Journal of thrombosis and haemostasis : JTH
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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.
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J. Thromb. Haemost. · Jun 2015
Randomized Controlled Trial Multicenter StudyFresh frozen plasma transfusion fails to influence the hemostatic balance in critically ill patients with a coagulopathy.
Coagulopathy has a high prevalence in critically ill patients. An increased International Normalized Ratio (INR) is a common trigger to transfuse fresh frozen plasma (FFP), even in the absence of bleeding. Therefore, FFP is frequently administered to these patients. However, the efficacy of FFP in correcting hemostatic disorders in non-bleeding recipients has been questioned. ⋯ In non-bleeding critically ill patients with a coagulopathy, FFP transfusion failed to induce a more procoagulant state.
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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.