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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 investigate the clinical features and prognosis of primary glomerular disease in children with IgM deposits. Methods The clinical data of 83 children with primary glomerular disease undergoing renal biopsy from May 2009 to June 2015 in Pediatric Hospital of Bo’ai Hospital of Zhongshan City, Guangdong Province were retrospectively analyzed. According to their renal histopathological immunity Fluorescent IgM deposits were divided into Ig M group (n = 49) and n Ig M group (n = 34), and their clinical manifestations, pathological features, curative effects and prognosis were analyzed respectively. Results Clinical diagnosis included hematuria in 37 cases, nephrotic syndrome in 30 cases, acute nephritic syndrome in 9 cases and proteinuria in 7 cases. 1 Pathology: The majority of mesangial proliferative glomerulonephritis (IgA) accounted for 59.2% (29 cases) in IgM group, which was significantly higher than 17.6% (n = 6) in n Ig M group (χ2 = 14.200, P <0.05) The slight lesion rate in IgM group was 28.6% (14 cases), which was lower than 70.6% (24 cases) in n Ig M group (χ2 = 14.280, P <0.05); focal segmental glomerulosclerosis IgM group and n Ig M Group 3 and 4 respectively. There were 3 cases of IgM with membranoproliferative glomerulonephritis, no cases of n Ig M, 4 cases of basilar membrane thickening and small artery wall lesions in IgM group, n Ig M 2 had no response to hormone therapy: hormone response was found in 44 patients with glucocorticoid, and those with Ig M and n Ig M were 56.0% and 73.7%, respectively, whereas those with drug resistance / dependence / frequent recurrence were 44.0 % And 26.3%, respectively. There was no significant difference between the two groups (P> 0.05) .3 Prognosis: The response rates of IgM and n IgM were 38.8% (19 cases) and 64.7 % (22 cases), the difference between the two groups was statistically significant (χ2 = 5.399, P = 0.020). Conclusions The clinical manifestations of primary glomerular disease with IgM deposition are similar to those without IgM deposition. The main pathological features of renal pathology are mesangial proliferative glomerulonephritis. In a few cases, thickening of basement membrane and arterioles may occur. Hormone treatment response and non-Ig M deposits were similar; follow-up found that urinary proteinuria lower remission rate should be long-term follow-up.