250448
Description
Flashcards by indysahota, updated more than 1 year ago
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Created by indysahota
about 11 years ago
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| Question | Answer |
| Hemophilia A is a deficiency in what factor? | VIII |
| Outline the pathophysiology and classifications of Hem A | - X-linked recessive - 1/5000 males - mild Hem A (>5% of normal factor level), moderate (1-5%), severe (<1%) |
| Describe the 5 H's of Hemophilia A (symptoms/signs). | Hemarthroses Hematoma Hematochezia Hematuria Head hemorrhage |
| List the investigations (and results) for hem A | - prolonged PTT, normal PT/INR - decreased factor VIII (<40% of normal) - vWF usually normal or increased |
| Outline the management of hemophilia A | - Desmopressin (DDAVP) in mild hemophilia A - recombinant Factor VIII concentrate for: prophylaxis, minor but not trivial bleeding, major potentially life threatening-bleeding - Antifibrinolytic agents (ex. transexamic acid) |
| Hemophilia B is a deficiency in what factor? | IX |
| Describe the pathogenesis of hem B | - AKA Christmas disease - X-linked recessive (like hem A) - 1/30,000 males |
| Outline the investigations for hemophilia B | Same as hemophilia A except Factor IX instead of VIII |
| Outline the management of hemophilia B | - Recombinant factor IX concentrate - Antifibrinolytic agents |
| What is the mechanism of desmopressin (DDAVP)? | - Promotes the release of vWF (with subsequent increase in factor VIII survival) in patients with vWD, mild hemophilia A and thrombocytopenia - It is not effective in the treatment of hemophilia B (factor IX deficiency), severe hemophilia A, or von Willebrand 2B. |
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