Elevated Dermatan and Heparan Sulfate
High concentrations of specific mucopolysaccharides, namely dermatan and heparan sulfate, in the urine, indicating an alpha-L-iduronidase deficiency.
A urine sample is tested using a dye-binding assay or chromatography to quantify the amount and type of glycosaminoglycans being excreted. High levels indicate a breakdown defect in the lysosomes.
High concentrations of specific mucopolysaccharides, namely dermatan and heparan sulfate, in the urine, indicating an alpha-L-iduronidase deficiency.