text
stringlengths
4
4.87k
entities
list
【临床表现】(一)起病常隐袭。
[ { "id": 0, "entity": "起病常隐袭", "start_offset": 9, "end_offset": 14, "label": "sym" } ]
(二)皮肤、黏膜开始皮肤病变见于双侧手指及面部,后向躯干蔓延。
[ { "id": 0, "entity": "皮肤", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "黏膜", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 2, "entity": "皮肤", "start_offset": 10, "end_offset": 12, "label": "bod" }, ...
经历水肿期(皮肤变厚、紧张、苍白和皮温降低)、硬化期(皮肤增厚、变硬如皮革,呈蜡样光泽、面部呈假面具状、皱纹消失和张口困难)、最后萎缩期(皮肤光滑而细薄如羊皮纸紧贴于皮下骨面)。
[ { "id": 0, "entity": "皮肤", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "皮肤变厚、紧张、苍白和皮温降低", "start_offset": 6, "end_offset": 21, "label": "sym" }, { "id": 2, "entity": "皮肤", "start_offset": 27, "end_offset": 29, "label"...
黏膜(如口腔及阴道黏膜)可硬化、萎缩。
[ { "id": 0, "entity": "黏膜", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "口腔", "start_offset": 4, "end_offset": 6, "label": "bod" }, { "id": 2, "entity": "阴道黏膜", "start_offset": 7, "end_offset": 11, "label": "bod" }, ...
(三)雷诺现象约为70%患者的首发症状,有时为硬皮病早期唯一表现,是该病的典型症状之一。
[ { "id": 0, "entity": "雷诺现象", "start_offset": 3, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "硬皮病", "start_offset": 23, "end_offset": 26, "label": "dis" } ]
(四)关节和肌肉关节炎或关节痛,以手指关节常见,指端可因缺血而造成指垫丧失,指骨溶解、吸收而缩短。肌肉无力和萎缩。
[ { "id": 0, "entity": "关节", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "肌肉关节炎", "start_offset": 6, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "关节", "start_offset": 12, "end_offset": 14, "label": "bod" ...
(五)消化系统食管受累而引起吞咽困难,反流性食道炎,吸收不良综合征等。
[ { "id": 0, "entity": "消化系统食管", "start_offset": 3, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "吞咽困难", "start_offset": 14, "end_offset": 18, "label": "sym" }, { "id": 2, "entity": "反流性食道炎", "start_offset": 19, "end_offset": 25, "label": ...
(六)肺脏间质性肺炎、纤维化,通气及换气功能受损。
[ { "id": 0, "entity": "肺脏", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "间质性肺炎", "start_offset": 5, "end_offset": 10, "label": "dis" }, { "id": 2, "entity": "肺脏间质性肺炎、纤维化,通气及换气功能受损", "start_offset": 3, "end_offset": 24, ...
(七)心脏心脏增大、心力衰竭、心包炎、心律失常和肺动脉高压等是死亡重要原因之一。
[ { "id": 0, "entity": "心脏", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "心脏", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "心脏增大", "start_offset": 5, "end_offset": 9, "label": "sym" }, ...
(八)肾脏约17%受累,蛋白尿、血尿,有时出现硬皮病危象(急进性高血压及进行性肾衰竭),是重要死因之一。
[ { "id": 0, "entity": "肾脏", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "蛋白尿", "start_offset": 12, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "血尿", "start_offset": 16, "end_offset": 18, "label": "sym" }...
(九)其他发热,多发性神经炎等。
[ { "id": 0, "entity": "发热", "start_offset": 5, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "多发性神经炎", "start_offset": 8, "end_offset": 14, "label": "dis" } ]
(十)分型1.局限型病变限于皮肤,预后较好。
[ { "id": 0, "entity": "局限型", "start_offset": 7, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "皮肤", "start_offset": 14, "end_offset": 16, "label": "bod" } ]
此型有一组特殊临床表现,称为CREST综合征(皮下钙化、雷诺征、食管运动功能障碍、硬指和毛细血管扩张)。
[ { "id": 0, "entity": "CREST综合征", "start_offset": 14, "end_offset": 22, "label": "sym" }, { "id": 1, "entity": "皮下", "start_offset": 23, "end_offset": 25, "label": "bod" }, { "id": 2, "entity": "食管", "start_offset": 32, "end_offset": 34, "label": "b...
2.弥漫型皮损累及全身,进展快,内脏器官受累。
[ { "id": 0, "entity": "弥漫型皮损", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "内脏器官", "start_offset": 16, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "内脏器官受累", "start_offset": 16, "end_offset": 22, "label": "...
3.重叠型局限或弥漫型伴有另一种结缔组织病。
[ { "id": 0, "entity": "重叠型", "start_offset": 2, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "弥漫型", "start_offset": 8, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "结缔组织病", "start_offset": 16, "end_offset": 21, "label": "dis" ...
【实验室检查】(一)血沉加快。
[ { "id": 0, "entity": "血沉", "start_offset": 10, "end_offset": 12, "label": "ite" } ]
(二)自身抗体1.抗核抗体阳性,斑点型及核仁型为主。
[ { "id": 0, "entity": "抗体", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "抗核抗体", "start_offset": 9, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "抗核抗体阳性", "start_offset": 9, "end_offset": 15, "label": "sym" ...
2.抗Scl-70抗体阳性,是弥漫型SSc标记抗体。
[ { "id": 0, "entity": "弥漫型SSc标记抗体", "start_offset": 15, "end_offset": 25, "label": "bod" } ]
3.抗着丝点抗体是局限型SSc标记抗体,特别是CREST综合征时阳性。
[ { "id": 0, "entity": "抗着丝点抗体", "start_offset": 2, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "局限型SSc标记抗体", "start_offset": 9, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "CREST综合征", "start_offset": 23, "end_offset": 31, "l...
4.抗核仁抗体阳性(三)皮肤活体组织检查胶原纤维增生、硬化和萎缩,结缔组织细胞浸润,小血管壁增厚,管腔变小和闭塞。
[ { "id": 0, "entity": "抗核仁抗体", "start_offset": 2, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "抗核仁抗体阳性", "start_offset": 2, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "皮肤活体组织检查", "start_offset": 12, "end_offset": 20, "label"...
(四)X线检查食道蠕动减弱、管壁僵硬。
[ { "id": 0, "entity": "X线检查", "start_offset": 3, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "食道蠕动减弱、管壁僵硬", "start_offset": 7, "end_offset": 18, "label": "sym" } ]
间质性肺炎及肺纤维化等。
[ { "id": 0, "entity": "间质性肺炎", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "肺纤维化", "start_offset": 6, "end_offset": 10, "label": "dis" } ]
(五)肺功能测定肺容量及弥散功能减低。
[ { "id": 0, "entity": "肺", "start_offset": 3, "end_offset": 4, "label": "bod" } ]
【诊断与鉴别诊断】(一)诊断标准2007年ACR与欧洲风湿病联盟共同修订的SSc分类标准:1.主要指标近端硬皮:对称性手指及掌指或跖趾近端皮肤增厚、紧硬,类似病变亦见于整个四肢、面颈及躯干(胸腹)。
[ { "id": 0, "entity": "风湿病", "start_offset": 27, "end_offset": 30, "label": "dis" }, { "id": 1, "entity": "手指", "start_offset": 59, "end_offset": 61, "label": "bod" }, { "id": 2, "entity": "掌指", "start_offset": 62, "end_offset": 64, "label": "bod" ...
2.次要指标(1)硬指:上述皮肤改变仅限于手指。
[ { "id": 0, "entity": "皮肤", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 1, "entity": "手指", "start_offset": 21, "end_offset": 23, "label": "bod" } ]
(2)外周血管:雷诺现象或指端可凹性瘢痕或指垫变薄、丧失。
[ { "id": 0, "entity": "外周血管", "start_offset": 3, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "雷诺现象", "start_offset": 8, "end_offset": 12, "label": "sym" }, { "id": 2, "entity": "指端", "start_offset": 13, "end_offset": 15, "label": "bod" ...
(3)胃肠道:胃十二指肠反流及吞咽困难。
[ { "id": 0, "entity": "胃肠道", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "胃十二指肠", "start_offset": 7, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "吞咽困难", "start_offset": 15, "end_offset": 19, "label": "sym"...
(4)呼吸系统:肺底部纤维化及肺动脉高压。
[ { "id": 0, "entity": "肺底部纤维化", "start_offset": 8, "end_offset": 14, "label": "dis" }, { "id": 1, "entity": "肺动脉高压", "start_offset": 15, "end_offset": 20, "label": "dis" } ]
(5)心脏:心律失常及心功能衰竭。
[ { "id": 0, "entity": "心脏", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "心律", "start_offset": 6, "end_offset": 8, "label": "ite" }, { "id": 2, "entity": "心律失常及心功能衰竭", "start_offset": 6, "end_offset": 16, "label": "sym"...
(6)肾脏:肾危象及肾血管性高血压。
[ { "id": 0, "entity": "肾脏", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "肾危象及肾血管性高血压", "start_offset": 6, "end_offset": 17, "label": "sym" } ]
(7)神经系统:出现神经系统病变骨骼肌肉(8)骨骼肌肉:肌腱摩擦音、关节炎和肌炎。
[ { "id": 0, "entity": "神经系统", "start_offset": 3, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "骨骼肌肉", "start_offset": 16, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "骨骼肌肉", "start_offset": 23, "end_offset": 27, "label": "bod...
(9)血清学:ANA阳性或特异性抗体(如Scl70)阳性。
[ { "id": 0, "entity": "特异性抗体", "start_offset": 13, "end_offset": 18, "label": "bod" }, { "id": 1, "entity": "ANA阳性或特异性抗体(如Scl70)阳性", "start_offset": 7, "end_offset": 28, "label": "sym" } ]
具备1项主要指标和2项次要指标者可诊断为SSc。
[ { "id": 0, "entity": "SSc", "start_offset": 20, "end_offset": 23, "label": "dis" } ]
(二)鉴别诊断1.局部性硬皮病局部皮肤变硬呈线状或斑点状,界限清楚,无血清学及内脏病变。
[ { "id": 0, "entity": "局部性硬皮病", "start_offset": 9, "end_offset": 15, "label": "dis" }, { "id": 1, "entity": "皮肤", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "局部皮肤变硬呈线状或斑点状,界限清楚", "start_offset": 15, "end_offset": 33, ...
2.混合性结缔组织病该病有手指肿胀及雷诺现象,易与SSc混淆,但它兼有狼疮及肌炎表现,如蛋白尿、肌无力及肌酶增高,高滴度抗RNP抗体可鉴别。
[ { "id": 0, "entity": "混合性结缔组织病", "start_offset": 2, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "手指", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "手指肿胀及雷诺现象", "start_offset": 13, "end_offset": 22, "labe...
3.嗜酸性筋膜炎肢体局部压痛、肿胀和硬结,但一般不影响手、足和面部,嗜酸粒细胞增多,无雷诺现象及内脏损害,自身抗体阴性,活体组织检查可见深筋膜、皮下组织广泛炎症和硬化。
[ { "id": 0, "entity": "嗜酸性筋膜炎", "start_offset": 2, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "肢体", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "肢体局部压痛、肿胀和硬结", "start_offset": 8, "end_offset": 20, "label"...
4.自限性硬肿病皮肤发硬,但①病损发展快,短期内可累及全身皮肤,手、足常不受累;②无雷诺现象;③抗Scl-70抗体等常阴性;④病程常自限性;⑤发病前常有感染史,如流感、咽炎及扁桃体炎等。
[ { "id": 0, "entity": "自限性硬肿病", "start_offset": 2, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "皮肤", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "皮肤发硬", "start_offset": 8, "end_offset": 12, "label": "sym" ...
(一)一般治疗保暖,营养,避免劳累及精神紧张,清除感染。
[ { "id": 0, "entity": "保暖", "start_offset": 7, "end_offset": 9, "label": "pro" }, { "id": 1, "entity": "营养", "start_offset": 10, "end_offset": 12, "label": "pro" }, { "id": 2, "entity": "避免劳累及精神紧张", "start_offset": 13, "end_offset": 22, "label": "pr...
(二)糖皮质激素用于系统性硬化症,缓解炎性肌病及肺纤维化,但不能阻止本病的进展。
[ { "id": 0, "entity": "糖皮质激素", "start_offset": 3, "end_offset": 8, "label": "dru" }, { "id": 1, "entity": "系统性硬化症", "start_offset": 10, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "炎性肌病", "start_offset": 19, "end_offset": 23, "label": "...
泼尼松1~2mg/(kg•d)分次服用,症状缓解后逐渐减量。
[ { "id": 0, "entity": "泼尼松", "start_offset": 0, "end_offset": 3, "label": "dru" } ]
(三)免疫抑制剂氨甲蝶呤0.25~0.5mg/kg,每周一次口服。
[ { "id": 0, "entity": "免疫抑制剂", "start_offset": 3, "end_offset": 8, "label": "dru" }, { "id": 1, "entity": "氨甲蝶呤", "start_offset": 8, "end_offset": 12, "label": "dru" } ]
(四)青霉胺适用于严重病例,3mg/kg,2个月后每月增加2~3mg/kg,最后达每日10~15mg/kg,一般剂量250~500mg/d。
[ { "id": 0, "entity": "青霉胺", "start_offset": 3, "end_offset": 6, "label": "dru" } ]
(五)扩张血管药硝苯地平0.5~1.0mg/(kg•d),分次服用。
[ { "id": 0, "entity": "扩张血管药", "start_offset": 3, "end_offset": 8, "label": "dru" }, { "id": 1, "entity": "硝苯地平", "start_offset": 8, "end_offset": 12, "label": "dru" } ]
(六)中药可选用复方参片口服或用复方丹参注射液静滴。
[ { "id": 0, "entity": "中药", "start_offset": 3, "end_offset": 5, "label": "dru" }, { "id": 1, "entity": "复方参片", "start_offset": 8, "end_offset": 12, "label": "dru" }, { "id": 2, "entity": "复方丹参", "start_offset": 16, "end_offset": 20, "label": "dru" ...
(七)血浆置换用于重症及药物疗效不佳患者。
[ { "id": 0, "entity": "血浆置换", "start_offset": 3, "end_offset": 7, "label": "pro" } ]
【预后】局限性硬皮病一般无生命危险,皮肤损害可持续进展多年,个别损害可逐渐变软,但很少完全恢复正常。
[ { "id": 0, "entity": "局限性硬皮病", "start_offset": 4, "end_offset": 10, "label": "dis" } ]
肺和心脏受累是本病的严重表现。
[ { "id": 0, "entity": "肺", "start_offset": 0, "end_offset": 1, "label": "bod" }, { "id": 1, "entity": "心脏", "start_offset": 2, "end_offset": 4, "label": "bod" } ]
肺纤维化和心功能不全是主要致死的原因。
[ { "id": 0, "entity": "肺纤维化", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "心功能不全", "start_offset": 5, "end_offset": 10, "label": "dis" } ]
第四节维生素K缺乏症维生素K分为两大类:一类是脂溶性维生素K1</sub>(从植物中提取)和K2</sub>(从微生物中提取,也可由肠内细菌制造),另一类是水溶性维生素K3</sub>和K4</sub>(由人工合成),其中以K1</sub>和K2</sub>最为重要。
[ { "id": 0, "entity": "维生素K缺乏症", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "维生素K", "start_offset": 10, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "脂溶性维生素K1", "start_offset": 23, "end_offset": 31, "labe...
维生素K是促进血液凝固的化学物质之一,是四种凝血蛋白(凝血酶原、转变加速因子、抗血友病因子和司徒因子)在肝内合成必不可少的物质。
[ { "id": 0, "entity": "维生素K", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "血液", "start_offset": 7, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "凝血蛋白", "start_offset": 22, "end_offset": 26, "label": "bod" ...
维生素K的缺乏将导致凝血功能失常而出现出血。
[ { "id": 0, "entity": "维生素K", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "凝血功能失常", "start_offset": 10, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "出血", "start_offset": 19, "end_offset": 21, "label": "sym...
【流行病学】维生素K缺乏是婴儿和新生儿出血性疾病的主要原因,其发病急,病死率高,严重危害婴儿健康,1991年城市颅内出血死亡率71.5/105</sup>,1993年为106.6/105</sup>。
[ { "id": 0, "entity": "维生素K缺乏", "start_offset": 6, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "出血性疾病", "start_offset": 19, "end_offset": 24, "label": "dis" }, { "id": 2, "entity": "颅内出血", "start_offset": 56, "end_offset": 60, "label": ...
本病发病高峰年龄为4~8周,发病的男女比例为2.62∶1,纯母乳喂养者占89%,92%患儿并发颅内出血;农村多于城市。
[ { "id": 0, "entity": "颅内出血", "start_offset": 47, "end_offset": 51, "label": "dis" } ]
根据死亡率推算:我国婴儿颅内出血每年死亡2.5万人。
[ { "id": 0, "entity": "颅内出血", "start_offset": 12, "end_offset": 16, "label": "dis" } ]
近几年来,随着母乳喂养率不断提高,母乳维生素K相对不足,可能导致婴儿维生素K缺乏,因此维生素K缺乏已是危害我国婴儿健康的严重疾病之一。
[ { "id": 0, "entity": "维生素K", "start_offset": 19, "end_offset": 23, "label": "bod" }, { "id": 1, "entity": "维生素K缺乏", "start_offset": 34, "end_offset": 40, "label": "dis" }, { "id": 2, "entity": "维生素K缺乏", "start_offset": 43, "end_offset": 49, "label"...
1997年,首都儿科研究所和全国维生素K协作组在7省自治区调查了31649名婴儿维生素K缺乏出血症的情况,其发生率为2.4‰。
[ { "id": 0, "entity": "维生素K", "start_offset": 16, "end_offset": 20, "label": "bod" }, { "id": 1, "entity": "维生素K缺乏出血症", "start_offset": 40, "end_offset": 49, "label": "dis" } ]
【病因】本病的发病原因是由于体内维生素K缺乏,使凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ在肝内合成不足,从而引起出血。
[ { "id": 0, "entity": "维生素K缺乏", "start_offset": 16, "end_offset": 22, "label": "dis" }, { "id": 1, "entity": "凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ", "start_offset": 24, "end_offset": 35, "label": "bod" }, { "id": 2, "entity": "肝", "start_offset": 36, "end_offset": 37, "labe...
在婴儿出生后第一小时内即可出现,可导致致命性出血。
[ { "id": 0, "entity": "致命性出血", "start_offset": 19, "end_offset": 24, "label": "sym" } ]
发病原因如下:1.母体缺乏维生素K,维生素K经胎盘转运不足,经放射免疫方法检测大部分新生儿脐血中维生素K缺乏。
[ { "id": 0, "entity": "维生素K", "start_offset": 13, "end_offset": 17, "label": "bod" }, { "id": 1, "entity": "维生素K", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "胎盘", "start_offset": 23, "end_offset": 25, "label": "bod...
2.孕期药物影响母亲怀孕期间服用影响维生素K代谢及合成的药物能导致新生儿期维生素K缺乏。
[ { "id": 0, "entity": "孕期药物", "start_offset": 2, "end_offset": 6, "label": "dru" }, { "id": 1, "entity": "维生素K", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "维生素K缺乏", "start_offset": 37, "end_offset": 43, "label": "d...
如果长期应用抑制肠道内细菌生长的药物,如广谱抗生素和肠道内不易吸收的磺胺类药物,能抑制肠道内寄生的非致病菌,减少肠道内维生素K的合成,导致维生素K的缺乏。
[ { "id": 0, "entity": "肠道", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "细菌", "start_offset": 11, "end_offset": 13, "label": "mic" }, { "id": 2, "entity": "广谱抗生素", "start_offset": 20, "end_offset": 25, "label": "dru" ...
摄入过量的维生素A,也能抑制维生素K2</sub>的肠内合成,并且因为维生素K1</sub>、K2</sub>均为脂溶性物质,其他脂溶性维生素(如A和D)都能影响其吸收。
[ { "id": 0, "entity": "维生素A", "start_offset": 5, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "维生素K2", "start_offset": 14, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "肠", "start_offset": 26, "end_offset": 27, "label": "bod" ...
口服抗凝药物(如双香豆素)的结构与维生素K相似,可与维生素K竞争,减少凝血酶原在肝脏内的合成;孕妇服用抗惊厥药物后,可经胎盘输送,并以类似抗凝药物的作用来抑制维生素K的生成,引起新生儿维生素K的缺乏。
[ { "id": 0, "entity": "抗凝药物", "start_offset": 2, "end_offset": 6, "label": "dru" }, { "id": 1, "entity": "双香豆素", "start_offset": 8, "end_offset": 12, "label": "dru" }, { "id": 2, "entity": "维生素K", "start_offset": 17, "end_offset": 21, "label": "bod"...
其原因为:1.单纯母乳喂养母乳喂养是婴儿最佳的喂养方式已得到公认,应该大力提倡和推广,但由于人乳中含维生素K的量极低,平均为15μg/L(牛奶中含量为60μg/L)。
[ { "id": 0, "entity": "维生素K", "start_offset": 50, "end_offset": 54, "label": "bod" } ]
故如单纯母乳喂养的婴儿未给予适当量的维生素K的补充,很容易导致维生素K的缺乏。
[ { "id": 0, "entity": "维生素K", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 1, "entity": "维生素K", "start_offset": 31, "end_offset": 35, "label": "bod" } ]
据相关文献报道,90%以上的维生素K缺乏出血是发生在母乳喂养的婴儿中。
[ { "id": 0, "entity": "维生素K缺乏出血", "start_offset": 14, "end_offset": 22, "label": "dis" } ]
2.吸收利用功能不良新生儿(特别是早产儿)胆汁分泌有限,且胆汁中胆酸含量低,脂肪及脂溶性维生素的吸收有限,影响维生素K的吸收;新生儿及早产儿肝脏功能未发育成熟,使凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ在肝内合成不足,以至维生素K依赖因子生成减少。
[ { "id": 0, "entity": "胆汁", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 1, "entity": "胆汁", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 2, "entity": "胆酸", "start_offset": 32, "end_offset": 34, "label": "bod" ...
肠道细菌可合成一部分维生素K,但新生儿出生时肠道内无细菌,维生素K合成减少。
[ { "id": 0, "entity": "肠道细菌", "start_offset": 0, "end_offset": 4, "label": "mic" }, { "id": 1, "entity": "维生素K", "start_offset": 10, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "肠道", "start_offset": 22, "end_offset": 24, "label": "bod" ...
发病原因如下:1.摄入不足新生儿吃奶量少且母乳中维生素含量低,初乳中几乎不含维生素K,如长期单纯母乳喂养,未及时添加辅食,未添加含维生素K丰富的蔬菜、水果,均可引起维生素K缺乏。
[ { "id": 0, "entity": "维生素", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 1, "entity": "维生素K", "start_offset": 38, "end_offset": 42, "label": "bod" }, { "id": 2, "entity": "维生素K", "start_offset": 65, "end_offset": 69, "label": "bo...
2.吸收不良因慢性腹泻、溃疡性结肠炎、肠切除、囊性纤维化等疾病引起的小儿肠道吸收不良维生素K吸收障碍;胆道阻塞、胆瘘等胆道梗阻性疾病、胆汁缺乏性疾病,也可影响维生素K的吸收。
[ { "id": 0, "entity": "慢性腹泻", "start_offset": 7, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "溃疡性结肠炎", "start_offset": 12, "end_offset": 18, "label": "dis" }, { "id": 2, "entity": "肠切除", "start_offset": 19, "end_offset": 22, "label": "p...
3.利用障碍新生儿肝炎、新生儿败血症及病毒感染等任何原因引起的肝脏损害均可影响维生素K依赖因子的合成。
[ { "id": 0, "entity": "肝炎", "start_offset": 9, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "新生儿败血症", "start_offset": 12, "end_offset": 18, "label": "dis" }, { "id": 2, "entity": "病毒感染", "start_offset": 19, "end_offset": 23, "label": "di...
4.合成减少肠道细菌也可合成部分维生素K,在婴儿于肠道菌落出现后,维生素K缺乏则明显减少,长期应用抗生素抑制肠道内的正常细菌的生长。
[ { "id": 0, "entity": "肠道细菌", "start_offset": 6, "end_offset": 10, "label": "mic" }, { "id": 1, "entity": "维生素K", "start_offset": 16, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "肠道菌落", "start_offset": 25, "end_offset": 29, "label": "mi...
【临床表现】临床上以出血为主要表现。
[ { "id": 0, "entity": "出血", "start_offset": 10, "end_offset": 12, "label": "sym" } ]
早发型者可有头颅血肿颅内、胸腔内出血脐带出血胃肠道出血脐部出血脐带结扎不良来解释,轻者为渗血,重者则出血不止;胃肠道出血则表现为不同程度吐血和便血。
[ { "id": 0, "entity": "头颅", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "头颅血肿", "start_offset": 6, "end_offset": 10, "label": "sym" }, { "id": 2, "entity": "颅内", "start_offset": 10, "end_offset": 12, "label": "bod" }...
其次是皮肤出血,多见于分娩时挤压处,轻者为瘀点和紫癜,重者可形成大片瘀斑和血肿;也可见于采血及注射部位、术后伤口处渗血不止。
[ { "id": 0, "entity": "皮肤出血", "start_offset": 3, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "分娩", "start_offset": 11, "end_offset": 13, "label": "pro" }, { "id": 2, "entity": "瘀点", "start_offset": 21, "end_offset": 23, "label": "sym" ...
颅内出血少见,但早产儿由于毛细血管脆性增加,往往预后不良。
[ { "id": 0, "entity": "颅内出血", "start_offset": 0, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "毛细血管", "start_offset": 13, "end_offset": 17, "label": "bod" } ]
同时可伴有出血性贫血。
[ { "id": 0, "entity": "出血性贫血", "start_offset": 5, "end_offset": 10, "label": "sym" } ]
【实验室检查】凝血酶原时间延长,多数延长至正常对照的2倍以上,轻度维生素K缺乏只有凝血酶原时间延长,临床无出血倾向。
[ { "id": 0, "entity": "实验室检查", "start_offset": 1, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "凝血酶原时间延长", "start_offset": 7, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "维生素K缺乏", "start_offset": 33, "end_offset": 39, "label"...
陶土部分凝血活酶时间延长,凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ因子活性明显降低,第Ⅶ因子首先降至最低,第Ⅶ因子减低后凝血酶原水平即下降但较缓慢,第Ⅸ、Ⅹ因子也有不同程度地减少。
[ { "id": 0, "entity": "陶土部分凝血活酶时间延长", "start_offset": 0, "end_offset": 12, "label": "sym" }, { "id": 1, "entity": "凝血因子Ⅱ、Ⅶ、Ⅸ、Ⅹ因子活性明显降低", "start_offset": 13, "end_offset": 32, "label": "sym" }, { "id": 2, "entity": "第Ⅶ因子首先降至最低", "start_offset": 33, "end_...
凝血酶原检测是维生素K缺乏的可靠证据。
[ { "id": 0, "entity": "凝血酶原检测", "start_offset": 0, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "维生素K缺乏", "start_offset": 7, "end_offset": 13, "label": "dis" } ]
如疑有颅内出血者应进行B超、CT或MRI检查,以了解出血情况。
[ { "id": 0, "entity": "颅内出血", "start_offset": 3, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "B超", "start_offset": 11, "end_offset": 13, "label": "pro" }, { "id": 2, "entity": "CT", "start_offset": 14, "end_offset": 16, "label": "pro" ...
必要时可行维生素K的检测。
[ { "id": 0, "entity": "维生素K的检测", "start_offset": 5, "end_offset": 12, "label": "pro" } ]
多见于单纯母乳喂养儿,生后3个月内的婴儿,未接受过维生素K预防。
[ { "id": 0, "entity": "维生素K", "start_offset": 25, "end_offset": 29, "label": "bod" } ]
(二)观察病情新生儿出血症多见于出生后1~7天,以胃肠道出血凝血酶原时间延长,血小板、出血时间均正常,予维生素K治疗效果良好,数小时或24小时后出血倾向明显好转。
[ { "id": 0, "entity": "新生儿出血症", "start_offset": 7, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "胃肠道", "start_offset": 25, "end_offset": 28, "label": "bod" }, { "id": 2, "entity": "胃肠道出血", "start_offset": 25, "end_offset": 30, "label": "...
不伴其他部位出血的患儿,易误诊为颅内感染,而迟发性新生儿出血症表现为突然起病,无明显感染中毒症状,贫血发展迅速而严重,故可与颅内感染相鉴别。
[ { "id": 0, "entity": "出血", "start_offset": 6, "end_offset": 8, "label": "sym" }, { "id": 1, "entity": "颅内感染", "start_offset": 16, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "迟发性新生儿出血症", "start_offset": 22, "end_offset": 31, "label": "...
辅助检查也有助于该诊断,脑脊液检查呈现均匀一致的血性和皱缩红细胞,但脑脊液检查正常也不可以完全排除此病,且病情危重者不宜进行该项检查。
[ { "id": 0, "entity": "辅助检查", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "脑脊液检查", "start_offset": 12, "end_offset": 17, "label": "pro" }, { "id": 2, "entity": "均匀一致的血性和皱缩红细胞", "start_offset": 19, "end_offset": 32, "la...
【治疗】有出血现象时,应立即注射维生素K2mg,可迅速改善出血,胃肠道出血者应暂禁食,给予静脉营养支持,止血后应根据适当情况纠正贫血,严重者可输全血或血浆10~20ml/kg。
[ { "id": 0, "entity": "出血", "start_offset": 5, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "注射维生素K2", "start_offset": 14, "end_offset": 21, "label": "pro" }, { "id": 2, "entity": "出血", "start_offset": 29, "end_offset": 31, "label": "sym"...
如有颅内出血,首先要加强护理,保持安静,维持通气,抬高头肩部,推迟喂奶,控制补液;如有高声尖叫、频繁呕吐、反复抽搐等表现,应对症止惊,降低颅内压,恢复脑细胞功能;同时要及时止血、纠正贫血。
[ { "id": 0, "entity": "颅内出血", "start_offset": 2, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "头", "start_offset": 27, "end_offset": 28, "label": "bod" }, { "id": 2, "entity": "肩", "start_offset": 28, "end_offset": 29, "label": "bod" },...
严重者可手术清除血肿。
[ { "id": 0, "entity": "手术清除血肿", "start_offset": 4, "end_offset": 10, "label": "pro" } ]
【预防】预防新生儿维生素K缺乏症应从孕妇开始,分娩前数周即可口服维生素K20mg,能预防新生儿维生素K缺乏所致的低凝血酶原血症。
[ { "id": 0, "entity": "维生素K缺乏症", "start_offset": 9, "end_offset": 16, "label": "dis" }, { "id": 1, "entity": "分娩", "start_offset": 23, "end_offset": 25, "label": "pro" }, { "id": 2, "entity": "维生素K", "start_offset": 32, "end_offset": 36, "label": "d...
乳母应多吃蔬菜、水果以提高乳汁中维生素K的含量。
[ { "id": 0, "entity": "维生素K", "start_offset": 16, "end_offset": 20, "label": "bod" } ]
自从1961年美国儿科学会营养委员会提出所有新生儿应在出生后肌内注射维生素K1</sub>0.5~1mg作为预防新生儿出血以来,维生素K1</sub>用来预防和根治新生儿维生素K缺乏性出血已在许多国家得到广泛应用。
[ { "id": 0, "entity": "儿科", "start_offset": 9, "end_offset": 11, "label": "dep" }, { "id": 1, "entity": "肌内注射维生素K1", "start_offset": 30, "end_offset": 39, "label": "pro" }, { "id": 2, "entity": "出血", "start_offset": 59, "end_offset": 61, "label": "p...
荷兰CornelissenEA等人实验证明,在新生儿出生后3个月内,每周口服维生素K1mg可有效纠正维生素K缺乏且不会引起维生素K在体内的积聚。
[ { "id": 0, "entity": "维生素K", "start_offset": 38, "end_offset": 42, "label": "dru" }, { "id": 1, "entity": "维生素K缺乏", "start_offset": 50, "end_offset": 56, "label": "dis" }, { "id": 2, "entity": "维生素K", "start_offset": 61, "end_offset": 65, "label": ...
加拿大儿科协会建议足月产的新生儿应在出生后6小时内口服或肌注维生素K1mg;早产儿、低体重儿及难产儿均需在产后6小时内肌注维生素K1mg;因脂肪吸收不良而有迟发性出血性疾病危险性的新生儿需每天口服维生素K1mg或每月肌注维生素K一次以预防维生素K缺乏性出血症。
[ { "id": 0, "entity": "儿科", "start_offset": 3, "end_offset": 5, "label": "dep" }, { "id": 1, "entity": "口服或肌注维生素K", "start_offset": 25, "end_offset": 34, "label": "pro" }, { "id": 2, "entity": "肌注维生素K", "start_offset": 59, "end_offset": 65, "label":...
我国林良明等于2002年报道中国7省协作对19751例活产婴儿进行对照研究发现,采用给婴儿出生后口服维生素K1</sub>2mg,以后每隔10天1次,服满3个月,共10次,对预防维生素K缺乏性出血有相当好的效果。
[ { "id": 0, "entity": "维生素K1", "start_offset": 50, "end_offset": 55, "label": "dru" }, { "id": 1, "entity": "维生素K缺乏性出血", "start_offset": 89, "end_offset": 98, "label": "dis" } ]
参考文献1.程国强,洪建国.痰液分析在哮喘临床研究中的作用.国外医学儿科学分册,2000,27(2):82-852.中华医学会儿科学分会呼吸学组儿科支气管镜协作组.儿科支气管镜术指南(2009年版).中华儿科杂志,2009,47(10):740-7403.EfratiO,Sadeh-GornikU,Modan-MosesD,etal.Flexiblebronchoscopyandbronchoalveolarlavageinpediatricpatientswithlungdisease.PediatrCritCareMed,2009,10(1):80-844.HasanRA,ReddyR.Sedationwithpropofolforflexiblebronchoscopyinchildren.PediatrPulmonol,2009,44(4):373-3735.NielsonDW,KuPLandEggerM.Topicallidocaineexaggerateslaryngomalaciaduringflexiblebronchoscopy.AmJRespirCritCareMed,2000,161(1):147-1476.Vuori-HolopainenE,PeltolaH.Reappraisaloflungtap:reviewofanoldmethodforbetteretiologicdiagnosisofchildhoodpneumonia.ClinInfectDis,2001,32(5):715-715
[ { "id": 0, "entity": "痰液", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 1, "entity": "哮喘", "start_offset": 19, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "儿科", "start_offset": 34, "end_offset": 36, "label": "dep" ...
参考文献1.蒋明.风湿病学.北京:科学出版社,19982.李永柏,杨锡强.自身抗体测定及其临床意义.中国实用儿科杂志,2000,15(11):691-6933.滕庆,何晓唬.隐匿性类风湿因子与幼年类风湿关节炎.实用儿科临床杂志,1997,12:2704.BehrmanRE,KliegmanRM,JensonHB.NelsonTextbookofPediatrics.16thed.Philadelphia:W.B.SaunersCo,20005.周厚清,董敏,马路.ANA、抗dsDNA和ENA多肽抗体与自身免疫性疾病的关系.中国卫生检验杂志,2008:18(12):2675-26756.李永柏.自身抗体检测及其临床应用.实用儿科临床杂志,2005,20(11):1062-10627.FerucciED,MajkaDS,ParrishLA,etal.AntibodiesagainstcycliccitrullinatedpeptideareassociatedwithHLA-DR4insimplexandmultiplexpolyarticular-onsetjuvenilerheumatoidarthritis.ArthritisRheum,2005Jan,52(1):239-239
[ { "id": 0, "entity": "风湿病", "start_offset": 9, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "隐匿性类风湿因子", "start_offset": 87, "end_offset": 95, "label": "bod" }, { "id": 2, "entity": "幼年类风湿关节炎", "start_offset": 96, "end_offset": 104, "lab...
五、免疫性溶血性贫血由于免疫因素如抗体、补体等导致红细胞损伤而过早地破坏,产生溶血和贫血症状者称为免疫性溶血性贫血。
[ { "id": 0, "entity": "免疫性溶血性贫血", "start_offset": 2, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "抗体", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "补体", "start_offset": 20, "end_offset": 22, "label": "bo...
可发生于任何年龄阶段,小儿时期最常见的是新生儿同族免疫性贫血(见有关章节),其次是自身免疫性溶血性贫血(autoimmunehemolyticanemia,AIHA)。
[ { "id": 0, "entity": "新生儿同族免疫性贫血", "start_offset": 20, "end_offset": 30, "label": "dis" }, { "id": 1, "entity": "自身免疫性溶血性贫血", "start_offset": 41, "end_offset": 51, "label": "dis" }, { "id": 2, "entity": "autoimmunehemolyticanemia", "start_offset": 52, ...
AIHA是一种获得性免疫性贫血,由患儿体内产生抗自身红细胞膜抗原的抗体,引起红细胞过早地破坏而产生溶血性贫血所致。
[ { "id": 0, "entity": "AIHA", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "获得性免疫性贫血", "start_offset": 7, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "红细胞膜", "start_offset": 26, "end_offset": 30, "label": "...