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Medicine and Health Sciences Commons

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Physical Sciences and Mathematics

Edith Cowan University

Series

Stargardt disease

Articles 1 - 2 of 2

Full-Text Articles in Medicine and Health Sciences

Analysis Of The Outer Retinal Bands In Abca4 And Prph2-Associated Retinopathy Using Oct, Rachael C. Heath Jeffery, Johnny Lo, Jennifer A. Thompson, Tina M. Lamey, Terri L. Mclaren, John N. De Roach, Lauren N. Ayton, Andrea L. Vincent, Abhishek Sharma, Fred K. Chen Feb 2024

Analysis Of The Outer Retinal Bands In Abca4 And Prph2-Associated Retinopathy Using Oct, Rachael C. Heath Jeffery, Johnny Lo, Jennifer A. Thompson, Tina M. Lamey, Terri L. Mclaren, John N. De Roach, Lauren N. Ayton, Andrea L. Vincent, Abhishek Sharma, Fred K. Chen

Research outputs 2022 to 2026

Purpose: To evaluate the outer retinal bands using OCT in ABCA4- and PRPH2-associated retinopathy and develop a novel imaging biomarker to differentiate between these 2 genotypes. Design: Multicenter case-control study. Participants: Patients with a clinical and genetic diagnosis of ABCA4- or PRPH2-associated retinopathy and an age-matched control group. Methods: Macular OCT was used to measure the thickness of the outer retinal bands 2 and 4 by 2 independent examiners at 4 retinal loci. Main Outcome Measures: Outcome measures included the thicknesses of band 2, band 4, and the band 2/band 4 ratio. Linear mixed modeling was used to make comparisons …


Genotype-Specific Lesion Growth Rates In Stargardt Disease, Rachael C. Heath Jeffery, Jennifer A. Thompson, Johnny Lo, Tina M. Lamey, Terri L. Mclaren, Ian L. Mcallister, Ian J. Constable, John N. De Roach, Fred K. Chen Dec 2021

Genotype-Specific Lesion Growth Rates In Stargardt Disease, Rachael C. Heath Jeffery, Jennifer A. Thompson, Johnny Lo, Tina M. Lamey, Terri L. Mclaren, Ian L. Mcallister, Ian J. Constable, John N. De Roach, Fred K. Chen

Research outputs 2014 to 2021

Reported growth rates (GR) of atrophic lesions in Stargardt disease (STGD1) vary widely. In the present study, we report the longitudinal natural history of patients with confirmed bial-lelic ABCA4 mutations from five genotype groups: c.6079C > T, c.[2588G > C;5603A > T], c.3113C > T, c.5882G > A and c.5603A > T. Fundus autofluorescence (AF) 30◦ × 30◦ images were manually seg-mented for boundaries of definitely decreased autofluorescence (DDAF). The primary outcome was the effective radius GR across five genotype groups. The age of DDAF formation in each eye was calculated using the x-intercept of the DDAF effective radius against age. Discordance between age at DDAF …