加深对木质结膜炎的认识:桥接性发病机制、诊断和治疗。

IF 2.1 3区 医学 Q2 OPHTHALMOLOGY
Talia N Shoshany, Andrew Thomson, Amy Shapiro, Amol Sura, Charles Nakar, Henry D Perry
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引用次数: 0

摘要

目的:本文回顾并报道木质性结膜炎(LC),一种由1型纤溶酶原缺乏症(PLGD-1)引起的罕见眼部疾病,表现为眼睑结膜上的木样富纤维蛋白膜。目的是为眼科医生(通常是第一个遇到这种情况的医生)提供对其系统性表现的全面了解,并强调当前的治疗策略,特别是静脉注射纤溶酶原浓缩物。方法:我们提出了一个临床LC病例,并对已发表的病例、病因和治疗方法进行了叙述回顾。结果:LC最初表现为眼睑结膜红斑病变,最终发展为纤维样木膜。它通常与PLGD-1相关,PLGD-1是一种纤溶酶原基因(PLG)的遗传性疾病,导致纤维蛋白溶解受损和纤维蛋白沉积的形成。LC通常出现在生命的第一年,但患者在诊断前可能会经历一段较长的时间。PLGD-1也与许多其他器官系统粘膜病变的发生有关。诱发因素包括慢性炎症、过敏原、感染和创伤。目前已经提出了许多治疗LC的方法,但最近批准的静脉血浆源性人纤溶酶原浓缩物替代是一个重要的进展。结论:LC是一种眼部疾病,具有潜在的视力和危及生命的并发症。信用证的处理历来具有挑战性;然而,认识到其与PLGD-1的关联已导致更有效的治疗,特别是静脉注射浓缩纤溶酶原。眼科医生和其他专家(如血液科医生)之间的早期诊断和协调治疗对于预防并发症(包括视力丧失)至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Advancing Understanding of Ligneous Conjunctivitis: Bridging Pathogenesis, Diagnosis, and Therapy.

Purpose: This review and case report address ligneous conjunctivitis (LC), a rare ocular condition caused by plasminogen deficiency type 1 (PLGD-1), which manifests as wood-like fibrin-rich membranes on the palpebral conjunctiva. The goal is to provide ophthalmologists-often the first physicians to encounter the condition-with a robust understanding of its systemic manifestations and to highlight current therapeutic strategies, with particular emphasis on the administration of intravenous plasminogen concentrate.

Methods: We present a clinical LC case alongside a narrative review of published cases, etiology, and treatment approaches.

Results: LC initially manifests as erythematous lesions on the palpebral conjunctiva, eventually developing into fibrinous, wood-like membranes. It is typically associated with PLGD-1, a genetic disorder of the plasminogen gene (PLG), which results in impaired fibrinolysis and the formation of fibrin deposits. LC often appears within the first year of life, but patients may experience a prolonged period before diagnosis. PLGD-1 is also associated with the development of mucosal lesions in many other organ systems. Contributing factors include chronic inflammation, allergens, infections, and trauma. There have been many proposed treatment approaches for LC, but the recent approval of intravenous plasma-derived human plasminogen concentrate replacement has been an important advance.

Conclusions: LC is an ocular disorder with potential vision- and life-threatening complications. The treatment of LC has historically been challenging; however, recognition of its association with PLGD-1 has led to more effective therapies, particularly intravenous plasminogen concentrate. Early diagnosis and coordinated care between ophthalmologists and other specialists, such as hematologists, are crucial to prevent complications, including vision loss.

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来源期刊
Cornea
Cornea 医学-眼科学
CiteScore
5.20
自引率
10.70%
发文量
354
审稿时长
3-6 weeks
期刊介绍: For corneal specialists and for all general ophthalmologists with an interest in this exciting subspecialty, Cornea brings together the latest clinical and basic research on the cornea and the anterior segment of the eye. Each volume is peer-reviewed by Cornea''s board of world-renowned experts and fully indexed in archival format. Your subscription brings you the latest developments in your field and a growing library of valuable professional references. Sponsored by The Cornea Society which was founded as the Castroviejo Cornea Society in 1975.
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