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Clinical Chemistry 37: 572-575, 1991;
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Clinical Chemistry, Vol 37, 572-575, Copyright © 1991 by American Association for Clinical Chemistry

The spot test is not a reliable screening procedure for mucopolysaccharidoses

JG de Jong, JJ Hasselman, AA van Landeghem, HL Vader and RA Wevers
Institute of Neurology, University of Nijmegen, St. Radboud Hospital, The Netherlands.

To check the reliability of the Ames MPS paper spot test, which is based on the Azure A dye, we sent a series of urine samples to three laboratories where the spot test is part of the metabolic screening for mucopolysaccharidoses. In these laboratories false-negative results ranged between 19% and 35% and false-positive results ranged between 12% and 29% of all samples submitted. In contrast, the quantitative dimethylmethylene blue test (Clin Chem 1989;35:1472-7) detected an increased glycosaminoglycan content in all urine samples from mucopolysaccharidosis patients and gave no false-positive results. In the latter procedure, glycosaminoglycan content is expressed per millimole of creatinine, and age-dependent reference values are used. We conclude that the Ames spot test and other spot tests are unreliable as a screening procedure for mucopolysaccharidoses and should not be used to screen for these diseases.




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Copyright © 1991 by the American Association for Clinical Chemistry.