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诊断为 X 连锁性鱼鳞病患者的眼表面分析。

Ocular surface analysis in patients diagnosed with X-linked ichthyosis.

机构信息

UGC Oftalmología, Hospital Universitario Virgen Macarena, Sevilla, España.

UGC Oftalmología, Hospital Universitario Virgen Macarena, Sevilla, España; Departamento de Cirugía, Universidad de Sevilla, Sevilla, España; RETICS-OFTARED.

出版信息

Arch Soc Esp Oftalmol (Engl Ed). 2020 Nov;95(11):565-568. doi: 10.1016/j.oftal.2020.04.023. Epub 2020 Jul 11.

DOI:10.1016/j.oftal.2020.04.023
PMID:32660766
Abstract

Seven patients (14 eyes) diagnosed with X-linked ichthyosis were studied using the Schirmer test, biomicroscopy, tonometry, endothelial count, optical coherence tomography, Pentacam®, ocular surface analyser, and confocal microscopy. The mean age was 33.83±20.17 years (range: 7-64 years). The most frequent findings in biomicroscopy were Meibomian glands dysfunction (83.3%) and stromal corneal opacities (33%). The tear break-up time was found shortened in 25% of the eyes. Confocal microscopy (both eyes) revealed activated keratocytes with hyper-reflective particles inside them in the anterior stroma and outside them in the posterior stroma. It is believed that the inclusion of the use of confocal microscopy will help in a better understanding of the corneal pathology associated with ichthyosis X, as well as new characteristics of these patients.

摘要

对 7 名(14 只眼)被诊断为 X 连锁鱼鳞癣的患者进行了 Schirmer 测试、生物显微镜检查、眼压测量、内皮细胞计数、光学相干断层扫描、Pentacam®、眼表面分析器和共焦显微镜检查。平均年龄为 33.83±20.17 岁(范围:7-64 岁)。生物显微镜检查最常见的发现是睑板腺功能障碍(83.3%)和基质角膜混浊(33%)。发现 25%的眼的泪膜破裂时间缩短。共焦显微镜(双眼)在前基质中发现了被激活的角膜细胞,其中有高反射颗粒,在后基质中发现了这些颗粒的外部。人们相信,共焦显微镜的使用将有助于更好地理解与 X 连锁鱼鳞癣相关的角膜病理学,以及这些患者的新特征。

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引用本文的文献

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Advanced Anterior Eye Segment Imaging for Ichthyosis.用于鱼鳞病的先进眼前节成像
J Clin Med. 2023 Sep 16;12(18):6006. doi: 10.3390/jcm12186006.
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Identification of a novel partial deletion of associated with pre-Descemet corneal dystrophy and X-linked ichthyosis.鉴定一种新型与前弹力层角膜营养不良和 X 连锁鱼鳞癣相关的 部分缺失。
Mol Vis. 2023 Apr 29;29:25-30. eCollection 2023.