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细菌感染一旦发展为败血症，死亡率为35%～75%，是移植后最常见的死亡原因。 | [
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病毒感染中以疱疹病毒族多见，CMV感染率占8%，也越来越多的引起重视，水痘感染往往是致死性。 | [
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（二）肾病复发常见于局灶节段型肾小球肾炎、IgA肾病、系膜增殖性肾炎、草酸盐沉积症以及溶血尿毒综合征；往往会发生急性肾小管坏死及肾功能减退，但长期随访结果移植肾存活率并不受复发影响。 | [
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"entity": "溶血尿毒综合征",
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"entity": "急性肾小管坏死",
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"entity": "肾功能减退",
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"entity": "肾",
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二次肾移植复发率略高于首次肾移植，首次肾移植复发后进行二次肾移植有75%～80%复发。 | [
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对复发的处理主张血浆置换与免疫抑制剂（CsA、CTX和MTX）联合应用可改善一些肾移植后复发的重症病例的存活率。 | [
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"entity": "肾移植",
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（三）高血压、高血脂及生长迟缓严重影响肾移植患儿的长期生活质量。 | [
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高血压常见于术后早期，与继发性肾动脉狭窄、大量糖皮质激素应用及排斥反应有关。 | [
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另有人提出成人供肾在小儿体内灌注不足造成肾素分泌增加而形成移植后高血压。 | [
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当高血压超过明显时必须使用抗高血压药物，ACEI类以及血管紧张素受体拮抗剂已作为首选控制血压的药物。 | [
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高血脂与高血压成为心血管并发症的重要危险因素。 | [
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通过饮食控制及降脂药物的应用控制血脂成为决定肾移植预后的又一因素。 | [
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国外对生长迟缓的肾移植患儿应用了人类重组生长激素rhGH进行治疗取得了良好效果，尤其在一些移植肾功能低下的患儿，必须应用rhGH才能使生长迟缓得以改善。 | [
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但长期rhGH的应用是导致排斥反应的危险因素。 | [
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（四）恶性肿瘤儿童肾移植可能并发恶性肿瘤，其中主要是恶性淋巴瘤与皮肤癌，这可能与免疫抑制剂应用导致的免疫紊乱有关。 | [
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德国报道儿童肾移植有2.6%发生恶性肿瘤。 | [
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儿童肾移植有着良好的前景，在我国的台湾、香港地区已广泛开展，而内地受经济、社会等因素制约尚未广泛开展，某些医疗单位已开始着手儿童肾移植的临床实践与基础研究，积累经验，通过多学科的协作逐步使之得到广泛开展。 | [
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四、先天性血小板功能异常性疾病的治疗先天性血小板功能异常性疾病至今仍缺乏特异性治疗药物及措施，但注意护理避免外伤，病儿有不同程度出血时，恰当地给予处理是必要的。 | [
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（二）局部出血不重时，多可用明胶海绵、凝血酶等压迫止血，青春期月经过多时可采用避孕药如复方炔诺酮以控制月经量。 | [
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（三）1-脱氧-8-精氨酸加压素（DDAVP）本药可提高血浆凝血因子Ⅷ活性和抗利尿作用，但对储存池的病人使用亦有效。 | [
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DDAVP0.2～0.3μg/kg皮下注射或溶于20ml生理盐水中缓慢静注，可使60%～70%的储存池病病人改善临床出血症状，出血时间缩短或恢复正常，但对BSS及GT病人常无效。 | [
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（四）严重出血需输注血小板浓缩制剂，反复输注易产生抗小板抗体而失效，因此有条件者宜做ABO及HLA配型，给予去白细胞的同型血小板制剂，不易引起同种免疫。 | [
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对于已产生抗血小板抗体的病人，可使用血浆交换以减少抗体后再输同型血小板制剂，有时可静脉给予丙种球蛋白亦有帮助。 | [
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（五）骨髓移植或基因治疗严重的病儿如能找到合适的供体可进行异基因骨髓移植、脐血干细胞移植，一旦成功即可根治。 | [
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二、发病机制环境因素在成人肿瘤的发病中起有重要作用，这些后天的环境因素包括个人生活习惯、环境污染、特殊感染等。 | [
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在儿童肿瘤的发病机制中涉及更多的先天性因素即基因因素。 | [
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1.胚胎期已由于某些打击存在的DNA多发性突变这些突变并非一定来自亲代，在这些出生时已存在的突变基础上，再次遭受打击时（如病毒感染）引起再次基因突变时（双突变理论）肿瘤的发病机会明显增高，导致临床上低年龄发病，但家族性发病的病例并不多见，可将此情况称为遗传性（或先天性）肿瘤发病倾向。 | [
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2.遗传因素致家族性发病倾向由亲代遗传获得某一致病基因，并由此导致肿瘤的形成。 | [
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这一因素在各种肿瘤中的比例不同，如40%视网膜母细胞瘤病例有遗传因素，而在白血病中仅2.5%病例可能有遗传因素参与。 | [
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药物性免疫性溶血性贫血药物性免疫性溶血性贫血（druginducedimmunehemolyticanemia，DIIHA）的临床特征为：①大多为急性溶血；②血红蛋白尿多见；③多数伴有脾大；④Coombs试验阳性。 | [
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"id": 8,
"entity": "Coombs试验",
"start_offset": 97,
"end_offset": 105,
"label": "pro"
}
] |
可诱发DIIHA的药物很多，主要包括抗感染药物（如青霉素类、头孢霉素类、抗结核药、磺胺类等）、消炎止痛药（安乃近、保泰松、吲哚美辛等）、镇静催眠及抗癫痫药（如苯妥英钠、氯丙嗪、氯氮等）及奎尼丁、奎宁、甲基多巴、左旋多巴、白消安等。 | [
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"id": 0,
"entity": "DIIHA",
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{
"id": 1,
"entity": "青霉素",
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"label": "dru"
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{
"id": 2,
"entity": "头孢霉素",
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"label": "dru"
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{
"id": 3,
"entity": "抗结核药",
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"label": "dru"
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{
"id": 4,
"entity": "磺胺类",
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"label": "dru"
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{
"id": 5,
"entity": "消炎止痛药",
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{
"id": 6,
"entity": "安乃近",
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{
"id": 7,
"entity": "保泰松",
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"label": "dru"
},
{
"id": 8,
"entity": "吲哚美辛",
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},
{
"id": 9,
"entity": "抗癫痫药",
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"label": "dru"
},
{
"id": 10,
"entity": "苯妥英钠",
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"label": "dru"
},
{
"id": 11,
"entity": "氯丙嗪",
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"label": "dru"
},
{
"id": 12,
"entity": "氯氮",
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"end_offset": 90,
"label": "dru"
},
{
"id": 13,
"entity": "奎尼丁",
"start_offset": 93,
"end_offset": 96,
"label": "dru"
},
{
"id": 14,
"entity": "奎宁",
"start_offset": 97,
"end_offset": 99,
"label": "dru"
},
{
"id": 15,
"entity": "甲基多巴",
"start_offset": 100,
"end_offset": 104,
"label": "dru"
},
{
"id": 16,
"entity": "左旋多巴",
"start_offset": 105,
"end_offset": 109,
"label": "dru"
},
{
"id": 17,
"entity": "白消安",
"start_offset": 110,
"end_offset": 113,
"label": "dru"
}
] |
临床上服药后出现急性溶血性贫血，且Coombs试验阳性者可确诊本病。 | [
{
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"entity": "急性溶血性贫血",
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{
"id": 1,
"entity": "Coombs试验阳性",
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] |
患儿一经确诊，应立刻停用可疑药物，并给予糖皮质激素治疗，严重贫血时，输注洗涤红细胞。 | [
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{
"id": 1,
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{
"id": 2,
"entity": "输注洗涤红细胞",
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"label": "pro"
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] |
在中国，医疗活动中善意的隐瞒（如确诊为恶性肿瘤而不告以实情）曾经被认为是积极的行为，新的《医疗事故处理办法》对告知的具体要求使得上述“积极行为”的合法性受到挑战。 | [
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"id": 0,
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"label": "dis"
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] |
（五）利益冲突儿科医生要维护患儿的利益，也要维护患儿家庭的利益，而有时患儿的利益和其家庭的利益是不一致的，这种利益冲突造成许多儿科医学上特有的伦理学的问题，也是伦理学上的重要命题。 | [
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第六章组织细胞增生症组织细胞起自骨髓造血干细胞，然后逐步分化成熟为单核-巨噬细胞及树突状细胞，完全分化成熟的组织细胞分布于全身各脏器组织中发挥它们的功能，不同部位的组织细胞各有其特定的名称，如在外周血中即为单核细胞，肝脏中称为库普弗细胞，在骨髓中称巨噬细胞，在皮肤中称朗格汉斯细胞。 | [
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{
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{
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{
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{
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{
"id": 6,
"entity": "脏器组织",
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{
"id": 7,
"entity": "组织细胞",
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{
"id": 8,
"entity": "外周血",
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{
"id": 9,
"entity": "单核细胞",
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{
"id": 10,
"entity": "肝脏",
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{
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"entity": "库普弗细胞",
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{
"id": 12,
"entity": "骨髓",
"start_offset": 120,
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"label": "bod"
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{
"id": 13,
"entity": "巨噬细胞",
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"label": "bod"
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{
"id": 14,
"entity": "皮肤",
"start_offset": 130,
"end_offset": 132,
"label": "bod"
},
{
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"entity": "朗格汉斯细胞",
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] |
单核-巨噬细胞主要功能为吞噬及杀伤入侵的微生物及处理衰老和变性的细胞，而树状突细胞多作为抗原提呈细胞，在主要组织相容性抗原参与下，将抗原提呈给T、B淋巴细胞，激发体内特异性细胞及体液免疫反应。 | [
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{
"id": 1,
"entity": "微生物",
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"label": "mic"
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{
"id": 2,
"entity": "衰老和变性的细胞",
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{
"id": 3,
"entity": "树状突细胞",
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{
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"entity": "细胞",
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{
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"entity": "T、B淋巴细胞",
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{
"id": 6,
"entity": "特异性细胞",
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"label": "bod"
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] |
该分类将组织细胞增生症分为Ⅰ、Ⅱ、Ⅲ型，Ⅰ型旧称为组织细胞增生症Ⅹ，病变中的组织细胞主要为郎罕细胞，因此目前已更名为郎罕组织细胞增生症。 | [
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{
"id": 1,
"entity": "组织细胞增生症Ⅹ",
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"entity": "组织细胞",
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{
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"entity": "郎罕细胞",
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{
"id": 4,
"entity": "郎罕组织细胞增生症",
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] |
组织细胞增生症Ⅲ型为恶性组织细胞疾病，包括急性单核细胞性白血病、恶性组织细胞增生症、组织细胞肉瘤和极少见的恶性朗格汉斯增生症。 | [
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{
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"entity": "恶性组织细胞疾病",
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{
"id": 2,
"entity": "急性单核细胞性白血病",
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{
"id": 3,
"entity": "恶性组织细胞增生症",
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{
"id": 4,
"entity": "组织细胞肉瘤",
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{
"id": 5,
"entity": "恶性朗格汉斯增生症",
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"label": "dis"
}
] |
第一节朗格汉斯细胞性组织细胞增生症朗格汉斯细胞性组织细胞增生症（Langerhan’scellhistiocytosis）为组织细胞增生症Ⅰ型。 | [
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] |
因其共同病理特点为郎罕组织细胞浸润，故重新命名为朗格汉斯细胞性组织细胞增多症，朗格汉斯细胞组织细胞为一种正常的抗原提呈细胞，但对抗原刺激呈失控性反应。 | [
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] |
根据发病年龄，病变范围和临床表现，将此类疾病分为三种亚型，包括勒-雪综合征（Letterer-Siwesyndrome），韩-薛-柯综合征（Hand-Schuller-Christiansyndrome）和骨嗜酸细胞性肉芽肿。 | [
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{
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"entity": "骨嗜酸细胞性肉芽肿",
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（一）勒-雪综合征（急性婴儿型）以内脏病变为主，此型临床表现最严重、也最多见，多于1岁以内发病，起病急而重，以内脏和皮肤受侵害为主。 | [
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{
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"entity": "内脏",
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表现为发热、皮疹、咳嗽、苍白、耳流脓、肝、脾很快增大，肺部广泛浸润，淋巴结轻度增大，皮疹较特殊，主要分布于躯干、颈部、头皮和发际，四肢少见。 | [
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{
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{
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{
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{
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{
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{
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{
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{
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{
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{
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{
"id": 17,
"entity": "四肢",
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}
] |
初起为淡红色斑丘疹，直径1～3mm，继而呈出血性或湿疹样、皮脂溢出样皮疹，以后皮疹表面结痂、脱屑、脱痂后留有白斑或色素沉着。 | [
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各期皮疹可同时存在，常成批发生，一批消退，一批又起，手触摸时有粗糙感。 | [
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出疹前常先发热，出疹同时伴肝增大，呼吸道感染时，症状急剧加重，极易发生肺炎，出现喘憋和发绀，但肺部体征多不明显，因系肺间质性病变，可并发气胸和皮下气肿。 | [
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{
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{
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{
"id": 9,
"entity": "皮下气肿",
"start_offset": 71,
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"label": "sym"
}
] |
呼吸衰竭是致死的主要原因。 | [
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此外常见耳流脓、营养不良、腹泻和贫血。 | [
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"entity": "耳",
"start_offset": 4,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "耳流脓",
"start_offset": 4,
"end_offset": 7,
"label": "sym"
},
{
"id": 2,
"entity": "营养不良",
"start_offset": 8,
"end_offset": 12,
"label": "sym"
},
{
"id": 3,
"entity": "腹泻",
"start_offset": 13,
"end_offset": 15,
"label": "sym"
},
{
"id": 4,
"entity": "贫血",
"start_offset": 16,
"end_offset": 18,
"label": "dis"
}
] |
也可同时有溶骨性骨骼病变，但与其他二型相比，相对较少。 | [
{
"id": 0,
"entity": "溶骨性骨骼病变",
"start_offset": 5,
"end_offset": 12,
"label": "dis"
}
] |
（二）韩-薛-柯综合征骨损害伴中度其他器官侵犯，又称慢性黄色瘤病，多发生于3～4岁小儿。 | [
{
"id": 0,
"entity": "韩-薛-柯综合征",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "骨损害",
"start_offset": 11,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "慢性黄色瘤病",
"start_offset": 26,
"end_offset": 32,
"label": "dis"
}
] |
颅骨缺损、突眼、尿崩症是此型的三大特征，这三大特征可先后出现或在病程中只见其中之一或二。 | [
{
"id": 0,
"entity": "颅骨",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "颅骨缺损、突眼",
"start_offset": 0,
"end_offset": 7,
"label": "sym"
},
{
"id": 2,
"entity": "尿崩症",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
}
] |
初起颅骨损害呈肿块状凸起，硬，有轻度压痛。 | [
{
"id": 0,
"entity": "颅骨",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "颅骨损害呈肿块状凸起，硬，有轻度压痛",
"start_offset": 2,
"end_offset": 20,
"label": "sym"
}
] |
当病变蚀穿颅骨外板后、肿物变软、触之有波动感，常可触及颅骨边缘，压痛不明显，以后肿块逐渐吸收、局部下凹，缺损大者可触及脑，并随脉搏跳动。 | [
{
"id": 0,
"entity": "颅骨外板",
"start_offset": 5,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "颅骨",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
},
{
"id": 2,
"entity": "肿物变软、触之有波动感，常可触及颅骨边缘，压痛不明显",
"start_offset": 11,
"end_offset": 37,
"label": "sym"
},
{
"id": 3,
"entity": "肿块",
"start_offset": 40,
"end_offset": 42,
"label": "bod"
},
{
"id": 4,
"entity": "脑",
"start_offset": 59,
"end_offset": 60,
"label": "bod"
},
{
"id": 5,
"entity": "肿块逐渐吸收、局部下凹，缺损大者可触及脑，并随脉搏跳动",
"start_offset": 40,
"end_offset": 67,
"label": "sym"
}
] |
尿崩症由垂体或下丘脑受累所致，不一定有蝶鞍破坏。 | [
{
"id": 0,
"entity": "尿崩症",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "垂体",
"start_offset": 4,
"end_offset": 6,
"label": "bod"
},
{
"id": 2,
"entity": "下丘脑",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 3,
"entity": "蝶鞍破坏",
"start_offset": 19,
"end_offset": 23,
"label": "sym"
}
] |
皮疹呈孤立、稀疏的黄色丘疹，似半个小米粒或黄豆状，突出在皮肤表面，若以玻片压于其上，可见皮疹中央发黄，是慢性黄色瘤病名称的来由。 | [
{
"id": 0,
"entity": "皮肤",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
},
{
"id": 1,
"entity": "皮疹呈孤立、稀疏的黄色丘疹，似半个小米粒或黄豆状，突出在皮肤表面",
"start_offset": 0,
"end_offset": 32,
"label": "sym"
},
{
"id": 2,
"entity": "玻片",
"start_offset": 35,
"end_offset": 37,
"label": "equ"
},
{
"id": 3,
"entity": "皮疹中央发黄",
"start_offset": 44,
"end_offset": 50,
"label": "sym"
},
{
"id": 4,
"entity": "慢性黄色瘤病",
"start_offset": 52,
"end_offset": 58,
"label": "dis"
}
] |
其后皮疹开始消退变软、变浅，慢慢吸收。 | [
{
"id": 0,
"entity": "皮疹",
"start_offset": 2,
"end_offset": 4,
"label": "sym"
}
] |
此外低热、轻度肝、脾增大，贫血，牙龈齿槽肿胀，发炎、坏死、萎缩、牙齿松动、脱落也常见。 | [
{
"id": 0,
"entity": "低热",
"start_offset": 2,
"end_offset": 4,
"label": "sym"
},
{
"id": 1,
"entity": "肝",
"start_offset": 7,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "脾",
"start_offset": 9,
"end_offset": 10,
"label": "bod"
},
{
"id": 3,
"entity": "轻度肝、脾增大",
"start_offset": 5,
"end_offset": 12,
"label": "sym"
},
{
"id": 4,
"entity": "贫血",
"start_offset": 13,
"end_offset": 15,
"label": "dis"
},
{
"id": 5,
"entity": "牙龈",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
},
{
"id": 6,
"entity": "牙龈齿槽肿胀",
"start_offset": 16,
"end_offset": 22,
"label": "sym"
},
{
"id": 7,
"entity": "发炎",
"start_offset": 23,
"end_offset": 25,
"label": "sym"
},
{
"id": 8,
"entity": "坏死",
"start_offset": 26,
"end_offset": 28,
"label": "sym"
},
{
"id": 9,
"entity": "萎缩",
"start_offset": 29,
"end_offset": 31,
"label": "sym"
},
{
"id": 10,
"entity": "牙齿",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 11,
"entity": "牙齿松动、脱落",
"start_offset": 32,
"end_offset": 39,
"label": "sym"
}
] |
（三）骨嗜酸细胞肉芽肿为单纯骨损害型，是本症中预后最好的一型，多发于4～7岁小儿，但也可见于婴儿或成人，任何骨骼均可受累，但以颅骨、四肢骨、脊椎、骨盆最常见。 | [
{
"id": 0,
"entity": "骨嗜酸细胞肉芽肿",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "骨骼",
"start_offset": 54,
"end_offset": 56,
"label": "bod"
},
{
"id": 2,
"entity": "颅骨",
"start_offset": 63,
"end_offset": 65,
"label": "bod"
},
{
"id": 3,
"entity": "四肢骨",
"start_offset": 66,
"end_offset": 69,
"label": "bod"
},
{
"id": 4,
"entity": "脊椎",
"start_offset": 70,
"end_offset": 72,
"label": "bod"
},
{
"id": 5,
"entity": "骨盆",
"start_offset": 73,
"end_offset": 75,
"label": "bod"
}
] |
不少患儿是在偶然情况下或出现病理性骨折时发现。 | [
{
"id": 0,
"entity": "病理性骨折",
"start_offset": 14,
"end_offset": 19,
"label": "dis"
}
] |
唯有脊椎病变，椎弓破坏者，常伴神经压迫症状，如肢体麻木、疼痛、无力、瘫痪，甚至大小便失禁成为疾病的主诉而就医。 | [
{
"id": 0,
"entity": "脊椎",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "脊椎病变",
"start_offset": 2,
"end_offset": 6,
"label": "sym"
},
{
"id": 2,
"entity": "椎弓破坏",
"start_offset": 7,
"end_offset": 11,
"label": "sym"
},
{
"id": 3,
"entity": "神经压迫",
"start_offset": 15,
"end_offset": 19,
"label": "sym"
},
{
"id": 4,
"entity": "肢体",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 5,
"entity": "肢体麻木",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
},
{
"id": 6,
"entity": "疼痛",
"start_offset": 28,
"end_offset": 30,
"label": "sym"
},
{
"id": 7,
"entity": "无力",
"start_offset": 31,
"end_offset": 33,
"label": "sym"
},
{
"id": 8,
"entity": "瘫痪",
"start_offset": 34,
"end_offset": 36,
"label": "sym"
},
{
"id": 9,
"entity": "大小便失禁",
"start_offset": 39,
"end_offset": 44,
"label": "sym"
}
] |
发热、贫血、肝脾大、耳流脓伴典型皮疹时要考虑郎罕细胞性组织细胞增生症Ⅰ型。 | [
{
"id": 0,
"entity": "发热",
"start_offset": 0,
"end_offset": 2,
"label": "sym"
},
{
"id": 1,
"entity": "贫血",
"start_offset": 3,
"end_offset": 5,
"label": "sym"
},
{
"id": 2,
"entity": "肝脾大",
"start_offset": 6,
"end_offset": 9,
"label": "sym"
},
{
"id": 3,
"entity": "耳流脓伴",
"start_offset": 10,
"end_offset": 14,
"label": "sym"
},
{
"id": 4,
"entity": "皮疹",
"start_offset": 16,
"end_offset": 18,
"label": "sym"
},
{
"id": 5,
"entity": "郎罕细胞性组织细胞增生症",
"start_offset": 22,
"end_offset": 34,
"label": "dis"
}
] |
突眼、尿崩症、颅骨缺损是Ⅱ型综合征典型表现，应疑及本病。 | [
{
"id": 0,
"entity": "突眼",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "尿崩症",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
},
{
"id": 2,
"entity": "颅骨缺损是Ⅱ型综合征",
"start_offset": 7,
"end_offset": 17,
"label": "dis"
}
] |
骨骼X线摄片示扁平骨和长骨发生溶骨性骨质破坏，形状不规则，边缘可呈锯齿状。 | [
{
"id": 0,
"entity": "骨骼",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "扁平骨",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "长骨",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 3,
"entity": "溶骨性骨质破坏",
"start_offset": 15,
"end_offset": 22,
"label": "sym"
}
] |
脊椎骨受压则呈扁平椎，但椎间隙不狭窄。 | [
{
"id": 0,
"entity": "脊椎",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
}
] |
上下颌骨破坏可致牙齿脱落，长骨的缺损多为囊状，单发或互相融合成分房状，骨皮质变薄。 | [
{
"id": 0,
"entity": "颌骨",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "牙齿",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "长骨",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 3,
"entity": "骨皮质",
"start_offset": 35,
"end_offset": 38,
"label": "bod"
}
] |
破坏明显处可有层状骨膜增生。 | [
{
"id": 0,
"entity": "骨膜",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
}
] |
以上典型的骨X线变化提示郎罕细胞性组织细胞增生症。 | [
{
"id": 0,
"entity": "骨X线",
"start_offset": 5,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "郎罕细胞性组织细胞增生症",
"start_offset": 12,
"end_offset": 24,
"label": "dis"
}
] |
皮疹印片或活检、骨病变，郎罕细胞是诊断的依据。 | [
{
"id": 0,
"entity": "活检",
"start_offset": 5,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "骨",
"start_offset": 8,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "骨病变",
"start_offset": 8,
"end_offset": 11,
"label": "sym"
},
{
"id": 3,
"entity": "郎罕细胞",
"start_offset": 12,
"end_offset": 16,
"label": "bod"
}
] |
S-100蛋白染色阳性，电镜找到Birbeck颗粒支持本病的诊断。 | [
{
"id": 0,
"entity": "电镜",
"start_offset": 12,
"end_offset": 14,
"label": "equ"
}
] |
（一）局限病灶手术治疗病变局限的骨嗜酸性肉芽肿应采取手术刮除或切除。 | [
{
"id": 0,
"entity": "骨嗜酸性肉芽肿",
"start_offset": 16,
"end_offset": 23,
"label": "dis"
}
] |
比较小的病灶应用局部氢化可的松注射亦可取得与手术刮除同样的效果。 | [
{
"id": 0,
"entity": "氢化可的松",
"start_offset": 10,
"end_offset": 15,
"label": "dru"
}
] |
（二）放射治疗适用于孤立的骨骼病变，尤以手术刮除有困难的部位如：眼眶周围、颌骨、乳突或负重后易发生骨折和神经损伤的脊椎等部位，以及早期的垂体病变，尿崩症出现时间较久（如6个月以上），放射治疗大多无效。 | [
{
"id": 0,
"entity": "骨骼",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 1,
"entity": "骨骼病变",
"start_offset": 13,
"end_offset": 17,
"label": "sym"
},
{
"id": 2,
"entity": "手术刮除",
"start_offset": 20,
"end_offset": 24,
"label": "pro"
},
{
"id": 3,
"entity": "眼眶",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 4,
"entity": "颌骨",
"start_offset": 37,
"end_offset": 39,
"label": "bod"
},
{
"id": 5,
"entity": "乳突",
"start_offset": 40,
"end_offset": 42,
"label": "bod"
},
{
"id": 6,
"entity": "骨折",
"start_offset": 49,
"end_offset": 51,
"label": "sym"
},
{
"id": 7,
"entity": "神经损伤",
"start_offset": 52,
"end_offset": 56,
"label": "sym"
},
{
"id": 8,
"entity": "脊椎",
"start_offset": 57,
"end_offset": 59,
"label": "bod"
},
{
"id": 9,
"entity": "垂体",
"start_offset": 68,
"end_offset": 70,
"label": "bod"
},
{
"id": 10,
"entity": "垂体病变",
"start_offset": 68,
"end_offset": 72,
"label": "sym"
},
{
"id": 11,
"entity": "尿崩症",
"start_offset": 73,
"end_offset": 76,
"label": "dis"
},
{
"id": 12,
"entity": "放射治疗",
"start_offset": 91,
"end_offset": 95,
"label": "pro"
}
] |
皮肤病变对放疗亦不敏感。 | [
{
"id": 0,
"entity": "皮肤病变",
"start_offset": 0,
"end_offset": 4,
"label": "sym"
}
] |
（三）药物治疗肾上腺皮质激素为首选药物，且多药联合治疗并不比采用单一药物效果好，但对多脏器受累病人应采用联合化疗。 | [
{
"id": 0,
"entity": "肾上腺皮质激素",
"start_offset": 7,
"end_offset": 14,
"label": "dru"
},
{
"id": 1,
"entity": "脏器",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
}
] |
常用药物：①肾上腺皮质激素：泼尼松45～60mg/（m2</sup>•d）或地塞米松8～10mg/（m2</sup>•d）口服，分3～4次，6周后减至半量，再用4周，然后逐渐减量，总疗程12周。 | [
{
"id": 0,
"entity": "肾上腺皮质激素",
"start_offset": 6,
"end_offset": 13,
"label": "dru"
},
{
"id": 1,
"entity": "泼尼松",
"start_offset": 14,
"end_offset": 17,
"label": "dru"
},
{
"id": 2,
"entity": "地塞米松",
"start_offset": 38,
"end_offset": 42,
"label": "dru"
}
] |
危重病人可静脉滴注氢化可的松250～300mg/M2</sup>。 | [
{
"id": 0,
"entity": "氢化可的松",
"start_offset": 9,
"end_offset": 14,
"label": "dru"
}
] |
急性症状消失后改为口服，此类药物对全身症状如发热、皮疹和贫血等效果较好。 | [
{
"id": 0,
"entity": "发热",
"start_offset": 22,
"end_offset": 24,
"label": "sym"
},
{
"id": 1,
"entity": "皮疹",
"start_offset": 25,
"end_offset": 27,
"label": "sym"
},
{
"id": 2,
"entity": "贫血",
"start_offset": 28,
"end_offset": 30,
"label": "dis"
}
] |
②长春花碱：每次6mg/m2</sup>，一周1次。 | [
{
"id": 0,
"entity": "长春花碱",
"start_offset": 1,
"end_offset": 5,
"label": "dru"
}
] |
或长春新碱每次1.4mg/m2</sup>，一周1次，静脉注射，连用4～6周，以后改为每月一次或停8～12周后，再给4～6周，并与泼尼松合用。 | [
{
"id": 0,
"entity": "长春新碱",
"start_offset": 1,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "泼尼松",
"start_offset": 65,
"end_offset": 68,
"label": "dru"
}
] |
若应用上述药物4～6周后效果不明显，可加用足叶乙甙（Vp16）100/（m2</sup>•d），静滴，每日1次，用3～5天，每月一疗程。 | [
{
"id": 0,
"entity": "足叶乙甙",
"start_offset": 21,
"end_offset": 25,
"label": "dru"
},
{
"id": 1,
"entity": "Vp16",
"start_offset": 26,
"end_offset": 30,
"label": "dru"
}
] |
亦可采用6-巯基嘌呤（6-MP）或6-硫鸟嘌呤（6-TG）60～75mg/（m2•d）口服。 | [
{
"id": 0,
"entity": "6-巯基嘌呤",
"start_offset": 4,
"end_offset": 10,
"label": "dru"
},
{
"id": 1,
"entity": "6-MP",
"start_offset": 11,
"end_offset": 15,
"label": "dru"
},
{
"id": 2,
"entity": "6-硫鸟嘌呤",
"start_offset": 17,
"end_offset": 23,
"label": "dru"
},
{
"id": 3,
"entity": "6-TG",
"start_offset": 24,
"end_offset": 28,
"label": "dru"
}
] |
甲氨蝶呤（MTX）每次15～20mg/m2</sup>，每周1次口服或静脉注射。 | [
{
"id": 0,
"entity": "甲氨蝶呤",
"start_offset": 0,
"end_offset": 4,
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{
"id": 1,
"entity": "MTX",
"start_offset": 5,
"end_offset": 8,
"label": "dru"
},
{
"id": 2,
"entity": "静脉注射",
"start_offset": 35,
"end_offset": 39,
"label": "pro"
}
] |
多脏器病变化疗时间一般不短于1年。 | [
{
"id": 0,
"entity": "脏器",
"start_offset": 1,
"end_offset": 3,
"label": "bod"
}
] |
若于用药后很快退热，精神食欲好转，1～2周后皮疹消退，肝、脾缩小，肺部症状减轻说明对治疗敏感，但骨骼X线，肺部病变消退较慢。 | [
{
"id": 0,
"entity": "皮疹消退",
"start_offset": 22,
"end_offset": 26,
"label": "sym"
},
{
"id": 1,
"entity": "肝",
"start_offset": 27,
"end_offset": 28,
"label": "bod"
},
{
"id": 2,
"entity": "脾",
"start_offset": 29,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "肝、脾缩小",
"start_offset": 27,
"end_offset": 32,
"label": "sym"
},
{
"id": 4,
"entity": "肺部",
"start_offset": 33,
"end_offset": 35,
"label": "bod"
},
{
"id": 5,
"entity": "骨骼X线",
"start_offset": 48,
"end_offset": 52,
"label": "pro"
},
{
"id": 6,
"entity": "肺部",
"start_offset": 53,
"end_offset": 55,
"label": "bod"
}
] |
有些病人在治疗过程中可有反复或发生新的病状，则应改换其他药物，如环磷酰胺10mg/（kg•d），连续3天，每三周1次。 | [
{
"id": 0,
"entity": "环磷酰胺",
"start_offset": 32,
"end_offset": 36,
"label": "dru"
}
] |
（四）免疫治疗胸腺素或胸腺肽，每次3～5mg静脉或肌肉注射，连用2～3个月，可与化疗联合使用，增加疗效。 | [
{
"id": 0,
"entity": "胸腺素",
"start_offset": 7,
"end_offset": 10,
"label": "dru"
},
{
"id": 1,
"entity": "胸腺肽",
"start_offset": 11,
"end_offset": 14,
"label": "dru"
},
{
"id": 2,
"entity": "肌肉",
"start_offset": 25,
"end_offset": 27,
"label": "bod"
}
] |
（五）支持治疗包括用SMZco预防卡氏肺囊虫病及积极控制感染，特别是中耳炎。 | [
{
"id": 0,
"entity": "SMZco",
"start_offset": 10,
"end_offset": 15,
"label": "dru"
},
{
"id": 1,
"entity": "卡氏肺囊虫病",
"start_offset": 17,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "中耳炎",
"start_offset": 34,
"end_offset": 37,
"label": "dis"
}
] |
如合并呼吸道感染导致呼吸衰竭，应及时给氧并需监护。 | [
{
"id": 0,
"entity": "呼吸道感染",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "呼吸衰竭",
"start_offset": 10,
"end_offset": 14,
"label": "sym"
}
] |
对尿崩症患者应给神经垂体激素治疗。 | [
{
"id": 0,
"entity": "尿崩症",
"start_offset": 1,
"end_offset": 4,
"label": "dis"
}
] |
此外预防出血、纠正贫血亦很重要。 | [
{
"id": 0,
"entity": "贫血",
"start_offset": 9,
"end_offset": 11,
"label": "dis"
}
] |
【预后】预后决定于发病年龄，受累器官的数目和有无脏器功能衰竭。 | [
{
"id": 0,
"entity": "脏器",
"start_offset": 24,
"end_offset": 26,
"label": "bod"
}
] |
参考文献1.BillmireD，VinocurC，RescorlaF，etal.Malignantmediastinalgermcelltumors：anintergroupstudy.JPediatrSurg，2001，36（1）：18-182.MannJR，RaafatF，RobinsonK，etal.TheUnitedKingdomChildren'sCancerStudyGroup'ssecondgermcelltumorstudy：carboplatin，etoposide，andbleomycinareeffectivetreatmentforchildrenwithmalignantextracranialgermcelltumors，withacceptabletoxicity.JClinOncol，2000，18（22）：3809-38093.PhilipAPizzo，DavidGPoplock.PrincipleandPracticeofPediatricOncology.4rded.Philadelphia.NewYork：LWW.com，2002.1091-10914.RescorlaF，BillmireD，StolarC，etal.Theeffectofcisplatindoseandsurgicalresectioninchildrenwithmalignantgermcelltumorsatthesacrococcygealregion：apediatricintergrouptrial（POG9049/CCG8882）.JPediatrSurg，2001，36（1）：12-125.VukyJ，BainsM，BacikJ，etal.Roleofpostchemotherapyadjunctivesurgeryinthemanagementofpatientswithnonseminomaarisingfromthemediastinum.JClinOncol，2001，19（3）：682-688 | [
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"id": 0,
"entity": "Malignantmediastinalgermcelltumors",
"start_offset": 40,
"end_offset": 74,
"label": "dis"
},
{
"id": 1,
"entity": "secondgermcelltumor",
"start_offset": 198,
"end_offset": 217,
"label": "dis"
},
{
"id": 2,
"entity": "carboplatin",
"start_offset": 223,
"end_offset": 234,
"label": "dru"
},
{
"id": 3,
"entity": "etoposide",
"start_offset": 235,
"end_offset": 244,
"label": "dru"
},
{
"id": 4,
"entity": "bleomycinare",
"start_offset": 248,
"end_offset": 260,
"label": "dru"
},
{
"id": 5,
"entity": "malignantextracranialgermcelltumors",
"start_offset": 293,
"end_offset": 328,
"label": "dis"
},
{
"id": 6,
"entity": "malignantgermcelltumor",
"start_offset": 595,
"end_offset": 617,
"label": "dis"
}
] |
第五节维生素B<sub>1</sub>缺乏症维生素B<sub>1</sub>又称硫胺素、抗脚气病因子或抗神经炎因子，它是最早发现的维生素之一。 | [
{
"id": 0,
"entity": "维生素B<sub>1</sub>缺乏症",
"start_offset": 3,
"end_offset": 22,
"label": "dis"
},
{
"id": 1,
"entity": "维生素B<sub>1</sub>",
"start_offset": 22,
"end_offset": 38,
"label": "bod"
},
{
"id": 2,
"entity": "硫胺素",
"start_offset": 40,
"end_offset": 43,
"label": "bod"
},
{
"id": 3,
"entity": "抗脚气病因子",
"start_offset": 44,
"end_offset": 50,
"label": "bod"
},
{
"id": 4,
"entity": "抗神经炎因子",
"start_offset": 51,
"end_offset": 57,
"label": "bod"
},
{
"id": 5,
"entity": "维生素",
"start_offset": 65,
"end_offset": 68,
"label": "bod"
}
] |
维生素B<sub>1</sub>在高温、特别是高温碱性溶液中易被破坏，在酸性溶液中，稳定性较好。 | [
{
"id": 0,
"entity": "维生素B<sub>1</sub>",
"start_offset": 0,
"end_offset": 16,
"label": "bod"
}
] |
在体内硫胺80%是以硫胺素焦磷酸盐（TPP）的形式存在，10%是以硫胺素三磷酸盐的形式存在，其余的为硫胺素单磷酸盐或游离的硫胺素。 | [
{
"id": 0,
"entity": "硫胺",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "硫胺素焦磷酸盐",
"start_offset": 10,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "TPP",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "硫胺素三磷酸盐",
"start_offset": 33,
"end_offset": 40,
"label": "bod"
},
{
"id": 4,
"entity": "硫胺素单磷酸盐",
"start_offset": 50,
"end_offset": 57,
"label": "bod"
},
{
"id": 5,
"entity": "硫胺素",
"start_offset": 61,
"end_offset": 64,
"label": "bod"
}
] |
维生素B<sub>1</sub>缺乏将引起一种典型的疾病，被称为脚气病。 | [
{
"id": 0,
"entity": "维生素B<sub>1</sub>缺乏",
"start_offset": 0,
"end_offset": 18,
"label": "dis"
},
{
"id": 1,
"entity": "脚气病",
"start_offset": 32,
"end_offset": 35,
"label": "dis"
}
] |
【流行病学】18～19世纪，脚气病在中国、日本（尤其在东南亚一带）广为流行，当时每年约有几十万人死于脚气病。 | [
{
"id": 0,
"entity": "脚气病",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "脚气病",
"start_offset": 50,
"end_offset": 53,
"label": "dis"
}
] |
第一个记录脚气病的是1592年荷兰医生JacobBontius。 | [
{
"id": 0,
"entity": "脚气病",
"start_offset": 5,
"end_offset": 8,
"label": "dis"
}
] |
1897年，一名驻爪哇的荷兰医生Eijkman以小鸡做实验，发现用精白米饲养小鸡，即出现一种类似脚气病的多发性神经炎，如用糙米饲养小鸡，则能预防这种疾病。 | [
{
"id": 0,
"entity": "脚气病",
"start_offset": 48,
"end_offset": 51,
"label": "dis"
},
{
"id": 1,
"entity": "多发性神经炎",
"start_offset": 52,
"end_offset": 58,
"label": "dis"
}
] |
1936年，Williams公布了硫胺素的化学结构，它是由含硫噻唑环联结氨基吡啶环组成，并开始人工合成。 | [
{
"id": 0,
"entity": "硫胺素",
"start_offset": 17,
"end_offset": 20,
"label": "bod"
}
] |
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