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Flashcards in Hereditary disorders of coagulation Deck (10)
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1
Q

Factor VIII Deficiency (Hemophilia A)- Eti

A

Congenital deficiency in coagulation factor VIII. X-linked recessive-affected males

2
Q

Factor VIII Deficiency (Hemophilia A)- Sx

A

Spontaneous bleeding in joints & soft tissues, arthropathy, significant bleeding. Recurrent hemarthrosees & arthropathy

3
Q

Factor VIII Deficiency (Hemophilia A)- Dx

A

Low factor VIII in absence of other conditions. Often dx in infants

4
Q

Factor VIII Deficiency (Hemophilia A)- Tx

A

Plasma derived or recombinant factor concentrate infusions

5
Q

Factor IX deficiency (hemophilia B)- Eti

A

Congenital deficiency of coagulation factor IX. Less common

6
Q

Factor IX deficiency (hemophilia B)- Sx, Dx. Tx

A

Same as Hemophilia A

7
Q

Von Willebrand disease- Eti

A

Most common inherited bleeding disorder. Von Willebrand factor binds platelets to endothelium. Autosomal inheritance. 3 types, type 1 most common

8
Q

Von Willebrand disease- Sx

A

Bleeding involving integumentary system, mucous membranes- mild to severe.Decreased PTT, corrects with mixing.

9
Q

Von Willebrand disease- Dx

A

Assessment of von willebrand factors and Factor VIII activity

10
Q

Von Willebrand disease- Tx

A

DDAVP- releases VWF and FVIII from endothelial stores