儿童IgA肾病的临床与病理分析

Analysis of clinicopathological features of IgA nephropathy in 37 children

目的探讨儿童IgA肾病(IgAN)的临床与病理特点及相关关系.方法对我院2000年2月～2005年2月经肾穿刺活检确诊为IgAN的37例患儿的临床表现,肾组织光镜、免疫组化及相关实验室检查进行回顾性分析总结.结果IgAN发病年龄(9.8±3.8)岁,男:女为3.11:1.37例中,临床表现为孤立性血尿11例(29.73%),肾病综合征(NS)10例(27.03%),急性肾小球肾炎(AGN)9例(24.32%),持续性血尿蛋白尿5例(13.51%),孤立性蛋白尿,急性进行性肾小球肾炎(RPGN)各1例(各2.70%).病理表现为系膜增生性肾炎(MsPGN)33例(89.19%),肾小球轻微病变(ML),毛细血管内增生性肾炎(EnPGN),快速进行性肾小球肾炎(RPGN),硬化性肾小球肾炎(SGN)各1例(各2.70%).免疫组化可见多种免疫球蛋白沉积.沉积类型为IgA+M+G(+C3)型14例(37.84%),IgA型13例(35.14%),IgA+M(+C3)型10例(27.03%).结论儿童IgA肾病以学龄期儿童多见,男性明显多于女性.临床表现多样,以孤立性血尿,肾病综合征,急性肾小球肾炎多见.病理表现以MsPGN为主.临床表现与免疫病理分型相关,与肾小球病理损害程度无相关性.

Objective To explore the clinical and pathological features and their relations of IgA nephropathy（IgAN） in children. Methods A retrospective study of 37 children with IgAN by renal biopsy from 2000.2 to 2005.2 was conducted, including their clinical and pathological features, immunohistochemical and laboratorial examinations. Results The average age of initial onset was 9.8±3.8 years . The ratio of boys to girls was 3. 11 ： 1. The clinical manifestation included isolated hematuria in 11 cases, nephrotic syndrome（NS） in 10, acute glomerulonephritis（AGN） in 9, durative hematuria and proteinuria in 5,isolated proteinuria and rapidly progressive glomerulonephritis（RPGN） in 1 respectively. The pathological classification included mesangial proliferative glomerulonephritis （MsPGN）in 33 cases, glomerular minor lesion（MI.）, endocapilary proliferative glomerulonephritis（EnPGN）, rapidly progressive glomerulonephritis（RPGN）, sclerosing glomerulonephritis（SGN） in 1 respectively. Immunohistoehemieal results showed multiple immunoglobulin deposits. The deposits included IgA＋M＋G（＋C3）in 14 cases, IgA in 13, IgA＋M （＋C3a）in10. Conclusion IgAN is common in school-aged children, boys far more than girls . There are various clinical presentations while isolated hematuria, NS and AGN are common. The main pathological presentation is MsPGN. Clinical manifestation is correlated with immunopathological changes,instead of glomerulus changes.