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A Case of Congenital Hypoprothrombinemia

Journal of the Korean Pediatric Society 1994;37(3):422-429.
Published online March 15, 1994.
A Case of Congenital Hypoprothrombinemia
Jong Kwang Lee, Hae Won Cheon, Jung Hwa Lee, Kwang Chul Lee, Soon Kyum Kim
Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea
선천성 hypoprothrombinemia 1례
이종광, 전혜원, 이정화, 이광철, 김순겸
고려대학교 의과대학 소아과학교실
Abstract
Congenital hypoprothrombinemia is a rare congenital coagulation defect. The clinical signs are manifestation of generalized bleeding tendency such as; mucosal bleeding, hypermenorrhea and post tooth extraction hemorrage. It is associated with prolongation of PT and PTT with normal thrombin time and decreased serum prothrombin level. A case with congenital hypoprothrombinemia was experienced by the authors. A 36 days old male baby was admitted with palor skin and vomiting started one day before admission. Right side brain parenchymal hemorrhage and left shift of lateral ventricle were on brain CT scan. Prolongation of prothrombin time and partial prothrombin time with decreased serum prothrombin level were resulted. Serum factor I, V, VII, VIII, IX and X were within normal level. We report a case congenital hypoprothrombinemia with a brief review of related literatures.
Key Words: Congenital hypoprothrombinemia


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