37例儿童IgA肾病临床与病理分析

Clinical and pathological features of 37 children with IgA nephropathy

为了探讨IgA肾病的临床与病理改变的关系,对37例IgA肾病进行临床分型并与肾小球、肾小管间质改变及免疫病理特点的关系进行比较。结果:临床分型中单纯血尿(血尿)18例占49％,肾病综合征(肾病)14例占38％,血尿和蛋白尿3例占8％,肾炎综合征(肾炎)2例占5％。肾小球病理损害以Ⅲ级为主占54％,临床各型与肾小球病理损害无相关性。肾小管间质改变24例,血尿组7例占39％,其中Ⅰ级为43％,Ⅱ级为57％。肾病组均有改变,其中Ⅱ级11例占78％,Ⅲ级3例占22％。血尿和蛋白尿组2例占66％。肾炎组1例占50％。免疫病理改变为IgA 16例,IgAG 6例,IgAM 10例,IgAGM 5例。血尿组以单纯IgA沉积为主占66％,肾病组则以IgAM型为主占50％。提示IgA肾病临床以单纯血尿为主,其次为肾病综合征;肾小球病理损害程度与临床分型无相关性,但肾病组肾小管间质均有改变且程度也较血尿组为重。免疫病理血尿组以单纯IgA为主,而肾病组以IgAM为主。

To explore the relationship between clinical findings and pathological changes of IgA nephropathy (IgAN), 37 children with IgAN were undertaken clinical classification and renal-pathological comparison including glomerulus change, renal tubule-interstitial change and immunopathology. The results showed that there were 18 (49%) cases with hematuria, 14 (38%) cases with nephrotic syndrome, 3(8%) cases with both of hematuria and proteineuria, and 2 (5%) cases with nephritic syndrome in the clinical classification. 54% of cases with glomerulus changes was as class Ⅲ. No significant relationship was found between clinical classification and glomerulus changes. There were 24 cases with renal-tubule interstitial changes and 7 cases with hematuria. 43% of them were classified as class Ⅰ and 57% as class Ⅱ. All cases with nephrotic syndrome developed renal tubule-interstitial change. 78% (11 cases) of them were as class Ⅱ and 22% (3 cases) as class Ⅲ. Besides, 2 of 3 cases with both of hematuria and proteineuria and 1 of 2 cases with nephritic syndrome also manifested renal tubule-interstitial change. There were four phenotypes were observed in immunopathology including 16 cases of them as IgA, 6 cases as IgAG, 10 cases as IgAM and 5 cases as IgAGM, respectively. 66% of cases with hematuria was found as IgA and 50% of cases with nephrotic syndrome as IgAM. It is concluded that hematuria can be a main clinical finding in both of IgAN and nephrotic syndrome. Glomerulus changes is usually not correlated with clinical classification. There is a significant renal tubule-interstitial change in cases with nephrotic syndrome. Hematuria is usually as IgA and nephrotic syndrome as IgAM in immunopathology.