Severe haemophilia B (Q89191): Difference between revisions
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Hemofilia B grave é uma forma de hemofilia B (veja este termo) caracterizada por uma deficiência importante de fator IX, levando a hemorragias espontâneas frequentes e a sangramento anormal como resultado de ferimentos menores ou subsequente a cirurgias ou extrações dentárias. Hemofilia B grave responde por cerca de 40% de todos os casos de hemofilia B. A atividade biológica do fator IX é inferior a 1%. A transmissão é recessiva ligada ao X, e o transtorno é causado por mutações no gene F9 (Xq28), que codifica o fator IX da coagulação. | |||
| description / en | description / en | ||
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. | |||
Revision as of 15:45, 15 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_1209364172 |
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| English | 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. |
