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伏格特-小柳-原田综合征及其多系统受累情况。

Vogt-Koyanagi-Harada's syndrome and its multisystem involvement.

作者信息

Mota Luiz Alberto Alves, Santos Arthur Borges Dos

机构信息

Faculdade de Ciências Médicas da Universidade de Pernambuco, Pernambuco, PE, Brazil.

出版信息

Rev Assoc Med Bras (1992). 2010 Sep-Oct;56(5):590-5. doi: 10.1590/s0104-42302010000500023.

Abstract

UNLABELLED

Vogt-Koyanagi-Harada's syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, inner ear and skin. Some ethnic groups have a higher probability of developing the disease, including Asians, Indians and Latin Americans and females are affected more often.

METHODS

Literature was reviewed in books, articles published on the internet and papers available in the online databases MEDLINE, LILACS and SciELO. Texts were selected that focused on otorhinolaryngological symptoms.

LITERATURE REVIEW

The disease probably has autoimmune etiology, with aggression occurring on the surface of melanocytes by promoting inflammatory reaction in which T lymphocytes predominate. The allele most often found in association with this disease is HLA DRB1*0405. Clinical manifestations are divided into four stages: prodromal, uveitic, chronic and recurrent. Otorhinolaryngological symptoms occur during the uveitic stage and are characterized by bilateral sensorineural hearing loss, tinnitus and vestibular symptoms. Diagnosis is made according to the diagnostic criteria for the disease. Treatment is primarily with corticosteroids.

CONCLUSIONS

It is important that professionals in other specialties are able to recognize Vogt-Koyanagi-Harada’s syndrome, because late diagnosis can lead to ocular and cutaneous sequelae.

摘要

未标注

伏格特-小柳-原田综合征是一种罕见疾病,会影响含黑素细胞的组织,如眼睛、中枢神经系统、内耳和皮肤。一些种族患该疾病的概率更高,包括亚洲人、印度人和拉丁美洲人,且女性受影响更为常见。

方法

对书籍、互联网上发表的文章以及在线数据库MEDLINE、LILACS和SciELO中的论文进行文献综述。选取聚焦于耳鼻喉科症状的文本。

文献综述

该疾病可能具有自身免疫病因,通过促进以T淋巴细胞为主的炎症反应,对黑素细胞表面发起攻击。与该疾病最常相关的等位基因是HLA DRB1*0405。临床表现分为四个阶段:前驱期、葡萄膜炎期、慢性期和复发期。耳鼻喉科症状出现在葡萄膜炎期,其特征为双侧感音神经性听力损失、耳鸣和前庭症状。根据该疾病的诊断标准进行诊断。主要治疗方法为使用皮质类固醇。

结论

其他专科的专业人员能够识别伏格特-小柳-原田综合征很重要,因为诊断延迟可能导致眼部和皮肤后遗症。

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