title: Validation of Urinary Glycosaminoglycans in Iranian patients with Mucopolysaccharidase type I: The effect of urine sedimentation characteristics.
abstract: OBJECTIVE
  The first line-screening test for mucopolysaccharidosis is based on measurement of urinary glycosaminoglycans. The most reliable test for measurement of urine glycosaminoglycans is the 1,9-dimethyleneblue colorimetric assay. Biological markers are affected by ethnical factors, for this reason, the World Health Organization recommends that the diagnostic test characteristics should be used to determine results for different populations. This study determines the diagnostic value of 1,9-dimethyleneblue tests for diagnosis of mucopolysaccharidosis type I patients in Iran.
  
  
  METHODS
  In addition to routine urine analysis, the qualitative and quantitative measurements of urine glucosaminoglycans were performed with the Berry spot test and 1,9-dimethyleneblue assay. Diagnostic values of the tests were determined using the ROC curve.
  
  
  RESULTS
  Urine total glycosaminoglycans were significantly higher in male subjects than in female subjects. Glycosaminoglycan concentration was markedly decreased in specimens with elevated white blood cell and epithelial cells count. Using a cut-off level of 10.37 mg/g creatinine, sensitivity, and specificity were 100% and 97.22%, respectively, for a 1,9-dimethyleneblue colorimetric assay.
  
  
  CONCLUSIONS
  Urine glycosaminoglycans concentration significantly differs in our studied population. In addition to determine diagnostic validity of the 1,9-dimethyleneblue test, our results demonstrate the usefulness of measuring glycosaminoglycans for early screening of mucopolysaccharidosis type I Iran.
authors: .Department of Clinical Biochemistry, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran.
authors: Mohammad
authors: M
authors: Abdi
authors: .Department of Biochemistry and Nutrition, Faculty of Medicine, Kurdistan University of Medical Sciences, Sanandaj, Iran.
authors: Mohammad Said
authors: MS
authors: Hakhamaneshi
authors: Department of Pediatric, Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors: Mohammad Reza
authors: MR
authors: Alaei
authors: .Department of Epidemiology and Biostatistics, Faculty of Medicine, Kurdistan University of Medical Sciences, Sanandaj, Iran.
authors: Namam-Ali
authors: NA
authors: Azadi
authors: Department of Pediatrics, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
authors: Rahim
authors: R
authors: Vakili
authors: Department of Pediatric, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.
authors: Daniel
authors: D
authors: Zamanfar
authors: .Department of Clinical Biochemistry, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran.
authors: Mohammad
authors: M
authors: Taghikhani
authors: Department of Biochemistry, Pasteur Institute of Iran, Tehran, Iran.
authors: Shohreh
authors: S
authors: Khatami
keywords: Berry spot test; Diagnostic value; Dimethyl methylene blue test; Glycosaminoglycans; Iran; Mucopolysaccharidosis type I
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