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Determinants of Quality of Life in Children with Inborn Errors of Metabolism Receiving a Restricted Diet

2021; Elsevier BV; Volume: 242; Linguagem: Inglês

10.1016/j.jpeds.2021.11.021

1097-6833

Abdoulaye Ouattara, Noémie Resseguier, Aline Cano, Pascale de Lonlay, Jean‐Baptiste Arnoux, Anaïs Brassier, Manuel Schiff, Samia Pichard, Alexandre Fabre, Célia Hoebeke, Nathalie Guffon, Alain Fouilhoux, Pierre Broué, Guy Touati, Dries Dobbelaere, Karine Mention, François Labarthe, M. Tardieu, L de Parscau, François Feillet, C. Bonnemains, Alice Kuster, Philippe Labrune, Magalie Barth, Léna Damaj, Delphine Lamireau, Julie Berbis, Pascal Auquier, B. Chabrol,

Childhood Cancer Survivors' Quality of Life

ObjectiveTo investigate the determinants of quality of life (QoL) in children with inborn errors of metabolism with restricted diet (IEMRDs) using a single theory-based multidimensional model.Study designIn this multicenter cross-sectional study, data from children aged 8-17 years with IEMRDs (except phenylketonuria) and their parents were collected from January 2015 to December 2017. Measurements included a child's self-reported QoL, self-rated behavioral problems and anxiety, and parental anxiety. Based on hypotheses from a literature-built theoretical model linking demographic, clinical, family environment, and psychosocial characteristics to QoL either directly or indirectly, associations of these factors with a child's self-rated QoL were examined using a structural equation modeling approach.ResultsA total of 312 children (mean [SD] age, 12.2 [2.6] years; 51% boys [n = 160]) were included. Higher levels of trait anxiety and behavioral problems in children were the most important factors associated with poorer QoL (standardized path coefficients, −0.71 and −0.23, respectively). In addition, higher parent trait anxiety, younger age at diagnosis, and a disease requiring an emergency diet were associated with poorer QoL in these children. The final model fit the data closely according to conventional goodness-of-fit statistics and explained 86% of the QoL variance.ConclusionsPsychosocial factors appear to be major determinants of QoL impairment in children with IEMRDs. These factors should be addressed in clinical practice as part of the global treatment plan for a child with IEMRD. Future studies based on a longitudinal design should consider coping strategies when exploring potential predictive factors of QoL.