Effective hemostasis during minor surgery in a case of hereditary combined deficiency of vitamin K-dependent clotting factors.
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.
Lapecorella
Mario
M
Haemophilia and Thrombosis Centre, University of L'Aquila, Ospedale San Salvatore, Via Vetoio 1, Coppito, L'Aquila, Italy. emofilia.aq@cc.univaq.it
Napolitano
Mariasanta
M
Bernardi
Francesco
F
Pinotti
Mirko
M
Sbrighi
Piero Sandro
PS
Marchetti
Giovanna
G
Canella
Alessandro
A
Caruso
Pierpaolo
P
Orecchioni
Assunta
A
Mariani
Guglielmo
G
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
D011994:Recombinant Proteins; D014812:Vitamin K; D005167:Factor VII; C103587:recombinant FVIIa; D006899:Mixed Function Oxygenases; D064417:Vitamin K Epoxide Reductases; D015942:Factor VIIa