ENVIRONMENTAL TREATMENT OF A HEREDITARY ILLNESS: WILSON'S DISEASE
Authors: I H SCHEINBERG, I STERNLIEB
Journal: Annals of Internal Medicine (1960)

Article1 December 1960ENVIRONMENTAL TREATMENT OF A HEREDITARY ILLNESS: WILSON'S DISEASEI. HERBERT SCHEINBERG, M.D., IRMIN STERNLIEB, M.D.I. HERBERT SCHEINBERG, M.D.Search for more papers by this author, IRMIN STERNLIEB, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-53-6-1151 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptINTRODUCTIONWilson'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.1This is the consequence of the autosomal inheritance of one abnormal gene from each parent of the patient.2The environmental factor is copper,3and the interaction of inheritance and environment appears to result in the retention of abnormally large amounts of dietary copper.4, 5, 6This copper is deposited in several organs, and seems ultimately to produce pathologic effects, most prominently in the brain, where degeneration, though...Bibliography1. ScheinbergGitlin IHD: Deficiency of ceruloplasmin in patients with hepatolenticular degeneration (Wilson's disease), Science 116: 484, 1952. CrossrefMedlineGoogle Scholar2. Bearn AG: Genetic aspects of Wilson's disease, Proc. Roy. Soc. Med. 52: 61, 1959. CrossrefMedlineGoogle Scholar3. Cumings JN: Copper and iron content of brain and liver in normal and in heptolenticular degeneration, Brain 71: 410, 1948. 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To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: New York, N. Y.*Received for publication April 29, 1960.Presented in part at the Forty-first Annual Session of The American College of Physicians, San Francisco, California, April 8, 1960.From the Department of Medicine, Albert Einstein College of Medicine, and the Bronx Municipal Hospital Center, New York, N. Y.†This work was supported in part by grants from the National Institute of Arthritis and Metabolic Disease of the U. S. Public Health Service (A-1059), and the Life Insurance Medical Research Fund (G-58-58).‡Postdoctoral Research Fellow, Division of General Medical Sciences, National Institutes of Health, U. S. Public Health Service.Requests for reprints should be addressed to I. Herbert Scheinberg, M.D., Albert Einstein College of Medicine, 1300 Morris Park Avenue, New York 61, N. Y. 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