Objective: To explore the clinical features and expression of PLA(2)R in renal tissue of children with idiopathic membranous nephropathy. Methods: Retrospective study was performed in patients with membranous nephropathy diagnosed through renal biopsy and the follow-up time was at least half a year in Shanghai Children's Hospital from January 2010 to February 2017. We compared their clinicopathological and pathological findings of IMN. Indirect immunofluorescence assay was used to detect glomerular PLA(2)R expression. We analyzed the differences of clinical features between the PLA(2)R negative and positive groups. T test, rank-sum test and Fisher exact test were used. Results: Eleven cases had hematuria and proteinuria, 9 cases presented with nephrotic syndrome, and 2 cases showed isolated proteinuria. Of the 22 cases of children with IMN, 16 patients had complete remission (complete remission rate was 72.8%), and 22 patients had partial remission. The renal function of all cases was normal and in all cases the estimated glomerular filtration rate was > 90 ml/(min·1.73m(2)). Of 22 cases with IMN, 7 cases were PLA(2)R-positive in renal tissue and 15 cases were PLA(2)R-negative. The age of positive group (10 years old) was older than the negative group (6 years old)(Z=-2.483, P<0.05) and the time of positive group (6 months) for urine protein to return to negative was longer than the negative group (2.5 months) through treatment. These differences were significantly different (Z=-2.072, P<0.05). Conclusions: Hematuria and proteinuria can be found in most children with idiopathic primary membranous nephropathy. Prednisone combined with immunosuppressant was effective. The positive rate of PLA(2)R in renal tissue of children with IMN was about 32%. The age of PLA(2)R positive group was older than the negative group. And the time of urine protein turning to negative in positive group was longer than that in the negative group.
目的： 分析儿童原发性膜性肾病（IMN）的临床特点及肾组织M型磷脂酶A(2)受体(PLA(2)R)的表达。 方法： 选取2010年1月—2017年2月在上海交通大学附属儿童医院经肾活检证实的，随访至少半年且临床和病理资料完整的IMN儿童22例；回顾性分析患儿发病至随访终点的临床资料、治疗方案、疗程及疗效进行比较。采用间接免疫荧光法检测PLA(2)R在肾组织的表达；分析阳性组与阴性组之间临床特点的区别。两组间差异使用t检验、秩和检验及Fisher确切概率法。 结果： 22例IMN患儿中11例临床表现为血尿和蛋白尿，9例表现为肾病综合征，2例表现为孤立性蛋白尿。22例IMN患儿中，经糖皮质激素或糖皮质激素联合免疫抑制剂治疗后，至随访终点时，完全缓解16例（73%），部分缓解6例，所有患儿肾功能均正常，肾小球滤过率估计值均> 90 ml/(min·1.73 m(2))。22例IMN患儿中，肾组织PLA(2)R阳性组7例（32%），阴性组15例，两组在年龄（10岁比6岁）及尿蛋白转阴时间（6.00个月比2.50个月）上的差异有统计学意义（Z值分别为－2.483及－2.072，P<0.05）。 结论： 儿童IMN临床以血尿和蛋白尿居多。应用糖皮质激素联合免疫抑制剂能取得较为满意的治疗效果。儿童IMN肾组织PLA(2)R表达阳性率约32%，与表达阴性组相比，肾组织PLA(2)R表达阳性患儿年龄偏大，尿蛋白转阴时间更长。.
Keywords: Child; Idiopathic membranous nephropathy; Immunosuppressive agents; M Type phospholipase A(2) receptor; Remission.