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小肠结肠炎的致病机制至今未明，有患者甚至在行粪便改道及结肠造瘘术后还有持久性的小肠结肠炎症状。 | [
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"entity": "小肠结肠炎",
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【诊断】ENS是小肠的“中枢”，与大脑相似，即使存在某些缺陷，一定程度上仍具有自主功能。 | [
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因此，既可以婴儿期肠梗阻表现收治入院，也可以儿童或成人期慢性便秘就诊。 | [
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（一）影像学检查仰卧位及直立位腹部平片发现结肠有液平，应怀疑HD，常规需行钡剂灌肠检查。 | [
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新生儿在钡剂灌肠前不必直肠指检或清洁灌肠，以免出现假阴性结果。 | [
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怀疑存在胎粪性肠梗阻或者胎粪栓塞综合征时，通常应选用水溶性造影剂取代钡剂以防止加重梗阻。 | [
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经典的X线表现为：直肠口径正常或远端狭窄，移行段呈漏斗型扩张，近端结肠明显扩张。 | [
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不能确诊的病例，可在24小时后重复摄片，如结肠中仍有造影剂潴留则有助诊断。 | [
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有10%HD和29%伴发HD的B型IND患儿，钡剂灌肠检查无法确诊。 | [
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全结肠无神经节细胞患儿，结肠可无典型狭窄表现，但若观察到钡剂反流入扩张回肠则有助诊断。 | [
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小肠结肠炎患儿的腹部平片可见肠壁增厚、黏液不规则以及肠曲明显扩张，由于肠道肌肉功能受炎症破坏，有正常神经支配的移行段结肠一般亦无典型表现。 | [
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运用放射性标记物评估肠道排空时间，有助于确定肠道病变的最高部位，确定手术切除的长度，判断可能伴发的肠神经元异常，如B型IND或神经节细胞缺乏的临床特征。 | [
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该方法也可用于根治术前已行肠造瘘的患儿，明确从远端造口到肛门排泄所需时间。 | [
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（二）直肠肛管测压直肠肛管测压诊断HD的准确率高达85%。 | [
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正常情况下，气囊充盈扩张直肠，可使内括约肌松弛。 | [
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神经节细胞缺乏患儿可见特异性多节段收缩波形；直肠气囊扩张时，肛管或直肠缺乏抑制性反应，直肠肛管测压直肠肛管测压曲线上升。 | [
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神经节细胞减少症或IND患者则可能表现为抑制性反应低下或消失。 | [
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但直肠肛管测压一般不用于新生儿，因为即使正常婴儿，直肠括约肌反射需在生后12天才发育完全；而那些胎龄＜39周或体重＜2.7kg的婴儿则尚未形成该反射。 | [
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总体而言，直肠肛管测压是筛查HD及相关疾病一种有效手段。 | [
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（三）直肠活体组织检查直肠活体组织检查可确诊HD。 | [
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理想的活检标本直径约为3.5mm，且包含黏液下层，但有吸引活检导致出血和穿孔的报道。 | [
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新生儿神经节细胞发育不成熟，需采用特殊染色技术（LDH及SDH还原反应），否则可能无法识别；吸引活检标本也可能不能完全反映受累肠段的真实情况，易误诊为神经节细胞缺乏症，导致不必要的肠切除。 | [
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"entity": "肠",
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若标本仅包含黏液下丛，则可能漏诊神经节细胞减少症。 | [
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因此若怀疑神经节细胞减少症或肠肌间神经丛异位，建议应取肠壁全层标本。 | [
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【鉴别诊断】临床上往往很难鉴别新生儿胎粪栓塞综合征、左半小结肠综合征、HD及相关ENS病。 | [
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巨膀胱-小结肠-肠蠕动不全综合征是导致新生儿慢性肠梗阻的一种罕见而致命的疾病，至今报道不足70例，病理表现为平滑肌细胞退行性变与大量结缔组织增生，但根本病因尚不清楚。 | [
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腹肌松弛和膀胱扩张导致腹胀，可伴有小肠旋转不良和结肠蠕动功能不良。 | [
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【治疗】包括胃肠减压、造瘘术及根治手术等。 | [
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（一）减压有肠梗阻表现的新生儿应立即放置鼻胃管，并置肛管行清洁灌肠，反复刺激排便。 | [
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"entity": "刺激排便",
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影像学、直肠测压和活体组织标本组化分析明确诊断后，若有必要，需行结肠造瘘术。 | [
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（二）结肠造瘘术新生儿HD肠梗阻时，应先考虑行肠造瘘术，一般认为新生儿期以后行根治术效果更佳。 | [
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造瘘术前必须行清洁灌肠，造瘘口一般位于右侧横结肠，若病变累及全部或几乎全部结肠，必须行回肠造瘘术。 | [
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（三）根治手术巨结肠根治术是最为有效的治疗手段。 | [
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1948年，Swenson和Bill首次施行了无神经节细胞肠段切除术。 | [
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以此为基础，逐步发展了其他四种基本术式：Swenson术式、Duhamel-GROB术式、Rehbein术式和Soave术式。 | [
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"label": "pro"
},
{
"id": 1,
"entity": "Duhamel-GROB术式",
"start_offset": 30,
"end_offset": 44,
"label": "pro"
},
{
"id": 2,
"entity": "Rehbein术式",
"start_offset": 45,
"end_offset": 54,
"label": "pro"
},
{
"id": 3,
"entity": "Soave术式",
"start_offset": 55,
"end_offset": 62,
"label": "pro"
}
] |
随着微创外科的发展及器械的改良，腹腔镜技术也已应用于巨结肠根治术。 | [
{
"id": 0,
"entity": "腹腔镜技术",
"start_offset": 16,
"end_offset": 21,
"label": "pro"
},
{
"id": 1,
"entity": "巨结肠根治术",
"start_offset": 26,
"end_offset": 32,
"label": "pro"
}
] |
在此基础上又出现了经肛门游离直肠和结肠，并将正常段结肠从肛门拖下、成形的方法。 | [
{
"id": 0,
"entity": "经肛门游离直肠",
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"label": "bod"
},
{
"id": 1,
"entity": "肛门",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
}
] |
主要适用于小婴儿和无神经节细胞肠段比较短的患儿，由于不进腹腔，具有手术创伤小、并发症少及恢复快的优点。 | [
{
"id": 0,
"entity": "神经节细胞肠段",
"start_offset": 10,
"end_offset": 17,
"label": "bod"
},
{
"id": 1,
"entity": "腹腔",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
}
] |
【预后】先天性巨结肠尽管病因不明，但常见的普通型病变在诊断和治疗上已比较定型，效果也比较肯定。 | [
{
"id": 0,
"entity": "先天性巨结肠",
"start_offset": 4,
"end_offset": 10,
"label": "dis"
}
] |
长段型和其他一些少见类型的处理尚不尽如人意，巨结肠并发小肠结肠炎仍是临床面临的非常棘手的问题。 | [
{
"id": 0,
"entity": "巨结肠并发小肠结肠炎",
"start_offset": 22,
"end_offset": 32,
"label": "dis"
}
] |
三、异体造血干细胞移植供体的选择（一）人类白细胞抗原（HLA）基本理论供受体之间主要组织相容性复合物（majorhistocompatibilitycomplex，MHC）的不同是移植排异反应的基础。 | [
{
"id": 0,
"entity": "异体造血干细胞移植",
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"end_offset": 12,
"label": "pro"
},
{
"id": 1,
"entity": "白细胞抗原",
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"end_offset": 27,
"label": "bod"
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{
"id": 2,
"entity": "HLA",
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"label": "bod"
},
{
"id": 3,
"entity": "主要组织相容性复合物",
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},
{
"id": 4,
"entity": "majorhistocompatibilitycomplex",
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"label": "bod"
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{
"id": 5,
"entity": "MHC",
"start_offset": 83,
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"label": "bod"
},
{
"id": 6,
"entity": "移植排异反应",
"start_offset": 91,
"end_offset": 97,
"label": "dis"
}
] |
MHC为一组抗原基因，在人类称为HLA系统，位于第6对染色体的p21区。 | [
{
"id": 0,
"entity": "MHC",
"start_offset": 0,
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{
"id": 1,
"entity": "抗原基因",
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"label": "bod"
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{
"id": 2,
"entity": "HLA系统",
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"label": "bod"
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{
"id": 3,
"entity": "染色体",
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"label": "bod"
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] |
此类基因集中在三个区，即Ⅰ、Ⅱ、Ⅲ类抗原区。 | [
{
"id": 0,
"entity": "基因",
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"end_offset": 4,
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] |
与移植排斥反应相关的主要是第Ⅰ、Ⅱ类MHC抗原。 | [
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"entity": "移植排斥反应",
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{
"id": 1,
"entity": "第Ⅰ、Ⅱ类MHC抗原",
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Ⅰ类MHC抗原包括HLA-A、B、C，近来又发现E、F、G三类。 | [
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"entity": "Ⅰ类MHC抗原",
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{
"id": 1,
"entity": "HLA-A、B、C",
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"label": "bod"
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] |
Ⅱ类MHC抗原包括HLA-D、DR、DQ、DP抗原。 | [
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"id": 0,
"entity": "Ⅱ类MHC抗原",
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{
"id": 1,
"entity": "HLA-D、DR、DQ、DP抗原",
"start_offset": 9,
"end_offset": 25,
"label": "bod"
}
] |
（二）HLA相配几率及移植物抗宿主病发生率1.同基因异体移植同卵双胎间HLA基因完全相配，基本无移植物抗宿主病（graft-versus-hostdisease，GVHD）。 | [
{
"id": 0,
"entity": "HLA",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "抗宿主病",
"start_offset": 14,
"end_offset": 18,
"label": "dis"
},
{
"id": 2,
"entity": "同基因异体移植",
"start_offset": 23,
"end_offset": 30,
"label": "pro"
},
{
"id": 3,
"entity": "HLA基因",
"start_offset": 35,
"end_offset": 40,
"label": "bod"
},
{
"id": 4,
"entity": "抗宿主病",
"start_offset": 51,
"end_offset": 55,
"label": "dis"
},
{
"id": 5,
"entity": "graft-versus-hostdisease",
"start_offset": 56,
"end_offset": 80,
"label": "dis"
},
{
"id": 6,
"entity": "GVHD",
"start_offset": 81,
"end_offset": 85,
"label": "dis"
}
] |
2.同胞HLA相合供者同胞间HLA相合机会为25%，其HLA相合可分为两种情况，即HLA表型相合及HLA基因型相合，其中多数为后者。 | [
{
"id": 0,
"entity": "HLA",
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"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "HLA",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "HLA",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "HLA",
"start_offset": 41,
"end_offset": 44,
"label": "bod"
},
{
"id": 4,
"entity": "HLA基因",
"start_offset": 49,
"end_offset": 54,
"label": "bod"
}
] |
HLA完全相合者，GVHD较轻。 | [
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"id": 0,
"entity": "HLA",
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"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "GVHD",
"start_offset": 9,
"end_offset": 13,
"label": "dis"
}
] |
3.家族HLA不完全相合供者包括1个、2个或3个位点不合，GVHD发病率与不相合位点成比例，不相合位点越多越易移植失败。 | [
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"entity": "HLA",
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{
"id": 1,
"entity": "GVHD",
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"label": "dis"
},
{
"id": 2,
"entity": "移植",
"start_offset": 55,
"end_offset": 57,
"label": "pro"
}
] |
4.无关供者约1万～2万无关供者中可找到1个HLA完全相配者，GVHD发生的严重度一般较同胞相合供体移植重。 | [
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"entity": "HLA",
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{
"id": 1,
"entity": "GVHD",
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"label": "dis"
},
{
"id": 2,
"entity": "移植",
"start_offset": 50,
"end_offset": 52,
"label": "pro"
}
] |
第四章白血病第一节儿童急性淋巴细胞白血病儿童急性淋巴细胞白血病（acutelymphoblasticleukemia，ALL）是最常见的儿童肿瘤性疾病。 | [
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{
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{
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"entity": "儿童急性淋巴细胞白血病",
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{
"id": 3,
"entity": "acutelymphoblasticleukemia",
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"label": "dis"
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{
"id": 4,
"entity": "ALL",
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"label": "dis"
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{
"id": 5,
"entity": "儿童肿瘤性疾病",
"start_offset": 68,
"end_offset": 75,
"label": "dis"
}
] |
近40年来，特别是20世纪80年代以后，对儿童ALL的基础和临床研究取得了巨大的成就，儿童ALL已成为可以治愈的恶性肿瘤，是当今疗效最好、治愈率最高的恶性肿瘤性疾病之一，给其他儿童恶性肿瘤的治疗带来了信心和合理的临床研究模式。 | [
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"entity": "儿童ALL",
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"id": 1,
"entity": "儿童ALL",
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{
"id": 2,
"entity": "恶性肿瘤",
"start_offset": 56,
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"label": "dis"
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{
"id": 3,
"entity": "恶性肿瘤性疾病",
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"label": "dis"
},
{
"id": 4,
"entity": "儿童恶性肿瘤",
"start_offset": 88,
"end_offset": 94,
"label": "dis"
}
] |
目前小儿ALL的完全缓解（CR）率可达95%以上，5年以上持续完全缓解（CCR）率可达65%～80%。 | [
{
"id": 0,
"entity": "小儿ALL",
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"label": "dis"
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] |
【发病率及流行病学】在儿童肿瘤性疾病中，ALL发病率最高，发病高峰年龄约为4岁。 | [
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"entity": "儿童肿瘤性疾病",
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"label": "dis"
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{
"id": 1,
"entity": "ALL",
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"label": "dis"
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] |
根据上海市肿瘤登记系统1986～1992年资料，0～14岁组ALL发病数占儿童肿瘤总发病数的20%，年发病率为20.90/100万，0～14岁累积发病率为312.55/100万，男性发病率高于女性，男∶女为1.5∶1。 | [
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"id": 0,
"entity": "ALL",
"start_offset": 30,
"end_offset": 33,
"label": "dis"
},
{
"id": 1,
"entity": "儿童肿瘤",
"start_offset": 37,
"end_offset": 41,
"label": "dis"
}
] |
【病因学】可能导致发生儿童白血病的因素包括遗传、环境、病毒感染、免疫缺陷因素，但对每一个白血病患儿来说常不能确定其个体的致病原因。 | [
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"id": 0,
"entity": "儿童白血病",
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{
"id": 1,
"entity": "病毒感染",
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"label": "dis"
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{
"id": 2,
"entity": "白血病",
"start_offset": 44,
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"label": "dis"
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] |
（一）环境因素接触电离辐射有利于白血病的发生，第二次世界大战时日本发生原子弹爆炸后，当地白血病发病率增高即证实这一点。 | [
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"entity": "白血病",
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{
"id": 1,
"entity": "白血病",
"start_offset": 44,
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] |
接触治疗性辐射也增加白血病的发病率。 | [
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"entity": "白血病",
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] |
长期接触苯等有毒化学物品与急性非淋巴细胞白血病有关。 | [
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"id": 0,
"entity": "急性非淋巴细胞白血病",
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"end_offset": 23,
"label": "dis"
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] |
其他与ALL发病可能有关的化学物品有除草剂、杀虫剂、孕妇酗酒、避孕药、烟草及化学溶剂，但这些因素与ALL发病的确切关系尚不肯定。 | [
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{
"id": 1,
"entity": "避孕药",
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"end_offset": 34,
"label": "dru"
},
{
"id": 2,
"entity": "ALL",
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"end_offset": 52,
"label": "dis"
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] |
（二）病毒感染EB病毒感染可能与L3</sub>型ALL相关，也有ALL发病与HIV感染相关的病例报告。 | [
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"entity": "病毒感染",
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{
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{
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"entity": "L3</sub>型ALL",
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{
"id": 3,
"entity": "ALL",
"start_offset": 33,
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"label": "dis"
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{
"id": 4,
"entity": "HIV感染",
"start_offset": 39,
"end_offset": 44,
"label": "dis"
}
] |
（三）免疫缺陷先天性免疫缺陷者淋巴系统恶性肿瘤的发病率增高。 | [
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"entity": "先天性免疫缺陷",
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{
"id": 1,
"entity": "淋巴系统恶性肿瘤",
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"label": "dis"
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] |
（四）先天性基因（遗传）因素有报告白血病患儿（包括ALL）同胞的白血病发病率比普通人群高2～4倍。 | [
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{
"id": 1,
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{
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"entity": "白血病",
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] |
单卵双胎中一个发生白血病后，另一个发生白血病的机会高达25%；发病年龄越小，另一个发病的机会越高；当发病年龄＞7岁时，另一个发病的机会明显减少。 | [
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{
"id": 1,
"entity": "白血病",
"start_offset": 19,
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] |
说明白血病的发生可以有先天性遗传因素参与，但确切的基因因素尚未十分明了。 | [
{
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] |
【临床表现】各类型小儿急性白血病临床表现相似，通常表现为进行性苍白、乏力、食欲减退、盗汗、虚弱、低热和出血倾向。 | [
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"entity": "急性白血病",
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{
"id": 1,
"entity": "进行性苍白",
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"label": "sym"
},
{
"id": 2,
"entity": "乏力",
"start_offset": 34,
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{
"id": 3,
"entity": "食欲减退",
"start_offset": 37,
"end_offset": 41,
"label": "sym"
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{
"id": 4,
"entity": "盗汗",
"start_offset": 42,
"end_offset": 44,
"label": "sym"
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{
"id": 5,
"entity": "虚弱",
"start_offset": 45,
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"label": "sym"
},
{
"id": 6,
"entity": "低热和出血倾向",
"start_offset": 48,
"end_offset": 55,
"label": "sym"
}
] |
从起病到诊断可长达数月，也可以骤然起病，以不规则发热、急速的进行性苍白、明显的出血症状和骨关节疼痛等症贫血、出血、发热和白血病细胞对全身各脏器浸润引起的症状贫血常早期出现，轻重不等，表现为苍白、乏力、气促、心悸、颜面水肿等，可进行性加重，贫血和出血程度常不成比例。 | [
{
"id": 0,
"entity": "不规则发热",
"start_offset": 21,
"end_offset": 26,
"label": "sym"
},
{
"id": 1,
"entity": "急速的进行性苍白",
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"label": "sym"
},
{
"id": 2,
"entity": "骨关节",
"start_offset": 44,
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},
{
"id": 3,
"entity": "明显的出血症状和骨关节疼痛等症",
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"label": "sym"
},
{
"id": 4,
"entity": "贫血",
"start_offset": 51,
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"label": "sym"
},
{
"id": 5,
"entity": "出血",
"start_offset": 54,
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"label": "sym"
},
{
"id": 6,
"entity": "白血病细胞",
"start_offset": 60,
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"label": "bod"
},
{
"id": 7,
"entity": "脏器",
"start_offset": 69,
"end_offset": 71,
"label": "bod"
},
{
"id": 8,
"entity": "发热和白血病细胞对全身各脏器浸润引起的症状",
"start_offset": 57,
"end_offset": 78,
"label": "sym"
},
{
"id": 9,
"entity": "贫血",
"start_offset": 78,
"end_offset": 80,
"label": "sym"
},
{
"id": 10,
"entity": "苍白",
"start_offset": 94,
"end_offset": 96,
"label": "sym"
},
{
"id": 11,
"entity": "乏力",
"start_offset": 97,
"end_offset": 99,
"label": "sym"
},
{
"id": 12,
"entity": "气促",
"start_offset": 100,
"end_offset": 102,
"label": "sym"
},
{
"id": 13,
"entity": "心悸",
"start_offset": 103,
"end_offset": 105,
"label": "sym"
},
{
"id": 14,
"entity": "颜面水肿等",
"start_offset": 106,
"end_offset": 111,
"label": "sym"
},
{
"id": 15,
"entity": "贫血",
"start_offset": 119,
"end_offset": 121,
"label": "sym"
},
{
"id": 16,
"entity": "出血",
"start_offset": 122,
"end_offset": 124,
"label": "sym"
}
] |
（二）出血极大部分患儿均有不同程度的皮肤和黏膜出血，表现为皮肤紫癜、乌青和瘀斑，甚至发生皮下血肿。 | [
{
"id": 0,
"entity": "出血",
"start_offset": 3,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "皮肤和黏膜出血",
"start_offset": 18,
"end_offset": 25,
"label": "dis"
},
{
"id": 2,
"entity": "皮肤紫癜",
"start_offset": 29,
"end_offset": 33,
"label": "sym"
},
{
"id": 3,
"entity": "乌青",
"start_offset": 34,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "瘀斑",
"start_offset": 37,
"end_offset": 39,
"label": "sym"
},
{
"id": 5,
"entity": "皮下血肿",
"start_offset": 44,
"end_offset": 48,
"label": "sym"
}
] |
齿龈出血、鼻出血、口腔黏膜渗血，严重者可出现眼底视网膜出血，导致视力减退、颅内压增高。 | [
{
"id": 0,
"entity": "齿龈",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "齿龈出血",
"start_offset": 0,
"end_offset": 4,
"label": "sym"
},
{
"id": 2,
"entity": "鼻",
"start_offset": 5,
"end_offset": 6,
"label": "bod"
},
{
"id": 3,
"entity": "鼻出血",
"start_offset": 5,
"end_offset": 8,
"label": "sym"
},
{
"id": 4,
"entity": "口腔黏膜",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 5,
"entity": "口腔黏膜渗血",
"start_offset": 9,
"end_offset": 15,
"label": "sym"
},
{
"id": 6,
"entity": "视网膜",
"start_offset": 24,
"end_offset": 27,
"label": "bod"
},
{
"id": 7,
"entity": "眼底视网膜出血",
"start_offset": 22,
"end_offset": 29,
"label": "sym"
},
{
"id": 8,
"entity": "视力减退",
"start_offset": 32,
"end_offset": 36,
"label": "sym"
},
{
"id": 9,
"entity": "颅内压增高",
"start_offset": 37,
"end_offset": 42,
"label": "sym"
}
] |
消化道和泌尿道出血，临床表现为便血、呕血和尿血。 | [
{
"id": 0,
"entity": "消化道和泌尿道出血",
"start_offset": 0,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "便血",
"start_offset": 15,
"end_offset": 17,
"label": "sym"
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{
"id": 2,
"entity": "呕血",
"start_offset": 18,
"end_offset": 20,
"label": "sym"
},
{
"id": 3,
"entity": "尿血",
"start_offset": 21,
"end_offset": 23,
"label": "sym"
}
] |
颅内出血时表现为头痛、呕吐、抽搐和昏迷等。 | [
{
"id": 0,
"entity": "颅内出血",
"start_offset": 0,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "头痛",
"start_offset": 8,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "呕吐",
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"end_offset": 13,
"label": "sym"
},
{
"id": 3,
"entity": "抽搐",
"start_offset": 14,
"end_offset": 16,
"label": "sym"
},
{
"id": 4,
"entity": "昏迷",
"start_offset": 17,
"end_offset": 19,
"label": "sym"
}
] |
（三）发热与感染多数患儿起病时有不同程度发热，可为低热、不规则发热、持续高热或弛张热，暂时性热退时常大汗淋漓。 | [
{
"id": 0,
"entity": "发热",
"start_offset": 3,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "感染",
"start_offset": 6,
"end_offset": 8,
"label": "dis"
},
{
"id": 2,
"entity": "发热",
"start_offset": 20,
"end_offset": 22,
"label": "dis"
},
{
"id": 3,
"entity": "低热",
"start_offset": 25,
"end_offset": 27,
"label": "sym"
},
{
"id": 4,
"entity": "不规则发热",
"start_offset": 28,
"end_offset": 33,
"label": "sym"
},
{
"id": 5,
"entity": "持续高热",
"start_offset": 34,
"end_offset": 38,
"label": "sym"
},
{
"id": 6,
"entity": "弛张热",
"start_offset": 39,
"end_offset": 42,
"label": "sym"
},
{
"id": 7,
"entity": "暂时性热退时常大汗淋漓",
"start_offset": 43,
"end_offset": 54,
"label": "sym"
}
] |
发热的原因包括肿瘤性发热和感染性发热，前者用抗生素治疗无效，而用吲哚美辛0.5mg/kg，每8小时口服，热可退净，以此鉴别肿瘤性发热和感染性发热。 | [
{
"id": 0,
"entity": "发热",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "肿瘤性发热",
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"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "感染性发热",
"start_offset": 13,
"end_offset": 18,
"label": "dis"
},
{
"id": 3,
"entity": "抗生素",
"start_offset": 22,
"end_offset": 25,
"label": "dru"
},
{
"id": 4,
"entity": "吲哚美辛",
"start_offset": 32,
"end_offset": 36,
"label": "dru"
},
{
"id": 5,
"entity": "肿瘤性发热",
"start_offset": 61,
"end_offset": 66,
"label": "dis"
},
{
"id": 6,
"entity": "感染性发热",
"start_offset": 67,
"end_offset": 72,
"label": "dis"
}
] |
常见的感染有：呼吸系统感染、败血症、口腔溃疡、皮肤疖肿、肠道感染等，肛周炎也颇为常见。 | [
{
"id": 0,
"entity": "呼吸系统感染",
"start_offset": 7,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "败血症",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "口腔溃疡",
"start_offset": 18,
"end_offset": 22,
"label": "dis"
},
{
"id": 3,
"entity": "皮肤疖肿",
"start_offset": 23,
"end_offset": 27,
"label": "dis"
},
{
"id": 4,
"entity": "肠道感染",
"start_offset": 28,
"end_offset": 32,
"label": "dis"
},
{
"id": 5,
"entity": "肛周炎",
"start_offset": 34,
"end_offset": 37,
"label": "dis"
}
] |
常见的病原菌为大肠埃希菌、铜绿假单胞菌、副大肠杆菌等革兰阴性杆菌、金黄色葡萄球菌和表皮葡萄球菌等革兰阳性球菌，其他还有粪链球菌、克雷白菌、阴沟杆菌、硝酸盐阴性杆菌、黏质沙雷菌、弗氏枸橼酸杆菌等条件致病菌和厌氧菌。 | [
{
"id": 0,
"entity": "大肠埃希菌",
"start_offset": 7,
"end_offset": 12,
"label": "mic"
},
{
"id": 1,
"entity": "铜绿假单胞菌",
"start_offset": 13,
"end_offset": 19,
"label": "mic"
},
{
"id": 2,
"entity": "副大肠杆菌",
"start_offset": 20,
"end_offset": 25,
"label": "mic"
},
{
"id": 3,
"entity": "革兰阴性杆菌",
"start_offset": 26,
"end_offset": 32,
"label": "mic"
},
{
"id": 4,
"entity": "金黄色葡萄球菌",
"start_offset": 33,
"end_offset": 40,
"label": "mic"
},
{
"id": 5,
"entity": "表皮葡萄球菌",
"start_offset": 41,
"end_offset": 47,
"label": "mic"
},
{
"id": 6,
"entity": "革兰阳性球菌",
"start_offset": 48,
"end_offset": 54,
"label": "mic"
},
{
"id": 7,
"entity": "粪链球菌",
"start_offset": 59,
"end_offset": 63,
"label": "mic"
},
{
"id": 8,
"entity": "克雷白菌",
"start_offset": 64,
"end_offset": 68,
"label": "mic"
},
{
"id": 9,
"entity": "阴沟杆菌",
"start_offset": 69,
"end_offset": 73,
"label": "mic"
},
{
"id": 10,
"entity": "硝酸盐阴性杆菌",
"start_offset": 74,
"end_offset": 81,
"label": "mic"
},
{
"id": 11,
"entity": "黏质沙雷菌",
"start_offset": 82,
"end_offset": 87,
"label": "mic"
},
{
"id": 12,
"entity": "弗氏枸橼酸杆菌",
"start_offset": 88,
"end_offset": 95,
"label": "mic"
},
{
"id": 13,
"entity": "致病菌",
"start_offset": 98,
"end_offset": 101,
"label": "mic"
},
{
"id": 14,
"entity": "厌氧菌",
"start_offset": 102,
"end_offset": 105,
"label": "mic"
}
] |
此外可有巨细胞包涵体病毒（CMV）、疱疹病毒、EB病毒感染。 | [
{
"id": 0,
"entity": "巨细胞包涵体病毒",
"start_offset": 4,
"end_offset": 12,
"label": "mic"
},
{
"id": 1,
"entity": "CMV",
"start_offset": 13,
"end_offset": 16,
"label": "mic"
},
{
"id": 2,
"entity": "疱疹病毒",
"start_offset": 18,
"end_offset": 22,
"label": "mic"
},
{
"id": 3,
"entity": "EB病毒",
"start_offset": 23,
"end_offset": 27,
"label": "mic"
}
] |
真菌感染也较常见，有白色念珠菌引起鹅口疮、肛周真菌症、真菌性肠炎和深部真菌感染。 | [
{
"id": 0,
"entity": "真菌感染",
"start_offset": 0,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "白色念珠菌",
"start_offset": 10,
"end_offset": 15,
"label": "mic"
},
{
"id": 2,
"entity": "鹅口疮",
"start_offset": 17,
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"label": "dis"
},
{
"id": 3,
"entity": "肛周真菌症",
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"label": "dis"
},
{
"id": 4,
"entity": "真菌性肠炎",
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"end_offset": 32,
"label": "dis"
},
{
"id": 5,
"entity": "真菌感染",
"start_offset": 35,
"end_offset": 39,
"label": "dis"
}
] |
上述各种感染可单独发生也可混合感染，临床常表现为不规则或弛张性发热。 | [
{
"id": 0,
"entity": "不规则或弛张性发热",
"start_offset": 24,
"end_offset": 33,
"label": "sym"
}
] |
（四）白血病细胞浸润表现ALL易有网状内皮系统的浸润，表现为肝、脾和淋巴结轻至重度肿大。 | [
{
"id": 0,
"entity": "白血病",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "ALL",
"start_offset": 12,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "网状内皮系统",
"start_offset": 17,
"end_offset": 23,
"label": "bod"
},
{
"id": 3,
"entity": "肝",
"start_offset": 30,
"end_offset": 31,
"label": "bod"
},
{
"id": 4,
"entity": "脾",
"start_offset": 32,
"end_offset": 33,
"label": "bod"
},
{
"id": 5,
"entity": "淋巴结",
"start_offset": 34,
"end_offset": 37,
"label": "bod"
},
{
"id": 6,
"entity": "肝、脾和淋巴结轻至重度肿大",
"start_offset": 30,
"end_offset": 43,
"label": "sym"
}
] |
骨关节浸润表现为持续性并阵发性加剧的骨、关节疼痛或肿痛，行动受碍，多见于膝、胫骨、胸骨、踝、肩、腕、肘关节处，易被误诊为风湿关节炎或骨髓炎。 | [
{
"id": 0,
"entity": "骨关节",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "持续性并阵发性加剧的骨、关节疼痛或肿痛",
"start_offset": 8,
"end_offset": 27,
"label": "sym"
},
{
"id": 2,
"entity": "行动受碍",
"start_offset": 28,
"end_offset": 32,
"label": "sym"
},
{
"id": 3,
"entity": "膝",
"start_offset": 36,
"end_offset": 37,
"label": "bod"
},
{
"id": 4,
"entity": "胫骨",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 5,
"entity": "胸骨",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 6,
"entity": "踝",
"start_offset": 44,
"end_offset": 45,
"label": "bod"
},
{
"id": 7,
"entity": "肩",
"start_offset": 46,
"end_offset": 47,
"label": "bod"
},
{
"id": 8,
"entity": "腕",
"start_offset": 48,
"end_offset": 49,
"label": "bod"
},
{
"id": 9,
"entity": "肘关节",
"start_offset": 50,
"end_offset": 53,
"label": "bod"
},
{
"id": 10,
"entity": "风湿关节炎",
"start_offset": 60,
"end_offset": 65,
"label": "dis"
},
{
"id": 11,
"entity": "骨髓炎",
"start_offset": 66,
"end_offset": 69,
"label": "dis"
}
] |
中枢神经系统浸润时，可表现为颅内压增高，有头痛、呕吐、视神经乳头水肿所致视力模糊，也可引起面瘫等脑神经损害症，甚至发生癫痫样发作，意识障碍等。 | [
{
"id": 0,
"entity": "中枢神经系统",
"start_offset": 0,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "颅内压增高",
"start_offset": 14,
"end_offset": 19,
"label": "sym"
},
{
"id": 2,
"entity": "头痛",
"start_offset": 21,
"end_offset": 23,
"label": "sym"
},
{
"id": 3,
"entity": "呕吐",
"start_offset": 24,
"end_offset": 26,
"label": "sym"
},
{
"id": 4,
"entity": "视神经乳头",
"start_offset": 27,
"end_offset": 32,
"label": "bod"
},
{
"id": 5,
"entity": "视神经乳头水肿所致视力模糊",
"start_offset": 27,
"end_offset": 40,
"label": "sym"
},
{
"id": 6,
"entity": "面瘫",
"start_offset": 45,
"end_offset": 47,
"label": "dis"
},
{
"id": 7,
"entity": "脑神经损害症",
"start_offset": 48,
"end_offset": 54,
"label": "dis"
},
{
"id": 8,
"entity": "癫痫样发作",
"start_offset": 59,
"end_offset": 64,
"label": "sym"
},
{
"id": 9,
"entity": "意识障碍",
"start_offset": 65,
"end_offset": 69,
"label": "sym"
}
] |
腮腺浸润时表现为两侧腮腺无痛性增大，质地较硬，无压痛或轻度压痛。 | [
{
"id": 0,
"entity": "腮腺",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "腮腺",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "腮腺无痛性增大",
"start_offset": 10,
"end_offset": 17,
"label": "sym"
},
{
"id": 3,
"entity": "质地较硬",
"start_offset": 18,
"end_offset": 22,
"label": "sym"
},
{
"id": 4,
"entity": "无压痛或轻度压痛",
"start_offset": 23,
"end_offset": 31,
"label": "sym"
}
] |
睾丸浸润时有单侧或双侧睾丸无痛性肿大，质地坚硬，压痛不明显，透光试验呈阴性。 | [
{
"id": 0,
"entity": "睾丸",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "睾丸",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "睾丸无痛性肿大",
"start_offset": 11,
"end_offset": 18,
"label": "sym"
},
{
"id": 3,
"entity": "质地坚硬",
"start_offset": 19,
"end_offset": 23,
"label": "sym"
},
{
"id": 4,
"entity": "压痛不明显",
"start_offset": 24,
"end_offset": 29,
"label": "sym"
},
{
"id": 5,
"entity": "透光试验呈阴性",
"start_offset": 30,
"end_offset": 37,
"label": "sym"
}
] |
ALL时肾浸润并不少见，可因水肿、尿量尿色改变而就诊，有时肾脏明显肿大可在两侧腹部触及，腹部B型超声或CT可见肾脏有多发性浸润灶。 | [
{
"id": 0,
"entity": "ALL",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "肾",
"start_offset": 4,
"end_offset": 5,
"label": "bod"
},
{
"id": 2,
"entity": "水肿",
"start_offset": 14,
"end_offset": 16,
"label": "sym"
},
{
"id": 3,
"entity": "尿量尿色改变",
"start_offset": 17,
"end_offset": 23,
"label": "sym"
},
{
"id": 4,
"entity": "肾脏明显肿大",
"start_offset": 29,
"end_offset": 35,
"label": "sym"
},
{
"id": 5,
"entity": "腹部",
"start_offset": 39,
"end_offset": 41,
"label": "bod"
},
{
"id": 6,
"entity": "腹部B型超声",
"start_offset": 44,
"end_offset": 50,
"label": "equ"
},
{
"id": 7,
"entity": "CT",
"start_offset": 51,
"end_offset": 53,
"label": "equ"
},
{
"id": 8,
"entity": "肾脏",
"start_offset": 55,
"end_offset": 57,
"label": "bod"
}
] |
其他如皮肤、胃肠道、肺、胸膜和心脏浸润时，引起相应脏器功能障碍的症状。 | [
{
"id": 0,
"entity": "皮肤",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "胃肠道",
"start_offset": 6,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "肺",
"start_offset": 10,
"end_offset": 11,
"label": "bod"
},
{
"id": 3,
"entity": "胸膜",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 4,
"entity": "心脏",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
},
{
"id": 5,
"entity": "脏器功能障碍",
"start_offset": 25,
"end_offset": 31,
"label": "sym"
}
] |
【实验室检查及临床分型】（一）外周血象ALL时外周血象红系、髓系和巨核系中常有≥2系的异常变化，多数患儿有贫血和血小板减少。 | [
{
"id": 0,
"entity": "ALL",
"start_offset": 19,
"end_offset": 22,
"label": "dis"
},
{
"id": 1,
"entity": "贫血",
"start_offset": 53,
"end_offset": 55,
"label": "sym"
},
{
"id": 2,
"entity": "血小板减少",
"start_offset": 56,
"end_offset": 61,
"label": "sym"
}
] |
低增生性ALL时白细胞数可很低，外周血象类似再生障碍性贫血，三系均降低，也未见幼稚细胞。 | [
{
"id": 0,
"entity": "ALL",
"start_offset": 4,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "白细胞",
"start_offset": 8,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "再生障碍性贫血",
"start_offset": 22,
"end_offset": 29,
"label": "dis"
},
{
"id": 3,
"entity": "幼稚细胞",
"start_offset": 39,
"end_offset": 43,
"label": "bod"
}
] |
高增生性时可高至数十万，较多患儿外周血中可见到幼稚细胞。 | [
{
"id": 0,
"entity": "外周血",
"start_offset": 16,
"end_offset": 19,
"label": "bod"
},
{
"id": 1,
"entity": "幼稚细胞",
"start_offset": 23,
"end_offset": 27,
"label": "bod"
}
] |
少数患儿可因外周血变化不明显或基本正常而被忽略并延误诊断。 | [
{
"id": 0,
"entity": "外周血",
"start_offset": 6,
"end_offset": 9,
"label": "bod"
}
] |
（二）骨髓象（细胞形态学检查）骨髓涂片是确诊白血病的主要依据。 | [
{
"id": 0,
"entity": "细胞形态学检查",
"start_offset": 7,
"end_offset": 14,
"label": "pro"
},
{
"id": 1,
"entity": "骨髓涂片",
"start_offset": 15,
"end_offset": 19,
"label": "ite"
},
{
"id": 2,
"entity": "白血病",
"start_offset": 22,
"end_offset": 25,
"label": "dis"
}
] |
除了白血病细胞明显增生外，有时可伴有不同程度的骨髓纤维组织增生，此时抽取骨髓液较为困难，称之为“干抽”现象。 | [
{
"id": 0,
"entity": "白血病",
"start_offset": 2,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "骨髓纤维组织",
"start_offset": 23,
"end_offset": 29,
"label": "bod"
},
{
"id": 2,
"entity": "骨髓液",
"start_offset": 36,
"end_offset": 39,
"label": "bod"
}
] |
ALL骨髓涂片组织化学染色的典型表现为糖原呈阳性或强阳性，过氧化物酶阴性，非特异性酯酶呈阴性。 | [
{
"id": 0,
"entity": "ALL",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "骨髓涂片",
"start_offset": 3,
"end_offset": 7,
"label": "ite"
},
{
"id": 2,
"entity": "糖原呈阳性或强阳性",
"start_offset": 19,
"end_offset": 28,
"label": "sym"
},
{
"id": 3,
"entity": "过氧化物酶阴性",
"start_offset": 29,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "非特异性酯酶呈阴性",
"start_offset": 37,
"end_offset": 46,
"label": "sym"
}
] |
根据法国、美国和英国（FAB）形态学分类，ALL分为L1</sub>、L2</sub>、L3</sub>型，90%儿童ALL形态分型为L1</sub>，细胞形态较小；L3</sub>型肿瘤细胞的形态与Burkitt's淋巴瘤细胞相似；L2</sub>细胞大小不均，介于L1</sub>和L3</sub>之间。 | [
{
"id": 0,
"entity": "ALL",
"start_offset": 21,
"end_offset": 24,
"label": "dis"
},
{
"id": 1,
"entity": "ALL",
"start_offset": 59,
"end_offset": 62,
"label": "dis"
},
{
"id": 2,
"entity": "肿瘤细胞",
"start_offset": 92,
"end_offset": 96,
"label": "bod"
},
{
"id": 3,
"entity": "淋巴瘤细胞",
"start_offset": 109,
"end_offset": 114,
"label": "bod"
}
] |
仅依靠骨髓细胞形态学不能鉴别ALL还是非霍奇金淋巴瘤骨髓浸润。 | [
{
"id": 0,
"entity": "骨髓细胞",
"start_offset": 3,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "ALL",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "非霍奇金淋巴瘤",
"start_offset": 19,
"end_offset": 26,
"label": "dis"
}
] |
（三）免疫分型根据单克隆抗体（McAb）对白血病细胞表面分化抗原、胞质免疫球蛋白链的反应，可将ALL分为T、B二大系列。 | [
{
"id": 0,
"entity": "抗体",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 1,
"entity": "McAb",
"start_offset": 15,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "白血病",
"start_offset": 21,
"end_offset": 24,
"label": "dis"
},
{
"id": 3,
"entity": "胞质免疫球蛋白链",
"start_offset": 33,
"end_offset": 41,
"label": "bod"
}
] |
1.T系淋巴细胞型（T-ALL）约占儿童ALL10%～15%，常表达T淋巴细胞分化抗原标志，如CD1</sub>、CD2</sub>、CD3</sub>、CD4</sub>、CD5</sub>、CD7</sub>、CD8</sub>以及TdT等。 | [
{
"id": 0,
"entity": "T系淋巴细胞型",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "T-ALL",
"start_offset": 10,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "T淋巴细胞",
"start_offset": 34,
"end_offset": 39,
"label": "bod"
},
{
"id": 3,
"entity": "抗原",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 4,
"entity": "CD1",
"start_offset": 47,
"end_offset": 50,
"label": "bod"
},
{
"id": 5,
"entity": "CD2",
"start_offset": 57,
"end_offset": 60,
"label": "bod"
},
{
"id": 6,
"entity": "CD3",
"start_offset": 67,
"end_offset": 70,
"label": "bod"
},
{
"id": 7,
"entity": "CD4",
"start_offset": 77,
"end_offset": 80,
"label": "bod"
},
{
"id": 8,
"entity": "CD5",
"start_offset": 87,
"end_offset": 90,
"label": "bod"
},
{
"id": 9,
"entity": "CD7",
"start_offset": 97,
"end_offset": 100,
"label": "bod"
},
{
"id": 10,
"entity": "CD8",
"start_offset": 107,
"end_offset": 110,
"label": "bod"
},
{
"id": 11,
"entity": "TdT",
"start_offset": 118,
"end_offset": 121,
"label": "bod"
}
] |
临床上可伴有纵隔增宽、外周血白细胞计数高。 | [
{
"id": 0,
"entity": "纵隔增宽",
"start_offset": 6,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "外周血白细胞计数高",
"start_offset": 11,
"end_offset": 20,
"label": "sym"
}
] |
2.B系淋巴细胞型（B-ALL）约占儿童ALL80%～90%，可大致分为未成熟型（以发育成熟过程依次包括B淋巴祖细胞性、早期前B淋巴细胞性和前B淋巴细胞型）和相对成熟型。 | [
{
"id": 0,
"entity": "B系淋巴细胞型",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "B-ALL",
"start_offset": 10,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "儿童ALL",
"start_offset": 18,
"end_offset": 23,
"label": "dis"
}
] |
胞质免疫球蛋白（CyIg）从无到有，继之细胞膜表面免疫球蛋白（SmIg）的出现反映了B细胞向成熟方向发育的过程。 | [
{
"id": 0,
"entity": "胞质免疫球蛋白",
"start_offset": 0,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "CyIg",
"start_offset": 8,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "细胞膜表面免疫球蛋白",
"start_offset": 20,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "SmIg",
"start_offset": 31,
"end_offset": 35,
"label": "bod"
},
{
"id": 4,
"entity": "B细胞",
"start_offset": 42,
"end_offset": 45,
"label": "bod"
}
] |
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