以肾病综合征为临床表现的儿童IgA肾病的临床研究

Clinical Study of IgA Nephropathy with Clinical Manifestations of Nephrotic Syndrome in Children

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 explore the clinical features and therapy of IgA nephropathy （IgAN） with clinical manifestations of nephrotic syndrome （NS） in children. Methods： The clinical date of 33 patients of IgAN with NS who were hospitalized in Children＇ s Hospital of Hunan Province from December 2006 to June 2012 was analyzed, following up one to five and half a year. According to the Lee＇ s classification, they were divided into two groups： group Ⅰ （13 children, Lee＇s grade ≤Ⅱ ） and group Ⅱ （20 children, Lee＇s grade ≥ Ⅲ ）. All children took oral prednisone, and steroid-resistant children combined with mycophenolate mofetil （MMF）. Results： The incidence of hematuria in group Ⅰ and group Ⅱ was 61.5% and 100% , there were significant difference between the two groups （X2= 9.07, P〈0.05）. The incidence of steroid-resistant in group I and group Ⅱ were 38.5% and 100% , There were also significant difference between the two groups （X2 = 16.25, P〈0.01 ）. All steroid-resistant children accompanied with hematuria. The remission rate of proteinuria in steroid-resistant children who combined with mycophenolate mofetil （MMF） was 83.3%. There were no significant difference between the two groups （X2 =0.05, P〉 0.05）. Conclusion： The children of IgAN with NS who were accompanied with hematuria or the Lee＇ s grade ≥Ⅲ have a high incidence of steroid-resistant, and need to combine with immunosuppressive agents early. MMF may be a good option in immunosuppressive agent.