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A quantitative anal...
A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD)
- Article/chapterEnglish2006
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LIBRIS-ID:oai:lup.lub.lu.se:917e9df2-c39e-4b86-88d5-27fdc1492924
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https://lup.lub.lu.se/record/416336URI
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https://doi.org/10.1111/j.1538-7836.2006.01847.xDOI
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Language:English
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Summary in:English
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Background: A quantitative description of bleeding symptoms in type 1 von Willebrand disease (VWD) has never been reported. Objectives: The aim was to quantitatively evaluate the severity of bleeding symptoms in type 1 VWD and its correlation with clinical and laboratory features. Patients and methods: Bleeding symptoms were retrospectively recorded in a European cohort of VWD type 1 families, and for each subject a quantitative bleeding score (BS) was obtained together with phenotypic tests. Results: A total of 712 subjects belonging to 144 families and 195 controls were available for analysis. The BS was higher in index cases than in affected family members (BS 9 vs. 5, P < 0.0001) and in unaffected family members than in controls (BS 0 vs. -1, P < 0.0001). There was no effect of ABO blood group. BS showed a strong significant inverse relation with either von Willebrand ristocetin cofactor (VWF:RCo), von Willebrand antigen (VWF:Ag) or factor VIII procoagulant activity (FVIII:C) measured at time of enrollment, even after adjustment for age, sex and blood group (P < 0.001 for all the four upper quintiles of BS vs. the first quintile, for either VWF:RCo, VWF:Ag or FVIII:C). Higher BS was related with increasing likelihood of VWD, and a mucocutaneous BS (computed from spontaneous, mucocutaneous symptoms) was strongly associated with bleeding after surgery or tooth extraction. Conclusions: Quantitative analysis of bleeding symptoms is potentially useful for a more accurate diagnosis of type 1 VWD and to develop guidelines for its optimal treatment.
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Rodeghiero, F
(author)
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Castaman, G
(author)
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Goodeve, A
(author)
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Federici, AB
(author)
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Batlle, J
(author)
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Meyer, D
(author)
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Fressinaud, E
(author)
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Mazurier, C
(author)
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Goudemand, J
(author)
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Eikenboom, J
(author)
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Schneppenheim, R
(author)
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Budde, U
(author)
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Ingerslev, J
(author)
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Vorlova, Z
(author)
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Habart, D
(author)
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Holmberg, LarsLund University,Lunds universitet,Pediatrik, Lund,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Paediatrics (Lund),Section V,Department of Clinical Sciences, Lund,Faculty of Medicine(Swepub:lu)pedi-lho
(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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Pasi, J
(author)
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Hill, F
(author)
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Peake, I
(author)
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Pediatrik, LundSektion V
(creator_code:org_t)
Related titles
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In:Journal of Thrombosis and Haemostasis: Elsevier BV4:4, s. 766-7731538-79331538-7836
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Tosetto, A
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Rodeghiero, F
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Castaman, G
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Goodeve, A
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Federici, AB
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Batlle, J
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show more...
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Meyer, D
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Fressinaud, E
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Mazurier, C
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Goudemand, J
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Eikenboom, J
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Schneppenheim, R
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Budde, U
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Ingerslev, J
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Vorlova, Z
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Habart, D
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Holmberg, Lars
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Lethagen, Stefan
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Pasi, J
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Hill, F
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Peake, I
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Lund University