hemophilia b is factor ix deficiency and is inherited as x-linked recessive disorder. the subject of carrier detection in hemophilias has received new impetus in the last several years. analysis of factor ix gene polymorphisms is considered the best approach for prenatal diagnosis and hemophilia b carrier detection, if the identification of the gene mutation is possible. allele frequencies of t...

Human plasma–derived factor IX (pdFIX) concentrates are routinely used to treat patients with hemophilia B, an X-linked bleeding disorder that affects 1 in 30 000 males, but concerns remain regarding transmission of blood-borne pathogens. Therefore, the safety and efficacy of recombinant human factor IX (rFIX) were evaluated. A 20-center international trial was conducted in previously treated p...

Two structurally different forms of activated human Factor IX (Factor IXaa and IXa8) have been previously reported to have essentially identical clotting activity in vitro. Although it has been shown that activated Factor IXChapel Hills an abnormal Factor IX isolated from the plasma of a patient with mild hemophilia B, and normal Factor IXaa are structurally very similar, the clotting activity ...

FEIBA (factor eight inhibitor by-passing activity) Immuno was used to achieve hemostasis in 46 patients with factor VIII inhibitors with titers greater than 4 Bethesda units, and 3 patients with factor IX inhibitors. One-hundred and sixty-five bleeding episodes were treated with 50-70 U/kg; 102 of these episodes occurred in joints. 20 in mucous membranes, 33 muscle and soft tissue, and 10 were ...

BACKGROUND Hemophilia is caused by deficiencies in coagulation factor VIII or IX, resulting in direct blockade of the intrinsic tenase complex and indirect blockade of the extrinsic tenase complex which is rapidly inhibited upon binding of factor Xa to tissue factor pathway inhibitor. We evaluated the ability of Gla-domainless factor Xa, a truncated form of factor Xa devoid of procoagulant prop...

Factor IX deficiency was associated with a hemorrhagic disorder in a woman who previously had experienced postpartum hypotension resulting in Sheehan’s syndrome. There was no family history of Christmas disease, and other known causes of factor IX deficiency were excluded. Plasma levels of factor IX were partially corrected by therapy with thyroid hormones and completely restored to normal by t...

lmmunoassays with a monoclonal antibody (A-i ) detect a prevalent dimorphism in plasma coagulation factor IX. The antibody was shown to react with a dimorphic segment of the normal factor IX sequence as follows. First. A-i bound to isolated activation peptide (residues 146 through 180) prepared from activated factor IX from a normal plasma pool. Second, binding of recombinant factor lXs with A-...

Hemophilia B is a recessive bleeding disorder resulting from mutations in the coagulation factor IX gene. As this disease is characterized by clinical and molecular heterogeneity, the building of relationship between its genotype and phenotype would be great helpful for better diagnosis, prognosis and treatment. We use a descriptively probabilistic method, cross-impact analysis, to couple the c...