title: An illustrative case and a review on the dosing of recombinant factor VIIa in congenital factor XI deficiency.
abstract: Recombinant activated factor VII (rFVIIa) has been used in a very limited number of patients with severe factor XI (FXI) deficiency. The dose and duration of treatment has varied greatly between these case reports. In a few of these cases there was also evidence of thrombotic complications. We present here a report on one additional patient with congenital FXI deficiency. For two major orthopaedic procedures in this patient we used rFVIIa as a single bolus dose followed by continuous infusion at a low rate. The data from these treatment episodes, together with those from a review of the published cases, lend support to the concept of using much lower doses than in haemophilia with inhibitors. A bolus dose of 20 microg kg(-1) and thereafter maintenance of the FVII activity at approximately 3 IU mL(-1) appears effective and safe.
authors.affiliation: Coagulation Unit, Department of Hematology, Karolinska University Hospital, Stockholm, Sweden. schulms@mcmaster.ca
authors.forename: S
authors.initials: S
authors.lastname: Schulman
authors.forename: G
authors.initials: G
authors.lastname: Németh
mesh_terms: D005167:Factor VII / Q000008:administration & dosage*; D015942:Factor VIIa; D005173:Factor XI Deficiency / Q000151:congenital* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D007275:Injections, Intravenous; D007430:Intraoperative Care / Q000379:methods; D007431:Intraoperative Complications; D008875:Middle Aged; D019637:Orthopedic Procedures; D011183:Postoperative Complications; D011994:Recombinant Proteins / Q000008:administration & dosage; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa