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Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency

Year 2011, Volume: 3 Issue: 2, 1 - 4, 07.04.2011

Baris Malbora Zekai Avcı Betul Orhan Serdar Arda Namik Ozbek

Abstract

Factor XI (FXI) deficiency is a rare inherited bleeding disorder caused by a mutation in the FXI gene, which is located on the distal arm of chromosome 4 (4q35). Factor XI (FXI) deficiency is defined when the activity of FXI in plasma is less than 15 U/dL. We report our experience about perioperative use of recombinant activated factor VII (rFVIIa) in a 17 year-old with congenital FXI deficiency and appendicitis. After appendectomy, the first intravenous dose of rFVIIa was administered. This same dosage was repeated and he was discharged on postoperative 72nd hour without complications.

Keywords

appendectomy factor XI deficiency recombinant factor VIIa

References

  • Salomon O, Seligsohn U. New observations on factor XI deficiency. Haemophilia 2004;10:184-7.
  • Seligsohn U. Factor XI deficiency in humans. J Thromb Haemost 2009;7 (Suppl. 1): 84–7.
  • Bolton-Maggs PH, Patterson DA, Wensley RT, Tuddenham EG. Definition of the bleeding tendency in factor XI deficient kindreds-a clinical and laboratory study. Thromb Haemos 1995;73:194-202.
  • Niamh M. O’Connell. Factor XI deficiency-from molecular genetics to clinical management. Blood Coagul Fibrinolysis 2003; 14 (suppl 1): 59-64.
  • Ramadan KM, McNulty O, Anderson JA, Jones FG, Winter PC. A novel factor XI deficiency in a symptomatic patient. Blood Coagul Fibrinolysis 2006; 17: 499-502.
  • Sidi A, Seligsoh U, Jonas P, Many M. Factor XI deficiency, detection and management during urological surgery Urol 1978; 119: 528-30.
  • Bolton-Maggs PH. Factor XI deficiency and its management. Haemophilia 2000; 6: 100-9.
  • Avci Z, Malbora B, Gokdemir M, Ozkan S, Ozbek N. Successful Use of Recombinant Factor VIIa (NovoSeven) During Cardiac Surgery in a Pediatric Patient with Congenital Factor XI Deficiency. Pediatr Cardiol 2008; 29: 220–2. ®
  • Berliner S, Horowitz I, Martinowitz U, Brenner B, Seligsohn U. Dental surgery in patients with severe factor XI deficiency without plasma replacement therapy. Blood Coagul Fibrinol 1992; 3: 465-8.
  • David AL, Paterson-Brown S, Letsky A. Factor XI deficiency presenting in pregnancy: diagnosis and management. Br J Obstet Gynaecol 2002; 109: 840–3.
  • Schulman S, Nemeth G. An illustrative case and a review on the dosing of recombinant factor VIIa in congenital factor XI deficiency. Haemophilia 2006; 12: 223-7.

Year 2011, Volume: 3 Issue: 2, 1 - 4, 07.04.2011

Baris Malbora Zekai Avcı Betul Orhan Serdar Arda Namik Ozbek

Abstract

References

  • Salomon O, Seligsohn U. New observations on factor XI deficiency. Haemophilia 2004;10:184-7.
  • Seligsohn U. Factor XI deficiency in humans. J Thromb Haemost 2009;7 (Suppl. 1): 84–7.
  • Bolton-Maggs PH, Patterson DA, Wensley RT, Tuddenham EG. Definition of the bleeding tendency in factor XI deficient kindreds-a clinical and laboratory study. Thromb Haemos 1995;73:194-202.
  • Niamh M. O’Connell. Factor XI deficiency-from molecular genetics to clinical management. Blood Coagul Fibrinolysis 2003; 14 (suppl 1): 59-64.
  • Ramadan KM, McNulty O, Anderson JA, Jones FG, Winter PC. A novel factor XI deficiency in a symptomatic patient. Blood Coagul Fibrinolysis 2006; 17: 499-502.
  • Sidi A, Seligsoh U, Jonas P, Many M. Factor XI deficiency, detection and management during urological surgery Urol 1978; 119: 528-30.
  • Bolton-Maggs PH. Factor XI deficiency and its management. Haemophilia 2000; 6: 100-9.
  • Avci Z, Malbora B, Gokdemir M, Ozkan S, Ozbek N. Successful Use of Recombinant Factor VIIa (NovoSeven) During Cardiac Surgery in a Pediatric Patient with Congenital Factor XI Deficiency. Pediatr Cardiol 2008; 29: 220–2. ®
  • Berliner S, Horowitz I, Martinowitz U, Brenner B, Seligsohn U. Dental surgery in patients with severe factor XI deficiency without plasma replacement therapy. Blood Coagul Fibrinol 1992; 3: 465-8.
  • David AL, Paterson-Brown S, Letsky A. Factor XI deficiency presenting in pregnancy: diagnosis and management. Br J Obstet Gynaecol 2002; 109: 840–3.
  • Schulman S, Nemeth G. An illustrative case and a review on the dosing of recombinant factor VIIa in congenital factor XI deficiency. Haemophilia 2006; 12: 223-7.
There are 11 citations in total.

Details

Primary Language English
Journal Section Case Reports
Authors

Baris Malbora

Zekai Avcı This is me

Betul Orhan This is me

Serdar Arda This is me

Namik Ozbek This is me

Publication Date April 7, 2011
Published in Issue Year 2011 Volume: 3 Issue: 2

Cite

APA Malbora, B., Avcı, Z., Orhan, B., Arda, S., et al. (2011). Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency. Journal of Pediatric Sciences, 3(2), 1-4. https://doi.org/10.17334/jps.73756
AMA Malbora B, Avcı Z, Orhan B, Arda S, Ozbek N. Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency. Journal of Pediatric Sciences. April 2011;3(2):1-4. doi:10.17334/jps.73756
Chicago Malbora, Baris, Zekai Avcı, Betul Orhan, Serdar Arda, and Namik Ozbek. “Succesful Use of Recombinant Factor VIIa (Novoseven®) During Apendectomy in an Adolescent Patient With Congenital Factor XI Deficiency”. Journal of Pediatric Sciences 3, no. 2 (April 2011): 1-4. https://doi.org/10.17334/jps.73756.
EndNote Malbora B, Avcı Z, Orhan B, Arda S, Ozbek N (April 1, 2011) Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency. Journal of Pediatric Sciences 3 2 1–4.
IEEE B. Malbora, Z. Avcı, B. Orhan, S. Arda, and N. Ozbek, “Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency”, Journal of Pediatric Sciences, vol. 3, no. 2, pp. 1–4, 2011, doi: 10.17334/jps.73756.
ISNAD Malbora, Baris et al. “Succesful Use of Recombinant Factor VIIa (Novoseven®) During Apendectomy in an Adolescent Patient With Congenital Factor XI Deficiency”. Journal of Pediatric Sciences 3/2 (April 2011), 1-4. https://doi.org/10.17334/jps.73756.
JAMA Malbora B, Avcı Z, Orhan B, Arda S, Ozbek N. Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency. Journal of Pediatric Sciences. 2011;3:1–4.
MLA Malbora, Baris et al. “Succesful Use of Recombinant Factor VIIa (Novoseven®) During Apendectomy in an Adolescent Patient With Congenital Factor XI Deficiency”. Journal of Pediatric Sciences, vol. 3, no. 2, 2011, pp. 1-4, doi:10.17334/jps.73756.
Vancouver Malbora B, Avcı Z, Orhan B, Arda S, Ozbek N. Succesful use of recombinant factor VIIa (Novoseven®) during apendectomy in an adolescent patient with congenital factor XI deficiency. Journal of Pediatric Sciences. 2011;3(2):1-4.