Liver transplant for congenital factor VII deficiency.
| Autor: | Acquazzino MA; Pediatric Hematology/Oncology/BMT, Medical College of Wisconsin, Milwaukee, Wisconsin., Rush ET, Quiros Tejeira RE, Beck JC |
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| Jazyk: | angličtina |
| Zdroj: | Pediatric blood & cancer [Pediatr Blood Cancer] 2014 Oct; Vol. 61 (10), pp. 1886-7. Date of Electronic Publication: 2014 Feb 28. |
| DOI: | 10.1002/pbc.25008 |
| Abstrakt: | 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. (© 2014 Wiley Periodicals, Inc.) |
| Databáze: | MEDLINE |
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