Spontaneous splenic rupture in a patient with congenital afibrinogenemia

Arcagök B. C., Özdemir N., Tekin A., ÖZCAN R., ELİÇEVİK M., Şenyüz O. F., ...More

Turk Pediatri Arsivi, vol.49, no.3, pp.247-249, 2014 (SCI-Expanded, ESCI, Scopus, TRDizin) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 49 Issue: 3
  • Publication Date: 2014
  • Doi Number: 10.5152/tpa.2014.1070
  • Journal Name: Turk Pediatri Arsivi
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.247-249
  • Istanbul University-Cerrahpasa Affiliated: No

Abstract

© 2014 by Turkish Pediatric Association.Afibrinogenemia is a rare bleeding disorder which is observed with an incidence of 1:1 000 000. It is an autosomal recessive disease and occurs as a result of mutation in one of the three genes which code the three polypeptide chains of fibrinogen. Basic clinical findings include spontaneous bleeding, bleeding after minor trauma or due to surgery. Splenic rupture in afibrinogenemia has been reported only in 6 cases so far. In this article, we present a 15-year old congenital afibrinogenemia patient with spontaneous splenic rupture.