Alport综合征男性患者蛋白尿特点分析  

Features of proteinuria in Chinese male patients with Alport syndrome

作　　者：王芳[1] 刘晓宇[1] 丁洁[1] 张宏文[1] 钟旭辉[1] 肖慧捷[1] 姚勇[1] 

机构地区：[1]北京大学第一医院儿科,100034

出　　处：《中华医学杂志》2012年第12期835-837,共3页National Medical Journal of China

基　　金：国家自然科学基金（30400482、81070545）;北京市自然科学基金（7102148）

摘　　要：目的分析Alport综合征（As）男性患者蛋白尿特点以助于临床早期干预、延缓疾病进展。方法回顾性分析1994-2009年北京大学第一医院儿科门诊和住院患者中118例AS男性患者，其蛋白尿发生时间、程度以及微量白蛋白尿的情况。结果106例患者有蛋白尿。其中婴、幼儿期出现或检测到蛋白尿的患者分别为5例（4．7％）和15例（14．2％）。43例蛋白尿达到肾病诊断标准的患者中，21例（48．8％）患者在6—12岁便具有符合肾病诊断标准的蛋白尿。53例患者1周之内进行了尿蛋白定量和晨尿微量白蛋白的检测，尿蛋白〈0．15g／24h有5例，4例检测到微量白蛋白尿，4例晨尿微量白蛋白浓度增高；尿蛋白／〉0．15g／24h有48例，均检测到微量白蛋白尿，46例晨尿微量白蛋白浓度增高。51例患者尿微量白蛋白浓度和尿微量白蛋白／肌酐改变一致，2例患者尿微量白蛋白浓度正常、而尿微量白蛋白／肌酐〉30mg／g。结论AS男性患者蛋白尿发生早，且进展速度快，其出现蛋白尿前存在微量白蛋白尿状态。早期通过晨尿微量白蛋白／肌酐或微量白蛋白浓度测定检测微量白蛋白尿，可以确定临床进行药物干预的时机。Objective To explore the timing and course of proteinuria in Chinese patients with Alport syndrome （AS）. Methods This retrospectively study included 118 unrelated male AS patients at Department of Pediatrics, Peking University First Hospital between 1994 and 2009. The clinical data of the onset age of proteinuria, the degree of proteinuria and the prevalence of microalbuminuria were analyzed. Urinary total protein was detected by the pyrogallol red protein dye-binding assay, urinary microalbumin by immunoturbidimetric assay and urinary creatinine level by alkaline kinetics. Microalbuminuria was detected when the microalbumin： creatinine ratio was 〉 30 mg/g on a single random midstream first morning urine sample. Results A total of 106 patients had proteinuria. The occurring or detecting age of proteinuria varied from 1 month to 27 years. Five patients （4. 7% ） were under 1 year of age and 15 patients （ 14. 2% ） at toddler＇s age. Among 43 cases with nephrotic-level proteinuria, 21 cases （48.8%） presented with nephrotic-level proteinuria at age 6 - 12 year. Both urine total protein and morning urine microalbumin measurements within 7 days were made in 53 patients. Microalbuminuria occurred in 4 of 5 patients with normal daily urinary protein excretion. All 48 patients had daily proteinuria 〉 0. 15 g. Increased urinary microalbumin was detected in 4 of 5 patients with normal daily urinary protein excretion and 46 patients with daily proteinuria 〉 0. 15 g. The consistency of urine mieroalbumin concentration and urine mieroalbumin-to- creatinine ratio was excellent in 51 patients. However, in 2 patients with slightly increased daily urine total protein, urine microalbumin concentration was normal while the urine mieroalbumin-to-ereatinine ratio abnormal （ 〉 30 mg/g）. Conclusions In AS males, proteinuria occurs earlier and progresses rapidly. Since microalbuminuria precedes the onset of proteinuria, either urine microalbumin concentration or urine microalbumin-to-creatinine ratio in morn

关 键 词：肾炎 遗传性 蛋白尿 男(雄)性 微量白蛋白尿 

分 类 号：R692.9[医药卫生—泌尿科学]