Dietary Intake and Hedonic Preferences for Sodium in Children, Adolescents and Young Adults with Barth Syndrome

Individuals with Barth syndrome (BTHS) present with decreased activity tolerance and high fatigability. Recent evidence suggests that differences in metabolic function in BTHS negatively impact the production of aerobic energy for activity and maintenance of muscle mass. Recommendations on a metabolically beneficial diet are complicated by selective eating behaviors often seen in individuals with BTHS. While known to be selective eaters with a preference for salty foods, there is limited evidence documenting sodium liking or discrimination in the BTHS population. The purpose of the current study was to 1) Quantify and compare food 'liking' across six food categories, 2) Quantify and compare the frequency with which food was eaten in each category in males with and without BTHS, and 3) Conduct a taste test to compare ratings of hedonic liking and intensity of sodium infused liquids and compare the results between males with and without BTHS.