Akram Sharmeen, Khabir Khabir
Section of Ophthalmology, Department of Surgery, The Aga Khan University Hospital, Karachi.
J Coll Physicians Surg Pak. 2014 Sep;24(9):692-4.
Vogt-Koyanagi-Harada (VKH) syndrome is a rare multisystem disease of melanocyte containing organs. It is characterized by diffuse granulomatous inflammation involving various organs including eye. VKH syndrome is usually sporadic, but some familial cases have also been reported indicating a hereditary basis. VKH is not associated with mortality but it may result in long-term complications such as decreased vision associated with cataract, glaucoma and choroidal neovascularization. For successful outcomes, early aggressive treatment using systemic steroids with gradual tapering is essential. This report describes a case of VKH syndrome in a 26-year-old male of Pakistan origin who was successfully treated with systemic steroids. The case is briefly contextualised within wider literature.
伏格特-小柳-原田(VKH)综合征是一种罕见的累及含黑素细胞器官的多系统疾病。其特征为包括眼部在内的多个器官出现弥漫性肉芽肿性炎症。VKH综合征通常为散发性,但也有一些家族性病例报道,提示存在遗传基础。VKH与死亡率无关,但可能导致长期并发症,如与白内障、青光眼和脉络膜新生血管形成相关的视力下降。为取得成功的治疗效果,早期积极使用全身类固醇并逐渐减量至关重要。本报告描述了一例26岁祖籍巴基斯坦男性的VKH综合征病例,该患者通过全身类固醇治疗获得成功。该病例在更广泛的文献背景中进行了简要介绍。