Energy expenditure in patients with propionic and methylmalonic acidaemias

C C van Hagen; E Carbasius Weber; Th A M van den Hurk; J H Oudshoorn; L Dorland; R Berger; T de Koning

doi:10.1023/b:boli.0000016678.78134.7d

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.

Authors

C C van Hagen¹, E Carbasius Weber, Th A M van den Hurk, J H Oudshoorn, L Dorland, R Berger, T de Koning

Affiliation

¹ Department of Metabolic Diseases, University Medical Centre Utrecht, The Netherlands.

PMID: 15065575
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