Hemostatic management in pediatric patients with type I von Willebrand disease undergoing oral surgery: case report and literature review.

摘要

Von Willebrand disease (vWD) (Online Mendelian Inheritance in Man 193400) is a hematologic condition characterized by reduced platelet adhesiveness linked to factor VIII (FVIII) and von Willebrand factor (vW) deficiencies.1'2 vWF, otherwise known as factor VIII-related protein is a large glycoprotein synthesized by endothelial cells and megakaryocytes, and represents the major component of the FVIII-vWF multimeric complex.3 vWF has bridging molecular functions between vessel walls and platelets. vWF level can be measured by immunoassay as plasmatic levels of vWF antigen (vWP:Ag).