Lancet:快速了解原发性硬化性胆管炎!

2018-02-21 MedSci MedSci原创

原发性硬化性胆管炎是一种罕见的慢性胆汁淤积性肝病,以肝内或肝外狭窄为特征,或兼而有之,伴有胆管纤维化。炎症以及胆管和肝纤维化后,进而形成胆汁或血流受损和进行性肝功能障碍。患者可能无症状或出现瘙痒、疲劳、右上腹疼痛、反复发作的胆管炎或门静脉高压症后遗症。诊断的关键要素是胆汁淤积性肝脏生化和胆管造影术中的胆管狭窄。遗传和环境因素在原发性硬化性胆管炎的病因中很重要,此外,肠道微生物组也越来越多地被认为在

原发性硬化性胆管炎是一种罕见的慢性胆汁淤积性肝病,以肝内或肝外狭窄为特征,或兼而有之,伴有胆管纤维化。

炎症以及胆管和肝纤维化后,进而形成胆汁或血流受损和进行性肝功能障碍。患者可能无症状或出现瘙痒、疲劳、右上腹疼痛、反复发作的胆管炎或门静脉高压症后遗症。诊断的关键要素是胆汁淤积性肝脏生化和胆管造影术中的胆管狭窄。

遗传和环境因素在原发性硬化性胆管炎的病因中很重要,此外,肠道微生物组也越来越多地被认为在疾病的发生中起着重要作用。大约70%的患者同时患有炎症性肠病,因此,患者需要进行结肠镜检查和监测。

原发性硬化性胆管炎与恶性肿瘤的风险增加有关,而早期胆管癌检测的策略有限。

目前,尚没有任何一种药物已被证明可以提高患者的无移植生存率。肝移植对于晚期疾病是有效的,但至少25%的患者在移植物中发生复发性疾病。

原始出处:
Dyson JK, Beuers U, et al. Primary sclerosing cholangitis. Lancet. 2018 Feb 13. pii: S0140-6736(18)30300-3. doi: 10.1016/S0140-6736(18)30300-3.

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    2018-09-26 howi
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    2018-05-03 jyzxjiangqin

    原发性硬化性胆管炎.

    0

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createdName=jyzxjiangqin, createdTime=Tue Apr 24 06:34:28 CST 2018, time=2018-04-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=305010, encodeId=512630501051, content=快速了解原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Thu Apr 12 06:37:53 CST 2018, time=2018-04-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300701, encodeId=4f99300e012e, content=原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=63, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Thu Mar 29 06:27:21 CST 2018, time=2018-03-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=298487, encodeId=1cda29848e05, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=60, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Thu Mar 22 06:19:08 CST 2018, time=2018-03-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297022, encodeId=15f829e02237, content=快速了解原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Sat Mar 17 07:18:25 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=293458, encodeId=f26a2934582a, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=22, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Tue Mar 06 07:07:39 CST 2018, time=2018-03-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=292899, encodeId=63802928998b, content=谢谢分享., beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/vi_32/DYAIOgq83er8sYHCPqtn0aUWywsfKibdCUbJOTrGuwSt8ryEbSChJ7SXJvDCzUR91FVEicCFW0PClAs1vYDiajqYw/132, createdBy=6f5c2263960, createdName=Blank_, createdTime=Sun Mar 04 10:49:30 CST 2018, time=2018-03-04, status=1, ipAttribution=)]
    2018-04-24 jyzxjiangqin

    了解原发性硬化性胆管炎.

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=1829489, encodeId=a712182948976, content=<a href='/topic/show?id=1b6210686b2' target=_blank style='color:#2F92EE;'>#Lancet#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=10686, encryptionId=1b6210686b2, topicName=Lancet)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3f0227, createdName=howi, createdTime=Wed Sep 26 17:37:00 CST 2018, time=2018-09-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1846643, encodeId=7217184664381, content=<a href='/topic/show?id=d3a33e0306d' target=_blank style='color:#2F92EE;'>#原发性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=37030, encryptionId=d3a33e0306d, topicName=原发性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=8e4c53, 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createdName=jyzxjiangqin, createdTime=Tue Apr 24 06:34:28 CST 2018, time=2018-04-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=305010, encodeId=512630501051, content=快速了解原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Thu Apr 12 06:37:53 CST 2018, time=2018-04-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300701, encodeId=4f99300e012e, content=原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=63, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Thu Mar 29 06:27:21 CST 2018, time=2018-03-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=298487, encodeId=1cda29848e05, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=60, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Thu Mar 22 06:19:08 CST 2018, time=2018-03-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297022, encodeId=15f829e02237, content=快速了解原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Sat Mar 17 07:18:25 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=293458, encodeId=f26a2934582a, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=22, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Tue Mar 06 07:07:39 CST 2018, time=2018-03-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=292899, encodeId=63802928998b, content=谢谢分享., beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/vi_32/DYAIOgq83er8sYHCPqtn0aUWywsfKibdCUbJOTrGuwSt8ryEbSChJ7SXJvDCzUR91FVEicCFW0PClAs1vYDiajqYw/132, createdBy=6f5c2263960, createdName=Blank_, createdTime=Sun Mar 04 10:49:30 CST 2018, time=2018-03-04, status=1, ipAttribution=)]
    2018-04-12 jyzxjiangqin

    快速了解原发性硬化性胆管炎.

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=1829489, encodeId=a712182948976, content=<a href='/topic/show?id=1b6210686b2' target=_blank style='color:#2F92EE;'>#Lancet#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=10686, encryptionId=1b6210686b2, topicName=Lancet)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3f0227, createdName=howi, createdTime=Wed Sep 26 17:37:00 CST 2018, time=2018-09-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1846643, encodeId=7217184664381, content=<a href='/topic/show?id=d3a33e0306d' target=_blank style='color:#2F92EE;'>#原发性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=37030, encryptionId=d3a33e0306d, topicName=原发性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=8e4c53, 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createdName=jyzxjiangqin, createdTime=Tue Apr 24 06:34:28 CST 2018, time=2018-04-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=305010, encodeId=512630501051, content=快速了解原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Thu Apr 12 06:37:53 CST 2018, time=2018-04-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300701, encodeId=4f99300e012e, content=原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=63, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Thu Mar 29 06:27:21 CST 2018, time=2018-03-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=298487, encodeId=1cda29848e05, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=60, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Thu Mar 22 06:19:08 CST 2018, time=2018-03-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297022, encodeId=15f829e02237, content=快速了解原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Sat Mar 17 07:18:25 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=293458, encodeId=f26a2934582a, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=22, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Tue Mar 06 07:07:39 CST 2018, time=2018-03-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=292899, encodeId=63802928998b, content=谢谢分享., beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/vi_32/DYAIOgq83er8sYHCPqtn0aUWywsfKibdCUbJOTrGuwSt8ryEbSChJ7SXJvDCzUR91FVEicCFW0PClAs1vYDiajqYw/132, createdBy=6f5c2263960, createdName=Blank_, createdTime=Sun Mar 04 10:49:30 CST 2018, time=2018-03-04, status=1, ipAttribution=)]
    2018-03-29 jyzxjiangqin

    原发性硬化性胆管炎.

    0

  7. [GetPortalCommentsPageByObjectIdResponse(id=1829489, encodeId=a712182948976, content=<a href='/topic/show?id=1b6210686b2' target=_blank style='color:#2F92EE;'>#Lancet#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=21, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=10686, encryptionId=1b6210686b2, topicName=Lancet)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=3f0227, createdName=howi, createdTime=Wed Sep 26 17:37:00 CST 2018, time=2018-09-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1846643, encodeId=7217184664381, content=<a href='/topic/show?id=d3a33e0306d' target=_blank style='color:#2F92EE;'>#原发性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=37030, encryptionId=d3a33e0306d, topicName=原发性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=8e4c53, 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createdName=jyzxjiangqin, createdTime=Tue Apr 24 06:34:28 CST 2018, time=2018-04-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=305010, encodeId=512630501051, content=快速了解原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Thu Apr 12 06:37:53 CST 2018, time=2018-04-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=300701, encodeId=4f99300e012e, content=原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=63, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Thu Mar 29 06:27:21 CST 2018, time=2018-03-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=298487, encodeId=1cda29848e05, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=60, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Thu Mar 22 06:19:08 CST 2018, time=2018-03-22, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=297022, encodeId=15f829e02237, content=快速了解原发性硬化性胆管炎., beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Sat Mar 17 07:18:25 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=293458, encodeId=f26a2934582a, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=22, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Tue Mar 06 07:07:39 CST 2018, time=2018-03-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=292899, encodeId=63802928998b, content=谢谢分享., beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/vi_32/DYAIOgq83er8sYHCPqtn0aUWywsfKibdCUbJOTrGuwSt8ryEbSChJ7SXJvDCzUR91FVEicCFW0PClAs1vYDiajqYw/132, createdBy=6f5c2263960, createdName=Blank_, createdTime=Sun Mar 04 10:49:30 CST 2018, time=2018-03-04, status=1, ipAttribution=)]
    2018-03-22 lofter

    学习了感谢分享

    0

  8. 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createdName=jyzxjiangqin, createdTime=Sat Mar 17 07:18:25 CST 2018, time=2018-03-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=293458, encodeId=f26a2934582a, content=学习了感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=22, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://q.qlogo.cn/qqapp/1103841572/C20CB8E4EBB36F5B821651C710BCB491/100, createdBy=18de1715848, createdName=lofter, createdTime=Tue Mar 06 07:07:39 CST 2018, time=2018-03-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=292899, encodeId=63802928998b, content=谢谢分享., beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/vi_32/DYAIOgq83er8sYHCPqtn0aUWywsfKibdCUbJOTrGuwSt8ryEbSChJ7SXJvDCzUR91FVEicCFW0PClAs1vYDiajqYw/132, createdBy=6f5c2263960, createdName=Blank_, createdTime=Sun Mar 04 10:49:30 CST 2018, time=2018-03-04, status=1, ipAttribution=)]
    2018-03-17 jyzxjiangqin

    快速了解原发性硬化性胆管炎.

    0

  9. 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    2018-03-06 lofter

    学习了感谢分享

    0

  10. 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    2018-03-04 Blank_

    谢谢分享.

    0

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