| A Case of Factor Ⅶ Deficiency Presenting as Hemarthroses |
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Han Seong Ko, Kee Hwan Yoo, Kwang Chul Lee, Young Sook Hong, Joo Won Lee, Soon Kyum Kim |
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Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea |
| 혈관절증으로 발현된 Factor Ⅶ 결핍증 1례 |
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고한성, 유기환, 이광철, 홍영숙, 이주원, 김순겸 |
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고려대학교 의과대학 소아과학교실 |
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| Abstract |
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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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