Factor VII deficiency.
As yet, there have been neither systematic reviews nor reports of randomized, controlled trials involving factor VII (FVII) deficiency. Hence, a picture of this disorder can only be drawn by reviewing and summarizing the data that is available. This article provides an overview of the understanding of this rare, inherited disorder of coagulation. In particular, the status of current knowledge of the disorder's prevalence, clinical presentation, diagnostic characteristics and molecular genetics is reviewed, followed by a summary of currently available options for its treatment and management.
Haemophilia Centre and Haemostasis Unit, Royal Free and University College Medical School, Pond Street, Hampstead, London, UK. D.perry@rfc.ucl.ac.uk
David J
DJ
Perry
D001779:Blood Coagulation Factors / Q000627:therapeutic use; D005167:Factor VII / Q000737:chemistry / Q000235:genetics / Q000627:therapeutic use; D005168:Factor VII Deficiency / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics; D015942:Factor VIIa; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D008958:Models, Molecular; D009154:Mutation; D010949:Plasma; D011110:Polymorphism, Genetic; D015995:Prevalence; D011487:Protein Conformation; D011994:Recombinant Proteins / Q000627:therapeutic use
D001779:Blood Coagulation Factors; D011994:Recombinant Proteins; C025667:prothrombin complex concentrates; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa