Abstract:

Objective　To summarize the clinicopathological features，treatment and prognosis of childhood idiopathic membranous nephropathy（IMN）. Methods　The clinical manifestations，pathologic features，treatment and prognosis of 22 IMN children who were diagnosed by pathology from January 2005 to February 2017 were retrospectively analyzed. Results　Twenty-two children（12 males and 10 females） had an onset age range of 3 to 15 years. Clinical manifestations：hematuria and proteinuria in 4 cases（18.18%）；nephrotic syndrome（NS） in 18 cases（81.82%）. Renal biopsy results：8 cases（36.36%） in stageⅠ，5 cases（22.73%） in stage Ⅰ-Ⅱ，5 cases（22.73%） in stage Ⅱ，1 case（4.54%） in stage Ⅱ-Ⅲ，atypical membranous nephropathy in 3 cases（13.64%）. Among them，12 cases（54.54%） of M-type phospholipase A2 receptor（PLA2R） positive were detected and 83.33%（10/12） PLA2R positive children with an onset age older than 10 years old. Immunofluorescence showed predominantly IgG and C3 deposition. Treatment programs：in the 4 cases whose clinical manifestations were hematuria and proteinuria，2 cases were given glucocorticoid and ACEI drug treatment，the other 2 cases were given ACEI drugs alone，and they had complete remission. The 18 patients with NS all were treated with glucocorticoid and ACEI，and immunosuppressive agents were given to steroid-resistant patients. During the follow-up，18 cases（81.82%） were completely relieved，3 cases（13.64%） were partially relieved，and 1 patient（4.54%） was lost of follow-up. Conclusion　Clinical manifestations of IMN are mostly nephritis nephrotic syndrome，mainly steroid-resistant nephrotic syndrome. Pathological performance mainly isⅠ-Ⅱstage. The positive rate of PLA2R in junior age was lower than adolescence. Glucocorticoid combined with immunosuppressive agents has a higher response rate.

Key words: child, idiopathic membranous nephropathy, M-type phospholipase A2 receptor, clinicopathological feature

摘要：

目的　总结儿童特发性膜性肾病（IMN）的临床病理特点、治疗及预后转归。方法　回顾性分析2005年1月至2017年2月山东大学附属省立医院小儿肾脏风湿免疫科经病理证实的22例IMN患儿的临床表现、病理特征、治疗及预后等资料。结果　22例患儿中男12例，女10例，发病年龄3～15岁。临床表现：血尿蛋白尿4例（18.18%）；原发性肾病综合征18例（81.82%）。初次肾活检结果：膜性肾病Ⅰ期8例（36.36%）、Ⅰ～Ⅱ期5例（22.73%），Ⅱ期5例（22.73%）、Ⅱ～Ⅲ期1例（4.54%）、不典型膜性肾病3例（13.64%）。22例患儿肾组织M型磷脂酶A2受体（PLA2R）阳性者12例（54.54%），83.33%（10/12）PLA2R阳性的患儿起病年龄≥10岁。治疗方案：4例血尿蛋白尿患儿中2例给予激素及血管紧张素转化酶抑制剂（ACEI）治疗，2例仅给予ACEI治疗，均获得完全缓解。18例肾病综合征患儿全部予以激素、ACEI治疗，激素耐药者加用免疫抑制剂治疗。随访发现22例患儿中18例（81.82%）获完全缓解，3例（13.64%）获部分缓解，1例（4.54%）失访。结论　儿童IMN临床多表现为肾炎型肾病综合征，以激素耐药型为主。病理表现以Ⅰ～Ⅱ期多见。儿童IMN的PLA2R阳性以青少年为主，小年龄儿童阳性率较低。激素联合ACEI、免疫抑制剂治疗缓解率较高。

关键词: 儿童, 特发性膜性肾病, M型磷脂酶A2受体, 临床病理特点