{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T10:44:02Z","timestamp":1761561842776},"reference-count":39,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2014,2,21]],"date-time":"2014-02-21T00:00:00Z","timestamp":1392940800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2014,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Diagnostics of von Willebrand disease (<jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>) includes assessment of factor <jats:styled-content style=\"fixed-case\">VIII<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content>) coagulant activity, von Willebrand factor (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>) antigen (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:Ag) and <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> ristocetin cofactor activity (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">RC<\/jats:styled-content>o), and more specific tests as multimeric and genetic analyses are necessary for the correct <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> classification. The <jats:styled-content style=\"fixed-case\">ACL<\/jats:styled-content> AcuStar\u2122 analyzer introduces chemiluminescence (<jats:styled-content style=\"fixed-case\">CL<\/jats:styled-content>) technology in detection of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> with automated <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:Ag and <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">RC<\/jats:styled-content>o assays. Compare <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:Ag\u2010<jats:styled-content style=\"fixed-case\">ELISA<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">RC<\/jats:styled-content>o by aggregometry conventional assays with new <jats:styled-content style=\"fixed-case\">CL VWF<\/jats:styled-content>:Ag\u2010<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">RC<\/jats:styled-content>o\u2010<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content> assays, investigate the ability to make accurate <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> diagnosis and concordance with multimeric and genetic analyses. 146 patients with congenital <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> (51 Type 1; 34 Type2A; 16 Type 2B; 31 Type 2M; 5 Type 2N; 9 Type 3) and 30 healthy normal subjects were included. A comparison was made between <jats:styled-content style=\"fixed-case\">CL<\/jats:styled-content> and conventional methods. Diagnostic evaluation included: <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">RC<\/jats:styled-content>o\/<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:Ag ratio, multimeric distribution (<jats:styled-content style=\"fixed-case\">sodium dodecyl sulfate [SDS]<\/jats:styled-content>\u2010agarose gel) of <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> and genetic analysis in 110 of 146 patients. <jats:styled-content style=\"fixed-case\">CL<\/jats:styled-content> and conventional methods revealed good correlation. Kappa test agreement diagnosis was &gt;0.8. <jats:styled-content style=\"fixed-case\">CL<\/jats:styled-content> diagnostic sensitivity was 100% and specificity 97%. Multimeric and genetic analysis were of help in clarifying 13 discrepancies of diagnosis between methods, of which six discrepancies were explained by lack of conventional methods\u2032 sensibility. <jats:styled-content style=\"fixed-case\">CL<\/jats:styled-content> methodology can detect <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> and discriminate between type 1, 3 and variant forms and offers an automated, faster, sensitive and less cumbersome method when compared to conventional assays, in particular <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>:<jats:styled-content style=\"fixed-case\">RC<\/jats:styled-content>o by aggregometry. In some cases, even with all phenotype and genetic analyses, discrepancies exist in the classification of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/hae.12380","type":"journal-article","created":{"date-parts":[[2014,2,24]],"date-time":"2014-02-24T17:28:47Z","timestamp":1393262927000},"page":"559-567","source":"Crossref","is-referenced-by-count":31,"title":["Diagnosis of inherited von Willebrand disease: comparison of two methodologies and analysis of the discrepancies"],"prefix":"10.1111","volume":"20","author":[{"given":"J.","family":"Costa\u2010Pinto","sequence":"first","affiliation":[{"name":"Servicio de Hematolog\u00eda y Hemoterapia Xerencia Xesti\u00f3n Integrada de A Coru\u00f1a Department of Medicine School of Medicine University of Santiago de Compostela A Coru\u00f1a Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"P\u00e9rez\u2010Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"Servicio de Hematolog\u00eda y Hemoterapia Xerencia Xesti\u00f3n Integrada de A Coru\u00f1a Department of Medicine School of Medicine University of Santiago de Compostela A Coru\u00f1a Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. del C.","family":"Gom\u00e9z\u2010del\u2010Castillo","sequence":"additional","affiliation":[{"name":"Servicio de Hematolog\u00eda y Hemoterapia Xerencia Xesti\u00f3n Integrada de A Coru\u00f1a Department of Medicine School of Medicine University of Santiago de Compostela A Coru\u00f1a Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Lour\u00e9s","sequence":"additional","affiliation":[{"name":"Servicio de Hematolog\u00eda y Hemoterapia Xerencia Xesti\u00f3n Integrada de A Coru\u00f1a Department of Medicine School of Medicine University of Santiago de Compostela A Coru\u00f1a Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c1.","family":"Rodr\u00edguez\u2010Trillo","sequence":"additional","affiliation":[{"name":"Servicio de Hematolog\u00eda y Hemoterapia Xerencia Xesti\u00f3n Integrada de A Coru\u00f1a Department of Medicine School of Medicine University of Santiago de Compostela A Coru\u00f1a Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Batlle","sequence":"additional","affiliation":[{"name":"Servicio de Hematolog\u00eda y Hemoterapia Xerencia Xesti\u00f3n Integrada de A Coru\u00f1a Department of Medicine School of Medicine University of Santiago de Compostela A Coru\u00f1a Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. 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