title: Total hip arthroplasty in severe congenital factor VII deficiency: successful use of recombinant activated factor VII for hemostasis.
abstract: Congenital factor VII (FVII) deficiency is a rare hemorrhagic disorder, and, in affected patients, surgery is likely to cause excessive bleeding. In this article, we report the first case of total hip arthroplasty in a patient with severe congenital FVII deficiency in which recombinant activated factor VII (rFVIIa, NovoSeven) was successfully used to manage bleeds. The patient was a 68-year-old woman with severe congenital FVII deficiency, who suffered from a hemorrhagic arthrosis of her left hip joint. We administered rFVIIa as intermittent bolus infusions both perioperatively and postoperatively; dosing was based on the results of a preoperative rFVIIa challenge test. During surgery and the 3-day postoperative treatment period, we observed normal hemostasis with no excessive bleeding or complications.
authors.affiliation: Department of Orthopaedic Surgery, Kobe University Graduate School of Medicine, Kobe, Hyogo 650-0017, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Niikura
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Nishikawa
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Saegusa
authors.forename: Takaaki
authors.initials: T
authors.lastname: Fujishiro
authors.forename: Shinichi
authors.initials: S
authors.lastname: Yoshiya
authors.forename: Masahiro
authors.initials: M
authors.lastname: Kurosaka
mesh_terms: D000368:Aged; D019644:Arthroplasty, Replacement, Hip; D016063:Blood Loss, Surgical / Q000503:physiopathology / Q000517:prevention & control*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005168:Factor VII Deficiency / Q000097:blood / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D015942:Factor VIIa / Q000008:administration & dosage* / Q000378:metabolism; D005260:Female; D006395:Hemarthrosis / Q000097:blood / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D007262:Infusions, Intravenous; D015207:Osteoarthritis, Hip / Q000097:blood / Q000000981:diagnostic imaging / Q000601:surgery*; D011292:Premedication; D011859:Radiography; D011994:Recombinant Proteins / Q000008:administration & dosage
chemical_list: D011994:Recombinant Proteins; D015942:Factor VIIa