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Kidney int：H因子自身抗体与儿童非典型溶血性尿毒症综合征有关！

2017-08-06 MedSci MedSci原创

H因子自身抗体非典型溶血性尿毒症综合征
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既往有研究表明，H因子自身抗体可以损害补体调节，导致非典型溶血性尿毒症综合征，尤其在儿童时期。但是，目前尚无试验评估对其的治疗，临床实践也只是通过回顾性队列分析进行。近期，一项发表在杂志Kidney int上的研究评估了175名英国和爱尔兰呈现非典型溶血性尿毒症综合征的儿童的H因子自身抗体，其中包括17名高于国际标准滴度的儿童。在17名患儿中，7名儿童在编码补体途径成分或调节物的基因中具有伴随的稀

既往有研究表明，H因子自身抗体可以损害补体调节，导致非典型溶血性尿毒症综合征，尤其在儿童时期。但是，目前尚无试验评估对其的治疗，临床实践也只是通过回顾性队列分析进行。近期，一项发表在杂志Kidney int上的研究评估了175名英国和爱尔兰呈现非典型溶血性尿毒症综合征的儿童的H因子自身抗体，其中包括17名高于国际标准滴度的儿童。在17名患儿中，7名儿童在编码补体途径成分或调节物的基因中具有伴随的稀有遗传变体。两名儿童得到支持治疗；但两者均发展成肾衰竭。11例治疗组中，7例进行血浆交换的患儿与肾脏恢复率差相关。使用eculizumab治疗的6例患者恢复肾功能。与全球实践相反，由于对治疗相关并发症的关注，不采用免疫抑制治疗预防血浆置换治疗的患者出现复发。没有免疫抑制治疗的复发率高（七分之五）。然而，重新使用治疗仍然可以恢复肾功能。所有用eculizumab治疗的患者均获得持续缓解。5例无特异性H因子自身抗体靶向治疗的患者接受肾移植出现复发，其中还有1例患者具有功能重要的CFI突变。此项研究的做法是启动eculizumab治疗H因子自身抗体介导的非典型溶血性尿毒症综合征，而不是进行非免疫抑制

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    2017-08-08 tcm99hq

    #尿毒症#

    0

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    2017-08-08 zhouqu_8

    #综合征#

    0

  3. 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    2017-08-08 sjzlzc010

    #非典型#

    0

  4. 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createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzaiabIFicf63hKvVMjL6aJlx4yX4UcXmzX9DyibCRcicq0QtCRWMaQKh8MYjE1D3EKUBopgiaGWYuqbeR/0, createdBy=0c2b1999806, createdName=三生有幸9135, createdTime=Sun Aug 06 15:54:05 CST 2017, time=2017-08-06, status=1, ipAttribution=)]
    2017-08-08 bioon5

    #非典型溶血性尿毒症综合征#

    0

  5. 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createdTime=Sun Aug 06 19:59:39 CST 2017, time=2017-08-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=230794, encodeId=0d9a230e94a2, content=谢谢分享学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=69, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Sun Aug 06 19:19:48 CST 2017, time=2017-08-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=230734, encodeId=7a20230e34a9, content=学习一下谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzaiabIFicf63hKvVMjL6aJlx4yX4UcXmzX9DyibCRcicq0QtCRWMaQKh8MYjE1D3EKUBopgiaGWYuqbeR/0, createdBy=0c2b1999806, createdName=三生有幸9135, createdTime=Sun Aug 06 15:54:05 CST 2017, time=2017-08-06, status=1, ipAttribution=)]
    2017-08-08 gwc389

    #kidney#

    0

  6. 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createdTime=Sun Aug 06 19:59:39 CST 2017, time=2017-08-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=230794, encodeId=0d9a230e94a2, content=谢谢分享学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=69, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Sun Aug 06 19:19:48 CST 2017, time=2017-08-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=230734, encodeId=7a20230e34a9, content=学习一下谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzaiabIFicf63hKvVMjL6aJlx4yX4UcXmzX9DyibCRcicq0QtCRWMaQKh8MYjE1D3EKUBopgiaGWYuqbeR/0, createdBy=0c2b1999806, createdName=三生有幸9135, createdTime=Sun Aug 06 15:54:05 CST 2017, time=2017-08-06, status=1, ipAttribution=)]
    2017-08-06 139****5926

    好好文章学习了

    0

  7. 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createdTime=Sun Aug 06 19:59:39 CST 2017, time=2017-08-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=230794, encodeId=0d9a230e94a2, content=谢谢分享学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=69, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Sun Aug 06 19:19:48 CST 2017, time=2017-08-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=230734, encodeId=7a20230e34a9, content=学习一下谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzaiabIFicf63hKvVMjL6aJlx4yX4UcXmzX9DyibCRcicq0QtCRWMaQKh8MYjE1D3EKUBopgiaGWYuqbeR/0, createdBy=0c2b1999806, createdName=三生有幸9135, createdTime=Sun Aug 06 15:54:05 CST 2017, time=2017-08-06, status=1, ipAttribution=)]
    2017-08-06 131****1460

    学习了受益匪浅。

    0

  8. 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createdTime=Sun Aug 06 19:59:39 CST 2017, time=2017-08-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=230794, encodeId=0d9a230e94a2, content=谢谢分享学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=69, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zSn0GqHrsfLGSUSR8zwnWWLT4FMl5XH8S58HuZslVkLsAPbXDXaliagK7TOxQfmngKibG1yrDonepCLDkcxGk8hS/0, createdBy=b8282062892, createdName=changjiu, createdTime=Sun Aug 06 19:19:48 CST 2017, time=2017-08-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=230734, encodeId=7a20230e34a9, content=学习一下谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzaiabIFicf63hKvVMjL6aJlx4yX4UcXmzX9DyibCRcicq0QtCRWMaQKh8MYjE1D3EKUBopgiaGWYuqbeR/0, createdBy=0c2b1999806, createdName=三生有幸9135, createdTime=Sun Aug 06 15:54:05 CST 2017, time=2017-08-06, status=1, ipAttribution=)]
    2017-08-06 changjiu

    谢谢分享学习

    0

  9. 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createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzaiabIFicf63hKvVMjL6aJlx4yX4UcXmzX9DyibCRcicq0QtCRWMaQKh8MYjE1D3EKUBopgiaGWYuqbeR/0, createdBy=0c2b1999806, createdName=三生有幸9135, createdTime=Sun Aug 06 15:54:05 CST 2017, time=2017-08-06, status=1, ipAttribution=)]
    2017-08-06 三生有幸9135

    学习一下谢谢分享

    0