Young people with biliary atresia requiring liver transplantation: a distinct population requiring specialist care.

BACKGROUND: Young people born with biliary atresia are an emerging population for adult hepatologists with 40-45% of children entering adolescence with their native liver intact. For those requiring liver transplantation during adolescence, disparity on the waiting list and post liver transplantation outcome for young adults compared to younger and older age groups has stimulated discussion regarding the optimal timing of listing. In this study we review our experience of young people with biliary atresia requiring liver transplantation during adolescence and young adulthood.

METHODS: Retrospective, single centre review of patients with biliary atresia requiring liver transplantation > 11 years.

RESULTS: Thirty-six young people (16 male) underwent liver transplantation between 1991-2014 at a median age of 16.6 (IQR 14.2,19.5) years. The commonest indications for listing were refractory cholangitis (31%), synthetic failure (25%) and variceal bleeding (14%). Patients listed by the adult team (n=14) waited longer than those listed by the paediatric team (10 (IQR 7.7,24.6) vs 5.8 (IQR 4.0,15.1) months (p<0.05)) and were more likely to require intensive care support at time of listing (29% vs 5%, p<0.05). Admission to intensive care unit at listing was associated with poorer patient and graft survival and support from a multidisciplinary liver transition service improved survival. Liver disease severity scores did not correlate with time on waiting list or outcome.

CONCLUSIONS: Young people with biliary atresia require close monitoring by specialists familiar with their condition and timing for liver transplantation needs to be fine-tuned in order to avoid clinical decompensation and improve long-term outcomes.