Severe haemophilia B (Q89191)

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Severe haemophilia B is a form of haemophilia B (see this term) characterised by a large deficiency of factor IX leading to frequent spontaneous haemorrhage and abnormal bleeding as a result of minor injuries, or following surgery or tooth extraction. Severe haemophilia B accounts for around 40% of all cases of haemophilia B. The biological activity of factor IX is below 1%. Transmission is X-linked recessive and the disorder is caused by mutations in the F9 gene (Xq28) encoding coagulation factor IX.
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    Severe haemophilia B
    Severe haemophilia B is a form of haemophilia B (see this term) characterised by a large deficiency of factor IX leading to frequent spontaneous haemorrhage and abnormal bleeding as a result of minor injuries, or following surgery or tooth extraction. Severe haemophilia B accounts for around 40% of all cases of haemophilia B. The biological activity of factor IX is below 1%. Transmission is X-linked recessive and the disorder is caused by mutations in the F9 gene (Xq28) encoding coagulation factor IX.

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