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【目的】建立一种快速、简便、可靠的尿粘多糖定量检测方法,用于临床筛查粘多糖病(mucopolysacchari-dosis,MPS)患者。【方法】采用1,9-二甲基亚甲蓝(1,9-dimethylmethylene blue,DMB)在碱性条件下与尿中粘多糖(glycosaminoglycan,GAG)反应生成粉红色产物及分光光度计比色原理,检测267例正常人、12例MPS患儿及30例非MPS患儿的尿GAG含量,并同时检测尿肌酐(creatinine,Cr),计算GAG/Cr比值。【结果】正常人尿GAG水平常低于70 mg/L,且与年龄无显著相关。而GAG/Cr比值随年龄的递增呈递减趋势。除1例IVA型MPS患者外,其余11例MPS患儿尿GAG浓度及GAG/Cr比值均显著高于同龄正常儿童参考值,而非MPS患儿的尿GAG及GAG/Cr比值均在正常范围内。GAG测定的批内变异系数为1.3%,批间变异系数为3.6%,硫酸类肝素的回收率为(102±6.0)%。【结论】DMB-Tris比色法测定尿GAG是一种快速、简便、标本用量少、特异性和敏感性均较高的定量检测方法,可作为MPS的筛查方法在临床上推广应用。
【Objective】 To establish a rapid, simple and reliable method for the quantitative determination of urine mucopolysaccharide for the clinical screening of patients with mucopolysacchari-dosis (MPS). 【Method】 1,9-dimethylmethylene blue (DMB) was used to react with glycosaminoglycan (GAG) in urine to produce pink products and spectrophotometer colorimetric The urinary GAG levels in 267 normal subjects, 12 MPS children and 30 non-MPS children were detected. Urinary creatinine (Cr) was measured at the same time. The GAG / Cr ratio was calculated. 【Results】 The urinary GAG level of normal people is often lower than 70 mg / L, and there is no significant correlation with age. The GAG / Cr ratio decreased with increasing age. Except for 1 patient with type IVA MPS, urinary GAG concentration and GAG / Cr ratio in 11 other MPS children were significantly higher than those of normal children of the same age, while urinary GAG and GAG / Cr ratios of non-MPS children were in the normal range Inside. The intra-assay CV of GAG was 1.3%, the inter-assay CV was 3.6%, and the recovery of HSH was (102 ± 6.0)%. 【Conclusion】 DMB-Tris colorimetry is a fast and simple method for quantitative determination of urinary GAG with low dosage, high specificity and high sensitivity. It can be used clinically as a screening method for MPS.