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重症Vogt-小柳-原田综合征的临床及组织病理学观察

Clinical and histopathologic observations in severe Vogt-Koyanagi-Harada syndrome.

作者信息

Perry H D, Font R L

出版信息

Am J Ophthalmol. 1977 Feb;83(2):242-54. doi: 10.1016/0002-9394(77)90623-7.

DOI:10.1016/0002-9394(77)90623-7
PMID:836666
Abstract

Vogt-Koyanagi (V-K) syndrome is a severe anterior uveitis associated with alopecia, vitiligo, poliosis, and dysacousia. Harada's disease (HD) is primarily a posterior uveitis accompanied by signs of meningeal irritation and abnormalities of the cerebrospinal fluid. The overlapping of clinical manifestations between the two justified considering them as part of a spectrum of one disease (V-K-H). In a clinicopathologic study of nine cases four patients (three with V-K syndrome and one with V-K-H syndrome) histopathologically displayed a granulomatous uveitis. Five patients (two with V-K syndrome and three with HD) showed a nongranulomatous uveitis. We emphasized this latter finding, since in the past the diagnosis of this syndrome has been discarded both clinically and histopathologically because of the absence of a granulomatous uveitis. We established the clinical and histopathologic differences between V-K-H and sympathetic ophthalmia. The designation "uveomeningoencephalitic syndrome" stresses the key features of V-K-H, namely the uveal involvement and signs of meningeal irritation (that is, headaches, personality changes, and cerebrospinal fluid alterations).

摘要

伏格特-小柳(V-K)综合征是一种严重的前葡萄膜炎,伴有脱发、白癜风、白发和听力障碍。原田病(HD)主要是一种后葡萄膜炎,伴有脑膜刺激征和脑脊液异常。两者临床表现的重叠使得将它们视为一种疾病谱系(V-K-H)的一部分是合理的。在一项对9例病例的临床病理研究中,4例患者(3例V-K综合征和1例V-K-H综合征)组织病理学显示为肉芽肿性葡萄膜炎。5例患者(2例V-K综合征和3例HD)表现为非肉芽肿性葡萄膜炎。我们强调了后一项发现,因为过去由于缺乏肉芽肿性葡萄膜炎,该综合征在临床和组织病理学上都被排除。我们确定了V-K-H与交感性眼炎之间的临床和组织病理学差异。“葡萄膜脑膜脑炎综合征”这一名称强调了V-K-H的关键特征,即葡萄膜受累和脑膜刺激征(即头痛、人格改变和脑脊液改变)。

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Clinical and histopathologic observations in severe Vogt-Koyanagi-Harada syndrome.重症Vogt-小柳-原田综合征的临床及组织病理学观察
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