肝豆状核变性患者尿肾小管损害相关标志物水平的改变及其意义

摘要

目的 探讨肝豆状核变性(HLD)患者尿肾小管损害相关标志物水平的改变及其意义.方法 检测196例HLD患者(HLD组,其中假性硬化型45例,脑内脏混合型41例,肝型58例,Wilson型52例)和47名正常对照者(正常对照组)尿常规；应用免疫比浊法检测尿肾小管损害相关标志物[包括N-乙酰-β-D氨基葡萄糖苷酶(NAG)、视黄醇结合蛋白(RBP)、α1-微球蛋白(α1 -MG)和β2-微球蛋白(β32-MG)]水平.结果 HLD组中,尿常规正常54例,异常142例；HLD组尿NAG、RBP、α1 -MG和β2-MG水平显著高于正常对照组(P ＜0.05 ～0.01)；HLD组各型间尿NAG、RBP、α1-MG和β2-MG水平差异无统计学意义；尿常规异常的HLD患者尿NAG、RBP和α1-MG水平显著高于尿常规正常的患者(均P＜0.01),但尿β2-MG水平差异无统计学意义.结论 HLD患者尿液中肾小管损害相关标志物水平均显著升高,尿NAG、RBP和α1-MG水平可以更敏感的反映HLD患者肾功能的损害.%Objective To explore the changes and their significance of the levels of urine renal tubule damage associated markers in patients with hepatolenticular degeneration (HLD). Methods The urine routine was examined in 196 HLD patients (HLD group, including false sclerosis type in 45 cases, brain-visceral mixed type in 41 cases, hepatic type in 58 cases and Wilson type in 52 cases) and 47 normal controls (normal control group). The levels of urine renal tubule damage associated markers [including N-acetyl-β-D-glucosaminidase (NAG) , retinol-binding protein (RBP) , α1-microglobulin (αl-MG) and β2-microglobulin (β2-MG) ]were detected by immunoturbidimetric assay. Results In HLD group, urine routine was normal in 54 cases, abnormal in 142 cases. The levels of urine NAG, RBP, α1-MG and (β2-MG in HLD group were significantly higher than those in the normal control group (P＜ 0.05-0.01), but those index among each HLD type were no statistical different. The levels of urine NAG, RBP and al-MG in HLD patients with abnormal urine routine were significantly higher than those in HLD patients with normal urine routine patients (all P ＜ 0. 01) , bur the level of urine β2-MCand between the two groups was no statistical different. Conclusions The levels of urine renal tubule damage associated markers in all types HLD patients are significantly increased. The levels of urine NAG, RBP and α1-MG can be more sensitive reflects renal dysfunction in HLD patients.