Document Detail

Progression of retinal pigment epithelial atrophy in stargardt disease.
MedLine Citation:
PMID:  22464366     Owner:  NLM     Status:  MEDLINE    
PURPOSE: To evaluate retinal pigment epithelial (RPE) atrophy in patients with Stargardt disease using autofluorescence imaging (AF).
DESIGN: Retrospective observational case series.
METHODS: Demographics, best-corrected visual acuity (BCVA), AF images, and electrophysiology responses (group 1, macular dysfunction; group 2, macula + cone dysfunction; group 3, macula + cone-rod dysfunction) were evaluated at presentation and follow-up in a group of 12 patients (24 eyes) with Stargardt disease. The existence, development, and rate of enlargement of areas of RPE atrophy over time were evaluated using AF imaging. A linear regression model was used to investigate the effects of AF and electrophysiology on rate of atrophy enlargement and BCVA, adjusting for age of onset and duration of disease.
RESULTS: Eight male and 4 female patients (median age 42 years; range 24-69 years) were followed for a median of 41.5 months (range 13-66 months). All 12 patients had reduced AF compatible with RPE atrophy at presentation and in all patients the atrophy enlarged during follow-up. The mean rate of atrophy enlargement for all patients was 1.58 mm(2)/y (SD 1.25 mm(2)/y; range 0.13-5.27 mm(2)/y). Only the pattern of functional loss present as detected by electrophysiology was statistically significantly associated with the rate of atrophy enlargement when correcting for other variables (P < .001), with patients in group 3 (macula + cone-rod dysfunction) having the fastest rate of atrophy enlargement (1.97 mm(2)/y, SD 0.70 mm(2)/y) (group 1 [macula] 1.09 mm(2)/y, SD 0.53 mm(2)/y; group 2 [macula + cone] 1.89 mm(2)/y, SD 2.27 mm(2)/y).
CONCLUSION: Variable rates of atrophy enlargement were observed in patients with Stargardt disease. The pattern of functional loss detected on electrophysiology was strongly associated with the rate of atrophy enlargement over time, thus serving as the best prognostic indicator for patients with this inherited retinal disease.
Vikki A McBain; John Townend; Noemi Lois
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Publication Detail:
Type:  Journal Article     Date:  2012-03-30
Journal Detail:
Title:  American journal of ophthalmology     Volume:  154     ISSN:  1879-1891     ISO Abbreviation:  Am. J. Ophthalmol.     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-06-19     Completed Date:  2012-08-29     Revised Date:  2013-07-10    
Medline Journal Info:
Nlm Unique ID:  0370500     Medline TA:  Am J Ophthalmol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  146-54     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2012 Elsevier Inc. All rights reserved.
Ophthalmology Department, Grampian University Hospitals-National Health Service Trust, Aberdeen, Scotland, United Kingdom.
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MeSH Terms
Disease Progression
Fluorescein Angiography
Macular Degeneration / congenital,  physiopathology*
Middle Aged
Retinal Pigment Epithelium / pathology*
Retrospective Studies
Visual Acuity / physiology
Young Adult

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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