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对于无明显症状的硬膜下出血患儿,外科手术并不能改善其远期预后,但需临床严密观察,若患儿病情稳定,无须手术。
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轻度出血若能早期诊断和及时治疗,预后较好。
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严重小脑幕和大脑镰撕裂者病死率较高,存活者常发生脑积水和其他后遗症。
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第六章溶血尿毒综合征详见“第六篇重症监护和急救”之“第八章”。
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二、继发性免疫性血小板减少性紫癜由于免疫介导的继发性血小板减少性紫癜种类较多(见表10-8)。
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本文简要介绍儿科常见的继发性血小板减少性紫癜(secondarythrombocytopenicpurpura)—药物免疫性血小板减少性紫癜(drugsinducedimmunethrombocytopenicpurpura)。
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【发病机制】临床上通过免疫机制引起血小板减少的药物可达上百种,如退热镇痛药、抗生素、植物碱、镇静剂、利尿剂、强心剂、化疗药物、杀虫剂等等。
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目前所知分子量为500~1000D的药物可作为半抗原与血小板膜的一种或多种蛋白成分相结合,形成抗原并刺激机体产生特异性的抗体。
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这种抗体对药物-血小板复合物有特异性,可直接与血小板的某些成分如膜糖蛋白GPIb/Ⅸ和GPⅡb/Ⅲa等相结合,被单核-巨噬细胞系统所清除,有的亦可直接激活补体系统引起血小板破坏。
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【临床表现】从摄入药物到引起免疫性血小板减少,临床上常有一潜伏期。
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该期长短不一,奎宁及奎尼丁类可短至数小时,安替比林类约2周,吲哚美辛、金盐等可长达数月。
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临床症状取决于血小板减少程度和机体的反应,出血是在骨髓巨核细胞失代偿后出现,可有皮肤瘀点、瘀斑,鼻出血、牙龈出血等。
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严重病例全身皮肤发红,继之发热、寒战,严重出血包括口腔黏膜出血大疱、胃肠道出血、血尿、肺出血、颅内出血等;某些服用奎宁的病例兼有微血管病性贫血并发急性肾衰竭。
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【实验室检查】血小板计数降低,重者常<1×109</sup>~10×109</sup>/L,出血时间延长,血块收缩不良;骨髓巨核细胞数正常或增加并伴有成熟障碍。
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体外有一些测定抗体的办法,例如测定奎宁、奎尼丁等诱导的抗体,可将病人的血清或血浆、正常人血小板、致敏药物混合后进行免疫测定,常用的有血块退缩抑制试验,即病人血清在有相关药物存在条件下抑制了相合血型的血块收缩,表明有与该药物有关的抗体存在。
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【诊断】病史中有可疑药物史,临床上以皮肤黏膜自发性出血为主,实验室可见血小板降低,如测得药物有关的抗体即可诊断。
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【治疗】停用一切可疑药物是治疗本症的关键;糖皮质激素能改善血管完整性而减少出血,根据病情选择口服或静滴。
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具有威胁生命的严重出血时可输注血小板,也可用血浆交换以减少抗体和药物浓度。
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此外高剂量丙种球蛋白静滴,免疫抑制剂如长春新碱、环磷酰胺的使用都有帮助。
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金制剂引起者恢复慢,临床可试用二巯基丙醇增加其排泄率。
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【预后】停用有关药物后,本病能在数天至数周内恢复,极个别病例可死于严重出血如肺出血、颅内出血。
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第二节诊断方法(一)结核杆菌素试验(结素试验)结核杆菌感染后4~8周,身体对于结核蛋白产生过敏状态,此时如果作结核杆菌素试验(包括旧结核杆菌素试验OT和提纯蛋白物质PPD),局部可发生反应,表示受试者已受结核菌感染。
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PPD第1次试验液为每0.1ml含1U,第2次试验液为每0.1ml中含5U,注射后48~72小时看结果,阴性者用高一级浓度再试。
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阳性标准如下:红晕及硬肿的平均直径5~9mm为+;10~19mm为++;>20mm为+++;除红晕及硬肿外,还有疱疹或坏死,或发热等全身症状为++++。
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但BCG接种后,结核菌素试验的诊断价值受到影响。
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目前区别自然感染与BCG接种后过敏反应的方法,是根据阳性反应的强度和持久情况。
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自然感染时,结素反应多为强阳性反应(+++以上),且终生不消失;而BCG接种后多为弱阳性反应(红晕及硬肿平均直径<10mm),且持续时间短,反应逐渐减弱,一般3~5年后完全消失。
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此外,结素反应仅表示结核杆菌感染,还要根据小儿的年龄、BCG接种史及结核病接触史,判断患儿感染的活动性。
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(二)血常规检查急性期时,白细胞可增高到10×109</sup>~20×109</sup>/L,伴有中性多形核粒细胞增高淋巴细胞减少单核细胞增多中性粒细胞核左移出现中毒性颗粒。
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好转时白细胞数目恢复正常淋巴细胞增加红细胞沉降率多加速血清学检测国内外结核病血清学研究报告很多,目前主要检测结核病特异性抗体水平,尤其是抗结核IgG抗体的检测对诊断有很重要的参考价值。
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从临床应用角度来看,仍以ELISA法应用最广泛,也较为成熟。
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(四)寻找结核杆菌1.痰液标本的检测痰涂片找抗酸杆菌,通常几个小时之内就能出结果,但在儿童结核病很少阳性。
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标本中要有大量的病原菌,即每毫升5000~10000个杆菌痰涂片中才能检测到抗酸杆菌。
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如果做培养,只需10~100个杆菌即可得到阳性结果原发结核病的患儿体内病原菌数目很少,因而,即使是活动结核病的患儿,其痰涂片有95%,而培养有60%都是阴性的。
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另外,痰涂片对结核杆菌不具有特异性。
[ { "id": 0, "entity": "痰涂片", "start_offset": 3, "end_offset": 6, "label": "equ" }, { "id": 1, "entity": "结核杆菌", "start_offset": 7, "end_offset": 11, "label": "mic" } ]
一份含有非结核分枝杆菌的标本也可能抗酸杆菌涂片阳性,因而病原菌一定要用标准的培养方法和DNA探针确认。
[ { "id": 0, "entity": "非结核分枝杆菌", "start_offset": 4, "end_offset": 11, "label": "mic" }, { "id": 1, "entity": "DNA探针", "start_offset": 43, "end_offset": 48, "label": "pro" } ]
在成人和儿童,涂片或培养阴性不能除外结核病。
[ { "id": 0, "entity": "结核病", "start_offset": 18, "end_offset": 21, "label": "dis" } ]
2.胃液或支气管肺泡灌洗液标本的检测由于儿童收集痰标本困难,在小婴幼儿肺结核患者,可通过清晨吸取胃液或通过支气管肺泡灌洗液收集标本。
[ { "id": 0, "entity": "胃液", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "支气管肺泡灌洗液", "start_offset": 5, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "痰", "start_offset": 24, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "肺结核", "start_offset": 35, "end_offset": 38, "label": "dis" }, { "id": 4, "entity": "胃液", "start_offset": 48, "end_offset": 50, "label": "bod" }, { "id": 5, "entity": "支气管肺泡灌洗液", "start_offset": 53, "end_offset": 61, "label": "bod" } ]
在夜间,呼吸道的纤毛把肺部的分泌物送到大气道,这些分泌物又被吞到胃内。
[ { "id": 0, "entity": "呼吸道", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "纤毛", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "肺部", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 3, "entity": "分泌物", "start_offset": 14, "end_offset": 17, "label": "bod" }, { "id": 4, "entity": "大气道", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 5, "entity": "分泌物", "start_offset": 25, "end_offset": 28, "label": "bod" }, { "id": 6, "entity": "胃内", "start_offset": 32, "end_offset": 34, "label": "bod" } ]
清晨患儿未进食或未完全清醒时,其胃没有蠕动,此时下胃管抽取胃液做结核培养可以提供最好的标本。
[ { "id": 0, "entity": "胃", "start_offset": 16, "end_offset": 17, "label": "bod" }, { "id": 1, "entity": "下胃管", "start_offset": 24, "end_offset": 27, "label": "pro" }, { "id": 2, "entity": "胃液", "start_offset": 29, "end_offset": 31, "label": "bod" } ]
另一种获取标本的途径是支气管肺泡灌洗液,和胃液的敏感性是一样的。
[ { "id": 0, "entity": "支气管肺泡灌洗液", "start_offset": 11, "end_offset": 19, "label": "bod" }, { "id": 1, "entity": "胃液", "start_offset": 21, "end_offset": 23, "label": "bod" } ]
3.肺外结核病的标本检测在肺外结核的患者,由于感染结核的部位相对于肺部来说更难取标本,而且结核杆菌的数目较少,使获得细菌学诊断依据更为困难。
[ { "id": 0, "entity": "肺外结核病", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "肺外结核", "start_offset": 13, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "结核", "start_offset": 25, "end_offset": 27, "label": "dis" }, { "id": 3, "entity": "肺部", "start_offset": 33, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "结核杆菌", "start_offset": 45, "end_offset": 49, "label": "mic" } ]
淋巴结结核通常需要针吸或手术切除后活检。
[ { "id": 0, "entity": "淋巴结结核", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "针吸", "start_offset": 9, "end_offset": 11, "label": "pro" }, { "id": 2, "entity": "手术切除", "start_offset": 12, "end_offset": 16, "label": "pro" }, { "id": 3, "entity": "活检", "start_offset": 17, "end_offset": 19, "label": "pro" } ]
标本应该送普通细菌培养和分枝杆菌培养。
[ { "id": 0, "entity": "分枝杆菌培养", "start_offset": 12, "end_offset": 18, "label": "pro" } ]
如果培养阴性,病理染色找抗酸杆菌和肉芽肿病变就更为重要。
[ { "id": 0, "entity": "抗酸杆菌", "start_offset": 12, "end_offset": 16, "label": "mic" } ]
其他部位的结核,如腹膜、骨或皮肤结核,一般需要做活检以取得足够的标本做检查。
[ { "id": 0, "entity": "结核", "start_offset": 5, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "腹膜", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "骨", "start_offset": 12, "end_offset": 13, "label": "bod" }, { "id": 3, "entity": "皮肤", "start_offset": 14, "end_offset": 16, "label": "bod" } ]
肾结核的患者则可以从尿液中找到结核菌。
[ { "id": 0, "entity": "肾结核", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "尿液", "start_offset": 10, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "结核菌", "start_offset": 15, "end_offset": 18, "label": "mic" } ]
结核性脑膜炎的患者脑脊液的培养经常是阴性的,这是因为脑脊液中结核菌的数目相对较少的缘故。
[ { "id": 0, "entity": "结核性脑膜炎", "start_offset": 0, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "脑脊液", "start_offset": 9, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "脑脊液", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 3, "entity": "结核菌", "start_offset": 30, "end_offset": 33, "label": "mic" } ]
10ml或更多的脑脊液标本有利于提高检出的几率。
[ { "id": 0, "entity": "脑脊液", "start_offset": 8, "end_offset": 11, "label": "bod" } ]
即使是在理想条件下,也只有大约40%能从结核病患儿的胃液或支气管肺泡灌洗液标本找到结核杆菌。
[ { "id": 0, "entity": "结核病", "start_offset": 20, "end_offset": 23, "label": "dis" }, { "id": 1, "entity": "胃液", "start_offset": 26, "end_offset": 28, "label": "bod" }, { "id": 2, "entity": "支气管肺泡灌洗液", "start_offset": 29, "end_offset": 37, "label": "bod" }, { "id": 3, "entity": "结核杆菌", "start_offset": 41, "end_offset": 45, "label": "mic" } ]
婴幼儿肺结核培养的阳性率(70%)高于1岁以上的患儿(40%)。
[ { "id": 0, "entity": "婴幼儿肺结核", "start_offset": 0, "end_offset": 6, "label": "dis" } ]
如果患儿结核菌素皮试阳性,具有和结核病一致的临床表现或X线片的改变,则已符合临床诊断结核病的标准。
[ { "id": 0, "entity": "结核病", "start_offset": 16, "end_offset": 19, "label": "dis" }, { "id": 1, "entity": "X线片", "start_offset": 27, "end_offset": 30, "label": "pro" }, { "id": 2, "entity": "结核病", "start_offset": 42, "end_offset": 45, "label": "dis" } ]
结核菌素试验阴性结核病的临床表现,并和有传染性的成人结核病患者有接触史者,也认为可以临床诊断。
[ { "id": 0, "entity": "结核菌素试验", "start_offset": 0, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "结核菌素试验阴性", "start_offset": 0, "end_offset": 8, "label": "sym" }, { "id": 2, "entity": "结核病", "start_offset": 8, "end_offset": 11, "label": "dis" }, { "id": 3, "entity": "结核病", "start_offset": 26, "end_offset": 29, "label": "dis" } ]
怀疑有结核病的患儿应该进一步取标本做培养,因为培养阳性可以证实临床诊断,而且药物敏感试验的结果对治疗也非常重要。
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如果和患儿有接触的成人结核病患者培养分离出阳性结果,其药物敏感试验结果可以指导患儿的治疗,因为儿童一般都是由于家庭成员患病或和结核患者共同生活在同一居室内而感染结核病的。
[ { "id": 0, "entity": "结核病", "start_offset": 11, "end_offset": 14, "label": "dis" }, { "id": 1, "entity": "药物敏感试验", "start_offset": 27, "end_offset": 33, "label": "pro" }, { "id": 2, "entity": "结核病", "start_offset": 80, "end_offset": 83, "label": "dis" } ]
4.结核杆菌检测的新方法结核杆菌传统的检测方法存在许多缺点。
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结核杆菌在培养基中生长缓慢,每18~24小时分离一代。
[ { "id": 0, "entity": "结核杆菌", "start_offset": 0, "end_offset": 4, "label": "mic" } ]
传统的结核培养在固体培养基中要8周才能出结果,可能还需要4~8周的时间做药物敏感试验。
[ { "id": 0, "entity": "结核", "start_offset": 3, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "药物敏感试验", "start_offset": 36, "end_offset": 42, "label": "pro" } ]
而结核抗酸杆菌涂片在儿科病例阳性率极低且没有特异性。
[ { "id": 0, "entity": "结核抗酸杆菌涂片", "start_offset": 1, "end_offset": 9, "label": "pro" } ]
放射或比色法系统如BACTEC使用Middlebrook7H12液体培养基,在这种培养基中加入了促使结核杆菌生长的物质,使其在1~3周之内即能快速生长,从而得到阳性结果。
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传统的固体培养基如改良罗氏培养基(LowenssteinJenssen)和BACTEC液体培养基结合,使菌落的形态学可以被确定。
[ { "id": 0, "entity": "改良罗氏培养基", "start_offset": 9, "end_offset": 16, "label": "equ" }, { "id": 1, "entity": "LowenssteinJenssen", "start_offset": 17, "end_offset": 35, "label": "equ" }, { "id": 2, "entity": "BACTEC液体培养基", "start_offset": 37, "end_offset": 48, "label": "equ" } ]
如果培养阳性,就要用DNA探针鉴别分枝杆菌的类型,确定是否为结核分枝杆菌。
[ { "id": 0, "entity": "DNA探针", "start_offset": 10, "end_offset": 15, "label": "pro" }, { "id": 1, "entity": "分枝杆菌", "start_offset": 17, "end_offset": 21, "label": "mic" }, { "id": 2, "entity": "结核分枝杆菌", "start_offset": 30, "end_offset": 36, "label": "mic" } ]
应用最广泛的是核酸杂交技术和高效液相色谱法。
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这些技术可以区别出结核杆菌菌属,以及鸟型分枝杆菌、堪萨斯分枝杆菌、戈登分枝杆菌。
[ { "id": 0, "entity": "结核杆菌", "start_offset": 9, "end_offset": 13, "label": "mic" }, { "id": 1, "entity": "鸟型分枝杆菌", "start_offset": 18, "end_offset": 24, "label": "mic" }, { "id": 2, "entity": "堪萨斯分枝杆菌", "start_offset": 25, "end_offset": 32, "label": "mic" }, { "id": 3, "entity": "戈登分枝杆菌", "start_offset": 33, "end_offset": 39, "label": "mic" } ]
目前的方法还不能鉴定其他分枝杆菌的类型。
[ { "id": 0, "entity": "分枝杆菌", "start_offset": 12, "end_offset": 16, "label": "mic" } ]
一旦培养阳性就要使用这些技术做鉴别,但这种技术不能用于临床标本如痰液或胃液的直接检测。
[ { "id": 0, "entity": "痰液", "start_offset": 32, "end_offset": 34, "label": "bod" }, { "id": 1, "entity": "胃液", "start_offset": 35, "end_offset": 37, "label": "bod" } ]
(五)核酸扩增技术核酸扩增技术如DNA快速复制靶序列的DNA或RNA。
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由于只需一部分DNA或RNA,正在治疗中的患者体内的非存活菌,以及结核感染后极少量的临床标本,都可以聚合酶链反应(PCR)检测结果阳性。
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实验室研究报道,这种技术可以在标本中少到只有10个细菌的情况下得到阳性结果。
[ { "id": 0, "entity": "细菌", "start_offset": 25, "end_offset": 27, "label": "mic" } ]
在抗酸杆菌涂片阳性的标本,PCR的敏感度是95%,特异度是98%。
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在抗酸杆菌涂片阴性,细菌数量少的标本,PCR的特异度仍有95%,但敏感度只有50%。
[ { "id": 0, "entity": "抗酸杆菌涂片", "start_offset": 1, "end_offset": 7, "label": "equ" }, { "id": 1, "entity": "细菌", "start_offset": 10, "end_offset": 12, "label": "mic" }, { "id": 2, "entity": "PCR", "start_offset": 19, "end_offset": 22, "label": "pro" } ]
因此,PCR结果阴性仍不能除外结核病。
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在儿科的研究表明,PCR的敏感度和培养相同,约为40%;如果PCR和培养结合,并和临床诊断比较,其敏感度可增加到60%。
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如果PCR阳性,可以证实诊断,但PCR阴性绝不能除外结核病的诊断。
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在临床怀疑结核病的患者,这一检测方法的敏感度是75%~88%,特异度达100%。
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(六)影像学由于至少有50%原发结核的患儿没有临床症状,因此,在结核菌素试验阳性的患儿,无论有无症状,都应拍胸片。
[ { "id": 0, "entity": "原发结核", "start_offset": 14, "end_offset": 18, "label": "dis" }, { "id": 1, "entity": "结核菌素试验", "start_offset": 32, "end_offset": 38, "label": "pro" }, { "id": 2, "entity": "胸片", "start_offset": 54, "end_offset": 56, "label": "pro" } ]
应同时拍胸正位片和侧位片以确认有无肺部浸润拍正位片,淋巴结肿大肺部CT扫描除了有利于发现隐蔽处病灶外,在显示纵隔肺门淋巴结,肺内空洞、钙化,支气管充气像支气管扩张X线检查敏感。
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因此,肺CT对诊断有重要的价值。
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附:美国儿科学会胎儿新生儿委员会对吸入NO治疗的推荐意见1.出现低氧性呼吸衰竭的新生儿应该在(或及时转运到)具有多种呼吸支持救治手段的医疗中心治疗。
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2.给予吸入NO必须参考气体说明提供的针对指征、剂量、给药方式、监测方法等。
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建议应用多普勒彩超诊断排除先心病。
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3.吸入NO应由专门训练的、会使用多种呼吸治疗的医师指导下进行,最好具备包括ECMO支持。
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4.一般吸入NO须在具备ECMO的医院进行,或先给予NO,同时与ECMO中心保持联系,一旦吸入NO失败,可以迅速转运到ECMO中心治疗。
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转运中必须保持吸入NO治疗NO治疗不中断。
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5.治疗NO的医院必须长期随访患儿健康和神经经运动发育。
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6.建立前瞻性资料收集,如治疗时间、不良反应、治疗失败、其他特殊呼吸治疗方式的应用、生存或死亡等。
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第二节慢性胰腺炎慢性胰腺炎(chronicpancreatitis)是指胰腺局部或弥漫性的慢性进行性炎症。
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日益加重的胰实质损害导致胰腺内、外分泌功能进行性衰退。
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慢性胰腺炎在儿童中很少见。
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【病因】引起慢性胰腺炎的常见原因有三类,具体见表13-13。
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表13-15儿童慢性胰腺炎的病因【病理生理】慢性胰腺炎有两种主要的病理类型:钙化性和阻塞性。
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在儿童中,慢性钙化性胰腺炎见于遗传性胰腺炎和特发性胰腺炎。
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胰腺坚硬,手术时可触及钙石。
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当含有多种蛋白如消化酶、黏多糖以及糖蛋白的黏性塞子融合在导管腔内时,就会发生阻塞。
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也有推测有毒的代谢物加重胰腺的损害。
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胰石蛋白水平降低以及继发性钙结石的形成,可能是许多原因导致钙化性胰腺炎的共同通路。
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先天畸形或后天获得性疾病如肿瘤、纤维变性或损伤性狭窄阻塞主胰管时就会发生阻塞性胰腺炎。
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胰腺上皮发生炎症,被纤维组织替代。
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【临床表现】许多患者有反复发作的急性胰腺炎史。
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98%的胰腺功能丧失时才会出现外分泌功能不足的表现。
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