title: Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER.
abstract: Excessive bleeding represents a major complication of surgical interventions and its control is especially relevant in patients with Congenital Bleeding Disorders (CBD). In factor VII (FVII) deficiency, scanty data on surgery is available to guide treatment strategies. The STER (Seven Treatment Evaluation Registry) is a multi-centre, prospective, observational, web-based study protocol providing the frame for a structured and detailed data collection. Inhibitor occurrence was checked in a centralized fashion. Forty-one surgical operations (24 'major' and 17 'minor') were performed in 34 subjects with a carefully characterized FVII deficiency under the coverage of recombinant activated Factor VII (rFVIIa). Bleeding occurred during three major interventions of orthopaedic surgery, but rFVIIa was given at very low dose in each case. An antibody to FVII was observed in one patient who underwent a multiple dental extraction. No thromboses were reported during the 30-d follow up period. Replacement therapy with rFVIIa proved effective when suitable doses were used, which, during the period of maximum bleeding risk (the day of operation), were calculated (Receiver Operated Characteristic analysis) to be of at least 13 μg/kg/body weight per single dose and no less than three administrations. This indication is important especially in the case of major surgery.
authors.affiliation: Department of Internal Medicine & Public Health, University of L'Aquila, Haematology, L'Aquila, Italy. gmariani@cc.univaq.it
authors.forename: Guglielmo
authors.initials: G
authors.lastname: Mariani
authors.forename: Alberto
authors.initials: A
authors.lastname: Dolce
authors.forename: Angelika
authors.initials: A
authors.lastname: Batorova
authors.forename: Günter
authors.initials: G
authors.lastname: Auerswald
authors.forename: Jean Francois
authors.initials: JF
authors.lastname: Schved
authors.forename: Sergio
authors.initials: S
authors.lastname: Siragusa
authors.forename: Mariasanta
authors.initials: M
authors.lastname: Napolitano
authors.forename: Jens Bjerre
authors.initials: JB
authors.lastname: Knudsen
authors.forename: Jørgen
authors.initials: J
authors.lastname: Ingerslev
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016063:Blood Loss, Surgical / Q000517:prevention & control*; D002648:Child; D002675:Child, Preschool; D003029:Coagulants / Q000627:therapeutic use*; D004341:Drug Evaluation / Q000379:methods; D004812:Epidemiologic Methods; D005168:Factor VII Deficiency / Q000150:complications / Q000188:drug therapy*; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006488:Hemostasis, Surgical / Q000379:methods; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011994:Recombinant Proteins / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D003029:Coagulants; D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa