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糖原累积症Ⅳ型由于糖原分支酶缺陷导致糖原在肝脏沉积，出现进行性肝硬化和肝功能障碍，该型低血糖不显著。 | [
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Zellweger综合征是另一种引起新生儿黄疸和肝功能障碍的代谢疾病，通常由于伴肌张力减低和畸形被发现，该病与过氧化物酶功能障碍有关。 | [
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患儿在无溶血时出现持续性间接胆红素升高提示Criger-Najjar综合征，该病的高胆红素血症是由于葡萄糖醛酸转移酶部分或全部缺乏造成。 | [
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"entity": "高胆红素血症",
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胸部X线检查早期即有肺浸润，呈非典型性肺炎变化。 | [
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"entity": "非典型性肺炎",
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横纹肌肉瘤占儿童实体肿瘤的15%，软组织肉瘤的50%。 | [
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1950年Stobbe报道对头颈部肿瘤加以放疗可提高疗效。 | [
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1965年Edland介绍的大剂量放疗成为治疗不能完整切除的肿瘤的标准方法。 | [
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化疗药物的应用包括长春新碱、放线菌素D和环磷酰胺（VCR方案），效果各有差异。 | [
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如有扩散转移，病人可有发热、厌食、体重下降、疼痛和衰弱等情况。 | [
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该肿瘤好发于两个年龄段：2～4岁和12～16岁。 | [
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发病最常见的部位是头颈部（35%），泌尿生殖系统（26%），四肢（19%）。 | [
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鼻咽癌和鼻咽部横纹肌肉瘤发病情况很相似，但鼻咽癌好发大龄儿童，颈部淋巴结转移率较高，预后较差。 | [
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除了原发病，横纹肌肉瘤转移到肺、骨、骨髓、淋巴结、脑、肝和乳腺，可出现相应的症状。 | [
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做血常规，肝、肾功能，尿液分析，骨髓穿刺等化验检查。 | [
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CT、MRI可以定位肿物和其向周围侵袭的情况。 | [
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儿童横纹肌肉瘤尚无特异性血浆或尿标记物。 | [
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最常见的横纹肌肉瘤组织类型是胚胎型，由梭状的横纹肌母细胞、具有嗜伊红细胞质和纵行条纹的小圆细胞组成。 | [
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息肉样结构由以圆形和梭状细胞为中心的黏液基质以及围绕的小圆细胞组成，好发年龄小于4岁，预后最好。 | [
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腺泡型是第二位常见的横纹肌肉瘤，仅次于胚胎型。 | [
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最多见于四肢和躯干，由具有嗜伊红细胞质的大圆细胞组成。 | [
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通过反转录聚合酶链反应（PCR）和荧光原位杂交技术，横纹肌肉瘤的诊断可达到分子遗传学水平，并对治疗有指导意义。 | [
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在腺泡型横纹肌肉瘤中发现2、13q35-q14基因位点中断，而在胚胎型横纹肌肉瘤中发现11号染色体上有肿瘤生长抑制基因。 | [
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Ⅱ级中39%的患儿为除脑膜外的头颈部肿瘤。 | [
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先做活检再行辅助治疗，使肿瘤缩小再做手术比将肿瘤部分切除效果好。 | [
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如果没有完全切除睾丸旁肿瘤，则要取腹膜后淋巴结活检。 | [
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对横纹肌肉瘤比较有效的药物有长春新碱、放线菌素D、环磷酰胺、阿霉素、异环磷酰胺、足叶乙甙、顺铂、卡铂、美法仑、氨甲蝶呤、达卡巴嗪以及丝裂霉素C。 | [
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但除高分级肿瘤外，所有的病例都可采用VCR方案，如加强长春新碱和放线菌素D方案（加强VA），或VAC冲击方案。 | [
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加强VA方案对Ⅰ级或Ⅱ级非腺泡型肿瘤有效，Ⅰ级存活率达85%，Ⅱ级达70%；Ⅲ、Ⅳ级病人大多数用冲击VAC方案，大约50%～70%完全敏感，另外20%部分敏感。 | [
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大剂量环磷酰胺连同泌尿系保护药物美司钠一起应用效果不错。 | [
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1979年Abramason的研究表明放疗加化疗对眼眶肿瘤是最有效的，从此不再进行眼眶内容物剜除术，其存活率可达90%。 | [
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化疗主要是长春新碱和放线菌素D。 | [
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脑膜旁的横纹肌肉瘤部位较隐蔽，不易诊断，预后较差。 | [
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强化治疗可提高生存率，其包括VCA、鞘内化疗、整个脑脊髓部的放疗等。 | [
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大约有50%的胸壁肿瘤是腺泡型横纹肌肉瘤，其他尚有骨骼外Ewing肉瘤和未分化肉瘤也可见这种组织类型。 | [
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脊柱旁肿瘤可表现出急性神经压迫症状，需急诊手术减压。 | [
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Ortega对IRSⅠ和Ⅱ的56个脊柱旁横纹肌肉瘤的患者作过总结，大部分发生在腰部和胸腰部，肿瘤直径大于5cm，为未分化型和腺泡型。 | [
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下肢比上肢多发，远端比近端常见，但预后差别不大。 | [
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以前膀胱或前列腺横纹肌肉瘤必须行盆腔内肿瘤及组织的根除术，随着综合治疗的开展，该术式已经不再进行。 | [
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IRSⅢ提出了加强的放疗和化疗方案，采用阿霉素、顺铂、VAC，在第6周放疗，大大提高了3年存活率（83%），而且60%的膀胱得以保留（表11-11）。 | [
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膀胱前列腺横纹肌肉瘤如果条件允许可以行部分膀胱切除，或先行病理活检，接受加强化疗和放疗。 | [
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"label": "pro"
},
{
"id": 2,
"entity": "病理活检",
"start_offset": 29,
"end_offset": 33,
"label... |
从临床结果来看，早期局部肿瘤尽管可得到控制，但镜下肿瘤残留却可能持续存在，所以大多数病人最终还是做了子宫、阴道切除术。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "镜",
"start_offset": 23,
"end_offset": 24,
"label": "equ"
},
{
"id": 2,
"entity": "肿瘤残留",
"start_offset": 25,
"end_offset": 29,
"label": "bod"
... |
确诊后要行睾丸根治切除术。 | [
{
"id": 0,
"entity": "睾丸根治切除术",
"start_offset": 5,
"end_offset": 12,
"label": "pro"
}
] |
如果以前作过阴囊活检，要行患侧阴囊切除，以免污染阴囊，同时也减少了髂腹股沟和主动脉旁淋巴结转移的机会。 | [
{
"id": 0,
"entity": "阴囊活检",
"start_offset": 6,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "阴囊切除",
"start_offset": 15,
"end_offset": 19,
"label": "pro"
},
{
"id": 2,
"entity": "阴囊",
"start_offset": 24,
"end_offset": 26,
"label": "bod"... |
经上述手术，存活率为50%；如术后化疗，或在有淋巴结转移和肿瘤残留的情况下放疗，存活率可达90%。 | [
{
"id": 0,
"entity": "手术",
"start_offset": 3,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "化疗",
"start_offset": 17,
"end_offset": 19,
"label": "pro"
},
{
"id": 2,
"entity": "淋巴结",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
}... |
对经CT检查无腹膜后淋巴结转移和肿瘤又完全切除者，可不需做腹膜后淋巴结活检。 | [
{
"id": 0,
"entity": "CT检查",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "腹膜",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "淋巴结",
"start_offset": 10,
"end_offset": 13,
"label": "bod"
}... |
但如怀疑有淋巴结浸润或原发肿瘤切除不完整，还是做系统的腹膜后淋巴结检查为好。 | [
{
"id": 0,
"entity": "淋巴结",
"start_offset": 5,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "原发肿瘤",
"start_offset": 11,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "腹膜后淋巴结检查",
"start_offset": 27,
"end_offset": 35,
"label": "... |
Heyn等人讨论了86例IRS睾丸旁横纹肌肉瘤的长期疗效。 | [
{
"id": 0,
"entity": "睾丸旁横纹肌肉瘤",
"start_offset": 15,
"end_offset": 23,
"label": "dis"
}
] |
1/3接受过环磷酰胺治疗的病人患出血性膀胱炎。 | [
{
"id": 0,
"entity": "环磷酰胺",
"start_offset": 6,
"end_offset": 10,
"label": "dru"
},
{
"id": 1,
"entity": "出血性膀胱炎",
"start_offset": 16,
"end_offset": 22,
"label": "dis"
}
] |
治疗一般采用强化疗和放疗，如近距离放疗或术中放疗。 | [
{
"id": 0,
"entity": "强化疗",
"start_offset": 6,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "放疗",
"start_offset": 10,
"end_offset": 12,
"label": "pro"
},
{
"id": 2,
"entity": "近距离放疗",
"start_offset": 14,
"end_offset": 19,
"label": "pro"
... |
偶有经二次探查术后转为对治疗敏感的，Ⅲ级病人的存活率为48%。 | [
{
"id": 0,
"entity": "二次探查术",
"start_offset": 3,
"end_offset": 8,
"label": "pro"
}
] |
会阴和肛周横纹肌肉瘤少见，通常为腺泡型，对放疗和化疗反应极差，存活率大约为20%。 | [
{
"id": 0,
"entity": "会阴和肛周横纹肌肉瘤",
"start_offset": 0,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "放疗",
"start_offset": 21,
"end_offset": 23,
"label": "pro"
},
{
"id": 2,
"entity": "化疗",
"start_offset": 24,
"end_offset": 26,
"label": "... |
女孩中约80%的中枢性性早熟患儿为特发性性早熟。 | [
{
"id": 0,
"entity": "中枢性性早熟",
"start_offset": 8,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "特发性性早熟",
"start_offset": 17,
"end_offset": 23,
"label": "dis"
}
] |
下丘脑错构瘤是最常引起真性性早熟的脑部病变之一，这一先天畸形由异位的神经组织所构成，含有分泌GnRH的神经元，并在功能上如同一个附加的GnRH脉冲源。 | [
{
"id": 0,
"entity": "下丘脑错构瘤",
"start_offset": 0,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "真性性早熟",
"start_offset": 11,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "脑部",
"start_offset": 17,
"end_offset": 19,
"label": "bo... |
其次包括星形细胞瘤、室管膜瘤、视神经胶质瘤及神经纤维瘤Ⅰ型等。 | [
{
"id": 0,
"entity": "星形细胞瘤",
"start_offset": 4,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "室管膜瘤",
"start_offset": 10,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "视神经胶质瘤",
"start_offset": 15,
"end_offset": 21,
"label": "... |
最常见的症状为呼吸道梗阻，呼气时出现喘鸣，常易反复发作急性呼吸道梗阻和呼吸道感染，但不呈现吞咽困难症状。 | [
{
"id": 0,
"entity": "呼吸道",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "呼吸道梗阻",
"start_offset": 7,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "呼气时出现喘鸣",
"start_offset": 13,
"end_offset": 20,
"label": "... |
体格检查可发现右侧肺部呼吸音减弱及肺膨胀不全的表现。 | [
{
"id": 0,
"entity": "体格检查",
"start_offset": 0,
"end_offset": 4,
"label": "ite"
},
{
"id": 1,
"entity": "右侧肺部",
"start_offset": 7,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "肺",
"start_offset": 17,
"end_offset": 18,
"label": "bod"
... |
在气管隆突与食管之间，可见异常左肺动脉的块影，左肺动脉在左肺门发出的分支位置较低，支气管镜或支气管造影可显示并存的气管、支气管异常。 | [
{
"id": 0,
"entity": "气管",
"start_offset": 1,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "食管",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "左肺动脉",
"start_offset": 15,
"end_offset": 19,
"label": "bod"
},... |
超声心动图可发现左肺动脉异常起源。 | [
{
"id": 0,
"entity": "超声心动图",
"start_offset": 0,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "左肺动脉",
"start_offset": 8,
"end_offset": 12,
"label": "bod"
}
] |
手术后早期死亡率高，主要致死原因为重度气管、支气管狭窄和左肺动脉因吻合不当发生扭曲和血栓形成。 | [
{
"id": 0,
"entity": "手术",
"start_offset": 0,
"end_offset": 2,
"label": "pro"
},
{
"id": 1,
"entity": "重度气管、支气管狭窄",
"start_offset": 17,
"end_offset": 27,
"label": "dis"
},
{
"id": 2,
"entity": "左肺动脉",
"start_offset": 28,
"end_offset": 32,
"label": ... |
术后静脉滴注少量地塞米松可减少拔除气管插管后气管黏膜水肿。 | [
{
"id": 0,
"entity": "术后静脉滴注",
"start_offset": 0,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "地塞米松",
"start_offset": 8,
"end_offset": 12,
"label": "dru"
},
{
"id": 2,
"entity": "气管",
"start_offset": 17,
"end_offset": 19,
"label": "bod"... |
可有剧烈的疼痛，并可引起耳鸣。 | [
{
"id": 0,
"entity": "可有剧烈的疼痛，并可引起耳鸣",
"start_offset": 0,
"end_offset": 14,
"label": "sym"
}
] |
肿瘤过大，可分期手术，必要时植皮。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "植皮",
"start_offset": 14,
"end_offset": 16,
"label": "pro"
}
] |
肿瘤主要在新生儿期扩大，少数在婴儿期长大。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
}
] |
治疗只需切除即可。 | [
{
"id": 0,
"entity": "切除",
"start_offset": 4,
"end_offset": 6,
"label": "pro"
}
] |
正常情况下主动脉瓣下无圆锥，大血管转位后其瓣下有圆锥部存在，造成与三尖瓣之间无纤维连续。 | [
{
"id": 0,
"entity": "主动脉瓣",
"start_offset": 5,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "大血管转位",
"start_offset": 14,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "其瓣下有圆锥部存在",
"start_offset": 20,
"end_offset": 29,
"label"... |
相反，肺动脉瓣与二尖瓣毗邻，两者之间出现纤维连续。 | [
{
"id": 0,
"entity": "肺动脉瓣",
"start_offset": 3,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "二尖瓣",
"start_offset": 8,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "纤维连续",
"start_offset": 20,
"end_offset": 24,
"label": "sym"
... |
大动脉转位最明显的特征是主肺动脉相对位置异常。 | [
{
"id": 0,
"entity": "大动脉转位",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "主肺动脉相对位置异常",
"start_offset": 12,
"end_offset": 22,
"label": "sym"
}
] |
常用的分类法是将“面对窦”硬性定为1号窦、2号窦。 | [
{
"id": 0,
"entity": "面对窦",
"start_offset": 9,
"end_offset": 12,
"label": "bod"
},
{
"id": 1,
"entity": "1号窦",
"start_offset": 17,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "2号窦",
"start_offset": 21,
"end_offset": 24,
"label": "bod"
... |
大动脉转位除合并卵圆孔、动脉导管未闭外不伴有其他心血管畸形时称为单纯型大动脉转位，半数的大动脉转位的患儿均属于此类型。 | [
{
"id": 0,
"entity": "大动脉转位",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "合并卵圆孔",
"start_offset": 6,
"end_offset": 11,
"label": "sym"
},
{
"id": 2,
"entity": "动脉导管未闭外",
"start_offset": 12,
"end_offset": 19,
"label": ... |
另约40%～45%的患儿伴有室间隔缺损，同时伴有室间隔缺损及明显左室流出道梗阻者约占10%。 | [
{
"id": 0,
"entity": "室间隔缺损",
"start_offset": 14,
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"label": "sym"
},
{
"id": 1,
"entity": "室间隔缺损及明显左室流出道梗阻",
"start_offset": 24,
"end_offset": 39,
"label": "sym"
}
] |
其他合并畸形尚包括房室瓣异常、主动脉梗阻。 | [
{
"id": 0,
"entity": "房室瓣异常",
"start_offset": 9,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "主动脉梗阻",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
}
] |
室间隔缺损是最常见的伴发畸形，大动脉转位仅伴有室间隔缺损时，室间隔缺损的位置及大小差异较大，也可为多发性缺损。 | [
{
"id": 0,
"entity": "室间隔缺损",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "大动脉转位",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "室间隔缺损",
"start_offset": 23,
"end_offset": 28,
"label": "... |
最常见是膜部缺损及肌部缺损。 | [
{
"id": 0,
"entity": "膜部缺损",
"start_offset": 4,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "肌部缺损",
"start_offset": 9,
"end_offset": 13,
"label": "dis"
}
] |
左心室流出道梗阻会引起肺动脉狭窄。 | [
{
"id": 0,
"entity": "左心室流出道梗阻",
"start_offset": 0,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "肺动脉狭窄",
"start_offset": 11,
"end_offset": 16,
"label": "sym"
}
] |
梗阻通常为瓣下型，可由纤维膜、纤维肌性隧道或流出道室间隔向后移位引起。 | [
{
"id": 0,
"entity": "梗阻",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "瓣下型",
"start_offset": 5,
"end_offset": 8,
"label": "dis"
},
{
"id": 2,
"entity": "纤维膜、纤维肌性隧道或流出道室间隔向后移位引起",
"start_offset": 11,
"end_offset": 34,
... |
另一类肺血管异常属于亚临床型，是指肺血流较易进入右肺，为左心室流出道位置异常而引起的，但这种异常的肺血流分布对总的临床症状的影响意义不大。 | [
{
"id": 0,
"entity": "肺血管异常",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "肺血流",
"start_offset": 17,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "右肺",
"start_offset": 24,
"end_offset": 26,
"label": "bod"
... |
当存在大的循环间分流，如大的动脉导管未闭或大的室间隔缺损时，因交换血流量较多，则表现为以充血性心力衰竭症状为主，伴有轻度发绀。 | [
{
"id": 0,
"entity": "动脉导管未闭",
"start_offset": 14,
"end_offset": 20,
"label": "sym"
},
{
"id": 1,
"entity": "室间隔",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
},
{
"id": 2,
"entity": "血流量",
"start_offset": 33,
"end_offset": 36,
"label": "i... |
伴有粗大的动脉导管未闭的患儿通常在生后1周内出现症状，典型的表现为水冲脉，连续性杂音可不明显；伴有大型室间隔缺损的婴儿，通常在生后2～4周出现心力衰竭症状。 | [
{
"id": 0,
"entity": "动脉导管未闭",
"start_offset": 5,
"end_offset": 11,
"label": "sym"
},
{
"id": 1,
"entity": "水冲脉",
"start_offset": 33,
"end_offset": 36,
"label": "sym"
},
{
"id": 2,
"entity": "连续性杂音可不明显",
"start_offset": 37,
"end_offset": 46,
"label... |
随着肺动脉高压的发展，逐渐出现第三心音、心力衰竭引起的奔马律及肺动脉第二音亢进，在心尖处可闻及因肺静脉血流增加而产生的舒张中期杂音。 | [
{
"id": 0,
"entity": "肺动脉高压",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "第三心音",
"start_offset": 15,
"end_offset": 19,
"label": "sym"
},
{
"id": 2,
"entity": "心力衰竭引起的奔马律",
"start_offset": 20,
"end_offset": 30,
"label... |
球囊房隔造口术可作为最初的姑息性手术，该手术在第一章节中详细介绍。 | [
{
"id": 0,
"entity": "球囊房隔造口术",
"start_offset": 0,
"end_offset": 7,
"label": "pro"
}
] |
主要有两种术式：Mustard手术和Senning手术。 | [
{
"id": 0,
"entity": "Mustard手术",
"start_offset": 8,
"end_offset": 17,
"label": "pro"
},
{
"id": 1,
"entity": "Senning手术",
"start_offset": 18,
"end_offset": 27,
"label": "pro"
}
] |
一些伴有多发孔洞型室间隔缺损的患儿，可先行肺动脉环缩一期姑息手术。 | [
{
"id": 0,
"entity": "多发孔洞型室间隔缺损",
"start_offset": 4,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "肺动脉环缩一期姑息手术",
"start_offset": 21,
"end_offset": 32,
"label": "pro"
}
] |
近来，对伴有室间隔缺损、左心室流出道梗阻并有严重低氧血症的新生儿，可先行体肺动脉分流术，然后再行Rastelli手术。 | [
{
"id": 0,
"entity": "室间隔缺损",
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"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "左心室流出道梗阻",
"start_offset": 12,
"end_offset": 20,
"label": "sym"
},
{
"id": 2,
"entity": "低氧血症",
"start_offset": 24,
"end_offset": 28,
"label"... |
尽管Rastelli手术的术后的成功率在1～2岁的幼儿较高，但在婴儿期仍可进行。 | [
{
"id": 0,
"entity": "Rastelli手术",
"start_offset": 2,
"end_offset": 12,
"label": "pro"
}
] |
Rastelli手术的缺点在于将来需做人工管道替换手术。 | [
{
"id": 0,
"entity": "Rastelli手术",
"start_offset": 0,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "人工管道替换手术",
"start_offset": 19,
"end_offset": 27,
"label": "pro"
}
] |
此方案避免了将来的人工管道替换术，具有一定的发展潜力，但术后可引起肺动脉反流。 | [
{
"id": 0,
"entity": "人工管道替换术",
"start_offset": 9,
"end_offset": 16,
"label": "pro"
},
{
"id": 1,
"entity": "肺动脉反流",
"start_offset": 33,
"end_offset": 38,
"label": "sym"
}
] |
当有严重水肿或主动脉断离伴主动脉发育不全时，可通过胸部正中切口同时完成大动脉转换术、室间隔缺损修补术及主动脉弓重建术，但手术危险性较大。 | [
{
"id": 0,
"entity": "严重水肿",
"start_offset": 2,
"end_offset": 6,
"label": "sym"
},
{
"id": 1,
"entity": "主动脉断离伴主动脉发育不全",
"start_offset": 7,
"end_offset": 20,
"label": "sym"
},
{
"id": 2,
"entity": "胸部",
"start_offset": 25,
"end_offset": 27,
"label"... |
术后最重要的并发症是晚期猝死，约占2%～8%，房性扑动可能是晚期死亡的高危因素。 | [
{
"id": 0,
"entity": "晚期猝死",
"start_offset": 10,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "房性扑动",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
}
] |
由于常同时并发心动过速和心动过缓，抗心律失常药应用难度较大。 | [
{
"id": 0,
"entity": "心动过速",
"start_offset": 7,
"end_offset": 11,
"label": "sym"
},
{
"id": 1,
"entity": "心动过缓",
"start_offset": 12,
"end_offset": 16,
"label": "sym"
},
{
"id": 2,
"entity": "抗心律失常药",
"start_offset": 17,
"end_offset": 23,
"label": "... |
尽管大多患儿并没有相应临床症状，术后有右心室功能不良已有大量的文献报道。 | [
{
"id": 0,
"entity": "右心室",
"start_offset": 19,
"end_offset": 22,
"label": "bod"
}
] |
Losay等对1095例大动脉转换术患儿的远期随访结果表明：单纯的大动脉转位患儿术后1年、10年、15年生存率均为92%。 | [
{
"id": 0,
"entity": "大动脉转换术",
"start_offset": 12,
"end_offset": 18,
"label": "pro"
},
{
"id": 1,
"entity": "大动脉转位",
"start_offset": 33,
"end_offset": 38,
"label": "dis"
}
] |
大动脉转换术后有近80%患儿术后10年无须再次治疗。 | [
{
"id": 0,
"entity": "大动脉转换术",
"start_offset": 0,
"end_offset": 6,
"label": "pro"
}
] |
重叠综合征的治疗较治疗单纯某一种疾病困难。 | [
{
"id": 0,
"entity": "重叠综合征",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
}
] |
与MCTD相比，重叠综合征的预后更差。 | [
{
"id": 0,
"entity": "MCTD",
"start_offset": 1,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "重叠综合征",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
}
] |
最早症状为厌食，继之出现体重减轻、低热、精神不振、恶心、呕吐、顽固性便秘、嗜睡、表情淡漠，年长儿诉头痛，重者或晚期可出现高热、多尿、烦躁、脱水、昏迷、抽搐等症状。 | [
{
"id": 0,
"entity": "厌食",
"start_offset": 5,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "体重减轻",
"start_offset": 12,
"end_offset": 16,
"label": "sym"
},
{
"id": 2,
"entity": "低热",
"start_offset": 17,
"end_offset": 19,
"label": "sym"
... |
严重者可因高钙血症导致主动脉瓣钙化及狭窄、肾钙化及肾衰竭而致死。 | [
{
"id": 0,
"entity": "高钙血症",
"start_offset": 5,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "主动脉瓣钙化及狭窄",
"start_offset": 11,
"end_offset": 20,
"label": "sym"
},
{
"id": 2,
"entity": "肾钙化",
"start_offset": 21,
"end_offset": 24,
"label": ... |
治疗原则为主要防治胱氨酸结石形成并治疗其并发症。 | [
{
"id": 0,
"entity": "胱氨酸结石",
"start_offset": 9,
"end_offset": 14,
"label": "dis"
}
] |
在睡前还可服用醋唑酰胺一次，剂量为5～10mg/kg。 | [
{
"id": 0,
"entity": "醋唑酰胺",
"start_offset": 7,
"end_offset": 11,
"label": "dru"
}
] |
本药不良反应常有皮疹、发热、关节痛、骨髓抑制、类狼疮反应以及肾损害（肾病综合征）等，因此，只用于上述一般治疗不能控制以及出现严重胱氨酸结石的病例。 | [
{
"id": 0,
"entity": "皮疹",
"start_offset": 8,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "发热",
"start_offset": 11,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "关节痛",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
... |
较新的药物如N-乙酰-D-青霉胺（N-acety1-D-penicillamine），毒性较低，有相同效果。 | [
{
"id": 0,
"entity": "N-乙酰-D-青霉胺",
"start_offset": 6,
"end_offset": 16,
"label": "dru"
},
{
"id": 1,
"entity": "N-acety1-D-penicillamine",
"start_offset": 17,
"end_offset": 41,
"label": "dru"
}
] |
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