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＜6个月婴儿，因生理性贫血等因素，血红蛋白变化较大，目前尚无统一标准。 | [
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一、贫血的分级小儿贫血分级见表10-10。 | [
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表10-1小儿贫血的分级 | [
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第十三章小儿呼吸机治疗和吸入一氧化氮疗法第一节常规机械通气一、呼吸机和通气模式常规机械通气（conventionalmechanicalventilation，CMV）治疗用呼吸机一般为成人/小儿兼用型，按对气流控制方式具备以下基本模式：容量控制（volumecontrol，VCV，也称定容通气）；压力控制（pressurecontrol，PCV，也称定压通气）。 | [
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辅助通气指患儿主要通过自主呼吸以适应、或以自主呼吸带动呼吸机供气，从而减轻呼吸做功。 | [
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在辅助通气时又分为持续气道正压通气（continuouspositiveairwaypressure，CPAP）和压力支持通气（pressuresupportventilation，PSV）两种基本功能，某些呼吸机也用患者触发的通气（patienttriggeredventilation，PTV）表示，新型呼吸机还有容量支持通气（volumesupportventilation，VSV）功能。 | [
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缺点为气道峰压不控制，随通气量、气道阻力和肺组织顺应性而变化。 | [
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适用于没有或较轻外周肺损伤、手术、中枢性病变、或神经肌肉病变时的通气。 | [
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目前新型呼吸机均设有潮气量目标化的气道压力自动调节的定容通气模式，也可以安全地应用于新生儿及小儿急性外周性肺损伤和呼吸衰竭。 | [
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每次供气压力限定时，通气量则随供气流量、气道阻力和肺组织顺应性而变化。 | [
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新型呼吸机均设置吸气上升时间控制，或平台压控制旋钮来控制气道压的形成状态。 | [
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适用于外周肺有病变损伤时的通气。 | [
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新型呼吸机具备供气和呼出气潮气量的精确监测，可以提高通气控制的稳定性。 | [
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（四）同步间歇指令通气和间歇指令通气SIMV按照患儿自主呼吸的要求，提供预设的通气，避免与患儿自己的呼吸相冲突、对抗。 | [
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作用原理：在一次呼吸周期的呼气相后期（或呼气相全程），一旦患儿出现自主呼吸，气道压力和气流产生变化，呼吸机在极短时间内感知并发出指令，呼吸机立即提供通气气流，完成一次通气。 | [
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IMV为非同步间歇指令通气，其主要缺点为机械通气时出现患者与呼吸机对抗，因而鼓励使用SIMV和A/C通气模式的同步触发通气功能。 | [
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在选择了SIMV（或A/C）模式时，应将触发控制功能打开，通过调节触发灵敏度来控制通气频率，以此获得目标分钟通气量和理想通气效果，SIMV应该与PSV联合应用（见后，呼吸机调节部分）。 | [
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（五）压力调节容量控制通气新型呼吸机（如瑞典西门子300型、德国德尔格Babylog8000型）设有微电脑控制的“压力调节容量控制”（pressureregulatedvolumecontrol，PRVC），或称为“容量保证”（volumeguaranty）功能，将定容和定压的优点结合在一起，避免了各自的缺点，成为目前儿科患儿机械通气治疗的主要模式之一。 | [
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（六）压力支持通气呼吸机在一次通气的呼气相设有一特殊供气方式。 | [
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在患儿自主呼吸发动时，经机内设置的触发反应机制将阀门打开，气流立即经气道进入肺内，在压力达到预设水平，且气流量衰减到最大峰流量的一定水平（如下降到峰流量的5%～25%），阀门关闭，供气气流中断，压力迅速回复到基线压水平。 | [
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这种通气的优点在于呼吸机随患者的需要而供气，可以提高并保证自主呼吸时的通气潮气量和每分通气量，而患者的吸气做功可以大大降低。 | [
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此外，在保持每分通气量相似的条件下，PSV时的平均气道压（MAP）可以较A/C或IMV时降低30%～50%，可以降低气压伤危险性。 | [
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（七）持续气道正压通气CPAP是在整个呼吸周期，经鼻塞、面罩或气道插管将连续气流加于小儿鼻腔或气道，并产生高于大气压的鼻腔/气道压力，使小儿在吸气相得到较高的供气气压和流量，降低吸气做功；同时在呼气相，得到高于外界大气压的压力，避免肺泡塌陷。 | [
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新型专用于婴儿的CPAP气流供气系统采用特制的CPAP压力发生装置，使通气时压力恒定，效率大大提高，并可将供氧浓度调节在0.21～0.40，降低了高氧导致新生儿肺损伤的危险。 | [
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参考文献1.ZulianF，WooP，AthreyaBH，etal.ThePediatricRheumatologyEuropeanSociety/AmericanCollegeofRheumatology/EuropeanLeagueagainstRheumatismprovisionalclassificationcriteriaforjuvenilesystemicsclerosis［J］.ArthritisRheum，2007，57（2）：203-2032.OchsHD，SmithCIE，PuckJM.PrimaryImmunodeficiencyDiseases：AMolecularandApproach.OxfordUniversityPress.NewYork，Oxford，19993.韩玉昆等.儿科实用诊断治疗学.合肥：安徽科技出版社，19994.杨锡强等.儿童免疫学.北京：人民卫生出版社，20015.StiehmER.ImmunologicDisorderinInfantsandChildren.3<sup>rd</sup>ed.W.B.SaundersCo，1996 | [
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第五节先天性卵巢发育不全综合征【概述】本病由Turner于1938年首先报道，故称为Turner综合征（TS）。 | [
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该病也是人类唯一能生存的单体综合征。 | [
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TS的主要临床特征是身材明显矮小、青春期不发育、原发性闭经、颈蹼及肘外翻等，其性腺呈纤维条索状。 | [
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【病理生理和发病机制】Turner综合征是由于细胞内X染色体缺失或结构发生改变所致。 | [
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有关X单体染色体的遗传基础还不清楚，目前已排除遗传印迹的可能性，认为可能的机制为：①亲代生殖细胞的减数分裂发生不分离；②合子卵裂中姐妹染色单体不分离；③在有丝分裂过程中X染色体的部分丢失。 | [
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由于在胚胎发育早期，女性两条X染色体之一就已失活而不转录，因此仅有一条X染色体在人体的发育中起积极作用。 | [
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由此看来，Turner综合征表型并非由X单体所致，可能涉及某些至今尚未明确的Turner综合征相关基因的缺陷。 | [
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在胚胎发育早期及X染色体失活之前，涉及人体细胞发育的X连锁基因可能即已表达，并呈双倍剂量的基因表达。 | [
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失活X染色体上的X连锁基因似乎并未完全失活，可能存在不完全失活区域，由于该区域的基因表达是造成Turner综合征临床表型的关键因素，因此提示Turner综合征相关基因很可能位于该区域内。 | [
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有关Y染色体的研究结果表明，由于部分Turner综合征患者伴有Y染色体着丝粒衍生物，因此人们认为性别决定基因和Turner综合征相关基因在遗传学上是两个完全独立的基因控制位点。 | [
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}
] |
研究还发现，部分患者中存在涉及Turner综合征相关的Y染色体基因（RPS4Y）与X染色体基因（RPS4X）连锁，并能编码一种40S的蛋白异构体，提示该连锁基因可能是Turner综合征的候选基因。 | [
{
"id": 0,
"entity": "Turner综合征",
"start_offset": 15,
"end_offset": 24,
"label": "dis"
},
{
"id": 1,
"entity": "Y染色体基因",
"start_offset": 27,
"end_offset": 33,
"label": "bod"
},
{
"id": 2,
"entity": "RPS4Y",
"start_offset": 34,
"end_offset": 39,
"label": "bod"
},
{
"id": 3,
"entity": "X染色体基因",
"start_offset": 41,
"end_offset": 47,
"label": "bod"
},
{
"id": 4,
"entity": "RPS4X",
"start_offset": 48,
"end_offset": 53,
"label": "bod"
},
{
"id": 5,
"entity": "40S的蛋白异构体",
"start_offset": 63,
"end_offset": 72,
"label": "bod"
},
{
"id": 6,
"entity": "Turner综合征",
"start_offset": 83,
"end_offset": 92,
"label": "dis"
},
{
"id": 7,
"entity": "候选基因",
"start_offset": 93,
"end_offset": 97,
"label": "bod"
}
] |
目前有关TS表型特征的相关分子遗传学研究显示，身材矮小相关基因（SHOX）和FOXC2基因分别与Turner骨骼异常及淋巴管膨胀及淋巴水肿有关。 | [
{
"id": 0,
"entity": "TS",
"start_offset": 4,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "FOXC2基因",
"start_offset": 38,
"end_offset": 45,
"label": "bod"
},
{
"id": 2,
"entity": "Turner骨骼异常",
"start_offset": 48,
"end_offset": 58,
"label": "dis"
},
{
"id": 3,
"entity": "淋巴管膨胀",
"start_offset": 59,
"end_offset": 64,
"label": "dis"
},
{
"id": 4,
"entity": "淋巴水肿",
"start_offset": 65,
"end_offset": 69,
"label": "dis"
}
] |
SHOX基因定位于Xp22的伪长染色体区1（pseudoautosomalregion1，PAR1），包含7个外显子，分别编码292和225个氨基酸残基组成的两种转录蛋白（SHOXa和SHOXb），目前推测SHOX基因缺陷所致相关蛋白单倍剂量表达不足是与TS患者矮身材及骨骼畸形有关，但对TS的其他表型无重要作用。 | [
{
"id": 0,
"entity": "Xp22的伪长染色体区1",
"start_offset": 9,
"end_offset": 21,
"label": "bod"
},
{
"id": 1,
"entity": "PAR1",
"start_offset": 45,
"end_offset": 49,
"label": "bod"
},
{
"id": 2,
"entity": "外显子",
"start_offset": 55,
"end_offset": 58,
"label": "bod"
},
{
"id": 3,
"entity": "氨基酸残基",
"start_offset": 71,
"end_offset": 76,
"label": "dis"
},
{
"id": 4,
"entity": "SHOXa",
"start_offset": 86,
"end_offset": 91,
"label": "bod"
},
{
"id": 5,
"entity": "SHOXb",
"start_offset": 92,
"end_offset": 97,
"label": "bod"
},
{
"id": 6,
"entity": "SHOX基因缺陷",
"start_offset": 103,
"end_offset": 111,
"label": "dis"
},
{
"id": 7,
"entity": "蛋白",
"start_offset": 115,
"end_offset": 117,
"label": "bod"
},
{
"id": 8,
"entity": "TS",
"start_offset": 127,
"end_offset": 129,
"label": "dis"
},
{
"id": 9,
"entity": "矮身材",
"start_offset": 131,
"end_offset": 134,
"label": "sym"
},
{
"id": 10,
"entity": "骨骼畸形",
"start_offset": 135,
"end_offset": 139,
"label": "sym"
},
{
"id": 11,
"entity": "TS",
"start_offset": 144,
"end_offset": 146,
"label": "dis"
}
] |
FOXC2基因定位于16号染色体长臂（16q），仅1个外显子，mRNA1.5kb，相关转录蛋白涉足胎儿相关发育旁路途径。 | [
{
"id": 0,
"entity": "FOXC2基因",
"start_offset": 0,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "16号染色体长臂",
"start_offset": 10,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "16q",
"start_offset": 19,
"end_offset": 22,
"label": "bod"
},
{
"id": 3,
"entity": "外显子",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 4,
"entity": "mRNA1.5kb",
"start_offset": 31,
"end_offset": 40,
"label": "bod"
},
{
"id": 5,
"entity": "转录蛋白",
"start_offset": 43,
"end_offset": 47,
"label": "bod"
}
] |
该基因缺陷可致患者淋巴管发育不全、淋巴阻塞和继发性淋巴水肿。 | [
{
"id": 0,
"entity": "淋巴管发育不全",
"start_offset": 9,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "淋巴阻塞",
"start_offset": 17,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "继发性淋巴水肿",
"start_offset": 22,
"end_offset": 29,
"label": "dis"
}
] |
总之，目前Turner综合征致病机制正在向分子遗传学方向深入。 | [
{
"id": 0,
"entity": "Turner综合征",
"start_offset": 5,
"end_offset": 14,
"label": "dis"
}
] |
【临床表现】典型的Turner综合征患者在出生时就有身高和体重发育落后，在新生儿时期可见颈后皮肤过度折叠以及手、足背发生水肿等特殊症状。 | [
{
"id": 0,
"entity": "Turner综合征",
"start_offset": 9,
"end_offset": 18,
"label": "dis"
},
{
"id": 1,
"entity": "身高和体重发育落后",
"start_offset": 26,
"end_offset": 35,
"label": "sym"
},
{
"id": 2,
"entity": "颈后皮肤过度折叠",
"start_offset": 44,
"end_offset": 52,
"label": "sym"
},
{
"id": 3,
"entity": "手、足背发生水肿等特殊症状",
"start_offset": 54,
"end_offset": 67,
"label": "sym"
}
] |
除生殖器、乳腺不发育、原发性闭经及缺乏第二性征之外，尚有呆板面容、智力正常或稍低，约有18%的患者有智能落后。 | [
{
"id": 0,
"entity": "生殖器",
"start_offset": 1,
"end_offset": 4,
"label": "sym"
},
{
"id": 1,
"entity": "乳腺不发育",
"start_offset": 5,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "呆板面容",
"start_offset": 28,
"end_offset": 32,
"label": "sym"
},
{
"id": 3,
"entity": "智力正常或稍低",
"start_offset": 33,
"end_offset": 40,
"label": "sym"
},
{
"id": 4,
"entity": "智能落后",
"start_offset": 50,
"end_offset": 54,
"label": "sym"
}
] |
最近有报道，用超声心动图检查Turner综合征患者，查出有34%病例并发主动脉瓣二叶型，但无狭窄。 | [
{
"id": 0,
"entity": "超声心动图检查",
"start_offset": 7,
"end_offset": 14,
"label": "pro"
},
{
"id": 1,
"entity": "Turner综合征",
"start_offset": 14,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "主动脉瓣二叶型",
"start_offset": 36,
"end_offset": 43,
"label": "dis"
},
{
"id": 3,
"entity": "无狭窄",
"start_offset": 45,
"end_offset": 48,
"label": "sym"
}
] |
患者还可并发肾脏畸形。 | [
{
"id": 0,
"entity": "肾脏畸形",
"start_offset": 6,
"end_offset": 10,
"label": "dis"
}
] |
【实验室检查】1.染色体核型分析临床确诊TS的关键检测手段，用外周血淋巴细胞培养技术进行核型分析，先天性卵巢发育不全综合征的异常核型有以下类型：（1）单体型，45，X是最多见的一种，约占60%。 | [
{
"id": 0,
"entity": "染色体核型分析",
"start_offset": 9,
"end_offset": 16,
"label": "pro"
},
{
"id": 1,
"entity": "TS",
"start_offset": 20,
"end_offset": 22,
"label": "dis"
},
{
"id": 2,
"entity": "外周血淋巴细胞培养技术",
"start_offset": 31,
"end_offset": 42,
"label": "pro"
},
{
"id": 3,
"entity": "核型分析",
"start_offset": 44,
"end_offset": 48,
"label": "pro"
},
{
"id": 4,
"entity": "先天性卵巢发育不全综合征",
"start_offset": 49,
"end_offset": 61,
"label": "dis"
},
{
"id": 5,
"entity": "单体型",
"start_offset": 75,
"end_offset": 78,
"label": "dis"
},
{
"id": 6,
"entity": "X",
"start_offset": 82,
"end_offset": 83,
"label": "bod"
}
] |
（2）嵌合型，核型为45，X/46，XX，约占该病的25%。 | [
{
"id": 0,
"entity": "嵌合型",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "X",
"start_offset": 13,
"end_offset": 14,
"label": "bod"
},
{
"id": 2,
"entity": "XX",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
}
] |
细胞类型以46，XX为主的个体临床症状较轻，约20%的患者可有月经来潮，部分有生育能力。 | [
{
"id": 0,
"entity": "XX",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
}
] |
若患者以45，X细胞为主，其表型与单体型相似。 | [
{
"id": 0,
"entity": "X细胞",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
}
] |
（3）X染色体结构异常，46，Xdel（Xq）或者46，Xdel（Xp），即1条X染色体长臂或短臂缺失，同时伴有X染色体易位；46，Xi（Xq），即一条X染色体的短臂缺失而形成了等长臂X染色体。 | [
{
"id": 0,
"entity": "X染色体结构异常",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "X染色体",
"start_offset": 40,
"end_offset": 44,
"label": "bod"
},
{
"id": 2,
"entity": "X染色体",
"start_offset": 56,
"end_offset": 60,
"label": "bod"
},
{
"id": 3,
"entity": "X染色体",
"start_offset": 76,
"end_offset": 80,
"label": "bod"
},
{
"id": 4,
"entity": "等长臂X染色体",
"start_offset": 89,
"end_offset": 96,
"label": "bod"
},
{
"id": 5,
"entity": "一条X染色体的短臂缺失而形成了等长臂X染色体",
"start_offset": 74,
"end_offset": 96,
"label": "sym"
}
] |
2.内分泌激素检查垂体促性腺激素黄体生成激素（LH）及促卵泡生长激素（FSH）明显升高，E2</sub>降低，提示卵巢功能衰竭。 | [
{
"id": 0,
"entity": "内分泌激素检查",
"start_offset": 2,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "垂体促性腺激素黄体生成激素",
"start_offset": 9,
"end_offset": 22,
"label": "ite"
},
{
"id": 2,
"entity": "LH",
"start_offset": 23,
"end_offset": 25,
"label": "ite"
},
{
"id": 3,
"entity": "促卵泡生长激素",
"start_offset": 27,
"end_offset": 34,
"label": "ite"
},
{
"id": 4,
"entity": "FSH",
"start_offset": 35,
"end_offset": 38,
"label": "ite"
},
{
"id": 5,
"entity": "垂体促性腺激素黄体生成激素（LH）及促卵泡生长激素（FSH）明显升高",
"start_offset": 9,
"end_offset": 43,
"label": "sym"
},
{
"id": 6,
"entity": "E2</sub>",
"start_offset": 44,
"end_offset": 52,
"label": "ite"
},
{
"id": 7,
"entity": "E2</sub>降低",
"start_offset": 44,
"end_offset": 54,
"label": "sym"
},
{
"id": 8,
"entity": "卵巢",
"start_offset": 57,
"end_offset": 59,
"label": "bod"
},
{
"id": 9,
"entity": "卵巢功能衰竭",
"start_offset": 57,
"end_offset": 63,
"label": "sym"
}
] |
部分患者血清生长激素（GH）激发峰值常可小于10ng/ml，血清胰岛素样生长因子1（IGF-1）分泌低下。 | [
{
"id": 0,
"entity": "血清生长激素",
"start_offset": 4,
"end_offset": 10,
"label": "ite"
},
{
"id": 1,
"entity": "GH",
"start_offset": 11,
"end_offset": 13,
"label": "ite"
},
{
"id": 2,
"entity": "血清生长激素（GH）激发峰值常可小于10ng/ml",
"start_offset": 4,
"end_offset": 29,
"label": "sym"
},
{
"id": 3,
"entity": "血清胰岛素",
"start_offset": 30,
"end_offset": 35,
"label": "bod"
},
{
"id": 4,
"entity": "血清胰岛素样生长因子1（IGF-1）分泌低下",
"start_offset": 30,
"end_offset": 52,
"label": "sym"
}
] |
3.B超检查显示子宫及卵巢发育不良，严重者呈纤维条索状。 | [
{
"id": 0,
"entity": "B超检查",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "子宫",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "卵巢",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 3,
"entity": "子宫及卵巢发育不良",
"start_offset": 8,
"end_offset": 17,
"label": "sym"
},
{
"id": 4,
"entity": "严重者呈纤维条索状",
"start_offset": 18,
"end_offset": 27,
"label": "sym"
}
] |
【诊断及鉴别诊断】如发现女孩身材矮小，肘外翻，颈蹼，青春期无第二性征，又伴有某些先天畸形时应怀疑此病。 | [
{
"id": 0,
"entity": "身材矮小",
"start_offset": 14,
"end_offset": 18,
"label": "sym"
},
{
"id": 1,
"entity": "肘外翻",
"start_offset": 19,
"end_offset": 22,
"label": "sym"
},
{
"id": 2,
"entity": "颈蹼",
"start_offset": 23,
"end_offset": 25,
"label": "sym"
},
{
"id": 3,
"entity": "青春期无第二性征",
"start_offset": 26,
"end_offset": 34,
"label": "sym"
},
{
"id": 4,
"entity": "先天畸形",
"start_offset": 40,
"end_offset": 44,
"label": "sym"
}
] |
确诊需女孩身材矮小学检查。 | [
{
"id": 0,
"entity": "女孩身材矮小",
"start_offset": 3,
"end_offset": 9,
"label": "sym"
}
] |
在鉴别诊断时应考虑下列疾病：1.先天畸形症患者多无畸形，其生长激素分泌不足，部分病人还出现促甲状腺激素及促肾上腺皮质激素不足。 | [
{
"id": 0,
"entity": "先天畸形",
"start_offset": 16,
"end_offset": 20,
"label": "sym"
},
{
"id": 1,
"entity": "患者多无畸形",
"start_offset": 21,
"end_offset": 27,
"label": "sym"
},
{
"id": 2,
"entity": "生长激素",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "其生长激素分泌不足",
"start_offset": 28,
"end_offset": 37,
"label": "sym"
},
{
"id": 4,
"entity": "促甲状腺激素",
"start_offset": 45,
"end_offset": 51,
"label": "bod"
},
{
"id": 5,
"entity": "促肾上腺皮质激素",
"start_offset": 52,
"end_offset": 60,
"label": "bod"
},
{
"id": 6,
"entity": "出现促甲状腺激素及促肾上腺皮质激素不足",
"start_offset": 43,
"end_offset": 62,
"label": "sym"
}
] |
2.青春期发育延迟虽青春期较正常儿延迟数年出现促甲状腺激素到发育正常水平，其内分泌功能亦正常，无血TSH及LH升高。 | [
{
"id": 0,
"entity": "出现促甲状腺激素",
"start_offset": 21,
"end_offset": 29,
"label": "sym"
},
{
"id": 1,
"entity": "血TSH及LH",
"start_offset": 48,
"end_offset": 55,
"label": "ite"
}
] |
3.出生低体重儿以足月出生，但体重明显低于正常儿以及体格发育始终无血TSH及LH升高为其特征。 | [
{
"id": 0,
"entity": "体重明显低于正常儿以及体格发育",
"start_offset": 15,
"end_offset": 30,
"label": "sym"
},
{
"id": 1,
"entity": "无血TSH及LH升高",
"start_offset": 32,
"end_offset": 42,
"label": "sym"
}
] |
其生长激素及骨龄接近正常。 | [
{
"id": 0,
"entity": "生长激素及骨龄接近正常",
"start_offset": 1,
"end_offset": 12,
"label": "sym"
}
] |
4.Noonan综合征临床表现与Turner综合征相似，智能发育迟缓者较多，部分病人合并心血管畸形，其中以肺动脉狭窄及房间隔缺损最常见，其核型为正常男性或者女性。 | [
{
"id": 0,
"entity": "Noonan综合征",
"start_offset": 2,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "Turner综合征",
"start_offset": 16,
"end_offset": 25,
"label": "dis"
},
{
"id": 2,
"entity": "智能发育迟缓者",
"start_offset": 28,
"end_offset": 35,
"label": "sym"
},
{
"id": 3,
"entity": "合并心血管畸形",
"start_offset": 42,
"end_offset": 49,
"label": "sym"
},
{
"id": 4,
"entity": "肺动脉",
"start_offset": 53,
"end_offset": 56,
"label": "bod"
},
{
"id": 5,
"entity": "房间隔",
"start_offset": 59,
"end_offset": 62,
"label": "bod"
},
{
"id": 6,
"entity": "肺动脉狭窄及房间隔缺损",
"start_offset": 53,
"end_offset": 64,
"label": "sym"
}
] |
【治疗】本病的治疗以改善其肺动脉狭窄身高、促进性征发育、辅助生殖技术、社会心理治疗及相关疾病防治为目标。 | [
{
"id": 0,
"entity": "肺动脉狭窄",
"start_offset": 13,
"end_offset": 18,
"label": "sym"
}
] |
重组人生长激素对TS患儿身高改善有一定作用，明确诊断后每晚临睡前皮下注射0.15U/kg。 | [
{
"id": 0,
"entity": "TS",
"start_offset": 8,
"end_offset": 10,
"label": "dis"
}
] |
2.雌性激素替代治疗在青春期可用雌激素进行替代疗法，一般从12～14岁开始，先用小剂量结合雌性激素疗6～12个月，逐步增加到成年人替代治疗剂量，以促使乳房及外阴发育。 | [
{
"id": 0,
"entity": "雌性激素替代治疗",
"start_offset": 2,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "雌激素",
"start_offset": 16,
"end_offset": 19,
"label": "dru"
},
{
"id": 2,
"entity": "雌性激素",
"start_offset": 45,
"end_offset": 49,
"label": "dru"
},
{
"id": 3,
"entity": "乳房",
"start_offset": 75,
"end_offset": 77,
"label": "bod"
},
{
"id": 4,
"entity": "外阴",
"start_offset": 78,
"end_offset": 80,
"label": "bod"
}
] |
2年后可进行周期性的雌激素-孕激素疗法（人工周期治疗），有助于患者的第二性征发育及提高生活质量。 | [
{
"id": 0,
"entity": "雌激素-孕激素疗法",
"start_offset": 10,
"end_offset": 19,
"label": "pro"
}
] |
由于性激素具有促进骨骺愈合，限制骨骼生长的作用，故在青春期前忌用，12岁后方可考虑使用。 | [
{
"id": 0,
"entity": "性激素",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "骨骺",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "骨骼",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
}
] |
诱导性激素性发育须遵循个体化原则。 | [
{
"id": 0,
"entity": "性激素",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
}
] |
极少数嵌合型患者可能有生育能力，但其流产或者死胎率极高，30%后代患有染色体畸变。 | [
{
"id": 0,
"entity": "流产",
"start_offset": 18,
"end_offset": 20,
"label": "sym"
},
{
"id": 1,
"entity": "死胎率",
"start_offset": 22,
"end_offset": 25,
"label": "sym"
},
{
"id": 2,
"entity": "染色体畸变",
"start_offset": 35,
"end_offset": 40,
"label": "sym"
}
] |
二、呼吸机参数设定及其呼吸生理基础（一）肺通气量有关通气量、压力和流量示意（图8-19）。 | [
{
"id": 0,
"entity": "呼吸机",
"start_offset": 2,
"end_offset": 5,
"label": "equ"
},
{
"id": 1,
"entity": "肺通气量",
"start_offset": 20,
"end_offset": 24,
"label": "ite"
}
] |
图8-19气道压力、肺容量、气流流量变化示意图1.潮气（tidalvolume，VT）每一次自主呼吸或机械通气时，进入或排出肺的气体量。 | [
{
"id": 0,
"entity": "机械通气",
"start_offset": 51,
"end_offset": 55,
"label": "pro"
},
{
"id": 1,
"entity": "肺",
"start_offset": 62,
"end_offset": 63,
"label": "bod"
}
] |
机械通气下，儿童与成人为5～7ml/kg体重，足月新生儿为6～8ml/kg，早产儿为8～10ml/kg。 | [
{
"id": 0,
"entity": "机械通气",
"start_offset": 0,
"end_offset": 4,
"label": "pro"
}
] |
2.通气频率（frequency）和吸呼比值（inspiration：expirationratio，I∶E）通气频率指每分钟机械通气的次数。 | [
{
"id": 0,
"entity": "机械通气",
"start_offset": 63,
"end_offset": 67,
"label": "pro"
}
] |
完成一次机械通气所需时间为通气周期，为频率的倒数×60。 | [
{
"id": 0,
"entity": "机械通气",
"start_offset": 4,
"end_offset": 8,
"label": "pro"
}
] |
吸呼比值则指一次机械通气时，供气与排气的时间比例，呼吸机一般只调节供气时间的长短以获得所需的吸呼比值，但有的呼吸机如美国Sechrist型、德国德尔格Babylog8000型，则可以分别改变供气和排气时间来改变吸呼比，且会影响到通气频率。 | [
{
"id": 0,
"entity": "机械通气",
"start_offset": 8,
"end_offset": 12,
"label": "pro"
},
{
"id": 1,
"entity": "呼吸机",
"start_offset": 25,
"end_offset": 28,
"label": "equ"
},
{
"id": 2,
"entity": "呼吸机",
"start_offset": 54,
"end_offset": 57,
"label": "equ"
},
{
"id": 3,
"entity": "美国Sechrist型",
"start_offset": 58,
"end_offset": 69,
"label": "equ"
},
{
"id": 4,
"entity": "德国德尔格Babylog8000型",
"start_offset": 70,
"end_offset": 87,
"label": "equ"
}
] |
3.每分通气量（minutevolume，MV）为潮气量和呼吸频率的乘积，指每分钟进出肺部的气量，用于判断通气量的大小，较单用潮气量要全面。 | [
{
"id": 0,
"entity": "肺部",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
}
] |
机械通气下，一个3～4kg小儿一般要维持在0.8～1.2L/min。 | [
{
"id": 0,
"entity": "机械通气",
"start_offset": 0,
"end_offset": 4,
"label": "pro"
}
] |
当血气值测定后要求对呼吸机参数调整时，也应设定新的每分通气量值。 | [
{
"id": 0,
"entity": "血气值测定",
"start_offset": 1,
"end_offset": 6,
"label": "ite"
},
{
"id": 1,
"entity": "呼吸机",
"start_offset": 10,
"end_offset": 13,
"label": "equ"
}
] |
一般呼吸机均有每分通气量上下限报警。 | [
{
"id": 0,
"entity": "呼吸机",
"start_offset": 2,
"end_offset": 5,
"label": "equ"
}
] |
如果上限报警，可能因通气频率加快（触发增加）或潮气量过大（定压模式）；如果下限报警，可能为供气量不足，供气回路管道或接口漏气，潮气量过低（定压模式），或呼吸机主供气气流不稳定（须检查压缩空气和氧气气源压力）。 | [
{
"id": 0,
"entity": "呼吸机",
"start_offset": 76,
"end_offset": 79,
"label": "equ"
}
] |
但在小婴儿，由于气道插管在声门处的漏气，或呼吸机监测装置装置的偏差，可以出现不一致。 | [
{
"id": 0,
"entity": "声门",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 1,
"entity": "呼吸机监测装置",
"start_offset": 21,
"end_offset": 28,
"label": "equ"
}
] |
（二）通气压力1.气道峰压（peakinspiratorypressure，PIP）PIP是指在一次机械通气周期内，气道内压力达到的最大值，其内涵包括：①供气压力高于大气压（基线压）的最大变化水平；②达到最大峰压值所需时间；③达到最大值后的维持时间（图8-8）。 | [
{
"id": 0,
"entity": "气道",
"start_offset": 58,
"end_offset": 60,
"label": "bod"
}
] |
机械通气或自主呼吸的吸气末和呼气末的气流为零。 | [
{
"id": 0,
"entity": "机械通气",
"start_offset": 0,
"end_offset": 4,
"label": "pro"
}
] |
PIP设置的高低在于使肺泡张开并维持其张开达到适当时间。 | [
{
"id": 0,
"entity": "肺泡",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
}
] |
不同的压力波型可以对肺泡张开和肺泡内压水平，产生不同效果，并可应用于不同的肺内病变。 | [
{
"id": 0,
"entity": "肺泡",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 1,
"entity": "肺泡",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "肺内病变",
"start_offset": 37,
"end_offset": 41,
"label": "dis"
}
] |
通过呼吸机设置吸气气流上升时间改变压力波形可以得到较安全有效的通气。 | [
{
"id": 0,
"entity": "呼吸机",
"start_offset": 2,
"end_offset": 5,
"label": "equ"
},
{
"id": 1,
"entity": "通气",
"start_offset": 31,
"end_offset": 33,
"label": "pro"
}
] |
有的呼吸机采用设置PIP水平高于PEEP水平，因此实际的PIP水平应该为其设置值加上设置的PEEP值。 | [
{
"id": 0,
"entity": "呼吸机",
"start_offset": 2,
"end_offset": 5,
"label": "equ"
}
] |
在弥漫性肺损伤和肺泡萎陷时，要获得比较好的氧合，需要在机械通气时保持压力的平台，以使肺泡持续扩张，气体在不同时间常数的肺泡单位内移动，达到压力平台，使得到较好的肺泡通气/灌流，减少肺内分流。 | [
{
"id": 0,
"entity": "弥漫性肺损伤",
"start_offset": 1,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "肺泡萎陷",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "机械通气",
"start_offset": 27,
"end_offset": 31,
"label": "pro"
},
{
"id": 3,
"entity": "肺泡",
"start_offset": 42,
"end_offset": 44,
"label": "bod"
}
] |
图8-20机械通气气道峰压示意图压力上升缓慢，没有气道峰压平台；压力上升较快，出现气道峰压平台；压力上升迅速，气道峰压平台时间较长，趋于平台4.呼气末正压（PEEP）呼吸机在排气管道设置的阻力阀门，使呼气相管道阻力增加，管道压力高于大气压。 | [
{
"id": 0,
"entity": "机械通气气道",
"start_offset": 5,
"end_offset": 11,
"label": "equ"
},
{
"id": 1,
"entity": "呼吸机",
"start_offset": 83,
"end_offset": 86,
"label": "equ"
}
] |
其效果主要为保持在呼气时肺泡不至于立即关闭，提高功能余气量，有利于气血交换，并减少呼吸做功。 | [
{
"id": 0,
"entity": "肺泡",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 1,
"entity": "气血",
"start_offset": 33,
"end_offset": 35,
"label": "bod"
}
] |
一般用ml/min或L/min表示，在肺功能监护仪上显示一个呼吸周期的通气流量随时间或潮气有大小、位相的变化。 | [
{
"id": 0,
"entity": "肺功能监护仪",
"start_offset": 19,
"end_offset": 25,
"label": "equ"
}
] |
当自主呼吸增强或患儿触发的机械通气次数增加，实测每分流量可以高于设定每分流量。 | [
{
"id": 0,
"entity": "机械通气",
"start_offset": 13,
"end_offset": 17,
"label": "pro"
}
] |
3.峰流量在一次通气开始时，供气阀门打开，气流在瞬间达到最大值，以后随气道阻力和肺顺应性的反应，供气气流下降，为减速气流，为目前呼吸机常设。 | [
{
"id": 0,
"entity": "通气",
"start_offset": 8,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "呼吸机",
"start_offset": 64,
"end_offset": 67,
"label": "equ"
}
] |
如果在气流达到最大后，尽管有气道阻力的增加，供气气流仍然维持在恒定水平，此为持续恒定气流，为一部分儿童型呼吸机供气的主要方式。 | [
{
"id": 0,
"entity": "儿童型呼吸机",
"start_offset": 49,
"end_offset": 55,
"label": "equ"
}
] |
在新型呼吸机上，小流量的偏流气流使流量触发功能得以发挥。 | [
{
"id": 0,
"entity": "新型呼吸机",
"start_offset": 1,
"end_offset": 6,
"label": "equ"
}
] |
第十三章硬皮病【概述】硬皮病（scleroderma）是儿童时期少见的慢性结缔组织病。 | [
{
"id": 0,
"entity": "硬皮病",
"start_offset": 4,
"end_offset": 7,
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{
"id": 1,
"entity": "硬皮病",
"start_offset": 11,
"end_offset": 14,
"label": "dis"
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{
"id": 2,
"entity": "scleroderma",
"start_offset": 15,
"end_offset": 26,
"label": "dis"
},
{
"id": 3,
"entity": "慢性结缔组织病",
"start_offset": 35,
"end_offset": 42,
"label": "dis"
}
] |
它可分为局限性硬皮病（localizedscleroderma）和系统性硬化症（systemicsclerosis）两种类型。 | [
{
"id": 0,
"entity": "局限性硬皮病",
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},
{
"id": 1,
"entity": "localizedscleroderma",
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"label": "dis"
},
{
"id": 2,
"entity": "系统性硬化症",
"start_offset": 33,
"end_offset": 39,
"label": "dis"
},
{
"id": 3,
"entity": "systemicsclerosis",
"start_offset": 40,
"end_offset": 57,
"label": "dis"
}
] |
前者以局限性皮肤增厚和纤维化为主，后者除皮肤弥漫性增厚和纤维化，内脏器官如心、肺、肾也可受侵犯。 | [
{
"id": 0,
"entity": "局限性皮肤增厚",
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},
{
"id": 1,
"entity": "纤维化",
"start_offset": 11,
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"label": "sym"
},
{
"id": 2,
"entity": "皮肤",
"start_offset": 20,
"end_offset": 22,
"label": "bod"
},
{
"id": 3,
"entity": "皮肤弥漫性增厚",
"start_offset": 20,
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},
{
"id": 4,
"entity": "纤维化",
"start_offset": 28,
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"label": "sym"
},
{
"id": 5,
"entity": "心",
"start_offset": 37,
"end_offset": 38,
"label": "bod"
},
{
"id": 6,
"entity": "肺",
"start_offset": 39,
"end_offset": 40,
"label": "bod"
},
{
"id": 7,
"entity": "肾",
"start_offset": 41,
"end_offset": 42,
"label": "bod"
},
{
"id": 8,
"entity": "内脏器官如心、肺、肾也可受侵犯",
"start_offset": 32,
"end_offset": 47,
"label": "sym"
}
] |
个别局限性硬皮病可转变为系统性硬化症而累及内脏，故认为两者是同一病理过程的不同类型。 | [
{
"id": 0,
"entity": "局限性硬皮病",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "系统性硬化症",
"start_offset": 12,
"end_offset": 18,
"label": "dis"
},
{
"id": 2,
"entity": "内脏",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
}
] |
【病因和发病机制】病因未明，很多报道提示是在遗传因素（如HLADR1</sub>和DR3</sub>频率较高）的参与下，由于某种因子刺激所导致的自身免疫性疾病。 | [
{
"id": 0,
"entity": "自身免疫性疾病",
"start_offset": 72,
"end_offset": 79,
"label": "dis"
}
] |
并认为本病的纤维化病变与5-羟色胺代谢异常有关。 | [
{
"id": 0,
"entity": "纤维化病变",
"start_offset": 6,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "5-羟色胺",
"start_offset": 12,
"end_offset": 17,
"label": "bod"
}
] |
内脏肌肉纤维变性、萎缩以及肌间纤维组织增生而造成脏器硬化。 | [
{
"id": 0,
"entity": "脏器",
"start_offset": 24,
"end_offset": 26,
"label": "bod"
},
{
"id": 1,
"entity": "脏器硬化",
"start_offset": 24,
"end_offset": 28,
"label": "sym"
}
] |
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