Divisions of Kidney Diseases, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Clin J Am Soc Nephrol. 2013 Aug;8(8):1304-11. doi: 10.2215/CJN.07680712. Epub 2013 Apr 25.
FSGS is the primary cause of childhood nephrotic syndrome leading to ESRD. Permeability factors, including circulating serum soluble urokinase-type plasminogen activator receptor (suPAR), have been postulated as putative causes in adults with primary FSGS. Similar results have yet to be proven in children.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This cross-sectional single-center study assessed the association of serum suPAR in children with FSGS or other glomerular and nonglomerular kidney diseases.
This study examined 110 samples retrieved from 99 individuals (between January 2011 and April 2012), aged 1-21 years; of these individuals, 20 had primary FSGS, 24 had non-FSGS glomerular disease, 26 had nonglomerular kidney disease, and 29 were healthy controls. suPAR levels were not significantly different in children with FSGS, non-FSGS glomerular disease, and healthy controls (P>0.05). However, suPAR levels (median [25%-75%]) were higher in children with nonglomerular kidney disease (3385 pg/ml [2695-4392]) versus FSGS (2487 pg/ml [2191-3351]; P<0.05). Female patients with nephrotic-range proteinuria (U-Pr/Cr >2) had lower suPAR levels than those without proteinuria (2380 pg/ml [2116-2571] versus 3125 pg/ml [2516-4198], respectively; P<0.001). This trend was not seen among male participants; suPAR levels in all female participants were lower than in male participants (P=0.03). Thirty-four patients studied were kidney transplant recipients; transplant status was not associated with suPAR levels in patients with FSGS or non-FSGS diagnoses, independent of proteinuria, race, or sex (P>0.05).
On the basis of these results, circulating suPAR is unlikely the leading cause for childhood idiopathic FSGS.
局灶节段性肾小球硬化症（FSGS）是导致儿童肾病综合征终末期肾衰竭的主要原因。循环血清可溶性尿激酶型纤溶酶原激活物受体（suPAR）等通透性因子被认为是成人原发性 FSGS 的潜在病因。但类似的结果尚未在儿童中得到证实。
设计、地点、参与者和测量方法：本横断面单中心研究评估了血清 suPAR 与 FSGS 或其他肾小球和非肾小球肾脏疾病患儿的相关性。
本研究共检测了 110 例来自 99 名个体（2011 年 1 月至 2012 年 4 月）的样本，年龄 1-21 岁；其中 20 例为原发性 FSGS，24 例为非 FSGS 肾小球疾病，26 例为非肾小球肾脏疾病，29 例为健康对照。FSGS、非 FSGS 肾小球疾病和健康对照组患儿的 suPAR 水平无显著差异（P>0.05）。然而，非肾小球肾脏疾病患儿的 suPAR 水平（中位数[25%-75%]）明显高于 FSGS 患儿（3385pg/ml [2695-4392] vs. 2487pg/ml [2191-3351]；P<0.05）。伴有肾病范围蛋白尿（U-Pr/Cr>2）的女性患儿的 suPAR 水平低于无蛋白尿患儿（2380pg/ml [2116-2571] vs. 3125pg/ml [2516-4198]；P<0.001）。这一趋势在男性参与者中并未出现；所有女性参与者的 suPAR 水平均低于男性参与者（P=0.03）。34 例研究对象为肾移植受者；FSGS 或非 FSGS 患者的移植状态与 suPAR 水平无关，与蛋白尿、种族或性别无关（P>0.05）。
基于这些结果，循环 suPAR 不太可能是导致儿童特发性 FSGS 的主要原因。
