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Flashcards in Hereditary disorders of coagulation Deck (10)
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1

Factor VIII Deficiency (Hemophilia A)- Eti

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

2

Factor VIII Deficiency (Hemophilia A)- Sx

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

3

Factor VIII Deficiency (Hemophilia A)- Dx

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

4

Factor VIII Deficiency (Hemophilia A)- Tx

Plasma derived or recombinant factor concentrate infusions

5

Factor IX deficiency (hemophilia B)- Eti

Congenital deficiency of coagulation factor IX. Less common

6

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

Same as Hemophilia A

7

Von Willebrand disease- Eti

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

8

Von Willebrand disease- Sx

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

9

Von Willebrand disease- Dx

Assessment of von willebrand factors and Factor VIII activity

10

Von Willebrand disease- Tx

DDAVP- releases VWF and FVIII from endothelial stores