儿童原发性IgM肾病49例临床分析及随访研究  

Clinical analysis and follow-up studies of primary IgM nephropathy in 49 children

作　　者：国琴[1,2] 吴玉斌[1] 赵成广[1] 杜悦[1] 

机构地区：[1]中国医科大学附属盛京医院小儿肾脏风湿免疫科,辽宁沈阳110004 [2]沈阳医学院奉天医院儿科

出　　处：《中国实用儿科杂志》2013年第4期289-291,共3页Chinese Journal of Practical Pediatrics

摘　　要：目的探讨儿童原发性IgM肾病的临床病理特征、疗效及预后,为临床诊治提供参考。方法总结分析2001年6月至2009年10月中国医科大学附属盛京医院儿肾内科49例原发性IgM肾病患儿临床表现、病理特征、治疗及预后。结果 (1)临床类型包括单纯蛋白尿型1例、蛋白尿伴肉眼血尿型1例、原发性肾病综合征型47例。(2)免疫荧光下均可见IgM颗粒状沉积于肾小球系膜区,6例有球囊改变,8例肾小管灶性萎缩,1例可见细胞纤维性新月体,6例肾小球硬化。(3)传统治疗效果不佳者应用其他免疫抑制剂治疗并随访观察,44例随访患者中,35例完全缓解后至今尚未复发,8例复发。结论原发性IgM肾病作为一种独立的疾病,主要临床类型为原发性肾病综合征,其中以难治性肾病最常见,组织学类型以中度系膜细胞增生较多见,新型免疫抑制剂治疗可取得较好的疗效,其远期预后有待进一步随访观察。Objective To investigate the clinical characters,pathological characters,treatment and prognosis of primary IgM nephropathy in children for reference. Methods The data of 49 cases of children with primary IgM nephropathy were reviewed from Jun.2001 to Oct.2009 Department of Nephrology,in Shengjing Hospital Affiliated to China Medical University.The clinical presentation,pathological features , curative effects and prognosis were analyzed. Results （1） clinical types include asymptomatic proteinuria（1/49）, gross hematuria with proteinuria（1/49）,primary nephrotic syndrome（47/49）. （2）IgM was seen as granular deposition in the mesangium under light-immunofluorescence. 7 cases were ball wall thickening or adhesion layer,8 cases were focal tubular atrophy, 1 case had cellular fibrous crescents,6 caess had focal segmental glomerulosclerosis. （3）Other immunosuppressants together with follow-up observation was applied in that conventional treatment unsatisfactly.Among 44 cases of follow-up observation ,35 cases after complete remission had not yet relapsed. Conclusion Primary IgM nephropathy should be seen as an independent entity,the main clinical type is primary nephrotic syndrome,refractory nephritic disease is the most common, moderate mesangial cell proliferation is more common histological type,new immunosuppressive agents are expected to gain the better results,long-term prognosis needs further follow-up study.

关 键 词：儿童 原发性IgM肾病 难治性肾病 

分 类 号：R72[医药卫生—儿科]