J Korean Assoc Pediatr Surg.  1999 Dec;5(2):137-140. 10.13029/jkaps.1999.5.2.137.

Spleen Rupture in Congenital Afibrinogenemia

Affiliations
  • 1Department of Surgery, Ulsan University College of Medicine, Seoul, Korea.

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.

Keyword

Congenital afibrinogenemia; Spleen rupture

MeSH Terms

Afibrinogenemia*
Fibrinogen
Hemorrhage
Humans
Male
Rupture*
Spleen*
Splenectomy
Splenic Rupture
Fibrinogen
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