Retinal Imaging in Patients With Inherited Retinal Degenerations

Retinitis Pigmentosa Clinical Trial

Official title:

High Resolution Retinal Imaging in Patients With Inherited Retinal Degenerations

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT00254605
• Other study ID #: H12225-27221-0+1
• Status: Recruiting
• Start date: November 2005
• Est. completion date: November 2028

Study information

• Verified date: November 2023
• Source: University of California, San Francisco
• Contact: Jacque L. Duncan, M.D.
• Phone: 415-514-4241
• Email: jacque.duncan[@]ucsf.edu
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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).

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 400
• Est. completion date: November 2028
• Est. primary completion date: October 2028
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 13 Years and older

Eligibility

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 University of California (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

Related Conditions & MeSH terms

• Retinal Degeneration
• Retinitis
• Retinitis Pigmentosa

Locations

Country	Name	City	State
United States	Department of Ophthalmology Retinal Degenerations Clinic, UCSF	San Francisco	California

Sponsors (2)

Lead Sponsor	Collaborator
University of California, San Francisco	University of California, Berkeley

Country where clinical trial is conducted

United States, 

References & Publications (3)

Duncan JL, Zhang Y, Gandhi J, Nakanishi C, Othman M, Branham KE, Swaroop A, Roorda A. High-resolution imaging with adaptive optics in patients with inherited retinal degeneration. Invest Ophthalmol Vis Sci. 2007 Jul;48(7):3283-91. doi: 10.1167/iovs.06-142 — View Citation

Roorda A, Zhang Y, Duncan JL. High-resolution in vivo imaging of the RPE mosaic in eyes with retinal disease. Invest Ophthalmol Vis Sci. 2007 May;48(5):2297-303. doi: 10.1167/iovs.06-1450. — View Citation

Yoon MK, Roorda A, Zhang Y, Nakanishi C, Wong LJ, Zhang Q, Gillum L, Green A, Duncan JL. Adaptive optics scanning laser ophthalmoscopy images in a family with the mitochondrial DNA T8993C mutation. Invest Ophthalmol Vis Sci. 2009 Apr;50(4):1838-47. doi: 1 — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Cone spacing	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.	24 months
Secondary	Visual acuity	Visual acuity will be measured every 6 months for 30 months with the primary outcome measure at 24 months.	24 months