Tags

Type your tag names separated by a space and hit enter

Hereditary deficiency of antithrombin III, protein C and protein S: prevalence in patients with a history of venous thrombosis and criteria for rational patient screening.
Blood Coagul Fibrinolysis. 1992 Oct; 3(5):547-53.BC

Abstract

Data in the literature on the prevalence of hereditary deficiency of the natural coagulation inhibitors are conflicting. We conducted a prospective study on 680 consecutive patients with a history of venous thrombosis to determine the prevalence of hereditary deficiency of antithrombin III (AT III), protein C(PC) and protein S(PS) and to establish selection criteria for rational patient screening. The mean age of the patients at investigation was 44.3 +/- 15.4 years, while that at the first thrombotic event was 38.5 +/- 14.8 years. The total prevalence of inhibitor deficiency states was 48/680 (7.1%). 19/680 patients (2.8%) had AT III-deficiency, 17 (2.5%) PC-deficiency, nine (1.3%) PS-deficiency and three (0.4%) a combined deficiency. In 37/48 deficient patients family studies were performed and the hereditary nature was established in 19 cases (2.8% of total patient population, six with AT III-deficiency, eight with PC-deficiency, four with PS-deficiency and one with a combined deficiency). Family studies in these 19 patients revealed 46 additional individual patients with a hereditary deficiency state. A positive family history was found in 15/19 (79%) with a proven hereditary deficiency state, in 153/619 (25%) of non-deficient patients and in 11/29 (38%) of deficient patients without established hereditary nature. The mean age at the first thrombotic event was significantly lower in patients with a hereditary deficiency state (26.8 years) compared with the other two groups (39.0 and 39.7 years, respectively). In all patients with a hereditary deficiency the first thrombotic event occurred before the age of 45 years.(

ABSTRACT

TRUNCATED AT 250 WORDS)

Authors+Show Affiliations

Clinic of Internal Medicine I, University of Vienna, Austria.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

1450321

Citation

Pabinger, I, et al. "Hereditary Deficiency of Antithrombin III, Protein C and Protein S: Prevalence in Patients With a History of Venous Thrombosis and Criteria for Rational Patient Screening." Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis, vol. 3, no. 5, 1992, pp. 547-53.
Pabinger I, Brücker S, Kyrle PA, et al. Hereditary deficiency of antithrombin III, protein C and protein S: prevalence in patients with a history of venous thrombosis and criteria for rational patient screening. Blood Coagul Fibrinolysis. 1992;3(5):547-53.
Pabinger, I., Brücker, S., Kyrle, P. A., Schneider, B., Korninger, H. C., Niessner, H., & Lechner, K. (1992). Hereditary deficiency of antithrombin III, protein C and protein S: prevalence in patients with a history of venous thrombosis and criteria for rational patient screening. Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis, 3(5), 547-53.
Pabinger I, et al. Hereditary Deficiency of Antithrombin III, Protein C and Protein S: Prevalence in Patients With a History of Venous Thrombosis and Criteria for Rational Patient Screening. Blood Coagul Fibrinolysis. 1992;3(5):547-53. PubMed PMID: 1450321.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hereditary deficiency of antithrombin III, protein C and protein S: prevalence in patients with a history of venous thrombosis and criteria for rational patient screening. AU - Pabinger,I, AU - Brücker,S, AU - Kyrle,P A, AU - Schneider,B, AU - Korninger,H C, AU - Niessner,H, AU - Lechner,K, PY - 1992/10/1/pubmed PY - 1992/10/1/medline PY - 1992/10/1/entrez SP - 547 EP - 53 JF - Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis JO - Blood Coagul Fibrinolysis VL - 3 IS - 5 N2 - Data in the literature on the prevalence of hereditary deficiency of the natural coagulation inhibitors are conflicting. We conducted a prospective study on 680 consecutive patients with a history of venous thrombosis to determine the prevalence of hereditary deficiency of antithrombin III (AT III), protein C(PC) and protein S(PS) and to establish selection criteria for rational patient screening. The mean age of the patients at investigation was 44.3 +/- 15.4 years, while that at the first thrombotic event was 38.5 +/- 14.8 years. The total prevalence of inhibitor deficiency states was 48/680 (7.1%). 19/680 patients (2.8%) had AT III-deficiency, 17 (2.5%) PC-deficiency, nine (1.3%) PS-deficiency and three (0.4%) a combined deficiency. In 37/48 deficient patients family studies were performed and the hereditary nature was established in 19 cases (2.8% of total patient population, six with AT III-deficiency, eight with PC-deficiency, four with PS-deficiency and one with a combined deficiency). Family studies in these 19 patients revealed 46 additional individual patients with a hereditary deficiency state. A positive family history was found in 15/19 (79%) with a proven hereditary deficiency state, in 153/619 (25%) of non-deficient patients and in 11/29 (38%) of deficient patients without established hereditary nature. The mean age at the first thrombotic event was significantly lower in patients with a hereditary deficiency state (26.8 years) compared with the other two groups (39.0 and 39.7 years, respectively). In all patients with a hereditary deficiency the first thrombotic event occurred before the age of 45 years.(ABSTRACT TRUNCATED AT 250 WORDS) SN - 0957-5235 UR - https://www.unboundmedicine.com/medline/citation/1450321/Hereditary_deficiency_of_antithrombin_III_protein_C_and_protein_S:_prevalence_in_patients_with_a_history_of_venous_thrombosis_and_criteria_for_rational_patient_screening_ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=1450321.ui DB - PRIME DP - Unbound Medicine ER -