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.

Effective Hemostasis During Minor Surgery in a Case of Hereditary Combined Deficiency of Vitamin K-dependent Clotting Factors.

BERNARDI, Francesco;PINOTTI, Mirko;MARCHETTI, Giovanna;CANELLA, Alessandro;CARUSO, Pierpaolo;
2010

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.
Lapecorella, M; Napolitano, M; Bernardi, Francesco; Pinotti, Mirko; Sbrighi, P; Marchetti, Giovanna; Canella, Alessandro; Caruso, Pierpaolo; Orecchioni, A; Mariani, G.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11392/533122
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