6533b7d6fe1ef96bd1266645
RESEARCH PRODUCT
Effective hemostasis during minor surgery in a case of hereditary combined deficiency of vitamin K-dependent clotting factors.
Assunta OrecchioniAlessandro CanellaPierpaolo CarusoMirko PinottiMario LapecorellaFrancesco BernardiGuglielmo MarianiPiero Sandro SbrighiM. NapolitanoGiovanna Marchettisubject
VitaminAdultmedicine.medical_specialtyVitamin KBiopsyFactor VII DeficiencyHereditary Combined Deficiency of Vitamin K-dependent Clotting FactorsBlood Loss SurgicalDiseaseFactor VIIaGastroenterologyMixed Function Oxygenaseschemistry.chemical_compoundBlood Coagulation Disorders InheritedInternal medicineVitamin K Epoxide ReductasesGastroscopymedicineHumansRare Inherited Bleeding disorders Minor SurgeryOral anticoagulationClotting factorbusiness.industryHomozygoteWarfarinHematologyGeneral MedicineFactor VIIHemostasis SurgicalRecombinant ProteinsSurgeryCoagulationchemistryHemostasisTooth ExtractionFemaleBlood Coagulation TestsbusinessProtein Cmedicine.drugdescription
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.
year | journal | country | edition | language |
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2009-01-13 | Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis |