A Natural History of the Progression of Stargardt Disease: Retrospective and Prospective Studies...
Stargardt DiseaseStargardt disease is currently an incurable and untreatable macular dystrophy that causes severe visual loss in children and young adults, thereby causing enormous morbidity with economic, psychological, emotional, and social implications. There are no FDA approved therapeutic treatments for this disease. Therefore, the objective of this study is to collect natural history data from a large population of children and adults in order to evaluate possible efficacy measures for planned clinical trials. Participants will be recruited from each Investigator's own patient population as the study requires the availability of both multiyear retrospective data, as well as ongoing prospectively collected data. A concurrent ancillary study (SMART study) is also being conducted with a subset of the prospective study patients during their regular ProgSTAR study visits to expand the collection of retinal images to include microperimetry measurements gathered under scotopic (low light) conditions.
Macular Pigment Density Evolution in Unilateral Wet AMD Versus Non AMD Patients With or Without...
Exudative Age-related Macular Degeneration•Purpose: Age-related Macular Degeneration ( AMD) is the leading cause of blindness and visual impairment in industrialized countries. The macular pigment, composed of carotenoid derivatives (lutein and zeaxanthin), may play an important role in the occurrence of AMD. An increase in macular pigment following dietary supplementation with lutein and zeaxanthin could allow early treatment with such supplements in subjects with a high risk of AMD, and encourage them to change their eating habits. •Primary outcome: Comparative analysis of the density and evolution of the density of macular pigment: In patients without any retinal pathology who underwent cataract surgery 1 month previously In the non-exudative eye of patients with exudative AMD in one eye by analyzing the density and evolution of the density of macular pigment in the non-exudative eye Secondary outcomes: Analysis of changes in macular pigment density after taking food supplements (Nutrof Total versus comparator): Time taken to reach the maximum plateau of macular pigment density (no increase in density between 2 measurements) Time taken to return to the baseline macular pigment density after cessation of supplementation Study design : Pilot study -Prospective, randomised, double-masked, comparative, multicenter.
Development of Core Outcomes for Age-related Macular Degeneration (AMD) Interventions- Caregivers'...
Age Related Macular DegenerationThis study will explore experience of AMD caregivers in order to develop a core outcome set (COS) for age related macular degeneration (AMD) randomised controlled trials (RCTs) trying to capture what research outcomes are important from their perspective. People 18 years of age and older who have been AMD caregivers for at least 6 months may be eligible for this study. The aim is to conduct three focus groups lasting approximately one hour. The plan is to enrol 18-24 participants (6-8 participants per each of 3 focus groups).Two researchers will be involved in conducting the focus groups. A moderator will ensure fluid discussion, while the second investigator will be taking notes and audio-recording the discussion.
Generation of Induced Pluripotent Stem (iPS) Cell Lines From Skin Fibroblast Cells of Participants...
AMDBackground: Age-related macular degeneration (AMD) is the leading cause of blindness in the United States. Currently, there is no safe way to obtain cells from the eye to study. But researchers now can turn other types of cells, like skin or blood, into induced pluripotent stem (iPS) cells. These can be grown in a lab and turned into other types of cells, like cells from the eye. This will allow researchers to understand and treat diseases of the eye such as AMD. Objectives: To establish a bank of samples that can be changed into other cell types, such as eye cells, to better understand diseases such as AMD. Also to test drugs in order to treat various eye diseases. Eligibility: People who provided DNA samples in another protocol (07-EI-0025) Design: Participants will be screened with their data from the previous protocol. Participants with select genetic variants will be chosen and contacted via phone. Participants will have a punch skin biopsy. The skin will be washed. A numbing medication will be injected. A small piece of skin will be removed with a biopsy tool. The site will be covered with a dressing. They will receive instructions on how to care for the area. They will have follow-up visits if needed for clinical care for the area. Participants may be asked to return if their first sample did not provide enough cells for the lab. Participants sample will be developed into eye cells. The cells will be used to understand diseases and test new drugs.
Evaluate the Effectiveness of Intravitreal (IVT) Aflibercept Injection in Clinical Practice in naïve...
Macular DegenerationThere is a need to investigate the effectiveness of IVT aflibercept injection in routine clinical practice. The aim of this study is to collect 12-month real-world clinical data from treatment-naïve nAMD patients who started first-line treatment with IVT aflibercept injection, according to the SmPC (Summary of Product Characteristics) and the SERV (Spanish Society of Retina and Vitreous) guidelines. The effectiveness of IVT aflibercept will be assessed by the changes in VA (Visual Acuity) and CRT (Central Retinal Thickness) during treatment.
The Potential Role of Dietary Antioxidant Capacity on Age-Related Macular Degeneration
Age Related Macular DegenerationIncluding eye health, nutrition plays a vital role for the sustainability of individuals health life. There is an increasing global concern about the issues related with eye health. In 2010, in order to take attention to these issues, World Health Organization (WHO) defined the main reasons of vision disorders as refraction defects of eye diseases (43 %), cataract (33 %), glaucoma (2 %), age-related macular degeneration (AMD) (1 %), diabetic retinopathy (1 %) and undetermined natural reasons (18 %). This report also stated the three biggest reasons of blindness as cataract (51 %), glaucoma (8 %) and AMD (5 %). AMD is a multi-factorial disease in which the genetic predisposition plays important role with environmental factors and metabolic conditions, except for age. Especially cigarette is the secondary important risk factor for dry-type AMD. In the Age Related Eye Disease Study (AREDS), it was stated that AMD prevalence is higher in white races, compared with races which are not white. At the same time, AREDS searched the effect of diet supplements on the progression of AMD disease. In terms of the patients followed for six years, it was reported that the formulation C and E vitamins, beta carotene and zinc decreased the progression risk of AMD from middle levels to advanced levels by 34 %. In AREDS-2 study completed in 2012, it was shown that the extraction of beta carotene from the formulation and the decrease of zinc did not affect the progression rate of the disease. In the group using beta carotene, including persons who were used to smoke but gave up at least one year ago, the rate of becoming lung cancer was observed as substantially high. Moreover, the use of lutein and zeaxanthin instead of beta carotene in the formula did not increase the risk of lung cancer. In addition, it was shown that omega-3 fatty acids did not decrease risk progression. In current data, the effect of the intake of carotenoid and antioxidant increased with diet on AMD is not coherent. Likewise, the epidemiological evidences about the relation between diet fat intake and AMD are contradictory. The consumption of fatty fish is related with increased poly-unsaturated fatty acid intake and decreases the risk of AMD. However, it was reported the high rate of total fat intake in other studies as risk factor for AMD. In another study, there was not any important relation found between diet fat intake and AMD occurrence after the correction of other variables. In the interventional AREDS-2 study, it was reported that the additional intake of long-chain omega-3 poly-unsaturated fatty acids did not have any beneficial effect. The pathophysiological mechanism responsible from the possible relation between obesity and AMD is not clearly known. There are various hypotheses about how obesity causes AMD. In the first hypothesis, obesity can cause AMD after obesity increases systematic oxidative stress. In the second hypothesis, obesity can play a role in AMD pathophysiology as the cause of hyperleptinemia. The studies also prescribed that inflammation could play a role in the progression of AMD and also showed that plasma fibrinogen and other inflammation indicators could be related with late AMD. In Pathologies Oculaires Liées à l'Age (POLA) study made with the participation of many Europeans, it was observed that the progression of late AMD increased by two times in obese individuals and in early AMD, obesity did not affect the progression of disease. In the treatment of this disease which have age-related progression, proper nutrition, vitamin/mineral/supplement usage and the development of precautionary strategies play an important role. When compared with Body Mass Index (BMI), it was found that the measure of abdominal obesity (waist/hip rate and waist circumference) was the better determinant of chronic diseases such as diabetics and cardiovascular disease. Some evidences in the United States of America indicated that the relation between waist/hip rate and AMD gave stronger results when compared with the relation between BMI and AMD. For middle age cohort, after six years of follow-up, a group of researchers reported that the decrease of waist/hip rate also decreased the risk of AMD and the results were the same for waist circumference, even if the evidences were weak. In another study, it was reported that the increase of waist/hip rate or waist circumference also increased the progression of AMD. This study was planned with the aim of determining the occurrence of AMD by evaluating dietary total antioxidant capacity, diet components and some anthropometric measures of individuals having age related macular degeneration (AMD). In the study, the possible effect of nutrition on the occurrence of disease was evaluated by comparing healthy individuals with dietary total antioxidant capacity and some anthropometric measures of individuals with AMD.
Effect of Aflibercept on Human Corneal Endothelial Cells in Neovascular Age-Related Macular Degeneration...
Age Related Macular DegenerationAflibercept is the most recently developed VEGF inhibitor with a recombinant fusion protein consisting of human VEGF receptor extracellular domains from receptors 1 and 2 (VEGFR1 and VEGFR2) fused to the Fc domain of human IgG. Although both ranibizumab and bevacizumab have been shown not to have harmful effects on corneal endothelium, the effect of intravitreal aflibercept on human corneal endothelium has not been reported so far. Considering the functional importance of the corneal endothelium, particularly in aged population, the present study was designed to evaluate the in vivo toxicity of aflibercept on human corneal endothelial cells in patients with neovascular AMD. This study showed that intravitreal injection of clinically effective doses of aflibercept for four times on average during the 6-month period do not induce any harmful effect on human corneal endothelium evaluated by specular microscopy. Further prospective, large-scale, prolonged studies are needed to confirm that intravitreal aflibercept can be used safely without any corneal toxicity to treat neovascular AMD.
Assessment of Psychological Strain and Perioperative Fear in Intravitreal Injections
Age-related Macular DegenerationStressIntravitreal injection can induce perioperative stress for the patients. Different factors can modulate the pre-operative fear and physiologic reaction.
Choroidal Blood Flow and Progression of Age-Related Macular Degeneration in the Fellow Eye in Patients...
Regional Blood FlowAge-related macular degeneration (AMD) is the chief cause of severe and irreversible loss of vision in developed countries. The prevalence of AMD increases dramatically with age. The early stage (or dry AMD) is associated with minimal visual impairment and is characterized by large drusen and pigmentary abnormalities in the macula. The late stage is a neovascular, exudative form. This so called exudative AMD includes serous or hemorrhagic detachment of retinal pigment epithelium and choroidal neovascularization leading to severe loss of vision (20/200 or worse). Patients with unilateral CNV (choroidal neovascularisation) have a significant risk of CNV developing in the second eye. Choroidal blood flow is of great importance for normal visual function. Several reports have provided evidence suggesting that choroidal blood flow is decreased in subjects with AMD. In late stages of AMD angiogenesis leads to the formation of choroidal neovascularization that can cause severe visual impairment by disrupting normal macular function. The purpose of this evaluation is to investigate a possible link between alterations in choroidal blood flow and the development of CNV and serous detachment in the fellow eye of patients with AMD and unilateral neovascular maculopathy. This longitudinal study may provide important findings with respect to natural history and visual prognosis of patients with neovascularized AMD. Ocular blood flow will be determined by non-invasive methods, including laser Doppler flowmetry and laser interferometry
Long-Term Use Of Pegaptanib In Patients For Subfoveal Choroidal Neovascularization Secondary To...
Macular DegenerationThe objective of this surveillance is to collect information about 1) adverse drug reaction not expected from the LPD (unknown adverse drug reaction), 2) the incidence of adverse drug reactions in this surveillance, and 3) factors considered to affect the safety and/or efficacy of this drug.