KIDNEY INT:高血压相关的血栓性微血管病患者可出现补体异常!

2017-02-14 xing.T MedSci原创

对于严重高血压患者和肾脏活检证实为TMA的患者进行遗传性补体异常的筛查是有必要的,以为患者采取合适的治疗方法和预防措施。

近日,肾脏病领域权威杂志kidney international上发表了一篇研究文章,研究人员在Steno-2研究的事后分析中,分析了强化的综合治疗对2型糖尿病合并微量白蛋白尿患者肾脏功能预后的影响。

血栓性微血管病(TMA)是一种内皮细胞损伤性疾病,可以发生在不同的临床疾病中,如恶性高血压。虽然病理生理机制有所不同,但是越来越多的证据提示补体功能失调参与了各种TMA,尤其是非典型溶血尿毒综合征。近日,肾脏病领域权威杂志kidney international上发表了一篇研究文章,研究人员评估了连续就诊的九例经活检证实为肾脏TMA严重的高血压患者补体系统在疾病发生发展中的作用。

典型的TMA血液症状并不常见。在纳入研究的九例患者中的六例患者研究人员发现了有3例患者存在C3突变,一例患者存在CFI突变,一例患者存在CD46突变和/或两例患者存在CFH突变,在四例患者存在或无CFH-H3单倍体风险。可溶性C5b-9以及肾脏沿血管和/或肾小球毛细血管壁C3c和C5b-9积聚水平升高,证实了体内补体激活。与没有遗传缺陷的患者相反,补体缺陷的患者一成不变地进展为终末期肾脏疾病,并且在肾脏移植后的疾病复发似乎很常见。因此,高血压相关的TMA亚组患者在补体介导的TMA有所下降,但其预后差。

由此可见,对于严重高血压患者和肾脏活检证实为TMA的患者进行遗传性补体异常的筛查是有必要的,以为患者采取合适的治疗方法和预防措施。

原始出处:
Sjoerd A.M.E.G. Timmermans, et al. Patients with hypertension-associated thrombotic microangiopathy may present with complement abnormalities. kidney international.2017;  http://dx.doi.org/10.1016/j.kint.2016.12.009

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    2017-02-16 mgqwxj
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    2017-02-16 gwc389
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    2017-02-14 tanxingdoctor

    不错,谢谢分享!

    0

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