Exact(8)
Jiang, M. et al. A novel missense mutation close to the charge-stabilizing system in a patient with congenital factor VII deficiency.
Dr. Dorgalaleh's primary interest is the diagnosis and management of congenital bleeding disorders, and he has a special interest in congenital factor XIII deficiency.
Congenital factor VII (FVII) deficiency is a rare autosomal recessive coagulation disorder that is characterized by prolongation of prothrombin time.
Currently, it is also approved to patients with congenital factor VII deficiency or the patients who have acquired antibodies to factor VIII, which is called as acquired hemophilia A. Furthermore, there have been increasing numbers of reports describing the off-label use of rFVIIa, which was effective in controlling refractory bleeding in nonhemophilic patients [1 4].
Hence the diagnosis of congenital factor V deficiency was made.
If prolongation of PT persists in spite of extra vitamin K, a congenital factor VII deficiency must be considered [ 18].
The hemostatic properties of recombinant activated factor VII (rFVIIa) are established in patients with inherited or acquired hemophilia with inhibitors and in patients with congenital factor VII deficiencies.
rFVIIa is a US Food and Drug Administration-approved treatment for congenital factor VII deficiency and hemophiliacs with inhibitors to factor VIII.
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