Book chapter
Congenital Bleeding Disorders
Concise Guide to Hematology, pp.112-130
Wiley‐Blackwell
11/04/2011
DOI: 10.1002/9781444345254.ch11
Abstract
Deficiencies of coagulation factors VIII, IX, and von Willebrand factor constitute the majority of inherited bleeding disorders while deficiencies of fibrinogen, prothrombin and factors V, VII, X, XI, and XIII constitute rare bleeding disorders (RBDs). Inherited deficiencies of inhibitors of fibrinolysis are extremely rare bleeding disorders and are included under the “miscellaneous” category. The clinical spectrum of bleeding symptoms of these disorders ranges from mucocutaneous bleeding to musculoskeletal bleeding. Rarely, these disorders are asymptomatic and present only after hemostatic challenge such as surgical procedures or trauma. Availability of clotting factor concentrates and adjuvant therapies, such as desmopressin (DDAVP) and antifibrinolytics, as well as comprehensive care within specialized hemophilia treatment centers, have transformed patient outcomes and quality of life. This chapter provides an overview of the clinical presentation, diagnostic testing and principles of management for patients with inherited bleeding disorders.
Details
- Title: Subtitle
- Congenital Bleeding Disorders
- Creators
- Anjali A Sharathkumar - Lurie Children's HospitalSteven W Pipe - C. S. Mott Children's Hospital
- Contributors
- Alvin H Schmaier (Editor) - Case Western Reserve UniversityHillard M Lazarus (Editor) - Case Western Reserve University
- Resource Type
- Book chapter
- Publication Details
- Concise Guide to Hematology, pp.112-130
- Publisher
- Wiley‐Blackwell; Oxford, UK
- DOI
- 10.1002/9781444345254.ch11
- Number of pages
- 19
- Language
- English
- Date published
- 11/04/2011
- Academic Unit
- Stead Family Department of Pediatrics; Hematology/Oncology
- Record Identifier
- 9984354006102771
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