Journal of the Korean Pediatric Society 2000;43(3):428-431.
Published online March 15, 2000.
A Case of Factor Ⅶ Deficiency Presenting as Hemarthroses
Han Seong Ko, Kee Hwan Yoo, Kwang Chul Lee, Young Sook Hong, Joo Won Lee, Soon Kyum Kim
Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea
혈관절증으로 발현된 Factor Ⅶ 결핍증 1례
고한성, 유기환, 이광철, 홍영숙, 이주원, 김순겸
고려대학교 의과대학 소아과학교실
Abstract
Factor Ⅶ deficiency has an estimated incidence of 1/500,000 in the general population and autosomal recessive pattern of inheritance. Factor Ⅶ 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 Ⅶ assay. The clinical features are variable and do not always correlate with the Factor Ⅶ level. We experienced a case of Factor Ⅶ 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 Ⅶ. So, we report a case of Factor Ⅶ deficiency with a brief review of the related literature.
Key Words: Factor Ⅶ deficiency, Hemarthroses


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