Caicedo MV, Raffan-Sanabria F, Duarte-Romero M

Language: Spanish
References: 20
Page: 177-185
PDF size: 64.43 Kb.

ABSTRACT

Classic hemophilia is a hereditary disease linked to sex, where the mother is typically the carrier and the disease is suffered from the male sons. These disease that takes part of the group of blood dyscrasias is characterized by a deficiency of VIII and IX factors, denominated hemophilia A and B respectively. Hemophilia C, less common, is characterized by a deficiency of XI factor linked to the IV chromosome. There is a rough incidence of one case out of 5,000 men for hemophilia type A, while the incidence for Hemophilia type B the figure is about of one case out of 32,000 men. There is a prevalence of 400,000 patients all over the world, from who around the 85% of the hemophilic patients are from hemophilia type A. The disease is hereditary in the 60% of the cases, due to mutation of genetic type, and without any familiar background in the 40% of the cases. However, nowadays this mentioned percentage is changing as a consequence of scientific improvements that allow sex election. The available literature regarding the anesthetic handling of hemophilic patients is scarce, the available data is isolated and there are no updated texts where anesthesiologists can get certain orientation about the perioperative management of this clinical condition. The Santa Fe de Bogotá University Hospital Foundation is the reference Center for hemophilic patients; what has increased the number of patients with this pathology that have been treated at our institution, with a low incidence of complications during the peri-anesthetic period. In this review, we unify the available and updated information, with our experience, giving concrete guidelines and practices for the handling of hemophilic patients in operating-rooms.

REFERENCES

1. White GC II, Rosendaal F, Aledort LM, et al. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001;85:560.

2. Seligsohn U, Peretz H. Molecular genetics aspects of factor XI deficiency and Glanzmann thrombasthenia. Haemostasis 1994;24:81-5.

3. Darby SC, Keeling DM, Spooner RJ, et al. The incidence of factor VIII and factor IX inhibitors in the hemophilia population of the UK and their effect on subsequent mortality, 1977–99. J Thromb Haemost 2004;2:1047–54.

4. Guidelines for the Management of Hemophilia, World Federation of Hemophilia 2005. www.wfh.org/2/docs/Publications/Diagnosis_and_Treatment/Gudelines_Mng_Hemophilia.pdf

5. Lee JW. Von Willebrand disease, hemophilia A and B, and other factor deficiencies. Int Anesthesiol Clin 2004;42:59-76.

6. Simioni P, Spiezia L, Meneghetti L, et al. Potential role of thromboplastography in the monitoring of acquired factor VIII inhibitor hemophilia A: Report on 78 year old woman with life threatening bleedings. Clin Appl Thromb Hemost 2008.

7. Kasper CK. Hereditary plasma clotting factor disorders and their management. Fifth edition, WFH 2008. www.wfh.org/2/docs/Publications/Diagnosis_and_Treatment/TOH-4-Hereditary-Plasma-Revised2008.pdf

8. Mahlangu JN, Gilham A, et al. Guideline for the treatment of haemophilia in South Africa. S Afr Med J 2008;98:126-40.

9. Ozier Y, Schlumberger S. Pharmacological approaches to reducing blood loss and transfusions in the surgical patient. Can J Anaesth 2006;53:s21-9.

10. Ruiz-Sáez A, Hong A, Arguello A, et al. Pharmacokinetics, thrombogenicity and safety of a double viral inactivated factor IX concentrate compared with a prothrombin complex concentrate. Haemophilia 2005;11:583-8.

11. Aledort LM. Factor VIII inhibitor bypassing activity (FEIBA) – addressing safety issues. Haemophilia 2007:1–4.

12. Young G, Blain R, Nakagawa P. Individualization of bypassing agent treatment for haemophilic patients with inhibitors utilizing thromboelastography. Haemophilia 2006;12:598–604.

13. Kraut EH, Aledort LM, Arkin S, Stine KC, Wong WY. Surgical interventions in a cohort of patients with haemophilia A and inhibitors: an experiential retrospective chart review. Haemophilia 2007;13:508–517.

14. Hind D, Lloyd-Jones M, Makris M, Paisley S. Concentrado de factor VIIa recombinante versus concentrados derivados de plasma para el tratamiento de hemorragias agudas en personas con hemofilia A e inhibidores. La Biblioteca Cochrane Plus 2007:1.

15. Tjønnfjord GE, Holme PA. Factor eight inhibitor bypass activity (FEIBA) in the management of bleeds in hemophilia patients with high-titer inhibitors. Vasc Health Risk Manag 2007;3:527-31.

16. Ewenstein BM, Valentino LA, Journeycake JM, et al. Consensus recommendations for use of central venous access devices in haemophilia. Haemophilia 2004;10:629-648.

17. Chi C, Lee CA, Shiltagh N, Khan A, Pollard D, Kadir RA. Pregnancy in carriers of haemophilia. Haemophilia 2007:1–9.

18. Dhar P, Abramovitz S, DiMichele D, Gibb CB, Gadalla F. Management of pregnancy in a patient with severe haemophilia A. Br J Anaesth 2003;91:432–5.

19. Kadir RA, Lee CA, Sabin CA, et al. Pregnancy in women with von Willebrand’s disease or factor XI deficiency. Br J Obstet Gynaecol 1998;105:314-21.

20. Kang SB, Runmball KM, Ettinger RS, et al. Continuous axillary brachial plexus analgesia in a patient with severe hemophilia. J Clin Anesth 2003;15:38-40.