Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease

Basit Öğe Kaydı
dc.contributor.authorBitirgen, Gulfidan
dc.contributor.authorTurkmen, Kultigin
dc.contributor.authorZengin, Nazmi
dc.contributor.authorMalik, Rayaz A.
dc.date.accessioned2024-02-23T14:16:44Z
dc.date.available2024-02-23T14:16:44Z
dc.date.issued2021
dc.departmentNEÜen_US
dc.description.abstractSymptoms of autonomic dysfunction are common in Fabry disease. In this study we aimed to evaluate alterations in the pupillary response to white light stimulation in patients with Fabry disease and their association with the severity of autonomic symptoms. Fourteen consecutive patients with Fabry disease and 14 healthy control participants were enrolled in this cross-sectional study. The Mainz Severity Score Index (MSSI) was used to measure the severity of Fabry disease and the Composite Autonomic Symptom Scale 31 (COMPASS 31) questionnaire was used to evaluate the severity of autonomic symptoms. The pupil light responses were assessed with an infrared dynamic pupillometry unit. There were significant reductions in the amplitude (P=0.048) and duration (P=0.048) of pupil contraction, and the latency of pupil dilation (P=0.048) in patients with Fabry disease compared to control subjects. The total weighted COMPASS 31 score correlated with MSSI (r=0.592; P=0.026) and the duration of pupil dilation (rho =0.561; P=0.037). The pupillomotor weighted sub-score of the COMPASS 31 correlated inversely with the duration of pupil contraction (r=-0.600; P=0.023) and latency of pupil dilation (rho=-0.541; P=0.046), and directly with the duration of pupil dilation (rho =0.877; P<0.001) and MSSI (r=0.533; P=0.049). In conclusion, abnormal pupillary function is demonstrated in patients with Fabry disease, which is associated with the severity of autonomic symptoms.en_US
dc.identifier.doi10.1038/s41598-021-87589-x
dc.identifier.issn2045-2322
dc.identifier.issue1en_US
dc.identifier.pmid33854145en_US
dc.identifier.scopus2-s2.0-85104241414en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.urihttps://doi.org/10.1038/s41598-021-87589-x
dc.identifier.urihttps://hdl.handle.net/20.500.12452/12770
dc.identifier.volume11en_US
dc.identifier.wosWOS:000640593400003en_US
dc.identifier.wosqualityQ2en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherNature Researchen_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.titleAltered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry diseaseen_US
dc.typeArticleen_US

Dosyalar

Koleksiyon

WoS İndeksli Yayınlar Koleksiyonu
PubMed İndeksli Yayın Koleksiyonu
Scopus İndeksli Yayınlar Koleksiyonu