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若肺血增加而心影正常则可能是肺静脉淤血；若肺血正常或减少而心影增大，应考虑为左向右分流之外的其他病变。 | [
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因此，肺血是否增多，需与心脏病变相联系，还应考虑众多背景因素包括年龄、呼吸深浅、X线的透光度。 | [
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"entity": "X线",
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1.非发绀型先心病肺充血如前所述，肺循环体循环血流比小于2∶1时肺血可正常。 | [
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如肺血流量继续增加，肺血管扩张，则肺血管影向肺外带延伸（图9-9）；在肺动脉血流增加的同时，肺静脉血回流至左心房亦增加，导致左心房扩大。 | [
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"entity": "肺血管",
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"entity": "肺外带",
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"entity": "肺动脉血流",
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"entity": "肺静脉血",
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"entity": "左心房",
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"entity": "左心房",
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"entity": "左心房扩大",
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由于心腔接纳大量的额外血流，心脏常扩大且X线可显示心影增大。 | [
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"entity": "X线",
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常见于：房间隔缺损（ASD）、室间隔缺损（VSD）、房室间隔缺损、动脉导管未闭（PDA）；左心房、左心室增大见于大的VSD、房室间隔缺损或ASD；右心房、右心室增大见于大的ASD。 | [
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"entity": "动脉导管未闭",
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"entity": "ASD",
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"entity": "ASD",
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如大量左向右分流逐渐减，即为肺血管病变逐渐进展而致肺血管逐渐减少，扩张的肺血管影及外周肺血管影均减少。 | [
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图9-2房室间隔缺损（正位片）示：高肺动脉血流量导致肺纹理增多和心脏扩大2.发绀型肺血增多肺血增多和心脏肥大也见于发绀型先天性心脏病（congenitalheartdisease），见于完全性大动脉转位（transpositionofthegreatarteries）、共同通道畸形（无梗阻性完全性肺静脉异位连接、心室双入口不伴肺动脉狭窄及永存动脉干）、右向左分流伴肺血流增加（四联症伴肺动脉闭锁（pulmonaryatresia）合并大型的主肺动脉侧支或分流形成）。 | [
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"entity": "完全性大动脉转位",
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"entity": "transpositionofthegreatarteries",
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"label": "dis"
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{
"id": 11,
"entity": "共同通道畸形",
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"label": "dis"
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"id": 12,
"entity": "无梗阻性完全性肺静脉异位连接",
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"id": 13,
"entity": "心室双入口不伴肺动脉狭窄",
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"id": 14,
"entity": "永存动脉干",
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"id": 15,
"entity": "右向左分流伴肺血流增加",
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"label": "dis"
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"id": 16,
"entity": "四联症伴肺动脉闭锁",
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"entity": "pulmonaryatresia",
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"entity": "主肺动脉侧支",
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"label": "bod"
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{
"id": 19,
"entity": "分流",
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3.发绀性肺血减少心影正常或稍增大伴有右向左分流所致的肺血减少，与先天性心脏病肺动脉狭窄和梗阻有关。 | [
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"entity": "梗阻",
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肺血减少程度与肺血流量有关，后者与肺动脉狭窄程度或导管依赖性病变的动脉导管大小有关。 | [
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"entity": "导管",
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"entity": "动脉导管",
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胸片表现为肺部过度充气。 | [
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典型病变有：法洛四联症伴或不伴肺动脉闭锁、室间隔完整型肺动脉闭锁（图9-9）、Ebstein畸形、三尖瓣闭锁（tricuspidatresia）伴肺动脉闭锁或狭窄。 | [
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"entity": "Ebstein畸形",
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"entity": "三尖瓣闭锁",
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"entity": "tricuspidatresia",
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"id": 6,
"entity": "肺动脉闭锁或狭窄",
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法洛四联症心影呈靴形，心尖上翘（图9-4）。 | [
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Ebstein畸形则表现为心影明显扩大。 | [
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4.肺静脉淤血肺静脉淤血伴肺动脉高压在儿童并不多见。 | [
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见于肺静脉异位连接、三房心、二尖瓣狭窄（mitralstenosis）或限制型心房间交通伴二尖瓣闭锁。 | [
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肺动脉毛细血管压超过12mmHg时，上下叶血管影相当或上叶略有增多，压力超过15mmHg时，可见肺间质水肿，位于外周的肺叶间隙水肿增厚，显示为KerleyB线，即从可见在肺下野两侧直达肺脏边缘的水平线，肺血管模糊不清。 | [
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"entity": "上叶",
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"entity": "肺间质水肿",
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"id": 4,
"entity": "位于外周的肺叶间隙水肿增厚",
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"id": 5,
"entity": "肺下野两侧",
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"entity": "肺血管",
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肺门部的肺叶间隙增厚，分散在中野或上野的KerleyA线也较明显。 | [
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肺毛细血管压超过25mmHg时，肺泡水肿形成蝶翼征。 | [
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该阴影在双侧肺门区域最明显，对称性向外逐渐消散（图9-9）。 | [
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图9-3室间隔完整型的肺动脉闭锁（正位片）示：肺血流量减少，肺野缺血图9-4法洛四联症（正位片）示：心脏呈典型的“靴形心”，心尖上翘，肺动脉影凹陷和右位主动脉弓（五）心血管结构异常判别1.水肿本病在较大儿童和青年伴心脏肥大者少见。 | [
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"entity": "心脏呈典型的“靴形心”",
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"entity": "心尖上翘",
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"entity": "肺动脉影凹陷和右位主动脉弓",
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"entity": "心血管结构异常",
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{
"id": 8,
"entity": "水肿",
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{
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"entity": "心脏肥大",
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有时因左锁骨下动脉扩张呈现狭窄部近端动脉扩张及降主动脉狭窄后扩张。 | [
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双侧肋骨切迹通常位于第3、4后肋下缘，与肋间动脉侧支血管形成有关。 | [
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{
"id": 1,
"entity": "肋间动脉侧支血管",
"start_offset": 20,
"end_offset": 28,
"label": "bod"
}
] |
2.右位主动脉弓（aorticarch）即主动脉弓上升于右支气管前，在气管的右侧形成弓后，向下走行。 | [
{
"id": 0,
"entity": "右位主动脉弓",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "aorticarch",
"start_offset": 9,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "主动脉弓",
"start_offset": 21,
"end_offset": 25,
"label": "bod"
},
{
"id": 3,
"entity": "右支气管",
"start_offset": 28,
"end_offset": 32,
"label": "bod"
},
{
"id": 4,
"entity": "气管",
"start_offset": 35,
"end_offset": 37,
"label": "bod"
},
{
"id": 5,
"entity": "主动脉弓上升于右支气管前，在气管的右侧形成弓后，向下走行",
"start_offset": 21,
"end_offset": 49,
"label": "sym"
}
] |
后者在先心病中较少见，但本病与法洛四联症、永存动脉干和大动脉转位均有密切关系。 | [
{
"id": 0,
"entity": "先心病",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "法洛四联症",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "永存动脉干",
"start_offset": 21,
"end_offset": 26,
"label": "dis"
},
{
"id": 3,
"entity": "大动脉转位",
"start_offset": 27,
"end_offset": 32,
"label": "dis"
}
] |
3.肺动脉瓣狭窄可见肺总动脉和左肺动脉扩张，其余肺动脉段发育正常，肺动脉扩张与狭窄后扩张有关。 | [
{
"id": 0,
"entity": "肺动脉瓣狭窄",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "肺总动脉",
"start_offset": 10,
"end_offset": 14,
"label": "bod"
},
{
"id": 2,
"entity": "左肺动脉",
"start_offset": 15,
"end_offset": 19,
"label": "bod"
},
{
"id": 3,
"entity": "肺总动脉和左肺动脉扩张",
"start_offset": 10,
"end_offset": 21,
"label": "sym"
},
{
"id": 4,
"entity": "其余肺动脉段",
"start_offset": 22,
"end_offset": 28,
"label": "bod"
},
{
"id": 5,
"entity": "肺动脉",
"start_offset": 33,
"end_offset": 36,
"label": "bod"
}
] |
（六）骨骼异常肋骨畸形、脊柱侧突和椎骨的异常如脊椎畸形，均与先心病有关。 | [
{
"id": 0,
"entity": "骨骼异常",
"start_offset": 3,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "肋骨畸形",
"start_offset": 7,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "脊柱侧突",
"start_offset": 12,
"end_offset": 16,
"label": "dis"
},
{
"id": 3,
"entity": "椎骨的异常",
"start_offset": 17,
"end_offset": 22,
"label": "dis"
},
{
"id": 4,
"entity": "脊椎畸形",
"start_offset": 23,
"end_offset": 27,
"label": "dis"
},
{
"id": 5,
"entity": "先心病",
"start_offset": 30,
"end_offset": 33,
"label": "dis"
}
] |
脊柱侧突和漏斗胸见于二尖瓣脱垂、马方综合征；蝶形椎体见于阿拉吉尔综合征（Alagillesyndrome）伴外周肺动脉狭窄。 | [
{
"id": 0,
"entity": "脊柱侧突",
"start_offset": 0,
"end_offset": 4,
"label": "sym"
},
{
"id": 1,
"entity": "漏斗胸",
"start_offset": 5,
"end_offset": 8,
"label": "sym"
},
{
"id": 2,
"entity": "二尖瓣脱垂",
"start_offset": 10,
"end_offset": 15,
"label": "dis"
},
{
"id": 3,
"entity": "马方综合征",
"start_offset": 16,
"end_offset": 21,
"label": "dis"
},
{
"id": 4,
"entity": "阿拉吉尔综合征",
"start_offset": 28,
"end_offset": 35,
"label": "dis"
},
{
"id": 5,
"entity": "Alagillesyndrome",
"start_offset": 36,
"end_offset": 52,
"label": "dis"
},
{
"id": 6,
"entity": "外周肺动脉狭窄",
"start_offset": 54,
"end_offset": 61,
"label": "dis"
}
] |
肺骨关节病可有骨髓腔扩大，继发于发绀型先天性心脏病的低氧血症。 | [
{
"id": 0,
"entity": "肺骨关节病",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "骨髓腔扩大",
"start_offset": 7,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "发绀型先天性心脏病的低氧血症",
"start_offset": 16,
"end_offset": 30,
"label": "dis"
}
] |
后肋下缘切迹如前所述继发于水肿侧支血管形成。 | [
{
"id": 0,
"entity": "水肿侧支血管",
"start_offset": 13,
"end_offset": 19,
"label": "dis"
}
] |
肋骨畸形也可继发于既往的心脏手术。 | [
{
"id": 0,
"entity": "肋骨畸形",
"start_offset": 0,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "心脏手术",
"start_offset": 12,
"end_offset": 16,
"label": "pro"
}
] |
图9-5完全性肺静脉异位引流伴右心房异构（正位片）示：因肺水肿胸片呈“蝶形”，肺部阴影集中在肺门部并向周边呈对称性消退（七）心脏和腹部脏器位置右位心（dextrocardia）是指心脏的大部位于胸骨右侧，心尖指向左侧。 | [
{
"id": 0,
"entity": "完全性肺静脉异位引流伴右心房异构",
"start_offset": 4,
"end_offset": 20,
"label": "dis"
},
{
"id": 1,
"entity": "肺水肿胸片呈“蝶形”",
"start_offset": 28,
"end_offset": 38,
"label": "sym"
},
{
"id": 2,
"entity": "肺部阴影集中在肺门部并向周边呈对称性消退",
"start_offset": 39,
"end_offset": 59,
"label": "sym"
},
{
"id": 3,
"entity": "心脏和腹部脏器位置",
"start_offset": 62,
"end_offset": 71,
"label": "dis"
},
{
"id": 4,
"entity": "右位心",
"start_offset": 71,
"end_offset": 74,
"label": "dis"
},
{
"id": 5,
"entity": "dextrocardia",
"start_offset": 75,
"end_offset": 87,
"label": "dis"
},
{
"id": 6,
"entity": "心脏",
"start_offset": 90,
"end_offset": 92,
"label": "bod"
},
{
"id": 7,
"entity": "胸骨",
"start_offset": 97,
"end_offset": 99,
"label": "bod"
},
{
"id": 8,
"entity": "心尖",
"start_offset": 102,
"end_offset": 104,
"label": "bod"
}
] |
当心脏的所有心腔反位伴有腹腔脏器全部反位时称为镜像性右位心（图9-6），镜像右位心不伴有任何先天性心脏病者临床上少见。 | [
{
"id": 0,
"entity": "心脏的所有心腔反位伴有腹腔脏器全部反位",
"start_offset": 1,
"end_offset": 20,
"label": "sym"
},
{
"id": 1,
"entity": "镜像性右位心",
"start_offset": 23,
"end_offset": 29,
"label": "sym"
},
{
"id": 2,
"entity": "镜像右位心",
"start_offset": 36,
"end_offset": 41,
"label": "sym"
}
] |
大多数右位心合并先天性心脏病，X线胸片表现为心尖指向右侧、胃泡在正常左侧位。 | [
{
"id": 0,
"entity": "右位心",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "先天性心脏病",
"start_offset": 8,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "X线胸片",
"start_offset": 15,
"end_offset": 19,
"label": "pro"
},
{
"id": 3,
"entity": "心尖指向右侧",
"start_offset": 22,
"end_offset": 28,
"label": "sym"
},
{
"id": 4,
"entity": "胃泡在正常左侧位",
"start_offset": 29,
"end_offset": 37,
"label": "sym"
}
] |
部分右位心可伴有右肺发育不全。 | [
{
"id": 0,
"entity": "右位心",
"start_offset": 2,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "右肺发育不全",
"start_offset": 8,
"end_offset": 14,
"label": "dis"
}
] |
图9-6右心房异构，房室间隔缺损和肺动脉瓣狭窄（正位片）示：右位心和肺野缺血，横位的肝脏居中提示合并复杂型先心病通过辨别胃泡及肝脏右叶的位置可判定内脏位置。 | [
{
"id": 0,
"entity": "右心房异构",
"start_offset": 4,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "房室间隔缺损",
"start_offset": 10,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "肺动脉瓣狭窄",
"start_offset": 17,
"end_offset": 23,
"label": "dis"
},
{
"id": 3,
"entity": "右位心",
"start_offset": 30,
"end_offset": 33,
"label": "dis"
},
{
"id": 4,
"entity": "肺野缺血",
"start_offset": 34,
"end_offset": 38,
"label": "dis"
},
{
"id": 5,
"entity": "横位的肝脏居中",
"start_offset": 39,
"end_offset": 46,
"label": "dis"
},
{
"id": 6,
"entity": "复杂型先心病",
"start_offset": 50,
"end_offset": 56,
"label": "dis"
},
{
"id": 7,
"entity": "胃泡",
"start_offset": 60,
"end_offset": 62,
"label": "bod"
},
{
"id": 8,
"entity": "肝脏右叶",
"start_offset": 63,
"end_offset": 67,
"label": "bod"
}
] |
绝大多数人表现为心房内脏正位，少数人两者均成呈镜像反位。 | [
{
"id": 0,
"entity": "心房内脏正位",
"start_offset": 8,
"end_offset": 14,
"label": "sym"
},
{
"id": 1,
"entity": "镜像反位",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
}
] |
有时可有双左或双右侧内脏结构（心耳、肺脏、支气管等），称为左或右侧异构，常与复杂先心病有关；亦可见肝脏横位或胃肠位于腹腔正中。 | [
{
"id": 0,
"entity": "双左或双右侧内脏结构",
"start_offset": 4,
"end_offset": 14,
"label": "sym"
},
{
"id": 1,
"entity": "心耳",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "肺脏",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
},
{
"id": 3,
"entity": "支气管",
"start_offset": 21,
"end_offset": 24,
"label": "bod"
},
{
"id": 4,
"entity": "左或右侧异构",
"start_offset": 29,
"end_offset": 35,
"label": "dis"
},
{
"id": 5,
"entity": "复杂先心病",
"start_offset": 38,
"end_offset": 43,
"label": "dis"
},
{
"id": 6,
"entity": "肝脏横位或胃肠位于腹腔正中",
"start_offset": 49,
"end_offset": 62,
"label": "sym"
}
] |
由于支气管X线断层扫描广泛应用，支气管位置也已成为定位依据。 | [
{
"id": 0,
"entity": "支气管X线断层扫描",
"start_offset": 2,
"end_offset": 11,
"label": "pro"
},
{
"id": 1,
"entity": "支气管",
"start_offset": 16,
"end_offset": 19,
"label": "bod"
}
] |
双右异构时，左侧、右侧主支气管短，上叶支气管向上走行；双左异构时则表现为两侧主支气管均较长。 | [
{
"id": 0,
"entity": "双右异构",
"start_offset": 0,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "左侧、右侧主支气管短",
"start_offset": 6,
"end_offset": 16,
"label": "sym"
},
{
"id": 2,
"entity": "上叶支气管向上走行",
"start_offset": 17,
"end_offset": 26,
"label": "sym"
},
{
"id": 3,
"entity": "双左异构",
"start_offset": 27,
"end_offset": 31,
"label": "dis"
},
{
"id": 4,
"entity": "两侧主支气管均较长",
"start_offset": 36,
"end_offset": 45,
"label": "sym"
}
] |
目前通过超声心动图探查腹主动脉与下腔静脉关系可准确反映心房的位置。 | [
{
"id": 0,
"entity": "超声心动图",
"start_offset": 4,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "腹主动脉",
"start_offset": 11,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "下腔静脉",
"start_offset": 16,
"end_offset": 20,
"label": "bod"
},
{
"id": 3,
"entity": "心房",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
}
] |
第六节低磷血症性抗维生素D佝偻病低磷血症性抗维生素D佝偻病，又称家族性低磷血症性维生素D难治性佝偻病，多为性联显性遗传性疾病。 | [
{
"id": 0,
"entity": "低磷血症性抗维生素D佝偻病",
"start_offset": 3,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "低磷血症性抗维生素D佝偻病",
"start_offset": 16,
"end_offset": 29,
"label": "dis"
},
{
"id": 2,
"entity": "家族性低磷血症性维生素D难治性佝偻病",
"start_offset": 32,
"end_offset": 50,
"label": "dis"
},
{
"id": 3,
"entity": "性联显性遗传性疾病",
"start_offset": 53,
"end_offset": 62,
"label": "dis"
}
] |
亦有人称本病为肾性磷丢失症。 | [
{
"id": 0,
"entity": "肾性磷丢失症",
"start_offset": 7,
"end_offset": 13,
"label": "dis"
}
] |
对一般治疗剂量的维生素D无效。 | [
{
"id": 0,
"entity": "维生素D",
"start_offset": 8,
"end_offset": 12,
"label": "dru"
}
] |
【病因和发病机制】本病多为性联显性遗传的肾小管功能缺陷，少数为常染色体显性或隐性遗传。 | [
{
"id": 0,
"entity": "性联显性遗传的肾小管功能缺陷",
"start_offset": 13,
"end_offset": 27,
"label": "dis"
},
{
"id": 1,
"entity": "常染色体显性或隐性遗传",
"start_offset": 31,
"end_offset": 42,
"label": "sym"
}
] |
由于染色体的先天病变致肾近曲小管细胞膜刷状缘钠-磷转运系统异常，肾小管对磷的重吸收障碍，引起大量磷从肾脏排出；另外，染色体的异常也造成成骨细胞功能不良，致成骨缺陷，造成临床上低磷血症及骨发育不良。 | [
{
"id": 0,
"entity": "染色体的先天病变",
"start_offset": 2,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "肾近曲小管细胞膜刷状缘",
"start_offset": 11,
"end_offset": 22,
"label": "bod"
},
{
"id": 2,
"entity": "肾近曲小管细胞膜刷状缘钠-磷转运系统异常",
"start_offset": 11,
"end_offset": 31,
"label": "sym"
},
{
"id": 3,
"entity": "肾小管",
"start_offset": 32,
"end_offset": 35,
"label": "bod"
},
{
"id": 4,
"entity": "肾小管对磷的重吸收障碍",
"start_offset": 32,
"end_offset": 43,
"label": "sym"
},
{
"id": 5,
"entity": "肾脏",
"start_offset": 50,
"end_offset": 52,
"label": "bod"
},
{
"id": 6,
"entity": "大量磷从肾脏排出",
"start_offset": 46,
"end_offset": 54,
"label": "sym"
},
{
"id": 7,
"entity": "染色体",
"start_offset": 58,
"end_offset": 61,
"label": "bod"
},
{
"id": 8,
"entity": "染色体的异常",
"start_offset": 58,
"end_offset": 64,
"label": "sym"
},
{
"id": 9,
"entity": "成骨细胞",
"start_offset": 67,
"end_offset": 71,
"label": "bod"
},
{
"id": 10,
"entity": "成骨细胞功能不良",
"start_offset": 67,
"end_offset": 75,
"label": "sym"
},
{
"id": 11,
"entity": "成骨",
"start_offset": 77,
"end_offset": 79,
"label": "bod"
},
{
"id": 12,
"entity": "成骨缺陷",
"start_offset": 77,
"end_offset": 81,
"label": "sym"
},
{
"id": 13,
"entity": "低磷血症",
"start_offset": 87,
"end_offset": 91,
"label": "dis"
},
{
"id": 14,
"entity": "骨发育不良",
"start_offset": 92,
"end_offset": 97,
"label": "dis"
}
] |
目前认为与下列发病机制有关：（一）肾小管保留磷和肠道吸收磷障碍认为肾小管本身功能障碍，可能缺乏一种磷结合蛋白，因为肾小管和肠黏膜上皮细胞的磷结合蛋白可能受同一基因位点的密码控制，当此基因缺陷，可出现尿磷丢失增加及肠道磷摄取障碍。 | [
{
"id": 0,
"entity": "肾小管保留磷和肠道吸收磷障碍",
"start_offset": 17,
"end_offset": 31,
"label": "dis"
},
{
"id": 1,
"entity": "肾小管本身功能障碍",
"start_offset": 33,
"end_offset": 42,
"label": "dis"
},
{
"id": 2,
"entity": "磷结合蛋白",
"start_offset": 49,
"end_offset": 54,
"label": "bod"
},
{
"id": 3,
"entity": "缺乏一种磷结合蛋白",
"start_offset": 45,
"end_offset": 54,
"label": "sym"
},
{
"id": 4,
"entity": "肾小管",
"start_offset": 57,
"end_offset": 60,
"label": "bod"
},
{
"id": 5,
"entity": "肠黏膜上皮细胞",
"start_offset": 61,
"end_offset": 68,
"label": "bod"
},
{
"id": 6,
"entity": "磷结合蛋白",
"start_offset": 69,
"end_offset": 74,
"label": "bod"
},
{
"id": 7,
"entity": "尿磷丢失增加",
"start_offset": 99,
"end_offset": 105,
"label": "sym"
},
{
"id": 8,
"entity": "肠道磷摄取障碍",
"start_offset": 106,
"end_offset": 113,
"label": "sym"
}
] |
（二）肾脏1-α羟化酶缺陷肾脏1-α羟化酶活性减低，使1，25（OH）<sub>2</sub>D3</sub>合成减少，肠道钙吸收减少，尿磷排出增加，同时引起继发性甲状旁腺功能亢进，甲状旁腺素（PTH）增加，加重尿磷排出，最后导致骨钙化不全而造成佝偻病或骨软化症。 | [
{
"id": 0,
"entity": "肾脏1-α羟化酶缺陷",
"start_offset": 3,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "肾脏1-α羟化酶",
"start_offset": 13,
"end_offset": 21,
"label": "bod"
},
{
"id": 2,
"entity": "肾脏1-α羟化酶活性减低",
"start_offset": 13,
"end_offset": 25,
"label": "sym"
},
{
"id": 3,
"entity": "1，25（OH）<sub>2</sub>D3</sub>",
"start_offset": 27,
"end_offset": 55,
"label": "bod"
},
{
"id": 4,
"entity": "1，25（OH）<sub>2</sub>D3</sub>合成减少",
"start_offset": 27,
"end_offset": 59,
"label": "sym"
},
{
"id": 5,
"entity": "肠道钙吸收减少",
"start_offset": 60,
"end_offset": 67,
"label": "sym"
},
{
"id": 6,
"entity": "尿磷",
"start_offset": 68,
"end_offset": 70,
"label": "bod"
},
{
"id": 7,
"entity": "尿磷排出增加",
"start_offset": 68,
"end_offset": 74,
"label": "sym"
},
{
"id": 8,
"entity": "甲状旁腺",
"start_offset": 82,
"end_offset": 86,
"label": "bod"
},
{
"id": 9,
"entity": "继发性甲状旁腺功能亢进",
"start_offset": 79,
"end_offset": 90,
"label": "sym"
},
{
"id": 10,
"entity": "甲状旁腺素",
"start_offset": 91,
"end_offset": 96,
"label": "bod"
},
{
"id": 11,
"entity": "PTH",
"start_offset": 97,
"end_offset": 100,
"label": "bod"
},
{
"id": 12,
"entity": "甲状旁腺素（PTH）增加",
"start_offset": 91,
"end_offset": 103,
"label": "sym"
},
{
"id": 13,
"entity": "尿磷",
"start_offset": 106,
"end_offset": 108,
"label": "bod"
},
{
"id": 14,
"entity": "加重尿磷排出",
"start_offset": 104,
"end_offset": 110,
"label": "sym"
},
{
"id": 15,
"entity": "骨钙化不全",
"start_offset": 115,
"end_offset": 120,
"label": "sym"
},
{
"id": 16,
"entity": "佝偻病",
"start_offset": 123,
"end_offset": 126,
"label": "dis"
},
{
"id": 17,
"entity": "骨软化症",
"start_offset": 127,
"end_offset": 131,
"label": "dis"
}
] |
患儿多在1岁半出现症状，常表现为生长发育障碍，身材矮小；骨骼呈佝偻病样表现，特别是下肢短小和畸形。 | [
{
"id": 0,
"entity": "生长发育障碍",
"start_offset": 16,
"end_offset": 22,
"label": "sym"
},
{
"id": 1,
"entity": "身材矮小",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
},
{
"id": 2,
"entity": "骨骼",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "骨骼呈佝偻病样",
"start_offset": 28,
"end_offset": 35,
"label": "sym"
},
{
"id": 4,
"entity": "下肢",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 5,
"entity": "下肢短小和畸形",
"start_offset": 41,
"end_offset": 48,
"label": "sym"
}
] |
成人则发生骨软化症。 | [
{
"id": 0,
"entity": "骨软化症",
"start_offset": 5,
"end_offset": 9,
"label": "dis"
}
] |
在较严重的病人，其临床特征与维生素D缺乏性佝偻病相同。 | [
{
"id": 0,
"entity": "维生素D缺乏性佝偻病",
"start_offset": 14,
"end_offset": 24,
"label": "dis"
}
] |
儿童在6岁左右可出现典型的佝偻病；严重骨骼畸形及侏儒症；剧烈骨痛。 | [
{
"id": 0,
"entity": "佝偻病",
"start_offset": 13,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "严重骨骼畸形",
"start_offset": 17,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "侏儒症",
"start_offset": 24,
"end_offset": 27,
"label": "dis"
},
{
"id": 3,
"entity": "剧烈骨痛",
"start_offset": 28,
"end_offset": 32,
"label": "sym"
}
] |
有些病人可因骨骼疼痛及至不能行走。 | [
{
"id": 0,
"entity": "骨骼",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "骨骼疼痛",
"start_offset": 6,
"end_offset": 10,
"label": "sym"
}
] |
小儿出现骨病前，早期常出现牙齿病变（牙折断、磨损、脱落、釉质矿质过少或发育不全）。 | [
{
"id": 0,
"entity": "骨病",
"start_offset": 4,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "牙齿",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "牙齿病变",
"start_offset": 13,
"end_offset": 17,
"label": "sym"
},
{
"id": 3,
"entity": "牙",
"start_offset": 18,
"end_offset": 19,
"label": "bod"
},
{
"id": 4,
"entity": "牙折断",
"start_offset": 18,
"end_offset": 21,
"label": "sym"
},
{
"id": 5,
"entity": "磨损",
"start_offset": 22,
"end_offset": 24,
"label": "sym"
},
{
"id": 6,
"entity": "脱落",
"start_offset": 25,
"end_offset": 27,
"label": "sym"
},
{
"id": 7,
"entity": "釉质矿质过少",
"start_offset": 28,
"end_offset": 34,
"label": "sym"
},
{
"id": 8,
"entity": "发育不全",
"start_offset": 35,
"end_offset": 39,
"label": "sym"
}
] |
维生素D治疗效果欠佳。 | [
{
"id": 0,
"entity": "维生素D",
"start_offset": 0,
"end_offset": 4,
"label": "dru"
}
] |
【实验室检查】血磷显著降低，常为0.32～0.78mmol/L（1～2.4mg/dl），24小时尿磷升高达21mg/kg以上（正常24小时12～20mg/kg）。 | [
{
"id": 0,
"entity": "血磷",
"start_offset": 7,
"end_offset": 9,
"label": "ite"
},
{
"id": 1,
"entity": "血磷显著降低",
"start_offset": 7,
"end_offset": 13,
"label": "sym"
},
{
"id": 2,
"entity": "尿磷",
"start_offset": 48,
"end_offset": 50,
"label": "ite"
},
{
"id": 3,
"entity": "24小时尿磷升高达21mg/kg以上",
"start_offset": 44,
"end_offset": 62,
"label": "sym"
}
] |
血钙正常，尿钙正常，血钙磷乘积降低，常＜30。 | [
{
"id": 0,
"entity": "血钙",
"start_offset": 0,
"end_offset": 2,
"label": "ite"
},
{
"id": 1,
"entity": "血钙正常",
"start_offset": 0,
"end_offset": 4,
"label": "sym"
},
{
"id": 2,
"entity": "尿钙",
"start_offset": 5,
"end_offset": 7,
"label": "ite"
},
{
"id": 3,
"entity": "尿钙正常",
"start_offset": 5,
"end_offset": 9,
"label": "sym"
},
{
"id": 4,
"entity": "血钙磷乘积",
"start_offset": 10,
"end_offset": 15,
"label": "ite"
},
{
"id": 5,
"entity": "血钙磷乘积降低",
"start_offset": 10,
"end_offset": 17,
"label": "sym"
}
] |
活动期血碱性磷酸酶升高，血甲状旁腺激素正常或轻度升高，血l，25-（OH）<sub>2</sub>D<sub>3</sub>多正常。 | [
{
"id": 0,
"entity": "血碱性磷酸酶",
"start_offset": 3,
"end_offset": 9,
"label": "ite"
},
{
"id": 1,
"entity": "活动期血碱性磷酸酶升高",
"start_offset": 0,
"end_offset": 11,
"label": "sym"
},
{
"id": 2,
"entity": "血甲状旁腺激素",
"start_offset": 12,
"end_offset": 19,
"label": "ite"
},
{
"id": 3,
"entity": "血甲状旁腺激素正常或轻度升高",
"start_offset": 12,
"end_offset": 26,
"label": "sym"
}
] |
骨X线表现为典型佝偻病及骨软化象征。 | [
{
"id": 0,
"entity": "骨X线",
"start_offset": 0,
"end_offset": 3,
"label": "pro"
},
{
"id": 1,
"entity": "典型佝偻病",
"start_offset": 6,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "骨软化象征",
"start_offset": 12,
"end_offset": 17,
"label": "sym"
}
] |
本病应与下述疾病作鉴别：①维生素D缺乏性佝偻病：有缺乏维生素D的病因，对常规剂量维生素D治疗后反应良好，可资鉴别。 | [
{
"id": 0,
"entity": "维生素D缺乏性佝偻病",
"start_offset": 13,
"end_offset": 23,
"label": "dis"
},
{
"id": 1,
"entity": "缺乏维生素D",
"start_offset": 25,
"end_offset": 31,
"label": "sym"
},
{
"id": 2,
"entity": "常规剂量维生素D治疗",
"start_offset": 36,
"end_offset": 46,
"label": "pro"
}
] |
此外，尿磷不增加，血甲状旁腺激素含量增加；尿cAMP升高，亦有助于鉴别。 | [
{
"id": 0,
"entity": "尿磷",
"start_offset": 3,
"end_offset": 5,
"label": "ite"
},
{
"id": 1,
"entity": "尿磷不增加",
"start_offset": 3,
"end_offset": 8,
"label": "sym"
},
{
"id": 2,
"entity": "血甲状旁腺激素",
"start_offset": 9,
"end_offset": 16,
"label": "ite"
},
{
"id": 3,
"entity": "血甲状旁腺激素含量增加",
"start_offset": 9,
"end_offset": 20,
"label": "sym"
},
{
"id": 4,
"entity": "尿cAMP",
"start_offset": 21,
"end_offset": 26,
"label": "ite"
},
{
"id": 5,
"entity": "尿cAMP升高",
"start_offset": 21,
"end_offset": 28,
"label": "sym"
}
] |
②还需注意与维生素D依赖性佝偻病、范可尼综合征、Lowe综合征、肾小管性酸中毒以及慢性肾功能不全等鉴别。 | [
{
"id": 0,
"entity": "维生素D依赖性佝偻病",
"start_offset": 6,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "范可尼综合征",
"start_offset": 17,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "Lowe综合征",
"start_offset": 24,
"end_offset": 31,
"label": "dis"
},
{
"id": 3,
"entity": "肾小管性酸中毒",
"start_offset": 32,
"end_offset": 39,
"label": "dis"
},
{
"id": 4,
"entity": "慢性肾功能不全",
"start_offset": 41,
"end_offset": 48,
"label": "dis"
}
] |
【治疗】（一）补充维生素D大剂量维生素D<sub>2</sub>，可用20000～100000U/d，或肌肉注射维生素D<sub>3</sub>25000～50000U/d。 | [
{
"id": 0,
"entity": "补充维生素D",
"start_offset": 7,
"end_offset": 13,
"label": "pro"
},
{
"id": 1,
"entity": "肌肉注射维生素D<sub>3</sub>25000～50000U/d",
"start_offset": 52,
"end_offset": 86,
"label": "pro"
}
] |
有条件应服用活性维生素D<sub>3</sub>0.5～1μg/d。 | [
{
"id": 0,
"entity": "服用活性维生素D<sub>3</sub>0.5～1μg/d",
"start_offset": 4,
"end_offset": 33,
"label": "pro"
}
] |
在治疗期间应根据病人的血钙、血磷、尿钙及骨X线征来调节剂量，防止发生高钙血症。 | [
{
"id": 0,
"entity": "血钙",
"start_offset": 11,
"end_offset": 13,
"label": "ite"
},
{
"id": 1,
"entity": "血磷",
"start_offset": 14,
"end_offset": 16,
"label": "ite"
},
{
"id": 2,
"entity": "尿钙",
"start_offset": 17,
"end_offset": 19,
"label": "ite"
},
{
"id": 3,
"entity": "骨X线征",
"start_offset": 20,
"end_offset": 24,
"label": "sym"
},
{
"id": 4,
"entity": "高钙血症",
"start_offset": 34,
"end_offset": 38,
"label": "dis"
}
] |
单独补充维生素D不能纠正低磷血症及生长迟缓。 | [
{
"id": 0,
"entity": "单独补充维生素D",
"start_offset": 0,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "低磷血症",
"start_offset": 12,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "生长迟缓",
"start_offset": 17,
"end_offset": 21,
"label": "sym"
}
] |
（二）补充磷用磷酸盐合剂20ml，每日4～5次。 | [
{
"id": 0,
"entity": "磷",
"start_offset": 5,
"end_offset": 6,
"label": "dru"
},
{
"id": 1,
"entity": "磷酸盐合剂",
"start_offset": 7,
"end_offset": 12,
"label": "dru"
}
] |
其配方为：磷酸二氢钠18g，磷酸氢二钠145g，水加至1000ml。 | [
{
"id": 0,
"entity": "磷酸二氢钠",
"start_offset": 5,
"end_offset": 10,
"label": "dru"
},
{
"id": 1,
"entity": "磷酸氢二钠",
"start_offset": 14,
"end_offset": 19,
"label": "dru"
}
] |
补给磷后可减少维生素D用量，口服磷和1，25（OH）<sub>2</sub>D<sub>3</sub>可使近90%的病例骨痛明显减轻。 | [
{
"id": 0,
"entity": "磷",
"start_offset": 2,
"end_offset": 3,
"label": "dru"
},
{
"id": 1,
"entity": "维生素D",
"start_offset": 7,
"end_offset": 11,
"label": "dru"
},
{
"id": 2,
"entity": "磷",
"start_offset": 16,
"end_offset": 17,
"label": "dru"
},
{
"id": 3,
"entity": "骨痛明显减轻",
"start_offset": 60,
"end_offset": 66,
"label": "sym"
}
] |
（三）其他治疗给予维生素C（降低尿pH）及钙剂可加强肾对磷的再吸收。 | [
{
"id": 0,
"entity": "维生素C",
"start_offset": 9,
"end_offset": 13,
"label": "dru"
},
{
"id": 1,
"entity": "尿pH",
"start_offset": 16,
"end_offset": 19,
"label": "ite"
},
{
"id": 2,
"entity": "钙剂",
"start_offset": 21,
"end_offset": 23,
"label": "dru"
},
{
"id": 3,
"entity": "肾",
"start_offset": 26,
"end_offset": 27,
"label": "bod"
},
{
"id": 4,
"entity": "磷",
"start_offset": 28,
"end_offset": 29,
"label": "dru"
}
] |
骨骼畸形明显而病情已静止，X线及生化检查已正常者，于12岁以后可作矫形手术，术前2周停服维生素D，以避免高钙血症的肾损害发生。 | [
{
"id": 0,
"entity": "骨骼",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "骨骼畸形明显",
"start_offset": 0,
"end_offset": 6,
"label": "sym"
},
{
"id": 2,
"entity": "X线",
"start_offset": 13,
"end_offset": 15,
"label": "pro"
},
{
"id": 3,
"entity": "生化检查",
"start_offset": 16,
"end_offset": 20,
"label": "pro"
},
{
"id": 4,
"entity": "矫形手术",
"start_offset": 33,
"end_offset": 37,
"label": "pro"
},
{
"id": 5,
"entity": "维生素D",
"start_offset": 44,
"end_offset": 48,
"label": "dru"
},
{
"id": 6,
"entity": "高钙血症",
"start_offset": 52,
"end_offset": 56,
"label": "dis"
},
{
"id": 7,
"entity": "肾损害",
"start_offset": 57,
"end_offset": 60,
"label": "dis"
}
] |
第三节缺铁性贫血缺铁性贫血（irondeficiencyanemia，IDA）是婴幼儿时期最常见的一种贫血。 | [
{
"id": 0,
"entity": "缺铁性贫血",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "缺铁性贫血",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "irondeficiencyanemia",
"start_offset": 14,
"end_offset": 34,
"label": "dis"
},
{
"id": 3,
"entity": "IDA",
"start_offset": 35,
"end_offset": 38,
"label": "dis"
},
{
"id": 4,
"entity": "贫血",
"start_offset": 51,
"end_offset": 53,
"label": "dis"
}
] |
其发生的根本病因是体内铁缺乏致使血红蛋白合成减少而发生的一种小细胞低色素性贫血。 | [
{
"id": 0,
"entity": "血红蛋白",
"start_offset": 16,
"end_offset": 20,
"label": "bod"
},
{
"id": 1,
"entity": "小细胞低色素性贫血",
"start_offset": 30,
"end_offset": 39,
"label": "dis"
}
] |
【铁的代谢】（一）铁在体内的分布体内大部分铁主要分布在血红蛋白中，少量存在于肌红蛋白中，两者占体内铁总量的60%～70%。 | [
{
"id": 0,
"entity": "铁",
"start_offset": 1,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "铁",
"start_offset": 9,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "血红蛋白",
"start_offset": 27,
"end_offset": 31,
"label": "bod"
},
{
"id": 3,
"entity": "肌红蛋白",
"start_offset": 38,
"end_offset": 42,
"label": "bod"
}
] |
细胞色素、过氧化氢酶及血浆中运输状态中的铁，仅占极小部分。 | [
{
"id": 0,
"entity": "细胞色素",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "过氧化氢酶",
"start_offset": 5,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "血浆",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
},
{
"id": 3,
"entity": "铁",
"start_offset": 20,
"end_offset": 21,
"label": "bod"
}
] |
其余约30%～40%的铁则以铁蛋白和含铁血黄素的形式贮存于骨髓、肝、脾、淋巴结等网状内皮系统中。 | [
{
"id": 0,
"entity": "骨髓",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 1,
"entity": "肝",
"start_offset": 32,
"end_offset": 33,
"label": "bod"
},
{
"id": 2,
"entity": "脾",
"start_offset": 34,
"end_offset": 35,
"label": "bod"
},
{
"id": 3,
"entity": "淋巴结",
"start_offset": 36,
"end_offset": 39,
"label": "bod"
},
{
"id": 4,
"entity": "网状内皮系统",
"start_offset": 40,
"end_offset": 46,
"label": "bod"
}
] |
内源性铁主要来自更新破坏的红细胞。 | [
{
"id": 0,
"entity": "红细胞",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
}
] |
这种衰老红细胞经机体网状内皮系统消化降解的铁可被重新利用。 | [
{
"id": 0,
"entity": "红细胞",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "网状内皮系统",
"start_offset": 10,
"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "铁",
"start_offset": 21,
"end_offset": 22,
"label": "bod"
}
] |
此外，小儿在正常发育过程中，还可以通过消化道、皮肤、泌尿生殖道黏膜上皮细胞脱落而丢失一部分铁。 | [
{
"id": 0,
"entity": "消化道",
"start_offset": 19,
"end_offset": 22,
"label": "bod"
},
{
"id": 1,
"entity": "皮肤",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 2,
"entity": "泌尿生殖道黏膜上皮细胞",
"start_offset": 26,
"end_offset": 37,
"label": "bod"
},
{
"id": 3,
"entity": "铁",
"start_offset": 45,
"end_offset": 46,
"label": "bod"
}
] |
因此，一个15岁以内的小儿每天从食物中吸收的铁至少需要1mg才能满足铁的正平衡。 | [
{
"id": 0,
"entity": "铁",
"start_offset": 34,
"end_offset": 35,
"label": "bod"
}
] |
铁吸收的主要部位在十二指肠及空肠上段，部分借助于位于十二指肠中的几种辅助铁吸收蛋白。 | [
{
"id": 0,
"entity": "十二指肠",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 1,
"entity": "空肠",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "十二指肠",
"start_offset": 26,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "辅助铁吸收蛋白",
"start_offset": 34,
"end_offset": 41,
"label": "bod"
}
] |
适量胃酸的存在对铁的吸收颇为重要，它们能将食物中的三价铁转化成二价铁；维生素C能将三价铁还原成二价铁而有助于铁的吸收。 | [
{
"id": 0,
"entity": "胃酸",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
}
] |
由于人体铁极少排泄，因此，铁吸收的调节是维持体内铁平衡的主要机制。 | [
{
"id": 0,
"entity": "铁",
"start_offset": 4,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "铁",
"start_offset": 24,
"end_offset": 25,
"label": "bod"
}
] |
肠黏膜上皮细胞具有控制铁吸收的能力，它们可根据体内铁的需要程度来增减铁的吸收量。 | [
{
"id": 0,
"entity": "肠黏膜上皮细胞",
"start_offset": 0,
"end_offset": 7,
"label": "bod"
}
] |
铁的吸收效率因食物种类不同而异，大多数素食中的铁吸收效率较差，平均仅5%左右（1.7%～7.9%），而黄豆、肉类和血红蛋白中铁的吸收效率可高达15%～20%。 | [
{
"id": 0,
"entity": "血红蛋白",
"start_offset": 57,
"end_offset": 61,
"label": "bod"
}
] |
从食物中所吸收的铁有两种去向，其中一部分铁吸收后进入肠黏膜上皮细胞内，与其中的去铁蛋白（apoferritin）结合而形成铁蛋白；大部分吸收铁直接进入血液循环，并与血浆中的转铁蛋白（transferrin）结合而被转运。 | [
{
"id": 0,
"entity": "肠黏膜上皮细胞",
"start_offset": 26,
"end_offset": 33,
"label": "bod"
},
{
"id": 1,
"entity": "去铁蛋白",
"start_offset": 39,
"end_offset": 43,
"label": "bod"
},
{
"id": 2,
"entity": "apoferritin",
"start_offset": 44,
"end_offset": 55,
"label": "bod"
},
{
"id": 3,
"entity": "铁蛋白",
"start_offset": 61,
"end_offset": 64,
"label": "bod"
},
{
"id": 4,
"entity": "血液",
"start_offset": 75,
"end_offset": 77,
"label": "bod"
},
{
"id": 5,
"entity": "血浆",
"start_offset": 82,
"end_offset": 84,
"label": "bod"
},
{
"id": 6,
"entity": "转铁蛋白",
"start_offset": 86,
"end_offset": 90,
"label": "bod"
},
{
"id": 7,
"entity": "transferrin",
"start_offset": 91,
"end_offset": 102,
"label": "bod"
}
] |
（五）铁的转运被吸收的二价铁进入血液循环后又被氧化成三价铁，并与转铁蛋白结合。 | [
{
"id": 0,
"entity": "血液",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
},
{
"id": 1,
"entity": "转铁蛋白",
"start_offset": 32,
"end_offset": 36,
"label": "bod"
}
] |
通常，二分子Fe3+</sup>需一分子的转铁蛋白。 | [
{
"id": 0,
"entity": "转铁蛋白",
"start_offset": 21,
"end_offset": 25,
"label": "bod"
}
] |
与转铁蛋白相结合的铁称为血清铁（serumiron，SI）。 | [
{
"id": 0,
"entity": "转铁蛋白",
"start_offset": 1,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "血清铁",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "serumiron",
"start_offset": 16,
"end_offset": 25,
"label": "bod"
},
{
"id": 3,
"entity": "SI",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
}
] |
SI被转运至骨髓幼红细胞和网织红细胞胞质内，约80%～90%进入幼红细胞的铁被线粒体摄取，并与原卟啉Ⅸ结合形成血红素，血红素再与珠蛋白结合而成血红蛋白。 | [
{
"id": 0,
"entity": "SI",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "骨髓幼红细胞",
"start_offset": 6,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "网织红细胞",
"start_offset": 13,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "胞质",
"start_offset": 18,
"end_offset": 20,
"label": "bod"
},
{
"id": 4,
"entity": "幼红细胞",
"start_offset": 32,
"end_offset": 36,
"label": "bod"
},
{
"id": 5,
"entity": "铁",
"start_offset": 37,
"end_offset": 38,
"label": "bod"
},
{
"id": 6,
"entity": "线粒体",
"start_offset": 39,
"end_offset": 42,
"label": "bod"
},
{
"id": 7,
"entity": "原卟啉Ⅸ",
"start_offset": 47,
"end_offset": 51,
"label": "bod"
},
{
"id": 8,
"entity": "血红素",
"start_offset": 55,
"end_offset": 58,
"label": "bod"
},
{
"id": 9,
"entity": "血红素",
"start_offset": 59,
"end_offset": 62,
"label": "bod"
},
{
"id": 10,
"entity": "珠蛋白",
"start_offset": 64,
"end_offset": 67,
"label": "bod"
},
{
"id": 11,
"entity": "血红蛋白",
"start_offset": 71,
"end_offset": 75,
"label": "bod"
}
] |
转铁蛋白存在于血浆β球蛋白组分中，是一种糖蛋白，主要在肝脏中合成，在465nm波长处有最大吸收峰。 | [
{
"id": 0,
"entity": "转铁蛋白",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "血浆",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "β球蛋白",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 3,
"entity": "糖蛋白",
"start_offset": 20,
"end_offset": 23,
"label": "bod"
},
{
"id": 4,
"entity": "肝脏",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
}
] |
转铁蛋白分子的表面有许多铁结合位点，在正常情况下，仅1/3的转铁蛋白铁结合位点被铁结合，换言之，转铁蛋白铁结合的饱和度仅33.3%。 | [
{
"id": 0,
"entity": "转铁蛋白分子",
"start_offset": 0,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "转铁蛋白",
"start_offset": 30,
"end_offset": 34,
"label": "bod"
},
{
"id": 2,
"entity": "转铁蛋白",
"start_offset": 48,
"end_offset": 52,
"label": "bod"
}
] |
转铁蛋白的主要功能是为骨髓造红细胞提供原料。 | [
{
"id": 0,
"entity": "转铁蛋白",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "骨髓造红细胞",
"start_offset": 11,
"end_offset": 17,
"label": "bod"
}
] |
它们将所结合的铁通过位于幼红细胞膜上转铁蛋白受体（TfR）的协助下转入幼红细胞和网织红细胞内后，在胞质低pH（5.5）条件下，转铁蛋白迅速释放出铁，而转铁蛋白本身又回到血浆中重新执行运铁的功能，如此循环往复，周而复始。 | [
{
"id": 0,
"entity": "幼红细胞膜",
"start_offset": 12,
"end_offset": 17,
"label": "bod"
},
{
"id": 1,
"entity": "转铁蛋白受体",
"start_offset": 18,
"end_offset": 24,
"label": "bod"
},
{
"id": 2,
"entity": "TfR",
"start_offset": 25,
"end_offset": 28,
"label": "bod"
},
{
"id": 3,
"entity": "幼红细胞",
"start_offset": 35,
"end_offset": 39,
"label": "bod"
},
{
"id": 4,
"entity": "网织红细胞",
"start_offset": 40,
"end_offset": 45,
"label": "bod"
},
{
"id": 5,
"entity": "胞质",
"start_offset": 49,
"end_offset": 51,
"label": "bod"
},
{
"id": 6,
"entity": "转铁蛋白",
"start_offset": 63,
"end_offset": 67,
"label": "bod"
},
{
"id": 7,
"entity": "转铁蛋白",
"start_offset": 75,
"end_offset": 79,
"label": "bod"
},
{
"id": 8,
"entity": "血浆",
"start_offset": 84,
"end_offset": 86,
"label": "bod"
}
] |
转铁蛋白的血浆半寿期平均为9（8～10.4）天。 | [
{
"id": 0,
"entity": "转铁蛋白",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "血浆",
"start_offset": 5,
"end_offset": 7,
"label": "bod"
}
] |
血清中转铁蛋白能结合SI的总量称为总铁结合力（totalironbindingcapacity，TIBC），未被铁结合的转铁蛋白铁结合能力称为未饱和铁结合力（unboundironbindingcapacity，UIBC）。 | [
{
"id": 0,
"entity": "血清",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "转铁蛋白",
"start_offset": 3,
"end_offset": 7,
"label": "bod"
},
{
"id": 2,
"entity": "总铁结合力",
"start_offset": 17,
"end_offset": 22,
"label": "ite"
},
{
"id": 3,
"entity": "totalironbindingcapacity",
"start_offset": 23,
"end_offset": 47,
"label": "ite"
},
{
"id": 4,
"entity": "TIBC",
"start_offset": 48,
"end_offset": 52,
"label": "ite"
},
{
"id": 5,
"entity": "转铁蛋白",
"start_offset": 60,
"end_offset": 64,
"label": "bod"
},
{
"id": 6,
"entity": "未饱和铁结合力",
"start_offset": 71,
"end_offset": 78,
"label": "ite"
},
{
"id": 7,
"entity": "unboundironbindingcapacity",
"start_offset": 79,
"end_offset": 105,
"label": "ite"
},
{
"id": 8,
"entity": "UIBC",
"start_offset": 106,
"end_offset": 110,
"label": "ite"
}
] |
因此，这三者之间的关系可用一个公式（TIBC=SI+UIBC）来表示。 | [
{
"id": 0,
"entity": "TIBC",
"start_offset": 18,
"end_offset": 22,
"label": "ite"
},
{
"id": 1,
"entity": "SI",
"start_offset": 23,
"end_offset": 25,
"label": "ite"
},
{
"id": 2,
"entity": "UIBC",
"start_offset": 26,
"end_offset": 30,
"label": "ite"
}
] |
幼红细胞中未被利用的铁以小粒的形式存在于胞质中，亚铁氰化钾可将其染成蓝色。 | [
{
"id": 0,
"entity": "幼红细胞",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "胞质",
"start_offset": 20,
"end_offset": 22,
"label": "bod"
},
{
"id": 2,
"entity": "亚铁氰化钾",
"start_offset": 24,
"end_offset": 29,
"label": "bod"
}
] |
在缺铁情况下，幼红细胞中的铁小粒显著减少甚至消失，而在体内贮存铁增多时，幼红细胞中铁小粒也增多。 | [
{
"id": 0,
"entity": "缺铁",
"start_offset": 1,
"end_offset": 3,
"label": "sym"
},
{
"id": 1,
"entity": "幼红细胞",
"start_offset": 7,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "幼红细胞中的铁小粒显著减少甚至消失",
"start_offset": 7,
"end_offset": 24,
"label": "sym"
},
{
"id": 3,
"entity": "幼红细胞",
"start_offset": 36,
"end_offset": 40,
"label": "bod"
},
{
"id": 4,
"entity": "幼红细胞中铁小粒也增多",
"start_offset": 36,
"end_offset": 47,
"label": "sym"
}
] |
含有铁粒的幼红细胞称为铁粒幼细胞。 | [
{
"id": 0,
"entity": "幼红细胞",
"start_offset": 5,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "铁粒幼细胞",
"start_offset": 11,
"end_offset": 16,
"label": "bod"
}
] |
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