.
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