Literature DB >> 22906074

von Willebrand's disease: a report from a meeting in the Åland islands.

E Berntorp1, I Peake, U Budde, M Laffan, R Montgomery, J Windyga, A Goodeve, P Petrini, M von Depka, W Miesbach, D Lillicrap, A B Federici, R Lassila, G White.   

Abstract

von Willebrand's disease (VWD) is probably the most common bleeding disorder, with some studies indicating that up to 1% of the population may have the condition. Over recent years interest in VWD has fallen compared to that of haemophilia, partly the result of focus on blood-borne diseases such as HIV and hepatitis. Now the time has come to revisit VWD, and in view of this some 60 international physicians with clinical and scientific interest in VWD met over 4 days in 2010 in the Åland islands to discuss state-of-the-art issues in the disease. The Åland islands are where Erik von Willebrand had first observed a bleeding disorder in a number of members of a family from Föglö, and 2010 was also the 140th anniversary of his birth. This report summarizes the main papers presented at the symposium; topics ranged from genetics and biochemistry through to classification of VWD, pharmacokinetics and laboratory assays used in the diagnosis of the disease, inhibitors, treatment guidelines in different age groups including the elderly who often have comorbid conditions that present challenges, and prophylaxis. Other topics included managing surgeries in patients with VWD and the role of FVIII in VWF replacement, a controversial subject.
© 2012 Blackwell Publishing Ltd.

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Year:  2012        PMID: 22906074      PMCID: PMC4170876          DOI: 10.1111/j.1365-2516.2012.02925.x

Source DB:  PubMed          Journal:  Haemophilia        ISSN: 1351-8216            Impact factor:   4.287


  43 in total

1.  Pharmacokinetics and hemostatic effect of different factor VIII/von Willebrand factor concentrates in von Willebrand's disease type III.

Authors:  S Lethagen; E Berntorp; I M Nilsson
Journal:  Ann Hematol       Date:  1992-12       Impact factor: 3.673

2.  Structures of the asparagine-linked oligosaccharide chains of human von Willebrand factor. Occurrence of blood group A, B, and H(O) structures.

Authors:  T Matsui; K Titani; T Mizuochi
Journal:  J Biol Chem       Date:  1992-05-05       Impact factor: 5.157

Review 3.  Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor.

Authors:  J E Sadler; U Budde; J C J Eikenboom; E J Favaloro; F G H Hill; L Holmberg; J Ingerslev; C A Lee; D Lillicrap; P M Mannucci; C Mazurier; D Meyer; W L Nichols; M Nishino; I R Peake; F Rodeghiero; R Schneppenheim; Z M Ruggeri; A Srivastava; R R Montgomery; A B Federici
Journal:  J Thromb Haemost       Date:  2006-08-02       Impact factor: 5.824

4.  Amino acid sequence of human von Willebrand factor.

Authors:  K Titani; S Kumar; K Takio; L H Ericsson; R D Wade; K Ashida; K A Walsh; M W Chopek; J E Sadler; K Fujikawa
Journal:  Biochemistry       Date:  1986-06-03       Impact factor: 3.162

Review 5.  Prophylaxis in von Willebrand disease.

Authors:  E Berntorp
Journal:  Haemophilia       Date:  2008-11       Impact factor: 4.287

6.  Incidence of bleeding complications in pediatric patients with type 1 von Willebrand disease undergoing adenotonsillar procedures.

Authors:  Char M Witmer; Lisa Elden; Regina B Butler; Catherine S Manno; Leslie J Raffini
Journal:  J Pediatr       Date:  2009-04-24       Impact factor: 4.406

7.  An influence of ABO blood group on the rate of proteolysis of von Willebrand factor by ADAMTS13.

Authors:  D J Bowen
Journal:  J Thromb Haemost       Date:  2003-01       Impact factor: 5.824

8.  Efficacy and safety of highly purified, doubly virus-inactivated VWF/FVIII concentrates in inherited von Willebrand's disease: results of an Italian cohort study on 120 patients characterized by bleeding severity score.

Authors:  A B Federici; G Barillari; E Zanon; M G Mazzucconi; R Musso; R Targhetta; P M Mannucci
Journal:  Haemophilia       Date:  2009-10-06       Impact factor: 4.287

Review 9.  Beyond lectins: the calnexin/calreticulin chaperone system of the endoplasmic reticulum.

Authors:  David B Williams
Journal:  J Cell Sci       Date:  2006-02-15       Impact factor: 5.285

Review 10.  How to estimate bleeding risk in mild bleeding disorders.

Authors:  F Rodeghiero; A Tosetto; G Castaman
Journal:  J Thromb Haemost       Date:  2007-07       Impact factor: 5.824

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  4 in total

1.  Peri-operative DDAVP Use Leading to Severe Hyponatremia after Total Shoulder Replacement in a Patient with von Willebrand's Disease.

Authors:  James S MacKenzie; Stuart C Kozinn
Journal:  HSS J       Date:  2015-07-17

Review 2.  Alloantibodies in von Willebrand disease.

Authors:  Paula D James; David Lillicrap; Pier M Mannucci
Journal:  Blood       Date:  2013-01-07       Impact factor: 22.113

3.  Utility of a Paediatric Bleeding Questionnaire as a screening tool for von Willebrand disease in apparently healthy children.

Authors:  N Mittal; R Naridze; P James; S Shott; L A Valentino
Journal:  Haemophilia       Date:  2015-05-16       Impact factor: 4.287

4.  Third Åland islands conference on von Willebrand disease, 26-28 September 2012: meeting report.

Authors:  E Berntorp; B Fuchs; M Makris; R Montgomery; V Flood; J S O'Donnell; A B Federici; D Lillicrap; P James; U Budde; M Morfini; P Petrini; S Austin; C Kannicht; V Jiménez-Yuste; C Lee
Journal:  Haemophilia       Date:  2013-03       Impact factor: 4.287

  4 in total

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