title: Effective hemostasis during minor surgery in a case of hereditary combined deficiency of vitamin K-dependent clotting factors.
abstract: Combined deficiency of the vitamin K-dependent clotting factors (VKCFD) is a rare bleeding disorder involving defective gamma-carboxylation of coagulation factors II , VII, IX and X as well as natural anticoagulants protein C and protein S. The disease is characterized by a cluster of different, often life threatening, bleeding symptoms occurring both spontaneously and in a surgical setting. In the present paper we describe two different treatment modalities to be used both in a programmed surgical procedure and in an emergency scenario. As this disease is a natural model that resembles oral anticoagulation, our experience discloses a possible rationale in the use of recombinant activated FVII for warfarin reversal.
authors.affiliation: Haemophilia and Thrombosis Centre, University of L'Aquila, Ospedale San Salvatore, Via Vetoio 1, Coppito, L'Aquila, Italy. emofilia.aq@cc.univaq.it
authors.forename: Mario
authors.initials: M
authors.lastname: Lapecorella
authors.forename: Mariasanta
authors.initials: M
authors.lastname: Napolitano
authors.forename: Francesco
authors.initials: F
authors.lastname: Bernardi
authors.forename: Mirko
authors.initials: M
authors.lastname: Pinotti
authors.forename: Piero Sandro
authors.initials: PS
authors.lastname: Sbrighi
authors.forename: Giovanna
authors.initials: G
authors.lastname: Marchetti
authors.forename: Alessandro
authors.initials: A
authors.lastname: Canella
authors.forename: Pierpaolo
authors.initials: P
authors.lastname: Caruso
authors.forename: Assunta
authors.initials: A
authors.lastname: Orecchioni
authors.forename: Guglielmo
authors.initials: G
authors.lastname: Mariani
mesh_terms: D000328:Adult; D001706:Biopsy; D025861:Blood Coagulation Disorders, Inherited / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D001780:Blood Coagulation Tests; D016063:Blood Loss, Surgical; D005167:Factor VII / Q000235:genetics; D005168:Factor VII Deficiency / Q000188:drug therapy / Q000235:genetics*; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D005773:Gastroscopy; D006488:Hemostasis, Surgical / Q000379:methods*; D006720:Homozygote; D006801:Humans; D006899:Mixed Function Oxygenases / Q000172:deficiency* / Q000235:genetics; D011994:Recombinant Proteins / Q000627:therapeutic use; D014081:Tooth Extraction; D014812:Vitamin K / Q000627:therapeutic use; D064417:Vitamin K Epoxide Reductases
chemical_list: D011994:Recombinant Proteins; D014812:Vitamin K; D005167:Factor VII; C103587:recombinant FVIIa; D006899:Mixed Function Oxygenases; D064417:Vitamin K Epoxide Reductases; D015942:Factor VIIa