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5.其他疾病史肝肾疾病、结缔组织病、再生障碍性贫血、白血病等可引起继发性出血。
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(二)体格检查1.出血特征注意出血部位、性质、程度,四肢高出皮面的紫癜,对称性分布者是过敏性紫癜的特点。
[ { "id": 0, "entity": "紫癜", "start_offset": 33, "end_offset": 35, "label": "sym" }, { "id": 1, "entity": "过敏性紫癜", "start_offset": 43, "end_offset": 48, "label": "sym" } ]
2.其他体征全身淋巴结及肝、脾大者提示恶性疾病如白血病、淋巴瘤等;巨脾应考虑到继发于寄生虫感染、溶血性疾病、肝硬化等的脾功能亢进;皮肤湿疹、反复感染及出血倾向应考虑到皮肤湿疹-感染-血小板减少综合征。
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(三)实验室检查实验室检查结果在出血性疾病的诊断中起着十分重要的作用,但是出、凝血的实验室检查项目繁多,临床医师必须仔细考虑病史和物理检查所揭示出的病人出血特点,结合血、大小便常规和骨髓涂片检查结果,排除由于全身性疾病引起的继发性出血,然后初步确立出血性疾病的实验室检查方向,以简易筛查试验来印证,再进一步检查确立最后诊断。
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所以,出血性疾病的诊断可分为三步,首先确定是否出血性疾病,其次明确在血管、血小板、凝血因素中何种功能障碍引起的出血,最后确立最后诊断。
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表10-13出血性疾病的简易筛查试验2.诊断试验根据临床特点及简易筛查试验结果,一般均能确定方向,明确是何种止血功能障碍引起的出血,接下来便是分门别类地确立进一步实验室检查的程序,以决定最后诊断。
[ { "id": 0, "entity": "出血性疾病", "start_offset": 6, "end_offset": 11, "label": "dis" } ]
例如疑为遗传性毛细血管扩张症者,应用毛细血管镜检查甲床可发现扭曲、排列不齐的扩张的毛细血管。
[ { "id": 0, "entity": "遗传性毛细血管扩张症", "start_offset": 4, "end_offset": 14, "label": "dis" }, { "id": 1, "entity": "毛细血管镜", "start_offset": 18, "end_offset": 23, "label": "equ" }, { "id": 2, "entity": "毛细血管", "start_offset": 41, "end_offset": 45, "label": "bod" } ]
血小板数量正常疑为功能障碍者,应分别检查阿司匹林耐量试验、血小板黏附、聚集功能试验、血小板因子3(PF3</sub>)、血小板膜糖蛋白等。
[ { "id": 0, "entity": "血小板", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "阿司匹林", "start_offset": 20, "end_offset": 24, "label": "dru" }, { "id": 2, "entity": "血小板", "start_offset": 29, "end_offset": 32, "label": "bod" }, { "id": 3, "entity": "血小板因子3", "start_offset": 42, "end_offset": 48, "label": "bod" }, { "id": 4, "entity": "血小板膜糖蛋白", "start_offset": 60, "end_offset": 67, "label": "bod" } ]
例如激活的部分凝血活酶时间(APTT)延长,凝血酶原消耗试验(PCT)不良,凝血酶原时间(PT)正常,凝血酶时间(TT)正常,当属凝血活酶生成障碍。
[ { "id": 0, "entity": "凝血活酶", "start_offset": 7, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "APTT", "start_offset": 14, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "凝血酶原", "start_offset": 22, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "PCT", "start_offset": 31, "end_offset": 34, "label": "bod" }, { "id": 4, "entity": "PT", "start_offset": 45, "end_offset": 47, "label": "ite" }, { "id": 5, "entity": "TT", "start_offset": 57, "end_offset": 59, "label": "ite" }, { "id": 6, "entity": "凝血活酶", "start_offset": 65, "end_offset": 69, "label": "bod" } ]
属于此阶段异常通常为血友病,可通过测定因子Ⅷ、Ⅸ、Ⅺ促凝血活性或APTT纠正试验以确定诊断。
[ { "id": 0, "entity": "血友病", "start_offset": 10, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "因子Ⅷ、Ⅸ、Ⅺ", "start_offset": 19, "end_offset": 26, "label": "bod" }, { "id": 2, "entity": "APTT", "start_offset": 32, "end_offset": 36, "label": "bod" } ]
当APTT延长或正常,PCT正常、PT延长、TT正常时,应属凝血酶生成障碍,涉及因子Ⅱ、Ⅴ、Ⅶ、Ⅹ等异常,可进一步作PT纠正试验。
[ { "id": 0, "entity": "APTT", "start_offset": 1, "end_offset": 5, "label": "ite" }, { "id": 1, "entity": "PCT", "start_offset": 11, "end_offset": 14, "label": "ite" }, { "id": 2, "entity": "PT", "start_offset": 17, "end_offset": 19, "label": "ite" }, { "id": 3, "entity": "TT", "start_offset": 22, "end_offset": 24, "label": "ite" }, { "id": 4, "entity": "凝血酶生成障碍", "start_offset": 30, "end_offset": 37, "label": "sym" }, { "id": 5, "entity": "PT", "start_offset": 58, "end_offset": 60, "label": "ite" } ]
参考文献1.胡亚美,江载芳.诸福棠实用儿科学.第7版.北京:人民卫生出版社,20022.江载芳.实用小儿呼吸病学.北京:人民卫生出版社,2010:2253.中华医学会儿科分会呼吸学组.儿童侵袭性肺部真菌感染诊治指南(儿童侵袭性真菌感染州医学.2009,22(3):183-1834.肖晶,周东风,孟浦.儿童侵袭性真菌感染早期诊断的研究进展.国际儿科学杂志,2009,36(5):523-5235.KlontRR,MenninkKerstenMH,RuegebrinkW,etal.ParadoxicalincreaseincirculatingHspergillusantigenduringtreatmentwithcaspofungininapatientwithpulmonaryaspergillosis.ClinInfectDis,2006,43(3):23-236.AgarwalR.Allergicbronchopulmonaryaspergillosis.Chest,2009,135:805-8267.ChabraSK,SahayandS,RamarajuK.Allergicbronchopulmonaryaspergillosiscomplicatingchildhoodasthma.IndianJPediatric,2009,76(3):331-331
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四、液体疗法时常用补液溶液常用液体包括非电解质和电解质溶液。
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其中非电解质溶液常用5%或10%葡萄糖液,因葡萄糖输入体内将被氧化成水,故属无张力溶液。
[ { "id": 0, "entity": "5%或10%葡萄糖液", "start_offset": 10, "end_offset": 20, "label": "dru" }, { "id": 1, "entity": "葡萄糖", "start_offset": 22, "end_offset": 25, "label": "dru" } ]
电解质溶液包括氯化钠、氯化钾、乳酸钠、碳酸氢钠和氯化铵等,以及它们的不同配制液(表2-2)。
[ { "id": 0, "entity": "氯化钠", "start_offset": 7, "end_offset": 10, "label": "dru" }, { "id": 1, "entity": "氯化钾", "start_offset": 11, "end_offset": 14, "label": "dru" }, { "id": 2, "entity": "乳酸钠", "start_offset": 15, "end_offset": 18, "label": "dru" }, { "id": 3, "entity": "碳酸氢钠", "start_offset": 19, "end_offset": 23, "label": "dru" }, { "id": 4, "entity": "氯化铵", "start_offset": 24, "end_offset": 27, "label": "dru" } ]
表2-11常用溶液成分续表口服补液盐(oralrehydrationsalts,ORS)ORS是世界卫生组织推荐用以治疗急性腹泻合并脱水的一种溶液,经临床应用取得了良好效果,对发展中国家尤其适用。
[ { "id": 0, "entity": "口服补液盐", "start_offset": 13, "end_offset": 18, "label": "dru" }, { "id": 1, "entity": "oralrehydrationsalts", "start_offset": 19, "end_offset": 39, "label": "dru" }, { "id": 2, "entity": "ORS", "start_offset": 40, "end_offset": 43, "label": "dru" }, { "id": 3, "entity": "ORS", "start_offset": 44, "end_offset": 47, "label": "dru" }, { "id": 4, "entity": "急性腹泻", "start_offset": 60, "end_offset": 64, "label": "dis" }, { "id": 5, "entity": "脱水", "start_offset": 66, "end_offset": 68, "label": "sym" } ]
其理论基础是基于小肠的Na+</sup>-葡萄糖偶联转运吸收机制,即小肠上皮细胞刷状缘的膜上存在着Na+</sup>-葡萄糖共同载体,此载体上有Na+</sup>-葡萄糖两个结合位点,当Na+</sup>-葡萄糖同时与结合位点相结合时即能运转,并显著增加钠和水的吸收。
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目前有多种ORS配方。
[ { "id": 0, "entity": "ORS", "start_offset": 5, "end_offset": 8, "label": "dru" } ]
WHO2002年推荐的低渗透压口服补液盐配方较传统配方减少了糖和盐的量,但同样有效,且更为安全。
[ { "id": 0, "entity": "低渗透压口服补液", "start_offset": 11, "end_offset": 19, "label": "dru" } ]
该配方中各种电解质浓度为Na+</sup>75mmol/L、K+</sup>20mmol/L、Cl-</sup>65mmol/L、枸橼酸根10mmol/L、葡萄糖75mmol/L。
[ { "id": 0, "entity": "Na+", "start_offset": 12, "end_offset": 15, "label": "ite" }, { "id": 1, "entity": "K+", "start_offset": 30, "end_offset": 32, "label": "ite" }, { "id": 2, "entity": "Cl-", "start_offset": 47, "end_offset": 50, "label": "ite" }, { "id": 3, "entity": "枸橼酸根", "start_offset": 65, "end_offset": 69, "label": "ite" }, { "id": 4, "entity": "葡萄糖", "start_offset": 78, "end_offset": 81, "label": "ite" } ]
可用NaCl2.6g、枸橼酸钠2.9g、氯化钾1.5g、葡萄糖13.5g,加水到1000ml配成。
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ORS一般适用于轻度或中度脱水无严重呕吐者,在用于补充继续损失量和生理需要量时需适当稀释。
[ { "id": 0, "entity": "ORS", "start_offset": 0, "end_offset": 3, "label": "dru" }, { "id": 1, "entity": "轻度或中度脱水", "start_offset": 8, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "严重呕吐", "start_offset": 16, "end_offset": 20, "label": "sym" } ]
第四章非青紫型先天性心脏病第一节动脉导管未闭动脉导管是由第六对支气管动脉弓远端演化而成。
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在胎儿循环时,它将大部分右室入肺动脉的血流导入降主动脉送往胎盘进行氧合。
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出生后,动脉导管未闭可作为一个独立病变存在(可单独存在),也可与其他心血管畸形合并存在,如主动脉弓缩窄或中断、严重的主动脉狭窄、左心发育不全综合征及肺动脉闭锁,严重的肺动脉狭窄或者作为血管环的一部分。
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单纯的动脉导管未闭占所有先天性心脏病的12%,占活产婴儿的0.04%~0.06%。
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【病理解剖】动脉导管常位于左侧,肺动脉端开口起自左肺动脉近主肺动脉分叉处,主动脉端开口位于主动脉弓与降主动脉之间。
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如为右位主动脉弓,动脉导管可位于右侧。
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少数左位动脉导管发生于右位主动脉弓,连接左肺动脉与左头臂动脉。
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极少数动脉导管为双侧。
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组织学上,动脉导管管壁中段主要由数层环状的沿长轴方向排列的平滑肌构成,而与肺动脉和主动脉连接之处主要由环状的弹力纤维构成。
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出生后12小时,由于中层平滑肌收缩,动脉导管出现功能性关闭。
[ { "id": 0, "entity": "平滑肌", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "动脉导管", "start_offset": 18, "end_offset": 22, "label": "bod" } ]
此后,由于内皮细胞增生、肌纤维由纤维组织替代而最终转化成纤维索,形成动脉韧带导致解剖性关闭,此过程约需3周到3个月。
[ { "id": 0, "entity": "内皮细胞", "start_offset": 5, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "肌纤维", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "纤维组织", "start_offset": 16, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "纤维索", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 4, "entity": "动脉韧带导致解剖性关闭", "start_offset": 34, "end_offset": 45, "label": "dis" } ]
【病理生理】单纯的动脉导管未闭(patentductusarteriosus)的血流方向通常由降主动脉向肺动脉分流,从而形成左向右分流。
[ { "id": 0, "entity": "动脉导管未闭", "start_offset": 9, "end_offset": 15, "label": "dis" }, { "id": 1, "entity": "patentductusarteriosus", "start_offset": 16, "end_offset": 38, "label": "dis" }, { "id": 2, "entity": "降主动脉", "start_offset": 47, "end_offset": 51, "label": "bod" }, { "id": 3, "entity": "肺动脉", "start_offset": 52, "end_offset": 55, "label": "bod" } ]
分流量取决于导管的粗细和体循环与肺循环血管阻力之间的压差。
[ { "id": 0, "entity": "导管", "start_offset": 6, "end_offset": 8, "label": "equ" }, { "id": 1, "entity": "血管", "start_offset": 19, "end_offset": 21, "label": "bod" } ]
生后早期肺血管阻力较高,分流量较小。
[ { "id": 0, "entity": "肺血管", "start_offset": 4, "end_offset": 7, "label": "bod" } ]
2个月后,随着肺血管阻力的降低,左向右分流逐渐增加。
[ { "id": 0, "entity": "肺血管", "start_offset": 7, "end_offset": 10, "label": "bod" } ]
如导管管径较粗,左向右分流量多,肺静脉回流增多,则使左心容量负荷过重,左心房、左心室扩大。
[ { "id": 0, "entity": "导管", "start_offset": 1, "end_offset": 3, "label": "equ" }, { "id": 1, "entity": "肺静脉", "start_offset": 16, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "左心", "start_offset": 26, "end_offset": 28, "label": "bod" }, { "id": 3, "entity": "左心房", "start_offset": 35, "end_offset": 38, "label": "bod" }, { "id": 4, "entity": "左心室", "start_offset": 39, "end_offset": 42, "label": "bod" } ]
左心室容量负荷过重使左室舒张末期压力增大,导致继发性左心房压力增高,最终出现左心衰竭症状。
[ { "id": 0, "entity": "左心室", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "左心房", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 2, "entity": "左心衰竭", "start_offset": 38, "end_offset": 42, "label": "sym" } ]
粗大的动脉导管未闭使肺循环血量增加过多,可造成肺动脉压力增高,体、肺循环的压力接近相等。
[ { "id": 0, "entity": "动脉导管未闭", "start_offset": 3, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "肺", "start_offset": 10, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "肺动脉", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "肺", "start_offset": 33, "end_offset": 34, "label": "bod" } ]
肺血流量的增多将会进一步减慢或阻止肺小动脉平滑肌的退化,导致持续肺动脉高压。
[ { "id": 0, "entity": "肺", "start_offset": 0, "end_offset": 1, "label": "bod" }, { "id": 1, "entity": "肺小动脉平滑肌", "start_offset": 17, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "持续肺动脉高压", "start_offset": 30, "end_offset": 37, "label": "dis" } ]
持续的大型左向右分流可使肺小动脉中膜增厚,内膜增生,导致肺小动脉破坏,形成不可逆性的肺血管病变。
[ { "id": 0, "entity": "肺小动脉中膜", "start_offset": 12, "end_offset": 18, "label": "bod" }, { "id": 1, "entity": "肺小动脉", "start_offset": 28, "end_offset": 32, "label": "bod" }, { "id": 2, "entity": "肺血管病变", "start_offset": 42, "end_offset": 47, "label": "dis" } ]
随着肺血管的进一步破坏以及肺血管阻力的增加,出现右向左分流,导致体循环血氧含量降低。
[ { "id": 0, "entity": "肺血管", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "肺血管", "start_offset": 13, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "体循环血氧", "start_offset": 32, "end_offset": 37, "label": "ite" } ]
如通过FrankStarling机制左心室舒张末期容量增大以增加每搏量;增强交感神经兴奋性以增加心率和心肌收缩力,使心输出量进一步增加。
[ { "id": 0, "entity": "左心室", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 1, "entity": "交感神经", "start_offset": 38, "end_offset": 42, "label": "bod" }, { "id": 2, "entity": "心率", "start_offset": 48, "end_offset": 50, "label": "ite" }, { "id": 3, "entity": "心肌", "start_offset": 51, "end_offset": 53, "label": "bod" }, { "id": 4, "entity": "心", "start_offset": 58, "end_offset": 59, "label": "bod" } ]
此外,大的动脉导管在心脏舒张期的窃血现象可造成肠系膜动脉在舒张期出现血流缺如甚至逆向血流,易导致坏死性小肠结肠炎。
[ { "id": 0, "entity": "动脉导管", "start_offset": 5, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "心脏", "start_offset": 10, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "窃血", "start_offset": 16, "end_offset": 18, "label": "sym" }, { "id": 3, "entity": "肠系膜动脉", "start_offset": 23, "end_offset": 28, "label": "bod" }, { "id": 4, "entity": "血流缺如", "start_offset": 34, "end_offset": 38, "label": "sym" }, { "id": 5, "entity": "逆向血流", "start_offset": 40, "end_offset": 44, "label": "sym" }, { "id": 6, "entity": "坏死性小肠结肠炎", "start_offset": 48, "end_offset": 56, "label": "dis" } ]
【临床表现】(一)早产儿血氧分压升高可使动脉导管收缩,而前列腺素E可使之扩张,此反应的灵敏程度与胎龄有关。
[ { "id": 0, "entity": "血氧分压", "start_offset": 12, "end_offset": 16, "label": "ite" }, { "id": 1, "entity": "动脉导管", "start_offset": 20, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "前列腺素E", "start_offset": 28, "end_offset": 33, "label": "dru" } ]
肺泡表面活性剂的应用改善了呼吸窘迫综合征的症状,使肺血管阻力降低,临床症状常在生后3~4天左右才开始出现。
[ { "id": 0, "entity": "肺泡", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "呼吸窘迫综合征", "start_offset": 13, "end_offset": 20, "label": "dis" }, { "id": 2, "entity": "肺血管", "start_offset": 25, "end_offset": 28, "label": "bod" } ]
肺动脉瓣区第二心音增强。
[ { "id": 0, "entity": "肺动脉瓣区", "start_offset": 0, "end_offset": 5, "label": "bod" } ]
未用呼吸机的患儿可表现为三凹征,腹部肝脏肿大。
[ { "id": 0, "entity": "呼吸机", "start_offset": 2, "end_offset": 5, "label": "equ" }, { "id": 1, "entity": "三凹征", "start_offset": 12, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "腹", "start_offset": 16, "end_offset": 17, "label": "ite" }, { "id": 3, "entity": "腹", "start_offset": 16, "end_offset": 17, "label": "bod" }, { "id": 4, "entity": "征,腹部", "start_offset": 14, "end_offset": 18, "label": "sym" } ]
(二)婴儿和年长儿婴儿或年长儿,较小的动脉导管未闭可不引起任何症状,只是在常规体检时偶然发现心脏杂音才引起重视,生长发育不受影响。
[ { "id": 0, "entity": "动脉导管未闭", "start_offset": 19, "end_offset": 25, "label": "dis" }, { "id": 1, "entity": "常规体检", "start_offset": 37, "end_offset": 41, "label": "pro" }, { "id": 2, "entity": "心脏杂音", "start_offset": 46, "end_offset": 50, "label": "sym" } ]
心搏出量正常或轻度增加,无心脏肥大或心脏搏动异常,第一、第二心音正常。
[ { "id": 0, "entity": "心搏出量", "start_offset": 0, "end_offset": 4, "label": "ite" }, { "id": 1, "entity": "无心脏肥大", "start_offset": 12, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "心脏搏动异常", "start_offset": 18, "end_offset": 24, "label": "sym" } ]
胸骨左上缘或左锁骨下可听到特征性的连续性杂音,杂音起初柔和,强度逐渐增强,到第二心音最响,至舒张期逐渐减弱。
[ { "id": 0, "entity": "胸骨左上缘或左锁骨下可听到特征性的连续性杂音", "start_offset": 0, "end_offset": 22, "label": "sym" } ]
细小的导管未闭临床可能仅表现为局限于收缩期的柔和的喷射性杂音。
[ { "id": 0, "entity": "细小的导管未闭", "start_offset": 0, "end_offset": 7, "label": "dis" } ]
心前区心尖搏动增强,呈抬举样搏动及心脏肥大的征象。
[ { "id": 0, "entity": "心前区心尖搏动增强", "start_offset": 0, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "呈抬举样搏动及心脏肥大的征象", "start_offset": 10, "end_offset": 24, "label": "sym" } ]
胸骨左上缘或左侧锁骨下可触及收缩期震颤。
[ { "id": 0, "entity": "胸骨左上缘", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "左侧锁骨下", "start_offset": 6, "end_offset": 11, "label": "bod" } ]
在有肺动脉高压时,右心室肥大,于胸骨左侧可触及心脏搏动。
[ { "id": 0, "entity": "肺动脉高压", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "右心室肥大", "start_offset": 9, "end_offset": 14, "label": "sym" }, { "id": 2, "entity": "胸骨左侧可触及心脏搏动", "start_offset": 16, "end_offset": 27, "label": "sym" } ]
听诊第二心音亢进,第一、第二心音可被响亮的动脉导管杂音所掩盖,胸骨左上缘可闻及多发的收缩期喀喇音,乃因来自动脉导管与右心室的相对血流冲撞产生湍流所致。
[ { "id": 0, "entity": "听诊", "start_offset": 0, "end_offset": 2, "label": "ite" }, { "id": 1, "entity": "第二心音亢进", "start_offset": 2, "end_offset": 8, "label": "sym" }, { "id": 2, "entity": "胸骨左上缘可闻及多发的收缩期喀喇音", "start_offset": 31, "end_offset": 48, "label": "sym" }, { "id": 3, "entity": "动脉导管", "start_offset": 53, "end_offset": 57, "label": "bod" }, { "id": 4, "entity": "右心室", "start_offset": 58, "end_offset": 61, "label": "bod" } ]
由于肺血管阻力的增加,左向右分流逐渐减少,左心衰竭症状减轻。
[ { "id": 0, "entity": "肺血管", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "左心衰竭", "start_offset": 21, "end_offset": 25, "label": "sym" } ]
症状的改变常出现于生后8~16个月,表现为脉搏减弱,心前区搏动减弱,第二心音增强且单一,舒张期杂音消失,收缩期杂音时相变短并逐渐消失。
[ { "id": 0, "entity": "脉搏减弱", "start_offset": 21, "end_offset": 25, "label": "sym" }, { "id": 1, "entity": "心前区搏动减弱", "start_offset": 26, "end_offset": 33, "label": "sym" }, { "id": 2, "entity": "第二心音增强且单一", "start_offset": 34, "end_offset": 43, "label": "sym" }, { "id": 3, "entity": "舒张期杂音消失", "start_offset": 44, "end_offset": 51, "label": "sym" }, { "id": 4, "entity": "收缩期杂音时相变短并逐渐消失", "start_offset": 52, "end_offset": 66, "label": "sym" } ]
随着右向左分流的产生,肢体末端出现发绀明显,心前区体征呈严重肺动脉高压时的表现。
[ { "id": 0, "entity": "肢体末端", "start_offset": 11, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "发绀", "start_offset": 17, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "心前区体征呈严重肺动脉高压时的表现", "start_offset": 22, "end_offset": 39, "label": "sym" } ]
生后15~18个月,肺血管即可发生不可逆性改变。
[ { "id": 0, "entity": "肺血管", "start_offset": 10, "end_offset": 13, "label": "bod" } ]
【鉴别诊断】无发绀患儿周围血管搏动明显伴连续性杂音,如杂音不在导管的典型部位,要考虑其他先天性或后天性心脏疾患,如主肺动脉窗、冠状动静脉瘘冠状动静脉瘘(coronaryarteriovenousfistula)、主动脉乏氏窦瘤破裂及伴室隔缺损的主动脉瓣反流。
[ { "id": 0, "entity": "无发绀患儿周围血管搏动明显伴连续性杂音", "start_offset": 6, "end_offset": 25, "label": "sym" }, { "id": 1, "entity": "主肺动脉窗", "start_offset": 57, "end_offset": 62, "label": "dis" }, { "id": 2, "entity": "冠状动静脉瘘", "start_offset": 63, "end_offset": 69, "label": "dis" }, { "id": 3, "entity": "冠状动静脉瘘", "start_offset": 69, "end_offset": 75, "label": "dis" }, { "id": 4, "entity": "coronaryarteriovenousfistula", "start_offset": 76, "end_offset": 104, "label": "dis" }, { "id": 5, "entity": "主动脉乏氏窦瘤破裂", "start_offset": 106, "end_offset": 115, "label": "dis" }, { "id": 6, "entity": "伴室隔缺损的主动脉瓣反流", "start_offset": 116, "end_offset": 128, "label": "dis" } ]
【检查】(一)X线胸片导管细小时胸片正常。
[ { "id": 0, "entity": "X线", "start_offset": 7, "end_offset": 9, "label": "pro" }, { "id": 1, "entity": "胸片", "start_offset": 9, "end_offset": 11, "label": "pro" }, { "id": 2, "entity": "导管", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 3, "entity": "胸片", "start_offset": 16, "end_offset": 18, "label": "pro" } ]
存在中到大量左向右分流时,左室增大,左心房增大明显,主动脉、肺动脉段突出,外周肺血管影增多,肺野充血。
[ { "id": 0, "entity": "左室", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "左心房", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 2, "entity": "主动脉", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 3, "entity": "肺动脉段", "start_offset": 30, "end_offset": 34, "label": "bod" }, { "id": 4, "entity": "肺血管", "start_offset": 39, "end_offset": 42, "label": "bod" } ]
早产儿增粗的肺血管影较难与呼吸窘迫综合征所致的肺实质病变及慢性肺部疾病相鉴别。
[ { "id": 0, "entity": "肺血管", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "呼吸窘迫综合征", "start_offset": 13, "end_offset": 20, "label": "dis" }, { "id": 2, "entity": "肺实质病变", "start_offset": 23, "end_offset": 28, "label": "dis" }, { "id": 3, "entity": "慢性肺部疾病", "start_offset": 29, "end_offset": 35, "label": "dis" } ]
(二)心电图导管细小时心电图完全正常,导管直径越大,左向右分流加大,心电图显示左室肥大,Ⅱ、Ⅲ、aVF、V5</sub>~V6</sub>等导联R波高耸,左胸导联T波倒置;左房增大呈宽大P波;如有肺动脉高压,则T波高尖,右胸导联R波增大。
[ { "id": 0, "entity": "心电图", "start_offset": 3, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "导管", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 2, "entity": "心电图", "start_offset": 11, "end_offset": 14, "label": "pro" }, { "id": 3, "entity": "导管", "start_offset": 19, "end_offset": 21, "label": "bod" }, { "id": 4, "entity": "心电图", "start_offset": 34, "end_offset": 37, "label": "pro" }, { "id": 5, "entity": "左室肥大", "start_offset": 39, "end_offset": 43, "label": "sym" }, { "id": 6, "entity": "肺动脉高压", "start_offset": 98, "end_offset": 103, "label": "dis" } ]
早产儿患肺部疾病时,右胸导联占优势。
[ { "id": 0, "entity": "肺部疾病", "start_offset": 4, "end_offset": 8, "label": "dis" } ]
(三)超声心动图二维超声和多普勒可显示动脉导管分流量的大小。
[ { "id": 0, "entity": "超声心动图", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "二维超声", "start_offset": 8, "end_offset": 12, "label": "pro" }, { "id": 2, "entity": "多普勒", "start_offset": 13, "end_offset": 16, "label": "pro" }, { "id": 3, "entity": "动脉导管", "start_offset": 19, "end_offset": 23, "label": "bod" } ]
在高位胸骨旁切面,探头置于胸骨左缘第一、二肋间隙,逆时针稍作旋转,即可得到导管大小及形态的清晰图像(图9-9)。
[ { "id": 0, "entity": "胸骨", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "探头", "start_offset": 9, "end_offset": 11, "label": "equ" }, { "id": 2, "entity": "胸骨左缘", "start_offset": 13, "end_offset": 17, "label": "bod" } ]
左心房、左心室大小可反映左心室容量负荷。
[ { "id": 0, "entity": "左心房", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "左心室", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "左心室", "start_offset": 12, "end_offset": 15, "label": "bod" } ]
M型超声左心房与主动脉根部内径比值超过1.3表示有较大的左向右的分流。
[ { "id": 0, "entity": "M型超声", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "左心房", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "主动脉", "start_offset": 8, "end_offset": 11, "label": "bod" } ]
彩色多普勒可快速显示细小的左向右分流,尤其适于检测手术后或经导管介入治疗后有无残余分流。
[ { "id": 0, "entity": "彩色多普勒", "start_offset": 0, "end_offset": 5, "label": "pro" }, { "id": 1, "entity": "经导管介入治疗", "start_offset": 29, "end_offset": 36, "label": "pro" } ]
连续多普勒超声适于估测穿过导管的射流速度,并可以此估测体循环与肺循环之间的压力差。
[ { "id": 0, "entity": "多普勒超声", "start_offset": 2, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "导管", "start_offset": 13, "end_offset": 15, "label": "equ" } ]
已知体循环动脉压后可用此方法估测肺动脉压力。
[ { "id": 0, "entity": "体循环动脉压", "start_offset": 2, "end_offset": 8, "label": "ite" }, { "id": 1, "entity": "肺动脉压力", "start_offset": 16, "end_offset": 21, "label": "ite" } ]
图9-14超声心动图示:中等大小的动脉导管未闭(箭头所示)(四)心导管现今的心导管术主要是用于动脉导管未闭封堵治疗而不是以诊断为目的。
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心导管检测肺动脉干氧饱和度升高表示动脉导管水平由左向右分流。
[ { "id": 0, "entity": "心导管检测肺动脉干氧饱和度", "start_offset": 0, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "动脉导管", "start_offset": 17, "end_offset": 21, "label": "bod" } ]
但这一指标并不特异,主肺动脉窗、双动脉下室缺亦有相似结果。
[ { "id": 0, "entity": "主肺动脉窗", "start_offset": 10, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "双动脉下室", "start_offset": 16, "end_offset": 21, "label": "bod" } ]
中到大型动脉导管未闭者肺动脉压升高。
[ { "id": 0, "entity": "动脉导管未闭", "start_offset": 4, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "肺动脉压", "start_offset": 11, "end_offset": 15, "label": "ite" } ]
因体循环舒张压下降,收缩压增加,导致体循环脉压增宽。
[ { "id": 0, "entity": "舒张压", "start_offset": 4, "end_offset": 7, "label": "ite" }, { "id": 1, "entity": "收缩压", "start_offset": 10, "end_offset": 13, "label": "ite" }, { "id": 2, "entity": "脉压", "start_offset": 21, "end_offset": 23, "label": "ite" } ]
降主动脉造影可显示动脉导管解剖学形态。
[ { "id": 0, "entity": "降主动脉造影", "start_offset": 0, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "动脉导管", "start_offset": 9, "end_offset": 13, "label": "bod" } ]
侧位造影可显示动脉导管的走向和形态(图9-9),左前斜位可减小动脉导管与降主动脉的重叠影。
[ { "id": 0, "entity": "侧位造影", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "动脉导管", "start_offset": 7, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "动脉导管", "start_offset": 31, "end_offset": 35, "label": "bod" }, { "id": 3, "entity": "降主动脉", "start_offset": 36, "end_offset": 40, "label": "bod" } ]
多数情况下主动脉端壶腹部较宽,越近肺动脉端直径越细。
[ { "id": 0, "entity": "主动脉", "start_offset": 5, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "肺动脉端", "start_offset": 17, "end_offset": 21, "label": "bod" } ]
无节段性狭窄的管样动脉导管较少见。
[ { "id": 0, "entity": "管样动脉", "start_offset": 7, "end_offset": 11, "label": "bod" } ]
心导管造影前及造影中均不可将导管穿过动脉导管,否则,由此造成的动脉导管痉挛会影响对其大小的判断,从而影响填塞装置大小的选择。
[ { "id": 0, "entity": "心导管造影", "start_offset": 0, "end_offset": 5, "label": "pro" }, { "id": 1, "entity": "导管", "start_offset": 14, "end_offset": 16, "label": "equ" }, { "id": 2, "entity": "动脉导管", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "动脉导管痉挛", "start_offset": 31, "end_offset": 37, "label": "sym" }, { "id": 4, "entity": "填塞装置", "start_offset": 52, "end_offset": 56, "label": "equ" } ]
图9-15降主动脉造影侧位片示:存在动脉导管未闭(箭头所示)【治疗】(一)早产儿对于早产儿,早期就需限制液体和钠盐的摄入量。
[ { "id": 0, "entity": "降主动脉造影", "start_offset": 5, "end_offset": 11, "label": "pro" }, { "id": 1, "entity": "动脉导管未闭", "start_offset": 18, "end_offset": 24, "label": "dis" } ]
但有肾功能不全、坏死性小肠结肠炎及有出血倾向的患儿禁用,且治疗过程中需密切检测肾功能。
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近来使用异丁苯乙酸(布洛芬)静脉滴注促进动脉导管闭合,疗效与吲哚美辛相当,且较少引起少尿。
[ { "id": 0, "entity": "异丁苯乙酸", "start_offset": 4, "end_offset": 9, "label": "dru" }, { "id": 1, "entity": "布洛芬", "start_offset": 10, "end_offset": 13, "label": "dru" }, { "id": 2, "entity": "静脉滴注", "start_offset": 14, "end_offset": 18, "label": "pro" }, { "id": 3, "entity": "动脉导管", "start_offset": 20, "end_offset": 24, "label": "bod" }, { "id": 4, "entity": "吲哚美辛", "start_offset": 30, "end_offset": 34, "label": "dru" }, { "id": 5, "entity": "少尿", "start_offset": 42, "end_offset": 44, "label": "sym" } ]
布洛芬分3次给予,首次剂量为10mg/kg,之后的24小时按每5mg/kg再用2次,导管闭合率可达70%。
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动脉导管结扎术可在新生儿重症监护室实施,以避免转往手术室过程中可能出现的紧急状态。
[ { "id": 0, "entity": "动脉导管结扎术", "start_offset": 0, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "新生儿重症监护室", "start_offset": 9, "end_offset": 17, "label": "dep" }, { "id": 2, "entity": "手术室", "start_offset": 25, "end_offset": 28, "label": "dep" } ]
对出生体重≤1000g的早产儿,在出生当天进行预防性的动脉导管结扎,经临床随机、对照研究发现可降低坏死性小肠结肠炎的发生率。
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(二)足月儿和年长儿有严重左向右分流的患儿,关闭动脉导管可以纠正心力衰竭并消除最终发展成为肺血管疾病的危险性。
[ { "id": 0, "entity": "左向右分流", "start_offset": 13, "end_offset": 18, "label": "dis" }, { "id": 1, "entity": "关闭动脉导管", "start_offset": 22, "end_offset": 28, "label": "pro" }, { "id": 2, "entity": "心力衰竭", "start_offset": 32, "end_offset": 36, "label": "dis" }, { "id": 3, "entity": "肺血管疾病", "start_offset": 45, "end_offset": 50, "label": "dis" } ]
为预防感染性心内膜,即使是小的分流也建议结扎动脉导管,尤其是这种手术的并发症和死亡率都比较低,但单纯为消除发生感染性心内膜炎的危险性而行动脉导管结扎术尚有争议。
[ { "id": 0, "entity": "感染性心内膜", "start_offset": 3, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "结扎动脉导管", "start_offset": 20, "end_offset": 26, "label": "pro" }, { "id": 2, "entity": "感染性心内膜炎", "start_offset": 55, "end_offset": 62, "label": "dis" }, { "id": 3, "entity": "动脉导管结扎术", "start_offset": 68, "end_offset": 75, "label": "pro" } ]
(三)经皮穿刺经导管动脉导管堵塞术目前,经导管介入治疗堵塞未闭的动脉导管的方法优于手术结扎。
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这一部分内容将在介入性心导管治疗术中详细介绍。
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从20世纪70年代早期到80年代后期Rashkind双伞装置被广泛使用之前,动脉导管经心导管阻塞术只是偶尔有过尝试(图9-16A)。
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但Rashkind双伞由于价格昂贵,需较大的经静脉管鞘及相对较高的近10%~20%的残余分流率,目前亦已少用。
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据报道有少数病人尚可发生血管内溶血和左肺动脉血流紊乱。
[ { "id": 0, "entity": "血管内溶血", "start_offset": 12, "end_offset": 17, "label": "dis" }, { "id": 1, "entity": "左肺动脉血流紊乱", "start_offset": 18, "end_offset": 26, "label": "dis" } ]
图9-16经导管用(A)Rashkind伞阻塞未闭的动脉导管已经过时,现多用(B)Gianturco旋管和(C)Amplatzer导管闭塞器近来,充满涤纶纤维的不锈钢弹簧圈正在越来越多地应用于小到中型的动脉导管的堵封闭(图9-9B)。
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具有可控性释放机制的可拆卸弹簧圈因其潜在的易控制、易于取回重新放置的优点而得到发展。
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弹簧圈的优势是价格便宜,只需要较小的递送导管,封堵率达95%以上。
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