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(四)心导管目前,由于超声心动图能提供足够的解剖学及血流动力学方面的信息,故诊断性的心导管检查一般很少用。
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但是,当存在中等大小的左向右分流时,仍然需通过心导管检查明确室间隔缺损的大小,以选择具体的手术方案。
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对怀疑可能有肺血管疾病的小儿,可行心导管检查明确肺血管病变的可逆程度。
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若测得的优势氧饱和度高于正常,即说明在心室水平存在左向右分流,通过Fick原理,可计算出分流量。
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当缺损呈中至大型,肺动脉压力可以升高,对于那些肺血管阻力过大而不能手术者,可通过吸入100%氧及NO气体,经心导管估计肺血管阻力下降程度。
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同样,虽然肺活体组织检查也可了解肺血管病变,但一旦取样不当仍可造成误诊。
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左心导管可测得心室缺损的数量、大小、位置。
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位于中部和心尖部的室间隔膜周部及肌部缺损及肌部缺损可通过长轴斜径或四腔位显示,而动脉下缺损和前室间隔肌部缺损可通过右前斜径显像。
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对于需行导管闭合术的病人,一个清晰的血管造影定位尤其重要。
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升主动脉造影术用来估计伴发的主动脉瓣脱垂主动脉瓣脱垂及主动脉反流的程度(图9-9)。
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右心室造影术可显示肺动脉漏斗部的狭窄程度。
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图9-20升主动脉血管造影右前斜位片显示:右冠状尖脱垂和畸形【治疗】(一)药物治疗小型缺损者无须治疗。
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然而,在进行可能导致短暂菌血症如牙科或其他创伤性治疗以前,为避免细菌性心内膜炎的发生,需事先用抗生素预防。
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③血管扩张剂如依那普利和卡托普利能有效降低体循环的超负荷状况。
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在长期使用这些药的过程中,应定期检测血电解质、地高辛水平、肾功能情况。
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当药物治疗无效,则表明需尽早实施手术治疗。
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(二)外科治疗对于不伴其他畸形的单纯室间隔缺损,手术指征为:药物不能控制心力衰竭;有大的左向右分流,出现活动受限、反应差的;肺高压反复肺部感染者。
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若肺∶主动脉血流量大于2,说明至少存在中型缺损,需要行手术关闭缺口;该比值不到2∶1,不会出现肺动脉高压,一般先以药物治疗,1~2岁后复查心导管了解左向右分流量的变化及肺血管阻力;如果5~6岁小儿肺动脉压力仍持续高于主动脉50%,为减少肺血管疾病的发生,也需要进行手术。
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大多数病人在3~12个月时做手术,在这年龄阶段行室间隔缺损修补术,肺动脉压力可恢复正常。
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在心血管治疗中心,现在行手术修补室间隔缺损的婴儿死亡率接近于0%。
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术后早期并发症有:心室功能不良引起的心脏低位流出道综合征,完全性传导阻滞,肺动脉高压危象。
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大多室间隔缺损可经心房路径修复,此外,通过该路径亦可切除肥厚的漏斗部肌肉;动脉下型缺损可经主动脉瓣路径,但对一些肌部缺损的关闭手术必须以左心室或右心室为入口;有多个孔洞的肌部缺损小儿手术难度较大,先行肺动脉环缩术可减少分流量,1~2年后再行缺损关闭手术。
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近几年镶嵌治疗,即手术加导管治疗在治疗多发孔洞型室间隔缺损,尤其是肌部室间隔缺损越来越成熟、普遍。
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动脉下缺损并发主动脉瓣疾病是早期手术的指征,而不是取决于分流量的大小。
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但是,对于无主动脉瓣畸形的室间隔缺损,手术治疗的必要性尚有争议:有人主张为了避免主动脉瓣并发症的出现,所有的动脉下型缺损均应手术治疗。
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而最近研究表明,小于5mm的缺损不可能引起主动脉瓣畸形及主动脉反流,缺损小于5mm没有临床症状的病人可仅以药物保守治疗;对伴有严重主动脉瓣脱垂和主动脉反流的患儿,除了行缺损关闭术外,还需行主动脉瓣修复手术。
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大型室缺引起严重肺高压者,在决定是否手术以前,先要仔细了解肺血管阻力和肺血管扩张术后肺血管阻力下降程度。
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行心导管术时,控制吸入100%氧及NO气体,可观察肺血管的反应程度。
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肺循环阻力大于8Wood单位•平方米,通常列为手术禁忌。
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(三)经导管介入治疗目前,已有很多填补装置用于经导管闭合室间隔缺损管闭合室间隔缺损的治疗中。
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用于填塞缺损的装置有Clamshell伞、Rashkind伞、Sideris纽扣等。
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这些装置最大的限制在于使用时需要有大的传导系统和相关的复杂置入技术,且对于填塞物的复位、调换及残留缺损的修复则无能为力。
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近来,Amplatzer室缺填塞装置,尤其对于肌部型缺损非常有用。
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Thanopoulos等报道其对8名2~10岁患儿肌部缺损的治疗,2名患儿缺损即刻关闭,其余5名在术后24小时内缺口也关闭,1名在术后6个月仍存在小的分流残余。
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不同于肌部缺损,膜周部缺损因其接近于主动脉和三尖瓣以及缺口较大,使缺损修补难度加大。
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常见的并发症包括填塞物移位、主动脉瓣穿孔等,改良的Rashkind伞、Sideris纽扣可用于晚期并发症的修复。
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最近,新的改良Amplatzer室缺填塞装置已在临床上使用,该装置为一个左侧偏心固定圆片,在填补膜周部室间隔缺损时,不影响主动脉瓣的活动。
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但是目前大型的膜周部缺损仍需以手术治疗。
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【预后】小型缺损预后良好,自然关闭率高达75%~80%,大多在5岁内关闭。
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但小儿有患感染性心内膜炎的可能。
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大约10%~15%婴儿会发展为充血性心衰,需要药物或手术治疗;早期伴有肺动脉高压的小儿,1岁时可产生肺血管疾病,那些出生后肺血管阻力未降的儿童,青紫可为首发症状;出现艾森门格综合征的病人,多可活到40岁以上,其死因包括咯血、心力衰竭、感染性心内膜炎、脑脓疡、血栓栓塞、妊娠及心律失常引起的猝死。
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大多手术病人的远期疗效良好,少数出现心脏完全性传导阻滞,需要安置起搏器。
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至于一过性的术后心脏传导阻滞也可发生晚期猝死。
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同样,术后心室异位也易引起心室心律失常导致猝死。
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可常见小的残余分流,通常这些残余室缺不会引起血流动力学紊乱,但是仍需终生用抗生素预防细菌性心内膜炎。
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结核病特别易在缺乏营养、住房拥挤、无医疗条件和无固定住所的人群中流行。
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根据卫生部2000年进行的第四次全国结核病流行病学调查结果,我国结核菌感染率31.7%,近4.2亿人感染了结核分枝杆菌,推算2000年的年感染率为0.72%。
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肺结核的患病率为367/10万,现有肺结核患者450余万。
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每年结核病死亡的人数约13万。
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0~14岁儿童平均感染率为9.0%,全年龄组的结核感染率随年龄的增长而增高。
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我国儿童结核病流行仍然处于较高水平,结核病仍是儿童常见的传染病。
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(二)传播结核病最主要的传播途径是呼吸道。
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当含有病原菌的微粒核被结核患者咳到空气中,又被易感者吸入肺内,到达肺泡,结核菌即得到传播。
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这种极小的含有病原菌的微粒直径1~5μm,可以悬浮在空气中几个小时。
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环境因素,如和患者接触区域的空间大小、通风情况等,都影响空气中病原菌的浓度。
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与患者接触的时间长短也影响结核病传播的可能性。
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许多专家认为,结核病的传播需要易感者暴露在有结核杆菌的空气中4~8小时,因此,家庭成员间的接触是最可能的传播途径。
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活动性空洞肺结核成人咳痰且痰涂片可见抗酸杆菌(AFB)者是结核病典型的传染源。
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成人肺外结核病,除了喉结核,一般不具有传染性,因为这些患者体内的病原菌无法在空气中传播。
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曾有在接触患者的尿液和分泌物后被感染的病例发生。
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如果活的病原菌被吞下,结核杆菌也可以经胃肠道传播。
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偶尔,病原菌可以通过引流的伤口或被污染的敷料在空气中传播。
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小于12岁的儿童即使是活动性肺结核通常也没有传染性,这是因为儿童患者体内所含的病原菌数目少,不如成人咳嗽的力度大,因而不能把结核杆菌咳出来。
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有报道,医护人员可能因为接触活动结核病患儿的胃液或支气管灌洗液而感染结核,但这种病例非常少见。
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四、平滑肌肉瘤平滑肌肉瘤(leiomyosarcoma)可以发生于任何含有平滑肌的器官或组织,最多见于胃肠道;成人、儿童均如此,但成人多见。
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肿瘤质地坚韧,大小不一,中央常有坏死,无被膜覆盖光镜下细胞呈长胞质丰富,核分裂象多见。
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儿童平滑肌肉瘤,主要发生部位除胃肠道外尚包括腹膜后、气管、支气管和肺内的平滑肌。
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一般胃肠道和皮下组织的肿瘤预后较好,腹膜后、肠系膜的预后较差。
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体检可触及肿块,患儿可有贫血、腹痛、消化道出血等胃肠道的诊断可做X线钡餐造影,典型征象为边缘整齐的充盈缺损,也可有溃疡表现纤维胃镜、肠镜在诊断上都有作用。
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肿瘤可经血行转移至肝脏,或种植于大网膜、腹膜,可有淋巴结转移。
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放疗和化疗有一定的疗效。
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六、医学伦理与遗传病从遗传学的观点看,人类既有大量优质基因,也存在相当数量的有害基因,两者构成我们的基因库。
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但是,对于晚发的遗传病,是否要早期告诉病人,让他(她)在发病前的几十年中生活在阴影之中?
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诸如:保险公司或人事部门是否有权力知道每个人所携带的致病基因(个人隐私权)?
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带有“癌症基因”或“糖尿病基因”者是否会在社会上受到“基因歧视”,人们常说“癌症基因”或“糖尿病基因”,但事实是基因只是决定病症的一部分原因。
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进一步明了基因功能将帮助人们准确判断环境因素的影响,并指导我们避免或者减轻致病基因的作用。
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第四节室上性心动过速室上性心动过速(简称室上速)是小儿最常见的异位快速心律失常。
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从广义上讲,室上速是指异位激动在希氏束分叉以上的心动过速,主要由折返性机制产生,少数为自律性增高或并行心律。
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折返性室上速以房室折返性心动过速(旁道参与)最常见,其次为房室结折返性心动过速(双径路),后者的发生近年有增加的趋势。
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房性和交界性异位心动过速通常与心脏病变(如心肌病)或先天性心脏病术后有关。
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【临床表现】折返性室上速的特点是突发突止,可由急性感染促发,通常在安静时发作。
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唯一的主诉可能是患儿感到心跳快。
[ { "id": 0, "entity": "心跳快", "start_offset": 12, "end_offset": 15, "label": "sym" } ]
如发作时心率极快,或发作持续时间长,可感到心前区不适,甚至可发生心力衰竭。
[ { "id": 0, "entity": "心率", "start_offset": 4, "end_offset": 6, "label": "ite" }, { "id": 1, "entity": "心率极快", "start_offset": 4, "end_offset": 8, "label": "sym" }, { "id": 2, "entity": "心前区", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 3, "entity": "心前区不适", "start_offset": 21, "end_offset": 26, "label": "sym" }, { "id": 4, "entity": "心力衰竭", "start_offset": 32, "end_offset": 36, "label": "sym" } ]
在这一年龄段,患儿不能主诉症状,通常情况下心率本身就快,即使无室上速,哭吵时心率更快。
[ { "id": 0, "entity": "心率", "start_offset": 21, "end_offset": 23, "label": "ite" }, { "id": 1, "entity": "室上速", "start_offset": 31, "end_offset": 34, "label": "dis" }, { "id": 2, "entity": "心率", "start_offset": 38, "end_offset": 40, "label": "ite" } ]
新生儿室上速发作时为窄QRS波群(<0.08秒),仅在50%~60%的新生儿可见到P波,但在食管电极多可见到。
[ { "id": 0, "entity": "室上速", "start_offset": 3, "end_offset": 6, "label": "dis" } ]
与窦性心动过速的鉴别比较困难。
[ { "id": 0, "entity": "窦性心动过速", "start_offset": 1, "end_offset": 7, "label": "dis" } ]
如发作时心率大于230次/分,P波电轴异常(正常P波在Ⅰ导联及avF正向),则室上速的可能性大。
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此外,室上速时心率比较固定,而窦性心动过速时心率易受迷走及交感张力的变化而变化。
[ { "id": 0, "entity": "室上速", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "心率", "start_offset": 7, "end_offset": 9, "label": "ite" }, { "id": 2, "entity": "窦性心动", "start_offset": 15, "end_offset": 19, "label": "dis" }, { "id": 3, "entity": "心率", "start_offset": 22, "end_offset": 24, "label": "ite" } ]
P波缺乏,QRS波群宽大,与窦性时不同,则室速的可能性大。
[ { "id": 0, "entity": "室速", "start_offset": 21, "end_offset": 23, "label": "dis" } ]
与室上速有关的旁道有两种:隐匿性旁道与显性旁道(WPW综合征或预激综合征)。
[ { "id": 0, "entity": "室上速", "start_offset": 1, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "WPW综合征", "start_offset": 24, "end_offset": 30, "label": "dis" }, { "id": 2, "entity": "预激综合征", "start_offset": 31, "end_offset": 36, "label": "dis" } ]
室上速发作时,如冲动自房室结前传、旁道逆传,则QRS波群正常;如冲动自旁道前传,房室结逆传,则QRS波群宽大畸形。
[ { "id": 0, "entity": "室上速", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "房室结前传", "start_offset": 11, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "旁道", "start_offset": 17, "end_offset": 19, "label": "bod" } ]
有前传功能的旁道比较容易发展成更严重的心律失常,特别是如发生房性颤动时。
[ { "id": 0, "entity": "心律失常", "start_offset": 19, "end_offset": 23, "label": "sym" }, { "id": 1, "entity": "房性颤动", "start_offset": 30, "end_offset": 34, "label": "sym" } ]
【实验室检查】24小时动态心电图(HOLTER)可用于监视治疗过程及发现短阵无症状性室上速。
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电生理检查时,多极导管可置于心脏的不同部位,根据激动顺序可识别异位起搏点或旁道的位置。
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电生理检查时还可诱发心动过速,并评价药物疗效。
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【治疗】刺激迷走神经,如在年长儿将脸浸于冰水,或在婴儿将冰袋置于脸上可终止发作。
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一旦患儿转为窦性心律,应选用长效药物维持治疗。
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应该认识到,大多数抗心律失常药有致心律失常作用及负性肌收缩力作用。
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生后3、4个月内发生室上速的患儿复发的机会较年长儿低。
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【几种特殊类型的室上性心动过速】(一)心房异位性心动过速在儿科不常见,特征为心率不固定(很少超过200次/分),可见P波,但电轴异常,可慢性持续性或慢性阵发性。
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