以肾病综合征为临床表现的儿童IgM肾病临床特征

IgM nephropathy with nephrotic syndrome in children

目的 探讨以肾病综合征为临床表现的IgM肾病患儿的临床特征.方法 以2005年6月至2012年6月在湖南省儿童医院肾内科住院,临床诊断为肾病综合征、病理诊断为IgM肾病的36例患儿为研究对象（A组）,以同期住院,病理诊断为微小病变的肾病综合征106例患儿为对照组（B组）.随访1～8年,分析其临床特征.结果 （1）对照组、研究组患儿伴有血尿者分别为3.8％及30.6％（x2=20.403,P＜0.05）.（2）研究组患儿肾脏病理构成：轻度系膜增生性病变26例（72.2％）,中度系膜增生性病变9例（25％）,1例为局灶节段性肾小球硬化.（3）将研究组按肾脏病理分成轻度病变组及中重度病变组,对照组、轻度病变组、中重度病变组患儿激素耐药率分别为12.3％、19.2％、77.8％（x2 =24.369,P＜0.05）,对照组与轻度病变组之间差异无统计学意义（P＞0.05）.（4）激素耐药者联用吗替麦考酚酯治疗,对照组、研究组激素耐药患儿的缓解率分别为50％及85.7％（x2=3.60,P＞0.05）.结论 以肾病综合征为临床表现的IgM肾病患儿血尿的发生率较高,肾脏病理为中度病变以上者激素耐药发生率较高,需早期联合应用免疫抑制剂治疗,吗替麦考酚酯可能成为较好的免疫抑制剂选择方案.

Objective To investigate the clinical charactors and treatment of IgM nephropathy with nephrotic syndrome（NS） in children.Methods Thirty-six IgM nephropathy patients hospitalized in Hunan Children＆#39;s Hospital as research group（group A）,from June 2005 to June 2012.One hundred and six patients with minimal change disease （MCD） as control group （group B）,followed up for 1 ～ 8 years.Results （1） Hematuria at presentation of the two groups respectly 3.8% vs 30.6% （x2 =20.403,P 〈 0.05）.（2） Renal pathology revealed that there were 26 （72.2%）patients with minimal change disease,9 cases （72.2 %）with moderate membranoproliferative glomerulonephritis,1 cases with focal segmental glomerulosclerosis.（3） According to renal pathology,group A patients were divided into two sub groups：mild group and moderate group.To compared with group B,the steroid-resistant incidence of the 3 group were respectly 12.3%,19.2%,77.8% （x2 =24.369,P 〈 0.05）.There was no significant difference between control group and mild group.（4）The remission rate of proteinuria in steroid-resistant patients who combined to use mycophenolate mofetil （MMF） with the two groups were respectively 50% and 85.7 % （x2 =3.60,P 〉 0.05）.Conclusion Incidence of hematuria is higher in IgM nephropathy patients and patients with renal pathology for moderate lesions have a high steroid-resistant,and need use immunosuppressive early.MMF may be a good immunosuppressive for theses patients.