It was characterized as an inborn error of carbohydrate metabolism in 1908. It is associated with a deficiency of L-xylulose reductase, necessary for xylitol metabolism. L-Xylulose is a reducing sugar, so it may give false diagnosis of diabetes, as it is found in high concentrations in urine.
Characterized as an innate defect in carbohydrate metabolism dating back to 1908, this condition is linked to a deficiency of the enzyme necessary for xylitol processing. As L-Xylulose is a reducing sugar, it may cause false positives for diabetes due to its presence in high amounts in urine.