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儿童肾病综合征肾小球糖皮质激素受体表达与临床病理类型

Glomerular Glucocorticoid Receptors Expression and Clinicopathological Types of Childhood Nephrotic Syndrome.

作者信息

Gamal Yasser, Badawy Ahlam, Swelam Salwa, Tawfeek Mostafa S K, Gad Eman Fathalla

机构信息

a Pathology Department , Assiut University Faculty of Medicine , Assiut , Egypt.

b Pediatric Department , Assiut University Faculty of Medicine , Assiut , Egypt.

出版信息

Fetal Pediatr Pathol. 2017 Feb;36(1):16-26. doi: 10.1080/15513815.2016.1225872. Epub 2016 Oct 3.

DOI:10.1080/15513815.2016.1225872
PMID:27690709
Abstract

Glucocorticoids are primary therapy of idiopathic nephrotic syndrome (INS). However, not all children respond to steroid therapy. We assessed glomerular glucocorticoid receptor expression in fifty-one children with INS and its relation to response to steroid therapy and to histopathological type. Clinical, laboratory and glomerular expression of glucocorticoid receptors were compared between groups with different steroid response. Glomerular glucocorticoid expression was slightly higher in controls than in minimal change early responders, which in turn was significantly higher than in minimal change late responders. There was significantly lower glomerular glucocorticoid receptor expression in steroid-resistance compared to early responders, late responders and controls. Glomerular glucocorticoid expression was significantly higher in all minimal change disease (MCD) compared to focal segmental glomerulosclerosis. In INS, response to glucocorticoid is dependent on glomerular expression of receptors and peripheral expression. Evaluation of glomerular glucocorticoid receptor expression at time of diagnosis of NS can predict response to steroid therapy.

摘要

糖皮质激素是特发性肾病综合征(INS)的主要治疗方法。然而,并非所有儿童对类固醇治疗都有反应。我们评估了51例INS患儿的肾小球糖皮质激素受体表达及其与类固醇治疗反应和组织病理学类型的关系。比较了不同类固醇反应组之间的临床、实验室指标及糖皮质激素受体的肾小球表达情况。对照组的肾小球糖皮质激素表达略高于微小病变早期反应者,微小病变早期反应者又显著高于微小病变晚期反应者。与早期反应者、晚期反应者和对照组相比,类固醇抵抗组的肾小球糖皮质激素受体表达显著降低。与局灶节段性肾小球硬化相比,所有微小病变疾病(MCD)的肾小球糖皮质激素表达显著更高。在INS中,对糖皮质激素的反应取决于受体的肾小球表达和外周表达。在诊断NS时评估肾小球糖皮质激素受体表达可预测对类固醇治疗的反应。

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