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目的探讨小儿IgA肾病的临床与病理特点。方法总结中南大学湘雅二医院儿科1997-05—2004-12通过肾活检确诊的64例小儿IgA肾病,根据病程、临床类型比较其临床与病理特点。结果64例小儿IgA肾病的临床类型主要为孤立性血尿型(29例)和肾病综合征型(18例),占73%(47/64)。以病程分组比较,主要的病理特征是炎症细胞浸润、肾小管变性和肾间质纤维化,其中肾间质纤维化的分布显著性不均衡,与肾病综合征型IgA肾病的分布有密切关系。肾病综合征型IgA肾病的炎症细胞浸润、肾小管变性和肾间质纤维化的发生率均显著性高于其他类型的IgA肾病。结论64例IgA肾病以孤立性血尿和蛋白尿为主,肾病综合征型IgA肾病或持续性蛋白尿是导致肾间质纤维化的主要原因,早期控制蛋白尿可能会延缓或减轻肾间质纤维化的发生。

Clinical and pathological features of children with IgA nephropathy

Objective To investigate clinical and pathological features of children with IgA nephropathy (IgAN).Methods To characterize the clinical manifestations and pathological changes of 64 children with IgAN depending upon the course of disease and clinical type.Results Isolate hematuria and nephrotic syndrome of IgAN were major clinical types in 64 cases,accounting for 73% (47/64).Upon the course of disease,three pathological changes such as inflammation cells infiltration,renal tubule degeneration and renal interstitial fibrosis predominated,of whom renal interstitial fibrosis unbalancedly distributed between the group of less than or equal to one year and more than one year,which was closely related to unbalanced distribution of nephrotic syndrome IgAN,in which the three changes were more obvious than other clinical type of IgAN.Conclusion In 64 children with IgAN,isolate hematuria and nephrotic syndrome are main clinical type of IgAN.Nephrotic syndrome IgAN or continuous proteinuria is the major cause leading to renal interstitial fibrosis.Early control of proteinuria would delay or decrease renal interstitial fibrosis.