A RAPID METHOD FOR DETERMINATION OF URINARY KERATAN SULFATE IN THE HEREDITARY MUCOPOLY-SACCHARIDOSIS IV (MORQUIO SYNDROME)

Abstract

A rapid method is described for the chemical determination of keratan sulfate in patient's urine with mucopolysaccharidosis IV (Morquio syndrome). The method of estimation of the glycosaminoglycan relies upon its resistance to enzymic degradation, and involves cellulose acetate-strip electrophoresis. This quantitative method can be carried out in a few days and provides means of a chemical diagnosis of mucopolysaccharidosis IV (Morquio syndrome) in the clinical laboratory. Keratan sulfate/heparan sulfate ratio was 15.8 in this type, while it was 1.5±0.67 in normal, 0.3 in mucopolysaccharidosis I and 0.4 in mucopolysaccharidosis II, respectively.