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Evidence for shrunken pore syndrome in children.
Scandinavian Journal of Clinical and Laboratory Investigation ( IF 2.1 ) Pub Date : 2019-11-22 , DOI: 10.1080/00365513.2019.1692231
Emil den Bakker 1 , Reinoud Jbj Gemke 1 , Joanna Ae van Wijk 1 , Isabelle Hubeek 2 , Birgit Stoffel-Wagner 3 , Arend Bökenkamp 1
Affiliation  

The link between cystatin C and mortality independent of glomerular filtration rate (GFR) in adults has prompted the "Shrunken Pore Syndrome" (SPS) hypothesis, where high serum cystatin C with normal creatinine is explained by smaller glomerular pores, through which creatinine can pass freely, while the larger cystatin C, beta-trace protein (BTP) and pro-inflammatory molecules are retained. This study set out to apply the definition of SPS to children. In 294 children who underwent inulin clearance (Cin) test, serum creatinine, cystatin C and BTP were measured. For all three markers eGFRx was calculated using the full age spectrum equations. The ratio eGFRcys/eGFRcrea was plotted against the error of eGFRBTP(%) (i.e. eGFRBTP-Cin)/Cin*100%). Patients with and without SPS according to different cut-off points of eGFRcys/eGFRcrea and eGFRcys/Cin (i.e. ≤0.6,0.7,0.8) were compared in terms of eGFRx, Cin, error of eGFRx(%) and eGFRBTP/eGFRcrea-ratio. The ratio eGFRcys/eGFRcrea and error of eGFRBTP(%) were positively correlated. The prevalence of SPS by eGFRcys/eGFRcrea with a cut-off of 0.6 was 4.8%. Patients with SPS had a more negative error of eGFRcys(%) and eGFRBTP(%) and higher Cin regardless of the definition. Overestimation of eGFRcrea in patients with SPS was only present when using the eGFRcys/eGFRcrea rather than the eGFRcys/Cin definition. Cystatin C and BTP are related independent of creatinine, suggesting glomerular pore size as a common denominator. The prevalence of SPS in children is comparable to adults. For research in SPS, a definition based on eGFRcys/exogenous clearance study may be useful to study the effect of SPS on creatinine metabolism.

中文翻译:

儿童收缩毛孔综合症的证据。

半胱氨酸蛋白酶抑制剂C和死亡率与成年人的肾小球滤过率(GFR)无关,这提示了“收缩毛孔综合症”(SPS)假说,其中较高的血清胱抑素C和正常肌酐可以通过较小的肾小球毛孔来解释,肌酐可以通过自由地保留较大的cystatin C,β-迹蛋白(BTP)和促炎分子。该研究旨在将SPS的定义应用于儿童。在294名接受菊粉清除率(Cin)测试的儿童中,测量了血清肌酐,胱抑素C和BTP。对于所有三个标记,使用完整的年龄谱方程计算eGFRx。将比率eGFRcys / eGFRcrea与eGFRBTP(%)的误差(即eGFRBTP-Cin)/ Cin * 100%)作图。根据eGFRx,ein,eGFRx(%)和eGFRBTP / eGFRcrea-ratio的eGFRx,Cin,eGFRx(%)的误差比较了根据eGFRcys / eGFRcrea和eGFRcys / Cin(即≤0.6、0.7、0.8)的不同临界点有无SPS的患者。eGFRcys / eGFRcrea的比率与eGFRBTP(%)的误差呈正相关。eGFRcys / eGFRcrea的SPS发生率为0.6%,为0.6。无论定义如何,SPS患者的eGFRcys(%)和eGFRBTP(%)的负误差更大,Cin更高。仅当使用eGFRcys / eGFRcrea而不是eGFRcys / Cin定义时,才出现SPS患者中eGFRcrea的高估。胱抑素C和BTP的相关性与肌酐无关,提示肾小球孔径是一个共同的指标。儿童中SPS的患病率与成人相当。对于SPS的研究,
更新日期:2019-11-01
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