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占儿童原发性脑瘤的10%~20%,后颅窝肿瘤的40%。
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肿瘤细胞可随脑脊液播散至蛛网膜下腔或转移至颅外,可达11%~43%。
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瘤组织呈灰红或紫红色,质软、易碎,边界不清。
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镜下肿瘤细胞密集,核呈圆形或椭圆形,深染,多有核分裂,胞质及间质较少,有的呈假菊形团排列。
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主要表现为颅内压增高和共济失调等小脑症状,侵及脑干者常有复视及多种脑神经障碍,小脑扁桃体疝时常有颈强直、斜颈表现。
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辅助检查CT及MRI可清楚地显示肿瘤。
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CT见肿瘤为高密度或等密度影,类圆形,边界清楚,周围水肿常为低密度。MRI瘤体呈长T1与长T2信号,注射Gd-DTPA(顺磁剂)后明显均一强化,少数可见厚薄不均的环状强化。
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术后放疗是十分重要的措施,除局部照射外,应包括全脑和椎管照射,以防止播散肿瘤发展。
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并加用化学治疗。
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早年髓母细胞瘤预后极差,近年来,随着放疗及化疗的改进,生存期已大大延长,有报道5年生存期可达85%。
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二、实验室检查主要包括病毒标志物和特异性抗体的检查。
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(一)病毒学检查(即病毒标志物检查)1.病毒颗粒检查用电镜或免疫电镜直接检测样本中病毒颗粒。
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2.病毒分离取样本接种于适宜细胞,培养分离病毒,常根据细胞病变或病毒标志物检查或利用其生物学特性如红细胞吸附、空斑形成等予以鉴定。
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此法能客观反映所感染病毒的存在及其种类,故被称为“黄金标准”。
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标准的病毒分离和电镜检查可用于检测未知病毒或发现新的病毒株。
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但此法费时、耗材、耗力,标本所含病毒数量极少时会出现假阴性。
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病毒复制不全、产生前病毒或与宿主细胞DNA整合时不能分离成功。
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有些病毒如乙型肝炎病毒(HBV)、丙型肝炎病毒(HCV)、庚型肝炎病毒(HGV)等目前尚缺乏敏感的培养细胞。
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3.病毒抗原检测用免疫标记技术直接检测体液和组织中病毒抗原是快速而实用的方法。
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这一方法要求标本含有一定量的抗原和高质量的特异性抗体。
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4.病毒基因检测应用核酸杂交法或聚合酶链反应(PCR),通过检测病毒特异性核酸(DNA或RNA)片段来发现相关病毒。
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此法灵敏度高,如PCR法能测出fg(10~15)水平的核酸,较杂交法测出pg(10~12)水平更为敏感。
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此法能发现不完整病毒如潜伏病毒DNA,故对疱疹类病毒如人巨细胞病毒(HCMV),单独检查病毒DNA不能区别活动性感染和潜伏感染。
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此外,新生儿娩出时,脐血中可混入母血,比血细胞更小的病毒核酸更易污染脐血。
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因此,用敏感的PCR法从脐血中检测病毒基因尚难确定系新生儿自身的感染。
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5.其他标志物检查如感染细胞内病毒颗粒聚集形成的包涵体(如疱疹类病毒)、内基小体(狂犬病毒)等特殊结构,常为活动性病毒感染的诊断依据。
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(二)血清学检查狭义的血清学检查是指特异性抗体测定。
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1.特异性IgG检测特异性IgG抗体多出现较晚,一般持续时间较长,乃至终生,单项阳性提示既往或正在感染该病毒。
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取急性期和恢复期双份血清检测特异性IgG,若抗体滴度≥4倍增高可回顾性诊断近期相应病毒感染。
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但也有例外,如原发EBV感染时,抗VCAIgG峰值在急性期,故不能使用上述诊断标准。
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IgG能通过胎盘,在评估6个月以下婴儿的IgG抗体时应考虑被动抗体的存在。
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在严重免疫缺陷患者,IgG抗体水平低下或缺如,可出现假阴性结果。
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2.特异性IgM检测机体在病毒初次感染早期或潜伏病毒活化时产生特异性IgM抗体(后者IgM抗体水平常较前者低),一般持续数月后消失,常作为急性(或近期)感染或活动性感染的诊断指标。
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受高滴度IgG抗体或类风湿因子等因素干扰可出现假阳性,采用IgM抗体捕捉ELISA或先去除IgG等方法可避免之。
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免疫抑制者和幼小婴儿因免疫反应低下易出现假阴性。
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3.特异性IgA检测某些病毒感染后,特异性IgA检出率较高,有助于诊断,如原发性带状疱疹病毒(VZV)感染和急性呼吸道合胞病毒(RSV)感染时。
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EB病毒(EBV)相关性鼻咽癌时,抗早期抗原IgA和抗病毒衣壳抗原IgA水平增高可用于早期诊断。
[ { "id": 0, "entity": "EB病毒", "start_offset": 0, "end_offset": 4, "label": "mic" }, { "id": 1, "entity": "EBV", "start_offset": 5, "end_offset": 8, "label": "mic" }, { "id": 2, "entity": "鼻咽癌", "start_offset": 12, "end_offset": 15, "label": "dis" }, { "id": 3, "entity": "抗早期抗原IgA", "start_offset": 17, "end_offset": 25, "label": "ite" }, { "id": 4, "entity": "抗病毒衣壳抗原IgA水平", "start_offset": 26, "end_offset": 38, "label": "ite" } ]
第七节特发性高钙尿症特发性高钙尿症(idiopathichypercalciuria,IH)是指病因不明尿钙排出增多而血钙正常而血钙正常的一组疾病。
[ { "id": 0, "entity": "特发性高钙尿症", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "特发性高钙尿症", "start_offset": 10, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "idiopathichypercalciuria", "start_offset": 18, "end_offset": 42, "label": "dis" }, { "id": 3, "entity": "IH", "start_offset": 43, "end_offset": 45, "label": "dis" }, { "id": 4, "entity": "尿钙", "start_offset": 52, "end_offset": 54, "label": "ite" }, { "id": 5, "entity": "血钙", "start_offset": 59, "end_offset": 61, "label": "ite" }, { "id": 6, "entity": "尿钙排出增多而血钙正常", "start_offset": 52, "end_offset": 63, "label": "sym" }, { "id": 7, "entity": "血钙", "start_offset": 64, "end_offset": 66, "label": "ite" }, { "id": 8, "entity": "血钙正常", "start_offset": 64, "end_offset": 68, "label": "sym" } ]
【病因与发病机制】肾脏和钙代谢关系非常密切,从肾小球滤过的钙50%~70%在近端肾小管被重吸收,30%~40%在远端肾小管重吸收,尿中排出的钙,离子钙占20%,复合钙占80%,以枸橼酸钙为主。
[ { "id": 0, "entity": "肾脏", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "钙", "start_offset": 12, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "肾小球", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "钙", "start_offset": 29, "end_offset": 30, "label": "bod" }, { "id": 4, "entity": "近端肾小管", "start_offset": 38, "end_offset": 43, "label": "bod" }, { "id": 5, "entity": "远端肾小管", "start_offset": 56, "end_offset": 61, "label": "bod" }, { "id": 6, "entity": "尿", "start_offset": 65, "end_offset": 66, "label": "bod" }, { "id": 7, "entity": "钙", "start_offset": 70, "end_offset": 71, "label": "bod" }, { "id": 8, "entity": "离子钙", "start_offset": 72, "end_offset": 75, "label": "bod" }, { "id": 9, "entity": "复合钙", "start_offset": 80, "end_offset": 83, "label": "bod" }, { "id": 10, "entity": "枸橼酸钙", "start_offset": 89, "end_offset": 93, "label": "bod" } ]
钙的重吸收和钠相似,是通过主动转运完成的,且受PTH和活性维生素D的调节。
[ { "id": 0, "entity": "钙", "start_offset": 0, "end_offset": 1, "label": "bod" }, { "id": 1, "entity": "钠", "start_offset": 6, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "PTH", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "活性维生素D", "start_offset": 27, "end_offset": 33, "label": "bod" } ]
特发性高钙尿症病因不明,此病有明显的家族史,可能系常染色体显性遗传,但也有认为家族中同一饮食或环境因素引起。
[ { "id": 0, "entity": "特发性高钙尿症", "start_offset": 0, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "常染色体显性遗传", "start_offset": 25, "end_offset": 33, "label": "sym" } ]
发病机制可能与以下机制有关:(一)肠钙吸收亢进(吸收型)主要由于空肠对钙选择性吸收过多,使血钙短暂升高致肾小球滤过钙增多及甲状旁腺分泌抑制而使肾小管重吸收钙减少。
[ { "id": 0, "entity": "肠钙吸收亢进", "start_offset": 17, "end_offset": 23, "label": "sym" }, { "id": 1, "entity": "空肠", "start_offset": 32, "end_offset": 34, "label": "bod" }, { "id": 2, "entity": "钙", "start_offset": 35, "end_offset": 36, "label": "bod" }, { "id": 3, "entity": "空肠对钙选择性吸收过多", "start_offset": 32, "end_offset": 43, "label": "sym" }, { "id": 4, "entity": "血钙", "start_offset": 45, "end_offset": 47, "label": "ite" }, { "id": 5, "entity": "血钙短暂升高", "start_offset": 45, "end_offset": 51, "label": "sym" }, { "id": 6, "entity": "肾小球", "start_offset": 52, "end_offset": 55, "label": "bod" }, { "id": 7, "entity": "钙", "start_offset": 57, "end_offset": 58, "label": "bod" }, { "id": 8, "entity": "肾小球滤过钙增多", "start_offset": 52, "end_offset": 60, "label": "sym" }, { "id": 9, "entity": "甲状旁腺", "start_offset": 61, "end_offset": 65, "label": "bod" }, { "id": 10, "entity": "甲状旁腺分泌抑制", "start_offset": 61, "end_offset": 69, "label": "sym" }, { "id": 11, "entity": "肾小管", "start_offset": 71, "end_offset": 74, "label": "bod" }, { "id": 12, "entity": "钙", "start_offset": 77, "end_offset": 78, "label": "bod" }, { "id": 13, "entity": "肾小管重吸收钙减少", "start_offset": 71, "end_offset": 80, "label": "sym" } ]
肠吸收钙亢进原因尚不明,可能系维生素D合成增多及调节功能障碍所致。
[ { "id": 0, "entity": "肠吸收钙亢进", "start_offset": 0, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "维生素D合成增多及调节功能障碍", "start_offset": 15, "end_offset": 30, "label": "sym" } ]
也有人认为与原发性肾失磷,致1,25(OH<sub>2</sub>D3</sub>合成增加,肠吸收钙增多所致。
[ { "id": 0, "entity": "原发性肾失磷", "start_offset": 6, "end_offset": 12, "label": "sym" }, { "id": 1, "entity": "1,25(OH<sub>2</sub>D3</sub>", "start_offset": 14, "end_offset": 41, "label": "bod" }, { "id": 2, "entity": "1,25(OH<sub>2</sub>D3</sub>合成增加", "start_offset": 14, "end_offset": 45, "label": "sym" }, { "id": 3, "entity": "肠吸收钙增多", "start_offset": 46, "end_offset": 52, "label": "sym" } ]
(二)肾小管重吸收钙障碍(肾漏型)由于肾小管重吸收钙缺陷致尿钙漏出增多,刺激甲状旁腺的分泌及1,25(OH)2</sub>D3</sub>合成增多,引起继发性肠钙吸收亢进并维持血钙正常。
[ { "id": 0, "entity": "肾小管", "start_offset": 3, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "肾小管重吸收钙障碍", "start_offset": 3, "end_offset": 12, "label": "sym" }, { "id": 2, "entity": "肾小管", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "钙", "start_offset": 25, "end_offset": 26, "label": "bod" }, { "id": 4, "entity": "肾小管重吸收钙缺陷", "start_offset": 19, "end_offset": 28, "label": "sym" }, { "id": 5, "entity": "尿钙漏出增多", "start_offset": 29, "end_offset": 35, "label": "sym" }, { "id": 6, "entity": "甲状旁腺", "start_offset": 38, "end_offset": 42, "label": "bod" }, { "id": 7, "entity": "1,25(OH)2</sub>D3</sub>", "start_offset": 46, "end_offset": 69, "label": "bod" }, { "id": 8, "entity": "1,25(OH)2</sub>D3</sub>合成增多", "start_offset": 46, "end_offset": 73, "label": "sym" }, { "id": 9, "entity": "血钙", "start_offset": 88, "end_offset": 90, "label": "ite" }, { "id": 10, "entity": "继发性肠钙吸收亢进并维持血钙正常", "start_offset": 76, "end_offset": 92, "label": "sym" } ]
一般认为高钙尿达10天以上会出现血尿,高钙尿可能损伤肾脏而出现血尿,但也并非高钙尿都有血尿。
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【临床表现】(一)血尿主要为镜下血尿,肉眼血尿一般为反复发作性,有时可见血丝。
[ { "id": 0, "entity": "血尿", "start_offset": 9, "end_offset": 11, "label": "sym" }, { "id": 1, "entity": "镜下血尿", "start_offset": 14, "end_offset": 18, "label": "sym" }, { "id": 2, "entity": "肉眼血尿", "start_offset": 19, "end_offset": 23, "label": "sym" }, { "id": 3, "entity": "反复发作性", "start_offset": 26, "end_offset": 31, "label": "sym" }, { "id": 4, "entity": "血丝", "start_offset": 36, "end_offset": 38, "label": "sym" } ]
尿中红细胞形态为非肾小球性。
[ { "id": 0, "entity": "尿", "start_offset": 0, "end_offset": 1, "label": "bod" }, { "id": 1, "entity": "红细胞形态", "start_offset": 2, "end_offset": 7, "label": "ite" }, { "id": 2, "entity": "尿中红细胞形态为非肾小球性", "start_offset": 0, "end_offset": 13, "label": "sym" } ]
(二)泌尿系症状少数病例有尿频、尿急、尿痛、排尿困难、遗尿及肾绞痛等症状。
[ { "id": 0, "entity": "尿频", "start_offset": 13, "end_offset": 15, "label": "sym" }, { "id": 1, "entity": "尿急", "start_offset": 16, "end_offset": 18, "label": "sym" }, { "id": 2, "entity": "尿痛", "start_offset": 19, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "排尿困难", "start_offset": 22, "end_offset": 26, "label": "sym" }, { "id": 4, "entity": "遗尿", "start_offset": 27, "end_offset": 29, "label": "sym" }, { "id": 5, "entity": "肾绞痛", "start_offset": 30, "end_offset": 33, "label": "sym" } ]
(三)尿路结石小儿肾结石中仅2%~5%系由本病引起。
[ { "id": 0, "entity": "尿路结石", "start_offset": 3, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "小儿肾结石", "start_offset": 7, "end_offset": 12, "label": "dis" } ]
(四)少数病人身体矮小,体重不增,肌无力,骨质稀疏等。
[ { "id": 0, "entity": "身体矮小", "start_offset": 7, "end_offset": 11, "label": "sym" }, { "id": 1, "entity": "体重不增", "start_offset": 12, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "肌无力", "start_offset": 17, "end_offset": 20, "label": "sym" }, { "id": 3, "entity": "骨质稀疏", "start_offset": 21, "end_offset": 25, "label": "sym" } ]
【实验室检查】血钙正常,血磷有时可降低;粪钙、磷减少;血碱性磷酸酶增高。
[ { "id": 0, "entity": "血钙", "start_offset": 7, "end_offset": 9, "label": "ite" }, { "id": 1, "entity": "血钙正常", "start_offset": 7, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "血磷", "start_offset": 12, "end_offset": 14, "label": "ite" }, { "id": 3, "entity": "血磷有时可降低", "start_offset": 12, "end_offset": 19, "label": "sym" }, { "id": 4, "entity": "粪钙", "start_offset": 20, "end_offset": 22, "label": "ite" }, { "id": 5, "entity": "磷", "start_offset": 23, "end_offset": 24, "label": "ite" }, { "id": 6, "entity": "粪钙、磷减少", "start_offset": 20, "end_offset": 26, "label": "sym" }, { "id": 7, "entity": "血碱性磷酸酶", "start_offset": 27, "end_offset": 33, "label": "ite" }, { "id": 8, "entity": "血碱性磷酸酶增高", "start_offset": 27, "end_offset": 35, "label": "sym" } ]
尿钙增多(尿钙/尿肌酐>0.18;24小时尿钙定量>0.1mmol/kg);X线及B型超声等影像学检查应常规进行。
[ { "id": 0, "entity": "X线及B型超声", "start_offset": 38, "end_offset": 45, "label": "pro" } ]
不能完全确诊,长期伴有血尿者可考虑做肾活体组织检查。
[ { "id": 0, "entity": "血尿", "start_offset": 11, "end_offset": 13, "label": "sym" }, { "id": 1, "entity": "肾活体组织检查", "start_offset": 18, "end_offset": 25, "label": "pro" } ]
【诊断】(一)随意尿Ca/Cr比值一般采用早餐后2小时随意尿标本测定,当尿Ca/Cr比值>0.21者,提示有高钙尿可能。
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因儿童收集24小时尿较为困难,经实验证明,可用随意尿Ca/Cr比值来做筛查。
[ { "id": 0, "entity": "尿Ca/Cr比值", "start_offset": 25, "end_offset": 33, "label": "ite" } ]
(二)24小时尿钙测定定量当尿Ca>4mg/(kg•d),可诊断高钙尿症,但尿钙排出量是受多种因素影响,如饮食中钙、钠、磷及蛋白质的含量,维生素D的摄入量等,一般上述检查应重复2次以上,排除各种已知病因引起的高钙尿后,可诊断为特发性高钙尿。
[ { "id": 0, "entity": "24小时尿钙测定定量", "start_offset": 3, "end_offset": 13, "label": "ite" }, { "id": 1, "entity": "尿Ca", "start_offset": 14, "end_offset": 17, "label": "ite" }, { "id": 2, "entity": "尿Ca>4mg/(kg•d)", "start_offset": 14, "end_offset": 28, "label": "sym" }, { "id": 3, "entity": "高钙尿症", "start_offset": 32, "end_offset": 36, "label": "dis" }, { "id": 4, "entity": "尿钙排出量", "start_offset": 38, "end_offset": 43, "label": "ite" }, { "id": 5, "entity": "高钙尿", "start_offset": 104, "end_offset": 107, "label": "dis" }, { "id": 6, "entity": "特发性高钙尿", "start_offset": 113, "end_offset": 119, "label": "dis" } ]
(三)钙负荷试验可做钙负荷试验进一步分型:给患儿低钙低钠饮食1周(停服乳品及钙剂,钙<250~300mg/d);试验前晚餐后禁食,于晚9时及午夜各饮水5~10ml/kg,试验日清晨7~9时留尿测空腹尿Ca/Cr比值;正常早餐后,口服葡萄糖酸钙糖浆(含钙1g/1.73m<sup>2</sup>或元素钙15~20mg/kg,),收集上午9时至下午1时共4小时尿,再测尿Ca/Cr比值。
[ { "id": 0, "entity": "钙负荷试验", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "钙负荷试验", "start_offset": 10, "end_offset": 15, "label": "pro" }, { "id": 2, "entity": "空腹尿Ca/Cr比值", "start_offset": 97, "end_offset": 107, "label": "ite" }, { "id": 3, "entity": "葡萄糖酸钙糖浆", "start_offset": 116, "end_offset": 123, "label": "dru" }, { "id": 4, "entity": "尿Ca/Cr比值", "start_offset": 182, "end_offset": 190, "label": "ite" } ]
如为吸收型IH,则空腹尿Ca/Cr比值正常,钙负荷后增高(>0.28),肾性IH则不受限钙影响,空腹尿Ca/Cr>0.21。
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【鉴别诊断】本病主要要与其他原因所致肾结石,如原发性甲状旁腺功能亢进症、肾小管酸中毒、维生素D中毒以及手术后制动等相鉴别;尚应注意排出其他病因所致的高钙尿症,如髓质海绵肾、结节病、肝豆状核变性、糖尿病以及长期皮质醇治疗,慢性镉/铅中毒、Wilson病及幼年类风湿关节炎等;可根据各原发病特点进行鉴别。
[ { "id": 0, "entity": "肾结石", "start_offset": 18, "end_offset": 21, "label": "dis" }, { "id": 1, "entity": "原发性甲状旁腺功能亢进症", "start_offset": 23, "end_offset": 35, "label": "dis" }, { "id": 2, "entity": "肾小管酸中毒", "start_offset": 36, "end_offset": 42, "label": "dis" }, { "id": 3, "entity": "维生素D中毒", "start_offset": 43, "end_offset": 49, "label": "dis" }, { "id": 4, "entity": "高钙尿症", "start_offset": 74, "end_offset": 78, "label": "dis" }, { "id": 5, "entity": "髓质海绵肾", "start_offset": 80, "end_offset": 85, "label": "dis" }, { "id": 6, "entity": "结节病", "start_offset": 86, "end_offset": 89, "label": "dis" }, { "id": 7, "entity": "肝豆状核变性", "start_offset": 90, "end_offset": 96, "label": "dis" }, { "id": 8, "entity": "糖尿病", "start_offset": 97, "end_offset": 100, "label": "dis" }, { "id": 9, "entity": "长期皮质醇治疗", "start_offset": 102, "end_offset": 109, "label": "pro" }, { "id": 10, "entity": "慢性镉/铅中毒", "start_offset": 110, "end_offset": 117, "label": "dis" }, { "id": 11, "entity": "Wilson病", "start_offset": 118, "end_offset": 125, "label": "dis" }, { "id": 12, "entity": "幼年类风湿关节炎", "start_offset": 126, "end_offset": 134, "label": "dis" } ]
(二)噻嗪类利尿剂对肾漏型者噻嗪类利尿剂治疗有效,可促进远端肾小管重吸收钙,使尿钙恢复正常,并调节甲状旁腺及1,25(OH)<sub>2</sub>D3</sub>至正常水平,使肠钙吸收正常。
[ { "id": 0, "entity": "噻嗪类利尿剂", "start_offset": 3, "end_offset": 9, "label": "dru" }, { "id": 1, "entity": "肾漏型", "start_offset": 10, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "噻嗪类利尿剂", "start_offset": 14, "end_offset": 20, "label": "dru" }, { "id": 3, "entity": "远端肾小管", "start_offset": 28, "end_offset": 33, "label": "bod" }, { "id": 4, "entity": "尿钙", "start_offset": 39, "end_offset": 41, "label": "ite" }, { "id": 5, "entity": "尿钙恢复正常", "start_offset": 39, "end_offset": 45, "label": "sym" }, { "id": 6, "entity": "甲状旁腺", "start_offset": 49, "end_offset": 53, "label": "ite" }, { "id": 7, "entity": "1,25(OH)<sub>2</sub>D3</sub>", "start_offset": 54, "end_offset": 82, "label": "ite" }, { "id": 8, "entity": "甲状旁腺及1,25(OH)<sub>2</sub>D3</sub>至正常水平", "start_offset": 49, "end_offset": 87, "label": "sym" }, { "id": 9, "entity": "肠钙吸收正常", "start_offset": 89, "end_offset": 95, "label": "sym" } ]
(三)磷酸纤维素钠为一种不被肠道吸收的离子交换树脂,能减少肠道钙的吸收,从而减少尿钙排出,对吸收型有效。
[ { "id": 0, "entity": "磷酸纤维素钠", "start_offset": 3, "end_offset": 9, "label": "dru" }, { "id": 1, "entity": "减少肠道钙的吸收", "start_offset": 27, "end_offset": 35, "label": "sym" }, { "id": 2, "entity": "尿钙", "start_offset": 40, "end_offset": 42, "label": "ite" }, { "id": 3, "entity": "减少尿钙排出", "start_offset": 38, "end_offset": 44, "label": "sym" } ]
不良反应为能影响肠道镁的吸收,可致血镁降低,应注意补充镁。
[ { "id": 0, "entity": "影响肠道镁的吸收", "start_offset": 6, "end_offset": 14, "label": "sym" }, { "id": 1, "entity": "血镁降低", "start_offset": 17, "end_offset": 21, "label": "sym" }, { "id": 2, "entity": "镁", "start_offset": 27, "end_offset": 28, "label": "dru" } ]
(四)口服锌或铁剂可减少钙的吸收而降低尿钙,适用于低锌血症或缺铁性贫血患儿。
[ { "id": 0, "entity": "锌", "start_offset": 5, "end_offset": 6, "label": "dru" }, { "id": 1, "entity": "铁剂", "start_offset": 7, "end_offset": 9, "label": "dru" } ]
(五)有人报告用未加工的麦麸服用治疗,能使80%患儿尿钙减少,其中近半数尿钙达正常水平。
[ { "id": 0, "entity": "麦麸", "start_offset": 12, "end_offset": 14, "label": "dru" }, { "id": 1, "entity": "尿钙", "start_offset": 26, "end_offset": 28, "label": "ite" }, { "id": 2, "entity": "尿钙减少", "start_offset": 26, "end_offset": 30, "label": "sym" }, { "id": 3, "entity": "尿钙", "start_offset": 36, "end_offset": 38, "label": "ite" } ]
其机制可能是影响肠钙吸收,故更适用于因肠吸收钙过多者。
[ { "id": 0, "entity": "肠吸收钙过多", "start_offset": 19, "end_offset": 25, "label": "sym" } ]
第七章气管、支气管疾病第一节先天性气道畸形一、气管软化气管软化(tracheomalacia)因气管软骨先天发育不良所致,可同时伴有喉软化(laryngomalacia),两者是先天性喉喘鸣的主要原因。
[ { "id": 0, "entity": "气管、支气管疾病", "start_offset": 4, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "先天性气道畸形", "start_offset": 15, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "气管软化", "start_offset": 24, "end_offset": 28, "label": "dis" }, { "id": 3, "entity": "气管软化", "start_offset": 28, "end_offset": 32, "label": "dis" }, { "id": 4, "entity": "tracheomalacia", "start_offset": 33, "end_offset": 47, "label": "dis" }, { "id": 5, "entity": "气管软骨先天发育不良", "start_offset": 49, "end_offset": 59, "label": "dis" }, { "id": 6, "entity": "喉软化", "start_offset": 67, "end_offset": 70, "label": "dis" }, { "id": 7, "entity": "laryngomalacia", "start_offset": 71, "end_offset": 85, "label": "dis" }, { "id": 8, "entity": "先天性喉喘鸣", "start_offset": 90, "end_offset": 96, "label": "dis" } ]
偶伴支气管软化(bronchomalacia)。
[ { "id": 0, "entity": "支气管软化", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "bronchomalacia", "start_offset": 8, "end_offset": 22, "label": "dis" } ]
动物实验表明,多柔比星诱发的食管闭锁常伴气管软化,说明两者可能存在共同的致病因素。
[ { "id": 0, "entity": "多柔比星", "start_offset": 7, "end_offset": 11, "label": "dru" }, { "id": 1, "entity": "食管闭锁", "start_offset": 14, "end_offset": 18, "label": "dis" }, { "id": 2, "entity": "气管软化", "start_offset": 20, "end_offset": 24, "label": "dis" } ]
易并发呼吸道感染。
[ { "id": 0, "entity": "呼吸道感染", "start_offset": 3, "end_offset": 8, "label": "dis" } ]
25%患儿伴有反应性气道疾病。
[ { "id": 0, "entity": "反应性气道疾病", "start_offset": 7, "end_offset": 14, "label": "dis" } ]
婴儿持续喘鸣应考虑到本病可能。
[ { "id": 0, "entity": "喘鸣", "start_offset": 4, "end_offset": 6, "label": "sym" } ]
气管镜检查可做出诊断。
[ { "id": 0, "entity": "气管镜检查", "start_offset": 0, "end_offset": 5, "label": "pro" } ]
在气管镜直视下可看到气管的前后壁随着呼吸运动而相互贴近,深呼吸时更为明显。
[ { "id": 0, "entity": "气管镜", "start_offset": 1, "end_offset": 4, "label": "equ" }, { "id": 1, "entity": "气管", "start_offset": 10, "end_offset": 12, "label": "bod" } ]
当气管镜探入隆突的下方时,由于支气管受到支撑,可使呼吸困难有所缓解。
[ { "id": 0, "entity": "气管镜", "start_offset": 1, "end_offset": 4, "label": "equ" }, { "id": 1, "entity": "支气管", "start_offset": 15, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "呼吸困难", "start_offset": 25, "end_offset": 29, "label": "sym" } ]
喂养时奶孔不宜过大,必要时胃管喂养。
[ { "id": 0, "entity": "胃管", "start_offset": 13, "end_offset": 15, "label": "equ" } ]
安静时喘鸣一般在5岁内消失。
[ { "id": 0, "entity": "喘鸣", "start_offset": 3, "end_offset": 5, "label": "sym" } ]
多发于冬春两季,呈散发性或流行性发病,后者称为流行性毛细支气管炎,又因该病是以喘憋为主要特征的一种特殊类型的肺炎,故又称喘憋性肺炎。
[ { "id": 0, "entity": "流行性毛细支气管炎", "start_offset": 23, "end_offset": 32, "label": "dis" }, { "id": 1, "entity": "喘憋", "start_offset": 39, "end_offset": 41, "label": "sym" }, { "id": 2, "entity": "肺炎", "start_offset": 54, "end_offset": 56, "label": "dis" }, { "id": 3, "entity": "喘憋性肺炎", "start_offset": 60, "end_offset": 65, "label": "dis" } ]
本病主要由病毒引起,呼吸道合胞病毒占50%以上,其次为副流感病毒3型、肺炎支原体、腺病毒等。
[ { "id": 0, "entity": "病毒", "start_offset": 5, "end_offset": 7, "label": "mic" }, { "id": 1, "entity": "呼吸道合胞病毒", "start_offset": 10, "end_offset": 17, "label": "mic" }, { "id": 2, "entity": "副流感病毒3型", "start_offset": 27, "end_offset": 34, "label": "mic" }, { "id": 3, "entity": "肺炎支原体", "start_offset": 35, "end_offset": 40, "label": "mic" }, { "id": 4, "entity": "腺病毒", "start_offset": 41, "end_offset": 44, "label": "mic" } ]
多数患儿先有上呼吸道感染症状,伴发热、食欲缺乏。
[ { "id": 0, "entity": "上呼吸道感染", "start_offset": 6, "end_offset": 12, "label": "sym" }, { "id": 1, "entity": "发热", "start_offset": 16, "end_offset": 18, "label": "sym" }, { "id": 2, "entity": "食欲缺乏", "start_offset": 19, "end_offset": 23, "label": "sym" } ]
逐渐出现呼吸困难、阵发性喘憋,发作时呼吸快而浅,并伴有呼气性喘鸣,明显鼻扇及三凹征。
[ { "id": 0, "entity": "呼吸困难", "start_offset": 4, "end_offset": 8, "label": "sym" }, { "id": 1, "entity": "阵发性喘憋", "start_offset": 9, "end_offset": 14, "label": "sym" }, { "id": 2, "entity": "呼吸快而浅,并伴有呼气性喘鸣", "start_offset": 18, "end_offset": 32, "label": "sym" }, { "id": 3, "entity": "鼻", "start_offset": 35, "end_offset": 36, "label": "bod" }, { "id": 4, "entity": "明显鼻扇", "start_offset": 33, "end_offset": 37, "label": "sym" }, { "id": 5, "entity": "三凹征", "start_offset": 38, "end_offset": 41, "label": "sym" } ]
严重病例有明显梗阻性肺气肿,常有极度烦躁不安、面色苍白及发绀。
[ { "id": 0, "entity": "梗阻性肺气肿", "start_offset": 7, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "极度烦躁不安", "start_offset": 16, "end_offset": 22, "label": "sym" }, { "id": 2, "entity": "面色苍白", "start_offset": 23, "end_offset": 27, "label": "sym" }, { "id": 3, "entity": "发绀", "start_offset": 28, "end_offset": 30, "label": "sym" } ]
胸部叩诊呈过清音,毛细支气管接近完全梗阻时,呼吸音明显减低或完全听不到,或仅有呼气延长及哮鸣音,喘憋时常听不到湿啰音,趋于缓解时则可有弥漫性中小水泡音、捻发音。
[ { "id": 0, "entity": "胸部叩诊", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "过清音", "start_offset": 5, "end_offset": 8, "label": "sym" }, { "id": 2, "entity": "毛细支气管", "start_offset": 9, "end_offset": 14, "label": "bod" }, { "id": 3, "entity": "完全梗阻", "start_offset": 16, "end_offset": 20, "label": "sym" }, { "id": 4, "entity": "呼吸音明显减低或完全听不到,或仅有呼气延长及哮鸣音", "start_offset": 22, "end_offset": 47, "label": "sym" }, { "id": 5, "entity": "喘憋时常听不到湿啰音", "start_offset": 48, "end_offset": 58, "label": "sym" }, { "id": 6, "entity": "趋于缓解时则可有弥漫性中小水泡音、捻发音", "start_offset": 59, "end_offset": 79, "label": "sym" } ]
胸片可见不同程度的梗阻性肺气肿,可伴支气管周围炎影像,部分患儿可有散在点片状阴影。
[ { "id": 0, "entity": "胸片", "start_offset": 0, "end_offset": 2, "label": "pro" }, { "id": 1, "entity": "梗阻性肺气肿", "start_offset": 9, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "支气管周围炎", "start_offset": 18, "end_offset": 24, "label": "dis" }, { "id": 3, "entity": "点片状阴影", "start_offset": 35, "end_offset": 40, "label": "sym" } ]
周围血白细胞总数及分类多属正常;鼻咽分泌物病毒抗原检测、病毒分离或病毒核酸检测可阳性。
[ { "id": 0, "entity": "周围血白细胞总数", "start_offset": 0, "end_offset": 8, "label": "ite" }, { "id": 1, "entity": "鼻咽分泌物病毒抗原检测", "start_offset": 16, "end_offset": 27, "label": "ite" }, { "id": 2, "entity": "病毒分离或病毒核酸检测", "start_offset": 28, "end_offset": 39, "label": "ite" }, { "id": 3, "entity": "阳性", "start_offset": 40, "end_offset": 42, "label": "sym" } ]
亦可通过测定血清特异IgM或双份血清抗体效价得出病原学诊断。
[ { "id": 0, "entity": "血清特异IgM", "start_offset": 6, "end_offset": 13, "label": "ite" }, { "id": 1, "entity": "双份血清抗体", "start_offset": 14, "end_offset": 20, "label": "ite" } ]
根据发病年龄偏小,发病初期即出现明显的喘憋,体格检查及X线检查在初期即出现明显肺气肿,诊断不难。
[ { "id": 0, "entity": "喘憋", "start_offset": 19, "end_offset": 21, "label": "sym" }, { "id": 1, "entity": "体格检查", "start_offset": 22, "end_offset": 26, "label": "pro" }, { "id": 2, "entity": "X线检查", "start_offset": 27, "end_offset": 31, "label": "pro" }, { "id": 3, "entity": "明显肺气肿", "start_offset": 37, "end_offset": 42, "label": "sym" } ]
本病应与哮喘相鉴别。
[ { "id": 0, "entity": "哮喘", "start_offset": 4, "end_offset": 6, "label": "dis" } ]
应放置在冷湿化环境中,或冷湿化给氧,以缓解低氧血症、减少不显性失水。
[ { "id": 0, "entity": "冷湿化给氧", "start_offset": 12, "end_offset": 17, "label": "pro" }, { "id": 1, "entity": "低氧血症", "start_offset": 21, "end_offset": 25, "label": "dis" } ]
避免使用镇静剂。
[ { "id": 0, "entity": "镇静剂", "start_offset": 4, "end_offset": 7, "label": "dru" } ]
有些患儿可发展成为反应性气道疾病。
[ { "id": 0, "entity": "反应性气道疾病", "start_offset": 9, "end_offset": 16, "label": "dis" } ]
多发生于儿童期,临床经过多样,儿童患者常表现为隐性感染和轻微的上呼吸道炎症。
[ { "id": 0, "entity": "上呼吸道炎症", "start_offset": 31, "end_offset": 37, "label": "dis" } ]
接触含有病毒的唾液是本病的主要传播方式。
[ { "id": 0, "entity": "病毒", "start_offset": 4, "end_offset": 6, "label": "mic" }, { "id": 1, "entity": "唾液", "start_offset": 7, "end_offset": 9, "label": "bod" } ]
偶可经输血传播。
[ { "id": 0, "entity": "输血", "start_offset": 3, "end_offset": 5, "label": "pro" } ]
自身抗体是导致血液系统异常变化的因素之一。
[ { "id": 0, "entity": "血液系统", "start_offset": 7, "end_offset": 11, "label": "bod" } ]
病理改变为淋巴结滤泡增多增大,生发中心增大,其核心见母细胞、组织细胞和淋巴细胞。
[ { "id": 0, "entity": "淋巴结滤泡", "start_offset": 5, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "淋巴结滤泡增多增大", "start_offset": 5, "end_offset": 14, "label": "sym" }, { "id": 2, "entity": "生发中心", "start_offset": 15, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "生发中心增大", "start_offset": 15, "end_offset": 21, "label": "sym" }, { "id": 4, "entity": "母细胞", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 5, "entity": "组织细胞", "start_offset": 30, "end_offset": 34, "label": "bod" }, { "id": 6, "entity": "淋巴细胞", "start_offset": 35, "end_offset": 39, "label": "bod" } ]
脾脏增大2~3倍,充血,伴局灶性出血,脾包膜和小梁水肿、增厚伴淋巴样细胞浸润。
[ { "id": 0, "entity": "脾脏", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "脾脏增大2~3倍,充血", "start_offset": 0, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "局灶性出血", "start_offset": 13, "end_offset": 18, "label": "sym" }, { "id": 3, "entity": "脾包膜", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 4, "entity": "小梁", "start_offset": 23, "end_offset": 25, "label": "bod" }, { "id": 5, "entity": "脾包膜和小梁水肿、增厚", "start_offset": 19, "end_offset": 30, "label": "sym" }, { "id": 6, "entity": "淋巴样细胞浸润", "start_offset": 31, "end_offset": 38, "label": "sym" } ]
神经系统病变包括神经元变性,血管周围出血和星状细胞增生,大脑皮层、基底节、小脑或脊髓等处小单核细胞浸润。
[ { "id": 0, "entity": "神经系统", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "神经元变性", "start_offset": 8, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "血管", "start_offset": 14, "end_offset": 16, "label": "bod" }, { "id": 3, "entity": "血管周围出血和星状细胞增生", "start_offset": 14, "end_offset": 27, "label": "sym" }, { "id": 4, "entity": "大脑皮层", "start_offset": 28, "end_offset": 32, "label": "bod" }, { "id": 5, "entity": "基底节", "start_offset": 33, "end_offset": 36, "label": "bod" }, { "id": 6, "entity": "小脑", "start_offset": 37, "end_offset": 39, "label": "bod" }, { "id": 7, "entity": "脊髓", "start_offset": 40, "end_offset": 42, "label": "bod" }, { "id": 8, "entity": "小单核细胞", "start_offset": 44, "end_offset": 49, "label": "bod" }, { "id": 9, "entity": "大脑皮层、基底节、小脑或脊髓等处小单核细胞浸润", "start_offset": 28, "end_offset": 51, "label": "sym" } ]
显性表现常较轻微,如上呼吸道感染、扁桃体炎、持续发热伴或不伴淋巴结肿大。
[ { "id": 0, "entity": "上呼吸道", "start_offset": 10, "end_offset": 14, "label": "bod" }, { "id": 1, "entity": "上呼吸道感染", "start_offset": 10, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "扁桃体炎", "start_offset": 17, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "持续发热", "start_offset": 22, "end_offset": 26, "label": "sym" }, { "id": 4, "entity": "淋巴结", "start_offset": 30, "end_offset": 33, "label": "bod" }, { "id": 5, "entity": "不伴淋巴结肿大", "start_offset": 28, "end_offset": 35, "label": "sym" } ]
纵隔淋巴结肿大可引起咳嗽和气促。
[ { "id": 0, "entity": "淋巴结", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "淋巴结肿大", "start_offset": 2, "end_offset": 7, "label": "sym" }, { "id": 2, "entity": "咳嗽", "start_offset": 10, "end_offset": 12, "label": "sym" }, { "id": 3, "entity": "气促", "start_offset": 13, "end_offset": 15, "label": "sym" } ]