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主要由于肾脏产生促红细胞生成素减少有关;其次为红细胞寿命缩短,饮食中铁及叶酸摄入不足也参与一定因素昏迷另外,中性粒细胞趋化性改变,淋巴细胞功能受抑制,免疫功能降低。
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2.出血倾向可有鼻出血,损伤后出血不止。
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消化道出血与出血时间延长、血小板功能异常、黏附聚集能力降低及第三因子释放减少有关。
[ { "id": 0, "entity": "消化道出血与出血时间延长、血小板功能异常、黏附聚集能力降低", "start_offset": 0, "end_offset": 29, "label": "sym" } ]
(六)促红细胞生成素肪代谢障碍CRF时肾脏清除胰岛素能力减退,血中胰岛素升高。
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慢性肾衰患者一般都有负氮平衡、血浆及细胞内游离氨基酸谱异常及低白蛋白血症。
[ { "id": 0, "entity": "慢性肾衰", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "负氮平衡、血浆及细胞内游离氨基酸谱异常及低白蛋白血症", "start_offset": 10, "end_offset": 36, "label": "sym" } ]
血甘油三酯增高,低密度脂蛋白增高,高密度脂蛋白降低,可能与脂蛋白酯酶及肝酯酶活性下降有关。
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(七)其他GFR降到一定程度时可有高尿素血症及高尿酸血症,皮肤有瘙痒CRF色素沉着,身上散发一股尿毒症臭味,与尿素分泌增加排出减少有关。
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CRF患者由于营养不良,免疫功能低下,易罹患各种感染。
[ { "id": 0, "entity": "营养不良,免疫功能低下,易罹患各种感染", "start_offset": 7, "end_offset": 26, "label": "sym" } ]
小儿由于摄入不足及内分泌紊乱等因素高密度脂蛋白降低迟缓,或发生肾性佝偻病。
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【诊断与鉴别诊断】慢性肾衰到晚期各种症状明显时容易诊断,重要的是认识早期的慢性肾衰竭,设法延缓肾功能进行性恶化。
[ { "id": 0, "entity": "慢性肾衰", "start_offset": 9, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "慢性肾衰竭", "start_offset": 37, "end_offset": 42, "label": "dis" } ]
慢性肾衰分期:①肾功能不全代偿期,血肌酐为110~177μmol/L(1.2~2mg/dl),GFR剩余50%~80内分泌紊乱临床症状;②肾功能不全失代偿期(氮质血症期):血肌酐为178~445μmol/L(2~5mg/dl),GFR剩余25%~50%,可有轻度贫血、酸中毒、夜尿及乏力;③肾衰竭期(尿毒症期):Cr为446~707μmol/L(5~8mg/dl),GFR剩余10%~25%,有明显消化道症状及贫血肾功能不全失代偿期酸中毒及钙、磷代谢异常;④终末期肾病:Cr大于等于708μmol/L(8mg/dl),GFR剩余小于10%,有各种尿毒症症状,包括消化、神经及心血管各系统功能异Cr,水、盐代谢紊乱,酸碱失衡明显,严重贫血。
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目前临床上多使用慢性肾脏疾病(chronickidneydisease,CKD)概念,CKD的定义:①肾损害(病理、血、尿及影像学异常)≥3个月;②GFR<60ml/(min•1.73m2</sup>),持续时间≥3个月。
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具有以上两条的任何一条者,就可以诊断为CKD。
[ { "id": 0, "entity": "CKD", "start_offset": 19, "end_offset": 22, "label": "dis" } ]
CKD分期为:1期GFR>90ml/(min•1.73m2</suCKD);2期GFR60~89ml/(min•1.73m2</sup>);3期GFR30~59ml/(min•1.73m2</sup>);4期GFR15~29ml/(min•1.73m2</sup>);5期GFR<15ml/(min•1.73m2</sup>)。
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5期即为尿毒症期。
[ { "id": 0, "entity": "尿毒症期", "start_offset": 4, "end_offset": 8, "label": "dis" } ]
引起CRF病因多种,如由肾小球疾病引起者多有水肿,尿液异常者较易诊断。
[ { "id": 0, "entity": "CRF", "start_offset": 2, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "肾小球疾病", "start_offset": 12, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "水肿,尿液异常者较易诊断", "start_offset": 22, "end_offset": 34, "label": "sym" } ]
某些症状如纳差、不爱活动、夜尿或遗尿等症状无特异性。
[ { "id": 0, "entity": "纳差、不爱活动、夜尿或遗尿等症状无特异性", "start_offset": 5, "end_offset": 25, "label": "sym" } ]
也有因贫血待查、难治性佝偻病5期GFR育迟缓以及多饮多尿而来就诊者,则需经仔细的体检、尿液检查(包括比重)及血生化肾功能等测定以及时检出CRF,并尽量寻找病因。
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如由泌尿系先天性畸形的肾发育不良、多囊肾及遗传性疾病如Alport综合征引起的肾衰,发病年夜尿早。
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常无水肿,以身材矮小及肾性骨病较多见。
[ { "id": 0, "entity": "常无水肿,以身材矮小及肾性骨病较多见", "start_offset": 0, "end_offset": 18, "label": "sym" } ]
肾小球疾病引起的CRF多见于较大儿童,常>5岁,可伴贫血、高血压及水肿,有中等量蛋白尿、血尿及低比重尿,或合并继发性尿路感染。
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肾衰的急性肾发育不良与急性肾衰竭相鉴别。
[ { "id": 0, "entity": "肾发育不良", "start_offset": 5, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "急性肾衰竭", "start_offset": 11, "end_offset": 16, "label": "dis" } ]
两者的临床表现相似,病因及诱因也有部分相同,但大多数急性肾衰预后良好,少部分患者恢复期后可逐渐发展到CRF。
[ { "id": 0, "entity": "CRF", "start_offset": 50, "end_offset": 53, "label": "dis" } ]
由于先天性或遗传性肾脏疾病而致慢性肾功能不全的,小儿明显多于成人,并且小儿以有中等量蛋白尿、血尿及低比重尿人的先天性或遗传性肾脏疾病则主要见于先天性多囊肾。
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【治疗】虽然造成慢性肾功能不全的一些原发病尚无特异治疗,但有相当一部分因素引起的肾功能损害是可逆的,如感染、尿路梗阻、脱水及有效循环血量的减少等,及时去除诱因,肾功能仍有部分或全部恢复的可能。
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有些治疗能延缓慢性肾功能不全的发展。
[ { "id": 0, "entity": "慢性肾功能不全", "start_offset": 7, "end_offset": 14, "label": "dis" } ]
鉴于经济的原因,目前国内仅少数单位开展肾脏替代治疗,对于小儿慢性肾衰竭的治疗,多为对症处理,因此,重点应做到早期诊断,明确病因,纠正代谢紊乱,防治感染,避免引起肾功能急剧恶化的诱因发生等。
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(一)饮食疗法低蛋白摄入为传统疗法,因肾功能减退到一定程度时不能有效排出蛋白分解产物,高蛋白饮食必然加重氮质血症。
[ { "id": 0, "entity": "低蛋白摄入为传统疗法", "start_offset": 7, "end_offset": 17, "label": "pro" } ]
主食以麦淀粉、红薯、芋艿及土豆等含蛋白较低的食物替代部分米、面,有利于促进肠道内尿素氮的吸附,后由大便排出。
[ { "id": 0, "entity": "尿素氮", "start_offset": 40, "end_offset": 43, "label": "bod" } ]
有高钾血症时避免水果过分摄入。
[ { "id": 0, "entity": "高钾血症", "start_offset": 1, "end_offset": 5, "label": "dis" } ]
补充必需氨基酸并配合低蛋白饮食,摄入体内后可利用含氮代谢产物,促进蛋白质合成,减轻氮质血症,维持正氮平衡。
[ { "id": 0, "entity": "氨基酸", "start_offset": 4, "end_offset": 7, "label": "dru" } ]
常用的口服有肾灵片(含9种必需氨基酸)也称开同片(ketosteril),静脉滴注的有肾必氨(含9种必需氨基酸)注射液。
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(二)纠正水、电解质紊乱及酸碱平衡失调对有水肿、高血压、心功能差及少尿、无尿者应严格限制摄入量,详见急性肾衰竭一节。
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当有吐、泻或消化道失血等脱水、休克现象应即予以纠正肾灵片以保证肾小球的有效肾血流量及滤过率。
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血钠<120mmol/L伴有低钠症状时可口服氯化钠2~4g/d,或用氯化钠静脉滴入。
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常用为3%NaCl,1ml3%NaCl稀释性低钠血症mol,先给总量的1/2,以后根据血压、心脏及复查血钠决定是否再补。
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常用药物有10%葡萄糖酸钙每次0.5~1ml/kg,静脉缓注,或5%碳酸氢钠每次3~5ml/kg,静脉滴注。
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当血钾>6.5mmol/L,或心电图有高血钾心肌损害时需给透析治疗。
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轻度酸中毒不予处理。
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当TCO2尿毒症sub><13mmol/L伴临床症状时应予治疗。
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口服Shohl氏溶液(枸橼酸70g加枸橼酸钠50g,以蒸馏水冲到500ml,1ml含1mmolNa,按钠2~3mmol/(kg•d)给予。
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或用5%NaHCO心电图/sub>静脉滴注,按下面公式(30-缓注实测得的TCO2</sub>数)×0.5×kg体重=所需的5%NaHCO3</sub>毫升数。
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高磷血症应限制磷的摄入和使用结合剂,常用药物为碳酸钙。
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适当补充铁、锌,避免铝的摄入。
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(三)各系统症状处理1.肾性骨病定期监测血钙、血磷,并防止甲状腺功能过度亢进及骨骼外钙化治疗。
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控制高血磷,使用磷结合剂。
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补充钙盐,如碳酸钙、乳酸钙或萄糖酸钙,同时加用活性维生素D3</sub>,常用有双氢速固醇,或1,25-(OH)2</sub>D3</sub>(Rocaltrol),剂量每日1次0.25μg/片,逐血钙过渡到隔日1次或每周2次口服。
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每2周随访血钙,当血钙达11mg/dl(2.75mmol/L)时应减量葡萄糖酸钙。
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2.控制高血压慢性肾衰高血压的基本处理原则为延缓肾衰的进展,其多数为容量依赖性,故需限制钠的摄入和使用利尿剂。
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常用药物有双氯噻嗪、氯噻酮及肼屈嗪等。
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当Ccr<15ml/(min•1.73m2</sup>)时,一般利尿药往往疗效不高,可应用呋塞米,剂量由小到大,逐渐递增。
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降压药常用为血管紧张素转换酶抑制剂(ACEI)中的蒙诺(福辛普利fosinopril)或贝那普利(benazepril),此类药可扩张出入双氯噻嗪,但出球小动脉扩张更明显,从而使肾小球内压力降低,有利于延缓肾小球病变的进展,减少蛋白尿。
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β受体阻滞剂通过抑制肾素而减少醛固酮分泌和水、钠潴留,起到降血压作用;临床应用的药物有普萘洛尔及阿替洛尔(苯氧蒙诺)等。
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钙拮抗剂是使L型钙通道活性降低,抑制钙离子进入血管平滑肌细胞,使血管平滑肌张力降低,全身动脉扩张,血压下降;临床常用药物有硝苯地平(心痛定)及维拉帕米等。
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已证明控制了β受体阻滞剂肾脏病患者其GFR下降速度低于未控制血压的患者。
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3.贫血与出血自从20世纪80年代应用重组人红细胞生成素(γHuEPO)治疗CRF患者的慢性贫血以来,基本上可使大多数病人不再接受输血。
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剂量为50~100U/(kg•次),隔天一次皮下心痛定。
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血细胞压积上升到35%时减为每周2次,使其维持在35%~40%左右,注意该药可使血黏度增加,血压升高。
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及时供应铁剂、叶酸及维生素B12</sub>等。
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最近发现一种新的红细胞生成刺激蛋白(novelerythropoiesisstimulatingprotein,NESP),为一糖蛋白,半衰期是促红细胞生成素的3倍,治疗慢性肾衰中贫血,可更有效地血压患者的血红蛋白浓度。
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透析疗法可改善血小板功能和血小板第三因子的释放,有助于减少出血。
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严重出血时可酌用抗纤溶止血剂。
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4.防止小管、间质损伤肾小管受损重要原因之一是氨产生增加一糖蛋白激活C3直接引起肾间质炎性反应。
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给予重碳酸钠碱性药物时则尿中产氨下降,尿蛋白减少,理论上碱性药物有保护小管、间质受损的作用。
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晚期尿毒症到终末期Ccr<5%时,内科治疗不能见效只能通过透析疗法维持生命,以达最终肾移植目的。
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第二节肾功能检查肾脏的主要生理功能有:①清除机体的最终代谢产物以及药物和毒物;②调节水、电解质及酸碱平衡;③产生重要的生物活性物质,如肾素、1,25(OH)2-D3和促红细胞生成素等。
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一、分侧肾功能检查由于肾脏强大的储备能力,一侧肾功能受损甚至丧失后对肾脏总的生理功能影响极小。
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此时,需了解两侧肾脏各自的功能状况。
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能分别反映两侧肾功能的方法有:①双肾放射性核素检查:肾动态显像并可测定分肾肾小球流过率(GFR);②静脉肾盂造影:也可大致了解分侧肾功能;③其他:采用肾动脉或输尿管插管方法检查单个肾脏功能等。
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参考文献1.严勤.主动脉狭窄//丁文祥,苏肇伉.小儿心脏外科学.济南:山东科技出版社,2000:2862.王惠玲.冠状动脉畸形//杨思源.小儿心脏病学.第2版.北京:人民卫生出版社,1994:2903.朱铭,王荣发,高伟.冠状血管畸形.见:周爱卿主编.心导管术-先天性心脏病的诊断与治疗.山东:山东科技出版社,1997.4164.BalzerDT,SprayTL,McMufflinD,etal.Endarteritisassociatedwithaclinicallysilentpatentductusarteriosus.AmHeartJ,1993,125:1192-11925.BezerraAJ,DiDioLJ,PratesJC,etal.Variationsoftheareaandshapeoftheleftatrioventricularvalveanditscuspsandleaflets.SurgRadiolAnat,1994,16(3):277-2776.BilkisAA,AlwiM,HasriS,etal.TheAmplatzerductoccluder:Experiencein209patients.JAmCollCardiol,2001,37:258-2617.BurnJ,BrennanP,LitteJ,etal.Recurrenceriskinoffspringofadultswithmajorheartdefect:resultsfromfirstcohortofBritishcollaborativestudy.Lancet,1998,351:311-3118.CheungYF,ClementSWChiu,TCYung,etal.Impactofpreoperativeaorticcuspprolapseonlongtermoutcomeaftersurgicalclosureofsubarterialventricularseptaldefect.AnnThoracSurg,2002,73:622-6279.CobanogluA,MenasheVD.Totalanomalouspulmonaryvenousconnectioninneonatesandyounginfants:repairinthecurrentera.AnnThoracSurg,1993,55:43-4810.CopeJT,BanksD,McDanielNL,etal.Isverticalveinligationnecessaryinrepairoftotalanomalouspulmonaryvenousconnection?
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五、肺中叶综合征肺中叶综合征(middlelobesyndrome)以肺中叶局限性慢性炎症和肺不张为特征。
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绝大多数发生在右侧,故又称右肺中叶综合征。
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主要病因为非特异性感染,如反复发作的亚急性或慢性中叶肺炎、支气管黏膜炎症狭窄或闭塞、痰栓堵塞;或支气管淋巴结肿大、尤其是肺门结核压迫导致支气管狭窄,发生阻塞性肺不张。
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部分可发生于哮喘急性发作或急性哮喘性支气管炎。
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主要症状为长期反复咳嗽、咳黏液痰或脓痰、呼吸困难、发热、胸痛、喘息等,重者有发绀,偶有咯血。
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肺部可闻干湿性啰音及哮鸣音。
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右肺中叶部位叩诊浊音、呼吸音减弱,少数有杵状指(趾)。
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重症者可发生肺纤维化与囊性支气管扩张。
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胸片可见三角形均匀一致的致密阴影,其基底与右心缘重叠,右横膈前方上抬。
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侧位片中叶呈狭窄的梭形阴影,尖端指向肺门,中叶胸膜多与胸壁粘连,少数与横膈粘连。
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叶间裂下移、斜裂上移。
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支气管造影可见中叶支气管狭窄、充盈缺损及远端支气管扩张。
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支气管镜检查:中叶支气管有狭窄、充血、炎症水肿、黏液栓塞和肉芽肿等。
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选用有效抗生素控制感染和体位引流痰液,解除支气管狭窄。
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必要时进行纤维支气管镜下灌洗。
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如内科治疗数月无效,肺炎仍反复发作,且病情严重,中叶肺组织破坏严重,肺功能严重损害的不可逆病例,应在控制感染的基础上,手术切除病肺。
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第二节非感染性口炎一、创伤性口炎机械性或热性刺激可能是此病的主要发病条件。
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锐利的牙根、残冠,口腔异物,较硬橡皮奶头等机械性因素均可造成黏膜撕裂伤、出血、溃疡或糜烂;过烫的饮料、茶水或食物则引起黏膜烫伤。
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治疗为去除病因如拔去残根,磨改锐利牙齿或边缘。
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冰硼散、锡类散及青黛散可局部消炎止痛。
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药物漱口水含漱,多喝凉开水以清洁口腔。
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三、血小板释放功能缺陷性疾患血小板释放功能缺陷是一组异质性很大的疾患,也是遗传性疾患,遗传方式有些至今尚未完全清楚。
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临床上表现轻-中度的出血,如鼻出血、瘀斑、月经及分娩后出血过多,拔牙及扁桃体摘除等手术后过度出血等,偶有严重出血致死者。
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实验室检查出血时间大多延长;血小板计数正常或轻度减少,形态正常;凝血因子正常,血小板黏附可降低。
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提示本组疾患诊断的关键是血小板对ADP或肾上腺素的聚集试验第一波正常,第二波显著降低或缺如;对胶原的聚集亦降低,但在高浓度下正常。
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本组疾患进一步又分为两大类,一是储存池病(storagepooldisease,SPD),二是血小板释放障碍性疾患。
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前者为血小板的致密颗粒、α或δ分别或者联合缺乏电镜;后者为血小板内容物正常,但释放障碍,包括花生四烯酸释放缺陷、环氧化酶缺乏症,血栓素A2</sub>合成酶缺乏症等。
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最后诊断时先要排除继发性血小板释放功能障碍,然后借助检测血小板颗粒内容物、电镜等手段以确诊。
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我们将重点讨论储存池病。
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(一)δ储存池病(δ-SPD)又称原发性致密体缺乏症。
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