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病程长者,可影响局部发育出现小颌畸形;累及喉杓(环状软骨及杓状软骨)可致声哑、喉喘鸣和饮食困难。
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部分患儿晨起关节活动障碍,但病变关节可不发红,即晨僵。
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偶见个别幼儿颌关节炎后导致口腔活动障碍,面部不对称而需要外科手术纠正。
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而在JIA总体阳性率不足15%,主要出现在多关节型JIA之中,RF阴性并不能除外诊断JIA。
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各型患儿活动期HIgM-RF均值高于缓解期,并与病情活动性有关。
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256例JIA患儿检测ANA阳性结果分析与发病年龄偏小、不对称性关节炎及虹膜睫状体炎的发生有关。
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因此建议将APF作为JIA诊断指标。
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研究发现抗Sa抗体与RF、RA3、SSA、SSB、RNP、Sm、Jo-1及Scl-70等多种自身抗体无交叉反应性,Sa抗体对JIA的诊断价值罕见报告。
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各种NSAIDs药理机制及副作用基本相似,因此不能两种NSAIDs联合使用。
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尽管为成人类风湿关节炎(RA)指南,对儿童亦有较好的指导作用,该指南第一项提出即一经诊断RA即可早期使用DMARDs治疗。
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CSA可用于全身型JIA,尤其是合并巨噬细胞活化综合征患儿。
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糖皮质激素治疗RA价值有争议,成人RA的“强化治疗”理念认为“应依据病情活动度制订个体化的早期联合治疗方案,此后密切随访,根据疗效及时调整用药,使患者的病情活动度能在最快时间内达临床缓解,防止关节破坏及关节外损伤”。
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HGA是由于嗜吞噬细胞无形体感染引起的经蜱传播的立克次体病,主要侵犯人中性粒细胞,临床以发热伴白细胞、血小板减少和多脏器功能损害为主要临床表现。
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我国已有报道黑龙江、蒙古及新疆等地的全沟硬蜱中检测到人粒细胞无形体核酸。
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免疫缺陷患者及应用糖皮质激素治疗者感染本病后病情多较危重。
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尿常规可出现蛋白尿、血尿、管型尿。
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血生化检查可出现肝、肾功能异常,心肌酶谱升高,少数患者出现血淀粉酶、尿淀粉酶和血糖升高。
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全血或血细胞标本PCR检测嗜吞噬细胞无形体特异性核酸阳性,或分离到病原体。
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按临床表现又可分为少尿型与非少尿型以及高分解型。
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败血症也可引起弥散性血管内凝血(DIC),导致急性肾衰。
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中毒性ATN的病变限于近端小管,呈局灶性分布,坏死的肾小管基膜完整,小管上皮再生良好。
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而缺血性ATN病变可涉及各段肾小管,呈弥漫性分布,坏死的小管基底膜断裂,上皮细胞再生较差。
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②氮质血症:血BUN及Cr增高,并出现由于毒素在体内储积而引起的全身各系统中毒症状,如厌食、恶心、呕吐、呕血、嗜睡、烦躁及贫血等。
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③水钠潴留:全身水肿、血压升高,并可出现肺水肿、脑水肿及心力衰竭等表现。
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高磷及低钙血症,可出现手足搐搦及惊厥等。
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⑤代谢性酸中毒:表现为疲乏、嗜睡、面色潮红、恶心、呕吐、呼吸深大,甚至昏迷、休克等。
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2.防治感染。
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3.加强营养,纠正贫血。
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胸水苏丹乙醇染色可见红色脂肪颗粒。
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胸水乙醚试验:胸水加少量乙醚振荡均匀静置片刻,胸水变清亮。
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反复胸腔穿刺可减轻压迫症状,但乳糜可迅速漏出重新积聚,并导致热量、蛋白质和淋巴细胞的丢失,继发免疫功能缺陷,如低免疫球蛋白血症和细胞免疫功能异常。
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多数学者主张乳糜胸患儿应给予低脂肪、高蛋白质、高热量饮食,适当限制盐的摄入。
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若内科保守治疗无效,可行手术治疗,如胸导管结扎、胸腹腔引流术。
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此外,文献报道还与单纯疱疹和带状疱疹病毒,流感A和B、流行性腮腺炎、麻疹、柯萨奇、甲型和乙型肝炎病毒,天花和人类免疫缺陷病毒等感染有关。
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目前空肠弯曲杆菌及GBS的相关性引起广泛关注,空肠弯曲杆菌(CJ)是引起急性胃肠炎的主要病原,也是最常见的GBS的前驱感染源。
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各亚型的临床及病理特征各异,但最主要的病理改变为周围神经中单核细胞浸润和节段性脱髓鞘。
[ { "id": 0, "entity": "单核细胞", "start_offset": 29, "end_offset": 33, "label": "bod" }, { "id": 1, "entity": "节段性脱髓鞘", "start_offset": 36, "end_offset": 42, "label": "bod" } ]
严重者24~48小时内发生呼吸肌麻痹,需立即机械通气。
[ { "id": 0, "entity": "呼吸肌麻痹", "start_offset": 13, "end_offset": 18, "label": "sym" } ]
感觉障碍包括麻木感、蚁行感、针刺感,以及烧灼感。
[ { "id": 0, "entity": "麻木感", "start_offset": 6, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "蚁行感", "start_offset": 10, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "针刺感", "start_offset": 14, "end_offset": 17, "label": "sym" }, { "id": 3, "entity": "烧灼感", "start_offset": 20, "end_offset": 23, "label": "sym" } ]
(4)抗Ga1NAc-GD1a抗体:此抗体与前驱空肠弯曲杆菌感染相关,研究表明伴有此抗体的GBS患者可出现快速进展,非常严重的肌无力(以远端肌群为主)。
[ { "id": 0, "entity": "抗Ga1NAc-GD1a抗体", "start_offset": 3, "end_offset": 17, "label": "bod" }, { "id": 1, "entity": "抗体", "start_offset": 19, "end_offset": 21, "label": "bod" }, { "id": 2, "entity": "前驱空肠弯曲杆菌感染", "start_offset": 22, "end_offset": 32, "label": "dis" }, { "id": 3, "entity": "抗体", "start_offset": 42, "end_offset": 44, "label": "bod" }, { "id": 4, "entity": "GBS", "start_offset": 45, "end_offset": 48, "label": "dis" }, { "id": 5, "entity": "严重的肌无力", "start_offset": 60, "end_offset": 66, "label": "dis" }, { "id": 6, "entity": "远端肌群", "start_offset": 68, "end_offset": 72, "label": "bod" } ]
8)变异型:①神经症状发生时发热。
[ { "id": 0, "entity": "神经症状", "start_offset": 7, "end_offset": 11, "label": "sym" }, { "id": 1, "entity": "发热", "start_offset": 14, "end_offset": 16, "label": "sym" } ]
最重要的是观察呼吸肌的力量,最方便的床旁方法是测肺活量,对高危患者应每隔2小时监测一次肺活量,当肺活量下降至15ml/kg时,即使患者未出现低氧血症,也需进行机械通气。
[ { "id": 0, "entity": "观察呼吸肌的力量", "start_offset": 5, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "测肺活量", "start_offset": 23, "end_offset": 27, "label": "pro" }, { "id": 2, "entity": "肺活量", "start_offset": 43, "end_offset": 46, "label": "ite" }, { "id": 3, "entity": "肺活量", "start_offset": 48, "end_offset": 51, "label": "ite" }, { "id": 4, "entity": "低氧血症", "start_offset": 70, "end_offset": 74, "label": "dis" }, { "id": 5, "entity": "机械通气", "start_offset": 79, "end_offset": 83, "label": "pro" } ]
因此对那些发病5天内不能吞咽的患者需给予营养支持。
[ { "id": 0, "entity": "营养支持", "start_offset": 20, "end_offset": 24, "label": "pro" } ]
勤翻身可避免褥疮及因长期卧床导致的深静脉栓塞及肺栓塞等并发症。
[ { "id": 0, "entity": "褥疮", "start_offset": 6, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "深静脉栓塞", "start_offset": 17, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "肺栓塞", "start_offset": 23, "end_offset": 26, "label": "dis" } ]
疼痛是GBS常见的症状,可能与多种因素有关,如神经根炎及神经炎,不能活动等造成的肌肉疼痛和痛觉过敏。
[ { "id": 0, "entity": "疼痛", "start_offset": 0, "end_offset": 2, "label": "sym" }, { "id": 1, "entity": "GBS", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 2, "entity": "神经根炎", "start_offset": 23, "end_offset": 27, "label": "dis" }, { "id": 3, "entity": "神经炎", "start_offset": 28, "end_offset": 31, "label": "dis" }, { "id": 4, "entity": "肌肉疼痛", "start_offset": 40, "end_offset": 44, "label": "sym" }, { "id": 5, "entity": "痛觉过敏", "start_offset": 45, "end_offset": 49, "label": "sym" } ]
短期应用大剂量肾上腺皮质激素有时也有效。
[ { "id": 0, "entity": "肾上腺皮质激素", "start_offset": 7, "end_offset": 14, "label": "dru" } ]
患者在入院后的1~2天内即可进行理疗,肢体做被动锻炼,但应避免骨折。
[ { "id": 0, "entity": "肢体", "start_offset": 19, "end_offset": 21, "label": "bod" } ]
据报道大剂量静注免疫球蛋白应用于重症GBS,可以降低气管插管及机械通气的需要,缩短患者在ICU的时间,以及促进其功能恢复。
[ { "id": 0, "entity": "大剂量静注免疫球蛋白", "start_offset": 3, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "重症GBS", "start_offset": 16, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "气管插管", "start_offset": 26, "end_offset": 30, "label": "pro" }, { "id": 3, "entity": "机械通气", "start_offset": 31, "end_offset": 35, "label": "pro" }, { "id": 4, "entity": "ICU", "start_offset": 44, "end_offset": 47, "label": "dep" } ]
有学者认为大剂量肾上腺皮质激素冲击疗效好,能抑制B细胞产生抗体,同时减轻神经组织水肿,方法为甲泼尼龙,开始剂量为15mg/(kg•d),3~5天后改为口服泼尼松,4周后减量,总疗程为6~7周。
[ { "id": 0, "entity": "大剂量肾上腺皮质激素冲击疗效", "start_offset": 5, "end_offset": 19, "label": "pro" }, { "id": 1, "entity": "B细胞", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 2, "entity": "抗体", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 3, "entity": "神经组织水肿", "start_offset": 36, "end_offset": 42, "label": "dis" }, { "id": 4, "entity": "甲泼尼龙", "start_offset": 46, "end_offset": 50, "label": "dru" }, { "id": 5, "entity": "泼尼松", "start_offset": 77, "end_offset": 80, "label": "dru" } ]
有报道指出肾上腺皮质激素与静脉注射丙球蛋白联合应用疗效显著。
[ { "id": 0, "entity": "肾上腺皮质激素", "start_offset": 5, "end_offset": 12, "label": "dru" }, { "id": 1, "entity": "静脉注射丙球蛋白", "start_offset": 13, "end_offset": 21, "label": "pro" } ]
总之,GBS的治疗以综合疗法为宜。
[ { "id": 0, "entity": "GBS", "start_offset": 3, "end_offset": 6, "label": "dis" } ]
此病变很少见于婴儿,手术切除后又可重新长出,且许多先天性心脏病手术后可长出主动脉瓣下隔膜,所以有人认为此系后天长出,但为先天的局部组织异常所致。
[ { "id": 0, "entity": "手术", "start_offset": 10, "end_offset": 12, "label": "pro" }, { "id": 1, "entity": "先天性心脏病", "start_offset": 25, "end_offset": 31, "label": "dis" }, { "id": 2, "entity": "主动脉瓣下隔膜", "start_offset": 37, "end_offset": 44, "label": "bod" } ]
此外,二尖瓣的许多畸形如降落伞样二尖瓣等亦可有左心室流出道的梗阻。
[ { "id": 0, "entity": "二尖瓣", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "降落伞样二尖瓣", "start_offset": 12, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "左心室流出道的梗阻", "start_offset": 23, "end_offset": 32, "label": "dis" } ]
临床上主动脉瓣下与瓣膜狭窄不易鉴别,左心室造影和超声对诊断有决定意义。
[ { "id": 0, "entity": "主动脉瓣", "start_offset": 3, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "瓣膜", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "左心室造影", "start_offset": 18, "end_offset": 23, "label": "pro" }, { "id": 3, "entity": "超声", "start_offset": 24, "end_offset": 26, "label": "pro" } ]
重者需手术矫治。
[ { "id": 0, "entity": "手术矫治", "start_offset": 3, "end_offset": 7, "label": "pro" } ]
患儿头小,前额后缩,前囟大及骨缝宽,颅顶头皮有溃疡。
[ { "id": 0, "entity": "患儿头小", "start_offset": 0, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "前额后缩", "start_offset": 5, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "前囟大", "start_offset": 10, "end_offset": 13, "label": "sym" }, { "id": 3, "entity": "骨缝宽", "start_offset": 14, "end_offset": 17, "label": "sym" }, { "id": 4, "entity": "颅顶头皮有溃疡", "start_offset": 18, "end_offset": 25, "label": "sym" } ]
睑裂呈水平线,可见不同程度的小眼至无眼,眼距宽,有白内障、虹膜缺损及视网膜发育异常。
[ { "id": 0, "entity": "睑裂呈水平线", "start_offset": 0, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "不同程度的小眼至无眼", "start_offset": 9, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "眼距宽", "start_offset": 20, "end_offset": 23, "label": "sym" }, { "id": 3, "entity": "白内障", "start_offset": 25, "end_offset": 28, "label": "sym" }, { "id": 4, "entity": "虹膜", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 5, "entity": "虹膜缺损", "start_offset": 29, "end_offset": 33, "label": "sym" }, { "id": 6, "entity": "视网膜", "start_offset": 34, "end_offset": 37, "label": "bod" }, { "id": 7, "entity": "视网膜发育异常", "start_offset": 34, "end_offset": 41, "label": "sym" } ]
可见独眼畸形及小下颌。
[ { "id": 0, "entity": "独眼畸形", "start_offset": 2, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "小下颌", "start_offset": 7, "end_offset": 10, "label": "sym" } ]
耳位低,耳轮较平而界限不清,且有耳聋。
[ { "id": 0, "entity": "耳位低", "start_offset": 0, "end_offset": 3, "label": "sym" }, { "id": 1, "entity": "耳轮较平而界限不清", "start_offset": 4, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "耳聋", "start_offset": 16, "end_offset": 18, "label": "sym" } ]
面、前额或颈背可有一个或多个血管瘤。
[ { "id": 0, "entity": "面", "start_offset": 0, "end_offset": 1, "label": "bod" }, { "id": 1, "entity": "前额", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 2, "entity": "颈背", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 3, "entity": "面、前额或颈背可有一个或多个血管瘤", "start_offset": 0, "end_offset": 17, "label": "sym" } ]
第12肋骨发育不良或缺如。
[ { "id": 0, "entity": "肋骨发育不良或缺如", "start_offset": 3, "end_offset": 12, "label": "sym" } ]
80%病例有先天性心脏病,主要为室间隔缺损、动脉导管未闭及房间隔缺损等。
[ { "id": 0, "entity": "先天性心脏病", "start_offset": 6, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "室间隔缺损", "start_offset": 16, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "动脉导管未闭", "start_offset": 22, "end_offset": 28, "label": "dis" }, { "id": 3, "entity": "房间隔缺损", "start_offset": 29, "end_offset": 34, "label": "dis" } ]
消化道畸形可见结肠旋转不良、胰腺或脾组织异位等。
[ { "id": 0, "entity": "消化道畸形", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "结肠旋转不良", "start_offset": 7, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "胰腺", "start_offset": 14, "end_offset": 16, "label": "dis" }, { "id": 3, "entity": "脾组织异位", "start_offset": 17, "end_offset": 22, "label": "dis" } ]
常见六指(趾)畸形,指甲过度凸出。
[ { "id": 0, "entity": "指", "start_offset": 3, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "六指(趾)畸形", "start_offset": 2, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "指甲过度凸出", "start_offset": 10, "end_offset": 16, "label": "sym" } ]
30%~60%患儿有泌尿系畸形,可见多囊肾、肾盂积水、双肾及双输尿管。
[ { "id": 0, "entity": "泌尿系畸形", "start_offset": 10, "end_offset": 15, "label": "sym" }, { "id": 1, "entity": "多囊肾", "start_offset": 18, "end_offset": 21, "label": "sym" }, { "id": 2, "entity": "肾盂积水", "start_offset": 22, "end_offset": 26, "label": "sym" }, { "id": 3, "entity": "肾", "start_offset": 28, "end_offset": 29, "label": "bod" }, { "id": 4, "entity": "双肾及双输尿管", "start_offset": 27, "end_offset": 34, "label": "sym" } ]
男性80%有隐睾,见阴囊畸形。
[ { "id": 0, "entity": "男性80%有隐睾", "start_offset": 0, "end_offset": 8, "label": "sym" }, { "id": 1, "entity": "阴囊畸形", "start_offset": 10, "end_offset": 14, "label": "sym" } ]
女性可有双角子宫、阴蒂肥大及双阴道。
[ { "id": 0, "entity": "女性可有双角子宫", "start_offset": 0, "end_offset": 8, "label": "sym" }, { "id": 1, "entity": "阴蒂肥", "start_offset": 9, "end_offset": 12, "label": "sym" }, { "id": 2, "entity": "双阴道", "start_offset": 14, "end_offset": 17, "label": "sym" } ]
核型分析类同外周血淋巴细胞染色体检查。
[ { "id": 0, "entity": "外周血淋巴细胞染色体检查", "start_offset": 6, "end_offset": 18, "label": "pro" } ]
约占所有先天性心脏病的1%~4%。
[ { "id": 0, "entity": "心脏病", "start_offset": 7, "end_offset": 10, "label": "dis" } ]
Collett及Edwards依据体、肺循环状况将之分为四型:①Ⅰ型:有短段的肺总动脉存在并发出肺动脉分支;②Ⅱ型:无肺总动脉段,左、右肺动脉分别发自动脉干,但两者起源部分紧贴;③Ⅲ型:无肺总动脉段,左、右肺动脉分别发自动脉干,但两者起源部分远离;④Ⅳ型:称假性永存动脉干,肺循环由降主动脉发出侧支循环供应。
[ { "id": 0, "entity": "体、肺循环", "start_offset": 17, "end_offset": 22, "label": "bod" }, { "id": 1, "entity": "肺总动脉", "start_offset": 39, "end_offset": 43, "label": "bod" }, { "id": 2, "entity": "肺动脉分支", "start_offset": 48, "end_offset": 53, "label": "bod" }, { "id": 3, "entity": "肺总动脉段", "start_offset": 59, "end_offset": 64, "label": "bod" }, { "id": 4, "entity": "左、右肺动脉", "start_offset": 65, "end_offset": 71, "label": "bod" }, { "id": 5, "entity": "动脉干", "start_offset": 75, "end_offset": 78, "label": "bod" }, { "id": 6, "entity": "肺总动脉段", "start_offset": 94, "end_offset": 99, "label": "bod" }, { "id": 7, "entity": "左、右肺动脉", "start_offset": 100, "end_offset": 106, "label": "bod" }, { "id": 8, "entity": "动脉干", "start_offset": 110, "end_offset": 113, "label": "bod" }, { "id": 9, "entity": "假性永存动脉干", "start_offset": 129, "end_offset": 136, "label": "dis" }, { "id": 10, "entity": "肺循环", "start_offset": 137, "end_offset": 140, "label": "bod" }, { "id": 11, "entity": "降主动脉", "start_offset": 141, "end_offset": 145, "label": "bod" } ]
患儿多有共同干瓣膜的异常增厚,并可伴有结节。
[ { "id": 0, "entity": "干瓣膜的异常增厚", "start_offset": 6, "end_offset": 14, "label": "sym" }, { "id": 1, "entity": "结节", "start_offset": 19, "end_offset": 21, "label": "sym" } ]
30%的患儿可有共同干瓣膜的狭窄,50%患儿可伴有共同干瓣膜的反流,两者将直接影响该病的预后。
[ { "id": 0, "entity": "干瓣膜的狭窄", "start_offset": 10, "end_offset": 16, "label": "sym" }, { "id": 1, "entity": "干瓣膜的反流", "start_offset": 27, "end_offset": 33, "label": "sym" } ]
65%~70%的共同干瓣膜为三瓣,四瓣畸形的发生率在9%~24%之间,6%~23%可为二瓣畸形,已有单瓣畸形的报道。
[ { "id": 0, "entity": "干瓣膜", "start_offset": 10, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "四瓣畸形", "start_offset": 17, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "二瓣畸形", "start_offset": 43, "end_offset": 47, "label": "dis" }, { "id": 3, "entity": "单瓣畸形", "start_offset": 50, "end_offset": 54, "label": "dis" } ]
永存动脉干的冠状动脉畸形将影响外科手术的矫治。
[ { "id": 0, "entity": "永存动脉干", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "冠状动脉畸形", "start_offset": 6, "end_offset": 12, "label": "dis" }, { "id": 2, "entity": "外科", "start_offset": 15, "end_offset": 17, "label": "dep" } ]
13%~18%的患儿可为单支冠状动脉畸形。
[ { "id": 0, "entity": "单支冠状动脉畸形", "start_offset": 12, "end_offset": 20, "label": "dis" } ]
永存动脉干的最常见合并畸形是主动脉弓异常。
[ { "id": 0, "entity": "永存动脉干", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "畸形", "start_offset": 11, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "主动脉弓异常", "start_offset": 14, "end_offset": 20, "label": "dis" } ]
右位主动脉弓、主动脉弓中断的发生率分别为33%和19%。
[ { "id": 0, "entity": "右位主动脉弓、主动脉弓中断", "start_offset": 0, "end_offset": 13, "label": "sym" } ]
此外,永存动脉干中有近12%合并有左上腔静脉残存。
[ { "id": 0, "entity": "永存动脉干", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "左上腔静脉", "start_offset": 17, "end_offset": 22, "label": "bod" } ]
其他尚可合并部分性肺静脉异位引流、三尖瓣闭锁及房室间隔缺损等。
[ { "id": 0, "entity": "肺静脉异位引流", "start_offset": 9, "end_offset": 16, "label": "sym" }, { "id": 1, "entity": "三尖瓣闭锁", "start_offset": 17, "end_offset": 22, "label": "sym" }, { "id": 2, "entity": "房室间隔缺损", "start_offset": 23, "end_offset": 29, "label": "sym" } ]
临床上与室间隔缺损合并动脉导管未闭或主肺动脉间隔缺损合并室间隔缺损相似。
[ { "id": 0, "entity": "室间隔缺损", "start_offset": 4, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "动脉导管未闭", "start_offset": 11, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "主肺动脉间隔缺损", "start_offset": 18, "end_offset": 26, "label": "dis" }, { "id": 3, "entity": "室间隔缺损", "start_offset": 28, "end_offset": 33, "label": "dis" } ]
如有共同干反流则可及舒张早期杂音,如共同干与肺动脉分支间存在压力阶差时还可闻及连续性杂音。
[ { "id": 0, "entity": "共同干反流", "start_offset": 2, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "舒张早期杂音", "start_offset": 10, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "共同干", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 3, "entity": "肺动脉分支", "start_offset": 22, "end_offset": 27, "label": "bod" }, { "id": 4, "entity": "连续性杂音", "start_offset": 39, "end_offset": 44, "label": "sym" } ]
对于2岁以上的患儿应常规测量肺动脉阻力,当肺动脉阻力高于8单位/平方米时不宜进行手术。
[ { "id": 0, "entity": "肺动脉阻力", "start_offset": 14, "end_offset": 19, "label": "ite" }, { "id": 1, "entity": "肺动脉阻力", "start_offset": 21, "end_offset": 26, "label": "ite" } ]
内科的抗心力衰竭治疗包括地高辛及利尿剂的应用;在伴有主动脉弓中断时还应临时应用前列腺素E1,以保持动脉导管开放直至手术。
[ { "id": 0, "entity": "抗心力衰竭治疗", "start_offset": 3, "end_offset": 10, "label": "pro" }, { "id": 1, "entity": "地高辛", "start_offset": 12, "end_offset": 15, "label": "dru" }, { "id": 2, "entity": "利尿剂", "start_offset": 16, "end_offset": 19, "label": "dru" }, { "id": 3, "entity": "主动脉弓中断", "start_offset": 26, "end_offset": 32, "label": "sym" }, { "id": 4, "entity": "前列腺素E1", "start_offset": 39, "end_offset": 45, "label": "dru" }, { "id": 5, "entity": "动脉", "start_offset": 49, "end_offset": 51, "label": "bod" } ]
手术的危险因素包括共同干瓣膜反流、合并主动脉弓中断、冠状动脉异常及手术年龄。
[ { "id": 0, "entity": "干瓣膜反流", "start_offset": 11, "end_offset": 16, "label": "sym" }, { "id": 1, "entity": "合并主动脉弓中断", "start_offset": 17, "end_offset": 25, "label": "sym" }, { "id": 2, "entity": "冠状动脉异常", "start_offset": 26, "end_offset": 32, "label": "sym" } ]
出生30天后肺动脉高压发生率高,患儿如果出生后100天以上手术,手术死亡率将明显增高。
[ { "id": 0, "entity": "肺动脉高压", "start_offset": 6, "end_offset": 11, "label": "sym" } ]
患儿在百日咳病程中突然发热、气急,呼吸增快与体温不成比例,严重者可出现呼吸困难、发绀。
[ { "id": 0, "entity": "百日咳", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "发热", "start_offset": 11, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "气急", "start_offset": 14, "end_offset": 16, "label": "sym" }, { "id": 3, "entity": "呼吸增快", "start_offset": 17, "end_offset": 21, "label": "sym" }, { "id": 4, "entity": "体温不成比例", "start_offset": 22, "end_offset": 28, "label": "sym" }, { "id": 5, "entity": "呼吸困难", "start_offset": 35, "end_offset": 39, "label": "sym" }, { "id": 6, "entity": "发绀", "start_offset": 40, "end_offset": 42, "label": "sym" } ]
肺部可闻及细湿啰音,或出现实变体征。
[ { "id": 0, "entity": "肺部", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "细湿啰音", "start_offset": 5, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "实变体征", "start_offset": 13, "end_offset": 17, "label": "sym" } ]
剧烈咳嗽有时可造成肺泡破裂引起气胸、纵隔气肿或皮下气肿。
[ { "id": 0, "entity": "剧烈咳嗽", "start_offset": 0, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "肺泡", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "气胸", "start_offset": 15, "end_offset": 17, "label": "sym" }, { "id": 3, "entity": "纵隔气肿", "start_offset": 18, "end_offset": 22, "label": "sym" }, { "id": 4, "entity": "皮下气肿", "start_offset": 23, "end_offset": 27, "label": "sym" } ]
继发细菌感染者应送检痰培养及血培养。
[ { "id": 0, "entity": "痰培养", "start_offset": 10, "end_offset": 13, "label": "ite" }, { "id": 1, "entity": "血培养", "start_offset": 14, "end_offset": 17, "label": "ite" } ]
治疗首选红霉素,10~14天为一疗程。
[ { "id": 0, "entity": "红霉素", "start_offset": 4, "end_offset": 7, "label": "dru" } ]
必要时加用氨苄西林或利福平等。
[ { "id": 0, "entity": "氨苄西林", "start_offset": 5, "end_offset": 9, "label": "dru" }, { "id": 1, "entity": "利福平", "start_offset": 10, "end_offset": 13, "label": "dru" } ]
本病为小儿所特有,发生于手指或足趾,有复发倾向。
[ { "id": 0, "entity": "手指", "start_offset": 12, "end_offset": 14, "label": "bod" }, { "id": 1, "entity": "足趾", "start_offset": 15, "end_offset": 17, "label": "bod" } ]
纤维结节仅发生于指或趾,手指较足趾的发病率要高,女孩略多于男孩,无家族遗传史。
[ { "id": 0, "entity": "纤维结节", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "指", "start_offset": 8, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "趾", "start_offset": 10, "end_offset": 11, "label": "bod" }, { "id": 3, "entity": "手指", "start_offset": 12, "end_offset": 14, "label": "bod" }, { "id": 4, "entity": "足趾", "start_offset": 15, "end_offset": 17, "label": "bod" } ]
结节呈圆顶型,基底较宽,表面光滑,覆盖的皮肤呈淡红色,体积小,很少超过2cm。
[ { "id": 0, "entity": "结节", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "皮肤", "start_offset": 20, "end_offset": 22, "label": "bod" } ]
治疗以手术为主,手术切除容易,有60%的病例复发。
[ { "id": 0, "entity": "手术", "start_offset": 3, "end_offset": 5, "label": "pro" }, { "id": 1, "entity": "手术切除", "start_offset": 8, "end_offset": 12, "label": "pro" } ]
多在新生儿期出现呼吸困难及发绀。
[ { "id": 0, "entity": "呼吸困难", "start_offset": 8, "end_offset": 12, "label": "sym" }, { "id": 1, "entity": "发绀", "start_offset": 13, "end_offset": 15, "label": "sym" } ]
体格检查可见胸部呼吸运动减弱,心率加快,心前区易听到收缩期杂音。
[ { "id": 0, "entity": "体格检查", "start_offset": 0, "end_offset": 4, "label": "ite" }, { "id": 1, "entity": "胸部呼吸运动减弱", "start_offset": 6, "end_offset": 14, "label": "sym" }, { "id": 2, "entity": "心率加快", "start_offset": 15, "end_offset": 19, "label": "sym" }, { "id": 3, "entity": "心前区易听到收缩期杂音", "start_offset": 20, "end_offset": 31, "label": "sym" } ]
胸部X线摄片示肺野网状或颗粒状细小斑点影,偶可出现一侧肺异常透亮。
[ { "id": 0, "entity": "胸部X线摄片", "start_offset": 0, "end_offset": 6, "label": "ite" }, { "id": 1, "entity": "肺野网状或颗粒状细小斑点影", "start_offset": 7, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "偶可出现一侧肺异常透亮", "start_offset": 21, "end_offset": 32, "label": "sym" } ]
肠套叠分为原发性和继发性两种。
[ { "id": 0, "entity": "肠套叠", "start_offset": 0, "end_offset": 3, "label": "dis" } ]
随着病情进展,腹痛间歇可出现淡漠、嗜睡。
[ { "id": 0, "entity": "腹痛间歇可出现淡漠、嗜睡", "start_offset": 7, "end_offset": 19, "label": "sym" } ]
体格检查,早期生命体征平稳,腹痛发作时,可听到亢进的肠鸣音。
[ { "id": 0, "entity": "早期生命体征平稳", "start_offset": 5, "end_offset": 13, "label": "sym" }, { "id": 1, "entity": "腹痛", "start_offset": 14, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "亢进的肠鸣音", "start_offset": 23, "end_offset": 29, "label": "sym" } ]