Universal Rare Gene Study: A Registry and Natural History Study of Retinal Dystrophies Associated With Rare Disease-Causing Genetic Variants
Start Date
5/11/2023
Completion Date
12/15/2030
Summary
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
Detailed Description
This study includes multiple phases. 1. Screening Phase The patient's current genetic report will be reviewed. Genetic testing will not be performed in this study. A prior conclusive genetic test will be assessed for screening analysis. Having at least one gene on the RD Rare Gene List meets one of the eligible Genetic Screening Criteria and other eligibility criteria can be evaluated based on medical history. 2. Genetic Screening Phase: Genetic reports for participants enrolled into the genetic screening phase will be uploaded to study website for review and confirmation by Central Genetics Auditor (CGA) as meeting Genetic Screening Criteria.Participants confirmed as meeting those criteria will be considered enrolled into the Registry. 3. Registry Phase: The flow of participants who are enrolled into the Registry depends on whether their causal gene is designated as a Natural History Study (NHS) Target Gene. If they are not Designated as NHS Target Gene, they will receive annual phone calls up to 48 months from the Registry/Screening visit or until the gene is designated as NHS Target Gene. If they are Designated as NHS Target Gene participants will be considered pending enrollment into the NHS. The Registry will establish genetically and clinically well-characterized cohorts of patients across hundreds of genetic variants associated with retinal dystrophy (RD). Characterization of these patients will accelerate eligibility screening for the Natural History Study, provide cross-sectional data on phenotype-genotype associations, and contribute to our knowledge of pathogenicity of these rare disease-causing variants. 4. Natural History Study (NHS) Phase Participants pending enrollment will return to the clinic for the NHS Enrollment/Baseline Visit and return to the clinic for follow-up visits. The Natural History Study will accelerate the identification and development of sensitive, reliable outcome measures for clinical trials, which will facilitate development of treatments for retinal dystrophies due to disease-causing genetic variants. The expected impact of the Natural History Study is as follows: 1. Describe the natural history of retinal degeneration in patients with rare disease-causing genetic variants 2. Identify sensitive structural and functional outcome 3. Identify well-defined subpopulations for future clinical trials of investigative treatments for rare inherited retinal degeneration
Eligibility Criteria
Age Range: 4 years to No maximum
Conditions
Locations
University of Arkansas, Jones Eye Institute
Little Rock, Arkansas 72205
United States
USC Roski Eye Institute
Los Angeles, California 90033
United States
University of California San Francisco
San Francisco, California 94158
United States
University of Florida Health Jacksonville
Jacksonville, Florida 32209
United States
University of Miami, Bascom Palmer Eye Institute
Miami, Florida 33136
United States
Emory University, Emory Eye Center
Atlanta, Georgia 30322
United States
Johns Hopkins University, Wilmer Eye Institute
Baltimore, Maryland 21236
United States
Harvard Univ., Massachusetts Eye and Ear Infirmary
Boston, Massachusetts 02114
United States
University of Michigan, Kellogg Eye Center
Ann Arbor, Michigan 48105
United States
Mayo Clinic
Rochester, Minnesota 55905
United States
Duke University, Duke Eye Center
Durham, North Carolina 27705
United States
Oregon Health & Science Univ., Casey Eye Institute
Portland, Oregon 97239
United States
University of Pennsylvania, Scheie Eye Institute
Philadelphia, Pennsylvania 19104
United States
UPMC Eye Center
Pittsburgh, Pennsylvania 15213
United States
Retina Foundation of the Southwest
Dallas, Texas 75231
United States
Baylor College of Medicine, Alkek Eye Center
Houston, Texas 77030
United States
University of Utah, John Moran Eye Center
Salt Lake City, Utah 84132
United States
University of Wisconsin Madison
Madison, Wisconsin 53711
United States
Medical College of Wisconsin Eye Institute
Milwaukee, Wisconsin 53226
United States
Centre for Eye Research Australia
East Melbourne, Victoria
Australia
Ghent University
Ghent,
Belgium
INRET Clínica e Centro de Pesquisa
Belo Horizonte, Minas Gerais 30150-270
Brazil
Instituto de Genética Ocular
São Paulo, São Paulo Province
Brazil
University of Alberta and Alberta Health Services
Edmonton, Alberta
Canada
University of Toronto, Hospital for Sick Children
Toronto, Ontario M5G 2L3
Canada
University Health Network
Toronto, M5T 2S8
Canada
Helsinki University Hospital
Helsinki, 00100
Finland
CHNO des Quinze-Vingts
Paris, 75012
France
Hadassah-Hebrew University Medical Center
Jerusalem, 9112001
Israel
Vista Vision Eye Clinic
Brescia, 25123
Italy
Retina and Genomics Institute
Yucatán, Merida
Mexico
Radboud University Medical Center
Nijmegen, 6525 GA
Netherlands
Oslo University Hospital
Oslo,
Norway
University Hospital Basel
Basel, Canton of Basel-City
Switzerland
University Hospital Jules-Gonin
Lausanne, 1004
Switzerland
Moorfields Eye Hospital
London, EC1V 2PD
United Kingdom