Progression of Stargardt Disease as Determined by Fundus Autofluorescence Over a 12-Month Period: ProgStar Report No. 11.

JAMA Ophthalmology
Rupert W StraussProgStar Study Group

Abstract

Sensitive outcome measures for disease progression are needed for treatment trials of Stargardt disease. To estimate the progression rate of atrophic lesions in the prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) study over a 12-month period. This multicenter prospective cohort study was conducted in an international selection of tertiary referral centers from October 21, 2013, to February 15, 2017. Patients who were affected by Stargardt disease, aged 6 years and older at baseline, and harboring disease-causing variants of the ABCA4 gene were enrolled at 9 centers in the United States, United Kingdom, and continental Europe. Data analysis occurred from November 2016 to January 2017. Autofluorescence images obtained with a standard protocol were sent to a central reading center, and areas of definitely decreased autofluorescence, questionably decreased autofluorescence, and the total combined area of decreased autofluorescence were outlined and quantified. Progression rates were estimated from linear mixed models with time as the independent variable. Yearly rate of progression, using the growth of atrophic lesions measured by autofluorescence imaging. A total of 259 study par...Continue Reading

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Mar 14, 2020·Current Opinion in Ophthalmology·Sophie CaiLejla Vajzovic
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