Diagnosis and management of von Willebrand disease in Spain.
| Autor: | Batlle J; Haematology and Haemotherapy Service and INIBIC, Complexo Hospitalario Universitario de A Coruna, A Coruna, Spain. Francisco.Javier.Batlle.Fonrodona@sergas.es, Perez-Rodriguez A, Pinto JC, Fraga EL, Rodriguez-Trillo Tch A, Fernanda Lopez-Fernandez M |
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| Jazyk: | angličtina |
| Zdroj: | Seminars in thrombosis and hemostasis [Semin Thromb Hemost] 2011 Jul; Vol. 37 (5), pp. 503-10. Date of Electronic Publication: 2011 Nov 18. |
| DOI: | 10.1055/s-0031-1281036 |
| Abstrakt: | The correct diagnosis and classification of Von Willebrand disease (VWD) is important for therapy and genetic counseling but is made difficult due to the variability of its clinical expression and limitations of laboratory methods. A national registry of VWD patients has been initiated in Spain. The results of a concise survey on the diagnosis of VWD show the frequency of VWD is fivefold greater in Spain than that expected from epidemiological studies in other European countries; this may result from overdiagnosis and/or a higher prevalence of VWD. These results clearly reinforce the need for the Spanish VWD registry. A consensus guideline for optimal treatment of VWD is being elaborated in Spain. Desmopressin (DDAVP) is the choice of treatment in responsive VWD patients. Von Willebrand factor concentrates (VWF/factor VIII) are used in individuals nonresponsive to DDAVP, when DDAVP is contraindicated, or in VWD types 2B and 3. (© Thieme Medical Publishers.) |
| Databáze: | MEDLINE |
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