Generation of three human induced pluripotent stem cell lines from retinitis pigmentosa 25 patient and two carriers but asymptomatic daughters.

Retinitis Pigmentosa type 25 (RP25) is a form of inherited retinal dystrophy characterized by a progressive loss of rod photoreceptors, subsequent degeneration of cone photoreceptors, and eventually, the retinal pigment epithelium. Caused by mutations in the EYS gene, it is believed to be critical for the structural and functional integrity of the retina. Using a non-integrative RNA reprogramming method, we have generated human induced pluripotent stem cell (hiPSC) lines from RP25 patient and from carriers but asymptomatic daughters.

Successful use of PRGF-endoret® and ILM peeling for full thickness macular hole in MacTel type 2: A case report and review of literature.

Purpose: To describe the successful use of plasma rich in growth factors (PRGF-Endoret®) and internal limiting membrane peeling for full thickness macular hole in Macular Telangiectasia type 2.

Case Presentation: A case report of a full thickness macular hole (FTMH) associated with Macular Telangiectasia (MacTel) type 2 is described. 25-G vitrectomy with internal limiting membrane (ILM) peeling and use of (PRGF-Endoret®) was performed.

Reverse Pupillary Block after Implantation of a Sutureless Scleral Fixation Carlevale Intraocular Lens.

Purpose: To describe the incidence, complications and management of reverse pupillary block (RPB) after implantation of Carlevale intraocular lens (IOL).

Design: Multicenter, retrospective, cross-sectional study.

Participants: Of a sample of 128 patients that had undergone Carlevale IOL implantation, 19 patients were found to present RPB.

Fight Retinal Blindness SPAIN. Report 3: clinical outcomes of vascular endothelial growth factor inhibitors in low vision eyes with neovascular age-related macular degeneration. A national database study.

Objectives: To compare visual outcomes for low vision eyes (LVE) (<35 letters LogMAR or <20/200 Snellen) versus non-low vision eyes (NLVE) (>35 letters LogMAR or >20/200 Snellen) at the time of the first injection in a clinical practice setting.

Methods: Subgroup analysis of a multicenter national database of treatment- naïve eyes neovascular age related macular degeneration (nAMD) treated with anti-VEGF intravitreal injections divided into LVE and NLVE. Demographics, visual acuity (VA) at baseline and subsequent timepoints (12, 24, and 36 months), number of injections and visits data were collected using a validated web-based tool (Fight Retinal Blindness!).

Optical coherence tomography biomarkers in MYO7A-inherited retinal dystrophy: longitudinal study in pediatric patients.

Purpose: This study aims to answer a key question: is MYO7A-inherited retinal dystrophy (MYO7A-IRD) a photoreceptor-first or retinal pigment epithelium-first disease? A second aim was to determine the most useful biomarkers to monitor disease progression in pediatric patients with Usher syndrome type 1B (USH1) secondary to MYO7A mutation.

Methods: Fifty-two eyes from 26 patients with genetically-confirmed MYO7A-IRD underwent swept-source optical coherence tomography (SS-OCT). Structural abnormalities were evaluated and correlated with follow-up time and best corrected visual acuity (BCVA).

Neovascular Progression and Retinal Dysfunction in the Laser-Induced Choroidal Neovascularization Mouse Model.

The mouse model of laser-induced choroidal neovascularization (LI-CNV) has been widely used to study neovascular age-related macular degeneration; however, it still lacks a comprehensive characterization. Here, CNV was induced in the eyes of 12-week-old C57BL/6J male mice by argon laser irradiation. We studied the CNV lesion progression of an LI-CNV mouse cohort by using multimodal imaging (color fundus, optical coherence tomography (OCT), and fluorescence angiography, focal electroretinography features for 14 days, and related cytokines, angiogenic factors, and reactive gliosis for 5 days.

Blocking Hemopexin With Specific Antibodies: A New Strategy for Treating Diabetic Retinopathy.

Unlabelled: Hemopexin (HPX) is overexpressed in the retina of patients with diabetes and induces the breakdown of the blood-retinal barrier in vitro. The aim of this study was to evaluate whether HPX blockade by specific antibodies (aHPX) could avoid vascular leakage in vivo and microvascular angiogenesis in vitro and ex vivo. For this purpose, the effect of intravitreal (IVT) injections of aHPX on vascular leakage was evaluated in db/db mice and rats with streptozotocin-induced diabetes using the Evans Blue method.

Clinical and Molecular Aspects of C2orf71/PCARE in Retinal Diseases.

Mutations in the photoreceptor-specific gene (also known as photoreceptor cilium actin regulator protein PCARE) cause autosomal recessive retinitis pigmentosa type 54 and cone-rod dystrophy. No treatments are available for patients with retinal ciliopathies exhibiting a severe clinical phenotype. Our understanding of the disease process and the role of PCARE in the healthy retina significantly limits our capacity to transfer recent technical developments into viable therapy choices.

Serum glial fibrillary acidic protein and neurofilament light chain as biomarkers of retinal neurodysfunction in early diabetic retinopathy: results of the EUROCONDOR study.

Aims: Neurodegeneration and glial activation are primary events in the pathogenesis of diabetic retinopathy. Serum glial fibrillary acidic protein (GFAP) and neurofilament light chain (NfL) are biomarkers of underlying neuroinflammatory and neurodegenerative disease processes. The aim of the present study was to assess the usefulness of these serum biomarkers for the identification and monitoring of retinal neurodysfunction in subjects with type 2 diabetes.

Multiresistant Candida Endophthalmitis Treated with Intravitreal Caspofungin: A Case Report.

Background: endophthalmitis is a severe complication of candidemia. Currently, the recommended treatment of fungal endophthalmitis is a combination of intravitreal and systemic antifungal drugs, and in some cases vitrectomy is also required. Intravitreal therapies that are commonly used are amphotericin B and voriconazole, although recently the use of intravitreal caspofungin has been described in a few case reports.

Repeated Topical Administration of 3 nm Cerium Oxide Nanoparticles Reverts Disease Atrophic Phenotype and Arrests Neovascular Degeneration in AMD Mouse Models.

Increased oxidative stress in the retina and retinal pigment epithelium is implicated in age-related macular degeneration (AMD). Antioxidant cerium oxide nanoparticles (CeONPs) have been used to treat degenerative retinal pathologies in animal models, although their delivery route is not ideal for chronic patient treatment. In this work, we prepared a formulation for ocular topical delivery that contains small (3 nm), nonaggregated biocompatible CeONPs.

All-trans retinoic acid modulates pigmentation, neuroretinal maturation, and corneal transparency in human multiocular organoids.

Background: All-trans retinoic acid (ATRA) plays an essential role during human eye development, being temporally and spatially adjusted to create gradient concentrations that guide embryonic anterior and posterior axis formation of the eye. Perturbations in ATRA signaling can result in severe ocular developmental diseases. Although it is known that ATRA is essential for correct eye formation, how ATRA influences the different ocular tissues during the embryonic development of the human eye is still not well studied.

A Screening Tool for Self-Evaluation of Risk for Age-Related Macular Degeneration: Validation in a Spanish Population.

Purpose: The objectives of this study were the creation and validation of a screening tool for age-related macular degeneration (AMD) for routine assessment by primary care physicians, ophthalmologists, other healthcare professionals, and the general population.

Methods: A simple, self-administered questionnaire (Simplified Théa AMD Risk-Assessment Scale [STARS] version 4.0) which included well-established risk factors for AMD, such as family history, smoking, and dietary factors, was administered to patients during ophthalmology visits.

Persistent reduction of retinal microvascular vessel density in patients with moderate and severe COVID-19 disease.

Objective: This study aims to analyse the possible recovery or worsening in retinal microvasculature after 8 months in a previously studied COVID-19 cohort.

Methods And Analysis: A cross-sectional case-control study and a prospective longitudinal cohort study. Participants were the subjects of our previous study who re-enrolled for a new examination including a fundus photograph (retinography), an optical coherence tomography (OCT) scan and an OCT angiography.

Multiocular organoids from human induced pluripotent stem cells displayed retinal, corneal, and retinal pigment epithelium lineages.

Background: Recently, great efforts have been made to design protocols for obtaining ocular cells from human stem cells to model diseases or for regenerative purposes. Current protocols generally focus on isolating retinal cells, retinal pigment epithelium (RPE), or corneal cells and fail to recapitulate the complexity of the tissue during eye development. Here, the generation of more advanced in vitro multiocular organoids from human induced pluripotent stem cells (hiPSCs) is demonstrated.

Repopulation of decellularized retinas with hiPSC-derived retinal pigment epithelial and ocular progenitor cells shows cell engraftment, organization and differentiation.

The retinal extracellular matrix (ECM) provides architectural support, adhesion and signal guidance that controls retinal development. Decellularization of the ECM affords great potential to tissue engineering; however, how structural retinal ECM affects in vitro development, differentiation and maturation of ocular cells remains to be elucidated. Here, mouse and porcine retinas were decellularized and the protein profile analyzed.

Fundus flavimaculatus-like in myotonic dystrophy: a case report.

Background: Myotonic dystrophy is an inherited disease characterized by progressive muscle weakness and myotonia. It is a multisystemic disorder that affects different parts of the body, including the eye. Dysfunction of ocular muscles, ptosis and cataract are the most common ophthalmologic manifestations, but it can also present with pigmentary changes in the retina.

Cell therapy with hiPSC-derived RPE cells and RPCs prevents visual function loss in a rat model of retinal degeneration.

Photoreceptor loss is the principal cause of blindness in retinal degenerative diseases (RDDs). Whereas some therapies exist for early stages of RDDs, no effective treatment is currently available for later stages, and once photoreceptors are lost, the only option to rescue vision is cell transplantation. With the use of the Royal College of Surgeons (RCS) rat model of retinal degeneration, we sought to determine whether combined transplantation of human-induced pluripotent stem cell (hiPSC)-derived retinal precursor cells (RPCs) and retinal pigment epithelial (RPE) cells was superior to RPE or RPC transplantation alone in preserving retinal from degeneration.

Role of intraretinal and subretinal fluid on clinical and anatomical outcomes in patients with neovascular age-related macular degeneration treated with bimonthly, treat-and-extend and as-needed ranibizumab in the In-Eye study.

Purpose: To assess the effect of fluid status at baseline (BL) and at the end of the loading phase (LP) of three different ranibizumab regimens: treat-and-extend (T&E), fixed bimonthly (FBM) injections and pro re nata (PRN), in patients with neovascular age-related macular degeneration (nAMD).

Design: Post hoc analysis of the In-Eye study (phase IV clinical trial).

Methods: Patients were randomized 1:1:1 to the three study arms and were treated accordingly.

Transcriptomics analysis of Ccl2/Cx3cr1/Crb1 deficient mice provides new insights into the pathophysiology of progressive retinal degeneration.

Chronic oxidative stress and immune dysregulation are key mechanisms involved in the pathogenesis of most retinal degenerative diseases, including age-related macular degeneration. The Ccl2/Cx3cr1/Crb1 mouse model develops a progressive degeneration phenotype, with photoreceptor atrophy, drusen-like lesions or pigment alterations at an early age; however, the role of oxidative stress and immune function in the pathogenesis of the model is poorly understood. We performed a comprehensive characterization of the Ccl2/Cx3cr1/Crb1 mouse to evaluate how these pathways influence pathogenesis.