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stringlengths 4
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| entities
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|---|---|
EPO主要在肾脏活化。
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红细胞增加可以提高携氧能力，代偿组织缺氧。
|
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但在急性呼吸衰竭时，低氧对骨髓的抑制，使EPO的作用产生缓慢或不起作用。
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（二）二氧化碳潴留及其对机体的影响1.中枢对二氧化碳的调节敏感性和反应性动脉血二氧化碳分压变化通过延髓和颈动脉体化学感受器影响呼吸运动强弱和通气量。
|
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"entity": "颈动脉",
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"entity": "体化学感受器",
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二氧化碳透过血-脑屏障，进入脑脊液，解离出氢离子，刺激感受器。
|
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反应机制中颈动脉体的作用占1/3，反应快；延脑作用占2/3，作用较持续。
|
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当二氧化碳进一步升高时，可以抑制大脑皮质下层，出现嗜睡和呼吸抑制。
|
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不同个体和不同疾病状况下，外周和中枢化学感受器对于二氧化碳的不同程度变化，其敏感性和反应性不同。
|
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对于呼吸衰竭出现兴奋烦躁、与呼吸机对抗者，可以通过应用镇静剂，以降低中枢对二氧化碳反应的敏感性，但仍然可以维持中枢对二氧化碳的调节反应性。
|
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呼吸肌长期负担过重而导致动力性衰竭，或长期二氧化碳潴留，可以出现敏感性和反应性同时下降。
|
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过多使用肌松剂导致呼吸肌失用性变性变性等，也可以导致对二氧化碳呼吸调节作用的下降。
|
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2.二氧化碳潴留对脏器功能的影响（1）呼吸系统：机体二氧化碳代谢特点表现为由组织-循环血-肺泡的二氧化碳分压递降。
|
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"entity": "循环血",
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"entity": "肺泡",
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呼吸衰竭时可因肺通气障碍，导致肺泡内二氧化碳排出困难，在通气不足时，组织、循环和肺泡内二氧化碳潴留，而呼出气二氧化碳分压降低。
|
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当通气改善而肺血流灌注不良时，出现组织和循环二氧化碳潴留，肺泡内气和呼出气二氧化碳分压降低。
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当通气-灌流和肺换气功能改善，但外周循环没有改善，则循环血和呼出气二氧化碳分压逐渐提高，而且差别减小；当外周循环改善后，会出现循环血和呼出气二氧化碳分压增高的阶段，然后随组织二氧化碳潴留的解除，循环血和呼出气二氧化碳分压水平恢复到正常。
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二氧化碳增加可以使肺血管收缩，肺血流量下降。
|
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（2）中枢神经系统：正常人脑循环对于二氧化碳敏感。
|
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当吸入气含CO<sub>2</sub>5%时，或PaCO<sub>2</sub>提高1～2kPa时，脑血流量可提高40%～50%，可以出现颅内压上升，致头痛、视神经乳头水肿、肌张力增高、瞳孔变化等症状和体征。
|
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颅高压严重者可以发展为脑疝，延髓受压迫后中枢呼吸停止而死亡。
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（3）心血管系统：二氧化碳分压升高可以使心率、血压、心输出量反射性增加。
|
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如果二氧化碳上升过高，可以出现心率、血压、心输出量降低，出现心律不齐，外周血管扩张症状。
|
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（4）肾脏：二氧化碳潴留存在轻度酸中毒时，肾血管血流增加，促进肾脏排尿作用。
|
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当呼吸性酸中毒失代偿时，pH显著下降，肾血管痉挛、血流减少，尿量和钠离子排出量亦显著减少。
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3.酸碱失衡和电解质紊乱二氧化碳潴留可以导致呼吸性酸中毒，并出现一系列电解质紊乱。
|
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（1）碳酸氢根/碳酸对酸中毒的调节失代偿：组织生成的CO<sub>2</sub>主要在红细胞和肾小管上皮细胞内，经碳酸酐酶催化生成碳酸，碳酸迅速解离成H<sup>+</sup>和离子。
|
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血液中5%的CO<sub>2</sub>溶解在血浆中，95%的CO<sub>2</sub>进入红细胞，而大部分的逸出红细胞外，相应的Cl<sup>-</sup>进入红细胞内（氯移现象），伴随血清氯离子减少。
|
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血浆中CO<sub>2</sub>总量包括溶解的CO<sub>2</sub>和。
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呼吸性酸中毒主要为PaCO<sub>2</sub>变化（升高）。
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当呼吸性酸中毒合并代谢性酸中毒时，可以出现的下降，还可出现严重电解质紊乱和阴离子间隙升高。
|
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（2）氢-钾交换：细胞内酸中毒时大量氢离子产生，细胞内钾离子和细胞外液中氢离子交换（3个钾离子与2个钠离子、1个氢离子交换），可以导致细胞内酸中毒和低钾。
|
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此外，远端肾小管氢-钾交换加强，随氢离子大量排出，血清钾水平升高。
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呼吸性酸中毒时血清钾离子水平与pH呈负相关，pH越低，血钾越高。
|
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理论上血浆阳离子总数和阴离子总数相等，Na<sup>+</sup>+UC=Cl<sup>-</sup>++UA，对上式移项得到：UA-UC=Na<sup>+</sup>-（Cl<sup>-</sup>+），正常范围为8～16mmol/L，平均12mmol/L。
|
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呼吸衰竭时，由于组织缺氧，组织无氧代谢-糖酵解增强，可以出现乳酸增高伴酸中毒，为乳酸性酸中毒，常见于呼吸性酸中毒时，可以通过测定血乳酸、AG加以诊断。
|
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{
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"entity": "乳酸",
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"entity": "血乳酸",
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"label": "ite"
},
{
"id": 7,
"entity": "AG",
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] |
尤其当治疗过程中补碱液、机体代偿使增加并恢复到正常范围，但酸中毒仍然存在时，如果AG增高，仍可以判断有代谢性紊乱。
|
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"entity": "代谢性紊乱",
"start_offset": 51,
"end_offset": 56,
"label": "dis"
}
] |
但危重呼吸衰竭时，应根据临床病情和处理综合判断。
|
[
{
"id": 0,
"entity": "危重呼吸衰竭",
"start_offset": 1,
"end_offset": 7,
"label": "dis"
}
] |
三、出血性疾病的鉴别诊断（一）血管止血功能障碍引起的出血性疾病1.遗传性出血性毛细血管扩张症同一患者反复同一部位的出血，儿童以鼻出血、牙龈出血最为常见，成人还可见胃肠道、泌尿道等内脏出血。
|
[
{
"id": 0,
"entity": "出血性疾病",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "血管止血功能障碍引起的出血性疾病",
"start_offset": 16,
"end_offset": 32,
"label": "dis"
},
{
"id": 2,
"entity": "遗传性出血性毛细血管扩张症",
"start_offset": 34,
"end_offset": 47,
"label": "dis"
},
{
"id": 3,
"entity": "出血",
"start_offset": 58,
"end_offset": 60,
"label": "sym"
},
{
"id": 4,
"entity": "出血",
"start_offset": 65,
"end_offset": 67,
"label": "sym"
},
{
"id": 5,
"entity": "胃肠道",
"start_offset": 82,
"end_offset": 85,
"label": "bod"
},
{
"id": 6,
"entity": "泌尿道",
"start_offset": 86,
"end_offset": 89,
"label": "bod"
},
{
"id": 7,
"entity": "内脏",
"start_offset": 90,
"end_offset": 92,
"label": "bod"
}
] |
体格检查可见口腔、鼻黏膜、甲床和手掌等部位的毛细血管扭曲扩张，呈细点状或结节状；全身皮肤、黏膜并无瘀点、瘀斑。
|
[
{
"id": 0,
"entity": "口腔",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "鼻黏膜",
"start_offset": 9,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "甲床",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 3,
"entity": "手掌",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
},
{
"id": 4,
"entity": "毛细血管",
"start_offset": 22,
"end_offset": 26,
"label": "bod"
},
{
"id": 5,
"entity": "皮肤",
"start_offset": 42,
"end_offset": 44,
"label": "bod"
},
{
"id": 6,
"entity": "黏膜",
"start_offset": 45,
"end_offset": 47,
"label": "bod"
}
] |
出血时间延长、束臂试验阳性，毛细血管镜下可见扩张的毛细血管。
|
[
{
"id": 0,
"entity": "毛细血管镜",
"start_offset": 14,
"end_offset": 19,
"label": "equ"
},
{
"id": 1,
"entity": "毛细血管",
"start_offset": 25,
"end_offset": 29,
"label": "bod"
}
] |
共济失调毛细血管扩张症也可见眼结合膜和皮肤的毛细血管扩张，但一般很少出血，常有反复呼吸道感染、进行性共济失调和联合免疫缺陷，易与遗传性出血性毛细血管扩张症鉴别。
|
[
{
"id": 0,
"entity": "眼结合膜",
"start_offset": 14,
"end_offset": 18,
"label": "bod"
},
{
"id": 1,
"entity": "皮肤",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
},
{
"id": 2,
"entity": "毛细血管",
"start_offset": 22,
"end_offset": 26,
"label": "bod"
},
{
"id": 3,
"entity": "呼吸道",
"start_offset": 41,
"end_offset": 44,
"label": "bod"
},
{
"id": 4,
"entity": "呼吸道感染",
"start_offset": 41,
"end_offset": 46,
"label": "sym"
},
{
"id": 5,
"entity": "进行性共济失调",
"start_offset": 47,
"end_offset": 54,
"label": "sym"
},
{
"id": 6,
"entity": "联合免疫缺陷",
"start_offset": 55,
"end_offset": 61,
"label": "sym"
},
{
"id": 7,
"entity": "遗传性出血性毛细血管扩张症",
"start_offset": 64,
"end_offset": 77,
"label": "dis"
}
] |
2.过敏性紫癜四肢及臂部反复出现高于皮面、大小不等的紫癜，呈对称性分布，可同时出现血尿、胃肠道出血。
|
[
{
"id": 0,
"entity": "过敏性紫癜",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
}
] |
半数以上病人束臂试验阳性，而出凝血时间、血小板计数、血块退缩试验等均正常。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 20,
"end_offset": 23,
"label": "bod"
}
] |
不典型的过敏性紫癜应与ITP、风湿性关节炎、肾炎及急腹症等相鉴别。
|
[
{
"id": 0,
"entity": "风湿性关节炎",
"start_offset": 15,
"end_offset": 21,
"label": "dis"
},
{
"id": 1,
"entity": "肾炎",
"start_offset": 22,
"end_offset": 24,
"label": "dis"
},
{
"id": 2,
"entity": "急腹症",
"start_offset": 25,
"end_offset": 28,
"label": "dis"
}
] |
3.单纯性紫癜多见于青春期女孩，月经期发作加重。
|
[
{
"id": 0,
"entity": "单纯性紫癜",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
}
] |
4.维生素C缺乏症维生素C不仅是体内强有力的还原剂，还参加体内多种物质如胶原纤维及细胞间粘合质等的合成，缺乏时引起出血和骨骼改变。
|
[
{
"id": 0,
"entity": "维生素C缺乏症",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "维生素C",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "出血",
"start_offset": 57,
"end_offset": 59,
"label": "sym"
},
{
"id": 3,
"entity": "骨骼",
"start_offset": 60,
"end_offset": 62,
"label": "bod"
}
] |
骨骼改变可见骨膜下出血，干骺脱位、分离或骨骺嵌入，膝、踝关节肿胀血浆维生素C含量＜4mg/L，四肢长骨远端X线检查有较特异改变。
|
[
{
"id": 0,
"entity": "骨骼",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "骨膜",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "出血",
"start_offset": 9,
"end_offset": 11,
"label": "sym"
},
{
"id": 3,
"entity": "骨骺",
"start_offset": 20,
"end_offset": 22,
"label": "bod"
},
{
"id": 4,
"entity": "膝",
"start_offset": 25,
"end_offset": 26,
"label": "bod"
},
{
"id": 5,
"entity": "踝关节",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 6,
"entity": "骨骺嵌入，膝、踝关节肿胀",
"start_offset": 20,
"end_offset": 32,
"label": "sym"
},
{
"id": 7,
"entity": "血浆",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 8,
"entity": "维生素C",
"start_offset": 34,
"end_offset": 38,
"label": "bod"
},
{
"id": 9,
"entity": "长骨",
"start_offset": 49,
"end_offset": 51,
"label": "bod"
}
] |
5.爱-唐（Ehlers-Danlos）综合征本病系常染色体显性遗传，因结缔组织中胶原纤维数量不足和质的异常，弹力纤维相对增多所致。
|
[
{
"id": 0,
"entity": "爱-唐（Ehlers-Danlos）综合征",
"start_offset": 2,
"end_offset": 23,
"label": "dis"
},
{
"id": 1,
"entity": "结缔组织",
"start_offset": 36,
"end_offset": 40,
"label": "bod"
}
] |
临床表现为皮肤弹性过度，牵拉后弹回原位时会伴以“拍击声”；关节过度伸展、行走困难；血管脆性增高，轻微外伤可致出血，如瘀斑、血肿等，后者钙化后X线检查时可见钙化小肿瘤；受累者可表现蓝色巩膜、血肿、斜视和视网膜血管纹等。
|
[
{
"id": 0,
"entity": "瘀斑",
"start_offset": 58,
"end_offset": 60,
"label": "sym"
},
{
"id": 1,
"entity": "血肿",
"start_offset": 61,
"end_offset": 63,
"label": "sym"
},
{
"id": 2,
"entity": "钙化小肿瘤",
"start_offset": 77,
"end_offset": 82,
"label": "sym"
},
{
"id": 3,
"entity": "蓝色巩膜",
"start_offset": 89,
"end_offset": 93,
"label": "sym"
},
{
"id": 4,
"entity": "血肿",
"start_offset": 94,
"end_offset": 96,
"label": "sym"
},
{
"id": 5,
"entity": "斜视",
"start_offset": 97,
"end_offset": 99,
"label": "sym"
},
{
"id": 6,
"entity": "视网膜",
"start_offset": 100,
"end_offset": 103,
"label": "bod"
},
{
"id": 7,
"entity": "视网膜血管纹",
"start_offset": 100,
"end_offset": 106,
"label": "sym"
}
] |
（二）血小板止血功能障碍引起的出血性疾病1.特发性血小板减少性紫癜ITP是一种自身免疫性疾病，约2/3的患者循环中存在以IgG为主的抗血小板抗体，ITP在起病前1～3周多有病毒感染史。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "出血性疾病",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "特发性血小板减少性紫癜",
"start_offset": 22,
"end_offset": 33,
"label": "dis"
},
{
"id": 3,
"entity": "ITP",
"start_offset": 33,
"end_offset": 36,
"label": "dis"
},
{
"id": 4,
"entity": "IgG",
"start_offset": 60,
"end_offset": 63,
"label": "bod"
},
{
"id": 5,
"entity": "抗血小板抗体",
"start_offset": 66,
"end_offset": 72,
"label": "bod"
},
{
"id": 6,
"entity": "ITP",
"start_offset": 73,
"end_offset": 76,
"label": "bod"
}
] |
临床以皮肤、黏膜的自发性出血为主，重者亦可有器官内出血。
|
[
{
"id": 0,
"entity": "皮肤",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "黏膜",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "自发性出血",
"start_offset": 9,
"end_offset": 14,
"label": "sym"
},
{
"id": 3,
"entity": "出血",
"start_offset": 25,
"end_offset": 27,
"label": "sym"
}
] |
实验室检查可见血小板计数减少，骨髓中巨核细胞数增多或正常，成熟障碍，血液中PAIgG增多或PAC3</sub>增多。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "骨髓",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "巨核细胞",
"start_offset": 18,
"end_offset": 22,
"label": "bod"
},
{
"id": 3,
"entity": "血液",
"start_offset": 34,
"end_offset": 36,
"label": "bod"
},
{
"id": 4,
"entity": "PAIgG",
"start_offset": 37,
"end_offset": 42,
"label": "bod"
}
] |
本病与继发性血小板减少性紫癜的鉴别，急慢性ITP型别的鉴别等请参见本章第一节内容。
|
[
{
"id": 0,
"entity": "继发性血小板减少性紫癜",
"start_offset": 3,
"end_offset": 14,
"label": "dis"
}
] |
2.血栓性血小板减少性紫癜（thromboticthrombocytopenicpurpura，TTP）TTP在儿童少见，主要因为微小血管内皮细胞增生、损伤造成微血栓形成并阻塞管腔所致。
|
[
{
"id": 0,
"entity": "血栓性血小板减少性紫癜",
"start_offset": 2,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "thromboticthrombocytopenicpurpura",
"start_offset": 14,
"end_offset": 47,
"label": "dis"
},
{
"id": 2,
"entity": "TTP",
"start_offset": 48,
"end_offset": 51,
"label": "dis"
},
{
"id": 3,
"entity": "TTP",
"start_offset": 52,
"end_offset": 55,
"label": "dis"
},
{
"id": 4,
"entity": "血管",
"start_offset": 67,
"end_offset": 69,
"label": "bod"
}
] |
临床病变广泛，主要表现为血小板减少性出血，溶血性贫血和神经系统受累，通常发病急骤并有高热，组织病理学检查可辅助诊断。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
}
] |
TTP发病机制尚未完全清楚，临床表现与实验室检查酷似Evans综合征，溶血尿毒综合征和DIC，更有人认为溶血尿毒综合征与TTP实际上是一种疾病的两种型别。
|
[
{
"id": 0,
"entity": "TTP",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "Evans综合征",
"start_offset": 26,
"end_offset": 34,
"label": "dis"
},
{
"id": 2,
"entity": "溶血尿毒综合征",
"start_offset": 35,
"end_offset": 42,
"label": "dis"
},
{
"id": 3,
"entity": "DIC",
"start_offset": 43,
"end_offset": 46,
"label": "dis"
},
{
"id": 4,
"entity": "溶血尿毒综合征",
"start_offset": 52,
"end_offset": 59,
"label": "dis"
},
{
"id": 5,
"entity": "TTP",
"start_offset": 60,
"end_offset": 63,
"label": "dis"
}
] |
3.原发性血小板增多症临床上血小板持续增多达600×109</sup>/L以上，伴反复自发性皮肤、黏膜出血、血栓形成和脾大为特征。
|
[
{
"id": 0,
"entity": "原发性血小板增多症",
"start_offset": 2,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
}
] |
4.血管瘤-血小板减少综合征先天性的皮肤或内脏海绵状血管瘤会随年龄增长而增大，血小板则相应减少。
|
[
{
"id": 0,
"entity": "血管瘤-血小板减少综合征",
"start_offset": 2,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "皮肤",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "内脏",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
},
{
"id": 3,
"entity": "血管瘤",
"start_offset": 26,
"end_offset": 29,
"label": "sym"
},
{
"id": 4,
"entity": "血小板",
"start_offset": 39,
"end_offset": 42,
"label": "bod"
}
] |
究其原因可能系血小板在血管中凝聚或形成血小板栓子所致。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "血管",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "血小板",
"start_offset": 19,
"end_offset": 22,
"label": "bod"
}
] |
病儿可在生后数周内也可致成年后发生皮肤、黏膜或内脏的出血，重者可致DIC甚至死亡。
|
[
{
"id": 0,
"entity": "皮肤",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 1,
"entity": "DIC",
"start_offset": 33,
"end_offset": 36,
"label": "dis"
}
] |
5.周期性血小板减少症血小板呈周期性减少伴出血，通常10～25天为一周期，重者可出血致死。
|
[
{
"id": 0,
"entity": "周期性血小板减少症",
"start_offset": 2,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 11,
"end_offset": 14,
"label": "bod"
}
] |
本症病因未明，临床可见有随月经周期变化的，有巨核细胞本身周期性减少、消失和伴成熟障碍的，也有血小板减少时骨髓巨核细胞反而增多等亚型。
|
[
{
"id": 0,
"entity": "巨核细胞",
"start_offset": 22,
"end_offset": 26,
"label": "bod"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 46,
"end_offset": 49,
"label": "bod"
},
{
"id": 2,
"entity": "骨髓",
"start_offset": 52,
"end_offset": 54,
"label": "bod"
},
{
"id": 3,
"entity": "巨核细胞",
"start_offset": 54,
"end_offset": 58,
"label": "bod"
}
] |
6.先天性血小板功能障碍性疾病本组疾病病情请参见本章第二节有关内容，注意与获得性血小板功能障碍性疾病相鉴别。
|
[
{
"id": 0,
"entity": "先天性血小板功能障碍性疾病",
"start_offset": 2,
"end_offset": 15,
"label": "dis"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 40,
"end_offset": 43,
"label": "bod"
}
] |
（三）凝血障碍引起的出血性疾病1.血友病凝血障碍性出血性疾病约占我国小儿出血性疾病的12%，其中血友病甲约占70%～80%，血友病乙约占10%～15%，血友病丙仅占1%～2%，前二者为性联隐性遗传，后者为不完全性常染色体隐性遗传。
|
[
{
"id": 0,
"entity": "凝血障碍引起的出血性疾病",
"start_offset": 3,
"end_offset": 15,
"label": "dis"
},
{
"id": 1,
"entity": "血友病",
"start_offset": 17,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "凝血障碍性出血性疾病",
"start_offset": 20,
"end_offset": 30,
"label": "dis"
},
{
"id": 3,
"entity": "血友病甲",
"start_offset": 48,
"end_offset": 52,
"label": "dis"
},
{
"id": 4,
"entity": "血友病乙",
"start_offset": 62,
"end_offset": 66,
"label": "dis"
},
{
"id": 5,
"entity": "血友病丙",
"start_offset": 76,
"end_offset": 80,
"label": "dis"
}
] |
血友病的特点是轻微损伤后出血不止的倾向，重者常有软组织的深部血肿和关节腔出血APTT多延长，PT及TT正常，APTT纠正试验及凝血因子活性测定有助诊断。
|
[
{
"id": 0,
"entity": "轻微损伤后出血不止的倾向",
"start_offset": 7,
"end_offset": 19,
"label": "sym"
},
{
"id": 1,
"entity": "软组织",
"start_offset": 24,
"end_offset": 27,
"label": "bod"
},
{
"id": 2,
"entity": "关节腔",
"start_offset": 33,
"end_offset": 36,
"label": "bod"
},
{
"id": 3,
"entity": "软组织的深部血肿和关节腔出血",
"start_offset": 24,
"end_offset": 38,
"label": "sym"
},
{
"id": 4,
"entity": "APTT",
"start_offset": 38,
"end_offset": 42,
"label": "ite"
},
{
"id": 5,
"entity": "PT",
"start_offset": 46,
"end_offset": 48,
"label": "ite"
},
{
"id": 6,
"entity": "APTT",
"start_offset": 54,
"end_offset": 58,
"label": "ite"
},
{
"id": 7,
"entity": "凝血因子",
"start_offset": 63,
"end_offset": 67,
"label": "bod"
}
] |
2.血管性血友病本病为常染色体显性遗传，少数为隐性遗传。
|
[
{
"id": 0,
"entity": "血管性血友病",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
}
] |
因子Ⅷ是由低分子量的因子Ⅷ∶C和高分子量的vWF组成的蛋白复合物，因为vWF合成障碍导致vWF缺乏或分子结构异常是本病的基本缺陷，继而引起血小板对损伤血管壁黏附功能障碍，对瑞斯托霉素诱导的聚集反应降低和Ⅷ∶C降低。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 69,
"end_offset": 72,
"label": "bod"
},
{
"id": 1,
"entity": "血管壁",
"start_offset": 75,
"end_offset": 78,
"label": "bod"
},
{
"id": 2,
"entity": "瑞斯托霉素",
"start_offset": 86,
"end_offset": 91,
"label": "dru"
}
] |
vWD以皮肤黏膜出血为多见，重者可有内脏出血及手术时异常出血，其中肌肉及关节出血少见。
|
[
{
"id": 0,
"entity": "皮肤",
"start_offset": 4,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "黏膜",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "肌肉",
"start_offset": 33,
"end_offset": 35,
"label": "bod"
}
] |
3.维生素K缺乏症凝血因子由前体蛋白转变成具有生物活性的过程中需维生素K参与。
|
[
{
"id": 0,
"entity": "维生素K缺乏症",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "凝血因子",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "维生素K",
"start_offset": 32,
"end_offset": 36,
"label": "bod"
}
] |
新生儿及小婴儿时有发病，前者系孕母维生素K通过胎盘少，或用过影响维生素K代谢药物，患儿吸收减少所致。
|
[
{
"id": 0,
"entity": "维生素K",
"start_offset": 17,
"end_offset": 21,
"label": "bod"
},
{
"id": 1,
"entity": "胎盘",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 2,
"entity": "维生素K",
"start_offset": 32,
"end_offset": 36,
"label": "bod"
}
] |
实验室检查凝血时间（CT）轻度延长或正常，凝血酶原时间延长，血小板正常即可确诊。
|
[
{
"id": 0,
"entity": "凝血酶原",
"start_offset": 21,
"end_offset": 25,
"label": "bod"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
}
] |
4.先天性纤维蛋白原缺乏症本病为常染色体隐性或不完全隐性遗传，临床罕见。
|
[
{
"id": 0,
"entity": "先天性纤维蛋白原缺乏症",
"start_offset": 2,
"end_offset": 13,
"label": "dis"
}
] |
杂合子多无出血，纯合子可有出血表现，其中APTT、PT、TT均延长、血浆纤维蛋白原量显著减少；成年人中须注意与获得性纤维蛋白原缺乏症相鉴别。
|
[
{
"id": 0,
"entity": "APTT",
"start_offset": 20,
"end_offset": 24,
"label": "ite"
},
{
"id": 1,
"entity": "PT",
"start_offset": 25,
"end_offset": 27,
"label": "ite"
},
{
"id": 2,
"entity": "TT",
"start_offset": 28,
"end_offset": 30,
"label": "ite"
},
{
"id": 3,
"entity": "血浆纤维蛋白",
"start_offset": 34,
"end_offset": 40,
"label": "bod"
},
{
"id": 4,
"entity": "获得性纤维蛋白原缺乏症",
"start_offset": 55,
"end_offset": 66,
"label": "dis"
}
] |
5.抗凝物质过多血友病甲反复应用因子Ⅷ制剂后，约6%～9%病例可产生抗体，在自身免疫性疾病、淋巴瘤、药物反应等中也可出现因子Ⅷ抗体。
|
[
{
"id": 0,
"entity": "血友病甲",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "因子Ⅷ制剂",
"start_offset": 16,
"end_offset": 21,
"label": "dru"
},
{
"id": 2,
"entity": "抗体",
"start_offset": 34,
"end_offset": 36,
"label": "bod"
},
{
"id": 3,
"entity": "自身免疫性疾病",
"start_offset": 38,
"end_offset": 45,
"label": "dis"
},
{
"id": 4,
"entity": "淋巴瘤",
"start_offset": 46,
"end_offset": 49,
"label": "dis"
},
{
"id": 5,
"entity": "因子Ⅷ抗体",
"start_offset": 60,
"end_offset": 65,
"label": "bod"
}
] |
抗原与抗体结合的结果，抑制了因子Ⅷ活性，临床表现与实验室检查结果均类似甲型血友病。
|
[
{
"id": 0,
"entity": "抗体",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "因子Ⅷ",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "甲型血友病",
"start_offset": 35,
"end_offset": 40,
"label": "dis"
}
] |
肝功能衰竭、放射病、恶性肿瘤或肝素使用过量等可引起循环中肝素或类肝素物质增多，抑制Ⅴ、Ⅷ、Ⅹ、Ⅻ和对抗凝血酶、结果CT显著延长，PT中度延长，使用鱼精蛋白或甲苯胺蓝可纠正CT延长即能诊断。
|
[
{
"id": 0,
"entity": "肝功能衰竭",
"start_offset": 0,
"end_offset": 5,
"label": "sym"
},
{
"id": 1,
"entity": "放射病",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 2,
"entity": "恶性肿瘤",
"start_offset": 10,
"end_offset": 14,
"label": "dis"
},
{
"id": 3,
"entity": "肝素",
"start_offset": 15,
"end_offset": 17,
"label": "dru"
},
{
"id": 4,
"entity": "肝素",
"start_offset": 28,
"end_offset": 30,
"label": "dru"
},
{
"id": 5,
"entity": "类肝素",
"start_offset": 31,
"end_offset": 34,
"label": "dru"
},
{
"id": 6,
"entity": "凝血酶",
"start_offset": 51,
"end_offset": 54,
"label": "bod"
},
{
"id": 7,
"entity": "CT",
"start_offset": 57,
"end_offset": 59,
"label": "ite"
},
{
"id": 8,
"entity": "PT",
"start_offset": 64,
"end_offset": 66,
"label": "ite"
},
{
"id": 9,
"entity": "鱼精蛋白",
"start_offset": 73,
"end_offset": 77,
"label": "dru"
},
{
"id": 10,
"entity": "甲苯胺蓝",
"start_offset": 78,
"end_offset": 82,
"label": "dru"
},
{
"id": 11,
"entity": "CT",
"start_offset": 85,
"end_offset": 87,
"label": "bod"
}
] |
6.DIC本症是多种因素引起的一组严重的出血综合征。
|
[
{
"id": 0,
"entity": "出血综合征",
"start_offset": 20,
"end_offset": 25,
"label": "dis"
}
] |
实验室检查发现有下列三项异常者可确定诊断：①血小板＜100×109</sup>/L或进行性下降；②血浆纤维蛋白原含量＜1.5g/L或进行性下降；③3P试验阳性或FDP＞20mg/L；或血浆D-二聚体水平升高；④PT缩短或延长3秒以上或呈动态变化；⑤外周血破碎红细胞＞2%。
|
[
{
"id": 0,
"entity": "血小板",
"start_offset": 22,
"end_offset": 25,
"label": "ite"
},
{
"id": 1,
"entity": "血浆纤维蛋白原",
"start_offset": 49,
"end_offset": 56,
"label": "ite"
},
{
"id": 2,
"entity": "3P试验",
"start_offset": 73,
"end_offset": 77,
"label": "pro"
},
{
"id": 3,
"entity": "PT",
"start_offset": 105,
"end_offset": 107,
"label": "ite"
},
{
"id": 4,
"entity": "外周血破碎红细胞",
"start_offset": 124,
"end_offset": 132,
"label": "ite"
}
] |
此外纤溶酶原和AT-Ⅲ的含量及活性降低，因子Ⅷ∶C活性＜50%等有助于疑难病例的诊断。
|
[
{
"id": 0,
"entity": "纤溶酶原",
"start_offset": 2,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "AT-Ⅲ",
"start_offset": 7,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "因子Ⅷ∶C",
"start_offset": 20,
"end_offset": 25,
"label": "bod"
}
] |
第三章儿童恶性肿瘤诊断治疗工作模式第一节多学科联合工作模式儿童肿瘤是一个较为复杂的疾病。
|
[
{
"id": 0,
"entity": "儿童恶性肿瘤",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "儿童肿瘤",
"start_offset": 29,
"end_offset": 33,
"label": "dis"
}
] |
诊断及治疗涉及外科、内科肿瘤专业、病理科、影像学科、放疗科、免疫学、遗传学等多个学科。
|
[
{
"id": 0,
"entity": "外科",
"start_offset": 7,
"end_offset": 9,
"label": "dep"
},
{
"id": 1,
"entity": "内科",
"start_offset": 10,
"end_offset": 12,
"label": "dep"
},
{
"id": 2,
"entity": "病理科",
"start_offset": 17,
"end_offset": 20,
"label": "dep"
},
{
"id": 3,
"entity": "影像学科",
"start_offset": 21,
"end_offset": 25,
"label": "dep"
},
{
"id": 4,
"entity": "放疗科",
"start_offset": 26,
"end_offset": 29,
"label": "dep"
}
] |
（一）外科在儿童肿瘤中的作用除血液系统恶性肿瘤外，外科在儿童恶性肿瘤的诊断与治疗中起着至关重要的作用，手术是儿童实体瘤的三大主要治疗手段之一。
|
[
{
"id": 0,
"entity": "外科",
"start_offset": 3,
"end_offset": 5,
"label": "dep"
},
{
"id": 1,
"entity": "儿童肿瘤",
"start_offset": 6,
"end_offset": 10,
"label": "dis"
},
{
"id": 2,
"entity": "血液系统恶性肿瘤",
"start_offset": 15,
"end_offset": 23,
"label": "dis"
},
{
"id": 3,
"entity": "外科",
"start_offset": 25,
"end_offset": 27,
"label": "dep"
},
{
"id": 4,
"entity": "儿童恶性肿瘤",
"start_offset": 28,
"end_offset": 34,
"label": "dis"
},
{
"id": 5,
"entity": "手术",
"start_offset": 51,
"end_offset": 53,
"label": "pro"
},
{
"id": 6,
"entity": "儿童实体瘤",
"start_offset": 54,
"end_offset": 59,
"label": "dis"
}
] |
随着诊断与治疗技术的不断发展，外科医生越来越需要其他相关专业参与儿童肿瘤的诊治工作。
|
[
{
"id": 0,
"entity": "外科",
"start_offset": 15,
"end_offset": 17,
"label": "dep"
},
{
"id": 1,
"entity": "儿童肿瘤",
"start_offset": 32,
"end_offset": 36,
"label": "dis"
}
] |
外科医生在儿童肿瘤中的主要作用有以下几个方面：1.向肿瘤专业、影像学、病理科等相关学科提供临床资料，如肿块大小、质地、粘连及术中情况，辅助肿瘤专业、影像学科和病理科作出完善而正确的诊断。
|
[
{
"id": 0,
"entity": "外科",
"start_offset": 0,
"end_offset": 2,
"label": "dep"
},
{
"id": 1,
"entity": "儿童肿瘤",
"start_offset": 5,
"end_offset": 9,
"label": "dis"
},
{
"id": 2,
"entity": "肿瘤专业",
"start_offset": 26,
"end_offset": 30,
"label": "dep"
},
{
"id": 3,
"entity": "影像学",
"start_offset": 31,
"end_offset": 34,
"label": "dep"
},
{
"id": 4,
"entity": "病理科",
"start_offset": 35,
"end_offset": 38,
"label": "dep"
},
{
"id": 5,
"entity": "肿瘤专业",
"start_offset": 69,
"end_offset": 73,
"label": "dep"
},
{
"id": 6,
"entity": "影像学科",
"start_offset": 74,
"end_offset": 78,
"label": "dep"
},
{
"id": 7,
"entity": "病理科",
"start_offset": 79,
"end_offset": 82,
"label": "dep"
}
] |
2.在肿瘤不能完全或大部分切除时作肿块活检，提供诊断用标本。
|
[
{
"id": 0,
"entity": "肿瘤",
"start_offset": 3,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "大部分切除",
"start_offset": 10,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "肿块活检",
"start_offset": 17,
"end_offset": 21,
"label": "pro"
}
] |
3.对能完全切除的肿瘤做根治性手术，达到治愈的目的。
|
[
{
"id": 0,
"entity": "肿瘤",
"start_offset": 9,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "手术",
"start_offset": 15,
"end_offset": 17,
"label": "pro"
}
] |
或做肿瘤大部分切除术切除术，结合其他治疗方法，增加治愈机会。
|
[
{
"id": 0,
"entity": "肿瘤",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "大部分切除术",
"start_offset": 4,
"end_offset": 10,
"label": "pro"
}
] |
4.以缓解症状改善生命质量为目的的姑息性手术，如造瘘术、转流术、减压术等。
|
[
{
"id": 0,
"entity": "姑息性手术",
"start_offset": 17,
"end_offset": 22,
"label": "pro"
},
{
"id": 1,
"entity": "造瘘术",
"start_offset": 24,
"end_offset": 27,
"label": "pro"
},
{
"id": 2,
"entity": "转流术",
"start_offset": 28,
"end_offset": 31,
"label": "pro"
},
{
"id": 3,
"entity": "减压术",
"start_offset": 32,
"end_offset": 35,
"label": "pro"
}
] |
（二）病理科的作用1.结合影像学、外科、肿瘤科及其他学科提供的临床资料作出正确的病理诊断、分型，指导临床治疗方案的选择。
|
[
{
"id": 0,
"entity": "病理科",
"start_offset": 3,
"end_offset": 6,
"label": "dep"
},
{
"id": 1,
"entity": "影像学",
"start_offset": 13,
"end_offset": 16,
"label": "dep"
},
{
"id": 2,
"entity": "外科",
"start_offset": 17,
"end_offset": 19,
"label": "dep"
},
{
"id": 3,
"entity": "肿瘤科",
"start_offset": 20,
"end_offset": 23,
"label": "dep"
}
] |
2.对切除的临近组织、器官、淋巴结作肿瘤浸润情况报告，提供临床分期依据，对治疗方案的强度选择至关重要。
|
[
{
"id": 0,
"entity": "组织",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "器官",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "淋巴结",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 3,
"entity": "肿瘤",
"start_offset": 18,
"end_offset": 20,
"label": "dis"
}
] |
3.对残留病灶的再活检，提供组织细胞病理水平完全缓解依据，指导进一步治疗计划的制定。
|
[
{
"id": 0,
"entity": "活检",
"start_offset": 9,
"end_offset": 11,
"label": "pro"
}
] |
（三）影像学科的作用通过肿瘤相关部位的X线平片、造影、超声检查、MRI、CT检查等向临床医生提供各种必要的信息。
|
[
{
"id": 0,
"entity": "影像学科",
"start_offset": 3,
"end_offset": 7,
"label": "dep"
},
{
"id": 1,
"entity": "肿瘤",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "X线平片",
"start_offset": 19,
"end_offset": 23,
"label": "pro"
},
{
"id": 3,
"entity": "造影",
"start_offset": 24,
"end_offset": 26,
"label": "pro"
},
{
"id": 4,
"entity": "超声检查",
"start_offset": 27,
"end_offset": 31,
"label": "pro"
},
{
"id": 5,
"entity": "MRI",
"start_offset": 32,
"end_offset": 35,
"label": "pro"
},
{
"id": 6,
"entity": "CT检查",
"start_offset": 36,
"end_offset": 40,
"label": "pro"
}
] |
1.向有关学科提供肿瘤影像学特征，如肿瘤大小、边界、钙化、液化及出血等，并作出影像学诊断，供临床医生及病理科参考。
|
[
{
"id": 0,
"entity": "肿瘤",
"start_offset": 18,
"end_offset": 20,
"label": "dis"
},
{
"id": 1,
"entity": "病理科",
"start_offset": 51,
"end_offset": 54,
"label": "dep"
}
] |
2.手术前向外科医生提供肿瘤与邻近脏器、组织、血管的关系，与外科医生共同确定手术的可行性及手术范围，完善手术方案。
|
[
{
"id": 0,
"entity": "手术",
"start_offset": 2,
"end_offset": 4,
"label": "pro"
},
{
"id": 1,
"entity": "外科",
"start_offset": 6,
"end_offset": 8,
"label": "dep"
},
{
"id": 2,
"entity": "肿瘤",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
},
{
"id": 3,
"entity": "脏器",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 4,
"entity": "组织",
"start_offset": 20,
"end_offset": 22,
"label": "bod"
},
{
"id": 5,
"entity": "血管",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 6,
"entity": "外科",
"start_offset": 30,
"end_offset": 32,
"label": "dep"
},
{
"id": 7,
"entity": "手术",
"start_offset": 38,
"end_offset": 40,
"label": "pro"
},
{
"id": 8,
"entity": "手术",
"start_offset": 45,
"end_offset": 47,
"label": "pro"
},
{
"id": 9,
"entity": "手术",
"start_offset": 52,
"end_offset": 54,
"label": "pro"
}
] |
3.从影像学角度确定病变扩散范围，提供肿瘤分期依据。
|
[
{
"id": 0,
"entity": "肿瘤",
"start_offset": 19,
"end_offset": 21,
"label": "dis"
}
] |
（四）肿瘤科的作用儿童肿瘤专业是一个新兴学科，既往儿童实体瘤主要在儿童外科诊治，随着化疗等技术的发展，许多儿童肿瘤在化疗介入后预后有了明显的改善。
|
[
{
"id": 0,
"entity": "肿瘤科",
"start_offset": 3,
"end_offset": 6,
"label": "dep"
},
{
"id": 1,
"entity": "儿童肿瘤",
"start_offset": 9,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "儿童实体瘤",
"start_offset": 25,
"end_offset": 30,
"label": "dis"
},
{
"id": 3,
"entity": "儿童外科",
"start_offset": 33,
"end_offset": 37,
"label": "dep"
},
{
"id": 4,
"entity": "化疗",
"start_offset": 42,
"end_offset": 44,
"label": "pro"
},
{
"id": 5,
"entity": "儿童肿瘤",
"start_offset": 53,
"end_offset": 57,
"label": "dis"
},
{
"id": 6,
"entity": "化疗",
"start_offset": 58,
"end_offset": 60,
"label": "pro"
}
] |
由于化疗及其他诊治技术越来越复杂，就需要有一组专业性人员来负责包括化疗在内的诊治方案的制定、实施及观察、随访，并负责各学科之间的协作。
|
[
{
"id": 0,
"entity": "化疗",
"start_offset": 2,
"end_offset": 4,
"label": "pro"
},
{
"id": 1,
"entity": "化疗",
"start_offset": 33,
"end_offset": 35,
"label": "pro"
}
] |
在进展中逐渐形成了儿童肿瘤专业，这一专业的人员通常来自熟悉儿童白血病化疗及支持治疗的血液专业。
|
[
{
"id": 0,
"entity": "儿童肿瘤",
"start_offset": 9,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "儿童白血病",
"start_offset": 29,
"end_offset": 34,
"label": "dis"
},
{
"id": 2,
"entity": "化疗",
"start_offset": 34,
"end_offset": 36,
"label": "pro"
}
] |
它的主要任务是与外科、病理科、放疗科、影像学等各学科协作，使诊断确切完善，治疗更合理有效，病人随访管理落实。
|
[
{
"id": 0,
"entity": "外科",
"start_offset": 8,
"end_offset": 10,
"label": "dep"
},
{
"id": 1,
"entity": "病理科",
"start_offset": 11,
"end_offset": 14,
"label": "dep"
},
{
"id": 2,
"entity": "放疗科",
"start_offset": 15,
"end_offset": 18,
"label": "dep"
},
{
"id": 3,
"entity": "影像学",
"start_offset": 19,
"end_offset": 22,
"label": "dep"
}
] |
2.结合外科、放疗科意见制定合理的综合治疗方案。
|
[
{
"id": 0,
"entity": "外科",
"start_offset": 4,
"end_offset": 6,
"label": "dep"
},
{
"id": 1,
"entity": "放疗科",
"start_offset": 7,
"end_offset": 10,
"label": "dep"
}
] |
（五）放疗科的作用与外科手术、内科化疗一样，放疗是儿童肿瘤三大主要治疗方法之一。
|
[
{
"id": 0,
"entity": "放疗科",
"start_offset": 3,
"end_offset": 6,
"label": "dep"
},
{
"id": 1,
"entity": "外科手术",
"start_offset": 10,
"end_offset": 14,
"label": "pro"
},
{
"id": 2,
"entity": "内科化疗",
"start_offset": 15,
"end_offset": 19,
"label": "pro"
},
{
"id": 3,
"entity": "放疗",
"start_offset": 22,
"end_offset": 24,
"label": "pro"
},
{
"id": 4,
"entity": "儿童肿瘤",
"start_offset": 25,
"end_offset": 29,
"label": "dis"
}
] |
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