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Background: 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. Methods: A non-experimental, cross-sectional design was employed which compared the BTHS group (n = 34) to age-matched controls (n = 34). A Food Behaviors and Preferences Inventory was completed by all participants. A structured Chicken Soup Taste Test was conducted to compare ratings of hedonic liking and intensity of sodium infused liquids and compare results between males with and without BTHS. Results: Males with BTHS were less likely to “like” or eat foods in the fruit, vegetable, protein, and grains & carbohydrate categories of the food inventory. Sodium liking and frequency of consumption was comparable between groups, however sodium infused foods appeared to make up a larger portion of the BTHS diet compared to controls. Results of the taste test suggested that the BTHS group had a slightly higher preference for sodium infused broths compared to the control group, but discrimination abilities were not significantly different. Conclusions: The study suggests that males with BTHS are able to discriminate sodium levels accurately and have a slightly higher hedonic preference for sodium infused foods compared to their age-matched peers. More research is needed to determine the health implications of a sodium rich diet for individuals with BTHS.

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