" /> Essential pentosuria - CISMeF





Preferred Label : Essential pentosuria;

ICD-11 definition : Pentosuria is an inborn error of metabolism which is characterized by the excretion of 1 to 4 g of the pentose L-xylulose in the urine per day. Pentosuria is benign and shows no symptoms.;

ICD-11 synonym : L-xylulosuria; L-xylulose reductase deficiency; Xylitol dehydrogenase deficiency;

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Pentosuria is an inborn error of metabolism which is characterized by the excretion of 1 to 4 g of the pentose L-xylulose in the urine per day. Pentosuria is benign and shows no symptoms.

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28/07/2025


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