Efficacy of a high-purity factor IX concentrate in hemophilia B patients undergoing surgery
نویسندگان
چکیده
منابع مشابه
Circulating factor IX antigen-inhibitor complexes in hemophilia B- following infusion of a factor IX concentrate.
A persistent low-titer factor IX Inhibitor was discovered in a patIent with severe hemophllia B. The Inhibitor was very likely an immunoglobulin, since it was present in serum, was not dialyzable, retained its potency after heating to 56#{176}C, and was bound by staphylococcal protein A (SPA). When the hemophilia B patient with the inhibItor was given therapeutic infusions of factor Ix concentr...
متن کاملMolecular Characterization of the Factor IX Gene in 28 Iranian Hemophilia B Patients
Background: Heterogeneous mutations in the human coagulation factor IX gene lead to an X-linked recessive bleeding disorder known as hemophilia B. The disease is distributed worldwide with no ethnic or geographical priority. Materials and Methods: The aim of this study was to characterize the factor IX gene mutations in 28 unrelated Iranian hemophilia B patients. Polymerase chain reaction (PCR)...
متن کاملStudies of factor IX concentrate therapy in hemophilia.
The effects of factor IX concentrate therapy on hemostasis in hemophilia patients were studied by means of the radiometric factor IXa assay, the coupled amidolytic assay for factor VIIa, and coagulant assays for factors II, IX, and X, and antithrombin III. Both activated and unactivated concentrates contained factors VIIa and IXa, with the highest levels in the activated concentrates. Factors V...
متن کاملFactor IX variants improve gene therapy efficacy for hemophilia B.
Intramuscular injection of adeno-associated viral (AAV) vector to skeletal muscle of humans with hemophilia B is safe, but higher doses are required to achieve therapeutic factor IX (F.IX) levels. The efficacy of this approach is hampered by the retention of F.IX in muscle extracellular spaces and by the limiting capacity of muscle to synthesize fully active F.IX at high expression rates. To ov...
متن کاملLabeled factor IX kinetics in patients with hemophilia-B.
Labeled factor IX was infused five time into four patients with hemophilia-B. Ten-minute plasma recovery average 35% (SD +/- 2) and the mean T 1/2 beta-phase elimination was 23 hr (+/- 5). No alteration in the postinfusion 125I-factor-IX could be detected by radioautography of plasma samples run on polyacrylamide gels or on crossed-immunoelectrophoresis. Label was excreted into the urine as fre...
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ژورنال
عنوان ژورنال: Revista Brasileira de Hematologia e Hemoterapia
سال: 2002
ISSN: 1516-8484
DOI: 10.1590/s1516-84842002000200007