Final answer:
Factor VII deficiency results in an elevated prothrombin time and is treated with recombinant factor VIIa or fresh frozen plasma; monitoring factor VII activity levels is critical during treatment.
Step-by-step explanation:
A congenital factor VII deficiency presents with an elevated prothrombin time (PT). Treatment includes recombinant factor VIIa (rFVIIa) or fresh frozen plasma.
Factor VII is part of the extrinsic pathway of blood coagulation, which responds quickly to tissue damage by forming a complex with tissue factor (factor III) and calcium to activate factor X, leading to clot formation. Factor VII deficiency can lead to inadequate clotting and is most noticeably detected with a prolonged PT, as this test measures the efficiency of the extrinsic and common clotting pathways. When treating congenital factor VII deficiency, it is important to monitor factor VII activity levels to ensure therapeutic effectiveness and to manage dosing.
Additionally, observing for possible adverse reactions to treatment, such as thrombosis or allergic reactions, is crucial in the management of the condition. In the future, genetic therapy may provide alternate treatment for such bleeding disorders.