Treatment of congenital bleeding disorders: future trends in management.

摘要

The coagulation pathway is comprised of a complex interaction of many elements, including various coagulation factors, which are essential for stopping the loss of blood at the site of an injury and laying the foundation for injury repair and wound healing. Disruption in any part of this pathway can lead to bleeding that may be serious and life threatening. Defects and/or deficiencies in coagulation factors are common causes of congenital bleeding disorders, such as haemophilia A (factor VIII deficiency), haemophilia B (factor IX deficiency), von Willebrand disease (von Willebrand factor deficiency or dysfunction), and the fibrinogen disorders afibrinogenemia, hypofibrinogenemia and dysfibrinogenemia (fibrinogen deficiency/defect).