Fuchs’ endothelial corneal dystrophy

Fuchs 内皮角膜营养不良
  • 文章类型: Journal Article
    (1)背景:Fuchs内皮角膜营养不良(FECD)患者可能合并白内障,因此,可能需要白内障手术,由于潜在的内皮细胞损伤,这带来了挑战。FECD是一种病因不明的退行性眼病,炎性细胞因子可能在其发生发展中起重要作用。本研究旨在研究患有白内障的FECD眼房水中的细胞因子谱。(2)方法:52例患者纳入研究,26以FECD+白内障和26以白内障为对照组。使用Bio-Plex200系统分析房水样品的促炎和抗炎细胞因子。(3)结果:与对照组/白内障组相比,FECD+白内障患者房水白细胞介素1受体拮抗剂(IL-1Ra)和白细胞介素IL-8水平明显增高。此外,与对照组相比,FECD+白内障组的抗炎IL-10水平呈较高趋势.相比之下,IL-1β无统计学差异,IL-6、IL-4、IL-10、IL-13、IL-17A、各组间肿瘤坏死因子TNF-α。(4)结论:本研究有助于更好地理解FECD的发病机制。IL-1Ra和IL-8水平的升高可能是患有FECD和共存白内障的人的防御机制。
    (1) Background: Patients with Fuchs\' endothelial corneal dystrophy (FECD) may have coexisting cataracts and, therefore, may require a cataract surgery, which poses challenges due to potential endothelial cell damage. FECD is a degenerative eye disease of unclear etiology, with inflammatory cytokines maybe playing an important role in its development and progression. The present study aimed to investigate the cytokine profile in the aqueous humor of FECD eyes with cataract. (2) Methods: Fifty-two patients were included in the study, 26 with FECD + cataract and 26 with cataract as a control group. Samples of the aqueous humor were analyzed for pro- and anti-inflammatory cytokines using a Bio-Plex 200 system. (3) Results: Interleukin 1 receptor antagonist (IL-1Ra) and interleukin IL-8 levels were significantly higher in the aqueous humor of FECD + cataract patients compared to the control/cataract group. Moreover, the levels of anti-inflammatory IL-10 showed a strong trend to be higher in the FECD + cataract group compared to the control group. In contrast, there were no statistically significant differences in IL-1β, IL-6, IL-4, IL-10, IL-13, IL-17A, and tumor necrosis factor TNF-α between the groups. (4) Conclusions: Presented research contributes to a better understanding of FECD pathogenesis. Elevated levels of IL-1Ra and IL-8 may serve as a defense mechanism in people with FECD and coexisting cataract.
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  • 文章类型: English Abstract
    OBJECTIVE: This study evaluates the long-term results of surgical treatment of patients with Fuchs\' endothelial corneal dystrophy and cataract.
    METHODS: The study included 24 patients (24 eyes) with primary Fuchs\' endothelial corneal dystrophy and cataract, who underwent cataract phacoemulsification with IOL implantation and of Descemet\'s membrane endothelial keratoplasty with a semicircular graft (hemi-DMEK). The effect of treatment was assessed by best corrected visual acuity (BCVA), central corneal thickness (CCT) and endothelial cell density (ECD).
    RESULTS: In total, surgical treatment involved 14 donor corneas that were divided in half during the preparation and isolation of the Descemet\'s membrane (DM). By month 12 after the surgery an increase in visual functions and graft transparency were observed in 23 patients (23 eyes) out of 24. Repeated keratoplasty was required in one case due to fibrosis of the posterior layers of recipient\'s corneal stroma. At 12 months postoperatively, the study group showed an increase in BCVA from 0.16±0.1 to 0.75±20, a decrease in CCT from 650.9±4.5 μm to 519.6±43.9, and a decreased in ECD from 2850.5±84.7 cells/mm2 up to 1285.5±277.2 cells/mm2. Thus, the loss of endothelial cells at one year after surgery amounted to 54.9%.
    CONCLUSIONS: The developed method for transplantation of a semicircular DM fragment provides a tissue-saving approach to endothelial keratoplasty, and considering the high percentage of transparent engraftment of grafts and complete visual rehabilitation, it can be recommended in the treatment of patients with cataract and Fuchs\' endothelial corneal dystrophy.
    UNASSIGNED: Провести анализ отдаленных клинико-функциональных результатов хирургического лечения пациентов с первичной эндотелиальной дистрофией роговицы Фукса и катарактой.
    UNASSIGNED: В исследование включены 24 пациента (24 глаза) с первичной эндотелиальной дистрофией роговицы Фукса, сочетанной с катарактой, прооперированных методом одномоментной факоэмульсификации катаракты с имплантацией интраокулярной линзы и трансплантацией полукруглого фрагмента десцеметовой мембраны (ДМ) с эндотелием. Для изучения эффективности представленной методики оценивали показатели максимально корригируемой остроты зрения (МКОЗ), центральной толщины роговицы (ЦТР) и плотности эндотелиальных клеток (ПЭК).
    UNASSIGNED: Для хирургического лечения использовали 14 донорских корнеосклеральных дисков, которые в ходе подготовки и выделения ДМ были разделены пополам. К 12-му месяцу наблюдения после операции прозрачное приживление трансплантата и повышение зрительных функций наблюдалось у 23 пациентов (23 глаза) из 24. В 1 случае в связи с фиброзом задних слоев стромы роговицы потребовалась повторная кератопластика. Показатель МКОЗ к 12-му месяцу послеоперационного наблюдения в исследуемой группе увеличился с предоперационных значений, составлявших 0,16±0,1, до 0,75±20. К 12-му месяцу после операции показатель ЦТР уменьшился с исходных 650,9±44,5 до 519,6±43,9 мкм, а ПЭК — с исходных 2850,5±84,7 до 1285,5±277,2 кл/мм2. Таким образом, потеря эндотелиальных клеток за 1-й год наблюдения составила 54,9%.
    UNASSIGNED: Учитывая высокий процент прозрачного приживления трансплантатов и полноценную зрительную реабилитацию, разработанный метод трансплантации фрагмента ДМ, обеспечивший тканесберегающий подход к эндотелиальной кератопластике, может быть рекомендован как операция выбора при лечении пациентов с эндотелиальной дистрофией роговицы Фукса и катарактой.
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  • 文章类型: Journal Article
    目的:本综述将总结FECD相关基因和病理生理学,诊断,目前的治疗方法,以及未来的治疗前景。
    方法:文献综述。
    结果:Fuchs'内皮角膜营养不良(FECD)是最常见的双侧角膜营养不良,占美国所有角膜移植的三分之一。FECD是由遗传因素和非遗传因素共同引起的,有两种类型:早发性FECD,从早期开始影响个体,通常更严重,和迟发性FECD,这更常见,通常表现在40岁左右。FECD的标志发现包括角膜内皮细胞的进行性丧失和在Descemet膜上形成局灶性增生(guttae)。这些病理生理变化导致进行性内皮功能障碍,导致后期视力下降和失明。本文将对FECD相关基因及其病理生理学进行综述,诊断,目前的治疗方法,以及未来的治疗前景。
    结论:随着对FECD相关基因的表征和理解以及对角膜内皮再生疗法的持续研究,我们希望将来在疾病的管理和护理方面看到更多的重大改进。
    OBJECTIVE: The present review will summarize FECD-associated genes and pathophysiology, diagnosis, current  therapeutic approaches, and future treatment perspectives.
    METHODS: Literature review.
    RESULTS: Fuchs\' endothelial corneal dystrophy (FECD) is the most common bilateral corneal dystrophy and accounts for one-third of all corneal transplants performed in the US. FECD is caused by a combination of genetic and non-heritable factors, and there are two types: early-onset FECD, which affects individuals from an early age and is usually more severe, and late-onset FECD, which is more common and typically manifests around the age of 40. The hallmark findings of FECD include progressive loss of corneal endothelial cells and the formation of focal excrescences (guttae) on the Descemet membrane. These pathophysiological changes result in progressive endothelial dysfunction, leading to a decrease in visual acuity and blindness in later stages. The present review will summarize FECD-associated genes and pathophysiology, diagnosis, current therapeutic approaches, and future treatment perspectives.
    CONCLUSIONS: With the characterization and understanding of FECD-related genes and ongoing research into regenerative therapies for corneal endothelium, we can hope to see more significant improvements in the future in the management and care of the disease.
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  • 文章类型: Journal Article
    角膜,作为眼睛的最外层,通过将光线聚焦到视网膜上,在视觉中起着至关重要的作用。各种疾病和伤害会损害其清晰度,导致视力受损。这篇综述旨在全面概述其药理特性,Rho相关蛋白激酶(ROCK)抑制剂在角膜疾病治疗中的治疗潜力和相关风险。本文重点介绍了四种关键的ROCK抑制剂:Y-27632,法舒地尔,里帕苏地尔,和netarsudil,提供比较检查。支持使用ROCK抑制剂的研究强调了它们在不同角膜病症中的功效。在Fuchs角膜内皮营养不良中,Y-27632、利帕舒地尔的应用研究,netarsudil在角膜透明度方面表现出显著的增强,内皮细胞密度,和视力。在假晶状体大疱性角膜病变中,将Y-27632与培养的角膜内皮细胞一起注射入前房导致角膜内皮细胞密度增加和视力改善.模拟角膜化学损伤的动物模型显示,应用法舒地尔后,新生血管形成和上皮缺损减少,在虹膜角膜内皮综合征的情况下,netarsudil改善了角膜水肿。解决安全问题,netarsudil和ripasudil,都是临床批准的,表现出不良事件,如结膜充血,结膜出血,角膜,结膜炎,和眼睑炎。在治疗期间监测患者对于平衡潜在的治疗益处与这些相关的风险至关重要。总之,岩石抑制剂,尤其是netarsudil和ripasudil,承诺管理角膜疾病。对其药理特性的比较分析和支持其功效的研究强调了其潜在的治疗意义。然而,正在进行的研究对于全面了解其在各种角膜疾病中的安全性和长期结果至关重要,指导其在临床实践中的最佳应用。
    The cornea, as the outermost layer of the eye, plays a crucial role in vision by focusing light onto the retina. Various diseases and injuries can compromise its clarity, leading to impaired vision. This review aims to provide a thorough overview of the pharmacological properties, therapeutic potential and associated risks of Rho-associated protein kinase (ROCK) inhibitors in the management of corneal diseases. The article focuses on four key ROCK inhibitors: Y-27632, fasudil, ripasudil, and netarsudil, providing a comparative examination. Studies supporting the use of ROCK inhibitors highlight their efficacy across diverse corneal conditions. In Fuchs\' endothelial corneal dystrophy, studies on the application of Y-27632, ripasudil, and netarsudil demonstrated noteworthy enhancements in corneal clarity, endothelial cell density, and visual acuity. In pseudophakic bullous keratopathy, the injection of Y-27632 together with cultured corneal endothelial cells into the anterior chamber lead to enhanced corneal endothelial cell density and improved visual acuity. Animal models simulating chemical injury to the cornea showed a reduction of neovascularization and epithelial defects after application of fasudil and in a case of iridocorneal endothelial syndrome netarsudil improved corneal edema. Addressing safety considerations, netarsudil and ripasudil, both clinically approved, exhibit adverse events such as conjunctival hyperemia, conjunctival hemorrhage, cornea verticillata, conjunctivitis, and blepharitis. Monitoring patients during treatment becomes crucial to balancing the potential therapeutic benefits with these associated risks. In conclusion, ROCK inhibitors, particularly netarsudil and ripasudil, offer promise in managing corneal diseases. The comparative analysis of their pharmacological properties and studies supporting their efficacy underscore their potential therapeutic significance. However, ongoing research is paramount to comprehensively understand their safety profiles and long-term outcomes in diverse corneal conditions, guiding their optimal application in clinical practice.
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  • 文章类型: Journal Article
    (1)目的:目的是使用眼镜辅助的NGENUITY®3D可视化系统(AlconLaboratories,沃思堡,TX,美国)。(2)方法:在Arruzafa医院进行的这项前瞻性研究中,使用NGENUITY®系统进行了5例连续的DMEK手术和4例连续的DSO,科尔多瓦,西班牙。每个患者只有一只眼睛接受手术。使用EDTRS图表的最佳矫正视力(CDVA),使用CasiaII光学相干层析成像仪(TomeyCo.,名古屋,Japan),和使用TomeyEM-4000(TomeyCo.,名古屋,日本)用于DMEK病例或NidekCEM-530(NidekCo.,Ltd.,Gamagori,日本)术前以及术后1和3个月记录了DSO病例的镜面显微镜。(3)结果:DMEK病例包括1名男性和4名女性受试者,平均年龄73.6±9.5岁。术后3个月CDVA的平均改善为0.46±0.16小数。术后1至3个月之间细胞计数的平均变化为360.75±289.38细胞/mm2。DSO病例包括四名女性受试者,平均年龄64.2±9.7岁。术后3个月CDVA的平均改善为0.09±0.17小数。所有病例均进行了超声乳化手术。手术后1至3个月细胞计数的平均变化为460±515.69细胞/mm2。在任何情况下,手术或随访期间均无相关并发症。(4)结论:除了在手术过程中使用3D可视化系统的已知好处外,本研究表明,该系统可以成功地用于DMEK和DSO手术,外科医生的学习曲线很短。
    (1) Purpose: The aim was to analyze the outcomes of Descemet\'s membrane endothelial keratoplasty (DMEK) and Descemet stripping only (DSO) surgeries using a glasses-assisted NGENUITY® 3D visualization system (Alcon Laboratories, Fort Worth, TX, USA). (2) Methods: Five consecutive cases of DMEK surgery and four consecutive cases of DSO were performed using the NGENUITY® system in this prospective study carried out at the Arruzafa Hospital, Córdoba, Spain. Only one eye from each patient received surgery. Best corrected distance visual acuity (CDVA) using EDTRS charts, central corneal thickness using the Casia II optical coherence tomograph (Tomey Co., Nagoya, Japan), and endothelial cell count using the Tomey EM-4000 (Tomey Co., Nagoya, Japan) for DMEK cases or the Nidek CEM-530 (Nidek Co., Ltd., Gamagori, Japan) specular microscopes for DSO cases were recorded preoperatively and at 1 and 3 months postsurgery. (3) Results: DMEK cases included one male and four female subjects, with a mean age of 73.6 ± 9.5 years. Average improvement in CDVA 3 months after surgery was 0.46 ± 0.16 decimal. Average change in cell count between 1 and 3 months postsurgery was 360.75 ± 289.38 cells/mm2. DSO cases included four female subjects, with a mean age of 64.2 ± 9.7 years. The average improvement in CDVA 3 months after surgery was 0.09 ± 0.17 decimal. All cases also had phacoemulsification carried out. He average change in cell count between 1 and 3 months after surgery was 460 ± 515.69 cells/mm2. There were no associated complications during surgery or the follow-up period in any of the cases. (4) Conclusions: In addition to the known benefits of the use of a 3D visualization system during surgery, the present study shows that the system can be successfully used in both DMEK and DSO procedures with a very short learning curve for the surgeon.
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  • 文章类型: Journal Article
    Fuchs内皮角膜营养不良(FECD)是由于内皮细胞营养不良引起的角膜水肿。Descemet膜内皮角膜移植术(DMEK)被认为是治疗的金标准。这项研究的目的是调查DMEK前后FECD患者角膜上皮厚度的变化,并将这些结果与健康对照组进行比较。在这个回顾性分析中,接受DMEK治疗的FECD患者的38只眼和35只健康对照眼接受了眼前节光学相干断层扫描(OCT;Optovue,XR-Avanti,弗里蒙特,CA,美国)。术前分析比较不同部位角膜上皮厚度,术后,和控制队列。中位随访时间为9个月。在中央的DMEK后,平均上皮厚度显着下降,paracentral,和角膜的中间周边区域(p<0.01)。角膜总厚度和基质厚度也显着降低。术后和对照组之间没有观察到显着差异。总之,与健康对照组相比,FECD患者的上皮厚度增加,DMEK后显着降低,并达到与健康对照眼相当的厚度水平。这项研究强调了在眼前段病理和外科手术中区分角膜层的重要性。此外,它强调了FECD的结构改变超出角膜基质的事实。
    Fuchs\' endothelial corneal dystrophy (FECD) is the occurrence of corneal edema due to endothelial cell dystrophy. Descemet membrane endothelial keratoplasty (DMEK) is considered to be the gold standard of treatment. The aim of this study was to investigate the changes in the corneal epithelial thickness of FECD patients before and after DMEK and to compare these results with a healthy control cohort. In this retrospective analysis, 38 eyes of patients with FECD that were treated with DMEK and 35 healthy control eyes received anterior segment optical coherence tomography (OCT; Optovue, XR-Avanti, Fremont, CA, USA). The corneal epithelial thicknesses in different locations were analyzed and compared between the preoperative, postoperative, and control cohorts. The median follow-up time was 9 months. There was a significant degression of the mean epithelial thickness after DMEK in the central, paracentral, and mid-peripheral zones (p < 0.01) of the cornea. The total corneal thickness and stromal thickness decreased significantly as well. No significant differences were observed between the postoperative and control cohorts. In conclusion, the FECD patients had an increased epithelial thickness compared to the healthy controls, which decreased significantly after DMEK and reached thickness levels comparable to those of healthy control eyes. This study emphasized the importance of distinguishing between the corneal layers in anterior segment pathologies and surgical procedures. Moreover, it accentuated the fact that the structural alterations in FECD extend beyond the corneal stroma.
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  • 文章类型: Journal Article
    目的:评估个体外科医师对Descemet膜内皮角膜移植术(DMEK)和Triple-DMEK的学习曲线,并根据经验评估结果。
    方法:回顾性纳入四位外科医生的前41例和后41例手术。记录手术时间和移植物准备时间。校正后的远距视力(CDVA,logMAR)和中央角膜厚度(CCT,µm)在术前6个月和12个月后收集,以及术后并发症,例如,再起泡或重复穿透性角膜移植术。
    结果:Triple-DMEK和DMEK的手术时间在两个时期之间显着减少了21分钟和14分钟(p<0.001;p<0.001)。移植物准备时间从第1期的13.3±5.2分钟(95CI12.8-14.3)显着减少到第2期的10.7±4.8分钟(95CI10.2-11.4)(p=0.002)。术后CDVA和CCT在两个时期的变化均不显著(p=0.900;p=0.263)。再起泡率从第1期的51.2%显著下降到第2期的26.2%(p<0.001)。重复穿透性角膜移植术(PKP)在第1阶段为7.3%,在第2阶段为3.7%(p=0.146)。Re-DMEK在第1阶段为6.1%,在第2阶段为4.9%(p=0.535)。几个参数显示两个时期外科医生之间的显着差异(手术持续时间:第1期:p<0.001,第2期p<0.001;移植物准备:第1期:p<0.001,第2期p<0.001)。
    结论:手术时间显著缩短,移植物准备时间,重新起泡率可以归因于获得的个人经验。
    OBJECTIVE: Evaluating the learning curve of individual surgeons for Descemet Membrane Endothelial Keratoplasty (DMEK) and Triple-DMEK and assessing outcome with experience.
    METHODS: The first 41 and the last 41 surgeries of each of the four surgeons were retrospectively included. Surgery duration and graft preparation time were recorded. Corrected distance visual acuity (CDVA, logMAR) and central corneal thickness (CCT, µm) were collected preoperatively after 6 and 12 months, as well as postoperative complications, e.g., re-bubbling or repeat penetrating keratoplasty.
    RESULTS: Surgical duration for Triple-DMEK and DMEK decreased significantly by 21 min and 14 min between the two periods (p < 0.001; p < 0.001). Graft preparation time decreased significantly from 13.3 ± 5.2 min (95%CI 12.8-14.3) in period 1 to 10.7 ± 4.8 min (95%CI 10.2-11.4) in period 2 (p = 0.002). The postoperative changes in CDVA and CCT over both periods were not significant (p = 0.900; p = 0.263). The re-bubbling rate decreased significantly from 51.2% in period 1 to 26.2% in period 2 (p < 0.001). The repeat penetrating keratoplasty (PKP) was 7.3% in period 1 and 3.7% in period 2 (p = 0.146). Re-DMEK was necessary in 6.1% in period 1 and 4.9% in period 2 (p = 0.535). Several parameters showed significant differences between the surgeons in both periods (surgical duration: period 1: p < 0.001, period 2 p < 0.001; graft preparation: period 1: p < 0.001, period 2 p < 0.001).
    CONCLUSIONS: Significant decrease in surgery duration, graft preparation time, and the re-bubbling rate can be attributed to gained individual experience.
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  • 文章类型: Journal Article
    综述了与Fuchs内皮角膜营养不良(FECD)上皮间质转化研究相关的miRNA表达。
    文献检索策略-PubMed中央数据库,使用“miRNA”或“microRNA”和“上皮间质转化”或“EMT”和“Fuchs”内皮角膜营养不良”或“FECD”作为关键字。包括在2009年至2022年之间发表的关于Fuchs内皮角膜营养不良中上皮间质转化的miRNA谱的英文发表的人类实验或临床研究。
    关于Fuchs内皮角膜营养不良中上皮间质转化的miRNA谱的出版物很少,但提供了一些关于区分衰老变化与以上皮间质转化为特征的早期疾病阶段的潜在生物标志物的有价值的信息。在FECD患者的角膜组织中,证明了miRNA-184种子区突变以及由miRNA-29导致的总miRNA表达的单向下调。对于早期诊断,应分析房水中上皮间质转化的miRNA并将其用作生物标志物。
    a review of miRNA expression connected to epithelial mesenchymal transition studies in Fuchs\' endothelial corneal dystrophy (FECD).
    literature search strategy-PubMed central database, using \"miRNA\" or \"microRNA\" and \"epithelial mesenchymal transition\" or \"EMT\" and \"Fuchs\' endothelial corneal dystrophy\" or \"FECD\" as keywords. Experimental or clinical studies on humans published in English regarding miRNA profiles of epithelial mesenchymal transition in Fuchs\' endothelial corneal dystrophy published between 2009 and 2022 were included.
    The publications regarding the miRNA profiles of epithelial mesenchymal transition in Fuchs\' endothelial corneal dystrophy are scarce but provide some valuable information about the potential biomarkers differentiating aging changes from early disease stages characterized by epithelial mesenchymal transition. In the corneal tissue of FECD patients, miRNA-184 seed-region mutation as well as unidirectional downregulation of total miRNA expression led by the miRNA-29 were demonstrated. For early diagnostics the miRNA of epithelial mesenchymal transition in aqueous humor should be analyzed and used as biomarkers.
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  • 文章类型: Journal Article
    未经证实:70%的Fuchs内皮角膜营养不良(FECD)病例是由转录因子4基因(TCF4)的内含子三核苷酸重复扩增引起的。这项研究的目的是表征有(RE)和没有三核苷酸重复扩增(RE-)的FECD患者的角膜基底下神经丛和角膜雾霾,并评估这些参数与疾病严重程度的相关性。
    未经评估:横截面,单中心研究。
    UNASSIGNED:该研究包括29名受试者的52只眼,其FECD严重程度的Krachmer等级从1到6。29名受试者中有15名携带扩展的TCF4等位基因长度≥40个胞嘧啶-胸腺嘧啶-鸟嘌呤重复序列(RE)。
    UNASSIGNED:体内共聚焦显微镜评估角膜神经纤维长度(CNFL),角膜神经分支密度,角膜神经纤维密度(CNFD),和前角膜基质反向散射(haze);Scheimpflug断层摄影术密度测定法测量前的haze,中央,和后角膜层。
    未经评估:使用共聚焦显微镜,我们在RE+受试者的眼睛中检测到FECD严重程度与CNFL和CNFD之间呈负相关(分别为Spearmanρ=-0.45,P=0.029和ρ=-0.62,P=0.0015),但在RE-受试者的眼睛中没有。此外,在RE+受试者中,CNFD与扩增等位基因的重复长度呈负相关(Spearmanρ=-0.42,P=0.038)。我们发现RE和RE-组的前基质背向散射与严重程度之间呈正相关(分别为ρ=0.60,P=0.0023和ρ=0.44,P=0.024)。前部,中央,和后Scheimpflug密度测定测量值也与RE组和RE-组的严重程度呈正相关(分别为P=5.5×10-5、2.5×10-4和2.9×10-4,在合并分析中调整扩展状态后。然而,对于患有严重FECD(克拉赫默5级和6级)的患者,RE+组的后路光密度测量值高于RE-组(P<0.05)。
    UNASSIGNED:FECD角膜神经丢失支持将TCF4三核苷酸重复扩增障碍分类为神经退行性疾病。前面的阴霾,中央,后角膜与严重程度相关,无论基因型。角膜神经和角膜雾霾的定量评估可能有助于评估和监测RE患者的FECD疾病严重程度。
    UNASSIGNED: Seventy percent of Fuchs\' endothelial corneal dystrophy (FECD) cases are caused by an intronic trinucleotide repeat expansion in the transcription factor 4 gene (TCF4). The objective of this study was to characterize the corneal subbasal nerve plexus and corneal haze in patients with FECD with (RE+) and without the trinucleotide repeat expansion (RE-) and to assess the correlation of these parameters with disease severity.
    UNASSIGNED: Cross-sectional, single-center study.
    UNASSIGNED: Fifty-two eyes of 29 subjects with a modified Krachmer grade of FECD severity from 1 to 6 were included in the study. Fifteen of the 29 subjects carried an expanded TCF4 allele length of ≥ 40 cytosine-thymine-guanine repeats (RE+).
    UNASSIGNED: In vivo confocal microscopy assessments of corneal nerve fiber length (CNFL), corneal nerve branch density, corneal nerve fiber density (CNFD), and anterior corneal stromal backscatter (haze); Scheimpflug tomography densitometry measurements of haze in anterior, central, and posterior corneal layers.
    UNASSIGNED: Using confocal microscopy, we detected a negative correlation between FECD severity and both CNFL and CNFD in the eyes of RE+ subjects (Spearman ρ = -0.45, P = 0.029 and ρ = -0.62, P = 0.0015, respectively) but not in the eyes of RE- subjects. Additionally, CNFD negatively correlated with the repeat length of the expanded allele in the RE+ subjects (Spearman ρ = -0.42, P = 0.038). We found a positive correlation between anterior stromal backscatter and severity in both the RE+ and RE- groups (ρ = 0.60, P = 0.0023 and ρ = 0.44, P = 0.024, respectively). The anterior, central, and posterior Scheimpflug densitometry measurements also positively correlated with severity in both the RE+ and RE- groups (P = 5.5 × 10-5, 2.5 × 10-4, and 2.9 × 10-4, respectively, after adjusting for the expansion status in a pooled analysis. However, for patients with severe FECD (Krachmer grades 5 and 6), the posterior densitometry measurements were higher in the RE+ group than in the RE- group (P < 0.05).
    UNASSIGNED: Loss of corneal nerves in FECD supports the classification of the TCF4 trinucleotide repeat expansion disorder as a neurodegenerative disease. Haze in the anterior, central, and posterior cornea correlate with severity, irrespective of the genotype. Quantitative assessments of corneal nerves and corneal haze may be useful to gauge and monitor FECD disease severity in RE+ patients.
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  • 文章类型: Journal Article
    目的:确定Fuchs内皮角膜营养不良(FECD)患者接受内皮角膜移植术(EK)和穿透性角膜移植术(PK)的相关因素。
    方法:回顾性队列研究。
    方法:医疗保险受益人65岁或以上,2011年至2019年间诊断为FECD。
    方法:查询100%Medicare按服务收费的行政索赔数据库,以获取未治疗的FECD患者。包括年龄,种族和民族,性别,地理,眼合并症和手术,Charlson合并症指数(CCI),和社会经济状况用于确定与接收EK和PK相关的因素。Kaplan-Meier生存分析用于确定白内障或复杂或其他眼前节手术后EK的发生率。
    方法:与收到EK或PK相关的因素,加上白内障或复杂或其他眼前节手术后的EK率。
    结果:在确定的719066名受益人中,31372(4.4%)收到EK,2426(0.3%)收到PK。在多变量分析中,女性性别降低了EK和PK的可能性(调整后的优势比0.83[95%置信区间0.81-0.85]和0.84[0.78-0.92],分别),与Southern相比,西部居住地(1.33[1.29-1.38];1.25[1.11-1.42])和复杂或其他眼前节手术史(1.62[1.54-1.70];5.52[4.97-6.12])增加了两者的可能性。与白人相比,布莱克的EK可能性降低(0.76[0.72-0.80]),亚洲或太平洋岛民(0.54[0.48-0.61]),西班牙裔或拉丁裔(0.62[0.55-0.70])种族和种族,而对于同一组,PK的可能性增加(Black1.32[1.14-1.53];亚洲/太平洋岛民1.46[1.13-1.89];和西班牙裔/拉丁裔1.62[1.25-2.11]).白内障或复杂/其他眼前段手术后,1年EK率分别为1.3%和3.3%,8年分别为2.3%和5.6%,分别。
    结论:在多变量分析中,与男性相比,女性受益人获得FECDEK或PK的可能性较小,与白人受益人相比,非白人受益人不太可能获得EK,而更有可能获得PK。
    To identify factors associated with receipt of endothelial keratoplasty (EK) and penetrating keratoplasty (PK) in patients with Fuchs\' endothelial corneal dystrophy (FECD).
    Retrospective cohort study.
    Medicare beneficiaries 65 years of age or older with a FECD diagnosis between 2011 and 2019.
    The 100% Medicare fee-for-service administrative claims database was queried for treatment-naïve FECD patients. A multivariate logistic regression model including age, race and ethnicity, sex, geography, ocular comorbidities and surgeries, Charlson comorbidity index (CCI), and socioeconomic status was used to identify factors associated with receipt of EK and PK. Kaplan-Meier survival analyses were used to determine the rate of EK after cataract or complex or other anterior segment surgery.
    Factors associated with receipt of an EK or PK, plus rate of EK after cataract or complex or other anterior segment surgery.
    Of 719 066 beneficiaries identified, 31 372 (4.4%) received an EK and 2426 (0.3%) received a PK. In a multivariate analysis, female sex decreased likelihood of both EK and PK (adjusted odds ratio 0.83 [95% confidence interval 0.81-0.85] and 0.84 [0.78-0.92], respectively), while Western residence (1.33 [1.29-1.38]; 1.25 [1.11-1.42]) compared to Southern and history of complex or other anterior segment surgery (1.62 [1.54-1.70]; 5.52 [4.97-6.12]) increased the likelihood of both. Compared to Whites, the likelihood of EK was decreased for Black (0.76 [0.72-0.80]), Asian or Pacific Islander (0.54 [0.48-0.61]), and Hispanic or Latino (0.62 [0.55-0.70]) race and ethnicity, while for the same groups likelihood of PK was increased (for Black 1.32 [1.14-1.53]; Asian/Pacific Islander 1.46 [1.13-1.89]; and Hispanic/Latino 1.62 [1.25-2.11]). Following cataract or complex/other anterior segment surgery, rates of EK were 1.3% and 3.3% at 1 year and 2.3% and 5.6% at 8 years, respectively.
    In a multivariate analysis, women beneficiaries are less likely to receive EK or PK for FECD compared with men, whereas non-White beneficiaries are less likely to receive EK and more likely to receive PK compared with White beneficiaries.
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