An investigation of three patients with Christmas disease due to an abnormal type of factor IX.
Three patients with Christmas disease whose plasma was shown to have a prolonged one-stage prothrombin time with ox brain thromboplastin have been investigated. These patients have an inhibitor for the reaction between factor X, factor VII, and ox brain extract. The abnormal constituent responsible for this inhibitor appears to be factor IX whuch is functionally inactive but antigenically indistinguishable from normal factor IX. It is proposed that patients might be classified into haemophilia B(+) for patients with this defect (Christmas disease(+)) and haemophilia B(-) (Christmas disease(-)) for patients who have classical Christmas disease.
D000818:Animals; D000941:Antigens; D001780:Blood Coagulation Tests; D002417:Cattle; D005164:Factor IX / Q000032:analysis*; D005167:Factor VII / Q000032:analysis; D005170:Factor X / Q000032:analysis; D002836:Hemophilia B / Q000097:blood* / Q000145:classification / Q000209:etiology; D006801:Humans; D005779:Immunodiffusion; D011516:Prothrombin / Q000032:analysis; D013925:Thromboplastin
D000941:Antigens; D005167:Factor VII; D011516:Prothrombin; D005164:Factor IX; D005170:Factor X; D013925:Thromboplastin