Mortality in hereditary antithrombin-III deficiency—1830 to 1989

Bruin, Kora de; Jp, Vandenbroucke; Briët, E.; Fr, Rosendaal; Heijboer, H.; Büller, Harry R.; Brandjes, D.P.M.; Hommes, DW

doi:10.1016/0140-6736(91)90867-o

Cited by 75 publications

(27 citation statements)

References 7 publications

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“…On the other hand, an excess of fatal events in relatives with a strong thrombophilic defect might have underestimated the risk of venous thrombosis. However, these defects were not associated with a reduced life expectancy in previous studies, 34,35 whereas our study did not show a difference in death rate between relatives of probands with a strong thrombophilic defect and relatives of probands with another defect.…”

Section: Discussioncontrasting

confidence: 54%

Selective testing for thrombophilia in patients with first venous thrombosis: results from a retrospective family cohort study on absolute thrombotic risk for currently known thrombophilic defects in 2479 relatives

Lijfering

Brouwer

Veeger

et al. 2009

Blood

203

154

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Thrombophilia screening is controversial. In a retrospective family cohort, where probands had thrombosis and a thrombophilic defect, 2479 relatives were tested for thrombophilia. In antithrombin-, protein C-, and protein S-deficient relatives, annual incidences of venous thrombosis were 1.77% (95% CI, 1.14-2.60), 1.52% (95% CI, 1.06-2.11), and 1.90% (95% CI, 1.32-2.64), respectively, at a median age of 29 years and a positive family history of more than 20% symptomatic

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Section: Discussioncontrasting

confidence: 54%

Selective testing for thrombophilia in patients with first venous thrombosis: results from a retrospective family cohort study on absolute thrombotic risk for currently known thrombophilic defects in 2479 relatives

Lijfering

Brouwer

Veeger

et al. 2009

Blood

203

154

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“…Third, we cannot provide detailed information on mortality in our study population. However, an excess of fatal events in relatives with antithrombin, or protein C or protein S deficiency is not likely as these deficiencies were not associated with a reduced life expectancy in previous studies, 34,35 whereas the overall mortality due to recurrent venous thrombosis is low. 36,37 Finally, subjects with clinical suspicion of venous thrombosis, who were treated with heparin and vitamin K antagonists for more than 3 months at a time when objective testing was not available yet, might have overestimated our recurrence rates.…”

Section: Discussionmentioning

confidence: 95%

A lower risk of recurrent venous thrombosis in women compared with men is explained by sex-specific risk factors at time of first venous thrombosis in thrombophilic families

Lijfering

Veeger

Middeldorp

et al. 2009

Blood

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Why men appear to have an increased risk of recurrent venous thrombosis compared with women is unknown. In a cohort study of families with thrombophilia, lifetime risk of recurrent venous thrombosis was assessed in men and women (n ‫؍‬ 816). Adjusted relative risk of recurrence was 1.6 (95% CI, 1.3-2.0) in men compared with women. Women were younger at time of their first event (mean, 34 years vs 44 years; P < .001) and at time of recurrence (40 years vs 48 years, P < .001). After excluding provoked first and recurrent venous thrombosis, adjusted relative risk was 1.2 (95% CI, 0.8-1.7), although mean age at recurrence was comparable in men and women (50 years vs 49 years, P ‫؍‬ .595). In women with a hormonal first event, median interval between first event and recurrence was 10.4 years versus 2.7 years in men (P < .001). This difference was not observed when only unprovoked events were considered (P ‫؍‬ .938). The difference in lifetime risk of recurrent venous thrombosis between men and women in thrombophilic families can be explained by a younger age of women at time of first venous thrombosis due to hormonal risk factors, and a longer interval between a provoked first episode of venous thrombosis and recurrence in women. (Blood.

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“…Moreover, hereditary deficiencies were not associated with a reduced life expectancy in previous studies. 32,33 Despite these limitations, we believe that this is the first study to document the age-dependent elevated risk for ATE conveyed by hereditary protein S or protein C deficiency but not antithrombin deficiency in these thrombophilic families.…”

Section: Discussionmentioning

confidence: 99%

Hereditary Deficiency of Protein C or Protein S Confers Increased Risk of Arterial Thromboembolic Events at a Young Age

et al. 2008

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Background-Whether hereditary protein S, protein C, or antithrombin deficiency is associated with arterial thromboembolism (ATE) and whether history of venous thromboembolism in these subjects predisposes them to subsequent ATE have yet to be determined. Methods and Results-On the basis of pedigree analysis, we enrolled a total of 552 subjects (52% women; mean age, 46Ϯ17 years), belonging to 84 different kindreds, in this retrospective family cohort study. Detailed information on previous episodes of venous thromboembolism, ATE, anticoagulant use, and atherosclerosis risk factors was collected. Primary study outcome was objectively verified symptomatic ATE. Of 552 subjects, 308 had protein S (35%), protein C (39%), or antithrombin (26%) deficiency. Overall, annual incidences of ATE were 0.34% (95% confidence interval [CI], 0.23 to 0.49) in deficient versus 0.17% (95% CI, 0.09 to 0.28) in nondeficient subjects; the hazard ratio was 2.3 (95% CI, 1.2 to 4.5). Because the risk hazards varied over lifetime, we performed a time-dependent analysis. After adjusting for atherosclerosis risk factors and clustering within families, we found that deficient subjects had a 4.7-fold (95% CI, 1.5 to 14.2; Pϭ0.007) higher risk for ATE before 55 years of age versus 1.1 (95% CI, 0.5 to 2.6) thereafter compared with nondeficient family members. For separate deficiencies, the risks were 4.6-(95% CI, 1.1 to 18.3), 6.9-(95% CI, 2.1 to 22.2), and 1.1-(95% CI, 0.1 to 10.9) fold higher in protein S-, protein C-, and antithrombin-deficient subjects, respectively, before 55 years of age. History of venous thromboembolism was not related to subsequent ATE (hazard ratio, 1.1; 95% CI, 0.5 to 2.2). Conclusions-Compared with nondeficient family members, subjects with protein S or protein C deficiency but not antithrombin deficiency have a higher risk for ATE before 55 years of age that is independent of prior venous thromboembolism. (Circulation. 2008;118:1659-1667.)

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Mortality in hereditary antithrombin-III deficiency—1830 to 1989

Cited by 75 publications

References 7 publications

Selective testing for thrombophilia in patients with first venous thrombosis: results from a retrospective family cohort study on absolute thrombotic risk for currently known thrombophilic defects in 2479 relatives

Selective testing for thrombophilia in patients with first venous thrombosis: results from a retrospective family cohort study on absolute thrombotic risk for currently known thrombophilic defects in 2479 relatives

A lower risk of recurrent venous thrombosis in women compared with men is explained by sex-specific risk factors at time of first venous thrombosis in thrombophilic families

Hereditary Deficiency of Protein C or Protein S Confers Increased Risk of Arterial Thromboembolic Events at a Young Age

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