Hemophilia A and B are X-chromosome linked bleeding disorders caused by deficiency of the respective coagulation factor VIII and IX. Affected individuals develop a variable phenotype of hemorrhage caused by a broad range of mutations within the Factor VIII or Factor IX gene. Here, were report the results of the molecular diagnosis in a five Costa Rican families affected with Hemophilia. Methods...

Endothelial cells expose specific receptors for blood clotting factors and, upon perturbation, can initiate and propagate the reactions of the extrinsic pathway of blood coagulation leading to fibrin formation on the cell surface. The existence of an intrinsic mechanism of Factor IX activation on cultured human umbilical vein cells (HUVECs) was investigated by studies of the interaction between...

In This Issue: • NovoSeven • Therapeutic Interchanges Introduction: Recombinant human activated Factor VII (rFVIIa), or NovoSeven, is a hemostatic agent which is structurally similar to human plasma-derived coagulation Factor VIIa. Recombinant FVIIa is currently licensed by the US Food and Drug Administration (FDA) in patients with hemophilia A or B with inhibitors to Factor VIII or Factor IX o...

Abstract In Coronavirus disease 2019 (COVID-19) subjects, recent evidence suggests the presence of unique coagulation abnormalities. this study, we performed clot waveform analyses to investigate whether specific modulations are observed in COVID-19 subjects. We analyzed second derivative absorbance routine APTT tests using an ACL-TOP system. high frequencies abnormal patterns second-derivative...

Hemophilia A and B are X-linked diseases that predominantly affect male patients. Patients can develop coagulation factor inhibitors, which exponentially increases the treatment cost. However, the prevalence of factor VIII and IX inhibitors in Saudi Arabia is unclear.This study aimed to determine the Saudi prevalence of factor VIII and IX inhibitors.This 4-year, 7-center, cross-sectional study ...

ACQUIRED HEMOPHILIA A occurs as a result of development of inhibitory autoantibodies to factor VIII in the coagulation cascade. These antibodies result in impaired hemostasis and bleeding disorders of variable severities through varied mechanisms including impairment of binding of factor VIII to membrane phospholipids, factor IX, and/or von Willebrand factor. Common presentation includes mucocu...

A global phase 3 study evaluated the pharmacokinetics, efficacy and safety of a recombinant fusion protein linking coagulation factor IX with albumin (rIX-FP) in 27 previously treated male children (1-11 years) with severe and moderately severe haemophilia B (factor IX [FIX] activity ≤2 IU/dl). All patients received routine prophylaxis once every seven days for up to 77 weeks, and treated any b...

Coagulation factor IX-binding protein isolated from Trimeresurus flavoviridis (IX-bp) is a C-type lectin-like protein. It is an anticoagulant protein consisting of homologous subunits A and B. The subunits both contain a Ca2+-binding site with differing affinity (Kd values of 14 and 130 microM at pH 7.5). These binding characteristics are pH-dependent; under acidic conditions, the affinity of t...

Factor XI (FXI) is the zymogen of an enzyme (FXIa) that contributes to hemostasis by activating factor IX. Although bleeding associated with FXI deficiency is relatively mild, there has been resurgence of interest in FXI because of studies indicating it makes contributions to thrombosis and other processes associated with dysregulated coagulation. FXI is an unusual dimeric protease, with struct...

background recombinant activated factor vii (rfviia; novoseven® rt, novo nordisk, bagsvaerd, denmark) is a synthetic pro-coagulation factor derived from hamster kidney cells. objectives the purpose of this study was to evaluate the prescribing patterns of recombinant factor viia (rfviia) at a single, tertiary care pediatric hospital by indication of usage and dose administered. materials and me...