Term
Patients of what disease are often employed at circuses as "India rubber men"? |
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Definition
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Term
A connective tissue disorder that causes bleeding due to increased fragility of subcutaneous vessels. The patients joints are hyperextensible. |
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Definition
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Term
Is Ehler's-Danlos Syndrome acquired or inherited? |
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Definition
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Term
An inherited disorder characterized by serious bleeding secondary to abnormal elastic fibers in the skin and the media of the arteries. |
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Definition
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Term
A connective tissue disorder with many other physical manifestations. Causes mild bleeding tendencies. Abraham Lincoln may have had this disorder. |
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Definition
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Term
"devil's pinches"; an acquired disorder of unknown cause; usually causing bruising in females |
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Definition
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Term
3 inherited bleeding disorders due to problems of the subendothelium |
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Definition
Ehler's-Danlos Syndrome, Pseudoxanthoma elasticum, Marfan's syndrome |
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Term
Bruises on hands and forearms, usually seen in older patients; caused by the loss of dermal and vascular support. |
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Definition
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Term
Vitamin C deficiency resulting in defective collagen and otehr supportive structures. Patients may have pale skin, loss of teeth, and sunken eyes. |
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Definition
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Term
Three acquired bleeding disorders due to problems of the subendothelium |
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Definition
Purpura simplex, senile purpura, scurvy |
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Term
Osler-Weber-Rendu syndrome is another name for what inherited disorder? |
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Definition
Hereditary Hemorrhagic Telangiectasia |
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Term
An autosomal dominant disorder characterized by cutaneous lesions called A-V malformations, which hold blood and appear as red-colored spots. |
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Definition
Hereditary Hemorrhagic Telangiectasia |
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Term
Why is Hereditary Hemorrhagic Telangiectasia more common than most other vascular diseases? |
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Definition
Because it's autosomal dominant |
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Term
allergic purpura, hepatitis B- associated vaculitis, various infections and reactions to drugs; all these inflammatory disorders are acquired disorders of what part of the vascular system? |
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Definition
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Term
The deficiency of the Glycoprotein IIb/IIIa complex on the platelet surface. Abnormal bleeding time and platelet aggregation tests. Autosomal recessive disease |
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Definition
Glansman's Thrombasthenia |
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Term
The deficiency of the Glycoprotein Ib receptor. Autosomal recessive. |
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Definition
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Term
Bernard Soulier patients are clinically identical to patients with what other disease? |
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Definition
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Term
Three diseases that produce substances which coat the platelet receptors? |
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Definition
uremia, multiple myeloma, anti-platelet antibodies |
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Term
The deficiency of the von Willebrand's Factor molecule which binds the platelet to the collagen |
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Definition
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Term
Which is more common, Bernard Soulier Disease or Von Willebrand's Disease? |
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Definition
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Term
DDAVP is used to treat what disease? |
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Definition
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Term
How is von Willebrand's disease inherited? |
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Definition
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Term
deficiency of Factor VIII due to a defective gene on the X chromosome |
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Definition
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Term
Which disease is characterized by bleeding into the joints? |
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Definition
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Term
Which disease is characterized by bleeding from the gums, nose bleeds, and heavy menstrual bleeding in females? |
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Definition
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Term
epistaxis is bleeding from what body part? |
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Definition
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Term
A bleeding disease that has a normal bleeding time, normal aggregation studies, prolonged APTT and decreased factor VIII level |
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Definition
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Term
People with hemophilia B have a decrease in what factor? |
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Definition
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Term
which version of hemophilia is treated by using plasma replacement or commercial Factor IX replacement? |
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Definition
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Term
name five factor deficiencies that are autosomal recessive and extremely rare. |
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Definition
Factor II, Factor V, Factor VII, Factor X, Factor XIII |
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Term
Hemophilia C is a deficiency of what factor? |
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Definition
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Term
Autosomal dominant factor deficiency with over 40 abnormal variants with a spectrum of clinical expression. |
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Definition
Factor I (fibrinogen) deficiency |
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Term
How is Hemophilia C inherited? |
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Definition
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Term
A phenomenon most commonly seen in the elderly and in post partum females; similar to Hemophelia A but much more difficult to treat. |
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Definition
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Term
An antibody, usually IgG, to the phospholipid platelet membrane. Can prolong both PT and APTT, but does not cause bleeding. |
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Definition
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Term
Vitamin K deficiency causes functional deficiency of what four factors? |
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Definition
Factors II, VII, IX and X |
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Term
If a woman poisons her husband with rat poison, he will die of what vitamin deficiency..? |
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Definition
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Term
In what organ are factors I, II, V, VII, IX and X produced? |
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Definition
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Term
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Definition
disseminated intravascular coagulation |
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Term
In primary fibrinolysis, are d-dimers produced? |
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Definition
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Term
A very common chronic condition of arterial damage and the formation of atherosclerotic plaque. The acute phase of the blockage is driven by a platelet clot at the injury site which blocks the blood supply. |
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Definition
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Term
an acquired disease of endothelial cells resulting in the loss of prostacyclin function and the release of vWF. |
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Definition
Thrombotic Thrombocytopenia Purpura (TTP) |
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Term
oral anticoagulants which inhibit Vitamin K |
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Definition
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Term
an anti-platelet drug that blocks cyclo-oxygenase |
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Definition
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Term
an anti-platelet drug that inhibits platelet adhesion |
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Definition
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Term
what test is used to monitor coumadin or warfarin therapy, and what is the desired result? |
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Definition
Prothrombin time/ the goal is to get a prolongation of PT of 2x normal |
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Term
an anticoagulant that binds and activates Antithrombin III, which inhibits the activated serine proteases. |
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Definition
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Term
what test is used to monitor patients on heparin therapy, and what is the desired result? |
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Definition
APTT, the goal is to be prolonged 2x normal |
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Term
A clot busting drug that was once produced from horse urine but is now produced from human renal cells in tissue culture |
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Definition
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Term
name two anticoagulant drugs |
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Definition
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Term
name two anti-platelet drugs |
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Definition
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Term
name three clot busting drugs |
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Definition
urokinase, streptokinase, tissue plasminogen activator |
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Term
the latest and most expensive clot busting drug. Made with new recombinant DNA technology |
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Definition
tissue plasminogen activator |
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Term
a product of beta hemolytic streptococci; still a widely used clot busting drug |
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Definition
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Term
name two general categories of disorders of the hemostatic system |
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Definition
bleeding disorders and clotting disorders |
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Term
another word for clotting |
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Definition
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Term
two subsets of antiphospholipid antibody syndrome |
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Definition
Lupus anticoagulant and Anti-cardiolipin antibody |
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Term
an autosomal dominant disorder that can lead to both arterial and venous thrombosis; characterized by spontaneous platelet aggregation in vitro |
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Definition
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Term
A mutated form of Factor V that leads to Activated Protein C resistance |
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Definition
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Term
what is the most common inherited form of hypercoagulability? |
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Definition
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Term
what inherited disorder is present in 3-15% of caucasians of northern european origin, but rare in persons of african or asian ancestry? |
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Definition
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Term
The screening test called Activated Protein C Resistance Test is used to test for what disorder? |
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Definition
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Term
A mutated prothrombin gene seen in 2-3% of general population and 5-18% of people with family history of deep vein thrombosis |
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Definition
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Term
anti-clotting drugs that inhibit ADP receptors |
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Definition
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Term
How is heparin therapy administered? |
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Definition
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Term
an overdose of warfarin is counteracted with what? |
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Definition
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Term
what drug is used to counteract a heparin overdose? |
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Definition
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Term
vasoconstriction and formation of platelet plug are what phase of hemostasis? |
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Definition
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Term
fibrin clot formation and fibrinolysis are in what phase of hemostasis? |
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Definition
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Term
two types of platelet function tests |
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Definition
platelet aggregation and clot retraction |
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Term
what does PRP stand for, and how do you get it? |
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Definition
Platelet rich plasma; you get it by centrifuging slowly |
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Term
what aggregating agents are used in the platelet aggregation test? |
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Definition
ristocetin, ADP, collagen, and arachidonic acid |
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Term
In a platelet aggregation test on a patient with von willebrand's disease, which aggregating agent will cause incomplete or no aggregation? |
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Definition
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Term
In a mixing study, if the pooled normal plasma does not correct the result, what is indicated? |
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Definition
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Term
in the mixing study test, if the addition of pooled normal plasma corrects the result, what is indicated? |
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Definition
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Term
what factor deficiency is associated with umbilical stump bleeding? |
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Definition
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Term
what factor deficiency is the 5 M Urea Test used to test for? |
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Definition
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Term
what are lupus anticoagulants directed against? |
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Definition
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Term
What two tests are good to use to test for lupus anticoagulant? |
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Definition
Kaolin clot time and platelet neutralization |
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Term
Fletcher factor is another name for what? |
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Definition
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Term
If a prolonged APTT is corrected by incubating the serum for 15 minutes instead of 5, what is indicated? |
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Definition
fletcher factor deficiency |
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Term
what does DRVVT stand for, and what is it used for? |
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Definition
Dilute Russel's Viper Venom Test, used to test for Lupus Anticoagulant |
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Term
what test can be used to rule out heparin contamination? |
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Definition
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Term
What is the cofactor to Protein C? |
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Definition
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Term
what activates Protein C? |
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Definition
Thrombin and thrombomodulin |
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Term
do immunologic assays measure function? |
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Definition
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Term
name four things that could cause acquired antithrombin III deficiency? |
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Definition
DIC, liver diease, sepsis, trauma |
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Term
in the antithrombin III chromogenic assay, what is the relationship between the amount of color and the amount of patient antithrombin III? |
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Definition
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Term
In the Activated Protein C resistance test, what result would indicate the presence of Factor V Leiden? |
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Definition
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Term
what is a quantitative but not functional test to check for von willebrand factor antigen? |
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Definition
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Term
How is prothrombin gene mutation detected? |
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Definition
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Term
what is a functional assay to test for von willebrand disease? |
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Definition
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