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Atrofia girata de la coroides y la retina: actualización en diagnóstico y tratamiento 脉络膜和视网膜回旋体萎缩:诊断和治疗的最新进展
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.03.006

Gyrate atrophy of the choroid and retina (GACR) is a rare autosomal recessive disease characterised by elevated plasma ornithine levels due to deficiency of the enzyme ornithine aminotransferase (OAT). The accumulation of this amino acid in plasma leads to the development of patches of chorioretinal atrophy in the peripheral retina extending into the macular area. Patients usually present with night blindness followed by constriction of the visual field and, finally, decreased central vision and blindness. The disease is diagnosed by the presence of the characteristic clinical picture, the presence of hyperornithinaemia in plasma and the detection of mutations in the OAT enzyme gene. There is currently no effective gene therapy and the most common therapeutic intervention mainly involves dietary modifications with arginine restriction.

This article aims to summarise the pathogenesis, clinical and diagnostic findings and treatment options in patients with GACR.

脉络膜和视网膜回旋萎缩症(GACR)是一种罕见的常染色体隐性遗传病,由于缺乏鸟氨酸氨基转移酶(OAT),导致血浆鸟氨酸水平升高。这种氨基酸在血浆中的积累会导致周边视网膜上出现延伸至黄斑区的脉络膜视网膜萎缩斑。患者通常表现为夜盲,随后视野缩小,最后中心视力下降并失明。该病的诊断依据是特征性临床表现、血浆中出现高鸟氨酸血症以及 OAT 酶基因突变。本文旨在总结 GACR 患者的发病机制、临床和诊断结果以及治疗方案。
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引用次数: 0
El «ictus de la retina»: un llamado a la acción para la conciencia y el tratamiento de las emergencias vasculares oculares 视网膜中风":认识和治疗眼部血管急症的行动号召
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.03.009
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引用次数: 0
Mujer vigoréxica con pérdida severa bilateral de visión 双侧视力严重下降的活力缺失妇女
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.04.008

We describe a 28-year-old Caucasian female with vigorexy, who had no previous ocular history. She presented with bilateral gradual painless reduction in vision over the past 3 weeks. She had been taking niacin supplements, averaging 500 mg daily, for 7 years. Fundus examination revealed bilateral CME, which was confirmed by ocular coherence tomography scan. Fundus fluorescein angiography did not reveal any fluid leakage. Niacin supplementation was discontinued, and after 2 months, the CME had completely resolved, and the best corrected visual acuities improved to 1 in both eyes.

我们描述了一名 28 岁的白种女性,她患有眩晕症,以前没有眼科病史。她在过去 3 周内出现双侧视力逐渐下降,且无疼痛感。7 年来,她一直服用烟酸补充剂,平均每天 500 毫克。眼底检查显示双侧均有 CME,眼相干断层扫描证实了这一点。眼底荧光素血管造影检查未发现任何液体渗漏。停止补充烟酸两个月后,CME完全消退,双眼最佳矫正视力提高到1。
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引用次数: 0
Bancos de ojos públicos de Andalucía: una década de mejora continua 安达卢西亚公共眼科银行:十年持续改进
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.05.002
M. Rodríguez-Calvo-de-Mora , J. Giménez-Almenara-Amo , B. Mataix-Albert , M. Arias-Alcalá , J. García-Montesinos-Gutiérrez , C. Rocha-de-Lossada , R. Villalba-Montoro , M.S. Díaz-Gallardo , N. Cuende , A. Álvarez-Márquez

Objectives

To show donation data, number of keratoplasties and the changes in transplant indications and techniques that occurred in Andalusia in the period from 2013 to 2022.

Materials and methods

The present work is a retrospective and descriptive study that included all keratoplasties performed between January 2013 and December 2022 in Andalusia, as well as the evolution of the cornea donation and transplant activity of the public and private hospitals pertaining to the waiting list management system of the Public Health System of Andalusia. Transplants performed in private centers with corneas from outside Andalusia were excluded.

Results

Cornea donation activity in Andalusia in the decade 2013-2022 has experienced a growth of more than 126%, while overall transplant activity has increased by 157% in public hospitals. Penetrating keratoplasty has decreased from 83% in 2013 to 43% in 2022, while lamellar techniques have increased from 17% to 57% in this same period. Since 2018, more lamellar transplants are performed than penetrating transplants. Regarding indications, endothelial conditions already represent the first cause of transplantation. In 2022 alone, the public Andalusian Eye Banks evaluated 1,054 corneas and prepared 281 endothelial grafts.

Conclusion

In the decade from 2013 to 2022 in Andalusia there has been an increase in donation activity and the number of keratoplasties. The public Eye Banks implementation in this period has played a key role in the widespread adoption of lamellar keratoplasty techniques and has enabled the transition to perform a greater number of lamellar keratoplasties compared to penetrating keratoplasty.

材料和方法本研究是一项回顾性和描述性研究,包括 2013 年 1 月至 2022 年 12 月在安达卢西亚进行的所有角膜移植手术,以及安达卢西亚公共卫生系统候选名单管理系统中公立和私立医院角膜捐赠和移植活动的演变情况。结果 2013-2022 年间,安达卢西亚的角膜捐献活动增长了 126% 以上,而公立医院的整体移植活动增长了 157%。穿透性角膜移植术从 2013 年的 83% 下降到 2022 年的 43%,而同期板层技术从 17% 增加到 57%。自2018年以来,板层移植手术的数量多于穿透性移植手术。在适应症方面,内皮病变已经成为移植手术的首要原因。仅在 2022 年,安达卢西亚公共眼库就评估了 1,054 例角膜,并准备了 281 例内皮移植。在此期间,公共眼库在广泛采用板层角膜移植技术方面发挥了关键作用,与穿透性角膜移植术相比,板层角膜移植术的数量增加了。
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引用次数: 0
Experiencia con el uso de bevacizumab intravítreo en el manejo de la retinopatía del prematuro tipo I en un país latinoamericano 拉丁美洲某国使用玻璃体内贝伐单抗治疗 I 型早产儿视网膜病变的经验
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.03.012

In retinopathy of prematurity (ROP) type I, the use of intravitreal bevacizumab (IVB), which is an inhibitor of endothelial growth factor (VEGF), has become popular despite not being a therapy approved by regulatory agencies. However, IVB has shown positive effects in halting disease progression at lower costs compared to other anti-VEGF therapies (ranibizumab or aflibercept). In this report, we present the experience during the treatment with IVB of 102 Colombian children with ROP type I, with a success rate of 98% (100). Complications occurred in 3.9% (4). Finally, we conclude that a single dose of IVB is an effective therapy for the management of ROP type I, with a lower risk of complications and retreatment.

在 I 型早产儿视网膜病变(ROP)中,玻璃体内贝伐单抗(IVB)是一种内皮生长因子(VEGF)抑制剂,尽管尚未获得监管机构的批准,但它的使用已变得非常普遍。然而,与其他抗血管内皮生长因子疗法(ranibizumab 或 aflibercept)相比,IVB 在阻止疾病进展方面已显示出积极的效果,而且成本更低。在本报告中,我们介绍了对 102 名哥伦比亚 I 型视网膜病变患儿进行 IVB 治疗的经验,成功率为 98%(100 例)。并发症发生率为 3.9%(4 例)。最后,我们得出结论:单剂量 IVB 是治疗 I 型视网膜病变的有效疗法,并发症和再治疗的风险较低。
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引用次数: 0
Características del desprendimiento de retina pseudofáquico y factores de riesgo de recidiva 假性视网膜脱离的特征和复发的风险因素
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.04.012

Background

The present study was designed to gain knowledge about the prognostic factors and evolution of pseudophakic retinal detachment (PRD) and to analyse the main characteristics of PRD, such as the mean time to retinal detachment following cataract surgery, as well as the clinical factors associated with the risk of recurrence and worse anatomical and functional outcomes.

Methods

This was a retrospective monocentric study of 330 patients with PRD who underwent surgery between 2012 and 2020. All patients were pseudophakic and were referred for retinal detachment surgery at Donostia University Hospital (Terciary Hospital, Spain).

Results

The mean age of the patients at the time of phacoemulsification was 63.06 ± 10.8 years, and 49.09% of them had moderate myopia (axial length [AL] [23.5-26.5 mm]). In our series, macular detachment was evident in 69.09% (n = 228) of the patients. The mean time that elapsed between phacoemulsification and PRD surgery was 4.04 ± 4.17 years, which was reduced by half in the event of surgical complications (2.24 ± 2.65). The rate of PRD was 28.79%. The anatomical results in terms of retinal reapplication were better with the combination of vitrectomy and scleral buckling (83.1% vs. 70.5%) (p = 0,127) compared with vitrectomy. The variables associated with a higher risk of recurrence were male sex (hazard ratio [HR]: 1.75), macular detachment (HR: 2.00) and the presence of proliferative vitreoretinopathy (PVR) (HR: 3.21).

Conclusions

PRD usually occurs within 4 years of phacoemulsification, although it may occur significantly earlier in the event of surgical complications. Macular involvement, PVR, and male sex are all associated with a higher risk of PRD recurrence.

背景本研究旨在了解假性视网膜脱离(PRD)的预后因素和演变情况,分析PRD的主要特征,如白内障手术后视网膜脱离的平均时间,以及与复发风险和更差的解剖和功能结果相关的临床因素。结果患者接受乳化手术时的平均年龄为(63.06 ± 10.8)岁,49.09%的患者患有中度近视(轴向长度 [AL] [23.5-26.5 mm])。在我们的系列研究中,69.09%的患者(n = 228)有明显的黄斑脱离。从超声乳化到黄斑脱离手术的平均间隔时间为 4.04 ± 4.17 年,如果出现手术并发症,间隔时间会缩短一半(2.24 ± 2.65)。PRD 率为 28.79%。与玻璃体切除术相比,联合使用玻璃体切除术和巩膜扣带术后视网膜重贴的解剖效果更好(83.1% 对 70.5%)(p = 0,127)。与复发风险较高相关的变量是男性(危险比 [HR]:1.75)、黄斑脱离(HR:2.00)和存在增殖性玻璃体视网膜病变(PVR)(HR:3.21)。黄斑受累、PVR 和男性性别都与 PRD 复发风险较高有关。
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引用次数: 0
Formación en cirugía de cataratas en España: análisis de resultados de una encuesta del European Board of Ophthalmology en una cohorte española 西班牙的白内障手术培训:欧洲眼科委员会对西班牙队列的调查结果分析
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.05.001

Introduction

A survey conducted by the European Board of Ophthalmology (EBO) revealed significant differences in the surgical training of the ophthalmology residents in Europe, including a disparity between the sexes and a variation in the experience on cataract surgery (CC) between them. This study is about the Spanish sub-cohort of the survey, and its objective is to present and analyse the peculiarities of ophthalmology training in Spain within the European context, as well as discussing ways to harmonise and improve that training throughout the EU.

Methods

We analyse data of the Spanish participants in the EBO exams, defining subgroups by the Autonomous Communities existing in Spain.

Results

93 of 135 requested participants (68.9%) responded. A 60.2% passed the EBO exam between 2021-2022, being mostly women (65.59%) aged 31 years old on average. The 91.4% were right-handed, coming from 13 of the 17 Spanish autonomous communities, although mostly from the Community of Valencia, Madrid and Catalonia. Respectively, 16.1%, 3.2% and 8.7% of the respondents said they have completed 10 or more training sessions on animal eyes, synthetic eyes and through the virtual reality simulator. This training was correlated with greater self-confidence in the management of a posterior capsular tear during surgery (p 0.025). All respondents manifested to have already performed stages of the CC. The average number of operations reported was 181.6 with regional disparities. A significant difference is observed between the sexes against women (-28.3%, p 0.03).

Discussion

Ophthalmologists in Spain, much more than other European countries, have greater opportunities for surgical training, with surgical procedures during the residency, that nearly triples those made by the others. Spanish women refer, like their European colleagues, to be in disadvantage in learning opportunities about cataract surgery. The Simulation Based Medical Education (SBME) allows to respond to the training deficit and complements the training on the patient. Although we demonstrate a significant correlation between the number of procedures carried out and self-confidence to operate simple cases, the SBME would be a complementary tool in self-confidence in front of a complication like capsular rupture.

Conclusion

Spain massively adopts the model named by us “surgery for all”, despite the underrepresentation of women in this area, emphasising a need for cultural change that the SBME could facilitate.

引言 欧洲眼科委员会(EBO)进行的一项调查显示,欧洲眼科住院医生的手术培训存在显著差异,其中包括性别差异和白内障手术(CC)经验差异。本研究的对象是该调查的西班牙子群,其目的是在欧洲范围内介绍和分析西班牙眼科培训的特殊性,并讨论如何在整个欧盟范围内协调和改善眼科培训。60.2%的人通过了 2021-2022 年间的 EBO 考试,其中大部分为女性(65.59%),平均年龄为 31 岁。91.4%的人是右撇子,来自西班牙 17 个自治区中的 13 个,但大部分来自巴伦西亚、马德里和加泰罗尼亚自治区。分别有 16.1%、3.2% 和 8.7% 的受访者表示,他们已经完成了 10 次或 10 次以上的动物眼睛、合成眼睛和虚拟现实模拟器培训。这种培训与在手术过程中处理后囊撕裂的自信心有关(P 0.025)。所有受访者都表示已经进行过几期 CC 手术。报告的平均手术次数为 181.6 次,各地区之间存在差异。讨论西班牙的眼科医生比其他欧洲国家的眼科医生有更多的机会接受手术培训,他们在实习期间的手术次数几乎是其他国家的三倍。西班牙女性眼科医生与欧洲女性眼科医生一样,在学习白内障手术方面处于劣势。模拟医学教育(SBME)可以解决培训不足的问题,并对患者培训起到补充作用。尽管我们证明了已实施手术的数量与操作简单病例的自信心之间存在着明显的相关性,但模拟医学教育将是一种补充工具,可增强患者在囊膜破裂等并发症面前的自信心。结论西班牙大规模采用了我们命名为 "全民手术 "的模式,尽管女性在这一领域的代表性不足,但这强调了文化变革的必要性,而模拟医学教育可促进文化变革。
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引用次数: 0
Histiocitosis de células de Langerhans que simula una dacriocistitis aguda 模仿急性泪囊炎的朗格汉斯细胞组织细胞增生症
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.04.004

Langerhans cell histiocytosis is a myeloid neoplasm characterized by clonal neoplastic proliferation of Langerhans-type dendritic cells associated with an inflammatory infiltrate predominantly composed of lymphocytes and eosinophils. In this article, we present an unusual case of Langerhans cell histiocytosis with significant swelling in the left lacrimal sac region in a 3-year-old child, clinically mimicking acute dacryocystitis. Microscopically, it showed intense inflammatory infiltrate and histiocytes with irregular nuclei. The tumor cells were positive for S-100 protein, CD1a, and CD207 (langerin). Molecular study was positive for the V600E/E2/D mutation (exon 15). This case emphasizes the importance of careful clinical, radiographic, and microscopic evaluation, as some neoplasms may mimic common benign lesions.

朗格汉斯细胞组织细胞增生症是一种髓系肿瘤,其特点是朗格汉斯型树突状细胞克隆性增生,并伴有主要由淋巴细胞和嗜酸性粒细胞组成的炎性浸润。本文介绍了一例罕见的朗格汉斯细胞组织细胞增生症病例,患者为一名 3 岁儿童,左侧泪囊区肿胀明显,临床症状类似急性泪囊炎。显微镜下,病变部位有强烈的炎症浸润和核不规则的组织细胞。肿瘤细胞的 S-100 蛋白、CD1a 和 CD207(langerin)均呈阳性。分子研究显示,V600E/E2/D 突变(外显子 15)呈阳性。该病例强调了仔细进行临床、影像学和显微镜评估的重要性,因为有些肿瘤可能会模仿常见的良性病变。
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引用次数: 0
Hemorragias subretinianas con afectación subfoveal tras traumatismo ocular contuso, en paciente con pseudoxantoma elástico y estrías angioides 一名患有假黄瘤和血管条纹的患者在眼部钝性外伤后出现视网膜下出血并累及眼底。
Q3 Medicine Pub Date : 2024-09-01 DOI: 10.1016/j.oftal.2024.05.010
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引用次数: 0
Estrategias farmacológicas para la corrección de la presbicia 矫正老花眼的药理策略
Q3 Medicine Pub Date : 2024-08-01 DOI: 10.1016/j.oftal.2024.03.005

Presbyopia affects between 1.7 and 2 billion people worldwide. Presbyopia significantly impacts productivity and quality of life in both developed and developing countries. During accommodation, the human eye changes its dioptric power by altering the shape of the lens, but the exact nature of this change has not been fully explained. Recently, topical treatments have been marketed for the treatment of presbyopia and others are under investigation.

In order to prepare a review of these novel therapies, we searched the major biomedical search engines. We found 15 randomized clinical trials and 12 reviews that met our review criteria. There are 2 different strategies for this purpose: the pinhole effect that increases depth of focus and “crystalline lens relaxation” for which parasympathetic-mimetics and lens oxidation intermediates have been used. The results are generally favorable in terms of improvement of near visual acuity, although the follow-up period of the studies is short. These are novel strategies in the early stages of research that could be useful in the treatment of presbyopia.

全世界有 17 亿至 20 亿人受到老花眼的影响。老花眼严重影响了发达国家和发展中国家的生产力和生活质量。在调节过程中,人眼会通过改变晶状体的形状来改变其屈光度,但这种变化的确切性质尚未得到充分解释。最近,市场上出现了治疗老花眼的外用疗法,其他疗法也在研究之中。为了编写这些新型疗法的综述,我们搜索了主要的生物医学搜索引擎。我们发现有 15 项随机临床试验和 12 篇综述符合我们的综述标准。为此,有两种不同的策略:增加焦距深度的针孔效应和 "晶状体松弛",其中 "晶状体松弛 "使用了副交感神经模拟物和晶状体氧化中间产物。虽然这些研究的随访时间较短,但从改善近视力的角度来看,结果普遍良好。这些都是处于早期研究阶段的新策略,可用于治疗老花眼。
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引用次数: 0
期刊
Archivos De La Sociedad Espanola De Oftalmologia
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