THE SUCCESSFUL MANAGEMENT OF HEPATOLENTICULAR DEGENERATION WITH PENICILLAMINE: STUDIES ON THREE GENERATIONS OF A FAMILY
Authors: Jack B. Strong, Hugh Dempsey, S. Richardson Hill
Journal: Annals of Internal Medicine (1961)

Article1 February 1961THE SUCCESSFUL MANAGEMENT OF HEPATOLENTICULAR DEGENERATION WITH PENICILLAMINE: STUDIES ON THREE GENERATIONS OF A FAMILYJACK B. STRONG JR., HUGH DEMPSEY, M.D., S. RICHARDSON HILL JR., M.D.JACK B. STRONG JR.Search for more papers by this author, HUGH DEMPSEY, M.D.Search for more papers by this author, S. RICHARDSON HILL JR., M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-54-2-198 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptINTRODUCTIONHepatolenticular degeneration (HLD, Wilson's disease)1is associated with abnormal deposition of copper in the brain, liver, kidneys, and other body tissues,2apparently due to an inherited defect in copper metabolism.3Clinically the disorder is manifested by signs and symptoms of basal ganglia disease, postnecrotic hepatic cirrhosis,4Kayser-Fleischer rings,5hypoceruloplasminemia, hypocupremia, hypouricemia, cupruresis, and aminoaciduria.6In light of our present state of knowledge concerning the pathogenesis of this disease, rational therapy should aim at producing a negative copper balance. This may be done both by decreasing copper intake and absorption, and by removing the excess copper deposition in tissue before...Bibliography1. Wilson SA: Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 34: 295, 1912. CrossrefGoogle Scholar2. CartwrightHodgesGublerMahoneyDaumWintrobeBean GERECJJPKMMWB: Studies on copper metabolism. XIII. Hepatolenticular degeneration. J. Clin. Invest. 33: 1487, 1954. CrossrefMedlineGoogle Scholar3. Bearn AG: Genetic and biochemical aspects of Wilson's disease. Amer. J. Med. 15: 442, 1953. CrossrefMedlineGoogle Scholar4. AndersonPopper PJH: Changes in hepatic structure in Wilson's disease. Amer. J. Path. 36: 483, 1960. MedlineGoogle Scholar5. WarnockNeill CGDW: The diagnosis and treatment of Wilson's disease. Brain 81: 258, 1958. CrossrefMedlineGoogle Scholar6. Bearn AG in The metabolic basis of inherited disease, pp. 809-838. The McGraw-Hill Book Company, Inc., New York, 1960. 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NealeFischer-Williams FCM: Copper metabolism in normal adults and in clinically normal relatives of patients with Wilson's disease. J. Clin. Path. 11: 441, 1958. CrossrefMedlineGoogle Scholar21. CartwrightMarkowitzShieldsWintrobe GEHGSMM: Studies on copper metabolism. XXIX. A critical analysis of serum copper and ceruloplasmin concentrations in normal subjects, patients with Wilson's disease and relatives of patients with Wilson's disease. Amer. J. Med. 28: 555, 1960. CrossrefMedlineGoogle Scholar22. Walshe JM: Current views on the pathogenesis and treatment of Wilson's disease. Arch. Intern. Med. 103: 155, 1959. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Birmingham, Alabama*Received for publication June 25, 1960.From the Division of Metabolism and Endocrinology, the Department of Medicine and the University Hospital, University of Alabama Medical Center, and the Medical Service, Veterans Administration Hospital, Birmingham, Alabama.†This study was aided in part by Grants 2A-5053, A-1265, and A-3555 from the National Institute of Arthritis and Metabolic Diseases of the National Institutes of Health, Bethesda, Md., and by funds from the Upjohn Company, Kalamazoo, Mich.‡Student Research Fellow in Metabolism, National Institutes of Health.Requests for reprints should be addressed to S. Richardson Hill, Jr., M.D., Associate Professor of Medicine, The University of Alabama Medical Center, Medical College of Alabama, Birmingham 3, Alabama. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byWhat Wilson's Disease and Its Treatment have Taught us About the Metabolism of Copper: Observations in 27 CasesMetabolic balance studies in hepatolenticular degeneration treated with diethyldithiocarbamateMedical genetics 1961Wilson's Disease 1 February 1961Volume 54, Issue 2Page: 198-204KeywordsBrain diseasesEndocrinologyHospital medicineKidneysLiverLiver diseasesMedical servicesMetabolic disordersPathogenesisSigns and symptoms ePublished: 1 December 2008 Issue Published: 1 February 1961 PDF downloadLoading ...