Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency

Abstract

Factor XI (FXI) deficiency is a rare inherited bleeding disorder caused by a mutation in the FXI gene, which is located on the distal arm of chromosome 4 (4q35). Factor XI (FXI) deficiency is defined when the activity of FXI in plasma is less than 15 U/dL. We report our experience about perioperative use of recombinant activated factor VII (rFVIIa) in a 17 year-old with congenital FXI deficiency and appendicitis. After appendectomy, the first intravenous dose of rFVIIa was administered. This same dosage was repeated and he was discharged on postoperative 72nd hour without complications.