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如果肺动脉及主动脉漏斗部出现吸收异常,可出现双漏斗部及漏斗部缺如畸形。
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5.动脉干的分隔动脉干位于心球的头端,在胚胎发育第5~7周时动脉干内膜左右两侧各发生一纵嵴,分别为Ⅰ嵴和Ⅲ嵴,分别与漏斗部的腹侧、背侧心球嵴相连。
[ { "id": 0, "entity": "动脉干", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "动脉干", "start_offset": 8, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "心球", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 3, "entity": "动脉干内膜", "start_offset": 30, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "纵嵴", "start_offset": 43, "end_offset": 45, "label": "bod" }, { "id": 5, "entity": "Ⅰ嵴", "start_offset": 49, "end_offset": 51, "label": "bod" }, { "id": 6, "entity": "Ⅲ嵴", "start_offset": 52, "end_offset": 54, "label": "bod" }, { "id": 7, "entity": "漏斗部", "start_offset": 58, "end_offset": 61, "label": "bod" }, { "id": 8, "entity": "背侧心球嵴", "start_offset": 65, "end_offset": 70, "label": "bod" } ]
Ⅰ嵴和Ⅲ嵴相互融合形成动脉干的纵隔即主肺动脉隔,将动脉干分成前后两部分,前侧为肺动脉,后侧为主动脉。
[ { "id": 0, "entity": "Ⅰ嵴", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "Ⅲ嵴", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 2, "entity": "动脉干", "start_offset": 11, "end_offset": 14, "label": "bod" }, { "id": 3, "entity": "纵隔", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 4, "entity": "肺动脉隔", "start_offset": 19, "end_offset": 23, "label": "bod" }, { "id": 5, "entity": "动脉干", "start_offset": 25, "end_offset": 28, "label": "bod" }, { "id": 6, "entity": "肺动脉", "start_offset": 39, "end_offset": 42, "label": "bod" }, { "id": 7, "entity": "主动脉", "start_offset": 46, "end_offset": 49, "label": "bod" } ]
6.心球纵隔、动脉干纵隔的扭转心球纵隔、动脉干纵隔融合后,心球纵隔作顺时针180°的扭转,然后动脉干纵隔同样顺时针方向扭转,最终使两者呈45°的旋转关系,因而肺动脉自右前方转向左后方,主动脉由左后方转向右前方。
[ { "id": 0, "entity": "心球纵隔", "start_offset": 2, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "动脉干纵隔", "start_offset": 7, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "心球纵隔", "start_offset": 15, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "动脉干纵隔", "start_offset": 20, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "心球纵隔", "start_offset": 29, "end_offset": 33, "label": "bod" }, { "id": 5, "entity": "动脉干纵隔", "start_offset": 47, "end_offset": 52, "label": "bod" }, { "id": 6, "entity": "肺动脉", "start_offset": 79, "end_offset": 82, "label": "bod" }, { "id": 7, "entity": "主动脉", "start_offset": 92, "end_offset": 95, "label": "bod" } ]
心球、动脉干隔的发育异常可分为三种类型:①分隔不均畸形:纵隔偏右可造成右侧漏斗部狭窄,反之可造成主动脉下狭窄。
[ { "id": 0, "entity": "心球", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "动脉干隔", "start_offset": 3, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "分隔", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 3, "entity": "纵隔", "start_offset": 28, "end_offset": 30, "label": "bod" }, { "id": 4, "entity": "右侧漏斗部", "start_offset": 35, "end_offset": 40, "label": "bod" }, { "id": 5, "entity": "主动脉", "start_offset": 48, "end_offset": 51, "label": "bod" } ]
②扭转不良畸形:动脉干纵隔扭转不全可导致主肺动脉异位性畸形(大动脉转位)。
[ { "id": 0, "entity": "动脉干纵隔", "start_offset": 8, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "主肺动脉", "start_offset": 20, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "大动脉", "start_offset": 30, "end_offset": 33, "label": "bod" } ]
③分隔不全畸形:动脉干纵隔发育不全可形成主肺动脉隔缺损;动脉干纵隔缺如即形成永存动脉干畸形。
[ { "id": 0, "entity": "分隔", "start_offset": 1, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "动脉干纵隔", "start_offset": 8, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "主肺动脉隔", "start_offset": 20, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "动脉干纵隔", "start_offset": 28, "end_offset": 33, "label": "bod" }, { "id": 4, "entity": "动脉干", "start_offset": 40, "end_offset": 43, "label": "bod" } ]
7.半月瓣的形成在胚胎5~7周时,动脉干内部分隔同时其表面发生两条相应的纵沟,使肺动脉及主动脉完全分离。
[ { "id": 0, "entity": "半月瓣", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "动脉干", "start_offset": 17, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "隔", "start_offset": 23, "end_offset": 24, "label": "bod" }, { "id": 3, "entity": "肺动脉", "start_offset": 40, "end_offset": 43, "label": "bod" }, { "id": 4, "entity": "主动脉", "start_offset": 44, "end_offset": 47, "label": "bod" } ]
纵沟的形成使动脉干纵嵴一分为二,主动脉及肺动脉各得一半,随后头端大部分消失,留下的近心端(位于动脉干与心球交界水平)逐渐各自分化为三个半月瓣。
[ { "id": 0, "entity": "纵沟", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "动脉干纵嵴", "start_offset": 6, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "主动脉", "start_offset": 16, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "肺动脉", "start_offset": 20, "end_offset": 23, "label": "bod" }, { "id": 4, "entity": "近心端", "start_offset": 41, "end_offset": 44, "label": "bod" }, { "id": 5, "entity": "动脉干", "start_offset": 47, "end_offset": 50, "label": "bod" }, { "id": 6, "entity": "心球", "start_offset": 51, "end_offset": 53, "label": "bod" }, { "id": 7, "entity": "半月瓣", "start_offset": 67, "end_offset": 70, "label": "bod" } ]
8.静脉窦的演变胚胎早期静脉窦分为左右两个,相互对称,一端与原始心房相连,另一端则各自连接三个左右对称的静脉:总主静脉、脐静脉及卵黄静脉。
[ { "id": 0, "entity": "静脉窦", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "静脉窦", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "原始心房", "start_offset": 30, "end_offset": 34, "label": "bod" }, { "id": 3, "entity": "静脉", "start_offset": 52, "end_offset": 54, "label": "bod" }, { "id": 4, "entity": "总主静脉", "start_offset": 55, "end_offset": 59, "label": "bod" }, { "id": 5, "entity": "脐静脉", "start_offset": 60, "end_offset": 63, "label": "bod" }, { "id": 6, "entity": "卵黄静脉", "start_offset": 64, "end_offset": 68, "label": "bod" } ]
以后两侧的脐静脉及左卵黄静脉退化,在胚胎发育第二个月末期右心房发育迅速,左、右静脉窦均被右房吸收。
[ { "id": 0, "entity": "脐静脉", "start_offset": 5, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "左卵黄静脉", "start_offset": 9, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "右心房", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 3, "entity": "左、右静脉窦", "start_offset": 36, "end_offset": 42, "label": "bod" }, { "id": 4, "entity": "右房", "start_offset": 44, "end_offset": 46, "label": "bod" } ]
右总主静脉发育成上腔静脉,右卵黄静脉发育成下腔静脉。
[ { "id": 0, "entity": "右总主静脉", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "上腔静脉", "start_offset": 8, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "右卵黄静脉", "start_offset": 13, "end_offset": 18, "label": "bod" }, { "id": 3, "entity": "下腔静脉", "start_offset": 21, "end_offset": 25, "label": "bod" } ]
原左静脉窦接收的左总主静脉发育差,形成心外的冠状窦及冠状静脉。
[ { "id": 0, "entity": "原左静脉窦", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "左总主静脉", "start_offset": 8, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "心", "start_offset": 19, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "冠状窦", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "冠状静脉", "start_offset": 26, "end_offset": 30, "label": "bod" } ]
9.左心房与肺静脉的发育胚胎36天左右时,原始窦-房腔的背侧中胚层组织呈现两个突起,位于静脉窦部的突起逐渐被吸收而消失,而位于心房的突起发展成肺静脉主干。
[ { "id": 0, "entity": "左心房", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "肺静脉", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "窦-房腔", "start_offset": 23, "end_offset": 27, "label": "bod" }, { "id": 3, "entity": "静脉窦部", "start_offset": 44, "end_offset": 48, "label": "bod" }, { "id": 4, "entity": "心房", "start_offset": 63, "end_offset": 65, "label": "bod" }, { "id": 5, "entity": "肺静脉主干", "start_offset": 71, "end_offset": 76, "label": "bod" } ]
肺静脉主干形成分支,左右各二,随后肺静脉主干被左心房吸收,使左右肺静脉直接进入左心房,左心房内四支肺静脉中间部分属静脉性起源,称为Keir前庭。
[ { "id": 0, "entity": "肺静脉主干", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "肺静脉主干", "start_offset": 17, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "左心房", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "左右肺静脉", "start_offset": 30, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "左心房", "start_offset": 39, "end_offset": 42, "label": "bod" }, { "id": 5, "entity": "左心房", "start_offset": 43, "end_offset": 46, "label": "bod" }, { "id": 6, "entity": "肺静脉", "start_offset": 49, "end_offset": 52, "label": "bod" }, { "id": 7, "entity": "Keir前庭", "start_offset": 65, "end_offset": 71, "label": "bod" } ]
第十六节鞘膜积液胚胎发育时,腹膜在下腹部形成一突起,进入腹股沟管并伸延至阴囊底部,称为鞘状突。
[ { "id": 0, "entity": "鞘膜积液", "start_offset": 4, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "腹膜", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "腹股沟管", "start_offset": 28, "end_offset": 32, "label": "bod" }, { "id": 3, "entity": "阴囊", "start_offset": 36, "end_offset": 38, "label": "bod" }, { "id": 4, "entity": "鞘状突", "start_offset": 43, "end_offset": 46, "label": "bod" } ]
鞘状突覆盖精索及睾丸的大部分。
[ { "id": 0, "entity": "鞘状突", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "精索", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "睾丸", "start_offset": 8, "end_offset": 10, "label": "bod" } ]
出生前鞘状突从腹股沟管内环处和近睾丸端两个部位开始闭塞,闭塞过程由两端向中间延续,使覆盖精索的鞘状突形成纤维索带,仅覆盖睾丸部的鞘膜留有间隙,成为睾丸固有鞘膜腔,与腹膜腔不通。
[ { "id": 0, "entity": "鞘状突", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "腹股沟管", "start_offset": 7, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "睾丸", "start_offset": 16, "end_offset": 18, "label": "bod" }, { "id": 3, "entity": "精索", "start_offset": 44, "end_offset": 46, "label": "bod" }, { "id": 4, "entity": "鞘状突", "start_offset": 47, "end_offset": 50, "label": "bod" }, { "id": 5, "entity": "纤维索带", "start_offset": 52, "end_offset": 56, "label": "bod" }, { "id": 6, "entity": "睾丸", "start_offset": 60, "end_offset": 62, "label": "bod" }, { "id": 7, "entity": "鞘膜", "start_offset": 64, "end_offset": 66, "label": "bod" }, { "id": 8, "entity": "睾丸固有鞘膜腔", "start_offset": 73, "end_offset": 80, "label": "bod" }, { "id": 9, "entity": "腹膜腔", "start_offset": 82, "end_offset": 85, "label": "bod" } ]
鞘状突的闭塞过程可能出现异常,在腹膜腔和睾丸固有鞘膜腔之间的不同水平,覆盖精索的鞘状突未完全闭合,使得腹腔液进入未完全闭合的鞘状突,并在已闭合的近端积聚,即形成临床所见的鞘膜积液。
[ { "id": 0, "entity": "鞘状突", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "腹膜腔", "start_offset": 16, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "鞘膜腔", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 3, "entity": "鞘状突", "start_offset": 40, "end_offset": 43, "label": "bod" }, { "id": 4, "entity": "腹腔液", "start_offset": 51, "end_offset": 54, "label": "bod" }, { "id": 5, "entity": "鞘状突", "start_offset": 62, "end_offset": 65, "label": "bod" }, { "id": 6, "entity": "鞘膜积液", "start_offset": 85, "end_offset": 89, "label": "sym" } ]
根据鞘突管闭合异常的部位,鞘膜积液基本上分为两个类型:①精索鞘膜积液:鞘状突在近睾丸部闭塞,而其近端至腹膜腔未闭,腹膜腔内液体经内环流入鞘状突临床表现为腹股沟囊性的肿块,但睾丸清晰可及睾丸鞘膜积液:整个鞘状突均未闭合,腹膜腔内液体经鞘状突固有鞘膜腔阴囊内囊性肿块睾丸被囊液所包围积液量大,睾丸难以触及睾丸鞘膜腔与腹膜腔之间未闭的鞘状突一般细小,液体不容易倒流回腹膜腔,故鞘膜腔内积液的张力很大未闭的鞘状突比较粗,则表现为交通性鞘膜积液,积液的张力一般不大,特征性的是,腹股沟或阴囊的肿块在患儿晚上上床时很明显一夜平卧后,晨起肿块明显缩小或消失。
[ { "id": 0, "entity": "鞘突管闭合异常", "start_offset": 2, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "鞘膜积液", "start_offset": 13, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "精索鞘膜积液", "start_offset": 28, "end_offset": 34, "label": "dis" }, { "id": 3, "entity": "鞘状突在近睾丸部闭塞,而其近端至腹膜腔未闭", "start_offset": 35, "end_offset": 56, "label": "sym" }, { "id": 4, "entity": "腹膜腔", "start_offset": 57, "end_offset": 60, "label": "bod" }, { "id": 5, "entity": "腹膜腔内液体经内环流入鞘状突", "start_offset": 57, "end_offset": 71, "label": "sym" }, { "id": 6, "entity": "临床表现为腹股沟囊性的肿块", "start_offset": 71, "end_offset": 84, "label": "sym" }, { "id": 7, "entity": "睾丸", "start_offset": 86, "end_offset": 88, "label": "bod" }, { "id": 8, "entity": "但睾丸清晰可及", "start_offset": 85, "end_offset": 92, "label": "sym" }, { "id": 9, "entity": "睾丸鞘膜积液", "start_offset": 92, "end_offset": 98, "label": "dis" }, { "id": 10, "entity": "鞘状突", "start_offset": 101, "end_offset": 104, "label": "bod" }, { "id": 11, "entity": "腹膜腔", "start_offset": 109, "end_offset": 112, "label": "bod" }, { "id": 12, "entity": "鞘状突", "start_offset": 116, "end_offset": 119, "label": "bod" }, { "id": 13, "entity": "鞘膜腔", "start_offset": 121, "end_offset": 124, "label": "bod" }, { "id": 14, "entity": "整个鞘状突均未闭合,腹膜腔内液体经鞘状突固有鞘膜腔", "start_offset": 99, "end_offset": 124, "label": "sym" }, { "id": 15, "entity": "阴囊", "start_offset": 124, "end_offset": 126, "label": "bod" }, { "id": 16, "entity": "阴囊内囊性肿块", "start_offset": 124, "end_offset": 131, "label": "sym" }, { "id": 17, "entity": "睾丸", "start_offset": 131, "end_offset": 133, "label": "bod" }, { "id": 18, "entity": "睾丸被囊液所包围", "start_offset": 131, "end_offset": 139, "label": "sym" }, { "id": 19, "entity": "睾丸", "start_offset": 144, "end_offset": 146, "label": "bod" }, { "id": 20, "entity": "积液量大,睾丸难以触及", "start_offset": 139, "end_offset": 150, "label": "sym" }, { "id": 21, "entity": "睾丸鞘膜腔", "start_offset": 150, "end_offset": 155, "label": "bod" }, { "id": 22, "entity": "腹膜腔", "start_offset": 156, "end_offset": 159, "label": "bod" }, { "id": 23, "entity": "鞘状突", "start_offset": 164, "end_offset": 167, "label": "bod" }, { "id": 24, "entity": "液体不容易倒流回腹膜腔", "start_offset": 172, "end_offset": 183, "label": "sym" }, { "id": 25, "entity": "鞘膜腔", "start_offset": 185, "end_offset": 188, "label": "bod" }, { "id": 26, "entity": "故鞘膜腔内积液的张力很大", "start_offset": 184, "end_offset": 196, "label": "sym" }, { "id": 27, "entity": "未闭的鞘状突比较粗,则表现为交通性鞘膜积液", "start_offset": 196, "end_offset": 217, "label": "sym" }, { "id": 28, "entity": "积液的张力一般不大", "start_offset": 218, "end_offset": 227, "label": "sym" }, { "id": 29, "entity": "腹股沟", "start_offset": 234, "end_offset": 237, "label": "bod" }, { "id": 30, "entity": "阴囊", "start_offset": 238, "end_offset": 240, "label": "bod" }, { "id": 31, "entity": "腹股沟或阴囊的肿块在患儿晚上上床时很明显", "start_offset": 234, "end_offset": 254, "label": "sym" }, { "id": 32, "entity": "一夜平卧后,晨起肿块明显缩小或消失", "start_offset": 254, "end_offset": 271, "label": "sym" } ]
鞘膜积液可双侧同时发生,女孩也有,称为Nuck囊肿,位于腹股沟。
[ { "id": 0, "entity": "鞘膜积液可双侧同时发生", "start_offset": 0, "end_offset": 11, "label": "sym" }, { "id": 1, "entity": "女孩也有,称为Nuck囊肿,位于腹股沟", "start_offset": 12, "end_offset": 31, "label": "sym" } ]
新生儿鞘膜积液相当常见,由于出生后鞘状突继续发生闭合,部分病例可逐渐自行消退,一般认为可观察至1岁,其后自行消退的可能性很小。
[ { "id": 0, "entity": "鞘膜积液", "start_offset": 3, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "鞘状突", "start_offset": 17, "end_offset": 20, "label": "bod" } ]
临床应引起重视的是睾丸肿瘤也可伴有鞘膜积液,在积液量多、张力高、未触及睾丸的情况下,尤其容易误诊。
[ { "id": 0, "entity": "睾丸肿瘤", "start_offset": 9, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "鞘膜积液", "start_offset": 17, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "张力高", "start_offset": 28, "end_offset": 31, "label": "sym" }, { "id": 3, "entity": "睾丸", "start_offset": 35, "end_offset": 37, "label": "bod" } ]
我们就有以鞘膜积液诊断手术而术中发现为睾丸肿瘤的经历。
[ { "id": 0, "entity": "鞘膜积液诊断手术", "start_offset": 5, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "睾丸肿瘤", "start_offset": 19, "end_offset": 23, "label": "dis" } ]
新生儿鞘膜积液至1岁后鞘膜鞘膜积液后发现的鞘膜积液均应手术治疗。
[ { "id": 0, "entity": "新生儿鞘膜积液", "start_offset": 0, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "鞘膜鞘膜积液", "start_offset": 11, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "鞘膜积液", "start_offset": 21, "end_offset": 25, "label": "dis" } ]
有主张如果积液张力较高,可能影响睾丸血液循环,导致睾丸萎缩,应早期手术治疗。
[ { "id": 0, "entity": "睾丸", "start_offset": 16, "end_offset": 18, "label": "bod" }, { "id": 1, "entity": "睾丸", "start_offset": 25, "end_offset": 27, "label": "bod" }, { "id": 2, "entity": "手术治疗", "start_offset": 33, "end_offset": 37, "label": "pro" } ]
鞘膜翻转缝合或鞘膜切除,主要用于成人鞘膜积液,小儿中完全结扎鞘状鞘状突放出积液关键是完全结扎鞘状突,放出积液。
[ { "id": 0, "entity": "鞘膜翻转缝合", "start_offset": 0, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "鞘膜切除", "start_offset": 7, "end_offset": 11, "label": "pro" }, { "id": 2, "entity": "成人鞘膜积液", "start_offset": 16, "end_offset": 22, "label": "dis" }, { "id": 3, "entity": "完全结扎鞘状鞘状突放出积液", "start_offset": 26, "end_offset": 39, "label": "pro" }, { "id": 4, "entity": "完全结扎鞘状突,放出积液", "start_offset": 42, "end_offset": 54, "label": "pro" } ]
第三章继发性免疫缺陷病临床实践中逐步发现很多免疫功能缺陷病为继发性免疫缺陷病(secondaryimmunodeficiencydisease,SID),SID与原发性免疫缺陷病(PID)的重要区别在于:①PID几乎都是特定的单基因缺失,导致相应的免疫活性细胞或免疫分子受损,表现出这种功能的完全缺失,且为不可逆的改变;而SID常为免疫系统多环节受损,但受损程度较PID轻,仅为部分功能受损,表现为免疫功能低下(immunocompromise);②PID系关键位基因突变,除非免疫重建,否则其免疫功能缺陷将为终身性,SID为后天环境因素致免疫功能缺陷,虽也能影响基因表达,但仅系基因不完全性表达障碍,去除不利因素之后,免疫功能将可能恢复正常。
[ { "id": 0, "entity": "继发性免疫缺陷病", "start_offset": 3, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "继发性免疫缺陷病", "start_offset": 30, "end_offset": 38, "label": "dis" }, { "id": 2, "entity": "secondaryimmunodeficiencydisease", "start_offset": 39, "end_offset": 71, "label": "dis" }, { "id": 3, "entity": "SID", "start_offset": 72, "end_offset": 75, "label": "dis" }, { "id": 4, "entity": "SID", "start_offset": 77, "end_offset": 80, "label": "dis" }, { "id": 5, "entity": "原发性免疫缺陷病", "start_offset": 81, "end_offset": 89, "label": "dis" }, { "id": 6, "entity": "PID", "start_offset": 90, "end_offset": 93, "label": "dis" }, { "id": 7, "entity": "PID", "start_offset": 103, "end_offset": 106, "label": "dis" }, { "id": 8, "entity": "免疫活性细胞", "start_offset": 124, "end_offset": 130, "label": "bod" }, { "id": 9, "entity": "免疫分子", "start_offset": 131, "end_offset": 135, "label": "bod" }, { "id": 10, "entity": "SID", "start_offset": 161, "end_offset": 164, "label": "dis" }, { "id": 11, "entity": "PID", "start_offset": 182, "end_offset": 185, "label": "dis" }, { "id": 12, "entity": "免疫功能低下", "start_offset": 199, "end_offset": 205, "label": "sym" }, { "id": 13, "entity": "immunocompromise", "start_offset": 206, "end_offset": 222, "label": "sym" }, { "id": 14, "entity": "PID", "start_offset": 225, "end_offset": 228, "label": "dis" }, { "id": 15, "entity": "SID", "start_offset": 259, "end_offset": 262, "label": "dis" } ]
【病因】(一)感染感染即是免疫缺陷病的临床表现之一,同时也是致SID的原因。
[ { "id": 0, "entity": "免疫缺陷病", "start_offset": 13, "end_offset": 18, "label": "dis" }, { "id": 1, "entity": "SID", "start_offset": 31, "end_offset": 34, "label": "dis" } ]
人类免疫缺陷病毒(HIV)感染致获得性免疫缺陷病(aquiredimmunodeficiencysyndrome,AIDS)是感染引起SID的典型例子。
[ { "id": 0, "entity": "人类免疫缺陷病毒", "start_offset": 0, "end_offset": 8, "label": "mic" }, { "id": 1, "entity": "HIV", "start_offset": 9, "end_offset": 12, "label": "mic" }, { "id": 2, "entity": "获得性免疫缺陷病", "start_offset": 16, "end_offset": 24, "label": "dis" }, { "id": 3, "entity": "aquiredimmunodeficiencysyndrome", "start_offset": 25, "end_offset": 56, "label": "dis" }, { "id": 4, "entity": "AIDS", "start_offset": 57, "end_offset": 61, "label": "dis" }, { "id": 5, "entity": "SID", "start_offset": 67, "end_offset": 70, "label": "dis" } ]
(二)营养紊乱(五)医源性因素药物是临床实践中较常见的免疫低下的重要原因,如糖皮质激素、细胞毒性药物及放射性照射等均可引起SID。
[ { "id": 0, "entity": "营养紊乱", "start_offset": 3, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "糖皮质激素", "start_offset": 38, "end_offset": 43, "label": "dru" }, { "id": 2, "entity": "细胞毒性药物", "start_offset": 44, "end_offset": 50, "label": "dru" }, { "id": 3, "entity": "放射性照射", "start_offset": 51, "end_offset": 56, "label": "pro" }, { "id": 4, "entity": "SID", "start_offset": 61, "end_offset": 64, "label": "dis" } ]
在自身免疫性疾病及肿瘤等多种疾病的治疗中采用这些手段均要警惕SID的发生。
[ { "id": 0, "entity": "自身免疫性疾病", "start_offset": 1, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "肿瘤", "start_offset": 9, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "SID", "start_offset": 30, "end_offset": 33, "label": "dis" } ]
引起SID因素复杂,现列简表如下(表17-17)。
[ { "id": 0, "entity": "SID", "start_offset": 2, "end_offset": 5, "label": "dis" } ]
表17-6各种维生素和微量元素缺乏引起的免疫功能缺陷注:+轻度抑制,++中度抑制,+++严重抑制,*</sup>动物模型结果,尚未在人体证实表17-7导致继发性免疫缺陷病的因素【临床表现与治疗】SID的临床表现与PID大致相同,但其程度往往轻于后者,治疗效果也较好,反复感染是突出表现,并发肿瘤与自身免疫性疾病的机会相对较少。
[ { "id": 0, "entity": "维生素", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "微量元素", "start_offset": 11, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "继发性免疫缺陷病", "start_offset": 77, "end_offset": 85, "label": "dis" }, { "id": 3, "entity": "SID", "start_offset": 97, "end_offset": 100, "label": "dis" }, { "id": 4, "entity": "PID", "start_offset": 106, "end_offset": 109, "label": "dis" }, { "id": 5, "entity": "肿瘤", "start_offset": 145, "end_offset": 147, "label": "dis" }, { "id": 6, "entity": "自身免疫性疾病", "start_offset": 148, "end_offset": 155, "label": "dis" } ]
SID治疗原则上是根治原发病及免疫替代治疗和对症治疗,替代治疗和对症治疗均要视免疫缺陷的类型及后果而定,因在何种因素影响下,产生何种特殊免疫异常并不十分明了,故只有根治原发病,才有可能彻底纠正SID。
[ { "id": 0, "entity": "SID", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "免疫替代治疗", "start_offset": 15, "end_offset": 21, "label": "pro" }, { "id": 2, "entity": "对症治疗", "start_offset": 22, "end_offset": 26, "label": "pro" }, { "id": 3, "entity": "替代治疗", "start_offset": 27, "end_offset": 31, "label": "pro" }, { "id": 4, "entity": "对症治疗", "start_offset": 32, "end_offset": 36, "label": "pro" }, { "id": 5, "entity": "SID", "start_offset": 96, "end_offset": 99, "label": "dis" } ]
三、CVS临床表现1.CVS分期和分级CVS分为4个时期:①间歇期:几乎没有症状;②前驱期:有接近于发作的表现,通过药物尚能控制;③呕吐期:持续而强烈的恶心、呕吐、干呕和其他症状;④恢复期:恶心很快停止,患者恢复食欲及精神状态。
[ { "id": 0, "entity": "CVS", "start_offset": 2, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "CVS", "start_offset": 11, "end_offset": 14, "label": "dis" }, { "id": 2, "entity": "CVS", "start_offset": 19, "end_offset": 22, "label": "dis" }, { "id": 3, "entity": "几乎没有症状", "start_offset": 34, "end_offset": 40, "label": "sym" }, { "id": 4, "entity": "有接近于发作的表现", "start_offset": 46, "end_offset": 55, "label": "sym" }, { "id": 5, "entity": "持续而强烈的恶心、呕吐、干呕和其他症状", "start_offset": 70, "end_offset": 89, "label": "sym" }, { "id": 6, "entity": "恶心很快停止", "start_offset": 95, "end_offset": 101, "label": "sym" }, { "id": 7, "entity": "患者恢复食欲及精神状态", "start_offset": 102, "end_offset": 113, "label": "sym" } ]
2.CVS临床表现特点CVS以反复发生、刻板发作的剧烈恶心、呕吐为特征,持续数小时到数天。
[ { "id": 0, "entity": "CVS", "start_offset": 2, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "CVS", "start_offset": 11, "end_offset": 14, "label": "dis" }, { "id": 2, "entity": "反复发生、刻板发作的剧烈恶心、呕吐", "start_offset": 15, "end_offset": 32, "label": "sym" }, { "id": 3, "entity": "持续数小时到数天", "start_offset": 36, "end_offset": 44, "label": "sym" } ]
间歇期无症状,可持续数周到数月。
[ { "id": 0, "entity": "间歇期无症状", "start_offset": 0, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "可持续数周到数月", "start_offset": 7, "end_offset": 15, "label": "sym" } ]
每日发作时间比较固定,通常在晚上或凌晨。
[ { "id": 0, "entity": "每日发作时间比较固定,通常在晚上或凌晨", "start_offset": 0, "end_offset": 19, "label": "sym" } ]
一旦发作,在最初的数小时内便达到最大强度,发作和停止却非常快速,呈一种“开-关”刻板模式。
[ { "id": 0, "entity": "一旦发作,在最初的数小时内便达到最大强度", "start_offset": 0, "end_offset": 20, "label": "sym" }, { "id": 1, "entity": "发作和停止却非常快速", "start_offset": 21, "end_offset": 31, "label": "sym" }, { "id": 2, "entity": "呈一种“开-关”刻板模式", "start_offset": 32, "end_offset": 44, "label": "sym" } ]
二、HSCT适应证HSCT医疗费用高、移植风险大,因此必须把握好移植指征,只有当移植治疗疗效明显优于其他治疗方法时才有必要采用。
[ { "id": 0, "entity": "HSCT", "start_offset": 2, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "HSCT", "start_offset": 9, "end_offset": 13, "label": "pro" }, { "id": 2, "entity": "移植", "start_offset": 19, "end_offset": 21, "label": "pro" }, { "id": 3, "entity": "移植", "start_offset": 32, "end_offset": 34, "label": "pro" }, { "id": 4, "entity": "指征", "start_offset": 34, "end_offset": 36, "label": "sym" }, { "id": 5, "entity": "移植", "start_offset": 40, "end_offset": 42, "label": "pro" } ]
(一)异基因HSCT常见适应证1.造血系统恶性疾病(1)急性淋巴细胞白血病(ALL):①高度难治或高危复发危险的ALL,如初治35天未达完全缓解者;有t(9;22)或t(4;11)并对早期治疗反应不良者(如诱导治疗第14~19天骨髓涂片肿瘤细胞仍大于5%)。
[ { "id": 0, "entity": "异基因HSCT", "start_offset": 3, "end_offset": 10, "label": "pro" }, { "id": 1, "entity": "造血系统恶性疾病", "start_offset": 17, "end_offset": 25, "label": "dis" }, { "id": 2, "entity": "急性淋巴细胞白血病", "start_offset": 28, "end_offset": 37, "label": "dis" }, { "id": 3, "entity": "ALL", "start_offset": 38, "end_offset": 41, "label": "dis" }, { "id": 4, "entity": "ALL", "start_offset": 56, "end_offset": 59, "label": "dis" }, { "id": 5, "entity": "诱导治疗", "start_offset": 103, "end_offset": 107, "label": "pro" }, { "id": 6, "entity": "骨髓涂片", "start_offset": 114, "end_offset": 118, "label": "pro" }, { "id": 7, "entity": "肿瘤细胞", "start_offset": 118, "end_offset": 122, "label": "bod" } ]
②复发ALL再CR者(低危停药半年以上晚期复发的CR2B-ALL仍应以化疗为主)。
[ { "id": 0, "entity": "复发ALL再CR", "start_offset": 1, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "低危停药半年以上晚期复发的CR2B-ALL", "start_offset": 11, "end_offset": 32, "label": "dis" }, { "id": 2, "entity": "化疗", "start_offset": 35, "end_offset": 37, "label": "pro" } ]
非CR状态的ALL无移植指征。
[ { "id": 0, "entity": "非CR状态的ALL", "start_offset": 0, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "移植", "start_offset": 10, "end_offset": 12, "label": "pro" }, { "id": 2, "entity": "指征", "start_offset": 12, "end_offset": 14, "label": "sym" } ]
(2)AML:①经正规诱导治疗1个疗程恢复造血后幼稚细胞仍然大于15%或2个疗程未CR者;②复发后再CR者;③高度难治,多个疗程后仍然未CR但幼稚细胞比例不很高者;④MDS转化而来的AML、治疗相关的AML患者、M0</sub>、M7</sub>患者[排除t(1;22)];⑤若为同胞相合供体,所有非M3、非低危AML均应考虑HSCT。
[ { "id": 0, "entity": "AML", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "诱导治疗", "start_offset": 11, "end_offset": 15, "label": "pro" }, { "id": 2, "entity": "幼稚细胞", "start_offset": 24, "end_offset": 28, "label": "bod" }, { "id": 3, "entity": "幼稚细胞", "start_offset": 71, "end_offset": 75, "label": "bod" }, { "id": 4, "entity": "MDS", "start_offset": 83, "end_offset": 86, "label": "dis" }, { "id": 5, "entity": "AML", "start_offset": 91, "end_offset": 94, "label": "dis" }, { "id": 6, "entity": "AML", "start_offset": 100, "end_offset": 103, "label": "dis" }, { "id": 7, "entity": "M0</sub>", "start_offset": 106, "end_offset": 114, "label": "dis" }, { "id": 8, "entity": "M7</sub>", "start_offset": 115, "end_offset": 123, "label": "dis" }, { "id": 9, "entity": "非M3", "start_offset": 149, "end_offset": 152, "label": "dis" }, { "id": 10, "entity": "非低危AML", "start_offset": 153, "end_offset": 159, "label": "dis" }, { "id": 11, "entity": "HSCT", "start_offset": 163, "end_offset": 167, "label": "pro" } ]
(3)慢性粒细胞性白血病。
[ { "id": 0, "entity": "慢性粒细胞性白血病", "start_offset": 3, "end_offset": 12, "label": "dis" } ]
(4)非霍奇金淋巴瘤伴有骨髓浸润并经正规治疗后复发者。
[ { "id": 0, "entity": "非霍奇金淋巴瘤", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "伴有骨髓浸润", "start_offset": 10, "end_offset": 16, "label": "sym" } ]
(5)骨髓增生异常综合征。
[ { "id": 0, "entity": "骨髓增生异常综合征", "start_offset": 3, "end_offset": 12, "label": "dis" } ]
2.预后不良的造血系统衰竭性疾病(1)重型再生障碍性贫血(SAA)。
[ { "id": 0, "entity": "造血系统衰竭性疾病", "start_offset": 7, "end_offset": 16, "label": "dis" }, { "id": 1, "entity": "重型再生障碍性贫血", "start_offset": 19, "end_offset": 28, "label": "dis" }, { "id": 2, "entity": "SAA", "start_offset": 29, "end_offset": 32, "label": "dis" } ]
(2)继发性造血系统衰竭(如放射病)。
[ { "id": 0, "entity": "继发性造血系统衰竭", "start_offset": 3, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "放射病", "start_offset": 14, "end_offset": 17, "label": "dis" } ]
3.先天性造血、免疫系统疾病(1)家族性噬血细胞增生征。
[ { "id": 0, "entity": "先天性造血、免疫系统疾病", "start_offset": 2, "end_offset": 14, "label": "dis" }, { "id": 1, "entity": "家族性噬血细胞增生征", "start_offset": 17, "end_offset": 27, "label": "dis" } ]
(2)免疫缺陷:重型联合免疫缺陷、慢性肉芽肿病、X-连锁高IgM血症、Wiskott-Aldrich综合征、Chediak-Higashi综合征等。
[ { "id": 0, "entity": "免疫缺陷", "start_offset": 3, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "重型联合免疫缺陷", "start_offset": 8, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "慢性肉芽肿病", "start_offset": 17, "end_offset": 23, "label": "dis" }, { "id": 3, "entity": "X-连锁高IgM血症", "start_offset": 24, "end_offset": 34, "label": "dis" }, { "id": 4, "entity": "Wiskott-Aldrich综合征", "start_offset": 35, "end_offset": 53, "label": "dis" }, { "id": 5, "entity": "Chediak-Higashi综合征", "start_offset": 54, "end_offset": 72, "label": "dis" } ]
(3)重型地中海贫血、先天性粒细胞缺乏症、药物治疗无效的先天性纯红再障。
[ { "id": 0, "entity": "重型地中海贫血", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "先天性粒细胞缺乏症", "start_offset": 11, "end_offset": 20, "label": "dis" }, { "id": 2, "entity": "先天性纯红再障", "start_offset": 28, "end_offset": 35, "label": "dis" } ]
4.先天代谢异常如黏多糖累积症Ⅰ、Ⅱ、Ⅵ型,肾上腺脑白质营养不良、甘露糖苷储积症、戈谢病、尼曼匹克病、石骨症等。
[ { "id": 0, "entity": "先天代谢异常", "start_offset": 2, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "黏多糖累积症Ⅰ、Ⅱ、Ⅵ型", "start_offset": 9, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "肾上腺脑白质营养不良", "start_offset": 22, "end_offset": 32, "label": "dis" }, { "id": 3, "entity": "甘露糖苷储积症", "start_offset": 33, "end_offset": 40, "label": "dis" }, { "id": 4, "entity": "戈谢病", "start_offset": 41, "end_offset": 44, "label": "dis" }, { "id": 5, "entity": "尼曼匹克病", "start_offset": 45, "end_offset": 50, "label": "dis" }, { "id": 6, "entity": "石骨症", "start_offset": 51, "end_offset": 54, "label": "dis" } ]
(二)自体移植的适应证1.无骨髓浸润的化疗一定程度敏感的晚期恶性实体瘤,如神经母细胞瘤、脑瘤、尤因肉瘤、横纹肌肉瘤、恶性生殖细胞性肿瘤等。
[ { "id": 0, "entity": "自体移植", "start_offset": 3, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "骨髓", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "化疗", "start_offset": 19, "end_offset": 21, "label": "pro" }, { "id": 3, "entity": "晚期恶性实体瘤", "start_offset": 28, "end_offset": 35, "label": "dis" }, { "id": 4, "entity": "神经母细胞瘤", "start_offset": 37, "end_offset": 43, "label": "dis" }, { "id": 5, "entity": "脑瘤", "start_offset": 44, "end_offset": 46, "label": "dis" }, { "id": 6, "entity": "尤因肉瘤", "start_offset": 47, "end_offset": 51, "label": "dis" }, { "id": 7, "entity": "横纹肌肉瘤", "start_offset": 52, "end_offset": 57, "label": "dis" }, { "id": 8, "entity": "恶性生殖细胞性肿瘤", "start_offset": 58, "end_offset": 67, "label": "dis" } ]
2.难治性自身免疫性疾病。
[ { "id": 0, "entity": "难治性自身免疫性疾病", "start_offset": 2, "end_offset": 12, "label": "dis" } ]
第四节成分输血与其他血制品的应用在血液系统、肿瘤性疾病和其他一些危重疾病的治疗过程中常涉及血制品的应用,不同的临床状况需应用不同的血制品。
[ { "id": 0, "entity": "成分输血", "start_offset": 3, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "血制品", "start_offset": 10, "end_offset": 13, "label": "dru" }, { "id": 2, "entity": "血液系统", "start_offset": 17, "end_offset": 21, "label": "bod" }, { "id": 3, "entity": "肿瘤性疾病", "start_offset": 22, "end_offset": 27, "label": "dis" }, { "id": 4, "entity": "血制品", "start_offset": 45, "end_offset": 48, "label": "dru" }, { "id": 5, "entity": "血制品", "start_offset": 65, "end_offset": 68, "label": "dru" } ]
一、血制品的种类及应用指征(一)血细胞成分制品1.全血即为未祛除任何成分的血液。
[ { "id": 0, "entity": "血制品", "start_offset": 2, "end_offset": 5, "label": "dru" }, { "id": 1, "entity": "血细胞", "start_offset": 16, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "全血", "start_offset": 25, "end_offset": 27, "label": "bod" }, { "id": 3, "entity": "血液", "start_offset": 37, "end_offset": 39, "label": "bod" } ]
正常人新鲜全血含有各种有效的血细胞及血浆成分,库存血随着库存时间的延长,根据半衰期不同逐渐丧失某些成分的活性,如新鲜全血中含有凝血因子Ⅷ,在无其他途径获得Ⅷ因子时,可采用新鲜全血作替代治疗,但Ⅷ因子半衰期仅为8~12小时,因此库存血就不能用作Ⅷ因子缺乏的替代治疗。
[ { "id": 0, "entity": "全血", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "血细胞", "start_offset": 14, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "血浆", "start_offset": 18, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "全血", "start_offset": 58, "end_offset": 60, "label": "bod" }, { "id": 4, "entity": "凝血因子Ⅷ", "start_offset": 63, "end_offset": 68, "label": "bod" }, { "id": 5, "entity": "Ⅷ因子", "start_offset": 77, "end_offset": 80, "label": "bod" }, { "id": 6, "entity": "全血", "start_offset": 87, "end_offset": 89, "label": "bod" }, { "id": 7, "entity": "Ⅷ因子", "start_offset": 96, "end_offset": 99, "label": "bod" }, { "id": 8, "entity": "Ⅷ因子", "start_offset": 121, "end_offset": 124, "label": "bod" } ]
目前全血的应用日趋减少,主要有以下原因:①绝大多数疾病状态仅缺乏血液的某些成分;②某些特定成分缺乏时如采用全血,很难达到有效水平,因此影响疗效;③血液成分的分离技术越来越完善;④采用全血时浪费所需成分以外的其他血液成分。
[ { "id": 0, "entity": "全血", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "血液", "start_offset": 32, "end_offset": 34, "label": "bod" }, { "id": 2, "entity": "全血", "start_offset": 53, "end_offset": 55, "label": "bod" }, { "id": 3, "entity": "血液", "start_offset": 73, "end_offset": 75, "label": "bod" }, { "id": 4, "entity": "全血", "start_offset": 91, "end_offset": 93, "label": "bod" }, { "id": 5, "entity": "血液", "start_offset": 105, "end_offset": 107, "label": "bod" } ]
2.以红细胞为主要成分的少浆血根据需要在全血中提取血浆、血小板、白细胞后的以红细胞为主要成分的悬液,是应用最为广泛的血液成分,根据被提取的成分不同可分别称为少浆全血、去白细胞少浆血、去血小板少浆血。
[ { "id": 0, "entity": "红细胞", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "浆血", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "全血", "start_offset": 20, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "血浆", "start_offset": 25, "end_offset": 27, "label": "bod" }, { "id": 4, "entity": "血小板", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 5, "entity": "白细胞", "start_offset": 32, "end_offset": 35, "label": "bod" }, { "id": 6, "entity": "红细胞", "start_offset": 38, "end_offset": 41, "label": "bod" }, { "id": 7, "entity": "悬液", "start_offset": 47, "end_offset": 49, "label": "bod" }, { "id": 8, "entity": "血液", "start_offset": 58, "end_offset": 60, "label": "bod" }, { "id": 9, "entity": "少浆全血", "start_offset": 78, "end_offset": 82, "label": "dru" }, { "id": 10, "entity": "去白细胞少浆血", "start_offset": 83, "end_offset": 90, "label": "dru" }, { "id": 11, "entity": "去血小板少浆血", "start_offset": 91, "end_offset": 98, "label": "dru" } ]
少浆血可应用于各种原因引起的贫血。
[ { "id": 0, "entity": "少浆血", "start_offset": 0, "end_offset": 3, "label": "dru" }, { "id": 1, "entity": "贫血", "start_offset": 14, "end_offset": 16, "label": "dis" } ]
在无明显失血或急性溶血时,输注10ml/kg少浆血可提高血色素20g/L(上海儿童医学中心100例输血疗效分析结果)。
[ { "id": 0, "entity": "失血", "start_offset": 4, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "急性溶血", "start_offset": 7, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "少浆血", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "血色素", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 4, "entity": "输血", "start_offset": 49, "end_offset": 51, "label": "pro" } ]
每次输血量一般为5~10ml/kg,对较为慢性病程的病人,贫血越严重,单次输血量应越少,并需缓慢输入,以避免因血容量急剧增多而致心力衰竭。
[ { "id": 0, "entity": "输血量", "start_offset": 2, "end_offset": 5, "label": "ite" }, { "id": 1, "entity": "贫血", "start_offset": 29, "end_offset": 31, "label": "dis" }, { "id": 2, "entity": "输血量", "start_offset": 37, "end_offset": 40, "label": "ite" }, { "id": 3, "entity": "血容量", "start_offset": 55, "end_offset": 58, "label": "ite" }, { "id": 4, "entity": "心力衰竭", "start_offset": 64, "end_offset": 68, "label": "dis" } ]
3.血小板通过血细胞分离仪从单个供血者采集的血小板称为单采血小板,从2000ml全血中分离的血小板量为1个单采单位,容量为200ml。
[ { "id": 0, "entity": "血小板", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "血细胞分离仪", "start_offset": 7, "end_offset": 13, "label": "equ" }, { "id": 2, "entity": "血小板", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "单采血小板", "start_offset": 27, "end_offset": 32, "label": "dru" }, { "id": 4, "entity": "全血", "start_offset": 40, "end_offset": 42, "label": "bod" }, { "id": 5, "entity": "血小板量", "start_offset": 46, "end_offset": 50, "label": "ite" } ]
从已采集的非同一供体的全血中分离的血小板为多采血小板,从200ml全血中分离的血小板量为1个多采单位,容量为25ml,10个多采单位相当于1个单采单位。
[ { "id": 0, "entity": "全血", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "血小板", "start_offset": 17, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "血小板", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "全血", "start_offset": 33, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "血小板量", "start_offset": 39, "end_offset": 43, "label": "ite" } ]
血小板输注主要用于血小板明显减少或功能异常并伴有出血倾向者。
[ { "id": 0, "entity": "血小板", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "输注", "start_offset": 3, "end_offset": 5, "label": "pro" }, { "id": 2, "entity": "血小板", "start_offset": 9, "end_offset": 12, "label": "bod" }, { "id": 3, "entity": "血小板明显减少或功能异常并伴有出血", "start_offset": 9, "end_offset": 26, "label": "sym" } ]
输注10ml/kg单采血小板可提高血小板50×109</sup>/L(上海儿童医学中心50例单采血小板输注疗效分析结果)。
[ { "id": 0, "entity": "血小板", "start_offset": 11, "end_offset": 14, "label": "bod" }, { "id": 1, "entity": "血小板", "start_offset": 17, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "血小板输注", "start_offset": 48, "end_offset": 53, "label": "pro" } ]
在血小板相关抗体明显增高时输注效果减低。
[ { "id": 0, "entity": "血小板", "start_offset": 1, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "抗体", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 2, "entity": "输注", "start_offset": 13, "end_offset": 15, "label": "pro" } ]
血小板寿命短,需新鲜采集使用。
[ { "id": 0, "entity": "血小板", "start_offset": 0, "end_offset": 3, "label": "bod" } ]
4.白细胞通过血细胞分离仪可分离得白细胞,但白细胞输注的疗效尚未被充分肯定,主要用于粒细胞极度低下及粒细胞功能障碍。
[ { "id": 0, "entity": "白细胞", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "血细胞分离仪", "start_offset": 7, "end_offset": 13, "label": "equ" }, { "id": 2, "entity": "白细胞", "start_offset": 17, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "白细胞输注", "start_offset": 22, "end_offset": 27, "label": "pro" }, { "id": 4, "entity": "粒细胞极度低下", "start_offset": 42, "end_offset": 49, "label": "dis" }, { "id": 5, "entity": "粒细胞功能障碍", "start_offset": 50, "end_offset": 57, "label": "dis" } ]
白细胞寿命短,也需新鲜采集使用。
[ { "id": 0, "entity": "白细胞", "start_offset": 0, "end_offset": 3, "label": "bod" } ]
5.造血干细胞输注见干细胞移植章节。
[ { "id": 0, "entity": "造血干细胞", "start_offset": 2, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "输注", "start_offset": 7, "end_offset": 9, "label": "pro" }, { "id": 2, "entity": "干细胞移植", "start_offset": 10, "end_offset": 15, "label": "pro" } ]
(二)血浆成分1.全血浆新鲜全血浆或新鲜冰冻全血浆含所有正常血浆成分,主要用于非失血性低血容量性休克的扩容、低蛋白血症、低免疫球蛋白血症、各种凝血因子缺乏症和其他血浆成分缺乏的替代治疗,常用剂量为5~10ml/kg。
[ { "id": 0, "entity": "血浆", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "全血浆", "start_offset": 9, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "全血浆", "start_offset": 14, "end_offset": 17, "label": "bod" }, { "id": 3, "entity": "血浆", "start_offset": 23, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "血浆", "start_offset": 30, "end_offset": 32, "label": "bod" }, { "id": 5, "entity": "非失血性低血容量性休克", "start_offset": 39, "end_offset": 50, "label": "dis" }, { "id": 6, "entity": "低蛋白血症", "start_offset": 54, "end_offset": 59, "label": "dis" }, { "id": 7, "entity": "低免疫球蛋白血症", "start_offset": 60, "end_offset": 68, "label": "dis" }, { "id": 8, "entity": "凝血因子缺乏症", "start_offset": 71, "end_offset": 78, "label": "dis" }, { "id": 9, "entity": "血浆", "start_offset": 81, "end_offset": 83, "label": "dis" } ]
2.丙种球蛋白静脉用丙种球蛋白主要用于低丙种球蛋白血症的替代治疗及感染性疾病的辅助支持治疗,常用剂量为200~500mg/kg。
[ { "id": 0, "entity": "丙种球蛋白", "start_offset": 2, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "静脉", "start_offset": 7, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "丙种球蛋白", "start_offset": 10, "end_offset": 15, "label": "bod" }, { "id": 3, "entity": "低丙种球蛋白血症", "start_offset": 19, "end_offset": 27, "label": "dis" }, { "id": 4, "entity": "替代治疗", "start_offset": 28, "end_offset": 32, "label": "pro" }, { "id": 5, "entity": "感染性疾病", "start_offset": 33, "end_offset": 38, "label": "dis" }, { "id": 6, "entity": "辅助支持治疗", "start_offset": 39, "end_offset": 45, "label": "pro" } ]
大剂量(≥1克/千克体重)时有免疫封闭作用,可作为特发性血小板减少性紫癜和再障的免疫抑制治疗。
[ { "id": 0, "entity": "特发性血小板减少性紫癜", "start_offset": 25, "end_offset": 36, "label": "dis" }, { "id": 1, "entity": "再障", "start_offset": 37, "end_offset": 39, "label": "dis" }, { "id": 2, "entity": "免疫抑制治疗", "start_offset": 40, "end_offset": 46, "label": "pro" } ]
3.凝血因子Ⅷ从血浆中提纯,主要用于血友病甲的替代治疗,1单位/千克体重可提高Ⅷ因子活力2%。
[ { "id": 0, "entity": "凝血因子Ⅷ", "start_offset": 2, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "血浆", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "血友病甲", "start_offset": 18, "end_offset": 22, "label": "dis" }, { "id": 3, "entity": "替代治疗", "start_offset": 23, "end_offset": 27, "label": "pro" }, { "id": 4, "entity": "体重", "start_offset": 34, "end_offset": 36, "label": "ite" }, { "id": 5, "entity": "Ⅷ因子", "start_offset": 39, "end_offset": 42, "label": "bod" } ]
4.凝血酶原复合物用于多种内外源凝血因子缺乏的补充及替代治疗,如Ⅶ、Ⅷ、Ⅸ、Ⅹ因子缺乏,包括血友病及维生素K依赖性凝血因子缺乏症。
[ { "id": 0, "entity": "凝血酶原复合物", "start_offset": 2, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "内外源凝血因子缺乏", "start_offset": 13, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "替代治疗", "start_offset": 26, "end_offset": 30, "label": "pro" }, { "id": 3, "entity": "Ⅶ、Ⅷ、Ⅸ、Ⅹ因子缺乏", "start_offset": 32, "end_offset": 43, "label": "sym" }, { "id": 4, "entity": "血友病", "start_offset": 46, "end_offset": 49, "label": "dis" }, { "id": 5, "entity": "维生素K依赖性凝血因子缺乏症", "start_offset": 50, "end_offset": 64, "label": "dis" } ]
5.纤维蛋白原复合物主要用于纤维蛋白原缺乏症和异常纤维蛋白原血症。
[ { "id": 0, "entity": "纤维蛋白原复合物", "start_offset": 2, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "纤维蛋白原缺乏症", "start_offset": 14, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "异常纤维蛋白原血症", "start_offset": 23, "end_offset": 32, "label": "dis" } ]
(三)特殊处理的血制品1.三洗红细胞用生理盐水三次洗涤的红细胞,主要洗去可加重免疫性溶血的补体,用于免疫性溶血性贫血需输血的病人。
[ { "id": 0, "entity": "血制品", "start_offset": 8, "end_offset": 11, "label": "dru" }, { "id": 1, "entity": "三洗红细胞", "start_offset": 13, "end_offset": 18, "label": "dru" }, { "id": 2, "entity": "生理盐水", "start_offset": 19, "end_offset": 23, "label": "dru" }, { "id": 3, "entity": "洗涤", "start_offset": 25, "end_offset": 27, "label": "pro" }, { "id": 4, "entity": "红细胞", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 5, "entity": "免疫性溶血", "start_offset": 39, "end_offset": 44, "label": "dis" }, { "id": 6, "entity": "补体", "start_offset": 45, "end_offset": 47, "label": "bod" }, { "id": 7, "entity": "免疫性溶血性贫血", "start_offset": 50, "end_offset": 58, "label": "dis" }, { "id": 8, "entity": "输血", "start_offset": 59, "end_offset": 61, "label": "pro" } ]
2.照光血制品强烈化放疗免疫功能极度抑制病人、细胞免疫功能不全、异体移植后免疫功能未完全重建者输注含有淋巴细胞的血制品时,血制品需先经1800cGy照光,以灭活可能导致移植物抗宿主病的淋巴细胞。
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3.去白细胞成分血正常人白细胞携带CMV机会较高,异体骨髓移植受体输注后易发生CMV感染并可导致死亡,对异体骨髓移植者可输注去白细胞成分血。
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五、弥散性血管内凝血弥散性血管内凝血(DIC)是许多疾病严重阶段的并发症,机体在某些致病因素作用下,凝血系统被激活,凝血过程加速,微循环内发生纤维蛋白沉积和血小板凝集,导致血管内广泛微血栓形成,由于凝血因子被消耗,发生广泛出血。
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新生儿由于易患严重疾病,DIC的发生率较高。
[ { "id": 0, "entity": "DIC", "start_offset": 12, "end_offset": 15, "label": "dis" } ]
【病因与发病机制】1.感染严重感染是导致新生儿DIC的主要原因。
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2.缺氧缺氧导致酸中毒、血黏度增高。
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3.寒冷损伤由于寒冷及皮下脂肪变硬,微循环灌流不足,毛细血管损伤。
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4.溶血由于红细胞破坏,释放大量磷脂类凝血活酶物质,促发DIC。
[ { "id": 0, "entity": "红细胞", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "磷脂类凝血活酶", "start_offset": 16, "end_offset": 23, "label": "bod" }, { "id": 2, "entity": "DIC", "start_offset": 28, "end_offset": 31, "label": "dis" } ]
5.其他早产儿因易发生各种疾病及凝血机制不完善,DIC发生率较高。
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某些产科因素,如胎盘早期剥离、前置胎盘、严重妊娠高血压等,由于胎盘损伤释放组织凝血活酶进入胎儿循环,促发DIC。
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【临床表现】1.出血因凝血因子大量被消耗、血小板减少及继发纤溶亢进,发生出血。
[ { "id": 0, "entity": "凝血因子", "start_offset": 11, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "血小板", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "继发纤溶亢进", "start_offset": 27, "end_offset": 33, "label": "dis" } ]
常见皮肤瘀点、瘀斑、脐部渗血、穿刺点渗血,严重者出现消化道、泌尿道、肺出血等全身广泛出血。
[ { "id": 0, "entity": "皮肤瘀点", "start_offset": 2, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "瘀斑", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 2, "entity": "脐部渗血", "start_offset": 10, "end_offset": 14, "label": "sym" }, { "id": 3, "entity": "穿刺点渗血", "start_offset": 15, "end_offset": 20, "label": "sym" }, { "id": 4, "entity": "消化道、泌尿道、肺出血", "start_offset": 26, "end_offset": 37, "label": "sym" }, { "id": 5, "entity": "全身广泛出血", "start_offset": 38, "end_offset": 44, "label": "sym" } ]
2.休克由于微循环广泛血栓形成,通路受阻,发生循环障碍,出现休克。
[ { "id": 0, "entity": "休克", "start_offset": 2, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "血栓形成", "start_offset": 11, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "通路受阻", "start_offset": 16, "end_offset": 20, "label": "sym" }, { "id": 3, "entity": "循环障碍", "start_offset": 23, "end_offset": 27, "label": "sym" }, { "id": 4, "entity": "休克", "start_offset": 30, "end_offset": 32, "label": "sym" } ]
3.栓塞由于微循环广泛血栓形成,受累脏器发生缺氧缺血损伤,出现多脏器功能衰竭。
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4.溶血因红细胞变形受损,发生微血管病性溶血,出现血红蛋白尿、黄疸、发热。
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2.DIC的主要指标监测D-二聚体、血小板、凝血时间、凝血酶原时间、3P试验等项目的动态变化。
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3.使用肝素早期使用小剂量肝素,20U/kg,皮下注射,每天2~3次。
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4.改善微循环低分子右旋糖酐,10~20ml/kg,静脉滴注。
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三、影像学检查影像学检查(超声,X线,CT,MRI等)主要是作为关节炎及其他器官损害的定位与程度评价,除极少数特征性骨关节病变外,是重要的鉴别诊断及疗效评价方法,基本上没有病因诊断价值。
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二、选择常见病原菌的敏感抗生素类型,进行初始经验性治疗不论CAP还是HAP,绝大多数肺炎患儿的治疗是经验性的;在初始选择抗生素时候,应当覆盖大多数可能的病原,同时避免使用多种或过于贵重以及毒性较强的抗生素。
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