An evaluation of the DDAVP infusion test with PFA-100 and vWF activity assays to distinguish vWD types in children

dc.contributor.authorAkin M.
dc.contributor.authorKarapinar D.Y.
dc.contributor.authorBalkan C.
dc.contributor.authorAy Y.
dc.contributor.authorKavakli K.
dc.date.accessioned2019-10-27T08:33:31Z
dc.date.available2019-10-27T08:33:31Z
dc.date.issued2011
dc.departmentEge Üniversitesien_US
dc.description.abstractvon Willebrand disease (vWD) is classified into partial (type 1), qualitative (type 2), and total deficiency (type 3).The aims of the study were to evaluate prospectively the potency of the DDAVP infusion test together with von Willebrand factor (vWF) ristocetin cofactor (vWF:RCo), vWF antigen (vWF:Ag), factor VIII coagulant activity (FVIII:C), and platelet function analyzer (PFA)-100 to distinguish vWD types. Genetic analysis and multimeric analysis of vWF was not applied. We classified the 112 patients and 47 healthy children phenotypically according to the laboratory test results and bleeding severity score. PFA-100 closure times (CT), FVIII:C, vWF:RCo, vWF:Ag, ristocetin-induced platelet aggregation (RIPA), and the response of FVIII:C and vWF parameters to desmopressin (DDAVP) were used to define types 1, 2, and 3 vWD. Type 1 vWD is mild in 34 cases (vWF:RCo % 40-55), moderate in 29 (vWF:RCo %27-40), severe type 1 vWD or nonclassical type 2 vWD in 12 cases (vWF:RCo % 4-16), and type 2 vWD in 23 cases (vWF:RCo %4-38).The response to DDAVP of vWF parameters is normal in all patients with mild/moderate type 1 vWD, 6 patients with severe type 1 vWD or nonclassical type 2 vWD and 11 patients with type 2 vWD. In conclusion, this study showed that measurement of vWF:RCo, vWF:Ag, FVIII:C, and PFA-100 parameters can differentiate vWD types but not severe type 1 vWD or nonclassical type 2 vWD. In the differentiation of severe type 1 vWD and nonclassical type 2 vWD, DDAVP response may be used. © SAGE Publications 2011.en_US
dc.identifier.doi10.1177/1076029610366440en_US
dc.identifier.endpage448en_US
dc.identifier.issn1076-0296
dc.identifier.issue5en_US
dc.identifier.pmid20460340en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage441en_US
dc.identifier.urihttps://doi.org/10.1177/1076029610366440
dc.identifier.urihttps://hdl.handle.net/11454/26798
dc.identifier.volume17en_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.relation.ispartofClinical and Applied Thrombosis/Hemostasisen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectDDAVPen_US
dc.subjectPFA-100en_US
dc.subjectvon Willebrand disease (vWD)en_US
dc.subjectvon Willebrand factor (vWF)en_US
dc.titleAn evaluation of the DDAVP infusion test with PFA-100 and vWF activity assays to distinguish vWD types in childrenen_US
dc.typeArticleen_US

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