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Progress in Retinal and Eye Research

James S Wolffsohn, Leon N Davies
Presbyopia is a global problem affecting over a billion people worldwide. The prevalence of unmanaged presbyopia is as high as 50% of those over 50 years of age in developing world populations due to a lack of awareness and accessibility to affordable treatment, and is even as high as 34% in developed countries. Definitions of presbyopia are inconsistent and varied, so we propose a redefinition that states "presbyopia occurs when the physiologically normal age-related reduction in the eye's focusing range reaches a point, when optimally corrected for distance vision, that the clarity of vision at near is insufficient to satisfy an individual's requirements"...
September 19, 2018: Progress in Retinal and Eye Research
Thibaud Mathis, Pauline Jardel, Olivier Loria, Benoit Delaunay, Anh-Minh Nguyen, Francesco Lanza, Carlo Mosci, Jean-Pierre Caujolle, Laurent Kodjikian, Juliette Thariat
The most frequent site of ocular metastasis is the choroid. The occurrence of choroidal metastases has increased steadily due to the longer survival of metastatic patients and the improvement of diagnostic tools. Fundoscopy, ultrasonography, and fluorescein angiography are now complemented by indocyanine green angiography and optical coherence tomography. Choroidal tumor biopsy may also confirm the metastatic nature of the tumor and help to determine the site of the primary malignancy. There is currently no consensus on the treatment strategy...
September 18, 2018: Progress in Retinal and Eye Research
Leah P Foltz, Dennis O Clegg
The human retina is a highly complex tissue that makes up an integral part of our central nervous system. It is astonishing that our retina works seamlessly to provide one of our most critical senses, and it is equally devastating when a disease destroys a portion of the retina and robs people of their vision. After decades of research, scientists are beginning to understand retinal cells in a way that can benefit the millions of individuals suffering from inherited blindness. This understanding has come about in part with the ability to culture human embryonic stem cells and the innovation of induced pluripotent stem cells, which can be cultured from patients and used to model their disease...
September 11, 2018: Progress in Retinal and Eye Research
Marta Fernández-Nogales, Verónica Murcia-Belmonte, Holly Yu Chen, Eloísa Herrera
Numerous degenerative diseases affecting visual function, including glaucoma and retinitis pigmentosa, are produced by the loss of different types of retinal cells. Cell replacement therapy has emerged as a promising strategy for treating these and other retinal diseases. The retinal margin or ciliary body (CB) of mammals has been proposed as a potential source of cells to be used in degenerative conditions affecting the retina because it has been reported it might hold neurogenic potential beyond embryonic development...
September 7, 2018: Progress in Retinal and Eye Research
Jia Hui Lee, Jiang-Hui Wang, Jinying Chen, Fan Li, Thomas L Edwards, Alex W Hewitt, Guei-Sheung Liu
Many clinical trials using gene therapy have shown significant therapeutic benefits and exceptional safety records. Increasing evidence is verifying the long sought-after promise that gene therapy will genetically 'cure' some severely disabling diseases. In particular, the first gene therapy bioproduct for RPE65-associated Leber's congenital amaurosis, which was approved by the US Food and Drug Administration in 2017, has provided tremendous encouragement to the field of gene therapy. Recent developments in genome editing technologies have significantly advanced our capability to precisely engineer genomes in eukaryotic cells...
August 29, 2018: Progress in Retinal and Eye Research
Stephen A Burns, Ann E Elsner, Kaitlyn A Sapoznik, Raymond L Warner, Thomas J Gast
Adaptive Optics (AO) retinal imaging has provided revolutionary tools to scientists and clinicians for studying retinal structure and function in the living eye. From animal models to clinical patients, AO imaging is changing the way scientists are approaching the study of the retina. By providing cellular and subcellular details without the need for histology, it is now possible to perform large scale studies as well as to understand how an individual retina changes over time. Because AO retinal imaging is non-invasive and when performed with near-IR wavelengths both safe and easily tolerated by patients, it holds promise for being incorporated into clinical trials providing cell specific approaches to monitoring diseases and therapeutic interventions...
August 27, 2018: Progress in Retinal and Eye Research
Ursula Schmidt-Erfurth, Amir Sadeghipour, Bianca S Gerendas, Sebastian M Waldstein, Hrvoje Bogunović
Major advances in diagnostic technologies are offering unprecedented insight into the condition of the retina and beyond ocular disease. Digital images providing millions of morphological datasets can fast and non-invasively be analyzed in a comprehensive manner using artificial intelligence (AI). Methods based on machine learning (ML) and particularly deep learning (DL) are able to identify, localize and quantify pathological features in almost every macular and retinal disease. Convolutional neural networks thereby mimic the path of the human brain for object recognition through learning of pathological features from training sets, supervised ML, or even extrapolation from patterns recognized independently, unsupervised ML...
August 1, 2018: Progress in Retinal and Eye Research
Sylvia B Smith, Jing Wang, Xuezhi Cui, Barbara A Mysona, Jing Zhao, Kathryn E Bollinger
Retinal degenerative diseases are major causes of untreatable blindness worldwide and efficacious treatments for these diseases are sorely needed. A novel target for treatment of retinal disease is the transmembrane protein Sigma 1 Receptor (Sig1R). This enigmatic protein is an evolutionary isolate with no known homology to any other protein. Sig1R was originally thought to be an opioid receptor. That notion has been dispelled and more recent pharmacological and molecular studies suggest that it is a pluripotent modulator with a number of biological functions, many of which are relevant to retinal disease...
August 1, 2018: Progress in Retinal and Eye Research
Enrico Borrelli, David Sarraf, K Bailey Freund, Srinivas R Sadda
The recent introduction of optical coherence tomography angiography (OCTA) has remarkably expanded our knowledge of the choroid through in vivo investigation of the anatomical and pathological features of this important vascular layer. New insights elucidating the morphological features of the choroid, in both physiological and pathological conditions, indicate that this vascular structure plays a crucial role in many chorioretinal disorders. In this article, a review of the salient histological and anatomical features of the choroid, essential for the proper interpretation of in vivo imaging, is followed by a discussion of the fundamental principles of OCTA and the application of this advanced imaging modality to study and understand the choroid...
July 27, 2018: Progress in Retinal and Eye Research
Michel Paques, Serge Meimon, Florence Rossant, David Rosenbaum, Sarah Mrejen, Florian Sennlaub, Kate Grieve
Adaptive optics (AO)-enhanced en face retinal imaging, termed here AO ophthalmoscopy (AOO) has reached a level of robustness which fuels its increasing use in research and clinical centers. Here we will review the contribution of clinical AOO to the understanding and monitoring of 1) age-related macular degeneration and 2) vascular diseases. The main contributions of AOO to the phenotyping of AMD are a better identification of drusen, a better delineation of the limits of atrophy, and the identification of novel features such as punctate hyperreflectivity and mobile melanin-containing clumps...
July 17, 2018: Progress in Retinal and Eye Research
Rabab Sharif, Sashia Bak-Nielsen, Jesper Hjortdal, Dimitrios Karamichos
Keratoconus (KC) is the most common ectatic corneal disease, with clinical findings that include discomfort, visual disturbance and possible blindness if left untreated. KC affects approximately 1:400 to 1:2000 people worldwide, including both males and females. The aetiology and onset of KC remains a puzzle and as a result, the ability to treat or reverse the disease is hampered. Sex hormones are known to play a role in the maintenance of the structure and integrity of the human cornea. Hormone levels have been reported to alter corneal thickness, curvature, and sensitivity during different times of menstrual cycle...
July 12, 2018: Progress in Retinal and Eye Research
Marina França Dias, Kwangsic Joo, Jessica A Kemp, Silvia Ligório Fialho, Armando da Silva Cunha, Se Joon Woo, Young Jik Kwon
No abstract text is available yet for this article.
September 2018: Progress in Retinal and Eye Research
Lewis E Fry, Eamonn Fahy, Vicki Chrysostomou, Flora Hui, Jessica Tang, Peter van Wijngaarden, Steven Petrou, Jonathan G Crowston
Retinal ganglion cell (RGC) degeneration causes vision loss in patients with glaucoma, and this has been generally considered to be irreversible due to RGC death. We question this assertion and summarise accumulating evidence that points to visual function improving in glaucoma patients with treatment, particularly in the early stages of disease. We propose that prior to death, RGCs enter periods of dysfunction but can recover with relief of RGC stress. We first summarise the clinical evidence for vision improvement in glaucoma and then detail our experimental work that points to the underlying processes that underpin clinical improvement...
July 2018: Progress in Retinal and Eye Research
Erin R Burnight, Joseph C Giacalone, Jessica A Cooke, Jessica R Thompson, Laura R Bohrer, Kathleen R Chirco, Arlene V Drack, John H Fingert, Kristan S Worthington, Luke A Wiley, Robert F Mullins, Edwin M Stone, Budd A Tucker
Gene correction is a valuable strategy for treating inherited retinal degenerative diseases, a major cause of irreversible blindness worldwide. Single gene defects cause the majority of these retinal dystrophies. Gene augmentation holds great promise if delivered early in the course of the disease, however, many patients carry mutations in genes too large to be packaged into adeno-associated viral vectors and some, when overexpressed via heterologous promoters, induce retinal toxicity. In addition to the aforementioned challenges, some patients have sustained significant photoreceptor cell loss at the time of diagnosis, rendering gene replacement therapy insufficient to treat the disease...
July 2018: Progress in Retinal and Eye Research
Ximena Corso-Díaz, Catherine Jaeger, Vijender Chaitankar, Anand Swaroop
Complex biological processes, such as organogenesis and homeostasis, are stringently regulated by genetic programs that are fine-tuned by epigenetic factors to establish cell fates and/or to respond to the microenvironment. Gene regulatory networks that guide cell differentiation and function are modulated and stabilized by modifications to DNA, RNA and proteins. In this review, we focus on two key epigenetic changes - DNA methylation and histone modifications - and discuss their contribution to retinal development, aging and disease, especially in the context of age-related macular degeneration (AMD) and diabetic retinopathy...
July 2018: Progress in Retinal and Eye Research
Simon J Epps, Joanne Boldison, Madeleine L Stimpson, Tarnjit K Khera, Philippa J P Lait, David A Copland, Andrew D Dick, Lindsay B Nicholson
Ocular function depends on a high level of anatomical integrity. This is threatened by inflammation, which alters the local tissue over short and long time-scales. Uveitis due to autoimmune disease, especially when it involves the retina, leads to persistent changes in how the eye interacts with the immune system. The normal pattern of immune surveillance, which for immune privileged tissues is limited, is re-programmed. Many cell types, that are not usually present in the eye, become detectable. There are changes in the tissue homeostasis and integrity...
July 2018: Progress in Retinal and Eye Research
Maria Diedrichs-Möhring, Ulrike Kaufmann, Gerhild Wildner
Autoimmune diseases usually follow a relapsing-remitting or a chronic progressive course. To understand the underlying immunopathogenesis we investigated experimental Lewis rat models displaying both disease types, which were only dependent on the autoantigen peptide used for immunization. Retinal S-Antigen-peptide PDSAg induces chronic, monophasic disease, whilst interphotoreceptor retinoid-binding protein (IRBP)-peptide R14 causes a spontaneously relapsing-remitting course. R14-mediated uveitis can be re-induced by immunization; PDSAg-induced disease is even preventable by prior CFA-injection...
July 2018: Progress in Retinal and Eye Research
Machelle T Pardue, Rachael S Allen
Diseases that affect the eye, including photoreceptor degeneration, diabetic retinopathy, and glaucoma, affect 11.8 million people in the US, resulting in vision loss and blindness. Loss of sight affects patient quality of life and puts an economic burden both on individuals and the greater healthcare system. Despite the urgent need for treatments, few effective options currently exist in the clinic. Here, we review research on promising neuroprotective strategies that promote neuronal survival with the potential to protect against vision loss and retinal cell death...
July 2018: Progress in Retinal and Eye Research
C B Tara Moore, Kathleen A Christie, John Marshall, M Andrew Nesbit
The potential of personalised genome editing reaching the clinic has come to light due to advancements in the field of gene editing, namely the development of CRISPR/Cas9. The different mechanisms of repair used to resolve the double strand breaks (DSBs) mediated by Cas9 allow targeting of a wide range of disease causing mutations. Collectively, the corneal dystrophies offer an ideal platform for personalised genome editing; the majority of corneal dystrophies are monogenic, highly penetrant diseases with a known pattern of inheritance...
July 2018: Progress in Retinal and Eye Research
Hemal Mehta, Adnan Tufail, Vincent Daien, Aaron Y Lee, Vuong Nguyen, Mehmet Ozturk, Daniel Barthelmes, Mark C Gillies
Clinical trials identified intravitreal vascular endothelial growth factor inhibitors (anti-VEGF agents) have the potential to stabilise or even improve visual acuity outcomes in neovascular age-related macular degeneration (AMD), a sight-threatening disease. Real-world evidence allows us to assess whether results from randomised controlled trials can be applied to the general population. We describe the development of global registries, in particular the Fight Retinal Blindness! registry that originated in Australia, the United Kingdom AMD Electronic Medical Records User Group and the IRIS registry in the USA...
July 2018: Progress in Retinal and Eye Research
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