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Reference: Lindner, M, Lambertus, S, Mauschitz, MM et al., (2017). Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease. Investigative Ophthalmology & Visual Science, 58, 1001-1007.Citable link to this page:

 

Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease

Abstract: Purpose: To compare the disease course of retinal pigment epithelium (RPE) atrophy secondary to age-related macula degeneratio (AMD) and late-onset Stargardt disease (STGD1). Methods: Patients were examined longitudinally by fundus autofluorescence, near-infrared reflectance imaging, and best-corrected visual acuity (BCVA). Areas of RPE atrophy were quantified using semi-automated software, and the status of the fovea was evaluated based on autofluorescence and near-infrared reflectance images. Mixed-effects models were used to compare atrophy progression rates. BCVA loss and loss of foveal integrity were analyzed using Turnbull's estimator. Results: A total of 151 patients (226 eyes) with RPE atrophy secondary to AMD and 38 patients (66 eyes) with RPE atrophy secondary to late-onset STGD1 were examined for a median time of 2.3 years (interquartile range, 2.7). Mean baseline age was 74.2 years (SD, 7.6) in AMD and 63.4 (SD, 9.9) in late-onset STGD1 (P = 1.1 × 10-7). Square root atrophy progression was significantly faster in AMD when compared with late-onset STGD1 (0.28 mm/year [SE, 0.01] vs. 0.23 [SE, 0.03]; P = 0.030). In late-onset STGD1, the median survival of the fovea was significantly longer when compared with eyes with AMD (8.60 vs. 3.35 years; P = 0.005) with a trend to a later BCVA loss of ≥3 lines (5.97 vs. 4.37 years; P = 0.382). Conclusions: These natural history data indicate differential disease progression in AMD versus late-onset STGD1. The results underline the relevance of refined phenotyping in elderly patients presenting with RPE atrophy in regard to prognosis and design of interventional trials.

Publication status:PublishedPeer Review status:Peer reviewedVersion:Publisher's versionDate of acceptance:2017-01-11 Funder: Deutsche Forschungsgemeinschaft   Funder: University of Bonn   Funder: Stichting A.F. Deutman Researchfonds Oogheelkunde   Funder: Nederlandse Oogonderzoek Stichting   Funder: the Stichting MaculaFonds   Funder: UitZicht   Funder: Landelijke Stichting voor Blinden en Slechtzienden,and Oogfonds   Notes:This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Bibliographic Details

Publisher: Association for Research in Vision and Ophthalmology

Publisher Website: http://www.arvo.org/

Journal: Investigative Ophthalmology & Visual Sciencesee more from them

Publication Website: http://iovs.arvojournals.org/

Volume: 58

Issue Date: 2017-02-01

pages:1001-1007Identifiers

Doi: https://doi.org/10.1167/iovs.16-20980

Issn: 0146-0404

Eissn: 1552-5783

Uuid: uuid:4467394b-6e0e-4a65-bca7-d78c71642dfe

Urn: uri:4467394b-6e0e-4a65-bca7-d78c71642dfe

Pubs-id: pubs:690079 Item Description

Type: journal-article;

Language: eng

Version: Publisher's versionKeywords: Journal Article

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Autor: Lindner, M - Oxford, MSD, Clinical Neurosciences St Cross College - - - Lambertus, S - - - Mauschitz, MM - - - Bax, NM - - - Kers

Fuente: https://ora.ox.ac.uk/objects/uuid:4467394b-6e0e-4a65-bca7-d78c71642dfe



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