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Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.
BACKGROUND Effective ways to prevent arthropathy in severe hemophilia are unknown. METHODS We randomly assigned young boys with severe hemophilia A to regular infusions of recombinant factor VIIIExpand
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Prospective, randomised trial of two doses of rFVIIa (NovoSeven) in haemophilia patients with inhibitors undergoing surgery.
Recombinant factor VIIa (rFVIIa: NovoSeven; Novo Nordisk) has proven efficacy in the treatment of haemophilic patients with inhibitors. This prospective, double-blind study compared rFVIIa (35 vs. 90Expand
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Modern haemophilia care
Haemophilia care has undergone substantial improvements during the past 40-50 years. Early clotting factor concentrates were not sufficiently refined to enable self-administered treatment at homeExpand
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Plasminogen deficiency
Summary.  Plasminogen deficiency has emerged as a well‐recognized disorder in which reduced levels of plasminogen lead to the development of pseudo membranes on mucosal surfaces, with subsequentExpand
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Home treatment of mild to moderate bleeding episodes using recombinant factor VIIa (Novoseven) in haemophiliacs with inhibitors.
OBJECTIVE To assess the safety and efficacy of a fixed dose of recombinant activated factor VII (rFVIIa; NovoSeven) in the home setting for mild to moderately severe joint, muscle; and mucocutaneousExpand
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The polygenic nature of inhibitors in hemophilia A: results from the Hemophilia Inhibitor Genetics Study (HIGS) Combined Cohort.
Studies of determinants of development of inhibitory Abs to factor VIII in people with hemophilia A indicate a complex process involving multiple factors. The Hemophilia Inhibitor Genetics StudyExpand
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Plasminogen activator inhibitor type 1 deficiency
Summary.  Plasminogen activator inhibitor type 1 (PAI‐1) is an important component of the coagulation system that down‐regulates fibrinolysis in the circulation. Reduced PAI‐1 levels may result inExpand
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Recombinant factor IX-Fc fusion protein (rFIXFc) demonstrates safety and prolonged activity in a phase 1/2a study in hemophilia B patients.
Current factor IX (FIX) products display a half-life (t(1/2)) of ∼ 18 hours, requiring frequent intravenous infusions for prophylaxis and treatment in patients with hemophilia B. This open-label,Expand
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Rare bleeding disorders: diagnosis and treatment.
Despite the worldwide prevalence of rare bleeding disorders (RBDs), knowledge of these conditions and their management is suboptimal; health care professionals often have little diagnostic andExpand
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Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor.
The diagnosis of von Willebrand disease relies on abnormalities in specific tests of von Willebrand factor (VWF), including VWF antigen (VWF:Ag) and VWF ristocetin cofactor activity (VWF:RCo). WhenExpand
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