The Nephrotic Syndrome as a Complication of Penicillamine Therapy for Hepatolenticular Degeneration (Wilson's Disease)
Authors: SHALOM Z. HIRSCHMAN
Journal: Annals of Internal Medicine (1965)

Case Studies1 June 1965The Nephrotic Syndrome as a Complication of Penicillamine Therapy for Hepatolenticular Degeneration (Wilson's Disease)SHALOM Z. HIRSCHMAN, M.D., KURT J. ISSELBACHER, M.D.SHALOM Z. HIRSCHMAN, M.D., KURT J. ISSELBACHER, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-62-6-1297 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptSince the introduction of penicillamine (β,β dimethylcysteine) as a copper chelating agent by Walshe (1) in 1956, this drug has been used extensively in the therapy of Wilson's disease (2-9) to increase cupruria and diminish body copper deposits. To a large extent penicillamine has replaced dimercaprol (BAL) in the treatment of this disorder, since it can be administered orally. However, allergic reactions to penicillamine are common. These include skin rashes (usually a maculopapular eruption, but at times a nonerythematous desquamation of the skin of the face and trunk), fever, lymphadenopathy, and, rarely, leukopenia and thrombocytopenia (6). Cessation of penicillamine therapy...References1. WALSHE JM: Penicillamine, a new oral therapy for Wilson's disease. Amer. J. Med. 21: 487, 1956. CrossrefMedlineGoogle Scholar2. WALSHE JM: Hepatolenticular degeneration (Wilson's disease). Brit. Med. Bull. 13: 132, 1957. CrossrefMedlineGoogle Scholar3. BEARN AG: Wilson's disease. An inborn error of metabolism with multiple manifestations. Amer. J. Med. 22: 747, 1957. CrossrefMedlineGoogle Scholar4. WARNOCKNEILL CGDW: Diagnosis and treatment of Wilson's disease. Brain 81: 258, 1958. CrossrefMedlineGoogle Scholar5. FISTERBOULDINGBAKER WPJERA: The treatment of hepatolenticular degeneration with penicillamine with report of two cases. Canad. Med. Ass. J. 78: 99, 1958. MedlineGoogle Scholar6. SCHEINBERGSTERNLIEB IHI: The long term management of hepatolenticular degeneration (Wilson's disease). Amer. J. Med. 29: 316, 1960. CrossrefMedlineGoogle Scholar7. BOULDING JE: Treatment of Wilson's disease with D-penicillamine, in Wilson's Disease, Some Current Concepts, Symposium on Wilson's disease, London, 1960, edited by WALSHE, J. M., and CUMMINGS, J. N., Blackwell, Oxford, 1961, p. 224-226. Google Scholar8. GARLANDSUMNER HD: The treatment of Wilson's disease with penicillamine. Ibid., p. 227-232. Google Scholar9. DENNY-BROWN D: Hepatolenticular degeneration (Wilson's disease), two different components. New Eng. J. Med. 270: 1149, 1964. CrossrefMedlineGoogle Scholar10. FELLERSSHAHIDI FXNT: The nephrotic syndrome induced by penicillamine therapy (abstract). Amer. J. Dis. Child. 98: 669, 1959. Google Scholar11. ADAMSGOLDMANMAXWELLLATTA ADRMHH: Nephrotic syndrome associated with penicillamine therapy of Wilson's disease. Amer. J. Med. 36: 330, 1964. CrossrefMedlineGoogle Scholar12. STERNLIEBSCHEINBERG IIH: Penicillamine therapy for hepatolenticular degeneration. JAMA 189: 748, 1964. CrossrefMedlineGoogle Scholar13. SPIRO D: Electron microscopic studies of renal biopsies on patients with proteinuria, in Proceedings of the Eleventh Annual Conference on the Nephrotic Syndrome, New York (1959), edited by METCOFF, J., National Kidney Disease Foundation, New York, 1959, p. 171-221. Google Scholar14. BEARNYÜGUTMAN AGTFAB: Renal functions in Wilson's disease. J. Clin. Invest. 36: 1107, 1957. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Boston, MassachusettsFrom the Department of Medicine, Harvard Medical School, and the Medical Service (Gastrointestinal Unit), Massachusetts General Hospital, Boston, Mass.This study was supported by grant AM-01392 from the National Institutes of Health, U. S. Public Health Service, Bethesda, Md.Requests for reprints should be addressed to Kurt J. Isselbacher, M.D., Massachusetts General Hospital, Fruit Street, Boston, Mass. 02114. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByWilson's diseasePenicillamine and nephrotic syndromeInteractions of Zinc with Other MicronutrientsAcquired sideroblastic anaemia during treatment of Wilson's disease with triethylene tetramine dihydrochlorideOral Zinc Therapy for Wilson's DiseaseGEORGE J. BREWER, M.D., GRETCHEN M. HILL, Ph.D., ANANDA S. PRASAD, M.D., Ph.D., ZAFRALLAH T. COSSACK, Ph.D., PARVIZ RABBANI, Ph.D.Evaluation of the Toxicity of Anti-Inflammatory DrugsPharmacology and therapeutic applications of agents used in heavy metal poisoningWilson's DiseaseToxic Disturbances of Renal FunctionsCystinuriaExcretion of Trace Elements in Two Penicillamine-treated Cases of Cystinuria 1 June 1965Volume 62, Issue 6Page: 1297-1300KeywordsDesquamationDrug therapyEdemaFeversHospital medicineHyperlipidemiaLesionsMedical servicesPrevention, policy, and public healthRashes Issue Published: 1 June 1965 PDF DownloadLoading ...