Long-Term Outcomes of Living Donor Liver Transplantation in Children With Alagille Syndrome: Results From Vanguard Multicenter Study of International Living Donor Liver Transplantation Group

Abstract

Background The morbidity and mortality after living donor liver transplantation (LDLT) in children with Alagille syndrome (AGS) are complex because of the multisystem involvement of the disease. Evidence on long-term outcomes and donor selection remains limited.Methods This multicenter retrospective study included 49 pediatric patients with AGS who underwent LDLT between 2001 and 2020 at eight institutions in Japan, Korea, and Turkey. Clinical characteristics, transplant indications, donor selection, posttransplant growth, renal function, and complications were analyzed.Results The median age at LDLT was 1.1 years. Major indications for transplantation were severe growth failure (91.8%) and advanced liver disease with hepatic dysfunction. Based on donor evaluations, 11% of candidates were excluded due to genetic or anatomical concerns. The 5-year patient and graft survival rates were 93.7% and 91.5%, respectively. LDLT before 2 years of age was associated with significantly greater catch-up in height and weight. Long-term renal deterioration was observed, particularly beyond 15 years after transplantation, and older age at LDLT was an independent predictor of lower long-term estimated glomerular filtration rate. Cardiac anomalies were manageable with appropriate preoperative evaluation, whereas vasculopathies were observed after adolescence. A history of Kasai portoenterostomy did not significantly affect posttransplant outcomes.Conclusions LDLT provides excellent short- and long-term outcomes in pediatric AGS. Timely LT may improve growth and preserve renal function before progressive multisystem complications become established. Long-term surveillance and comprehensive donor evaluation remain essential.