肾病综合征表现的儿童IgA肾病15例临床和病理分析

目的分析儿童IgA肾病中表现肾病综合征的患儿临床及病理特征。方法总结2000～2006年重庆医科大学附属儿童医院62例肾活检病理诊断为原发IgA肾病中,临床表现为肾病综合征的15例患儿临床病理特点结果 62例中表现为肾病综合征者15例,占24.2%;按WHO病理组织分类,病理分级为Ⅱ级1例,Ⅲ级8例,均为肾炎性肾病,IV级5例,单纯性肾病2例,肾炎性肾病3例,V级1例,为肾炎性肾病;免疫荧光分型IgA+C33例,IgA+C3+IgG1例,IgA+C3+IgG+IgM7例,IgA+C3+IgM3例,IgA+IgM1例,以IgA+C3+IgG+IgM多见,小管间质损害轻型7例,中度损害3例。结论儿童IgA肾病表现为肾病综合征者病理改变较重,病理损害以III～IV级为主,免疫荧光以IgA+C3+IgG+IgM多见,小管间质损害以轻-中度损害多见。

Clinical and pathological analysis of 15 cases IgA nephropathy with manifestation of nephritic Syndrome in Children

Objective To analyse clinncal and pathologic features of immunoglobulina nephropathy（IgAN） with manifestation of nephritic syndrome in children.Methods A retrospective review of 62 cases of primary IgAN within 6 years（2000～2006） were conducted based on renal biospy,tosummarize clinical manifestactions and pathological features of IgAN with manifestation of nephritic syndrome.Results According to WHO pathological changes of 1 case was grade II（6.7%）,1 case with nephritis nephropathy;8 cases were grade III（53.3%）,all cases with nephritis nephropathy.5 cases were grade IV（33.3%）,2 cases with simple nephropathy,3 cases with nephritis.1 case was grade V（6.7%）.Immunofluorescence typing IgA＋C33 cases（20%）,IgA＋C3＋IgG,1 case（6.7%）IgA＋C3＋IgG＋IgM7 cases（46.7%）,IgA＋C3＋IgM,3 cases（20%）,IgA＋IgM 1 case（6.7%）,IgA＋C3＋IgG＋IgM was frequently found.Conclusion The clinical representation and tissue damage of IgA nephropathy presenting with rephritic syndrome are serious,grades Ⅲ and Ⅳ.Pathologically and type IgA＋C3＋IgG＋IgM immunologically are common.