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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.
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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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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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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Detection of Carriers of Haemophilia B
British Journal of Haematology, 1979Summary. Plasma levels of factor IX activity and factor IX antigen were determined in 18 definite carriers of haemophilia B‐, 10 definite carriers of haemophilia B+ and 40 control subjects. Factor IX antigen was determined by the electroimmunoassay technique of Laurell using a rabbit antiserum against factor IX.
K H, Orstavik +3 more
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Subperiosteal Haemorrhage in Haemophilia A and B
Acta Paediatrica, 1961SummaryDescription of two cases of extensive subperiosteal haematoma of the heel in a patient with haemophilia A and in a patient with haemophilia B. These haema‐tomata gave rise to extensive destruction of the calcaneus. Discussion of the means by which recovery was obtained.We are grateful to Dr. H. A. E.
, van CREVELD, M J, KINGMA
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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 A and haemophilia B. Are there relevant clinical differences?].
Hamostaseologie, 2010Haemophilia B (HB) was described in 1952 as a single disease for the first time. In comparison to haemophilia A (HA) the bleeding tendency seemed to be less severe. The aim of this study was to investigate this hypothesis in all patients with HA and HB treated in the haemophilia care center of the Vivantes Klinikum.All patients with severe HA and HB ...
R, Klamroth +3 more
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The Relative Frequency of Haemophilia A and B
Thrombosis and Haemostasis, 1960SummaryTwo 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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2012
Haemophilia A and B are hereditary, X-linked bleeding disorders caused, respectively, by deficiencies of coagulation factors FVIII and FIX. Haemophilia is among the most common hereditary human disorders; both haemophilia A and B have been identified in domestic animals and, as in humans, haemophilia A is the most common form.
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Haemophilia A and B are hereditary, X-linked bleeding disorders caused, respectively, by deficiencies of coagulation factors FVIII and FIX. Haemophilia is among the most common hereditary human disorders; both haemophilia A and B have been identified in domestic animals and, as in humans, haemophilia A is the most common form.
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Gene therapy for haemophilia B
Haemophilia, 2012Summary. AAV virus mediated transfer of factor IX to humans is safe and effective at three dose levels. Two subjects treated at highest dose level developed immune mediated transaminitis which resolved on a short course of Prednisolone. Beneficial effects in terms of continuous elevation of factor IX level above base line was seen in all subjects ...
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