Energy expenditure in patients with propionic and methylmalonic acidaemias

J Inherit Metab Dis. 2004;27(1):111-2. doi: 10.1023/b:boli.0000016678.78134.7d.

Abstract

Resting energy expenditure (REE) was investigated in 8 children with propionic and methylmalonic acidaemias because a lowered REE has been reported in the literature. We observed a marginally elevated REE and think that adequate caloric intake and the use of a synthetic amino acid mixture are responsible for this.

MeSH terms

  • Amino Acid Metabolism, Inborn Errors / drug therapy
  • Amino Acid Metabolism, Inborn Errors / metabolism*
  • Amino Acids / therapeutic use
  • Child
  • Child, Preschool
  • Drug Combinations
  • Energy Intake
  • Energy Metabolism*
  • Female
  • Humans
  • Infant
  • Male
  • Methylmalonic Acid / metabolism*
  • Propionates / metabolism*

Substances

  • Amino Acids
  • Drug Combinations
  • Propionates
  • Methylmalonic Acid