Document Detail


Fundus Autofluorescence and Optical Coherence Tomography in Relation to Visual Function in Usher Syndrome Type 1 and 2.
MedLine Citation:
PMID:  23000274     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Purpose of this study was to characterise retinal disease in Usher syndrome using fundus autofluorescence and optical coherence tomography. Study included 54 patients (26 male, 28 female) aged 7-70 years. There were 18 (33%) USH1 and 36 (67%) USH 2 patients. 49/52 (94%) patients were found to carry at least one mutation in Usher genes. Ophthalmological examination included assessment of Snellen visual acuity, color vision with Ishihara tables, Goldmann visual fields (targets II/1-4 and V/4), microperimetry, fundus autofluorescence imaging and optical coherence tomography. Average age at disease onset (nyctalopia) was significantly lower in USH1 than USH2 patients (average 9 vs. 17 years, respectively; p<0.01); however no significant differences were found regarding type of autofluorescence patterns, frequency of foveal lesions and CME, rate of disease progression and age at legal blindness. All representative eyes had abnormal fundus autofluorescence of either hyperautofluorescent ring (55%), hyperautofluorescent foveal patch (35%) or foveal atrophy (10%). Disease duration of more than 30 years was associated with a high incidence of abnormal central fundus autofluorescence and visual acuity loss.
Authors:
Ana Fakin; Damjan Glavač; Crystel Bonnet; Christine Petit; Marko Hawlina
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-9-18
Journal Detail:
Title:  Vision research     Volume:  -     ISSN:  1878-5646     ISO Abbreviation:  Vision Res.     Publication Date:  2012 Sep 
Date Detail:
Created Date:  2012-9-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0417402     Medline TA:  Vision Res     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012. Published by Elsevier Ltd.
Affiliation:
Eye Hospital, University Medical Centre Ljubljana, Slovenia, Grablovičeva 46, 1000 Ljubljana, Slovenia. Electronic address: ana.fakin@gmail.com.
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