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- Bree Zimmerman, MD*
- Leonard A. Valentino, MD*
- *Rush University Medical Center, Chicago, IL.
Author Disclosure
Dr Zimmerman has disclosed no financial relationships relevant to this commentary. Dr. Valentino has disclosed he is a consultant and on the advisory board of Baxter Healthcare Corp., Bayer HealthCare Pharmaceuticals, Biogen Idec, CSL Behring, GTC Biotherapeutics, Inspiration Bioscience, NovoNordisk, and Pfizer, for which Rush University receives grant support and honoraria payments. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.
- a:
- activated
- F:
- factor
- PT:
- prothrombin time
- PTT:
- partial thromboplastin time
- TF:
- tissue factor
- VWF:
- von Willebrand factor
Educational Gap
General pediatricians rarely encounter bleeding in a neonate or a child, so it is important to know the genetics of hemophilia and to be aware of the clinical manifestations of bleeding disorders in order to appropriately identify those children at risk, ensure early diagnosis and treatment, and prevent complications.
Objectives
After completing this article, readers should be able to:
Describe the coagulation system and the basic physiology of blood clotting.
Know the genetics of hemophilia and be able to estimate the relative risk of hemophilia in carriers of the mutation.
Discuss the clinical manifestations and the potential complications of hemophilia.
List the initial laboratory studies needed to evaluate children who have bleeding.
Understand the role of comprehensive care in the treatment of patients with hemophilia.
Hemophilia A (deficiency in factor [F] VIII) and hemophilia B (deficiency in FIX) are the most common serious congenital coagulation factor deficiencies. Hemophilia A is more common, occurring in 1:5000 male births, whereas hemophilia B occurs in 1:30,000 male births. Hemophilia is found in all ethnic groups; there is no geographic or racial predilection. Although easy bruising and excessive mucosal bleeding may be the first signs of hemophilia, severe soft tissue bleeding and hemarthroses are the classic bleeding symptoms. Other bleeding disorders should be considered when evaluating a child with unexpected bleeding. Factor deficiencies (FV, FVII, FX, FXI, FXIII, and fibrinogen) may present with bleeding or abnormal …
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