Focal and segmental sclerosed lesions in the glomeruli are found in several pathological entities and more often are found in the corticomedullary junction where renal blood flow and filtration pressure is maximal. Experimental data suggest that hyperfiltration injury results in focal and segmental glomerulosclerosis (FSGS). In keeping with this concept, malignant hypertension is a known cause of nephrotic-range proteinuria and nephrotic syndrome pathalobically represented by FSGS. We report a case of unilateral renal artery stenosis associated with nephrotic syndrome and FSGS in the contralateral kidney only. The kidney with the stenosed renal artery showed normal glomeruli with juxtaglomerular hyperplasia, suggesting that protection from hyperfiltration injury was provided by the presence of high-grade stenosis. Serum creatinine concentration, blood pressure, and proteinuria normalized after aorto-renal bypass surgery. This case shows the importance of hemodynamic factors on the pathogenesis of secondary FSGS and the progression of renal disease.

译文

肾小球的局灶性和节段性硬化性病变在几种病理实体中发现,而在肾血流量和过滤压力最大的皮质管交界处更常见。实验数据表明,超滤损伤会导致局灶性和节段性肾小球硬化 (FSGS)。与这个概念保持一致,恶性高血压是由FSGS病理代表的肾病范围蛋白尿和肾病综合征的已知原因。我们报告了仅在对侧肾脏中与肾病综合征和FSGS相关的单侧肾动脉狭窄病例。肾动脉狭窄的肾脏显示正常的肾小球并伴有近肾小球增生,这表明高级别狭窄的存在可保护高滤过损伤。主动脉-肾搭桥手术后血清肌酐浓度,血压和蛋白尿正常化。该病例显示了血流动力学因素对继发性FSGS发病机理和肾脏疾病进展的重要性。

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