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  • Castaman, G. (author)

Hemorrhagic symptoms and bleeding risk in obligatory carriers of type 3 von Willebrand disease: an international, multicenter study

  • Article/chapterEnglish2006

Publisher, publication year, extent ...

  • Elsevier BV,2006

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  • LIBRIS-ID:oai:lup.lub.lu.se:677d7ab2-55d8-41c7-bc6a-e584955a9f85
  • https://lup.lub.lu.se/record/686163URI
  • https://doi.org/10.1111/j.1538-7836.2006.02070.xDOI
  • http://kipublications.ki.se/Default.aspx?queryparsed=id:1944076URI

Supplementary language notes

  • Language:English
  • Summary in:English

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

Notes

  • Objectives: We undertook an international, multicenter study to describe the clinical picture and to estimate the bleeding risk in a group of obligatory carriers of type 3 von Willebrand disease (VWD). Patients and methods: Obligatory carriers (OC) of type 3 VWD were identified by the presence of offspring with type 3 VWD or by being an offspring of a type 3 patient. Normal controls were age- and sex-matched with the obligatory carriers. A physician-administered standardized questionnaire was used to evaluate hemorrhagic symptoms at presentation. A score system ranging from 0 (no symptom) to 3 (hospitalization, replacement therapy, blood transfusion) was used to quantitate bleeding manifestations. Odds ratios were computed for each symptom. Results: Ten centers participated to the study, enrolling a total of 35 type 3 VWD families, with 70 OC. A total of 215 normal controls and 42 OC for type 1 VWD were also included. About 40% of type 3 OC had at least one bleeding symptom compared to 23% of normal controls and 81.8% of type 1 OC (P < 0.0001 by chi-squared test), showing that type 3 OC clearly represent a distinct population from type 1 OC. The clinical situations associated with an increase of bleeding risk in type 3 OC were epistaxis [odds ratio 3.6; 90% confidence intervals (CI) 1.84-21.5], cutaneous bleeding (odds ratio 5.5; 90% CI 2.5-14.1) and postsurgical bleeding (odds ratio 16.3; 90% CI 4.5-59). The severity of bleeding score correlated with the degree of factor (F) VIII reduction in plasma. Conclusions: OC for type 3 VWD represent a distinctive population from type 1 OC. These patients, however, present with more frequent bleeding symptoms in comparison to normal controls, especially in case of significantly low FVIII. Desmopressin and/or tranexamic acid might be useful to prevent or treat bleeding in these cases.

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Added entries (persons, corporate bodies, meetings, titles ...)

  • Rodeghiero, F. (author)
  • Tosetto, A. (author)
  • Cappelletti, A. (author)
  • Baudo, F. (author)
  • Eikenboom, J. C. J. (author)
  • Federici, A. B. (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)
  • Linari, S. (author)
  • Lusher, J. (author)
  • Nishino, M. (author)
  • Petrini, P. (author)
  • Srivastava, A. (author)
  • Ungerstedt, J. S.Karolinska Institutet (author)
  • Institutionen för kliniska vetenskaper, MalmöMedicinska fakulteten (creator_code:org_t)

Related titles

  • In:Journal of Thrombosis and Haemostasis: Elsevier BV4:10, s. 2164-21691538-79331538-7836

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