Psychosocial factors, including higher anxiety levels and behavioral problems, significantly contributed to poorer quality of life (QoL) in children with inborn errors of metabolism such as long chain fatty acid oxidation disorder (LCFAOD).

Diseases involving emergency restricted diets, higher parental anxiety, and diagnoses at younger ages also decreased QoL scores in these children, as published online in the Journal of Pediatrics.

Researchers conducted a multicenter cross-sectional study in France that analyzed data from 312 children between the ages of 8 and 17 years with inborn errors of metabolism on restricted diets from January 2015 to December 2017.

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The investigators excluded children with phenylketonuria. Boys (n=160) comprised over half (51%) of the children in this study, and the average age of all children was 12.2 ± 2.6 years.

Read more about LCFAOD comorbidities

Primary outcomes of the study included self-reported QoL, self-rated behavioral problems and anxiety, and parental anxiety. The researchers analyzed the relationships between these factors and how they ultimately influenced QoL by using a structural equation modeling approach.

This analysis indicated that higher anxiety levels (standardized path coefficient: -0.71) and behavioral problems (standardized path coefficient: -0.23) promoted worse self-reported QoL scores in these children. The investigators observed that the final model, which aligned best with the data, explained 86% of the variances in QoL.

Based on the results of their study, the authors strongly advocated for addressing the psychosocial elements of children with inborn errors of metabolism as part of a global treatment plan.

“Future studies based on a longitudinal design should consider coping strategies when exploring potential predictive factors of quality of life,” they concluded.


Ouattara A, Resseguier N, Cano A, et al. Determinants of quality of life in children with inborn errors of metabolism receiving a restricted diet. J Pediatr. Published online November 14, 2021. doi:10.1016/j.jpeds.2021.11.021