LAUSR

Congenital factor VII (FVII) deficiency is an autosomal recessive disorder with an estimated prevalence of 1/500,000 individuals without ethnic or gender predilection, the gene for factor VII is found on chromosome 13, adjacent to the gene for factor X. Factor VII deficiency can cause bleeding particularly in cases where factor VII is extremely low, but a few cases where factor VII function is lacking entirely or sub-totally may not present with a history of bleeding. Patients with congenital Factor VII deficiency who require surgery can be treated efficiently and safely with rFVIIa or antifibrinolytic agents. Most reports have recommended replacement therapy for patients with congenital factor VII deficiency that must undergo surgery. It is generally believed that substitution therapy should be administered to patients undergoing surgery and having FVII activity of less than 10% and to patients with a history of recurrent bleeding.