心血管

订阅

MedSci主页 / 所有栏目 / 心血管

2014 EAS家族性高胆固醇血症临床管理指南

2014-07-25 MedSci MedSci原创

高胆固醇血症指南
关注

近日，欧洲动脉粥样硬化学会(EAS)发布了纯合子家族性高胆固醇血症(HoFH)临床管理指南，全文7月22日在线发表于《欧洲心脏杂志》(Eur Heart J)。以下为指南摘要内容。 诊断 指南建议， HoFH的早期诊断并及时启动饮食和降脂治疗至关重要。基因学检测可确诊，但如果不可用，低密度脂蛋白胆固醇(LDL-C)显著升高和＜10岁出现皮肤或 肌腱黄色瘤，或者未经处理的LDL

近日，欧洲动脉粥样硬化学会(EAS)发布了纯合子家族性高胆固醇血症(HoFH)临床管理指南，全文7月22日在线发表于《欧洲心脏杂志》(Eur Heart J)。以下为指南摘要内容。

诊断

指南建议， HoFH的早期诊断并及时启动饮食和降脂治疗至关重要。基因学检测可确诊，但如果不可用，低密度脂蛋白胆固醇(LDL-C)显著升高和＜10岁出现皮肤或 肌腱黄色瘤，或者未经处理的LDL-C水平升高伴有父母患有杂合子FH也提示患者有HoFH。

治疗

指南推荐，疑为HoFH的患者应及时就诊于专科医师，以综合评估动脉粥样硬化性心血管疾病(ACVD)并进行临床管理。生活方式干预和最大量他汀类药物是治疗主要手段，理想治疗时间是于出生后第一年或初次确诊时，常与依折麦布和其他降脂治疗方法联用。


因患者LDL-C很少能达标，如果可以，推荐辅助脂蛋白单采，最好是于患者5岁时启动，不晚于8岁。Lomitapide和mipomersen可作为新的治疗方法使用。


HoFH治疗方案流程图

随访

鉴于ACVD的严重性，指南推荐常规随访，包括每年进行多普勒超声心动图检查和主动脉检查以及压力测试，如果可以，每5年进行CT冠状动脉造影检查，或如有必要此年限可更短。

原始出处：

Cuchel M, Bruckert E, Ginsberg HN, Raal FJ, Santos RD, Hegele RA, Kuivenhoven JA, Nordestgaard BG, Descamps OS, Steinhagen-Thiessen E, Tybjærg-Hansen A, Watts GF, Averna M, Boileau C, Borén J, Catapano AL, Defesche JC, Hovingh GK, Humphries SE, Kovanen PT, Masana L, Pajukanta P, Parhofer KG, Ray KK, Stalenhoef AF, Stroes E, Taskinen MR, Wiegman A, Wiklund O, Chapman MJ; for the European Atherosclerosis Society Consensus Panel on Familial Hypercholesterolaemia.Homozygous familial hypercholesterolaemia: new insights and guidance for clinicians to improve detection and clinical management. A position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society.Eur Heart J. 2014 Jul 22. pii: ehu274

下载：eurheartj_ehu274_full.pdf

版权声明：
本网站所有内容来源注明为“梅斯医学”或“MedSci原创”的文字、图片和音视频资料，版权均属于梅斯医学所有。非经授权，任何媒体、网站或个人不得转载，授权转载时须注明来源为“梅斯医学”。其它来源的文章系转载文章，或“梅斯号”自媒体发布的文章，仅系出于传递更多信息之目的，本站仅负责审核内容合规，其内容不代表本站立场，本站不负责内容的准确性和版权。如果存在侵权、或不希望被转载的媒体或个人可与我们联系，我们将立即进行删除处理。
在此留言
评论区 (4)
#插入话题
  1. [GetPortalCommentsPageByObjectIdResponse(id=1936272, encodeId=d6b919362e2b0, content=<a href='/topic/show?id=daa1e60387b' target=_blank style='color:#2F92EE;'>#管理指南#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=76038, encryptionId=daa1e60387b, topicName=管理指南)], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIUEDhSibBFt8TicgFCCSBtW10SxVJicDpibTCucns8L1YaiaKpibWOkv8hYpJ0LeTVUH1Ab4sfica9bxiccA/132, createdBy=dd282500126, createdName=ms3830150583600765, createdTime=Fri Apr 10 22:00:00 CST 2015, time=2015-04-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1380011, encodeId=834713800110e, content=<a href='/topic/show?id=bef6102602a9' target=_blank style='color:#2F92EE;'>#高胆固醇血症#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=102602, encryptionId=bef6102602a9, topicName=高胆固醇血症)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=d1d1470, createdName=刘阔, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1487051, encodeId=5838148e05106, content=<a href='/topic/show?id=2b902295e5b' target=_blank style='color:#2F92EE;'>#临床管理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=22957, encryptionId=2b902295e5b, topicName=临床管理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d89d8256855, createdName=shuangle, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1629463, encodeId=d0431629463be, content=<a href='/topic/show?id=c61d4641821' target=_blank style='color:#2F92EE;'>#家族性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=46418, encryptionId=c61d4641821, topicName=家族性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=26fc21339997, createdName=wetgdt, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=)]
    2015-04-10 ms3830150583600765

    #管理指南#

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=1936272, encodeId=d6b919362e2b0, content=<a href='/topic/show?id=daa1e60387b' target=_blank style='color:#2F92EE;'>#管理指南#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=76038, encryptionId=daa1e60387b, topicName=管理指南)], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIUEDhSibBFt8TicgFCCSBtW10SxVJicDpibTCucns8L1YaiaKpibWOkv8hYpJ0LeTVUH1Ab4sfica9bxiccA/132, createdBy=dd282500126, createdName=ms3830150583600765, createdTime=Fri Apr 10 22:00:00 CST 2015, time=2015-04-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1380011, encodeId=834713800110e, content=<a href='/topic/show?id=bef6102602a9' target=_blank style='color:#2F92EE;'>#高胆固醇血症#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=102602, encryptionId=bef6102602a9, topicName=高胆固醇血症)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=d1d1470, createdName=刘阔, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1487051, encodeId=5838148e05106, content=<a href='/topic/show?id=2b902295e5b' target=_blank style='color:#2F92EE;'>#临床管理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=22957, encryptionId=2b902295e5b, topicName=临床管理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d89d8256855, createdName=shuangle, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1629463, encodeId=d0431629463be, content=<a href='/topic/show?id=c61d4641821' target=_blank style='color:#2F92EE;'>#家族性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=46418, encryptionId=c61d4641821, topicName=家族性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=26fc21339997, createdName=wetgdt, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=)]
    2014-07-27 刘阔

    #高胆固醇血症#

    0

  3. [GetPortalCommentsPageByObjectIdResponse(id=1936272, encodeId=d6b919362e2b0, content=<a href='/topic/show?id=daa1e60387b' target=_blank style='color:#2F92EE;'>#管理指南#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=76038, encryptionId=daa1e60387b, topicName=管理指南)], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIUEDhSibBFt8TicgFCCSBtW10SxVJicDpibTCucns8L1YaiaKpibWOkv8hYpJ0LeTVUH1Ab4sfica9bxiccA/132, createdBy=dd282500126, createdName=ms3830150583600765, createdTime=Fri Apr 10 22:00:00 CST 2015, time=2015-04-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1380011, encodeId=834713800110e, content=<a href='/topic/show?id=bef6102602a9' target=_blank style='color:#2F92EE;'>#高胆固醇血症#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=102602, encryptionId=bef6102602a9, topicName=高胆固醇血症)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=d1d1470, createdName=刘阔, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1487051, encodeId=5838148e05106, content=<a href='/topic/show?id=2b902295e5b' target=_blank style='color:#2F92EE;'>#临床管理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=22957, encryptionId=2b902295e5b, topicName=临床管理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d89d8256855, createdName=shuangle, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1629463, encodeId=d0431629463be, content=<a href='/topic/show?id=c61d4641821' target=_blank style='color:#2F92EE;'>#家族性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=46418, encryptionId=c61d4641821, topicName=家族性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=26fc21339997, createdName=wetgdt, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=)]
    2014-07-27 shuangle

    #临床管理#

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=1936272, encodeId=d6b919362e2b0, content=<a href='/topic/show?id=daa1e60387b' target=_blank style='color:#2F92EE;'>#管理指南#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=30, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=76038, encryptionId=daa1e60387b, topicName=管理指南)], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIUEDhSibBFt8TicgFCCSBtW10SxVJicDpibTCucns8L1YaiaKpibWOkv8hYpJ0LeTVUH1Ab4sfica9bxiccA/132, createdBy=dd282500126, createdName=ms3830150583600765, createdTime=Fri Apr 10 22:00:00 CST 2015, time=2015-04-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1380011, encodeId=834713800110e, content=<a href='/topic/show?id=bef6102602a9' target=_blank style='color:#2F92EE;'>#高胆固醇血症#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=102602, encryptionId=bef6102602a9, topicName=高胆固醇血症)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=d1d1470, createdName=刘阔, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1487051, encodeId=5838148e05106, content=<a href='/topic/show?id=2b902295e5b' target=_blank style='color:#2F92EE;'>#临床管理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=22957, encryptionId=2b902295e5b, topicName=临床管理)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d89d8256855, createdName=shuangle, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1629463, encodeId=d0431629463be, content=<a href='/topic/show?id=c61d4641821' target=_blank style='color:#2F92EE;'>#家族性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=46418, encryptionId=c61d4641821, topicName=家族性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=26fc21339997, createdName=wetgdt, createdTime=Sun Jul 27 12:00:00 CST 2014, time=2014-07-27, status=1, ipAttribution=)]
    2014-07-27 wetgdt

    #家族性#

    0

相关资讯

他汀不降低家族性高胆固醇血症辅酶Q10浓度

　《动脉粥样硬化》（Atherosclerosis）杂志2011年6月6日在线发表的一项研究表明，长期、大剂量他汀治疗不能使显性或遗传型家族性高胆固醇血症（FH）患者的血浆辅酶Q10（CoQ10）浓度降低至正常值以下。与未接受他汀治疗的对照组相比，FH患者的动脉僵硬度增加，低CoQ10水平与动脉僵硬度增加相关。 　　研究者评价了接受他汀治疗的杂合性FH患者，与同等剂量他汀治疗的低密度脂蛋白受体

Sci Transl Med ：迷你猪助力高胆固醇血症研究

近日，刊登在美国《科学—转化医学》上的一项新研究报告称，猪也会像人那样，发展出胆固醇增高及动脉粥样硬化（动脉堵塞）等症状。由于猪与人有着相似的生理机能，因此，来自丹麦的一组研究人员制造出一种迷你猪新模型，对诸如高胆固醇血症、动脉粥样硬化及心脏病等疾病新疗法的研究十分有帮助。新研究还使研发让体内粥样硬化可视化的成像技术成为可能。 奥尔胡斯大学临床医学部的Rozh Al-Mashhadi及其同事用一

EHJ：心血管危险与LDLR多态性间的关系

　　过量的低密度脂蛋白受体（LDLR）基因突变是导致家族性高胆固醇血症（FH）临床表型的根本原因。但鉴别致病性突变与非致病性突变对FH的诊断非常重要。与非功能性变异相比，真正的致病性突变是否确实与冠状动脉疾病（CAD）的发生有关，荷兰科学家对此进行了评估。非功能变异应该与CAD的发生没有关系。 　　研究纳入29365名受试者，检测了64种最常见的LDLR 变异。科学家首先确定每一种序列

氯吡格雷能否“走进”手术室？

　　 　　 　　 　　一项荟萃分析对34项研究中22854例因急性冠状动脉综合征（ACS）接受冠状动脉旁路移植术（CABG）治疗的患者进行研究后发现，术前停用氯吡格雷与否，对死亡、CABG术后心肌梗死、卒中和联合主要不良心血管事件发生率无影响。研究5月24日在线发表于《欧洲心脏杂志》（Eur Heart J）。 　　在CABG术前，氯吡格雷要不要停药，这是一个问题。为此，我们采访了该荟萃分