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Cross-Sectional and Longitudinal Assessment of Retinal Sensitivity in Patients With Childhood-Onset Stargardt Disease

PURPOSE: We assess cross-sectional and longitudinal microperimetry and full-field static perimetry-derived retinal sensitivity with conventional and volumetric indices of retinal function in childhood-onset Stargardt disease (STGD1). METHODS: Subjects with molecularly confirmed childhood-onset STGD1...

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Autores principales: Tanna, Preena, Georgiou, Michalis, Aboshiha, Jonathan, Strauss, Rupert W., Kumaran, Neruban, Kalitzeos, Angelos, Weleber, Richard G., Michaelides, Michel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Association for Research in Vision and Ophthalmology 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6262645/
https://www.ncbi.nlm.nih.gov/pubmed/30510854
http://dx.doi.org/10.1167/tvst.7.6.10
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author Tanna, Preena
Georgiou, Michalis
Aboshiha, Jonathan
Strauss, Rupert W.
Kumaran, Neruban
Kalitzeos, Angelos
Weleber, Richard G.
Michaelides, Michel
author_facet Tanna, Preena
Georgiou, Michalis
Aboshiha, Jonathan
Strauss, Rupert W.
Kumaran, Neruban
Kalitzeos, Angelos
Weleber, Richard G.
Michaelides, Michel
author_sort Tanna, Preena
collection PubMed
description PURPOSE: We assess cross-sectional and longitudinal microperimetry and full-field static perimetry-derived retinal sensitivity with conventional and volumetric indices of retinal function in childhood-onset Stargardt disease (STGD1). METHODS: Subjects with molecularly confirmed childhood-onset STGD1 underwent full-field static perimetry and/or microperimetry using custom designed grids. Mean sensitivity (MS) and total volume (V(TOT)) were computed for each microperimetry test. MS, V(TOT), and central field volume (V(30)) were computed for each full-field static perimetry test. Subjects under 18 years old at baseline were classified as children and subjects 18 years or older as adults. RESULTS: A total of 43 children (mean age at baseline, 13.0 years; range, 8–17) and 13 adults (mean age at baseline, 23.1 years; range, 18–32) were included in the analysis. For full-field static perimetry and microperimetry for both subgroups, intraclass correlation coefficient results for MS and volumetric indices were good to excellent, indicating strong test–retest reliability. Interocular symmetry in terms of baseline measurements and the annual rate of progression was observed. A greater rate of progression was observed in childhood. CONCLUSIONS: To our knowledge, this is the first prospective study of retinal sensitivity in a large cohort of molecularly confirmed subjects with childhood-onset STGD1 demonstrating that children with STGD1 can reliably undertake detailed functional testing. Moreover, using custom designed grids and subsequent topographic analysis, volumetric indices of retinal function provide a reliable measure of retinal sensitivity. TRANSLATIONAL RELEVANCE: This study highlights the use of microperimetry and full-field static perimetry, as well as volumetric indices of retinal function, in monitoring disease progression.
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spelling pubmed-62626452018-12-03 Cross-Sectional and Longitudinal Assessment of Retinal Sensitivity in Patients With Childhood-Onset Stargardt Disease Tanna, Preena Georgiou, Michalis Aboshiha, Jonathan Strauss, Rupert W. Kumaran, Neruban Kalitzeos, Angelos Weleber, Richard G. Michaelides, Michel Transl Vis Sci Technol Articles PURPOSE: We assess cross-sectional and longitudinal microperimetry and full-field static perimetry-derived retinal sensitivity with conventional and volumetric indices of retinal function in childhood-onset Stargardt disease (STGD1). METHODS: Subjects with molecularly confirmed childhood-onset STGD1 underwent full-field static perimetry and/or microperimetry using custom designed grids. Mean sensitivity (MS) and total volume (V(TOT)) were computed for each microperimetry test. MS, V(TOT), and central field volume (V(30)) were computed for each full-field static perimetry test. Subjects under 18 years old at baseline were classified as children and subjects 18 years or older as adults. RESULTS: A total of 43 children (mean age at baseline, 13.0 years; range, 8–17) and 13 adults (mean age at baseline, 23.1 years; range, 18–32) were included in the analysis. For full-field static perimetry and microperimetry for both subgroups, intraclass correlation coefficient results for MS and volumetric indices were good to excellent, indicating strong test–retest reliability. Interocular symmetry in terms of baseline measurements and the annual rate of progression was observed. A greater rate of progression was observed in childhood. CONCLUSIONS: To our knowledge, this is the first prospective study of retinal sensitivity in a large cohort of molecularly confirmed subjects with childhood-onset STGD1 demonstrating that children with STGD1 can reliably undertake detailed functional testing. Moreover, using custom designed grids and subsequent topographic analysis, volumetric indices of retinal function provide a reliable measure of retinal sensitivity. TRANSLATIONAL RELEVANCE: This study highlights the use of microperimetry and full-field static perimetry, as well as volumetric indices of retinal function, in monitoring disease progression. The Association for Research in Vision and Ophthalmology 2018-11-27 /pmc/articles/PMC6262645/ /pubmed/30510854 http://dx.doi.org/10.1167/tvst.7.6.10 Text en Copyright 2018 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
spellingShingle Articles
Tanna, Preena
Georgiou, Michalis
Aboshiha, Jonathan
Strauss, Rupert W.
Kumaran, Neruban
Kalitzeos, Angelos
Weleber, Richard G.
Michaelides, Michel
Cross-Sectional and Longitudinal Assessment of Retinal Sensitivity in Patients With Childhood-Onset Stargardt Disease
title Cross-Sectional and Longitudinal Assessment of Retinal Sensitivity in Patients With Childhood-Onset Stargardt Disease
title_full Cross-Sectional and Longitudinal Assessment of Retinal Sensitivity in Patients With Childhood-Onset Stargardt Disease
title_fullStr Cross-Sectional and Longitudinal Assessment of Retinal Sensitivity in Patients With Childhood-Onset Stargardt Disease
title_full_unstemmed Cross-Sectional and Longitudinal Assessment of Retinal Sensitivity in Patients With Childhood-Onset Stargardt Disease
title_short Cross-Sectional and Longitudinal Assessment of Retinal Sensitivity in Patients With Childhood-Onset Stargardt Disease
title_sort cross-sectional and longitudinal assessment of retinal sensitivity in patients with childhood-onset stargardt disease
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6262645/
https://www.ncbi.nlm.nih.gov/pubmed/30510854
http://dx.doi.org/10.1167/tvst.7.6.10
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