Journal of the Korean Pediatric Society 1998;41(12):1726-1730.
Published online December 15, 1998.
A Case of Congenital Factor Ⅶ Deficiency Associated with Intraventricular Hemorrhage and Hydrocephalus
Hyoung Jun Kim, Jin Hwa Jung, Jeong Ho Lee, Jong Dae Jo
Department of Pediatrics, Maryknoll Hospital, Pusan, Korea
뇌실내 출혈과 수두증을 동반한 선천성 Ⅶ인자 결핍증 1례
김형준, 정진화, 이정호, 조종대
부산 메리놀병원 소아과
Hyoung Jun Kim, Email: 1
Congenital factor Ⅶ deficiency is a rare bleeding disorder with an estimated incidence of 1 in 500,000. It is inherited as an autosomal recessive pattern with variable expression and high penetrance. In severely affected patients, repeated hemarthroses, chronic crippling hemarthropathy, and dangerous hematomas can occur. Other types of hemorrhage include epistaxis, menorrhagia, hematuria, gastrointestinal and gingival bleeding. Fetal cerebral hemorrhage has been reported, although less frequently than in severe hemophilia A or B. It is characterized by normal partial thromboplastin time and prolonged prothrombin time. Definitive diagnosis rests on a specific assay for factor Ⅶ clotting activity. Replacement therapy is necessary to control the hemorrhage. Conventional prophylaxis and therapy in this disorder have consisted of fresh frozen plasma(FFP) or prothrombin complex concentrate. We experienced a case of intraventricular hemorrhage and hydrocephalus in a 4-year-old girl who had been diagnosed with congenital factor Ⅶ deficiency during her neonatal period. She presented with episodes of frontal headache, frequent vomiting and malnutrition. We report this case with a brief review and related literatures.
Key Words: Congenital factor Ⅶ deficiency, Intraventricular hemorrhage, Hydrocephalus

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