49例IgM沉积儿童原发性肾小球疾病的临床特征及预后  

Clinical features and prognosis of 49 cases of childhood primary glomerulopathy with IgM deposition

作　　者：潘晓芬[1] 刘玉玲[1] 李小琳[1] 付四毛[1] 周涛[1] 雷辛衍[1] 罗序峰[1] 林国模[1] 黄东明[1] 杨赛[1] 

机构地区：[1]南方医科大学附属中山市博爱医院儿科,广东中山528403

出　　处：《中国妇幼健康研究》2016年第7期828-830,共3页Chinese Journal of Woman and Child Health Research

摘　　要：目的探讨Ig M沉积儿童原发性肾小球疾病的临床特点及预后。方法回顾分析2009年5月至2015年6月在广东省中山市博爱医院儿肾科住院并行肾穿刺活检的儿童原发性肾小球疾病患儿83例的临床资料,根据其肾组织病理免疫荧光是否有Ig M沉积分为Ig M组(49例)和n Ig M组(34例),分别对其临床表现、病理特点、疗效及预后进行分析。结果临床诊断包括单纯血尿37例,肾病综合征30例,急性肾炎综合征9例,蛋白尿7例。1病理情况:Ig M组以系膜增生性肾小球肾炎为主,占59.2%(29例),显著高于n Ig M组的17.6%(6例),χ2=14.200,P<0.05;而Ig M组轻微病变率28.6%(14例)低于n Ig M组的70.6%(24例)χ2=14.280,P<0.05;局灶节段性肾小球硬化Ig M组和n Ig M组分别为3例和4例;膜增生性肾小球肾炎Ig M组3例、n Ig M组未见病例,Ig M组基底膜增厚及小动脉管壁病变各4例,n Ig M组均未见病例;2对激素治疗反应:有44例使用糖皮质激素,Ig M组和n Ig M组激素敏感分别为56.0%和73.7%,而耐药/依赖/频繁复发情况分别为44.0%和26.3%,,两组差异无统计学意义(P>0.05);3预后:随访6个月至6.5年,Ig M组和n Ig M组缓解率分别占38.8%(19例)和64.7%(22例),两组差异有统计学意义(χ2=5.399,P=0.020)。结论 Ig M沉积的原发性肾小球疾病临床表现与无Ig M沉积病例相似;肾脏病理以系膜增生性肾小球肾炎为主,少部分病例可发生基底膜增厚和小动脉病变;激素的治疗反应与无Ig M沉积者相似;随访发现血尿蛋白尿缓解率较低,应长期跟踪。Objective To explore the clinical features and prognosis of childhood primary glomerulopathy with Ig M deposition. Methods The clinical data of 83 children with primary glomerulopathy,who were hospitalized and underwent puncture biopsy in pediatric nephrology of Zhongshan Boai Hospital during May 2009 to June 2015,was analyzed. According to whether there was Ig M deposition in renal tissue pathology immunofluorescence test,patients were divided into Ig M group（ n = 49） and non-Ig M group（ n = 34）,and their clinical features,pathological characteristics,curative effect and prognosis were analyzed. Results Clinical diagnosis indicated 37 cases of simple hematuria,30 cases of nephrotic syndrome,9 cases of acute nephritic syndrome,and 7 cases of proteinuria. In Ig M group mesangial proliferative glomerulonephritis was the major lesion and occupied 59. 2%（ 29 cases）,which was significantly higher than that（ 17. 6%,6 cases） in non-Ig M group（ χ2= 14. 200,P〈0. 05）. However,rate of minor glomerular abnormalities in Ig M group（ 28. 6%,14） was lower than that in non-Ig M group（ 70. 6%,24）（ χ2= 14. 280,P〈0. 05）. In Ig M group and non-Ig M group there were 3 and 4 cases of focal segmental glomerulosclerosis,and 3 and 0 case of membrano-proliferative glomerulonephritis,respectively. There were 4 cases of basement membrane thickening and 4 cases of small artery wall lesions in Ig M group,but no case was found in non-Ig M group. Totally 44 cases were provided with corticosteroids,and the sensitive rate to steroid was 56. 0% and 73. 7% in Ig M group and non-Ig M group,respectively. However,in drug resistance,dependence or frequently relapse,the rate was 44. 0% and 26. 3%,respectively,and there was no significant difference between two groups（ P〈0. 05）. Follow-up for 6 months to 6. 5 years revealed that there were 19 cases of remission（ 38. 8%） in Ig M group and 22 cases（ 62. 9%） in non-Ig M group,respectively,and the difference between two groups was significant（ χ2= 5. 339,

关 键 词：IgM沉积 原发性肾小球疾病 儿童 临床特征 

分 类 号：R725.5[医药卫生—儿科]