KMID : 0374920110320010183
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Inje Medical Journal 2011 Volume.32 No. 1 p.183 ~ p.187
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A Case of Congenital Factor VII Deficiency in a Newborn Infant Presented with Intraventricular Hemorrhage
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Park Hyung-Jun
Kim Young-Dae Kim Woo-Kyung Park Yong-Won
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Abstract
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Congenital factor VII deficiency is a rare bleeding disorder that has an estimated prevalence of 1/300,000 to 1/500,000. Transmission is autosomal recessive. Because factor VII is important in initiation the coagulation cascade, a factor VII deficiency can result in significant bleeding with pr oiongation of the prothrombin time. In severely affected patients, bleeding starts early in life and encompasses life-threatening hemorrhages such as Intracranial and gastrointestinal hemorrhages. These are the most frequent bleeding events in the first 6 months of life. Definitive diagnosis tests on a specific assay for factor VII clotting activity. Human factor VII from plasma is used to treat factor VII deficiency. Either fresh frozen plasma or factor VII concentrate can be used. We experienced a case of intraventricular hemorrhage in a newborn infant who had been diagnosed as congenital factor VII deficiency. So, we report the case with a review of literatures.
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KEYWORD
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Congenital factor VII deficiency, Intraventricular hemorrhage, Newborn infant
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