UNI-RARE

Universal Rare Gene Study: A Registry and Natural History Study of Retinal Dystrophies Associated with Rare Disease-Causing Genetic Variants

  • Sponsor: Jaeb Center for Health Research
  • Principal Investigator: Kimberly Stepien
  • Study Coordinator: Nickie Stangel

Study Objective:

To 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.

Study Design:

The Universal Registry is open to rare RD genes, to cross-sectionally characterize patients within rare RD genes (mild, moderate, and severe vision loss), so they are ready to be enrolled into a subsequent universal longitudinal natural history study as their gene is selected.

Inclusion Criteria:

  • Age ≥ 4 years
  • Must have a single gene on the RD Rare Gene List which meets one of the Genetic Screening Criteria from a clinically certified lab (or from a research lab which has been approved by the study Genetics Committee)

Exclusion Criteria:

  • 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
  • Current vitreous hemorrhage
  • 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
  • History of intraocular surgery (for example, but not limited to, cataract surgery, vitrectomy, penetrating keratoplasty, or LASIK) within 3 months of Registry/Screening Visit
  • 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)
  • Current or any history of retinal vascular occlusion or proliferative diabetic retinopathy
  • 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:
  1. Any use of ocular stem cell or gene therapy Any treatment with ocriplasmin Treatment with Ozurdex (dexamethasone), Iluvien, or Yutiq (fluocinolone acetonide) intravitreal implant

 

Contact Nickie Stangel with questions, (608) 263-8783

For more information about this study, visit clinicaltrials.gov: Universal Rare Gene Study: A Registry and Natural History Study of Retinal Dystrophies Associated With Rare Disease-Causing Genetic Variants – Full Text View – ClinicalTrials.gov