J Korean Pediatr Soc.
2000 Mar;43(3):428-431.
A Case of Factor Vll Deficiency Presenting as Hemarthroses
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- 1Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea.
Abstract
- Factor Vll deficiency has an estimated incidence of 1/500,000 in the general population and autosomal recessive pattern of inheritance. Factor Vll deficiency is characterized by prolonged prothrombin time (PT), and normal activated partial thromboplastin time (aPTT) and bleeding time (BT). Definite diagnosis of this condition requires a specific Factor Vll assay. The clinical features are variable and do not always correlate with the Factor Vll level. We experienced a case of Factor Vll deficiency presenting as hemarthroses in a 2-years-old girl, whose chief complaint was pain, swelling of right knee joint and limping gait. The laboratory findings were prolonged PT and prominent deficiency of factor Vll. So, we report a case of Factor Vll deficiency with a brief review of the related literature.
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