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Datos sobre la Hemofilia.


En general, la hemofilia es un trastorno hereditario en el cual la sangre no coagula normalmente.


La hemofilia afecta casi siempre a varones, mientras que las madres son portadoras genéticas de esta afección


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Viviendo con Hemofilia.


Guillermo vive en la ciudad de Córdoba, Argentina, donde trabaja como diseñador gráfico para una agencia de publicidad.


Fuera de su actividad laboral, va al gimnasio tres veces por semana, dedica tiempo...


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Comprendiendo
la vida con
Hemofilia.


Si bien muchas personas con hemofilia tienen un buen control de su afección y llevan adelante la vida que desean, este trastorno puede plantear desafíos para algunos.


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Últimas noticias

Federación Mundial
de Hemofilia



Fundación Novo Nordisk
para la Hemofilia



 

FUENTES
· Haemophilia worldwide: hemophilia.org · HERO study: novonordisk.com · CDC.gov: haemophilia.org; wfh.org

Referencias (NovoSeven®)

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  3. 3.  Liebman HA, Chediak J, Fink KI, et al. Activated recombinant human coagulation factor VII (rFVIIa) therapy for abdominal bleeding in patients with inhibitory antibodies to factor VIII. Am J Hematol 2000;63:109-113.
  4. 4.  Lusher JM, Roberts HR, Davignon G, et al. A randomized, double-blind comparison of two dosage levels of recombinant factor VIIa in the treatment of joint, muscle and mucocutaneous haemorrhages in persons with haemophilia A and B, with and without inhibitors. rFVIIa Study Group. Haemophilia 1998;4:790-798.
  5. 5.  McPherson J, Teague L, Lloyd J, et al. Experience with recombinant factor VIIa in Australia and New Zealand. Haemostasis 1996;26 Suppl 1:109-117.
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  7. 8.  Scharrer I. Recombinant factor VIIa for patients with inhibitors to factor VIII or IX or factor VII deficiency. Haemophilia 1999;5:253-259.
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  25. 26.  Mariani G, Dolce A, Batorova A, et al. Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER. Haemophilia 2011;152:340-346.
  26. 27.  Nicolaisen EM. Antigenicity of activated recombinant factor VII followed through nine years of clinical experience. Blood Coagul Fibrinolysis 1998;9 Suppl 1:S119-123.
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  28. 29.  Novo Nordisk A/S, Denmark. Data on file.
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  32. 33.  Galan AM, Tonda R, Pino M, et al. Increased local procoagulant action: a mechanism contributing to the favorable hemostatic effect of recombinant FVIIa in PLT disorders. Transfusion (Paris) 2003;43:885-892.
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  36. 37.  Poon MC, d'Oiron R. Recombinant activated factor VII (NovoSeven) treatment of platelet-related bleeding disorders. International Registry on Recombinant Factor VIIa and Congenital Platelet Disorders Group. Blood Coagul Fibrinolysis 2000;11 Suppl 1:S55-68.
  37. 38.  d'Oiron R, Menart C, Trzeciak MC, et al. Use of recombinant factor VIIa in 3 patients with inherited type I Glanzmann's thrombasthenia undergoing invasive procedures. Thromb Haemost 2000;83:644-647.
  38. 39.  Poon MC, D'Oiron R, Von Depka M, et al. Prophylactic and therapeutic recombinant factor VIIa administration to patients with Glanzmann's thrombasthenia: results of an international survey. . J Thromb Haemost 2004;2:1096-1103.
  39. 40.  Poon MC, d'Oiron R, Hann I, et al. Use of recombinant factor VIIa (NovoSeven) in patients with Glanzmann thrombasthenia. Semin Hematol 2001;38:21-25.
  40. 41.  Poon MC, Zotz R, Di Minno G, et al. Glanzmann's thrombasthenia treatment: a prospective observational registry on the use of recombinant human activated factor VII and other hemostatic agents. Semin Hematol 2006;43:S33-36.
  41. 42.  NCT01476423. Observational Registry of the Treatment of Glanzmann's Thrombasthenia.
  42. 43.  Key NS, Nelsestuen GL. Views on methods for monitoring recombinant factor VIIa in inhibitor patients. Semin Hematol 2004;41:51-54.
  43. 44.  Shirahata A, Kamiya T, Takamatsu J, et al. Clinical trial to investigate the pharmacokinetics, pharmacodynamics, safety, and efficacy of recombinant factor VIIa in Japanese patients with hemophilia with inhibitors. Int J Hematol 2001;73:517-525.
  44. 45.  Barrowcliffe TW. Monitoring inhibitor patients with the right assays. Semin Hematol 2008;45:S25-30.
  45. 46.  Hedner U. Treatment of patients with factor VIII and factor IX inhibitors with special focus on the use of recombinant factor VIIa. Thromb Haemost 1999;82:531-539.
  46. 47.  Dargaud Y, Sorensen B, Shima M, et al. Global haemostasis and point of care testing. Haemophilia 2012;18 Suppl 4:81-88.
  47. 48.  Kenet G, Stenmo CB, Blemings A, et al. Intra-patient variability of thromboelastographic parameters following in vivo and ex vivo administration of recombinant activated factor VII in haemophilia patients. A multi-centre, randomised trial. Thromb Haemost 2010;103:351-359.
  48. 49.  Young G, Ebbesen LS, Viuff D, et al. Evaluation of thromboelastography for monitoring recombinant activated factor VII ex vivo in haemophilia A and B patients with inhibitors: a multicentre trial. Blood Coagul Fibrinolysis 2008;19:276-282.
  49. 50.  Roberts HR, Monroe DM, 3rd, Hoffman M. Safety profile of recombinant factor VIIa. Semin Hematol 2004;41:101-108.
  50. 51.  Hoffman M, Monroe DM, 3rd. A cell-based model of hemostasis. Thromb Haemost 2001;85:958-965.
  51. 52.  Jurlander B, Thim L, Klausen NK, et al. Recombinant activated factor VII (rFVIIa): characterization, manufacturing, and clinical development. Semin Thromb Hemost 2001;27:373-384.
  52. 53.  Monroe DM, Hoffman M, Oliver JA, et al. Platelet activity of high-dose factor VIIa is independent of tissue factor. Haemophilia 1997;99:542-547.
  53. 54.  Monroe DM, Hoffman M, Oliver JA, et al. A possible mechanism of action of activated factor VII independent of tissue factor. Blood Coagul Fibrinolysis 1998;9 Suppl 1:S15-20.
  54. 55.  Brinkhous KM, Hedner U, Garris JB, et al. Effect of recombinant factor VIIa on the hemostatic defect in dogs with hemophilia A, hemophilia B, and von Willebrand disease. Proc Natl Acad Sci U S A 1989;86:1382-1386.
  55. 56.  Bregengaard C, Diness V, Hedner U. Effect of recombinant factor VIIa on endotoxin-induced disseminated intravascular coagulation in rabbits. Thromb Haemost 1993;69:749 [ABSTRACT].
  56. 57.  Abshire T, Kenet G. Safety update on the use of recombinant factor VIIa and the treatment of congenital and acquired deficiency of factor VIII or IX with inhibitors. Haemophilia 2008;14:898-902.
  57. 58.  Abshire T, Kenet G. Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors. . J Thromb Haemost 2004;2:899-909.
  58. 59.  Girard P, Nony P, Erhardtsen E, et al. Population pharmacokinetics of recombinant factor VIIa in volunteers anticoagulated with acenocoumarol. Thromb Haemost 1998;80:109-113.
  59. 60.  Fridberg MJ, Hedner U, Roberts HR, et al. A study of the pharmacokinetics and safety of recombinant activated factor VII in healthy Caucasian and Japanese subjects. Blood Coagul Fibrinolysis 2005;16:259-266.
  60. 61.  Lindley CM, Sawyer WT, Macik BG, et al. Pharmacokinetics and pharmacodynamics of recombinant factor VIIa. Clin Pharmacol Ther 1994;55:638-648.
  61. 62.  Villar A, Aronis S, Morfini M, et al. Pharmacokinetics of activated recombinant coagulation factor VII (NovoSeven) in children vs. adults with haemophilia A. Haemophilia 2004;10:352-359.
  62. 63.  Berrettini M, Mariani G, Schiavoni M, et al. Pharmacokinetic evaluation of recombinant, activated factor VII in patients with inherited factor VII deficiency. Haematologica 2001;86:640-645.
  63. 64.  Hedner U. Factor VIIa and its potential therapeutic use in bleeding-associated pathologies. Thromb Haemost 2008;100:557-562.
  64. 65.  Negrier C, Goudemand J, Sultan Y, et al. Multicenter retrospective study on the utilization of FEIBA in France in patients with factor VIII and factor IX inhibitors. French FEIBA Study Group. Factor Eight Bypassing Activity. Thromb Haemost 1997;77:1113-1119.
  65. 66.  Johannessen M, Andreasen RB, Nordfang O. Decline of factor VIII and factor IX inhibitors during long-term treatment with NovoSeven. Blood Coagul Fibrinolysis 2000;11:239-242.
  66. 67.  Nicolaisen EM. Long-term follow-up with regard to potential immunogenicity: clinical experience with NovoSeven(recombinant factor VIIa). Haemostasis 1996;26 Suppl 1:98-101.
  67. 68.  Young G, Cooper DL, Gut RZ, et al. Dosing and effectiveness of recombinant activated factor VII (rFVIIA) in congenital haemophilia with inhibitors by bleed type and location: the experience of the Haemophilia and Thrombosis Research Society (HTRS) Registry (2004-2008). Haemophilia 2012;18:990-996.
  68. 69.  Lusher JM. Acute hemarthroses: the benefits of early versus late treatment with recombinant activated factor VII. Blood Coagul Fibrinolysis 2000;11 Suppl 1:S45-49.
  69. 70.  Stephens JM, Joshi AV, Sumner M, et al. Health economic review of recombinant activated factor VII for treatment of bleeding episodes in hemophilia patients with inhibitors. Expert Opin Pharmacother 2007;8:1127-1136.
  70. 71.  Mariani G, Konkle BA, Ingerslev J. Congenital factor VII deficiency: therapy with recombinant activated factor VII -- a critical appraisal. Haemophilia 2006;12:19-27.