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青霉素过敏,或青霉素治疗无效者可应用含β-内酰胺酶抑制剂的复合β-内酰胺类,或头孢霉素、大环内酯类抗生素。
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羟氨苄青霉素每日一次疗法可能成为青霉素治疗链球菌咽炎的替代方案。
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应注意呼吸道隔离,预防并发症。
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其临床表现以阴离子间隙正常的高氯性代谢性酸中毒、肾钙化及肾结石为特征。
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原发性为常染色体显性或隐性遗传,继发性者常是由于其他疾病影响到肾小管功能所致,可见于高丙种球蛋白血症、原发性甲状旁腺功能亢进、维生素D中毒、移植肾排斥反应、髓质海绵肾、梗阻性肾病、特发性高钙尿症肾钙化、Wilson病、失盐性先天性肾上腺皮质增生症、药物及毒素导致肾损害(如锂、两性霉素B、甲苯及地高辛等)。
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原发性dRTA的基因突变有两种。
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常染色质隐性遗传则涉及质子泵B亚基的缺陷(ATP6B1)。
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后者常在2岁后出现症状,以女性多见,为染色体显性遗传。
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不完全型dRTA可无酸中毒表现而仅出现低钾、肌无力或肾钙化。
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血钾降低也是dRTA的重要表现,甚至为不完全性dRTA的唯一表现。
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一般情况下可使用Shohl合剂或单用10%枸橼酸钾口服,剂量2~4ml/(kg•d);有低钙血症者可适当补充钙剂,如10%葡萄糖酸钙2ml/(kg•d),总量<20ml/d。
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对高钙尿症,可服用上述枸橼酸制剂治疗,必要时还可加双氢克尿噻,2mg/(kg•d)口服,常可减轻高钙尿症,并促进溶石与排石。
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内源性感染的放线菌多为构成人和动物黏膜,特别是口腔黏膜固有菌群中的兼性厌氧菌。
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临床上的放线菌病、泪小管炎、牙周炎和龋齿均由这类菌诱发引起。
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主要致病菌有诺卡菌属、马杜拉放线菌和链霉菌属中的一些种。
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临床上一般将广义的放线菌病因损害的部位不同分为以下几型:颈面型放线菌病、胸部型放线菌病、腹部型放线菌病、皮肤型放线菌病、脑型放线菌病、其他组织的放线菌病、放线菌性足菌病。
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脑型放线菌病的临床表现与细菌性脑部病变感染略似,有以下两型:①局限性脑脓肿型:多见于大脑半球、少数发生在第三脑室和颅后窝。
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主要表现为脑部占位性病变的体征,如颅压增高、脑神经损害、头痛、恶心、呕吐、复视、视神经乳头水肿等,常无发热,白细胞总数及分类正常。
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当青霉素过敏、无效或其他原因不能耐受时,可选用红霉素、林可霉素、克林霉素、头孢氨苄霉素、氯霉素、链霉素等。
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颈面部放线菌病预后良好,其他类型若能适当治疗,可减少废用、畸形等后遗症。
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尤因肉瘤可以发生在全身各个骨骼器,但半数以上的肿瘤发生在肢体的长骨,特别是股骨。
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骨盆、腓骨、胫骨、肱骨、肋骨、锁骨也经常累及。
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疼痛可以是间歇性的,但随着病情的发展多转为持续性,部分病例早期有原因不明的间歇性发热,肿瘤广泛累及骨骼后甚至会有病理性骨折的发生,但不常见。
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特别是当骨肿瘤存在时,早期检查肺部对于治疗方案的制定有重要的意义。
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尤因肉瘤的X线表现为肿瘤多数位于骨干的中部,呈混合性骨破坏影像,皮质骨上可以有斑点状的骨破坏。
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CT和MRI检查对于肿瘤浸润周围软组织可以有较明确的描述。
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当肿瘤组织呈现为半液体状态时,应该同慢性骨髓炎做鉴别,必要的时候要做组织的细菌培养。
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过去,放射治疗应用较多,原因是高度增长扩大的尤文肿瘤,对于放射线有着较为敏感的反应,甚至有时简单的X线摄片都会引起肿瘤生长的暂时性停顿。
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但近年来的研究越来越多地表明,单纯依赖肿瘤组织对于放射的敏感反应尚不能达到满意的疗效,很多病例,经过化疗后,应及时采取手术肿瘤切除的方法,患者长期生存率的提高比单纯接受放射治疗要好。
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双胎输血综合征的供血者出现贫血,受血者则表现为:多血,红细胞增多,血黏度增高,心、肝、肾、胰及肾上腺增大,羊水多,高胆红素血症,充血性心力衰竭等。
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对贫血较重者,需输血。
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体液免疫紊乱也可引起再障的发生,部分再障患儿血浆中可有抗造血细胞抗体存在。
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8.非血液学综合征如Down,Dubowitz,Seckel综合征等。
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4.当慢性再障在病程中病情恶化临床表现、血象及骨髓象与急性再障相同时,称为重型再障Ⅱ型(SAA-Ⅱ)。
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网织红细胞<1%;白细胞总数大多降低,但也有正常者,此时常出现淋巴细胞相对值增高。
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儿童再障以后两型多见。
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3.血清铁、镁、锌升高。
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4.血清EPO、游离红细胞原卟啉(FEP)增加。
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鉴别的主要依据为骨髓涂片、骨髓活检及相应的细胞和分子生物学检查。
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因此,积极预防和治疗感染是降低死亡率的重要措施。
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一旦出现感染,应及早使用强力有效的抗生素。
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在没有明确病原体感染之前,通常需要广谱抗生素、抗真菌药及抗病毒药联合应用。
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上述方案主要用于急性或重型再障的治疗。
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用药一周末至两周内可发生血清病,出现发热、皮疹(荨麻疹、麻疹样或猩红热样)、淋巴结增大、关节酸痛,严重表现有面部及四肢水肿、少尿、喉头水肿、哮喘、末梢神经炎、头痛、谵妄,甚至惊厥。
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一旦出现上述任何表现者均应严密监护,仅有皮疹者则可给予异丙嗪、止痒洗剂等对症处理,较重表现者则可给予甲基泼尼松龙10mg/(kg•d)一次静注,连用3~4日。
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(4)抗T淋巴细胞单克隆抗体(单抗)。
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(5)大剂量丙种球蛋白。
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(6)异基因造血干细胞移植:适用于重型再障,病程早期进行移植成活率极高。
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慢性再障治疗与急性再障治疗有所区别,急性再障以免疫抑制剂为主,而慢性再障则以雄性激素为主的综合疗法。
[ { "id": 0, "entity": "慢性再障", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "急性再障", "start_offset": 7, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "急性再障", "start_offset": 18, "end_offset": 22, "label": "dis"...
(4)莨菪浸膏片:每次10mg,每日3次,口服,每日递增10~20mg至每次240~300mg,30日为一疗程,休7日后重复。
[ { "id": 0, "entity": "莨菪浸膏片", "start_offset": 3, "end_offset": 8, "label": "dru" } ]
急性再障预后甚差,如未能得到有效治疗者,绝大多数一年内死亡,有的甚至2~3月内夭亡。
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如髋关节内翻、膝关节内翻或外翻等。
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近年来,除了传统的手术活组织检查,穿刺活组织检查,特别是超声波或放射线引导下的微创性组织活检技术得到了很大的发展。
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是一种地方性寄生虫病,由并殖吸虫寄生于人体所引起。
[ { "id": 0, "entity": "地方性寄生虫病", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "并殖吸虫", "start_offset": 12, "end_offset": 16, "label": "mic" } ]
约经1~3周后绝大多数幼虫由腹腔穿过横膈进入胸腔(主要在右侧沿肝表面向上移行),可引起胸膜炎症状。
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根据虫体寄生部位的不同,有不同的症状,但以胸部、腹部、脑部为重点。
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初期即发生胸膜炎者,有咳嗽、胸闷,胸痛及上腹痛明显,可痰中带血。
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肺内囊肿、脓肿可穿破至胸腔,发生胸水、脓胸或脓气胸等体征。
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胸X线片显示肺浸润、囊状或结节状阴影或薄壁空洞,有时呈液平面。
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此外尚可累及腹腔内其他器官,而可出现黄疸、脓尿、血尿、肝脾肿大等症状,有时甚至与急腹症不易鉴别。
[ { "id": 0, "entity": "腹腔", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "黄疸", "start_offset": 18, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "脓尿", "start_offset": 21, "end_offset": 23, "label": "sym" },...
此外尚可有癔症症状,表现为精神失常,记忆力差,有幻觉、幻视等。
[ { "id": 0, "entity": "癔症", "start_offset": 5, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "精神失常", "start_offset": 13, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "记忆力差", "start_offset": 18, "end_offset": 22, "label": "sym" ...
必要时可做气脑、脑室造影及肺部断层摄影。
[ { "id": 0, "entity": "气脑", "start_offset": 5, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "脑室造影", "start_offset": 8, "end_offset": 12, "label": "pro" }, { "id": 2, "entity": "肺部断层摄影", "start_offset": 13, "end_offset": 19, "label": "pro"...
散发性甲低是由于先天性甲状腺发育不良、异位或甲状腺激素合成途径缺陷所致;地方性甲低多见于甲状腺肿流行的地区,系由于地区性水、土和食物中碘缺乏所致。
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吞咽反射不协调,贲门括约肌松弛,胃容量小,早产儿容易发生溢乳和呛咳。
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早产儿发生败血症和脑膜炎的机会是足月新生儿的4倍,败血症死亡率高达30%。
[ { "id": 0, "entity": "败血症", "start_offset": 5, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "脑膜炎", "start_offset": 9, "end_offset": 12, "label": "dis" }, { "id": 2, "entity": "败血症", "start_offset": 25, "end_offset": 28, "label": "dis" ...
尿常规有蛋白尿及管型尿。
[ { "id": 0, "entity": "尿常规", "start_offset": 0, "end_offset": 3, "label": "ite" }, { "id": 1, "entity": "蛋白尿", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "管型尿", "start_offset": 8, "end_offset": 11, "label": "bod" } ...
血浆β-血栓球蛋白和Ⅷ因子相关抗原测定有助于随访疗效以及反映血管炎活动程度。
[ { "id": 0, "entity": "血浆β-血栓球蛋白", "start_offset": 0, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "Ⅷ因子相关抗原", "start_offset": 10, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "血管炎", "start_offset": 30, "end_offset": 33, "label...
B组链球菌(GBS)则是新生儿肺炎的主要病原。
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3型vWD患儿可有关节、软组织出血,极少数因颅内出血而危及生命。
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反复出血患儿易导致缺铁性贫血。
[ { "id": 0, "entity": "缺铁性贫血", "start_offset": 9, "end_offset": 14, "label": "dis" } ]
2型又分4种亚型:血浆中大和中等大小的多聚体缺乏者为2A型,缺乏大多聚体且与血小板GPⅠb亲和性增加为2B型,vWF与血小板GPⅠb结合力降低为2M型,与因子Ⅷ结合力降低者为2N型。
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该类药物可单纯使用或与DDAVP合用;②口服避孕药:用于治疗月经过多;③肾上腺皮质激素:对产生vWF抗体者更为有效;④因慢性缺铁引起缺铁性贫血者,应给予铁剂治疗。
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补充磷及维生素D对有佝偻病表现的患者有较好的效果。
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Depape-Brigger和Goldman试用二硫苏糖醇(dithiotreitol,DIT)治疗2例也未获效果。
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其不良反应主要有恶心、呕吐、腐臭味较难忍受以及血清病样反应等。
[ { "id": 0, "entity": "恶心", "start_offset": 8, "end_offset": 10, "label": "sym" }, { "id": 1, "entity": "呕吐", "start_offset": 11, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "腐臭味", "start_offset": 14, "end_offset": 17, "label": "sym" ...
上肢缺血引起肢体无力和麻木。
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胸部X线片可显示主动脉钙化或主动脉增宽,超声波检查可显示周围动脉或主动脉等狭窄部位及程度,动脉造影和MRI可显示狭窄或扩张的部位及程度,以及血流减少的程度。
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经多代学者的研究努力,肾母细胞瘤的低存活率迅速得到提高,其生存率1940年为25%,至今已上升至90%左右。
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肾母细胞瘤可能由于未分化形成小管和小球的后肾胚芽异常增生所致。
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有时同时有先天性白内障,还可有中枢神经异常,如小头畸形、头面异形、耳廓异常、泌尿系统畸形和智能迟缓等,近年对虹膜缺如合并肾母细胞瘤患儿的细胞遗传学研究表明,其均有11号染色体短臂移位或部分缺如的现象。
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此外,近年来还发现肾母细胞瘤伴外生殖器雌雄难辨的两性畸形患儿,Raifer报道10例,其中7例发生在单侧肾母细胞瘤,3例双侧肾母细胞瘤。
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肾母细胞瘤淋巴结转移并不多见,且大多限于局部淋巴结,但血源性扩散甚为多见,首先是经肾静脉,其中常有肿瘤栓子,可漫延至下腔静脉,甚至到右心房,血行性扩散80%到肺部,有时到肝,偶尔到骨骼。
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患儿往往有含糊不清的消化道症状,如恶心、呕吐和食欲减退等。
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近年还有人从肾母细胞瘤的浸出液中作肾素的定量分析,其量较正常肾皮质所含高得多。
[ { "id": 0, "entity": "肾母细胞瘤", "start_offset": 6, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "肾素", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "肾皮质", "start_offset": 30, "end_offset": 33, "label": "bod"...
尿显微镜检查不少有血尿和蛋白尿,但尿中多不能找到癌细胞。
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肿瘤主要经血流转移,故向肺转移最为多见,转移后鲜有咳嗽、咯血等症状,故X线肺部检查至为重要。
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Ⅱ期肿瘤已扩散至肾周组织,但能完整切除。
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Ⅲ期腹部残留有非血源性肿瘤,并有下列一种或多种情况:①肾门和腹主动脉旁淋巴结浸润;②弥漫性腹腔播散,术前或术中肿瘤散落;③切除面镜检或肉眼示有肿瘤残留;④腹膜有肿瘤种植;⑤由于局部浸润粘连,肿瘤无法被完整切除。
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Ⅳ期有血源性转移至肺、肝、骨和脑。
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双侧肾母细胞瘤术前应仔细考虑手术方案,一般作较大肿瘤患侧肾切除,较小的对侧作半肾切除或仅作肿瘤摘除。
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1.放线菌素D(AMD)术后当日开始应用,每日静脉注射15μg/kg,连续5日,总剂量75μg/kg,为1疗程。
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放线菌素D可引起恶心、呕吐、口腔炎、腹泻、脱发和骨髓抑制等。
[ { "id": 0, "entity": "放线菌素D", "start_offset": 0, "end_offset": 5, "label": "dru" } ]
目前了解的分子病理改变有垂体加压素基因(AVP-NPⅡ)。
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部分家族性单纯性尿崩症患者发现AVP-NPⅡ基因有突变,大多为基因点突变,且突变类型及位点具有一定的异质性,有的呈现常染色体显性遗传,也有常染色体隐性遗传。
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确需吸入氧气治疗(如发生呼吸暂停),应监测和控制用氧浓度吸入时间,同时监测血氧饱和度,使其保持在88%~93%为宜。
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氧气吸入可能和早产儿视网膜眼病(ROP)的发生有关。
[ { "id": 0, "entity": "氧气吸入", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "早产儿视网膜眼病", "start_offset": 7, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "ROP", "start_offset": 16, "end_offset": 19, "label": "d...
环境被蛔虫卵污染,是造成小儿感染蛔虫病的主要来源。
[ { "id": 0, "entity": "蛔虫", "start_offset": 3, "end_offset": 5, "label": "mic" }, { "id": 1, "entity": "蛔虫病", "start_offset": 16, "end_offset": 19, "label": "dis" } ]
人体蛔虫病由于雌虫产卵量极大和具有对恶劣环境条件的抵抗力而广泛流行,虫卵在泥土中生存数月仍具有感染力,在5~10℃较冷气候条件下可生存2年。
[ { "id": 0, "entity": "蛔虫病", "start_offset": 2, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "雌虫", "start_offset": 7, "end_offset": 9, "label": "mic" }, { "id": 2, "entity": "虫卵", "start_offset": 34, "end_offset": 36, "label": "mic" } ]
因此蛔虫病可在温暖季节传播,有时经年都可传播。
[ { "id": 0, "entity": "蛔虫病", "start_offset": 2, "end_offset": 5, "label": "dis" } ]
肠道蛔虫可无任何症状,或有食欲不佳和腹痛、腹胀、恶心、呕吐、轻泻或便秘。
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