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
|