• Journal of Yeungnam Medical Science
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• 제21권2호
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• pp.231-236
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• 2004

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.

• Archives of Plastic Surgery
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• 제36권1호
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• pp.93-95
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• 2009

Purpose: Congenital factor VII (FVII) deficiency is a rare bleeding disorder and surgery can cause excessive bleeding due to an extrinsic pathway problem. It can be diagnosed by increased PT and decreased FVII level in coagulation test. Symptom varies according to the level of FVII, but it is essential to prevent intraoperative excessive bleeding. Methods: In this report, we described the orthognatic surgery experience in a mandibular prognathism patient with congenital FVII deficiency, in which recombinant activated factor VII (rFVIIa) was used to manage the bleeding. Rsults: We could get a successful result without any complication and there was minimal intraoperative bleeding. Conclusion: The orthognathic surgery could therefore be safely performed in patients with congenital factor VII deficiency using rFVIIa.

• Clinical and Experimental Pediatrics
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• 제53권10호
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• pp.913-916
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• 2010

Congenital factor VII deficiency is a rare autosomal-recessive bleeding disorder. Bleeding manifestations and clinical findings vary widely, ranging from asymptomatic subjects to patients with hemorrhages that may cause significant handicaps. Treatment has traditionally involved factor VII(FVII) replacement therapy using fresh frozen plasma, prothrombin complex concentrates or plasma-derived FVII concentrates. Recombinant activated FVII ($NovoSeven^{(R)}$) is currently considered the first-line treatment for replacement therapy of FVII deficiency. Here we present a case of severe intracerebral and intraventricular hemorrhage in a neonate with congenital FVII deficiency.