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A Case of Congenital Hypofibrinogenemia.

Journal of the Korean Pediatric Society 1990;33(7):1009-1011.
Published online July 31, 1990.
A Case of Congenital Hypofibrinogenemia.
Ji In Park, In Seok Lim, Chul Ha Kim, Byoung Hoon Yoo
Department of Pediatrics, College of Medicine, Chung Ang University, Seoul, Korea
선천성 저피브리노겐혈증 1례
박지인, 임인석, 김철하, 유병훈
중앙대학교 의과대학 소아과학교실
Received: 20 February 1990   • Accepted: 24 April 1990
Abstract
Congenital hypofibrinogenemia is a rare congenital bleeding disorder, inherited by autosomal dominant or recessive trait. We experienced one case of congenital hypofibrinogenemia in 4 year-old female patient with chief complaints of headache and ecchymosis. History of continuous umbilical cord bleeding and frequent bleeding tendency against minor trauma were revealed on her past medical history. On laboratory data, bleeding time, platelet aggregation test were normal and factor assays were all within normal limit. PT, aPTT were markedly prolongated and fibrinogen level was decreased to 23 mg/dl. Subdural hematoma and communicating hydrocephalus were detected on brain CT film. We report 1 case of congenital hypofibrinogenemia with a brief review of literatures.
Key Words: Congenital hypofibrinogenemia


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