Literature DB >> 17964291

Stability of urinary glycosaminoglycans in patients with mucopolysaccharidoses.

Fernando Andrade1, José Angel Prieto, Javier Elorz, Sergio Martín, Pablo Sanjurjo, Luis Aldámiz-Echevarría.   

Abstract

OBJECTIVE: The aim of this work is to investigate the degradation of urinary glycosaminoglycans (GAGs) at different storage temperatures, in order to identify whether frozen transportation to reference laboratories is necessary. An improved method for the determination of total GAGs with 1,9-dimethylmethylene blue (DMB) is presented. DESIGN AND METHODS: Urine samples of 37 patients suffering from mucopolysaccharidoses (MPS) were analyzed in this study (13 Hunter, 6 Maroteaux-Lamy, 6 Morquio, 6 Sanfilippo, 5 Hurler-Scheie, and 1 Sly). Stability was assayed at room temperature, 5 degrees C and -30 degrees C, and analyses were repeated for at least 15 days. Spectrophotometric quantitation of GAGs with DMB was used for all determinations, using a variable wavelength for quantitation.
RESULTS: The concentration of urinary GAGs was stable for 10 days at room temperature, but it was found to be stable for more than 15 days at 5 degrees C and -30 degrees C.
CONCLUSIONS: The stability of GAGs allows urine samples to be sent for quantitation at a clinical laboratory without the need to freeze samples, as this would not affect results. This issue is important for the rapid detection of MPS at hospitals or primary health care centres, where GAGs determination is not performed.

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Year:  2007        PMID: 17964291     DOI: 10.1016/j.cca.2007.10.007

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN: 0009-8981            Impact factor:   3.786


  4 in total

1.  Reliability of a visual test for the rapid detection of mucopolysaccharidoses: GAG-test(®).

Authors:  Sergio Lage; José A Prieto; Fernando Andrade; Amaia Sojo; Pablo Sanjurjo; Luis J Aldámiz-Echevarría
Journal:  J Clin Lab Anal       Date:  2011       Impact factor: 2.352

2.  Study of inborn errors of metabolism in urine from patients with unexplained mental retardation.

Authors:  Angela Sempere; Angela Arias; Guillermo Farré; Judith García-Villoria; Pilar Rodríguez-Pombo; Lurdes R Desviat; Begoña Merinero; Angels García-Cazorla; Maria A Vilaseca; Antonia Ribes; Rafael Artuch; Jaume Campistol
Journal:  J Inherit Metab Dis       Date:  2010-01-05       Impact factor: 4.982

Review 3.  Mucopolysaccharidosis type III (Sanfilippo syndrome) and misdiagnosis of idiopathic developmental delay, attention deficit/hyperactivity disorder or autism spectrum disorder.

Authors:  Frits A Wijburg; Grzegorz Węgrzyn; Barbara K Burton; Anna Tylki-Szymańska
Journal:  Acta Paediatr       Date:  2013-02-06       Impact factor: 2.299

4.  Sanfilippo type A: new clinical manifestations and neuro-imaging findings in patients from the same family in Israel: a case report.

Authors:  Rajech Sharkia; Muhammad Mahajnah; Abdelnaser Zalan; Chrysovalantis Sourlis; Peter Bauer; Ludger Schöls
Journal:  J Med Case Rep       Date:  2014-02-28
  4 in total

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