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动态脑电图是脑电图的一个重要分支，与常规脑电图相比，动态脑电图有以下特点：①导联组合不能随意变换，一般动态脑电图以时间/事件为记录目的，采用24小时导联不变的记录方式；②记录脑电图信息不等，常规脑电图仅记录20～30分钟的包括额、中央、顶、枕及颞五个部位的脑电活动，动态脑电图可记录24小时脑电活动，信息量相当于普通脑电图的46～71倍；③记录状态不同常规脑电图通常在静息状态描记，而动态脑电图可记录静息、活动、立、卧、坐等不同体位不同状态下的随意脑电图。 | [
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动态脑电图在儿科的适应证：①监测正常人群中异常脑电活动情况，及早发现潜在病灶；②用于癫痫诊断和脑电图鉴别及分型；③用于昏迷病人及危重病人的实时脑电监测；④各种器质性病变严重程度及危险性估计；⑤麻醉的保护；⑥晕厥等不明原因的发作性疾患的病因诊断。 | [
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动态脑电图只能记录脑电图的表现，而病人的临床症状医生很难观察到，视频脑电在临床实时记录病人发作期或发作间期的脑电活动及体态活动，为医生提供最为直接、准确的诊断依据，是一非常重要的神经诊断技术。 | [
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视频脑电图在儿科的适应证：①可疑癫痫病人的确诊。 | [
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对多数病人，常规脑电图已能提供足够的脑电活动信息，但约20%的病人在临床上没有明显的发作，通过实时脑电图记录，对癫痫类型的判定和癫痫分类，尤其对全身性癫痫棘波灶的起源和定位提供了前所未有的有利依据。 | [
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②癫痫的鉴别诊断。 | [
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一些非癫痫发作疾病（如屏气发作、晕厥、睡眠障碍及心因性疾患等）被错误诊断为癫痫的发作性疾患，依靠视频脑电图在临床发作期的敏感性和特异性，可得到鉴别。 | [
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③难治性癫痫术前定位。 | [
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可有助于局灶性难治性癫痫的致痫灶的定位，通过临床表现、影像学、功能性检查尤其是视频脑电图发现的病灶进行联合定位，对手术切除癫痫灶提供可靠信息。 | [
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惊跳及呼吸不规则等新生儿和婴儿期特有的行为有时被误认为癫痫样发作，可经实时脑电监控得到识别。 | [
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⑤儿童癫痫综合征的分类。 | [
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大田原综合征及West综合征等经常规脑电图如未能发现特异性脑电变化的，可经视频脑电描记，结合同时记录的临床发作而得以及早诊断。 | [
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第六节真菌性肺炎引起真菌性肺炎（fungalpneumonia）的病原有白色念珠菌、隐球菌、曲菌、毛霉菌、放线菌、组织胞质菌、芽生霉菌等，其中以白色念珠菌最常见，致病力最强。 | [
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由于该菌广泛存在于自然界，可寄生在正常人的皮肤、口腔、肠道、阴道等处黏膜上，在正常情况下不致病，当人体抵抗力低下时可致病。 | [
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叶启慈报道经病理解剖证实的真菌性肺炎35例，以念珠菌最常见（48.5%），曲菌次之，其中新生儿14例（占40%），继发于其他疾病19例，胸腺发育异常25例（71%）。 | [
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胸部X线检查有点片状阴影，可似粟粒性结核，以两肺中下部多见，或肺门阴影增浓、肺纹理增多，可有大片实变病灶，少数有胸腔积液及心包积液等。 | [
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有基础疾病的患儿，肺炎病程迁延不愈，抗生素治疗无效甚至恶化，应考虑本病可能。 | [
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从痰等下呼吸道标本中找到真菌孢子及假菌丝，或培养阳性可诊断。 | [
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两性霉素B脂质体可提高疗效，减少不良反应。 | [
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广谱抗真菌药氟康唑对念珠菌、隐球菌抗菌活性最强，但对曲菌的作用差，可用于治疗念珠菌、隐球菌感染。 | [
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可酌情加用转移因子等免疫调节剂。 | [
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二、恶性纤维组织细胞瘤恶性纤维组织细胞瘤（malignantfibroushistocytoma，MFH），也称纤维组织细胞肉瘤、纤维黄色肉瘤、恶性纤维黄色瘤等。 | [
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肿瘤多呈结节状大小不一，从2cm到20cm，多无包膜切面为灰白色，鱼肉状，可有出血坏死，有时含有囊腔。 | [
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显微镜下肿瘤主要由组织细胞样细胞和成纤维细胞样细胞构成，呈多形性，典型和不典型核分裂象多见于组织细胞样细胞。 | [
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肿瘤多发于四肢，其次是躯干，腹腔内、腹膜后、骨骼、乳房也均有可能。 | [
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"label": "bod"
},
{
"id": 2,
"entity": "躯干",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 3,
"entity": "腹腔内",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 4,
"entity": "腹膜后",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 5,
"entity": "骨骼",
"start_offset": 22,
"end_offset": 24,
"label": "bod"
},
{
"id": 6,
"entity": "乳房",
"start_offset": 25,
"end_offset": 27,
"label": "bod"
}
] |
肿块呈单个结节状，开始时可很少，但常易侵犯局部深筋膜，有时呈溃疡状。 | [
{
"id": 0,
"entity": "肿块",
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"label": "bod"
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{
"id": 1,
"entity": "肿块呈单个结节状，开始时可很少",
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"label": "sym"
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{
"id": 2,
"entity": "深筋膜",
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},
{
"id": 3,
"entity": "有时呈溃疡状",
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"label": "sym"
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] |
肿瘤早期即可向区域淋巴结和远处转移，深部肿瘤可发生远处转移灶的症状先于原发灶的情况。 | [
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"id": 0,
"entity": "肿瘤",
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{
"id": 1,
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{
"id": 2,
"entity": "肿瘤",
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"end_offset": 22,
"label": "dis"
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] |
第一节川崎病川崎病（Kawasakidisease，KD）又称皮肤黏膜淋巴结综合征（mucocutaneouslymphnodesyndrome，MCLS），是一种病因未明的全身性血管炎综合征。 | [
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"id": 0,
"entity": "川崎病",
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"label": "bod"
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{
"id": 1,
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{
"id": 2,
"entity": "Kawasakidisease",
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{
"id": 3,
"entity": "KD",
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{
"id": 4,
"entity": "皮肤黏膜淋巴结综合征",
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{
"id": 5,
"entity": "mucocutaneouslymphnodesyndrome",
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{
"id": 6,
"entity": "MCLS",
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{
"id": 7,
"entity": "全身性血管炎综合征",
"start_offset": 87,
"end_offset": 96,
"label": "bod"
}
] |
表现为发热，皮疹，球结合膜、口腔黏膜充血，手足红斑、硬性水肿及颈淋巴结肿大。 | [
{
"id": 0,
"entity": "发热",
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"end_offset": 5,
"label": "sym"
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{
"id": 1,
"entity": "皮疹",
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"end_offset": 8,
"label": "sym"
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{
"id": 2,
"entity": "球结合膜、口腔黏膜充血",
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{
"id": 3,
"entity": "手足红斑",
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{
"id": 4,
"entity": "硬性水肿",
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"label": "sym"
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{
"id": 5,
"entity": "颈淋巴结肿大",
"start_offset": 31,
"end_offset": 37,
"label": "sym"
}
] |
主要病理改变为全身性中、小动脉炎，最严重的危害是病程中、后期发生的中大动脉损伤，尤其是冠状动脉损害，是儿童最重要的后天性心脏病之一。 | [
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"entity": "全身性中、小动脉炎",
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{
"id": 1,
"entity": "中大动脉损伤",
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{
"id": 2,
"entity": "冠状动脉损害",
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{
"id": 3,
"entity": "后天性心脏病",
"start_offset": 57,
"end_offset": 63,
"label": "dis"
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] |
据统计，发热10天内未经及时治疗者，冠状动脉病变发生率达20%～25%。 | [
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"entity": "发热",
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{
"id": 1,
"entity": "冠状动脉",
"start_offset": 18,
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"label": "bod"
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] |
即使经阿司匹林治疗也有约15%患儿发生冠脉病变，因而其危害性应予高度关注。 | [
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"id": 0,
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{
"id": 1,
"entity": "冠脉",
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"end_offset": 21,
"label": "bod"
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] |
【病因与发病机制】病因不明，感染是本病重要的致病因素之一，在川崎患儿体内常可发现链球菌、葡萄球菌、反转录病毒以及支原体等病原感染的证据。 | [
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"id": 0,
"entity": "链球菌",
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"end_offset": 43,
"label": "mic"
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{
"id": 1,
"entity": "葡萄球菌",
"start_offset": 44,
"end_offset": 48,
"label": "mic"
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{
"id": 2,
"entity": "反转录病毒",
"start_offset": 49,
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"label": "mic"
},
{
"id": 3,
"entity": "支原体",
"start_offset": 56,
"end_offset": 59,
"label": "mic"
}
] |
有关发病机制有下列学说：（一）免疫过度活化研究认为免疫反应亢进是致病的重要环节，突出表现在急性期CD30+</sup>T细胞增多，部分细胞因子分泌异常，B细胞多克隆活化，循环中有抗内皮细胞毒性抗体及抗中性粒胞浆抗体等体液免疫反应亢进现象；异常增高的IL-6能抑制淋巴细胞p53基因表达，淋巴细胞凋亡时间明显延迟，最终导致免疫细胞过度活化。 | [
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"id": 0,
"entity": "T细胞",
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"end_offset": 62,
"label": "bod"
},
{
"id": 1,
"entity": "细胞因子",
"start_offset": 67,
"end_offset": 71,
"label": "bod"
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{
"id": 2,
"entity": "B细胞",
"start_offset": 76,
"end_offset": 79,
"label": "bod"
},
{
"id": 3,
"entity": "抗内皮细胞毒性抗体",
"start_offset": 89,
"end_offset": 98,
"label": "bod"
},
{
"id": 4,
"entity": "抗中性粒胞浆抗体",
"start_offset": 99,
"end_offset": 107,
"label": "bod"
},
{
"id": 5,
"entity": "IL-6",
"start_offset": 124,
"end_offset": 128,
"label": "bod"
},
{
"id": 6,
"entity": "淋巴细胞p53基因",
"start_offset": 131,
"end_offset": 140,
"label": "bod"
},
{
"id": 7,
"entity": "淋巴细胞",
"start_offset": 143,
"end_offset": 147,
"label": "bod"
},
{
"id": 8,
"entity": "免疫细胞",
"start_offset": 160,
"end_offset": 164,
"label": "bod"
}
] |
（二）超抗原近年有人证实葡萄球菌内毒素和链球菌红斑毒素可作为一种超抗原启动暂时性异常免疫反应。 | [
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"id": 0,
"entity": "葡萄球菌",
"start_offset": 12,
"end_offset": 16,
"label": "mic"
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{
"id": 1,
"entity": "链球菌",
"start_offset": 20,
"end_offset": 23,
"label": "mic"
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{
"id": 2,
"entity": "红斑毒素",
"start_offset": 23,
"end_offset": 27,
"label": "mic"
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] |
超抗原可不经抗原递呈细胞（APC）处理即能直接激活T细胞或与APC表面MHCⅡ类抗原结合后刺激T细胞活化，释放大量淋巴因子如IL-4及IL-6，从而介导或放大自身免疫损伤。 | [
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"id": 0,
"entity": "T细胞",
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{
"id": 1,
"entity": "APC表面MHCⅡ类抗原",
"start_offset": 30,
"end_offset": 42,
"label": "bod"
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{
"id": 2,
"entity": "T细胞",
"start_offset": 47,
"end_offset": 50,
"label": "bod"
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{
"id": 3,
"entity": "淋巴因子",
"start_offset": 57,
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"label": "bod"
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{
"id": 4,
"entity": "IL-4",
"start_offset": 62,
"end_offset": 66,
"label": "bod"
},
{
"id": 5,
"entity": "IL-6",
"start_offset": 67,
"end_offset": 71,
"label": "bod"
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] |
（三）热休克蛋白因细菌体热休克蛋白65</sub>（HSP65</sub>）成分与人类HSP60</sub>有高度同源性，川崎患儿细菌感染后，其HSP65</sub>诱导人体局限于血管组织的HSP60</sub>表达增强，通过抗原分子间的模拟机制刺激机体产生了针对自身血管的免疫损伤。 | [
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"id": 0,
"entity": "细菌",
"start_offset": 9,
"end_offset": 11,
"label": "mic"
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{
"id": 1,
"entity": "HSP65",
"start_offset": 26,
"end_offset": 31,
"label": "mic"
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{
"id": 2,
"entity": "HSP60",
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{
"id": 3,
"entity": "细菌",
"start_offset": 65,
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"label": "mic"
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{
"id": 4,
"entity": "HSP65",
"start_offset": 72,
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"label": "mic"
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{
"id": 5,
"entity": "血管组织",
"start_offset": 90,
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"label": "bod"
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{
"id": 6,
"entity": "HSP60",
"start_offset": 95,
"end_offset": 100,
"label": "mic"
},
{
"id": 7,
"entity": "血管",
"start_offset": 134,
"end_offset": 136,
"label": "bod"
}
] |
【病理】病初以小血管炎为主，以后累及主动脉等中、大动脉，特别好发于冠状动脉及其分支，未经及时治疗的病例其病理改变大致可分为4期：Ⅰ期：1～9天，主要是小血管炎、微血管周围炎以及中等大小动脉周围炎，如冠状动脉周围炎；在心肌间质、心包及心内膜有中性粒细胞、嗜酸性粒细胞和淋巴细胞浸润。 | [
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"id": 0,
"entity": "小血管炎",
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"end_offset": 11,
"label": "dis"
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{
"id": 1,
"entity": "主动脉",
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"label": "bod"
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{
"id": 2,
"entity": "中、大动脉",
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"label": "bod"
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{
"id": 3,
"entity": "冠状动脉",
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{
"id": 4,
"entity": "小血管炎",
"start_offset": 75,
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{
"id": 5,
"entity": "微血管周围炎",
"start_offset": 80,
"end_offset": 86,
"label": "dis"
},
{
"id": 6,
"entity": "中等大小动脉周围炎",
"start_offset": 88,
"end_offset": 97,
"label": "dis"
},
{
"id": 7,
"entity": "冠状动脉周围炎",
"start_offset": 99,
"end_offset": 106,
"label": "dis"
},
{
"id": 8,
"entity": "心肌间质",
"start_offset": 108,
"end_offset": 112,
"label": "bod"
},
{
"id": 9,
"entity": "心包",
"start_offset": 113,
"end_offset": 115,
"label": "bod"
},
{
"id": 10,
"entity": "心内膜",
"start_offset": 116,
"end_offset": 119,
"label": "bod"
},
{
"id": 11,
"entity": "中性粒细胞",
"start_offset": 120,
"end_offset": 125,
"label": "bod"
},
{
"id": 12,
"entity": "嗜酸性粒细胞",
"start_offset": 126,
"end_offset": 132,
"label": "bod"
},
{
"id": 13,
"entity": "淋巴细胞",
"start_offset": 133,
"end_offset": 137,
"label": "bod"
}
] |
Ⅱ期：12～25天，小血管炎减轻，冠状动脉主要分支等中等大小动脉全层血管炎（内膜、外膜及中膜均有炎性细胞浸润）突出，伴有坏死、水肿，血管弹力纤维和肌层断裂，出现冠状动脉扩张，易发生冠状动脉瘤及血栓。 | [
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"id": 0,
"entity": "小血管炎",
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"label": "dis"
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{
"id": 1,
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{
"id": 2,
"entity": "中等大小动脉",
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"label": "bod"
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{
"id": 3,
"entity": "血管炎",
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{
"id": 4,
"entity": "内膜",
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"label": "bod"
},
{
"id": 5,
"entity": "外膜",
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{
"id": 6,
"entity": "中膜",
"start_offset": 44,
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"label": "bod"
},
{
"id": 7,
"entity": "炎性细胞",
"start_offset": 48,
"end_offset": 52,
"label": "bod"
},
{
"id": 8,
"entity": "坏死、水肿",
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"end_offset": 65,
"label": "sym"
},
{
"id": 9,
"entity": "冠状动脉",
"start_offset": 80,
"end_offset": 84,
"label": "bod"
},
{
"id": 10,
"entity": "冠状动脉扩张",
"start_offset": 80,
"end_offset": 86,
"label": "sym"
},
{
"id": 11,
"entity": "冠状动脉瘤",
"start_offset": 90,
"end_offset": 95,
"label": "dis"
},
{
"id": 12,
"entity": "血栓",
"start_offset": 96,
"end_offset": 98,
"label": "dis"
}
] |
Ⅲ期：28～45天，小血管及微血管炎消退，中动脉发生肉芽肿及血栓，纤维组织增生，血管内膜增厚，冠状动脉一些分支可全部或部分阻塞，有冠状动脉瘤破裂急性血管炎消失，已经发生的血管内膜增厚，瘢痕、动脉瘤或血栓有一个漫长的吸收、修复过程。 | [
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"id": 0,
"entity": "小血管及微血管炎",
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"label": "dis"
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{
"id": 1,
"entity": "中动脉",
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{
"id": 2,
"entity": "肉芽肿",
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"label": "dis"
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{
"id": 3,
"entity": "血栓",
"start_offset": 30,
"end_offset": 32,
"label": "dis"
},
{
"id": 4,
"entity": "纤维组织",
"start_offset": 33,
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"label": "bod"
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{
"id": 5,
"entity": "血管内膜",
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{
"id": 6,
"entity": "冠状动脉",
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"label": "bod"
},
{
"id": 7,
"entity": "冠状动脉瘤",
"start_offset": 65,
"end_offset": 70,
"label": "dis"
},
{
"id": 8,
"entity": "纤维组织增生，血管内膜增厚，冠状动脉一些分支可全部或部分阻塞，有冠状动脉瘤破裂",
"start_offset": 33,
"end_offset": 72,
"label": "sym"
},
{
"id": 9,
"entity": "急性血管炎",
"start_offset": 72,
"end_offset": 77,
"label": "dis"
},
{
"id": 10,
"entity": "血管内膜增厚",
"start_offset": 85,
"end_offset": 91,
"label": "sym"
},
{
"id": 11,
"entity": "瘢痕",
"start_offset": 92,
"end_offset": 94,
"label": "sym"
},
{
"id": 12,
"entity": "动脉瘤",
"start_offset": 95,
"end_offset": 98,
"label": "dis"
},
{
"id": 13,
"entity": "血栓",
"start_offset": 99,
"end_offset": 101,
"label": "dis"
}
] |
狭窄、阻塞的血管可能修复、再通，心肌可能遗留永久的疤痕。 | [
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"id": 0,
"entity": "血管",
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{
"id": 1,
"entity": "心肌",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
}
] |
早期严重心肌炎、中后期动脉瘤破裂与血管栓塞是本病死亡的主要风险。 | [
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"id": 0,
"entity": "心肌炎",
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{
"id": 1,
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{
"id": 2,
"entity": "血管栓塞",
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] |
【临床表现】（一）主要症状1.发热若无早期治疗，一般持续7～12天，少数有更长时间（2周至月余），多在39℃以上，呈稽留热或弛张热，抗生素治疗无效。 | [
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"id": 0,
"entity": "发热",
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{
"id": 1,
"entity": "稽留热或弛张热",
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},
{
"id": 2,
"entity": "抗生素",
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"label": "dru"
}
] |
2.皮疹为多形性弥漫性红斑，有些近似麻疹样，一般无疱疹与结痂，躯干部多见，面部及四肢也可见上述皮疹。 | [
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"id": 0,
"entity": "皮疹",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "麻疹",
"start_offset": 18,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "躯干部",
"start_offset": 31,
"end_offset": 34,
"label": "bod"
},
{
"id": 3,
"entity": "面部",
"start_offset": 37,
"end_offset": 39,
"label": "bod"
},
{
"id": 4,
"entity": "四肢",
"start_offset": 40,
"end_offset": 42,
"label": "bod"
},
{
"id": 5,
"entity": "多形性弥漫性红斑，有些近似麻疹样，一般无疱疹与结痂，躯干部多见，面部及四肢也可见",
"start_offset": 5,
"end_offset": 45,
"label": "sym"
},
{
"id": 6,
"entity": "皮疹",
"start_offset": 47,
"end_offset": 49,
"label": "dis"
}
] |
发热后2～4天出疹，持续4～5天后消退。 | [
{
"id": 0,
"entity": "发热后2～4天出疹，持续4～5天后消退",
"start_offset": 0,
"end_offset": 19,
"label": "sym"
}
] |
3.双眼球结合膜充血无脓性分泌物，一般无糜烂。 | [
{
"id": 0,
"entity": "双眼球结合膜充血",
"start_offset": 2,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "无脓性分泌物，一般无糜烂",
"start_offset": 10,
"end_offset": 22,
"label": "sym"
}
] |
4.唇红、干燥、皲裂口咽黏膜充血，舌乳头隆起似杨梅充血症状持续于整个发热期。 | [
{
"id": 0,
"entity": "唇红、干燥、皲裂",
"start_offset": 2,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "舌乳头",
"start_offset": 17,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "口咽黏膜充血，舌乳头隆起似杨梅",
"start_offset": 10,
"end_offset": 25,
"label": "sym"
},
{
"id": 3,
"entity": "充血",
"start_offset": 25,
"end_offset": 27,
"label": "sym"
},
{
"id": 4,
"entity": "发热",
"start_offset": 34,
"end_offset": 36,
"label": "sym"
}
] |
5.手足硬肿手足掌现弥漫性红斑，趾、指末端硬肿突出，伴疼痛和僵直，9～14天开始出现特征性趾、指末端沿甲床膜状或薄片状脱屑，肛周也见类似脱屑。 | [
{
"id": 0,
"entity": "手足硬肿",
"start_offset": 2,
"end_offset": 6,
"label": "sym"
},
{
"id": 1,
"entity": "弥漫性红斑",
"start_offset": 10,
"end_offset": 15,
"label": "sym"
},
{
"id": 2,
"entity": "趾、指末端",
"start_offset": 16,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "趾、指末端硬肿突出，伴疼痛和僵直",
"start_offset": 16,
"end_offset": 32,
"label": "sym"
},
{
"id": 4,
"entity": "趾、指末端",
"start_offset": 45,
"end_offset": 50,
"label": "bod"
},
{
"id": 5,
"entity": "肛周",
"start_offset": 62,
"end_offset": 64,
"label": "bod"
},
{
"id": 6,
"entity": "特征性趾、指末端沿甲床膜状或薄片状脱屑，肛周也见类似脱屑",
"start_offset": 42,
"end_offset": 70,
"label": "sym"
}
] |
6.颈部非化脓性淋巴结炎一过性淋巴结肿大，直径约0.5～1.5cm，多为颈侧淋巴结，单侧多见，压痛轻，质较硬，不化脓。 | [
{
"id": 0,
"entity": "颈部非化脓性淋巴结炎",
"start_offset": 2,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "淋巴结",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "淋巴结肿大",
"start_offset": 15,
"end_offset": 20,
"label": "sym"
},
{
"id": 3,
"entity": "颈侧淋巴结",
"start_offset": 36,
"end_offset": 41,
"label": "bod"
},
{
"id": 4,
"entity": "单侧多见，压痛轻，质较硬，不化脓",
"start_offset": 42,
"end_offset": 58,
"label": "sym"
}
] |
（二）其他症状心脏损害并不少见，可因冠状动脉炎伴动脉瘤和血栓栓塞而引起猝死。 | [
{
"id": 0,
"entity": "心脏",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "冠状动脉炎",
"start_offset": 18,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "动脉瘤",
"start_offset": 24,
"end_offset": 27,
"label": "dis"
},
{
"id": 3,
"entity": "血栓栓塞",
"start_offset": 28,
"end_offset": 32,
"label": "dis"
},
{
"id": 4,
"entity": "猝死",
"start_offset": 35,
"end_offset": 37,
"label": "sym"
}
] |
有不同程度心肌炎、心包炎、心内膜炎和心律失常，偶可闻奔马律、心音低钝和心音分裂，可发生心肌梗死、心力衰竭、高血压及心源性休克等；少数患儿有惊厥、昏迷、中枢性、外周性神经麻痹以及精神、情绪异常等无菌性脑炎、脑膜炎症状；也有关节痛、关节炎，咳嗽、间质性肺炎的报告，上述症状多于病程1～6周出现。 | [
{
"id": 0,
"entity": "心肌炎",
"start_offset": 5,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "心包炎",
"start_offset": 9,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "心内膜炎",
"start_offset": 13,
"end_offset": 17,
"label": "dis"
},
{
"id": 3,
"entity": "心律失常",
"start_offset": 18,
"end_offset": 22,
"label": "sym"
},
{
"id": 4,
"entity": "偶可闻奔马律、心音低钝和心音分裂",
"start_offset": 23,
"end_offset": 39,
"label": "sym"
},
{
"id": 5,
"entity": "心肌梗死",
"start_offset": 43,
"end_offset": 47,
"label": "dis"
},
{
"id": 6,
"entity": "心力衰竭",
"start_offset": 48,
"end_offset": 52,
"label": "dis"
},
{
"id": 7,
"entity": "高血压",
"start_offset": 53,
"end_offset": 56,
"label": "dis"
},
{
"id": 8,
"entity": "心源性休克",
"start_offset": 57,
"end_offset": 62,
"label": "dis"
},
{
"id": 9,
"entity": "无菌性脑炎",
"start_offset": 96,
"end_offset": 101,
"label": "dis"
},
{
"id": 10,
"entity": "脑膜炎",
"start_offset": 102,
"end_offset": 105,
"label": "dis"
},
{
"id": 11,
"entity": "惊厥、昏迷、中枢性、外周性神经麻痹以及精神、情绪异常等无菌性脑炎、脑膜炎症状",
"start_offset": 69,
"end_offset": 107,
"label": "sym"
},
{
"id": 12,
"entity": "关节炎",
"start_offset": 114,
"end_offset": 117,
"label": "dis"
},
{
"id": 13,
"entity": "间质性肺炎",
"start_offset": 121,
"end_offset": 126,
"label": "dis"
},
{
"id": 14,
"entity": "关节痛、关节炎，咳嗽、间质性肺炎",
"start_offset": 110,
"end_offset": 126,
"label": "sym"
}
] |
极少数患儿可合并巨噬细胞活化（MAS）等严重并发症，甚至可威胁生命。 | [
{
"id": 0,
"entity": "巨噬细胞活化",
"start_offset": 8,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "MAS",
"start_offset": 15,
"end_offset": 18,
"label": "dis"
}
] |
【辅助检查】轻度贫血，外周血白细胞数增加，以中性粒细胞增加为主，有核左移现象血小板早期正常，第2～3周显著增高，血液呈高凝状态，血浆黏度增高，血浆纤维蛋白原增加。 | [
{
"id": 0,
"entity": "中性粒细胞",
"start_offset": 22,
"end_offset": 27,
"label": "bod"
},
{
"id": 1,
"entity": "轻度贫血，外周血白细胞数增加，以中性粒细胞增加为主，有核左移现象",
"start_offset": 6,
"end_offset": 38,
"label": "sym"
},
{
"id": 2,
"entity": "血小板",
"start_offset": 38,
"end_offset": 41,
"label": "bod"
}
] |
血清IgG、IgA、IgM、IgE和血循环免疫复合物升高，类风湿因子及抗核抗体均阴性。 | [
{
"id": 0,
"entity": "血清IgG、IgA、IgM、IgE和血循环免疫复合物升高",
"start_offset": 0,
"end_offset": 28,
"label": "sym"
},
{
"id": 1,
"entity": "类风湿因子",
"start_offset": 29,
"end_offset": 34,
"label": "bod"
},
{
"id": 2,
"entity": "抗核抗体",
"start_offset": 35,
"end_offset": 39,
"label": "bod"
}
] |
部分患儿ALT和AST升高，血清蛋白电泳可见球蛋白升高。 | [
{
"id": 0,
"entity": "ALT",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "AST",
"start_offset": 8,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "血清蛋白",
"start_offset": 14,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "电泳",
"start_offset": 18,
"end_offset": 20,
"label": "pro"
},
{
"id": 4,
"entity": "球蛋白升高",
"start_offset": 22,
"end_offset": 27,
"label": "sym"
}
] |
尿沉渣中白细胞数增多，轻度蛋白尿。 | [
{
"id": 0,
"entity": "白细胞",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "尿沉渣中白细胞数增多",
"start_offset": 0,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "轻度蛋白尿",
"start_offset": 11,
"end_offset": 16,
"label": "sym"
}
] |
病程第1周常见各类心电图异常，如心动过速，ST-T改变，各种房室传导阻滞，T波改变及心律紊乱。 | [
{
"id": 0,
"entity": "心电图",
"start_offset": 9,
"end_offset": 12,
"label": "pro"
},
{
"id": 1,
"entity": "心动过速",
"start_offset": 16,
"end_offset": 20,
"label": "sym"
},
{
"id": 2,
"entity": "ST-T改变",
"start_offset": 21,
"end_offset": 27,
"label": "sym"
},
{
"id": 3,
"entity": "各种房室传导阻滞",
"start_offset": 28,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "T波改变及心律紊乱",
"start_offset": 37,
"end_offset": 46,
"label": "sym"
}
] |
【诊断标准】多采用日本MCLS研究会或第三次国际川崎病研讨会提出的诊断标准。 | [
{
"id": 0,
"entity": "MCLS",
"start_offset": 11,
"end_offset": 15,
"label": "bod"
},
{
"id": 1,
"entity": "川崎病",
"start_offset": 24,
"end_offset": 27,
"label": "bod"
}
] |
1.不明原因发热5天以上；2.双侧球结膜弥漫性充血，无渗出物；3.口唇潮红，皲裂，口咽黏膜充血，杨梅舌；4.病初（1～9天）手足指趾肿胀，掌跖潮红，恢复期（9～21天）出现指趾端膜状脱屑或肛周脱屑;5.躯干及四肢多形充血性红斑;6.颈淋巴结非化脓性肿大，直径达1.5cm或更大。 | [
{
"id": 0,
"entity": "不明原因发热5天以上",
"start_offset": 2,
"end_offset": 12,
"label": "sym"
},
{
"id": 1,
"entity": "双侧球结膜弥漫性充血，无渗出物",
"start_offset": 15,
"end_offset": 30,
"label": "sym"
},
{
"id": 2,
"entity": "口咽黏膜",
"start_offset": 41,
"end_offset": 45,
"label": "bod"
},
{
"id": 3,
"entity": "口唇潮红，皲裂，口咽黏膜充血，杨梅舌",
"start_offset": 33,
"end_offset": 51,
"label": "sym"
},
{
"id": 4,
"entity": "手足指趾肿胀",
"start_offset": 62,
"end_offset": 68,
"label": "sym"
},
{
"id": 5,
"entity": "掌跖潮红",
"start_offset": 69,
"end_offset": 73,
"label": "sym"
},
{
"id": 6,
"entity": "肛周",
"start_offset": 94,
"end_offset": 96,
"label": "bod"
},
{
"id": 7,
"entity": "指趾端膜状脱屑或肛周脱屑",
"start_offset": 86,
"end_offset": 98,
"label": "sym"
},
{
"id": 8,
"entity": "四肢",
"start_offset": 104,
"end_offset": 106,
"label": "bod"
},
{
"id": 9,
"entity": "躯干及四肢多形充血性红斑",
"start_offset": 101,
"end_offset": 113,
"label": "sym"
},
{
"id": 10,
"entity": "颈淋巴结非化脓性肿大",
"start_offset": 116,
"end_offset": 126,
"label": "sym"
}
] |
【鉴别诊断】（一）猩红热皮疹发生早（1～2天），粟粒样均匀丘疹，疹间皮肤潮红，发病年龄普遍＞3岁，青霉素治疗有效。 | [
{
"id": 0,
"entity": "猩红热",
"start_offset": 9,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "皮疹",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "粟粒样均匀丘疹",
"start_offset": 24,
"end_offset": 31,
"label": "sym"
},
{
"id": 3,
"entity": "疹间皮肤潮红",
"start_offset": 32,
"end_offset": 38,
"label": "sym"
},
{
"id": 4,
"entity": "青霉素",
"start_offset": 49,
"end_offset": 52,
"label": "dru"
}
] |
（二）幼年特发性关节炎（JIA）可为高热，反复隐现多型皮疹（热退疹隐），热程反复、迁延，常为关节肿痛。 | [
{
"id": 0,
"entity": "幼年特发性关节炎",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "JIA",
"start_offset": 12,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "高热",
"start_offset": 18,
"end_offset": 20,
"label": "sym"
},
{
"id": 3,
"entity": "多型皮疹",
"start_offset": 25,
"end_offset": 29,
"label": "sym"
},
{
"id": 4,
"entity": "热退疹隐",
"start_offset": 30,
"end_offset": 34,
"label": "sym"
},
{
"id": 5,
"entity": "关节肿痛",
"start_offset": 46,
"end_offset": 50,
"label": "sym"
}
] |
（三）渗出性红斑常见口唇、眼角多处黏膜糜烂，常有脓性渗出，假膜形成。 | [
{
"id": 0,
"entity": "渗出性红斑",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "口唇、眼角多处黏膜糜烂，常有脓性渗出，假膜形成",
"start_offset": 10,
"end_offset": 33,
"label": "sym"
}
] |
皮疹广泛、大片，有水泡和结痂。 | [
{
"id": 0,
"entity": "皮疹广泛、大片，有水泡和结痂",
"start_offset": 0,
"end_offset": 14,
"label": "sym"
}
] |
（四）系统性红斑狼疮面部蝶形、盘状红斑、脱发、关节炎、白细胞减少、血小板减少及抗核抗体阳性阿司匹林发热时用量30～50mg/（kg•d），热退后2～3天可根据血小板数及血凝状态调整剂量，一般为5～10mg/（kg•d）再用6～8周。 | [
{
"id": 0,
"entity": "系统性红斑狼疮",
"start_offset": 3,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "关节炎",
"start_offset": 23,
"end_offset": 26,
"label": "dis"
},
{
"id": 2,
"entity": "白细胞",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "血小板",
"start_offset": 33,
"end_offset": 36,
"label": "bod"
},
{
"id": 4,
"entity": "面部蝶形、盘状红斑、脱发、关节炎、白细胞减少、血小板减少及抗核抗体阳性",
"start_offset": 10,
"end_offset": 45,
"label": "sym"
},
{
"id": 5,
"entity": "阿司匹林",
"start_offset": 45,
"end_offset": 49,
"label": "dru"
},
{
"id": 6,
"entity": "血小板",
"start_offset": 79,
"end_offset": 82,
"label": "bod"
}
] |
有冠状动脉病变者用药疗程延至冠状动脉病变恢复正常。 | [
{
"id": 0,
"entity": "冠状动脉病变",
"start_offset": 1,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "冠状动脉病变",
"start_offset": 14,
"end_offset": 20,
"label": "dis"
}
] |
（二）静脉注射用丙种球蛋白（IVIG）治疗本病疗效突出。 | [
{
"id": 0,
"entity": "静脉注射",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "丙种球蛋白",
"start_offset": 8,
"end_offset": 13,
"label": "dru"
},
{
"id": 2,
"entity": "IVIG",
"start_offset": 14,
"end_offset": 18,
"label": "dru"
}
] |
发热及充血症状可在24小时左右缓解。 | [
{
"id": 0,
"entity": "发热及充血",
"start_offset": 0,
"end_offset": 5,
"label": "sym"
}
] |
与单用阿司匹林治疗比较，冠状动脉病变发生率从18%降至4%左右，单次大剂量（2g/kg）比分次给药（每天400mg/kg，连用5天）有更佳疗效，急性期症状缓解更快，冠状动脉病变发生率更低。 | [
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{
"id": 2,
"entity": "冠状动脉病变",
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] |
但IVIG给药若在10天之后，冠状动脉病变预防效果将显著降低。 | [
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"entity": "IVIG",
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{
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"entity": "冠状动脉病变",
"start_offset": 15,
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] |
IVIG输注同时和输注后1～2个月仍需合用阿司匹林以取得最佳疗效，防止恢复期高凝状态。 | [
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] |
IVIG虽为目前最佳药物，但鉴于经济原因也有人主张适用于冠状动脉病变高危患儿。 | [
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"id": 0,
"entity": "IVIG",
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}
] |
高危因素包括：①年龄＜1岁，男性；②血细胞压积＜0.35；③血浆蛋白＜35g/L；④血C反应蛋白强阳性；⑤血小板数第1周低于200×109</sup>/L。 | [
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},
{
"id": 3,
"entity": "血小板",
"start_offset": 53,
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"label": "bod"
}
] |
目前有关对IVIG治疗无反应（5%～10%）的临床报告在逐渐增加。 | [
{
"id": 0,
"entity": "IVIG",
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] |
（三）双嘧达莫加用双嘧达莫3～5mg/（kg•d），可抗血小板聚集的作用。 | [
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"entity": "双嘧达莫",
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] |
（四）皮质激素具有强大抗炎、抗过敏，抑制免疫反应的药理作用，能减轻血管内皮损伤，进而减轻冠脉损害。 | [
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{
"id": 1,
"entity": "冠脉",
"start_offset": 44,
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"label": "bod"
}
] |
近年主张皮质激素用于IVIG无反应川崎患儿，在首剂给予IVIG2g/kg后36小时发热不退，追加IVIG2g/kg，36小时仍发热，除外感染之后，可考虑短程小剂量使用皮质激素。 | [
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{
"id": 4,
"entity": "皮质激素",
"start_offset": 83,
"end_offset": 87,
"label": "dru"
}
] |
（五）其他治疗急性期很快发生冠状动脉或心外动脉血栓者可用尿激酶或腹蛇抗栓酶治疗。 | [
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"entity": "冠状动脉",
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{
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},
{
"id": 3,
"entity": "腹蛇抗栓酶",
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"label": "dru"
}
] |
Kato等人用尿激酶（8000～10000U/kg），通过插管滴入冠状动脉内治疗15例巨大冠脉内血栓形成患者，结果血栓完全消失3/15例，部分消失4/15例，1例已有心肌梗死者出现再通，7例无变化，随访2年无死亡或心肌梗死病例发生。 | [
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{
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"entity": "血栓",
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},
{
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"entity": "心肌梗死",
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},
{
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},
{
"id": 6,
"entity": "心肌梗死",
"start_offset": 107,
"end_offset": 111,
"label": "dis"
}
] |
对于极少数难治性川崎病，近年来有使用生物制剂治疗的报道。 | [
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"entity": "川崎病",
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{
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}
] |
（六）随访本病退热出院后2个月内每2～4周随访心电图、B超及血小板一次，此后应每3个月随访一次到1～2年，有冠脉病变者要随访至病变消失后数年。 | [
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},
{
"id": 2,
"entity": "血小板",
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"label": "bod"
}
] |
三、新生儿的神经心理发育儿童发育的基础与神经系统，尤其是脑的发育有密切的关系。 | [
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] |
新生儿脑重约390g，占出生体重的8%，为成人脑重的1/3。 | [
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] |
大脑皮质细胞的分化从胎儿第5个月开始，出生时已具备了成人脑所具备的沟和回，但比成人的浅。 | [
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在组织学上也具备了大脑皮质层的6层基本结构。 | [
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] |
新生儿大脑的皮质下中枢，如苍白球、纹状体系统发育较成熟，而皮质的发育尚未成熟，所以新生儿出现肌张力增高及不自主的动作，兴奋及抑制过程容易扩散。 | [
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] |
随着大脑皮质的发育，对皮质下起的抑制作用也逐渐明显。 | [
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] |
（一）感知觉的发育1.视感知（1）眼的生长发育：正常足月新生儿出生时眼的大小为成人的3/4，在生后第1年内，发育最快，以后发育速度降低。 | [
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}
] |
一般来说，在出生时眼的前部结构相对较大，而较后部结构发育慢。 | [
{
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"entity": "眼",
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}
] |
这种发育特征使小儿眼球形状处在不断变化之中，最后眼球近似球形。 | [
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{
"id": 1,
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}
] |
新生儿角膜相对较大，角膜弯曲度随年龄的增加而趋于平坦，因此眼的屈光度也在不断变化之中。 | [
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"entity": "角膜",
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{
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},
{
"id": 3,
"entity": "屈光度",
"start_offset": 31,
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"label": "ite"
}
] |
正常角膜无色透明，未成熟儿可呈暂时性的乳白色迷雾状新生儿前房较浅，并有角形结构眼内压的维持是重要的。 | [
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"id": 0,
"entity": "角膜",
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{
"id": 1,
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},
{
"id": 3,
"entity": "新生儿前房较浅，并有角形结构",
"start_offset": 25,
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"label": "sym"
},
{
"id": 4,
"entity": "眼内压",
"start_offset": 39,
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"label": "ite"
}
] |
新生儿瞳孔色泽较成人浅，脉络膜血管高度清晰可见，黄斑特别是中央凹的光反射界限不够清楚眼底镜检查。 | [
{
"id": 0,
"entity": "瞳孔",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "脉络膜血管",
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},
{
"id": 2,
"entity": "新生儿瞳孔色泽较成人浅，脉络膜血管高度清晰可见，黄斑特别是中央凹的光反射界限不够清楚",
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},
{
"id": 3,
"entity": "眼底镜检查",
"start_offset": 42,
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"label": "pro"
}
] |
有瞳孔对光反应，但感觉敏锐度较差，出生后1天的视力为20/150。 | [
{
"id": 0,
"entity": "瞳孔",
"start_offset": 1,
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"label": "bod"
}
] |
视物最早的刺激是母亲的脸，特别是在哺乳时，在安静觉醒时，能注视人脸。 | [
{
"id": 0,
"entity": "脸",
"start_offset": 11,
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"label": "bod"
}
] |
新生儿喜欢轮廓鲜明和深浅颜色对比强烈的图形，可能这种图形对视网膜刺激更大，因此黑白相间的棋盘比一块单纯白布更能吸引新生儿的注意力。 | [
{
"id": 0,
"entity": "视网膜",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
}
] |
2.听感知发育很多研究证明胎儿在宫内即有听力，已能区别声音的强弱、声调的高低、熟悉或不熟悉的声音，甚至已能辨别声音来源的方向。 | [
{
"id": 0,
"entity": "宫内",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
}
] |
新生儿从一出生即有声音的定向力，在新生儿觉醒状态，头向前方，在距小儿耳旁10～15cm发出柔和的格格声和铃声，新生儿会眨眼或转动头向声音发出的方向。 | [
{
"id": 0,
"entity": "头",
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{
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"entity": "新生儿会眨眼或转动头向声音发出的方向",
"start_offset": 55,
"end_offset": 73,
"label": "sym"
}
] |
3.皮肤感觉的发育触觉器官最大，全身皮肤都有灵敏的触觉，出生后的新生儿身体喜欢紧贴着温暖的环境，因此在怀抱新生儿时，他们会紧贴在抱者的怀里。 | [
{
"id": 0,
"entity": "皮肤",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "触觉器官",
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"label": "bod"
},
{
"id": 2,
"entity": "皮肤",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
}
] |
尤其当他们哭闹时，成人通过触觉刺激，将手放在新生儿的腹部，并按住他们两个手臂，就能使他们安静下来。 | [
{
"id": 0,
"entity": "手",
"start_offset": 19,
"end_offset": 20,
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},
{
"id": 1,
"entity": "腹部",
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"label": "bod"
},
{
"id": 2,
"entity": "手臂",
"start_offset": 36,
"end_offset": 38,
"label": "bod"
}
] |
新生儿的触觉有高度的灵敏性，尤其是在眼、前额、口周、手掌、足底等部位，而大腿、前臂、躯干处却比较迟钝，这可以解释新生儿吸吮手指躯干的有些反射出现与触觉的敏感性有关。 | [
{
"id": 0,
"entity": "眼",
"start_offset": 18,
"end_offset": 19,
"label": "bod"
},
{
"id": 1,
"entity": "前额",
"start_offset": 20,
"end_offset": 22,
"label": "bod"
},
{
"id": 2,
"entity": "口周",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 3,
"entity": "手掌",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 4,
"entity": "足底",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 5,
"entity": "大腿",
"start_offset": 36,
"end_offset": 38,
"label": "bod"
},
{
"id": 6,
"entity": "前臂",
"start_offset": 39,
"end_offset": 41,
"label": "bod"
},
{
"id": 7,
"entity": "躯干",
"start_offset": 42,
"end_offset": 44,
"label": "bod"
},
{
"id": 8,
"entity": "手指",
"start_offset": 61,
"end_offset": 63,
"label": "bod"
},
{
"id": 9,
"entity": "吸吮手指",
"start_offset": 59,
"end_offset": 63,
"label": "sym"
},
{
"id": 10,
"entity": "躯干",
"start_offset": 63,
"end_offset": 65,
"label": "bod"
}
] |
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