Orthotopic liver transplantation for adults with Alagille syndrome.
BACKGROUND
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).


OBJECTIVE
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).


CONCLUSIONS
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.
Department of Pediatrics, Mount Sinai School of Medicine, New York, NY, USA. ronen.arnon@ mountsinai.org
Ronen
R
Arnon
Rachel
R
Annunziato
Thomas
T
Schiano
Tamir
T
Miloh
Margaret
M
Baisley
Hiroshi
H
Sogawa
Alan G
AG
Contreras
Samantha
S
Lee
Nanda
N
Kerkar
D000328:Adult; D016738:Alagille Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D001656:Biliary Atresia / Q000150:complications / Q000601:surgery; D002675:Child, Preschool; D005260:Female; D006085:Graft Survival; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D015996:Survival Rate