A Case of Congenital Factor VII Deficiency Presented with Subacute Subdural Hematoma

A congenital factor VII deficiency is a rare disorder with an estimated incidence in the western contries of one in 500,000. Because factor VII is important in initiation the coagulation cascade, a factor VII deficiency can result in significant bleeding with prolongation of the prothrombin time. We present a case of a factor VII deficiency with a subdural hematoma in an 18-year-old boy whose plasma activity of factor VII was ${\leq}10%$. Previously, he did not have any symptoms, such as hemarthrosis, easy bruising or bleeding after a minor trauma. He was administered fresh frozen plasma and a trephination was performed. His sister also had 51% lower level of factor VII.

제 VII 혈액응고인자는 외인계 혈액응고기전에 매우 중요한 역할을 한다. 선천성 제 VII 혈액응고인자 결핍증은 구미에서는 인구 500,000명당 한명의 발생률을 보이는 드문 질환으로 상염색체 열성으로 유전되는 것으로 알려져 있으며 국내에서의 보고는 드물다. 이에 저자들은 경막하 출혈로 입원한 환자에서 발견된 선천성 제 VII 혈액응고인자 결핍증을 경험하고 보고하는 바이다.