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Radiology：肥厚性心肌病与Anderson-Fabry 病的鉴别点有哪些？

2018-05-23 shaosai MedSci原创

Anderson-Fabry病肥厚性心肌病心脏磁共振
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本研究旨在利用心脏磁共振（MR）成像T1值比较肥厚性心肌病（HCM）与Anderson-Fabry 病（AFD）左心室（LV）和右心室（RV）的T1值，评价除年龄、性别和传统成像特点之外T1值的价值，并将结果发表在Radiology上。

本研究旨在利用心脏磁共振（MR）成像T1值比较肥厚性心肌病（HCM）与Anderson-Fabry 病（AFD）左心室（LV）和右心室（RV）的T1值，评价除年龄、性别和传统成像特点之外T1值的价值，并将结果发表在Radiology上。

本研究共纳入了30例基因阳性AFD（37%男性，平均年龄为45.0±14.1）和30例HCM（57%男性，平均年龄49.3岁±13.5）的患者。对患者行心脏MR成像（包括T1 Mapping）。评价LV和RV T1值。统计学方法包括独立样本T检验、ROC分析、多变量Logistic回归和似然比检验。

结果为，AFD组室间隔LV、整体LV和RV的原始T1值要明显低于HCM组(1161 msec ± 47 vs 1296 msec ± 55 [P < .001]； 1192 msec ± 52 vs 1268 msec ± 55 [P < .001]； and 1221 msec ± 54 vs 1271 msec ± 37 [P = .001])以室间隔LV T1值≤1220 msec作为鉴别AFD与HCM最佳截断值的敏感性、特异性及准确性分别为97%、93%和95%。经调整年龄、性别和传统成像特征后以室间隔LV T1值≤1220 msec作为鉴别AFD与HCM(OR为0.94； 95% CI： 0.91， 0.98； P = < .001)。在年龄、性别和传统成像特征嵌套的Logistic回归模型中，通过纳入室间隔LV T1值改善模型效能(χ2 [df = 1] = 33.4； P < .001)。

本研究表明，AFD患者心脏MR成像原始T1值要明显低于HCM患者，从而提供了两者除年龄、性别和传统成像特征之外的诊断信息。

原始出处：

Karur GR， Robison S， Iwanochko RM. et al.Use of Myocardial T1 Mapping at 3.0 T to Differentiate Anderson-Fabry Disease from Hypertrophic Cardiomyopathy.Radiology.DOI：10.1148/radiol.2018172613

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  1. [GetPortalCommentsPageByObjectIdResponse(id=1785949, encodeId=83961e859498b, content=<a href='/topic/show?id=f61fe20776' target=_blank style='color:#2F92EE;'>#Fabry#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=51, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=7207, encryptionId=f61fe20776, topicName=Fabry)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a12645, createdName=智智灵药, createdTime=Sun Aug 05 08:24:00 CST 2018, time=2018-08-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878269, encodeId=bd0118e8269de, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=37, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=30bd35, createdName=一叶知秋, createdTime=Thu Dec 13 10:24:00 CST 2018, time=2018-12-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622330, encodeId=7823162233042, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Fri May 25 07:24:00 CST 2018, time=2018-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317936, encodeId=eac431e93646, content=学习学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/RZHiaqL82mmuQyM2yENjM6xkO8GA8Ioyv4o13u0RQmPwDahVYWYGKTAHctbqbCl76iaXcM3YBcibGvzJL5zfBhvgg/132, createdBy=43302363581, createdName=影像超人, createdTime=Wed May 23 22:49:18 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317901, encodeId=b75731e901e7, content=学习了受益匪浅, beContent=null, objectType=article, channel=null, level=null, likeNumber=73, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLDicHg2ldsSNmib46PmdzpO7CQEmPjuULp1v45zPWwMYhrnXgvl3OtmUCZSQfgITWn96X9moL94nUGA/0, createdBy=28072041016, createdName=131****1460, createdTime=Wed May 23 18:59:30 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317883, encodeId=08ca31e8836f, content=学习了.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=60791943465, createdName=1e0f8808m18(暂无匿称), createdTime=Wed May 23 17:10:09 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317880, encodeId=b4be31e880b2, content=牛啊.好厉害, beContent=null, objectType=article, channel=null, level=null, likeNumber=87, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=/v1.0.0/img/user_icon.png, createdBy=f4cf2011334, createdName=AndoR, createdTime=Wed May 23 16:34:22 CST 2018, time=2018-05-23, status=1, ipAttribution=)]
    2018-08-05 智智灵药

    #Fabry#

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=1785949, encodeId=83961e859498b, content=<a href='/topic/show?id=f61fe20776' target=_blank style='color:#2F92EE;'>#Fabry#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=51, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=7207, encryptionId=f61fe20776, topicName=Fabry)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a12645, createdName=智智灵药, createdTime=Sun Aug 05 08:24:00 CST 2018, time=2018-08-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878269, encodeId=bd0118e8269de, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=37, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=30bd35, createdName=一叶知秋, createdTime=Thu Dec 13 10:24:00 CST 2018, time=2018-12-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622330, encodeId=7823162233042, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Fri May 25 07:24:00 CST 2018, time=2018-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317936, encodeId=eac431e93646, content=学习学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/RZHiaqL82mmuQyM2yENjM6xkO8GA8Ioyv4o13u0RQmPwDahVYWYGKTAHctbqbCl76iaXcM3YBcibGvzJL5zfBhvgg/132, createdBy=43302363581, createdName=影像超人, createdTime=Wed May 23 22:49:18 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317901, encodeId=b75731e901e7, content=学习了受益匪浅, beContent=null, objectType=article, channel=null, level=null, likeNumber=73, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLDicHg2ldsSNmib46PmdzpO7CQEmPjuULp1v45zPWwMYhrnXgvl3OtmUCZSQfgITWn96X9moL94nUGA/0, createdBy=28072041016, createdName=131****1460, createdTime=Wed May 23 18:59:30 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317883, encodeId=08ca31e8836f, content=学习了.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=60791943465, createdName=1e0f8808m18(暂无匿称), createdTime=Wed May 23 17:10:09 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317880, encodeId=b4be31e880b2, content=牛啊.好厉害, beContent=null, objectType=article, channel=null, level=null, likeNumber=87, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=/v1.0.0/img/user_icon.png, createdBy=f4cf2011334, createdName=AndoR, createdTime=Wed May 23 16:34:22 CST 2018, time=2018-05-23, status=1, ipAttribution=)]
    2018-12-13 一叶知秋

    #ERS#

    0

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    2018-05-25 爆笑小医

    #肌病#

    0

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    2018-05-23 影像超人

    学习学习

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=1785949, encodeId=83961e859498b, content=<a href='/topic/show?id=f61fe20776' target=_blank style='color:#2F92EE;'>#Fabry#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=51, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=7207, encryptionId=f61fe20776, topicName=Fabry)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a12645, createdName=智智灵药, createdTime=Sun Aug 05 08:24:00 CST 2018, time=2018-08-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878269, encodeId=bd0118e8269de, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=37, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=30bd35, createdName=一叶知秋, createdTime=Thu Dec 13 10:24:00 CST 2018, time=2018-12-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622330, encodeId=7823162233042, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Fri May 25 07:24:00 CST 2018, time=2018-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317936, encodeId=eac431e93646, content=学习学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/RZHiaqL82mmuQyM2yENjM6xkO8GA8Ioyv4o13u0RQmPwDahVYWYGKTAHctbqbCl76iaXcM3YBcibGvzJL5zfBhvgg/132, createdBy=43302363581, createdName=影像超人, createdTime=Wed May 23 22:49:18 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317901, encodeId=b75731e901e7, content=学习了受益匪浅, beContent=null, objectType=article, channel=null, level=null, likeNumber=73, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLDicHg2ldsSNmib46PmdzpO7CQEmPjuULp1v45zPWwMYhrnXgvl3OtmUCZSQfgITWn96X9moL94nUGA/0, createdBy=28072041016, createdName=131****1460, createdTime=Wed May 23 18:59:30 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317883, encodeId=08ca31e8836f, content=学习了.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=60791943465, createdName=1e0f8808m18(暂无匿称), createdTime=Wed May 23 17:10:09 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317880, encodeId=b4be31e880b2, content=牛啊.好厉害, beContent=null, objectType=article, channel=null, level=null, likeNumber=87, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=/v1.0.0/img/user_icon.png, createdBy=f4cf2011334, createdName=AndoR, createdTime=Wed May 23 16:34:22 CST 2018, time=2018-05-23, status=1, ipAttribution=)]
    2018-05-23 131****1460

    学习了受益匪浅

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=1785949, encodeId=83961e859498b, content=<a href='/topic/show?id=f61fe20776' target=_blank style='color:#2F92EE;'>#Fabry#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=51, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=7207, encryptionId=f61fe20776, topicName=Fabry)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a12645, createdName=智智灵药, createdTime=Sun Aug 05 08:24:00 CST 2018, time=2018-08-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878269, encodeId=bd0118e8269de, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=37, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=30bd35, createdName=一叶知秋, createdTime=Thu Dec 13 10:24:00 CST 2018, time=2018-12-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622330, encodeId=7823162233042, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Fri May 25 07:24:00 CST 2018, time=2018-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317936, encodeId=eac431e93646, content=学习学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/RZHiaqL82mmuQyM2yENjM6xkO8GA8Ioyv4o13u0RQmPwDahVYWYGKTAHctbqbCl76iaXcM3YBcibGvzJL5zfBhvgg/132, createdBy=43302363581, createdName=影像超人, createdTime=Wed May 23 22:49:18 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317901, encodeId=b75731e901e7, content=学习了受益匪浅, beContent=null, objectType=article, channel=null, level=null, likeNumber=73, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLDicHg2ldsSNmib46PmdzpO7CQEmPjuULp1v45zPWwMYhrnXgvl3OtmUCZSQfgITWn96X9moL94nUGA/0, createdBy=28072041016, createdName=131****1460, createdTime=Wed May 23 18:59:30 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317883, encodeId=08ca31e8836f, content=学习了.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=60791943465, createdName=1e0f8808m18(暂无匿称), createdTime=Wed May 23 17:10:09 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317880, encodeId=b4be31e880b2, content=牛啊.好厉害, beContent=null, objectType=article, channel=null, level=null, likeNumber=87, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=/v1.0.0/img/user_icon.png, createdBy=f4cf2011334, createdName=AndoR, createdTime=Wed May 23 16:34:22 CST 2018, time=2018-05-23, status=1, ipAttribution=)]
    2018-05-23 1e0f8808m18(暂无匿称)

    学习了.谢谢分享!

    0

  7. [GetPortalCommentsPageByObjectIdResponse(id=1785949, encodeId=83961e859498b, content=<a href='/topic/show?id=f61fe20776' target=_blank style='color:#2F92EE;'>#Fabry#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=51, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=7207, encryptionId=f61fe20776, topicName=Fabry)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a12645, createdName=智智灵药, createdTime=Sun Aug 05 08:24:00 CST 2018, time=2018-08-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1878269, encodeId=bd0118e8269de, content=<a href='/topic/show?id=c157696175' target=_blank style='color:#2F92EE;'>#ERS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=37, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=6961, encryptionId=c157696175, topicName=ERS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=30bd35, createdName=一叶知秋, createdTime=Thu Dec 13 10:24:00 CST 2018, time=2018-12-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622330, encodeId=7823162233042, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Fri May 25 07:24:00 CST 2018, time=2018-05-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317936, encodeId=eac431e93646, content=学习学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/RZHiaqL82mmuQyM2yENjM6xkO8GA8Ioyv4o13u0RQmPwDahVYWYGKTAHctbqbCl76iaXcM3YBcibGvzJL5zfBhvgg/132, createdBy=43302363581, createdName=影像超人, createdTime=Wed May 23 22:49:18 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317901, encodeId=b75731e901e7, content=学习了受益匪浅, beContent=null, objectType=article, channel=null, level=null, likeNumber=73, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLDicHg2ldsSNmib46PmdzpO7CQEmPjuULp1v45zPWwMYhrnXgvl3OtmUCZSQfgITWn96X9moL94nUGA/0, createdBy=28072041016, createdName=131****1460, createdTime=Wed May 23 18:59:30 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317883, encodeId=08ca31e8836f, content=学习了.谢谢分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=60791943465, createdName=1e0f8808m18(暂无匿称), createdTime=Wed May 23 17:10:09 CST 2018, time=2018-05-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=317880, encodeId=b4be31e880b2, content=牛啊.好厉害, beContent=null, objectType=article, channel=null, level=null, likeNumber=87, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=/v1.0.0/img/user_icon.png, createdBy=f4cf2011334, createdName=AndoR, createdTime=Wed May 23 16:34:22 CST 2018, time=2018-05-23, status=1, ipAttribution=)]
    2018-05-23 AndoR

    牛啊.好厉害

    0