Summary

Eligibility
for people ages 13 years and up (full criteria)
Healthy Volunteers
healthy people welcome
Location
at UCSF
Dates
study started
completion around
Principal Investigator
by Jacque L. Duncan, M.D. (ucsf)
Headshot of Jacque L. Duncan
Jacque L. Duncan

Description

Summary

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).

Official Title

High Resolution Retinal Imaging in Patients With Inherited Retinal Degenerations

Details

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.

Keywords

Retinitis Pigmentosa, imaging, adaptive optics scanning laser ophthalmoscope, optical coherence tomography, electroretinography, Retinitis, Retinal Degeneration

Eligibility

You can join if…

Open to people ages 13 years and up

  • 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 University of California (UC) Berkeley.
  • Subjects are financially responsible for their travel to the San Francisco area if they are not San Francisco residents.

You CAN'T join if...

  • Cataract
  • Irregular corneal astigmatism (keratoconus)
  • Prior refractive surgery

Location

  • Department of Ophthalmology Retinal Degenerations Clinic, UCSF accepting new patients
    San Francisco California 94143 United States

Lead Scientist at University of California Health

  • Jacque L. Duncan, M.D. (ucsf)
    Retinitis pigmentosa (RP) affects about 1 in 3,500 people worldwide. Age-related macular degeneration (AMD) affects as many as 1 in 4 people by the age of 75 and is the leading cause of blindness in people over age 50 in the United States. Both RP and AMD have a hereditary basis, and currently, there is no cure for either of these types of hereditary retinal degeneration.

Details

Status
accepting new patients
Start Date
Completion Date
(estimated)
Sponsor
University of California, San Francisco
ID
NCT00254605
Study Type
Observational
Participants
Expecting 400 study participants
Last Updated