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Anti- and procoagulant activities in factor VII-deficient subjects

Astermark, Jan LU ; Tengborn, L; Hedner, Ulla LU and Berntorp, Erik LU (2001) In Thrombosis Research 101(6). p.435-440
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... (More)
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 (PAI-1) and tissue factor pathway inhibitor (TFPI). Seven of the patients were found to have the Factor V Leiden mutation, but none of them had experienced any thromboembolic event. The present data support the notion that compensatory hemostatic mechanisms might exist in that the protein C activity was found to be decreased in the factor VII-deficient subjects. Whether this could influence the clinical feature, including the risk of thromboembolic events in association with replacement therapy, remains to be evaluated. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
TFPI, Protein C, Hemostasis, Factor VII deficiency, Thrombosis, Anticoagulant activity
in
Thrombosis Research
volume
101
issue
6
pages
435 - 440
publisher
Elsevier Ltd
external identifiers
  • pmid:11323000
  • scopus:0035868253
ISSN
1879-2472
DOI
10.1016/S0049-3848(00)00410-2
language
English
LU publication?
yes
id
b8cf1e24-0548-49e8-baed-09300e62db3e (old id 1120921)
date added to LUP
2008-06-24 11:49:17
date last changed
2018-05-29 10:17:50
@article{b8cf1e24-0548-49e8-baed-09300e62db3e,
  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 &lt; 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 (PAI-1) and tissue factor pathway inhibitor (TFPI). Seven of the patients were found to have the Factor V Leiden mutation, but none of them had experienced any thromboembolic event. The present data support the notion that compensatory hemostatic mechanisms might exist in that the protein C activity was found to be decreased in the factor VII-deficient subjects. Whether this could influence the clinical feature, including the risk of thromboembolic events in association with replacement therapy, remains to be evaluated.},
  author       = {Astermark, Jan and Tengborn, L and Hedner, Ulla and Berntorp, Erik},
  issn         = {1879-2472},
  keyword      = {TFPI,Protein C,Hemostasis,Factor VII deficiency,Thrombosis,Anticoagulant activity},
  language     = {eng},
  number       = {6},
  pages        = {435--440},
  publisher    = {Elsevier Ltd},
  series       = {Thrombosis Research},
  title        = {Anti- and procoagulant activities in factor VII-deficient subjects},
  url          = {http://dx.doi.org/10.1016/S0049-3848(00)00410-2},
  volume       = {101},
  year         = {2001},
}