Activation of the alternative pathway of complement during the acute phase of typical haemolytic uraemic syndrome
Activation of the alternative pathway of complement during the acute phase of typical haemolytic uraemic syndrome
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DOI：
10.1111/cei.12601
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发表时间：
2015-07-01
影响因子：
4.6
通讯作者：
Mendez, C. F.
中科院分区：
文献类型：
--
作者：
Ferraris, J. R.;Ferraris, V.;Mendez, C. F.
溶血性尿毒综合征（HUS）的特征是溶血性贫血、血小板减少和急性肾衰竭。我们通过对18名患者和17名年龄匹配的健康对照进行前瞻性研究，以评估C3、C3 c、C4、C4d、Bb和SC 5 b-9水平，研究了滋贺毒素相关HUS急性期补体经典和旁路途径的激活状态。与健康和终末期肾病对照组相比，入院时所有患者的SC 5 b-9水平均显著升高，但与保留利尿的患者相比，少尿患者的SC 5 b-9水平显著升高。与对照组相比，非少尿组入院时C3和C4水平显著升高。C4d值无显著差异，而因子Bb在所有患者中升高，与对照组和非少尿个体相比，少尿患者中显著升高。当Bb形成作为入院时血浆SC 5 b-9的函数作图时，检测到正相关和显著相关。在第一周内，血脑屏障水平迅速下降，在第3天和第5天，非寡尿组和寡尿组的血脑屏障水平分别与对照组无显著差异。我们的数据表明在Stx相关HUS的急性期补体旁路途径的激活。这一发现表明，补体激活可能代表了在综合征期间发生的细胞损伤的重要触发因素。
Haemolytic uraemic syndrome (HUS) is characterized by haemolytic anaemia, thrombocytopenia and acute renal failure. We studied the activation state of classical and alternative pathways of complement during the acute phase of Shiga toxin-associated HUS by performing a prospective study of 18 patients and 17 age-matched healthy controls to evaluate C3, C3c, C4, C4d, Bb and SC5b-9 levels. SC5b-9 levels were increased significantly in all patients at admission compared to healthy and end-stage renal disease controls, but were significantly higher in patients presenting with oliguria compared to those with preserved diuresis. C3 and C4 levels were elevated significantly at admission in the non-oliguric group when compared to controls. No significant differences were found for C4d values, whereas factor Bb was elevated in all patients and significantly higher in oliguric patients when compared to both controls and non-oliguric individuals. A positive and significant association was detected when Bb formation was plotted as a function of plasma SC5b-9 at admission. Bb levels declined rapidly during the first week, with values not significantly different from controls by days 3 and 5 for non-oligurics and oligurics, respectively. Our data demonstrate the activation of the alternative pathway of complement during the acute phase of Stx-associated HUS. This finding suggests that complement activation may represent an important trigger for the cell damage that occurs during the syndrome.