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小儿角膜缘干细胞缺乏症:一种鲜有研究的病理学概述。

Pediatric limbal stem cell deficiency: An overview of a rarely studied pathology.

作者信息

Tutas Günaydın Nesrin, Barut Selver Ozlem

机构信息

Department of Ophtalmology, Istanbul Arel University, Bahçelievler Memorial Hospital, Istanbul, Turkey.

Department of Ophthalmology, School of Medicine, Ege University, Izmir, Turkey.

出版信息

Eur J Ophthalmol. 2025 May;35(3):821-833. doi: 10.1177/11206721241291995. Epub 2024 Oct 30.

DOI:10.1177/11206721241291995
PMID:39473431
Abstract

The cornea is the outermost transparent layer of the eye, and the continuous renewal of the corneal epithelium is important for its transparency. This process is primarily facilitated by corneal stem cells, most of which are found at the limbus. However, any deterioration or damage in this region leads to corneal conjunctivalization and consequent limbal stem cell deficiency (LSCD), which compromises corneal transparency. LSCD is an important condition, especially in the pediatric population, as it can lead to corneal vascularization, opacity and ultimately loss of vision and subsequent amblyopia, unlike adults. Although pediatric LSCD is often due to chemical injuries, as in adults, it is also caused by conditions such as congenital aniridia, Steven Johnson Syndrome and various other immunological disorders. Appropriate and timely treatment in pediatric LSCD is of particular importance in preventing progression to amblyopia, unlike adults. Accurate staging of the disease is essential for the necessary medical and surgical treatment decision. While medical approaches are at the forefront to eliminate the causative agent and improve the ocular surface in reversible cases, it is essential to replace the limbal stem cells lost in advanced disease. For these replacement procedures, it is noteworthy that there is a tendency for minimally invasive methods compared to adults to avoid possible complications due to long life expectancy in children. In conclusion, although there are various reviews on limbal stem cell deficiency (LSCD) in adults, we believe that this review for childhood LSCD will make an important contribution to the literature, since the relevant literature currently reported for the pediatric population is limited.

摘要

角膜是眼睛最外层的透明组织,角膜上皮的持续更新对其透明度至关重要。这一过程主要由角膜干细胞推动,其中大部分位于角膜缘。然而,该区域的任何恶化或损伤都会导致角膜结膜化以及随之而来的角膜缘干细胞缺乏症(LSCD),进而损害角膜透明度。LSCD是一种重要病症,尤其在儿童群体中,因为它会导致角膜血管化、混浊,最终导致视力丧失及随后的弱视,这与成人情况不同。尽管儿童LSCD通常像成人一样是由化学损伤引起,但也由先天性无虹膜、史蒂文斯-约翰逊综合征及其他各种免疫性疾病等情况导致。与成人不同,儿童LSCD的恰当及时治疗对于预防发展为弱视尤为重要。准确的疾病分期对于必要的药物和手术治疗决策至关重要。在可逆性病例中,药物治疗方法是消除病因和改善眼表的前沿手段,但在晚期疾病中,替换丢失的角膜缘干细胞至关重要。对于这些替换程序,值得注意的是,与成人相比,倾向于采用微创方法,以避免因儿童预期寿命长而可能出现的并发症。总之,尽管有关于成人角膜缘干细胞缺乏症(LSCD)的各种综述,但我们认为这篇关于儿童LSCD的综述将对文献做出重要贡献,因为目前报道的针对儿童群体的相关文献有限。

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