Intracranial hemorrhage in congenital deficiency of factor XIII.

Abstract

We describe a male infant with congenital deficiency of coagulation Factor XIII who presented in the immediate postnatal period with umbilical stump bleeding and suffered a severe intracranial hemorrhage at 2 months of age. Factor XIII, also known as "fibrin-stabilizing factor," is a transpeptidase that produces strong covalent bonds between soluble fibrin monomers formed during coagulation. Presumptive diagnosis of Factor XIII deficiency was made with a clot solubility screening test, and confirmation was accomplished by demonstrating the absence of cross-linked fibrin chains by electrophoresis. This patient had received replacement therapy for 2 years, initially with intravenous fresh frozen plasma, and recently with Fibrogammin (Hoechst-Roussel Pharmaceuticals), a European Factor XIII concentrate soon to be available in the United States. Factor XIII deficiency is associated with a high incidence of life-threatening complications, notably intracranial hemorrhage. In light of the long half-life of this factor and the relatively low risk associated with new Factor XIII concentrates, such as Fibrogammin, prophylactic life-long replacement therapy should be considered for patients with severe Factor XIII deficiency.

• Authors: S L Abbondanzo, J E Gootenberg, R S Lofts, R A McPherson
• Journal: The American journal of pediatric hematology/oncology (Am J Pediatr Hematol Oncol) Vol. 10 Issue 1 Pg. 65-8 ( 1988) ISSN: 0192-8562 [Print] United States
• PMID: 3189718 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Cerebral Hemorrhage (etiology)
• Factor XIII Deficiency (complications, therapy)
• Humans
• Infant, Newborn
• Male