Evaluation of Bleeding Phenotype of Inherited Factor VII Deficiency in Children With a Bleeding Assessment Tool and Global Assays

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dc.contributor.authorToret, Ersin
dc.contributor.authorAy, Yilmaz
dc.contributor.authorKarapinar, Tuba H.
dc.contributor.authorOymak, Yesim
dc.contributor.authorKavakli, Kaan
dc.contributor.authorVergin, Raziye C.
dc.date.accessioned2020-12-01T11:58:44Z
dc.date.available2020-12-01T11:58:44Z
dc.date.issued2020
dc.departmentEge Üniversitesien_US
dc.description.abstractIntroduction:Inherited factor VII (FVII) deficiency is the most common of the rare bleeding disorders and shows a heterogenous distribution of bleeding phenotypes independent of factor activity level. the bleeding score (BS) evaluates the phenotype of patients with rare bleeding disorders. Thromboelastography (TEG) and thrombin generation assays (TGAs) are 2 methods to evaluate global hemostasis, and controversially both tests are useful for identifying different bleeding tendency phenotypes. the purpose of this study was to investigate the use of the BS and global assays (TEG and TGAs) to predict the bleeding phenotype of inherited FVII deficiency.Materials and Methods:A total of 27 patients with FVII deficiency were evaluated with the BS and global hemostasis assays.Results:The BS was compatible with disease severity according to the FVII activity level (P<0.05) but the BS and bleeding grade of patients did not show a statistically significant correlation with factor activity level (P>0.05). No significant correlation was observed between the factor activity level and any TEG parameter (P>0.05). the factor activity level was negatively correlated with the lag time of the TGA on the contrary positively correlated with the peak thrombin time of the TGA (P<0.05).Conclusions:The global assays do not successfully predict the bleeding phenotype. the BS is a more suitable tool than conventional and global assays for predicting the bleeding phenotype.en_US
dc.identifier.doi10.1097/MPH.0000000000001564en_US
dc.identifier.endpageE530en_US
dc.identifier.issn1077-4114
dc.identifier.issn1536-3678
dc.identifier.issue6en_US
dc.identifier.pmid31343480en_US
dc.identifier.scopus2-s2.0-85088610361en_US
dc.identifier.scopusqualityQ3en_US
dc.identifier.startpageE527en_US
dc.identifier.urihttps://doi.org/10.1097/MPH.0000000000001564
dc.identifier.urihttps://hdl.handle.net/11454/62096
dc.identifier.volume42en_US
dc.identifier.wosWOS:000562759500042en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherLippincott Williams & Wilkinsen_US
dc.relation.ispartofJournal of Pediatric Hematology Oncologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectbleeding phenotypeen_US
dc.subjectglobal hemostasisen_US
dc.subjectFVII deficiencyen_US
dc.titleEvaluation of Bleeding Phenotype of Inherited Factor VII Deficiency in Children With a Bleeding Assessment Tool and Global Assaysen_US
dc.typeArticleen_US

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