CARBOHYDRATE METABOLISM IN PENTOSURIA
Authors: H H HIATT
Journal: Annals of Internal Medicine (1960)

Article1 August 1960CARBOHYDRATE METABOLISM IN PENTOSURIAHOWARD H. HIATT, M.D.HOWARD H. HIATT, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-53-2-372 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptPentosuria, or chronic essential pentosuria, is one of four hereditary disturbances which Garrod1over 50 years ago classified as inborn errors of metabolism. It is an innocuous condition, presumably present from birth, and has been described almost exclusively in Jews. The characteristic urinary excretion of a relatively constant amount ofL-xylulose, varying from 1 to 4 gm. per 24 hours,2unrelated to dietary factors, distinguishes hereditary pentosuria from several other situations in which milligram quantities of certain 5-carbon sugars other thanL-xylulose are found in the urine. These include alimentary pentosuria, in which arabinose or xylose may be excreted following...Bibliography1. Garrod AE: Inborn errors of metabolism, Lancet 2: 217, 1908. Google Scholar2. EnklewitzLasker MM: The origin of L-xyloketose (urine pentose), J. Biol. Chem. 110: 443, 1935. CrossrefGoogle Scholar3. TowerPetersPogorelskin DBELMA: Nature and significance of pentosuria in neuromuscular disease, Neurology 6: 37, 125, 1956. CrossrefMedlineGoogle Scholar4. FuttermanRoe SJH: The identification of ribulose and L-xylulose in human and rat urine, J. Biol. Chem. 215: 257, 1955. CrossrefMedlineGoogle Scholar5. HoreckerHiatt BLHH: Pathways of carbohydrate metabolism in normal and neoplastic cells, New England J. Med. 258: 177, 225, 1958. CrossrefMedlineGoogle Scholar6. HoreckerMehler BLAH: Carbohydrate metabolism, Ann. Rev. Biochem. 24: 207, 1955. CrossrefMedlineGoogle Scholar7. Margolis JI: Chronic pentosuria and migraine, Am. J. M. Sc. 177: 348, 1929. CrossrefGoogle Scholar8. 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Author, Article, and Disclosure InformationAffiliations: Boston, Massachusetts*Received for publication April 4, 1960.Presented in part at the Forty-first Annual Session of The American College of Physicians, San Francisco, California, April 4, 1960.From the Department of Medicine, Harvard Medical School, and the Department of Medical Research, Beth Israel Hospital, Boston, Massachusetts.This work was supported by grants from the U. S. Public Health Service, the American Cancer Society, and the Jane Coffin Childs Memorial Fund for Medical Research.Requests for reprints should be addressed to Howard H. Hiatt, M.D., Beth Israel Hospital, 330 Brookline Avenue, Boston 15, Massachusetts. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byThe complex human urinary sugar profile: determinants revealed in the cross-sectional KarMeN studyDehydrogenases: Glucose-6-Phosphate Dehydrogenase, 6-Phosphogluconate Dehydrogenase, Glutathione Reductase, Methemoglobin Reductase, Polyol DehydrogenasesIncidence of Pentosuria 1 August 1960Volume 53, Issue 2Page: 372-379KeywordsCarbohydrate metabolismExcretionHealth services researchHospital medicineInborn errors of metabolismJewish peopleMedical servicesResearch fundingUrineXylose Issue Published: 1 August 1960 PDF downloadLoading ...