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June 1, 1996Arteriosclerosis Thrombosis and Vascular Biology

Thrombotic Risk in Hereditary Antithrombin III, Protein C, or Protein S Deficiency

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Population

230 patients from 71 families with a documented hereditary deficiency of a natural coagulation inhibitor

Design

Cohort

Authors

IPIngrid PabingerVascular MedicineBSBarbara SchneiderAltonaer Kinderkrankenhaus

Discussion

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Implication

High thrombosis risk in hereditary deficiencies may inform anticoagulation decisions after first event; leaves open randomized confirmation of benefit.

Structured PICO

P
Population
230 patients from 71 families with a documented hereditary deficiency of a natural coagulation inhibitor (69 patients from 25 families with AT-III deficiency, 86 patients from 27 families with PC deficiency, and 75 patients from 19 families with PS deficiency)
O
Outcome
Probability of developing thrombosis by 50 to 60 years of agehard clinical

Patients with hereditary AT-III, PC, or PS deficiency have an 80% to 90% lifetime risk of thrombosis and a 63% recurrence rate, supporting the use of long-term oral anticoagulation after a first event.

Cite This Study

Pabinger et al. (1996) studied this question.

synapsesocial.com/papers/6a7e452837c44f9ef44f9b26https://doi.org/10.1161/01.atv.16.6.742

Topics

Pulmonary embolism
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Thrombosis and Pregnancy in Congenital Deficiencies in AT III, Protein C or Protein S: Study of 78 Women1990 · 285 citations
  2. 2Thromboembolic Disease – Critical Evaluation of Laboratory Investigation1992 · 113 citations
  3. 3The Risk of Thromboembolism in Asymptomatic Patients with Protein C and Protein S Deficiency: A Prospective Cohort Study1994 · 112 citations
  4. 4An abnormal plasma distribution of protein S occurs in functional protein S deficiency1986 · 285 citations
  5. 5Hereditary deficiency of antithrombin III, protein C and protein S1992 · 114 citations