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Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
PURPOSE: The aim of this case control study was to evaluate the prognostic value of automatically quantified retinal vessel tortuosity from fundus images and vessel density from OCT-A in Fabry disease and to evaluate the correlation of these with systemic disease parameters. METHODS: Automatically q...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8605526/ https://www.ncbi.nlm.nih.gov/pubmed/34801073 http://dx.doi.org/10.1186/s13023-021-02080-0 |
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author | Atiskova, Yevgeniya Wildner, Jan Spitzer, Martin Stephan Aries, Charlotte Muschol, Nicole Dulz, Simon |
author_facet | Atiskova, Yevgeniya Wildner, Jan Spitzer, Martin Stephan Aries, Charlotte Muschol, Nicole Dulz, Simon |
author_sort | Atiskova, Yevgeniya |
collection | PubMed |
description | PURPOSE: The aim of this case control study was to evaluate the prognostic value of automatically quantified retinal vessel tortuosity from fundus images and vessel density from OCT-A in Fabry disease and to evaluate the correlation of these with systemic disease parameters. METHODS: Automatically quantified perimacular retinal vessel tortuosity (MONA REVA software), acquired by fundus imaging, and perifoveal retinal vessel density, acquired by optic coherence tomography angiography (OCT-A) were compared between 26 FD patients and 26 controls. Gender and FD phenotype were analyzed to the obtained retinovascular data and correlated to the Mainz severity score index (MSSI) and plasma lyso-Gb3. RESULTS: Automatically quantified retinal vessel tortuosity indices of FD patients were significantly lower, reflecting an increased vessel tortuosity, compared to controls (p = 0.008). Males with a classical phenotype showed significantly lower retinal vessel tortuosity indices compared to males with an oligosymptomatic phenotype and females with a classical or oligosymptomatic phenotype (p < 0.001). The retinal vessel tortuosity index correlated significantly with systemic disease severity parameters [global MSSI (r = − 0.5; p < 0.01), cardiovascular MSSI (r = − 0.5; p < 0.01), lyso-Gb3 (r = − 0.6; p < 0.01)]. CONCLUSION: We advocate fundus imaging based automatically quantified retinal vessel tortuosity index over OCT-A imaging as it is a quick, non-invasive, easily assessable, objective and reproducible marker. |
format | Online Article Text |
id | pubmed-8605526 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-86055262021-11-22 Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease Atiskova, Yevgeniya Wildner, Jan Spitzer, Martin Stephan Aries, Charlotte Muschol, Nicole Dulz, Simon Orphanet J Rare Dis Research PURPOSE: The aim of this case control study was to evaluate the prognostic value of automatically quantified retinal vessel tortuosity from fundus images and vessel density from OCT-A in Fabry disease and to evaluate the correlation of these with systemic disease parameters. METHODS: Automatically quantified perimacular retinal vessel tortuosity (MONA REVA software), acquired by fundus imaging, and perifoveal retinal vessel density, acquired by optic coherence tomography angiography (OCT-A) were compared between 26 FD patients and 26 controls. Gender and FD phenotype were analyzed to the obtained retinovascular data and correlated to the Mainz severity score index (MSSI) and plasma lyso-Gb3. RESULTS: Automatically quantified retinal vessel tortuosity indices of FD patients were significantly lower, reflecting an increased vessel tortuosity, compared to controls (p = 0.008). Males with a classical phenotype showed significantly lower retinal vessel tortuosity indices compared to males with an oligosymptomatic phenotype and females with a classical or oligosymptomatic phenotype (p < 0.001). The retinal vessel tortuosity index correlated significantly with systemic disease severity parameters [global MSSI (r = − 0.5; p < 0.01), cardiovascular MSSI (r = − 0.5; p < 0.01), lyso-Gb3 (r = − 0.6; p < 0.01)]. CONCLUSION: We advocate fundus imaging based automatically quantified retinal vessel tortuosity index over OCT-A imaging as it is a quick, non-invasive, easily assessable, objective and reproducible marker. BioMed Central 2021-11-20 /pmc/articles/PMC8605526/ /pubmed/34801073 http://dx.doi.org/10.1186/s13023-021-02080-0 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Research Atiskova, Yevgeniya Wildner, Jan Spitzer, Martin Stephan Aries, Charlotte Muschol, Nicole Dulz, Simon Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease |
title | Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease |
title_full | Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease |
title_fullStr | Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease |
title_full_unstemmed | Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease |
title_short | Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease |
title_sort | retinal vessel tortuosity as a prognostic marker for disease severity in fabry disease |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8605526/ https://www.ncbi.nlm.nih.gov/pubmed/34801073 http://dx.doi.org/10.1186/s13023-021-02080-0 |
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