Results 231 to 240 of about 12,413,959 (307)
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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.
Paula H B, Bolton-Maggs, K John, Pasi
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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.
Paula H B, Bolton-Maggs, K John, Pasi
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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
S, Elödi, E, Puskás
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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
S, Elödi, E, Puskás
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Haemophilia B Leyden in Greece
Thrombosis and Haemostasis, 1986SummaryIn this paper, a five generation Greek family is described with haemophilia B. The disease is characterized by a normal ox-brain prothrombin time, normal levels of the vitamin-K dependent clotting factors VII and X and a proportional reduction of factor IX acttivity and antigen levels all of which is consistent with the cross-reacting material ...
T, Mandalaki +3 more
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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
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.
A, 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.
A, Yoshioka +4 more
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Thrombosis and Haemostasis, 1962
SummaryA 6 year old girl with symptoms of severe haemophilia and a haemophilia B factor B content of 2%—3% of normal is described. Investigation of the family revealed that the mother and the sister of the girl were carriers of the haemophilia B gene, while the father was normal. Chromosome studies showed a pattern of 2 X chromosomes.
J E, NILEHN, I M, NILSSON
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SummaryA 6 year old girl with symptoms of severe haemophilia and a haemophilia B factor B content of 2%—3% of normal is described. Investigation of the family revealed that the mother and the sister of the girl were carriers of the haemophilia B gene, while the father was normal. Chromosome studies showed a pattern of 2 X chromosomes.
J E, NILEHN, I M, NILSSON
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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).
P M, Mannucci, M, Franchini
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Haemophilia, 2019
Haemophilia B is a recessive, X‐linked bleeding disorder due to inherited deficiency in vitamin K‐dependent coagulation factor IX (FIX). FIX activity levels, as a basis for the definition of disease severity, do not clearly correlate with bleeding ...
A. Tjärnlund-Wolf, R. Lassila
semanticscholar +1 more source
Haemophilia B is a recessive, X‐linked bleeding disorder due to inherited deficiency in vitamin K‐dependent coagulation factor IX (FIX). FIX activity levels, as a basis for the definition of disease severity, do not clearly correlate with bleeding ...
A. Tjärnlund-Wolf, R. Lassila
semanticscholar +1 more source