Orthotopic liver transplantation for children with Alagille syndrome.
AGS is an inherited disorder involving the liver, heart, eyes, face, and skeleton.


OBJECTIVE
To determine the outcome of LT in children with AGS compared to those with BA.


METHODS
Children with AGS and BA who had a LT between 10/1987 and 5/2008 were identified from the UNOS database.


RESULTS
Of 11 467 children who received a liver transplant, 461 (4.0%) had AGS and 3056 (26.7%) had BA. One- and five-yr patient survival was significantly lower in patients with AGS in comparison with patients with BA (AGS; 82.9%, 78.4%, BA; 89.9%, 84%, respectively). Early death (<30 days from transplant) was significantly higher in AGS than in BA. One- and five-yr graft survival was significantly lower in AGS than in BA (AGS; 74.7%, 61.5%, BA; 81.6%, 70.0%, respectively). Death from graft failure, neurological, and cardiac complications was significantly higher in patients with AGS than in patients with BA. Serum creatinine at transplant, prior LT, and cold ischemic time >12 h were identified as risk factors for death.


CONCLUSIONS
Children with AGS were older at the time of LT and their one- and five-yr patient and graft survival were significantly lower compared to BA. Risk factors for poor outcome in AGS after LT were identified.
Arnon
Ronen
R
Pediatrics, Mount Sinai Medical Center, New York, NY 10029, USA. Ronen.Arnon@mountsinai.org
Annunziato
Rachel
R
Miloh
Tamir
T
Suchy
Frederick
F
Sakworawich
Arnond
A
Sogawa
Hiroshi
H
Hiroshi
Sogawa
S
Kishore
Iyer
I
Kerkar
Nanda
N
D000293:Adolescent; D016738:Alagille Syndrome / Q000601:surgery*; D001656:Biliary Atresia; D002648:Child; D002675:Child, Preschool; D005260:Female; D006085:Graft Survival; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D008297:Male; D012307:Risk Factors