Archives of Plastic Surgery

  • 제36권1호
  • /
  • Pages.93-95
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  • 2009
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  • 2234-6163(pISSN)
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  • 2234-6171(eISSN)
대한성형외과학회 (Korean Society of Plastic and Reconstructive Surgeons)
권호 표지

응고인자 VII 부족 환자에서의 악교정 수술: 증례보고

Orthognathic surgery in a patient with Factor VII deficiency: A Case Report

  • 백롱민 (분당서울대학교병원 성형외과) ;
  • 오명준 (분당서울대학교병원 성형외과) ;
  • 이상우 (분당서울대학교병원 성형외과)
  • Baek, Rong Min (Department of Plastic Surgery Seoul National University Bundang Hospital) ;
  • Oh, Myung June (Department of Plastic Surgery Seoul National University Bundang Hospital) ;
  • Lee, Sang Woo (Department of Plastic Surgery Seoul National University Bundang Hospital)
  • 발행 : 2009.01.15
⟨ 이전 논문 다음 논문 ⟩

초록

Purpose: Congenital factor VII (FVII) deficiency is a rare bleeding disorder and surgery can cause excessive bleeding due to an extrinsic pathway problem. It can be diagnosed by increased PT and decreased FVII level in coagulation test. Symptom varies according to the level of FVII, but it is essential to prevent intraoperative excessive bleeding. Methods: In this report, we described the orthognatic surgery experience in a mandibular prognathism patient with congenital FVII deficiency, in which recombinant activated factor VII (rFVIIa) was used to manage the bleeding. Rsults: We could get a successful result without any complication and there was minimal intraoperative bleeding. Conclusion: The orthognathic surgery could therefore be safely performed in patients with congenital factor VII deficiency using rFVIIa.

키워드

참고문헌

  1. Ragni MV, Lewis JH, Spero JA, Hasiba U: Factor VII deficiency. Am J Hematol 10: 79, 1981 https://doi.org/10.1002/ajh.2830100112
  2. Mariani G, Herrmann FH, Dolce A, Batorova A, Etro D, Peyvandi F, Wulff K, Schved JF, Auerswald G, Ingerslev J, Bernardi F; International factor VII Deficiency study Group: Clinical phenotypes and factor VII genotype in congenital factor VII deficiency. Thromb Haemost 93: 481, 2005
  3. Hunault M, Bauer KA: Recombinant factor VIIa for the treatment of congenital factor VII deficiency. Semin Thromb Hemost 26: 401, 2000 https://doi.org/10.1055/s-2000-8459
  4. Aldouri M: The use of recombinant factor VIIa in controlling surgical bleeding in non-haemophiliac patients. Pathophysiol Haemost Thromb 32: 41, 2002 https://doi.org/10.1159/000057301
  5. Grounds M: Recombinant factor VIIa(rFVIIa) and its use in severe bleeding in surgery and trauma: a review. Blood Rev 17: S11, 2003 https://doi.org/10.1016/S0268-960X(03)90002-6
  6. O'Connell NM, Perry DJ, Hodgson AJ, O'Shaughnessy DF, Laffan MA, Smith OP: Recombinant FVIIa in the management of uncontrolled hemorrhage. Transfusion 43: 1711, 2003 https://doi.org/10.1046/j.0041-1132.2003.00577.x
  7. Tcheng WY, Donkin J, Konzal S, Wong WY: Recombinant factor VIIa prophylaxis in a patient with severe congenital factor VII deficiency. Haemophilia 10: 295, 2004 https://doi.org/10.1111/j.1365-2516.2004.00885.x
  8. Lanigan DT: Vascular complications associated with orthognathic surgery. Oral Maxillofac Surg Clin North Am 9: 231, 1997