Kidney Int:妊娠相关非典型溶血性尿毒症综合征知多少?

2017-09-21 MedSci MedSci原创

妊娠相关的非典型溶血性尿毒症综合征(aHUS)是指在怀孕期间或产后,由不受控制的补体激活引起的血栓性微血管病变。妊娠相关的aHUS是一种严重的疾病,目前对这种疾病的临床理解和治疗经验十分有限。近期,一项发表在杂志Kidney Int上的回顾性研究分析了来自西班牙aHUS注册处不同治疗方法的22例妊娠相关aHUS患者的临床资料和预后资料。在第一次妊娠期间有16例患者出现了妊娠相关aHUS,多达9名患

妊娠相关的非典型溶血性尿毒症综合征(aHUS)是指在怀孕期间或产后,由不受控制的补体激活引起的血栓性微血管病变。妊娠相关的aHUS是一种严重的疾病,目前对这种疾病的临床理解和治疗经验十分有限。


近期,一项发表在杂志Kidney Int上的回顾性研究分析了来自西班牙aHUS注册处不同治疗方法的22例妊娠相关aHUS患者的临床资料和预后资料。

在第一次妊娠期间有16例患者出现了妊娠相关aHUS,多达9名患者在诊断时需要血液透析。22例患者中有9例出现遗传性补体异常,其中CFH突变以及CFH和CFHR1基因转换是与该病相关的最常见遗传改变(66%)。

在13例患者中,不存在遗传或获得性补体改变的情况下,妊娠并发症足以引起血栓性微血管病变。

产后时间是该病发生风险最高的时期,此项研究显示了剖腹产与妊娠相关性aHUS之间具有一定的相关性。17例接受血浆治疗的患者中仅有3例出现肾脏阳性反应。相比之下,无论有无携带遗传性补体异常,10名接受了依库丽单抗(eculizumab)治疗的患者都出现了非常好的肾脏反应。

此项研究数据表明妊娠相关aHUS与其他类型的aHUS没有差异,并表明了eculizumab治疗优于血浆治疗的疗效。

原始出处:
Huerta A, Arjona E, et al. A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome. Kidney Int. 2017 Sep 11. pii: S0085-2538(17)30482-9. doi: 10.1016/j.kint.2017.06.022.

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    2017-09-23 tcm99hq
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    2017-09-23 gwc389
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    2017-09-22 1ddf0692m34(暂无匿称)

    学习了.涨知识

    0

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