Hemophilia A & B

Frage	Antworten
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.

Zusammenfassung der Ressource

	Erstellt von indysahota vor etwa 11 Jahre