MosaicED Brain Teaser!
Listen to this jam, show 'em what you got
First things first, I'm the realest (Realest)
Drop this and let the whole world feel it (Let 'em feel it)
And I'm still in the Murda Bizness.
You know what else is first and the realest? Primary haemostasis
Which of the following do NOT contribute to problems in primary haemostasis?
- A-Bernard–Soulier syndrome
- B-Ehlers–Danlos syndrome
- C-Glanzmann thrombasthenia
- D-Inherited factor IX deficiency
- E-Storage pool deficiency
- G-Vascular endothelium abnormalities
- E-Von Willebrand deficiency
C-Inherited factor IX deficiency. Primary haemostasis = formation of a platelet plug, secondary hemostasis = coagulation and fibrinolysis. Primary hemostasis starts immediately after damage of the vessel wall with vasoconstriction as a result of local contraction of vascular smooth muscle cells. Next, von Willebrand factor binds to the exposed subendothelial collagens. Platelets are tethered to the site of endothelial cell injury through the binding of von Willebrand factor to the glycoprotein Ib receptor of the platelet. Platelets roll over von Willebrand factor in the direction of flow and get slightly activated. Platelets finally attach to the subendothelium by participation of other receptors, such as GPIIbIIIa, and the collagen receptors GPVI and α2β1. Primary haemostasis is caused by vascular abnormalities, von Willebrand disease, thrombocytopenia, and platelet dysfunction. Diseases that cause vascular anomalies, like Ehlers-Danlos syndrome and hereditary hemorrhagic telangiectasia cause bleeding abnormalities. Von Willebrand disease is caused by a deficiency in von Willebrand factor, a very large multimeric glycoprotein, which promotes the adhesion of platelets to the injured vessel and to each other, and binds and stabilizes factor VIII. It is the most common inherited bleeding disorder (about 1% of the population). It is autosomally dominant. Patients have mild to moderate bleeding evidenced by nosebleeds, heavy menstrual flow, gingival bleeding, easy bruising, and bleeding associated with surgery or trauma. Bernard–Soulier syndrome, Glanzmann thrombasthenia, and Storage pool deficiency are very rare platelet function disorders. Inherited factor IX deficiency, also known as haemophilia B or Christmas disease, is a factor IX deficiency disorder. It is x linked recessive and far rarer than haemophilia A which is an x linked recessive factor VIII deficiency. They are both diseases that affect secondary haemostasis.