Purpose Medical foods for methylmalonic acidemias (MMAs) and propionic acidemias
contain minimal valine, isoleucine, methionine, and threonine but have been formulated with
increased leucine. We aimed to assess the effects of imbalanced branched-chain amino
acid intake on metabolic and growth parameters in a cohort of patients with MMA
ascertained via a natural history study. Methods Cross-sectional anthropometric and body-
composition measurements were correlated with diet content and disease-related …