The Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) Studies: Design and Baseline Characteristics: ProgStar Report No. 1

Ophthalmology
Rupert W StraussProgression of Stargardt Disease Study Group

Abstract

To describe the design and baseline characteristics of patients enrolled into 2 natural history studies of Stargardt disease (STGD1). Multicenter retrospective and prospective cohort studies. Three hundred sixty-five unique patients aged 6 years and older at baseline harboring disease-causing variants in the ABCA4 gene and with specified ocular lesions were enrolled from 9 centers in the United States and Europe. In the retrospective study, patients contributed medical record data from at least 2 and up to 4 visits for at least 1 examination modality: fundus autofluorescence (FAF), spectral-domain (SD) optical coherence tomography (SD OCT), and/or microperimetry (MP). The total observational period was at least 2 years and up to 5 years between single visits. Demographic and visual acuity (VA) data also were obtained. In the prospective study, eligible patients were examined at baseline using a standard protocol, with 6-month follow-up visits planned for a 2-year period for serial Early Treatment Diabetic Retinopathy Study (ETDRS) best-corrected VA, SD OCT, FAF, and MP. Design and rationale of a multicenter study to determine the progression of STGD1 in 2 large retrospective and prospective international cohorts. Detailed basel...Continue Reading

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Citations

Jun 15, 2016·American Journal of Ophthalmology·Rupert W StraussMichel Michaelides
Oct 30, 2016·The British Journal of Ophthalmology·Preena TannaMichel Michaelides
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