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

IF 1.4 4区 医学 Q3 OPHTHALMOLOGY
Nesrin Tutas Günaydın, Ozlem Barut Selver
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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.

小儿角膜缘干细胞缺乏症:罕见病理概述
角膜是眼睛最外面的透明层,角膜上皮的不断更新对角膜的透明度非常重要。这一过程主要由角膜干细胞促进,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,进而导致角膜缘干细胞缺乏症(LSCD),影响角膜透明度。角膜缘干细胞缺乏症是一种重要的疾病,尤其是在儿童群体中,因为它与成人不同,可导致角膜血管化、不透明,最终导致视力丧失,继而引发弱视。虽然小儿角膜缺损通常与成人一样是由化学损伤引起的,但也可由先天性无角膜症、史蒂文-约翰逊综合征和其他各种免疫性疾病引起。与成人不同,对小儿 LSCD 进行适当和及时的治疗对于防止其发展为弱视尤为重要。对疾病进行准确分期对于做出必要的药物和手术治疗决定至关重要。在可逆性病例中,药物治疗是消除致病因子和改善眼表的首要方法,而在晚期病例中,补充丧失的角膜缘干细胞则至关重要。值得注意的是,与成人相比,这些替代手术更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,尽管已有各种关于成人眼睑缘干细胞缺乏症(LSCD)的综述,但我们相信,由于目前针对儿科人群的相关文献报道有限,这篇关于儿童眼睑缘干细胞缺乏症的综述将为文献做出重要贡献。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
3.60
自引率
0.00%
发文量
372
审稿时长
3-8 weeks
期刊介绍: The European Journal of Ophthalmology was founded in 1991 and is issued in print bi-monthly. It publishes only peer-reviewed original research reporting clinical observations and laboratory investigations with clinical relevance focusing on new diagnostic and surgical techniques, instrument and therapy updates, results of clinical trials and research findings.
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