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儿童IgM肾病:与微小病变病的比较。

Childhood IgM nephropathy: comparison with minimal change disease.

作者信息

Al-Eisa A, Carter J E, Lirenman D S, Magil A B

机构信息

Department of Pediatrics, University of British Columbia, Vancouver, Canada.

出版信息

Nephron. 1996;72(1):37-43. doi: 10.1159/000188804.

Abstract

The distinctiveness of IgM nephropathy (IgMN) as a clinicopathologic entity is controversial. Twenty-seven children (16 males, 11 females) with IgMN as defined immunohistochemically by diffuse mesangial staining of glomeruli for IgM were compared to a group of 63 children (40 males, 23 females) with minimal change disease (MCD). While mesangial expansion was significantly greater in IgMN than in MCD (p = 0.0014), there were no significant differences between the two groups with respect to the other biopsy factors. IgMN showed a significantly higher incidence of hypertension at presentation. More than 90% of patients in both groups presented with the nephrotic syndrome which in most initially responded to prednisone. Frequently relapsing/steroid-dependent nephrotic syndrome was the most common indication for biopsy in both groups. Approximately 60% of patients from both groups received cytotoxic therapy. Eight percent of IgMN and 7% of MCD patients failed to respond to therapy. Relapse rates and mean dose of prednisone at relapse were very similar in both groups prior to biopsy. Relapse rates diminished significantly after treatment in the postbiopsy interval, but mean dose of prednisone at relapse did not change appreciably over time. None of the patients developed renal failure or hypertension in the follow-up period. At last visit 23% of IgMN and 27% of MCD had proteinuria. The results indicate that IgMN and MCD are indistinguishable clinically in children who are biopsied for the nephrotic syndrome.

摘要

IgM肾病(IgMN)作为一种临床病理实体的独特性存在争议。将27名经免疫组织化学定义为肾小球IgM弥漫性系膜染色的IgMN患儿(16名男性,11名女性)与一组63名微小病变病(MCD)患儿(40名男性,23名女性)进行比较。虽然IgMN的系膜扩张明显大于MCD(p = 0.0014),但两组在其他活检因素方面没有显著差异。IgMN在就诊时高血压的发生率显著更高。两组中超过90%的患者表现为肾病综合征,大多数最初对泼尼松有反应。频繁复发/激素依赖型肾病综合征是两组活检最常见的指征。两组中约60%的患者接受了细胞毒性治疗。8%的IgMN患者和7%的MCD患者对治疗无反应。活检前两组的复发率和复发时泼尼松的平均剂量非常相似。活检后的随访期间治疗后复发率显著降低,但复发时泼尼松的平均剂量随时间没有明显变化。随访期间没有患者出现肾衰竭或高血压。在最后一次就诊时,23%的IgMN患者和27%的MCD患者有蛋白尿。结果表明,在因肾病综合征接受活检的儿童中,IgMN和MCD在临床上难以区分。

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