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肠套叠初期,结肠蠕动增加,肠腔内压升高,患儿排出少量正常粪便;后期粪便中出现血迹,随之因肠缺血坏死而排暗红色血块或果酱样大便。
[ { "id": 0, "entity": "结肠", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "粪便", "start_offset": 28, "end_offset": 30, "label": "bod" }, { "id": 2, "entity": "粪便", "start_offset": 33, "end_offset": 35, "label": "bod" }, { "id": 3, "entity": "血", "start_offset": 38, "end_offset": 39, "label": "bod" } ]
发作间歇期触诊可有右下腹平坦空虚感,这是由于盲肠和回盲部套入横结肠至右上腹所致;此外还可触及部位不固定的包块。
[ { "id": 0, "entity": "盲肠", "start_offset": 22, "end_offset": 24, "label": "bod" } ]
梗阻时间过长的患儿可能出现脱水及菌血症,导致心动过速和发热,偶见低血容量性或感染性休克。
[ { "id": 0, "entity": "梗阻", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "脱水", "start_offset": 13, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "菌血症", "start_offset": 16, "end_offset": 19, "label": "dis" }, { "id": 3, "entity": "心动过速", "start_offset": 22, "end_offset": 26, "label": "sym" }, { "id": 4, "entity": "低血容量性或感染性休克", "start_offset": 32, "end_offset": 43, "label": "dis" } ]
若全身情况良好,可即刻行空气或液体(钡剂)灌肠。
[ { "id": 0, "entity": "空气或液体(钡剂)灌肠", "start_offset": 12, "end_offset": 23, "label": "pro" } ]
目前国内已普遍采用空气灌肠复位法,复位成功率为75%~94%。
[ { "id": 0, "entity": "空气灌肠复位法", "start_offset": 9, "end_offset": 16, "label": "pro" } ]
有的将有流出道狭窄的称为梗阻性心肌病。
[ { "id": 0, "entity": "梗阻性心肌病", "start_offset": 12, "end_offset": 18, "label": "dis" } ]
如左右心室都肥厚的称为对称性,否则称为非对称性。
[ { "id": 0, "entity": "左右心室", "start_offset": 1, "end_offset": 5, "label": "bod" } ]
一般对称性多数为非梗阻性,不对称多数为梗阻性,但也有左心室壁与室间隔肥厚,右心室壁不肥厚而左心室流出道不狭窄的,即只有不对称而无梗阻的。
[ { "id": 0, "entity": "左心室壁", "start_offset": 26, "end_offset": 30, "label": "bod" }, { "id": 1, "entity": "室间隔", "start_offset": 31, "end_offset": 34, "label": "bod" }, { "id": 2, "entity": "右心室壁", "start_offset": 37, "end_offset": 41, "label": "bod" }, { "id": 3, "entity": "左心室", "start_offset": 45, "end_offset": 48, "label": "bod" } ]
有的患儿室间隔特别肥厚,突入到左心室腔间,尤其在主动脉瓣下,表现为左心室流出道狭窄称为特发性肥厚性主动脉瓣下狭窄。
[ { "id": 0, "entity": "室间隔", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "左心室", "start_offset": 15, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "主动脉", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 3, "entity": "左心室流出道狭窄", "start_offset": 33, "end_offset": 41, "label": "sym" }, { "id": 4, "entity": "特发性肥厚性主动脉瓣下狭窄", "start_offset": 43, "end_offset": 56, "label": "dis" } ]
HCM通常以常染色体显性方式遗传,目前已知多个基因与典型的家族性肥厚性心肌病有关,这些基因均编码肌节蛋白,如β肌凝蛋白重链等。
[ { "id": 0, "entity": "HCM", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "肥厚性心肌病", "start_offset": 32, "end_offset": 38, "label": "dis" } ]
年小儿可表现为生长落后,心力衰竭的发生率较年长儿高。
[ { "id": 0, "entity": "生长落后", "start_offset": 7, "end_offset": 11, "label": "sym" }, { "id": 1, "entity": "心力衰竭", "start_offset": 12, "end_offset": 16, "label": "dis" } ]
体格检查部分病例在心尖可闻及全收缩期杂音,并向左腋下放射,此杂音是由于二尖瓣反流所致。
[ { "id": 0, "entity": "体格检查", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "心尖可闻及全收缩期杂音", "start_offset": 9, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "左腋下", "start_offset": 23, "end_offset": 26, "label": "bod" } ]
在婴儿,偶可应用心内膜心肌活体组织检查来确定病因,如线粒体肌病、糖原累积病等。
[ { "id": 0, "entity": "线粒体肌病", "start_offset": 26, "end_offset": 31, "label": "dis" }, { "id": 1, "entity": "糖原累积病", "start_offset": 32, "end_offset": 37, "label": "dis" } ]
不过现今骨骼肌活体组织检查更方便,且创伤更小。
[ { "id": 0, "entity": "骨骼肌活体组织检查", "start_offset": 4, "end_offset": 13, "label": "pro" } ]
β受体阻滞剂普萘洛尔(propranolol)为本病治疗的主要药物,它减慢心率,降低心肌收缩力,从而减轻左心室流出道梗阻;且可减低心肌的张力,使氧需量减少,缓解心绞痛心绞痛;此外,普萘洛尔尚有一定的抗心律失常作用。
[ { "id": 0, "entity": "β受体阻滞剂普萘洛尔", "start_offset": 0, "end_offset": 10, "label": "dru" }, { "id": 1, "entity": "propranolol", "start_offset": 11, "end_offset": 22, "label": "dru" }, { "id": 2, "entity": "心率", "start_offset": 37, "end_offset": 39, "label": "ite" }, { "id": 3, "entity": "心肌", "start_offset": 42, "end_offset": 44, "label": "bod" }, { "id": 4, "entity": "左心室流出道", "start_offset": 52, "end_offset": 58, "label": "bod" }, { "id": 5, "entity": "心肌", "start_offset": 65, "end_offset": 67, "label": "bod" }, { "id": 6, "entity": "心绞痛", "start_offset": 80, "end_offset": 83, "label": "sym" }, { "id": 7, "entity": "普萘洛尔", "start_offset": 90, "end_offset": 94, "label": "dru" }, { "id": 8, "entity": "心律", "start_offset": 100, "end_offset": 102, "label": "ite" } ]
其他临床上应用的选择性β受体阻滞剂有阿替洛尔(atenolol)、美托洛尔(metoprolol)等。
[ { "id": 0, "entity": "β受体阻滞剂", "start_offset": 11, "end_offset": 17, "label": "dru" }, { "id": 1, "entity": "阿替洛尔", "start_offset": 18, "end_offset": 22, "label": "dru" }, { "id": 2, "entity": "atenolol", "start_offset": 23, "end_offset": 31, "label": "dru" }, { "id": 3, "entity": "美托洛尔", "start_offset": 33, "end_offset": 37, "label": "dru" }, { "id": 4, "entity": "metoprolol", "start_offset": 38, "end_offset": 48, "label": "dru" } ]
维拉帕米(verapamil)主要用于成人HCM患者。
[ { "id": 0, "entity": "维拉帕米", "start_offset": 0, "end_offset": 4, "label": "dru" }, { "id": 1, "entity": "verapamil", "start_offset": 5, "end_offset": 14, "label": "dru" }, { "id": 2, "entity": "HCM", "start_offset": 21, "end_offset": 24, "label": "dis" } ]
其他手术方式有二尖瓣换置术及心尖主动脉管道,但因疗效不确切,且并发症多、在儿科均极少应用。
[ { "id": 0, "entity": "二尖瓣换置术", "start_offset": 7, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "心尖主动脉管道", "start_offset": 14, "end_offset": 21, "label": "pro" }, { "id": 2, "entity": "儿科", "start_offset": 37, "end_offset": 39, "label": "dep" } ]
心脏移植是另一治疗手段。
[ { "id": 0, "entity": "心脏移植", "start_offset": 0, "end_offset": 4, "label": "pro" } ]
目前已知此类疾病多达40余种,如神经纤维瘤病、结节性硬化症、脑面血管瘤病、色素失调症、伊藤色素减少症、面部半侧萎缩症、神经皮肤鱼鳞病、着色性干皮病、小脑视网膜血管瘤病、皮肤脑脊膜脊髓血管瘤病、黑棘皮病以及线状皮脂痣等。
[ { "id": 0, "entity": "神经纤维瘤病", "start_offset": 16, "end_offset": 22, "label": "dis" }, { "id": 1, "entity": "结节性硬化症", "start_offset": 23, "end_offset": 29, "label": "dis" }, { "id": 2, "entity": "脑面血管瘤病", "start_offset": 30, "end_offset": 36, "label": "dis" }, { "id": 3, "entity": "色素失调症", "start_offset": 37, "end_offset": 42, "label": "dis" }, { "id": 4, "entity": "伊藤色素减少症", "start_offset": 43, "end_offset": 50, "label": "dis" }, { "id": 5, "entity": "面部半侧萎缩症", "start_offset": 51, "end_offset": 58, "label": "dis" }, { "id": 6, "entity": "神经皮肤鱼鳞病", "start_offset": 59, "end_offset": 66, "label": "dis" }, { "id": 7, "entity": "着色性干皮病", "start_offset": 67, "end_offset": 73, "label": "dis" }, { "id": 8, "entity": "小脑视网膜血管瘤病", "start_offset": 74, "end_offset": 83, "label": "dis" }, { "id": 9, "entity": "皮肤脑脊膜脊髓血管瘤病", "start_offset": 84, "end_offset": 95, "label": "dis" }, { "id": 10, "entity": "黑棘皮病", "start_offset": 96, "end_offset": 100, "label": "dis" }, { "id": 11, "entity": "线状皮脂痣", "start_offset": 102, "end_offset": 107, "label": "dis" } ]
这些疾病多数属于常染色体显性遗传,有一个较高的、不完全的外显率。
[ { "id": 0, "entity": "常染色体显性遗传", "start_offset": 8, "end_offset": 16, "label": "sym" } ]
NFⅡ主要临床特点为双侧听神经瘤。
[ { "id": 0, "entity": "NFⅡ", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "双侧听神经瘤", "start_offset": 10, "end_offset": 16, "label": "dis" } ]
通常好发于躯干,随年龄增长有增多、扩大的趋势。
[ { "id": 0, "entity": "好发于躯干", "start_offset": 2, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "随年龄增长有增多、扩大的趋势", "start_offset": 8, "end_offset": 22, "label": "sym" } ]
皮肤纤维瘤和纤维软瘤主要分布于躯干、面部,也累及四肢。
[ { "id": 0, "entity": "皮肤纤维瘤", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "纤维软瘤", "start_offset": 6, "end_offset": 10, "label": "dis" }, { "id": 2, "entity": "躯干", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 3, "entity": "面部", "start_offset": 18, "end_offset": 20, "label": "bod" }, { "id": 4, "entity": "四肢", "start_offset": 24, "end_offset": 26, "label": "bod" } ]
部分累及脊髓和周围神经干。
[ { "id": 0, "entity": "脊髓", "start_offset": 4, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "周围神经干", "start_offset": 7, "end_offset": 12, "label": "bod" } ]
血管系统可见到肾动脉或颈动脉狭窄等。
[ { "id": 0, "entity": "血管系统", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "肾动脉或颈动脉狭窄", "start_offset": 7, "end_offset": 16, "label": "sym" } ]
此外,肾上腺、心、肺、消化道及纵隔等均可发生肿瘤。
[ { "id": 0, "entity": "肾上腺", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "心", "start_offset": 7, "end_offset": 8, "label": "bod" }, { "id": 2, "entity": "肺", "start_offset": 9, "end_offset": 10, "label": "bod" }, { "id": 3, "entity": "消化道", "start_offset": 11, "end_offset": 14, "label": "bod" }, { "id": 4, "entity": "纵隔", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 5, "entity": "肿瘤", "start_offset": 22, "end_offset": 24, "label": "dis" } ]
一般肿瘤症状在青春期或青春期以后出现,如前庭及耳蜗神经症状表现为耳鸣、眩晕和听力丧失(开始时往往是单侧);邻近颅神经受损症状表现为面部疼痛、面肌抽搐、面部感觉减退以及周围性轻面瘫等。
[ { "id": 0, "entity": "肿瘤", "start_offset": 2, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "在青春期或青春期以后出现", "start_offset": 6, "end_offset": 18, "label": "sym" }, { "id": 2, "entity": "前庭及耳蜗神经症状", "start_offset": 20, "end_offset": 29, "label": "sym" }, { "id": 3, "entity": "耳鸣", "start_offset": 32, "end_offset": 34, "label": "sym" }, { "id": 4, "entity": "眩晕", "start_offset": 35, "end_offset": 37, "label": "sym" }, { "id": 5, "entity": "听力丧失", "start_offset": 38, "end_offset": 42, "label": "sym" }, { "id": 6, "entity": "开始时往往是单侧", "start_offset": 43, "end_offset": 51, "label": "sym" }, { "id": 7, "entity": "邻近颅神经受损", "start_offset": 53, "end_offset": 60, "label": "dis" }, { "id": 8, "entity": "面部疼痛", "start_offset": 65, "end_offset": 69, "label": "sym" }, { "id": 9, "entity": "面肌抽搐", "start_offset": 70, "end_offset": 74, "label": "sym" }, { "id": 10, "entity": "面部感觉减退", "start_offset": 75, "end_offset": 81, "label": "sym" }, { "id": 11, "entity": "周围性轻面瘫", "start_offset": 83, "end_offset": 89, "label": "sym" } ]
异物刚进入时,患儿除表现为呛咳憋气、作呕、面色苍白、呼吸困难外,喘鸣音是特征性体征,并可见吸气性三凹征。
[ { "id": 0, "entity": "呛咳憋气", "start_offset": 13, "end_offset": 17, "label": "sym" }, { "id": 1, "entity": "作呕", "start_offset": 18, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "面色", "start_offset": 21, "end_offset": 23, "label": "ite" }, { "id": 3, "entity": "面色苍白", "start_offset": 21, "end_offset": 25, "label": "sym" }, { "id": 4, "entity": "呼吸", "start_offset": 26, "end_offset": 28, "label": "ite" }, { "id": 5, "entity": "呼吸困难", "start_offset": 26, "end_offset": 30, "label": "sym" }, { "id": 6, "entity": "喘鸣音", "start_offset": 32, "end_offset": 35, "label": "sym" }, { "id": 7, "entity": "吸气性三凹征", "start_offset": 45, "end_offset": 51, "label": "dis" } ]
异物较大时可造成呼吸道阻塞窒息甚至死亡;异物较小可进一步进入支气管和细支气管,听诊时可发现一侧呼吸音减轻,同时,由于异物的刺激可出现肺炎体征,炎症如未能控制,还可出现肺不张,气胸、纵隔气肿等。
[ { "id": 0, "entity": "呼吸道阻塞窒息", "start_offset": 8, "end_offset": 15, "label": "sym" }, { "id": 1, "entity": "死亡", "start_offset": 17, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "支气管", "start_offset": 30, "end_offset": 33, "label": "bod" }, { "id": 3, "entity": "细支气管", "start_offset": 34, "end_offset": 38, "label": "bod" }, { "id": 4, "entity": "听诊", "start_offset": 39, "end_offset": 41, "label": "pro" }, { "id": 5, "entity": "一侧呼吸音减轻", "start_offset": 45, "end_offset": 52, "label": "sym" }, { "id": 6, "entity": "肺炎", "start_offset": 66, "end_offset": 68, "label": "dis" }, { "id": 7, "entity": "肺不张", "start_offset": 83, "end_offset": 86, "label": "sym" }, { "id": 8, "entity": "气胸", "start_offset": 87, "end_offset": 89, "label": "sym" }, { "id": 9, "entity": "纵隔气肿", "start_offset": 90, "end_offset": 94, "label": "sym" } ]
通常分为两大类:①原发性者:包括芬兰型先天性肾病综合征、弥漫性系膜硬化、微小病变及局灶节段性硬化;②继发性者:可继发于感染(先天梅毒、先天性毒浆原虫病、先天性巨细胞包涵体病、风疹、肝炎、疟疾及艾滋病等)、汞中毒、婴儿系统性红斑狼疮、溶血尿毒综合征、甲髌综合征、Drash综合征及肾静脉血栓形成等。
[ { "id": 0, "entity": "芬兰型先天性肾病综合征", "start_offset": 16, "end_offset": 27, "label": "dis" }, { "id": 1, "entity": "弥漫性系膜硬化", "start_offset": 28, "end_offset": 35, "label": "sym" }, { "id": 2, "entity": "微小病变及局灶节段性硬化", "start_offset": 36, "end_offset": 48, "label": "sym" }, { "id": 3, "entity": "感染", "start_offset": 59, "end_offset": 61, "label": "dis" }, { "id": 4, "entity": "先天梅毒", "start_offset": 62, "end_offset": 66, "label": "dis" }, { "id": 5, "entity": "先天性毒浆原虫病", "start_offset": 67, "end_offset": 75, "label": "dis" }, { "id": 6, "entity": "先天性巨细胞包涵体病", "start_offset": 76, "end_offset": 86, "label": "dis" }, { "id": 7, "entity": "风疹", "start_offset": 87, "end_offset": 89, "label": "dis" }, { "id": 8, "entity": "肝炎", "start_offset": 90, "end_offset": 92, "label": "dis" }, { "id": 9, "entity": "疟疾", "start_offset": 93, "end_offset": 95, "label": "dis" }, { "id": 10, "entity": "艾滋病", "start_offset": 96, "end_offset": 99, "label": "dis" }, { "id": 11, "entity": "汞中毒", "start_offset": 102, "end_offset": 105, "label": "dis" }, { "id": 12, "entity": "婴儿系统性红斑狼疮", "start_offset": 106, "end_offset": 115, "label": "dis" }, { "id": 13, "entity": "溶血尿毒综合征", "start_offset": 116, "end_offset": 123, "label": "dis" }, { "id": 14, "entity": "甲髌综合征", "start_offset": 124, "end_offset": 129, "label": "dis" }, { "id": 15, "entity": "Drash综合征", "start_offset": 130, "end_offset": 138, "label": "dis" }, { "id": 16, "entity": "肾静脉血栓", "start_offset": 139, "end_offset": 144, "label": "dis" } ]
免疫荧光检查病早期无Ig和补体沉着。
[ { "id": 0, "entity": "免疫荧光检查病", "start_offset": 0, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "Ig", "start_offset": 10, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "早期无Ig和补体沉着", "start_offset": 7, "end_offset": 17, "label": "sym" } ]
电镜示内皮细胞肿胀,上皮细胞足突融合,基膜皱缩等。
[ { "id": 0, "entity": "电镜", "start_offset": 0, "end_offset": 2, "label": "equ" }, { "id": 1, "entity": "内皮细胞", "start_offset": 3, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "内皮细胞肿胀", "start_offset": 3, "end_offset": 9, "label": "sym" }, { "id": 3, "entity": "上皮细胞", "start_offset": 10, "end_offset": 14, "label": "bod" }, { "id": 4, "entity": "上皮细胞足突融合", "start_offset": 10, "end_offset": 18, "label": "sym" }, { "id": 5, "entity": "基膜", "start_offset": 19, "end_offset": 21, "label": "bod" }, { "id": 6, "entity": "基膜皱缩", "start_offset": 19, "end_offset": 23, "label": "sym" } ]
出生后常见特殊的外貌,如:鼻梁低、眼距宽、低位耳、颅缝宽、前囟和后囟宽大,还常见髋、膝及肘部呈屈曲畸形。
[ { "id": 0, "entity": "鼻梁低", "start_offset": 13, "end_offset": 16, "label": "sym" }, { "id": 1, "entity": "眼距宽", "start_offset": 17, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "低位耳", "start_offset": 21, "end_offset": 24, "label": "sym" }, { "id": 3, "entity": "颅缝宽", "start_offset": 25, "end_offset": 28, "label": "sym" }, { "id": 4, "entity": "前囟", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 5, "entity": "后囟", "start_offset": 32, "end_offset": 34, "label": "bod" }, { "id": 6, "entity": "前囟和后囟宽大", "start_offset": 29, "end_offset": 36, "label": "sym" }, { "id": 7, "entity": "髋", "start_offset": 40, "end_offset": 41, "label": "bod" }, { "id": 8, "entity": "膝", "start_offset": 42, "end_offset": 43, "label": "bod" }, { "id": 9, "entity": "肘部", "start_offset": 44, "end_offset": 46, "label": "bod" }, { "id": 10, "entity": "髋、膝及肘部呈屈曲畸形", "start_offset": 40, "end_offset": 51, "label": "sym" } ]
其后常见腹胀、腹水及脐疝。
[ { "id": 0, "entity": "腹胀", "start_offset": 4, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "腹水", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "脐疝", "start_offset": 10, "end_offset": 12, "label": "sym" } ]
小婴儿有不能解释的肾病综合征伴外生殖器异常,则应考虑到Drash综合征。
[ { "id": 0, "entity": "肾病综合征", "start_offset": 9, "end_offset": 14, "label": "dis" }, { "id": 1, "entity": "外生殖器", "start_offset": 15, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "外生殖器异常", "start_offset": 15, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "Drash综合征", "start_offset": 27, "end_offset": 35, "label": "dis" } ]
对严重低白蛋白血症,或伴低血容量表现者可输注无盐白蛋白。
[ { "id": 0, "entity": "严重低白蛋白血症", "start_offset": 1, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "低血容量", "start_offset": 12, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "输注", "start_offset": 20, "end_offset": 22, "label": "pro" }, { "id": 3, "entity": "无盐白蛋白", "start_offset": 22, "end_offset": 27, "label": "dru" } ]
芬兰有主张自生后4周即静脉输白蛋白,维持其血浆蛋白在15g/L以上,则此时一般可无水肿且生长发育接近正常。
[ { "id": 0, "entity": "静脉", "start_offset": 11, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "白蛋白", "start_offset": 14, "end_offset": 17, "label": "dru" }, { "id": 2, "entity": "血浆蛋白", "start_offset": 21, "end_offset": 25, "label": "ite" }, { "id": 3, "entity": "无水肿", "start_offset": 40, "end_offset": 43, "label": "sym" }, { "id": 4, "entity": "生长发育接近正常", "start_offset": 44, "end_offset": 52, "label": "sym" } ]
继发性CNS随着其病因的不同而有着不同的预后,如感染所致者,采用强有力的抗感染治疗,病情常明显好转。
[ { "id": 0, "entity": "继发性CNS", "start_offset": 0, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "感染", "start_offset": 24, "end_offset": 26, "label": "dis" }, { "id": 2, "entity": "抗感染治疗", "start_offset": 36, "end_offset": 41, "label": "pro" } ]
因在胸片上右肺下野见一特征性新月形阴影,故又可称“弯刀综合征”。
[ { "id": 0, "entity": "胸片", "start_offset": 2, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "右肺", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "弯刀综合征", "start_offset": 25, "end_offset": 30, "label": "dis" } ]
伴有房间隔缺损者,体检以房缺体征为主。
[ { "id": 0, "entity": "房间隔缺损", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "体检", "start_offset": 9, "end_offset": 11, "label": "ite" }, { "id": 2, "entity": "房缺", "start_offset": 12, "end_offset": 14, "label": "sym" } ]
若肺循环∶体循环大于2∶1,则需手术纠正。
[ { "id": 0, "entity": "肺", "start_offset": 1, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "手术纠正", "start_offset": 16, "end_offset": 20, "label": "pro" } ]
右上肺静脉异位引流伴静脉窦型房缺患儿,术后30%~40%可发生病态窦房结综合征。
[ { "id": 0, "entity": "右上肺静脉异位引流", "start_offset": 0, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "静脉窦型房缺", "start_offset": 10, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "病态窦房结综合征", "start_offset": 31, "end_offset": 39, "label": "dis" } ]
几乎全部出现贫血症状,可有面色苍白、乏力、心率增快,心前区可闻及吹风样收缩期杂音。
[ { "id": 0, "entity": "出现贫血症状", "start_offset": 4, "end_offset": 10, "label": "sym" }, { "id": 1, "entity": "面色苍白", "start_offset": 13, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "乏力", "start_offset": 18, "end_offset": 20, "label": "sym" }, { "id": 3, "entity": "心率增快", "start_offset": 21, "end_offset": 25, "label": "sym" }, { "id": 4, "entity": "心前区可闻及吹风样收缩期杂音", "start_offset": 26, "end_offset": 40, "label": "sym" } ]
严重者可出现心力衰竭和呼吸衰竭。
[ { "id": 0, "entity": "心力衰竭", "start_offset": 6, "end_offset": 10, "label": "sym" }, { "id": 1, "entity": "呼吸衰竭", "start_offset": 11, "end_offset": 15, "label": "sym" } ]
注意排除继发性肺含铁血黄素沉着症,如继发于二尖瓣狭窄以及任何原因引起的左心衰竭,或肺内毛细血管压力长期增高的心脏病患者及结缔组织疾病如结节性多发性动脉炎、类风湿病、过敏性紫癜。
[ { "id": 0, "entity": "继发性肺含铁血黄素沉着症", "start_offset": 4, "end_offset": 16, "label": "dis" }, { "id": 1, "entity": "二尖瓣", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "左心衰竭", "start_offset": 35, "end_offset": 39, "label": "dis" }, { "id": 3, "entity": "肺", "start_offset": 41, "end_offset": 42, "label": "bod" }, { "id": 4, "entity": "毛细血管", "start_offset": 43, "end_offset": 47, "label": "bod" }, { "id": 5, "entity": "心脏病", "start_offset": 54, "end_offset": 57, "label": "dis" }, { "id": 6, "entity": "结缔组织", "start_offset": 60, "end_offset": 64, "label": "bod" }, { "id": 7, "entity": "结节性多发性动脉炎", "start_offset": 67, "end_offset": 76, "label": "dis" }, { "id": 8, "entity": "类风湿病", "start_offset": 77, "end_offset": 81, "label": "dis" }, { "id": 9, "entity": "过敏性紫癜", "start_offset": 82, "end_offset": 87, "label": "dis" } ]
本病尚有两种特殊类型:①Goodpasture综合征:是一种免疫复合物病,具体病因不明,病变同时累及肺和肾脏,病情严重,可见发热、咳嗽、咯血,常发生呼吸困难,有显著贫血,尿中有蛋白质、红细胞、管型。
[ { "id": 0, "entity": "Goodpasture综合征", "start_offset": 12, "end_offset": 26, "label": "dis" }, { "id": 1, "entity": "肺", "start_offset": 50, "end_offset": 51, "label": "bod" }, { "id": 2, "entity": "肾脏", "start_offset": 52, "end_offset": 54, "label": "bod" }, { "id": 3, "entity": "发热", "start_offset": 62, "end_offset": 64, "label": "sym" }, { "id": 4, "entity": "咳嗽", "start_offset": 65, "end_offset": 67, "label": "sym" }, { "id": 5, "entity": "咯血", "start_offset": 68, "end_offset": 70, "label": "sym" }, { "id": 6, "entity": "呼吸困难", "start_offset": 74, "end_offset": 78, "label": "sym" }, { "id": 7, "entity": "显著贫血", "start_offset": 80, "end_offset": 84, "label": "sym" }, { "id": 8, "entity": "蛋白质", "start_offset": 88, "end_offset": 91, "label": "bod" }, { "id": 9, "entity": "红细胞", "start_offset": 92, "end_offset": 95, "label": "bod" }, { "id": 10, "entity": "管型", "start_offset": 96, "end_offset": 98, "label": "bod" } ]
重症或急性期以静脉给药为主,如甲基泼尼松龙、氢化可的松、地塞米松等,病情好转后减量口服,以泼尼松维持,疗程至少3个月,一般为半年至1年,反复发作者可适当延长。
[ { "id": 0, "entity": "甲基泼尼松龙", "start_offset": 15, "end_offset": 21, "label": "dru" }, { "id": 1, "entity": "氢化可的松", "start_offset": 22, "end_offset": 27, "label": "dru" }, { "id": 2, "entity": "地塞米松", "start_offset": 28, "end_offset": 32, "label": "dru" }, { "id": 3, "entity": "泼尼松", "start_offset": 45, "end_offset": 48, "label": "dru" } ]
肾上腺皮质激素治疗无效者可试用免疫抑制剂,如硫唑嘌呤、环磷酰胺,疗程一般3个月,可与肾上腺皮质激素联合应用。
[ { "id": 0, "entity": "肾上腺皮质激素", "start_offset": 0, "end_offset": 7, "label": "dru" }, { "id": 1, "entity": "免疫抑制剂", "start_offset": 15, "end_offset": 20, "label": "dru" }, { "id": 2, "entity": "硫唑嘌呤", "start_offset": 22, "end_offset": 26, "label": "dru" }, { "id": 3, "entity": "环磷酰胺", "start_offset": 27, "end_offset": 31, "label": "dru" }, { "id": 4, "entity": "肾上腺皮质激素", "start_offset": 42, "end_offset": 49, "label": "dru" } ]
输血和铁剂治疗虽可改善贫血,但由于可能增加肺内铁沉积,故应慎用。
[ { "id": 0, "entity": "输血", "start_offset": 0, "end_offset": 2, "label": "pro" }, { "id": 1, "entity": "铁剂治疗", "start_offset": 3, "end_offset": 7, "label": "pro" }, { "id": 2, "entity": "贫血", "start_offset": 11, "end_offset": 13, "label": "dis" }, { "id": 3, "entity": "肺", "start_offset": 21, "end_offset": 22, "label": "bod" } ]
代谢性碱中毒无特征性临床表现。
[ { "id": 0, "entity": "代谢性碱中毒", "start_offset": 0, "end_offset": 6, "label": "dis" } ]
轻度代碱可无明显症状,重症者表现为呼吸抑制,精神萎靡。
[ { "id": 0, "entity": "呼吸抑制", "start_offset": 17, "end_offset": 21, "label": "sym" }, { "id": 1, "entity": "精神萎靡", "start_offset": 22, "end_offset": 26, "label": "sym" } ]
国外学者认为心肌炎的发生率通常被低估。
[ { "id": 0, "entity": "心肌炎", "start_offset": 6, "end_offset": 9, "label": "dis" } ]
在特发性扩张性心肌病成人患者,心肌炎的发生率为3%~63%。
[ { "id": 0, "entity": "心肌炎", "start_offset": 15, "end_offset": 18, "label": "dis" } ]
有时病毒可同时侵犯其他系统如,肌肉、大脑等,并出现相应症状及体征。
[ { "id": 0, "entity": "病毒", "start_offset": 2, "end_offset": 4, "label": "mic" }, { "id": 1, "entity": "肌肉", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "大脑", "start_offset": 18, "end_offset": 20, "label": "bod" } ]
现分述病毒性心肌炎各期主要症状、体征。
[ { "id": 0, "entity": "病毒性心肌炎", "start_offset": 3, "end_offset": 9, "label": "dis" } ]
轻型症状以乏力为主,其次有多汗、苍白、心悸、气短、胸闷、头晕、精神食欲缺乏等。
[ { "id": 0, "entity": "苍白", "start_offset": 16, "end_offset": 18, "label": "sym" }, { "id": 1, "entity": "心悸", "start_offset": 19, "end_offset": 21, "label": "sym" }, { "id": 2, "entity": "气短", "start_offset": 22, "end_offset": 24, "label": "sym" }, { "id": 3, "entity": "胸闷", "start_offset": 25, "end_offset": 27, "label": "sym" }, { "id": 4, "entity": "头晕", "start_offset": 28, "end_offset": 30, "label": "sym" }, { "id": 5, "entity": "精神食欲缺乏", "start_offset": 31, "end_offset": 37, "label": "sym" } ]
起病较急,除前述症状外,乏力突出,年长儿常诉心前区疼痛。
[ { "id": 0, "entity": "心前区疼痛", "start_offset": 22, "end_offset": 27, "label": "sym" } ]
起病较急者可伴恶心、呕吐。
[ { "id": 0, "entity": "恶心", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "呕吐", "start_offset": 10, "end_offset": 12, "label": "sym" } ]
患儿极度乏力、头晕、烦躁、呕吐、心前区疼痛或压迫感。
[ { "id": 0, "entity": "乏力", "start_offset": 4, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "头晕", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "烦躁", "start_offset": 10, "end_offset": 12, "label": "sym" }, { "id": 3, "entity": "呕吐", "start_offset": 13, "end_offset": 15, "label": "sym" }, { "id": 4, "entity": "心前区疼痛或压迫感", "start_offset": 16, "end_offset": 25, "label": "sym" } ]
有的发生急性左心力衰竭、肺水肿。
[ { "id": 0, "entity": "急性左心力衰竭", "start_offset": 4, "end_offset": 11, "label": "sym" }, { "id": 1, "entity": "肺", "start_offset": 12, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "肺水肿", "start_offset": 12, "end_offset": 15, "label": "sym" } ]
若血清中CK-MB明显增高则多提示心肌受累,与CK总活性相比,对判断心肌损伤有较高的特异性和敏感性。
[ { "id": 0, "entity": "血清", "start_offset": 1, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "心肌", "start_offset": 34, "end_offset": 36, "label": "bod" } ]
处理原则与一般心力衰竭及心源性休克相似,包括洋地黄的应用、血管扩张剂、磷酸二酯酶抑制剂、利尿剂及扩容纠正酸中毒等,但在洋地黄应用时应注意在病毒性心肌炎急性期,心肌对洋地黄敏感,易出现毒性反应,应避免快饱和,用药剂量也应适当减少。
[ { "id": 0, "entity": "心肌", "start_offset": 79, "end_offset": 81, "label": "bod" } ]
室上性心动过速洋地黄治疗有效,室性心动过速可用利多卡因或胺碘酮静滴。
[ { "id": 0, "entity": "室上性心动过速", "start_offset": 0, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "洋地黄", "start_offset": 7, "end_offset": 10, "label": "dru" }, { "id": 2, "entity": "室性心动过速", "start_offset": 15, "end_offset": 21, "label": "dis" } ]
如室性心律失常虽经积极治疗仍快速进展至室性纤颤(这种情况在小婴儿更易发生)应即刻予以直流电复律。
[ { "id": 0, "entity": "室性心律失常", "start_offset": 1, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "室性纤颤", "start_offset": 19, "end_offset": 23, "label": "dis" }, { "id": 2, "entity": "直流电复律", "start_offset": 42, "end_offset": 47, "label": "pro" } ]
严重的二尖瓣梗阻将出现外周循环灌注不良,脉搏微弱。
[ { "id": 0, "entity": "严重的二尖瓣梗阻", "start_offset": 0, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "外周循环灌注不良", "start_offset": 11, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "脉搏", "start_offset": 20, "end_offset": 22, "label": "ite" }, { "id": 3, "entity": "脉搏微弱", "start_offset": 20, "end_offset": 24, "label": "sym" } ]
与风湿性心瓣膜病相反,绝大多数的患儿第一心音柔和,无二尖瓣开放音。
[ { "id": 0, "entity": "风湿性心瓣膜病", "start_offset": 1, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "第一心音柔和", "start_offset": 18, "end_offset": 24, "label": "sym" }, { "id": 2, "entity": "二尖瓣", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 3, "entity": "无二尖瓣开放音", "start_offset": 25, "end_offset": 32, "label": "sym" } ]
二维超声心动图及多普勒超声心动图可清晰显示肺静脉解剖及血流正常,肺静脉回流至副房并通过其与真左房间隔膜的狭窄开口进入真左房。
[ { "id": 0, "entity": "二维超声心动图", "start_offset": 0, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "多普勒超声心动图", "start_offset": 8, "end_offset": 16, "label": "pro" }, { "id": 2, "entity": "肺静脉", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 3, "entity": "肺静脉", "start_offset": 32, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "副房", "start_offset": 38, "end_offset": 40, "label": "bod" }, { "id": 5, "entity": "真左房间隔膜", "start_offset": 45, "end_offset": 51, "label": "bod" }, { "id": 6, "entity": "真左房", "start_offset": 58, "end_offset": 61, "label": "bod" } ]
彩色多普勒超声心动图可提供二尖瓣舒张期正向湍流的彩色直观显示,并可对狭窄或反流的位置进行定位。
[ { "id": 0, "entity": "彩色多普勒超声心动图", "start_offset": 0, "end_offset": 10, "label": "pro" }, { "id": 1, "entity": "二尖瓣", "start_offset": 13, "end_offset": 16, "label": "bod" } ]
有时尚需进行吸氧或肺血管扩张试验,以判断肺血管床病理改变。
[ { "id": 0, "entity": "吸氧", "start_offset": 6, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "肺血管扩张", "start_offset": 9, "end_offset": 14, "label": "pro" }, { "id": 2, "entity": "肺血管床", "start_offset": 20, "end_offset": 24, "label": "bod" } ]
当患儿有明显肺动脉高压表现时应进行心血管造影。
[ { "id": 0, "entity": "肺动脉高压", "start_offset": 6, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "心血管造影", "start_offset": 17, "end_offset": 22, "label": "pro" } ]
患儿高热,中毒症状明显,可伴有多种器官损害,如肺炎、肝炎、肾炎、心肌炎及关节炎等,甚至发生循环衰竭。
[ { "id": 0, "entity": "高热", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "中毒症状明显", "start_offset": 5, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "伴有多种器官损害", "start_offset": 13, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "肺炎", "start_offset": 23, "end_offset": 25, "label": "dis" }, { "id": 4, "entity": "肝炎", "start_offset": 26, "end_offset": 28, "label": "dis" }, { "id": 5, "entity": "肾炎", "start_offset": 29, "end_offset": 31, "label": "dis" }, { "id": 6, "entity": "心肌炎", "start_offset": 32, "end_offset": 35, "label": "dis" }, { "id": 7, "entity": "关节炎", "start_offset": 36, "end_offset": 39, "label": "dis" } ]
皮疹迅速融合成片,成水肿样,其上形成水疱、大疱,疱破后形成糜烂面,似Ⅱ度烧伤,渗出大量浆液或出血性浆液,若无继发感染,1~4周后结痂脱屑,有色素沉着,不留瘢痕。
[ { "id": 0, "entity": "皮疹", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "水肿", "start_offset": 10, "end_offset": 12, "label": "dis" }, { "id": 2, "entity": "水疱、大疱,疱破后形成糜烂面", "start_offset": 18, "end_offset": 32, "label": "sym" }, { "id": 3, "entity": "Ⅱ度烧伤", "start_offset": 34, "end_offset": 38, "label": "dis" }, { "id": 4, "entity": "渗出大量浆液或出血性浆液", "start_offset": 39, "end_offset": 51, "label": "sym" }, { "id": 5, "entity": "结痂脱屑", "start_offset": 64, "end_offset": 68, "label": "sym" }, { "id": 6, "entity": "有色素沉着", "start_offset": 69, "end_offset": 74, "label": "sym" }, { "id": 7, "entity": "不留瘢痕", "start_offset": 75, "end_offset": 79, "label": "sym" } ]
若存活期长,还易发生肿瘤和自身免疫性疾病。
[ { "id": 0, "entity": "肿瘤", "start_offset": 10, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "自身免疫性疾病", "start_offset": 13, "end_offset": 20, "label": "dis" } ]
也可见全身感染,如脓毒血症、败血症、脑膜炎和骨、关节炎。
[ { "id": 0, "entity": "全身感染", "start_offset": 3, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "脓毒血症", "start_offset": 9, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "败血症", "start_offset": 14, "end_offset": 17, "label": "dis" }, { "id": 3, "entity": "脑膜炎", "start_offset": 18, "end_offset": 21, "label": "dis" }, { "id": 4, "entity": "骨、关节炎", "start_offset": 22, "end_offset": 27, "label": "dis" } ]
B细胞淋巴瘤最多见,T细胞和霍奇金病也可发生。
[ { "id": 0, "entity": "B细胞淋巴瘤", "start_offset": 0, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "T细胞", "start_offset": 10, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "霍奇金病", "start_offset": 14, "end_offset": 18, "label": "dis" } ]
临床发现伴发淋巴瘤的PID常为SCID、嘌呤核苷磷酸化酶(PNP)缺乏、XLA、X连锁淋巴组织增生症、高IgM血症(HM)、IgA缺乏症、IgG亚类缺陷、常见变异型免疫缺陷病(CVID)、湿疹血小板减少伴免疫缺陷(WAS)、毛细血管扩张共济失调综合征(AT)以及胸腺发育不全等。
[ { "id": 0, "entity": "淋巴瘤", "start_offset": 6, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "PID", "start_offset": 10, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "SCID", "start_offset": 15, "end_offset": 19, "label": "dis" }, { "id": 3, "entity": "嘌呤核苷磷酸化酶(PNP)缺乏", "start_offset": 20, "end_offset": 35, "label": "dis" }, { "id": 4, "entity": "X连锁淋巴组织增生症", "start_offset": 40, "end_offset": 50, "label": "dis" }, { "id": 5, "entity": "高IgM血症", "start_offset": 51, "end_offset": 57, "label": "dis" }, { "id": 6, "entity": "HM", "start_offset": 58, "end_offset": 60, "label": "dis" }, { "id": 7, "entity": "IgA缺乏症", "start_offset": 62, "end_offset": 68, "label": "dis" }, { "id": 8, "entity": "IgG亚类缺陷", "start_offset": 69, "end_offset": 76, "label": "dis" }, { "id": 9, "entity": "变异型免疫缺陷病", "start_offset": 79, "end_offset": 87, "label": "dis" }, { "id": 10, "entity": "CVID", "start_offset": 88, "end_offset": 92, "label": "dis" }, { "id": 11, "entity": "湿疹血小板减少", "start_offset": 94, "end_offset": 101, "label": "dis" }, { "id": 12, "entity": "免疫缺陷", "start_offset": 102, "end_offset": 106, "label": "dis" }, { "id": 13, "entity": "WAS", "start_offset": 107, "end_offset": 110, "label": "dis" }, { "id": 14, "entity": "毛细血管扩张共济失调综合征", "start_offset": 112, "end_offset": 125, "label": "dis" }, { "id": 15, "entity": "AT", "start_offset": 126, "end_offset": 128, "label": "dis" }, { "id": 16, "entity": "胸腺发育不全", "start_offset": 131, "end_offset": 137, "label": "dis" } ]
也有伴发白血病、胶质瘤、肝胆管瘤以及横纹肌肉瘤的报道。
[ { "id": 0, "entity": "白血病", "start_offset": 4, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "胶质瘤", "start_offset": 8, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "肝胆管瘤", "start_offset": 12, "end_offset": 16, "label": "dis" }, { "id": 3, "entity": "横纹肌肉瘤", "start_offset": 18, "end_offset": 23, "label": "dis" } ]
有一定抗体反应者可考虑给予死疫苗接种,细胞免疫缺陷病人不宜接种存活的疫苗如口服灰髓炎疫苗,以免感染患儿。
[ { "id": 0, "entity": "抗体", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "疫苗", "start_offset": 34, "end_offset": 36, "label": "dru" }, { "id": 2, "entity": "灰髓炎疫苗", "start_offset": 39, "end_offset": 44, "label": "dru" } ]
已确诊为T细胞缺陷患儿不宜输新鲜血制品,以防发生移植物抗宿主反应(GVHR),必需输血或新鲜血制品时,应先用射线(2000~3000rad)处理,血制品还要严格筛查CMV,以防血源性CMV感染。
[ { "id": 0, "entity": "T细胞缺陷", "start_offset": 4, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "输新鲜血制品", "start_offset": 13, "end_offset": 19, "label": "pro" }, { "id": 2, "entity": "移植物抗宿主反应", "start_offset": 24, "end_offset": 32, "label": "dis" }, { "id": 3, "entity": "GVHR", "start_offset": 33, "end_offset": 37, "label": "dis" }, { "id": 4, "entity": "输血", "start_offset": 41, "end_offset": 43, "label": "pro" }, { "id": 5, "entity": "新鲜血制品", "start_offset": 44, "end_offset": 49, "label": "dru" }, { "id": 6, "entity": "射线", "start_offset": 54, "end_offset": 56, "label": "pro" }, { "id": 7, "entity": "血制品", "start_offset": 73, "end_offset": 76, "label": "dru" }, { "id": 8, "entity": "筛查CMV", "start_offset": 80, "end_offset": 85, "label": "pro" }, { "id": 9, "entity": "血源性CMV感染", "start_offset": 88, "end_offset": 96, "label": "dis" } ]
大约80%PID患儿伴有不同程度IgG或其他抗体缺乏,因此补充IgG(IVIG)是最常见的替代治疗措施。
[ { "id": 0, "entity": "PID", "start_offset": 5, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "IgG", "start_offset": 16, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "抗体缺乏", "start_offset": 22, "end_offset": 26, "label": "dis" }, { "id": 3, "entity": "补充IgG", "start_offset": 29, "end_offset": 34, "label": "pro" }, { "id": 4, "entity": "IVIG", "start_offset": 35, "end_offset": 39, "label": "pro" }, { "id": 5, "entity": "替代治疗", "start_offset": 45, "end_offset": 49, "label": "pro" } ]
其他替代治疗包括特异性免疫血清,输白细胞,细胞因子(转移因子,胸腺素)。
[ { "id": 0, "entity": "替代治疗", "start_offset": 2, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "特异性免疫血清", "start_offset": 8, "end_offset": 15, "label": "dru" }, { "id": 2, "entity": "输白细胞", "start_offset": 16, "end_offset": 20, "label": "pro" }, { "id": 3, "entity": "细胞因子", "start_offset": 21, "end_offset": 25, "label": "dru" }, { "id": 4, "entity": "转移因子", "start_offset": 26, "end_offset": 30, "label": "dru" }, { "id": 5, "entity": "胸腺素", "start_offset": 31, "end_offset": 34, "label": "dru" } ]
约50%的LQTS为家族性:Romano-Ward综合征为常染色体显性遗传,可伴有先天性耳聋;Jirvell-Lange-Nielsen综合征为常染色体隐性遗传。
[ { "id": 0, "entity": "LQTS", "start_offset": 5, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "Romano-Ward综合征", "start_offset": 14, "end_offset": 28, "label": "dis" }, { "id": 2, "entity": "染色体", "start_offset": 30, "end_offset": 33, "label": "bod" }, { "id": 3, "entity": "先天性耳聋", "start_offset": 41, "end_offset": 46, "label": "dis" }, { "id": 4, "entity": "Jirvell-Lange-Nielsen综合征", "start_offset": 47, "end_offset": 71, "label": "dis" }, { "id": 5, "entity": "染色体", "start_offset": 73, "end_offset": 76, "label": "bod" } ]
遗传学研究显示心脏的钾、钠通道突变。
[ { "id": 0, "entity": "心脏", "start_offset": 7, "end_offset": 9, "label": "bod" } ]
药物可直接(terfenadine,阿司咪唑)或通过抑制代谢(红霉素,酮康唑)来延长QT间期。
[ { "id": 0, "entity": "terfenadine", "start_offset": 6, "end_offset": 17, "label": "dru" }, { "id": 1, "entity": "阿司咪唑", "start_offset": 18, "end_offset": 22, "label": "dru" }, { "id": 2, "entity": "红霉素", "start_offset": 31, "end_offset": 34, "label": "dru" }, { "id": 3, "entity": "酮康唑", "start_offset": 35, "end_offset": 38, "label": "dru" } ]
LQTS的临床表现最常见的为晕厥发作,多由运动或惊吓引起。
[ { "id": 0, "entity": "LQTS", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "晕厥发作", "start_offset": 14, "end_offset": 18, "label": "sym" } ]
患儿也可表现为抽搐、前晕厥及心悸,约10%的患儿一开始即表现为心搏骤停。
[ { "id": 0, "entity": "抽搐", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "晕厥", "start_offset": 11, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "心悸", "start_offset": 14, "end_offset": 16, "label": "sym" }, { "id": 3, "entity": "心搏骤停", "start_offset": 31, "end_offset": 35, "label": "sym" } ]
LQTS的诊断主要基于心电图及临床标准。
[ { "id": 0, "entity": "LQTS", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "心电图", "start_offset": 11, "end_offset": 14, "label": "pro" } ]
LQTS的治疗包括β受体阻滞剂,其剂量为能降低运动时的心率反应为度。
[ { "id": 0, "entity": "LQTS", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "β受体阻滞剂", "start_offset": 9, "end_offset": 15, "label": "dru" }, { "id": 2, "entity": "心率", "start_offset": 27, "end_offset": 29, "label": "ite" } ]
如仍有症状且心率慢者可安置起搏器。
[ { "id": 0, "entity": "心率", "start_offset": 6, "end_offset": 8, "label": "ite" }, { "id": 1, "entity": "起搏器", "start_offset": 13, "end_offset": 16, "label": "equ" } ]
手术切除左星状交感神经节也是治疗方法之一。
[ { "id": 0, "entity": "手术切除左星状交感神经节", "start_offset": 0, "end_offset": 12, "label": "pro" } ]
引起发热的原因可分为感染性与非感染性两方面。
[ { "id": 0, "entity": "发热", "start_offset": 2, "end_offset": 4, "label": "dis" } ]
由下丘脑所调控的体内正常体温调节平衡会由于下列因素而发生改变:感染、疫苗接种、生物学因素(如粒细胞-巨噬细胞集落刺激因子、干扰素、白细胞介素、肿瘤坏死因子)、组织损伤(如血管闭塞、肺栓塞、外伤、肌内注射、烧伤)、恶性肿瘤(白血病、淋巴肿瘤、肝癌、转移瘤)、药物(如药物热、可卡因、两性霉素B)、风湿性疾病(如系统性红斑狼疮、类风湿关节炎)、炎症性疾病(如炎性结肠病)、肉芽肿病(如结节病)、内分泌疾病(如甲状腺毒症、嗜铬细胞瘤)、代谢性疾病(如痛风、尿毒症、Ⅰ型高脂血症)以及原因不明或知之甚少的病变(如家族性地中海热)。
[ { "id": 0, "entity": "粒细胞-巨噬细胞", "start_offset": 46, "end_offset": 54, "label": "bod" }, { "id": 1, "entity": "干扰素", "start_offset": 61, "end_offset": 64, "label": "bod" }, { "id": 2, "entity": "白细胞介素", "start_offset": 65, "end_offset": 70, "label": "bod" }, { "id": 3, "entity": "肿瘤坏死因子", "start_offset": 71, "end_offset": 77, "label": "bod" } ]
发热可增高颅内压,使大脑皮质过度兴奋(烦躁、头痛或高热惊厥)或高度抑制,对6个月~5岁年龄组的儿童增加良性热性惊厥发生的危险性,而对有原发癫痫的患儿则会增加其癫痫的发作次数。
[ { "id": 0, "entity": "发热", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "可增高颅内压,使大脑皮质过度兴奋(烦躁、头痛或高热惊厥)或高度抑制,对6个月~5岁年龄组的儿童增加良性热性惊厥发生的危险性", "start_offset": 2, "end_offset": 63, "label": "sym" }, { "id": 2, "entity": "原发癫痫", "start_offset": 67, "end_offset": 71, "label": "dis" }, { "id": 3, "entity": "会增加其癫痫的发作次数", "start_offset": 75, "end_offset": 86, "label": "sym" } ]
发热时消化道分泌减少,消化酶活力降低,胃肠运动缓慢,可引起食欲缺乏、腹胀和便秘等症状。
[ { "id": 0, "entity": "发热", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "消化道", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 2, "entity": "消化酶", "start_offset": 11, "end_offset": 14, "label": "bod" }, { "id": 3, "entity": "胃肠", "start_offset": 19, "end_offset": 21, "label": "bod" }, { "id": 4, "entity": "消化道分泌减少,消化酶活力降低,胃肠运动缓慢,可引起食欲缺乏、腹胀和便秘等症状", "start_offset": 3, "end_offset": 42, "label": "sym" } ]
新生儿可能是社区获得性感染或新生儿脓毒败血症。
[ { "id": 0, "entity": "社区获得性感染", "start_offset": 6, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "新生儿脓毒败血症", "start_offset": 14, "end_offset": 22, "label": "dis" } ]
3个月以内婴儿的发热常提示严重细菌感染性疾病(约10%~15%)或自限性非特异性病毒感染。
[ { "id": 0, "entity": "发热", "start_offset": 8, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "严重细菌感染性疾病", "start_offset": 13, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "自限性非特异性病毒感染", "start_offset": 33, "end_offset": 44, "label": "dis" } ]
前者包括脓毒症、脑膜炎、泌尿道感染、胃肠炎、骨关节炎和化脓性关节炎。
[ { "id": 0, "entity": "脓毒症", "start_offset": 4, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "脑膜炎", "start_offset": 8, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "泌尿道感染", "start_offset": 12, "end_offset": 17, "label": "dis" }, { "id": 3, "entity": "胃肠炎", "start_offset": 18, "end_offset": 21, "label": "dis" }, { "id": 4, "entity": "骨关节炎", "start_offset": 22, "end_offset": 26, "label": "dis" }, { "id": 5, "entity": "化脓性关节炎", "start_offset": 27, "end_offset": 33, "label": "dis" } ]
约5%的发热婴儿有菌血症,其病原包括李斯特菌、β链球菌(可导致新生儿后期脓毒症、脑膜炎)以及社区获得性病原菌,如沙门菌属、大肠埃希菌、脑膜炎奈瑟菌、肺炎链球菌、b型流感嗜血杆菌和金黄色葡萄球菌。
[ { "id": 0, "entity": "发热", "start_offset": 4, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "菌血症", "start_offset": 9, "end_offset": 12, "label": "dis" }, { "id": 2, "entity": "李斯特菌", "start_offset": 18, "end_offset": 22, "label": "mic" }, { "id": 3, "entity": "β链球菌", "start_offset": 23, "end_offset": 27, "label": "mic" }, { "id": 4, "entity": "脓毒症", "start_offset": 36, "end_offset": 39, "label": "dis" }, { "id": 5, "entity": "脑膜炎", "start_offset": 40, "end_offset": 43, "label": "dis" }, { "id": 6, "entity": "社区获得性病原菌", "start_offset": 46, "end_offset": 54, "label": "mic" }, { "id": 7, "entity": "沙门菌属", "start_offset": 56, "end_offset": 60, "label": "mic" }, { "id": 8, "entity": "大肠埃希菌", "start_offset": 61, "end_offset": 66, "label": "mic" }, { "id": 9, "entity": "脑膜炎奈瑟菌", "start_offset": 67, "end_offset": 73, "label": "mic" }, { "id": 10, "entity": "肺炎链球菌", "start_offset": 74, "end_offset": 79, "label": "mic" }, { "id": 11, "entity": "b型流感嗜血杆菌", "start_offset": 80, "end_offset": 88, "label": "mic" }, { "id": 12, "entity": "金黄色葡萄球菌", "start_offset": 89, "end_offset": 96, "label": "mic" } ]
此外,这个年龄组还可能发生中耳炎、肺炎、脐炎、乳腺炎和皮肤、软组织感染。
[ { "id": 0, "entity": "中耳炎", "start_offset": 13, "end_offset": 16, "label": "dis" }, { "id": 1, "entity": "肺炎", "start_offset": 17, "end_offset": 19, "label": "dis" }, { "id": 2, "entity": "脐炎", "start_offset": 20, "end_offset": 22, "label": "dis" }, { "id": 3, "entity": "乳腺炎", "start_offset": 23, "end_offset": 26, "label": "dis" }, { "id": 4, "entity": "皮肤", "start_offset": 27, "end_offset": 29, "label": "bod" }, { "id": 5, "entity": "软组织", "start_offset": 30, "end_offset": 33, "label": "bod" } ]