GAUCHER'S DISEASE IN 29 CASES: HEMATOLOGIC COMPLICATIONS AND EFFECT OF SPLENECTOMY
Authors: A S MEDOFF, E D BAYRD
Journal: Annals of Internal Medicine (1954)

Article1 March 1954GAUCHER'S DISEASE IN 29 CASES: HEMATOLOGIC COMPLICATIONS AND EFFECT OF SPLENECTOMYALAN S. MEDOFF, M.D., EDWIN D. BAYRD, M.D.ALAN S. MEDOFF, M.D.Search for more papers by this author, EDWIN D. BAYRD, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-40-3-481 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptGaucher's disease is a rare, heredofamilial disorder of cerebroside metabolism characterized clinically in the adult by an insidious onset, splenomegaly, pigmentation, pingueculae, a protracted course, and usually some hematologic abnormality. In an effort to determine the incidence and nature of these hematologic variations and, in addition, to study the effects of splenectomy on patients having this disease, we have reviewed all proved cases of Gaucher's disease seen at the Mayo Clinic through the year 1950. Twenty-nine such cases were found (tables 1 and 2). Since the majority of clinical reports dealing with this disease contain only small numbers of cases,...Bibliography1. Atkinson FR: Gaucher's disease in children, Brit. J. Child. Dis. 35: 1-22 (Jan.-Mar.) 1938. Google Scholar2. Logan VW: The results of splenectomy in Gaucher's disease, Surg., Gynec. and Obst. 72: 807-814 (Apr.) 1941. Google Scholar3. Morgans ME: Gaucher's disease without splenomegaly, Lancet 2: 576-578 (Oct. 18) 1947. CrossrefMedlineGoogle Scholar4. Gordon GL: Osseous Gaucher's disease; report of two cases in siblings, Am. J. Med. 8: 332-341 (Mar.) 1950. CrossrefMedlineGoogle Scholar5. Thannhauser SJ: Lipidoses; diseases of the cellular lipid metabolism, 2nd Ed., 1950, Oxford University Press, New York. Google Scholar6. BloemGroenPostma TFJC: Gaucher's disease, Quart. J. Med. 5: 517-527 (Oct.) 1936. Google Scholar7. Pick L: I. A classification of the diseases of lipoid metabolism and Gaucher's disease, Am. J. M. Sc. 185: 453-469 (Apr.) 1933. CrossrefGoogle Scholar8. ReichSeifeKessler CMBJ: Gaucher's disease: a review, and discussion of twenty cases, Medicine 30: 1-20 (Feb.) 1951. CrossrefMedlineGoogle Scholar9. HoffmanMakler SJMI: Gaucher's disease; a review of the literature and report of a case diagnosed from section of an inguinal lymph gland, Am. J. Dis. Child. 38: 775-793 (Oct.) 1929. CrossrefGoogle Scholar10. CarlingCarlillPulvertaft ERHRJ: Splenectomy in Gaucher's disease with haemoglobinuria, Proc. Roy. Soc. Med. 26 (pt. 1): 361 (Feb.) 1932. Google Scholar11. Donovan EJ: Splenectomy in a child eleven months of age for Gaucher's disease, S. Clin. North America 11: 517-520 (June) 1931. Google Scholar12. ErdmannMoorhead JFJJ: Splenectomy for splenomegaly (Gaucher type), Am. J. M. Sc. 147: 213-224 (Feb.) 1914. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Rochester, Minnesota*Received for publication August 10, 1953.From the Division of Medicine of the Mayo Clinic, Rochester, Minnesota. Nextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byClinical and radiological evaluation of dentomaxillofacial involvement in Type I Gaucher diseaseNovel Management and Screening Approaches for Haematological Complications of Gaucher’s DiseaseCauses of death in 184 patients with type 1 Gaucher disease from the United States who were never treated with enzyme replacement therapyGaucher Disease2 Gaucher Disease11This is manuscript 8587-MEM from The Scripps Research Institute. Supported by National Institutes of Health Grants DK36639 and RR00833 and the Sam Stein and Rose Stein Charitable Trust Fund.A 27-Year experience with splenectomy for Gaucher's diseaseGaucher diseaseGenetic Heterogeneity of Gaucher Disease: Enzymatic and Immunologic StudiesGenetic heterogeneity in Gaucher disease: Physicokinetic and immunologic studies of the residual enzyme in cultured fibroblasts from non-neuronopathic and neuronopathic patientsSplenectomy for splenomegaly and secondary hypersplenismHematological findings in the Norrbottnian type of Gaucher diseaseSevere pulmonary involvement in adult Gaucher's diseaseDisorders of Mononuclear Phagocyte Proliferation, Maturation and FunctionGaucher's diseaseThrombocytopenia in Gaucher's DiseaseDAVID GREEN, M.D., HECTOR A. BATTIFORA, M.D., ROY T. SMITH, M.D., ENNIO C. ROSSI, M.D.Splenectomy for hematologic disordersAsymptomatic SplenomegalySpeicherkrankheitenLungenveränderungen bei StoffwechselerkrankungenGaucher’s DiseaseDyslipidosesThrombocytopeniaThe Genetic Mechanism of Gaucher's Disease,Gaucher's DiseaseObservations on a patient with Gaucher's diseaseTHE OCCURRENCE OF INTRACELLULAR IRON IN GAUCHER'S DISEASE*†MORTIMER LORBER, M.D.Gaucher's disease in pregnancyGAUCHER'S DISEASE IN IDENTICAL TWINS*RICHARD I. CRONE, JAMES J. BERGINDie SphingolipoidosenLiteraturGAUCHER'S DISEASE AND PREGNANCY: TWO CASE REPORTS, INCLUDING OBSERVATIONS ON THE EFFECTS OF ADRENAL STEROIDS*BARRY DECKER, C. A. MCWHORTER, M.D.Current pediatric references 1 March 1954Volume 40, Issue 3Page: 481-492KeywordsSplenectomySplenomegaly ePublished: 1 December 2008 Issue Published: 1 March 1954 Copyright & PermissionsCopyright, 1954, by The American College of PhysiciansPDF downloadLoading ...