Energy expenditure in patients with propionic and methylmalonic acidaemias.
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.
van Hagen
C C
CC
Department of Metabolic Diseases, University Medical Centre Utrecht, The Netherlands.
Carbasius Weber
E
E
van den Hurk
Th A M
TA
Oudshoorn
J H
JH
Dorland
L
L
Berger
R
R
de Koning
T
T
D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy / Q000378:metabolism*; D000596:Amino Acids / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004338:Drug Combinations; D002149:Energy Intake; D004734:Energy Metabolism; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008764:Methylmalonic Acid / Q000378:metabolism*; D011422:Propionates / Q000378:metabolism*
D000596:Amino Acids; D004338:Drug Combinations; D011422:Propionates; D008764:Methylmalonic Acid