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Energy expenditure in patients with propionic and methylmalonic acidaemias
C C van Hagen1, E Carbasius Weber, Th A M van den Hurk
1Department of Metabolic Diseases, University Medical Centre Utrecht, The Netherlands.
Journal of Inherited Metabolic Disease
|April 7, 2004
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.