PROGRESSION OF ABCA4-RELATED RETINOPATHY: Prognostic value of demographic, functional, genetic, and imaging parameters.

Retina
Philipp L MüllerFrank G Holz

Abstract

To investigate the prognostic value of demographic, functional, genetic, and imaging parameters on retinal pigment epithelium atrophy progression secondary to ABCA4-related retinopathy. Patients with retinal pigment epithelium atrophy secondary to ABCA4-related retinopathy were examined longitudinally with fundus autofluorescence imaging. Lesion area, perimeter, circularity, caliper diameters, and focality of areas with definitely decreased autofluorescence were determined. A model was used to predict the lesion enlargement rate based on baseline variables. Sample size calculations were performed to model the power in a simulated interventional study. Sixty-eight eyes of 37 patients (age range, 14-78 years) with a follow-up time of 10 to 100 months were included. The mean annual progression of retinal pigment epithelium atrophy was 0.89 mm. The number of atrophic areas, the retina-wide functional impairment, and the age-of-onset category constituted significant predictors for future retinal pigment epithelium atrophy growth, explaining 25.7% of the variability. By extension of a simulated study length and/or specific patient preselection based on these baseline characteristics, the required sample size could significantly be re...Continue Reading

References

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Aug 30, 2011·Investigative Ophthalmology & Visual Science·Steffen Schmitz-ValckenbergFrank G Holz
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Sep 18, 2015·Investigative Ophthalmology & Visual Science·Artur V CideciyanSamuel G Jacobson
Jan 1, 2016·Investigative Ophthalmology & Visual Science·Philipp L MüllerPeter Charbel Issa
Jul 15, 2016·Retina·Philipp L MüllerPeter Charbel Issa
Oct 5, 2016·Investigative Ophthalmology & Visual Science·Stanley LambertusUNKNOWN Foveal sparing Atrophy Study Team (FAST)
Mar 14, 2017·Investigative Ophthalmology & Visual Science·Moritz LindnerUNKNOWN Foveal sparing Atrophy Study Team (FAST)
May 22, 2018·Retina·Maximilian PfauUNKNOWN Fundus Autofluorescence in Age-Related Macular Degeneration Study Group
Aug 25, 2018·Investigative Ophthalmology & Visual Science·Philipp L MüllerMonika Fleckenstein
Oct 4, 2018·Translational Vision Science & Technology·Philipp L MüllerPhilipp Herrmann
Jan 19, 2019·Investigative Ophthalmology & Visual Science·Chantal DysliPhilipp Herrmann

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Citations

Sep 7, 2020·American Journal of Ophthalmology·Philipp L MüllerPeter Charbel Issa
Jan 16, 2021·Scientific Reports·Philipp L MüllerMaximilian Pfau
Aug 6, 2020·Journal of Clinical Medicine·Philipp L MüllerFrank G Holz
Apr 30, 2020·The British Journal of Ophthalmology·Maximilian PfauPhilipp L Müller
May 21, 2021·Clinical & Experimental Ophthalmology·Rachael C Heath Jeffery, Fred K Chen
Aug 31, 2021·Ophthalmic Genetics·Di HuangFred K Chen

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