Relationship of Vessel Density to Vessel Length Density in Patients with Treated Fabry Disease.
Diagnostics (Basel)
; 13(7)2023 Mar 24.
Article
in En
| MEDLINE
| ID: mdl-37046445
ABSTRACT
BACKGROUND:
Fabry disease (FD) is a potentially lethal lysosomal disorder with systemic vascular changes. Previous studies demonstrated retinal vascular involvement using optical coherence tomography angiography (OCTA) in affected patients;Aim:
To analyze and quantify the retinal vasculature measuring vessel density (VD), vessel length density (VLD), and the ratio of VD to VLD (VD/VLD) in superficial capillary plexuses (SCP) and deep capillary plexuses (DCP) using OCTA in patients with FD and to show whether they differ from healthy controls (HC); Patients andmethods:
Single-center, retrospective, consecutive cohort study of patients with genetically proven FD. Patients underwent an ophthalmological examination including OCTA. VD, VLD, foveal avascular zone (FAZ), and the VD/VLD were compared to an HC group using a linear mixed model;Results:
A statistically significant difference in the VLD and VD/VLD of DCP was observed between the two groups (p < 0.001). Using ROC curves with AUC and Youden's Index, a cut-off value for differentiating both groups using VD/VLD in DCP FD with high specificity and high sensitivity was established;Conclusions:
FD and HC groups seem to be separable using the VD/VLD ratio in DCP. This new biomarker might differentiate changes in the retinal microvasculature that are not detectable by VD or VLD alone.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Type of study:
Observational_studies
Language:
En
Journal:
Diagnostics (Basel)
Year:
2023
Document type:
Article
Affiliation country: