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Validation of Urinary Glycosaminoglycans in Iranian patients with Mucopolysaccharidase type I: The effect of urine sedimentation characteristics.

Abdi M, Hakhamaneshi MS, Alaei MR, Azadi NA, Vakili R, Zamanfar D, Taghikhani M, Khatami S - Iran J Child Neurol (2014)

Bottom Line: 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.Urine glycosaminoglycans concentration significantly differs in our studied population.

View Article: PubMed Central - PubMed

Affiliation: .Department of Clinical Biochemistry, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran.

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.

Materials & 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.

Conclusion: 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.

No MeSH data available.


Related in: MedlinePlus

ROC curves for the DMB test. DMB = 1,9 Dimethylane blue; AUC = area under the curve; ROC = receiver operating characteristic
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Figure 2: ROC curves for the DMB test. DMB = 1,9 Dimethylane blue; AUC = area under the curve; ROC = receiver operating characteristic

Mentions: The sensitivity and specificity of the Berry spot test was 83.33% and 88.88%, respectively. Table 2 shows the other diagnostic utility for this test. The means and standard deviations of urinary GAGs for MPSI and control groups were 12.54 ± 2.14 mg/g creatinine and 4.67 ± 3.29 mg/g creatinine, respectively. Using a cut-off level of 10.73 mg/g creatinine for urinary GAGs, sensitivity, and specificity were 100% and 97.22%, respectively. The area under the curve for GAGs by Analysis of ROC curves (Figure 2) of the DMB test was 0.99, 95% CI=0.973-1. The positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio, and negative likelihood ratio of the tests were also determined. Table 2 shows the abovementioned values for urine samples of the studied subjects. The highest (97.22%) specificity and highest (0.75) PPV could be obtained by quantitative measurements of GAGs. Within run and between run CVs were obtained for the DMB test was 2% and 7%, respectively.


Validation of Urinary Glycosaminoglycans in Iranian patients with Mucopolysaccharidase type I: The effect of urine sedimentation characteristics.

Abdi M, Hakhamaneshi MS, Alaei MR, Azadi NA, Vakili R, Zamanfar D, Taghikhani M, Khatami S - Iran J Child Neurol (2014)

ROC curves for the DMB test. DMB = 1,9 Dimethylane blue; AUC = area under the curve; ROC = receiver operating characteristic
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4307367&req=5

Figure 2: ROC curves for the DMB test. DMB = 1,9 Dimethylane blue; AUC = area under the curve; ROC = receiver operating characteristic
Mentions: The sensitivity and specificity of the Berry spot test was 83.33% and 88.88%, respectively. Table 2 shows the other diagnostic utility for this test. The means and standard deviations of urinary GAGs for MPSI and control groups were 12.54 ± 2.14 mg/g creatinine and 4.67 ± 3.29 mg/g creatinine, respectively. Using a cut-off level of 10.73 mg/g creatinine for urinary GAGs, sensitivity, and specificity were 100% and 97.22%, respectively. The area under the curve for GAGs by Analysis of ROC curves (Figure 2) of the DMB test was 0.99, 95% CI=0.973-1. The positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio, and negative likelihood ratio of the tests were also determined. Table 2 shows the abovementioned values for urine samples of the studied subjects. The highest (97.22%) specificity and highest (0.75) PPV could be obtained by quantitative measurements of GAGs. Within run and between run CVs were obtained for the DMB test was 2% and 7%, respectively.

Bottom Line: 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.Urine glycosaminoglycans concentration significantly differs in our studied population.

View Article: PubMed Central - PubMed

Affiliation: .Department of Clinical Biochemistry, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran.

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.

Materials & 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.

Conclusion: 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.

No MeSH data available.


Related in: MedlinePlus