Anti- and procoagulant activities in factor VII-deficient subjects
(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)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/1120921
- author
- Astermark, Jan LU ; Tengborn, L ; Hedner, Ulla LU and Berntorp, Erik LU
- organization
- publishing date
- 2001
- 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
- external identifiers
-
- pmid:11323000
- scopus:0035868253
- ISSN
- 1879-2472
- DOI
- 10.1016/S0049-3848(00)00410-2
- language
- English
- LU publication?
- yes
- additional info
- The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Clinical Coagulation Research Unit (013242510), Emergency medicine/Medicine/Surgery (013240200)
- id
- b8cf1e24-0548-49e8-baed-09300e62db3e (old id 1120921)
- date added to LUP
- 2016-04-01 12:35:47
- date last changed
- 2022-04-21 17:38:42
@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 < 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}},
keywords = {{TFPI; Protein C; Hemostasis; Factor VII deficiency; Thrombosis; Anticoagulant activity}},
language = {{eng}},
number = {{6}},
pages = {{435--440}},
publisher = {{Elsevier}},
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}},
doi = {{10.1016/S0049-3848(00)00410-2}},
volume = {{101}},
year = {{2001}},
}