de Jong J G, Heijs W M, Wevers R A
Laboratory of Pediatrics and Neurology, University Hospital Nijmegen, The Netherlands.
Ann Clin Biochem. 1994 May;31 ( Pt 3):267-71. doi: 10.1177/000456329403100309.
The dimethylmethylene blue (DMB)-based screening procedure for mucopolysaccharidosis and the alcian blue (AB)-based procedure both measure glycosaminoglycan (GAG) content directly in urine. We compared the two procedures. Absorbance per microgram of GAG for DMB was 25 times that obtained with AB, resulting in a requirement for 10 times smaller sample volumes for the former. Recoveries of added heparan sulphate for the AB assay in the absence and presence of added protein (2.5 g/L) were comparable (78-95% and 75-111%), as was the case with the DMB assay. Here, recoveries were generally better (94-103% and 96-100%). The coefficient of correlation (CC) between the two methods measured on 86 reference urine samples was 0.861. For mucopolysaccharidosis urines the CC was higher (0.928). Age-dependent reference values were comparable. Performance of the assays was compared by measurement of GAG content in 24 mucopolysaccharidosis urines. Standard deviation scores (measured value minus mean of reference values divided by standard deviation in reference values) and probabilities for classification in the patient group were calculated and compared. Especially for urines from patients with less distinctly increased GAG content (Sanfilippo, Scheie), scores and probabilities were higher with the DMB assay.
基于二甲基亚甲基蓝（DMB）的黏多糖贮积症筛查程序和基于阿尔新蓝（AB）的程序都可直接测量尿液中的糖胺聚糖（GAG）含量。我们对这两种程序进行了比较。DMB每微克GAG的吸光度是AB的25倍，这使得前者所需的样本体积小10倍。在不存在和存在添加蛋白（2.5 g/L）的情况下，AB法对添加硫酸乙酰肝素的回收率相当（78 - 95%和75 - 111%），DMB法也是如此。在此，回收率总体上更好（94 - 103%和96 - 100%）。在86份参考尿液样本上测量的两种方法之间的相关系数（CC）为0.861。对于黏多糖贮积症尿液，CC更高（0.928）。年龄相关的参考值相当。通过测量24份黏多糖贮积症尿液中的GAG含量来比较这两种检测方法的性能。计算并比较了标准差分数（测量值减去参考值的均值再除以参考值的标准差）以及患者组中分类的概率。特别是对于GAG含量增加不太明显的患者（Sanfilippo、Scheie）的尿液，DMB法的分数和概率更高。
