Factor IX, deficiency

The severity of bleeding associated with hemophilia correlates with the degree of factor VIII or factor IX deficiency as measured against the normal plasma standard. Table 64-1 summarizes the age at onset and laboratory and clinical manifestations of hemophilia.3... 

VIII.c. Factor IX Deficiency - Christmas Disease or Haemophilia B... 

Contraindications Active G1 bleeding, factor Vll or factor IX deficiencies, hypersensitivity to aspirin or NSAlDs... 

Clinical pharmacology Activated factor IX in combination with activated factor VIII activates factor X. This results ultimately in the conversion of prothrombin to thrombin. Thrombin then converts fibrinogen to fibrin, and a clot can be formed. Factor IX is the specific clotting factor deficient in patients with hemophilia B and in patients with acquired factor IX deficiencies. The administration of Coagulation Factor IX (Recombinant) increases plasma levels of factor IX and can temporarily correct the coagulation defect in these patients. 

Kay, M.A., Landen, C.N., Rothenberg, S.R., et al. (1994). In vivo hepatic gene therapy complete albeit transient correction of factor IX deficiency in hemophilia dogs. Proc. Natl. Acad. Sci. U.S.A., 91, 2353-2357. 

There are two types of hemophilias that have been identified, hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency). The incidence of hemophilia A is I in every 5000 live male births and for hemophilia B the incidence is I in every 30,000 live male births. Both types of hemophilia are... 

Factor IX-deficient dog PDAPP Tg mice Aged marmosets rcd1 dogs W/Wv mice... 

Lin H-F, Maeda N, Sithies O et al. (1997) A coagulation factor IX-deficient mouse model for human hemophilia B. Blood 90 3962-3966... 

Before the screening of blood and plasma and before virus inactivation procedures were applied to coagulation factor products (for example factor VIII and factor IX), many hemophiliacs who were treated with substitution therapy were exposed to infection with HIV. In the USA about 70% of tested persons with hemophiha A (factor VIII deficiency) and 35% with hemophilia B (factor IX deficiency) were HIV-seropositive (177). 

In two patients with severe factor IX deficiency and high concentrations of factor IX inhibitors who... 

Gregoire S, Kennedy A, Smiley RK. Acute hepatitis in a patient with mild factor IX deficiency after anesthesia with isoflurane. CMAJ 1986 135(6) 645-6. 

Trade names BeneFIX (Wyeth) Factor IX rFIX Indications Treatment and prophylaxis of bleeding in patients with haemophilia B (congenital factor IX deficiency). 

Factor IX complex, a blood derivative with hemostatic properties, is used in factor IX deficiency (hemophilia B or Christmas disease) in patients with factor VIII inhibition, in factor VII deficiency, and in overdosage with anticoagulant (see also Tables 17 and 18 and Figure 92). 

Factor IX is an antihemophilic agent that restores hemostasis in patients with factor IX deficiency. It is indicated in the control and prevention of hemorrhagic episodes in patients with hemophilia B (factor IX deficiency) (Proplex T only) bleeding episodes in patients with inhibitors to factor VIII, and prevention or control of bleeding episodes in patients with factor VII deficiency. 

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