text stringlengths 4 4.87k | entities list |
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真菌细胞壁内含有固醇的细胞膜，是两性霉素B、制霉菌素、丙烯胺类和唑类抗真菌药物的作用场所。 | [
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"id": 3,
"entity": "唑类抗真菌药物",
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1.多烯类抗生素主要有两性霉素B、制霉菌素等。 | [
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儿童剂量为0.5～1mg/（kg•d）；静脉注射时常见的药物反应有：高热、寒战、恶心、呕吐、食欲缺乏、静脉炎、低血压及心脏毒性；长期应用会出现低钾血症、低镁血症、贫血和肾毒性；大剂量时可致肾小管坏死、钙化。 | [
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"entity": "恶心",
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"entity": "呕吐",
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"id": 5,
"entity": "食欲缺乏",
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"entity": "静脉炎",
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"entity": "心脏",
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"id": 9,
"entity": "心脏毒性",
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"id": 10,
"entity": "低钾血症",
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"entity": "低镁血症",
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"entity": "贫血",
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"entity": "肾毒性",
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"entity": "肾小管",
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"entity": "肾小管坏死",
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"entity": "钙化",
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而两性霉素B脂质体既能降低毒性又不降低抗菌作用，已经试用于儿童。 | [
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两性霉素B脂质复合物3～5mg/（kg•d）。 | [
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（2）制霉菌素：具广谱抗真菌作用，对念珠菌属的抗菌活性尤为明显，新型隐球菌和曲霉菌对该药也敏感。 | [
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"entity": "曲霉菌",
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2.唑类抗生素包含咪唑类和三唑类，能干扰真菌细胞膜的合成，常用的有氟康唑、咪康唑、克霉唑、酮康唑、伊曲康唑、伏立康唑等。 | [
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"entity": "氟康唑",
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"entity": "克霉唑",
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"entity": "伊曲康唑",
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"entity": "伏立康唑",
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口服制剂6～8mg/（kg•d），分2次服用。 | [
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2～12岁：每次7mg/kg，每12小时1次，静脉注射；或第1天6mg/kg，每12小时1次，静脉注射，随后每次4mg/kg，每12小时1次，静脉注射。 | [
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口服剂量：＜40kg，100mg/次，每12小时1次；＞40kg，200mg/次，每12小时1次。 | [
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3.棘白菌素类卡泊芬净：适应证为念珠菌和曲霉感染，对隐球菌、镰刀霉菌属以及毛霉菌属无活性。 | [
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"entity": "毛霉菌",
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抗真菌治疗的时间长短，因病情而异，患侵袭性肺部真菌病的患儿一般均在免疫功能低下的情况下发病，给药时间不宜过短，一般要6～12周，甚至更长，一般治疗至临床证候消失，影像学示病变基本吸收。 | [
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第二节临床资料的整理和分析（一）资料归纳将病史问诊、体格检查和各项辅助检查的结果进行整理，去粗存精，有条理、系统地进行归类并列出条目。 | [
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要求有高度的概括性，围绕主诉、突出重点，将主要症状的特点、体格检查阳性发现及重要的阴性体征、实验室检查的异常结果列出条目。 | [
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以下是一病例临床归纳的特点：1.男性，1岁；2.持续发热2周伴不规则皮疹；3.咽充血双侧扁桃体Ⅱ度肿大颈部浅表淋巴结轻度肿大肝中度肿大，脾轻度肿大；6.外周血象白细胞总数正常淋巴细胞为主，轻度贫血，血小板计数正常尿常规正常；7.一般情况可，无头痛呕吐，无咳嗽气急，无腹痛、腹泻，无尿频、尿急、尿痛。 | [
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"entity": "白细胞总数",
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"entity": "外周血象白细胞总数正常",
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"entity": "淋巴细胞",
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"id": 14,
"entity": "血小板计数",
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"entity": "血小板计数正常",
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"id": 16,
"entity": "尿常规正常",
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"id": 17,
"entity": "头痛",
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"id": 18,
"entity": "呕吐",
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"id": 19,
"entity": "咳嗽气急",
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"label": "sym"
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{
"id": 20,
"entity": "腹痛",
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{
"id": 21,
"entity": "腹泻",
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{
"id": 22,
"entity": "尿频",
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"id": 23,
"entity": "尿急",
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"label": "sym"
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{
"id": 24,
"entity": "尿痛",
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而这个初步印象会在接下来的问诊、体格检查过程中起一定的导向作用。 | [
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以此为基础，进一步收集临床资料如辅助检查，尤其是一些具有特异性诊断价值的项目，以确诊或排除。 | [
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在儿科疾病诊断的临床思维过程中，具体还应注意下列问题：1.首先考虑常见病儿科疾病谱中，先天性、遗传性和感染性疾病占较大比例，在诊断时应首先考虑。 | [
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如遇发热待查患儿，病因有很多，如感染、结缔组织病、恶性肿瘤及血液病、变态反应性疾病、体温中枢病变或调节失常、组织破坏与吸收、代谢和内分泌失调等。 | [
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"id": 4,
"entity": "血液病",
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"label": "dis"
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"id": 5,
"entity": "变态反应性疾病",
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"label": "dis"
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{
"id": 6,
"entity": "体温中枢病变",
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"label": "dis"
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"id": 7,
"entity": "调节失常",
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"label": "dis"
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"entity": "组织破坏",
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"label": "dis"
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"id": 9,
"entity": "内分泌失调",
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"label": "dis"
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但婴幼儿由于免疫功能低下，以感染性疾病最为常见，故诊断思路应首先想到感染性疾病。 | [
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在病原方面，也应多考虑常见的细菌或病毒，其次再考虑支原体、衣原体、真菌、寄生虫。 | [
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然后通过一系列的实验室检查，如外周血象、C-反应蛋白、血培养、血清学检查、分子生物学等方法来证实推断。 | [
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如惊厥是儿科的常见症状之一，如果发生于新生儿，首先考虑围生期因素或代谢异常，如缺氧缺血性脑病、颅内出血、低血糖、低血钙等。 | [
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{
"id": 3,
"entity": "低血糖",
"start_offset": 52,
"end_offset": 55,
"label": "dis"
},
{
"id": 4,
"entity": "低血钙",
"start_offset": 56,
"end_offset": 59,
"label": "dis"
}
] |
如果发生于小婴儿，首先考虑颅内感染、热性惊厥等。 | [
{
"id": 0,
"entity": "颅内感染",
"start_offset": 13,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "热性惊厥",
"start_offset": 18,
"end_offset": 22,
"label": "dis"
}
] |
如果是较大儿童，多考虑脑炎、癫痫等。 | [
{
"id": 0,
"entity": "脑炎",
"start_offset": 11,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "癫痫",
"start_offset": 14,
"end_offset": 16,
"label": "dis"
}
] |
3.切忌生搬硬套有些疾病缺乏特异性的实验室检查，而依靠一些非特异性的临床及辅助检查指标来进行诊断。 | [
{
"id": 0,
"entity": "实验室检查",
"start_offset": 18,
"end_offset": 23,
"label": "pro"
},
{
"id": 1,
"entity": "辅助检查",
"start_offset": 37,
"end_offset": 41,
"label": "pro"
}
] |
4.重视典型临床表现的积累图2-1发绀的鉴别诊断有些疾病凭外观直觉就立即能作出诊断，如21-三体综合征有特殊的面容，过敏性紫癜有典型的皮肤表现；另外可以通过关联思维来获得诊断，如新生儿有阴茎短小并伴有低血糖，很容易想到先天性垂体功能低下的诊断。 | [
{
"id": 0,
"entity": "阴茎短小",
"start_offset": 93,
"end_offset": 97,
"label": "dis"
},
{
"id": 1,
"entity": "低血糖",
"start_offset": 100,
"end_offset": 103,
"label": "dis"
},
{
"id": 2,
"entity": "先天性垂体功能低下",
"start_offset": 109,
"end_offset": 118,
"label": "dis"
}
] |
它将关键的临床表现和辅助检查按顺序及逻辑关系进行排列，形成流程表。 | [
{
"id": 0,
"entity": "辅助检查",
"start_offset": 10,
"end_offset": 14,
"label": "pro"
}
] |
6.注意诊断的全面性及完整性诊断必须全面完整的诊断应包括主要诊断：系统器官定位（肺、肝）、性质（炎症、出血）、病程（如急性、慢性）、可能的病原（细菌性、支原体）、病理（如支气管肺炎、大叶性肺炎）、病情程度（轻、重）以及并发症（脓胸、气胸）、功能诊断（如呼吸衰竭）等。 | [
{
"id": 0,
"entity": "肺",
"start_offset": 40,
"end_offset": 41,
"label": "bod"
},
{
"id": 1,
"entity": "肝",
"start_offset": 42,
"end_offset": 43,
"label": "bod"
},
{
"id": 2,
"entity": "炎症",
"start_offset": 48,
"end_offset": 50,
"label": "dis"
},
{
"id": 3,
"entity": "出血",
"start_offset": 51,
"end_offset": 53,
"label": "dis"
},
{
"id": 4,
"entity": "支原体",
"start_offset": 76,
"end_offset": 79,
"label": "mic"
},
{
"id": 5,
"entity": "支气管肺炎",
"start_offset": 85,
"end_offset": 90,
"label": "dis"
},
{
"id": 6,
"entity": "大叶性肺炎",
"start_offset": 91,
"end_offset": 96,
"label": "dis"
},
{
"id": 7,
"entity": "脓胸",
"start_offset": 113,
"end_offset": 115,
"label": "dis"
},
{
"id": 8,
"entity": "气胸",
"start_offset": 116,
"end_offset": 118,
"label": "dis"
},
{
"id": 9,
"entity": "呼吸衰竭",
"start_offset": 126,
"end_offset": 130,
"label": "dis"
}
] |
有时还有次要诊断如贫血、血小板减少症等，都应完整列出。 | [
{
"id": 0,
"entity": "贫血",
"start_offset": 9,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "血小板减少症",
"start_offset": 12,
"end_offset": 18,
"label": "dis"
}
] |
二、阻力呼吸系统阻力从物理性质可分为三种：弹性阻力、黏性阻力、惯性阻力，三者之和为呼吸总阻抗；呼吸总阻抗可用强迫振荡法测定。 | [
{
"id": 0,
"entity": "呼吸系统阻力",
"start_offset": 4,
"end_offset": 10,
"label": "ite"
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{
"id": 1,
"entity": "弹性阻力",
"start_offset": 21,
"end_offset": 25,
"label": "ite"
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{
"id": 2,
"entity": "黏性阻力",
"start_offset": 26,
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"label": "ite"
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{
"id": 3,
"entity": "惯性阻力",
"start_offset": 31,
"end_offset": 35,
"label": "ite"
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{
"id": 4,
"entity": "呼吸总阻抗",
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"end_offset": 46,
"label": "ite"
},
{
"id": 5,
"entity": "呼吸总阻抗",
"start_offset": 47,
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"label": "ite"
},
{
"id": 6,
"entity": "强迫振荡法",
"start_offset": 54,
"end_offset": 59,
"label": "ite"
}
] |
按部位可分为：气道阻力、肺阻力和胸廓阻力。 | [
{
"id": 0,
"entity": "气道阻力",
"start_offset": 7,
"end_offset": 11,
"label": "ite"
},
{
"id": 1,
"entity": "肺阻力",
"start_offset": 12,
"end_offset": 15,
"label": "ite"
},
{
"id": 2,
"entity": "胸廓阻力",
"start_offset": 16,
"end_offset": 20,
"label": "ite"
}
] |
气道阻力是指气道的黏性阻力，是单位流量所需的压力差（R=△P/V）。 | [
{
"id": 0,
"entity": "气道阻力",
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"label": "ite"
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{
"id": 1,
"entity": "气道",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
}
] |
气道阻力取决于管径大小和气体流速，管道气流与管腔半径的4次方成反比，故小儿气道阻力大于成人。 | [
{
"id": 0,
"entity": "气道阻力",
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"label": "ite"
},
{
"id": 1,
"entity": "气道阻力",
"start_offset": 37,
"end_offset": 41,
"label": "ite"
}
] |
成人气道阻力一般在1～3cmH<sub>2</sub>O/（L•s），婴幼儿气道较狭窄，其阻力较高，约为成人的10倍。 | [
{
"id": 0,
"entity": "气道阻力",
"start_offset": 2,
"end_offset": 6,
"label": "ite"
},
{
"id": 1,
"entity": "气道",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
}
] |
气道管径随发育而增大，阻力随年龄而递减。 | [
{
"id": 0,
"entity": "气道",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
}
] |
婴幼儿肺炎时，气道管腔黏膜肿胀，分泌物增加，支气管痉挛，故管腔更为狭小，气道阻力增加。 | [
{
"id": 0,
"entity": "肺炎",
"start_offset": 3,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "气道管腔",
"start_offset": 7,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "气道管腔黏膜肿胀",
"start_offset": 7,
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"label": "sym"
},
{
"id": 3,
"entity": "分泌物增加",
"start_offset": 16,
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"label": "sym"
},
{
"id": 4,
"entity": "支气管",
"start_offset": 22,
"end_offset": 25,
"label": "bod"
},
{
"id": 5,
"entity": "支气管痉挛",
"start_offset": 22,
"end_offset": 27,
"label": "sym"
},
{
"id": 6,
"entity": "管腔",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 7,
"entity": "气道阻力",
"start_offset": 36,
"end_offset": 40,
"label": "ite"
}
] |
七、pH监测采用柔软的pH电极（儿科常用单晶锑电极），放置在食管或胃中监测pH，数据存储在可携带的pH记录仪上，可持续监测24～96小时，由电脑进行数据处理。 | [
{
"id": 0,
"entity": "pH监测",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "pH电极",
"start_offset": 11,
"end_offset": 15,
"label": "equ"
},
{
"id": 2,
"entity": "儿科",
"start_offset": 16,
"end_offset": 18,
"label": "dep"
},
{
"id": 3,
"entity": "单晶锑电极",
"start_offset": 20,
"end_offset": 25,
"label": "equ"
},
{
"id": 4,
"entity": "食管",
"start_offset": 30,
"end_offset": 32,
"label": "bod"
},
{
"id": 5,
"entity": "胃",
"start_offset": 33,
"end_offset": 34,
"label": "bod"
},
{
"id": 6,
"entity": "pH记录仪",
"start_offset": 49,
"end_offset": 54,
"label": "equ"
},
{
"id": 7,
"entity": "电脑",
"start_offset": 70,
"end_offset": 72,
"label": "equ"
}
] |
目前常用DigitrapperMK型pH监测仪。 | [
{
"id": 0,
"entity": "DigitrapperMK型pH监测仪",
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"label": "equ"
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] |
（一）食管pH监测检查前禁食4～6小时，检查时将pH电极准确置于下端食道括约肌上缘以上3cm处，受检时照常进食，但忌酸性饮料和食物，并记录进餐、体位变化的起止时间和症状发生的时间，不限制活动，力求接近生理状态。 | [
{
"id": 0,
"entity": "食管pH监测",
"start_offset": 3,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "pH电极",
"start_offset": 24,
"end_offset": 28,
"label": "equ"
},
{
"id": 2,
"entity": "下端食道括约肌上缘",
"start_offset": 32,
"end_offset": 41,
"label": "bod"
}
] |
食管pH监测主要用于胃食管反流病（GERD）的诊断，判断GERD治疗效果。 | [
{
"id": 0,
"entity": "食管pH监测",
"start_offset": 0,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "胃食管反流病",
"start_offset": 10,
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"label": "dis"
},
{
"id": 2,
"entity": "GERD",
"start_offset": 17,
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"label": "dis"
},
{
"id": 3,
"entity": "GERD",
"start_offset": 28,
"end_offset": 32,
"label": "dis"
}
] |
可以发现反流，区分生理性反流还是病理性反流，了解反流与症状、体位进食的关系。 | [
{
"id": 0,
"entity": "反流",
"start_offset": 4,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "生理性反流",
"start_offset": 9,
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"label": "dis"
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{
"id": 2,
"entity": "病理性反流",
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"label": "dis"
},
{
"id": 3,
"entity": "反流",
"start_offset": 24,
"end_offset": 26,
"label": "dis"
}
] |
（二）胃pH监测将pH电极置于下端食管括约肌下5cm，主要监测胃pH值平均数，胃pH中位数，胃pH值＞1、＞2、＞3、＞4的时间百分比，以及胃pH值密度分布。 | [
{
"id": 0,
"entity": "胃pH监测",
"start_offset": 3,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "pH电极",
"start_offset": 9,
"end_offset": 13,
"label": "equ"
},
{
"id": 2,
"entity": "下端食管括约肌",
"start_offset": 15,
"end_offset": 22,
"label": "bod"
},
{
"id": 3,
"entity": "胃",
"start_offset": 31,
"end_offset": 32,
"label": "bod"
},
{
"id": 4,
"entity": "胃",
"start_offset": 39,
"end_offset": 40,
"label": "bod"
},
{
"id": 5,
"entity": "胃pH值",
"start_offset": 46,
"end_offset": 50,
"label": "ite"
},
{
"id": 6,
"entity": "胃",
"start_offset": 70,
"end_offset": 71,
"label": "bod"
}
] |
主要用于评价酸相关性疾病的疗效，以及探讨消化性溃疡及胃泌素瘤等疾病的发病机制和愈合过程与胃酸变化的关系，对检测十二指肠胃反流有帮助。 | [
{
"id": 0,
"entity": "消化性溃疡",
"start_offset": 20,
"end_offset": 25,
"label": "dis"
},
{
"id": 1,
"entity": "胃泌素瘤",
"start_offset": 26,
"end_offset": 30,
"label": "dis"
},
{
"id": 2,
"entity": "十二指肠胃反流",
"start_offset": 55,
"end_offset": 62,
"label": "dis"
}
] |
第二节范可尼综合征Fanconi（范可尼）于1931年首先描述1例小儿有蛋白尿、非糖尿病性葡萄糖尿、生长迟缓伴低血磷性佝偻病，此综合征因此得名。 | [
{
"id": 0,
"entity": "范可尼综合征",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "范可尼",
"start_offset": 17,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "蛋白尿",
"start_offset": 36,
"end_offset": 39,
"label": "sym"
},
{
"id": 3,
"entity": "非糖尿病性葡萄糖尿",
"start_offset": 40,
"end_offset": 49,
"label": "sym"
},
{
"id": 4,
"entity": "生长迟缓",
"start_offset": 50,
"end_offset": 54,
"label": "sym"
},
{
"id": 5,
"entity": "低血磷性佝偻病",
"start_offset": 55,
"end_offset": 62,
"label": "dis"
}
] |
本病以多种肾小管功能紊乱为特征，导致氨基酸、葡萄糖、磷酸盐、碳酸氢盐和其他由近端或远端肾小管处的有机物或无机物从尿中丢失过多，因而出现酸中毒、低磷酸盐血症、低钙血症、脱水、佝偻病、骨质疏松以及生长过缓等表现。 | [
{
"id": 0,
"entity": "肾小管功能紊乱",
"start_offset": 5,
"end_offset": 12,
"label": "sym"
},
{
"id": 1,
"entity": "氨基酸",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 2,
"entity": "葡萄糖",
"start_offset": 22,
"end_offset": 25,
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},
{
"id": 3,
"entity": "磷酸盐",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
},
{
"id": 4,
"entity": "碳酸氢盐",
"start_offset": 30,
"end_offset": 34,
"label": "bod"
},
{
"id": 5,
"entity": "近端或远端肾小管",
"start_offset": 38,
"end_offset": 46,
"label": "bod"
},
{
"id": 6,
"entity": "氨基酸、葡萄糖、磷酸盐、碳酸氢盐和其他由近端或远端肾小管处的有机物或无机物从尿中丢失过多",
"start_offset": 18,
"end_offset": 62,
"label": "sym"
},
{
"id": 7,
"entity": "酸中毒",
"start_offset": 67,
"end_offset": 70,
"label": "dis"
},
{
"id": 8,
"entity": "低磷酸盐血症",
"start_offset": 71,
"end_offset": 77,
"label": "dis"
},
{
"id": 9,
"entity": "低钙血症",
"start_offset": 78,
"end_offset": 82,
"label": "dis"
},
{
"id": 10,
"entity": "脱水",
"start_offset": 83,
"end_offset": 85,
"label": "sym"
},
{
"id": 11,
"entity": "佝偻病",
"start_offset": 86,
"end_offset": 89,
"label": "dis"
},
{
"id": 12,
"entity": "骨质疏松",
"start_offset": 90,
"end_offset": 94,
"label": "dis"
},
{
"id": 13,
"entity": "生长过缓",
"start_offset": 96,
"end_offset": 100,
"label": "sym"
}
] |
【病因和分类】幼儿大多与遗传有关，年长儿多继发于免疫性疾病、毒物或药物中毒以及各种肾脏病。 | [
{
"id": 0,
"entity": "免疫性疾病",
"start_offset": 24,
"end_offset": 29,
"label": "dis"
},
{
"id": 1,
"entity": "毒物或药物中毒",
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"label": "dis"
},
{
"id": 2,
"entity": "肾脏病",
"start_offset": 41,
"end_offset": 44,
"label": "dis"
}
] |
（一）原发性（原因不明或无全身性疾病）包括遗传性［常染色体显性（AD）、常染色体隐性（AR）及X连锁隐性（XLR）］、散发性及特殊型（即刷状缘缺失型）。 | [
{
"id": 0,
"entity": "常染色体显性",
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"label": "dis"
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{
"id": 1,
"entity": "AD",
"start_offset": 32,
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{
"id": 2,
"entity": "常染色体隐性",
"start_offset": 36,
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"label": "dis"
},
{
"id": 3,
"entity": "AR",
"start_offset": 43,
"end_offset": 45,
"label": "dis"
},
{
"id": 4,
"entity": "X连锁隐性",
"start_offset": 47,
"end_offset": 52,
"label": "dis"
},
{
"id": 5,
"entity": "XLR",
"start_offset": 53,
"end_offset": 56,
"label": "dis"
},
{
"id": 6,
"entity": "刷状缘缺失型",
"start_offset": 68,
"end_offset": 74,
"label": "dis"
}
] |
（二）继发性（症状型）1.先天性代谢障碍（1）氨基酸代谢障碍：①胱氨酸病（常染色体隐性，AR）；②酪氨酸血症Ⅰ型（AR）；③Busby综合征（AR）；④Ludersheldon综合征（AD）。 | [
{
"id": 0,
"entity": "先天性代谢障碍",
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"end_offset": 20,
"label": "dis"
},
{
"id": 1,
"entity": "氨基酸代谢障碍",
"start_offset": 23,
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"label": "dis"
},
{
"id": 2,
"entity": "胱氨酸病",
"start_offset": 32,
"end_offset": 36,
"label": "dis"
},
{
"id": 3,
"entity": "常染色体隐性",
"start_offset": 37,
"end_offset": 43,
"label": "dis"
},
{
"id": 4,
"entity": "AR",
"start_offset": 44,
"end_offset": 46,
"label": "dis"
},
{
"id": 5,
"entity": "酪氨酸血症Ⅰ型",
"start_offset": 49,
"end_offset": 56,
"label": "dis"
},
{
"id": 6,
"entity": "AR",
"start_offset": 57,
"end_offset": 59,
"label": "dis"
},
{
"id": 7,
"entity": "Busby综合征",
"start_offset": 62,
"end_offset": 70,
"label": "dis"
},
{
"id": 8,
"entity": "AR",
"start_offset": 71,
"end_offset": 73,
"label": "dis"
},
{
"id": 9,
"entity": "Ludersheldon综合征",
"start_offset": 76,
"end_offset": 91,
"label": "dis"
},
{
"id": 10,
"entity": "AD",
"start_offset": 92,
"end_offset": 94,
"label": "dis"
}
] |
（2）碳水化合物代谢障碍：①糖原累积病Ⅰ型（Fanconi-Bickel综合征，AR）；②半乳糖血症（AR）；③遗传性果糖不耐受症（AR）。 | [
{
"id": 0,
"entity": "碳水化合物代谢障碍",
"start_offset": 3,
"end_offset": 12,
"label": "sym"
},
{
"id": 1,
"entity": "糖原累积病Ⅰ型",
"start_offset": 14,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "Fanconi-Bickel综合征",
"start_offset": 22,
"end_offset": 39,
"label": "dis"
},
{
"id": 3,
"entity": "AR",
"start_offset": 40,
"end_offset": 42,
"label": "dis"
},
{
"id": 4,
"entity": "半乳糖血症",
"start_offset": 45,
"end_offset": 50,
"label": "dis"
},
{
"id": 5,
"entity": "AR",
"start_offset": 51,
"end_offset": 53,
"label": "dis"
},
{
"id": 6,
"entity": "遗传性果糖不耐受症",
"start_offset": 56,
"end_offset": 65,
"label": "dis"
},
{
"id": 7,
"entity": "AR",
"start_offset": 66,
"end_offset": 68,
"label": "dis"
}
] |
（3）其他：①Lowe综合征（XLR）；②肝豆状核变性（AR）；③细胞色素C氧化酶缺陷（AR）；④Dent病（家族性近端肾小管疾病，XLR）；⑤Pearson综合征；⑥Wilson病；⑦维生素B12</sub>缺乏。 | [
{
"id": 0,
"entity": "Lowe综合征",
"start_offset": 7,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "XLR",
"start_offset": 15,
"end_offset": 18,
"label": "dis"
},
{
"id": 2,
"entity": "肝豆状核变性",
"start_offset": 21,
"end_offset": 27,
"label": "sym"
},
{
"id": 3,
"entity": "AR",
"start_offset": 28,
"end_offset": 30,
"label": "sym"
},
{
"id": 4,
"entity": "细胞色素C氧化酶",
"start_offset": 33,
"end_offset": 41,
"label": "bod"
},
{
"id": 5,
"entity": "细胞色素C氧化酶缺陷",
"start_offset": 33,
"end_offset": 43,
"label": "sym"
},
{
"id": 6,
"entity": "AR",
"start_offset": 44,
"end_offset": 46,
"label": "sym"
},
{
"id": 7,
"entity": "Dent病",
"start_offset": 49,
"end_offset": 54,
"label": "dis"
},
{
"id": 8,
"entity": "家族性近端肾小管疾病",
"start_offset": 55,
"end_offset": 65,
"label": "dis"
},
{
"id": 9,
"entity": "XLR",
"start_offset": 66,
"end_offset": 69,
"label": "dis"
},
{
"id": 10,
"entity": "Pearson综合征",
"start_offset": 72,
"end_offset": 82,
"label": "dis"
},
{
"id": 11,
"entity": "Wilson病",
"start_offset": 84,
"end_offset": 91,
"label": "dis"
},
{
"id": 12,
"entity": "维生素B12</sub>",
"start_offset": 93,
"end_offset": 105,
"label": "bod"
},
{
"id": 13,
"entity": "维生素B12</sub>缺乏",
"start_offset": 93,
"end_offset": 107,
"label": "sym"
}
] |
2.获得性疾病如：①多发性骨髓瘤；②肾病综合征；③肾移植；④肿瘤；⑤糖尿病；⑥急、慢性间质性肾炎；⑦急性肾小管坏死；⑧营养不良；⑨巴尔干肾病；⑩严重低钾血症。 | [
{
"id": 0,
"entity": "多发性骨髓瘤",
"start_offset": 10,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "肾病综合征",
"start_offset": 18,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "肾移植",
"start_offset": 25,
"end_offset": 28,
"label": "dis"
},
{
"id": 3,
"entity": "肿瘤",
"start_offset": 30,
"end_offset": 32,
"label": "dis"
},
{
"id": 4,
"entity": "糖尿病",
"start_offset": 34,
"end_offset": 37,
"label": "dis"
},
{
"id": 5,
"entity": "急、慢性间质性肾炎",
"start_offset": 39,
"end_offset": 48,
"label": "dis"
},
{
"id": 6,
"entity": "急性肾小管坏死",
"start_offset": 50,
"end_offset": 57,
"label": "dis"
},
{
"id": 7,
"entity": "营养不良",
"start_offset": 59,
"end_offset": 63,
"label": "dis"
},
{
"id": 8,
"entity": "巴尔干肾病",
"start_offset": 65,
"end_offset": 70,
"label": "dis"
},
{
"id": 9,
"entity": "严重低钾血症",
"start_offset": 72,
"end_offset": 78,
"label": "dis"
}
] |
3.药物损伤及中毒如：①重金属（汞、钠、铅及镉）；②化学毒剂马来酸、来苏儿、甲苯、甲酚及硝苯等；③过期四环素及丙酸；④顺铂、异环磷酰胺（Ifosfamide）、氨基糖苷类抗生素及维生素中毒；⑤雷米替丁、西咪替丁及中草药如马兜铃肾损害等。 | [
{
"id": 0,
"entity": "药物损伤",
"start_offset": 2,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "中毒",
"start_offset": 7,
"end_offset": 9,
"label": "dis"
},
{
"id": 2,
"entity": "四环素",
"start_offset": 51,
"end_offset": 54,
"label": "dru"
},
{
"id": 3,
"entity": "丙酸",
"start_offset": 55,
"end_offset": 57,
"label": "dru"
},
{
"id": 4,
"entity": "顺铂、异环磷酰胺（Ifosfamide）、氨基糖苷类抗生素及维生素中毒",
"start_offset": 59,
"end_offset": 94,
"label": "dis"
},
{
"id": 5,
"entity": "雷米替丁",
"start_offset": 96,
"end_offset": 100,
"label": "dru"
},
{
"id": 6,
"entity": "西咪替丁",
"start_offset": 101,
"end_offset": 105,
"label": "dru"
},
{
"id": 7,
"entity": "中草药",
"start_offset": 106,
"end_offset": 109,
"label": "dru"
},
{
"id": 8,
"entity": "马兜铃肾损害",
"start_offset": 110,
"end_offset": 116,
"label": "dis"
}
] |
【发病机制】本病发病机制尚未完全清楚，有以下几种可能：①内流缺陷，管腔内向组织内流减少，见于刷状缘缺失型；②细胞内回漏到肾小管腔增加，如马来酸中毒型；③通过基底侧细胞膜回流减少，致细胞内物质堆积；影响回吸收，如Fanconi-Bickel综合征；④从血液向细胞灌注增加，通过细胞紧密连接处反流管腔增加，如细胞色素C氧化酶缺乏型。 | [
{
"id": 0,
"entity": "内流缺陷",
"start_offset": 28,
"end_offset": 32,
"label": "sym"
},
{
"id": 1,
"entity": "管腔内向组织内流减少",
"start_offset": 33,
"end_offset": 43,
"label": "sym"
},
{
"id": 2,
"entity": "刷状缘缺失型",
"start_offset": 46,
"end_offset": 52,
"label": "sym"
},
{
"id": 3,
"entity": "肾小管腔",
"start_offset": 60,
"end_offset": 64,
"label": "bod"
},
{
"id": 4,
"entity": "细胞内回漏到肾小管腔增加",
"start_offset": 54,
"end_offset": 66,
"label": "sym"
},
{
"id": 5,
"entity": "马来酸中毒型",
"start_offset": 68,
"end_offset": 74,
"label": "dis"
},
{
"id": 6,
"entity": "基底侧细胞膜回流减少",
"start_offset": 78,
"end_offset": 88,
"label": "sym"
},
{
"id": 7,
"entity": "细胞内物质堆积",
"start_offset": 90,
"end_offset": 97,
"label": "sym"
},
{
"id": 8,
"entity": "Fanconi-Bickel综合征",
"start_offset": 105,
"end_offset": 122,
"label": "dis"
},
{
"id": 9,
"entity": "血液向细胞灌注增加",
"start_offset": 125,
"end_offset": 134,
"label": "sym"
},
{
"id": 10,
"entity": "通过细胞紧密连接处反流管腔增加",
"start_offset": 135,
"end_offset": 150,
"label": "sym"
},
{
"id": 11,
"entity": "细胞色素C氧化酶缺乏型",
"start_offset": 152,
"end_offset": 163,
"label": "dis"
}
] |
肾小管膜的输送异常在病理组织学检查中未见特异性表现。 | [
{
"id": 0,
"entity": "肾小管膜的输送异常",
"start_offset": 0,
"end_offset": 9,
"label": "sym"
}
] |
有实验提示本征的细胞内ATP活性的转运功能不全是由于磷酸盐耗竭，引起细胞内腺嘌呤核苷酸降解，因而发生ATP消耗。 | [
{
"id": 0,
"entity": "细胞内ATP活性的转运功能不全",
"start_offset": 8,
"end_offset": 23,
"label": "sym"
},
{
"id": 1,
"entity": "磷酸盐耗竭",
"start_offset": 26,
"end_offset": 31,
"label": "sym"
},
{
"id": 2,
"entity": "腺嘌呤核苷酸",
"start_offset": 37,
"end_offset": 43,
"label": "bod"
},
{
"id": 3,
"entity": "细胞内腺嘌呤核苷酸降解",
"start_offset": 34,
"end_offset": 45,
"label": "sym"
}
] |
图12-1范可尼综合征病理生理与临床表现【临床表现】本病临床表现取决于肾小管功能障碍的类型和程度。 | [
{
"id": 0,
"entity": "范可尼综合征",
"start_offset": 5,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "肾小管功能障碍",
"start_offset": 35,
"end_offset": 42,
"label": "dis"
}
] |
全氨基酸尿、糖尿以及高磷酸盐尿导致低磷血症为本症的三大特征，但不完全性Fanconi综合征不是全部具备上述三个特征，往往只具备其中1、2项。 | [
{
"id": 0,
"entity": "全氨基酸尿",
"start_offset": 0,
"end_offset": 5,
"label": "sym"
},
{
"id": 1,
"entity": "糖尿",
"start_offset": 6,
"end_offset": 8,
"label": "sym"
},
{
"id": 2,
"entity": "高磷酸盐尿",
"start_offset": 10,
"end_offset": 15,
"label": "sym"
},
{
"id": 3,
"entity": "低磷血症",
"start_offset": 17,
"end_offset": 21,
"label": "dis"
},
{
"id": 4,
"entity": "不完全性Fanconi综合征",
"start_offset": 31,
"end_offset": 45,
"label": "dis"
}
] |
（一）原发性Fanconi综合征1.婴儿型也称急性型，特点有：①起病早，6～12月龄发病；②常因烦渴、多饮、多尿、脱水、消瘦、呕吐、便秘及无力而就诊；③生长迟缓及发育障碍，出现抗维生素D佝偻病及营养不良、骨质疏松甚至骨折等表现；④肾性全氨基酸尿，但血浆氨基酸正常；⑤低血钾，低血磷，碱性磷酸酶活性增高，高氯血症性代谢性酸中毒，尿中可滴定酸及可减少，尿糖微量或增多，血糖正常；⑥预后较差，可死于尿毒症性酸中毒或继发感染。 | [
{
"id": 0,
"entity": "原发性Fanconi综合征",
"start_offset": 3,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "烦渴",
"start_offset": 48,
"end_offset": 50,
"label": "sym"
},
{
"id": 2,
"entity": "多饮",
"start_offset": 51,
"end_offset": 53,
"label": "sym"
},
{
"id": 3,
"entity": "多尿",
"start_offset": 54,
"end_offset": 56,
"label": "sym"
},
{
"id": 4,
"entity": "脱水",
"start_offset": 57,
"end_offset": 59,
"label": "sym"
},
{
"id": 5,
"entity": "消瘦",
"start_offset": 60,
"end_offset": 62,
"label": "sym"
},
{
"id": 6,
"entity": "呕吐",
"start_offset": 63,
"end_offset": 65,
"label": "sym"
},
{
"id": 7,
"entity": "便秘",
"start_offset": 66,
"end_offset": 68,
"label": "sym"
},
{
"id": 8,
"entity": "无力",
"start_offset": 69,
"end_offset": 71,
"label": "sym"
},
{
"id": 9,
"entity": "生长迟缓及发育障碍",
"start_offset": 76,
"end_offset": 85,
"label": "sym"
},
{
"id": 10,
"entity": "抗维生素D佝偻病",
"start_offset": 88,
"end_offset": 96,
"label": "dis"
},
{
"id": 11,
"entity": "营养不良",
"start_offset": 97,
"end_offset": 101,
"label": "sym"
},
{
"id": 12,
"entity": "骨质疏松",
"start_offset": 102,
"end_offset": 106,
"label": "sym"
},
{
"id": 13,
"entity": "骨折",
"start_offset": 108,
"end_offset": 110,
"label": "sym"
},
{
"id": 14,
"entity": "血浆氨基酸正常",
"start_offset": 124,
"end_offset": 131,
"label": "sym"
},
{
"id": 15,
"entity": "低血钾",
"start_offset": 133,
"end_offset": 136,
"label": "sym"
},
{
"id": 16,
"entity": "低血磷",
"start_offset": 137,
"end_offset": 140,
"label": "sym"
},
{
"id": 17,
"entity": "碱性磷酸酶活性增高",
"start_offset": 141,
"end_offset": 150,
"label": "sym"
},
{
"id": 18,
"entity": "高氯血症性代谢性酸中毒",
"start_offset": 151,
"end_offset": 162,
"label": "dis"
},
{
"id": 19,
"entity": "尿糖微量或增多",
"start_offset": 174,
"end_offset": 181,
"label": "dis"
},
{
"id": 20,
"entity": "血糖正常",
"start_offset": 182,
"end_offset": 186,
"label": "dis"
},
{
"id": 21,
"entity": "尿毒症性酸中毒",
"start_offset": 196,
"end_offset": 203,
"label": "dis"
},
{
"id": 22,
"entity": "感染",
"start_offset": 206,
"end_offset": 208,
"label": "dis"
}
] |
2.幼儿型起病较晚（2岁以后），症状较婴儿型轻，以抗维生素D佝偻病及生长迟缓为最突出表现。 | [
{
"id": 0,
"entity": "抗维生素D佝偻病",
"start_offset": 25,
"end_offset": 33,
"label": "dis"
},
{
"id": 1,
"entity": "生长迟缓",
"start_offset": 34,
"end_offset": 38,
"label": "sym"
}
] |
3.成人型特点有：①10～20岁或更晚发病；②多种肾小管功能障碍；如糖尿、全氨基酸尿、高磷酸盐尿、低血钾及高氯酸中毒；③软骨病往往是突出表现；④晚期可出现肾衰竭。 | [
{
"id": 0,
"entity": "肾小管功能障碍",
"start_offset": 25,
"end_offset": 32,
"label": "sym"
},
{
"id": 1,
"entity": "糖尿",
"start_offset": 34,
"end_offset": 36,
"label": "sym"
},
{
"id": 2,
"entity": "全氨基酸尿",
"start_offset": 37,
"end_offset": 42,
"label": "sym"
},
{
"id": 3,
"entity": "高磷酸盐尿",
"start_offset": 43,
"end_offset": 48,
"label": "sym"
},
{
"id": 4,
"entity": "低血钾",
"start_offset": 49,
"end_offset": 52,
"label": "sym"
},
{
"id": 5,
"entity": "高氯酸中毒",
"start_offset": 53,
"end_offset": 58,
"label": "dis"
},
{
"id": 6,
"entity": "软骨病",
"start_offset": 60,
"end_offset": 63,
"label": "dis"
},
{
"id": 7,
"entity": "肾衰竭",
"start_offset": 77,
"end_offset": 80,
"label": "dis"
}
] |
（二）继发性Fanconi综合征因病因不同表现有所不同（表12-4）。 | [
{
"id": 0,
"entity": "继发性Fanconi综合征",
"start_offset": 3,
"end_offset": 16,
"label": "dis"
}
] |
表12-4常见范可尼综合征的特征及鉴别注：PAH，对氨基马尿酸；+，轻度受损；++，中度受损；+++，严重受损；AD，常染色体显性；AR，常染色体隐性；XLR，X连锁隐性遗传【诊断与鉴别诊断】本病无特异诊断试验，根据生长迟缓、佝偻病、多尿及脱水、酸中毒、电解质紊乱相应的临床表现，血生化检查见低血钾、低血磷、低血钠、低血氯性酸中毒、高AKP、低血尿酸、糖尿而血糖正常，全氨基酸尿、尿pH低而尿氨和可滴定酸低，X线检查有骨质疏松及佝偻病表现均有助于骨质疏松注意询问家族史。 | [
{
"id": 0,
"entity": "范可尼综合征",
"start_offset": 7,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "AD",
"start_offset": 56,
"end_offset": 58,
"label": "sym"
},
{
"id": 2,
"entity": "常染色体显性",
"start_offset": 59,
"end_offset": 65,
"label": "sym"
},
{
"id": 3,
"entity": "AR",
"start_offset": 66,
"end_offset": 68,
"label": "sym"
},
{
"id": 4,
"entity": "常染色体隐性",
"start_offset": 69,
"end_offset": 75,
"label": "sym"
},
{
"id": 5,
"entity": "XLR",
"start_offset": 76,
"end_offset": 79,
"label": "sym"
},
{
"id": 6,
"entity": "X连锁隐性遗传",
"start_offset": 80,
"end_offset": 87,
"label": "sym"
},
{
"id": 7,
"entity": "生长迟缓",
"start_offset": 108,
"end_offset": 112,
"label": "sym"
},
{
"id": 8,
"entity": "佝偻病",
"start_offset": 113,
"end_offset": 116,
"label": "sym"
},
{
"id": 9,
"entity": "多尿",
"start_offset": 117,
"end_offset": 119,
"label": "sym"
},
{
"id": 10,
"entity": "脱水",
"start_offset": 120,
"end_offset": 122,
"label": "sym"
},
{
"id": 11,
"entity": "酸中毒",
"start_offset": 123,
"end_offset": 126,
"label": "dis"
},
{
"id": 12,
"entity": "电解质紊乱",
"start_offset": 127,
"end_offset": 132,
"label": "sym"
},
{
"id": 13,
"entity": "低血钾",
"start_offset": 146,
"end_offset": 149,
"label": "sym"
},
{
"id": 14,
"entity": "低血磷",
"start_offset": 150,
"end_offset": 153,
"label": "sym"
},
{
"id": 15,
"entity": "低血钠",
"start_offset": 154,
"end_offset": 157,
"label": "sym"
},
{
"id": 16,
"entity": "低血氯性酸中毒",
"start_offset": 158,
"end_offset": 165,
"label": "dis"
},
{
"id": 17,
"entity": "高AKP",
"start_offset": 166,
"end_offset": 170,
"label": "sym"
},
{
"id": 18,
"entity": "低血尿酸",
"start_offset": 171,
"end_offset": 175,
"label": "sym"
},
{
"id": 19,
"entity": "糖尿",
"start_offset": 176,
"end_offset": 178,
"label": "sym"
},
{
"id": 20,
"entity": "全氨基酸尿",
"start_offset": 184,
"end_offset": 189,
"label": "sym"
},
{
"id": 21,
"entity": "尿pH低",
"start_offset": 190,
"end_offset": 194,
"label": "sym"
},
{
"id": 22,
"entity": "尿氨和可滴定酸低",
"start_offset": 195,
"end_offset": 203,
"label": "sym"
},
{
"id": 23,
"entity": "X线检查",
"start_offset": 204,
"end_offset": 208,
"label": "pro"
},
{
"id": 24,
"entity": "骨质疏松",
"start_offset": 209,
"end_offset": 213,
"label": "sym"
},
{
"id": 25,
"entity": "佝偻病",
"start_offset": 214,
"end_offset": 217,
"label": "dis"
},
{
"id": 26,
"entity": "骨质疏松",
"start_offset": 223,
"end_offset": 227,
"label": "dis"
}
] |
由于多种类型Fanconi综合征可通过特异性治疗及对症处理取得良好疗效，因此病因诊断尤为重要，表12-4为各种不同病因下Fanconi综合征的特点和区别。 | [
{
"id": 0,
"entity": "Fanconi综合征",
"start_offset": 6,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "Fanconi综合征",
"start_offset": 60,
"end_offset": 70,
"label": "dis"
}
] |
【治疗】（一）病因治疗对已了解代谢缺陷类型的继发性Fanconi综合征，可进行特异性治疗。 | [
{
"id": 0,
"entity": "代谢缺陷",
"start_offset": 15,
"end_offset": 19,
"label": "sym"
},
{
"id": 1,
"entity": "继发性Fanconi综合征",
"start_offset": 22,
"end_offset": 35,
"label": "dis"
}
] |
通过饮食疗法减少或避免有毒代谢产物积聚的疾病有半乳糖血症、遗传性果糖不耐受以及酪氨酸血症Ⅰ型。 | [
{
"id": 0,
"entity": "有毒代谢产物积聚",
"start_offset": 11,
"end_offset": 19,
"label": "sym"
},
{
"id": 1,
"entity": "半乳糖血症",
"start_offset": 23,
"end_offset": 28,
"label": "dis"
},
{
"id": 2,
"entity": "遗传性果糖不耐受",
"start_offset": 29,
"end_offset": 37,
"label": "sym"
},
{
"id": 3,
"entity": "酪氨酸血症Ⅰ型",
"start_offset": 39,
"end_offset": 46,
"label": "dis"
}
] |
通过促进排泄治疗的疾病有Wilson病和重金属中毒。 | [
{
"id": 0,
"entity": "Wilson病",
"start_offset": 12,
"end_offset": 19,
"label": "dis"
},
{
"id": 1,
"entity": "重金属中毒",
"start_offset": 20,
"end_offset": 25,
"label": "dis"
}
] |
由药物引起的Fanconi综合征，清除体内药物可纠正肾小管功能障碍。 | [
{
"id": 0,
"entity": "Fanconi综合征",
"start_offset": 6,
"end_offset": 16,
"label": "dis"
}
] |
对于由肾脏疾病或全身疾病后引起的Fanconi综合征则相应针对原发病治疗。 | [
{
"id": 0,
"entity": "Fanconi综合征",
"start_offset": 16,
"end_offset": 26,
"label": "dis"
}
] |
。（二）对症治疗1.纠正酸中毒根据肾小管受损的程度给予碱性药物，剂量2～10mmol/（kg•d），可采用碳酸氢钠或者枸橼酸钠钾合剂，全天剂量分4～5次口服，然后根据血中浓度调整剂量。 | [
{
"id": 0,
"entity": "纠正酸中毒",
"start_offset": 10,
"end_offset": 15,
"label": "pro"
},
{
"id": 1,
"entity": "肾小管受损",
"start_offset": 17,
"end_offset": 22,
"label": "sym"
},
{
"id": 2,
"entity": "碳酸氢钠",
"start_offset": 53,
"end_offset": 57,
"label": "dru"
},
{
"id": 3,
"entity": "枸橼酸钠钾",
"start_offset": 59,
"end_offset": 64,
"label": "dru"
}
] |
应注意同时补钾，如碱性药物用量过大，可合用氢氯噻嗪，促进回吸收。 | [
{
"id": 0,
"entity": "氢氯噻嗪",
"start_offset": 21,
"end_offset": 25,
"label": "dru"
}
] |
2.纠正低磷血症口服中性磷酸盐以纠正低磷血症，剂量为1～3g/d，分次服，每4～5小时用药1次，不良反应有胃肠不适和腹泻，减少用量可减轻上述症状。 | [
{
"id": 0,
"entity": "纠正低磷血症",
"start_offset": 2,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "中性磷酸盐",
"start_offset": 10,
"end_offset": 15,
"label": "dru"
},
{
"id": 2,
"entity": "纠正低磷血症",
"start_offset": 16,
"end_offset": 22,
"label": "pro"
},
{
"id": 3,
"entity": "胃肠",
"start_offset": 53,
"end_offset": 55,
"label": "bod"
},
{
"id": 4,
"entity": "胃肠不适",
"start_offset": 53,
"end_offset": 57,
"label": "sym"
},
{
"id": 5,
"entity": "腹泻",
"start_offset": 58,
"end_offset": 60,
"label": "sym"
}
] |
在部分患者，应用磷酸盐可加重低钙血症，诱发甲状旁腺功能亢进，可口服钙剂和维生素D预防。 | [
{
"id": 0,
"entity": "磷酸盐",
"start_offset": 8,
"end_offset": 11,
"label": "dru"
},
{
"id": 1,
"entity": "低钙血症",
"start_offset": 14,
"end_offset": 18,
"label": "dis"
},
{
"id": 2,
"entity": "加重低钙血症",
"start_offset": 12,
"end_offset": 18,
"label": "sym"
},
{
"id": 3,
"entity": "甲状旁腺功能亢进",
"start_offset": 21,
"end_offset": 29,
"label": "sym"
},
{
"id": 4,
"entity": "口服钙剂和维生素D",
"start_offset": 31,
"end_offset": 40,
"label": "pro"
}
] |
中性磷酸盐配方：Na<sub>2</sub>HPO<sub>4</sub>•7H<sub>2</sub>O145g，NaH<sub>2</sub>PO<sub>4</sub>•H<sub>2</sub>O18.2g，加水至1000ml，每1000ml供磷2g。 | [
{
"id": 0,
"entity": "中性磷酸盐",
"start_offset": 0,
"end_offset": 5,
"label": "dru"
}
] |
3.其他应补充血容量，防脱水，纠正低钾血症。 | [
{
"id": 0,
"entity": "补充血容量",
"start_offset": 5,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "防脱水",
"start_offset": 11,
"end_offset": 14,
"label": "pro"
},
{
"id": 2,
"entity": "纠正低钾血症",
"start_offset": 15,
"end_offset": 21,
"label": "pro"
}
] |
对于低尿酸血症、氨基酸尿、糖尿及蛋白尿，目前尚缺乏有效的治疗方法。 | [
{
"id": 0,
"entity": "低尿酸血症",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "氨基酸尿",
"start_offset": 8,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "糖尿",
"start_offset": 13,
"end_offset": 15,
"label": "sym"
},
{
"id": 3,
"entity": "蛋白尿",
"start_offset": 16,
"end_offset": 19,
"label": "sym"
}
] |
肾功能不全者，则酌情采用保守式肾脏替代治疗。 | [
{
"id": 0,
"entity": "肾功能",
"start_offset": 0,
"end_offset": 3,
"label": "ite"
},
{
"id": 1,
"entity": "肾功能不全",
"start_offset": 0,
"end_offset": 5,
"label": "sym"
},
{
"id": 2,
"entity": "保守式肾脏替代治疗",
"start_offset": 12,
"end_offset": 21,
"label": "pro"
}
] |
【预后】本病预后取决于所累及的脏器以及治疗开始的早晚、持续性和原发病等因素，严重患者最终多死于严重水、电解质紊乱及肾衰竭。 | [
{
"id": 0,
"entity": "严重水、电解质紊乱",
"start_offset": 47,
"end_offset": 56,
"label": "sym"
},
{
"id": 1,
"entity": "肾衰竭",
"start_offset": 57,
"end_offset": 60,
"label": "dis"
}
] |
冷抗体型免疫性溶血性贫血可分为冷凝集素病和阵发性寒冷性血红蛋白尿两种。 | [
{
"id": 0,
"entity": "冷抗体型免疫性溶血性贫血",
"start_offset": 0,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "冷凝集素病",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "阵发性寒冷性血红蛋白尿",
"start_offset": 21,
"end_offset": 32,
"label": "dis"
}
] |
1.冷凝集素病常继发于肺炎支原体、EB病毒感染或继发于淋巴增生性疾病，产生识别I/i系统寡糖抗原的抗体，通常为IgM，需补体参与。 | [
{
"id": 0,
"entity": "冷凝集素病",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "肺炎支原体",
"start_offset": 11,
"end_offset": 16,
"label": "mic"
},
{
"id": 2,
"entity": "EB病毒感染",
"start_offset": 17,
"end_offset": 23,
"label": "dis"
},
{
"id": 3,
"entity": "淋巴增生性疾病",
"start_offset": 27,
"end_offset": 34,
"label": "dis"
},
{
"id": 4,
"entity": "抗体",
"start_offset": 49,
"end_offset": 51,
"label": "bod"
},
{
"id": 5,
"entity": "IgM",
"start_offset": 55,
"end_offset": 58,
"label": "bod"
}
] |
急性型者继发于支原体肺炎或传染性单核细胞增多症，呈一过性，预后良好；慢性型者寒冷季节恶化，温热季节缓解，病情长期持续反复。 | [
{
"id": 0,
"entity": "支原体肺炎",
"start_offset": 7,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "传染性单核细胞增多症",
"start_offset": 13,
"end_offset": 23,
"label": "dis"
}
] |
实验室检查：冷凝集素滴度常在1∶10000左右，肺炎支原体感染时，可高达1∶30000以上。 | [
{
"id": 0,
"entity": "冷凝集素",
"start_offset": 6,
"end_offset": 10,
"label": "dru"
},
{
"id": 1,
"entity": "肺炎支原体感染",
"start_offset": 24,
"end_offset": 31,
"label": "dis"
}
] |
直接Coombs试验可阳性，37℃时，间接试验阴性，但在低温条件下则阳性。 | [
{
"id": 0,
"entity": "Coombs试验",
"start_offset": 2,
"end_offset": 10,
"label": "pro"
}
] |
由IgM型冷凝抗体引起者，脾切除效果不佳，因为这类受损红细胞很少由脾脏清除。 | [
{
"id": 0,
"entity": "红细胞",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 1,
"entity": "脾脏",
"start_offset": 33,
"end_offset": 35,
"label": "bod"
}
] |
2.阵发性寒冷性血红蛋白尿本病占儿童AIHA病例的30%，多见于先天性梅毒患儿，也可继发于麻疹、流行性腮腺炎、水痘、传染性单核细胞增多症等病，少数为原发性。 | [
{
"id": 0,
"entity": "阵发性寒冷性血红蛋白尿",
"start_offset": 2,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "AIHA",
"start_offset": 18,
"end_offset": 22,
"label": "dis"
},
{
"id": 2,
"entity": "先天性梅毒",
"start_offset": 32,
"end_offset": 37,
"label": "dis"
},
{
"id": 3,
"entity": "麻疹",
"start_offset": 45,
"end_offset": 47,
"label": "dis"
},
{
"id": 4,
"entity": "流行性腮腺炎",
"start_offset": 48,
"end_offset": 54,
"label": "dis"
},
{
"id": 5,
"entity": "水痘",
"start_offset": 55,
"end_offset": 57,
"label": "dis"
},
{
"id": 6,
"entity": "传染性单核细胞增多症",
"start_offset": 58,
"end_offset": 68,
"label": "dis"
}
] |
它是与自身免疫和寒冷有关的急性溶血性贫血。 | [
{
"id": 0,
"entity": "急性溶血性贫血",
"start_offset": 13,
"end_offset": 20,
"label": "dis"
}
] |
患儿体内诱生的主要为IgG冷抗体即D-L型冷溶血素，该抗体在低温（＜20℃）时能与红细胞膜上的抗原结合，并可固定大量补体，当温度上升时，大量红细胞发生破裂而溶血。 | [
{
"id": 0,
"entity": "抗体",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 1,
"entity": "D-L型冷溶血素",
"start_offset": 17,
"end_offset": 25,
"label": "bod"
},
{
"id": 2,
"entity": "抗体",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
},
{
"id": 3,
"entity": "红细胞膜",
"start_offset": 41,
"end_offset": 45,
"label": "bod"
},
{
"id": 4,
"entity": "红细胞",
"start_offset": 70,
"end_offset": 73,
"label": "bod"
}
] |
本病也可呈慢性溶血过程，而与寒冷无关。 | [
{
"id": 0,
"entity": "溶血",
"start_offset": 7,
"end_offset": 9,
"label": "dis"
}
] |
临床上主要表现为身体暴露于低温条件下10～20分钟后复温时，发生溶血。 | [
{
"id": 0,
"entity": "溶血",
"start_offset": 32,
"end_offset": 34,
"label": "dis"
}
] |
发作时患儿出现寒战、高热、头痛、腰痛、肢痛及血红蛋白尿，并可出现恶性、呕吐、腹泻，肌强直等，症状持续数小时，发作后可有黄疸，反复发作者可出现轻度脾大。 | [
{
"id": 0,
"entity": "寒战",
"start_offset": 7,
"end_offset": 9,
"label": "sym"
},
{
"id": 1,
"entity": "高热",
"start_offset": 10,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "头痛",
"start_offset": 13,
"end_offset": 15,
"label": "sym"
},
{
"id": 3,
"entity": "腰痛",
"start_offset": 16,
"end_offset": 18,
"label": "sym"
},
{
"id": 4,
"entity": "肢痛",
"start_offset": 19,
"end_offset": 21,
"label": "sym"
},
{
"id": 5,
"entity": "血红蛋白尿",
"start_offset": 22,
"end_offset": 27,
"label": "sym"
},
{
"id": 6,
"entity": "恶性",
"start_offset": 32,
"end_offset": 34,
"label": "sym"
},
{
"id": 7,
"entity": "呕吐",
"start_offset": 35,
"end_offset": 37,
"label": "sym"
},
{
"id": 8,
"entity": "腹泻",
"start_offset": 38,
"end_offset": 40,
"label": "sym"
},
{
"id": 9,
"entity": "肌强直",
"start_offset": 41,
"end_offset": 44,
"label": "sym"
},
{
"id": 10,
"entity": "黄疸",
"start_offset": 59,
"end_offset": 61,
"label": "sym"
},
{
"id": 11,
"entity": "轻度脾大",
"start_offset": 70,
"end_offset": 74,
"label": "sym"
}
] |
实验室检查：Donath-Landsteiner试验阳性为确诊本病的依据。 | [
{
"id": 0,
"entity": "Donath-Landsteiner试验",
"start_offset": 6,
"end_offset": 26,
"label": "pro"
}
] |
溶血发作时Coombs直接试验强阳性，发作后转为阴性；间接试验均阴性。 | [
{
"id": 0,
"entity": "溶血",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "Coombs直接试验",
"start_offset": 5,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "间接试验",
"start_offset": 27,
"end_offset": 31,
"label": "pro"
}
] |
冷凝集试验阴性。 | [
{
"id": 0,
"entity": "冷凝集试验",
"start_offset": 0,
"end_offset": 5,
"label": "pro"
}
] |
慢性者可应用糖皮质激素或免疫抑制剂。 | [
{
"id": 0,
"entity": "糖皮质激素",
"start_offset": 6,
"end_offset": 11,
"label": "dru"
},
{
"id": 1,
"entity": "免疫抑制剂",
"start_offset": 12,
"end_offset": 17,
"label": "dru"
}
] |
重度贫血者可输注洗涤红细胞。 | [
{
"id": 0,
"entity": "贫血",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "红细胞",
"start_offset": 10,
"end_offset": 13,
"label": "bod"
}
] |
表10-12出血性疾病的临床鉴别（一）病史1.家族史家族中已有同样患者，提示为遗传性疾患，需仔细询问遗传方式等，如女性传递男性发病者多见于血友病甲或血友病乙；每一代中都有患者的为常染色体显性遗传如血管性血友病、毛细血管扩张症、储存池病、巨大血小板综合征等。 | [
{
"id": 0,
"entity": "出血性疾病",
"start_offset": 6,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "血友病甲",
"start_offset": 69,
"end_offset": 73,
"label": "dis"
},
{
"id": 2,
"entity": "血友病乙",
"start_offset": 74,
"end_offset": 78,
"label": "dis"
},
{
"id": 3,
"entity": "染色体",
"start_offset": 90,
"end_offset": 93,
"label": "bod"
},
{
"id": 4,
"entity": "血管性血友病",
"start_offset": 98,
"end_offset": 104,
"label": "dis"
},
{
"id": 5,
"entity": "毛细血管扩张症",
"start_offset": 105,
"end_offset": 112,
"label": "dis"
},
{
"id": 6,
"entity": "储存池病",
"start_offset": 113,
"end_offset": 117,
"label": "dis"
},
{
"id": 7,
"entity": "巨大血小板综合征",
"start_offset": 118,
"end_offset": 126,
"label": "dis"
}
] |
2.发病年龄与性别遗传性出血性疾病发病年龄偏小，获得性者发病年龄多偏大，但维生素K缺乏症、原发性血小板减少性紫癜的发病年龄可小至数月至数天。 | [
{
"id": 0,
"entity": "遗传性出血性疾病",
"start_offset": 9,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "维生素K缺乏症",
"start_offset": 37,
"end_offset": 44,
"label": "dis"
},
{
"id": 2,
"entity": "原发性血小板减少性紫癜",
"start_offset": 45,
"end_offset": 56,
"label": "dis"
}
] |
男性患者伴关节腔出血者多为血友病甲或乙。 | [
{
"id": 0,
"entity": "关节腔出血",
"start_offset": 5,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "血友病甲或乙",
"start_offset": 13,
"end_offset": 19,
"label": "dis"
}
] |
3.出血方式皮肤、黏膜自发性出血、青春期月经量过多常提示血小板质或量的异常；轻微外伤后出血不止提示凝血功能异常。 | [
{
"id": 0,
"entity": "皮肤",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "黏膜",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "自发性出血",
"start_offset": 11,
"end_offset": 16,
"label": "sym"
},
{
"id": 3,
"entity": "月经量过多",
"start_offset": 20,
"end_offset": 25,
"label": "sym"
},
{
"id": 4,
"entity": "血小板质",
"start_offset": 28,
"end_offset": 32,
"label": "bod"
}
] |
4.药物史不少药物分别可损伤血管壁，影响血小板功能，抑制凝血，常用的如磺胺药、异烟肼、阿司匹林、双嘧达莫、肝素等；抗癌药则可直接抑制骨髓使血小板减少。 | [
{
"id": 0,
"entity": "血管壁",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 20,
"end_offset": 23,
"label": "bod"
},
{
"id": 2,
"entity": "磺胺药",
"start_offset": 35,
"end_offset": 38,
"label": "dru"
},
{
"id": 3,
"entity": "异烟肼",
"start_offset": 39,
"end_offset": 42,
"label": "dru"
},
{
"id": 4,
"entity": "阿司匹林",
"start_offset": 43,
"end_offset": 47,
"label": "dru"
},
{
"id": 5,
"entity": "双嘧达莫",
"start_offset": 48,
"end_offset": 52,
"label": "dru"
},
{
"id": 6,
"entity": "肝素",
"start_offset": 53,
"end_offset": 55,
"label": "dru"
},
{
"id": 7,
"entity": "抗癌药",
"start_offset": 57,
"end_offset": 60,
"label": "dru"
},
{
"id": 8,
"entity": "骨髓",
"start_offset": 66,
"end_offset": 68,
"label": "bod"
},
{
"id": 9,
"entity": "血小板",
"start_offset": 69,
"end_offset": 72,
"label": "bod"
}
] |
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