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The impact of bleed...
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
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Castaman, Giancarlo (författare)
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Tosetto, Alberto (författare)
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Goodeve, Anne (författare)
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Federici, Augusto B. (författare)
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- Lethagen, Stefan (författare)
- Lund University,Lunds universitet,Institutionen för kliniska vetenskaper, Malmö,Medicinska fakulteten,Department of Clinical Sciences, Malmö,Faculty of Medicine
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Budde, Ulrich (författare)
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Batlle, Javier (författare)
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Meyer, Dominique (författare)
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Mazurier, Claudine (författare)
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Goudemand, Jenny (författare)
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Eikenboom, Jeroen (författare)
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Schneppenheim, Reinhard (författare)
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Ingerslev, Jorgen (författare)
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Habart, David (författare)
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Hill, Frank (författare)
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Peake, Ian (författare)
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Rodeghiero, Francesco (författare)
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(creator_code:org_t)
- 2010-08-25
- 2010
- Engelska.
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Ingår i: British Journal of Haematology. - : Wiley. - 0007-1048. ; 151:3, s. 245-251
- Relaterad länk:
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http://dx.doi.org/10...
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https://onlinelibrar...
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https://lup.lub.lu.s...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- 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.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Nyckelord
- bleeding score
- PFA-100 closure time
- disorders
- inherited bleeding
- von Willebrand disease
- von Willebrand factor
Publikations- och innehållstyp
- art (ämneskategori)
- ref (ämneskategori)
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Till lärosätets databas
- Av författaren/redakt...
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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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Lethagen, Stefan
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Budde, Ulrich
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visa fler...
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Batlle, Javier
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Meyer, Dominique
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Mazurier, Claudi ...
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Goudemand, Jenny
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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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Peake, Ian
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Rodeghiero, Fran ...
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visa färre...
- Om ämnet
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- MEDICIN OCH HÄLSOVETENSKAP
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MEDICIN OCH HÄLS ...
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och Klinisk medicin
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och Hematologi
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British Journal ...
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Lunds universitet