Quantifying the Progression of Stargardt Disease in Double-Null ABCA4 Carriers Using Fundus Autofluorescence Imaging.

ABSTRACT:

Purpose

To score real-world fundus autofluorescence (FAF) images of pediatric patients with ABCA4-related Stargardt disease (STGD1), in a way that is automatable, scales with the disease progression, and is applicable to a wide time interval in the natural history of the disease.

Methods

We developed the score based on a series of Optos wide-field FAF images of pediatric STGD1 patients (73 images; 14 individuals) and controls (27 images; 8 individuals). The patients' images were obtained over up to 6 years, and the controls over up to 5 years. In each image, we manually selected an artifact-free region, within which we evaluated an average of the pixel-level intensity score, constructed so that the average increases with progression of the disease.

Results

The score w

SUBMITTER: Mihalek I 

PROVIDER: S-EPMC11925222 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature