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Clinical Chemistry 18: 266-269, 1972;
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Clinical Chemistry, Vol 18, 266-269, Copyright © 1972 by the American Association for Clinical Chemistry

Test for Mucopolysaccharidoses: Simple Method for Quantitative Estimation of Urinary Glycosaminoglycans

Alberto Calatroni 1

1 Centro per lo Studio delle Mucopolisaccaridosi, Istituto di Chimica Biologica della Facoltà di Scienze, Università di Pavia, 27100 Pavia, Italy.

A simple method is described for measurement of urinary glycosaminoglycans (GAG) in normal and pathological urine: a fixed fraction of the 24-h urine is passed over a small plug of ECTEOLA-modified cellulose and the GAG are eluted with a few milliliters of NaCl solution (3 mol/liter) and measured as hexuronic acid. Because results are compared for equal aliquots of complete 24-h urines, GAG are quickly detected, thus providing means for a chemical diagnosis of mucopolysaccharidosis. The procedure requires less than 1 h, and several tests may be conveniently run simultaneously. When urine specimens collected from normal children or children affected by mucopolysaccharidosis were analyzed, the results obtained with the proposed procedure agree well with those of more elaborate, widely accepted methods.


Key Words: ECTEOLA-cellulose • glucuronic acid • colorimetry

Submitted on December 6, 1971
Accepted on December 10, 1971






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Copyright © 1972 by the American Association for Clinical Chemistry.