Liver transplant for congenital factor VII deficiency.

Abstract

Congenital factor VII (FVII) deficiency is a rare, autosomal recessive bleeding disorder with a spectrum of phenotypes ranging from asymptomatic to life-threatening intra-cranial hemorrhage (ICH). Orthotopic liver transplantation has been described for definitive treatment in a few patients with severe manifestations. We report a patient with congenital FVII deficiency and recurrent ICH, despite twice-weekly prophylaxis with recombinant activated FVII. At 17 months of age, he underwent an orthotopic liver transplant. He is now 1-year post-transplant, on maintenance immunosuppression with no hemorrhage or other complications.

• Authors: Melissa A Acquazzino, Eric T Rush, Ruben E Quiros Tejeira, Jill C Beck
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 61 Issue 10 Pg. 1886-7 (Oct 2014) ISSN: 1545-5017 [Electronic] United States
• PMID: 24585521 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2014 Wiley Periodicals, Inc.

Topics

• Child, Preschool
• Factor VII Deficiency (complications, surgery)
• Humans
• Infant
• Intracranial Hemorrhages (etiology)
• Liver Transplantation
• Male