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Concept information

Hemic and Lymphatic Diseases > Hematologic Diseases > Hemorrhagic Disorders > Hemophilia B
Hemic and Lymphatic Diseases > Hematologic Diseases > Blood Coagulation Disorders > Coagulation Protein Disorders > Hemophilia B
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Blood Coagulation Disorders, Inherited > Hemophilia B
Hemic and Lymphatic Diseases > Hematologic Diseases > Blood Coagulation Disorders > Blood Coagulation Disorders, Inherited > Hemophilia B
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Genetic Diseases, X-Linked > Hemophilia B

Terme préférentiel

Hemophilia B  

Type

  • mesh:Descriptor

Définition

  • 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)

Concept générique

Concepts associés

Synonyme(s)

  • Christmas Disease
  • Deficiency, Factor IX
  • F9 Deficiency
  • Factor IX Deficiency
  • Haemophilia B
  • Hemophilia B Leyden
  • Hemophilia B(M)
  • Plasma Thromboplastin Component Deficiency

Qualificatif(s) autorisé(s)

Traductions

  • Hémophilie B

    français

  • Carence héréditaire en facteur IX
  • Déficit en facteur IX
  • Déficit en FIX
  • Déficit héréditaire en facteur IX
  • Hémophilie par déficit en facteur IX
  • Maladie de Christmas

URI

http://data.loterre.fr/ark:/67375/JVR-ZWVQTNBQ-N

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RDF/XML TURTLE JSON-LD Date de création 01/01/1999, dernière modification le 28/06/2016