糖皮质激素受体在儿童激素耐药性肾病综合征中的低表达。

Low expression of glucocorticoid receptors in children with steroid-resistant nephrotic syndrome.

机构信息

Department of Pediatrics, Faculty of Medicine, Mansoura University, Mansoura, Egypt.

出版信息

Pediatr Nephrol. 2013 May;28(5):759-63. doi: 10.1007/s00467-012-2385-4. Epub 2013 Jan 9.

DOI:10.1007/s00467-012-2385-4

PMID:23299233

Abstract

BACKGROUND

About 10-20 % of children with idiopathic nephrotic syndrome (NS) are steroid-resistant (SR). Low expression of glucocorticoid receptors (GRs) has been associated with poor response to steroids in a variety of autoimmune diseases. This study was done to assess the expression of cytoplasmic GRs for CD3 and CD14 in children with NS.

METHODS

Expression of cytoplasmic GRs in lymphocytes (CD3(+)/GR) and monocytes (CD14(+)/GR) in the peripheral blood were assessed in 51 children with NS before the start of therapy by flow cytometry. Patients were divided into two groups: 30 children who were steroid-sensitive (SSNS) and 21 children who had initial steroid resistance (SRNS). Twenty age- and sex-matched healthy children served as controls.

RESULTS

Expression of CD3(+)/GR was significantly lower in SRNS in comparison to SSNS patients and controls (p < 0.0001). Similarly, expression of CD14(+)/GR was significantly lower in SRNS in comparison to SSNS patients (p < 0.0001) and controls (p = 0.002). CD3(+)/GR and CD14(+)/GR expression were not significantly different in SSNS patients compared with controls (p = 0.06 and 0.07 respectively).

CONCLUSIONS

Patients with initial SRNS showed decreased GR expression in peripheral blood mononuclear cells (PBMC) before starting therapy, and this low expression may be one of the pathophysiological mechanisms of steroid resistance in these children.

摘要

背景

约 10-20%的特发性肾病综合征（NS）患儿对类固醇治疗无反应（SR）。糖皮质激素受体（GRs）的低表达与多种自身免疫性疾病中对类固醇的反应不佳有关。本研究旨在评估 NS 患儿外周血淋巴细胞（CD3(+)/GR）和单核细胞（CD14(+)/GR）中细胞质 GR 的表达。

方法

通过流式细胞术评估 51 例 NS 患儿在开始治疗前外周血中淋巴细胞（CD3(+)/GR）和单核细胞（CD14(+)/GR）中细胞质 GR 的表达。患者分为两组：30 例为激素敏感型（SSNS），21 例为初发激素抵抗型（SRNS）。20 名年龄和性别匹配的健康儿童作为对照组。

结果

与 SSNS 患儿和对照组相比，SRNS 患儿的 CD3(+)/GR 表达显著降低（p<0.0001）。同样，与 SSNS 患儿（p<0.0001）和对照组（p=0.002）相比，SRNS 患儿的 CD14(+)/GR 表达也显著降低。与对照组相比，SSNS 患儿的 CD3(+)/GR 和 CD14(+)/GR 表达无显著差异（p=0.06 和 0.07）。