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

在几个病理实体中发现了肾小球的局灶性和节段性硬化病变,更常见于肾肾血流和滤过压力最大的皮质肾交界处。实验数据表明,超滤损伤可导致局灶性和节段性肾小球硬化(FSGS)。与这个概念一致,恶性高血压是FSGS病态性代表的肾病范围蛋白尿和肾病综合征的已知病因。我们报道了仅在对侧肾脏中伴有肾病综合征和FSGS的单侧肾动脉狭窄的病例。肾动脉狭窄的肾脏显示肾小球正常,并伴有肾小球增生,提示存在高度狭窄可防止过度滤过损伤。主动脉-肾脏搭桥手术后,血清肌酐浓度,血压和蛋白尿恢复正常。该病例表明血液动力学因素对继发性FSGS的发病机理和肾脏疾病进展的重要性。

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