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与原发性肾上腺皮质功能不全相关的角膜缘干细胞缺乏症。

Limbal stem cell defficiency associated with primary adrenocortical insufficiency.

作者信息

Örnek Nurgül, Örnek Kemal, Ogurel Tevfik

机构信息

Department of Ophthalmology, School of Medicine, Kırıkkale University, Yahşihan, Turkey.

Department of Ophthalmology, Kudret Eye Hospital, Ankara, Turkey.

出版信息

GMS Ophthalmol Cases. 2019 Feb 6;9:Doc02. doi: 10.3205/oc000091. eCollection 2019.

DOI:10.3205/oc000091
PMID:30828512
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6381395/
Abstract

To report a female patient with bilateral limbal stem cell deficiency (LSCD) due to primary adrenocortical insufficiency (PAI). Case report A 40-year-old female patient had blurry vision, foreign body sensation, tearing, and photophobia for several years. On examination, corneal epithelial haze, surface irregularity, and superficial neovascularization were observed. There was a dull and irregular reflex from the conjunctivalized corneal surface. Medical history revealed that she had a diagnosis of PAI for 11 years and received hormone replacement (fludrocortisone acetate) therapy. With the clinical presentation and examination, the diagnosis was compatible with LSCD. Frequent ocular lubricant and topical steroid drops were initially started and topical cyclosporine treatment was planned for the long term. After 3 weeks, there was no corneal superficial neovascularization and epithelial haze, peripheral stromal haze was still observed. LSCD may rarely be associated with PAI. In patients with LSCD, systemic evaluation should be made to rule out PAI.

摘要

报告一例因原发性肾上腺皮质功能不全(PAI)导致双侧角膜缘干细胞缺乏(LSCD)的女性患者。病例报告 一名40岁女性患者数年来一直有视力模糊、异物感、流泪和畏光症状。检查发现角膜上皮混浊、表面不规则以及浅层新生血管形成。结膜化的角膜表面反射暗淡且不规则。病史显示她已确诊PAI 11年,并接受了激素替代(醋酸氟氢可的松)治疗。结合临床表现和检查,诊断为LSCD。最初开始频繁使用眼部润滑剂和局部类固醇滴眼液,并计划长期使用局部环孢素治疗。3周后,角膜浅层新生血管形成和上皮混浊消失,但仍观察到周边基质混浊。LSCD可能很少与PAI相关。对于LSCD患者,应进行全身评估以排除PAI。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3606/6381395/462172490ca9/OC-09-02-g-002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3606/6381395/3ce85a6f19e9/OC-09-02-g-001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3606/6381395/462172490ca9/OC-09-02-g-002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3606/6381395/3ce85a6f19e9/OC-09-02-g-001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3606/6381395/462172490ca9/OC-09-02-g-002.jpg

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The diagnosis of limbal stem cell deficiency.角膜缘干细胞缺乏的诊断。
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2
Primary Adrenocortical Insufficiency Case Series: Genetic Etiologies More Common than Expected.原发性肾上腺皮质功能不全病例系列:遗传病因比预期更常见。
Horm Res Paediatr. 2016;85(1):35-42. doi: 10.1159/000441843. Epub 2015 Dec 10.
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Early-onset hypoparathyroidism and chronic keratitis revealing APECED.早发性甲状旁腺功能减退症和慢性角膜炎揭示了自身免疫性多内分泌腺病-念珠菌病-外胚层营养不良综合征
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