[HTML][HTML] Variable dietary management of methylmalonic acidemia: metabolic and energetic correlations
NS Hauser, I Manoli, JC Graf, J Sloan… - The American journal of …, 2011 - Elsevier
Background: Isolated methylmalonic acidemia (MMA) is managed by dietary protein
restriction and medical food supplementation. Resting energy expenditure (REE) can be
depressed in affected individuals for undefined reasons. Objective: The objective was to
document the spectrum of nutritional approaches used to treat patients with MMA, measure
REE, and analyze the dependence of REE on body composition, biochemical, and
nutritional variables. Design: Twenty-nine patients with isolated MMA (22 mut, 5 cblA, 2 cblB; …
restriction and medical food supplementation. Resting energy expenditure (REE) can be
depressed in affected individuals for undefined reasons. Objective: The objective was to
document the spectrum of nutritional approaches used to treat patients with MMA, measure
REE, and analyze the dependence of REE on body composition, biochemical, and
nutritional variables. Design: Twenty-nine patients with isolated MMA (22 mut, 5 cblA, 2 cblB; …