Corneal confocal microscopy detects corneal nerve damage and increased dendritic cells in Fabry disease

dc.contributor.authorBitirgen, Gulfidan
dc.contributor.authorTurkmen, Kultigin
dc.contributor.authorMalik, Rayaz A.
dc.contributor.authorOzkagnici, Ahmet
dc.contributor.authorZengin, Nazmi
dc.date.accessioned2024-02-23T14:16:44Z
dc.date.available2024-02-23T14:16:44Z
dc.date.issued2018
dc.departmentNEÜen_US
dc.description.abstractFabry disease is characterised by neuropathic pain and accelerated vascular disease. This study evaluates the utility of corneal confocal microscopy (CCM) to non-invasively quantify corneal nerve and endothelial cell morphology and dendritic cell (DC) density in relation to disease severity in subjects with Fabry disease. Seventeen consecutive participants with Fabry disease and 17 healthy control subjects were included in this cross-sectional study. Fabry disease severity was measured using the Mainz Severity Score Index (MSSI). Central corneal sensitivity was assessed with a contact corneal esthesiometer. There was a significant reduction in the corneal sensitivity (5.75 [5.25-6.00] vs. 6.00 [6.00-6.00] cm, P = 0.014), nerve fiber density (NFD) (26.4 +/- 10.1 vs. 33.7 +/- 7.9 fibers/mm(2), P = 0.025) and nerve fiber length (NFL) (15.9 +/- 3.4 vs. 19.5 +/- 4.4 mm/mm(2), P = 0.012) and an increase in DC density (38.3 [17.5-97.3] vs. 13.5 [0-29.4] cells/mm(2), P = 0.004) in subjects with Fabry disease compared to the healthy control subjects. The total MSSI score correlated with NFD (rho = -0.686; P = 0.006), NFL (rho = -0.692; P = 0.006), endothelial cell density (rho = -0.511; P = 0.036), endothelial cell area (rho = 0.514; P = 0.036) and alpha-galactosidase A enzyme activity (rho = -0.723; P = 0.008). This study demonstrates reduced corneal sensitivity, corneal nerve fiber damage and increased DCs in subjects with Fabry disease.en_US
dc.identifier.doi10.1038/s41598-018-30688-z
dc.identifier.issn2045-2322
dc.identifier.pmid30116053en_US
dc.identifier.scopus2-s2.0-85051639043en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.urihttps://doi.org/10.1038/s41598-018-30688-z
dc.identifier.urihttps://hdl.handle.net/20.500.12452/12765
dc.identifier.volume8en_US
dc.identifier.wosWOS:000441775900008en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherNature Publishing Groupen_US
dc.relation.ispartofScientific Reportsen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subject[Keyword Not Available]en_US
dc.titleCorneal confocal microscopy detects corneal nerve damage and increased dendritic cells in Fabry diseaseen_US
dc.typeArticleen_US

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