单中心86例儿童终末期肾病病因分析

Etiology analysis of 86 children with end - stage renal disease in a single - center

目的探讨儿童终末期肾病（ESRD）的病因构成，为其预防、诊治提供参考资料。方法对2005年1月至2013年10月在北京大学第一医院儿科住院治疗的ESRD患儿的病因构成、发病情况等进行回顾性分析。ESRD患儿诊断标准参照2002年美国肾脏基金会制定的慢性肾脏病临床实践指南（NKF／KDOQI）慢性肾脏病（CKD）5期诊断标准。结果共86例ESRD患儿人选，其中男53例，女33例，男女比例1．61：1．00；平均发病年龄（7．084-4．23）岁；确诊年龄（9．25±4．17）岁；确诊前病程中位数为0．84（0．01～13．67）年。ESRD病因构成中以获得性。肾小球疾病所占比例最高，占43．02％（37／86例），主要为慢性。肾小球肾炎（18／86例，20．93％）和肾病综合征（16／86例，18．60％）；其次为先天泌尿系统发育异常，占40．70％（35／86例），其中以。肾发育不良（18／86例，20．93％）和囊性肾脏疾病（11／86例，12．79％）最多见。在3岁以下年龄组患儿病因中以先天泌尿系统发育异常最多（6／10例，60．00％），3岁以上年龄组患儿病因中以获得性肾小球疾病最多（37／76例，48．68％），肾脏病理类型主要为增生硬化／硬化性肾小球肾炎（6／23例，26．09％）、局灶节段性。肾小球硬化（5／23例，21．74％）和间质性。肾炎（3／23例，13．04％）。结论儿童ESRD的主要病因为肾小球疾病和先天泌尿系统发育异常，应重视围生期泌尿系统超声筛查和儿童尿筛查，积极干预慢性肾脏疾病进展，减少ESRD的发生。

Objective To investigate the etiology composition of end- stage renal disease （ESRD） in chil- dren,in order to provide reference for the prevention and treatment. Methods The children with ESRD who were diag- nosed in Peking University First Hospital from January 2005 to October 2013 were selected, and the etiology composition and incidence of the children with ESRD were retrospectively analyzed. Diagnostic criteria for children with ESRD refer to the clinical practice guidelines for chronic renal disease （NKF/KDOQI） , developed by the American kidney founda- tion in 2002. Results Eighty - six children with ERSD were enrolled including 53 cases of males ,33 cases of females, with the male to female ratio of 1.61 ： 1.00 and the mean onset age was （ 7.08 ± 4.23 ） years old, and their average diagnosis age was（9.25 ± 4.17 ） years old. The median duration of ERSD before diagnosis was 0.84 （ 0.01 ±13.67 ） years. The main cause of ESRD was acquired renal disease, accounting for 43.02% （37/86 cases） , mainly the chronic glomemlonephritis （ 18/86 cases ,20.93% ） and nephrotic syndrome （ 16/86 cases, 18.60% ） ;followed by urinary con- genital abnormity, accounting for 40.70% （35/86 cases） , in which the most common were renal dysplasia （18/86 ca- ses ,20.93% ） and cystic renal disease （ I1/86 cases, 12.79% ）. Children under 3 years old mainly showed congenital urinary tract abnormalities （6/10 cases ,50.00% ）. But children over 3 years old mainly showed acquired renal diseases （37/76 cases ,58.7% ）, and pathologic classification of glomerular disease were proliferative mesangial glomerulone- phritis （6/23 cases,26.09% ）,focal segmental glomerulosclerosis （5/23 cases,21.74% ） and interstitial nephritis（3/ 23 cases, 13.04% ）. Conclusions The main etiology of ESRD is glomerular disease and congenital abnormal develop- ment of urinary system ,therefore, more attention should be paid on the ultrasound screening of the urinary tract in the perinatal period and urine screening in children. There are great significances in reducing the incidence of ESRD and intervening actively the progression to chronic kidney disease.