Developmental and functional outcomes in children with chronic liver disease undergoing liver transplant: a longitudinal cohort study

Abstract

Background: Chronic liver disease has multiple negative implications and has been shown to result in impairments in neurodevelopment and health-related quality of life. Minimal research has been conducted in a South African context on the effects of liver disease on neurodevelopment and health-related quality of life in children undergoing liver transplantation. Objectives: To determine neurodevelopment and health-related quality of life both pre- and post- liver transplantation in children with chronic liver disease under the age of five years undergoing liver transplant and to evaluate change over time. Methods: Neurodevelopmental assessment using the Ages and Stages Questionnaire and health-related quality of life assessment using the Paediatric Quality of Life Inventory Scale were conducted pre-liver transplant and at three month post-liver transplant in ten children with chronic liver disease under the age of five years. Functional status was determined using the Functional Status Score and evaluated pre-operatively, immediately post-operatively, at ICU and hospital discharge and at a three month follow up. Comparisons and change over time were determined for each participant. Results: Ten children, with chronic liver disease, under the age of five years undergoing liver transplant were assessed. Of that, three were at risk for delay in communication, five showed delays in gross motor skills, four showed delays in fine motor skills, four showed delays in problem solving skills and one showed delay in personal social skills on pre-operative assessment. Post operatively, two showed delays in communication skills, four showed delays in gross motor skills, three showed delays in fine motor skills, two showed delays in problem solving skills and two showed delays in personal social skills. Lower scores were shown in health-related quality of life physical health scores pre-operatively. Post-operatively three children had a reduced total score on Paediatric Quality of Life Inventory Scale compared to pre- operative scores and four showed improvement in overall health-related quality of life post-transplant. Conclusion: Chronic liver disease can result in impairments in neurodevelopment and health-related quality of life in children under five years of age. Despite liver transplantation, some children still presented with delay in neurodevelopment post- operatively and showed a reduction in health-related quality of life at three months post-transplant. Improvement in health-related quality of life was seen in the majority of participants post-liver transplant.