title: Use of recombinant, activated factor VII in the treatment of congenital factor VII deficiencies.
abstract: OBJECTIVE
  Factor VII (FVII) deficiency is a rare coagulation disorder, historically treated with prothrombin complex concentrates or plasma-derived FVII concentrates. We treated such patients (n = 17) with a recombinant, activated FVII preparation.
  
  
  METHODS
  Twenty-seven spontaneous bleeding episodes were treated and 7 major and 13 minor surgical interventions were carried out. The dosages employed ranged from 8.08 to 70.5 Ig/kg body weight.
  
  
  RESULTS
  A mean dose between 22 and 26 Ig/kg was sufficient to normalise the prothrombin time. Fifteen haemarthroses were treated with single doses and results were excellent in 13 cases. In 5/6 bleeding episodes of other types, the treatment gave either excellent or at least effective results. Haemostasis was secured in the 7 major and 13 minor surgical interventions. One patient developed antibodies 4-5 weeks after an extremely high dose. Otherwise, there were no side effects and no evidence of a thrombotic tendency.
  
  
  CONCLUSIONS
  This recombinant concentrate is efficacious in FVII-deficient patients. It is safe since any risk of transmission of blood-borne viruses is eliminated.
authors.affiliation: University of Palermo, Italy. mariangu@tin.it
authors.forename: G
authors.initials: G
authors.lastname: Mariani
authors.forename: M G
authors.initials: MG
authors.lastname: Testa
authors.forename: T
authors.initials: T
authors.lastname: Di Paolantonio
authors.forename: R
authors.initials: R
authors.lastname: Molskov Bech
authors.forename: U
authors.initials: U
authors.lastname: Hedner
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002675:Child, Preschool; D004305:Dose-Response Relationship, Drug; D005168:Factor VII Deficiency / Q000151:congenital* / Q000188:drug therapy* / Q000601:surgery; D015942:Factor VIIa / Q000008:administration & dosage* / Q000037:antagonists & inhibitors / Q000276:immunology; D006395:Hemarthrosis / Q000188:drug therapy; D006487:Hemostasis / Q000187:drug effects; D006801:Humans; D007223:Infant; D007518:Isoantibodies / Q000097:blood; D008297:Male; D008875:Middle Aged; D011516:Prothrombin / Q000187:drug effects; D011994:Recombinant Proteins / Q000008:administration & dosage; D013917:Thrombin / Q000187:drug effects; D013997:Time Factors
chemical_list: D007518:Isoantibodies; D011994:Recombinant Proteins; D011516:Prothrombin; D015942:Factor VIIa; D013917:Thrombin