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NEJM：波兰综合征-病例报道

2018-01-04 xing.T MedSci原创

波兰综合征畸形短指
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波兰综合征是一种罕见的先天性疾病，其特征是胸壁肌肉缺如或发育不全，肢体异常，包括患侧肢体并指、短指。受影响的人可能有潜在的肋骨、长骨、乳房或肾脏畸形，腋毛可能不生长。虽然波兰综合症的病因尚不清楚，但有人认为，胚胎组织中血液供应中断可能在引起胸壁和手部异常中发挥着重要作用。

患者为一名86岁的男性，因进行例行全身皮肤检查而到皮肤科门诊就诊，可以明显观察到右侧胸大肌缺陷（如图A所示）和右侧手指较短（如图B所示）。患者报告说这些发现从其出生以来就已经存在。他的力量没有减弱的或运动范围没有减少，临床诊断为波兰综合征。



波兰综合征是一种罕见的先天性疾病，其特征是胸壁肌肉缺如或发育不全，肢体异常，包括患侧肢体并指、短指。受影响的人可能有潜在的肋骨、长骨、乳房或肾脏畸形，腋毛可能不生长。虽然波兰综合症的病因尚不清楚，但有人认为，胚胎组织中血液供应中断可能在引起胸壁和手部异常中发挥着重要作用。

在一些患者中，可以进行整形手术来重建胸壁或乳房，物理治疗可能有助于改善受限的活动。

这名患者的发现可以诊断为波兰综合征，未进行进一步评估或随访。

原始出处：

Kelsey R. Kennedy,et al. Poland Syndrome.N Engl J Med 2018. http://www.nejm.org/doi/full/10.1056/NEJMicm1709713

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    2018-01-06 虈亣靌

    学习了谢谢作者分享!

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=276051, encodeId=b9512e605151, content=学习了谢谢作者分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=104, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Jan 06 16:41:08 CST 2018, time=2018-01-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275870, encodeId=0e492e5870ab, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=11a82232786, createdName=12128b66m44暂无昵称, createdTime=Sat Jan 06 00:57:07 CST 2018, time=2018-01-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431063, encodeId=18dd143106377, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Jan 05 22:47:00 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275756, encodeId=06152e575627, content=谢谢分享学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=109, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Q3auHgzwzM7GibvIBibibnnnvpajk2stlDF5oichYahI6Bia8uxeTBFoPbdoHWebyXf9eal9FNgUF2lNcrwoZicM3BvQ/0, createdBy=a3742066385, createdName=戒馋，懒，贪, createdTime=Fri Jan 05 17:55:06 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275658, encodeId=bffd2e565839, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=99, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c3da1990174, createdName=yfjms, createdTime=Fri Jan 05 09:46:59 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275318, encodeId=5f2a2e531836, content=学习谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mStl88fu4Nd44v3xHnnictn9UpctwtH62EPQ59TMdI9lnqHMgQ16nH7GmGCfpzicsHgjibYVk6vwSpXrC86gMibRkoaIzf5HwSz7/0, createdBy=78cc1691107, createdName=龙胆草, createdTime=Thu Jan 04 08:45:08 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275313, encodeId=ea972e5313cd, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c3da1990174, createdName=yfjms, createdTime=Thu Jan 04 08:24:45 CST 2018, time=2018-01-04, status=1, ipAttribution=)]
    2018-01-06 12128b66m44暂无昵称

    学习

    0

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    2018-01-05 zhouqu_8

    #综合征#

    0

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    2018-01-05 戒馋，懒，贪

    谢谢分享学习了

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=276051, encodeId=b9512e605151, content=学习了谢谢作者分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=104, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Jan 06 16:41:08 CST 2018, time=2018-01-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275870, encodeId=0e492e5870ab, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=11a82232786, createdName=12128b66m44暂无昵称, createdTime=Sat Jan 06 00:57:07 CST 2018, time=2018-01-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431063, encodeId=18dd143106377, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Jan 05 22:47:00 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275756, encodeId=06152e575627, content=谢谢分享学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=109, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Q3auHgzwzM7GibvIBibibnnnvpajk2stlDF5oichYahI6Bia8uxeTBFoPbdoHWebyXf9eal9FNgUF2lNcrwoZicM3BvQ/0, createdBy=a3742066385, createdName=戒馋，懒，贪, createdTime=Fri Jan 05 17:55:06 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275658, encodeId=bffd2e565839, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=99, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c3da1990174, createdName=yfjms, createdTime=Fri Jan 05 09:46:59 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275318, encodeId=5f2a2e531836, content=学习谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mStl88fu4Nd44v3xHnnictn9UpctwtH62EPQ59TMdI9lnqHMgQ16nH7GmGCfpzicsHgjibYVk6vwSpXrC86gMibRkoaIzf5HwSz7/0, createdBy=78cc1691107, createdName=龙胆草, createdTime=Thu Jan 04 08:45:08 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275313, encodeId=ea972e5313cd, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c3da1990174, createdName=yfjms, createdTime=Thu Jan 04 08:24:45 CST 2018, time=2018-01-04, status=1, ipAttribution=)]
    2018-01-05 yfjms

    学习

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=276051, encodeId=b9512e605151, content=学习了谢谢作者分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=104, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Jan 06 16:41:08 CST 2018, time=2018-01-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275870, encodeId=0e492e5870ab, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=11a82232786, createdName=12128b66m44暂无昵称, createdTime=Sat Jan 06 00:57:07 CST 2018, time=2018-01-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431063, encodeId=18dd143106377, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Jan 05 22:47:00 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275756, encodeId=06152e575627, content=谢谢分享学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=109, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Q3auHgzwzM7GibvIBibibnnnvpajk2stlDF5oichYahI6Bia8uxeTBFoPbdoHWebyXf9eal9FNgUF2lNcrwoZicM3BvQ/0, createdBy=a3742066385, createdName=戒馋，懒，贪, createdTime=Fri Jan 05 17:55:06 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275658, encodeId=bffd2e565839, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=99, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c3da1990174, createdName=yfjms, createdTime=Fri Jan 05 09:46:59 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275318, encodeId=5f2a2e531836, content=学习谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mStl88fu4Nd44v3xHnnictn9UpctwtH62EPQ59TMdI9lnqHMgQ16nH7GmGCfpzicsHgjibYVk6vwSpXrC86gMibRkoaIzf5HwSz7/0, createdBy=78cc1691107, createdName=龙胆草, createdTime=Thu Jan 04 08:45:08 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275313, encodeId=ea972e5313cd, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c3da1990174, createdName=yfjms, createdTime=Thu Jan 04 08:24:45 CST 2018, time=2018-01-04, status=1, ipAttribution=)]
    2018-01-04 龙胆草

    学习谢谢分享

    0

  7. [GetPortalCommentsPageByObjectIdResponse(id=276051, encodeId=b9512e605151, content=学习了谢谢作者分享!, beContent=null, objectType=article, channel=null, level=null, likeNumber=104, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mONcle9pic3zMoyicyo6ia9f4IuLQAwZoxD6Hx4ibd5CMcOCYhStY6oDibbKK6O2X8iaicldO5ib8j1iapOIobIKCGiczU2A/0, createdBy=56251941490, createdName=虈亣靌, createdTime=Sat Jan 06 16:41:08 CST 2018, time=2018-01-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275870, encodeId=0e492e5870ab, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=11a82232786, createdName=12128b66m44暂无昵称, createdTime=Sat Jan 06 00:57:07 CST 2018, time=2018-01-06, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1431063, encodeId=18dd143106377, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Fri Jan 05 22:47:00 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275756, encodeId=06152e575627, content=谢谢分享学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=109, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/Q3auHgzwzM7GibvIBibibnnnvpajk2stlDF5oichYahI6Bia8uxeTBFoPbdoHWebyXf9eal9FNgUF2lNcrwoZicM3BvQ/0, createdBy=a3742066385, createdName=戒馋，懒，贪, createdTime=Fri Jan 05 17:55:06 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275658, encodeId=bffd2e565839, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=99, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c3da1990174, createdName=yfjms, createdTime=Fri Jan 05 09:46:59 CST 2018, time=2018-01-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275318, encodeId=5f2a2e531836, content=学习谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=98, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mStl88fu4Nd44v3xHnnictn9UpctwtH62EPQ59TMdI9lnqHMgQ16nH7GmGCfpzicsHgjibYVk6vwSpXrC86gMibRkoaIzf5HwSz7/0, createdBy=78cc1691107, createdName=龙胆草, createdTime=Thu Jan 04 08:45:08 CST 2018, time=2018-01-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=275313, encodeId=ea972e5313cd, content=学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=54, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c3da1990174, createdName=yfjms, createdTime=Thu Jan 04 08:24:45 CST 2018, time=2018-01-04, status=1, ipAttribution=)]
    2018-01-04 yfjms

    学习

    0

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