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Hematology/Oncology Clinics of North America, 2022
Adeno-associated virus (AAV)-mediated gene transfer has successfully raised, and in some cases transiently normalized, FVIII or FIX activity levels in adults with severe hemophilia. Raising FVIII/IX levels, particularly greater than ∼15 IU/dL (mild deficiency), corresponds to a marked decrease in spontaneous and provoked bleeding, dramatic reduction in
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Adeno-associated virus (AAV)-mediated gene transfer has successfully raised, and in some cases transiently normalized, FVIII or FIX activity levels in adults with severe hemophilia. Raising FVIII/IX levels, particularly greater than ∼15 IU/dL (mild deficiency), corresponds to a marked decrease in spontaneous and provoked bleeding, dramatic reduction in
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Acta Haematologica, 2001
Hemophilia B is rare in females and only a few cases have been reported. In this report, we describe a girl with a clinically severe course of hemophilia B but with a normal 46,XX karyotype. She had no signs of Turner’s syndrome or any other dysmorphic features.
Shrimati Shetty +2 more
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Hemophilia B is rare in females and only a few cases have been reported. In this report, we describe a girl with a clinically severe course of hemophilia B but with a normal 46,XX karyotype. She had no signs of Turner’s syndrome or any other dysmorphic features.
Shrimati Shetty +2 more
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Seminars in Thrombosis and Hemostasis, 2018
AbstractHemophilia B (HB) is an X-linked bleeding disorder caused by deficiency of factor IX (FIX). Patients with the severe form (FIX <1%) account approximately for 30 to 45% of persons with HB and usually suffer from recurrent joint, soft-tissue, and muscle bleeds.
Erminia Baldacci +6 more
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AbstractHemophilia B (HB) is an X-linked bleeding disorder caused by deficiency of factor IX (FIX). Patients with the severe form (FIX <1%) account approximately for 30 to 45% of persons with HB and usually suffer from recurrent joint, soft-tissue, and muscle bleeds.
Erminia Baldacci +6 more
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Hemophilia A and Hemophilia B: Different Types of Diseases?
Seminars in Thrombosis and Hemostasis, 2013Hemophilia A and B are traditionally considered clinically indistinguishable; however, differences in bleeding frequency, clinical scores, use of prophylaxis, and need for orthopedic surgery have been reported, suggesting that the bleeding tendency associated with factor IX deficiency may be less severe with consequent better outcomes in the long term ...
Maria Rosaria Fasulo, Elena Santagostino
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Molecular therapeutics of hemophilia A and B
Expert Review of Hematology, 2022Hemophilia A (HA) or B (HB) is an X-linked recessive disorder caused by a defect in the factor VIII (FVIII) or factor IX (FIX) gene which leads to the dysfunction of blood coagulation. Protein replacement therapy (PRT) uses recombinant proteins and plasma-derived products, which incurs high cost and inconvenience requiring routine intravenous infusions
Jie, Gong, Hao-Lin, Wang, Lung-Ji, Chang
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2018
This chapter outlines the pathophysiology, clinical presentation, inheritance, and incidence of hemophilia A and B. The chapter describes the interaction with pregnancy and discusses the medical and anesthetic management of pregnant women with hemophilia A and B, including treatment of acute hemorrhage.
Joanne Douglas, James P. R. Brown
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This chapter outlines the pathophysiology, clinical presentation, inheritance, and incidence of hemophilia A and B. The chapter describes the interaction with pregnancy and discusses the medical and anesthetic management of pregnant women with hemophilia A and B, including treatment of acute hemorrhage.
Joanne Douglas, James P. R. Brown
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Emerging drugs for hemophilia B
Expert Opinion on Emerging Drugs, 2014Hemophilia B is a rare congenital bleeding disorder characterized by a deficiency of coagulation factor IX (FIX). Hemophilia B patients experience mild-to-severe bleeding complications according to the degree of FIX defect. Prophylaxis, with regular infusion of FIX concentrates, is nowadays, the mainstay of hemophilia care.
Pier Mannuccio Mannucci +1 more
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Current Management of Hemophilia B
Hematology/Oncology Clinics of North America, 1993The management of hemorrhagic episodes in patients with hemophilia B is in transition as a result of the availability of new products for replacement therapy. Although the basic principles of therapy have not changed, the new highly purified Factor IX concentrates, AlphaNine and Mononine, represent a break-through, as they appear to be safe in terms of
Harold R. Roberts, Mary E. Eberst
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The Genetic Heterogeneity of Hemophilia B
New England Journal of Medicine, 1970Abstract At least three genetic variants of hemophilia B exist: a variant with positive crossreacting material associated with a normal oxbrain prothrombin time; one with positive material but with...
Paul E. Brown +2 more
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