Biological and clinical response to desmopressin (DDAVP) in a retrospective cohort study of children with low von Willebrand factor levels and bleeding history

• DOI: 10.1160/th10-04-0220
• Publication Date: 2010-10-01T05:22:21Z
• Authors: Susana Meschengieser; Adriana Woods; Roberto Chuit; Karina Turdó; Alicia Blanco; María Lazzari; Analía Sánchez-Lu...
• Keywords: Desmopressin; von Willebrand Disease; Antifibrinolytic; Bleeding time

Summary The diagnosis and management of von Willebrand disease (VWD) in paediatrics is challenging. Our aim was to review patient’s characteristics related biological clinical response DDAVP children with low factor (VWF) levels bleeding history from a single institution. We included retrospective cohort 221 (median age 11 years; 137 females): 27 type 1 (VWF within 15–30 IU dL-1) 194 possible 31–49 dL-1). infusion-test performed 214/221 children, 93.4% whom showed good response. Patients were at higher risk DDAVP-test failure: 9/26 (34.6%) vs. 18/188 (9.6%) (RR 3.44, 1.75–6.79; p= 0.002, Fisher’s exact test). In 68 the evaluated 87 times: i) stop bleeding: menorrhagia (13), mucocutaneous (12), haemarthrosis (1); ii) prevent surgical adenotonsillectomy (17), major (15) minor surgery (10); dental procedures (19). No adverse events or observed. treatment effective one dose almost all patients, without antifibrinolytic local therapy, except girl severe haemorrhage during menarche who required replacement therapy. conclusion, patients VWD no infusion-test. this series, proved safe for VWD. Since safe, affordable we consider that wider use should be promoted, especially developing countries.