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Iminoglycinuria

ORPHA:42062· ICD-10 E72.0

Definition

A rare inborn error of metabolism characterized by elevated levels of imino acids (proline, hydroxyproline) and glycine in urine due to defective reabsorption in the kidney. The condition is considered benign and not associated with any specific clinical phenotype. Mode of inheritance is autosomal recessive.

Prevalence
1-9 / 100 000
Inheritance
Autosomal recessive
Age of onset
All ages