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维生素B<sub>12</sub>主要存在于动物性食物中,人体肠道内细菌也能大量合成,由于其吸收需在胃壁细胞分泌的内因子作用下在回肠部被吸收,故当胃或回肠切除后等胃肠功能减退时会发生缺乏,也可见于严格素食者和老年人中。
[ { "id": 0, "entity": "肠道内细菌", "start_offset": 31, "end_offset": 36, "label": "bod" }, { "id": 1, "entity": "回肠部", "start_offset": 64, "end_offset": 67, "label": "bod" }, { "id": 2, "entity": "胃", "start_offset": 73, "end_offset": 74, "label": "bod" }, { "id": 3, "entity": "回肠", "start_offset": 75, "end_offset": 77, "label": "bod" }, { "id": 4, "entity": "胃肠", "start_offset": 81, "end_offset": 83, "label": "bod" } ]
(六)烟酸又称尼克酸或维生素PP,经小肠吸收后形成脱氢酶辅酶,在体内代谢中起着递氢的作用。
[ { "id": 0, "entity": "脱氢酶辅酶", "start_offset": 25, "end_offset": 30, "label": "bod" } ]
严重缺乏时会出现皮炎、腹泻和痴呆的癞皮病典型症状。
[ { "id": 0, "entity": "皮炎", "start_offset": 8, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "腹泻", "start_offset": 11, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "痴呆的癞皮病", "start_offset": 14, "end_offset": 20, "label": "dis" } ]
皮肤症状表现为肢体暴露部位的对称性皮炎,包括急性红斑和褶烂、慢性肥厚和萎缩、色素沉着等;消化系统症状包括舌炎、口角炎、恶心呕吐、慢性胃炎、便秘或腹泻等;神经系统症状可产生精神错乱、神志不清甚至痴呆等。
[ { "id": 0, "entity": "皮肤", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "肢体暴露部位的对称性皮炎", "start_offset": 7, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "急性红斑和褶烂", "start_offset": 22, "end_offset": 29, "label": "sym" }, { "id": 3, "entity": "慢性肥厚和萎缩", "start_offset": 30, "end_offset": 37, "label": "sym" }, { "id": 4, "entity": "色素沉着", "start_offset": 38, "end_offset": 42, "label": "sym" }, { "id": 5, "entity": "消化系统", "start_offset": 44, "end_offset": 48, "label": "bod" }, { "id": 6, "entity": "舌炎", "start_offset": 52, "end_offset": 54, "label": "sym" }, { "id": 7, "entity": "口角炎", "start_offset": 55, "end_offset": 58, "label": "sym" }, { "id": 8, "entity": "恶心呕吐", "start_offset": 59, "end_offset": 63, "label": "sym" }, { "id": 9, "entity": "慢性胃炎", "start_offset": 64, "end_offset": 68, "label": "sym" }, { "id": 10, "entity": "便秘", "start_offset": 69, "end_offset": 71, "label": "sym" }, { "id": 11, "entity": "腹泻", "start_offset": 72, "end_offset": 74, "label": "sym" }, { "id": 12, "entity": "神经系统", "start_offset": 76, "end_offset": 80, "label": "bod" }, { "id": 13, "entity": "精神错乱", "start_offset": 85, "end_offset": 89, "label": "sym" }, { "id": 14, "entity": "神志不清", "start_offset": 90, "end_offset": 94, "label": "sym" }, { "id": 15, "entity": "痴呆", "start_offset": 96, "end_offset": 98, "label": "sym" } ]
由于抗结核药异烟肼与烟酸拮抗,故应用该药时需注意烟酸的补充。
[ { "id": 0, "entity": "异烟肼", "start_offset": 6, "end_offset": 9, "label": "dru" } ]
除了食物中很易得到外,肠内细菌也能合成,故通常不会缺乏。
[ { "id": 0, "entity": "肠内细菌", "start_offset": 11, "end_offset": 15, "label": "bod" } ]
缺乏时表现为皮炎、舌乳头萎缩、恶心呕吐和食欲减退等。
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(九)叶酸在体内的主要生理功能是促进红细胞的生成,缺乏时红细胞的发育和成熟会受到影响,引起巨幼红细胞性贫血;还与胎儿的神经管的发育有关,孕妇叶酸缺乏时可致胎儿神经管的发育畸形。
[ { "id": 0, "entity": "红细胞", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 1, "entity": "红细胞", "start_offset": 28, "end_offset": 31, "label": "dis" }, { "id": 2, "entity": "巨幼红细胞性贫血", "start_offset": 45, "end_offset": 53, "label": "dis" }, { "id": 3, "entity": "神经管", "start_offset": 59, "end_offset": 62, "label": "dis" }, { "id": 4, "entity": "叶酸缺乏", "start_offset": 70, "end_offset": 74, "label": "dis" }, { "id": 5, "entity": "胎儿神经管的发育畸形", "start_offset": 77, "end_offset": 87, "label": "dis" } ]
近年研究发现叶酸缺乏可引起高同型半胱氨酸血症,被认为是心血管疾病的危险因素,可影响胚胎早期心血管的发育。
[ { "id": 0, "entity": "高同型半胱氨酸血症", "start_offset": 13, "end_offset": 22, "label": "dis" }, { "id": 1, "entity": "心血管疾病", "start_offset": 27, "end_offset": 32, "label": "dis" }, { "id": 2, "entity": "心血管", "start_offset": 45, "end_offset": 48, "label": "bod" } ]
第四节肺泡微石症肺泡微石症(pulmonaryalveolarmicrolithiasis)以肺泡内形成以钙为主成分、广泛存在的播散性小结石为特征。
[ { "id": 0, "entity": "肺泡微石症", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "肺泡微石症", "start_offset": 8, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "pulmonaryalveolarmicrolithiasis", "start_offset": 14, "end_offset": 45, "label": "dis" }, { "id": 3, "entity": "肺泡内", "start_offset": 47, "end_offset": 50, "label": "bod" }, { "id": 4, "entity": "钙", "start_offset": 53, "end_offset": 54, "label": "bod" } ]
病因不明,体内无钙、磷或其他代谢障碍。
[ { "id": 0, "entity": "体内无钙、磷或其他代谢障碍", "start_offset": 5, "end_offset": 18, "label": "sym" } ]
胸片示细砂粒、粟粒状播散钙化影,以中肺野及肺底部最明显,以后阴影于肺门处融合,并蔓延到肺尖及周边,有时肺尖部可见气肿性肺大疱。
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无特殊方法,以对症治疗及支持疗法为主,注意预防呼吸道感染。
[ { "id": 0, "entity": "对症治疗", "start_offset": 7, "end_offset": 11, "label": "pro" }, { "id": 1, "entity": "支持疗法", "start_offset": 12, "end_offset": 16, "label": "pro" }, { "id": 2, "entity": "呼吸道感染", "start_offset": 23, "end_offset": 28, "label": "dis" } ]
第五节风疹风疹(rubella,Germanmeasles)是由风疹病毒引起的急性出疹性传染病,以前驱期短、发热1~2天出疹及耳后、枕后和颈部淋巴结肿大为其临床特征。
[ { "id": 0, "entity": "风疹", "start_offset": 3, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "风疹", "start_offset": 5, "end_offset": 7, "label": "dis" }, { "id": 2, "entity": "rubella", "start_offset": 8, "end_offset": 15, "label": "dis" }, { "id": 3, "entity": "Germanmeasles", "start_offset": 16, "end_offset": 29, "label": "dis" }, { "id": 4, "entity": "风疹病毒", "start_offset": 32, "end_offset": 36, "label": "mic" }, { "id": 5, "entity": "急性出疹性传染病", "start_offset": 39, "end_offset": 47, "label": "dis" }, { "id": 6, "entity": "耳后", "start_offset": 63, "end_offset": 65, "label": "bod" }, { "id": 7, "entity": "枕后", "start_offset": 66, "end_offset": 68, "label": "bod" }, { "id": 8, "entity": "颈部淋巴结", "start_offset": 69, "end_offset": 74, "label": "bod" }, { "id": 9, "entity": "前驱期短、发热1~2天出疹及耳后、枕后和颈部淋巴结肿大", "start_offset": 49, "end_offset": 76, "label": "sym" } ]
胎儿早期感染可致严重先天畸形。
[ { "id": 0, "entity": "感染", "start_offset": 4, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "严重先天畸形", "start_offset": 8, "end_offset": 14, "label": "dis" } ]
【病原和流行病学】风疹病毒(rubellaVirus)属披膜病毒科,由核衣壳和包膜构成。
[ { "id": 0, "entity": "风疹病毒", "start_offset": 9, "end_offset": 13, "label": "mic" }, { "id": 1, "entity": "rubellaVirus", "start_offset": 14, "end_offset": 26, "label": "mic" }, { "id": 2, "entity": "披膜病毒", "start_offset": 28, "end_offset": 32, "label": "mic" } ]
病毒核酸为单股正链RNA。
[ { "id": 0, "entity": "病毒核酸", "start_offset": 0, "end_offset": 4, "label": "mic" } ]
包膜含2种蛋白,E1具凝血作用,能刺激机体产生中和抗体和血凝抑制抗体,E2抗原性不如E1强,亦能诱导中和抗体。
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此病毒较不稳定,可被脂溶剂、甲醛、紫外线、强酸和热等灭活,干燥冰冻保存9个月。
[ { "id": 0, "entity": "病毒", "start_offset": 1, "end_offset": 3, "label": "mic" }, { "id": 1, "entity": "脂溶剂", "start_offset": 10, "end_offset": 13, "label": "dru" }, { "id": 2, "entity": "甲醛", "start_offset": 14, "end_offset": 16, "label": "dru" } ]
风疹病毒可在各种猴、兔和人原代、传代及二倍体细胞中增殖。
[ { "id": 0, "entity": "风疹病毒", "start_offset": 0, "end_offset": 4, "label": "mic" } ]
病毒存在于患者或隐性感染者鼻咽分泌物、血、粪和尿中。
[ { "id": 0, "entity": "病毒", "start_offset": 0, "end_offset": 2, "label": "mic" }, { "id": 1, "entity": "隐性感染", "start_offset": 8, "end_offset": 12, "label": "sym" }, { "id": 2, "entity": "鼻咽分泌物", "start_offset": 13, "end_offset": 18, "label": "bod" }, { "id": 3, "entity": "血", "start_offset": 19, "end_offset": 20, "label": "bod" }, { "id": 4, "entity": "粪", "start_offset": 21, "end_offset": 22, "label": "bod" }, { "id": 5, "entity": "尿", "start_offset": 23, "end_offset": 24, "label": "bod" } ]
在出疹前7天和疹退后14天内可从鼻咽部毒。
[ { "id": 0, "entity": "出疹", "start_offset": 1, "end_offset": 3, "label": "sym" }, { "id": 1, "entity": "鼻咽", "start_offset": 16, "end_offset": 18, "label": "ite" }, { "id": 2, "entity": "鼻咽", "start_offset": 16, "end_offset": 18, "label": "bod" } ]
先天性风疹综合征患者生后排大量病毒可达数月至数年(1年者约11%)。
[ { "id": 0, "entity": "先天性风疹综合征", "start_offset": 0, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "病毒", "start_offset": 15, "end_offset": 17, "label": "mic" } ]
孕妇感染时,在病毒血症血症期可将病毒经胎盘传给胎儿。
[ { "id": 0, "entity": "感染", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "病毒血症", "start_offset": 7, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "病毒", "start_offset": 16, "end_offset": 18, "label": "mic" }, { "id": 3, "entity": "胎盘", "start_offset": 19, "end_offset": 21, "label": "bod" } ]
感染者排毒污染手或环境后,可经污染物—手—呼吸道或手—呼吸道途径传播病毒。
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我国育龄妇女风疹病毒IgG抗体阳性率不同地区调查结果不同,为76%~98%,平均88%。
[ { "id": 0, "entity": "风疹病毒IgG抗体", "start_offset": 6, "end_offset": 15, "label": "bod" } ]
早期妇女特异性IgM阳性率为0.46%。
[ { "id": 0, "entity": "特异性IgM", "start_offset": 4, "end_offset": 10, "label": "bod" } ]
【发病机制和病理改变】病毒侵入上呼吸道,在黏膜和颈部、颈下和耳后淋巴结内增殖,而后入血,形成2次病毒血症。
[ { "id": 0, "entity": "病毒", "start_offset": 11, "end_offset": 13, "label": "mic" }, { "id": 1, "entity": "上呼吸道", "start_offset": 15, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "黏膜", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 3, "entity": "颈部", "start_offset": 24, "end_offset": 26, "label": "bod" }, { "id": 4, "entity": "颈下", "start_offset": 27, "end_offset": 29, "label": "bod" }, { "id": 5, "entity": "耳", "start_offset": 30, "end_offset": 31, "label": "bod" }, { "id": 6, "entity": "淋巴结", "start_offset": 32, "end_offset": 35, "label": "bod" }, { "id": 7, "entity": "血", "start_offset": 42, "end_offset": 43, "label": "bod" }, { "id": 8, "entity": "病毒血症", "start_offset": 48, "end_offset": 52, "label": "dis" } ]
风疹病毒所致的抗原抗体复合物引起真皮上层毛细血管炎,形成皮疹。
[ { "id": 0, "entity": "风疹病毒", "start_offset": 0, "end_offset": 4, "label": "mic" }, { "id": 1, "entity": "真皮上层毛细血管炎", "start_offset": 16, "end_offset": 25, "label": "dis" }, { "id": 2, "entity": "皮疹", "start_offset": 28, "end_offset": 30, "label": "dis" } ]
淋巴结肿大,呼吸道见轻度炎症。
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先天性风疹的发病机制并不十分明确。
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风疹病毒导致血管内皮细胞受损是胎儿供血不足、组织细胞代谢失调和脏器发育不良的重要原因;病毒抑制感染细胞有丝分裂,致染色体断裂,使器官组织分化发育障碍;特异性免疫复合物和自身抗体形成可能是组织脏器损伤的另一机制。
[ { "id": 0, "entity": "风疹病毒", "start_offset": 0, "end_offset": 4, "label": "mic" }, { "id": 1, "entity": "血管内皮细胞", "start_offset": 6, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "血管内皮细胞受损", "start_offset": 6, "end_offset": 14, "label": "sym" }, { "id": 3, "entity": "血", "start_offset": 18, "end_offset": 19, "label": "bod" }, { "id": 4, "entity": "胎儿供血不足", "start_offset": 15, "end_offset": 21, "label": "sym" }, { "id": 5, "entity": "组织细胞", "start_offset": 22, "end_offset": 26, "label": "bod" }, { "id": 6, "entity": "组织细胞代谢失调", "start_offset": 22, "end_offset": 30, "label": "sym" }, { "id": 7, "entity": "脏器", "start_offset": 31, "end_offset": 33, "label": "bod" }, { "id": 8, "entity": "脏器发育不良", "start_offset": 31, "end_offset": 37, "label": "sym" }, { "id": 9, "entity": "病毒", "start_offset": 43, "end_offset": 45, "label": "mic" }, { "id": 10, "entity": "感染细胞", "start_offset": 47, "end_offset": 51, "label": "bod" }, { "id": 11, "entity": "染色体", "start_offset": 57, "end_offset": 60, "label": "bod" }, { "id": 12, "entity": "染色体断裂", "start_offset": 57, "end_offset": 62, "label": "sym" }, { "id": 13, "entity": "器官组织", "start_offset": 64, "end_offset": 68, "label": "bod" }, { "id": 14, "entity": "器官组织分化发育障碍", "start_offset": 64, "end_offset": 74, "label": "sym" }, { "id": 15, "entity": "组织脏器损伤", "start_offset": 93, "end_offset": 99, "label": "dis" } ]
风疹病毒持续性感染可解释患儿生后出现的迟发性疾病,如生后某时期出现的听力障碍、白内障及进行性全脑炎。
[ { "id": 0, "entity": "风疹病毒持续性感染", "start_offset": 0, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "迟发性疾病", "start_offset": 19, "end_offset": 24, "label": "dis" }, { "id": 2, "entity": "听力障碍", "start_offset": 34, "end_offset": 38, "label": "dis" }, { "id": 3, "entity": "白内障", "start_offset": 39, "end_offset": 42, "label": "dis" }, { "id": 4, "entity": "进行性全脑炎", "start_offset": 43, "end_offset": 49, "label": "dis" } ]
【临床表现】(一)后天性风疹潜伏期14~21天,平均18天。
[ { "id": 0, "entity": "后天性风疹", "start_offset": 9, "end_offset": 14, "label": "dis" } ]
可有低热、不适、轻微上呼吸道炎症表现,如咳嗽、流涕、结合膜充血和咽红等。
[ { "id": 0, "entity": "低热", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "上呼吸道炎症", "start_offset": 10, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "轻微上呼吸道炎症", "start_offset": 8, "end_offset": 16, "label": "sym" }, { "id": 3, "entity": "咳嗽", "start_offset": 20, "end_offset": 22, "label": "sym" }, { "id": 4, "entity": "流涕", "start_offset": 23, "end_offset": 25, "label": "sym" }, { "id": 5, "entity": "结合膜", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 6, "entity": "结合膜充血", "start_offset": 26, "end_offset": 31, "label": "sym" }, { "id": 7, "entity": "咽红", "start_offset": 32, "end_offset": 34, "label": "sym" } ]
软腭上可见细小红疹,能融合成片。
[ { "id": 0, "entity": "软腭", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "软腭上可见细小红疹,能融合成片", "start_offset": 0, "end_offset": 15, "label": "sym" } ]
2.出疹期通常于发热第1~2天开始出疹。
[ { "id": 0, "entity": "发热", "start_offset": 8, "end_offset": 10, "label": "sym" }, { "id": 1, "entity": "出疹", "start_offset": 17, "end_offset": 19, "label": "sym" } ]
皮疹首先见于脸部,然后迅速遍及颈部、躯干和四肢。
[ { "id": 0, "entity": "脸部", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "颈部", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "躯干", "start_offset": 18, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "四肢", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 4, "entity": "皮疹首先见于脸部,然后迅速遍及颈部、躯干和四肢", "start_offset": 0, "end_offset": 23, "label": "sym" } ]
出疹第一天末,全身遍布浅红色斑丘疹,略大于猩红热的疹点,散在分布。
[ { "id": 0, "entity": "全身遍布浅红色斑丘疹,略大于猩红热的疹点,散在分布", "start_offset": 7, "end_offset": 32, "label": "sym" } ]
次日脸部皮疹开始消退,很少脱皮。
[ { "id": 0, "entity": "脸部", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "次日脸部皮疹开始消退,很少脱皮", "start_offset": 0, "end_offset": 15, "label": "sym" } ]
另一典型表现为枕后、耳后或颈部淋巴结肿大,可在皮疹出现前发生,持续1周或更久。
[ { "id": 0, "entity": "枕后、耳后或颈部淋巴结肿大", "start_offset": 7, "end_offset": 20, "label": "dis" }, { "id": 1, "entity": "皮疹", "start_offset": 23, "end_offset": 25, "label": "sym" } ]
有的患者伴轻度脾脏肿大,可在3~4周后恢复正常。
[ { "id": 0, "entity": "轻度脾脏肿大", "start_offset": 5, "end_offset": 11, "label": "dis" } ]
部分患者可无皮疹,仅有淋巴结肿大。
[ { "id": 0, "entity": "皮疹", "start_offset": 6, "end_offset": 8, "label": "sym" }, { "id": 1, "entity": "淋巴结", "start_offset": 11, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "淋巴结肿大", "start_offset": 11, "end_offset": 16, "label": "sym" } ]
(二)先天性风疹综合征先天感染风疹病毒后可发生死胎、流产、畸形新生儿;或在出生时正常,以后出现病损,也可为隐性感染。
[ { "id": 0, "entity": "先天性风疹综合征", "start_offset": 3, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "风疹病毒", "start_offset": 15, "end_offset": 19, "label": "mic" }, { "id": 2, "entity": "死胎", "start_offset": 23, "end_offset": 25, "label": "dis" }, { "id": 3, "entity": "流产", "start_offset": 26, "end_offset": 28, "label": "dis" }, { "id": 4, "entity": "畸形", "start_offset": 29, "end_offset": 31, "label": "dis" }, { "id": 5, "entity": "隐性感染", "start_offset": 53, "end_offset": 57, "label": "sym" } ]
重者出生时有低体重、肝脾肿大、血小板减少性紫癜、先天性心脏病、白内障、小头畸形、骨发育不良和脑脊液异常等。
[ { "id": 0, "entity": "低体重", "start_offset": 6, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "肝脾", "start_offset": 10, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "肝脾肿大", "start_offset": 10, "end_offset": 14, "label": "sym" }, { "id": 3, "entity": "血小板", "start_offset": 15, "end_offset": 18, "label": "bod" }, { "id": 4, "entity": "血小板减少性紫癜", "start_offset": 15, "end_offset": 23, "label": "sym" }, { "id": 5, "entity": "先天性心脏病", "start_offset": 24, "end_offset": 30, "label": "dis" }, { "id": 6, "entity": "白内障", "start_offset": 31, "end_offset": 34, "label": "dis" }, { "id": 7, "entity": "小头畸形", "start_offset": 35, "end_offset": 39, "label": "dis" }, { "id": 8, "entity": "骨", "start_offset": 40, "end_offset": 41, "label": "bod" }, { "id": 9, "entity": "骨发育不良", "start_offset": 40, "end_offset": 45, "label": "sym" }, { "id": 10, "entity": "脑脊液", "start_offset": 46, "end_offset": 49, "label": "bod" }, { "id": 11, "entity": "脑脊液异常", "start_offset": 46, "end_offset": 51, "label": "sym" } ]
迟发性疾病包括听力丧失、内分泌病、白内障或青光眼和进行性全脑炎。
[ { "id": 0, "entity": "听力丧失", "start_offset": 7, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "内分泌病", "start_offset": 12, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "白内障", "start_offset": 17, "end_offset": 20, "label": "dis" }, { "id": 3, "entity": "青光眼", "start_offset": 21, "end_offset": 24, "label": "dis" }, { "id": 4, "entity": "进行性全脑炎", "start_offset": 25, "end_offset": 31, "label": "dis" } ]
【病原学诊断】(一)病毒分离普通风疹患者取出疹前5天至出疹后6天鼻咽分泌物分离病毒的阳性率较高。
[ { "id": 0, "entity": "病毒", "start_offset": 10, "end_offset": 12, "label": "mic" }, { "id": 1, "entity": "风疹", "start_offset": 16, "end_offset": 18, "label": "dis" }, { "id": 2, "entity": "鼻咽分泌物", "start_offset": 32, "end_offset": 37, "label": "bod" }, { "id": 3, "entity": "病毒", "start_offset": 39, "end_offset": 41, "label": "mic" } ]
孕妇原发感染后,取羊水或胎盘绒毛分离病毒是诊断胎儿风疹病毒感染最可靠的方法之一。
[ { "id": 0, "entity": "原发感染", "start_offset": 2, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "羊水", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "胎盘绒毛", "start_offset": 12, "end_offset": 16, "label": "bod" }, { "id": 3, "entity": "病毒", "start_offset": 18, "end_offset": 20, "label": "mic" }, { "id": 4, "entity": "胎儿风疹病毒感染", "start_offset": 23, "end_offset": 31, "label": "dis" } ]
先天性风疹患儿常采集鼻咽分泌物、尿、脑脊液、骨髓等标本分离病毒。
[ { "id": 0, "entity": "先天性风疹", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "鼻咽分泌物", "start_offset": 10, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "尿", "start_offset": 16, "end_offset": 17, "label": "bod" }, { "id": 3, "entity": "脑脊液", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 4, "entity": "骨髓", "start_offset": 22, "end_offset": 24, "label": "bod" }, { "id": 5, "entity": "病毒", "start_offset": 29, "end_offset": 31, "label": "mic" } ]
(二)特异性抗体检测特异性IgM在出疹时出现,疹后5~14天水平较高,持续约30天,是近期感染的指标。
[ { "id": 0, "entity": "特异性抗体检测", "start_offset": 3, "end_offset": 10, "label": "pro" }, { "id": 1, "entity": "特异性IgM", "start_offset": 10, "end_offset": 16, "label": "ite" }, { "id": 2, "entity": "出疹", "start_offset": 17, "end_offset": 19, "label": "sym" }, { "id": 3, "entity": "感染", "start_offset": 45, "end_offset": 47, "label": "sym" } ]
双份血清(间隔1~2周采血)特异性IgG≥4倍升高有诊断意义。
[ { "id": 0, "entity": "血清", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "采血", "start_offset": 11, "end_offset": 13, "label": "pro" }, { "id": 2, "entity": "特异性IgG", "start_offset": 14, "end_offset": 20, "label": "ite" } ]
先天性风疹患儿特异性IgM在生后6个月内持续升高,1岁以内均可检测到。
[ { "id": 0, "entity": "先天性风疹", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "特异性IgM", "start_offset": 7, "end_offset": 13, "label": "ite" } ]
胎血中检出特异性IgM可证实胎儿感染,但由于胎儿在20周后才能产生IgM,故妊娠头3个月胎儿的风疹病毒感染不能依靠IgM检测。
[ { "id": 0, "entity": "胎血", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "特异性IgM", "start_offset": 5, "end_offset": 11, "label": "ite" }, { "id": 2, "entity": "胎儿", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 3, "entity": "胎儿", "start_offset": 22, "end_offset": 24, "label": "bod" }, { "id": 4, "entity": "IgM", "start_offset": 33, "end_offset": 36, "label": "ite" }, { "id": 5, "entity": "胎儿的风疹病毒感染", "start_offset": 44, "end_offset": 53, "label": "dis" }, { "id": 6, "entity": "IgM检测", "start_offset": 57, "end_offset": 62, "label": "pro" } ]
(三)病毒抗原检测采用免疫印迹法检测胎盘绒毛或胎儿活检标本中风疹病毒抗原。
[ { "id": 0, "entity": "病毒抗原检测", "start_offset": 3, "end_offset": 9, "label": "pro" }, { "id": 1, "entity": "免疫印迹法", "start_offset": 11, "end_offset": 16, "label": "pro" }, { "id": 2, "entity": "胎盘绒毛", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "胎儿活检", "start_offset": 23, "end_offset": 27, "label": "pro" }, { "id": 4, "entity": "风疹病毒抗原", "start_offset": 30, "end_offset": 36, "label": "ite" } ]
(四)病毒基因测定采用核酸杂交技术或PCR法检测羊水、绒毛膜或绒毛中病毒基因。
[ { "id": 0, "entity": "病毒基因测定", "start_offset": 3, "end_offset": 9, "label": "pro" }, { "id": 1, "entity": "核酸杂交技术", "start_offset": 11, "end_offset": 17, "label": "pro" }, { "id": 2, "entity": "PCR法", "start_offset": 18, "end_offset": 22, "label": "pro" }, { "id": 3, "entity": "羊水、绒毛膜或绒毛中病毒基因", "start_offset": 24, "end_offset": 38, "label": "ite" } ]
【预防和治疗】(一)一般预防预防重点是妊娠期妇女,尤其在孕早期,无论是否患过风疹或接种过风疹疫苗,均应尽量避免与风疹患者接触,以免感染或再感染。
[ { "id": 0, "entity": "风疹", "start_offset": 38, "end_offset": 40, "label": "dis" }, { "id": 1, "entity": "接种", "start_offset": 41, "end_offset": 43, "label": "pro" }, { "id": 2, "entity": "风疹疫苗", "start_offset": 44, "end_offset": 48, "label": "dru" }, { "id": 3, "entity": "风疹", "start_offset": 56, "end_offset": 58, "label": "dis" }, { "id": 4, "entity": "感染", "start_offset": 65, "end_offset": 67, "label": "sym" }, { "id": 5, "entity": "感染", "start_offset": 69, "end_offset": 71, "label": "sym" } ]
(二)主动免疫风疹减毒活疫苗已广泛应用,接种者95%产生抗体。
[ { "id": 0, "entity": "风疹减毒活疫苗", "start_offset": 7, "end_offset": 14, "label": "dru" }, { "id": 1, "entity": "抗体", "start_offset": 28, "end_offset": 30, "label": "bod" } ]
不良反应主要有小关节疼痛、一过性发热或皮疹。
[ { "id": 0, "entity": "小关节", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "小关节疼痛", "start_offset": 7, "end_offset": 12, "label": "sym" }, { "id": 2, "entity": "一过性发热", "start_offset": 13, "end_offset": 18, "label": "sym" }, { "id": 3, "entity": "皮疹", "start_offset": 19, "end_offset": 21, "label": "sym" } ]
尚无疫苗致畸的证据。
[ { "id": 0, "entity": "疫苗", "start_offset": 2, "end_offset": 4, "label": "dru" }, { "id": 1, "entity": "畸", "start_offset": 5, "end_offset": 6, "label": "dis" } ]
(三)被动免疫妊娠早期孕妇于接触风疹患者3天内肌内注射高效价免疫球蛋白20ml,可起到预防作用。
[ { "id": 0, "entity": "风疹", "start_offset": 16, "end_offset": 18, "label": "dis" }, { "id": 1, "entity": "肌内注射高效价免疫球蛋白", "start_offset": 23, "end_offset": 35, "label": "pro" } ]
(四)治疗主要为对症治疗,宜卧床休息,给予富营养又易消化的食物。
[ { "id": 0, "entity": "卧床休息", "start_offset": 14, "end_offset": 18, "label": "pro" }, { "id": 1, "entity": "给予富营养又易消化的食物", "start_offset": 19, "end_offset": 31, "label": "pro" } ]
可给清热解毒类中药。
[ { "id": 0, "entity": "给清热解毒类中药", "start_offset": 1, "end_offset": 9, "label": "pro" } ]
对先天性风疹综合征患者的各种缺陷,应作相应处理。
[ { "id": 0, "entity": "先天性风疹综合征", "start_offset": 1, "end_offset": 9, "label": "dis" } ]
二、幼年型慢性粒细胞白血病【临床特征】幼年型慢性粒细胞白血病(juvenilechronicmyelogenousleukemia,JCML)在FAB分类中划入骨髓增生异常综合征(MDS),与CML一样、JCML累及多能造血干细胞,外周血白细胞数增高、脾大、碱性磷酸酶活力减低CML来说尚有以下特征:外周血单核细胞比例>10%,幼稚细胞比例<5%;骨髓未成熟单核细胞增高,幼稚细胞比例<30%;Hb-F升高;高丙种球蛋白血症。
[ { "id": 0, "entity": "幼年型慢性粒细胞白血病", "start_offset": 2, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "幼年型慢性粒细胞白血病", "start_offset": 19, "end_offset": 30, "label": "dis" }, { "id": 2, "entity": "juvenilechronicmyelogenousleukemia", "start_offset": 31, "end_offset": 65, "label": "dis" }, { "id": 3, "entity": "JCML", "start_offset": 66, "end_offset": 70, "label": "dis" }, { "id": 4, "entity": "骨髓增生异常综合征", "start_offset": 80, "end_offset": 89, "label": "dis" }, { "id": 5, "entity": "MDS", "start_offset": 90, "end_offset": 93, "label": "dis" }, { "id": 6, "entity": "CML", "start_offset": 96, "end_offset": 99, "label": "dis" }, { "id": 7, "entity": "JCML", "start_offset": 102, "end_offset": 106, "label": "dis" }, { "id": 8, "entity": "多能造血干细胞", "start_offset": 108, "end_offset": 115, "label": "bod" }, { "id": 9, "entity": "白细胞", "start_offset": 119, "end_offset": 122, "label": "bod" }, { "id": 10, "entity": "脾", "start_offset": 126, "end_offset": 127, "label": "bod" }, { "id": 11, "entity": "碱性磷酸酶", "start_offset": 129, "end_offset": 134, "label": "bod" }, { "id": 12, "entity": "外周血白细胞数增高、脾大、碱性磷酸酶活力减低", "start_offset": 116, "end_offset": 138, "label": "sym" }, { "id": 13, "entity": "CML", "start_offset": 138, "end_offset": 141, "label": "dis" }, { "id": 14, "entity": "外周血单核细胞", "start_offset": 150, "end_offset": 157, "label": "ite" }, { "id": 15, "entity": "幼稚细胞", "start_offset": 164, "end_offset": 168, "label": "ite" }, { "id": 16, "entity": "骨髓未成熟单核细胞增高", "start_offset": 174, "end_offset": 185, "label": "sym" }, { "id": 17, "entity": "幼稚细胞", "start_offset": 186, "end_offset": 190, "label": "ite" }, { "id": 18, "entity": "Hb-F升高", "start_offset": 197, "end_offset": 203, "label": "sym" }, { "id": 19, "entity": "高丙种球蛋白血症", "start_offset": 204, "end_offset": 212, "label": "dis" } ]
MDS伴有7号染色体单体时与JCML临床表现相似,但Hb-F不高。
[ { "id": 0, "entity": "MDS", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "染色体单体", "start_offset": 7, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "JCML", "start_offset": 14, "end_offset": 18, "label": "dis" }, { "id": 3, "entity": "Hb-F不高", "start_offset": 26, "end_offset": 32, "label": "sym" } ]
JCML与CML的鉴别见表11-11。
[ { "id": 0, "entity": "JCML", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "CML", "start_offset": 5, "end_offset": 8, "label": "dis" } ]
表11-11JCML与成人型CML的鉴别【治疗与疗效】化疗疗效有限,可单独用6-巯基鸟嘌呤或与阿糖胞苷联合应用。
[ { "id": 0, "entity": "JCML", "start_offset": 6, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "成人型CML", "start_offset": 11, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "化疗", "start_offset": 27, "end_offset": 29, "label": "pro" }, { "id": 3, "entity": "6-巯基鸟嘌呤", "start_offset": 38, "end_offset": 45, "label": "dru" }, { "id": 4, "entity": "阿糖胞苷", "start_offset": 47, "end_offset": 51, "label": "dru" } ]
在多数病例中单纯的对症及支持治疗与采用化疗者生存期相似。
[ { "id": 0, "entity": "化疗", "start_offset": 19, "end_offset": 21, "label": "pro" } ]
联合强化疗在少数病人中取得达2年的缓解期。
[ { "id": 0, "entity": "化疗", "start_offset": 3, "end_offset": 5, "label": "pro" } ]
移植后复发仍是个棘手的问题。
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第十节原发性膀胱输尿管反流正常的输尿管膀胱连接部具有活瓣样功能,其只允许尿液从输尿管流进膀胱,而不允许尿液从膀胱向输尿管的倒流。
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输尿管膀胱连接部抗反流机制在公元150年已被认识到,1883年Pozzi在动物实验和人体中观察到膀胱输尿管反流,但尚不能确定是否为异常现象。
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其后Bumpus和Gruber等发现反流与泌尿系感染之间的关系,但当时更多的认为膀胱输尿管反流是继发于膀胱颈部梗阻,故1950年前后,人们施行了许多不必要的手术如膀胱颈部成形去解除并不存在的膀胱颈梗阻。
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从20世纪60年代起,出现了大量关于膀胱输尿管反流与尿路感染和肾瘢痕之间关系的研究,并逐步认识到膀胱输尿管反流可导致高血压和肾衰竭。
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【发病率】原发性膀胱输尿管反流在正常婴儿和儿童的发生率很难知道,统计的发生率为1%~18.5%,但70%发生在尿路感染的患儿中。
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反流有一定的性别倾向,女孩占85%,男孩如有尿路感染其反流可能性大,包皮环切对其发生也有影响,环切者,反流率低。
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年龄与反流发生率成反比,4岁20%,12岁15%,成人5.2%,由此也提示随着膀胱及输尿管的发育(输尿管隧道延长),反流可自然缓解。
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【病因和机制】1.输尿管膀胱连接部正常解剖和抗反流机制输尿管全部的肌层几乎都是由松散的、不规则的螺旋形肌纤维构成,只有膀胱壁段输尿管的肌纤维才是纵行。
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进入膀胱后肌纤维成扇形构成三角区肌肉的浅层,并向前延伸达后尿道精阜部。
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输尿管穿入膀胱壁时由一纤维鞘(Waldeyer)包绕,位于膀胱黏膜下,使其能适应膀胱的充盈和空虚状态。
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输尿管膀胱连接部的活瓣作用取决于膀胱内黏膜下段输尿管的长度和三角区肌层保持这个长度的能力,以及膀胱逼尿肌对该段输尿管后壁足够的支撑作用。
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当膀胱内压上升时,黏膜下段输尿管被压缩而不产生反流,这种活瓣机制是被动的。
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但输尿管的蠕动能力和输尿管口的关闭能力在防止反流中也起一部分作用。
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2.反流原因输尿管膀胱连接部的先天性异常,主要是输尿管膀胱壁内段的纵行肌肉发育不良,致使输尿管口外移,黏膜下段输尿管缩短,从而失去抗反流的能力。
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另一原因是黏膜下段输尿管的长度与其口径不相称。
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正常无反流时,黏膜下段输尿管的长度与其直径的比例为5∶1,而有反流者仅为1.4∶1。
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【反流分级】一般根据排尿性膀胱尿道造影(VCUG)的结果,将原发性膀胱输尿管反流分为反流仅达输尿管-12):Ⅰ级反流仅达输尿管;Ⅱ级反流至肾盂肾盏,但输尿管无扩张;Ⅲ级输尿管轻度扩张或(和)弯曲,肾盂轻度扩张和穹隆轻度变钝;轻度扩张或(和)弯曲,肾盂轻度扩张和穹隆轻度变钝;Ⅳ级输尿管中度扩张和弯曲,肾盂肾盏中度扩张,但多数肾盏仍维持乳头形态;Ⅴ级输尿管严重扩张和迂曲,肾盂肾盏严重扩张,多数肾盏中乳头形态消失。
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【反流演进】反流使部分尿液在膀胱排空后仍停留在输尿管中,从而为细菌从膀胱上行到肾脏提供了通路,因此反流常并发尿路感染,可表现为急性肾盂肾炎或无症状的慢性肾盂肾炎过程,80%的反流肾其组织学改变与肾盂肾炎一致。
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肾瘢痕的产生与反流的严重程度相关,反流越严重,发生瘢痕进展或新瘢痕的机会越高。
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新生儿及婴儿的集合管相对粗大,易于发生肾内反流,故患重度反流的小婴儿更易产生肾瘢痕。
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图12-7膀胱输尿管反流分级反流对肾功能的影响与尿路不完全性梗阻对肾脏的影响是一样的。
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反流时上尿路内压增加,远端肾单位首受其害,因此肾小管功能受损早于肾小球。
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无菌反流影响肾小管的浓缩能力,且持续时间较长。
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肾小球功能在有肾实质损害时受影响,并与肾实质损害的程度成正比。
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反流可以影响肾脏的发育,如抑制其胚胎发生,导致肾发育不全或肾发育异常或肾发育异常;而长期反流的患儿发生肾脏不生长等情况。
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反流病人发生高血压的机会较高。
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如反流未能有效控制,肾瘢痕进行性发展可导致肾衰竭。
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原发性膀胱输尿管反流一般随年龄增长逐渐好转,可能是因输尿管膀胱壁内段和膀胱三角区肌肉的生长和成熟之故。
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如静脉尿路造影显示输尿管口径正常,原发反流85%可自然消失。
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反流的自然消失与反流累及的范围也有关系,单侧反流自然消失率可达65%;双侧反流,输尿管无扩张者,自然消失率是50%,有输尿管扩张的仅10%左右。
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