Spellacy E, Bankes J L, Crow J, Dourmashkin R, Shah D, Watts R W
Br J Ophthalmol. 1980 Oct;64(10):773-8. doi: 10.1136/bjo.64.10.773.
The electron microscopic appearances of the corneoscleral and iris tissue removed at operation from a child with Hurler disease and glaucoma showed distinctive swollen cells with intracellular inclusions similar to those which are observed in other tissues in these patients and which are due to abnormal lysosomal storages of mucopolysaccharides. Some recent observations on the possible relationship between mucopolysaccharides and the drainage of fluid from the anterior chamber are briefly reviewed and correlated with the present observations. The development of glaucoma in this patient is thought to be associated with the presence of the mucopolysaccharide-containing cells in the region of the aqueous drainage channels.
从一名患有Hurler病和青光眼的儿童手术中切除的角膜巩膜和虹膜组织的电子显微镜观察显示,细胞明显肿胀,细胞内有包涵体,类似于在这些患者其他组织中观察到的情况,这是由于粘多糖在溶酶体中异常蓄积所致。本文简要回顾了最近关于粘多糖与前房液体引流之间可能关系的一些观察结果,并将其与目前的观察结果相关联。该患者青光眼的发生被认为与房水引流通道区域中含粘多糖细胞的存在有关。