Abstract
Low von Willebrand factor (VWF), defined as either VWF antigen (VWF:Ag) or Ristocetin cofactor (VWF:RCo) level ≥ 30 and < 50 iu/dl, is a common finding in paediatric patients tested for von Willebrand Disease (VWD), the most common inherited bleeding disorder. DDAVP (1-deamino-8-D-arginine vasopressin, desmopressin), a synthetic derivative of vasopressin that promotes the release of VWF multimers from Weibel-Palade bodies in the vascular endothelium, is safe and effective in preventing and treating bleeding in children with VWF levels < 50 iu/dl (Gilly, et al 2002, Khair, et al 2007, Leissinger, et al 2001). In our paediatric population, children are often tested for VWD due to a family history of VWD, personal bleeding history or prolonged partial thromboplastin time, as well as, but not limited to, part of a pre-operative evaluation. If found to have VWF:Ag or VWF:RCo <50 iu/dl, patients automatically undergo a DDAVP challenge test to assess laboratory response prior to the administration of DDAVP for the prevention or treatment of bleeding.
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