[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"$f65-g5LTUgHIk8dGZIHhGe2pKpxnfW0g-O4HGxk3yxcs":3,"$fH3S7g6I6K7vh8Q8Qokh49weG81f-0uQg-icFRTO_aL0":8},{"code":4,"msg":5,"message":6,"data":6,"success":7},401,"认证失败，无法访问系统资源",null,false,{"code":9,"msg":10,"message":6,"data":11,"success":60},200,"操作成功",{"id":12,"name":13,"describes":6,"symptoms":6,"checkup":6,"treatment":6,"seoTitle":14,"seoKeywords":15,"seoDescription":16,"coverVertical":6,"coverAcross":6,"introduceClassifications":17,"departments":58},6824,"α-贮存池病"," α-贮存池病：症状、诊断与治疗指南\n"," α-贮存池病, α-贮存池病症状, α-贮存池病诊断, α-贮存池病治疗, 血液疾病, α-贮存池病管理, α-贮存池病研究\n"," 了解α-贮存池病的症状、诊断方法及最新治疗指南。本页面为您提供全面的α-贮存池病信息，帮助您认识和管理这种罕见的血液疾病。",[18,23,28,33,38,43,48,53],{"id":19,"classification":20,"description":21,"sort":22,"diseaseId":12},116009,"概述","\u003Cp>　　α-贮存池病(α-storage pool disease，α-SPD)自幼即有轻度出血，出血时间延长，血小板减少，血小板、巨核细胞形态异常，细胞质颗粒明显减少，瑞氏染色血涂片上血小板呈灰色，故名“灰色血小板综合征”。\u003C/p>",0,{"id":24,"classification":25,"description":26,"sort":27,"diseaseId":12},116010,"病因","\u003Cp>为常染色体显性遗传。血小板α颗粒内容物如PF4,β-TG、纤维蛋白原、凝血酶敏感蛋白、vWF、纤维连接蛋白等减少，血浆中血小板特异蛋白如β-TG和PF4浓度正常或增高，巨核细胞免疫电镜表明vWF、PF4等合成正常，提示本病是由于合成的蛋白不能贮存于α颗粒所致。\u003C/p>\u003Cp>　　\u003C/p>",1,{"id":29,"classification":30,"description":31,"sort":32,"diseaseId":12},116011,"症状","\u003Cp>　　常见症状：鼻出血、 刷牙出血、 牙龈出血、 血小板减少、 骨髓巨核细胞成熟障碍\u003C/p>\u003Cp>　　主要表现为出血，但一般症状较轻。\u003C/p>\u003Cp>　　根据临床表现，实验室检查确诊。\u003C/p>\u003Cp>\u003C/p>",2,{"id":34,"classification":35,"description":36,"sort":37,"diseaseId":12},116012,"检查","\u003Cp>　　检查项目：出血时间、 涂片、 血涂片、 骨髓象分析 、血液检查\u003C/p>\u003Cp>　　1.血小板轻度至中度减少，大小不一，平均直径略有增加，在多嗜性染色的血涂片中呈灰色的鬼影样，卵圆形。\u003C/p>\u003Cp>　　2.出血时间延长。\u003C/p>\u003Cp>　　3.骨髓象 网状蛋白纤维化。\u003C/p>\u003Cp>　　4.血浆PF4及β-TG浓度正常或升高。\u003C/p>\u003Cp>　　5.血小板对ADP、肾上腺素、瑞斯托霉素等的聚集反应正常或接近正常，对胶原或凝血酶聚集反应常缺乏。\u003C/p>\u003Cp>　　6.血小板α颗粒内容物PF4、β-TG、纤维蛋白原、vWF、凝血因子Ⅴ，纤连蛋白及TSP明显减少，致密颗粒内容物S-HT、ATP、ADP正常。\u003C/p>",3,{"id":39,"classification":40,"description":41,"sort":42,"diseaseId":12},116013,"鉴别","\u003Cp>　　本病应与Quebec血小板病鉴别，后者是新近发现的一种遗传性血小板功能缺陷性疾病。两者均有多种血小板糖蛋白异常和血小板减少。但Quebec血小板病呈常染色体显性遗传，其α颗粒形态正常，肾上腺素诱导的血小板聚集反应缺如，muhimerin缺乏，输注血小板无效。\u003C/p>",4,{"id":44,"classification":45,"description":46,"sort":47,"diseaseId":12},116014,"预防","\u003Cp>　　建立遗传咨询，严格婚前检查，加强产前诊断，减少患儿的出生。\u003C/p>",5,{"id":49,"classification":50,"description":51,"sort":52,"diseaseId":12},116015,"治疗","\u003Cp>　　DDAVP对部分患者可缩短出血时间，增强止血功能。严重出血患者输注血小板有效。\u003C/p>",6,{"id":54,"classification":55,"description":56,"sort":57,"diseaseId":12},116016,"饮食","\u003Cp>α-贮存池病饮食保健应注意因为患者主要表现为出血，所以在饮食保健方面要注意加强营养，饮食要丰富营养均衡。特别注意摄入富含蛋白质、维生素的食物。保证身体代谢所需的能量。同时可以用中药黄芪、当归、白芍、山药等补气养血。保健方面要严格婚前检查，加强产前诊断，减少患儿的出生。\u003C/p>",7,[59],"血液内科",true]