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目的:探讨肾病综合征(NS)型儿童IgA肾病(IgAN)的特征及治疗方案。方法:分析2006年12月至2012年6月在湖南省儿童医院肾内科住院的33例NS型IgAN患儿的临床资料并随访1年~5年6个月。依据Lee氏分级将病例分为两组:Ⅰ组13例,Lee氏≤Ⅱ级;Ⅱ组20例,Lee氏≥Ⅲ级。全部患儿均口服泼尼松,对于激素耐药者联用吗替麦考酚酯(MMF)。结果:Ⅰ组、Ⅱ组患儿血尿发生率分别为61.5%、100%(χ2=9.07,P<0.05);激素耐药发生率分别为38.5%、100%(χ2=16.25,P<0.01),所有激素耐药的患儿均伴有血尿。激素耐药者联用MMF治疗,蛋白尿的缓解率为83.3%,Ⅰ组蛋白尿缓解率80.00%,Ⅱ组为84.2%,两组比较差异无统计学意义(χ2=0.05,P>0.05)。结论:NS型IgAN患儿临床表现伴有血尿者,或病理Lee氏≥Ⅲ级者激素耐药发生率高,需早期联用免疫抑制剂治疗,联用MMF可能为较好的免疫抑制剂选择方案。
Objective: To investigate the characteristics and treatment of IgAN in children with nephrotic syndrome (NS). Methods: The clinical data of 33 cases of NS-type IgAN hospitalized in Department of Nephrology, Children’s Hospital of Hunan Province from December 2006 to June 2012 were analyzed and followed up for 1 year to 5 years and 6 months. According to Lee’s classification, the patients were divided into two groups: group Ⅰ 13 cases, Lee’s ≤ Ⅱ grade; group Ⅱ 20 cases, Lee’s ≥ Ⅲ grade. All children were given prednisone orally, and mifeprone (MMF) for steroid-resistant patients. Results The incidence of hematuria in group Ⅰ and group Ⅱ was 61.5% and 100%, respectively (χ2 = 9.07, P <0.05). The rates of hormonal resistance were 38.5% and 100% respectively (χ2 = 16.25, P <0.01) All children with hormonal resistance are associated with hematuria. Hormone resistance associated with MMF treatment, proteinuria remission rate was 83.3%, Ⅰ proteinuria remission rate was 80.00%, Ⅱ group was 84.2%, there was no significant difference between the two groups (χ2 = 0.05, P> 0.05) . Conclusion: The clinical manifestations of NS-IgAN patients with hematuria or pathological Lee’s grade Ⅲ hormone-refractory high incidence of need for early treatment with immunosuppressive agents, combined with MMF may be a better choice of immunosuppressive agents Program.