Health-related quality of life in pre-adolescent liver transplant recipients with biliary atresia: A cross-sectional study

Objective: Pediatric recipients of liver transplantation (LT) often report lower Health-Related Quality of Life (HRQOL) than healthy controls when assessed on generic HRQOL measurement tools. The recent addition of the Pediatric Liver Transplant Quality of Life (PeLTQL), a novel disease-specific HRQOL instrument for pediatric LT recipients, into the clinical armamentarium of tools now routinely available to clinical care teams, provides the unique opportunity to identify disease-related challenges in children who have undergone this life-saving intervention. This study assesses HRQOL in pre-adolescent aged patients with a primary diagnosis of biliary atresia (BA) who underwent LT as an infant, using both generic and disease-specific HRQOL instruments validated for children. We also examined modifiable factors associated with HRQOL after pediatric LT. Methods: HRQOL was the primary outcome of this study assessed using the disease-specific PeLTQL and the generic Pediatric Quality of Life Inventory 4.0 (PedsQL). Exposure variablesof interest included medication status (e.g., monotherapy, dual therapy) and participation insports. Results: A total of 70 (56% female, mean age 9.89 +/- 1.25 years) pediatric LT recipients (meaninterval since LT was 9.0 +/- 1.26 years) comprised the study cohort. LT recipients reported significantly lower PedsQL Scores relative to the general population. Immunosuppression monotherapywas associated with higher patient-reported PeLTQL Scores, and sports participation was associated with higher parent-reported PedsQL Scores. Conclusions: Pre-adolescents who underwent LT as an infant with BA, self-report low HRQOL onboth disease-specific and generic HRQOL tools. Further research targeting sports participationand simplifying immunosuppression may further optimize quality of life years restored by lifesaving LT. (C) 2018 Elsevier Masson SAS. All rights reserved.