Results of multicenter registry for patients with inherited factor VII deficiency in Turkey

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dc.authoridTuna Deveci, Rumeysa/0000-0003-1821-3947
dc.authoridAr, Muhlis Cem/0000-0002-0332-9253
dc.authoridAydın, Sultan/0000-0002-8801-7776
dc.authoridDemirci, Ufuk/0000-0001-6923-1470
dc.authorwosidTuna Deveci, Rumeysa/AAU-4592-2020
dc.authorwosidPekpak Şahinoğlu, Esra/B-1004-2017
dc.authorwosidAr, Muhlis Cem/S-7530-2016
dc.authorwosidAydın, Sultan/AAN-9164-2020
dc.authorwosidKeklik, Fatma/IZQ-0529-2023
dc.contributor.authorAkdeniz, Aydan
dc.contributor.authorUnuvar, Aysegul
dc.contributor.authorAr, Muhlis Cem
dc.contributor.authorPekpak, Esra
dc.contributor.authorAkyay, Arzu
dc.contributor.authorMehtap, Ozgur
dc.contributor.authorKaradag, Fatma Keklik
dc.date.accessioned2024-06-12T11:01:46Z
dc.date.available2024-06-12T11:01:46Z
dc.date.issued2022
dc.departmentTrakya Üniversitesien_US
dc.description.abstractIntroduction: Inherited factor VII (FVII) deficiency (FVIID) is the most common of inherited rare bleeding disorders. Other determinants of clinical severity apart from FVII level (FVIIL) include genetic and environmental factors. We aimed to identify the cut-off FVIILs for general and severe bleedings in patients with FVIID by using an online national registry system including clinical, laboratory, and demographic characteristics of patients. Methods: Demographic, clinical, and laboratory data of patients with FVIID extracted from the national database, constituted by the Turkish Society of Hematology, were examined. Bleeding phenotypes, general characteristics, and laboratory features were assessed in terms of FVIILs. Bleeding rates and prophylaxis during special procedures/interventions were also recorded. Results: Data from 197 patients showed that 46.2% of patients had FVIIL< 10%. Most bleeds were of mucosal origin (67.7%), and severe bleeds tended to occur in younger patients (median age: 15 (IQR:6-29)). Cut-off FVIILs for all and severe bleeds were 16.5% and 7.5%, respectively. The major reason for long-term prophylaxis was observed as central nervous system bleeding (80%). Conclusion: Our data are consistent with most of the published literature in terms of cut-off FVIIL for bleeding, as well as reasons for prophylaxis, showing both an increased severity of bleeding and younger age at diagnosis with decreasing FVIIL. However, in order to offer a classification similar to that in Hemophilia A or B, data of a larger cohort with information about environmental and genetic factors are required.en_US
dc.identifier.doi10.1080/00365513.2021.2013524
dc.identifier.endpage36en_US
dc.identifier.issn0036-5513
dc.identifier.issn1502-7686
dc.identifier.issue1en_US
dc.identifier.pmid34915774en_US
dc.identifier.scopus2-s2.0-85121735923en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage28en_US
dc.identifier.urihttps://doi.org/10.1080/00365513.2021.2013524
dc.identifier.urihttps://hdl.handle.net/20.500.14551/21009
dc.identifier.volume82en_US
dc.identifier.wosWOS:000731213900001en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherTaylor & Francis Ltden_US
dc.relation.ispartofScandinavian Journal Of Clinical & Laboratory Investigationen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectFactor VIIen_US
dc.subjectRare Diseasesen_US
dc.subjectFVII Deficiencyen_US
dc.subjectHemorrhageen_US
dc.subjectBlood Coagulation Disorderen_US
dc.subjectRare Coagulation Disordersen_US
dc.subjectBleeding Disordersen_US
dc.subjectFvii Deficiencyen_US
dc.subjectProphylaxisen_US
dc.subjectDiagnosisen_US
dc.subjectPhenotypesen_US
dc.subjectSeverityen_US
dc.subjectSymptomsen_US
dc.subjectGenotypeen_US
dc.subjectWomenen_US
dc.titleResults of multicenter registry for patients with inherited factor VII deficiency in Turkeyen_US
dc.typeArticleen_US

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