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C1q或IgM肾病能否预测微小病变肾病综合征患儿的疾病严重程度?

Do C1q or IgM nephropathies predict disease severity in children with minimal change nephrotic syndrome?

作者信息

Vintar Spreitzer Mateja, Vizjak Alenka, Ferluga Dušan, Kenda Rajko B, Kersnik Levart Tanja

机构信息

Department of Pediatric Nephrology, University Medical Centre, Bohoričeva 20, 1000, Ljubljana, Slovenia.

出版信息

Pediatr Nephrol. 2014 Jan;29(1):67-74. doi: 10.1007/s00467-013-2551-3. Epub 2013 Jul 13.

Abstract

BACKGROUND

It has been suggested that C1q and immunoglobulin M (IgM) nephropathy are variants of minimal change nephrotic syndrome (MCNS). Many researchers believe that these two conditions signify a worse prognosis for children with MCNS in comparison with immunofluorescence (IF)-negative MCNS. The aim of our study was to determine the prognostic significance of C1q nephropathy and IgM nephropathy in children with MCNS.

METHODS

Fifty-five children with MCNS who had been biopsied over the course of 24 years at our institution were retrospectively categorized into three groups on the basis of IF microscopy findings: IF-negative MCNS (29/55 patients), MCNS with IgM nephropathy (19/55 patients), and MCNS with C1q nephropathy (7/55 patients). Clinical characteristics at disease presentation, clinical course, and renal outcome were compared between groups during the median follow-up period of 16.9 years (minimum 1.0, maximum 31.1 years).

RESULTS

No statistically significant differences in clinical characteristics at disease presentation, clinical course, and renal outcome were found. Children with IgM nephropathy, C1q nephropathy, and IF-negative MCNS were clinically indistinguishable.

CONCLUSIONS

We concluded that C1q or IgM nephropathy variants do not seem to signify a worse prognosis in children with MCNS in comparison with IF-negative MCNS.

摘要

背景

有人提出C1q和免疫球蛋白M(IgM)肾病是微小病变肾病综合征(MCNS)的变异型。许多研究人员认为,与免疫荧光(IF)阴性的MCNS相比,这两种情况表明MCNS患儿的预后更差。我们研究的目的是确定C1q肾病和IgM肾病在MCNS患儿中的预后意义。

方法

回顾性地将在我们机构24年间接受活检的55例MCNS患儿根据IF显微镜检查结果分为三组:IF阴性的MCNS(29/55例患者)、伴有IgM肾病的MCNS(19/55例患者)和伴有C1q肾病的MCNS(7/55例患者)。在16.9年的中位随访期(最短1.0年,最长31.1年)内,比较各组疾病表现时的临床特征、临床病程和肾脏结局。

结果

在疾病表现时的临床特征、临床病程和肾脏结局方面未发现统计学上的显著差异。伴有IgM肾病、C1q肾病和IF阴性的MCNS患儿在临床上难以区分。

结论

我们得出结论,与IF阴性的MCNS相比,C1q或IgM肾病变异型似乎并未表明MCNS患儿的预后更差。

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