这病,警惕短期内大咯血死亡

2016-10-01 刘岩 首都医科大学附属北京朝阳医院急诊科 医学界急诊与重症频道

某日,急诊接受一年轻女性患者,主诉:发热伴水肿 10 天,加重伴胸闷 3 天。体温最高 39.0℃,口服药物可自行退热。既往体健,在大理石厂工作 4 年,饮酒史 7 年,约 2 两/日。吸烟史 7 年,约 15 支/日,未戒烟及戒酒。检查及进展患者入院后检查:血常规 WBC :13.14 × 10*9/L,NE :81.6%  HGB :72g/L ,PLT :302 × 10*9/L。

某日,急诊接受一年轻女性患者,主诉:发热伴水肿 10 天,加重伴胸闷 3 天。体温最高 39.0℃,口服药物可自行退热。既往体健,在大理石厂工作 4 年,饮酒史 7 年,约 2 两/日。吸烟史 7 年,约 15 支/日,未戒烟及戒酒。


检查及进展


患者入院后检查:血常规 WBC :13.14 × 10*9/L,NE :81.6%  HGB :72g/L ,PLT :302 × 10*9/L。生化全项:BUN 33.81mmol/L,CREA 1349.8umol/L,Na+:130.5mmol/L     K+:5.2mmol/L。凝血四项:PT:14.2S,PA:75.2%,INR:1.17,APTT:44, TT:18.2S, Fbg:418.8 mg/dl D-Dimer 5.57 mg/FEU。肺 CT 显示双肺渗出性改变伴水肿,少量胸腔积液。


给予抗炎、利尿治疗后病情无明显缓解,并出现咯血表现,转入急诊 ICU 继续治疗。


入 ICU 后予血浆置换、持续血滤及洗涤后输入等对症治疗,完善检查示抗肾小球基底膜抗体(+),肺出血肾炎综合征诊断明确。


经治疗患者症状及化验指标均有明显好转,遗憾的是患者家属最终因经济原因放弃治疗。


什么是肺出血肾炎综合征(Goodpasture syndrome)?


Ernest Goodpasture 于 1919 年首次报道 1 例患者在流感后咯血、贫血而死亡,尸检证实病人存在肺出血及肾炎。


1958 年 Stanton 及 Tange 建议将具有肺出血及肾炎的综合征命名为 Goodpasture 综合征。


1967 年 Lerner 等发现此类肺出血合并肾炎的病人相当一部分是由于抗肾小球基膜(GBM)抗体致病,确诊 Goodpasture 综合征需具备 3 个条件:肺出血、肾小球肾炎及抗 GBM 抗体阳性。


肺出血-肾炎综合征可能系病毒感染和(或)吸入某些化学物质引起原发性肺损害。由于肺泡壁毛细血管基膜和肾小球基底膜存在交叉反应抗原,故可以引起继发性肾损伤。本病的特征为咯血、肺部浸润、肾小球肾炎、血浆和受累组织中有抗基底膜抗体。


本病目前尚无明确治疗手段,且该病多数病程进展迅速,短期内可因尿毒症及大咯血死亡,故早期诊断及治疗是改善预后的关键。因此早期甄别此病具有重要临床意义。

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    2016-11-10 rgjl
  2. [GetPortalCommentsPageByObjectIdResponse(id=2052699, encodeId=8bd12052699c6, content=<a href='/topic/show?id=dc444365158' target=_blank style='color:#2F92EE;'>#大咯血#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43651, encryptionId=dc444365158, topicName=大咯血)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=8b48400, createdName=rgjl, createdTime=Thu Nov 10 13:56:00 CST 2016, time=2016-11-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1468543, encodeId=6c4d146854338, content=<a href='/topic/show?id=49dd3980615' target=_blank style='color:#2F92EE;'>#咯血#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=22, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=39806, encryptionId=49dd3980615, topicName=咯血)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=0e3d6916225, createdName=feather80, createdTime=Mon Oct 03 13:56:00 CST 2016, time=2016-10-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=139484, encodeId=7f9d13948422, content=肺出血-肾炎综合征可能系病毒感染和(或)吸入某些化学物质引起原发性肺损害。由于肺泡壁毛细血管基膜和肾小球基底膜存在交叉反应抗原,故可以引起继发性肾损伤。本病的特征为咯血、肺部浸润、肾小球肾炎、血浆和受累组织中有抗基底膜抗体, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4cfc1960308, createdName=励志做个好医生, createdTime=Mon Oct 03 11:34:51 CST 2016, time=2016-10-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=138808, encodeId=fb3113880821, content=学习了谢谢。, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2017/04/21/72142930668371951a41ef4ea4b233e6.jpg, createdBy=dcbc1715228, createdName=刘煜, createdTime=Sun Oct 02 07:18:25 CST 2016, time=2016-10-02, status=1, ipAttribution=)]
    2016-10-03 feather80
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    2016-10-03 励志做个好医生

    肺出血-肾炎综合征可能系病毒感染和(或)吸入某些化学物质引起原发性肺损害。由于肺泡壁毛细血管基膜和肾小球基底膜存在交叉反应抗原,故可以引起继发性肾损伤。本病的特征为咯血、肺部浸润、肾小球肾炎、血浆和受累组织中有抗基底膜抗体

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=2052699, encodeId=8bd12052699c6, content=<a href='/topic/show?id=dc444365158' target=_blank style='color:#2F92EE;'>#大咯血#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=43651, encryptionId=dc444365158, topicName=大咯血)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=8b48400, createdName=rgjl, createdTime=Thu Nov 10 13:56:00 CST 2016, time=2016-11-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1468543, encodeId=6c4d146854338, content=<a href='/topic/show?id=49dd3980615' target=_blank style='color:#2F92EE;'>#咯血#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=22, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=39806, encryptionId=49dd3980615, topicName=咯血)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=0e3d6916225, createdName=feather80, createdTime=Mon Oct 03 13:56:00 CST 2016, time=2016-10-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=139484, encodeId=7f9d13948422, content=肺出血-肾炎综合征可能系病毒感染和(或)吸入某些化学物质引起原发性肺损害。由于肺泡壁毛细血管基膜和肾小球基底膜存在交叉反应抗原,故可以引起继发性肾损伤。本病的特征为咯血、肺部浸润、肾小球肾炎、血浆和受累组织中有抗基底膜抗体, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4cfc1960308, createdName=励志做个好医生, createdTime=Mon Oct 03 11:34:51 CST 2016, time=2016-10-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=138808, encodeId=fb3113880821, content=学习了谢谢。, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cdnapi.center.medsci.cn/medsci/head/2017/04/21/72142930668371951a41ef4ea4b233e6.jpg, createdBy=dcbc1715228, createdName=刘煜, createdTime=Sun Oct 02 07:18:25 CST 2016, time=2016-10-02, status=1, ipAttribution=)]
    2016-10-02 刘煜

    学习了谢谢。

    0