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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=67, 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=53, 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=36, 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=71, 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=67, 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=61, 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=26, 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

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    2018-01-05 yfjms

    学习

    0

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    2018-01-04 龙胆草

    学习谢谢分享

    0

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    2018-01-04 yfjms

    学习

    0