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大量文献报道，该方法弥补了症状分析及内镜检查的局限性，对鉴别生理性与病理性GER，深入了解GER与食管炎的关系，特别是对GERD的诊断与疗效判定提供了可靠的依据。 | [
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【治疗】GERD的治疗一般根据症状的轻重不同可分为非系统性治疗、系统性内科治疗和外科手术治疗。 | [
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目的在于加强食管的抗反流防御机制，减少胃食管反流；减缓症状，预防和治疗并发症以及防止复发。 | [
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（一）非系统性治疗对于症状较轻、无器质性病变的患儿可采用保守疗法，通过改变饮食和体位来达到治疗目的。 | [
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在此基础上如仍有症状可服用抗酸剂制酸剂、黏膜保护剂及促胃动力药。 | [
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1.抑制酸分泌药（1）H2</sub>受体阻滞剂：它能阻断组胺与壁细胞膜上H2</sub>受体结合，从而减少胃酸分泌，减少反流物的酸度和量。 | [
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临床上常用的有西咪替丁、雷尼替丁和法莫替丁等。 | [
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（2）质子泵抑制剂：它通过抑制壁细胞上的H+-K+-ATP酶活力阻断胃酸的分泌。 | [
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目前认为，质子泵抑制剂能更快地缓解反流症状，加速反流性食管炎的愈合，尤其对中重度食管炎及其并发症，此药应作为首选。 | [
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有研究证实，质子泵抑制剂在成人中长期使用（1年以上）能有效控制GERD并且安全。 | [
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在儿童，曾有研究人员对患有GERD的弱智儿童群体长期随访，证实该类药物对各种程度的反流性食管炎都相当有效，且未发现不良反应。 | [
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由此可见，质子泵抑制剂是一种有效且安全的GERD治疗药。 | [
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2.黏膜保护剂常用的为铝碳酸镁。 | [
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其独特的网络状结构，不仅可以迅速中和胃酸，还能吸附胆汁，对胃酸和胆汁反流引起的症状均有较好的疗效。 | [
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另外，临床上还经常使用硫糖铝及蒙脱石散，能增加黏膜对酸的抵抗力及促进黏膜上皮皮的修复。 | [
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3.促胃动力药GERD是一种上消化道动力障碍性疾病，因此，对GERD的治疗首先应该改善消化道动力。 | [
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（1）甲氧氯普胺：为周围及中枢神经系统多巴胺受体拮抗剂，能促进内源性乙酰胆碱的释放，增加食管收缩幅度并促进胃排空。 | [
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但因其对神经系统副作用明显，故临床上逐渐少用。 | [
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（2）多潘立酮：此药为外周多巴胺受体拮抗剂，能促进胃排空，协调胃、十二指肠运动，增强食管蠕动和LES张力。 | [
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该药对血-脑屏障渗透力差，对脑内多巴胺受体几乎无抑制作用，故无精神与神经不良反应，但1岁以下婴儿血-脑屏障功能发育尚不完全，仍应慎用。 | [
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（3）西沙比利：为第三代胃肠动力药。 | [
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它通过促进胃肠道肌层神经丛副交感神经节后纤维乙酰胆碱释放来加强食管、胃、小肠及结肠的推进性运动，加快胃肠道排空，增加食管下端括约肌张力。 | [
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（三）抗反流手术儿科GERD需要进行手术治疗的比较少见，大约仅占5%～15%，这些患儿往往是由于食管外症状，如反复吸入性肺炎及窒息等呼吸道症状，才需要手术治疗。 | [
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当前，抗反流手术的方式很多，国外开展最多的是Nissan胃底折叠术。 | [
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其机制是人工造成一个加强的食管下端高压区以利抵抗胃内容物反流。 | [
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另外，近年来利用腹腔镜腔镜下行Nissan胃底折叠术日益增多。 | [
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Lobe和Schier分别在1993和1994年报道了小儿GERD在腹腔镜下的Nissan术。 | [
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理论上，腹腔镜下胃底折叠术有手术更安全、损伤更小以及恢复时间更快等优点，但对它的远期疗效尚有争议。 | [
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有研究显示，这种方法的远期疗效无论从临床上还是各种检查上，都显示出很高的失败率，尤其在重度GERD患者中。 | [
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然而，这一技术无疑为小儿GERD的治疗开辟了新途径，并且随着这一新技术的日益成熟，它必将在GERD治疗中发挥重要作用。 | [
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参考文献1.BredaL，NozziM，DeSanctisS，etal.Laboratorytestsinthediagnosisandfollow-upofpediatricrheumaticdiseases：anupdate.SeminArthritisRheum.2010，40（1）：53-532.MokMY.TheimmunologicalbasisofB-celltherapyinsystemiclupuserythematosus.IntRheumDis.2010，13（1）：3-33.陈瑜，王宏伟，周建华.儿童系统性红斑狼疮国内外诊断标准比较.实用儿科临床杂志，2007，22（9）：715-7154.BehrmanRE，KliegmanRM，JensonHB.NelsonTextbookofPediatrics.16th</sup>ed.Philadelphia：W.B.SaunersCo，20005.杨锡强等主编.儿童免疫学.北京：人民卫生出版社，20016.PeterM.Izmirly，CarolinaLlanos，LelaA.Lee，etal.Cutaneousmanifestationsofneonatallupusandriskofsubsequentcongenitalheartblock.Arthritis＆Rheumatism，2010，62（4）：1153-1153 | [
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第六章中枢神经系统肿瘤一、概述中枢神经系统肿瘤是小儿时期比较常见的肿瘤之一，其发生率在15岁以下儿童肿瘤中占据第二位。 | [
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美国1973～1989年流行病学调查中发现，每年10万个儿童中就有约2.8例患颅内肿瘤。 | [
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颅内肿瘤好发年龄主要在二个年龄段，第一高峰在10岁以内，发病率为2.2/10万～2.5/10万人口/年，男性稍多于女性（1.1∶1）。 | [
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第二高峰从30～40岁开始，60岁以上达到顶点，这一阶段为肿瘤的最好发时期。 | [
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【病因】中枢神经系统肿瘤尚未发现确切的病因，但是目前根据某些肿瘤发病特点、病理以及一些基础实验研究，提出几种学说。 | [
{
"id": 0,
"entity": "中枢神经系统肿瘤",
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"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "肿瘤",
"start_offset": 30,
"end_offset": 32,
"label": "dis"
}
] |
1.遗传学说在神经外科领域中，某些肿瘤具有明显的家族倾向性，如视网膜母细胞瘤、血管网织细胞瘤、多发性神经纤维瘤等，一般认为它们均为常染色体显性遗传性肿瘤，外显率很高。 | [
{
"id": 0,
"entity": "神经外科",
"start_offset": 7,
"end_offset": 11,
"label": "dep"
},
{
"id": 1,
"entity": "肿瘤",
"start_offset": 17,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "视网膜母细胞瘤",
"start_offset": 31,
"end_offset": 38,
"label": "dis"
},
{
"id": 3,
"entity": "血管网织细胞瘤",
"start_offset": 39,
"end_offset": 46,
"label": "dis"
},
{
"id": 4,
"entity": "常染色体",
"start_offset": 65,
"end_offset": 69,
"label": "bod"
},
{
"id": 5,
"entity": "肿瘤",
"start_offset": 74,
"end_offset": 76,
"label": "dis"
}
] |
2.病毒学说实验研究表明，一些病毒包括DNA病毒和RNA病毒，若接种于动物脑内可诱发脑瘤。 | [
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"id": 0,
"entity": "DNA病毒",
"start_offset": 19,
"end_offset": 24,
"label": "mic"
},
{
"id": 1,
"entity": "RNA病毒",
"start_offset": 25,
"end_offset": 30,
"label": "mic"
},
{
"id": 2,
"entity": "脑瘤",
"start_offset": 42,
"end_offset": 44,
"label": "dis"
}
] |
3.理化学说物理因素中被确认的具有致肿瘤可能的是放射线，已有许多关于头颅放疗后引起颅内肿瘤的报道。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 18,
"end_offset": 20,
"label": "dis"
},
{
"id": 1,
"entity": "头颅放疗",
"start_offset": 34,
"end_offset": 38,
"label": "pro"
},
{
"id": 2,
"entity": "颅内肿瘤",
"start_offset": 41,
"end_offset": 45,
"label": "dis"
}
] |
4.免疫抑制学说器官移植免疫抑制剂的应用，会增加颅内或外周肿瘤发生的风险。 | [
{
"id": 0,
"entity": "器官移植",
"start_offset": 8,
"end_offset": 12,
"label": "pro"
},
{
"id": 1,
"entity": "免疫抑制剂",
"start_offset": 12,
"end_offset": 17,
"label": "dru"
},
{
"id": 2,
"entity": "颅内或外周肿瘤",
"start_offset": 24,
"end_offset": 31,
"label": "dis"
}
] |
5.胚胎残余学说颅咽管瘤、上皮样及皮样囊肿、畸胎瘤、脊索瘤明显发生于残留于脑内的胚胎组织，这些残余组织具有增殖分化的潜力，在一定条件下可发展为肿瘤。 | [
{
"id": 0,
"entity": "颅咽管瘤",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "上皮样及皮样囊肿",
"start_offset": 13,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "畸胎瘤",
"start_offset": 22,
"end_offset": 25,
"label": "dis"
},
{
"id": 3,
"entity": "脊索瘤",
"start_offset": 26,
"end_offset": 29,
"label": "dis"
},
{
"id": 4,
"entity": "脑内",
"start_offset": 37,
"end_offset": 39,
"label": "bod"
},
{
"id": 5,
"entity": "胚胎组织",
"start_offset": 40,
"end_offset": 44,
"label": "bod"
},
{
"id": 6,
"entity": "肿瘤",
"start_offset": 71,
"end_offset": 73,
"label": "dis"
}
] |
【分类】中枢神经系统颅内肿瘤分类方法很多，比较具有代表性的主要有以下几种（见表11-11）。 | [
{
"id": 0,
"entity": "中枢神经系统颅内肿瘤",
"start_offset": 4,
"end_offset": 14,
"label": "dis"
}
] |
Balley和Cushing的分类能反映肿瘤组织的来源及恶性程度，长期以来被世界多数地区的神经外科和病理科所采用。 | [
{
"id": 0,
"entity": "肿瘤组织",
"start_offset": 20,
"end_offset": 24,
"label": "bod"
},
{
"id": 1,
"entity": "神经外科",
"start_offset": 45,
"end_offset": 49,
"label": "dep"
},
{
"id": 2,
"entity": "病理科",
"start_offset": 50,
"end_offset": 53,
"label": "dep"
}
] |
在此基础上衍生出一些新的分类方法，如Kernohan的Ⅰ～Ⅳ级分类法，Russell的胶质瘤分类。 | [
{
"id": 0,
"entity": "Russell的胶质瘤",
"start_offset": 35,
"end_offset": 46,
"label": "dis"
}
] |
但此分类法也有缺陷，如同一肿瘤在不同部位，细胞分化即可不同，有些混合瘤无法分级，而有些肿瘤分级意义不大。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 13,
"end_offset": 15,
"label": "dis"
},
{
"id": 1,
"entity": "肿瘤",
"start_offset": 43,
"end_offset": 45,
"label": "dis"
}
] |
在此基础上世界卫生组织制定了新的分类法，其特点是分类细致，包括了颅内发生的各种肿瘤类型，吸收了既往各种分类的特点和长处，既反映了肿瘤的形态学，又表明肿瘤的来源，并采用间变这一概念，可认为是目前最好的分类。 | [
{
"id": 0,
"entity": "颅内",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 1,
"entity": "肿瘤",
"start_offset": 39,
"end_offset": 41,
"label": "dis"
},
{
"id": 2,
"entity": "肿瘤",
"start_offset": 64,
"end_offset": 66,
"label": "dis"
},
{
"id": 3,
"entity": "肿瘤",
"start_offset": 74,
"end_offset": 76,
"label": "dis"
}
] |
【临床表现】中枢神经系统肿瘤的发病机制及其临床表现，具有缓慢进行性加重的特征。 | [
{
"id": 0,
"entity": "中枢神经系统肿瘤",
"start_offset": 6,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "具有缓慢进行性加重的特征",
"start_offset": 26,
"end_offset": 38,
"label": "sym"
}
] |
不同的年龄，不同部位的肿瘤，其临床表现各不相同。 | [
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"id": 0,
"entity": "肿瘤",
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] |
1.颅内压增高中枢神经系统肿瘤分类比较肿瘤压迫、浸润脑组织、阻塞脑脊液增高头痛呕吐、嗜睡及视神经乳头水肿间隙性头痛学习成绩下降、易疲劳及性格改变。 | [
{
"id": 0,
"entity": "颅内压",
"start_offset": 2,
"end_offset": 5,
"label": "ite"
},
{
"id": 1,
"entity": "颅内压增高",
"start_offset": 2,
"end_offset": 7,
"label": "sym"
},
{
"id": 2,
"entity": "中枢神经系统肿瘤",
"start_offset": 7,
"end_offset": 15,
"label": "dis"
},
{
"id": 3,
"entity": "脑组织",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
},
{
"id": 4,
"entity": "脑脊液",
"start_offset": 32,
"end_offset": 35,
"label": "bod"
},
{
"id": 5,
"entity": "肿瘤压迫、浸润脑组织、阻塞脑脊液增高",
"start_offset": 19,
"end_offset": 37,
"label": "sym"
},
{
"id": 6,
"entity": "头",
"start_offset": 37,
"end_offset": 38,
"label": "bod"
},
{
"id": 7,
"entity": "头痛",
"start_offset": 37,
"end_offset": 39,
"label": "sym"
},
{
"id": 8,
"entity": "呕吐",
"start_offset": 39,
"end_offset": 41,
"label": "sym"
},
{
"id": 9,
"entity": "视神经乳头",
"start_offset": 45,
"end_offset": 50,
"label": "bod"
},
{
"id": 10,
"entity": "嗜睡及视神经乳头水肿",
"start_offset": 42,
"end_offset": 52,
"label": "sym"
},
{
"id": 11,
"entity": "头",
"start_offset": 55,
"end_offset": 56,
"label": "bod"
},
{
"id": 12,
"entity": "间隙性头痛",
"start_offset": 52,
"end_offset": 57,
"label": "sym"
},
{
"id": 13,
"entity": "学习成绩下降",
"start_offset": 57,
"end_offset": 63,
"label": "sym"
},
{
"id": 14,
"entity": "疲劳及性格改变",
"start_offset": 65,
"end_offset": 72,
"label": "sym"
}
] |
在婴幼儿期多表现激惹、食欲下降、生长发育延迟甚至退步、头围异常增大骨缝分离及两眼太阳落山征神经系统定位症状。 | [
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"id": 0,
"entity": "激惹",
"start_offset": 8,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "食欲下降",
"start_offset": 11,
"end_offset": 15,
"label": "sym"
},
{
"id": 2,
"entity": "生长发育延迟甚至退步",
"start_offset": 16,
"end_offset": 26,
"label": "sym"
},
{
"id": 3,
"entity": "头",
"start_offset": 27,
"end_offset": 28,
"label": "bod"
},
{
"id": 4,
"entity": "头围异常增大",
"start_offset": 27,
"end_offset": 33,
"label": "sym"
},
{
"id": 5,
"entity": "骨",
"start_offset": 33,
"end_offset": 34,
"label": "bod"
},
{
"id": 6,
"entity": "两眼",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 7,
"entity": "骨缝分离及两眼太阳落山征",
"start_offset": 33,
"end_offset": 45,
"label": "sym"
},
{
"id": 8,
"entity": "神经系统",
"start_offset": 45,
"end_offset": 49,
"label": "bod"
}
] |
续表2.癫痫癫痫是颅内肿瘤常见的症状之一，大约1/4的儿童幕上肿瘤是以癫痫为首发症状，其发生率仅次于头痛，位居第二。 | [
{
"id": 0,
"entity": "癫痫",
"start_offset": 4,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "癫痫",
"start_offset": 6,
"end_offset": 8,
"label": "dis"
},
{
"id": 2,
"entity": "颅内肿瘤",
"start_offset": 9,
"end_offset": 13,
"label": "dis"
},
{
"id": 3,
"entity": "儿童幕上肿瘤",
"start_offset": 27,
"end_offset": 33,
"label": "dis"
},
{
"id": 4,
"entity": "癫痫",
"start_offset": 35,
"end_offset": 37,
"label": "dis"
},
{
"id": 5,
"entity": "头痛",
"start_offset": 50,
"end_offset": 52,
"label": "dis"
}
] |
出现以下情况应高度怀疑颅内占位性病变的存在：有癫痫史的病人其癫痫类型发生了改变；癫痫持续难治性癫痫伴有局部神经系统损害的癫痫癫痫以及多数难以解释的全身发作性癫痫需做CT或MRI检查，了解有无颅内占位。 | [
{
"id": 0,
"entity": "颅内",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 1,
"entity": "癫痫",
"start_offset": 30,
"end_offset": 32,
"label": "dis"
},
{
"id": 2,
"entity": "癫痫",
"start_offset": 40,
"end_offset": 42,
"label": "dis"
},
{
"id": 3,
"entity": "癫痫持续",
"start_offset": 40,
"end_offset": 44,
"label": "sym"
},
{
"id": 4,
"entity": "癫痫",
"start_offset": 47,
"end_offset": 49,
"label": "dis"
},
{
"id": 5,
"entity": "难治性癫痫",
"start_offset": 44,
"end_offset": 49,
"label": "sym"
},
{
"id": 6,
"entity": "神经系统",
"start_offset": 53,
"end_offset": 57,
"label": "bod"
},
{
"id": 7,
"entity": "癫痫",
"start_offset": 60,
"end_offset": 62,
"label": "dis"
},
{
"id": 8,
"entity": "伴有局部神经系统损害的癫痫",
"start_offset": 49,
"end_offset": 62,
"label": "sym"
},
{
"id": 9,
"entity": "癫痫",
"start_offset": 62,
"end_offset": 64,
"label": "dis"
},
{
"id": 10,
"entity": "癫痫",
"start_offset": 78,
"end_offset": 80,
"label": "dis"
},
{
"id": 11,
"entity": "CT",
"start_offset": 82,
"end_offset": 84,
"label": "pro"
},
{
"id": 12,
"entity": "MRI检查",
"start_offset": 85,
"end_offset": 90,
"label": "pro"
},
{
"id": 13,
"entity": "颅内",
"start_offset": 95,
"end_offset": 97,
"label": "bod"
}
] |
3.局部症状因肿瘤所在部位和大小而异。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 7,
"end_offset": 9,
"label": "dis"
}
] |
若大脑半球肿瘤接近中央前回者可有对侧偏瘫；在左侧额下回后部者可有运动性失语；蝶鞍区肿瘤可有视神经原发性萎缩及视力视野改变；肿瘤影响垂体-视丘下部可有生长发育紊乱、肥胖或消瘦、多饮、多尿及体温调节障碍；幕下肿瘤多有走路不稳、眼球震颤肌张力及肌腱反射减退脑干部位肿瘤则有一侧脑神经损害及对侧锥体束征（交叉性麻痹）松果体区肿瘤可有眼球上视障碍及性早熟等。 | [
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"id": 0,
"entity": "大脑半球",
"start_offset": 1,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "肿瘤",
"start_offset": 5,
"end_offset": 7,
"label": "dis"
},
{
"id": 2,
"entity": "有对侧偏瘫",
"start_offset": 15,
"end_offset": 20,
"label": "sym"
},
{
"id": 3,
"entity": "额下回后部",
"start_offset": 24,
"end_offset": 29,
"label": "bod"
},
{
"id": 4,
"entity": "有运动性失语",
"start_offset": 31,
"end_offset": 37,
"label": "sym"
},
{
"id": 5,
"entity": "蝶鞍",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 6,
"entity": "肿瘤",
"start_offset": 41,
"end_offset": 43,
"label": "dis"
},
{
"id": 7,
"entity": "视神经",
"start_offset": 45,
"end_offset": 48,
"label": "bod"
},
{
"id": 8,
"entity": "有视神经原发性萎缩及视力",
"start_offset": 44,
"end_offset": 56,
"label": "sym"
},
{
"id": 9,
"entity": "视野改变",
"start_offset": 56,
"end_offset": 60,
"label": "sym"
},
{
"id": 10,
"entity": "肿瘤",
"start_offset": 61,
"end_offset": 63,
"label": "dis"
},
{
"id": 11,
"entity": "垂体-视丘下部",
"start_offset": 65,
"end_offset": 72,
"label": "bod"
},
{
"id": 12,
"entity": "有生长发育紊乱",
"start_offset": 73,
"end_offset": 80,
"label": "sym"
},
{
"id": 13,
"entity": "肥胖或消瘦",
"start_offset": 81,
"end_offset": 86,
"label": "sym"
},
{
"id": 14,
"entity": "多饮",
"start_offset": 87,
"end_offset": 89,
"label": "sym"
},
{
"id": 15,
"entity": "多尿及体温调节障碍",
"start_offset": 90,
"end_offset": 99,
"label": "sym"
},
{
"id": 16,
"entity": "幕下",
"start_offset": 100,
"end_offset": 102,
"label": "bod"
},
{
"id": 17,
"entity": "肿瘤",
"start_offset": 102,
"end_offset": 104,
"label": "dis"
},
{
"id": 18,
"entity": "有走路不稳",
"start_offset": 105,
"end_offset": 110,
"label": "sym"
},
{
"id": 19,
"entity": "眼球",
"start_offset": 111,
"end_offset": 113,
"label": "bod"
},
{
"id": 20,
"entity": "眼球震颤",
"start_offset": 111,
"end_offset": 115,
"label": "sym"
},
{
"id": 21,
"entity": "肌腱",
"start_offset": 119,
"end_offset": 121,
"label": "bod"
},
{
"id": 22,
"entity": "肌张力及肌腱反射减退",
"start_offset": 115,
"end_offset": 125,
"label": "sym"
},
{
"id": 23,
"entity": "脑干",
"start_offset": 125,
"end_offset": 127,
"label": "bod"
},
{
"id": 24,
"entity": "肿瘤",
"start_offset": 129,
"end_offset": 131,
"label": "dis"
},
{
"id": 25,
"entity": "脑神经",
"start_offset": 135,
"end_offset": 138,
"label": "bod"
},
{
"id": 26,
"entity": "一侧脑神经损害及对侧锥体束征（交叉性麻痹）",
"start_offset": 133,
"end_offset": 154,
"label": "sym"
},
{
"id": 27,
"entity": "松果体区",
"start_offset": 154,
"end_offset": 158,
"label": "bod"
},
{
"id": 28,
"entity": "肿瘤",
"start_offset": 158,
"end_offset": 160,
"label": "dis"
},
{
"id": 29,
"entity": "有眼球上视障碍及性早熟等",
"start_offset": 161,
"end_offset": 173,
"label": "sym"
}
] |
第十一篇儿童常见肿瘤0～15岁儿童恶性肿瘤发病率只占全部肿瘤病人的一小部分，在工业发达地区约占2%。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 8,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "儿童恶性肿瘤",
"start_offset": 15,
"end_offset": 21,
"label": "dis"
}
] |
但在儿童死亡原因中，恶性肿瘤却高达10%，是主要原因之一。 | [
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第一章儿童恶性肿瘤的流行病学一、常用的流行病学术语比、百分比与率是最常用的流行病学术语。 | [
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] |
比（ratio）说明两个数字之间的关系，如儿童急性白血病（acuteleukemia）男女发病例数比为869∶695，或1.29∶1。 | [
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{
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"entity": "acuteleukemia",
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率（rate）表示在一定期限内危险人群中的事件发生数，即发病率（incidence），也是一种成分比，肿瘤发病率常以百万计算，如某地区在3960740个0～14岁人口中1年内共有514例新发儿童肿瘤，即该地区0～14岁儿童年发病率为514/3960740约129.77/百万。 | [
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第十一章婴儿猝死综合征【概述】婴儿猝死综合征（suddeninfantdeathsyndrome，SIDS）是指婴儿时期突然死亡，死亡前后均不能从病史、症状体征甚至死亡后尸检等各种检查中得到相关疾病的诊断。 | [
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"id": 3,
"entity": "SIDS",
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据近年来的大量研究认为，SIDS并非由单一因素所致，而是由小儿发育、周围环境及多种病理生理因素造成。 | [
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目前研究仍未能揭示整个发病过程，仅在一些解剖和生理方面发现这些患儿存在一些问题，主要集中在肺部、脑干及一些神经功能方面（图6-6）。 | [
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{
"id": 2,
"entity": "脑干",
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图6-6SIDS发病机制尽管常规病理解剖不能发现明显的致死原因，但从死者解剖研究仍可发现SIDS患者存在一些病理征象，如轻度肺水肿、肺淤血和皮肤瘀点、瘀斑。 | [
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{
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{
"id": 9,
"entity": "瘀斑",
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"label": "sym"
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] |
研究证实有2/3死者存在慢性窒息，这些患者脑脊液中内皮细胞生长因子低于正常婴儿。 | [
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] |
SIDS患者被发现存在脑干神经结构和神经递质异常。 | [
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异常包括局灶性星形胶质细胞增生、树突状棘突及髓鞘发育不良、髓内星形细胞反应性增多。 | [
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{
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"entity": "异常包括局灶性星形胶质细胞增生、树突状棘突及髓鞘发育不良、髓内星形细胞反应性增多",
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位于延髓心血管呼吸中枢，与唤醒、自主神经及化学感受神经有关的反射弧发育不良，该部位的神经受体亦存在功能低下和受体量减少，如钾通道受体、毒蕈碱胆碱能受体等，二氧化碳、血压等其他神经感受器亦受累及。 | [
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{
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{
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"entity": "位于延髓心血管呼吸中枢，与唤醒、自主神经及化学感受神经有关的反射弧发育不良，该部位的神经受体亦存在功能低下和受体量减少，如钾通道受体、毒蕈碱胆碱能受体等，二氧化碳、血压等其他神经感受器亦受累及",
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此外，脑干区域、迷走神经核及脑干网状结构的酪氨酸羟化酶改变亦提示肾上腺素和去甲肾上腺素神经功能异常。 | [
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{
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"entity": "去甲肾上腺素",
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] |
近期临床研究主要集中于SIDS的危险因素。 | [
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基础研究方面，除解剖生理外，基因研究主要就心脏离子通道异常基因（钠通道、钾通道）、五羟色胺转运基因（5-HTT）以及自主神经系统发育和炎症反应的某些基因调控。 | [
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表6-14SIDS的高危因素【临床表现】本病发病年龄高峰为生后第2～4个月，早产儿为1～2个月，以后发病率减少。 | [
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前驱可有呼吸道感染和发热病史。 | [
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本病一般于午夜至清晨时段发病，患儿起病前常无明显、烦躁不适症状，在睡眠中呼吸、心跳突然停止。 | [
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] |
过度出汗提示存在发热、过度约束或存在自主神经功能缺陷。 | [
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很难在制定SIDS，其内容和项目除应尽可能精确鉴别和找出那些最终会死于SIDS的患儿，也应考虑到表格的有效性和实用性，必须忽略假阴性和允许存在一定程度的假阳性率。 | [
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{
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"entity": "终会死于",
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此外，呼吸描记图（pneumogram）和多导睡眠图（polysomnogram）可用于持续观察呼吸形态和心跳异常情况，但目前研究未证实其有足够的敏感度和特异性，临床上尚不能作为SIDS的筛查方法。 | [
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{
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亦未证实有早产儿呼吸暂停史婴儿的SIDS危险度高于无呼吸暂停的适于胎龄儿。 | [
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关于SIDS的鉴别诊断见图6-7。 | [
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图6-7SIDS的鉴别诊断【治疗】关于SIDS的防治，至今尚无可行的有效干预方法和用于初生婴儿生后SIDS的发病的预测方法。 | [
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{
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心电节律、呼吸类型以及自主神经系统的异常变化至今未能找出可用于临床观察的敏感监测指标，因而无法对SIDS病情作出即时和准确的判断，也无法制定相应的针对性干预措施。 | [
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虽然部分患者在电子监护中发现存在QT延长性心律失常，但对婴儿患者尚未建立统一的安全治疗规范。 | [
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咖啡因和茶碱已用于治疗早产儿呼吸暂停。 | [
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在成年人中，咖啡因可降低听觉唤醒阈值，但在婴儿尚无相应研究报道。 | [
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亦无该药会增加SIDS危险度的有关报道。 | [
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【预防】由于SIDS在临床观察上存在困难，目前主要针对有高危因素的婴儿进行临床保护性干预。 | [
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] |
部分欧美国家报道，通过这些措施后，近10年SIDS发病率下降了0.5个百分点。 | [
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表6-15SIDS预防措施（美国儿科学会）（陆铸今） | [
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二、通气功能所谓通气是指肺泡气体与外环境进行气体交换的过程（图8-8）。 | [
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图8-8最大用力流速-容量环VOKYNE：容积，FLOW：流速，TLC：肺总量，RV：残气量，FIF<sub>50</sub>：50%肺活量时的吸气流速，PEF：呼气峰流速，MEF<sub>75</sub>（FEF<sub>25</sub>）：75%肺活量时呼气流速；MEF<sub>50</sub>（FEF<sub>50</sub>）：50%肺活量时的呼流速；MEF<sub>25</sub>（FEF<sub>75</sub>）：25%肺活量时的呼气速（一）每分通气量（MV）是指每分钟呼出或吸入的气量，即潮气与呼吸频率的乘积。 | [
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},
{
"id": 17,
"entity": "肺活量",
"start_offset": 172,
"end_offset": 175,
"label": "ite"
},
{
"id": 18,
"entity": "MEF<sub>25</sub>",
"start_offset": 181,
"end_offset": 197,
"label": "ite"
},
{
"id": 19,
"entity": "FEF<sub>75</sub>",
"start_offset": 198,
"end_offset": 214,
"label": "ite"
},
{
"id": 20,
"entity": "肺活量",
"start_offset": 219,
"end_offset": 222,
"label": "ite"
},
{
"id": 21,
"entity": "每分通气量",
"start_offset": 230,
"end_offset": 235,
"label": "ite"
},
{
"id": 22,
"entity": "MV",
"start_offset": 236,
"end_offset": 238,
"label": "ite"
},
{
"id": 23,
"entity": "潮气",
"start_offset": 254,
"end_offset": 256,
"label": "ite"
},
{
"id": 24,
"entity": "呼吸频率",
"start_offset": 257,
"end_offset": 261,
"label": "ite"
}
] |
足月儿每分通气量140～220ml/kg，相当于3500～4000ml/m<sup>2</sup>，与成人相似。 | [
{
"id": 0,
"entity": "每分通气量",
"start_offset": 3,
"end_offset": 8,
"label": "ite"
}
] |
（二）肺泡通气量（V<sub>A</sub>）在静息状态下每分钟吸入气量中能到达肺泡进行有效气体交换的通气量为：（潮气量-无效腔气量）×呼吸频率，足月儿是100～150ml/kg。 | [
{
"id": 0,
"entity": "肺泡通气量",
"start_offset": 3,
"end_offset": 8,
"label": "ite"
},
{
"id": 1,
"entity": "V<sub>A</sub>",
"start_offset": 9,
"end_offset": 22,
"label": "ite"
},
{
"id": 2,
"entity": "每分钟吸入气量",
"start_offset": 29,
"end_offset": 36,
"label": "ite"
},
{
"id": 3,
"entity": "肺泡",
"start_offset": 40,
"end_offset": 42,
"label": "bod"
},
{
"id": 4,
"entity": "潮气量",
"start_offset": 57,
"end_offset": 60,
"label": "ite"
},
{
"id": 5,
"entity": "无效腔气量",
"start_offset": 61,
"end_offset": 66,
"label": "ite"
},
{
"id": 6,
"entity": "呼吸频率",
"start_offset": 68,
"end_offset": 72,
"label": "ite"
}
] |
（三）最大通气量（MVV）是指在单位时间内以最深最快的呼吸所能达到的最大通气量，通常以每分钟计算。 | [
{
"id": 0,
"entity": "最大通气量",
"start_offset": 3,
"end_offset": 8,
"label": "ite"
},
{
"id": 1,
"entity": "MVV",
"start_offset": 9,
"end_offset": 12,
"label": "ite"
},
{
"id": 2,
"entity": "最大通气量",
"start_offset": 34,
"end_offset": 39,
"label": "ite"
}
] |
（四）时间肺活量（又称用力肺活量，FVC）是指深吸气至肺总量，然后用力快速呼气直至残气位时测得的肺活量。 | [
{
"id": 0,
"entity": "时间肺活量",
"start_offset": 3,
"end_offset": 8,
"label": "ite"
},
{
"id": 1,
"entity": "用力肺活量",
"start_offset": 11,
"end_offset": 16,
"label": "ite"
},
{
"id": 2,
"entity": "FVC",
"start_offset": 17,
"end_offset": 20,
"label": "ite"
},
{
"id": 3,
"entity": "肺总量",
"start_offset": 27,
"end_offset": 30,
"label": "ite"
},
{
"id": 4,
"entity": "肺活量",
"start_offset": 48,
"end_offset": 51,
"label": "ite"
}
] |
婴幼儿无法配合，故有人研究用啼哭肺活量（CVC）作为婴幼儿FVC指标。 | [
{
"id": 0,
"entity": "啼哭肺活量",
"start_offset": 14,
"end_offset": 19,
"label": "ite"
},
{
"id": 1,
"entity": "CVC",
"start_offset": 20,
"end_offset": 23,
"label": "ite"
},
{
"id": 2,
"entity": "FVC",
"start_offset": 29,
"end_offset": 32,
"label": "ite"
}
] |
测定第1秒时间内呼出的气量称为第一秒用力呼气量（FEV<sub>1</sub>）或简称一秒量，用FEV<sub>1</sub>除以最大肺活量即为一秒率，一秒量和秒率是常规肺功能检测中反映气道阻塞最重要的两个指标。 | [
{
"id": 0,
"entity": "第一秒用力呼气量",
"start_offset": 15,
"end_offset": 23,
"label": "ite"
},
{
"id": 1,
"entity": "FEV<sub>1</sub>",
"start_offset": 24,
"end_offset": 39,
"label": "ite"
},
{
"id": 2,
"entity": "一秒量",
"start_offset": 43,
"end_offset": 46,
"label": "ite"
},
{
"id": 3,
"entity": "FEV<sub>1</sub>",
"start_offset": 48,
"end_offset": 63,
"label": "ite"
},
{
"id": 4,
"entity": "最大肺活量",
"start_offset": 65,
"end_offset": 70,
"label": "ite"
},
{
"id": 5,
"entity": "一秒率",
"start_offset": 72,
"end_offset": 75,
"label": "ite"
},
{
"id": 6,
"entity": "一秒量",
"start_offset": 76,
"end_offset": 79,
"label": "ite"
},
{
"id": 7,
"entity": "秒率",
"start_offset": 80,
"end_offset": 82,
"label": "ite"
},
{
"id": 8,
"entity": "常规肺功能检测",
"start_offset": 83,
"end_offset": 90,
"label": "pro"
},
{
"id": 9,
"entity": "气道",
"start_offset": 93,
"end_offset": 95,
"label": "bod"
},
{
"id": 10,
"entity": "气道阻塞",
"start_offset": 93,
"end_offset": 97,
"label": "sym"
}
] |
（五）呼气峰流速（PEF）即呼气相最高流速。 | [
{
"id": 0,
"entity": "呼气峰流速",
"start_offset": 3,
"end_offset": 8,
"label": "ite"
},
{
"id": 1,
"entity": "PEF",
"start_offset": 9,
"end_offset": 12,
"label": "ite"
},
{
"id": 2,
"entity": "呼气相最高流速",
"start_offset": 14,
"end_offset": 21,
"label": "ite"
}
] |
在呼气中，流速与肺弹性回缩力以及气道阻力有关。 | [
{
"id": 0,
"entity": "流速",
"start_offset": 5,
"end_offset": 7,
"label": "ite"
},
{
"id": 1,
"entity": "肺",
"start_offset": 8,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "气道",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
}
] |
最有意义的是呼气峰流速，其与年龄、身高、体重、胸围等均有关系，尤与身高关系密切。 | [
{
"id": 0,
"entity": "呼气峰流速",
"start_offset": 6,
"end_offset": 11,
"label": "ite"
},
{
"id": 1,
"entity": "年龄",
"start_offset": 14,
"end_offset": 16,
"label": "ite"
},
{
"id": 2,
"entity": "身高",
"start_offset": 17,
"end_offset": 19,
"label": "ite"
},
{
"id": 3,
"entity": "体重",
"start_offset": 20,
"end_offset": 22,
"label": "ite"
},
{
"id": 4,
"entity": "胸围",
"start_offset": 23,
"end_offset": 25,
"label": "ite"
},
{
"id": 5,
"entity": "身高",
"start_offset": 33,
"end_offset": 35,
"label": "ite"
}
] |
在阻塞性疾病（如婴幼儿哮喘），患者由于气道痉挛、痰液阻塞、小气道提早关闭，故PEF下降。 | [
{
"id": 0,
"entity": "阻塞性疾病",
"start_offset": 1,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "婴幼儿哮喘",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "气道",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "气道痉挛",
"start_offset": 19,
"end_offset": 23,
"label": "sym"
},
{
"id": 4,
"entity": "痰液",
"start_offset": 24,
"end_offset": 26,
"label": "bod"
},
{
"id": 5,
"entity": "痰液阻塞",
"start_offset": 24,
"end_offset": 28,
"label": "sym"
},
{
"id": 6,
"entity": "小气道",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 7,
"entity": "小气道提早关闭",
"start_offset": 29,
"end_offset": 36,
"label": "sym"
},
{
"id": 8,
"entity": "PEF",
"start_offset": 38,
"end_offset": 41,
"label": "ite"
}
] |
PEF还存在昼夜波动，据此结合临床症状，可对哮喘进行分级：PEF昼夜波动率=（日内最高PEF-日内最低PEF）/1/2（同日内最高PEF+最低PEF）×100%哮喘患儿发作时此值往往大于15%。 | [
{
"id": 0,
"entity": "PEF",
"start_offset": 0,
"end_offset": 3,
"label": "ite"
},
{
"id": 1,
"entity": "PEF还存在昼夜波动",
"start_offset": 0,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "哮喘",
"start_offset": 22,
"end_offset": 24,
"label": "dis"
},
{
"id": 3,
"entity": "PEF昼夜波动率",
"start_offset": 29,
"end_offset": 37,
"label": "ite"
},
{
"id": 4,
"entity": "PEF",
"start_offset": 43,
"end_offset": 46,
"label": "ite"
},
{
"id": 5,
"entity": "PEF",
"start_offset": 51,
"end_offset": 54,
"label": "ite"
},
{
"id": 6,
"entity": "PEF",
"start_offset": 65,
"end_offset": 68,
"label": "ite"
},
{
"id": 7,
"entity": "PEF",
"start_offset": 71,
"end_offset": 74,
"label": "ite"
},
{
"id": 8,
"entity": "哮喘",
"start_offset": 80,
"end_offset": 82,
"label": "dis"
}
] |
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