Liver transplant for congenital factor VII deficiency. Issue 10 (28th February 2014)

• Record Type: Journal Article
• Authors: Acquazzino, Melissa A.; Rush, Eric T.; Quiros Tejeira, Ruben E.; Beck, Jill C.
• Is Part Of: Pediatric blood & cancer. Volume 61:Issue 10(2014:Oct.)
• Page Start: 1886
• Page End: 1887
• Publication Date: 2014-02-28
• Subjects: Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.25008
• Languages: English
• ISSNs: 1545-5009

Abstract

<abstract abstract-type="main"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="pbc25008-sec-0001" sec-type="section"> <p>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.</p> </sec> </abstract>