Concept information
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Genetic Diseases, Inborn
Genetic Diseases, X-Linked
Hemic and Lymphatic Diseases
Hematologic Diseases
Blood Coagulation Disorders
Blood Coagulation Disorders, Inherited
Preferred term
Hemophilia B
Type
-
Topical Descriptor
Broader concept
Entry terms
- Christmas Disease
- Deficiencies, F9
- Deficiencies, Factor IX
- Deficiency, F9
- Deficiency, Factor IX
- Disease, Christmas
- F9 Deficiencies
- F9 Deficiency
- Factor IX Deficiencies
- Factor IX Deficiency
- Haemophilia B
- Haemophilia Bs
- Hemophilia B Leyden
- Hemophilia B(M)
- Hemophilia Bs (M)
- Plasma Thromboplastin Component Deficiency
Scope note
- A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)
History note
- 1999(1966)
In other languages
-
Finnish
-
B-hemofilia
-
Christmas disease
-
Christmasin tauti
-
deficientia hereditaria factoris IX
-
haemophilia B
-
hyytymistekijä IX:n perinnöllinen puute
-
hyytymistekijä IX:n puute
-
hyytymistekijä IX:n puutos
-
hyytymistekijä IX:n vajaatoiminta
-
morbus Christmas
-
plasman tromboplastiinikomponentin puute
-
PTC-puute
-
Swedish
-
Christmas sjukdom
URI
http://www.yso.fi/onto/mesh/D002836
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