title: Diagnosis and management of von Willebrand disease in Spain.
abstract: 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.
authors.affiliation: Haematology and Haemotherapy Service and INIBIC, Complexo Hospitalario Universitario de A Coruna, A Coruna, Spain. Francisco.Javier.Batlle.Fonrodona@sergas.es
authors.forename: Javier
authors.initials: J
authors.lastname: Batlle
authors.forename: Almudena
authors.initials: A
authors.lastname: Perez-Rodriguez
authors.forename: Joana Costa
authors.initials: JC
authors.lastname: Pinto
authors.forename: Esther Loures
authors.initials: EL
authors.lastname: Fraga
authors.forename: Angela
authors.initials: A
authors.lastname: Rodriguez-Trillo Tch
authors.forename: Maria
authors.initials: M
authors.lastname: Fernanda Lopez-Fernandez
mesh_terms: D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005169:Factor VIII / Q000627:therapeutic use; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D017410:Practice Guidelines as Topic; D015995:Prevalence; D013030:Spain / Q000453:epidemiology; D011795:Surveys and Questionnaires; D014842:von Willebrand Diseases / Q000175:diagnosis* / Q000188:drug therapy* / Q000453:epidemiology; D014841:von Willebrand Factor / Q000627:therapeutic use
chemical_list: D006490:Hemostatics; D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin