• Journal of Yeungnam Medical Science
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• v.21 no.2
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• pp.231-236
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• 2004

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.

• Archives of Plastic Surgery
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• v.36 no.1
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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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• v.53 no.10
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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.