Orthotopic liver transplantation for adults with Alagille syndrome

摘要: Arnon R, Annunziato Schiano T, Miloh 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. © John Wiley & Sons A/S. Abstract:  Introduction:  syndrome (AGS) is an inherited multisystem disorder, and (LT) may be required in pediatric patients AGS (P-AGS). There are limited data regarding the outcomes of LT (A-AGS). Aim:  To determine compare A-AGS vs. P-AGS as well biliary atresia (A-BA). Methods:  Adults (>18 yr), BA, children (≤18 yr), who underwent isolated first between 10/1987 5/2008, were identified from UNOS database. Results:  Forty-four 79 400 transplanted compared 407 56 A-BA, respectively. had a significantly higher rate encephalopathy, lower serum albumin, creatinine comparison P-AGS. One- five-yr patient graft survival not different either or A-BA (A-AGS survival: 95.5%, 90.9%, P-AGS: 88. 7%, 86.2%, A-BA: 89.3%, 87.5%; 84.1%, 79. 5%, 80.3%, 76%. 1%, 82.1%, 78.6%, respectively). Conclusion:  The outcome excellent overall reported adult survival. Although sicker than at transplant, their comparable that