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Objective To investigate the clinical features, causes of blindness and diagnosis of Vogt Koyanagi Harada syndrome Methods The data of 157 patients with VKH syndrome were reviewed and analyzed Patients were carefully examined with slit lamp, ophthalmoscope, three mirror lens, fundus fluorescein angiography, indocyanine green angiography and HLA typing Results Headache was noted in 73 5% of these patients Simultaneous involvement of both eyes occurred in 80 8% of these patients Chroiditis,papilledema and edema of the retina adjacent to the optic nerve were noted in 100% of these patients in the posterior uveitis stage, whereas recurrent granulomatous anterior uveitis (98 4%),"sunset glow" fundus (95 8%) and Dalen Fuchs nodules (71 2%) were the common ocular findings in the recurrent anterior uveitis stage The common causes of blindness were papillitis, exudative retinal detachment and complicated cataract in the posterior uveitis stage, anterior uveal involvement stage and its recurrent stage Poliosis (36 3%) and alopecia (35 0%) were the most common extraocular findings Early irregular patches of fluorescence, followed by localized hyperfluorescent spots were the typical findings of FFA Dilation of choroidal vessels and leakage of ICG from the choroidal vessels were the common ICGA findings The prevalence of HLA DR4 and HLA DRw53 in patients (54 9% and 71 8% respectively) was significantly higher than that in controls (14 7% and 38 2% respectively) Conclusions VKH syndrome is characterized by chroiditis, papillitis or neuroretinitis in the posterior uveitis stage, followed by a generalized uveitis with a typical recurrent granulomatous anterior uveitis Extraocular findings and relevant examinations including FFA, ICGA and HLA typing are helpful to the diagnosis of VKH syndrome

目的探讨Vogt-Koyanagi-Harada综合征患者的临床特征、盲目原因及诊断等有关问题。方法对在1996年1月至2000年12月间就诊资料完整的157例VKH综合征患者进行回顾性分析,并对裂隙灯、眼底镜、三面镜、荧光素眼底血管造影(fundus fluorescein angiography,FFA)、吲哚青绿血管造影(indocyanine green angiography,ICGA)及人类白细胞抗原分型等检查结果进行分析。结果 VKH综合征最常见的前驱症状为头痛(102例,73.5%),双眼同时患病118例(80.8%);后葡萄膜炎期眼部主要表现为脉络膜炎、视乳头及附近视网膜水肿(100.0%);前葡萄膜炎反复发作期眼部表现为复发性肉芽肿性前葡萄膜炎(128例,98.4%)、晚霞状眼底改变(95.8%)及Dalen-Fuchs结节(71.2%);后葡萄膜炎期、前葡萄膜受累期及前葡萄膜炎反复发作期导致盲目的主要原因分别为视乳头炎、视网膜脱离及并发性白内障;毛发变白(36.3%)及脱发(35.0%)是最常见的眼外表现;炎症活动期FFA典型表现为斑驳状高荧光,ICGA发现脉络膜血管扩张、通透性增高等改变;VKH综合征患者HLA-DR4及HLA-DRw53的阳性率(54.9%及71.8%)显著高于正常对照组(14.7%及38.2%)。结论 VKH综合征患者在后葡萄膜炎期眼部典型表现为双侧脉络膜炎、视乳头炎或神经视网膜炎,随后出现以反复发作的肉芽肿性前葡萄膜炎为特征的全葡萄膜炎。眼外症状及相关的辅助检查包括FFA、ICGA 及HLA分型等有助于VKH综合征的诊断。

Fruit baccate; endocarp membranous or fleshy; seeds without endosperm; flowers bisexual or bisexual and male; stamens at least 2 × as many as petals or rarely fewer; functional gynoecium 2- to many loculed, syncarpous

果浆果状;内果皮膜或者肉;没有胚乳的种子;花两性或两性和男性;雄蕊至少2 *更少的花瓣或很少的同数;职能雌蕊群2-到多室,合心皮 14

An opening or orifice, as in a bone or in the covering of the ovule of a plant.

褐色帽子白色肉垂的菌类,茎干的中部有一个膜状的环儿。

Any of various swimming and diving birds of the family Podicipedidae, having a pointed bill and lobed, fleshy membranes along each toe.

科中能游泳并潜水的任一种鸟类,有尖尖的喙,脚趾间有蹼状的肉膜

Bulb narrowly ovoid or cylindric; tunic black-brown, scarious; bulbels many, with several fleshy scales and hard tunics.

鳞茎狭卵球形或圆筒状;鳞茎皮黑棕色,干膜质;珠芽很多,带有几个肉的鳞片和硬鳞茎皮。

Seeds pyriform or rarely irregular in shape; seed coat membranous or thin and brittle; endosperm copious or ± scant, fleshy; embryo relatively large, bent double or rarely broadly horseshoe-shaped; cotyledons plano-convex and linear or ± flattened and elliptic-oblong; hypocotyl superior.

种子梨形或很少外形不规则;种皮膜质或者薄而脆;丰富的胚乳或者不够,肉的±;胚较大,弯曲或者很少宽马蹄形;子叶平凸和线形的或多少扁平和椭圆状长圆形;下胚轴上位。

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