A selection of talks on Clinical Practice

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0:00
Hello, I'm Snejana Krassova. I'm a medical doctor and a former Vice President and Head of Medical Affairs at Bayer Hematology. I'm presenting today on Factor VII Deficiency.
0:14
We will delve into the background, epidemiology, review the clinical picture, treatment options, and a little bit on patient education.
0:25
Factor VII is a vitamin K-dependent clotting factor and it's very crucial in triggering blood coagulation. There are two factor VII Deficiencies: inherited and acquired. Today we're focusing on inherited factor VII deficiency. Inherited deficiency, this is an autosomal recessive disorder for factor VII gene. Factor VII gene is located on chromosome 13. 1% of activated factor VII circulates in its active form and its half-life is 3.5 hours. It's the most common amongst the rare coagulation disorders.
1:03
When we look at the coagulation pathways, this is an issue of the extrinsic system. The extrinsic pathway is where you need activated factor VIIa to create a thrombin burst in the coagulation cascade. It needs to have a co-factor, tissue factor, that plays a role when we do essays, and substrate factor VIIa-tissue factor complex is absolutely necessary further downstream, to activate factor X and IX. Obviously, deficiencies of factor VII will affect factor VIIa and thrombin burst down the road. Therefore, it actually affects the whole coagulation cascade and that's why factor VII deficiency does present a clinical challenge and needs to be managed in certain patients, which we'll go into right now on epidemiology.

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