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PBPV有两种方法，即单球囊肺动脉瓣膜成形术和双球囊肺动脉瓣成形术。
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为了达到足够的球囊/瓣环比值，有些病例需作双球囊扩张术，分别由左右股静脉进行穿刺插入球囊导管，方法同单球囊扩张术。
|
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以下简述单球囊法。
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先以端孔导管或球囊端孔漂浮导管，经股静脉、下腔静脉、右心房、肺总动脉，最后到达肺小动脉，然后插入直头或弯头导引钢丝至肺下叶动脉，撤去端孔导管，用扩张管扩大股静脉穿刺口，使球囊导管顺利插入。
|
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"entity": "右心房",
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"entity": "肺总动脉",
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"entity": "肺小动脉",
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"entity": "直头或弯头导引钢丝",
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"entity": "肺下叶动脉",
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球囊导管插入前应检查有无破损及漏气，同时应用稀释造影剂，扩张或吸瘪球囊数次以驱除空气，防止球囊破裂时空气释出而引起气栓并发症。
|
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推送球囊导管直至肺动脉瓣处。
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先以少量造影剂注入球囊扩张导管，使球囊扩张以观察球囊是否恰跨在瓣环中心并呈腰凹症，如果球囊已到位，则用稀释造影剂以约3atm扩张球囊，开始显示肺动脉瓣狭窄处腰凹，随球囊腔内压力增加，腰凹随之消失。
|
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球囊扩张时一旦球囊全部扩张，腰凹消失，即可吸瘪球囊。
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通常从开始扩张球囊至吸瘪球囊总时间＜10秒，这样可减少由于右心室流出道血流中断时间过长而引起并发症。
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如果经过球囊扩张术后效果不满意是由于球/瓣比值不足引起，则可调用更大球囊或用双球囊进行扩张。
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PBPV后，如扩张满意，即撤回球囊导管直至股静脉穿刺处，以堵塞穿刺口渗血。
|
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15分钟后，由另一侧股静脉插入右心导管，重点血流动力学检测。
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通常PBPV后ΔP＜25mmHg，则效果良好。
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并作左侧位右心室造影观察球囊扩张后效果及右心室漏斗部是否存在反应性狭窄。
|
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3.球囊扩张术后反应性右心室漏斗部狭窄一部分病人在PBPV后发现瓣口梗阻虽已解除，但由于发生反应性漏斗部狭窄，使右心室压力不能满意下降，但连续曲线示肺动脉与漏斗部压差已解除，表明PBPV术有效。
|
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"entity": "肺动脉",
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10%～20%PBPV术后发生程度不一的漏斗部反应性狭窄。
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术后可给予普萘洛尔（心得安）口服，6个月内均可恢复正常。
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4.并发症PBPV虽为安全而有效的治疗肺动脉瓣狭窄的非开胸方法，但仍有5%左右并发症，总死亡率＜0.5%，多见于新生儿、小婴儿及重症病例。
|
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可能出现的并发症为球囊加压扩张时可引起一过性血压下降、心动过缓及期前收缩，另外血管损伤、右房室瓣损伤致关闭不全及心脏穿孔等偶有发生。
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"entity": "心脏穿孔",
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5.疗效评价与随访PBPV后即时及中长期随访表明，经皮球囊瓣膜成形术治疗肺动脉瓣狭窄效果最好，并发症最少，可达到开胸术疗效以替代外科开胸手术。
|
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术后随访包括临床、心电图、胸片及多普勒（彩色）超声心动图检查以评价疗效。
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（二）经皮球囊主动脉瓣成形术1984年，Lababidi等首先报道应用经皮球囊主动脉瓣成形术（PBAV）成功地治疗先天性或后天性主动脉瓣狭窄，引起广泛的重视。
|
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由于我国发病率较欧美国家为少，另外PBAV在技术上有别于PBPV，且有严重并发症发生，因此国内除上海新华医院一组报告外，尚无系统开展该技术的报道。
|
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根据1982～1986年204例小儿经皮球囊主动脉瓣形成术总结，95.1%获得即期成功。
|
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随后对PBAV方法学上进行较多的研究，包括单球囊与双球囊扩张术及采用长的球囊进行扩张术，以后把球囊瓣膜成形术应用到新生儿病例，甚至进行胎儿主动脉瓣球囊成形术。
|
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目前，正在对PBAV病人进行中、长期随访，以观察其疗效与心功能的改变。
|
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1.经皮球囊主动脉瓣成形术的指征及反指征（1）主要指征：1）血流动力学：跨瓣收缩压差＞50mmHg伴正常心排量；无或仅轻度主动脉瓣反流。
|
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"end_offset": 67,
"label": "sym"
}
] |
2）主动脉瓣狭窄形态学：非瓣膜发育不良型、瓣膜薄且活动良好、无明显瓣环发育不良者为良好指征。
|
[
{
"id": 0,
"entity": "主动脉瓣狭窄",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "瓣膜薄且活动良好",
"start_offset": 21,
"end_offset": 29,
"label": "sym"
},
{
"id": 2,
"entity": "无明显瓣环发育不良",
"start_offset": 30,
"end_offset": 39,
"label": "sym"
}
] |
（2）PBAV禁忌证：中度以上主动脉瓣反流以及心导管术禁忌者。
|
[
{
"id": 0,
"entity": "PBAV",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "主动脉瓣反流",
"start_offset": 15,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "心导管术",
"start_offset": 23,
"end_offset": 27,
"label": "pro"
}
] |
2.方法可通过以下途径进行PBAV术：①逆行股动脉插管法，临床常用；②脐动脉、腋动脉及颈动脉插管法，适用于小婴儿；③经房间隔穿刺法球囊主动脉瓣成形术，该法一般适用于不宜经股动脉穿刺法者。
|
[
{
"id": 0,
"entity": "PBAV",
"start_offset": 13,
"end_offset": 17,
"label": "pro"
},
{
"id": 1,
"entity": "逆行股动脉插管法",
"start_offset": 20,
"end_offset": 28,
"label": "pro"
},
{
"id": 2,
"entity": "脐动脉、腋动脉及颈动脉插管法",
"start_offset": 35,
"end_offset": 49,
"label": "pro"
},
{
"id": 3,
"entity": "经房间隔穿刺法球囊主动脉瓣成形术",
"start_offset": 58,
"end_offset": 74,
"label": "pro"
}
] |
3.疗效评价及随访PBAV的成功率为80%～97%，平均压差下降55%～70%。
|
[
{
"id": 0,
"entity": "PBAV",
"start_offset": 9,
"end_offset": 13,
"label": "pro"
}
] |
与PBPV相比有较多严重并发症，主要为动脉栓塞（特别是婴幼儿）、明显主动脉反流、心律失常、心功能不全、心肌穿孔等。
|
[
{
"id": 0,
"entity": "PBPV",
"start_offset": 1,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "动脉栓塞",
"start_offset": 19,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "明显主动脉反流",
"start_offset": 32,
"end_offset": 39,
"label": "dis"
},
{
"id": 3,
"entity": "心律失常",
"start_offset": 40,
"end_offset": 44,
"label": "dis"
},
{
"id": 4,
"entity": "心功能不全",
"start_offset": 45,
"end_offset": 50,
"label": "dis"
},
{
"id": 5,
"entity": "心肌穿孔",
"start_offset": 51,
"end_offset": 55,
"label": "dis"
}
] |
二、先天性肺囊肿先天性肺囊肿（congenitalpulmonarycysts）是常见的肺发育异常，系胚胎期肺芽发育障碍所致。
|
[
{
"id": 0,
"entity": "先天性肺囊肿",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "先天性肺囊肿",
"start_offset": 8,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "congenitalpulmonarycysts",
"start_offset": 15,
"end_offset": 39,
"label": "dis"
}
] |
病变肺组织可出现单个或多个囊肿，累及一个或多个肺叶。
|
[
{
"id": 0,
"entity": "肺组织",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "肺叶",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
}
] |
当囊肿黏液潴留过多或继发化脓性感染时，囊腔易与支气管相通，常形成单向活瓣样通气，导致肺泡腔内压力不断升高，形成张力性气囊肿，出现严重压迫症状。
|
[
{
"id": 0,
"entity": "囊肿黏液潴留过多",
"start_offset": 1,
"end_offset": 9,
"label": "sym"
},
{
"id": 1,
"entity": "化脓性感染",
"start_offset": 12,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "囊腔",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "支气管相通",
"start_offset": 23,
"end_offset": 28,
"label": "bod"
},
{
"id": 4,
"entity": "单向活瓣样通气",
"start_offset": 32,
"end_offset": 39,
"label": "sym"
},
{
"id": 5,
"entity": "肺泡腔内",
"start_offset": 42,
"end_offset": 46,
"label": "bod"
},
{
"id": 6,
"entity": "张力性气囊肿",
"start_offset": 55,
"end_offset": 61,
"label": "sym"
}
] |
囊肿小者可长期不出现任何症状，直至胸部X线检查时被发现。
|
[
{
"id": 0,
"entity": "胸部X线检查",
"start_offset": 17,
"end_offset": 23,
"label": "ite"
}
] |
1岁以内患儿多因呼吸困难就诊，而肺部感染是年长儿的主要临床特征。
|
[
{
"id": 0,
"entity": "呼吸困难",
"start_offset": 8,
"end_offset": 12,
"label": "sym"
},
{
"id": 1,
"entity": "肺部感染",
"start_offset": 16,
"end_offset": 20,
"label": "dis"
}
] |
单发闭合性肺囊肿在X线下显示一个圆形或类圆形阴影，密度均匀，边缘清晰，周围一般无明显浸润病灶。
|
[
{
"id": 0,
"entity": "单发闭合性肺囊肿",
"start_offset": 0,
"end_offset": 8,
"label": "dis"
}
] |
多发性肺囊肿在X线平片上显示多数大小不等的圆形或类圆形阴影，阴影内可出现液平面，周围可伴炎性浸润。
|
[
{
"id": 0,
"entity": "多发性肺囊肿",
"start_offset": 0,
"end_offset": 6,
"label": "dis"
}
] |
巨大的张力性气囊肿，有时易与张力性气胸相混淆，但张力性气囊肿在肺野的边缘如肺尖或肋膈角处仍可看到肺组织，而张力性气胸患侧肺组织被压到肺门区，肺野边缘部分看不到肺组织，且往往伴有胸膜反应。
|
[
{
"id": 0,
"entity": "巨大的张力性气囊肿",
"start_offset": 0,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "张力性气胸",
"start_offset": 14,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "张力性气囊肿",
"start_offset": 24,
"end_offset": 30,
"label": "dis"
},
{
"id": 3,
"entity": "肺野的边缘",
"start_offset": 31,
"end_offset": 36,
"label": "bod"
},
{
"id": 4,
"entity": "肺尖",
"start_offset": 37,
"end_offset": 39,
"label": "bod"
},
{
"id": 5,
"entity": "肋膈角",
"start_offset": 40,
"end_offset": 43,
"label": "bod"
},
{
"id": 6,
"entity": "肺组织",
"start_offset": 48,
"end_offset": 51,
"label": "bod"
},
{
"id": 7,
"entity": "张力性气胸",
"start_offset": 53,
"end_offset": 58,
"label": "dis"
},
{
"id": 8,
"entity": "肺组织",
"start_offset": 60,
"end_offset": 63,
"label": "bod"
},
{
"id": 9,
"entity": "肺门区",
"start_offset": 66,
"end_offset": 69,
"label": "bod"
},
{
"id": 10,
"entity": "肺野边缘部分",
"start_offset": 70,
"end_offset": 76,
"label": "bod"
},
{
"id": 11,
"entity": "肺组织",
"start_offset": 79,
"end_offset": 82,
"label": "bod"
},
{
"id": 12,
"entity": "胸膜反应",
"start_offset": 88,
"end_offset": 92,
"label": "sym"
}
] |
对已出现张力性病变而引起严重压迫症状者，可先行胸腔减压，然后再手术。
|
[
{
"id": 0,
"entity": "张力性病变而引起严重压迫症状者",
"start_offset": 4,
"end_offset": 19,
"label": "dis"
},
{
"id": 1,
"entity": "胸腔减压",
"start_offset": 23,
"end_offset": 27,
"label": "pro"
},
{
"id": 2,
"entity": "手术",
"start_offset": 31,
"end_offset": 33,
"label": "pro"
}
] |
三、其他病原学检查支原体病原学检查与病毒病原学检查相似。
|
[
{
"id": 0,
"entity": "病原学检查",
"start_offset": 4,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "支原体病原学检查",
"start_offset": 9,
"end_offset": 17,
"label": "pro"
},
{
"id": 2,
"entity": "病毒病原学检查",
"start_offset": 18,
"end_offset": 25,
"label": "pro"
}
] |
呼吸道分泌物支原体分离和双份血清抗体测定可作为支原体感染诊断的金标准。
|
[
{
"id": 0,
"entity": "呼吸道分泌物支原体分离",
"start_offset": 0,
"end_offset": 11,
"label": "pro"
},
{
"id": 1,
"entity": "双份血清抗体测定",
"start_offset": 12,
"end_offset": 20,
"label": "pro"
},
{
"id": 2,
"entity": "支原体感染",
"start_offset": 23,
"end_offset": 28,
"label": "dis"
}
] |
临床上常应用咽拭子支原体DNA检测、支原体特异抗原测定及血清特异抗体IgM测定进行快速诊断，指导临床用药。
|
[
{
"id": 0,
"entity": "咽拭子支原体DNA检测",
"start_offset": 6,
"end_offset": 17,
"label": "pro"
},
{
"id": 1,
"entity": "支原体特异抗原测定",
"start_offset": 18,
"end_offset": 27,
"label": "pro"
},
{
"id": 2,
"entity": "血清特异抗体IgM测定",
"start_offset": 28,
"end_offset": 39,
"label": "pro"
}
] |
与病毒不同的是，由于支原体可在细胞外单独存活，无需借助宿主细胞的细胞器，因而对呼吸道标本支原体的检测只需咽拭子，而不需要脱落细胞。
|
[
{
"id": 0,
"entity": "病毒",
"start_offset": 1,
"end_offset": 3,
"label": "mic"
},
{
"id": 1,
"entity": "支原体",
"start_offset": 10,
"end_offset": 13,
"label": "mic"
},
{
"id": 2,
"entity": "呼吸道",
"start_offset": 39,
"end_offset": 42,
"label": "bod"
},
{
"id": 3,
"entity": "支原体",
"start_offset": 44,
"end_offset": 47,
"label": "mic"
},
{
"id": 4,
"entity": "咽拭子",
"start_offset": 52,
"end_offset": 55,
"label": "equ"
}
] |
冷凝集试验是较早用于肺炎支原体感染早期诊断的一种方法，其原理是检查在4℃时有无凝集人类红细胞的IgM自身抗体。
|
[
{
"id": 0,
"entity": "冷凝集试验",
"start_offset": 0,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "肺炎支原体感染",
"start_offset": 10,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "红细胞",
"start_offset": 43,
"end_offset": 46,
"label": "bod"
},
{
"id": 3,
"entity": "IgM",
"start_offset": 47,
"end_offset": 50,
"label": "bod"
}
] |
但该方法并非特异性，当腺病毒等感染时亦可出现阳性，因而只能作为辅助诊断。
|
[
{
"id": 0,
"entity": "腺病毒",
"start_offset": 11,
"end_offset": 14,
"label": "mic"
},
{
"id": 1,
"entity": "阳性",
"start_offset": 22,
"end_offset": 24,
"label": "sym"
}
] |
对疑有真菌感染者，可采集痰标本、气管分泌物或血液等送检。
|
[
{
"id": 0,
"entity": "真菌感染",
"start_offset": 3,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "痰",
"start_offset": 12,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "气管分泌物",
"start_offset": 16,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "血液",
"start_offset": 22,
"end_offset": 24,
"label": "bod"
}
] |
直接涂片检查若发现真菌菌丝或孢子，可初步判定为真菌感染。
|
[
{
"id": 0,
"entity": "直接涂片检查",
"start_offset": 0,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "真菌菌丝或孢子",
"start_offset": 9,
"end_offset": 16,
"label": "mic"
},
{
"id": 2,
"entity": "真菌感染",
"start_offset": 23,
"end_offset": 27,
"label": "dis"
}
] |
培养诊断价值较高，但多次培养阳性才有意义。
|
[
{
"id": 0,
"entity": "阳性",
"start_offset": 14,
"end_offset": 16,
"label": "sym"
}
] |
亦可采用血清学、免疫学或分子生物学技术，如血清冷凝集试验、补体结合试验、免疫酶技术、免疫荧光技术、PCR技术等进行真菌病原的快速诊断。
|
[
{
"id": 0,
"entity": "血清冷凝集试验",
"start_offset": 21,
"end_offset": 28,
"label": "pro"
},
{
"id": 1,
"entity": "补体结合试验",
"start_offset": 29,
"end_offset": 35,
"label": "pro"
},
{
"id": 2,
"entity": "免疫酶技术",
"start_offset": 36,
"end_offset": 41,
"label": "pro"
},
{
"id": 3,
"entity": "免疫荧光技术",
"start_offset": 42,
"end_offset": 48,
"label": "pro"
},
{
"id": 4,
"entity": "PCR技术",
"start_offset": 49,
"end_offset": 54,
"label": "pro"
},
{
"id": 5,
"entity": "真菌",
"start_offset": 57,
"end_offset": 59,
"label": "mic"
}
] |
第六节溶血性贫血一、总论溶血性贫血（hemolyticanemia）是由多种病因引起红细胞寿命的缩短和过早地破坏，且红细胞的破坏超过了骨髓生血功能的代偿能力而发生循环中红细胞数和血红蛋白含量减少贫血。
|
[
{
"id": 0,
"entity": "溶血性贫血",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "溶血性贫血",
"start_offset": 12,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "hemolyticanemia",
"start_offset": 18,
"end_offset": 33,
"label": "dis"
},
{
"id": 3,
"entity": "红细胞",
"start_offset": 42,
"end_offset": 45,
"label": "bod"
},
{
"id": 4,
"entity": "红细胞",
"start_offset": 58,
"end_offset": 61,
"label": "bod"
},
{
"id": 5,
"entity": "骨髓",
"start_offset": 67,
"end_offset": 69,
"label": "bod"
},
{
"id": 6,
"entity": "红细胞数",
"start_offset": 84,
"end_offset": 88,
"label": "ite"
},
{
"id": 7,
"entity": "血红蛋白含量",
"start_offset": 89,
"end_offset": 95,
"label": "ite"
},
{
"id": 8,
"entity": "循环中红细胞数和血红蛋白含量减少",
"start_offset": 81,
"end_offset": 97,
"label": "sym"
},
{
"id": 9,
"entity": "贫血",
"start_offset": 97,
"end_offset": 99,
"label": "dis"
}
] |
正常红细胞的寿命为110～120日左右。
|
[
{
"id": 0,
"entity": "红细胞",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
}
] |
正常情况下，每日约有1%的衰老红细胞在脾脏中被吞噬和破坏，由新生的红细胞补充代替之，从而维持红细胞数量的恒定以发挥正常的生理功能。
|
[
{
"id": 0,
"entity": "红细胞",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 1,
"entity": "脾脏",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
},
{
"id": 2,
"entity": "红细胞",
"start_offset": 33,
"end_offset": 36,
"label": "bod"
},
{
"id": 3,
"entity": "红细胞",
"start_offset": 46,
"end_offset": 49,
"label": "bod"
}
] |
正常小儿骨髓造血潜能很大，一般可增加到正常的6～8倍。
|
[
{
"id": 0,
"entity": "骨髓",
"start_offset": 4,
"end_offset": 6,
"label": "bod"
}
] |
【分类】根据溶血因素存在的部位不同，可将溶血性贫血分为红细胞内和红细胞外两大类。
|
[
{
"id": 0,
"entity": "溶血",
"start_offset": 6,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "溶血性贫血",
"start_offset": 20,
"end_offset": 25,
"label": "dis"
},
{
"id": 2,
"entity": "红细胞",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "红细胞",
"start_offset": 32,
"end_offset": 35,
"label": "bod"
}
] |
（一）红细胞内因素1.红细胞膜的缺陷（1）遗传性球形细胞增多症。
|
[
{
"id": 0,
"entity": "红细胞",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "红细胞膜",
"start_offset": 11,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "遗传性球形细胞增多症",
"start_offset": 21,
"end_offset": 31,
"label": "dis"
}
] |
（2）遗传性椭圆形细胞增多症。
|
[
{
"id": 0,
"entity": "遗传性椭圆形细胞增多症",
"start_offset": 3,
"end_offset": 14,
"label": "dis"
}
] |
（5）遗传性棘细胞增生症。
|
[
{
"id": 0,
"entity": "遗传性棘细胞增生症",
"start_offset": 3,
"end_offset": 12,
"label": "dis"
}
] |
（6）阵发性睡眠性血红蛋白尿。
|
[
{
"id": 0,
"entity": "阵发性睡眠性血红蛋白尿",
"start_offset": 3,
"end_offset": 14,
"label": "dis"
}
] |
2.红细胞酶的缺陷（1）红细胞糖分解酶缺乏：1）丙酮酸激酶缺乏。
|
[
{
"id": 0,
"entity": "红细胞酶的缺陷",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "红细胞糖分解酶缺乏",
"start_offset": 12,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "丙酮酸激酶缺乏",
"start_offset": 24,
"end_offset": 31,
"label": "dis"
}
] |
3）磷酸果糖激酶缺乏。
|
[
{
"id": 0,
"entity": "磷酸果糖激酶缺乏",
"start_offset": 2,
"end_offset": 10,
"label": "dis"
}
] |
4）丙糖磷酸异构酶缺乏。
|
[
{
"id": 0,
"entity": "丙糖磷酸异构酶缺乏",
"start_offset": 2,
"end_offset": 11,
"label": "dis"
}
] |
5）己糖激酶缺乏。
|
[
{
"id": 0,
"entity": "己糖激酶缺乏",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
}
] |
6）磷酸甘油酸盐激酶缺乏。
|
[
{
"id": 0,
"entity": "磷酸甘油酸盐激酶缺乏",
"start_offset": 2,
"end_offset": 12,
"label": "dis"
}
] |
7）醛缩酶缺乏。
|
[
{
"id": 0,
"entity": "醛缩酶缺乏",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
}
] |
8）二磷酸甘油酸盐变位酶缺乏。
|
[
{
"id": 0,
"entity": "二磷酸甘油酸盐变位酶缺乏",
"start_offset": 2,
"end_offset": 14,
"label": "dis"
}
] |
（2）红细胞核苷酸代谢异常：1）嘧啶5-核苷酸酶缺乏。
|
[
{
"id": 0,
"entity": "红细胞核苷酸代谢异常",
"start_offset": 3,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "嘧啶5-核苷酸酶缺乏",
"start_offset": 16,
"end_offset": 26,
"label": "dis"
}
] |
2）腺苷脱氨酶过多。
|
[
{
"id": 0,
"entity": "腺苷脱氨酶过多",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
}
] |
4）腺苷酸激酶缺乏。
|
[
{
"id": 0,
"entity": "腺苷酸激酶缺乏",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
}
] |
（3）戊糖磷酸盐通路及谷胱苷肽代谢有关的酶缺乏：1）葡萄糖-6-磷酸脱氢酶（G-6-PD）缺乏。
|
[
{
"id": 0,
"entity": "戊糖磷酸盐通路及谷胱苷肽代谢有关的酶缺乏",
"start_offset": 3,
"end_offset": 23,
"label": "dis"
},
{
"id": 1,
"entity": "葡萄糖-6-磷酸脱氢酶（G-6-PD）缺乏",
"start_offset": 26,
"end_offset": 47,
"label": "dis"
}
] |
2）谷氨酰半胱氨酸合成酶缺乏。
|
[
{
"id": 0,
"entity": "谷氨酰半胱氨酸合成酶缺乏",
"start_offset": 2,
"end_offset": 14,
"label": "dis"
}
] |
3）谷胱甘肽合成酶缺乏。
|
[
{
"id": 0,
"entity": "谷胱甘肽合成酶缺乏",
"start_offset": 2,
"end_offset": 11,
"label": "dis"
}
] |
3.血红蛋白异常（1）珠蛋白生成障碍性贫血。
|
[
{
"id": 0,
"entity": "血红蛋白",
"start_offset": 2,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "珠蛋白生成障碍性贫血",
"start_offset": 11,
"end_offset": 21,
"label": "dis"
}
] |
（4）不稳定血红蛋白病。
|
[
{
"id": 0,
"entity": "不稳定血红蛋白病",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
}
] |
（5）其他同型合子血红蛋白病（CC，DI，EE）。
|
[
{
"id": 0,
"entity": "其他同型合子血红蛋白病",
"start_offset": 3,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "CC",
"start_offset": 15,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "DI",
"start_offset": 18,
"end_offset": 20,
"label": "dis"
},
{
"id": 3,
"entity": "EE",
"start_offset": 21,
"end_offset": 23,
"label": "dis"
}
] |
（6）双杂合子紊乱（HbSC，镰状细胞珠蛋白生成障碍性贫血）。
|
[
{
"id": 0,
"entity": "双杂合子紊乱",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "HbSC",
"start_offset": 10,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "镰状细胞珠蛋白生成障碍性贫血",
"start_offset": 15,
"end_offset": 29,
"label": "dis"
}
] |
（二）红细胞外因素1.免疫性溶血性贫血（1）错输血型不匹配血。
|
[
{
"id": 0,
"entity": "红细胞",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "免疫性溶血性贫血",
"start_offset": 11,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "血",
"start_offset": 29,
"end_offset": 30,
"label": "bod"
}
] |
（3）自身免疫性溶血性贫血：1）温抗体所致的自身免疫性溶血性贫血。
|
[
{
"id": 0,
"entity": "自身免疫性溶血性贫血",
"start_offset": 3,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "抗体",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "自身免疫性溶血性贫血",
"start_offset": 22,
"end_offset": 32,
"label": "dis"
}
] |
2）冷抗体所致的自身免疫性溶血性贫血。
|
[
{
"id": 0,
"entity": "抗体",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "自身免疫性溶血性贫血",
"start_offset": 8,
"end_offset": 18,
"label": "dis"
}
] |
2.创伤性及微血管性溶血性贫血（1）人工瓣膜及其他心脏异常。
|
[
{
"id": 0,
"entity": "创伤性及微血管性溶血性贫血",
"start_offset": 2,
"end_offset": 15,
"label": "dis"
},
{
"id": 1,
"entity": "人工瓣膜",
"start_offset": 18,
"end_offset": 22,
"label": "bod"
},
{
"id": 2,
"entity": "心脏",
"start_offset": 25,
"end_offset": 27,
"label": "bod"
}
] |
（5）血栓性血小板减少性紫癜。
|
[
{
"id": 0,
"entity": "血栓性血小板减少性紫癜",
"start_offset": 3,
"end_offset": 14,
"label": "dis"
}
] |
（6）溶血尿毒综合征。
|
[
{
"id": 0,
"entity": "溶血尿毒综合征",
"start_offset": 3,
"end_offset": 10,
"label": "dis"
}
] |
3.脾功能亢进4.血浆因素（1）肝脏疾病：如血浆胆固醇、磷脂过高所致脂肪肝、肝硬化等引起靴刺细胞（spurcell）贫血。
|
[
{
"id": 0,
"entity": "脾功能亢进",
"start_offset": 2,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "血浆",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "血浆胆固醇",
"start_offset": 22,
"end_offset": 27,
"label": "dis"
},
{
"id": 3,
"entity": "磷脂",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
},
{
"id": 4,
"entity": "脂肪肝",
"start_offset": 34,
"end_offset": 37,
"label": "dis"
},
{
"id": 5,
"entity": "肝硬化",
"start_offset": 38,
"end_offset": 41,
"label": "dis"
},
{
"id": 6,
"entity": "靴刺细胞（spurcell）贫血",
"start_offset": 44,
"end_offset": 60,
"label": "dis"
}
] |
5.感染性（1）原虫：疟原虫、毒浆原虫、黑热病原虫等。
|
[
{
"id": 0,
"entity": "原虫",
"start_offset": 8,
"end_offset": 10,
"label": "mic"
},
{
"id": 1,
"entity": "疟原虫",
"start_offset": 11,
"end_offset": 14,
"label": "mic"
},
{
"id": 2,
"entity": "毒浆原虫",
"start_offset": 15,
"end_offset": 19,
"label": "mic"
},
{
"id": 3,
"entity": "黑热病原虫",
"start_offset": 20,
"end_offset": 25,
"label": "mic"
}
] |
（2）细菌：梭状菌属感染（如梭状芽胞杆菌）、霍乱、伤寒等。
|
[
{
"id": 0,
"entity": "细菌",
"start_offset": 3,
"end_offset": 5,
"label": "mic"
},
{
"id": 1,
"entity": "梭状菌属感染",
"start_offset": 6,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "梭状芽胞杆菌",
"start_offset": 14,
"end_offset": 20,
"label": "mic"
},
{
"id": 3,
"entity": "霍乱",
"start_offset": 22,
"end_offset": 24,
"label": "dis"
},
{
"id": 4,
"entity": "伤寒",
"start_offset": 25,
"end_offset": 27,
"label": "dis"
}
] |
6.肝豆状核变性（Wilson病）7.化学品、药物及蛇毒（1）氧化性药物及化学制剂。
|
[
{
"id": 0,
"entity": "肝豆状核变性",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "Wilson病",
"start_offset": 9,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "氧化性药物",
"start_offset": 31,
"end_offset": 36,
"label": "dru"
}
] |
（2）非氧化性药物。
|
[
{
"id": 0,
"entity": "非氧化性药物",
"start_offset": 3,
"end_offset": 9,
"label": "dru"
}
] |
（3）新生儿维生素E缺乏。
|
[
{
"id": 0,
"entity": "新生儿维生素E缺乏",
"start_offset": 3,
"end_offset": 12,
"label": "dis"
}
] |
（4）并存于尿毒症、血液透析。
|
[
{
"id": 0,
"entity": "尿毒症",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "血液透析",
"start_offset": 10,
"end_offset": 14,
"label": "pro"
}
] |
【诊断】溶血性贫血的诊断主要依靠临床表现和实验室检查。
|
[
{
"id": 0,
"entity": "溶血性贫血",
"start_offset": 4,
"end_offset": 9,
"label": "dis"
}
] |
当患儿有贫血伴有网织红细胞增高时，应考虑到溶血性贫血存在的可能性，应选择下列试验寻找红细胞破坏增加的直接和间接证据。
|
[
{
"id": 0,
"entity": "贫血",
"start_offset": 4,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "网织红细胞",
"start_offset": 8,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "溶血性贫血",
"start_offset": 21,
"end_offset": 26,
"label": "dis"
},
{
"id": 3,
"entity": "红细胞",
"start_offset": 42,
"end_offset": 45,
"label": "bod"
}
] |
（一）红细胞破坏增加的直接证据1.Na2</sub>51</sup>CrO4</sub>标记红细胞测定红细胞寿命，其半寿命明显缩短。
|
[
{
"id": 0,
"entity": "红细胞",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "红细胞",
"start_offset": 46,
"end_offset": 49,
"label": "bod"
},
{
"id": 2,
"entity": "红细胞",
"start_offset": 51,
"end_offset": 54,
"label": "bod"
}
] |
2.血浆游离血红蛋白增多。
|
[
{
"id": 0,
"entity": "血浆",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "血红蛋白",
"start_offset": 6,
"end_offset": 10,
"label": "bod"
}
] |
5.出现血红蛋白尿时，联苯胺试验阳性。
|
[
{
"id": 0,
"entity": "血红蛋白尿",
"start_offset": 4,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "联苯胺试验",
"start_offset": 11,
"end_offset": 16,
"label": "pro"
}
] |
（二）红细胞破坏增加的间接证据1.网织红细胞明显增加。
|
[
{
"id": 0,
"entity": "红细胞",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "网织红细胞",
"start_offset": 17,
"end_offset": 22,
"label": "bod"
}
] |
3.骨髓红系增生活跃，粒∶红比例降低甚至倒置。
|
[
{
"id": 0,
"entity": "骨髓",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
}
] |
4.由于红细胞生成代偿性增快，红细胞大小不一，形状不等，红细胞带有核或核残余。
|
[
{
"id": 0,
"entity": "红细胞",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "生成代偿性增快",
"start_offset": 7,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "红细胞",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "大小不一",
"start_offset": 18,
"end_offset": 22,
"label": "sym"
},
{
"id": 4,
"entity": "形状不等",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
},
{
"id": 5,
"entity": "红细胞",
"start_offset": 28,
"end_offset": 31,
"label": "bod"
},
{
"id": 6,
"entity": "带有核或核残余",
"start_offset": 31,
"end_offset": 38,
"label": "sym"
}
] |
一旦红细胞破坏过多存在，溶血性贫血的诊断成立，然后，再根据抗人球蛋白（Coombs）直接及间接试验的阳性与否区分免疫性（Coombs试验阳性，血型不合溶血除外）与非免疫性（Coombs试验阴性）；非免疫性溶血性贫血可根据红细胞形态、脆性试验、葡萄糖孵育脆性试验、高铁血红蛋白还原试验、酸溶血试验（Ham）、红细胞酶谱分析、红细胞CD55/CD59流式细胞仪分析、珠蛋白小体（Hein’sbody）、血红蛋白电泳等试验区分各种原因引起的溶血性贫血。
|
[
{
"id": 0,
"entity": "红细胞",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "溶血性贫血",
"start_offset": 12,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "抗人球蛋白（Coombs）直接及间接试验",
"start_offset": 29,
"end_offset": 49,
"label": "pro"
},
{
"id": 3,
"entity": "非免疫性溶血性贫血",
"start_offset": 98,
"end_offset": 107,
"label": "dis"
},
{
"id": 4,
"entity": "红细胞",
"start_offset": 110,
"end_offset": 113,
"label": "bod"
},
{
"id": 5,
"entity": "脆性试验",
"start_offset": 116,
"end_offset": 120,
"label": "pro"
},
{
"id": 6,
"entity": "葡萄糖孵育脆性试验",
"start_offset": 121,
"end_offset": 130,
"label": "pro"
},
{
"id": 7,
"entity": "酸溶血试验",
"start_offset": 142,
"end_offset": 147,
"label": "pro"
},
{
"id": 8,
"entity": "Ham",
"start_offset": 148,
"end_offset": 151,
"label": "pro"
},
{
"id": 9,
"entity": "红细胞酶谱分析",
"start_offset": 153,
"end_offset": 160,
"label": "pro"
},
{
"id": 10,
"entity": "红细胞CD55/CD59流式细胞仪分析",
"start_offset": 161,
"end_offset": 180,
"label": "pro"
},
{
"id": 11,
"entity": "珠蛋白小体",
"start_offset": 181,
"end_offset": 186,
"label": "pro"
},
{
"id": 12,
"entity": "Hein’sbody",
"start_offset": 187,
"end_offset": 197,
"label": "pro"
},
{
"id": 13,
"entity": "血红蛋白电泳",
"start_offset": 199,
"end_offset": 205,
"label": "pro"
},
{
"id": 14,
"entity": "溶血性贫血",
"start_offset": 217,
"end_offset": 222,
"label": "dis"
}
] |
【治疗】溶血性贫血种类多种多样，因此，其治疗应根据病因、类型拟定治疗方案。
|
[
{
"id": 0,
"entity": "溶血性贫血",
"start_offset": 4,
"end_offset": 9,
"label": "dis"
}
] |
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