[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"$f65-g5LTUgHIk8dGZIHhGe2pKpxnfW0g-O4HGxk3yxcs":3,"$fpbSiqlxjGEOGqCWMf_gxTnRHdsXA5BO3V3nANPGl8m4":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":35},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":33},2815,"小儿先天性白细胞颗粒异常综合"," 小儿先天性白细胞颗粒异常综合症：症状、诊断与治疗指南\n"," 小儿先天性白细胞颗粒异常综合症, 白细胞颗粒异常, 先天性白细胞疾病, 儿童免疫系统疾病, 小儿疾病诊断, 小儿疾病治疗, 小儿血液疾病\n"," 了解小儿先天性白细胞颗粒异常综合症的详细信息，包括症状、诊断方法及治疗指南。为家长和医护人员提供全面的知识，帮助有效应对这种罕见的儿童免疫系统疾病。",[18,23,28],{"id":19,"classification":20,"description":21,"sort":22,"diseaseId":12},137199,"概述","\u003Cp>　　先天性白细胞颗粒异常综合征(Chédiak-Higashi syndrome)又称Chédiak-Higashi综合征、Chédiak-Higashi-Steinbrinck综合征、遗传性白细胞颗粒异常综合征、先天性过氧化物酶颗粒症、先天性白细胞异常白化综合征(congenital leukocytic abnormaly albinism syndrome)等。为一罕见的伴有神经系统及血液系统异常的白化病，属常染色体隐性遗传性疾病，多见于近亲结婚的后代。\u003C/p>",0,{"id":24,"classification":25,"description":26,"sort":27,"diseaseId":12},137200,"病因","\u003Cp>　　(一)发病原因\u003C/p>\u003Cp>　　常染色体隐性遗传性疾病，多见于近亲结婚的后代。人类CHS1基因突变引起。\u003C/p>\u003Cp>　　(二)发病机制\u003C/p>\u003Cp>　　本病征的发病机制可能是粒细胞内细胞膜界的广泛异常，粒细胞趋化性异常可能与细胞膜或胞浆内巨大颗粒有关，使之通过微孔发生障碍，对趋化刺激因子的反应差。白细胞异常在电镜超微白细胞化学结构上，白细胞组织内部有巨大溶酶颗粒体、酸性水解酶、磷脂水解酶、过氧化酶等，异常的溶酶颗粒结合在溶酶体膜上，因而被认为是溶酶体疾病，由于多种水解酶因溶酶体膜缺陷而不能被释放到吞噬空泡内，脱颗粒作用亦延迟，结果这种中性粒细胞表现为向化性能力减弱，杀灭细菌能力差，在脊髓内易于破坏而致中性粒细胞减少。\u003C/p>",1,{"id":29,"classification":30,"description":31,"sort":32,"diseaseId":12},137201,"检查","\u003Cp>　　检查项目：脑脊液的化学检查、血常规、血清溶菌酶（LYSO）、骨髓象分析、免疫病理检查、胸部平片、脑电图检查、B型超声波检查\u003C/p>",2,[34],"皮肤科",true]