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Diagnosis and Management of a Patient with Newly Diagnosed Hemophilia B

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Pediatric Bleeding Disorders

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Abstract

Hemophilia B (HB) is an X-linked bleeding disorder that results from the congenital deficiency of coagulation factor IX (FIX) and occurs in approximately 1 in 20,000–25,000 male births. The bleeding diathesis in HB is indistinguishable from that of hemophilia A, and measurement of plasma FIX levels is crucial for diagnosis. Replacement of the missing FIX by FIX concentrates is the cornerstone of current therapy. Several novel agents and gene therapy for the treatment of HB are currently in clinical trials.

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Author information

Authors and Affiliations

  1. Department of Pediatrics, St. Louis Children’s Hospital, St. Louis, MO, USA

    Surbhi Saini

  2. Washington University in St. Louis School of Medicine, St. Louis, MO, USA

    Surbhi Saini

  3. Nationwide Children’s Hospital, Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Columbus, OH, USA

    Amy L. Dunn

  4. Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA

    Amy L. Dunn

Authors
  1. Surbhi Saini
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  2. Amy L. Dunn
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Corresponding author

Correspondence to Amy L. Dunn .

Editor information

Editors and Affiliations

  1. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Amy L. Dunn

  2. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Bryce A. Kerlin

  3. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Sarah H. O'Brien

  4. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Melissa J. Rose

  5. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Riten Kumar

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Saini, S., Dunn, A.L. (2020). Diagnosis and Management of a Patient with Newly Diagnosed Hemophilia B. 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_3

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  • DOI: https://doi.org/10.1007/978-3-030-31661-7_3

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-31660-0

  • Online ISBN: 978-3-030-31661-7

  • eBook Packages: MedicineMedicine (R0)

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