Liver transplant for congenital factor VII deficiency

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 sev...

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Bibliographic Details
Published in:Pediatric blood & cancer 2014-10, Vol.61 (10), p.1886-1887
Main Authors: Acquazzino, Melissa A., Rush, Eric T., Quiros Tejeira, Ruben E., Beck, Jill C.
Format: Article
Language:English
Subjects:
Child, Preschool
factor VII deficiency
Factor VII Deficiency - complications
Factor VII Deficiency - surgery
Hematology
Humans
Infant
Intracranial Hemorrhages - etiology
liver transplant
Liver Transplantation
Male
Oncology
Pediatrics
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Summary: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.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.25008