[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"$f65-g5LTUgHIk8dGZIHhGe2pKpxnfW0g-O4HGxk3yxcs":3,"$foNjrnM7cSYDCAuNCjKW93oAhWKfeJuEgG5gDEaF7Hq4":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":67},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":63},6366,"岩藻糖苷贮积症"," 岩藻糖苷贮积症：症状、诊断及治疗方法\n"," 岩藻糖苷贮积症, 岩藻糖苷贮积症症状, 岩藻糖苷贮积症诊断, 岩藻糖苷贮积症治疗, 罕见疾病, 遗传病\n"," 了解岩藻糖苷贮积症的详细信息，包括症状、诊断方式及治疗方法。这是一种罕见的遗传性疾病，了解相关信息有助于提高对该病的认识和正确应对病情。",[18,23,28,33,38,43,48,53,58],{"id":19,"classification":20,"description":21,"sort":22,"diseaseId":12},105716,"概述","\u003Cp>　　岩藻糖苷贮积症(fucosidosis)是一种因α岩藻糖苷酶缺陷而引起的类黏多糖贮积症。临床上以神经系统异常、反复呼吸道感染、智力低下及心脏病变为特征，无黏多糖尿。\u003C/p>",0,{"id":24,"classification":25,"description":26,"sort":27,"diseaseId":12},105717,"病因","\u003Cp>　　遗传基因缺陷，病人体内α-岩藻糖苷酶缺陷是本病的主要病因。\u003C/p>",1,{"id":29,"classification":30,"description":31,"sort":32,"diseaseId":12},105718,"症状","\u003Cp>　　常见症状：反复上呼吸道感染、肌张力减低、出汗异常、异常矮小、智力发育迟缓、儿童精神运动发育异常、抽风、脊柱和四肢畸形\u003C/p>\u003Cp>　　Schoonderwaldt等(1980)根据症状出现的年龄将该病分为Ⅰ、Ⅱ、Ⅲ型，Ⅰ、Ⅱ型多于婴幼儿期，发病症状重，故也称幼儿型。Ⅲ型在成人发病，症状轻，又称成人型。幼儿型在一岁左右就可出现明显的临床特征，常表现有反复发作的呼吸道感染，全身肌张力低下，出汗过多和体态短小。进行性智力和运动发育迟缓可以是其最早的表现。自2岁开始，患儿神经症状进行性加重，伴以频发的抽风。有些患儿呈现轻度黏多糖贮积症Ⅰ型面容，肝脾肿大，心脏扩大，皮肤增厚，腰背侧弯。另一些患儿的面容更像黏多糖贮积症Ⅰ型，表现有前额突出，眼间距过宽，鼻梁塌陷，厚嘴唇和伸舌等丑陋面容。神经症状不明显，角膜一般清晰。神经系统症状恶化始于出生后6个月，多死于10岁以内。\u003C/p>\u003Cp>　　成人型临床表现与幼儿型相似，但也有所不同。成人型除可出现进行性智力和运动发育障碍、生长迟缓、肌无力和肌张力低下、面容粗笨，无肝脾肿大、无角膜浑浊之外，其最特征性表现为皮肤有弥漫性血管角质瘤，表现为针尖大小蓝褐色隆起的皮损，起初分布于腹背部，以后可扩展至上、下肢。有时可出现皮肤无汗症，一旦感染就可出现高热和抽风。\u003C/p>",2,{"id":34,"classification":35,"description":36,"sort":37,"diseaseId":12},105719,"检查","\u003Cp>　　检查项目：汗液电解质检查、肝组织活检、X线检查\u003C/p>\u003Cp>　　在周围血液淋巴细胞内可见有PAS染色弱阳性的空泡，在汗液中，氯化物和钠的含量较正常人高3～9倍，尿中无过多黏多糖排出，但有过多含岩藻糖的低聚糖和双糖排出，肝组织活检组织细胞生化分析显示有α-岩藻糖苷酶缺陷。\u003C/p>\u003Cp>　　X线检查：有多发性骨发育不良，骨骺成熟延迟，颅骨增厚，腰背部侧弯，伴以椎体双凸畸形，椎体发育不全，椎体前上部呈鸟嘴状。\u003C/p>",3,{"id":39,"classification":40,"description":41,"sort":42,"diseaseId":12},105720,"鉴别","\u003Cp>\u003C/p>\u003Cp>　　在鉴别诊断方面需注意与其他几种黏脂贮积症相鉴别。\u003C/p>",4,{"id":44,"classification":45,"description":46,"sort":47,"diseaseId":12},105721,"并发症","\u003Cp>　　本病可并发神经系统症状，呼吸道感染，全身肌张力低下。成人型除可并发进行性智力和运动发育障碍。\u003C/p>",5,{"id":49,"classification":50,"description":51,"sort":52,"diseaseId":12},105722,"预防","\u003Cp>　　开展婚姻和生育指导、努力降低人群中遗传病发生率、提高人口素质。通常的措施包括：婚前检查、遗传咨询、产前检查和遗传病的早期治疗。\u003C/p>\u003Cp>　　如给垂体性侏儒患者以生长激素，给血友病患者以抗血友病蛋白(凝血因子)，给遗传性免疫缺陷病人以相应的免疫球蛋白。\u003C/p>",6,{"id":54,"classification":55,"description":56,"sort":57,"diseaseId":12},105723,"治疗","\u003Cp>　　无特效疗法，可用手术矫正骨骼畸形和用抗生素控制感染。\u003C/p>",7,{"id":59,"classification":60,"description":61,"sort":62,"diseaseId":12},105724,"饮食","\u003Cp>　　1.不宜吃得过多、过杂，以免肠道细菌过度生长，引起细菌移位，导致原发性腹膜炎。\u003C/p>\u003Cp>　　2.忌饮酒，最好滴酒不沾，以免加重肝损害。\u003C/p>\u003Cp>　　3.避免各种刺激性辛辣、过热、生硬食物，含植物纤维较多的蔬菜也应慎吃。服药时，较大的药片应研碎服用。\u003C/p>\u003Cp>　　4.如有水肿或腹水，宜低盐饮食。\u003C/p>",8,[64,65,66],"呼吸内科","神经内科","内分泌科",true]