title: Desmopressin responsiveness at a capped dose of 15  μg in type 1 von Willebrand disease and mild hemophilia A.
abstract: Desmopressin (DDAVP) is commonly used in the treatment of patients with type 1 von Willebrand disease (VWD) and mild hemophilia A. A patient's responsiveness to DDAVP based on a 0.3  μg/kg dose determines future therapeutic efficacy of the drug. The aim of the study was to determine whether a capped dose of 15 μg subcutaneous DDAVP is able to achieve the same level of DDAVP responsiveness as previously reported. This is a retrospective chart review of patients from 1995 to 2013 in adults and children with type 1 VWD and hemophilia A weighing more than 50 kg. Levels of factor VIII, ristocetin cofactor, and von Willebrand factor antigen were measured before and after 1 h of administration of 15 μg of DDAVP. In patients with type 1 VWD, the complete response rate was 82.5% with a partial response rate of 12.5% and 5% nonresponders. In patients with mild hemophilia A, the complete response rate was 53.8% with a partial response rate of 38.5% and 7.7% nonresponders. These results using a capped 15-μg dose of DDAVP are similar to previously published reports using the 0.3-μg/kg dose.
authors.affiliation: Bleeding Disorders Program, University of Western Ontario, London, Ontario, Canada.
authors.forename: Dou-Anne
authors.initials: DA
authors.lastname: Siew
authors.forename: Joy
authors.initials: J
authors.lastname: Mangel
authors.forename: Lori
authors.initials: L
authors.lastname: Laudenbach
authors.forename: Sheila
authors.initials: S
authors.lastname: Schembri
authors.forename: Leonard
authors.initials: L
authors.lastname: Minuk
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage*; D004334:Drug Administration Schedule; D016903:Drug Monitoring; D005169:Factor VIII / Q000378:metabolism; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000188:drug therapy*; D006490:Hemostatics / Q000008:administration & dosage*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D056725:von Willebrand Disease, Type 1 / Q000097:blood / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D006490:Hemostatics; D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin