Management of an Infant with Hemophilia A

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Pediatric Bleeding Disorders

Abstract

Hemophilia A is the most common X-linked bleeding disorder, occurring in approximately 1 in 5000 males across all races and ethnicities. A congenital deficiency or absence of coagulation factor VIII leaves these patients prone to excessive and prolonged bleeding, especially joint and muscle hemorrhages as well as postsurgical, oral, and soft tissue bleeding. Recurrent bleeding into joints leads to chronic arthropathy. Replacement of the deficient factor VIII with plasma-derived or recombinant factor concentrates is the cornerstone of management. Dosing and frequency are determined by the desired plasma factor level, clinical response, and product characteristics.

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References

  1. Mannucci PM, Tuddenham EG. The hemophilias–from royal genes to gene therapy.[see comment][erratum appears in N Engl J Med. 2001;345(5):384]. [Review] [64 refs]. N Engl J Med 2001;344(23):1773–9.

    Google Scholar 

  2. Kulkarni R, Soucie JM, Lusher J, Presley R, Shapiro A, Gill J, et al. Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: a report from the Centers for Disease Control and Prevention’s (CDC) Universal Data Collection (UDC) project. Haemophilia. 2009;15(6):1281–90.

    Article  CAS  Google Scholar 

  3. Mansouritorghabeh H, Banihashem A, Modaresi A, Manavifar L. Circumcision in males with bleeding disorders. Mediterr J Hematol Infect Dis. 2013;5(1):e2013004.

    Article  Google Scholar 

  4. Rodriguez V, Titapiwatanakun R, Moir C, Schmidt KA, Pruthi RK. To circumcise or not to circumcise? Circumcision in patients with bleeding disorders. Haemophilia. 2010;16(2):272–6.

    Article  CAS  Google Scholar 

  5. Lakich D, Kazazian HH, Antonarakis SE, Gitschier J. Inversions disrupting the factor VIII gene are a common cause of severe haemophilia a. Nat Genet. 1993;5(3):236–41.

    Article  CAS  Google Scholar 

  6. van Galen KPM, Engelen ET, Mauser-Bunschoten EP, van Es RJJ, Schutgens REG. Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease undergoing minor oral surgery or dental extractions. Cochrane Database Syst Rev. 2015;12:CD011385.

    Google Scholar 

  7. Yilmaz D, Akin M, Ay Y, Balkan C, ÇElik A, ErgÜN O, et al. A single centre experience in circumcision of haemophilia patients: Izmir protocol. Haemophilia. 2010;16(6):888–91.

    Article  CAS  Google Scholar 

  8. Pipe SW, Kessler CM. Evidence-based guidelines support integrated disease management as the optimal model of haemophilia care. Haemophilia. 2016;22:3–5.

    Article  Google Scholar 

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Correspondence to Amy L. Dunn .

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Saini, S., Dunn, A.L. (2020). Management of an Infant with Hemophilia A. In: Dunn, A., Kerlin, B., O'Brien, S., Rose, M., Kumar, R. (eds) Pediatric Bleeding Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-31661-7_1

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  • DOI: https://doi.org/10.1007/978-3-030-31661-7_1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-31660-0

  • Online ISBN: 978-3-030-31661-7

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