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Retinal Imaging in Patients With Inherited Retinal Degenerations

This study is currently recruiting participants.
Verified October 2016 by Jacque Duncan, University of California, San Francisco
Sponsor:
ClinicalTrials.gov Identifier:
NCT00254605
First Posted: November 16, 2005
Last Update Posted: October 26, 2016
The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details.
Collaborator:
University of California, Berkeley
Information provided by (Responsible Party):
Jacque Duncan, University of California, San Francisco
  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 Jacque Duncan, University of California, San Francisco:

Primary Outcome Measures:
  • Cone spacing [ Time Frame: 24 months ]
    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 ]
    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 2019
Estimated Primary Completion Date: October 2017 (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

Information from the National Library of Medicine

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Ages Eligible for Study:   13 Years and older   (Child, Adult, Senior)
Sexes Eligible for Study:   All
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
Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00254605


Contacts
Contact: Jacque L. Duncan, M.D. 415-514-4241 jacque.duncan@ucsf.edu
Contact: Arshia Mian, B.S. 415-476-0444 arshia.mian@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    arshia.mian@ucsf.edu   
Contact: Jacque Duncan, MD    415-514-4241    jacque.duncan@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:
Publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):
Zayit-Soudry S, Sippl-Swezey N, Porco TC, Lynch SK, Syed R, Ratnam K, Menghini M, Roorda AJ, Duncan JL. Repeatability of Cone Spacing Measures in Eyes With Inherited Retinal Degenerations. Invest Ophthalmol Vis Sci. 2015 Sep 1;56(10):6179-89. doi: 10.1167/iovs.15-17010.
Wang Q, Tuten WS, Lujan BJ, Holland J, Bernstein PS, Schwartz SD, Duncan JL, Roorda A. Adaptive optics microperimetry and OCT images show preserved function and recovery of cone visibility in macular telangiectasia type 2 retinal lesions. Invest Ophthalmol Vis Sci. 2015 Jan 13;56(2):778-86. doi: 10.1167/iovs.14-15576.
Menghini M, Lujan BJ, Zayit-Soudry S, Syed R, Porco TC, Bayabo K, Carroll J, Roorda A, Duncan JL. Correlation of outer nuclear layer thickness with cone density values in patients with retinitis pigmentosa and healthy subjects. Invest Ophthalmol Vis Sci. 2014 Dec 16;56(1):372-81. doi: 10.1167/iovs.14-15521.
Zayit-Soudry S, Duncan JL, Syed R, Menghini M, Roorda AJ. Cone structure imaged with adaptive optics scanning laser ophthalmoscopy in eyes with nonneovascular age-related macular degeneration. Invest Ophthalmol Vis Sci. 2013 Nov 15;54(12):7498-509. doi: 10.1167/iovs.13-12433.
Ratnam K, Carroll J, Porco TC, Duncan JL, Roorda A. Relationship between foveal cone structure and clinical measures of visual function in patients with inherited retinal degenerations. Invest Ophthalmol Vis Sci. 2013 Aug 28;54(8):5836-47. doi: 10.1167/iovs.13-12557.
Syed R, Sundquist SM, Ratnam K, Zayit-Soudry S, Zhang Y, Crawford JB, MacDonald IM, Godara P, Rha J, Carroll J, Roorda A, Stepien KE, Duncan JL. High-resolution images of retinal structure in patients with choroideremia. Invest Ophthalmol Vis Sci. 2013 Feb 1;54(2):950-61. doi: 10.1167/iovs.12-10707.
Ratnam K, Västinsalo H, Roorda A, Sankila EM, Duncan JL. Cone structure in patients with usher syndrome type III and mutations in the Clarin 1 gene. JAMA Ophthalmol. 2013 Jan;131(1):67-74. doi: 10.1001/2013.jamaophthalmol.2.
Duncan JL, Ratnam K, Birch DG, Sundquist SM, Lucero AS, Zhang Y, Meltzer M, Smaoui N, Roorda A. Abnormal cone structure in foveal schisis cavities in X-linked retinoschisis from mutations in exon 6 of the RS1 gene. Invest Ophthalmol Vis Sci. 2011 Dec 20;52(13):9614-23. doi: 10.1167/iovs.11-8600.
Mkrtchyan M, Lujan BJ, Merino D, Thirkill CE, Roorda A, Duncan JL. Outer retinal structure in patients with acute zonal occult outer retinopathy. Am J Ophthalmol. 2012 Apr;153(4):757-68, 768.e1. doi: 10.1016/j.ajo.2011.09.007. Epub 2011 Nov 20.

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
First Submitted: November 14, 2005
First Posted: November 16, 2005
Last Update Posted: October 26, 2016
Last Verified: October 2016

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

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


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