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The impact of bleed...
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Castaman, Giancarlo
(author)
The impact of bleeding history, von Willebrand factor and PFA-100 (R) on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM-1VWD
- Article/chapterEnglish2010
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LIBRIS-ID:oai:lup.lub.lu.se:8c45b0a6-b880-4d18-aef8-fc0cb5fc7eba
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https://lup.lub.lu.se/record/1721017URI
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https://doi.org/10.1111/j.1365-2141.2010.08333.xDOI
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Language:English
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Summary in:English
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Subject category:art swepub-publicationtype
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Subject category:ref swepub-contenttype
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P>The relationships between the Platelet Function Analyzer (PFA)-100 and von Willebrand factor (VWF) levels and bleeding score (BS) were evaluated within a multicentre project on Molecular and Clinical Markers for the Diagnosis and Management of type 1 von Willebrand disease (MCMDM-1VWD). PFA-100 closure time, either with epinephrine (EPI) or adenosine diphosphate (ADP)-cartridges, was measured in 107 index cases, 105 affected and 71 unaffected family members, and 79 healthy controls. By regression analysis VWF levels were strongly related to both closure times, with a non-linear progression. In a multiple stepwise regression model, age- and sex-adjusted PFA-100 ADP and VWF ristocetin cofactor activity (VWF:RCo) were independently associated with BS. Most of the variation of BS was predicted by PFA-100 ADP and VWF:RCo alone. In the subgroup of patients with subtle abnormalities of the multimeric pattern, VWF was invariably reduced and closure time prolonged in almost all of them. Neither PFA-100 ADP nor EPI closure times appeared to significantly improve the diagnostic capability of VWF antigen (VWF:Ag) measurement. Thus, in an unselected population a normal PFA-100 would be useful to exclude VWD, but whether it could replace the more specific VWF assay in patients with significant mucocutaneous bleeding symptoms remains to be investigated prospectively.
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Tosetto, Alberto
(author)
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Goodeve, Anne
(author)
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Federici, Augusto B.
(author)
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Lethagen, StefanLund University,Lunds universitet,Institutionen för kliniska vetenskaper, Malmö,Medicinska fakulteten,Department of Clinical Sciences, Malmö,Faculty of Medicine(Swepub:lu)medf-sle
(author)
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Budde, Ulrich
(author)
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Batlle, Javier
(author)
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Meyer, Dominique
(author)
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Mazurier, Claudine
(author)
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Goudemand, Jenny
(author)
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Eikenboom, Jeroen
(author)
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Schneppenheim, Reinhard
(author)
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Ingerslev, Jorgen
(author)
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Habart, David
(author)
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Hill, Frank
(author)
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Peake, Ian
(author)
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Rodeghiero, Francesco
(author)
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Institutionen för kliniska vetenskaper, MalmöMedicinska fakulteten
(creator_code:org_t)
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In:British Journal of Haematology: Wiley151:3, s. 245-2510007-1048
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Castaman, Gianca ...
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Tosetto, Alberto
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Goodeve, Anne
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Federici, August ...
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Budde, Ulrich
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Batlle, Javier
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Meyer, Dominique
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Eikenboom, Jeroe ...
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Schneppenheim, R ...
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Ingerslev, Jorge ...
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Habart, David
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Hill, Frank
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