Retinal Imaging in Patients With Inherited Retinal Degenerations

This study is currently recruiting participants. (see Contacts and Locations)
Verified August 2014 by University of California, San Francisco
Sponsor:
Collaborator:
University of California, Berkeley
Information provided by (Responsible Party):
Jacque Duncan, University of California, San Francisco
ClinicalTrials.gov Identifier:
NCT00254605
First received: November 14, 2005
Last updated: August 14, 2014
Last verified: August 2014
  Purpose

The purpose of this study is to determine whether the structure and function of the human retina can be studied with high resolution in patients with inherited retinal degenerations using the Adaptive Optics Scanning Laser Ophthalmoscope (AOSLO).


Condition Phase
Retinitis Pigmentosa
Phase 1

Study Type: Observational
Study Design: Observational Model: Case Control
Time Perspective: Prospective
Official Title: High Resolution Retinal Imaging in Patients With Inherited Retinal Degenerations

Resource links provided by NLM:


Further study details as provided by University of California, San Francisco:

Primary Outcome Measures:
  • Cone spacing [ Time Frame: 24 months ] [ Designated as safety issue: No ]
    The current study will assess cone spacing twice at baseline and every 6 months for 30 months. The primary outcome will be measured at 24 months.


Secondary Outcome Measures:
  • Visual acuity [ Time Frame: 24 months ] [ Designated as safety issue: No ]
    Visual acuity will be measured every 6 months for 30 months with the primary outcome measure at 24 months.


Estimated Enrollment: 400
Study Start Date: November 2005
Estimated Study Completion Date: November 2016
Estimated Primary Completion Date: October 2016 (Final data collection date for primary outcome measure)
Detailed Description:

Retinal degenerations are a group of inherited diseases that result in progressive death of the vision cells, or photoreceptors. Currently there is no treatment or cure for any of these diseases and they ultimately cause blindness in affected patients. We propose to investigate the structure and function of the human retina in patients with inherited retinal degenerations using the Adaptive Optics Scanning Laser Ophthalmoscope (AOSLO). We will correlate the images of retinal structure produced by the AOSLO with Optical Coherence Tomography (OCT) images of the retina. In addition, we will study the vision of individual photoreceptors using the AOSLO to perform a novel technique, microperimetry, in patients with retinal degenerations. We will compare the results of microperimetry with standard measures of vision used in Ophthalmology clinics, including visual acuity, automated perimetry, fundus photography and multifocal electroretinography (mfERG).

The results of this work will provide insight into the mechanism of vision loss among patients with diverse retinal disorders. Specifically, we will study cone structure and function in patients with retinal degenerations with different etiologies: retinitis pigmentosa, a disease usually caused by rod-specific mutations; cone-rod dystrophy, which primarily affects cones rather than rods; and Best's disease, a disease caused by a defect in the retinal pigment epithelium (RPE). In addition, we will study the effect that lipofuscin, a byproduct of photoreceptor metabolism that accumulates in the RPE in diseases such as Stargardt's disease, Best's disease and age-related macular degeneration (AMD), has on cone structure and function, with the goal of understanding how these diseases cause blindness. Better understanding of the mechanisms of vision loss in patients with retinal degeneration should ultimately lead to treatments for these blinding conditions.

  Eligibility

Ages Eligible for Study:   13 Years and older
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population

Patients with inherited retinal degenerations, including retinitis pigmentosa, choroideremia, x-linked retinoschisis and Usher syndrome. We are also studying a small number of patients with age-related macular degeneration.

Criteria

Inclusion Criteria:

  • Subjects must speak and understand English
  • Subjects must have pupils that dilate to at least 6 millimeters diameter.
  • Subjects must be willing to travel to UC Berkeley.
  • Subjects are financially responsible for their travel to the San Francisco area if they are not San Francisco residents.

Exclusion Criteria:

  • Cataract
  • Irregular corneal astigmatism (keratoconus)
  • Prior refractive surgery
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00254605

Contacts
Contact: Jacque L. Duncan, M.D. 415-514-4241 duncanj@vision.ucsf.edu
Contact: Arshia Mian, B.S. 415-476-0444 Miana@vision.ucsf.edu

Locations
United States, California
Department of Ophthalmology Retinal Degenerations Clinic, UCSF Recruiting
San Francisco, California, United States, 94143
Contact: Arshia Mian, B.S.    415-476-0444    MianA@vision.ucsf.edu   
Contact: Jacque Duncan, MD    415-514-4241    duncanj@vision.ucsf.edu   
Sponsors and Collaborators
University of California, San Francisco
University of California, Berkeley
Investigators
Principal Investigator: Jacque L. Duncan, M.D. University of California, San Francisco
  More Information

Publications:
Additional publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):

Responsible Party: Jacque Duncan, Professor, Clinical Ophthalmology, University of California, San Francisco
ClinicalTrials.gov Identifier: NCT00254605     History of Changes
Other Study ID Numbers: H12225-27221-0+1
Study First Received: November 14, 2005
Last Updated: August 14, 2014
Health Authority: United States: Institutional Review Board

Keywords provided by University of California, San Francisco:
imaging
adaptive optics scanning laser ophthalmoscope
optical coherence tomography
electroretinography

Additional relevant MeSH terms:
Retinal Degeneration
Retinitis Pigmentosa
Eye Diseases
Eye Diseases, Hereditary
Genetic Diseases, Inborn
Retinal Diseases
Retinal Dystrophies

ClinicalTrials.gov processed this record on October 23, 2014