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微小病变型肾病综合征和局灶节段性肾小球硬化症患儿肾小球上皮细胞空泡化和足细胞足突消失的诊断及预后意义:一项超微结构研究

Diagnostic and prognostic significance of glomerular epithelial cell vacuolization and podocyte effacement in children with minimal lesion nephrotic syndrome and focal segmental glomerulosclerosis: an ultrastructural study.

作者信息

Chiang M L, Hawkins E P, Berry P L, Barrish J, Hill L L

机构信息

Department of Pediatric Nephrology, Baylor College of Medicine, Houston, TX 77030.

出版信息

Clin Nephrol. 1988 Jul;30(1):8-14.

PMID:3061695
Abstract

Children with minimal lesion nephrotic syndrome (MLNS) may later develop focal segmental glomerulosclerosis (FSGS). It has been suggested that a low percentage of epithelial podocyte effacement (EPE) and a high degree of epithelial cell vacuolization (ECV) in nonsclerotic glomeruli presage FSGS, and that extensive epithelial cell vacuolization in biopsies clearly showing FSGS predicts a poor clinical outcome. To investigate these contentions, we examined by electron microscopy three glomeruli from each of the first biopsies of 30 patients. Ten patients (group 1) had MLNS, 10 (group 2) had FSGS, and 10 (group 3) had MLNS which progressed to FSGS. Clinical data was obtained by retrospective review of medical records. The percent of epithelial podocyte effacement was calculated by computerized linear tracing and epithelial cell vacuolization was scored semiquantitatively from 0-3. (formula; see text) The percent podocyte effacement in each group was the same and does not distinguish MLNS from FSGS. Group 2 had more extensive epithelial cell vacuolization than group 1 (p less than 0.04) and the same as group 3 (p = 0.16). The combined ECV score for groups 2 and 3, however, was significantly greater than for group 1 (p less than 0.025) suggesting that epithelial cell vacuolization may indeed be a marker of FSGS. The extent of epithelial cell vacuolization did not correlate with creatinine clearance at latest follow-up, and thus does not predict clinical outcome.

摘要

微小病变型肾病综合征(MLNS)患儿日后可能会发展为局灶节段性肾小球硬化(FSGS)。有人提出,在非硬化性肾小球中,上皮足细胞足突消失(EPE)的比例较低和上皮细胞空泡化(ECV)程度较高预示着FSGS,并且在活检中显示为FSGS的广泛上皮细胞空泡化预示着临床预后不良。为了研究这些观点,我们通过电子显微镜检查了30例患者首次活检时的每个患者的3个肾小球。10例患者(第1组)患有MLNS,10例(第2组)患有FSGS,10例(第3组)患有进展为FSGS的MLNS。通过回顾病历获得临床数据。上皮足细胞足突消失的百分比通过计算机化线性追踪计算,上皮细胞空泡化进行0-3分的半定量评分。(公式;见正文)每组中足细胞足突消失的百分比相同,无法区分MLNS和FSGS。第2组的上皮细胞空泡化比第1组更广泛(p小于0.04),与第3组相同(p = 0.16)。然而,第2组和第3组的ECV综合评分显著高于第1组(p小于0.025),这表明上皮细胞空泡化可能确实是FSGS的一个标志物。上皮细胞空泡化的程度与最近一次随访时的肌酐清除率无关,因此不能预测临床预后。

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