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Orthotopic liver transplantation for adults with Alagille syndrome


  • 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@


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.