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Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
by
Aries, Charlotte
, Spitzer, Martin Stephan
, Muschol, Nicole
, Atiskova, Yevgeniya
, Wildner, Jan
, Dulz, Simon
in
Angiography
/ Blood vessels
/ Case-Control Studies
/ Development and progression
/ Enzymes
/ Eye
/ Fabry disease
/ Fabry Disease - genetics
/ Fabry's disease
/ Female
/ Human Genetics
/ Humans
/ Male
/ Medical examination
/ Medical personnel
/ Medical prognosis
/ Medical research
/ Medicine
/ Medicine & Public Health
/ MONA REVA
/ OCT-A
/ Patient outcomes
/ Pharmacology/Toxicology
/ Phenotypes
/ Prognosis
/ Rare diseases
/ Retina
/ Retinal Vessels
/ Severity of Illness Index
/ Software
/ Statistical analysis
/ Systemic diseases
/ Tomography, Optical Coherence - methods
/ Vascular tortuosity
2021
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Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
by
Aries, Charlotte
, Spitzer, Martin Stephan
, Muschol, Nicole
, Atiskova, Yevgeniya
, Wildner, Jan
, Dulz, Simon
in
Angiography
/ Blood vessels
/ Case-Control Studies
/ Development and progression
/ Enzymes
/ Eye
/ Fabry disease
/ Fabry Disease - genetics
/ Fabry's disease
/ Female
/ Human Genetics
/ Humans
/ Male
/ Medical examination
/ Medical personnel
/ Medical prognosis
/ Medical research
/ Medicine
/ Medicine & Public Health
/ MONA REVA
/ OCT-A
/ Patient outcomes
/ Pharmacology/Toxicology
/ Phenotypes
/ Prognosis
/ Rare diseases
/ Retina
/ Retinal Vessels
/ Severity of Illness Index
/ Software
/ Statistical analysis
/ Systemic diseases
/ Tomography, Optical Coherence - methods
/ Vascular tortuosity
2021
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Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
by
Aries, Charlotte
, Spitzer, Martin Stephan
, Muschol, Nicole
, Atiskova, Yevgeniya
, Wildner, Jan
, Dulz, Simon
in
Angiography
/ Blood vessels
/ Case-Control Studies
/ Development and progression
/ Enzymes
/ Eye
/ Fabry disease
/ Fabry Disease - genetics
/ Fabry's disease
/ Female
/ Human Genetics
/ Humans
/ Male
/ Medical examination
/ Medical personnel
/ Medical prognosis
/ Medical research
/ Medicine
/ Medicine & Public Health
/ MONA REVA
/ OCT-A
/ Patient outcomes
/ Pharmacology/Toxicology
/ Phenotypes
/ Prognosis
/ Rare diseases
/ Retina
/ Retinal Vessels
/ Severity of Illness Index
/ Software
/ Statistical analysis
/ Systemic diseases
/ Tomography, Optical Coherence - methods
/ Vascular tortuosity
2021
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Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
Journal Article
Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
2021
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Overview
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.
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