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Pentosuria

ORPHA:2843· ICD-10 E74.8

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.

Prevalence
Unknown
Inheritance
Autosomal recessive
Age of onset
Infancy, Neonatal