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IgA肾病(IgAN)是一种具有特殊临床病理改变的疾病。在免疫荧光检查下可表现为肾小球系膜区IgA沉积和少量IgG、IgM、C3。许多研究发现,IgAN时常伴IgA免疫系统的异常,如血清IgA浓度增高,体外IgA生成增加、IgA特异的T抑制细胞活性降低和T辅助细胞活性增加,IgA耐受细胞的数量增多,由于CD8细胞数的减少导致CD4/CD8比值上升,并有循环IgA免疫复合物的存在。有关这些现象和发病机制、疾病进展之间的关系仍不清楚。作者认为伴肉眼血尿的IgAN患儿具有较良性的病程。重复肾活检表明持续性蛋尿白尿的患儿肾小球常出现进行性改变,而在蛋白尿消失的患儿则罕见。因此,进行性IgAN的最重要临床表现是持续性蛋白尿而不是肉眼血尿。
IgA nephropathy (IgAN) is a disease with a special clinicopathological change. Immunofluorescence can be expressed as glomerular mesangial IgA deposition and a small amount of IgG, IgM, C3. Many studies have found that IgAN is often accompanied by IgA immune system abnormalities such as increased serum IgA concentration, increased in vitro IgA production, IgA-specific T suppressor cell activity decreased and T helper cell activity increased, the number of IgA resistant cells increased due to CD8 cells The decrease in numbers leads to an increase in the CD4 / CD8 ratio and the presence of circulating IgA immune complexes. The relationship between these phenomena and the pathogenesis of disease progression is still unclear. The author believes IgAN children with gross hematuria have a more benign course. Repeated renal biopsy showed that persistent glomerular proteinuria in children with progressive changes often occur, but disappeared in the proteinuria is rare. Therefore, the most important clinical manifestation of progressive IgAN is persistent proteinuria but not gross hematuria.