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Hemophilia B. World Federation of Hemophilia Global Survey 2008 Demographics Number of countries in this survey: 108 Percentage of world population covered.

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Presentation on theme: "Hemophilia B. World Federation of Hemophilia Global Survey 2008 Demographics Number of countries in this survey: 108 Percentage of world population covered."— Presentation transcript:

1 Hemophilia B

2 World Federation of Hemophilia Global Survey 2008 Demographics Number of countries in this survey: 108 Percentage of world population covered by 2008 survey report: 91% Number of people identified with hemophilia A and B: 148,971 Number of people identified with VWD: 52,330 Number of people identified with other bleeding disorders: 21,510 Total number of people with bleeding disorders identified: 219,699

3 Male or Female?

4 Prevalence in the Philippines 2008 PopulationNumber of People with Hemophilia Number of people with von Willebrand Disease Number of people with Other Bleeding disorder 97,976, n/a Hemophilia A TotalHemophilia B TotalHemophilia type unknown Total vWD Total

5 Pathogenesis

6 X-linked recessive hemorrhagic disease due to mutations in F9 gene Harrison’s Principles of Internal Medicine, 17 th ed.

7 Pathogenesis

8 Clinical Features

9

10 Early in life Bleeding after circumcision or rarely as intracranial hemorrhages Severe and moderate forms Hemarthroses Bleeding episodes into soft tissues & muscles after minor trauma or even spontaneously Harrison’s Principles of Internal Medicine, 17 th ed.

11 Clinical Features Severe forms Recurrent hemarthroses – most common bleeding manifestation – can affect every joint but mainly affect knees, elbows, ankles, shoulders, & hips Acute hemarthroses - painful, local swelling and erythema Chronic hemarthroses - debilitating, with synovial thickening & synovitis Harrison’s Principles of Internal Medicine, 17 th ed.

12 Clinical Features Mild forms Infrequent bleeding usually secondary to trauma Other manifestations Bleeding into oropharyngeal spaces, central nervous system, or retroperitoneum - requires immediate therapy Hematuria - self-limited Harrison’s Principles of Internal Medicine, 17th ed.


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