Anti- and procoagulant activities in factor VII-deficient subjects

Thrombosis Research
J AstermarkE Berntorp

Abstract

The clinical feature in patients with congenital factor VII deficiency is in part dependent on the underlying genetic defect, but the mechanisms influencing the genotype-phenotype correlation remain to be fully elucidated. In addition, thromboembolic events have been reported. Compensatory mechanisms involving vitamin K-dependent factors have been suggested. We have measured anticoagulant activities in 25 factor VII-deficient subjects (factor VII activity < or =36%) and 23 age-matched controls and correlated these to the vitamin K-dependent procoagulant activities. Two of the patients had a history of thromboembolism. The factor VII-deficient patients were found to have a significantly lower protein C activity than the controls [0.84 U/ml (95% CI 0.78; 0.89) vs. 0.98 U/ml (95% CI 0.91; 1.05), P=.004]. In addition, the protein C activity was correlated to that of factor VII (r=.36; P=.014), factor IX (r=.45; P=.002) and factor X (r=.50; P=.0006), respectively. The level of prothrombin fragment 1+2 was correlated to the protein C (r=.40; P=.012) and to the factor VII activity (r=.42; P=.011). No differences between patients and controls were seen regarding total and free protein S, antithrombin, plasminogen activator inhibitor-1 ...Continue Reading

References

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Citations

Oct 1, 2004·Journal of Thrombosis and Haemostasis : JTH·K Brummel ZiedinsK G Mann
Oct 3, 2003·Journal of Thrombosis and Haemostasis : JTH·G MarianiUNKNOWN International Factor VII Deficiency Study Group
Mar 5, 2005·Journal of Cardiothoracic and Vascular Anesthesia·Rosaleen ChunDavid Royston

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