系统性黏多糖贮积症I-S型中的急性青光眼

Acute glaucoma in systemic mucopolysaccharidosis I-S.

作者信息

Quigley H A, Maumenee A E, Stark W J

出版信息

Am J Ophthalmol. 1975 Jul;80(1):70-2. doi: 10.1016/0002-9394(75)90871-5.

Abstract

Two siblings, a 56-year-old white man and a 44-year-old white woman, with systemic mucopolysaccharidosis I-S (Scheie's syndrome) developed acute glaucoma successively in each eye. Their management was complicated by an inability to visualize the anterior chamber angle due to corneal clouding by acid mucopolysaccharide (AMP) deposition. The mechanism of their glaucoma resembled angle closure, which may have been related to thickening of anterior ocular structures due to abnormal AMP storage.

摘要

两名患有系统性黏多糖贮积症I-S型（谢伊综合征）的兄弟姐妹，分别是一名56岁的白人男性和一名44岁的白人女性，先后在每只眼睛中都患上了急性青光眼。由于酸性黏多糖（AMP）沉积导致角膜混浊，致使前房角无法看清，这给他们的治疗带来了复杂性。他们青光眼的发病机制类似于闭角型青光眼，这可能与异常的AMP蓄积导致眼前部结构增厚有关。