Measurement of Urinary Glycosaminoglycans with Quaternary Ammonium Salts : An Extension of the Method

The method for the measurement of urinary glycosaminoglycans, based on their precipitation with quaternary ammonium salts, has been extended to include the measurement of low molecular weight, hexuronic acid-containing anionic compounds, tentatively considered to be degradation products of glycosaminoglycans.The ratio mg urinary glycosaminoglycans/mg 'degradation products', measured either on a 24-hour or on a sporadic urine specimen, has been found to be less than 1.0 in normal individuals and consistently greater than 1.0 in children affected by various mucopolysaccharide-storage diseases. From the results obtained, the method appears to be of diagnostic importance, especially when a complete 24-hour urine specimen may not be available.