Progression of Stargardt Disease as Determined by Fundus Autofluorescence in the Retrospective Progression of Stargardt Disease Study (ProgStar Report No. 9).

ABSTRACT:

Importance

Sensitive outcome measures for disease progression are needed for treatment trials of Stargardt disease.

Objective

To describe the yearly progression rate of atrophic lesions in the retrospective Progression of Stargardt Disease study.

Design, setting, and participants

A multicenter retrospective cohort study was conducted at tertiary referral centers in the United States and Europe. A total of 251 patients aged 6 years or older at baseline, harboring disease-causing variants in ABCA4 (OMIM 601691), enrolled in the study from 9 centers between August 2, 2013, and December 12, 2014; of these patients, 215 had at least 2 gradable fundus autofluorescence images with atrophic lesion(s) present in at least 1 eye.

Exposures

Areas of definitely decreased au

SUBMITTER: Strauss RW 

PROVIDER: S-EPMC5710470 | biostudies-literature | 2017 Nov

REPOSITORIES: biostudies-literature