Pentosuria Explained

Pentosuria

Pentosuria is a condition where the sugar xylitol,[1] a pentose, presents in the urine in unusually high concentrations. It was characterized as an inborn error of carbohydrate metabolism in 1908.[2] It is associated with a deficiency of L-xylulose reductase, necessary for xylitol metabolism.[3] L-Xylulose is a reducing sugar, so it may give false diagnosis of diabetes, as it is found in high concentrations in urine. However glucose metabolism is normal in people with pentosuria, and they are not diabetic.[4] Patients of pentosuria have a low concentration of the sugar d-xyloketose.[5] Using phenyl pentosazone crystals, phloroglucin reaction, and absorption spectrum, pentose can be traced back as the reducing substance in urine, with those that have pentosuria.[6]

Research has shown that pentosuria appears in 3 forms. The most widely studied is essential pentosuria, where a couple of grams of L-xylusol are released into a person's system daily.[7] L-xylulose reductase, contained in red blood cells, is composed of both a major and minor isozyme.[8] For those diagnosed with essential pentosuria, the major isozyme appears to be the same as the minor one. Alimentary pentosuria can be acquired through fruits high in pentose. Finally, drug-induced pentosuria can be developed by those exposed to morphine, fevers, allergies, and some hormones.

Those diagnosed with Pentosuria are predominantly of Jewish root. However, it is a harmless defect, and no cure is needed.[9]

Notes and References

  1. Encyclopedia: pentosuria. Encyclopædia Britannica. March 26, 2013.
  2. Scriver CR . Garrod's Croonian Lectures (1908) and the charter 'Inborn Errors of Metabolism': albinism, alkaptonuria, cystinuria, and pentosuria at age 100 in 2008 . J. Inherit. Metab. Dis. . 31 . 5 . 580–98 . October 2008 . 18850300 . 10.1007/s10545-008-0984-9 . 10154482 .
  3. Book: Julia A. McMillan. Ralph D. Feigin. Catherine DeAngelis. M. Douglas Jones. Oski's pediatrics: principles & practice. 5 January 2011. 1 April 2006. Lippincott Williams & Wilkins. 978-0-7817-3894-1. 1–.
  4. Knox. W. Eugene. Sir Archibald Garrod's "Inborn Errors of Metabolism" IV.Pentosuria. The American Journal of Human Genetics. December 1958. 10. 4. 385–97. 13606116. 1931884.
  5. Greenwald. Isidor. The Nature of the Sugar in Four Cases of Pentosuria. The Journal of Biological Chemistry. 17 May 1930. 88. 1,2. 10.1016/S0021-9258(18)76792-6. free.
  6. Stookey. LB. Pentosuria. Experimental Biology and Medicine. 1 May 1909. 6. 5. 135–136. 10.3181/00379727-6-72. 209361414.
  7. B.Tower. Donald . Edmund L. Peters . Milton A. Pogorelskin. Nature and Significance of Pentosuria in Neuromuscular Disease. Neurology. January 1956. 6. 137. 37–49. 13280026. 10.1212/wnl.6.1.37. 24664586 .
  8. Lane. A.B.. On the Nature of L-Xylulose Reductase Deficiency in Essential Pentosuria. Biochemical Genetics. February 1984. 23. 1–2. 61–72. 3994659. 10.1007/bf00499113. 23655195.
  9. Oscar Touster. Touster. Oscar. Pentose Metabolism and Pentosuria. The American Journal of Medicine. May 1959. 26. 5. 13649698. 10.1016/0002-9343(59)90231-1. 724–39.

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