This is an international, multicenter study with two components:
Registry
* A standardized genetic screening and a prospective, standardized, cross-sectional clinical data collection
* Enrollment is open to all genes on the RD Rare Gene List
Natural History Study
* A prospective, standardized, longitudinal Natural History Study
* Enrollment opens gene-by-gene, based on funding and within-gene Registry enrollment The study objectives are as follows.
Registry Objectives
1. Genotype Characterization
2. Cross-Sectional Phenotype Characterization (within gene)
3. Establish a Link to My Retina Tracker Registry (MRTR)
4. Ancillary Exploratory Studies - Pooling of Genes
Natural History Study Objectives
1. Natural History (within gene)
2. Structure-Function Relationship (within gene)
3. Risk Factors for Progression (within gene)
4. Ancillary Exploratory Studies - Pooling of Genes
Eligibility
Sex
ALL
Min age
4 Years
Max age
—
Healthy volunteers
No
Inclusion Criteria: Participants must meet all the following inclusion criteria at the Registry/Screening Visit to be eligible to enroll into the genetic screening phase:
1. Willing to participate in the study and able to communicate consent during the consent process
2. Willing and able to complete all applicable Registry/Screening Visit assessments
3. Age ≥ 4 years
4. Must have a single gene on the RD Rare Gene List which meets one of the Genetic Screening Criteria below based on a genetic report\* from a clinically certified lab (or from a research lab which has been approved by the study Genetics Committee):
Inheritance Pattern is Recessive and has at least 2 disease-causing variants which are homozygous or heterozygous in trans
OR
Inheritance Pattern is Recessive and has 2 disease-causing variants with unknown phase and meets all the following additional informatic criteria that is consistent with likely segregation in trans:
1. Investigator confirms genotype and phenotype are consistent with autosomal recessive inheritance
2. The 2 disease-causing variants have not been reported in cis in variant databases
3. No additional potentially pathogenic variants were found on the gene (and the sequencing data for the gene were sufficiently robust to detect any additional potentially pathogenic variants)
4. No potentially pathogenic variants were found in other common, likely candidate genes for the proposed condition
OR
Inheritance Pattern is Dominant, X-linked, or Mitochondrial and has at least 1 disease-causing variant
Both eyes must meet the following criteria at the Registry/Screening Visit to enroll into the genetic screening phase:
1. Both eyes must have a clinical diagnosis of retinal dystrophy
2. Both eyes must permit good quality photographic imaging (e.g., but not limited to, clear ocular media, adequate pupil dilation, stable fixation)
Exclusion Criteria:
Participants must not meet any of the following exclusion criteria at the Registry/Screening Visit to be eligible to enroll into the genetic screening phase:
1\. History of more than 1 year of cumulative treatment, at any time, with an agent associated with pigmentary retinopathy including amiodarone, chloroquine, deferoxamine, hydroxychloroquine, pentosan polysulfate, tamoxifen, and deferoxamine Note: Since this is an observational study, pregnant women will not be specifically excluded from participation. However, minors that are pregnant shall be precluded from participation until they become the age of majority.
Ocular Exclusion Criteria:
If either eye has any of the following ocular exclusion criteria at the Registry/Screening Visit, then the participant is not eligible to enroll into the genetic screening phase:
1. Current vitreous hemorrhage
2. Current complications of pathological myopia (for example, but not limited to, myopic maculopathy including atrophy, scar, choroidal neovascularization, schisis) that could inhibit ability to obtain good quality photographic imaging
3. History of intraocular surgery (for example, but not limited to, cataract surgery, vitrectomy, penetrating keratoplasty, or LASIK) within 3 months of Registry/Screening Visit
4. Current or any history of confirmed diagnosis of glaucoma (for example, but not limited to, glaucomatous VF changes or nerve changes, or history of glaucoma filtering surgery)
5. Current or any history of retinal vascular occlusion or proliferative diabetic retinopathy
6. History or current evidence of ocular disease that, in the opinion of the Investigator, may confound assessment of visual function (for example, but not limited to, tractional or rhegmatogenous retinal detachment, any vitreoretinal surgery, retinal vascular occlusion, proliferative diabetic retinopathy)
7. The following medications and treatments are prohibited as they can affect progression of retinitis pigmentosa (RP). The participant must not have received the following treatments:
Any use of ocular stem cell or gene therapy Any treatment with ocriplasmin Treatment with Ozurdex (dexamethasone), Iluvien, or Yutiq (fluocinolone acetonide) intravitreal implant
8. The following medications and treatments are excluded within the specified timeframe:
Treatment with an ophthalmic oligonucleotide within the last 9 months (last treatment date is less than 9 months prior to Registry/Screening Visit date)
Treatment with any other product within five times the expected half-life of the product (time from last treatment date to Registry/Screening Visit date is at least 5 times the half-life of the given product)
Primary outcome measure(s)
Functional Outcome: Characterize change using Visual field sensitivity measured with quantitative topographic analysis (hill of vision [HOV]) — Baseline and every year until study completion (4 years) Measured by Static Perimetry (SP) using Octopus 900 Pro
Functional Outcome: Characterize Change Using Early Treatment of Diabetic Retinopathy Study (ETDRS) / HOTV Best Corrected Visual Acuity (BCVA) letter score — Baseline and every year until study completion (4 years) Measured by Electronic Visual Acuity (EVA) system or ETDRS/HOTV charts
Functional Outcome: Characterize Change Using Low visual acuity test - for participants unable to see ETDRS letters — Baseline and every year until study completion (4 years) Measured by Berkeley Rudimentary Vision Test (BRVT) for Low Visual Acuity
Functional Outcome: Characterize Change Using ETDRS/HOTV best corrected low luminance visual acuity letter score — Baseline and every year until study completion (4 years) Measured by Electronic Visual Acuity (EVA) system or ETDRS/HOTV charts
Functional Outcome: Characterize Change in Mean retinal sensitivity — Baseline and every year until study completion (4 years) Measured by Fundus guided Microperimetry (MP) using MAIA
Functional Outcome: Characterize Change in Contrast sensitivity function — Baseline and every year until study completion (4 years) Measured by Contrast sensitivity CSV-1000E chart
Functional Outcome: Characterize Change in Retinal function using amplitudes and timing in response to rod- and cone-specific stimuli — Baseline and at study completion (4 years) Measured by Full-field Electroretinogram (ffERG) Diagnosys Espion
Functional Outcome: Characterize Change in Full-field retinal sensitivity — Baseline and every year until study completion (4 years) Measured by Full-field stimulus threshold (FST) testing to blue, white, and red stimuli using Diagnosys Espion
Functional Outcome: Characterize Change in Color vision function — Baseline and every year until study completion (4 years) Measured by Color vision testing using Lanthony D15
Structural Outcome: Characterize Change in Ellipsoid zone (EZ) area; outer nuclear layer and ganglion cell layer thicknesses — Baseline and every year until study completion (4 years) Measured by Spectral Domain Optical Coherence Tomography (SD-OCT) using Heidelberg Spectralis
Structural Outcome: Characterize Change Using Qualitative and quantitative assessments of autofluorescence pattern — Baseline and every year until study completion (4 years) Measured by Fundus Autofluorescence (FAF) using Optos
Trial sites (36)
Facility
City
Region
Status
University of Arkansas, Jones Eye Institute
Little Rock
Arkansas
Recruiting
USC Roski Eye Institute
Los Angeles
California
Recruiting
University of California San Francisco
San Francisco
California
Recruiting
University of Florida Health Jacksonville
Jacksonville
Florida
Recruiting
University of Miami, Bascom Palmer Eye Institute
Miami
Florida
Recruiting
Emory University, Emory Eye Center
Atlanta
Georgia
Recruiting
Johns Hopkins University, Wilmer Eye Institute
Baltimore
Maryland
Recruiting
Harvard Univ., Massachusetts Eye and Ear Infirmary
Boston
Massachusetts
Recruiting
University of Michigan, Kellogg Eye Center
Ann Arbor
Michigan
Recruiting
Mayo Clinic
Rochester
Minnesota
Recruiting
Duke University, Duke Eye Center
Durham
North Carolina
Recruiting
Oregon Health & Science Univ., Casey Eye Institute
Portland
Oregon
Recruiting
University of Pennsylvania, Scheie Eye Institute
Philadelphia
Pennsylvania
Recruiting
UPMC Eye Center
Pittsburgh
Pennsylvania
Recruiting
Retina Foundation of the Southwest
Dallas
Texas
Recruiting
Baylor College of Medicine, Alkek Eye Center
Houston
Texas
Recruiting
University of Utah, John Moran Eye Center
Salt Lake City
Utah
Recruiting
University of Wisconsin Madison
Madison
Wisconsin
Recruiting
Medical College of Wisconsin Eye Institute
Milwaukee
Wisconsin
Recruiting
Centre for Eye Research Australia
East Melbourne
Victoria
Recruiting
Ghent University
Ghent
Belgium
Not Yet Recruiting
INRET Clínica e Centro de Pesquisa
Belo Horizonte
Minas Gerais
Recruiting
Instituto de Genética Ocular
São Paulo
São Paulo Province
Recruiting
University of Alberta and Alberta Health Services
Edmonton
Alberta
Recruiting
University of Toronto, Hospital for Sick Children
Toronto
Ontario
Recruiting
University Health Network
Toronto
Canada
Recruiting
Helsinki University Hospital
Helsinki
Finland
Recruiting
CHNO des Quinze-Vingts
Paris
France
Recruiting
Hadassah-Hebrew University Medical Center
Jerusalem
Israel
Recruiting
Vista Vision Eye Clinic
Brescia
Italy
Recruiting
Retina and Genomics Institute
Yucatán
Merida
Recruiting
Radboud University Medical Center
Nijmegen
Netherlands
Recruiting
Oslo University Hospital
Oslo
Norway
Recruiting
University Hospital Basel
Basel
Canton of Basel-City
Recruiting
University Hospital Jules-Gonin
Lausanne
Switzerland
Recruiting
Moorfields Eye Hospital
London
United Kingdom
Recruiting
More Jaeb Center for Health Research trials in Italy
This page summarises publicly available registry data for informational purposes — not medical advice. Eligibility is determined by each study team; patients should discuss participation with their clinician.
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