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目的探讨儿童IgA肾病(IgAN)的临床、病理特点及其相关关系。方法对本院2005年5月-2011年8月经肾穿刺活检确诊为IgAN的72例患儿的临床表现、临床分型、病理特点及免疫分型进行回顾性总结,并分析它们之间的相关关系。结果本组72例。男48例,女24例;年龄1岁5个月～17岁[(8.99±2.94)岁];入院时病程2 d～9 a(平均12.86个月)。临床以血尿起病者58例(包括38例肉眼血尿及5例伴水肿者),以单纯水肿起病者12例,以蛋白尿起病者2例。临床分型为肾病综合征型28例(38.89%)、孤立性血尿型19例(26.39%)、血尿和蛋白尿型13例(18.05%)、急性肾炎型10例(13.89%)、孤立性蛋白尿型2例(2.78%)。病理改变:系膜增生型肾小球肾炎40例,局灶增生型肾炎25例、毛细血管内增生型肾炎6例、新月体型肾炎1例。其中伴新月体形成者17例(占23.61%)。免疫组织化学可见多种免疫球蛋白沉积。沉积类型为满堂亮型1例、IgA+IgG+C32例、IgA型8例、IgA+IgM+IgG+C3型17例、IgA+IgM+C3型44例。结论 IgAN的临床表现形式多样,其病情轻重与起病形式无关。病理表现以系膜增生型肾小球肾炎为主,免疫球蛋白沉积以复合型为主。临床表现为肾病综合征型及血尿和蛋白尿型者病理较重,应尽早行肾穿,及时治疗。
Objective To investigate the clinical and pathological features of IgA nephropathy (IgAN) in children and their relationship. Methods The clinical manifestations, clinical types, pathological features and immunophenotype of 72 children diagnosed as IgAN by renal biopsy in our hospital from May 2005 to August 2011 were retrospectively reviewed and their correlations were analyzed relationship. Results The group of 72 cases. There were 48 males and 24 females. The patients were 1 year old and 5 months to 17 years old [(8.99 ± 2.94) years old). Their duration of admission was from 2 days to 9 years (average 12.86 months). Clinical onset of hematuria in 58 cases (including 38 cases of gross hematuria and 5 cases with edema), with simple edema onset in 12 cases, the onset of proteinuria in 2 cases. There were 28 cases (38.89%) with nephrotic syndrome, 19 cases (26.39%) with isolated hematuria, 13 cases (18.05%) with hematuria and proteinuria, 10 cases with acute nephritis (13.89%), Proteinuria in 2 cases (2.78%). Pathological changes: 40 cases of mesangial proliferative glomerulonephritis, 25 cases of focal proliferative nephritis, capillary proliferative nephritis in 6 cases, 1 case of crescentic nephritis. Among them, 17 cases (23.61%) had crescent body formation. Immunohistochemistry shows a variety of immunoglobulin deposits. The type of deposition was full bright type in 1 case, IgA + IgG + C32 cases, IgA in 8 cases, IgA + IgM + IgG + C3 in 17 cases, IgA + IgM + C3 in 44 cases. Conclusion The clinical manifestation of IgAN is varied, and the severity of IgAN has nothing to do with the onset of disease. The main pathological manifestations of mesangial proliferative glomerulonephritis, immunoglobulin deposition based composite type. Clinical manifestations of nephrotic syndrome and hematuria and proteinuria type pathological heavier kidney should be worn as soon as possible, and timely treatment.