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24 Cards in this Set

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Factor XII
Hageman Factor - binds to exposed collagen; Activated by HMWK and PK
Factor XI
PTA - activated by factor XIIa
Factor IX
Christmas Factor, PTC - activated by factor XIa in presence of Ca2+
Factor VII
Pronvertin SPCA - Activated by Thrombin in the presence of Ca2+; in tissue
Factor X
Stuart-Prower Factor - Activates on surface of platelets, VIIa, TF, Ca2+
Factor II
Prothrombin - Activated on surface of platelets, prothrombinase complex
Factor VIII
Antihemophilia factor a, AHG - activated by thrombin; factor VIIIa is cofactor for X by IX activation
Factor III (TF)
Tissue Factor - sub endothelial cell surface glycoprotein; cofactor for VII
Factor I
Fibrinogen - cleaved by thrombin to form fibrin clot
Factor XIII
Fibrin Stabilizer - Activated by thrombin in presence of Ca2+
von Willebrand Factor
associated with sub endothelial connective tissue; serves as bridge between platelets glycoprotein GbIb/IX and collagen
Protein C
Activated to Ca by thrombin bound to thrombomodulin; degrades factors VIIIa and Va
Protein S
Cofactor for protein C; both contain gla residues
Thrombomodulin
Protein on surface of endothelial cells; binds thrombin to activate protein C
Antithrombin III
Most important coagulation inhibitor; controls activities of IIa, factors IXa, Xa, XIa and XIIa
Prekallikrein - PK
Fletcher Factor
High Molecular Weigh Kininogen - HMWK
Contact activation factor
Factor IV
CALCIUM yo!!!
Hemophilia A
-Factor VIII
-X-linked w/ severe, mod, mild
-150 mutations
-1:5000, 1:10,000 males
-joint and muscle hemorrhage
-easy bruising & prolonged bleeding
-treatment w/ VIII concentrates
Hemophilia B
-Factor IX
-1/10 of A prevelance
-X-linked w/ severe, mod, mild
-300 mutations
-85% point mutations
-3% short nucleotide deletions or insertions
-12% gross gene alterations
Disorders of Fibrinogen & Factor XIII
-elevated levels in patients with CHD, hperlipidemias, diabetes and hypertension
-in pregnancy, menopause, women on oral contraceptives and smokers
-other rare disorders show complete lack or deficiency
-Afibringenemia: neonatal umbilical cord hemorrhage, mucosal hemorrhagen and recurrent abortion
-Factor XIII - rare
von Willebrand Disease
-most common
-due to inherited deficiency in vWF
-most commonly inherited bleeding disorder
-defective platelet adhesion (VIII)
-heterogeneous disorder type 1, 2, and 3
-Autosomal dominant
Factor XI and Contact Adhesion
-deficiency of Factor XI results in injury-related bleeding
-common in Ashkenazic jews
-autosomal dominantL: three independent point mutations
Antithrombin Deficiency
-Deficiency ranges from 1:2000 to 1:5000 in general pop
-deficiency arise from mutations that affect synthesis or stability and from mutations that affect protease and/or heparin binding sites
-clinical consequences are deep vein thrombosis and pulmonary embolism
-acute episodes and treated with heparin infusion