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.
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.
Authors: Miguel Silva Vieira; Christopher J Arthurs; Tarique Hussain; Reza Razavi; Carlos Alberto Figueroa Journal: PLoS One Date: 2018-11-08 Impact factor: 3.240