[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"$f65-g5LTUgHIk8dGZIHhGe2pKpxnfW0g-O4HGxk3yxcs":3,"$f28-F26qaCFMUh1uN5KKyz1k5IRAmS4k0uH7rRIyTr8o":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":52},200,"操作成功",{"id":12,"name":13,"describes":6,"symptoms":6,"checkup":6,"treatment":6,"seoTitle":6,"seoKeywords":6,"seoDescription":6,"coverVertical":6,"coverAcross":6,"introduceClassifications":14,"departments":50},7775,"白细胞异常色素减退综合征",[15,20,25,30,35,40,45],{"id":16,"classification":17,"description":18,"sort":19,"diseaseId":12},119074,"概述","\u003Cp>　　白细胞异常色素减退综合征(Chediak-Higashi syndrome)是一种常染色体隐性遗传性疾病本病是一种先天性溶酶体异常，发病机制不很清楚，多见于近亲结婚的后代主要表现为色素缺乏，眼色素层及眼底色素减少，伴畏光、眼球震颤;部分皮肤、毛发白化，似假性白化病。\u003C/p>",0,{"id":21,"classification":22,"description":23,"sort":24,"diseaseId":12},119075,"病因","\u003Cp>　　本病是一种先天性溶酶体异常，属于常染色体隐性遗传。\u003C/p>",1,{"id":26,"classification":27,"description":28,"sort":29,"diseaseId":12},119076,"症状","\u003Cp>　　常见症状：眼色素层及眼底色素减少、伴畏光、眼球震颤、部分皮肤毛发白化、易反复发生重症呼吸道、皮肤的细菌感染\u003C/p>\u003Cp>　　患者多见于近亲结婚的后代。主要表现为色素缺乏，眼色素层及眼底色素减少，伴畏光、眼球震颤;部分皮肤、毛发白化，似假性白化病;易反复发生重症呼吸道、皮肤的细菌感染，常见为金黄色葡萄球菌、链球菌、肺炎球菌等感染;肝脾、淋巴结肿大;可伴中枢和周围神经病变和弥漫性恶性淋巴瘤。因反复感染及恶性肿瘤，患者往往在5岁前死亡。\u003C/p>",2,{"id":31,"classification":32,"description":33,"sort":34,"diseaseId":12},119077,"检查","\u003Cp>　　检查项目：血常规、细胞学检查、眼科检查\u003C/p>\u003Cp>　　患儿中性粒细胞减少，细胞内溶酶体异常。白细胞的趋化性、吞噬和杀菌功能缺陷，晚期患者可呈全血细胞减少，白细胞过氧化物酶染色阳性。患儿中性粒细胞、单核细胞和淋巴细胞的胞浆中易检出巨大的过氧化酶阳性的嗜苯胺蓝颗粒为具有诊断意义的特征。\u003C/p>",3,{"id":36,"classification":37,"description":38,"sort":39,"diseaseId":12},119078,"并发症","\u003Cp>　　肝、脾、淋巴结肿大;可伴中枢和周围神经病变和弥漫性恶性淋巴瘤。\u003C/p>",4,{"id":41,"classification":42,"description":43,"sort":44,"diseaseId":12},119079,"预防","\u003Cp>　　加强护理和营养：以提高患者的抵抗力和免疫力。预防感染：应注意隔离，尽量减少与病原体的接触避免近亲结婚生子女是关键。\u003C/p>",5,{"id":46,"classification":47,"description":48,"sort":49,"diseaseId":12},119080,"治疗","\u003Cp>　　1.一般疗法\u003C/p>\u003Cp>　　(1)加强护理和营养：以提高患者的抵抗力和免疫力。\u003C/p>\u003Cp>　　(2)预防感染：应注意隔离，尽量减少与病原体的接触。\u003C/p>\u003Cp>　　2.抗感染疗法 由于吞噬细胞本身和吞噬能力缺陷机体无法杀灭感染的细菌，因此，一旦发生感染，应针对病原菌选择广谱的杀菌性抗生素进行治疗。磺胺甲噁唑/甲氧苄啶(复方新诺明)对控制慢性肉芽肿病的感染有一定的效果。\u003C/p>\u003Cp>　　3.免疫替补疗法\u003C/p>\u003Cp>　　(1)输注粒细胞：针对粒细胞吞噬杀菌功能的缺陷，可通过输注粒细胞起到暂时的替补作用。尤其是应用细胞分离器获得的白细胞悬液中含有更多的粒细胞(1010的细胞数)，可供临床使用。\u003C/p>\u003Cp>　　(2)输注新鲜全血：先天性调理系统缺陷可引起一些反复感染，应用新鲜血浆可以纠正，每次10～20ml/kg，每周或每2周1次。，对严重感染的病例，可输入调理蛋白，通过加强对病菌的调理作用促进机体对病菌的识别、吞噬和清除作用，从而达到控制感染的目的。\u003C/p>",6,[51],"血液内科",true]