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Arch Intern Med (Chic). 1943;72(1):31-36. doi:10.1001/archinte.1943.00210070039003.
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There has been no evidence to show that essential pentosuria is related in any way to diabetes mellitus, and no complete report of their combined occurrence can be found in the literature.1 In the first case presented here the patient was suffering from both essential pentosuria and diabetes mellitus. By way of comparison a case of uncomplicated essential pentosuria is also reported.

Essential pentosuria is a hereditary abnormality of metabolism characterized by continuous excretion of small amounts of pentose in the urine, unaffected by alterations in the diet. The first case of this disorder was reported by Salkowski and Jastrowitz2 in 1892. Since then reports of more than 170 cases have appeared in the literature. The incidence of this disorder in the general population is estimated by Blatherwick3 to be 1:50,000.

The pentose excreted has been found in most instances to be levorotatory xylulose, formerly called l


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