Correlations of questionnaire scores with objective measures will be analyzed. Multifocal electroretinography (mfERG) will be analyzed by subfield and possible ring analyses. Functional testing with photopic perimetry and scoptopic perimetry and kinetics will be quantified. Macular thickness as measured by optical coherence tomography (OCT), as well as ellipsoid zone band length will be quantified. Secondary outcome measures will include analysis of parameter testing based on severity groups. Outcome Measures: The primary outcome measure will be the limits of agreement in repeatability calculations of the tests performed. The goal of Aim 4 is to survey the cytokine/lymphokine profile. The goal of Aim 3 is to assess the participant s performance on questionnaires assessing visual function. The goal of Aim 2 is to evaluate structural measures for RP. The goal of Aim 1 is to identify measures that could be used in future studies to track the extent of functional retina over time. Up toģ0 healthy volunteers will also be enrolled for a total accrual ceiling of 150.ĭesign: This is a single center, observational, cross-sectional repeatability study of patients with retinitis pigmentosa. Study Population: Up to 120 participants with a diagnosis of RP will be enrolled. Developing these measures has the potential to advance the field by elucidating the process of photoreceptor degeneration as well as being a scaffold for which candidate therapies could be trialed.
Employing new imaging methods to visualize and analyze structural changes in the retina will allow for the evaluation of structural changes that underlie disease progression.
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Correlation of novel spatial functional maps with other functional measures (such as visual acuity and multifocal electroretinography) will help provide a full description of functional change.
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Objective: The objective of this study is to investigate the nature of photoreceptor dysfunction in retinitis pigmentosa (RP) patients using focal static and kinetic psychophysical tests to develop functional outcome measures for the clinical trial study in RP. Sponsoring Institute: National Eye Institute Condition or disease After the study ends, they may also be asked to give consent for researchers to continue to use their study information. Minors will give written consent to stay in the study when they turn 18. A small electrode taped to their forehead will record signals from their retinas. Then they will take eye-numbing drops and wear contacts as lights flash. Look into a bowl and press a button when they see lights. View a bright background then press a button when they see lights. Sit in a dark room and press a button when they see lights. Participants will take several eye tests. Pictures of participants retinas will be taken. Their pupils will be dilated with eye drops. Participants will give their medical and eye history. Each visit will last 5 6 hours, or a visit can be split into 2 days. Participants will have 2 visits about 6 weeks apart. Participants will be screened in another protocol. To find out how well certain tests track changes in retinitis pigmentosa. Researchers want to see how well these tests capture stages of RP. There is no cure, butut treatment and diagnosis can be guided by certain tests. RP affects about 2 million people globally. It is caused mostly by mutations in photoreceptor-expressed genes. Retinitis pigmentosa (RP) is a group of blinding eye diseases.
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