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【诊断】仔细询问病史，如患者存在维生素A摄入不足，或者存在维生素A的吸收、利用障碍，或引起维生素A消耗过多的疾病，同时合并暗适应障碍、夜盲、结膜干燥、角膜软化或四肢伸侧有毛囊性角化丘疹，通过暗适应检查和血浆维生素A浓度的测定可基本作出诊断。 | [
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"entity": "血浆维生素A浓度的测定",
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WHO推荐的诊断标准为：血清维生素A＜0.7μmolL为维生素A缺乏；0.7～1.4μmolL为亚临床维生素A缺乏（维生素A存在不足）；1.4～2.79μmolL为维生素贮存充足。 | [
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若血清维生素A水平在正常低值，此时肝内维生素A的储存也可能已耗竭。 | [
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在这种可疑的情况下，可采用敏感而可靠的相对剂量反应试验来进一步确定亚临床维生素A的缺乏。 | [
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亚临床维生素A缺乏已成为儿童广泛的营养缺乏症而受关注。 | [
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亚临床维生素A缺乏是指儿童因维生素A摄入不足导致的轻度维生素A缺乏，其特点是无典型的临床表现。 | [
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【治疗】如患儿因为疾病引起维生素A缺乏，应首先去除病因，同时给予维生素A丰富的饮食。 | [
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每天补充维生素A2.5万U（1U的维生素A=0.3μg的视黄醇），口服或肌注均可，共1～2周（或大剂量1次20万U），同时给予高蛋白饮食，以后再给予预防量。 | [
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如有角膜软化则给水溶性维生素A10万U，1周后再给20万U，然后给预防量。 | [
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夜盲症可于治疗后数小时好转，干眼于2～3天后改善。 | [
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必要时保持两眼清洁，使用抗生素眼膏，角膜溃疡者用1%阿托品滴眼防止虹膜粘连。 | [
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【预防】应提倡母乳喂养，对稍大的儿童，应及时添加含有维生素A的辅食，如鱼肝油、动物肝脏、肾脏、蛋黄、胡萝卜汁及番茄汁等，避免偏食，增加维生素A的摄入量，避免维生素A的缺乏。 | [
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如小儿因患有疾病而影响了维生素A吸收和利用时，应首先去除病因，然后及时补充维生素A。 | [
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维生素A每天推荐摄入量婴儿期为1500U，12岁以下的儿童为1500～2500U，如饮食中维生素A含量丰富，可不必另外补充维生素A。 | [
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四、治疗ICH治疗原则是迅速控制出血，适时进行外科手术治疗，预防并发症与后遗症。 | [
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（一）稳定治疗稳定治疗措施包括优化呼吸管理、控制体循环高血压、防治癫痫发作和针对颅内压增高的医学管理等。 | [
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ICH急性期应绝对卧床，保持安静，不宜搬动，避免引起血压增高和颅内压增高的因素。 | [
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如因特殊情况如急诊检查和手术治疗等，需要搬动病人，应保持头部固定。 | [
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还应保持水电解质平衡及足够的热量供给。 | [
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另外，针对蛛网膜下腔出血患儿来说，控制血管痉挛。 | [
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而对于一般出血病例，需要待病人病情稳定后再实施手术治疗，包括清除血肿和对局部畸形血管的处理等，通常以发病后2周左右为宜。 | [
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目前尚无明显证据显示幕上实质内血肿外科手术摘除术对任何年龄都有效。 | [
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Mendelow及其同事研究显示，在1033名非外伤性幕上出血的成人随机试验中，在血肿发生24小时内进行血肿取出术对病人无明显受益。 | [
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另外一项小样本研究，给予了较早（小于4小时）血肿取出术的11名病例中，有4例因为再出血给予了暂停早期血肿清除手术。 | [
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也有无对照研究证据显示，在选择人群中血肿清除可能缓解脑疝发生。 | [
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这种外科手术对于小脑出血以及大脑半球较大范围出血病灶病人可能获益更多。 | [
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反复腰穿放脑脊液适用于脑室及蛛网膜下腔出血患者，可减少脑积水的发生，并可迅速缓解蛛网膜下腔出血所引起的颅内高压，减轻脑膜刺激症状。 | [
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但如果患儿头痛剧烈、呕吐频繁或极度烦躁，甚至已出现脑疝的早期征象，则应禁忌腰穿，以免诱发脑疝。 | [
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硬膜下穿刺适用于硬膜下出血的治疗，前囟未闭。 | [
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穿刺1次，穿刺成功后应让液体自动流出，不应抽吸，每次引流量不宜过大，一般不超过15ml，否则可能诱发再出血。 | [
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可穿刺10～15次，液体量不多者逐渐延长间隔并停止穿刺。 | [
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脑血管畸形的手术处理可以防止再次破裂出血。 | [
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对凝血缺陷和血液系统疾病的治疗可减少继发性出血的危险。 | [
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血小板计数在200×109</sup>/L以上时脑出血很少发生。 | [
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即使血小板数很低，在没有创伤的情况下，自发性颅内出血极少见。 | [
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"end_offset": 5,
"label": "bod"
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] |
获得性同种免疫血小板减少症患者的脑出血通常伴有全身性病毒感染，可能是由于感染刺激机体产生大量的抗血小板抗体，导致血小板减少。 | [
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{
"id": 3,
"entity": "血小板减少",
"start_offset": 56,
"end_offset": 61,
"label": "sym"
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] |
同样，Ⅶ因子缺乏患儿通过补充Ⅶ因子可减少或预防外伤性颅内出血。 | [
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] |
（四）康复治疗ICH患儿在病情好转后即应进行医学康复训练，包括物理治疗、作业治疗和语言治疗等。 | [
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"entity": "ICH",
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"label": "dis"
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"id": 1,
"entity": "医学康复训练",
"start_offset": 22,
"end_offset": 28,
"label": "pro"
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{
"id": 2,
"entity": "物理治疗",
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"label": "pro"
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"id": 3,
"entity": "作业治疗",
"start_offset": 36,
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"label": "pro"
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{
"id": 4,
"entity": "语言治疗",
"start_offset": 41,
"end_offset": 45,
"label": "pro"
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] |
还应辅以针灸、推拿、理疗以及高压氧等，以减轻神经损害后遗症。 | [
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"entity": "高压氧",
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同时可给予心理支持和行为治疗。 | [
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儿童ICH治疗和评估推荐如下：1.Ⅰ级推荐（1）对于非外伤性脑出血患儿，当非侵入性检查不能确定原因时，应该进行全面的危险因素评估，包括常规脑血管造影，从而在再次脑出血发生前确定可治疗的危险因素（Ⅰ级，C水平证据）。 | [
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（2）严重凝血因子缺乏症患儿需要接受凝血因子替代治疗（Ⅰ级，A水平证据）。 | [
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（3）先天性血管异常患儿有发生反复脑出血的危险，这种损害应该予以及时识别和矫治。 | [
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（4）脑出血病人的稳定治疗包括呼吸治疗、控制血压、控制癫痫发作和降低颅内压（Ⅰ级，C水平证据）。 | [
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"id": 4,
"entity": "降低颅内压",
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"end_offset": 37,
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] |
2.Ⅱ级推荐（1）对于无临床症状的颅内动脉瘤病人，有必要每1～5年应用颅脑MRA进行追踪随访（Ⅱa级，C水平证据）。 | [
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"entity": "颅脑MRA",
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当病人出现可以用颅内动脉瘤解释的临床症状时，即使颅脑MRA未发现颅内动脉瘤，也应考虑采用CT血管造影（CTangiography，CTA）或导管血管造影（catheterangiography，CA）检查（Ⅱb级，C水平证据）。 | [
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"entity": "CTangiography",
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"id": 5,
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] |
然而，对于可能发生脑疝或颅内压很高的患者，应该进行外科手术治疗。 | [
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（2）尽管有证据表明镰状细胞病（sicklecelldisease，SCD）患者周期性的输血会造成缺血性梗死，但没有证据表明镰状细胞病患者周期性的输血能够减少ICH的发生率（Ⅲ级，B水平证据）。 | [
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"id": 3,
"entity": "ICH",
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] |
参考文献1.中华医学会.临床诊疗指南（癫痫病分册）.北京：人民卫生出版社，20072.吴逊，林庆.神经病学（第13卷：癫痫和发作性疾病）.北京：人民军医出版社，20013.周水珍，孙道开.抗癫痫新药的临床应用.中国实用儿科杂志，1997，12（6）：372-3724.周水珍.儿童癫痫发作与非癫痫发作.中国临床神经科学杂志，2000，8（4）：2975.周水珍.小儿癫痫抗癫痫药物治疗相关问题及其对策.实用儿科临床杂志，2006，21（24）：1686-16866.吴希如.癫痫遗传学的研究进展.中华儿科杂志，2000，38（11）：717-7177.周水珍，陈天兰，孙道开.非惊厥性癫痫持续状态的临床分析.中华儿科杂志，1995，33（5）：2908.林庆.小儿癫痫发作的分类及最新进展.中华儿科杂志，2002，40（5）：313-3139.蒋梨，蔡方成.小儿癫痫的诊断.中华儿科杂志，2002，40（6）：380-38010.魏克伦，孙梅，刘春峰（主译）.儿科临床手册（HandbookofPediatrics）.北京：人民卫生出版社，200111.ToniaSabo-Graham，AlanR.ManagementofStatusEpilepticusinChildren.PediatricinReview.1998；19（9）：306-30612.ChristopherC，Giza，RamanSankar.Pathogenesisofthedevelopmentalepilepsis.CurrentOpinioninPediatrics.1998，10：567-56713.AbdelazizY，HarbA，HishamM.TextbookofClinicalPediatrics.USA：LippincottWilliamsandWilkins.200114.EngelJJr.Aproposeddiagnosticschemeforpeoplewithepilepticseizuresandwithepilepsy：reportoftheILAEtaskforceonclassificationandterminology.Epilepsia.2001，42：796-80315.RamaMaganti，PaulaGerber，CorneliaDrees，et，al.Nonconvulsivestatusepilepticus.Epilepsy＆Behavior.12（2008）572-57216.JeanAicardi，MartinB，ChristopherGillberg.ClinicinDevelopmentalMedicine：DiseasesoftheNervoussysteminChildhood3rd</sup>Edition.MacKeithPress，2009 | [
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{
"id": 3,
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{
"id": 5,
"entity": "癫痫",
"start_offset": 95,
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{
"id": 6,
"entity": "儿科",
"start_offset": 109,
"end_offset": 111,
"label": "dep"
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{
"id": 7,
"entity": "儿童癫痫",
"start_offset": 138,
"end_offset": 142,
"label": "dis"
},
{
"id": 8,
"entity": "非癫痫发作",
"start_offset": 145,
"end_offset": 150,
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"entity": "临床",
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"entity": "小儿癫痫",
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"id": 20,
"entity": "儿科",
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] |
四、超声内镜超声内镜术（EUS）是超声与内镜相结合的一种新的检测技术，在内镜的顶端安置微型超声探头，当内镜插入消化道后，既可通过内镜直接观察消化道黏膜的形态学变化，又可通过内镜顶端的超声探头直接接触消化道黏膜进行扫描，从而获得管壁各层次的组织学特性及周围重要脏器的超声影像，增加了内镜的诊断范畴，提高了内镜的诊断能力。 | [
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"start_offset": 6,
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{
"id": 4,
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"label": "equ"
},
{
"id": 5,
"entity": "内镜",
"start_offset": 36,
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"label": "equ"
},
{
"id": 6,
"entity": "微型超声探头",
"start_offset": 43,
"end_offset": 49,
"label": "equ"
},
{
"id": 7,
"entity": "内镜",
"start_offset": 51,
"end_offset": 53,
"label": "equ"
},
{
"id": 8,
"entity": "消化道",
"start_offset": 55,
"end_offset": 58,
"label": "bod"
},
{
"id": 9,
"entity": "内镜",
"start_offset": 64,
"end_offset": 66,
"label": "equ"
},
{
"id": 10,
"entity": "消化道黏膜",
"start_offset": 70,
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"label": "bod"
},
{
"id": 11,
"entity": "内镜顶端的超声探头",
"start_offset": 86,
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"label": "equ"
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"id": 12,
"entity": "消化道黏膜",
"start_offset": 99,
"end_offset": 104,
"label": "bod"
},
{
"id": 13,
"entity": "管壁",
"start_offset": 113,
"end_offset": 115,
"label": "bod"
},
{
"id": 14,
"entity": "脏器",
"start_offset": 129,
"end_offset": 131,
"label": "bod"
},
{
"id": 15,
"entity": "内镜",
"start_offset": 140,
"end_offset": 142,
"label": "equ"
},
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"id": 16,
"entity": "内镜",
"start_offset": 151,
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] |
内镜超声扫描有2种方式：①水囊法：于内镜顶端超声探头的周围固定一橡皮囊，通过内镜的固定管道孔注入脱气水3～5ml。 | [
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] |
适用于食管、十二指肠升部和降部的超声扫描。 | [
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②脱气水充盈法：通过内镜的固定管道向胃腔内注入脱气水300～600ml，使胃壁膨胀，超声探头完全浸入水中。 | [
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{
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},
{
"id": 3,
"entity": "超声探头",
"start_offset": 42,
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"label": "equ"
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] |
适用于观察胃壁的各层结构及胃周邻近脏器，例如肝、胆、胰、脾脏及门静脉等。 | [
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"entity": "胃壁",
"start_offset": 5,
"end_offset": 7,
"label": "bod"
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"id": 1,
"entity": "肝",
"start_offset": 22,
"end_offset": 23,
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},
{
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"entity": "胆",
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},
{
"id": 4,
"entity": "门静脉",
"start_offset": 31,
"end_offset": 34,
"label": "bod"
}
] |
此外，在消化道腔内进行超声扫描明显缩短了超声探头与靶器官的距离，避免了腹壁脂肪、肠管积气和骨骼系统对超声波传导的影响和干扰，使位于腹壁深部的器官病变显示更清晰。 | [
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{
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{
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},
{
"id": 7,
"entity": "腹壁深部",
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}
] |
因而超声胃镜不仅具备内镜和超声双重功能，而且提高了内镜和超声在某些方面的诊断水平，弥补了这两种影像诊断的不足点。 | [
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五、尿液微生物学检查对诊断感染性疾病有意义。 | [
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}
] |
包括：（一）细菌学检查1.直接细菌学检查取尿10ml，1500转/分离心30分钟，沉渣涂片行革兰染色、抗酸染色（结核杆菌）以及吕氏美蓝染色（淋病萘瑟菌）以发现病原菌。 | [
{
"id": 0,
"entity": "细菌学检查",
"start_offset": 6,
"end_offset": 11,
"label": "pro"
},
{
"id": 1,
"entity": "直接细菌学检查",
"start_offset": 13,
"end_offset": 20,
"label": "pro"
},
{
"id": 2,
"entity": "尿",
"start_offset": 21,
"end_offset": 22,
"label": "ite"
},
{
"id": 3,
"entity": "涂片",
"start_offset": 43,
"end_offset": 45,
"label": "pro"
},
{
"id": 4,
"entity": "革兰染色",
"start_offset": 46,
"end_offset": 50,
"label": "pro"
},
{
"id": 5,
"entity": "抗酸染色",
"start_offset": 51,
"end_offset": 55,
"label": "pro"
},
{
"id": 6,
"entity": "结核杆菌",
"start_offset": 56,
"end_offset": 60,
"label": "mic"
},
{
"id": 7,
"entity": "吕氏美蓝染色",
"start_offset": 63,
"end_offset": 69,
"label": "pro"
},
{
"id": 8,
"entity": "淋病萘瑟菌",
"start_offset": 70,
"end_offset": 75,
"label": "mic"
},
{
"id": 9,
"entity": "病原菌",
"start_offset": 79,
"end_offset": 82,
"label": "mic"
}
] |
2.细菌培养可作一般细菌、结核杆菌、厌氧菌及L型菌培养（滴养）并进行菌落计数，以鉴别是否为尿路感染。 | [
{
"id": 0,
"entity": "细菌培养",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "结核杆菌",
"start_offset": 13,
"end_offset": 17,
"label": "mic"
},
{
"id": 2,
"entity": "厌氧菌",
"start_offset": 18,
"end_offset": 21,
"label": "mic"
},
{
"id": 3,
"entity": "L型菌",
"start_offset": 22,
"end_offset": 25,
"label": "mic"
},
{
"id": 4,
"entity": "滴养",
"start_offset": 28,
"end_offset": 30,
"label": "pro"
},
{
"id": 5,
"entity": "菌落计数",
"start_offset": 34,
"end_offset": 38,
"label": "ite"
},
{
"id": 6,
"entity": "尿路感染",
"start_offset": 45,
"end_offset": 49,
"label": "dis"
}
] |
菌落数＞105</sup>/ml则为感染，菌落数＜103</sup>/ml多为污染，介于两者之间为可疑。 | [
{
"id": 0,
"entity": "菌落数",
"start_offset": 0,
"end_offset": 3,
"label": "ite"
},
{
"id": 1,
"entity": "感染",
"start_offset": 18,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "菌落数",
"start_offset": 21,
"end_offset": 24,
"label": "ite"
}
] |
如果定量接种前在室温下放置时间超过2小时，即使计数≥105</sup>/ml亦可能是污染。 | [
{
"id": 0,
"entity": "定量接种",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
}
] |
3.免疫荧光检查检查抗体包裹细菌可用于区别上、下泌尿道感染以及非淋菌性尿道炎的诊断。 | [
{
"id": 0,
"entity": "免疫荧光检查",
"start_offset": 2,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "检查抗体包裹细菌",
"start_offset": 8,
"end_offset": 16,
"label": "pro"
},
{
"id": 2,
"entity": "上、下泌尿道感染",
"start_offset": 21,
"end_offset": 29,
"label": "dis"
},
{
"id": 3,
"entity": "非淋菌性尿道炎",
"start_offset": 31,
"end_offset": 38,
"label": "dis"
}
] |
（二）其他病原微生物包括病毒、支原体和衣原体等，一般采用培养分离，免疫荧光检查等方法。 | [
{
"id": 0,
"entity": "病原微生物",
"start_offset": 5,
"end_offset": 10,
"label": "mic"
},
{
"id": 1,
"entity": "病毒",
"start_offset": 12,
"end_offset": 14,
"label": "mic"
},
{
"id": 2,
"entity": "支原体",
"start_offset": 15,
"end_offset": 18,
"label": "mic"
},
{
"id": 3,
"entity": "衣原体",
"start_offset": 19,
"end_offset": 22,
"label": "mic"
},
{
"id": 4,
"entity": "采用培养分离",
"start_offset": 26,
"end_offset": 32,
"label": "pro"
},
{
"id": 5,
"entity": "免疫荧光检查",
"start_offset": 33,
"end_offset": 39,
"label": "pro"
}
] |
第三节恶性组织细胞增生症本组疾病中包括急性单核细胞性白血病（急单）、真正的恶性组织细胞病、恶性组织细胞肉瘤和少见的恶性郎罕细胞病，参见恶性肿瘤有关章节。 | [
{
"id": 0,
"entity": "恶性组织细胞增生症",
"start_offset": 3,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "急性单核细胞性白血病",
"start_offset": 19,
"end_offset": 29,
"label": "dis"
},
{
"id": 2,
"entity": "急单",
"start_offset": 30,
"end_offset": 32,
"label": "dis"
},
{
"id": 3,
"entity": "恶性组织细胞病",
"start_offset": 37,
"end_offset": 44,
"label": "dis"
},
{
"id": 4,
"entity": "恶性组织细胞肉瘤",
"start_offset": 45,
"end_offset": 53,
"label": "dis"
},
{
"id": 5,
"entity": "恶性郎罕细胞病",
"start_offset": 57,
"end_offset": 64,
"label": "dis"
},
{
"id": 6,
"entity": "恶性肿瘤",
"start_offset": 67,
"end_offset": 71,
"label": "dis"
}
] |
第八节右位心右位心（dextrocardia）由Fabricius在1606年首先报道，右位心伴内脏位置正常者在活产婴儿中的发生率在1∶7500～1∶29000之间，而在儿科中，单纯性右位心较右位心伴内脏反位右位心及90%的孤立性右位心伴有先天性心脏畸形，多数属青紫型。 | [
{
"id": 0,
"entity": "右位心",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "右位心",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 2,
"entity": "dextrocardia",
"start_offset": 10,
"end_offset": 22,
"label": "dis"
},
{
"id": 3,
"entity": "右位心",
"start_offset": 44,
"end_offset": 47,
"label": "dis"
},
{
"id": 4,
"entity": "内脏",
"start_offset": 48,
"end_offset": 50,
"label": "bod"
},
{
"id": 5,
"entity": "右位心",
"start_offset": 96,
"end_offset": 99,
"label": "dis"
},
{
"id": 6,
"entity": "内脏",
"start_offset": 100,
"end_offset": 102,
"label": "bod"
},
{
"id": 7,
"entity": "内脏反位",
"start_offset": 100,
"end_offset": 104,
"label": "sym"
},
{
"id": 8,
"entity": "右位心",
"start_offset": 104,
"end_offset": 107,
"label": "dis"
},
{
"id": 9,
"entity": "右位心",
"start_offset": 115,
"end_offset": 118,
"label": "dis"
},
{
"id": 10,
"entity": "先天性心脏畸形",
"start_offset": 120,
"end_offset": 127,
"label": "dis"
}
] |
【胚胎发生学】正常情况下原始心管在胚胎发育22～23天时向右侧成襻，在此后10～12天，心脏及大血管开始分隔，心尖开始由右侧胸腔迁移至左侧胸腔的正常位置。 | [
{
"id": 0,
"entity": "心管",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 1,
"entity": "心脏",
"start_offset": 44,
"end_offset": 46,
"label": "bod"
},
{
"id": 2,
"entity": "血管",
"start_offset": 48,
"end_offset": 50,
"label": "bod"
},
{
"id": 3,
"entity": "心尖",
"start_offset": 55,
"end_offset": 57,
"label": "bod"
},
{
"id": 4,
"entity": "胸腔",
"start_offset": 62,
"end_offset": 64,
"label": "bod"
},
{
"id": 5,
"entity": "胸腔",
"start_offset": 69,
"end_offset": 71,
"label": "bod"
}
] |
在内脏位置正常时，右位心被认为是由于此迁移过程缺如所致。 | [
{
"id": 0,
"entity": "内脏",
"start_offset": 1,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "右位心",
"start_offset": 9,
"end_offset": 12,
"label": "dis"
}
] |
同样，在内脏位置反位者，同样已有从左向右的心脏迁移过程。 | [
{
"id": 0,
"entity": "内脏",
"start_offset": 4,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "心脏",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
}
] |
【命名与分类】（一）右移心（dextroposition）或称为继发性右位心，由于外部因素将心脏整体推向右侧胸腔。 | [
{
"id": 0,
"entity": "右移心",
"start_offset": 10,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "dextroposition",
"start_offset": 14,
"end_offset": 28,
"label": "dis"
},
{
"id": 2,
"entity": "继发性右位心",
"start_offset": 32,
"end_offset": 38,
"label": "dis"
},
{
"id": 3,
"entity": "心脏",
"start_offset": 46,
"end_offset": 48,
"label": "bod"
},
{
"id": 4,
"entity": "胸腔",
"start_offset": 54,
"end_offset": 56,
"label": "bod"
}
] |
（二）孤立性右位心（isolateddextrocardia）内脏位置正常，心尖位于右侧胸腔。 | [
{
"id": 0,
"entity": "孤立性右位心",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "isolateddextrocardia",
"start_offset": 10,
"end_offset": 30,
"label": "dis"
},
{
"id": 2,
"entity": "内脏",
"start_offset": 31,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "心尖",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 4,
"entity": "胸腔",
"start_offset": 44,
"end_offset": 46,
"label": "bod"
}
] |
（三）混合性右位心（mixeddextrocardia）右位心伴房室连接不一致。 | [
{
"id": 0,
"entity": "混合性右位心",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "mixeddextrocardia",
"start_offset": 10,
"end_offset": 27,
"label": "dis"
},
{
"id": 2,
"entity": "房室",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
}
] |
（四）镜像右位心（mirrordextrocardia）内脏反位，心脏正常位于右侧胸腔。 | [
{
"id": 0,
"entity": "mirrordextrocardia",
"start_offset": 9,
"end_offset": 27,
"label": "dis"
},
{
"id": 1,
"entity": "内脏",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
},
{
"id": 2,
"entity": "心脏",
"start_offset": 33,
"end_offset": 35,
"label": "bod"
},
{
"id": 3,
"entity": "胸腔",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
}
] |
因此，应先将右移心排除后，依据内脏位置确定右位心的类型。 | [
{
"id": 0,
"entity": "右移心",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "内脏",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "右位心",
"start_offset": 21,
"end_offset": 24,
"label": "dis"
}
] |
【临床诊断】尽管右位心可通过体格检查发现，但通常需经胸部X线平片进行诊断。 | [
{
"id": 0,
"entity": "右位心",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "体格检查",
"start_offset": 14,
"end_offset": 18,
"label": "pro"
}
] |
右位心可在在新生儿表现为青紫、呼吸困难和心脏杂音右位心是在常规体格检查时发现。 | [
{
"id": 0,
"entity": "右位心",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "青紫",
"start_offset": 12,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "心脏",
"start_offset": 20,
"end_offset": 22,
"label": "bod"
},
{
"id": 3,
"entity": "心脏杂音",
"start_offset": 20,
"end_offset": 24,
"label": "sym"
},
{
"id": 4,
"entity": "右位心",
"start_offset": 24,
"end_offset": 27,
"label": "dis"
},
{
"id": 5,
"entity": "体格检查",
"start_offset": 31,
"end_offset": 35,
"label": "pro"
}
] |
右位心伴发有膈疝等其他先天畸形时提示伴有心内结构异常。 | [
{
"id": 0,
"entity": "右位心",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "心内结构异常",
"start_offset": 20,
"end_offset": 26,
"label": "sym"
}
] |
胸片不但可用于排除心外原因引起的右移心肝脏及胃泡位置、气管支气管类型的判断确定内脏位置而对右位心进行分类。 | [
{
"id": 0,
"entity": "胸片",
"start_offset": 0,
"end_offset": 2,
"label": "ite"
},
{
"id": 1,
"entity": "心",
"start_offset": 9,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "右移心",
"start_offset": 16,
"end_offset": 19,
"label": "dis"
},
{
"id": 3,
"entity": "肝脏",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
},
{
"id": 4,
"entity": "胃泡",
"start_offset": 22,
"end_offset": 24,
"label": "bod"
},
{
"id": 5,
"entity": "气管",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
},
{
"id": 6,
"entity": "支气管",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 7,
"entity": "内脏",
"start_offset": 39,
"end_offset": 41,
"label": "bod"
},
{
"id": 8,
"entity": "右位心",
"start_offset": 45,
"end_offset": 48,
"label": "dis"
}
] |
同时尚需明确有无间隔缺损瓣膜反流或狭窄肺静脉回流心脏伴发畸形心导管造影检查明确诊断。 | [
{
"id": 0,
"entity": "间隔",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "间隔缺损",
"start_offset": 8,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "瓣膜",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 3,
"entity": "瓣膜反流或狭窄",
"start_offset": 12,
"end_offset": 19,
"label": "sym"
},
{
"id": 4,
"entity": "肺静脉",
"start_offset": 19,
"end_offset": 22,
"label": "bod"
},
{
"id": 5,
"entity": "肺静脉回流",
"start_offset": 19,
"end_offset": 24,
"label": "sym"
},
{
"id": 6,
"entity": "心脏",
"start_offset": 24,
"end_offset": 26,
"label": "bod"
},
{
"id": 7,
"entity": "心脏伴发畸形",
"start_offset": 24,
"end_offset": 30,
"label": "sym"
},
{
"id": 8,
"entity": "心导管造影检查",
"start_offset": 30,
"end_offset": 37,
"label": "pro"
}
] |
【伴发畸形】（一）内脏正位孤立性右位心绝大多数伴有心内结构畸形。 | [
{
"id": 0,
"entity": "内脏",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 1,
"entity": "孤立性右位心",
"start_offset": 13,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "心内结构畸形",
"start_offset": 25,
"end_offset": 31,
"label": "sym"
}
] |
心室大血管连接不一致发生率较高，近半数合并有房室连接不一致及心室大血管连接不一致，即纠正型大血管转位。 | [
{
"id": 0,
"entity": "心室大血管连接不一致",
"start_offset": 0,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "房室连接不一致",
"start_offset": 22,
"end_offset": 29,
"label": "sym"
},
{
"id": 2,
"entity": "心室大血管连接不一致",
"start_offset": 30,
"end_offset": 40,
"label": "sym"
},
{
"id": 3,
"entity": "纠正型大血管转位",
"start_offset": 42,
"end_offset": 50,
"label": "sym"
}
] |
肺动脉狭窄或闭锁常见，占60%，右位主动脉弓少见，占5%。 | [
{
"id": 0,
"entity": "肺动脉狭窄或闭锁",
"start_offset": 0,
"end_offset": 8,
"label": "sym"
},
{
"id": 1,
"entity": "右位主动脉弓",
"start_offset": 16,
"end_offset": 22,
"label": "sym"
}
] |
短弯刀综合征是一种少见但合并右位心的心血管畸形。 | [
{
"id": 0,
"entity": "短弯刀综合征",
"start_offset": 0,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "右位心",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "心血管畸形",
"start_offset": 18,
"end_offset": 23,
"label": "dis"
}
] |
主要表现为右位心、心房内脏位置正位右肺发育不良异常体动脉血流供应右肺右肺静脉异常引流入上腔静脉胸部X线平片上，异位引流的肺静脉在右肺野表现为“短弯刀”样阴影。 | [
{
"id": 0,
"entity": "右位心",
"start_offset": 5,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "心房",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "内脏",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 3,
"entity": "心房内脏位置正位",
"start_offset": 9,
"end_offset": 17,
"label": "sym"
},
{
"id": 4,
"entity": "右肺",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 5,
"entity": "右肺发育不良",
"start_offset": 17,
"end_offset": 23,
"label": "sym"
},
{
"id": 6,
"entity": "体动脉",
"start_offset": 25,
"end_offset": 28,
"label": "bod"
},
{
"id": 7,
"entity": "右肺",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 8,
"entity": "异常体动脉血流供应右肺",
"start_offset": 23,
"end_offset": 34,
"label": "sym"
},
{
"id": 9,
"entity": "右肺",
"start_offset": 34,
"end_offset": 36,
"label": "bod"
},
{
"id": 10,
"entity": "静脉",
"start_offset": 36,
"end_offset": 38,
"label": "bod"
},
{
"id": 11,
"entity": "上腔静脉",
"start_offset": 43,
"end_offset": 47,
"label": "bod"
},
{
"id": 12,
"entity": "右肺静脉异常引流入上腔静脉",
"start_offset": 34,
"end_offset": 47,
"label": "sym"
},
{
"id": 13,
"entity": "胸部X线平片",
"start_offset": 47,
"end_offset": 53,
"label": "pro"
},
{
"id": 14,
"entity": "肺静脉",
"start_offset": 60,
"end_offset": 63,
"label": "bod"
},
{
"id": 15,
"entity": "右肺野",
"start_offset": 64,
"end_offset": 67,
"label": "bod"
}
] |
临床上，在婴儿可表现为青紫、呼吸困难、活动后气促，通常伴有肺动脉高压心内复杂畸形反复上呼吸道感染胸部X线表现。 | [
{
"id": 0,
"entity": "青紫",
"start_offset": 11,
"end_offset": 13,
"label": "sym"
},
{
"id": 1,
"entity": "呼吸困难",
"start_offset": 14,
"end_offset": 18,
"label": "sym"
},
{
"id": 2,
"entity": "活动后气促",
"start_offset": 19,
"end_offset": 24,
"label": "sym"
},
{
"id": 3,
"entity": "肺动脉",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 4,
"entity": "肺动脉高压",
"start_offset": 29,
"end_offset": 34,
"label": "sym"
},
{
"id": 5,
"entity": "心内",
"start_offset": 34,
"end_offset": 36,
"label": "bod"
},
{
"id": 6,
"entity": "心内复杂畸形",
"start_offset": 34,
"end_offset": 40,
"label": "sym"
},
{
"id": 7,
"entity": "呼吸道",
"start_offset": 43,
"end_offset": 46,
"label": "bod"
},
{
"id": 8,
"entity": "反复上呼吸道感染",
"start_offset": 40,
"end_offset": 48,
"label": "sym"
},
{
"id": 9,
"entity": "胸部X线",
"start_offset": 48,
"end_offset": 52,
"label": "pro"
}
] |
（二）内脏反位右位心伴内脏反位较前者少见。 | [
{
"id": 0,
"entity": "内脏反位",
"start_offset": 3,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "右位心伴内脏反位",
"start_offset": 7,
"end_offset": 15,
"label": "dis"
}
] |
在已报告的病例中，近1/3至2/3合并有右心室双出口、肺动脉狭窄或闭锁及室间隔缺损。 | [
{
"id": 0,
"entity": "右心室双出口",
"start_offset": 20,
"end_offset": 26,
"label": "sym"
},
{
"id": 1,
"entity": "肺动脉狭窄",
"start_offset": 27,
"end_offset": 32,
"label": "sym"
},
{
"id": 2,
"entity": "室间隔缺损",
"start_offset": 36,
"end_offset": 41,
"label": "sym"
}
] |
主动脉弓通常为右位。 | [
{
"id": 0,
"entity": "主动脉弓通常为右位",
"start_offset": 0,
"end_offset": 9,
"label": "sym"
}
] |
表9-9右位心伴内脏正位及右位心伴内脏反位伴发畸形比较尽管绝大多数内脏反位的患儿心脏结构正常，但肺部疾患的发病率较正常儿有明显升高。 | [
{
"id": 0,
"entity": "右位心",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "内脏",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "畸形",
"start_offset": 23,
"end_offset": 25,
"label": "sym"
},
{
"id": 3,
"entity": "内脏反位",
"start_offset": 33,
"end_offset": 37,
"label": "dis"
},
{
"id": 4,
"entity": "心脏",
"start_offset": 40,
"end_offset": 42,
"label": "bod"
},
{
"id": 5,
"entity": "肺部",
"start_offset": 48,
"end_offset": 50,
"label": "bod"
}
] |
第二节新生儿窒息新生儿窒息（asphyxiaofnewborn）是指由于产前、产时或产后的各种病因，在生后1分钟内无自主呼吸或未能建立规律呼吸，导致低氧血症和高碳酸血症，若持续存在，可出现代谢性酸中毒。 | [
{
"id": 0,
"entity": "新生儿窒息",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "新生儿窒息",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "asphyxiaofnewborn",
"start_offset": 14,
"end_offset": 31,
"label": "dis"
},
{
"id": 3,
"entity": "低氧血症",
"start_offset": 74,
"end_offset": 78,
"label": "dis"
},
{
"id": 4,
"entity": "高碳酸血症",
"start_offset": 79,
"end_offset": 84,
"label": "dis"
},
{
"id": 5,
"entity": "代谢性酸中毒",
"start_offset": 94,
"end_offset": 100,
"label": "dis"
}
] |
在分娩过程中，胎儿的呼吸和循环系统经历剧烈变化，绝大多数胎儿能够顺利完成这种从子宫内到子宫外环境的转变，从而建立有效的呼吸和循环，保证机体新陈代谢和各器官功能的正常，仅有少数患儿发生窒息。 | [
{
"id": 0,
"entity": "呼吸和循环系统",
"start_offset": 10,
"end_offset": 17,
"label": "bod"
},
{
"id": 1,
"entity": "子宫内",
"start_offset": 39,
"end_offset": 42,
"label": "bod"
},
{
"id": 2,
"entity": "子宫外",
"start_offset": 43,
"end_offset": 46,
"label": "bod"
},
{
"id": 3,
"entity": "窒息",
"start_offset": 91,
"end_offset": 93,
"label": "dis"
}
] |
国外文献报道活产婴儿的围生期窒息发生率约为1%～1.5%，而胎龄大于36周仅为5‰。 | [
{
"id": 0,
"entity": "窒息",
"start_offset": 14,
"end_offset": 16,
"label": "dis"
}
] |
【病因】窒息的本质是缺氧，凡能造成胎儿或新生儿血氧浓度降低的因素均可引起窒息，一种病因可通过不同途经影响机体，也可多种病因同时作用。 | [
{
"id": 0,
"entity": "窒息",
"start_offset": 4,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "缺氧",
"start_offset": 10,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "血氧浓度",
"start_offset": 23,
"end_offset": 27,
"label": "ite"
},
{
"id": 3,
"entity": "胎儿或新生儿血氧浓度降低",
"start_offset": 17,
"end_offset": 29,
"label": "sym"
},
{
"id": 4,
"entity": "窒息",
"start_offset": 36,
"end_offset": 38,
"label": "dis"
}
] |
常见病因如下：1.孕母因素①缺氧性疾病：如呼吸衰竭、青紫型先天性心脏病、严重贫血及CO中毒等；②障碍胎盘循环的疾病：如充血性心力衰竭、妊娠高血压综合征、慢性肾炎、失血、休克、糖尿病和感染性疾病等；③其他：孕母吸毒、吸烟或被动吸烟、孕母年龄≥35岁或＜16岁、多胎妊娠等，其胎儿窒息发生率增高。 | [
{
"id": 0,
"entity": "缺氧性疾病",
"start_offset": 14,
"end_offset": 19,
"label": "dis"
},
{
"id": 1,
"entity": "呼吸衰竭",
"start_offset": 21,
"end_offset": 25,
"label": "dis"
},
{
"id": 2,
"entity": "青紫型先天性心脏病",
"start_offset": 26,
"end_offset": 35,
"label": "dis"
},
{
"id": 3,
"entity": "严重贫血",
"start_offset": 36,
"end_offset": 40,
"label": "dis"
},
{
"id": 4,
"entity": "CO中毒",
"start_offset": 41,
"end_offset": 45,
"label": "dis"
},
{
"id": 5,
"entity": "胎盘",
"start_offset": 50,
"end_offset": 52,
"label": "bod"
},
{
"id": 6,
"entity": "充血性心力衰竭",
"start_offset": 59,
"end_offset": 66,
"label": "dis"
},
{
"id": 7,
"entity": "妊娠高血压综合征",
"start_offset": 67,
"end_offset": 75,
"label": "dis"
},
{
"id": 8,
"entity": "慢性肾炎",
"start_offset": 76,
"end_offset": 80,
"label": "dis"
},
{
"id": 9,
"entity": "失血",
"start_offset": 81,
"end_offset": 83,
"label": "dis"
},
{
"id": 10,
"entity": "休克",
"start_offset": 84,
"end_offset": 86,
"label": "dis"
},
{
"id": 11,
"entity": "糖尿病",
"start_offset": 87,
"end_offset": 90,
"label": "dis"
},
{
"id": 12,
"entity": "感染性疾病",
"start_offset": 91,
"end_offset": 96,
"label": "dis"
},
{
"id": 13,
"entity": "多胎妊娠",
"start_offset": 129,
"end_offset": 133,
"label": "sym"
},
{
"id": 14,
"entity": "窒息",
"start_offset": 138,
"end_offset": 140,
"label": "dis"
}
] |
2.胎盘异常如前置胎盘、胎盘早剥和胎盘功能不全等。 | [
{
"id": 0,
"entity": "胎盘",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "前置胎盘",
"start_offset": 7,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "胎盘早剥",
"start_offset": 12,
"end_offset": 16,
"label": "dis"
},
{
"id": 3,
"entity": "胎盘功能不全",
"start_offset": 17,
"end_offset": 23,
"label": "dis"
}
] |
3.脐带异常如脐带受压、过短、过长致绕颈或绕体、脱垂、扭转或打结等。 | [
{
"id": 0,
"entity": "脐带",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "脐带",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "脐带受压、过短、过长致绕颈或绕体、脱垂、扭转或打结",
"start_offset": 7,
"end_offset": 32,
"label": "sym"
}
] |
4.分娩因素如难产、高位产钳、臀位、胎头吸引不顺利；产程中麻醉药、镇痛药及催产药使用不当等。 | [
{
"id": 0,
"entity": "难产",
"start_offset": 7,
"end_offset": 9,
"label": "sym"
},
{
"id": 1,
"entity": "高位产钳",
"start_offset": 10,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "麻醉药",
"start_offset": 29,
"end_offset": 32,
"label": "dru"
},
{
"id": 3,
"entity": "镇痛药",
"start_offset": 33,
"end_offset": 36,
"label": "dru"
},
{
"id": 4,
"entity": "催产药",
"start_offset": 37,
"end_offset": 40,
"label": "dru"
}
] |
5.胎儿因素①早产儿、小于胎龄儿、巨大儿等；②各种畸形如后鼻孔闭锁、喉蹼、肺膨胀不全、先天性心脏病及宫内感染所致神经系统受损等；③胎粪吸入致使呼吸道阻塞等。 | [
{
"id": 0,
"entity": "畸形",
"start_offset": 25,
"end_offset": 27,
"label": "sym"
},
{
"id": 1,
"entity": "后鼻孔",
"start_offset": 28,
"end_offset": 31,
"label": "bod"
},
{
"id": 2,
"entity": "后鼻孔闭锁",
"start_offset": 28,
"end_offset": 33,
"label": "sym"
},
{
"id": 3,
"entity": "喉蹼",
"start_offset": 34,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "肺膨胀不全",
"start_offset": 37,
"end_offset": 42,
"label": "sym"
},
{
"id": 5,
"entity": "先天性心脏病",
"start_offset": 43,
"end_offset": 49,
"label": "dis"
},
{
"id": 6,
"entity": "宫内感染",
"start_offset": 50,
"end_offset": 54,
"label": "dis"
},
{
"id": 7,
"entity": "神经系统",
"start_offset": 56,
"end_offset": 60,
"label": "bod"
},
{
"id": 8,
"entity": "神经系统受损",
"start_offset": 56,
"end_offset": 62,
"label": "sym"
},
{
"id": 9,
"entity": "胎粪",
"start_offset": 65,
"end_offset": 67,
"label": "bod"
},
{
"id": 10,
"entity": "呼吸道",
"start_offset": 71,
"end_offset": 74,
"label": "bod"
},
{
"id": 11,
"entity": "呼吸道阻塞",
"start_offset": 71,
"end_offset": 76,
"label": "sym"
}
] |
若由于上述各种病因导致窒息，则出现一系列病理生理变化。 | [
{
"id": 0,
"entity": "窒息",
"start_offset": 11,
"end_offset": 13,
"label": "dis"
}
] |
（一）窒息后细胞损伤缺氧可导致细胞代谢及功能障碍和结构异常甚至死亡，是细胞损伤从可逆到不可逆的演变过程。 | [
{
"id": 0,
"entity": "窒息",
"start_offset": 3,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "缺氧",
"start_offset": 10,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "细胞",
"start_offset": 35,
"end_offset": 37,
"label": "bod"
}
] |
不同细胞对缺氧的易感性各异，其中脑细胞最敏感，其次是心肌、肝和肾上腺细胞，而纤维、上皮及骨骼肌细胞对缺氧的耐受性较强。 | [
{
"id": 0,
"entity": "细胞",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "缺氧",
"start_offset": 5,
"end_offset": 7,
"label": "sym"
},
{
"id": 2,
"entity": "脑细胞",
"start_offset": 16,
"end_offset": 19,
"label": "bod"
},
{
"id": 3,
"entity": "心肌",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 4,
"entity": "肝",
"start_offset": 29,
"end_offset": 30,
"label": "bod"
},
{
"id": 5,
"entity": "肾上腺细胞",
"start_offset": 31,
"end_offset": 36,
"label": "bod"
},
{
"id": 6,
"entity": "纤维",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 7,
"entity": "上皮",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 8,
"entity": "骨骼肌细胞",
"start_offset": 44,
"end_offset": 49,
"label": "bod"
},
{
"id": 9,
"entity": "缺氧",
"start_offset": 50,
"end_offset": 52,
"label": "sym"
}
] |
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