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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。
Objective To analyze the clinical and pathological features of children with nephrotic syndrome in childhood IgA nephropathy. Methods: From 2000 to 2006, 62 cases of renal biopsy in Children’s Hospital Affiliated to Chongqing Medical University were diagnosed as primary IgA nephropathy. The clinical features of 15 cases with nephrotic syndrome were analyzed. Of the 62 cases, those with nephrotic syndrome were 15 Cases, accounting for 24.2%; according to the WHO pathological tissue classification, pathological grade was grade Ⅱ in 1 case, grade Ⅲ in 8 cases, all nephritis, grade IV in 5 cases, simple nephropathy in 2 cases, nephritis nephropathy in 3 cases, V grade 1 case was nephritis kidney disease; immunofluorescence typing IgA + C33 cases, IgA + C3 + IgG1 cases, IgA + C3 + IgG + IgM7 cases, IgA + C3 + IgM3 cases, IgA + IgM1 cases, IgA + C3 + More common IgG + IgM, tubulointerstitial damage in 7 cases of light, 3 cases of moderate damage. Conclusions Children with IgA nephropathy showed severe pathological changes in nephrotic syndrome. The pathological changes were mainly from III to IV. Immunofluorescence was more common in IgA + C3 + IgG + IgM. More common in mild to moderate lesions were tubulointerstitial damage .