Ivana Mihalek, Hanna De Bruyn, Tomislav Glavan, Annie M Lancos, Caitlin M Ciolfi, Katarzyna Malendowicz, Sigrid Aslaksen, Laurie L Molday, Robert S Molday, Anne B Fulton
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引用次数: 0
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 we propose provides a statistically robust measure of disease progression (91% Spearman correlation with the absolute age, 97% with the estimated time from onset, when averaged over both eyes), comparable across timepoints and patients.
Conclusions: FAF is a reliable tool in STGD1 diagnostics, but its quantitative description must be modified to be applicable to tracking the disease progression. Analyzing images obtained in the course of clinical care of pediatric patients poses special challenges that make complete automation difficult.
Translational relevance: Our methodology provides a quantitative tool for investigating the natural progression of the Stargardt disease, and, potentially, the effects of genotype, environment, and therapeutic intervention on its course.
期刊介绍：
Translational Vision Science & Technology (TVST), an official journal of the Association for Research in Vision and Ophthalmology (ARVO), an international organization whose purpose is to advance research worldwide into understanding the visual system and preventing, treating and curing its disorders, is an online, open access, peer-reviewed journal emphasizing multidisciplinary research that bridges the gap between basic research and clinical care. A highly qualified and diverse group of Associate Editors and Editorial Board Members is led by Editor-in-Chief Marco Zarbin, MD, PhD, FARVO.
The journal covers a broad spectrum of work, including but not limited to:
Applications of stem cell technology for regenerative medicine,
Development of new animal models of human diseases,
Tissue bioengineering,
Chemical engineering to improve virus-based gene delivery,
Nanotechnology for drug delivery,
Design and synthesis of artificial extracellular matrices,
Development of a true microsurgical operating environment,
Refining data analysis algorithms to improve in vivo imaging technology,
Results of Phase 1 clinical trials,
Reverse translational ("bedside to bench") research.
TVST seeks manuscripts from scientists and clinicians with diverse backgrounds ranging from basic chemistry to ophthalmic surgery that will advance or change the way we understand and/or treat vision-threatening diseases. TVST encourages the use of color, multimedia, hyperlinks, program code and other digital enhancements.
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