1. Desmopressin responsiveness at a capped dose of 15 μg in type 1 von Willebrand disease and mild hemophilia A
- Author
-
Dou-Anne Siew, Sheila Schembri, Joy Mangel, Lori Laudenbach, and Leonard Minuk
- Subjects
Adult ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Adolescent ,Hemophilia A ,urologic and male genital diseases ,Severity of Illness Index ,von Willebrand Disease, Type 1 ,Gastroenterology ,Drug Administration Schedule ,Hemostatics ,Ristocetin Cofactor ,Von Willebrand factor ,Mild hemophilia A ,hemic and lymphatic diseases ,Internal medicine ,von Willebrand Factor ,Severity of illness ,medicine ,Von Willebrand disease ,Humans ,Deamino Arginine Vasopressin ,In patient ,Desmopressin ,Aged ,Retrospective Studies ,Aged, 80 and over ,Factor VIII ,biology ,business.industry ,Retrospective cohort study ,Hematology ,General Medicine ,Middle Aged ,medicine.disease ,biology.protein ,Female ,Drug Monitoring ,business ,hormones, hormone substitutes, and hormone antagonists ,medicine.drug - 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.
- Published
- 2014
- Full Text
- View/download PDF