Retinitis pigmentosa (RP) is characterized by degeneration of rod and cone photoreceptors that progresses to irreversible blindness. Now, there are no mutation-agnostic approaches to treat RP. Here, we utilized a single adeno-associated virus (AAV)-based CRISPR activation system to activate phosphodiesterase 6B (Pde6b) to mitigate the severe degeneration in mice.
View Article and Find Full Text PDFPurpose: To evaluate the clinical outcomes and prognostic factors in unilateral Coats disease in the era of anti-VEGF therapy.
Design: Global, multicenter, retrospective case series.
Subjects: 656 eyes of 656 subjects with Coats disease were included in this study.
Am J Ophthalmol Case Rep
December 2024
Purpose: To report a case of neovascular glaucoma in an 8-year-old male, secondary to a racemose hemangioma without associated intracranial arteriovenous malformation, highlighting the challenges in management and novel findings on optical coherence tomography angiography (OCTA).
Observations: An 8-year-old male initially presented with pain, redness, and blurred vision in the right eye. The patient was diagnosed with secondary neovascular glaucoma due to a racemose hemangioma.
Purpose: Premature infants at risk of retinopathy of prematurity (ROP) miss placental transfer of the carotenoids lutein (L) and zeaxanthin (Z) during the third trimester. We previously demonstrated that prenatal L and Z supplementation raised carotenoid levels in infants at birth in the Lutein and Zeaxanthin in Pregnancy (L-ZIP) study (NCT03750968). Based on their antioxidant effects and bioavailability, we hypothesized that prenatal maternal supplementation with macular carotenoids would reduce the risk of ROP.
View Article and Find Full Text PDFImportance: Literature and anecdotal evidence suggest a relationship between male sex and retinopathy of prematurity (ROP). It is not known whether a difference, if present, is sex-related pathophysiologic predisposition or sex difference in meeting ROP screening criteria.
Objective: To evaluate the association of sex with the development of treatment-warranted ROP.
Ophthalmic Surg Lasers Imaging Retina
January 2022
Universal newborn eye screening facilitates early diagnosis of ocular abnormalities and mitigates vision loss. "Referral warranted" eye disease is present at birth in about 5.5% of term infants, with "macular hemorrhage impinging on the fovea" representing about 50% of referral warranted disease.
View Article and Find Full Text PDFBackground: The American Indian Navajo and Goshute peoples are underserved patient populations residing in the Four Corners area of the United States and Ibupah, Utah, respectively.
Methods: We conducted a cross-sectional study of epidemiological factors and lipid biomarkers that may be associated with type II diabetes, hypertension and retinal manifestations in tribal and non-tribal members in the study areas (n = 146 participants). We performed multivariate analyses to determine which, if any, risk factors were unique at the tribal level.
Purpose: To investigate late retinal findings and complications of eyes with a history of retinopathy of prematurity (ROP) that did not meet treatment criteria and did not receive treatment during infancy.
Design: Retrospective, nonconsecutive, noncomparative, multicenter case series.
Participants: Three hundred sixty-three eyes of 186 patients.
Understanding disease risk is challenging in multifactorial conditions as it can differ by environment, ethnicity and race. The Confederated Tribes of the Goshute Reservation are one of the most isolated populations in the United States. Retinal changes are a reliable indicator for systemic disease.
View Article and Find Full Text PDF: Abnormal activation of signaling pathways related to angiogenesis, inflammation, and oxidative stress has been implicated in the pathophysiology of retinopathy of prematurity (ROP), a leading cause of blindness in pre-term infants. Therapies for ROP include laser and anti-vascular endothelial growth factor agents. However, these therapies have side effects, and even with adequate treatment, visual acuity can be impaired.
View Article and Find Full Text PDFOphthalmic Surg Lasers Imaging Retina
April 2019
Treatment of severe retinopathy of prematurity (ROP) has evolved over the last decade. This article reviews recent clinical trials and experimental evidence that supports clinical outcomes and observations, including the efficacy of anti-vascular endothelial growth factor (VEGF) agents in reducing the vascular activity of severe ROP, and the mechanisms behind recurrent stage 3 ROP and plus disease in some infants treated with anti-VEGF agents. Also discussed will be current imaging modalities that link experimental models of ROP with longitudinal human studies and which provide exciting future opportunities to enhance the understanding of pathophysiology of ROP and improve treatments.
View Article and Find Full Text PDFBackground And Objective: Retinovascular anomalies in the fellow eyes of patients with Coats' disease have been described, but the clinical significance is unknown, as well as whether these lesions progress over time.
Patients And Methods: This is an international, multicenter, retrospective, observational cohort study of fellow-eye abnormalities on widefield fluorescein angiography in patients with Coats' disease.
Results: Three hundred fifty eyes of 175 patients with Coats' disease were analyzed.
Int J Retina Vitreous
June 2018
Background: Ultra-widefield angiography is the latest technology in the evolution of fundus fluorescein angiography. With the ability to capture up to 200° of the fundus in a single image, far peripheral retinal pathology can be imaged. Generally, obtaining high-quality fundus fluorescein angiography in a child without sedation in the outpatient setting is exceedingly challenging.
View Article and Find Full Text PDFPurpose: Intravitreal bevacizumab is increasingly used to treat severe retinopathy of prematurity (ROP), but it enters the bloodstream, and there is concern that it may alter development of other organs. Previously we reported short-term outcomes of 61 infants enrolled in a dose de-escalation study, and we report the late recurrences and additional treatments.
Design: Masked, multicenter, dose de-escalation study.
JAMA Ophthalmol
August 2018
Importance: There are no approved drug treatments for autosomal dominant retinitis pigmentosa, a relentlessly progressive cause of adult and childhood blindness.
Objectives: To evaluate the potential efficacy and assess the safety of orally administered valproic acid (VPA) in the treatment of autosomal dominant retinitis pigmentosa.
Design, Setting, And Participants: Multicenter, phase 2, prospective, interventional, placebo-controlled, double-masked randomized clinical trial.
Erythropoietin (EPO) is recognized for neuroprotective and angiogenic effects and has been associated with aging and neovascular age-related macular degeneration (AMD). We hypothesized that systemic EPO facilitates the development of choroidal neovascularization (CNV). Wild type mice expressed murine EPOR (mWtEPOR) in RPE/choroids at baseline and had significantly increased serum EPO after laser treatment.
View Article and Find Full Text PDFAm J Ophthalmol Case Rep
April 2017
Purpose: To highlight good visual outcome with intravitreal amikacin administered 18 days following trauma-inducing vancomycin resistant enterococcal endophthalmitis treated initially with vitrectomy and oral linezolid.
Observations: Despite initial vitrectomy, intravitreal vancomycin, ceftazidime and oral linezolid, smoldering vitreous infiltrates prompted treatment with intravitreal amikacin 18 days later and restored vision to 20/40 in a vancomycin-resistant traumatic endophthalmitis.
Conclusions And Importance: Good visual outcome was attained with intravitreal injection of amikacin 18 days following penetrating trauma and vancomycin resistant enterococcal endophthalmitis that smoldered following initial treatment of vitrectomy, intravitreal antibiotics and oral linezolid.
Importance: Intravitreous bevacizumab (0.25 to 0.625 mg) is increasingly used to treat type 1 retinopathy of prematurity (ROP), but there remain concerns about systemic toxicity.
View Article and Find Full Text PDFSurv Ophthalmol
September 2017
The understanding, diagnosis, and treatment of retinopathy of prematurity have changed in the 70 years since the original description of retrolental fibroplasia associated with high oxygenation. It is now recognized that retinopathy of prematurity differs in appearance worldwide and as ever smaller and younger premature infants survive. New methods are being evaluated to image the retina, diagnose severe retinopathy of prematurity, and determine windows of time for treatment to save eyes and improve visual and neural outcomes.
View Article and Find Full Text PDFObjective: The purpose of this study was to: (a) describe the extent to which ophthalmologists and glaucoma patients discuss vision quality-of-life during office visits, and (b) examine the association between patient and ophthalmologist characteristics and provider-patient communication about vision quality-of-life.
Methods: Patients with glaucoma who were newly prescribed or on glaucoma medications were recruited at six ophthalmology clinics. Patients' visits were video-tape recorded and quality-of-life communication variables were coded.
Purpose: Medication self-efficacy, or patients' confidence that they can perform medication-related behaviors, is associated with better glaucoma medication adherence. Little is known about how to enhance glaucoma patients' medication self-efficacy. Our purpose is to examine whether patient-provider communication increases glaucoma patients' medication self-efficacy.
View Article and Find Full Text PDFEducation about how to administer eye drops may improve a patient's ability to instill his or her eye drops correctly. Our objectives were to (a) document the methods providers use to educate glaucoma patients about eye drop technique; (b) determine whether eye drop technique education varies by provider and patient characteristics; and (c) evaluate whether education predicts improved patient technique. We conducted an 8-month longitudinal study of 279 glaucoma patients and 15 providers in which we recorded on videotape the content of glaucoma office visits at two time points (baseline and 4- to 6-week follow-up) and videotaped patient eye drop technique at three time points (baseline, 4- to 6-week follow-up, and 8-month follow-up).
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