Congenital Factor VII Deficiency
Congenital Factor VII deficiency is an autosomal recessive disorder
that occurs in 1 of every 300,000 to 500,000 people.89 The
most common symptoms of congenital FVII deficiency, other
than excessive bleeding after injury or invasive procedures,
include the following:
- Frequent nosebleeds
- Bleeding from the gums
- Very heavy, prolonged menstrual bleeding
- Head bleeds in newborns
- Heavier-than-normal bleeding at circumcision
- Intestinal bleeding
Congenital FVII deficiency can be diagnosed as early as
the time of birth. A clotting assay will confirm a congenital
FVII deficiency. A prolonged prothrombin time (PT) and normal
activated partial thromboplastin time (aPTT) are indicators
of the deficiency.
Patients with congenital FVII deficiency are grouped by
severity:
- Mild or moderate congenital FVII deficiency—Patients
have 2–10% of the normal level of FVII. They may or
may not experience spontaneous bleeding episodes of any kind.
- Severe congenital FVII deficiency—Patients
have less than 2% of the normal level of FVII. They experience
spontaneous bleeding episodes similar to someone with hemophilia.
During a spontaneous bleeding episode or during a surgical
procedure, a congenitally FVII-deficient patient needs enough
FVII to promote effective hemostasis.
Recombinant activated FVII (NovoSeven® RT) replaces Factor VII in the clotting process. It also works at the site of injury, and small dosing volumes and intervals ensure that patients get only the amount needed to resolve the bleed.
Get information about Efficacy for congenital Factor VII deficiency.
Get information about Dosing and Administration for NovoSeven® RT for congenital Factor VII deficiency.
