HOWARD H. HIATT, M.D.
Pentosuria, or chronic essential pentosuria, is one of four hereditary disturbances which Garrod1 over 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 of L-xylulose, varying from 1 to 4 gm. per 24 hours,2 unrelated to dietary factors, distinguishes hereditary pentosuria from several other situations in which milligram quantities of certain 5-carbon sugars other than L-xylulose are found in the urine. These include alimentary pentosuria, in which arabinose or xylose may be excreted following
HIATT HH. CARBOHYDRATE METABOLISM IN PENTOSURIA1. Ann Intern Med. 1960;53:372–379. doi: 10.7326/0003-4819-53-2-372
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Published: Ann Intern Med. 1960;53(2):372-379.
Hospital Medicine, Nephrology.
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