Journal List > J Korean Assoc Pediatr Surg > v.5(2) > 1102627

Kim, Kim, and Kim: Spleen Rupture in Congenital Afibrinogenemia

Abstract

Congenital afibrinogenemia is a rare disorder that refers to a congenital lack of production of fibrinogen, a key component of the hemostatic system. Bleeding manifestations of congenital afibrinogenemia vary in severity from mild to catastrophic. This is a case report of splenic rupture occurred in an eight-year-old boy with congenital afibrinogenemia. Nonoperative treatment with cryoprecipitate and virally inactivated, purified fibrinogen concentrates successfully avoided splenectomy.

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