Diagnosis and management of a patient with newly diagnosed hemophilia B

Surbhi Saini; Amy L. Dunn

doi:10.1007/978-3-030-31661-7_3

Diagnosis and management of a patient with newly diagnosed hemophilia B

Research output: Chapter in Book/Report/Conference proceeding › Chapter › peer-review

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.

Original language	English
Title of host publication	Pediatric Bleeding Disorders
Subtitle of host publication	A Clinical Casebook
Publisher	Springer International Publishing
Pages	25-33
Number of pages	9
ISBN (Electronic)	9783030316617
ISBN (Print)	9783030316600
DOIs	https://doi.org/10.1007/978-3-030-31661-7_3
State	Published - Jan 1 2020

Keywords

Arthropathy
Christmas disease
Factor IX
Hemophilia B

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10.1007/978-3-030-31661-7_3

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AB - 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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ER -

Diagnosis and management of a patient with newly diagnosed hemophilia B

Abstract

Keywords

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