Outcomes of adults who received liver transplant as young children

Abstract

Background: Patient and graft survival 20-years after pediatric liver transplantation (pLT) are excellent. In children, attainment of normal growth, education and social adaptation to be an independent adult are equally important. This is particularly relevant for children who receive liver transplant at a young age, where infantile-onset liver disease, surgery and immunosuppression can adversely affect growth and neurodevelopment. The aim of this study was to evaluate the long-term physical and psychosocial outcomes of pLT recipients with normal graft function. We coin the term 'meaningful survival'. Methods: We performed a cross-sectional study of pLT recipients who received transplants between 1985 and 2004. A 20-year evaluation of physical health (growth, renal function), mental wellbeing and social outcomes (substance abuse, adherence, education, employment) was performed. All patients included were considered to have normal graft function. Findings: Eighty-four patients met study criteria. Median age at transplantation was 1.3 years (IQR 0¢7À3¢3 years), with median duration of follow-up of 20.2 years (18¢0À23¢5). At median of 20-years, 19 patients (23%) had chronic renal dysfunction and 3 patients (4%) had a BMI of >30 (mean 20¢4). Evaluation of longterm psychosocial outcomes demonstrated 22 patients (26%) with mental health disorders. Substance abuse was lower than national average. 62 patients (74%) were in education, employment or training. Overall, only 26% of our cohort achieved a composite outcome of 'meaningful survival'. Interpretation: This is the largest reported long-term study of biopsychosocial outcomes of pLT recipients with normal liver biochemistry, with follow-up upon completion of physical growth and senior school education. Importantly, despite normal liver function, many patients did not demonstrate 'meaningful survival'. We must refocus our efforts towards better understanding the long-term outcomes of children. A 'meaningful survival' rather than mere survival should be our goal.

Factors associated with improved patient and graft survival beyond 1 year in pediatric liver transplantation

“…In addition, a reporting bias may be present resulting in only the selection of available results for analysis. Finally, we note that graft and patient survival are not comprehensive measures, and registry studies have historically not included patient‐reported outcomes that define a more “meaningful survival.” [ 20 ] However, we also note that our report highlights opportunities for future efforts that could better inform the community and further improve long‐term outcomes. For one, it is notable that the percentage of living donor LTs (LDLTs) did not increase between eras.…”

Liver Transplantation for Tyrosinemia Type 1 in the Developing World: Is It Really the Best We Can Offer?: Authors’ Reply

“…We agree that it is an alternative, but do not agree that it is either entirely safe or definitive. We have thus argued in our response that LT is not as safe as medical therapy and comes with its own set of problems in the long term [3,4]. We also need to remember that the reported experience is from a single center and only of 9 patients, thereby limiting its generalizability across the country [5].…”

Liver disease in adolescents

“…The ability to return to a near normal life is an important gauge for the success of a medical intervention. We recently showed that only a quarter of our adolescent and young adult cohort (26%) achieved a composite outcome of ‘meaningful survival’ 20 years after liver transplantation52 (table 2).…”