Orthotopic liver transplantation for children with Alagille syndrome

Ronen Arnon, Rachel Annunziato, Tamir Miloh, Frederick Suchy, Arnond Sakworawich, Hiroshi Sogawa, Sogawa Hiroshi, Iyer Kishore, Nanda Kerkar
Pediatric Transplantation 2010, 14 (5): 622-8

UNLABELLED: AGS is an inherited disorder involving the liver, heart, eyes, face, and skeleton.

AIM: 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.

CONCLUSION: 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.

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