title: 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.affiliation: Pediatric Hematology/Oncology/BMT, Medical College of Wisconsin, Milwaukee, Wisconsin.
authors.forename: Melissa A
authors.initials: MA
authors.lastname: Acquazzino
authors.forename: Eric T
authors.initials: ET
authors.lastname: Rush
authors.forename: Ruben E
authors.initials: RE
authors.lastname: Quiros Tejeira
authors.forename: Jill C
authors.initials: JC
authors.lastname: Beck
mesh_terms: D002675:Child, Preschool; D005168:Factor VII Deficiency / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D020300:Intracranial Hemorrhages / Q000209:etiology; D016031:Liver Transplantation; D008297:Male
keywords: factor VII deficiency; liver transplant