Journal of thrombosis and haemostasis : JTH
-
Tranexamic acid (TXA) reduces blood loss by inhibiting the enzymatic breakdown of fibrin. It is often used in surgery to decrease bleeding and the need for blood transfusion. In 2011, results from a multi-center, randomized, and placebo-controlled trial (CRASH-2 trial) showed that TXA (1 g loading dose over 10 min followed by an infusion of 1 g over 8 h) safely reduces mortality in bleeding trauma patients. ⋯ Because it does not have any serious adverse effects, TXA can be administered to a wide spectrum of bleeding trauma patients. Limiting its use to the most severely injured or those with a diagnosis of 'hyperfibrinolysis' would result in thousands of avoidable deaths. A clinical trial (CRASH-3 trial) of TXA in patients with traumatic brain injury is now in progress.
-
J. Thromb. Haemost. · Jun 2015
Multicenter Study Comparative StudyEvaluation of von Willebrand factor phenotypes and genotypes in Hemophilia A patients with and without identified F8 mutations.
Hemophilia A (HA) is an X-linked bleeding disorder caused by a deficiency in factor VIII (FVIII). von Willebrand disease (VWD) is characterized by a quantitative or qualitative defect in von Willebrand factor (VWF). Patients with VWD with severely low VWF or VWD Type 2N (VWD2N), a VWD subtype distinguished by defective VWF binding to FVIII, may have reduced FVIII levels secondary to their VWD. These patients superficially resemble patients with HA and pose a potential for misdiagnosis. ⋯ These data emphasize that some patients diagnosed with HA require VWF assessments in order to achieve a comprehensive diagnosis and an optimal treatment strategy.
-
J. Thromb. Haemost. · Jun 2015
Comparative StudyQualitative point-of-care D-dimer testing compared with quantitative D-dimer testing in excluding pulmonary embolism in primary care.
General practitioners can safely exclude pulmonary embolism (PE) by using the Wells PE rule combined with D-dimer testing. ⋯ Combined with the Wells PE rule, both tests are safe to use in excluding PE. The quantitative test seemed to be safer than the POC test, albeit not statistically significant. The specificity of the POC test was higher, resulting in more patients in whom PE could be excluded.
-
J. Thromb. Haemost. · Jun 2015
Multicenter StudyRecombinant factor VIII Fc fusion protein for the prevention and treatment of bleeding in children with severe hemophilia A.
Prophylactic factor replacement, which prevents hemarthroses and thereby reduces the musculoskeletal disease burden in children with hemophilia A, requires frequent intravenous infusions (three to four times weekly). ⋯ Twice-weekly infusions with rFVIIIFc were well tolerated and yielded low bleeding rates in children with severe hemophilia A.
-
J. Thromb. Haemost. · Jun 2015
ReviewDysfibrinogenemia: from molecular anomalies to clinical manifestations and management.
Congenital dysfibrinogenemia is a qualitative congenital fibrinogen disorder characterized by normal antigen levels of a dysfunctional fibrinogen. The diagnosis is usually based on discrepancies between fibrinogen activity and antigen levels, but could require more specialized techniques for the assessment of fibrinogen function, owing to some limitations in routine assays. Molecular abnormalities, which are frequently heterozygous missense mutations localized in exon 2 of FGA and exon 8 of FGG, lead to defects in one or more phases of fibrinogen to fibrin conversion, fibrin network formation, and other important functions of fibrinogen. ⋯ Functional analysis of polymerization and fibrinolysis, structural studies of the fibrin network and the viscoelastic properties of fibrin clot could help to predict the phenotype of congenital dysfibrinogenemia, but have not yet been evaluated in detail. The management is essentially based on personal and family history; however, even individuals who are still asymptomatic and without a family history should be carefully assessed and monitored. Particular situations, such as pregnancy, delivery, and surgery, require a multidisciplinary approach.