text stringlengths 4 4.87k | entities list |
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【分类】AIHA的分类有两种，一种是根据病因进行分类，另一种是根据抗体的种类进行分类。 | [
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（2）继发性：发病率约占80%，常见病因有：1）感染：可由细菌、病毒、支原体或疫苗接种等引起，病原体包括伤寒、链球菌、金黄色葡萄球菌、结核、肝炎病毒、巨细胞包涵体病毒、EB病毒、疱疹病毒、流感病毒、腺病毒、腮腺炎病毒及肺炎支原体等。 | [
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"entity": "链球菌",
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"entity": "结核",
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"entity": "肝炎病毒",
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"entity": "巨细胞包涵体病毒",
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"entity": "EB病毒",
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"entity": "肺炎支原体",
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2）免疫性疾病：常见于系统性红斑狼疮、类风湿性关节炎、皮肌炎、免疫性血小板减少症、无丙种球蛋白血症、异常丙种球蛋白血症等。 | [
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"entity": "无丙种球蛋白血症",
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"entity": "异常丙种球蛋白血症",
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3）恶性肿瘤：如白血病、淋巴瘤、霍奇金病等。 | [
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4）多种药物：可通过半抗原药物依赖性非特异性抗体（如青霉素类、头孢霉素类等）或通过免疫复合物（如奎宁、奎尼丁等）或诱导真性自身抗体（如甲基多巴、左旋多巴等）而破坏红细胞，发生溶血性贫血。 | [
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2.根据抗体种类分类根据自身抗体作用在红细胞引起溶血所需要的最适温度可将AIHA分为温抗体型和冷抗体型两种，前者通常为IgG，少数为IgA和IgM；IgG亚类则以IgG1</sub>和IgG3</sub>为主，而IgG2</sub>及IgG4</sub>少见，最适温度为37℃；后者较少见，抗体属IgM，少数为IgG，最适温度为4℃。 | [
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两类抗体引起的AIHA，其发病机制、诊断、治疗方法和预后不尽相同。 | [
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【发病机制】AIHA的发病机制尚未完全阐明。 | [
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自身温抗体型通过IgG可变区Fab段吸附于红细胞膜上，其恒定区Fc段则暴露于膜外，一是通过激活补体破坏红细胞，二是Fc段可被位于单核巨噬细胞膜上的Fc受体所识别，借此单核巨噬细胞便可进行吞噬和毒性溶解被抗体包被的红细胞。 | [
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冷抗体型免疫性溶血性贫血可分为冷凝集素综合征（coldhemagglutininsyndrome）或冷凝集病和阵发性寒冷性血红蛋白尿；前者由病儿自身冷凝集素IgM引起，少数可由IgG或IgA引起；后者为IgG型冷抗体。 | [
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冷抗体型常继发于各种感染，可能由各种病原微生物和人类红细胞表面抗原相类似引起，所谓交叉抗原性（crossantigenicity）；也有人认为病原微生物代谢产物在体内与红细胞膜的蛋白质结合，使蛋白变性，成为一种新的抗原，因而刺激人体免疫系统产生自身抗体。 | [
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AIHA还可与免疫系统增生性疾病并发，如淋巴细胞白血病、恶性淋巴瘤等。 | [
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此外，在胶原血管疾病中也常有AI淋巴细胞白血病药物诱发的AIHA主要有三种类型：①青霉素型：亦称药物吸附型。 | [
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药物吸附于红细胞表面形成新的抗原，免疫系统制造抗体，通常是IgG与之结合而发生溶血。 | [
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青霉素、先锋霉素、四环素等所引起的AIHA均属这一类型。 | [
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②甲基青霉素型（methyldopatype）：α甲基多巴引起的AIHA属自身免疫性，60%见于HLA-B7阳性患儿；③免疫复合物型：这是由于IgM与药物反应，激活了补体系统，C3b</sub>沉积于红细胞表面，进而导致巨噬细胞对带有C3b</sub>的红细胞发生攻击和吞噬。 | [
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这种抗体与红细胞膜上的血型P抗原结合，通过激活补体而发生溶血性贫血。 | [
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温抗体型自身免疫性溶血性贫血：【临床表现】有三种临床类型：1.急性暂时型占70%～8温抗体型自身免疫性溶血性贫血的儿童，偶见于新生儿，男多于女。 | [
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起病大多急骤，伴有虚脱、苍白、黄疸、发热、血红蛋白尿等，病程呈自限性，通常2周内自行停止，最长不超过6个月。 | [
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严重溶血者，可发生急性肾功能不全，出现少尿、无尿和氮质血症等。 | [
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部分病例起病也可稍缓慢，主要表现为疲劳和苍白。 | [
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由青霉素引起者，与青霉素剂量有关，若每日用量超过120万单位，则很少出现溶血。 | [
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偶尔继发于系统性红斑狼疮等结缔组织病。 | [
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起病缓慢，主要症状有贫血、黄疸、肝脾大等，溶血可持续数月或数年，最长可达20年，可反复发作。 | [
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合并感染可加重病情，甚肝脾大溶血危象。 | [
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常并发其他血细胞成分异常，如合并中性粒细胞或血小板减少（Even综合征）。 | [
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Even综合征者，常可并发慢性疾病如系统性红斑狼疮等，因此预后大多不良。 | [
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3.抗人球蛋白试验（Coombs）阴性型与红细胞膜结合的抗体分子数过少（＜260）有关。 | [
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【实验室检查】1.外周血象大多数病例贫血严重，血红蛋白＜60g/L，球形和嗜多色性红细胞多见，网织红细胞可高达50%，可见有核红细胞。 | [
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慢性迁延型者，网织红细胞大多减少，主要原因是IgG抗体可以与幼红细胞和网织红细胞结合，使骨髓中的幼红细胞和网织红细胞减少，严重时可发生再障危象。 | [
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"entity": "幼红细胞",
"start_offset": 48,
"end_offset": 52,
"label": "bod"
}
] |
白细胞总数通常升高，可出现类白血病反应。 | [
{
"id": 0,
"entity": "白血病",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
}
] |
3.Coombs试验直接试验强阳性，而间接试验（测定血清中游离的抗红细胞抗体）阴性，偶尔间接试验也可呈阳性，与预后有关。 | [
{
"id": 0,
"entity": "Coombs试验",
"start_offset": 2,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "抗红细胞抗体",
"start_offset": 32,
"end_offset": 38,
"label": "bod"
}
] |
从有些病人血清中或从病人红细胞上洗脱下来的抗体还可以与其他人的红细胞发生凝集，这些抗体被认为是泛凝集素（panagglutinins），事实上，它们约与70%左右病人的红细胞Rh系统抗原呈特异性结合反应。 | [
{
"id": 0,
"entity": "红细胞",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
},
{
"id": 1,
"entity": "凝集素",
"start_offset": 48,
"end_offset": 51,
"label": "dru"
}
] |
部分病例的红细胞上可检测到与IgG结合的补体成分，通常为C3b</sub>。 | [
{
"id": 0,
"entity": "红细胞",
"start_offset": 5,
"end_offset": 8,
"label": "bod"
}
] |
一般来说，红细胞表面至少有260～500个抗体分子时，Coombs试验才能出现阳性，因此少数病例Coombs试验可因敏感度不足而呈阴性反应。 | [
{
"id": 0,
"entity": "抗体",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
}
] |
此时，需要采用特殊的诊断试验（如125</sup>I葡萄球菌蛋白A试验、放射免疫直接抗人球蛋白试验等）才能证实抗红细胞抗体的存在。 | [
{
"id": 0,
"entity": "抗体",
"start_offset": 59,
"end_offset": 61,
"label": "bod"
}
] |
4.胆红素和珠蛋白测定血清间接胆红素增加，尿中尿胆原增加，结合珠蛋白降低或消失。 | [
{
"id": 0,
"entity": "胆红素",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
}
] |
【诊断与鉴别诊断】近数月内无输胆红素特殊药物接触史，根据临床表现和实验室检查，尤其是直结合珠蛋白mbs试验阳性者可确诊，但后者阴性时不能否定。 | [
{
"id": 0,
"entity": "胆红素",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 1,
"entity": "直结合珠蛋白",
"start_offset": 42,
"end_offset": 48,
"label": "bod"
}
] |
本病应与其他溶血性贫血如珠蛋白生成障碍性贫血、溶血尿毒综合征、血栓性血小板减少性紫癜、传染性单核细胞增多症合并溶血等鉴别。 | [
{
"id": 0,
"entity": "溶血尿毒综合征",
"start_offset": 23,
"end_offset": 30,
"label": "dis"
}
] |
【治疗】1.一般治疗积极控制原发病，防治感染，以免引起溶血危象。 | [
{
"id": 0,
"entity": "感染",
"start_offset": 20,
"end_offset": 22,
"label": "sym"
}
] |
危重病例需注意水电解质平衡及心肾功能，溶血危象者宜采取碱化尿液的措施，应用低分子右旋糖苷以防DIC发生等。 | [
{
"id": 0,
"entity": "低分子右旋糖苷",
"start_offset": 37,
"end_offset": 44,
"label": "dru"
}
] |
2.药物治疗（1）激素：为温抗体型AIHA治疗的首选药，其主要药理机制是通过抑制抗体的产生、减少红细胞表面被覆盖的抗体量、干扰巨噬细胞表面的IgG及补体C3b</sub>受体以阻止其吞噬红细胞作用。 | [
{
"id": 0,
"entity": "红细胞",
"start_offset": 48,
"end_offset": 51,
"label": "bod"
},
{
"id": 1,
"entity": "红细胞",
"start_offset": 93,
"end_offset": 96,
"label": "bod"
}
] |
当出现溶血危象或再生障碍危象的重症或常规剂量无效者，可试用大剂量冲击疗法。 | [
{
"id": 0,
"entity": "溶血危象",
"start_offset": 3,
"end_offset": 7,
"label": "dis"
}
] |
也可应用大剂量地塞米松10mg/（M2</sup>•d）静滴，有效后改用泼尼松口服。 | [
{
"id": 0,
"entity": "地塞米松",
"start_offset": 7,
"end_offset": 11,
"label": "dru"
}
] |
（2）丙种球蛋白静滴：剂量400mg/（kg•d），连用5天为1个疗程，每隔3～5天可再用，至Hb达正常水平后1个月，再用1～2个疗程至Coombs试验转阴性后停药。 | [
{
"id": 0,
"entity": "丙种球蛋白静滴",
"start_offset": 3,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "Coombs试验",
"start_offset": 68,
"end_offset": 76,
"label": "pro"
}
] |
（3）免疫抑制剂：皮质激素治疗无效或泼尼松维持量每日＞10～15mg者，可应用免疫抑制剂或联合用药。 | [
{
"id": 0,
"entity": "皮质激素治疗",
"start_offset": 9,
"end_offset": 15,
"label": "pro"
},
{
"id": 1,
"entity": "泼尼松",
"start_offset": 18,
"end_offset": 21,
"label": "dru"
},
{
"id": 2,
"entity": "免疫抑制剂",
"start_offset": 39,
"end_offset": 44,
"label": "dru"
}
] |
常用制剂有硫唑嘌呤（AZP）、6-巯基嘌呤（6-MP）、环磷酰胺（CTX）、环胞霉素A（CsA）等。 | [
{
"id": 0,
"entity": "硫唑嘌呤",
"start_offset": 5,
"end_offset": 9,
"label": "dru"
},
{
"id": 1,
"entity": "AZP",
"start_offset": 10,
"end_offset": 13,
"label": "dru"
},
{
"id": 2,
"entity": "6-巯基嘌呤",
"start_offset": 15,
"end_offset": 21,
"label": "dru"
},
{
"id": 3,
"entity": "6-MP",
"start_offset": 22,
"end_offset": 26,
"label": "dru"
},
{
"id": 4,
"entity": "环磷酰胺",
"start_offset": 28,
"end_offset": 32,
"label": "dru"
},
{
"id": 5,
"entity": "CTX",
"start_offset": 33,
"end_offset": 36,
"label": "dru"
},
{
"id": 6,
"entity": "环胞霉素A",
"start_offset": 38,
"end_offset": 43,
"label": "dru"
},
{
"id": 7,
"entity": "CsA",
"start_offset": 44,
"end_offset": 47,
"label": "dru"
}
] |
3.脾切除对上述治疗疗效均差的温抗体型AIHA者，可考虑行脾切除术，有效率可达60%。 | [
{
"id": 0,
"entity": "脾切除",
"start_offset": 2,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "脾切除术",
"start_offset": 29,
"end_offset": 33,
"label": "pro"
}
] |
4.输血及血浆置换AIHA患儿一般不宜作输血治疗，因为，体内自身抗红细胞抗体可致自体或异体红细胞破坏溶血，且自身抗体尚可干扰血型配型等特点，输血后很容易发生更严重的溶血反应。 | [
{
"id": 0,
"entity": "输血治疗",
"start_offset": 20,
"end_offset": 24,
"label": "pro"
},
{
"id": 1,
"entity": "抗体",
"start_offset": 36,
"end_offset": 38,
"label": "bod"
},
{
"id": 2,
"entity": "自体或异体红细胞破坏溶血",
"start_offset": 40,
"end_offset": 52,
"label": "sym"
},
{
"id": 3,
"entity": "溶血",
"start_offset": 82,
"end_offset": 84,
"label": "dis"
}
] |
如果确因病情严重而需输血者，也应严格掌握适应证：①急性溶血进展迅速而致严重贫血（Hb＜40g/L），或发生溶血危象，心功能失代偿或严重脑缺氧和全身衰竭者；②急性溶血不能用糖皮质激素和免疫抑制剂控制者。 | [
{
"id": 0,
"entity": "心功能失代偿",
"start_offset": 58,
"end_offset": 64,
"label": "sym"
},
{
"id": 1,
"entity": "免疫抑制剂",
"start_offset": 91,
"end_offset": 96,
"label": "dru"
}
] |
用洗涤浓缩红细胞可减少输血反应。 | [
{
"id": 0,
"entity": "红细胞",
"start_offset": 5,
"end_offset": 8,
"label": "bod"
}
] |
六、α1-抗胰蛋白酶缺乏症α1-抗胰蛋白酶缺乏症（α1-antitrypsindeficiency）简称α1-AT缺乏症，是一种常染色体隐性遗传性疾病。 | [
{
"id": 0,
"entity": "α1-抗胰蛋白酶缺乏症",
"start_offset": 2,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "α1-抗胰蛋白酶缺乏症",
"start_offset": 13,
"end_offset": 24,
"label": "dis"
},
{
"id": 2,
"entity": "α1-antitrypsindeficiency",
"start_offset": 25,
"end_offset": 49,
"label": "dis"
},
{
"id": 3,
"entity": "α1-AT缺乏症",
"start_offset": 52,
"end_offset": 60,
"label": "dis"
}
] |
以婴儿期出现胆汁淤积性黄疸、进行性肝功能损害和青年后期出现肺气肿为主要临床表现，常有家族史。 | [
{
"id": 0,
"entity": "胆汁淤积性黄疸",
"start_offset": 6,
"end_offset": 13,
"label": "sym"
},
{
"id": 1,
"entity": "进行性肝功能损害",
"start_offset": 14,
"end_offset": 22,
"label": "sym"
},
{
"id": 2,
"entity": "青年后期出现肺气肿",
"start_offset": 23,
"end_offset": 32,
"label": "sym"
}
] |
儿童期累及肺部者罕见。 | [
{
"id": 0,
"entity": "肺部",
"start_offset": 5,
"end_offset": 7,
"label": "bod"
}
] |
目前普遍认为蛋白酶溶解学说是肺气肿的发病机制。 | [
{
"id": 0,
"entity": "蛋白酶",
"start_offset": 6,
"end_offset": 9,
"label": "mic"
},
{
"id": 1,
"entity": "肺气肿",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
}
] |
α1-AT和其他抗蛋白酶在灭活死亡细菌及中性粒细胞释放的蛋白溶解酶过程中起重要作用。 | [
{
"id": 0,
"entity": "α1-AT",
"start_offset": 0,
"end_offset": 5,
"label": "mic"
},
{
"id": 1,
"entity": "抗蛋白酶",
"start_offset": 8,
"end_offset": 12,
"label": "mic"
},
{
"id": 2,
"entity": "细菌",
"start_offset": 17,
"end_offset": 19,
"label": "mic"
},
{
"id": 3,
"entity": "中性粒细胞",
"start_offset": 20,
"end_offset": 25,
"label": "mic"
},
{
"id": 4,
"entity": "蛋白溶解酶",
"start_offset": 28,
"end_offset": 33,
"label": "mic"
}
] |
α1-AT严重缺乏者在炎症等刺激时不能提高分泌，而中性粒细胞和巨噬细胞在防御作用中释放的蛋白溶解酶过多积聚，引起肺组织蛋白溶解破坏和肺气肿。 | [
{
"id": 0,
"entity": "α1-AT",
"start_offset": 0,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "炎症",
"start_offset": 11,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "不能提高分泌",
"start_offset": 17,
"end_offset": 23,
"label": "sym"
},
{
"id": 3,
"entity": "中性粒细胞",
"start_offset": 25,
"end_offset": 30,
"label": "bod"
},
{
"id": 4,
"entity": "巨噬细胞",
"start_offset": 31,
"end_offset": 35,
"label": "bod"
},
{
"id": 5,
"entity": "蛋白溶解酶",
"start_offset": 44,
"end_offset": 49,
"label": "mic"
},
{
"id": 6,
"entity": "肺组织蛋白",
"start_offset": 56,
"end_offset": 61,
"label": "bod"
},
{
"id": 7,
"entity": "肺气肿",
"start_offset": 66,
"end_offset": 69,
"label": "dis"
}
] |
少数患儿可出现呼吸困难、咳喘、弥漫性肺气肿及桶状胸、杵状指（趾），肺部叩诊为过清音，伴生长发育障碍。 | [
{
"id": 0,
"entity": "呼吸困难",
"start_offset": 7,
"end_offset": 11,
"label": "sym"
},
{
"id": 1,
"entity": "咳喘",
"start_offset": 12,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "弥漫性肺气肿",
"start_offset": 15,
"end_offset": 21,
"label": "sym"
},
{
"id": 3,
"entity": "桶状胸",
"start_offset": 22,
"end_offset": 25,
"label": "sym"
},
{
"id": 4,
"entity": "杵状指（趾）",
"start_offset": 26,
"end_offset": 32,
"label": "sym"
},
{
"id": 5,
"entity": "肺部",
"start_offset": 33,
"end_offset": 35,
"label": "bod"
},
{
"id": 6,
"entity": "过清音",
"start_offset": 38,
"end_offset": 41,
"label": "sym"
},
{
"id": 7,
"entity": "生长发育障碍",
"start_offset": 43,
"end_offset": 49,
"label": "sym"
}
] |
胸部X线检查可见两侧肺气肿和膈肌下降。 | [
{
"id": 0,
"entity": "胸部X线检查",
"start_offset": 0,
"end_offset": 6,
"label": "ite"
},
{
"id": 1,
"entity": "肺气肿",
"start_offset": 10,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "膈肌下降",
"start_offset": 14,
"end_offset": 18,
"label": "dis"
}
] |
吸烟可显著增加发生肺气肿的危险性。 | [
{
"id": 0,
"entity": "肺气肿",
"start_offset": 9,
"end_offset": 12,
"label": "dis"
}
] |
血清α1-AT定量及胰蛋白酶抑制活性测定有助诊断。 | [
{
"id": 0,
"entity": "血清α1-AT定量及胰蛋白酶抑制活性测定",
"start_offset": 0,
"end_offset": 20,
"label": "ite"
}
] |
酶替代治疗可能成为本症的主要治疗方法。 | [
{
"id": 0,
"entity": "酶替代治疗",
"start_offset": 0,
"end_offset": 5,
"label": "pro"
}
] |
美国FDA已批准使用人血源性纯化酶用于某些纯合子患者。 | [
{
"id": 0,
"entity": "人血源性纯化酶",
"start_offset": 10,
"end_offset": 17,
"label": "mic"
}
] |
通过重组DNA技术亦已获得纯化酶。 | [
{
"id": 0,
"entity": "重组DNA技术",
"start_offset": 2,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "纯化酶",
"start_offset": 13,
"end_offset": 16,
"label": "mic"
}
] |
重症患者可能需要外科干预，包括肺减容术和肺移植。 | [
{
"id": 0,
"entity": "肺减容术",
"start_offset": 15,
"end_offset": 19,
"label": "pro"
},
{
"id": 1,
"entity": "肺移植",
"start_offset": 20,
"end_offset": 23,
"label": "pro"
}
] |
三、室管膜瘤室管膜瘤（ependymoma）发生于脑室的室管膜细胞，占儿童中枢神经系统原发性肿瘤5%～10%。 | [
{
"id": 0,
"entity": "室管膜瘤",
"start_offset": 2,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "室管膜瘤",
"start_offset": 6,
"end_offset": 10,
"label": "dis"
},
{
"id": 2,
"entity": "ependymoma",
"start_offset": 11,
"end_offset": 21,
"label": "dis"
},
{
"id": 3,
"entity": "脑室",
"start_offset": 25,
"end_offset": 27,
"label": "bod"
},
{
"id": 4,
"entity": "室管膜细胞",
"start_offset": 28,
"end_offset": 33,
"label": "bod"
},
{
"id": 5,
"entity": "儿童中枢神经系统原发性肿瘤",
"start_offset": 35,
"end_offset": 48,
"label": "dis"
}
] |
好发于后颅窝，约60%。 | [
{
"id": 0,
"entity": "后颅窝",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
}
] |
瘤体多位于脑室内，少数在脑室旁组织内，呈缓慢、浸润性生长。 | [
{
"id": 0,
"entity": "瘤体",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "脑室",
"start_offset": 5,
"end_offset": 7,
"label": "bod"
},
{
"id": 2,
"entity": "脑室旁",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
},
{
"id": 3,
"entity": "组织",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
}
] |
瘤细胞亦可脱落于蛛网膜下腔产生播散性种植。 | [
{
"id": 0,
"entity": "瘤细胞",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "蛛网膜下腔",
"start_offset": 8,
"end_offset": 13,
"label": "bod"
}
] |
肿瘤呈灰白色或紫红色质软，有的较硬，呈颗粒状，可有钙化。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "肿瘤呈灰白色或紫红色",
"start_offset": 0,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "质软",
"start_offset": 10,
"end_offset": 12,
"label": "sym"
},
{
"id": 3,
"entity": "有的较硬",
"start_offset": 13,
"end_offset": 17,
"label": "sym"
},
{
"id": 4,
"entity": "呈颗粒状",
"start_offset": 18,
"end_offset": 22,
"label": "sym"
},
{
"id": 5,
"entity": "可有钙化",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
}
] |
有包膜分界体积较大时多有囊肿形成瘤细胞较致密排列成腺泡或腺管状，形成假菊形团。 | [
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"id": 0,
"entity": "包膜",
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"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "有包膜分界",
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{
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"entity": "囊肿",
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{
"id": 3,
"entity": "体积较大时多有囊肿形成",
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{
"id": 4,
"entity": "瘤细胞",
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"end_offset": 19,
"label": "bod"
},
{
"id": 5,
"entity": "瘤细胞较致密",
"start_offset": 16,
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},
{
"id": 6,
"entity": "排列成腺泡或腺管状",
"start_offset": 22,
"end_offset": 31,
"label": "sym"
}
] |
核为圆形或椭圆形，间质内有胶质纤维形成的网状结构，血管较多。 | [
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"entity": "间质",
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{
"id": 1,
"entity": "胶质纤维",
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"label": "bod"
},
{
"id": 2,
"entity": "血管",
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"label": "bod"
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] |
初期可有头痛、呕吐颅内压增高症状。 | [
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"entity": "头痛",
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"end_offset": 6,
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{
"id": 1,
"entity": "呕吐",
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{
"id": 2,
"entity": "颅内压增高",
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] |
辅助检查CT及MRI检查可清楚地显示肿瘤大小、形状、有无钙化及其与周围结构的关系。 | [
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"id": 0,
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{
"id": 2,
"entity": "肿瘤",
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"end_offset": 20,
"label": "bod"
}
] |
手术切除为治疗本病的主要手段，如肿瘤自第四脑室底部长出，术中可残留一薄层肿瘤组织，以免脑干损伤，术中务必使原先脑脊液梗阻放疗和化疗。 | [
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"id": 0,
"entity": "手术切除",
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{
"id": 1,
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{
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"entity": "第四脑室底部",
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{
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{
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"entity": "脑干",
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{
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"entity": "脑脊液",
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"entity": "脑脊液梗阻",
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"entity": "放疗",
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"label": "pro"
},
{
"id": 8,
"entity": "化疗",
"start_offset": 63,
"end_offset": 65,
"label": "pro"
}
] |
间变性肿瘤或幕下肿瘤，应做全脑及脊髓轴的放疗，防止肿瘤种植到脑的其他部位或脊髓；低度肿瘤放疗范围尚有争议，目前多数主张局部照射，而不作大范围预防性放疗。 | [
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"entity": "间变性肿瘤",
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{
"id": 1,
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{
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{
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"entity": "放疗",
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{
"id": 9,
"entity": "放疗",
"start_offset": 73,
"end_offset": 75,
"label": "pro"
}
] |
本病的预后与肿瘤切除程度有关。 | [
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"id": 0,
"entity": "肿瘤切除",
"start_offset": 6,
"end_offset": 10,
"label": "pro"
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] |
三、肾穿刺操作步骤1.负压抽吸法嘱患儿排空膀胱后俯卧于检查台上，体位端正，腹部肋下垫一直径为10～15cm的硬枕，以固定肾脏，两上肢置于头前。 | [
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"entity": "肾穿刺",
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"label": "pro"
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{
"id": 1,
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{
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{
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"entity": "腹部",
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{
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"entity": "肾脏",
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{
"id": 5,
"entity": "上肢",
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"label": "bod"
},
{
"id": 6,
"entity": "头",
"start_offset": 68,
"end_offset": 69,
"label": "bod"
}
] |
常规皮肤消毒、铺巾，B超定位后行局部麻醉（2%利多卡因1ml皮下注射）；以22号腰穿针为探针，如探及肾包囊，可见针芯随呼吸头尾摆动，计算测得距离；在穿刺点上做一小切口，进针深度=皮肾距离（cm）+取肾组织长度（1.0～2.0cm）+肾退让距离（1～1.5cm）；进针方向与肾纵轴垂直。 | [
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"id": 0,
"entity": "皮肤消毒",
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"label": "pro"
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{
"id": 1,
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{
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{
"id": 3,
"entity": "利多卡因",
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"label": "dru"
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{
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"entity": "皮下注射",
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"label": "pro"
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{
"id": 5,
"entity": "腰穿针",
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{
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"entity": "探针",
"start_offset": 44,
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"label": "equ"
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{
"id": 7,
"entity": "肾包囊",
"start_offset": 50,
"end_offset": 53,
"label": "bod"
},
{
"id": 8,
"entity": "针芯",
"start_offset": 56,
"end_offset": 58,
"label": "equ"
},
{
"id": 9,
"entity": "皮肾",
"start_offset": 89,
"end_offset": 91,
"label": "bod"
},
{
"id": 10,
"entity": "肾组织",
"start_offset": 99,
"end_offset": 102,
"label": "bod"
},
{
"id": 11,
"entity": "肾",
"start_offset": 116,
"end_offset": 117,
"label": "bod"
},
{
"id": 12,
"entity": "肾",
"start_offset": 136,
"end_offset": 137,
"label": "bod"
}
] |
将穿刺针直至肾包膜处，拔出针芯，于针管内放置针栓（防止肾组织吸至负压注射器内），连接负压装置，嘱受检者屏气，肾固定后在助手配合下进行穿刺，一次穿刺动作于0.5秒内完成。 | [
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"id": 0,
"entity": "穿刺针",
"start_offset": 1,
"end_offset": 4,
"label": "equ"
},
{
"id": 1,
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"start_offset": 6,
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"label": "bod"
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{
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{
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{
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"entity": "针栓",
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{
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"entity": "肾组织",
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{
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{
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"entity": "负压装置",
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{
"id": 8,
"entity": "肾",
"start_offset": 54,
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{
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"entity": "穿刺",
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},
{
"id": 10,
"entity": "穿刺",
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"label": "pro"
}
] |
2.自动穿刺枪20世纪90年代后出现了应用穿刺枪作经皮肾穿刺活检的报道，穿刺枪体积小，重量轻，便于一人控制和操作。 | [
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"entity": "自动穿刺枪",
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{
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{
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{
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] |
穿刺部位皮肤切口小，在B超引导下进针，进针方向与肾纵轴垂直线呈10°～15°夹角，穿刺针至肾包膜后，嘱患者屏气，扣动开关，在快速的强力弹簧驱动下，活检针迅速精确地进针穿刺。 | [
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"id": 0,
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},
{
"id": 1,
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{
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"entity": "B超",
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{
"id": 3,
"entity": "肾",
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{
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"entity": "肾包膜",
"start_offset": 45,
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"label": "bod"
},
{
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"entity": "活检针",
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},
{
"id": 7,
"entity": "穿刺",
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"label": "pro"
}
] |
穿刺枪的应用使操作简化，单手操作整个过程，取材成功率高，减少穿刺后出血等并发症，对周围组织的损伤最小。 | [
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{
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{
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"entity": "出血",
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{
"id": 3,
"entity": "组织",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
}
] |
3.肾活检后的处理穿刺后常规按压穿刺部位15分钟，以纱布覆盖伤口，用多头带包紧腰部，患者仰卧用推床推床将小儿送回病房，平卧24小时。 | [
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"id": 0,
"entity": "肾活检",
"start_offset": 2,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
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"label": "pro"
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{
"id": 2,
"entity": "穿刺",
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{
"id": 3,
"entity": "纱布",
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{
"id": 4,
"entity": "头带",
"start_offset": 35,
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},
{
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"entity": "腰部",
"start_offset": 39,
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},
{
"id": 6,
"entity": "推床",
"start_offset": 47,
"end_offset": 49,
"label": "equ"
}
] |
穿刺后每小时测血压及脉搏，连续3～4次，如无异常可改为4小时测1次至24小时止。 | [
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"id": 0,
"entity": "穿刺",
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{
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"label": "ite"
},
{
"id": 2,
"entity": "脉搏",
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] |
肾穿刺后连续检查尿液3次，若无血尿则可停止检查监测，若有血尿则应连续监测，直到血尿消失。 | [
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"id": 0,
"entity": "肾穿刺",
"start_offset": 0,
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{
"id": 1,
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"label": "ite"
},
{
"id": 2,
"entity": "血尿",
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},
{
"id": 3,
"entity": "血尿",
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},
{
"id": 4,
"entity": "血尿",
"start_offset": 39,
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"label": "sym"
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] |
有肉眼血尿者则应给予补液，或多饮水，使尿量增加以防血块形成堵塞输尿管。 | [
{
"id": 0,
"entity": "血尿",
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{
"id": 1,
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{
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{
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},
{
"id": 4,
"entity": "输尿管",
"start_offset": 31,
"end_offset": 34,
"label": "bod"
}
] |
患儿应在1周内适当限制活动，观察有无肾周血肿的出现，如腰酸、腹部胀痛，腰部出现包块或血红蛋白下降等，可疑时应做超声波检查。 | [
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"id": 0,
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},
{
"id": 1,
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{
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{
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"entity": "腰部出现包块",
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},
{
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"entity": "血红蛋白下降",
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},
{
"id": 5,
"entity": "超声波检查",
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"label": "pro"
}
] |
例如，维生素E是生物膜和脂蛋白最重要的氧自由基清除剂，抑制脂质过氧化作用，对预防动脉粥样硬化和婴幼儿视网膜病变很重要；维生素C可抑制膳食中亚硝胺的致癌作用；许多流行病学调查证明，体内β-胡萝卜素水平的增加，可减少癌症和心血管疾病的危险性。 | [
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"entity": "生物膜",
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"label": "bod"
},
{
"id": 1,
"entity": "脂蛋白",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
},
{
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"entity": "动脉粥样硬化",
"start_offset": 40,
"end_offset": 46,
"label": "dis"
},
{
"id": 3,
"entity": "婴幼儿视网膜病变",
"start_offset": 47,
"end_offset": 55,
"label": "dis"
},
{
"id": 4,
"entity": "癌症",
"start_offset": 106,
"end_offset": 108,
"label": "dis"
},
{
"id": 5,
"entity": "心血管疾病",
"start_offset": 109,
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"label": "dis"
}
] |
（一）维生素A主要功能是促进生长发育、维持表皮的完整性和视觉功能、促进生殖功能和维持骨细胞的代谢平衡等，近年研究表明其还有抗肿瘤作用。 | [
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{
"id": 1,
"entity": "肿瘤",
"start_offset": 62,
"end_offset": 64,
"label": "dis"
}
] |
（二）维生素D主要功能是促进钙和磷在肠道内的吸收（钙和磷的比例为1～2∶1时吸收最佳）、增加肾脏对钙的重吸收，对骨骼形成极为重要，促使骨的生长和软骨骨化，与甲状旁腺一起维持血钙正常水平，防止骨质疏松和低钙痉挛。 | [
{
"id": 0,
"entity": "肠道",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
},
{
"id": 1,
"entity": "肾脏",
"start_offset": 46,
"end_offset": 48,
"label": "bod"
},
{
"id": 2,
"entity": "骨骼",
"start_offset": 56,
"end_offset": 58,
"label": "bod"
},
{
"id": 3,
"entity": "骨",
"start_offset": 67,
"end_offset": 68,
"label": "bod"
},
{
"id": 4,
"entity": "软骨",
"start_offset": 72,
"end_offset": 74,
"label": "bod"
},
{
"id": 5,
"entity": "甲状旁腺",
"start_offset": 78,
"end_offset": 82,
"label": "bod"
},
{
"id": 6,
"entity": "血钙",
"start_offset": 86,
"end_offset": 88,
"label": "ite"
},
{
"id": 7,
"entity": "骨质疏松",
"start_offset": 95,
"end_offset": 99,
"label": "dis"
},
{
"id": 8,
"entity": "低钙痉挛",
"start_offset": 100,
"end_offset": 104,
"label": "dis"
}
] |
（四）维生素K维生素K是凝血酶原的主要成分，还能促使肝脏合成凝血酶原，临床上常作为止血药应用。 | [
{
"id": 0,
"entity": "凝血酶原",
"start_offset": 12,
"end_offset": 16,
"label": "bod"
},
{
"id": 1,
"entity": "肝脏",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 2,
"entity": "凝血酶原",
"start_offset": 30,
"end_offset": 34,
"label": "bod"
}
] |
一部分维生素K可由人体回肠内细菌合成被吸收利用；另一部分由食物获得，主要来源于绿叶蔬菜、动物内脏、肉类和奶类。 | [
{
"id": 0,
"entity": "回肠内细菌",
"start_offset": 11,
"end_offset": 16,
"label": "bod"
}
] |
估计每天总的需要量为2μg/kg，如肠道功能不正常或长期应用抗生素者，有时需要补充一定量的维生素K来预防出血倾向。 | [
{
"id": 0,
"entity": "肠道",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
}
] |
主要概括为以下方面：促进胶原和神经递质的合成，类固醇化合物羟化，抗体生成，促使叶酸的活化；可防治坏血病，保护细胞膜，提高铁的吸收和利用，无论在治疗缺铁性还是巨幼红细胞性贫血胆固醇有协同作用；有抗氧化和清除自由基作用；另外，还可促进胆固醇的排出，防止动脉粥样硬化形成，并有提高机体免疫、增加白细胞的吞噬功能。 | [
{
"id": 0,
"entity": "胶原",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 1,
"entity": "神经递质",
"start_offset": 15,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "抗体",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 3,
"entity": "细胞膜",
"start_offset": 54,
"end_offset": 57,
"label": "bod"
},
{
"id": 4,
"entity": "胆固醇",
"start_offset": 86,
"end_offset": 89,
"label": "bod"
},
{
"id": 5,
"entity": "动脉粥样硬化",
"start_offset": 124,
"end_offset": 130,
"label": "dis"
},
{
"id": 6,
"entity": "白细胞",
"start_offset": 144,
"end_offset": 147,
"label": "bod"
}
] |
（二）维生素B<sub>1</sub>维生素B<sub>1</sub>又称硫胺素，是构成脱羧酶的辅酶，参与丙酮酸等的氧化脱羧反应，如缺乏可使丙酮酸在神经组织和末梢血管沉积而致脚气病。 | [
{
"id": 0,
"entity": "神经组织",
"start_offset": 74,
"end_offset": 78,
"label": "bod"
},
{
"id": 1,
"entity": "末梢血管",
"start_offset": 79,
"end_offset": 83,
"label": "bod"
},
{
"id": 2,
"entity": "丙酮酸在神经组织和末梢血管沉积",
"start_offset": 70,
"end_offset": 85,
"label": "sym"
},
{
"id": 3,
"entity": "脚气病",
"start_offset": 87,
"end_offset": 90,
"label": "dis"
}
] |
（三）维生素B<sub>2</sub>维生素B<sub>2</sub>又称核黄素，其主要功能是构成核黄素辅酶参与体内多种物质的氧化还原反应，是一种重要的营养素，如缺乏将影响物质和能量代谢，会出现多种临床症状，常见的有舌炎、口角炎、口腔溃疡、脂溢性皮炎等。 | [
{
"id": 0,
"entity": "舌炎",
"start_offset": 108,
"end_offset": 110,
"label": "sym"
},
{
"id": 1,
"entity": "口角炎",
"start_offset": 111,
"end_offset": 114,
"label": "sym"
},
{
"id": 2,
"entity": "口腔",
"start_offset": 115,
"end_offset": 117,
"label": "bod"
},
{
"id": 3,
"entity": "口腔溃疡",
"start_offset": 115,
"end_offset": 119,
"label": "sym"
},
{
"id": 4,
"entity": "脂溢性皮炎",
"start_offset": 120,
"end_offset": 125,
"label": "sym"
}
] |
（四）维生素B<sub>6</sub>维生素B<sub>6</sub>又称吡哆素，在体内与磷酸结合构成多种酶的辅酶，参与三大营养素的代谢，并与血红素的合成有关，缺乏时可引起低色素性贫血。 | [
{
"id": 0,
"entity": "低色素性贫血",
"start_offset": 86,
"end_offset": 92,
"label": "dis"
}
] |
主要生理功能是提高叶酸的利用率，促进红细胞的发育和成熟；还与神经髓鞘的物质代谢密切相关。 | [
{
"id": 0,
"entity": "神经髓鞘",
"start_offset": 30,
"end_offset": 34,
"label": "bod"
}
] |
故缺乏时可导致巨细胞性贫血并出现神经系统症状。 | [
{
"id": 0,
"entity": "巨细胞性贫血",
"start_offset": 7,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "神经系统",
"start_offset": 16,
"end_offset": 20,
"label": "bod"
}
] |
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