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电子显微镜检查结果提示激素抵抗型肾病综合征患者存在局灶节段性肾小球硬化。

Electron microscopic findings suggestive of focal and segmental glomerulosclerosis in patients with steroid-resistant nephrotic syndrome.

作者信息

Nili Fatemeh, Saboori Fatemeh, Jahanzad Issa, Mehrazma Mitra

机构信息

a Department of Pathology , Imam Khomeini Hospital Complex, Tehran University of Medical Sciences , Tehran , I.R. Iran.

b Department of Pathology , Shahid Hasheminejad Hospital, Iran University of Medical Sciences , Tehran , I.R. Iran.

出版信息

Ultrastruct Pathol. 2019;43(1):6-12. doi: 10.1080/01913123.2019.1584258. Epub 2019 Mar 5.

Abstract

Distinction between minimal change disease and unsampled Focal Segmental Glomerulosclerosis is a challenging concept in kidney biopsy of patients with nephrotic syndrome with minimal histopathological findings. This study was performed to compare electron microscopic findings in patients with steroid-resistant nephrotic syndrome with minimal histopathological abnormalities and cases with Focal Segmental Glomerulosclerosis. This Cohort study was conducted in Cancer Institute, Imam Khomeini Hospital Complex, Tehran, Iran. Twenty patients with steroid-resistant nephrotic syndrome and minimal changes on the light microscopic study were selected as case group. Similarly, 20 patients with Focal Segmental Glomerulosclerosis were selected as the control group. Ultrastructural findings were re-evaluated and scored qualitatively (0-3+). In patients with minimal changes on light microscopic evaluation, clinical course of the disease was followed after 5 years. Mean ages of the patients (8 women and 12 men) in case and control groups were 12.9 and 15.9 years, respectively (p > 0.05). There was no significant difference in number of examined glomeruli and sampling from cortico-medullary junction area between the groups. The mean percentage of sclerotic glomeruli in control group was 15.4%. Tubular atrophy and interstitial fibrosis were more frequent in control patients. Podocyte proliferation, GBM duplication (involving more than 10% of capillary walls), and moderate to severe multifocal expansion of mesangial matrix were significantly more obvious in FSGS patient samples (p < 0.05). No statistically significant difference was found in severity of cytoplasmic vacuolization, GBM wrinkling and splitting between the groups. Most of (80%) the patients with minimal changes improved during the 5-year follow-up. Generally, we concluded that Podocyte proliferation, GBM remodeling, and moderate to severe mesangial matrix expansion are the most reliable findings on electron microscopic examination in favor of FSGS.

摘要

在组织病理学表现轻微的肾病综合征患者的肾活检中,区分微小病变病和未采样的局灶节段性肾小球硬化是一个具有挑战性的概念。本研究旨在比较组织病理学异常轻微的激素抵抗性肾病综合征患者与局灶节段性肾小球硬化患者的电子显微镜检查结果。这项队列研究在伊朗德黑兰伊玛目霍梅尼医院综合大楼的癌症研究所进行。选取20例激素抵抗性肾病综合征且光镜检查变化轻微的患者作为病例组。同样,选取20例局灶节段性肾小球硬化患者作为对照组。对超微结构检查结果进行重新评估并进行定性评分(0 - 3+)。对光镜评估变化轻微的患者,随访5年观察疾病的临床病程。病例组和对照组患者(8名女性和12名男性)的平均年龄分别为12.9岁和15.9岁(p > 0.05)。两组间检查的肾小球数量以及从皮质 - 髓质交界区采样情况无显著差异。对照组硬化肾小球的平均百分比为15.4%。对照组患者肾小管萎缩和间质纤维化更常见。足细胞增殖、肾小球基底膜重复(累及超过10%的毛细血管壁)以及系膜基质中度至重度多灶性扩张在局灶节段性肾小球硬化患者样本中明显更显著(p < 0.05)。两组间细胞质空泡化、肾小球基底膜皱缩和分裂的严重程度无统计学显著差异。大多数(80%)变化轻微的患者在5年随访期间病情改善。总体而言,我们得出结论,足细胞增殖、肾小球基底膜重塑以及中度至重度系膜基质扩张是电子显微镜检查中支持局灶节段性肾小球硬化的最可靠发现。

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