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Liver transplant for congenital factor VII deficiency
Author(s) -
Acquazzino Melissa A.,
Rush Eric T.,
Quiros Tejeira Ruben E.,
Beck Jill C.
Publication year - 2014
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.25008
Subject(s) - medicine , pediatrics , liver transplantation , oncology , transplantation
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. Pediatr Blood Cancer 2014; 61:1886–1887. © 2014 Wiley Periodicals, Inc.