Congenital factor VII deficiency : A case report.

Abstract

Congenital factor VII deficiency is a rare bleeding disorder. Bleeding attributable to factor VII deficiency is manifest mainly as epistaxis, easybruising, intra or periarticular hemorrhage,gastrointestinal bleeding. Intracranial hemorrhages is a frequent cause of death. Factor VII is a substance active in the second phase of blood coagulation which is concerned with the conversion of prothrombin to thrombin. Thrombin is the enzyme essential for formation of fibrin from fibrinogen and factor VII is a vitamin K - dependent factor, but vitamin k administration is of no therapeutic benefit in the treatment of hereditary deficiencies.

Homeostasis screening test in factor VII deficiency reveal a normal PTT and normal thrombin time with a prolonged PT. Specific factor assays for factor VII confirm this diagnosis

A thai female newborn, 23 days - of age came to see doctor with convulsion marked anemia and coagulation disorder at Trat hospital. Ultrasound brain show intracerebellar hemorrhages and hydrocephalus. Specific factor assays confirm congenital factor VII deficiency.