IgM肾病临床与病理特点

目的探讨小儿ＩｇＭ肾病（ＩｇＭＮ）临床与病特点及疗效观察。方法对原发性肾小球疾病（ＰＧＤ）肾活检符合ＩｇＭＮ诊断７２５例进行临床、病理及肾上腺皮质激素疗效分析，并与微小病变肾病（ＭＣＮＳ）比较。结果ＩｇＭＮ６１例，表现肾病综合征（ＮＳ）４９例，血尿伴蛋白尿４例及单纯性血尿８例。肾组织免疫荧光（ＩＦ）均以ＩｇＭ单独或ＩｇＭ为主在系膜区弥漫沉积。随访５７例，ＮＳ４５例，７７．８％激素敏感，低于ＭＣＮＳ

The clinical and pathologic features of IgM nephropathy in chil-dren

Objective To investigate the clinical and pathologic features and to observe the effectiveness of adrenocortical hormone for IgMN in children.Methods The clinical and pathologic features were observed and effectiveness of adrenocortical hormone was analyzed,as compared with minute change nephritic syndrome(MCNS), diagnosed by renal biopsy in 725 cases of primary glomerular disease.Result Of 61 cases of IgMN, nephritic syndrome (NS) was shown in 49 cases, 8 cases of simple hematuria and 4 cases of hematuria with proteiuria,which was diffusedly deposited in mesenteric area by immunofluorescence (IF). 57 cases were followed up,the hormone sensitive rate being 77.8 % in 45 NS cases,which was lower than that in MCNS(100 %),the rate of RNS being 57.8 %, which was higher than that in MCNS. The total ratio of remission and the ratio of relapse were 76.3 % and 48.3 % respectively.There was no significant difference compared with 94.4 % and 66.7 % in MCNS.In 12 cases of non-NS with hematuria,diffuse thin basement membrane(DTBM) was observed in 4 cases.None of them developed renal failure for the following 3 years.Conclusion In this group the IgMN accounted for 8.4 % of patients in PGD,and the main manifestations of IgMN were NS (80.3 %).The pathologic features of IgMN was that IgMN deposited diffusely in mesenteric area by IF.The clinical and laboratory findings of IgMN were not significantly different from that of MCNS.The effectiveness of adrenocortical hormone for IgMN was less than that for MCNS and partly with DTBM.