青春期儿童特发性膜性肾病的临床特点及预后  

Clinical characteristics and prognosis of idiopathic membranous nephropathy in adolescent

作　　者：杨茜[1] 高二志[1] 左科[1] 徐维玮[1] 徐峰[1] 程震[1] Yang Xi;Gao Erzhi;Zuo Ke;Xu Weiwei;Xu Feng;Cheng Zhen(Department of Nephrology,Nanjing General Hospital of Nanjing Military Command,National Clinical Research Center of Kidney Diseases ,Nanjing 210016,China)

机构地区：[1]南京军区南京总医院肾脏科,国家肾脏疾病临床医学研究中心,全军肾脏病研究所,210016

出　　处：《国际泌尿系统杂志》2019年第1期121-125,共5页International Journal of Urology and Nephrology

摘　　要：目的分析阐明青春期儿童特发性膜性肾病(idiopathic membranous nephropathy,IMN)的临床特点及预后。方法选取本院2005年1月至2014年12月间经肾活检明确诊断为IMN且随访1年以上的青春期儿童患者90例,并随机抽取同期成人90例IMN患者为对照组,回顾性对比分析其临床表现、实验室检查及预后。结果①青春期IMN患儿起病时肾功能正常,与成人IMN相比低蛋白血症明显(P=0.0235),血IgG水平低(P<0.0001),但外周血CD4、CD20计数明显升高(P<0.0001),而尿蛋白定量及肾小管损伤指标、镜下血尿无差别;②青春期IMN患儿血抗PLA2R抗体阳性率为58.82%(40/68),与成人IMN阳性率55.9%(33/59)相当(P=0.857);③青春期IMN患儿平均随访12~130个月(中位时间40个月),累计有效率为88.89%(80/90),肾存活率2年为98.8%,3年为97%,6年为87.3%,与成人IMN相比无统计学差异(P=0.175);④多因素Cox回归分析显示高血压、尿RBP、肾活检时血肌酐为IMN患儿出现肌酐倍增的独立危险因素。结论青春期儿童IMN血抗PLA2R抗体的阳性检出率与成人IMN相当,其治疗反应和预后和成人IMN类似。Objective Idiopathic membranous nephropathy (IMN) is one of the most common causes of nephrotic syndrome in adults, but it is relatively rare in adolescent. The aim of this study was to elucidate the clinical features and prognosis of IMN in adolescent. Methods All patients were admitted to our hospital from January 2005 to December 2014 with biopsy proven IMN, and at least 12 months follow-up in this retrospective study. Adult patients with IMN were served as the controls, and their clinical manifestations, laboratory tests and prognosis were collected. Results ①The renal function of IMN in adolescent were normal at onset, they had lower serum albumin (P=0.0235) and serum IgG levels (P<0.0001) compared with adult IMN, but the peripheral blood CD4 and CD20 count were significantly increased (P=0.0256, P<0.0001), also there were no difference of 24 h urinary protein, renal tubular injury index and hematuria. ②The positive rate of PLA2R antibody in adolescent was 58.82% (40/68), which was similar to adults (P=0.857). ③Adolescent IMN were followed up 12~130 months (median 40 months), the total efficiency (complete and partial remission rate) was 88.89%(80/90), and the renal survival rate was 98.8% , 97.0% and 87.3% at 2, 3 and 6 years after renal biopsy. ④Multivariate Cox regression analysis showed that hypertension, urinary RBP, and renal creatinine were the independent risk factors for double creatinine. Conclusions The positive rate of PLA2R antibody in adolescent IMN is similar to that of adult IMN, and its treatment response and prognosis are similar to adult IMN.

关 键 词：肾小球肾炎 膜性 青少年 预后 

分 类 号：R726.9[医药卫生—儿科]