小柳原田氏综合征

小柳原田氏综合征 概述 小柳-原田综合征又称葡萄膜大脑炎(uveoencephalitis)综合征，也称色素膜脑膜脑炎或眼-脑-耳-皮综合征。本病好发于青壮年，男性稍多。发病率与性别无明显关系，但黄种人多见，全病程常持续数月至1 年后可自然缓解，但少数患者容易反复再发，病程可达数年或数十年之久。本病有眼葡萄膜炎、视网膜出血和剥离、白癜风、秃头、白毛症(头发、眉毛、睫毛变灰白)和神经系统损害，可先后出现。 描述 小柳-原田综合征又称葡萄膜大脑炎(uveoencephalitis)综合征，也称色素膜脑膜脑炎或眼-脑-耳-皮综合征。Vogt(1906)和小柳(1941)先后报道一种伴有视网膜脱离的双眼渗出性葡萄膜炎(色素膜炎)，发病前有脑膜刺激症状，根据临床观察，两者有相似之处，故称为Vogt-小柳-原田(Vogt-Koyanagi-Harada)综合征。 原因 病因目前尚无定论，文献报道有内分泌学说、病毒学说和免疫学说等三种学说。 病理生理变化 1.内分泌学说主张者认为病变范围包括脑下垂体、卵巢、甲状腺及肾上腺等。 2.病毒学说认为某种病毒作为诱因而触发易感机体。 3.Yokoyama 等发现葡萄膜大脑炎患者的血清中IgD 水平增高，推测与免疫反应有关。此外，近年来证实色素细胞既是抗原又是靶细胞。本病可能与色素细胞抗原有关。 病理改变:小柳-原田综合征的病理改变特征是弥漫性的中、小血管炎性改变为主，引起一系列的脑膜、脑实质及脑脊神经根的损害，而血管损害又以弥漫性小血管损害为主。可见软脑膜、蛛网膜、脑实质、脑神经及蛛网膜下隙小血管周围有单核细胞浸润，还可见到局部脑组织水肿、缺血性改变及胶质细胞增生等。 症状体征 本病好发于青壮年，男性稍多。发病率与性别无明显关系，但黄种人多见，全病程常持续数月至1 年后可自然缓解，但少数患者容易反复再发，病程可达数年或数十年之久。本病有眼葡萄rAt present, we have formed a "three-three" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on development needs in the next decade, based on the original, and then extended the 6 square kilometers. Of the Park, to further improve the roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, to achieve full coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determine 1 leading industries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road company" Park, industrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park as the starting area of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a timely manner ... ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimize government service. Low efficiency of government services, departments do not coordinate the views of businesses large, poor business environment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examination and approval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examination and approval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancel, are not allowed to keep or disguised approval. To do list list of administrative powers and responsibilities 膜炎、视网膜出血和剥离、白癜风、秃头、白毛症(头发、眉毛、睫毛变灰白)和神经系统损害。可先后出现。临床一般分为三期: 1.前驱期 又称脑炎与脑膜炎期。常突然发病，多数有感冒症状、全身不适、发热、头痛、头晕，常伴有脑膜刺激征、嗜睡、耳鸣、听力障碍及意识障碍，偶可见偏瘫、失语、脑神经瘫痪。约有50%的患者出现耳鸣和听力减退，常为一过性，少数可有严重耳聋。Vogt-小柳综合征，大约50%患者在起病期有脑膜刺激征，而原田综合征患者约有90%有脑膜刺激征症状。此期常持续数月后逐渐缓解。 2.眼病期 一般在前驱症状5,6 天后出现眼病，双眼可同时或先后出现弥漫性渗出性葡萄膜炎，视力高度减退。Vogt-小柳综合征患者虹膜睫状体炎明显，角膜后壁有大小不等的角膜沉积物(KP)和虹膜结节，易引起虹膜后粘连，屈光间质浑浊。原田综合征患者视盘充血水肿，黄斑部水肿更明显，后极视网膜斑状水肿发灰，常引起视网膜脱离。 3.恢复期 一般6 个月,1 年，眼部炎症逐渐消退，视网膜脱离复位，眼底有广泛脱色素和色素斑片增生，形成“晚霞”样眼底。严重者在发病1,2 周陆续出现各种并发症，如继发性青光眼、白内障、视神经炎、耳聋及前庭性平衡障碍，严重者可失明，少数可表现柯萨柯夫(Korsakoff)综合征。数月后可出现如秃发、发灰变和皮肤白斑等皮肤症状。 诊断检查 诊断:根据本综合征的临床表现如发热、头痛、呕吐、颈项强直，以后渐出现眼部症状、耳鸣、听力减退、发眉变白、白癜风、斑秃等一系列典型症状，结合腰穿CSF 检查、EEG 及MRI 检查，诊断一般不困难。 实验室检查:腰穿CSF 检查:CSF 的细胞数可增高，以淋巴细胞增多为主，蛋白含量可轻度增高，糖和氯化物一般无异常。 其他辅助检查: 1.脑电图 可显示脑实质受累的改变，可呈弥漫性异常。 2.影像学检查 脑CT 检查可无异常，脑MRI 可有异常发现，但无特异性。 鉴别诊断 imely manner ... ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimtarting area of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a tthe s company" Park, industrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park asading industries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road 1 le to achieve full coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determineimprove the roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, ade, based on the original, and then extended the 6 square kilometers. Of the Park, to furtherthree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on development needs in the next dec-rAt present, we have formed a "threenot allowed to keep or disguised approval. To do list list of administrative powers and responsibilities l, aretion and approval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancen and approval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examinainatiosiness environment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examvernment service. Low efficiency of government services, departments do not coordinate the views of businesses large, poor buize go2 早期应与各种原因的头痛和中枢神经系统感染相鉴别。 治疗 1.早期用足量的皮质类固醇治疗有明显疗效 常用地塞米松10,20mg/d 静脉滴注，7,14 天，或甲泼尼龙(甲基强的松龙)500,1000mg/d 静脉滴注，3,5 天，以后改为泼尼松40,60mg/d 口服，应逐渐减量，维持量要服用数月，停药前应注射ACTH 以免复发。 2.若激素疗效欠佳，可加用免疫抑制药如环磷酰胺等治疗，有助于眼部症状和脑膜炎的好转。应注意激素的副作用。 3.有明显颅内压增高者，应用20%甘露醇脱水，以降低颅内压。此外，可给予如维生素C、冻干人胎盘血丙种球蛋白(胎盘球蛋白)等，以增强机体抵抗力。近年来，应用大剂量免疫球蛋白0.4g/(kg?d)，连用3,5 天，以及血浆置换疗法治疗急性期患者，疗效颇佳。眼部治疗，应充分用1%阿托品扩瞳，并维持瞳孔扩大至炎症消失，这是保持视力的关键所在。 4.局部还可湿热敷，加强血液循环，提高局部抵抗力，同时可加快眼部毒素排出，促进炎症消失。 预防及预后 预后:全病程一般6 个月,1 年，可自然缓解，但少数患者容易反复再发，病程可达数年或数十年之久。 预防:尚无较好预防，应早期诊断及早期治疗，在缓解期，激素维持量要服用数月，可改善预后。停药前应注射ACTH 以免复发。眼部治疗，保持视力的关键是维持瞳孔扩大至炎症消失。 并发症 青光眼、白内障、视神经炎、严重者可失明，部分患者出现耳聋及前庭性平衡障碍，少数可表现柯萨柯夫综合征。 流行病学 目前国内尚未查到相关的发病率统计学资料。本病好发于青壮年，但黄种人多见，发病率与性别无明显关系，但男性稍多。 owed to keep or disguised approval. To do list list of administrative powers and responsibilitiesval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancel, are not allappro proval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examination andment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examination and apvironservice. Low efficiency of government services, departments do not coordinate the views of businesses large, poor business en .. ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimize governmentner .rea of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a timely manindustrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park as the starting a Park, dustries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road company"ll coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determine 1 leading inve futhe roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, to achie t needs in the next decade, based on the original, and then extended the 6 square kilometers. Of the Park, to further improvethree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on developmen-rAt present, we have formed a "three3 葡萄膜炎患者的饮食疗法 葡萄膜炎是一种多发于青壮年的眼病，近几年发病群体逐渐扩大，特别是青少年发病率呈上升趋势。葡萄膜炎是既可以单独发病又是白塞氏病临床表现中给患者伤害最为严重的临床表现之一。葡萄膜炎指的是虹膜、睫状体、脉络膜的炎症;虹膜和睫状体的血液供给同为虹膜大环，故二者经常同时发炎，而总称为虹膜睫状体炎。如果脉络膜也同时发炎，则称为葡萄膜炎。由于发病急，变化快，反复发作;并出现严重并发症，严重影响视力，甚至失明，给患者带来巨大痛苦。葡萄膜炎的病因复杂，除了诊断病因及对症治疗外，辨证配制一些食疗套餐，有利于疾病的康复。现代医学研究院医院白塞氏病康复中为葡萄膜炎患者推荐一些常用配餐: ? 金银花菊花茶:金银花50克，菊花50克，绿茶20克。上药混合共为粗末，用纱布分装成袋，每袋15克。每次1包，代茶饮用。可清凉解热、疏风明目。用于头眼胀痛、目睛红赤患者。 :蔓荆子15克，粳米50克。将蔓荆子捣碎，加水500? 蔓荆子粥 毫升，浸泡后煎取汁，入粳米煮粥，空腹食用。每日1剂。可辛凉解散，用于目赤头痛患者。 ? 青葙子茶:青葙子15克，绿茶5克。将青葙子和绿茶置于纱布袋中，沸水泡10分钟饮用。每日1剂。可祛风热、清肝火、适用于目赤肿痛患者。 ? 石膏粥:生石膏50克，粳米100克。先将石膏水煎半小时，去渣后放入粳米熬粥。每日1剂。可辛凉清热、除烦止渴，适用于眼红痛、口干重的患者。 ? 绿豆藕羹:藕1节，绿豆30克。将藕洗净切成小块，与绿豆同煮至熟烂后食用。每日1剂。可清热凉血、去赤止痛，适用于眼热赤痛患者。 ? 二仁粥:生薏仁30克，杏仁6克(捣碎)，粳米100克。三物共用水煮，至米开粥稠即可食用。每日1剂。可清热利湿，宣畅气机，适用于葡萄膜炎反复发患者。 ? 香菇烧冬瓜:冬瓜300克，香菇20克，调料适量。冬瓜去皮瓤、洗净、切片。香菇浸泡透，洗净。二味用油炒后，烧熟。每日1剂。可清湿热、益胃气，适用于脾胃湿热重的葡萄膜炎患者。 vernment service. Low efficiency of government services, departments do not coordinate the views of businesses large, poor buize goimely manner ... ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimtarting area of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a tthe s company" Park, industrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park asading industries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road 1 le to achieve full coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determineimprove the roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, ade, based on the original, and then extended the 6 square kilometers. Of the Park, to furtherthree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on development needs in the next dec-rAt present, we have formed a "threenot allowed to keep or disguised approval. To do list list of administrative powers and responsibilities l, aretion and approval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancen and approval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examinainatiosiness environment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative exam4 葡萄膜炎患者日常生活注意事项 1、少食或不食辛辣煎炸烧烤食品; 2、稳定情绪，避免急燥沮丧; 3、已婚患者节戒房事，安心调养; 4、戒烟忌酒; 5、患病期间少用视力，户外活动宜戴有色眼睛，避免强光刺激; 6、坚持用熏洗、热敷; 7、食疗: ? 乌龟土茯苓汤:乌龟1只去头足内脏洗净，土茯苓100g加适量水文火煲1-2小时，调味食用; ? 瘦肉夏枯草汤:瘦肉200g配适量夏枯草洗净加适量水，文火堡1小时，调味食用。 中医中治疗葡萄膜炎 河南现代医学研究院医院白塞氏病康复中心根据葡萄膜炎的病因病机并紧密结合每一位患者的临床具体情况采用辩症施治，对症配伍，精心炮制，确定治疗方案。在临床治疗中要紧扣热、毒、瘀、湿、阴虚五个方面进行辩症，灵活掌握清热解毒、化湿、祛瘀、养阴的要点。 1、肝经风热 辩证:发病急骤，目珠坠痛，热泪频流，怕光差明，视物模糊，抱轮红赤，黑睛内壁有灰白色点状沉着物，神水混浊，黄仁晦暗，纹理不清，瞳神紧小，全身可兼有头额疼痛，发热、口干舌红、舌苔薄白或薄黄、脉浮数。 治法:疏风清肝明目，散邪消滞。 常用方:新制柴连汤加减。 2、肝胆湿热 辩证:发病急、眼痛拒按，热泪频流、视力急降、抱轮红甚或白睛湿赤，黑睛内壁、灰白沉着物密集，黄仁肿胀，纹理不清，瞳神紧小，多处与晶珠粘连，神水混浊或伴有黄液上冲，甚则血灌瞳神，烦 val matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancel, are not allappro proval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examination andment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examination and apvironservice. Low efficiency of government services, departments do not coordinate the views of businesses large, poor business en .. ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimize governmentner .rea of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a timely manindustrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park as the starting a Park, dustries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road company"ll coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determine 1 leading inve futhe roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, to achie t needs in the next decade, based on the original, and then extended the 6 square kilometers. Of the Park, to further improvethree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on developmen-rAt present, we have formed a "threeowed to keep or disguised approval. To do list list of administrative powers and responsibilities5 燥易怒，口苦咽干，溺短便结，舌质红，苔黄腻，脉弦数。 治法:清肝泻胆、凉血散瘀，明目; 常用方:龙胆泻肝汤加减。 3、风热夹湿 辩证:发病有急有缓，病程缠绵反复发作，眼前有黑点或黑花飘舞，抱轮红赤久不退，黑睛内壁沉着物多为灰白点状，神水混浊，黄仁纹理模糊、瞳神紧小或干缺不圆，甚至可见白膜附着，头闷身重，胸脘满闷，肢节酸软，舌质红或淡红，舌苔厚腻，脉濡数。 治法:清热除湿，祛风散滞，活血通经; 常用方:抑阳酒连散加减。 4、阴虚火旺 辩证:病势较轻或病到后期，白睛红赤不甚、眼内干涩不适，眼疼时轻时重，黑睛内壁沉着物小而量少，但久不消退，神水混浊不明显，瞳神紧小或干缺，口干咽燥，虚烦不眠，手足心热，舌红苔薄，脉细数。 治法:滋阴补肝肾，清降虚火; 常用方:知柏地黄汤，六味地黄汤加减。 5、脾肾阳虚 辩证:病程迁延，白睛不红或红赤不甚，视物昏花，黑睛内壁沉着物或灰白色，黄仁晦暗。瞳神紧小或干缺，四肢不温，形寒气怯，腰酸膝冷，下利精谷，小便不利，舌质淡胖，脉沉迟细弱。 治法:补脾扶阳、温经散寒; 常用方:附子理冲汤，肾气加减。 n and approval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examinainatiosiness environment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examvernment service. Low efficiency of government services, departments do not coordinate the views of businesses large, poor buize goimely manner ... ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimtarting area of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a tthe s company" Park, industrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park asading industries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road 1 le to achieve full coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determineimprove the roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, ade, based on the original, and then extended the 6 square kilometers. Of the Park, to furtherthree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on development needs in the next dec-rAt present, we have formed a "threenot allowed to keep or disguised approval. To do list list of administrative powers and responsibilities l, aretion and approval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cance6 小柳原田氏综合症 小柳原田氏综合征，又称特发性葡萄膜大脑炎、色素膜一脑膜炎，是一种原因不明的累及全身多器官如眼、耳、皮肤、脑膜的综合征。AlfredVogt(1906)和小柳美三(1941)先后报道了一种伴有毛发变白、脱发、皮肤脱色斑及听力损害的双眼葡萄膜炎，发病前有脑膜刺激症状，称为vkh综合症。1926年原田永之助报道了一种伴有视网膜脱离的双眼渗出性葡萄膜炎，发病前亦有脑膜刺激症状，称为原田 氏病。此后，根据不断观察，发现两者之间并无明显界线，实际上均属于弥漫性渗出性葡萄膜炎，仅病变程度、主要受害部位和各种症状、体征出现的早晚略有不同而已，故被统称为Vogt—Koyanai—Halada综合征(VKH)。 本病多见于黄种人，容易反复再发，病程有长达数年及至数十年者。发病年龄以30——40岁居多，50岁以后少见，我国文献中最小发病年龄为10岁。发病率与性别 无关。以双侧弥漫性葡萄膜炎，伴有头痛、颈项强直等脑膜刺激症状，以及耳鸣、听力下降、皮肤色素脱失、白瘢风、全身毛发变白等全身情况为主要特征。典型病例临床容易诊断，但多数患者神经系统、皮肤、毛发的症状出现较晚或不全出现，因而多数病人早期容易被误诊。 小柳原田氏综合症(VKH综合症)发病原因 小柳原田氏综合症发病一种说法是与病毒感染有关;一种说法是属于自身免疫性疾病;由于本病多见于有色人种，故推测是针对色素上皮的免疫反应。目前认为该病很可能是在遗传素质基础上先有致病因子如病毒作用于易感的机体，引起非特异性前躯期症状，另方面致病因子引起色素细胞抗原性改变，而发生自体免疫反应，出现全身性色素细胞受损害的各种临床体征。小柳原田氏综合征的病理改变特征是弥 漫性的中、小血管炎性改变为主，引起一系列的脑膜、脑实质及脑脊神经根的损害，而血管损害又以弥漫性小血管损害为主。可见软脑膜、蛛网膜、脑实质、脑神经及蛛网膜下隙小血管周围有单核细胞浸润，还可见到局部脑组织水肿、缺血性改变及胶质细胞增生等。 小柳原田氏综合症(VKH综合症)临床表现 临床表现分为三个阶段: val matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancel, are not allappro proval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examination andment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examination and apvironservice. Low efficiency of government services, departments do not coordinate the views of businesses large, poor business en .. ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimize governmentner .rea of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a timely manindustrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park as the starting a Park, dustries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road company"ll coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determine 1 leading inve futhe roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, to achie t needs in the next decade, based on the original, and then extended the 6 square kilometers. Of the Park, to further improvethree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on developmen-rAt present, we have formed a "threeowed to keep or disguised approval. To do list list of administrative powers and responsibilities7 1(前驱期:又称脑炎与脑膜炎期。病人在眼症状出现前常有头痛、头晕、耳鸣、全身不适等感冒症状，严重者可出现高热、头痛、颈项强直以至昏迷，(小柳型50%患者有脑膜刺激症状;原田型可高达90%)，脑脊液检查可发现蛋白和淋巴细胞数增加，并表现病理性脑电图。此期5,6天。脱发、眉毛变白、白癜风、斑秃和听力障碍等一系列症状则在漫长的病程中逐渐出现。 2(眼病期:双眼同时或先后出现弥漫性、渗出性葡萄膜炎。双眼视力迅速下降，伴有羞明流泪、眼疼、闪光、飞蚊、变形。眼部检查:?小柳氏型:整个色素膜均可受累，但以前部色素膜炎为主。检查可见睫状体充血、角膜后沉着物，虹膜结节及后粘连等，短期内可形成瞳孔闭锁或膜闭，因而眼底不能窥见。常因继发青光眼而失明。?原田氏型:主要表现为急性弥漫性渗出性脉络膜炎，视网膜高度水肿或视网膜脱离，视力可降至光感。前部色素膜炎症较轻，仅见角膜后沉着物和房水闪光，玻璃体虽然混浊，但多能窥见眼底，数周后视网膜水肿及脱离可自行消退。眼底因色素脱失似晚霞之赤色，脉络膜血管外露，并可见大小不等黑白点交杂的脉络膜斑，常有反复发作的倾向。 3(恢复期:一般6个月至1年。眼内炎症逐渐消失，视网膜脱离复位，眼底有色素沉着，由于视网膜色素上皮崩溃脱失，眼底呈晚霞状。严重者可有各种并发症，如白内障、继发性青光眼，甚至失明。 辅助检查 1.腰椎穿刺:脑脊液压力升高，CSF细胞数可增高，以淋巴细胞增多为主，蛋白升高、免疫球蛋白升高，糖和氯化物一般正常。 2.头颅MRI:头MR可发现脑实质损害或脑膜强化;病情可复发;应与颅内炎症、多发性硬化等鉴别;糖皮质激素治疗有效。有时MRI平扫可见双侧脉络膜呈对称性增厚并脱离，强化扫描可见增厚脱离的脉络膜显著异常强化，眼巩膜正常。 3.眼超声检查:提示脉络膜脱离。 4.眼底荧光造影(FFA):早期视网膜色素上皮的渗漏，以及晚期出现的特征性的多湖状或多囊状的荧光素贮存，这些对VKH综合征早期诊断有辅助作用。 5.HLA分型检查:HLA-DR4、HLA-DRw53有较高的阳性率。 诊断及鉴别诊断 not allowed to keep or disguised approval. To do list list of administrative powers and responsibilities l, aretion and approval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancen and approval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examinainatiosiness environment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examvernment service. Low efficiency of government services, departments do not coordinate the views of businesses large, poor buize goimely manner ... ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimtarting area of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a tthe s company" Park, industrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park asading industries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road 1 le to achieve full coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determineimprove the roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, ade, based on the original, and then extended the 6 square kilometers. Of the Park, to furtherthree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on development needs in the next dec-rAt present, we have formed a "three8 1.临床症状:患者突然发病，常伴有头痛、耳鸣以及感冒样的前驱症状，严重者可有脑膜脑炎的临床表现。以后渐出现眼部症状，多两眼同时受累，视力急剧下降，眼底改变，后极部视网膜水肿，浆液性视网膜脱离，视乳头充血水肿，玻璃体混浊，炎症消退后视网膜复位，呈“晚霞”样眼底，或可表现虹膜睫状体炎的改变。 2.病程中可出现耳鸣、重听、毛发变白、疏发以及白癜风等病征。 3.常需与中枢神经系统感染、各种原因的头痛、全葡萄膜炎病人、白塞氏病患者进行鉴别。 【关键词】 Vogt-小柳原田综合征/治疗 【摘要】 目的 探讨应用中西医结合治疗Vogt-小柳原田综合征的疗效。方法 Vogt-小柳原田综合征的病人19例38眼，早期静脉滴注地塞米松，后改口服强的松并逐渐减量。初起时中医辨证为肝经风热者，予新制柴连汤加减;肝胆火炽者，予龙胆泻肝汤加减;湿热蕴蒸者，予三仁汤。后期予知柏地黄丸。结果 治疗1周后，矫正视力至0.3,0.9，平均0.60;与治疗前比较，差异有非常显著性(P<0.01)。急性症状控制时间5,14天，平均8天，至痊愈停药时间3,13个月。追踪观察2,23个月，最短矫正视力0.3,1.5，平均0.83。出现晚霞状眼底者13例，占68.4%。结论 联合应用中医辨证论治有其优越性。急性期糖皮质激素联合中药应用，症状控制快，使大剂量糖皮质激素应用间缩短。恢复期合用知柏地黄丸可起到稳定病情、减少复发的作用。 关键词 Vogt-小柳原田综合征/治疗 Vogt-小柳原田综合征是一种常伴有脑膜刺激征、听功能障碍、皮肤和毛发改变等症状的双眼葡萄膜炎。糖皮质激素是治疗Vogt-小柳原田综合征的主要药物，但长期糖皮质激素应用的副作用及患者病情的反复发作，也成为广大眼科医生面临的棘手问题。现就我院2000年4月,2004年2月中西医结合治疗19例38眼Vogt-小柳原田综合征的疗效及追踪观察情况作一。 1、资料与方法 1.1 一般资料 在我科被确诊为Vogt-小柳原田综合征的病人19例，男13例，女6例;年龄25,51岁，平均年龄35.6岁。起病后就诊时 ment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examination and apvironservice. Low efficiency of government services, departments do not coordinate the views of businesses large, poor business en .. ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimize governmentner .rea of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a timely manindustrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park as the starting a Park, dustries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road company"ll coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determine 1 leading inve futhe roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, to achie t needs in the next decade, based on the original, and then extended the 6 square kilometers. Of the Park, to further improvethree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on developmen-rAt present, we have formed a "threeowed to keep or disguised approval. To do list list of administrative powers and responsibilitiesval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancel, are not allappro proval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examination and9 间4天,1个月。最佳矫正视力0.04,0.4，平均视力0.20。角膜后壁羊脂状kp7例，前房闪辉及房水细胞11例，虹膜后粘连、瞳孔膜闭、继发青光眼2例视乳头充血、水肿，视乳头周围视网膜水肿17例(另2例因瞳孔膜闭、晶体混浊，眼底不能窥入)。行眼底荧光素血管造影(FFA)检查17例，均表现不同程度的视乳头渗漏，视网膜色素上皮水平多发性点状高荧光并迅速渗漏。伴皮肤、毛发变白8例，听力下降7例，头痛11例。 1.2 方法 (1)所有病例早期静脉滴注地塞米松10mg，每日1次，3,7天，至角膜后壁羊脂状kp、前房闪辉减少及房水细胞消失，视乳头水肿减轻，视力开始提高;改为静脉滴注地塞米松5mg，每日1次，3,5天，改口服强的松40,30mg，至角膜后壁羊脂状kp、前房闪辉基本消失，视乳头边界可见，视力明显提高;口服强的松逐渐减量至20mg，角膜后壁羊脂状kp、前房闪辉完全消失，视乳头边界清晰，视力明显提高，维持口服强的松20mg1个月以上。(2)11例有角膜后壁kp、前房闪辉者，频点点必舒眼液，点复方托品酰胺眼液扩瞳。2例瞳孔膜闭者，1%阿托品眼膏涂眼。(3)起初时全身症见头痛发热，口干舌红，苔薄，脉浮数者，中医辨证为肝经风热，予新制柴连汤加减;全身症见口干咽苦，烦躁易怒，舌红苔黄，脉弦数者，中医辨证为肝胆火炽，予龙胆泻肝汤加减;全身症见头重胸闷，肢节酸痛，苔黄腻，脉弦数或濡数者，中医辨证为湿热蕴蒸，予三仁汤。后期予知柏地黄丸。 2、结果 2.1 疗效 治疗1周后，最佳矫正视力0.3,0.9，平均0.60。与治疗前比较，差异有非常显著性(t=7.137>t 0.01(18) 2.878，P<0.01)。强的松完全停用，可停止治疗时，最佳矫正视力0.3,1.4，平均1.1。均无角膜后壁kp、前房闪辉，视乳头边界清晰。复查FFA检查11例，视乳头无渗漏，多发性视网膜脱色素改变，但无荧光素渗漏。 2.2 疗程 急性症状控制(角膜后壁羊脂状kp、前房闪辉减少及房水细胞消失，视乳头水肿减轻，视力提高大于2行)时间5,14天，平均8天。继续治疗至痊愈(角膜后壁羊脂状kp、前房闪辉完全消失，视乳头边界清晰，视力明显提高)停药时间3,13个月。 l, aretion and approval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancen and approval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examinainatiosiness environment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examvernment service. Low efficiency of government services, departments do not coordinate the views of businesses large, poor buize goimely manner ... ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimtarting area of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a tthe s company" Park, industrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park asading industries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road 1 le to achieve full coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determineimprove the roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, ade, based on the original, and then extended the 6 square kilometers. Of the Park, to furtherthree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on development needs in the next dec-rAt present, we have formed a "threenot allowed to keep or disguised approval. To do list list of administrative powers and responsibilities10 2.3 预后 门诊追踪观察2,23个月，最终矫正视力0.3,1.5，平均0.83。出现晚霞状眼底者13例，占68.4%。3 讨论Vogt-小柳原田综合征的病理基础是机体对全身黑色素细胞的选择性损害，从而出现双侧性葡萄膜炎及中枢神经、内耳、皮肤毛发的损害。易发生于含色素较多的人群，据报道中国葡萄膜炎者中约14%为Vogt-小柳原田综合征。故其为一种较常见的全身性免疫性疾病。早期的诊断、正确治疗，对其预后及减少复发有非常重要的意义。糖皮质激素是治疗早期Vogt-小柳原田综合征的主要药物。早期大剂量激素冲击治疗可缩短病程，改善预后。有前葡萄膜表现者，局部应用激素以点糖皮质激素眼液为主，不主张结膜下注射。应用短效或中效扩瞳剂点眼以活动瞳孔，防止虹膜前、后粘连。瞳孔膜闭者，1%阿托品眼涂眼，以防瞳孔阻滞。 Vogt-小柳原田综合征发病者为青壮年，急性炎症期多为实证，经辨证论治，属肝经风热者，宜祛风散邪、清肝泻热，应用新制柴连汤;属肝胆火炽者，宜清泻肝胆，应用龙胆泻肝汤;属湿热蕴蒸者，宜宣化畅中、清热利湿，应用三仁汤。急性期糖皮质激素联合中药应用，症状控制快，使大剂量糖皮质激素应用时间缩短。本组病例经上述治疗，葡萄膜炎症迅速控制，视力明显提高。但需强调大剂量激素冲击后应逐渐减量并在葡萄膜炎表现已控制后，继续维持量最少1个月，并注意监控血糖、水电解质平衡及血压等。 Vogt-小柳原田综合征作为一种自身免疫性疾病，且该年龄组为青壮年，易受劳累、精神紧张等因素刺激，极易复发，使病程慢长迁延不愈。往往在急性炎症控制2周,1个月后病情再次反复，使减至维持量的激素又要加大剂量。长期糖皮质激素应用有其副作用。 恢复期或慢性复发期因病程长久，久病伤阴，阴虚火旺，合用知柏地黄丸以扶正祛邪、滋阴降火，可起到稳定病情、减少复发的作用。我们体会联合应用中医辨证论治有其优越性。 只要早期诊断、正确治疗，就能获得较好的预后，减少并发症的出现。即使出现晚霞状眼底，仍可保持较好视力。 owed to keep or disguised approval. To do list list of administrative powers and responsibilitiesval matters, strengthen the existing supervision in matters of administrative power, has made it clear to cancel, are not allappro proval system, good digestion, implement the Government canceled, adjustment and devolution of administrative examination andment, it is ... ... Short Board, decide to change. We will continue to deepen reform of the administrative examination and apvironservice. Low efficiency of government services, departments do not coordinate the views of businesses large, poor business en .. ... Transfer of the wood industry. This opportunity slip by, so the timing must be tight. Second is to optimize governmentner .rea of the industrial park, planners need to take out plan, start construction as soon as possible, undertake in a timely manindustrial agglomeration. Currently, to focus on building better ... ... Industrial Park, ... ... Wood Park as the starting a Park, dustries, in particular stone Materials, cans, gloves, through guided, force and other measures to promote the "road company"ll coverage of infrastructure and interconnection with the facilities and raise capacity. Each park to determine 1 leading inve futhe roads, power supply, communications, water supply and drainage and sewage pipe network and other infrastructure, to achie t needs in the next decade, based on the original, and then extended the 6 square kilometers. Of the Park, to further improvethree" Park. …… Industrial Park, according to ... ... The instructions of comrade, focus on developmen-rAt present, we have formed a "three11