A critical reappraisal of dietary practices in methylmalonic acidemia raises concerns about the safety of medical foods. Part 1: isolated methylmalonic acidemias.
Irini ManoliJennifer G MylesJennifer L SloanOleg A ShchelochkovCharles P VendittiPublished in: Genetics in medicine : official journal of the American College of Medical Genetics (2015)
Increased leucine intake in patients with MMA resulted in iatrogenic amino acid deficiencies and was associated with adverse growth outcomes. Medical foods for propionate oxidation disorders need to be redesigned and studied prospectively to ensure efficacy and safety.Genet Med 18 4, 386-395.