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Orthotopic liver transplantation for adults with Alagille syndrome

Authors


  • Conflict of interest: None.

Corresponding author: Ronen Arnon, MD, One Gustave L. Levy Place, Box 1104, New York, NY 10029.
Tel.: 212 659 8060; fax: 212 241 2064; e-mail: ronen.arnon@ mountsinai.org

Abstract

Arnon R, Annunziato R, Schiano T, Miloh T, Baisley M, Sogawa H, Contreras AG, Lee S, Kerkar N. Orthotopic liver transplantation for adults with Alagille syndrome.
Clin Transplant 2011 DOI: 10.1111/j.1399-0012.2011.01574.x.
© 2011 John Wiley & Sons A/S.

Abstract:  Introduction:  Alagille syndrome (AGS) is an inherited multisystem disorder, and liver transplantation (LT) may be required in pediatric patients with AGS (P-AGS). There are limited data regarding the outcomes of LT in adults with AGS (A-AGS).

Aim:  To determine and compare the outcomes of LT in A-AGS vs. P-AGS as well as A-AGS vs. adults with biliary atresia (A-BA).

Methods:  Adults (>18 yr), with AGS and BA, and children (≤18 yr), with AGS who underwent isolated first LT between 10/1987 and 5/2008, were identified from the UNOS database.

Results:  Forty-four of 79 400 adults transplanted for AGS were compared with 407 P-AGS and 56 A-BA, respectively. A-AGS patients had a significantly higher rate of encephalopathy, lower serum albumin, and higher serum creatinine in comparison with P-AGS. One- and five-yr patient and graft survival in A-AGS who underwent LT were not significantly different in comparison with either P-AGS or A-BA (A-AGS patient survival: 95.5%, 90.9%, P-AGS: 88. 7%, 86.2%, A-BA: 89.3%, 87.5%; A-AGS graft survival: 84.1%, 79. 5%, P-AGS: 80.3%, 76%. 1%, A-BA: 82.1%, 78.6%, respectively).

Conclusion:  The outcome of first LT in A-AGS is excellent compared with the overall reported adult patient and graft survival. Although A-AGS were sicker than P-AGS at transplant, their outcomes were comparable with that of P-AGS.

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