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近年,随着生活水平的提高,人们食不厌精、脍不厌细,使维生素B<sub>1</sub>缺乏发病率有升高趋势,使乳母及婴幼儿体内的维生素B<sub>1</sub>严重不足。
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许秀举等抽样选择包头郊区4个地区的6~8岁儿童409名,采用荧光法测定其4小时负荷尿中维生素B<sub>1</sub>的含量,结果缺乏率为14.42%,不足率为18.58%。
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新华医院对25例以夜寐不安为主要表现,同时伴有烦躁、食欲缺乏的<2岁小儿进行临床观察和红细胞转酮醇酶活力(TPP效应)的测定,结果显示16%的小儿属边缘型维生素B<sub>1</sub>缺乏,20%属严重缺乏。
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【病因】(一)摄入不足母乳中维生素B<sub>1</sub>的含量较牛乳低,母乳中的含量为16μg/ml,牛乳中的含量为42μg/ml,但母乳中的维生素B<sub>1</sub>含量,对婴儿的生长需要已足够。
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但如果乳母膳食中维生素B<sub>1</sub>的摄入量缺乏,则会引起母乳中的维生素B<sub>1</sub>不足,如不及时补充,也将引起婴儿维生素B<sub>1</sub>缺乏症。
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对于已添加辅食的小儿,如长期使用精白米、面以及淀粉为主食,或煮饭时为增加其黏稠度而加入少量的碱,将破坏维生素B<sub>1</sub>。
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(二)吸收障碍如患有消化系统疾病,如慢性腹泻、慢性痢疾、胆囊纤维化、肠道感染等疾病,均可减少维生素B<sub>1</sub>的吸收。
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肝、肾疾病将影响TPP的合成,造成维生素B<sub>1</sub>缺乏。
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维生素B<sub>1</sub>缺乏使胃液中酸度降低,从而在胃肠道中维生素B<sub>1</sub>复合物内的维生素B<sub>1</sub>释放减少,影响了维生素B<sub>1</sub>的吸收。
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(三)维生素B<sub>1</sub>药物的需要量增加儿童生长发育速度较快,需要量也相对较多;如小儿患结核、麻疹、水痘、肺炎以及高热时,或患有如甲状腺功能亢进等代谢率增加的疾病时,维生素B<sub>1</sub>的消耗增加,如此时未予及时补充,则造成维生素B<sub>1</sub>的缺乏。
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(四)遗传代谢障碍遗传性维生素B<sub>1</sub>代谢与功能障碍引起的维生素B<sub>1</sub>缺乏症,一般具有高度的家族性遗传性疾病史,或父母近亲结婚史。
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【病理生理】在身体中,硫胺80%是以TPP的形式存在,它是丙酮酸氧化脱羧酶系的辅助因子,也是磷酸己糖氧化支路中转羧乙醛酶的辅酶。
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因此,维生素B<sub>1</sub>与糖代谢密切相关,其缺乏使糖代谢受阻,能量产生减少,会产生一系列的病理变化。
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(一)神经系统尤其是末梢神经受损严重、髓鞘退化及色素沉着。
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中枢神经系统和周围神经系统的神经纤维的髓鞘发育不良,因此表现为易激惹。
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重者神经轴被破坏,以坐骨神经及其分支受累较为常见,并且出现较早。
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其他如前臂神经等亦可累及。
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(二)心血管系统由于能量缺乏,心肌无力,严重时发生心力衰竭,周围血管平滑肌张力下降,小血管扩张。
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心脏扩张肥厚,尤以右心明显。
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心肌水肿,其心肌纤维粗硬。
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血管充血,但组织结构正常。
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(三)组织水肿及浆膜腔积液组织水肿多见于下肢,当体腔浆液渗出时,可见心包腔、胸腔及腹腔积液。
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(四)肌肉萎缩出现于受累神经支配的肌肉。
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镜下可见肌纤维横纹消失、混浊肿胀及脂肪变性。
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(五)消化系统消化道平滑肌张力下降,影响胃肠蠕动,消化功能减弱。
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【临床表现】维生素B<sub>1</sub>缺乏将导致脚气病。
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脚气病是维生素B<sub>1</sub>摄入不足的最终结果。
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本病主要影响心血管和神经系统。
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主要表现为多发性神经炎、肌肉萎缩、组织水肿、心脏扩大、循环失调及胃肠症状。
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消化系统症状:发病初期主要表现为消化系统症状,如食欲缺乏、厌食、恶心、呕吐、腹痛、便秘或腹泻。
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神经系统症状:消化道系统症状出现后不久就出现神经系统症状,神经系统症状突出者可分为脑型或神经炎型。
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跟腱反射和膝反射初期增强,随后减弱,最后消失。
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心血管症状:出现心悸、心动过速,婴儿可出现奔马律,呼吸困难,晚期出现发绀、心脏扩大、心力衰竭、肺充血及肝淤血。
[ { "id": 0, "entity": "心血管", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "心", "start_offset": 8, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "心悸", "start_offset": 8, "end_offset": 10, "label": "sym" }, { "id": 3, "entity": "心", "start_offset": 11, "end_offset": 12, "label": "bod" }, { "id": 4, "entity": "心动过速", "start_offset": 11, "end_offset": 15, "label": "sym" }, { "id": 5, "entity": "奔马律", "start_offset": 21, "end_offset": 24, "label": "sym" }, { "id": 6, "entity": "呼吸困难", "start_offset": 25, "end_offset": 29, "label": "sym" }, { "id": 7, "entity": "发绀", "start_offset": 34, "end_offset": 36, "label": "sym" }, { "id": 8, "entity": "心脏", "start_offset": 37, "end_offset": 39, "label": "bod" }, { "id": 9, "entity": "心脏扩大", "start_offset": 37, "end_offset": 41, "label": "sym" }, { "id": 10, "entity": "心", "start_offset": 42, "end_offset": 43, "label": "bod" }, { "id": 11, "entity": "心力衰竭", "start_offset": 42, "end_offset": 46, "label": "sym" }, { "id": 12, "entity": "肺", "start_offset": 47, "end_offset": 48, "label": "bod" }, { "id": 13, "entity": "肺充血", "start_offset": 47, "end_offset": 50, "label": "sym" }, { "id": 14, "entity": "肝", "start_offset": 51, "end_offset": 52, "label": "bod" }, { "id": 15, "entity": "肝淤血", "start_offset": 51, "end_offset": 54, "label": "sym" } ]
如不及时治疗,很快死亡。
[ { "id": 0, "entity": "死亡", "start_offset": 9, "end_offset": 11, "label": "sym" } ]
水肿及浆膜腔积液:水肿可遍及全身,多发生于下肢,浆膜腔积液,可发生于心包腔、胸腔和腹腔。
[ { "id": 0, "entity": "水肿", "start_offset": 0, "end_offset": 2, "label": "sym" }, { "id": 1, "entity": "浆膜腔", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 2, "entity": "浆膜腔积液", "start_offset": 3, "end_offset": 8, "label": "sym" }, { "id": 3, "entity": "水肿", "start_offset": 9, "end_offset": 11, "label": "sym" }, { "id": 4, "entity": "下肢", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 5, "entity": "浆膜腔", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 6, "entity": "浆膜腔积液", "start_offset": 24, "end_offset": 29, "label": "sym" }, { "id": 7, "entity": "心包腔", "start_offset": 34, "end_offset": 37, "label": "bod" }, { "id": 8, "entity": "胸腔", "start_offset": 38, "end_offset": 40, "label": "bod" }, { "id": 9, "entity": "腹腔", "start_offset": 41, "end_offset": 43, "label": "bod" } ]
由于喉的水肿而出现失声,或出现特殊的喉鸣(脚气病哭声)。
[ { "id": 0, "entity": "喉", "start_offset": 2, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "水肿", "start_offset": 4, "end_offset": 6, "label": "sym" }, { "id": 2, "entity": "失声", "start_offset": 9, "end_offset": 11, "label": "sym" }, { "id": 3, "entity": "特殊的喉鸣", "start_offset": 15, "end_offset": 20, "label": "sym" }, { "id": 4, "entity": "脚气病", "start_offset": 21, "end_offset": 24, "label": "dis" }, { "id": 5, "entity": "脚气病哭声", "start_offset": 21, "end_offset": 26, "label": "sym" } ]
先天性维生素B<sub>1</sub>代谢缺陷有关的遗传性疾病包括枫糖尿症、婴儿慢性乳酸酸中毒、婴儿及儿童的亚急性坏死性脑病及对维生素B<sub>1</sub>有反应的巨幼红细胞贫血。
[ { "id": 0, "entity": "先天性维生素B<sub>1</sub>代谢缺陷", "start_offset": 0, "end_offset": 23, "label": "dis" }, { "id": 1, "entity": "遗传性疾病", "start_offset": 26, "end_offset": 31, "label": "dis" }, { "id": 2, "entity": "枫糖尿症", "start_offset": 33, "end_offset": 37, "label": "dis" }, { "id": 3, "entity": "婴儿慢性乳酸酸中毒", "start_offset": 38, "end_offset": 47, "label": "dis" }, { "id": 4, "entity": "亚急性坏死性脑病", "start_offset": 54, "end_offset": 62, "label": "dis" }, { "id": 5, "entity": "维生素B<sub>1</sub>", "start_offset": 64, "end_offset": 80, "label": "bod" }, { "id": 6, "entity": "巨幼红细胞贫血", "start_offset": 84, "end_offset": 91, "label": "dis" } ]
1.枫糖尿症枫糖尿病的病因是由于缺乏支链α-酮酸脱氢酶复合物,患者的相应α-酮酸不能通过氧化脱羧作用而降解,而引起支链氨基酸(亮氨酸、异亮氨酸、缬氨酸)代谢异常。
[ { "id": 0, "entity": "枫糖尿症", "start_offset": 2, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "枫糖尿病", "start_offset": 6, "end_offset": 10, "label": "dis" }, { "id": 2, "entity": "支链α-酮酸脱氢酶复合物", "start_offset": 18, "end_offset": 30, "label": "bod" }, { "id": 3, "entity": "α-酮酸", "start_offset": 36, "end_offset": 40, "label": "bod" } ]
此病是常染色体隐性遗传性疾病,可出现精神及身体发育延迟、嗜睡、喂养困难、注意力减退、肌张力交替性升高和减弱。
[ { "id": 0, "entity": "常染色体隐性遗传性疾病", "start_offset": 3, "end_offset": 14, "label": "dis" }, { "id": 1, "entity": "精神及身体发育延迟", "start_offset": 18, "end_offset": 27, "label": "sym" }, { "id": 2, "entity": "嗜睡", "start_offset": 28, "end_offset": 30, "label": "sym" }, { "id": 3, "entity": "喂养困难", "start_offset": 31, "end_offset": 35, "label": "sym" }, { "id": 4, "entity": "注意力减退", "start_offset": 36, "end_offset": 41, "label": "sym" }, { "id": 5, "entity": "肌张力交替性升高和减弱", "start_offset": 42, "end_offset": 53, "label": "sym" } ]
给予口服大剂量维生素B<sub>1</sub>治疗,可减轻临床症状,血清支链氨基酸水平恢复正常,如停止给予维生素B<sub>1</sub>时,血清支链氨基酸水平再度升高。
[ { "id": 0, "entity": "口服", "start_offset": 2, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "维生素B<sub>1</sub>", "start_offset": 7, "end_offset": 23, "label": "dru" }, { "id": 2, "entity": "维生素B<sub>1</sub>", "start_offset": 53, "end_offset": 69, "label": "dru" } ]
2.婴儿慢性乳酸酸中毒主要以乳酸和丙酮酸酸中毒、神经性异常以及发育迟缓为特征。
[ { "id": 0, "entity": "婴儿慢性乳酸酸中毒", "start_offset": 2, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "乳酸和丙酮酸酸中毒", "start_offset": 14, "end_offset": 23, "label": "dis" }, { "id": 2, "entity": "神经性异常", "start_offset": 24, "end_offset": 29, "label": "sym" }, { "id": 3, "entity": "发育迟缓", "start_offset": 31, "end_offset": 35, "label": "sym" } ]
对大剂量维生素B<sub>1</sub>治疗有效者考虑为维生素B<sub>1</sub>代谢有缺陷,对维生素B<sub>1</sub>无效者可能为丙酮酸脱羧酶缺少。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 4, "end_offset": 20, "label": "dru" }, { "id": 1, "entity": "维生素B<sub>1</sub>", "start_offset": 28, "end_offset": 44, "label": "bod" }, { "id": 2, "entity": "维生素B<sub>1</sub>代谢有缺陷", "start_offset": 28, "end_offset": 49, "label": "sym" }, { "id": 3, "entity": "维生素B<sub>1</sub>", "start_offset": 51, "end_offset": 67, "label": "dru" }, { "id": 4, "entity": "丙酮酸脱羧酶缺少", "start_offset": 73, "end_offset": 81, "label": "dis" } ]
但有文献报道,丙酮酸脱羧酶缺少的婴儿,接受大剂量维生素B<sub>1</sub>治疗后好转。
[ { "id": 0, "entity": "丙酮酸脱羧酶缺少", "start_offset": 7, "end_offset": 15, "label": "dis" }, { "id": 1, "entity": "维生素B<sub>1</sub>", "start_offset": 24, "end_offset": 40, "label": "dru" } ]
3.婴儿及儿童的亚急性坏死型脑病(Leighs脑病)是婴儿期和儿童发育早期的一种致命性疾病,伴有虚弱、厌食、说话和眼球震颤、抽搐、瘫痪及复合感觉障碍,甚至生长停止。
[ { "id": 0, "entity": "亚急性坏死型脑病", "start_offset": 8, "end_offset": 16, "label": "dis" }, { "id": 1, "entity": "Leighs脑病", "start_offset": 17, "end_offset": 25, "label": "dis" }, { "id": 2, "entity": "虚弱", "start_offset": 48, "end_offset": 50, "label": "sym" }, { "id": 3, "entity": "厌食", "start_offset": 51, "end_offset": 53, "label": "sym" }, { "id": 4, "entity": "说话和眼球震颤", "start_offset": 54, "end_offset": 61, "label": "sym" }, { "id": 5, "entity": "抽搐", "start_offset": 62, "end_offset": 64, "label": "sym" }, { "id": 6, "entity": "瘫痪", "start_offset": 65, "end_offset": 67, "label": "sym" }, { "id": 7, "entity": "复合感觉障碍", "start_offset": 68, "end_offset": 74, "label": "sym" }, { "id": 8, "entity": "生长停止", "start_offset": 77, "end_offset": 81, "label": "sym" } ]
其血中的乳酸和丙酮酸升高,机制目前仍不详,考虑与TPP降低有关。
[ { "id": 0, "entity": "血", "start_offset": 1, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "乳酸", "start_offset": 4, "end_offset": 6, "label": "bod" }, { "id": 2, "entity": "丙酮酸", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 3, "entity": "TPP", "start_offset": 24, "end_offset": 27, "label": "bod" } ]
4.对维生素B<sub>1</sub>有反应的巨幼红细胞贫血是婴儿期和儿童期的一种罕见疾病,其特点是巨幼红细胞性贫血,并伴有感觉神经性耳聋和糖尿病,也可能出现心脏异常。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 3, "end_offset": 19, "label": "bod" }, { "id": 1, "entity": "巨幼红细胞贫血", "start_offset": 23, "end_offset": 30, "label": "dis" }, { "id": 2, "entity": "巨幼红细胞性贫血", "start_offset": 50, "end_offset": 58, "label": "dis" }, { "id": 3, "entity": "感觉神经性耳聋", "start_offset": 62, "end_offset": 69, "label": "dis" }, { "id": 4, "entity": "糖尿病", "start_offset": 70, "end_offset": 73, "label": "dis" }, { "id": 5, "entity": "心脏异常", "start_offset": 79, "end_offset": 83, "label": "dis" } ]
此病与继发于维生素B<sub>1</sub>在细胞内的转运和吸收障碍所引起的维生素B<sub>1</sub>缺乏状态有关。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 6, "end_offset": 22, "label": "bod" }, { "id": 1, "entity": "维生素B<sub>1</sub>缺乏", "start_offset": 38, "end_offset": 56, "label": "dis" } ]
【维生素B<sub>1</sub>营养水平评价】评价维生素B<sub>1</sub>的营养状况,可通过测定维生素B<sub>1</sub>负荷前后的尿维生素B<sub>1</sub>排泄量,血清维生素B<sub>1</sub>水平、红细胞转酮醇酶(ETK)活性及空腹一次测定尿液中维生素B<sub>1</sub>/肌酐比率进行评价。
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(一)维生素B<sub>1</sub>负荷前后的尿维生素B<sub>1</sub>排泄量摄入过多的维生素B<sub>1</sub>会从尿中排出,故可利用测定尿中的维生素B<sub>1</sub>来估计体内维生素B<sub>1</sub>的状态,因为维生素B<sub>1</sub>的需要量与其尿排泄量之间具有一定的关系,因此维生素B<sub>1</sub>负荷试验可以测定维生素B<sub>1</sub>的营养状况。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 3, "end_offset": 19, "label": "dru" }, { "id": 1, "entity": "尿维生素B<sub>1</sub>排泄量", "start_offset": 24, "end_offset": 44, "label": "ite" }, { "id": 2, "entity": "维生素B<sub>1</sub>", "start_offset": 49, "end_offset": 65, "label": "dru" }, { "id": 3, "entity": "尿", "start_offset": 67, "end_offset": 68, "label": "bod" }, { "id": 4, "entity": "尿", "start_offset": 78, "end_offset": 79, "label": "bod" }, { "id": 5, "entity": "维生素B<sub>1</sub>", "start_offset": 81, "end_offset": 97, "label": "bod" }, { "id": 6, "entity": "维生素B<sub>1</sub>", "start_offset": 102, "end_offset": 118, "label": "bod" }, { "id": 7, "entity": "维生素B<sub>1</sub>", "start_offset": 124, "end_offset": 140, "label": "bod" }, { "id": 8, "entity": "尿排泄量", "start_offset": 146, "end_offset": 150, "label": "ite" }, { "id": 9, "entity": "维生素B<sub>1</sub>负荷试验", "start_offset": 162, "end_offset": 182, "label": "ite" }, { "id": 10, "entity": "维生素B<sub>1</sub>", "start_offset": 186, "end_offset": 202, "label": "bod" } ]
通常用荧光法或微生物法进行维生素B<sub>1</sub>的测定,被测者于清晨排尿后禁食,给维生素B<sub>1</sub>(口服5mg或肌注1mg),然后饮水200ml,收集4小时尿,测定尿中维生素B<sub>1</sub>量,若在100μg以上者为正常,脚气患病常低于50μg。
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(二)血清维生素B<sub>1</sub>水平因为血中的游离维生素B<sub>1</sub>及其磷酸盐的含量很低,故测血中的维生素B<sub>1</sub>水平作为维生素B<sub>1</sub>营养状况的指标一直未被广泛采用,但是,最近采用灵敏的高效液相色谱法,此方法简单而可靠,易于标准化,但因其参考值幅度较广,血中含量不稳定,不能及时反映早期缺乏状况,故临床很少采用。
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正常参考值为103~306nmol/L(3.1~9.2μg/dl),如血清维生素B<sub>1</sub>水平<100nmol/L(3μg/dl),则提示维生素B<sub>1</sub>缺乏。
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(三)红细胞转酮醇酶(ETK)活性这是测定维生素B<sub>1</sub>营养状况的特异性指标,也是评价维生素B<sub>1</sub>营养状况的最有效指标。
[ { "id": 0, "entity": "红细胞转酮醇酶(ETK)活性", "start_offset": 3, "end_offset": 17, "label": "ite" }, { "id": 1, "entity": "维生素B<sub>1</sub>", "start_offset": 21, "end_offset": 37, "label": "bod" }, { "id": 2, "entity": "维生素B<sub>1</sub>", "start_offset": 52, "end_offset": 68, "label": "bod" } ]
在临床维生素B<sub>1</sub>缺乏的症状出现之前,ETK已有改变,故称为亚临床诊断或边缘状态的检查。
[ { "id": 0, "entity": "维生素B<sub>1</sub>缺乏", "start_offset": 3, "end_offset": 21, "label": "dis" }, { "id": 1, "entity": "ETK", "start_offset": 29, "end_offset": 32, "label": "bod" } ]
通过测定溶解的红细胞中戊糖消失率或己糖出现率来测量ETK活性。
[ { "id": 0, "entity": "红细胞", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "ETK活性", "start_offset": 25, "end_offset": 30, "label": "ite" } ]
采用体外不加(基础)或加入TPP(刺激)后测定ETK的活性,通常以基础活性(ETKA)或以刺激后活性与基础活性之差占基础活性的百分率(ETK-AC活性系数或TPP效应)来表示。
[ { "id": 0, "entity": "TPP", "start_offset": 13, "end_offset": 16, "label": "dru" }, { "id": 1, "entity": "ETK", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 2, "entity": "ETKA", "start_offset": 38, "end_offset": 42, "label": "ite" }, { "id": 3, "entity": "ETK-AC活性系数", "start_offset": 67, "end_offset": 77, "label": "ite" }, { "id": 4, "entity": "TPP效应", "start_offset": 78, "end_offset": 83, "label": "ite" } ]
硫胺素缺乏与ETKA的降低与ETK-AC的增加有联系;ETK-AC值越高,则维生素B1缺乏越严重。
[ { "id": 0, "entity": "硫胺素缺乏", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "ETKA", "start_offset": 6, "end_offset": 10, "label": "ite" }, { "id": 2, "entity": "ETK-AC", "start_offset": 14, "end_offset": 20, "label": "ite" }, { "id": 3, "entity": "ETK-AC", "start_offset": 27, "end_offset": 33, "label": "ite" }, { "id": 4, "entity": "维生素B1缺乏", "start_offset": 38, "end_offset": 45, "label": "dis" } ]
TPP效应的正常参考值为0%~15%,维生素B<sub>1</sub>低水平时为16%~20%,缺乏时>20%。
[ { "id": 0, "entity": "TPP效应", "start_offset": 0, "end_offset": 5, "label": "ite" }, { "id": 1, "entity": "维生素B<sub>1</sub>", "start_offset": 19, "end_offset": 35, "label": "bod" } ]
(四)空腹一次测定尿液中的维生素B<sub>1</sub>/肌酐比率其正常值为176μg/g肌酐,幼儿如低于120μg/g肌酐,4~12岁小儿低于60μg/g肌酐则为维生素B<sub>1</sub>缺乏。
[ { "id": 0, "entity": "尿液", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "维生素B<sub>1</sub>/肌酐比率", "start_offset": 13, "end_offset": 34, "label": "ite" }, { "id": 2, "entity": "肌酐", "start_offset": 46, "end_offset": 48, "label": "bod" }, { "id": 3, "entity": "肌酐", "start_offset": 61, "end_offset": 63, "label": "bod" }, { "id": 4, "entity": "肌酐", "start_offset": 79, "end_offset": 81, "label": "bod" }, { "id": 5, "entity": "维生素B<sub>1</sub>缺乏", "start_offset": 83, "end_offset": 101, "label": "dis" } ]
【诊断】依靠病史、临床症状和体征、实验室检查和实验性维生素B<sub>1</sub>治疗可作出可靠诊断。
[ { "id": 0, "entity": "体征", "start_offset": 14, "end_offset": 16, "label": "ite" }, { "id": 1, "entity": "实验室检查", "start_offset": 17, "end_offset": 22, "label": "pro" }, { "id": 2, "entity": "实验性维生素B<sub>1</sub>治疗", "start_offset": 23, "end_offset": 44, "label": "pro" } ]
(一)病史患儿是否有维生素B<sub>1</sub>摄入不足,已添加辅食的小儿,是否有长期食用精白米、面及有无偏食。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 10, "end_offset": 26, "label": "dru" } ]
有无妨碍维生素B<sub>1</sub>吸收和利用的疾病,如慢性消耗疾病、胃肠道疾病、肝胆系统疾病等。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 4, "end_offset": 20, "label": "dru" }, { "id": 1, "entity": "慢性消耗疾病", "start_offset": 30, "end_offset": 36, "label": "dis" }, { "id": 2, "entity": "胃肠道疾病", "start_offset": 37, "end_offset": 42, "label": "dis" }, { "id": 3, "entity": "肝胆系统疾病", "start_offset": 43, "end_offset": 49, "label": "dis" } ]
患者是否存在硫胺素需要量增加的因素,如生长发育阶段、发热及甲状腺功能亢进等。
[ { "id": 0, "entity": "硫胺素", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "发热", "start_offset": 26, "end_offset": 28, "label": "sym" }, { "id": 2, "entity": "甲状腺功能亢进", "start_offset": 29, "end_offset": 36, "label": "dis" } ]
(二)临床特点有无周围神经炎的表现,如肌肉萎缩、感觉异常、跟腱及膝反射异常。
[ { "id": 0, "entity": "周围神经炎", "start_offset": 9, "end_offset": 14, "label": "dis" }, { "id": 1, "entity": "肌肉", "start_offset": 19, "end_offset": 21, "label": "bod" }, { "id": 2, "entity": "肌肉萎缩", "start_offset": 19, "end_offset": 23, "label": "sym" }, { "id": 3, "entity": "感觉异常", "start_offset": 24, "end_offset": 28, "label": "sym" }, { "id": 4, "entity": "跟腱", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 5, "entity": "膝", "start_offset": 32, "end_offset": 33, "label": "bod" }, { "id": 6, "entity": "跟腱及膝反射异常", "start_offset": 29, "end_offset": 37, "label": "sym" } ]
有无进行性水肿、心脏扩张肥厚、心率增加、脉压加大。
[ { "id": 0, "entity": "进行性水肿", "start_offset": 2, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "心脏", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "心脏扩张肥厚", "start_offset": 8, "end_offset": 14, "label": "sym" }, { "id": 3, "entity": "心率", "start_offset": 15, "end_offset": 17, "label": "ite" }, { "id": 4, "entity": "心率增加", "start_offset": 15, "end_offset": 19, "label": "sym" }, { "id": 5, "entity": "脉压", "start_offset": 20, "end_offset": 22, "label": "ite" }, { "id": 6, "entity": "脉压加大", "start_offset": 20, "end_offset": 24, "label": "sym" } ]
能除外其他心脏病的心力衰竭。
[ { "id": 0, "entity": "心脏病", "start_offset": 5, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "心力衰竭", "start_offset": 9, "end_offset": 13, "label": "sym" } ]
有无其他营养缺乏的征象。
[ { "id": 0, "entity": "营养缺乏", "start_offset": 4, "end_offset": 8, "label": "sym" } ]
(三)实验室检验可通过测定维生素B<sub>1</sub>负荷前后尿维生素B<sub>1</sub>排泄量、血清维生素B<sub>1</sub>水平、红细胞转酮醇酶(ETK)活性及空腹一次测定尿液中的维生素B<sub>1</sub>/肌酐比率等实验室检查帮助诊断。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 13, "end_offset": 29, "label": "dru" }, { "id": 1, "entity": "尿维生素B<sub>1</sub>排泄量", "start_offset": 33, "end_offset": 53, "label": "ite" }, { "id": 2, "entity": "血清维生素B<sub>1</sub>水平", "start_offset": 54, "end_offset": 74, "label": "ite" }, { "id": 3, "entity": "红细胞转酮醇酶(ETK)活性", "start_offset": 75, "end_offset": 89, "label": "ite" }, { "id": 4, "entity": "尿液", "start_offset": 96, "end_offset": 98, "label": "bod" }, { "id": 5, "entity": "维生素B<sub>1</sub>/肌酐比率", "start_offset": 100, "end_offset": 121, "label": "ite" }, { "id": 6, "entity": "实验室检查", "start_offset": 122, "end_offset": 127, "label": "pro" } ]
【治疗和预防】(一)去除病因仔细询问病史,查明缺乏维生素B<sub>1</sub>的原因,治疗造成维生素B<sub>1</sub>缺乏的原发性疾病,如发热、感染、甲状腺功能亢进等。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 25, "end_offset": 41, "label": "bod" }, { "id": 1, "entity": "维生素B<sub>1</sub>缺乏", "start_offset": 49, "end_offset": 67, "label": "sym" }, { "id": 2, "entity": "发热", "start_offset": 75, "end_offset": 77, "label": "dis" }, { "id": 3, "entity": "感染", "start_offset": 78, "end_offset": 80, "label": "dis" }, { "id": 4, "entity": "甲状腺功能亢进", "start_offset": 81, "end_offset": 88, "label": "dis" } ]
(二)饮食增加含维生素B<sub>1</sub>丰富的食物的摄入量,并注意合理配合。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 8, "end_offset": 24, "label": "dru" } ]
如果乳母维生素B<sub>1</sub>缺乏,应及时予以补充,避免婴儿发生维生素B<sub>1</sub>缺乏症。
[ { "id": 0, "entity": "维生素B<sub>1</sub>缺乏", "start_offset": 4, "end_offset": 22, "label": "sym" }, { "id": 1, "entity": "维生素B<sub>1</sub>缺乏症", "start_offset": 37, "end_offset": 56, "label": "dis" } ]
未精制的粮谷类中维生素B<sub>1</sub>丰富,故碾磨精度不宜过度。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 8, "end_offset": 24, "label": "dru" } ]
豆类、坚果类、瘦肉及内脏维生素B<sub>1</sub>也较为丰富。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 12, "end_offset": 28, "label": "dru" } ]
蛋类、绿叶菜(芹菜叶、莴笋叶)等也是维生素B<sub>1</sub>的良好来源,应充分加以利用。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 18, "end_offset": 34, "label": "dru" } ]
(三)应用维生素B<sub>1</sub>治疗小儿症状较轻,一般维生素B<sub>1</sub>的剂量为5mg/d;重症则需10mg/d静脉注射,每天2次,如症状缓解,则可改为口服。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 5, "end_offset": 21, "label": "dru" }, { "id": 1, "entity": "维生素B<sub>1</sub>", "start_offset": 32, "end_offset": 48, "label": "dru" }, { "id": 2, "entity": "静脉注射", "start_offset": 68, "end_offset": 72, "label": "pro" }, { "id": 3, "entity": "口服", "start_offset": 88, "end_offset": 90, "label": "pro" } ]
用维生素B<sub>1</sub>治疗,神经症状一般于24小时内缓解,心脏症状一般于24~48小时缓解,而水肿则需48~72小时缓解,运动无力的恢复一般时间较长,需1~3个月。
[ { "id": 0, "entity": "维生素B<sub>1</sub>", "start_offset": 1, "end_offset": 17, "label": "dru" }, { "id": 1, "entity": "神经", "start_offset": 20, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "心脏", "start_offset": 35, "end_offset": 37, "label": "bod" }, { "id": 3, "entity": "水肿", "start_offset": 53, "end_offset": 55, "label": "sym" }, { "id": 4, "entity": "运动无力", "start_offset": 67, "end_offset": 71, "label": "sym" } ]
如口服有严重不能耐受的不良反应;长期腹泻、呕吐或大部分小肠切除后需要全肠外营养维持者可通过肠外途径予以补充。
[ { "id": 0, "entity": "口服", "start_offset": 1, "end_offset": 3, "label": "pro" }, { "id": 1, "entity": "长期腹泻、呕吐", "start_offset": 16, "end_offset": 23, "label": "sym" }, { "id": 2, "entity": "小肠", "start_offset": 27, "end_offset": 29, "label": "bod" }, { "id": 3, "entity": "全肠", "start_offset": 34, "end_offset": 36, "label": "bod" }, { "id": 4, "entity": "肠", "start_offset": 45, "end_offset": 46, "label": "bod" } ]
九、其他微生物感染性肺炎(一)支原体肺炎胸片改变多样化,但以单个的密度增高的大片状阴影为主,边缘模糊,常有胸膜反应,病灶吸收缓慢。
[ { "id": 0, "entity": "微生物感染性肺炎", "start_offset": 4, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "支原体肺炎", "start_offset": 15, "end_offset": 20, "label": "dis" }, { "id": 2, "entity": "胸片", "start_offset": 20, "end_offset": 22, "label": "pro" }, { "id": 3, "entity": "单个的密度增高的大片状阴影", "start_offset": 30, "end_offset": 43, "label": "sym" }, { "id": 4, "entity": "边缘模糊", "start_offset": 46, "end_offset": 50, "label": "sym" }, { "id": 5, "entity": "胸膜反应", "start_offset": 53, "end_offset": 57, "label": "sym" }, { "id": 6, "entity": "病灶吸收缓慢", "start_offset": 58, "end_offset": 64, "label": "sym" } ]
(二)衣原体肺炎常以细小点片状阴影出现,分布在两肺野,大多数两侧对称性分布,新生儿时期常与粟粒性肺结核相混淆,需提高警惕。
[ { "id": 0, "entity": "衣原体肺炎", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "细小点片状阴影", "start_offset": 10, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "肺野", "start_offset": 24, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "分布在两肺野,大多数两侧对称性分布", "start_offset": 20, "end_offset": 37, "label": "sym" }, { "id": 4, "entity": "粟粒性肺结核", "start_offset": 45, "end_offset": 51, "label": "dis" } ]
(三)真菌性肺炎病变弥漫广泛,其形态为小斑片状、结节状、绒毛状不等,边缘尚清,病灶内可出现空洞,有时出现胸膜炎、胸腔积液。
[ { "id": 0, "entity": "真菌性肺炎", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "边缘尚清", "start_offset": 34, "end_offset": 38, "label": "sym" }, { "id": 2, "entity": "病灶内可出现空洞", "start_offset": 39, "end_offset": 47, "label": "sym" }, { "id": 3, "entity": "胸膜炎", "start_offset": 52, "end_offset": 55, "label": "dis" }, { "id": 4, "entity": "胸腔积液", "start_offset": 56, "end_offset": 60, "label": "sym" } ]
第三节隐球菌病新型隐球菌(cryptococcusneoformans)是隐球菌病的病原菌。
[ { "id": 0, "entity": "隐球菌病", "start_offset": 3, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "新型隐球菌", "start_offset": 7, "end_offset": 12, "label": "mic" }, { "id": 2, "entity": "cryptococcusneoformans", "start_offset": 13, "end_offset": 35, "label": "mic" }, { "id": 3, "entity": "隐球菌病", "start_offset": 37, "end_offset": 41, "label": "dis" }, { "id": 4, "entity": "病原菌", "start_offset": 42, "end_offset": 45, "label": "mic" } ]
该菌首次由Buschke于1894年在德国从一全身芽生菌病患者身上分离出的。
[ { "id": 0, "entity": "全身芽生菌病", "start_offset": 23, "end_offset": 29, "label": "dis" } ]
主要侵犯肺和中枢神经系统,侵犯皮肤、骨骼等比较少见。
[ { "id": 0, "entity": "肺", "start_offset": 4, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "中枢神经系统", "start_offset": 6, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "皮肤", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 3, "entity": "骨骼", "start_offset": 18, "end_offset": 20, "label": "bod" } ]
【临床流行病学】隐球菌属有17个种和9个变种,分布广泛,其中只有新型隐球菌是人类的病原菌。
[ { "id": 0, "entity": "隐球菌", "start_offset": 8, "end_offset": 11, "label": "mic" }, { "id": 1, "entity": "新型隐球菌", "start_offset": 32, "end_offset": 37, "label": "mic" }, { "id": 2, "entity": "病原菌", "start_offset": 41, "end_offset": 44, "label": "mic" } ]
新型隐球菌为酵母菌,在寄生状态下,可见圆球形带宽阔荚膜的菌体,部分菌体可有出芽,菌体内可见一个或多个反光颗粒。
[ { "id": 0, "entity": "新型隐球菌", "start_offset": 0, "end_offset": 5, "label": "mic" }, { "id": 1, "entity": "酵母菌", "start_offset": 6, "end_offset": 9, "label": "mic" } ]
与非致病隐球菌不同的是,它能在37℃的环境中生长。
[ { "id": 0, "entity": "非致病隐球菌", "start_offset": 1, "end_offset": 7, "label": "mic" } ]
新型隐球菌荚膜的血清型(A~D和AD)与致病性有关,我国有A、B、D共3型存在,且以A型为最多见。
[ { "id": 0, "entity": "新型隐球菌", "start_offset": 0, "end_offset": 5, "label": "mic" } ]
从患者分离到的新型隐球菌多是A型和D型。
[ { "id": 0, "entity": "新型隐球菌", "start_offset": 7, "end_offset": 12, "label": "mic" } ]
此菌是土壤、鸽子粪、牛乳、水果的腐生菌,可从呼吸道、消化道、皮肤等进入人体引起隐球菌病。
[ { "id": 0, "entity": "腐生菌", "start_offset": 16, "end_offset": 19, "label": "mic" }, { "id": 1, "entity": "呼吸道", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 2, "entity": "消化道", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 3, "entity": "皮肤", "start_offset": 30, "end_offset": 32, "label": "bod" }, { "id": 4, "entity": "隐球菌病", "start_offset": 39, "end_offset": 43, "label": "dis" } ]
隐球菌分布很广,世界各地均有报道,在我国也有发现。
[ { "id": 0, "entity": "隐球菌", "start_offset": 0, "end_offset": 3, "label": "mic" } ]
尚未证实人与人之间传播的隐球菌感染。
[ { "id": 0, "entity": "隐球菌感染", "start_offset": 12, "end_offset": 17, "label": "dis" } ]
虽然在免疫正常的儿童可以发生播散型隐球菌病,但感染最常发生在有细胞免疫缺陷的免疫抑制患者,如先天性或获得性免疫缺陷病、器官移植的患者、胶原性血管性疾病、应用皮质类固醇等。
[ { "id": 0, "entity": "播散型隐球菌病", "start_offset": 14, "end_offset": 21, "label": "dis" }, { "id": 1, "entity": "细胞免疫缺陷", "start_offset": 31, "end_offset": 37, "label": "dis" }, { "id": 2, "entity": "先天性或获得性免疫缺陷病", "start_offset": 46, "end_offset": 58, "label": "dis" }, { "id": 3, "entity": "器官移植", "start_offset": 59, "end_offset": 63, "label": "dis" }, { "id": 4, "entity": "胶原性血管性疾病", "start_offset": 67, "end_offset": 75, "label": "dis" }, { "id": 5, "entity": "皮质类固醇", "start_offset": 78, "end_offset": 83, "label": "dru" } ]
有报道儿童最常见的原发疾病是急性淋巴细胞白血病、系统性红斑狼疮,以及恶性肿瘤和呼吸道疾病等。
[ { "id": 0, "entity": "急性淋巴细胞白血病", "start_offset": 14, "end_offset": 23, "label": "dis" }, { "id": 1, "entity": "系统性红斑狼疮", "start_offset": 24, "end_offset": 31, "label": "dis" }, { "id": 2, "entity": "恶性肿瘤", "start_offset": 34, "end_offset": 38, "label": "dis" }, { "id": 3, "entity": "呼吸道疾病", "start_offset": 39, "end_offset": 44, "label": "dis" } ]
【发病机制和病理生理】存在于土壤及鸽粪中的隐球菌,可随尘埃一起被人吸入。
[ { "id": 0, "entity": "隐球菌", "start_offset": 21, "end_offset": 24, "label": "mic" } ]
隐球菌直径仅1μm,能进入肺泡。
[ { "id": 0, "entity": "隐球菌", "start_offset": 0, "end_offset": 3, "label": "mic" }, { "id": 1, "entity": "肺泡", "start_offset": 13, "end_offset": 15, "label": "bod" } ]
隐球菌在体外无荚膜包裹,进入体内后很快形成荚膜,带荚膜的隐球菌具有致病力。
[ { "id": 0, "entity": "隐球菌", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "隐球菌", "start_offset": 28, "end_offset": 31, "label": "mic" } ]
侵入人体的隐球菌不一定致病,细胞免疫在防止隐球菌感染中起主要的作用。
[ { "id": 0, "entity": "隐球菌", "start_offset": 5, "end_offset": 8, "label": "mic" }, { "id": 1, "entity": "隐球菌感染", "start_offset": 21, "end_offset": 26, "label": "dis" } ]
免疫功能低下的各种疾病如白血病、淋巴瘤、结节病、糖尿病等患者,均易继发隐球菌病。
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隐球菌侵入肺部后,局部病变进展缓慢,常无临床症状,但可由此经血进入中枢神经系统,导致隐球菌病中最为常见的脑膜炎。
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颅底软脑膜病变较显著,蛛网膜下腔有广泛的渗出物积聚,内含单核细胞、淋巴细胞及隐球菌等。
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