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Déborah Mathis¹, Jean-Christophe Prost¹, Gabriela Maeder¹

¹University Institute of Clinical Chemistry, Inselspital, Bern University Hospital, University of Bern Bern Switzerland.

|March 6, 2024

概括

粘多糖症 (MPS) 查可以使用液态染色体质谱法 (LC-MS) 进行改进,以分析特定的葡萄糖氨基酸糖 (GAG). 这种方法提高了MPS类型的诊断准确性,并将其与尿路感染 (UTI) 区分开来.

科学领域:

生物化学生物化学
医学诊断医学诊断医学诊断
代谢障碍代谢障碍代谢障碍

背景情况:

目前的粘多糖症 (MPS) 查依赖于1,9-二甲基乙烯蓝色 (DMB) 光度测定总糖氨基 (GAG) 的测定,该测试存在不准确性,包括错误阳性和负性.
差异化GAG分析,传统上通过凝电泳和最近通过LC-MS,提供了更高的特异性.
在尿路感染 (UTI) 中观察到二次GAG升高,可能会混MPS诊断.

研究的目的:

为了评估尿液GAG测量的诊断准确性,LC-MS用于MPS类型.
为特定的GAG (CS,DS,HS,KS) 和它们的比率建立年龄相关的参考值和截止值.
评估尿路感染对GAG测量的影响及其错误分类的可能性.

主要方法:

在68名未经治疗的MPS/ML患者,183名对照患者和153名尿道感染样本中,对尿道GAG (ondroitin硫酸盐,dermatan硫酸盐,heparan硫酸盐,keratan硫酸盐) 的定量LC-MS分析.
计算特定的GAG比率 (例如HS/DS,KS/HS) 并将GAG度与肌素的正常化.
将LC-MS结果与DMB测定结果进行比较,并注意到假负结果.

主要成果:

HS/DS比率与肌素正常化GAG度相结合,提高了MPS类型I,II,VI和VII的诊断准确性,正确重新分类了15个样本.
增加的KS/HS比率和升高的KS水平增加了对MPS类型IV的歧视,不包括假阳性.
尿路检查样本显示高的特定GAG (主要是CS) 和KS/HS比率,造成错误分类为MPS类型IV的风险.
该DMB测定显示了94%的灵敏度,在MPS/ML患者中确定了四个虚假阴性.

结论:

特定的GAG比率,当与定量LC-MSGAG值一起使用时,显著改善了MPS类型的歧视.
排除尿路感染患者可以提高诊断准确度,特别是MPS类型IV,但对其他MPS类型的影响较小.
与传统的DMB测定相比,LC-MS为MPS类型提供了更准确的方法.

关键词:

这就是为什么GAG是GAG.在 LCMS 中使用.在MPS中,MPS是MPS.顺德里二硫酸盐顺德里二硫酸盐德马坦硫酸盐德马坦硫酸盐是一种诊断诊断诊断诊断诊断诊断双甲基乙烯蓝色染料结合 (DMB) 试验法葡萄糖氨基酸糖甘油葡萄糖甘油肝素硫酸盐是什么肝素硫酸盐是什么凯拉坦硫酸盐是一种酸盐.粘多糖糖症是什么粘多糖糖症是什么它们的比率.参考值是指指标值的参考值.尿路感染尿路感染

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