| Normal central retinal function and structure preserved in retinitis pigmentosa. | |
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MedLine Citation:
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PMID: 19797198 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE: To determine whether normal function and structure, as recently found in forms of Usher syndrome, also occur in a population of patients with nonsyndromic retinitis pigmentosa (RP). METHODS: Patients with simplex, multiplex, or autosomal recessive RP (n = 238; ages 9-82 years) were studied with static chromatic perimetry. A subset was evaluated with optical coherence tomography (OCT). Co-localized visual sensitivity and photoreceptor nuclear layer thickness were measured across the central retina to establish the relationship of function and structure. Comparisons were made to patients with Usher syndrome (n = 83, ages 10-69 years). RESULTS: Cross-sectional psychophysical data identified patients with RP who had normal rod- and cone-mediated function in the central retina. There were two other patterns with greater dysfunction, and longitudinal data confirmed that progression can occur from normal rod and cone function to cone-only central islands. The retinal extent of normal laminar architecture by OCT corresponded to the extent of normal visual function in patients with RP. Central retinal preservation of normal function and structure did not show a relationship with age or retained peripheral function. Usher syndrome results were like those in nonsyndromic RP. CONCLUSIONS: Regional disease variation is a well-known finding in RP. Unexpected was the observation that patients with presumed recessive RP can have regions with functionally and structurally normal retina. Such patients will require special consideration in future clinical trials of either focal or systemic treatment. Whether there is a common molecular mechanism shared by forms of RP with normal regions of retina warrants further study. |
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Authors:
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Samuel G Jacobson; Alejandro J Roman; Tomas S Aleman; Alexander Sumaroka; Waldo Herrera; Elizabeth A M Windsor; Lori A Atkinson; Sharon B Schwartz; Janet D Steinberg; Artur V Cideciyan |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2009-09-24 |
Journal Detail:
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Title: Investigative ophthalmology & visual science Volume: 51 ISSN: 1552-5783 ISO Abbreviation: Invest. Ophthalmol. Vis. Sci. Publication Date: 2010 Feb |
Date Detail:
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Created Date: 2010-01-19 Completed Date: 2010-02-17 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7703701 Medline TA: Invest Ophthalmol Vis Sci Country: United States |
Other Details:
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Languages: eng Pagination: 1079-85 Citation Subset: IM |
Affiliation:
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Department of Ophthalmology, Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA. jacobsos@mail.med.upenn.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Aged Aged, 80 and over Child Color Vision / physiology Contrast Sensitivity / physiology* Dark Adaptation / physiology Female Humans Male Middle Aged Perimetry Photoreceptor Cells, Vertebrate / pathology, physiology* Retinitis Pigmentosa / diagnosis, physiopathology* Retrospective Studies Tomography, Optical Coherence Usher Syndromes / diagnosis, physiopathology Visual Fields / physiology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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