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  • Tosetto, A (author)

A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD)

  • Article/chapterEnglish2006

Publisher, publication year, extent ...

  • Elsevier BV,2006

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  • LIBRIS-ID:oai:lup.lub.lu.se:917e9df2-c39e-4b86-88d5-27fdc1492924
  • https://lup.lub.lu.se/record/416336URI
  • https://doi.org/10.1111/j.1538-7836.2006.01847.xDOI

Supplementary language notes

  • Language:English
  • Summary in:English

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  • Subject category:art swepub-publicationtype
  • Subject category:ref swepub-contenttype

Notes

  • 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)
  • Castaman, G (author)
  • Goodeve, A (author)
  • Federici, AB (author)
  • Batlle, J (author)
  • Meyer, D (author)
  • Fressinaud, E (author)
  • Mazurier, C (author)
  • Goudemand, J (author)
  • Eikenboom, J (author)
  • Schneppenheim, R (author)
  • Budde, U (author)
  • Ingerslev, J (author)
  • Vorlova, Z (author)
  • Habart, D (author)
  • 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)
  • 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)
  • Pasi, J (author)
  • Hill, F (author)
  • Peake, I (author)
  • Pediatrik, LundSektion V (creator_code:org_t)

Related titles

  • In:Journal of Thrombosis and Haemostasis: Elsevier BV4:4, s. 766-7731538-79331538-7836

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