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[黏多糖贮积症Ⅰ型和 Lowe 综合征中的先天性青光眼。临床及电子显微镜检查结果]

[Congenital glaucoma in Hurler's syndrome and in Lowe's syndrome. Clinical and electron microscopy findings].

作者信息

Lalive d'Epinay S, Remé C E

出版信息

Adv Ophthalmol. 1978;36:80-9.

PMID:96681
Abstract

2 cases of congenital glaucoma combined with a general metabolic disease are described. 1 patient displayed the symptoms of a mucopolysaccharidosis (Hurler's syndrome), the other patient showed the characteristics of Lowe's oculo-cerebrorenal syndrome. In both patients a surgical trabeculectomy was performed. The excised tissues were analysed by electron microscopy. Distinct vacuolar or fibrillar deposits were observed in both cases in the trabecular region and in Schlemm's canal. Possibly, these alterations could have contributed to the raised intraocular pressure in both patients.

摘要

本文描述了2例先天性青光眼合并一种全身性代谢疾病的病例。1例患者表现出黏多糖贮积症(Hurler综合征)的症状,另1例患者表现出Lowe眼脑肾综合征的特征。两名患者均接受了小梁切除术。对切除的组织进行了电子显微镜分析。在两例患者的小梁区域和施莱姆管中均观察到明显的空泡状或纤维状沉积物。这些改变可能是导致两名患者眼压升高的原因。

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