An investigation of three patients with Christmas disease due to an abnormal type of factor ix

AUTOR(ES)

Denson, K. W. E.

RESUMO

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.

Documentos Relacionados

• Purification and characterization of an abnormal factor IX (Christmas factor) molecule. Factor IX Chapel Hill.
• Activation of human factor IX (Christmas factor).
• Factor IX antigen by radioimmunoassay. Abnormal factor IX protein in patients on warfarin therapy and with hemophilia B.
• Cobalamin malabsorption in three siblings due to an abnormal intrinsic factor that is markedly susceptible to acid and proteolysis.
• CHRISTMAS FACTOR (IX) DEFECT IN A CASE OF HAEMORRHAGIC THROMBOCYTHAEMIA