The procoagulant effects of factor V Leiden may be balanced againts decreased levels of factor V and do not reflect in vivo thrombin formation in newborns

Thrombosis and Haemostasis

Volumn 95, Issue 3, 2006, Pages 434-440

  Hyytiäinen, Satu ^a   Wartiovaara Kautto, Ulla ^b   Ulander, Veli Matti ^a   Kaaja, Risto ^a   Heikinheimo, Markku ^a,c,d   Petäjä, Jari ^a  

^a UNIVERSITY OF HELSINKI   (Finland)

^b HELSINKI UNIVERSITY CENTRAL HOSPITAL   (Finland)

^c TAMPERE UNIVERSITY HOSPITAL   (Finland)

^d WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Activated protein C; Factor V Leiden; Newborn; Platelet rich plasma; Thrombin

Indexed keywords

ACTIVATED PROTEIN C; ANTITHROMBIN; BLOOD CLOTTING FACTOR 5 LEIDEN; BLOOD CLOTTING FACTOR 7; DALTEPARIN; ENOXAPARIN; PROTEIN C; PROTEIN S; THROMBIN; THROMBOPLASTIN;

ADULT; APGAR SCORE; ARTICLE; BIRTH WEIGHT; BLOOD SAMPLING; CLINICAL ARTICLE; CONTROLLED STUDY; FEMALE; GENE MUTATION; GESTATIONAL AGE; HETEROZYGOSITY; HUMAN; IN VIVO STUDY; MALE; NEWBORN; PRIORITY JOURNAL; SENSITIVITY ANALYSIS; STATISTICAL ANALYSIS; STATISTICAL SIGNIFICANCE; THROMBOCYTE POOR PLASMA; THROMBOCYTE RICH PLASMA; THROMBOPHILIA; UMBILICAL CORD BLOOD;

BLOOD COAGULATION; FACTOR V; FEMALE; HETEROZYGOTE; HUMANS; INFANT, NEWBORN; MALE; PREGNANCY; PREGNANCY COMPLICATIONS, HEMATOLOGIC; PROTEIN C; THROMBIN; THROMBOPHILIA; TIME FACTORS;

EID: 33645582478     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1160/TH05-05-0375     Document Type: Article

References (36)

1. Andrew M, Paes B, Milner R, et al. Development of the human coagulation system in the full term infant. Blood 1987; 70: 165-72.
2. Andrew M, Paes B, Johnston M. Development of the human coagulation system in the neonate and young infant. Am J Pediatr Hematol Oncol 1990; 12: 95-104.
3. Yuen PMP, Yin JA, Lao TTH. Fibrinopeptide A levels in maternal and newborn plasma. Eur J Obstet Gynecol Reprod Biol 1989; 30: 239-44.
4. Schneider DM, von Tempelhoff GF, Herrle B, et al. Maternal and cord blood hemostasis at delivery. J Perinat Med 1997; 25: 55-61.
5. Suarez CR, Walenga J, Mangogna LC, et al. Neonatal and maternal fibrinolysis: activation at time of birth. Am J Hematol 1985; 19: 365-72.
6. Lao TTH, Yin JA, Yuen PMP. Coagulation and anticoagulation systems in newborns - correlation with their mothers at delivery. Gynecol Obstet Invest 1990; 29: 181-4.
7. Bertina RM, Koeleman BP, Koster T, et al. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature 1994; 343: 64-7.
8. Koster T, Rosendaal FR, de Ronde H, et al. Venous thromboembolism due to poor anticoagulant response to activated protein C: Leiden Thrombophilia Study. Lancet 1993; 342: 1503-6.
9. Svensson PJ, Dahlbäck B. Resistance to activated protein C as a basis for venous thrombosis. N Engl J Med 1994; 330: 517-22.
10. Dahlbäck B. The discovery of activated protein C resistance. J Thromb Haemost 2003; 1: 3-9.
11. Nicolaes GAF, Dahlbäck B. Factor V and thrombotic disease. Description of a janus-faced protein. Arterioscler Thromb Vasc Biol 2002; 22: 530-8.
12. Castoldi E, Rosing J. Factor V Leiden: a disorder of factor V anticoagulant function. Curr Opin Hematol 2004; 11: 176-81.
13. Andrew M, David M, Adams M, et al. Venous thromboembolic complications (VTE) in children: First analysis of the Canadian registry of VTE. Blood 1994; 83: 1251-7.
14. Hagstrom JN, Walter J, Bluebond-Langner R, et al. Prevalence of the factor V Leiden mutation in children and neonates with thromboembolic disease. J Pediatr 1998; 133: 777-81.
15. Petäjä J, Hiltunen L, Fellman V. Increased risk of intraventricular hemorrhage in newborn infants with thrombophilia. Pediatr Res 2001; 49: 643-6.
16. Schmidt B, Ofusu FA, Mitchell L, et al. Anticoagulant effects of heparin in neonatal plasma. Pediatr Res 1989; 25: 405-8.
17. Andrew M, Schmidt B, Mitchell L, et al. Thrombin generation in newborn plasma is critically dependent of the concentration of prothrombin. Thromb Haemost 1990; 63: 27-30.
18. Cvirn G, Gallistl S, Leschnik B, et al. Low tissue factor pathway inhibitor (TFPI) together with low anti-thrombin allows sufficient thrombin generation in neonates. J Thromb Haemost 2003; 1: 263-8.
19. Petäjä J, Fernandéz JA, Fellman V, et al. Upregulation of the antithrombotic protein C pathway at birth. Pediatr Hematol Oncol 1998; 15: 489-99.
20. Schwartz HP, Muntean W, Wazke H, et al. Low total protein S antigen but high protein S activity due to decreased C4b-binding protein in neonates. Blood 1988; 71: 562-5.
21. Menashi S, Aurosseau MH, Gozin D, et al. High levels of circulating thrombomodulin in human foetuses and children. Thromb Haemost 1999; 81: 906-9.
22. Cvirn G, Gallistl S, Rehak G, et al. Elevated thrombin-forming capacity of tissue factor-activated cord compared with adult plasma. J Thromb Haemost 2003; 1: 1785-90.
23. Cvirn G, Gallistl S, Koestenberger M, et al. The anticoagulant action of recombinant human activated protein C (rhAPC, Drotrecogin α activated): comparison between cord and adult plasma. Thromb Haemost 2004; 91: 912-8.
24. Hemker HC, Giesen P, Dieri RA, et al. Calibrated automated thrombin generation measurement in clotting plasma. Pathophysiol Haemost Thromb 2003; 33: 4-15.
25. Hemker HC, Giesen PLA, Ramjee M, et al. The Thrombogram: Monitoring thrombin generation in platelet rich plasma. Thromb Haemost 2000; 83: 389-91.
26. Vanschoonbeek K, Feijge MAH, van Kampen JW, et al. Initiating and potentiating role of platelets in tissue factor-induced thrombin generation in the presence of plasma: subject-dependent variation in thrombogram characteristics. J Thromb Haemost 2004; 2: 476-84.
27. Fernandez JA, Hackeng TM, Kojima K, et al. The carbohydrate moiety of factor V modulates inactivation by activated protein C. Blood 1997; 89: 4348-54.
28. Gould WR, Silveira JR, Tracy PB. Unique in vivo modifications of coagulation factor V produce a physically and functionally distinct platelet-derived cofactor. J Biol Chem 2004; 279: 2383-93.
29. Smirnov MD, Esmon CT. Phoshpatidylethanolamine incorporation into vesicles selectively enhances factor Va inactivation by activated protein C. J Biol Chem 1994; 269: 816-9.
30. Nicolaes GAF, Thomassen MCLGD, Tans G, et al. Effect of activated protein C on thrombin generation and on the thrombin potential in plasma of normal and APC-resistant individuals. Blood Coagul Fibrinol 1997; 8: 28-38.
31. Castoldi E, Brugge JM, Nicolaes GAF, et al. Impaired APC cofactor activity of factor V plays a major role in the APC resistance associated with the factor V Leiden (R506Q) and R2 (H1299R) mutations. Blood 2004; 103: 4173-9.
32. Zöller B, Holm J, Svensson P, et al. Elevated levels of prothrombin activation fragment 1+2 in plasma from patients with heterozygous Arg506 to Gln mutation in the factor V gene (APC-resistance) and/or inherited protein S deficiency. Thromb Haemost 1996; 75; 270-4.
33. Simioni P, Scarano L, Gavasso S, et al. Prothrombin fragment 1+2 and thrombin-antithrombin complex levels in patients with inherited APC resistance due to factor V Leiden mutation. Br J Haematol 1996; 92: 435-41.
34. Nowak-Göttl U, Vielhaber H. Elevated levels of soluble thrombomodulin in plasma from children with Arg 506 to Gln mutation in the factor V gene. Ear J Haematol 1997; 58: 51-5.
35. Rosendaal FR. Venous thrombosis: a multicausal disease. Lancet 1999; 353: 1167-73.
36. Petäjä J, Manco-Johnson MJ. Protein C pathway in infants and children. Semin Thromb Hemost 2003; 29: 349-61.