伴新月体形成的IgA肾病患儿临床病理特点和预后分析  

Clinicopathological features and prognosis of IgA nephropathy with crescentic formation in children

作　　者：许瑞英[1] 刘华卫[1] 葛伟[1] 董俊华[1] 甄军晖[2] Xu Ruiying;Liu Huawei;Ge Wei;Dong Junhua;Zhen Junhui(Department of Pediatrics,Qilu Hospital of Shandong University,Jinan 250012,China)

机构地区：[1]山东大学齐鲁医院儿科,济南250012 [2]山东大学医学院病理教研室

出　　处：《中华肾脏病杂志》2018年第7期494-499,共6页Chinese Journal of Nephrology

摘　　要：目的观察伴新月体形成的IgA肾病患儿的临床、病理特点和预后。方法回顾性分析山东大学齐鲁医院儿科经肾活检确诊的儿童IgA肾病38例，根据是否有新月体形成分为新月体形成组（新月体形成≥10％，n=18）；选择同期肾活检、临床资料完整以及年龄、性别、病程相匹配、无新月体形成的IgA肾病患儿作为对照（无新月体形成组，n=20）。比较两组患儿的临床、病理特点及预后生存率的差异。结果新月体形成组患儿14例（77．78％）伴有肉眼血尿，12例（66．67％）有高血压；非新月体形成组8例（40％）有肉眼血尿，6例（30％）有高血压，差异有统计学意义（均P〈0．05）。新月体形成组患儿血Alb、估算肾小球滤过率（eGFR）明显低于非新月体形成组；24h尿蛋白量、Scr明显高于非新月体形成组（均P〈0．05）。新月体形成组患儿肾组织病理改变分级以Ⅱ、Ⅲ级为主，合并肾小管间质损害较多见；除IgA沉积外，多伴有IgG和（或）IgM等免疫球蛋白沉积。非新月体形成组患儿肾组织病理改变分级以Ⅰ、Ⅱ级为主，合并有肾小管间质损害者较少见，免疫球蛋白沉积以IgA沉积为主。患儿经激素和（或）免疫抑制剂治疗12周后，新月体形成组7例尿蛋白转阴，7例24h尿蛋白量较用药前下降50％以上，4例24h尿蛋白量无明显下降；非新月体形成组12例尿蛋白转阴，6例24h尿蛋白量较用药前下降50％以上，2例尿蛋白量无明显下降。随访1-3年，新月体形成组14例肾功能正常，3例Scr轻度升高，1例需透析治疗；非新月体形成组19例肾功能正常，1例Scr轻度升高，未达透析标准。结论伴新月体形成的IgA肾病患儿临床病理改变表现重，易出现肾功能不全，提示出现新月体形成是病情预后不良的指标之一。Objective To observe the clinical, pathological features and prognosis of IgA nephropathy in children with crescent formation. Methods A total of 38 cases of children with IgA nephropathy diagnosed by renal biopsy at Qilu Hospital of Shandong University were retrospectively analyzed. According to whether there were crescent formation, they were divided into crescents formation group （crescent formation≥10%, n=18） and control group consisting of children with complete clinical data and matching age, gender and course of disease （no crescent formation, n=20）. The clinical, pathological features and prognosis of the two groups were compared. Results Fourteen cases （77.78%） of crescents formation group were associated with nausea hematuria, and 12 cases （66.67%） had hypertension, 8 patients in control group （40%） had gross hematuria, and 6 cases （30%） had high blood pressure. The differences were statistically significant （P 〈 0.05）. In crescents formationgroup, Alb and eGFR were significantly lower than those of control group. 24 h urine protein and Scr were significantly higher than those of control group and there were significant statistically differences between two groups （P 〈 0.05）. Nephropathological changes in crescent formation group were mainly in grade Ⅱ and grade Ⅲ and were usually combined with tubular interstitial damages. In addition to IgA immunoglobulin deposition, they often accompanied by IgG and IgM immunoglobulin deposition. In control group, nephropathological changes were mainly in grade Ⅰ and grade Ⅱ and tubular interstitial damages were rare. IgA was the main form of deposition in control group. After 12 weeks steroid treatment and （or） immunosuppressive therapy, urinary protein turned negative in 7 eases in crescent formation group, 24 h urinary protein level declined by half than before in 7 eases and no obvious change in 4 eases. In control group, urinary protein turned negative in 12 cases, 6 cases showed a more than 50% decline in urinar

关 键 词：肾小球肾炎 IGA 儿童 病理学 临床 新月体 预后 

分 类 号：R726.9[医药卫生—儿科]