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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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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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Gene Therapy with Fidanacogene Elaparvovec in Adults with Hemophilia B.
New England Journal of MedicineBACKGROUND Fidanacogene elaparvovec, an adeno-associated virus (AAV) gene-therapy vector for hemophilia B containing a high-activity human factor IX variant (FIX-R338L/FIX-Padua), was associated with sustained factor IX activity in a phase 1-2a study ...
A. Cuker +22 more
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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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Dental Extractions in Hemophilia A and B
JAMA: The Journal of the American Medical Association, 1971To the Editor.— In their article on the effective surgical treatment of patients with classic hemophilia and Christmas disease with human coagulation factor concentrates ( 214 : 1673,1970) Grant and Breckenridge also reported on uncomplicated dental extractions of deciduous and adult teeth in classic hemophilia and in Christmas disease with high ...
S. van Creveld, R. Buchner
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Tympanoplasty in a patient with hemophilia B
Auris Nasus Larynx, 1999We report the case of a 36-year-old hemophilia B who suffered from cholesteatoma and underwent tympanoplasty. Though the factor IX activity was less than 1% of normal before surgery, adequate replacement of factor IX led to the achievement of hemostasis during and after surgery.
Takashi Matsunaga +5 more
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2009
Publisher Summary This chapter discusses the bleeding disorder known as hemophilia B. The disease results from a congenital deficiency or absence of Factor IX (FIX), leading to lifelong bleeding risk and it is X-linked recessive disorder and as such the majority of affected patients are male.
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Publisher Summary This chapter discusses the bleeding disorder known as hemophilia B. The disease results from a congenital deficiency or absence of Factor IX (FIX), leading to lifelong bleeding risk and it is X-linked recessive disorder and as such the majority of affected patients are male.
openaire +3 more sources

