Optical coherence tomography angiography analysis of fabry disease

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dc.authoridcakmak, ayse idil/0000-0003-4377-7314
dc.authoridAtalay, Eray/0000-0002-2536-4279
dc.authoridTURGUT, FARUK/0000-0003-1910-7433
dc.contributor.authorCakmak, Ayse Idil
dc.contributor.authorAtalay, Eray
dc.contributor.authorCankurtaran, Veysel
dc.contributor.authorYasar, Erdogan
dc.contributor.authorTurgut, Faruk Hilmi
dc.date.accessioned2024-09-18T20:19:59Z
dc.date.available2024-09-18T20:19:59Z
dc.date.issued2020
dc.departmentHatay Mustafa Kemal Üniversitesien_US
dc.description.abstractPurpose Fabry disease (FD) is characterized by a deficiency in alpha-galactosidase A activity that leads to the cumulative deposition of unmetabolized glycosphingolipids within organs, including the vascular endothelium and the eyes. The purpose of this study was to assess the effects of FD on the retinal microvasculature, foveal avascular zone (FAZ), macular thickness and retinal nerve fiber layer (RNFL) using optical coherence tomography angiography (OCT-A). Methods Twenty-five patients (14 female and 11 male; mean age 33.16 +/- 11.44) with genetically verified FD were compared with 37 age- and sex-matched healthy controls (mean age 32.36 +/- 15.54). The vessel density (VD) values of the superficial and deep capillary plexuses (SCP and DCP), the area of the FAZ, the density of radial peripapillary capillaries (RPC), the macular thickness and the retinal nerve fiber layer thickness were measured by OCT-A examination. Results The patients showed significantly lower VD values than controls in the foveal regions of both SCP and the DCP (21.15 +/- 5.56 vs. 23.79 +/- 4.64 (p = 0.048), 37.92 +/- 6.78 vs. 41.11 +/- 5.59 (p = 0.048), respectively). The FAZ was significantly larger in the FD group than in the control group (0.3 +/- 0.1 vs. 0.24 +/- 0.08 (p = 0.011)). No significant difference was identified in measurements of RPC density, peripapillary RNFL thickness or macular thickness between the two groups (p > 0.05 for all). Conclusion Decreased VD and an enlarged foveal avascular area suggest possible changes in the retinal microvasculature of patients with FD. OCT-A can serve as a useful, noninvasive, quantitative tool for diagnosing FD and monitoring its progression.en_US
dc.description.sponsorshipScientific Research Projects Management Unit of Hatay Mustafa Kemal Universityen_US
dc.description.sponsorshipScientific Research Projects Management Unit of Hatay Mustafa Kemal University (TR).en_US
dc.identifier.doi10.1007/s10792-020-01486-2
dc.identifier.endpage3032en_US
dc.identifier.issn0165-5701
dc.identifier.issn1573-2630
dc.identifier.issue11en_US
dc.identifier.pmid32607948en_US
dc.identifier.scopus2-s2.0-85087297236en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage3023en_US
dc.identifier.urihttps://doi.org/10.1007/s10792-020-01486-2
dc.identifier.urihttps://hdl.handle.net/20.500.12483/9989
dc.identifier.volume40en_US
dc.identifier.wosWOS:000544534800001en_US
dc.identifier.wosqualityQ3en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringeren_US
dc.relation.ispartofInternational Ophthalmologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectFabry diseaseen_US
dc.subjectOptical coherence tomography angiographyen_US
dc.subjectRetinal vessel densityen_US
dc.subjectRadial peripapillary capillariesen_US
dc.subjectPeripapillary retinal nerve fiber layeren_US
dc.titleOptical coherence tomography angiography analysis of fabry diseaseen_US
dc.typeArticleen_US

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