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.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Biopsy
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Blood Coagulation Disorders, Inherited / complications*
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Blood Coagulation Disorders, Inherited / drug therapy
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Blood Coagulation Disorders, Inherited / genetics
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Blood Coagulation Tests
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Blood Loss, Surgical*
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Factor VII / genetics
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Factor VII Deficiency / drug therapy
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Factor VII Deficiency / genetics*
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Factor VIIa / therapeutic use
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Female
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Gastroscopy
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Hemostasis, Surgical / methods*
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Homozygote
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Humans
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Mixed Function Oxygenases / deficiency*
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Mixed Function Oxygenases / genetics
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Recombinant Proteins / therapeutic use
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Tooth Extraction*
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Vitamin K / therapeutic use
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Vitamin K Epoxide Reductases
Substances
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Recombinant Proteins
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Vitamin K
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Factor VII
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recombinant FVIIa
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Mixed Function Oxygenases
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Vitamin K Epoxide Reductases
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Factor VIIa