DDAVP (desmopressin; 1-deamino-cys-8-D-arginine-vasopressin) treatment in children with haemophilia B

Br J Haematol. 2000 Dec;111(4):1260-2. doi: 10.1046/j.1365-2141.2000.02504.x.

Abstract

We tested the response to desmopressin (1-deamino-cys-8-D-arginine-vasopressin; DDAVP) in four patients with haemophilia B [factor IX (F IX) at diagnosis 1.4-5%]. The activated partial thromboplastin time (aPTT) was significantly shortened in all patients. Although there was an up to 1.4-fold increase in F IX levels in three patients, maximal F IX activity remained below 10%. Much more prominent were the increases in F VIII (three- to fourfold), in von Willebrand factor antigen (VWF:Ag; 2.5-fold) and particularly in VWF collagen-binding activity (VWF:CBA; fivefold). These changes were reflected by the prophylactic efficacy of DDAVP for dental surgery. After pretesting, DDAVP could be a useful drug for reducing the need for plasma products for prevention of minor surgical bleeding in patients with mild to moderate haemophilia B.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Deamino Arginine Vasopressin / therapeutic use*
  • Dentistry, Operative
  • Factor IX / metabolism
  • Factor VIII / metabolism
  • Hemophilia B / complications*
  • Hemophilia B / drug therapy
  • Hemostatics / therapeutic use*
  • Humans
  • Male
  • Oral Hemorrhage / etiology*
  • Oral Hemorrhage / prevention & control
  • Partial Thromboplastin Time
  • Postoperative Hemorrhage / etiology
  • Postoperative Hemorrhage / prevention & control
  • Premedication*
  • von Willebrand Factor / metabolism

Substances

  • Hemostatics
  • von Willebrand Factor
  • Factor VIII
  • Factor IX
  • Deamino Arginine Vasopressin