Variable dietary management of methylmalonic acidemia: metabolic and energetic correlations.

ABSTRACT:

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; 15 males, 14 females; age range: 2-35 y) underwent evaluation. REE was measured with open-circuit calorimetry and compared with predicted values by using age-appropriate equations.

Results

Nutritional regimens were as follows: protein restriction with medica

SUBMITTER: Hauser NS 

PROVIDER: S-EPMC3001598 | biostudies-literature | 2011 Jan

REPOSITORIES: biostudies-literature