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婴幼儿拒奶,警惕心内膜弹力纤维增生症

2016-08-30 武警总医院 顽疾归赵 医学界儿科频道

门急诊工作中,会碰到婴儿期出现严重拒奶,一般状况差,肝脾肿大,听诊可以听到心音弱,心动过速,可闻及奔马律,这时候需要考虑婴幼儿心肌病的一种——心内膜弹力纤维增生症。此病病因目前尚未明确,目前有胎儿期病毒感染、宫内缺氧引起心脏发育障碍、遗传因素、继发于先天性心脏病等多种假说,在婴儿心肌病中还属于比较多见的一种。一、心内膜弹力纤维增生症症状1、暴发型:起病急骤,可突然出现呼吸困难、呕吐、拒食、口周发绀

门急诊工作中,会碰到婴儿期出现严重拒奶,一般状况差,肝脾肿大,听诊可以听到心音弱,心动过速,可闻及奔马律,这时候需要考虑婴幼儿心肌病的一种——心内膜弹力纤维增生症。此病病因目前尚未明确,目前有胎儿期病毒感染、宫内缺氧引起心脏发育障碍、遗传因素、继发于先天性心脏病等多种假说,在婴儿心肌病中还属于比较多见的一种。


一、心内膜弹力纤维增生症症状


1、暴发型:起病急骤,可突然出现呼吸困难、呕吐、拒食、口周发绀、面色苍白、烦躁不安、心动过速。肺部听诊可有散在哮鸣音或干啰音,肝脏肿大,还可见水肿,均为充血性心力衰竭所致,部分患儿可出现休克体征,如面白脉细四肢湿冷等表现。多在6月龄内,可引起心源性猝死。


2、急性型:起病急,但充血性心衰发展较暴发型缓慢,常并发肺炎、伴有发热、肺部有湿罗音。部分患儿可因腹壁血栓脱落而发生脑栓塞。可因心衰死亡,但经过积极治疗可缓解。


3、慢性型:发病缓慢,年龄在6月龄以上,症状同急性型,进展缓慢,但会影响婴儿生长发育。经治疗可缓解,可活至成人,但最终会因反复心衰去世。


二、心内膜弹力纤维增生症诊断依据


1、1岁以内婴儿于2-6月突然出现心力衰竭;


2、X线胸片提示心胸比增宽大,心脏扩大以左心室为主,心搏减弱;


3、心脏听诊无明显杂音;


4、心电图提示左心室肥厚,或V5、V6导联T波倒置;


5、超声心动图表现为左心室扩大,心内膜回声增粗,左心室收缩功能及舒张功能降低;


6、确诊需要心内膜心肌病理活检。


三、治疗


主要治疗以控制心力衰竭为主。


急性期:应用洋地黄类药物,或其他正性肌力药和强效利尿剂,并应长期应用地高辛维持,可达2-3年或数年之久,至心脏回缩至正常,过早停药可导致病情恶化。


慢性期:在常规治疗基础上,加用ACEI类药物,可改善心功能及预后。发生肺部感染时,应积极抗感染治疗。对于心脏重度扩大,射血分数明显降低者及药物治疗反应差的患儿,考虑进行心脏移植。可采用免疫抑制剂治疗,主要为泼尼松1.5 mg/kg.d,服用8周后逐渐减量,每隔2周减2.5-1.25 mg,至每天0.25-0.5 mg/kg作为维持量,至心电图正常,X线片心脏接近正常,逐渐停药,疗程1-1.5年。

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  2. [GetPortalCommentsPageByObjectIdResponse(id=1708350, encodeId=7c151e0835069, content=<a href='/topic/show?id=4c2d3051535' target=_blank style='color:#2F92EE;'>#内膜#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=30515, encryptionId=4c2d3051535, topicName=内膜)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=730431558473, createdName=12498974m61暂无昵称, createdTime=Fri Nov 04 17:20:00 CST 2016, time=2016-11-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=111408, encodeId=7cd3111408c7, content=很好,不错,以后会多学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=102, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzUn81b5CF5ibVPib8jWI92iciaxmqGHlwruOnK4SVZykd4iahqo1hicklIibyZoxm55X7EM8BW9eX1h1hQ2/0, createdBy=8aa81937526, createdName=李东泽, createdTime=Mon Sep 05 10:48:00 CST 2016, time=2016-09-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=111159, encodeId=f3eb111159a2, content=好文,深入学习。, beContent=null, objectType=article, channel=null, level=null, likeNumber=95, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo7s7ZCKOmmbBTD6nE7gcd5OZpvyGEzkxBP3S275k5ZJVrsPkotZhBglRHM9rKvcj1OgkssqhcnoPw8zIaMZTF8c/0, createdBy=4c441942088, createdName=ylzr123, createdTime=Mon Sep 05 09:06:00 CST 2016, time=2016-09-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=109459, encodeId=daef10945992, content=给我一个提醒, beContent=null, objectType=article, channel=null, level=null, likeNumber=133, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a3f71945931, createdName=1e101048m86(暂无匿称), createdTime=Fri Sep 02 10:37:00 CST 2016, time=2016-09-02, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=108354, encodeId=eb5f108354e8, content=继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=119, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Aug 31 13:05:00 CST 2016, time=2016-08-31, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=108355, encodeId=71aa10835513, content=继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Aug 31 13:05:00 CST 2016, time=2016-08-31, status=1, ipAttribution=)]
    2016-09-05 李东泽

    很好,不错,以后会多学习

    0

  3. [GetPortalCommentsPageByObjectIdResponse(id=1708350, encodeId=7c151e0835069, content=<a href='/topic/show?id=4c2d3051535' target=_blank style='color:#2F92EE;'>#内膜#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=30515, encryptionId=4c2d3051535, topicName=内膜)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=730431558473, createdName=12498974m61暂无昵称, createdTime=Fri Nov 04 17:20:00 CST 2016, time=2016-11-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=111408, encodeId=7cd3111408c7, content=很好,不错,以后会多学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=102, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzUn81b5CF5ibVPib8jWI92iciaxmqGHlwruOnK4SVZykd4iahqo1hicklIibyZoxm55X7EM8BW9eX1h1hQ2/0, createdBy=8aa81937526, createdName=李东泽, createdTime=Mon Sep 05 10:48:00 CST 2016, time=2016-09-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=111159, encodeId=f3eb111159a2, content=好文,深入学习。, beContent=null, objectType=article, channel=null, level=null, likeNumber=95, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo7s7ZCKOmmbBTD6nE7gcd5OZpvyGEzkxBP3S275k5ZJVrsPkotZhBglRHM9rKvcj1OgkssqhcnoPw8zIaMZTF8c/0, createdBy=4c441942088, createdName=ylzr123, createdTime=Mon Sep 05 09:06:00 CST 2016, time=2016-09-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=109459, encodeId=daef10945992, content=给我一个提醒, beContent=null, objectType=article, channel=null, level=null, likeNumber=133, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a3f71945931, createdName=1e101048m86(暂无匿称), createdTime=Fri Sep 02 10:37:00 CST 2016, time=2016-09-02, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=108354, encodeId=eb5f108354e8, content=继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=119, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Aug 31 13:05:00 CST 2016, time=2016-08-31, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=108355, encodeId=71aa10835513, content=继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Aug 31 13:05:00 CST 2016, time=2016-08-31, status=1, ipAttribution=)]
    2016-09-05 ylzr123

    好文,深入学习。

    0

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    2016-09-02 1e101048m86(暂无匿称)

    给我一个提醒

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=1708350, encodeId=7c151e0835069, content=<a href='/topic/show?id=4c2d3051535' target=_blank style='color:#2F92EE;'>#内膜#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=30515, encryptionId=4c2d3051535, topicName=内膜)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=730431558473, createdName=12498974m61暂无昵称, createdTime=Fri Nov 04 17:20:00 CST 2016, time=2016-11-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=111408, encodeId=7cd3111408c7, content=很好,不错,以后会多学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=102, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzUn81b5CF5ibVPib8jWI92iciaxmqGHlwruOnK4SVZykd4iahqo1hicklIibyZoxm55X7EM8BW9eX1h1hQ2/0, createdBy=8aa81937526, createdName=李东泽, createdTime=Mon Sep 05 10:48:00 CST 2016, time=2016-09-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=111159, encodeId=f3eb111159a2, content=好文,深入学习。, beContent=null, objectType=article, channel=null, level=null, likeNumber=95, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo7s7ZCKOmmbBTD6nE7gcd5OZpvyGEzkxBP3S275k5ZJVrsPkotZhBglRHM9rKvcj1OgkssqhcnoPw8zIaMZTF8c/0, createdBy=4c441942088, createdName=ylzr123, createdTime=Mon Sep 05 09:06:00 CST 2016, time=2016-09-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=109459, encodeId=daef10945992, content=给我一个提醒, beContent=null, objectType=article, channel=null, level=null, likeNumber=133, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a3f71945931, createdName=1e101048m86(暂无匿称), createdTime=Fri Sep 02 10:37:00 CST 2016, time=2016-09-02, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=108354, encodeId=eb5f108354e8, content=继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=119, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Aug 31 13:05:00 CST 2016, time=2016-08-31, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=108355, encodeId=71aa10835513, content=继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Aug 31 13:05:00 CST 2016, time=2016-08-31, status=1, ipAttribution=)]
    2016-08-31 doctorJiangchao

    继续学习

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=1708350, encodeId=7c151e0835069, content=<a href='/topic/show?id=4c2d3051535' target=_blank style='color:#2F92EE;'>#内膜#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=88, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=30515, encryptionId=4c2d3051535, topicName=内膜)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=730431558473, createdName=12498974m61暂无昵称, createdTime=Fri Nov 04 17:20:00 CST 2016, time=2016-11-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=111408, encodeId=7cd3111408c7, content=很好,不错,以后会多学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=102, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzUn81b5CF5ibVPib8jWI92iciaxmqGHlwruOnK4SVZykd4iahqo1hicklIibyZoxm55X7EM8BW9eX1h1hQ2/0, createdBy=8aa81937526, createdName=李东泽, createdTime=Mon Sep 05 10:48:00 CST 2016, time=2016-09-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=111159, encodeId=f3eb111159a2, content=好文,深入学习。, beContent=null, objectType=article, channel=null, level=null, likeNumber=95, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo7s7ZCKOmmbBTD6nE7gcd5OZpvyGEzkxBP3S275k5ZJVrsPkotZhBglRHM9rKvcj1OgkssqhcnoPw8zIaMZTF8c/0, createdBy=4c441942088, createdName=ylzr123, createdTime=Mon Sep 05 09:06:00 CST 2016, time=2016-09-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=109459, encodeId=daef10945992, content=给我一个提醒, beContent=null, objectType=article, channel=null, level=null, likeNumber=133, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a3f71945931, createdName=1e101048m86(暂无匿称), createdTime=Fri Sep 02 10:37:00 CST 2016, time=2016-09-02, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=108354, encodeId=eb5f108354e8, content=继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=119, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Aug 31 13:05:00 CST 2016, time=2016-08-31, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=108355, encodeId=71aa10835513, content=继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=89, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Aug 31 13:05:00 CST 2016, time=2016-08-31, status=1, ipAttribution=)]
    2016-08-31 doctorJiangchao

    继续关注

    0

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