Health status of children alive 10 years after pediatric liver transplantation performed in the US and Canada: Report of the studies of pediatric liver transplantation experience

Objectives: To determine clinical and health-related quality of life outcomes, and to derive an "ideal" composite profile of children alive 10 years after pediatric liver transplantation (LT) performed in the US and Canada. Study design: This was a multicenter cross-sectional analysis characterizing patients enrolled in the Studies of Pediatric Liver Transplantation database registry who have survived >10 years from LT. Results: A total of 167 10-year survivors were identified, all of whom received daily immunosuppression therapy. Comorbidities associated with the post-LT course included post-transplantation lymphoproliferative disease (in 5% of patients), renal dysfunction (9%), and impaired linear growth (23%). Health-related quality of life, as assessed by the PedsQL 4.0 Generic Core Scales, revealed lower patient self-reported total scale scores for 10-year survivors compared with matched healthy children (77.2 ± 12.9 vs 84.9 ± 11.7; P <.001). At 10 years post-LT, only 32% of patients achieved an ideal profile of a first allograft stable on immunosuppression monotherapy, normal growth, and absence of common immunosuppression-induced sequelae. Conclusion: Success after pediatric LT has moved beyond patient survival. Availability of an ideal composite profile at follow-up provides opportunities for patients, families, and healthcare providers to identify broader sets of outcomes at earlier stages, ultimately contributing to improved outcomes after pediatric LT.