Response of patients with hemophilia A and von Willebrand disease to desmopressin (DDAVP).
Factor VIII (FVIII) response to desmopressin (deamino-8-D-arginine vasopressin, abbreviated DDAVP) was studied in patients with mild Hemophilia A and von Willebrand Disease (vWD) who attend our Hemophilia Clinic. Thirty eight children and 9 adults had their F VIII components assayed 60 minutes after intravenous (IV) administration of DDAVP, 0.35 microgram/kg. Among 27 hemophiliacs, F VIII coagulant activity (F VIII: C) increased from a mean of 16.8 to 59.2 u/dL; with an average 3.2-fold increase. In 20 vWD patients, the mean F VIII:C and von Willebrand Factor increased from a mean of 50.1 to 136%; and from 22.6 to 75.6%; with an average 3.0 and 5.7-fold increase, respectively. The overall F VIII:C response was good or excellent in 81.5% of the hemophiliacs, and in 89.5% of the vWD patients tested. No significant side effects were observed. This study has demonstrated that IV DDAVP can cause an increase of F VIII:C and vWF to hemostatic levels, and thus it may be useful for the control of bleeding episodes in most of the patients tested in our clinic.
Santiago-Borrero
P J
PJ
Department of Pediatrics, University of Puerto Rico Medical Sciences, School of Medicine, San Juan.
Casanova
R
R
D000328:Adult; D002648:Child; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D004341:Drug Evaluation; D005169:Factor VIII / Q000032:analysis; D006467:Hemophilia A / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D014842:von Willebrand Diseases / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000032:analysis
D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin