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Demographics, clinical profile and treatment landscape of patients with haemophilia B in China
Haemophilia, 2022Dear Editor, Haemophilia B (HB) is a rare inherited bleeding disorder resulting from mutations in the F9 gene encoding clotting factor IX (FIX) responsible for blood clotting.
Wenhui Zhang +8 more
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Factor IX inhibitors in haemophilia B: A report of National Haemophilia Registry in China
Haemophilia, 2022The development of inhibitors against factor FIX (FIX) is the most serious complication of FIX replacement therapy in haemophilia B (HB) patients. Currently, only few cohorts of HB inhibitor patients have been reported worldwide.
Xueqing Dou +19 more
semanticscholar +1 more source
Haemophilia, 2021
Inhibitors develop less frequently in haemophilia B (HB) than haemophilia A (HA). However, when present, the success of tolerization by immune tolerance induction (ITI) therapy is lower and the risk of complications higher.
J. Astermark +13 more
semanticscholar +1 more source
Inhibitors develop less frequently in haemophilia B (HB) than haemophilia A (HA). However, when present, the success of tolerization by immune tolerance induction (ITI) therapy is lower and the risk of complications higher.
J. Astermark +13 more
semanticscholar +1 more source
The Lancet, 2003
The haemophilias are inherited disorders in which one of the coagulation factors is deficient. Although deficiencies of factor VIII (haemophilia A) and factor IX (haemophilia B) are well recognised, von Willebrand's disease is much more common. Rare defects can occur in any of the coagulation factors.
K. John Pasi, Paula H. B. Bolton-Maggs
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The haemophilias are inherited disorders in which one of the coagulation factors is deficient. Although deficiencies of factor VIII (haemophilia A) and factor IX (haemophilia B) are well recognised, von Willebrand's disease is much more common. Rare defects can occur in any of the coagulation factors.
K. John Pasi, Paula H. B. Bolton-Maggs
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Clinical & Laboratory Haematology, 2008
Haemophilia B is extremely rare in females and so far 20 cases have been reported. A 9-year-old girl with severe haemophilia symptoms is described, who shows a very low level of factor IX activity (1.5%) and antigen (less than 10%), normal XX female karyotype and negative family history of bleeding tendency or consanguinity.
Akira Yoshioka +4 more
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Haemophilia B is extremely rare in females and so far 20 cases have been reported. A 9-year-old girl with severe haemophilia symptoms is described, who shows a very low level of factor IX activity (1.5%) and antigen (less than 10%), normal XX female karyotype and negative family history of bleeding tendency or consanguinity.
Akira Yoshioka +4 more
openaire +3 more sources
Haemophilia, 2020
Extended half‐life factor IX concentrates (EHL‐FIX) can be administered weekly to prevent bleeding for persons with severe haemophilia B. We report the experience of a large UK haemophilia comprehensive care centre using low dose EHL‐FIX for persons with
A. Rampotas +7 more
semanticscholar +1 more source
Extended half‐life factor IX concentrates (EHL‐FIX) can be administered weekly to prevent bleeding for persons with severe haemophilia B. We report the experience of a large UK haemophilia comprehensive care centre using low dose EHL‐FIX for persons with
A. Rampotas +7 more
semanticscholar +1 more source
Thrombosis and Haemostasis, 1972
SummaryVariants of haemophilia B were examined by antibody-neutralization technique and by the ox brain thromboplastin time method. On the basis of the presence or absence of anomalous factor IX and of prothrombin time, four variants of haemophilia B were discerned. It is shown that the prolongation of prothrombin time, measured in our cases, cannot be
Éva Puskás, Susan Elödi
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SummaryVariants of haemophilia B were examined by antibody-neutralization technique and by the ox brain thromboplastin time method. On the basis of the presence or absence of anomalous factor IX and of prothrombin time, four variants of haemophilia B were discerned. It is shown that the prolongation of prothrombin time, measured in our cases, cannot be
Éva Puskás, Susan Elödi
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Is haemophilia B less severe than haemophilia A?
Haemophilia, 2013SummaryA number of observations suggest that severe factor IX deficiency (<1%) may be less clinically severe than the corresponding factor VIII deficiency: (i) Less factor consumption. There is evidence that patients with haemophilia B (HB) consume yearly less FIX for replacement therapy than patients with haemophilia (HA).
M. Franchini, P. M. Mannucci
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The molecular basis of haemophilia B
Haemophilia, 1998Summary. The cloning and isolation of the human factor VIII (FVIII) gene in the mid‐1980s has lead to 10 years of increasing understanding of the genetic and hence the molecular basis of haemophilia A. These studies are not only of enormous potential benefit for accurate carrier detection and prenatal diagnosis in families with haemophilia A, but ...
C. A. Lee +5 more
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The Relative Frequency of Haemophilia A and B [PDF]
Thrombosis and Haemostasis, 1960 SummaryTwo tables show the haemophilia cases observed in Zurich and the relative frequency of haemophilia A and haemophilia B in Switzerland and some other countries.
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