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即使在无症状患者中肝铜含量通常也可升高。 | [
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肝活体组织检查对临床症状不典型、其他检查不能确诊、症状出现前的患者，以及对WD患者家族成员的筛查有诊断意义。 | [
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铜的沉积主要在门静脉区及纤维隔处，因此在进行诊断及治疗后随访时，要考虑铜分布的显著差异。 | [
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另外，其他疾病也可导致肝铜含量增高，如慢性活动性肝炎、胆汁淤积性肝硬化及任何原因所致的长时间的肝外胆管阻塞的病人，但血浆铜蓝蛋白正常或增加可与WD相鉴别。 | [
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4.影像学检查MRI对WD的诊断优于CT，测定T1</sub>及T2</sub>迟缓时间能反映WD治疗结果，判断症状改善或变化。 | [
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MRI改变呈双侧对称一致类型。 | [
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T2</sub>加权像低信号为本病较具特殊性的改变，其主要改变位于壳核及尾状核，其次为脑萎缩及皮质下白质损害，病变还见于顶盖、大脑导水管周围灰质、红核和小脑深部齿状核，且分布与某些神经体征有很好的相关性。 | [
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5.基因诊断随着分子生物学的发展，基因诊断成为本病最可靠、最有前途的诊断方法。 | [
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20世纪80年代应用探针确定WD的基因位点在13号染色体长臂13q14-q21，此后经连锁分析，认为本病的基因位点D13S13远端0.4cM、D13S59近端1.2cM处，并提出13q14.3区的D13S31远端0.4cM和D13S59间长约2cM的DNA区是筛选WD后选基因的最佳片段处。 | [
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WD蛋白有3个功能区：第一区为金属离子结合区；第二区为功能区，WD基因突变常涉及此区；第三区为跨膜区。 | [
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WD患者的DNA分析显示也能有500多种突变形式，且大多数患者为复杂的杂合子，可能同时携带2种不同的突变形式，使DNA研究更为复杂。 | [
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另外，WD存在高度异质性，排除了大多数患者在DNA水平进行诊断的可能，但是在不同的人群中对常见的突变形式进行分析仍不失为一诊断手段。 | [
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①间接基因诊断：在有先证者的情况下，可采用多态标记连锁分析对家系中其他成员进行间接基因诊断。 | [
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目前限制性片段长度多态性连锁分析（RFLP）已用于WD诊断、症状前诊断、产前诊断和携带者的检测。 | [
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因此对常规检查不能确诊的患者可采用RFLP，达到早期诊断的目的。 | [
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保证RFLP准确有效的前提是家族中先有WD患者，家系中能提供组织标本成员的数量。 | [
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②直接基因诊断：对临床可疑但家系中无先证者的患者，可直接监测ATP7B基因突变。 | [
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本病的基因位于13q14.3，我国Wilson病患者的ATP7B基因突变有3个突变热点，即R778L、P992L及T935M，占所有突变的60%左右。 | [
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综上所述，结合家族史、临床表现及上述实验诊断方法可较准确地诊断WD。 | [
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【治疗】治疗的目的是重新维持肝内铜的自身稳定，降低组织内铜的含量，并解除铜的毒性作用。 | [
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（一）螯合剂青霉胺可通过与铜螯合促进WD患儿尿铜排泄增加铜在症状前患者体内的蓄积。 | [
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青霉胺治疗的试验性口服剂量开始一般为15～25mg/（kg•d），如果能够耐受逐渐增大剂量至40mg/（kg•d），每日分4次，饭前2小时口服，大多数患者在服药后4个月症状消失。 | [
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但是由于螯合剂治疗后可引起体内铜的再分布，肝铜入脑，累及基底神经节，从而加重神经系统症状。 | [
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因此，有报道20%有明显神经系统症状WD患儿接受青霉胺治疗后反而神经系统症状铜含量降低到一定程度（血清游离铜含量＜10mg/dl，尿铜排泄＜80mg/d），则给予常规治疗剂量的一半终身维持治疗。 | [
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坚持服药至关重要，因为据报道突然停用青霉胺的患者会导致病情急剧恶化。 | [
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在应用青霉胺治疗的过程中，需定期监测尿常规和血常规，因为过敏反应如发热、皮疹、淋巴结病以及血恶液质等不良反应较为常见。 | [
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此外，20%～25%的WD患者服用青霉胺后可出现严重的不良反应如SLE和肾病综合征。 | [
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一旦发生上述青霉胺诱发的自身免疫性病症，需立即停药，并改用曲恩汀（化学名为二盐酸三甲烯羟化四甲胺，triethylenetetra-minedihydrochloride）。 | [
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曲恩汀也是一种络合剂，可促进铜的排出，有时可作为伴有神经系统症状WD患者的一线药物，对各型患者均有效，一般剂量为40～50mg/（kg•d）。 | [
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某些患者应用后出现血浆铁浓度下降青霉胺同时加用维生素B6</sub>，可避免维生素B6</sub>缺乏引起的癫痫发作，服用时加用左旋多巴疗效更好。 | [
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"label": "bod"
},
{
"id": 1,
"entity": "血浆铁浓度下降",
"start_offset": 9,
"end_offset": 16,
"label": "sym"
},
{
"id": 2,
"entity": "青霉胺",
"start_offset": 16,
"end_offset": 19,
"label": "dru"
},
{
"id": 3,
"entity": "维生素B6",
"start_offset": 23,
"end_offset": 28,
"label": "dru"
},
{
"id": 4,
"entity": "维生素B6",
"start_offset": 38,
"end_offset": 43,
"label": "dru"
},
{
"id": 5,
"entity": "癫痫",
"start_offset": 54,
"end_offset": 56,
"label": "dis"
},
{
"id": 6,
"entity": "左旋多巴",
"start_offset": 64,
"end_offset": 68,
"label": "dru"
}
] |
二巯基丙磺酸钠推荐用于有轻、中、重度肝损害和神经精神症状患者。 | [
{
"id": 0,
"entity": "二巯基丙磺酸钠",
"start_offset": 0,
"end_offset": 7,
"label": "dru"
},
{
"id": 1,
"entity": "重度肝损害",
"start_offset": 16,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "神经精神症状",
"start_offset": 22,
"end_offset": 28,
"label": "dis"
}
] |
（二）锌剂用于WD起始治疗和维持治疗，也用于不能耐受青霉胺患者的替代治疗和螯合剂治疗导致锌缺乏的补充治疗。 | [
{
"id": 0,
"entity": "锌剂",
"start_offset": 3,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "WD",
"start_offset": 7,
"end_offset": 9,
"label": "dis"
},
{
"id": 2,
"entity": "青霉胺",
"start_offset": 26,
"end_offset": 29,
"label": "dru"
},
{
"id": 3,
"entity": "螯合剂治疗",
"start_offset": 37,
"end_offset": 42,
"label": "pro"
},
{
"id": 4,
"entity": "锌缺乏",
"start_offset": 44,
"end_offset": 47,
"label": "dis"
}
] |
锌剂的作用机制是使WD患者肠道内金属硫因（MT）合成增加，MT对铜有高度亲和力，肠道内MT增加，阻止铜吸收入血。 | [
{
"id": 0,
"entity": "锌剂",
"start_offset": 0,
"end_offset": 2,
"label": "dru"
},
{
"id": 1,
"entity": "WD",
"start_offset": 9,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "肠道",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 3,
"entity": "金属硫因",
"start_offset": 16,
"end_offset": 20,
"label": "bod"
},
{
"id": 4,
"entity": "MT",
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"label": "bod"
},
{
"id": 5,
"entity": "MT",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 6,
"entity": "铜",
"start_offset": 32,
"end_offset": 33,
"label": "bod"
},
{
"id": 7,
"entity": "肠道",
"start_offset": 40,
"end_offset": 42,
"label": "bod"
},
{
"id": 8,
"entity": "MT",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
},
{
"id": 9,
"entity": "铜",
"start_offset": 50,
"end_offset": 51,
"label": "bod"
},
{
"id": 10,
"entity": "血",
"start_offset": 54,
"end_offset": 55,
"label": "bod"
}
] |
同时肠道上皮细胞更新加剧，细胞脱落，铜由粪便排出，逐渐形成一个慢性铜负平衡。 | [
{
"id": 0,
"entity": "肠道上皮细胞",
"start_offset": 2,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "细胞",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "铜",
"start_offset": 18,
"end_offset": 19,
"label": "bod"
},
{
"id": 3,
"entity": "铜",
"start_offset": 33,
"end_offset": 34,
"label": "bod"
}
] |
锌剂安全有效、低毒，但起效慢。 | [
{
"id": 0,
"entity": "锌剂",
"start_offset": 0,
"end_offset": 2,
"label": "dru"
}
] |
锌剂用法：5～7.5mg/（kg•d），餐前1小时服用，与青霉胺间隔至少1小时。 | [
{
"id": 0,
"entity": "锌剂",
"start_offset": 0,
"end_offset": 2,
"label": "dru"
},
{
"id": 1,
"entity": "青霉胺",
"start_offset": 29,
"end_offset": 32,
"label": "dru"
}
] |
（三）低铜饮食在治疗初期，饮食中铜的含量应少于1mg/d，之后随着症状的控制可以增加至1.0～1.5mg/d。 | [
{
"id": 0,
"entity": "低铜饮食",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "铜",
"start_offset": 16,
"end_offset": 17,
"label": "bod"
}
] |
含铜量低的食物有精白米面、萝卜、藕、芹菜、小白菜、瘦猪肉、瘦鸡、瘦鸭、牛奶以及马铃薯等，牛奶不仅含铜量低，长期服用尚有轻度排铜作用。 | [
{
"id": 0,
"entity": "铜",
"start_offset": 1,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "铜",
"start_offset": 49,
"end_offset": 50,
"label": "bod"
},
{
"id": 2,
"entity": "铜",
"start_offset": 62,
"end_offset": 63,
"label": "bod"
}
] |
相反，铜含量高的食物有肥猪肉、动物内脏和小牛肉等肉类；蟹、虾、鱼和贝类等，黄豆、青豆和扁豆等豆类；花生、芝麻和胡桃等坚果类；蘑菇、牡蛎、蜈蚣以及全蝎等。 | [
{
"id": 0,
"entity": "铜",
"start_offset": 3,
"end_offset": 4,
"label": "bod"
}
] |
这些含铜量高的食物应禁止食用。 | [
{
"id": 0,
"entity": "铜",
"start_offset": 3,
"end_offset": 4,
"label": "bod"
}
] |
（四）肝移植患有进行肝衰竭、螯合剂治疗无效或由暴发性肝炎引起的急性肝衰竭患者，可考虑肝脏移植。 | [
{
"id": 0,
"entity": "肝移植",
"start_offset": 3,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "进行肝衰竭",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "螯合剂治疗",
"start_offset": 14,
"end_offset": 19,
"label": "pro"
},
{
"id": 3,
"entity": "暴发性肝炎",
"start_offset": 23,
"end_offset": 28,
"label": "dis"
},
{
"id": 4,
"entity": "急性肝衰竭",
"start_offset": 31,
"end_offset": 36,
"label": "dis"
},
{
"id": 5,
"entity": "肝脏移植",
"start_offset": 42,
"end_offset": 46,
"label": "pro"
}
] |
肝脏移植可以在6个月内使铜的自身稳定达到正常水平，并能持续改善临床症状，包括神经和精神性疾病。 | [
{
"id": 0,
"entity": "肝脏移植",
"start_offset": 0,
"end_offset": 4,
"label": "pro"
},
{
"id": 1,
"entity": "铜",
"start_offset": 12,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "神经和精神性疾病",
"start_offset": 38,
"end_offset": 46,
"label": "dis"
}
] |
由于肝细胞增殖能力有限，很难长时间维持其功能，同时存在的免疫排斥反应，尚需进一步研究。 | [
{
"id": 0,
"entity": "肝细胞",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
}
] |
（五）基因治疗随着WD基因研究的进一步深入，基因治疗将为WD病的治疗开拓新的领域。 | [
{
"id": 0,
"entity": "基因治疗",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "WD基因",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "WD",
"start_offset": 28,
"end_offset": 30,
"label": "dis"
}
] |
然而，想通过此方法有效治疗WD病，尚需对基因产物的特征有更多的了解，尚需有更有效的方法将基因导入所有肝细胞内。 | [
{
"id": 0,
"entity": "WD",
"start_offset": 13,
"end_offset": 15,
"label": "dis"
},
{
"id": 1,
"entity": "肝细胞",
"start_offset": 50,
"end_offset": 53,
"label": "bod"
}
] |
中华医学会神经病学分会帕金森病及运动障碍学组及中华医学会神经病学分会神经遗传病学组肝豆状核变性的诊断与治疗指南（2008年）：1.推荐症状前患者的治疗以及治疗有效患者的维持治疗，可用络合剂和锌剂。 | [
{
"id": 0,
"entity": "神经病",
"start_offset": 5,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "帕金森病",
"start_offset": 11,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "运动障碍",
"start_offset": 16,
"end_offset": 20,
"label": "dis"
},
{
"id": 3,
"entity": "神经病",
"start_offset": 28,
"end_offset": 31,
"label": "dis"
},
{
"id": 4,
"entity": "肝豆状核变性",
"start_offset": 41,
"end_offset": 47,
"label": "dis"
},
{
"id": 5,
"entity": "络合剂",
"start_offset": 91,
"end_offset": 94,
"label": "dru"
},
{
"id": 6,
"entity": "锌剂",
"start_offset": 95,
"end_offset": 97,
"label": "dru"
}
] |
2.药物治疗的监测开始用药后应检查肝肾功能、24小时尿铜以及血尿常规等，前3个月每月复查一次，病情稳定后3个月检查一次。 | [
{
"id": 0,
"entity": "肝肾功能",
"start_offset": 17,
"end_offset": 21,
"label": "ite"
},
{
"id": 1,
"entity": "24小时尿铜",
"start_offset": 22,
"end_offset": 28,
"label": "ite"
},
{
"id": 2,
"entity": "血尿常规",
"start_offset": 30,
"end_offset": 34,
"label": "ite"
}
] |
每3～6个月检查一次肝脾B超。 | [
{
"id": 0,
"entity": "肝脾B超",
"start_offset": 10,
"end_offset": 14,
"label": "pro"
}
] |
3.除严重肢体痉挛、畸形、严重构音障碍的脑型患者及对青霉胺过敏的患者慎用或不用外，其他类型患者均适用青霉胺。 | [
{
"id": 0,
"entity": "严重肢体痉挛",
"start_offset": 3,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "畸形",
"start_offset": 10,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "严重构音障碍",
"start_offset": 13,
"end_offset": 19,
"label": "dis"
},
{
"id": 3,
"entity": "青霉胺",
"start_offset": 26,
"end_offset": 29,
"label": "dru"
},
{
"id": 4,
"entity": "青霉胺",
"start_offset": 50,
"end_offset": 53,
"label": "dru"
}
] |
由于青霉胺疗效肯定、药源充足、价格低廉以及使用方便，目前在我国仍作为治疗本病的主要药物。 | [
{
"id": 0,
"entity": "青霉胺",
"start_offset": 2,
"end_offset": 5,
"label": "dru"
}
] |
4.对症治疗①静止性且幅度较小的震颤首选苯海索1mg，每日2次，如症状缓解不明显，可加用复方多巴类制剂。 | [
{
"id": 0,
"entity": "静止性且幅度较小的震颤",
"start_offset": 7,
"end_offset": 18,
"label": "sym"
},
{
"id": 1,
"entity": "苯海索",
"start_offset": 20,
"end_offset": 23,
"label": "dru"
},
{
"id": 2,
"entity": "复方多巴类制剂",
"start_offset": 44,
"end_offset": 51,
"label": "dru"
}
] |
意向性震颤且粗大震颤首选氯硝西泮0.5mg，每日1次或2次。 | [
{
"id": 0,
"entity": "意向性震颤且粗大震颤",
"start_offset": 0,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "氯硝西泮",
"start_offset": 12,
"end_offset": 16,
"label": "dru"
}
] |
合并严重肌张力增高者可选用氯氮平或奥氮平。 | [
{
"id": 0,
"entity": "严重肌张力增高",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "氯氮平",
"start_offset": 13,
"end_offset": 16,
"label": "dru"
},
{
"id": 2,
"entity": "奥氮平",
"start_offset": 17,
"end_offset": 20,
"label": "dru"
}
] |
⑥白细胞和血小板减少白细胞药物，仍不能纠正时应减用或停用青霉胺，改用其他驱铜药物。 | [
{
"id": 0,
"entity": "白细胞",
"start_offset": 1,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "血小板",
"start_offset": 5,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "白细胞和血小板减少",
"start_offset": 1,
"end_offset": 10,
"label": "sym"
},
{
"id": 3,
"entity": "白细胞",
"start_offset": 10,
"end_offset": 13,
"label": "bod"
},
{
"id": 4,
"entity": "青霉胺",
"start_offset": 28,
"end_offset": 31,
"label": "dru"
},
{
"id": 5,
"entity": "铜",
"start_offset": 37,
"end_offset": 38,
"label": "bod"
}
] |
如仍无效，可施行脾切除术。 | [
{
"id": 0,
"entity": "脾切除术",
"start_offset": 8,
"end_offset": 12,
"label": "pro"
}
] |
⑦暴发性肝功能衰竭铜（血液透析以及新鲜冰冻血浆进行血浆置换），尽快给予肝脏移植手术。 | [
{
"id": 0,
"entity": "肝功能",
"start_offset": 4,
"end_offset": 7,
"label": "ite"
},
{
"id": 1,
"entity": "暴发性肝功能衰竭",
"start_offset": 1,
"end_offset": 9,
"label": "sym"
},
{
"id": 2,
"entity": "铜",
"start_offset": 9,
"end_offset": 10,
"label": "bod"
},
{
"id": 3,
"entity": "血液透析",
"start_offset": 11,
"end_offset": 15,
"label": "pro"
},
{
"id": 4,
"entity": "新鲜冰冻血浆",
"start_offset": 17,
"end_offset": 23,
"label": "dru"
},
{
"id": 5,
"entity": "血浆置换",
"start_offset": 25,
"end_offset": 29,
"label": "pro"
},
{
"id": 6,
"entity": "肝脏移植手术",
"start_offset": 35,
"end_offset": 41,
"label": "pro"
}
] |
第十五章中枢神经系统慢感染中枢神经系统慢感染（slowinfectionsofthecentralnervoussystem）潜伏期很长（一般1年以上），起病后持续发展，缺乏有效治疗手段，预后不良。 | [
{
"id": 0,
"entity": "中枢神经系统慢感染",
"start_offset": 4,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "中枢神经系统慢感染",
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"end_offset": 22,
"label": "dis"
},
{
"id": 2,
"entity": "slowinfectionsofthecentralnervoussystem",
"start_offset": 23,
"end_offset": 62,
"label": "dis"
}
] |
中枢神经系统慢感染可分为两类：①慢病毒感染（slowvirusinfection），由传统的病毒引起；②非传统病毒引起的慢感染（slowinfectionswithunconventionalviruses），病原体很小，但与一般病毒的结构不同，其所引起的疾病统称为可传播性海绵样脑病（transmissiblespongiformencephalopathies），又称为朊蛋白病（priondiseases），包括疯牛（羊）病和人类克-雅病（Creutzfeldt-Jakobdisease，CJD）及库鲁（Kuru）病。 | [
{
"id": 0,
"entity": "中枢神经系统慢感染",
"start_offset": 0,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "慢病毒感染",
"start_offset": 16,
"end_offset": 21,
"label": "dis"
},
{
"id": 2,
"entity": "slowvirusinfection",
"start_offset": 22,
"end_offset": 40,
"label": "dis"
},
{
"id": 3,
"entity": "病毒",
"start_offset": 46,
"end_offset": 48,
"label": "mic"
},
{
"id": 4,
"entity": "非传统病毒引起的慢感染",
"start_offset": 52,
"end_offset": 63,
"label": "dis"
},
{
"id": 5,
"entity": "slowinfectionswithunconventionalviruses",
"start_offset": 64,
"end_offset": 103,
"label": "dis"
},
{
"id": 6,
"entity": "病原体",
"start_offset": 105,
"end_offset": 108,
"label": "mic"
},
{
"id": 7,
"entity": "病毒",
"start_offset": 115,
"end_offset": 117,
"label": "mic"
},
{
"id": 8,
"entity": "可传播性海绵样脑病",
"start_offset": 133,
"end_offset": 142,
"label": "dis"
},
{
"id": 9,
"entity": "transmissiblespongiformencephalopathies",
"start_offset": 143,
"end_offset": 182,
"label": "dis"
},
{
"id": 10,
"entity": "朊蛋白病",
"start_offset": 187,
"end_offset": 191,
"label": "dis"
},
{
"id": 11,
"entity": "priondiseases",
"start_offset": 192,
"end_offset": 205,
"label": "dis"
},
{
"id": 12,
"entity": "疯牛（羊）病",
"start_offset": 209,
"end_offset": 215,
"label": "dis"
},
{
"id": 13,
"entity": "人类克-雅病",
"start_offset": 216,
"end_offset": 222,
"label": "dis"
},
{
"id": 14,
"entity": "Creutzfeldt-Jakobdisease",
"start_offset": 223,
"end_offset": 247,
"label": "dis"
},
{
"id": 15,
"entity": "CJD",
"start_offset": 248,
"end_offset": 251,
"label": "dis"
},
{
"id": 16,
"entity": "库鲁（Kuru）病",
"start_offset": 253,
"end_offset": 262,
"label": "dis"
}
] |
本组疾病罕见，共同特点包括：①初始病毒或朊蛋白感染，可有典型表现，如麻疹，也可为隐匿性感染；②在一段较长（通常1年以上）的无症状的潜伏期之后再次出现新的症状，通常仅表现为脑病；③感染的影响局限于神经系统发病后病情持续加重，一般经数月至数年最终导致死亡。 | [
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{
"id": 4,
"entity": "脑病",
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{
"id": 5,
"entity": "神经系统",
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{
"id": 6,
"entity": "感染的影响局限于神经系统",
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},
{
"id": 7,
"entity": "发病后病情持续加重，一般经数月至数年最终导致死亡",
"start_offset": 101,
"end_offset": 125,
"label": "sym"
}
] |
第一节慢病毒感染慢病毒疾病系指一组由普通病毒引起的以慢性进行性脑病为主要表现的综合征，是神经系统慢性持续性病毒感染的结果。 | [
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"entity": "慢性进行性脑病",
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"entity": "神经系统慢性持续性病毒感染",
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] |
包括：①亚急性硬化性全脑炎（subacutesclerosingpanencephalitis，SSPE）；②进行性风疹全脑炎（progressiverubellapanencephalitis）；③进行性多灶性白质脑炎（progressivemultifocalleukoencephalitis）；④直接逆转录病毒脑病（directretrovirusencephalopathy）。 | [
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"entity": "subacutesclerosingpanencephalitis",
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"entity": "progressivemultifocalleukoencephalitis",
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{
"id": 7,
"entity": "直接逆转录病毒脑病",
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"label": "dis"
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{
"id": 8,
"entity": "directretrovirusencephalopathy",
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"end_offset": 192,
"label": "dis"
}
] |
以下主要介绍SSPE。 | [
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【病因和发病机制】SSPE是由麻疹病毒引起的慢病毒感染，是波及全脑的炎症性变性病。 | [
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{
"id": 4,
"entity": "炎症性变性病",
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"label": "dis"
}
] |
有人认为病毒突变是一个重要原因；也有人认为SSPE病人有轻微的免疫缺陷，例如婴儿期患麻疹后发生SSPE的危险性增加，提示免疫功能不成熟可能与之有关。 | [
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{
"id": 3,
"entity": "SSPE",
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"label": "dis"
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] |
SSPE病人脑中并未找到完整的麻疹病毒颗粒，也未见到其基质蛋白（Mprotein），但是麻疹病毒编码各种蛋白质所需的遗传物质全都存在，且是功能完整的。 | [
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"id": 4,
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{
"id": 6,
"entity": "蛋白质",
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"label": "bod"
}
] |
对此，有的研究提示，基质蛋白虽然已被编码，但由于有突变，故不能与病毒粒子结合，导致不完整麻疹病毒的产生和蓄积，因此病人的抗体和细胞免疫均不能将其清除。 | [
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"entity": "细胞",
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] |
本病神经系统症状大多出现于麻疹病毒感染后7～11年，平均8年。 | [
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根据其典型的临床表现，本病可分为4期：第1期（早期）典型表现包括行为改变、嗜睡、疲倦、学校适应困难、非频发性癫痫发作、多动以及性格变化等。 | [
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"entity": "非频发性癫痫发作",
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{
"id": 6,
"entity": "性格变化",
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] |
通过神经功能不全量表（neurologicdisabilityscale）定量研究发现，此期神经功能下降水平不超过30%。 | [
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{
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"entity": "神经功能",
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"label": "ite"
}
] |
不同病人此期进展速度各异，取决于灰质脑炎的严重程度以及病变向皮层下发展的快慢。 | [
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] |
当大脑皮层灰质病变恶化并开始向下波及皮层下白质和深部灰质时，肌阵挛逐渐明显，即进展到本病的第2期。 | [
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"entity": "肌阵挛",
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"label": "sym"
}
] |
第2期肌阵挛是本期的特征性表现，常随病程发展渐渐发生，并逐渐累及全身所有肌群，特别是躯干轴部肌群。 | [
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"entity": "躯干轴部肌群",
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] |
肌阵挛的特点包括弥漫性、重复性和频发性，大多为对称性出现，常有相对固定的间隔，全身性肌阵挛一般每5～10秒发生一次。 | [
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其发生是锥体外系广泛的刺激性病变所致，而非大脑皮层神经元异常放电所致的癫痫发作。 | [
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{
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] |
除了有明显的不自主运动外，出现了中枢神经运动或感觉长束受累癫痫和痴呆也进一步恶化。 | [
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{
"id": 3,
"entity": "痴呆",
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] |
第3期开始于病变进展累及皮层下灰质核团和脑干以后，以进行性智力和运动衰退为标志。 | [
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"entity": "运动衰退",
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] |
代表锥体外系刺激性病变的肌阵挛消失。 | [
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] |
第4期由于大脑功能丧失脑干、脊髓上段的广泛受累出现严重的自主神经功能异常全身重度弛缓或强直以及自主神经功能衰竭死亡。 | [
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{
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{
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{
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{
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"entity": "自主神经功能",
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{
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"entity": "自主神经功能衰竭",
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{
"id": 10,
"entity": "死亡",
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] |
SSPE病人发病后大多按上述4期顺序发展，每期持续数月，于2～4年左右死亡。 | [
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{
"id": 1,
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] |
但有些病例病情进展十分迅速，很快导致死亡，上述各期难以准确区分。 | [
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] |
而另一些病人进展很慢，至死亡时尚未进展至第4期。 | [
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] |
此外，在SSPE的任何一期都有可能出现病情相对静止，或呈波动性病程。 | [
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] |
分析各期神经功能丧失的程度发现，SSPE的病期和神经功能丧失的程度之间存在非常密切的相关性。 | [
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] |
如果将所有4期神经功能丧失的总量定为100%，则在1～4期中神经功能丧失的构成比例依次为0%～30%，31%～55%，55%～80%，81%～100%。 | [
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{
"id": 1,
"entity": "神经功能丧失",
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] |
典型SSPE每一病期可持续几个月。 | [
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}
] |
由于存在较大的个体差异，有人建议根据病情进展的速度将其分为3型：①急性型：发病后3个月以内神经功能至少丧失66%，在6个月以内出现明确的4期和死亡，或神经功能丧失90%以上；②亚急性型：在9个月以内神经功能丧失至少66%，有典型分期；③慢性型：发病9个月以后神经功能丧失不足66%，没有典型的临床分期。 | [
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{
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{
"id": 4,
"entity": "神经功能丧失",
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"label": "sym"
}
] |
慢性型病人的肌阵挛或第2期的其他症状可能大大延续。 | [
{
"id": 0,
"entity": "肌阵挛",
"start_offset": 6,
"end_offset": 9,
"label": "sym"
}
] |
【实验室检查与诊断】目前对SSPE的诊断有赖于对病人临床特点和实验室资料的综合分析。 | [
{
"id": 0,
"entity": "SSPE",
"start_offset": 13,
"end_offset": 17,
"label": "dis"
}
] |
过去曾提出以下5点作为SSPE的诊断线索：①典型的临床表现；②特征性脑电图式样；③经脑活检或尸解证实的典型组织学改变；④脑脊液球蛋白增高总蛋白量的20%；⑤血清和脑脊液麻疹病毒抗体滴度升高血清中＞1∶24，脑脊液中＞1∶8（补体结合抗体）。 | [
{
"id": 0,
"entity": "SSPE",
"start_offset": 11,
"end_offset": 15,
"label": "dis"
},
{
"id": 1,
"entity": "脑电图",
"start_offset": 34,
"end_offset": 37,
"label": "pro"
},
{
"id": 2,
"entity": "脑活检",
"start_offset": 42,
"end_offset": 45,
"label": "pro"
},
{
"id": 3,
"entity": "尸解",
"start_offset": 46,
"end_offset": 48,
"label": "pro"
},
{
"id": 4,
"entity": "脑脊液球蛋白",
"start_offset": 60,
"end_offset": 66,
"label": "bod"
},
{
"id": 5,
"entity": "脑脊液球蛋白增高",
"start_offset": 60,
"end_offset": 68,
"label": "sym"
},
{
"id": 6,
"entity": "总蛋白量",
"start_offset": 68,
"end_offset": 72,
"label": "ite"
},
{
"id": 7,
"entity": "血清",
"start_offset": 78,
"end_offset": 80,
"label": "bod"
},
{
"id": 8,
"entity": "脑脊液麻疹病毒抗体滴度",
"start_offset": 81,
"end_offset": 92,
"label": "ite"
},
{
"id": 9,
"entity": "血清和脑脊液麻疹病毒抗体滴度升高",
"start_offset": 78,
"end_offset": 94,
"label": "sym"
},
{
"id": 10,
"entity": "血清",
"start_offset": 94,
"end_offset": 96,
"label": "bod"
},
{
"id": 11,
"entity": "脑脊液",
"start_offset": 103,
"end_offset": 106,
"label": "bod"
},
{
"id": 12,
"entity": "补体",
"start_offset": 112,
"end_offset": 114,
"label": "bod"
},
{
"id": 13,
"entity": "抗体",
"start_offset": 116,
"end_offset": 118,
"label": "bod"
}
] |
CT和MRI技术的发展为本病诊断提供了无创性方法，且可作动态观察，可作为诊断的重要参考。 | [
{
"id": 0,
"entity": "CT",
"start_offset": 0,
"end_offset": 2,
"label": "pro"
},
{
"id": 1,
"entity": "MRI",
"start_offset": 3,
"end_offset": 6,
"label": "pro"
}
] |
脑脊液麻疹抗体检测迄今仍是诊断本病的特异性方法，因为已经证实脑脊液麻疹病毒抗体只能在中枢神经系统合成。 | [
{
"id": 0,
"entity": "脑脊液麻疹抗体检测",
"start_offset": 0,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "脑脊液麻疹病毒抗体",
"start_offset": 30,
"end_offset": 39,
"label": "bod"
},
{
"id": 2,
"entity": "中枢神经系统",
"start_offset": 42,
"end_offset": 48,
"label": "bod"
}
] |
综上所述，目前对SSPE的诊断只要具备相应的临床表现（不一定十分典型）以及脑脊液麻疹抗体升高两项条件即可建立诊断，如果还具备一些支持条件如麻疹病史或接种史、典型分期、脑电图异常脑脊液球蛋白升高异丙肌苷（isoprinosine）可能增加病人存活时间，对改善某些症状有所益处。 | [
{
"id": 0,
"entity": "SSPE",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "脑脊液麻疹抗体",
"start_offset": 37,
"end_offset": 44,
"label": "bod"
},
{
"id": 2,
"entity": "麻疹病",
"start_offset": 69,
"end_offset": 72,
"label": "dis"
},
{
"id": 3,
"entity": "脑电图",
"start_offset": 83,
"end_offset": 86,
"label": "pro"
},
{
"id": 4,
"entity": "脑电图异常",
"start_offset": 83,
"end_offset": 88,
"label": "sym"
},
{
"id": 5,
"entity": "脑脊液球蛋白",
"start_offset": 88,
"end_offset": 94,
"label": "bod"
},
{
"id": 6,
"entity": "脑脊液球蛋白升高",
"start_offset": 88,
"end_offset": 96,
"label": "sym"
},
{
"id": 7,
"entity": "异丙肌苷",
"start_offset": 96,
"end_offset": 100,
"label": "dru"
},
{
"id": 8,
"entity": "isoprinosine",
"start_offset": 101,
"end_offset": 113,
"label": "dru"
}
] |
2.干扰素鞘内注射或静脉注射，可延缓病情进展速度。 | [
{
"id": 0,
"entity": "干扰素",
"start_offset": 2,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "鞘内注射",
"start_offset": 5,
"end_offset": 9,
"label": "pro"
},
{
"id": 2,
"entity": "静脉注射",
"start_offset": 10,
"end_offset": 14,
"label": "pro"
}
] |
近有个例报告表明，鞘内注射IFN-α可能改善临床症状和MRI异常。 | [
{
"id": 0,
"entity": "鞘内注射IFN-α",
"start_offset": 9,
"end_offset": 18,
"label": "pro"
},
{
"id": 1,
"entity": "MRI",
"start_offset": 27,
"end_offset": 30,
"label": "pro"
}
] |
3.对症治疗包括止惊、防治感染、理疗及护理等，可减少并发症，延缓死亡，改善病人及家庭的生活质量。 | [
{
"id": 0,
"entity": "对症治疗",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "止惊",
"start_offset": 8,
"end_offset": 10,
"label": "pro"
},
{
"id": 2,
"entity": "防治感染",
"start_offset": 11,
"end_offset": 15,
"label": "pro"
},
{
"id": 3,
"entity": "理疗",
"start_offset": 16,
"end_offset": 18,
"label": "pro"
},
{
"id": 4,
"entity": "护理",
"start_offset": 19,
"end_offset": 21,
"label": "pro"
}
] |
六、氢呼气技术口服乳果糖2.0g/kg，收集呼气标本，测定氢浓度，确定收集到的呼出气体中氢气量突然增加的时间，该时间即为乳果糖到达盲肠的时间——小肠传递时间。 | [
{
"id": 0,
"entity": "氢呼气技术",
"start_offset": 2,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "乳果糖",
"start_offset": 9,
"end_offset": 12,
"label": "dru"
},
{
"id": 2,
"entity": "盲肠",
"start_offset": 65,
"end_offset": 67,
"label": "bod"
},
{
"id": 3,
"entity": "小肠",
"start_offset": 72,
"end_offset": 74,
"label": "bod"
}
] |
由于该检查受较多因素影响，因此，用于判定小肠传递时间仅限于成人。 | [
{
"id": 0,
"entity": "小肠",
"start_offset": 20,
"end_offset": 22,
"label": "bod"
}
] |
在小儿较多应用于诊断乳糖酶缺乏和碳水化合物吸收不良。 | [
{
"id": 0,
"entity": "乳糖酶缺乏",
"start_offset": 10,
"end_offset": 15,
"label": "dis"
},
{
"id": 1,
"entity": "碳水化合物吸收不良",
"start_offset": 16,
"end_offset": 25,
"label": "dis"
}
] |
六、肾移植后的并发症儿童肾移植后除了急慢性排斥反应外，另一些并发症，包括感染、高血压、高血脂、生长障碍以及恶性肿瘤等与肾病复发，都不同程度地影响着移植的预后。 | [
{
"id": 0,
"entity": "肾移植",
"start_offset": 2,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "儿童肾移植",
"start_offset": 10,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "急慢性排斥反应",
"start_offset": 18,
"end_offset": 25,
"label": "sym"
},
{
"id": 3,
"entity": "感染",
"start_offset": 36,
"end_offset": 38,
"label": "dis"
},
{
"id": 4,
"entity": "高血压",
"start_offset": 39,
"end_offset": 42,
"label": "dis"
},
{
"id": 5,
"entity": "高血脂",
"start_offset": 43,
"end_offset": 46,
"label": "dis"
},
{
"id": 6,
"entity": "生长障碍",
"start_offset": 47,
"end_offset": 51,
"label": "dis"
},
{
"id": 7,
"entity": "恶性肿瘤",
"start_offset": 53,
"end_offset": 57,
"label": "dis"
},
{
"id": 8,
"entity": "肾病",
"start_offset": 59,
"end_offset": 61,
"label": "dis"
},
{
"id": 9,
"entity": "移植",
"start_offset": 73,
"end_offset": 75,
"label": "pro"
}
] |
（一）感染儿童肾移植后的感染包括：细菌、真菌、病毒以及原虫等，多发生于尿路、肺部、伤口、动静脉瘘及中枢神经系统，其诱因包括导尿管的持续放置、大剂量激素的应用、粒细胞缺乏、肾周脓肿、尿瘘及糖尿病等。 | [
{
"id": 0,
"entity": "感染",
"start_offset": 3,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "儿童肾移植",
"start_offset": 5,
"end_offset": 10,
"label": "pro"
},
{
"id": 2,
"entity": "感染",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
},
{
"id": 3,
"entity": "细菌",
"start_offset": 17,
"end_offset": 19,
"label": "mic"
},
{
"id": 4,
"entity": "真菌",
"start_offset": 20,
"end_offset": 22,
"label": "mic"
},
{
"id": 5,
"entity": "病毒",
"start_offset": 23,
"end_offset": 25,
"label": "mic"
},
{
"id": 6,
"entity": "尿路",
"start_offset": 35,
"end_offset": 37,
"label": "bod"
},
{
"id": 7,
"entity": "肺部",
"start_offset": 38,
"end_offset": 40,
"label": "bod"
},
{
"id": 8,
"entity": "伤口",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 9,
"entity": "动静脉瘘",
"start_offset": 44,
"end_offset": 48,
"label": "bod"
},
{
"id": 10,
"entity": "中枢神经系统",
"start_offset": 49,
"end_offset": 55,
"label": "bod"
},
{
"id": 11,
"entity": "导尿管",
"start_offset": 61,
"end_offset": 64,
"label": "equ"
},
{
"id": 12,
"entity": "激素",
"start_offset": 73,
"end_offset": 75,
"label": "dru"
},
{
"id": 13,
"entity": "粒细胞缺乏",
"start_offset": 79,
"end_offset": 84,
"label": "sym"
},
{
"id": 14,
"entity": "肾周脓肿",
"start_offset": 85,
"end_offset": 89,
"label": "sym"
},
{
"id": 15,
"entity": "尿瘘",
"start_offset": 90,
"end_offset": 92,
"label": "sym"
},
{
"id": 16,
"entity": "糖尿病",
"start_offset": 93,
"end_offset": 96,
"label": "sym"
}
] |
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