Resting and total energy expenditure of patients with long-chain fatty acid oxidation disorders (LC-FAODs).
Energy expenditure
Fatty acid oxidation disorders
Resting energy expenditure
Total energy expenditure
Journal
Molecular genetics and metabolism
ISSN: 1096-7206
Titre abrégé: Mol Genet Metab
Pays: United States
ID NLM: 9805456
Informations de publication
Date de publication:
03 2023
03 2023
Historique:
received:
21
12
2022
revised:
13
01
2023
accepted:
14
01
2023
pmc-release:
01
03
2024
pubmed:
26
1
2023
medline:
7
3
2023
entrez:
25
1
2023
Statut:
ppublish
Résumé
The basis of medical nutrition therapy for patients with LC-FAODs is to provide adequate energy to maintain anabolism and prevent catabolism. In practice, energy needs are estimated based on formulas derived from normal populations but it is unknown if energy expenditure among patients with LC-FAODs is similar to the normal population. We measured resting energy expenditure (REE), total energy expenditure (TEE) and body composition in 31 subjects with LC-FAODs ranging in age from 7 to 64 years. Measured REE was lower than estimated REE by various prediction equations and measured TEE was lower than estimated TEE. It is possible that the lower energy expenditure based on prediction formulas from the normal population is due to differences in body composition; we compared body composition to normal data from the 2017-18 National Health and Nutrition Examination Survey (NHANES). Fat free mass and fat mass was similar between subjects with an LC-FAOD and NHANES normal data suggesting no difference in body composition. We then compared measured REE and TEE to normal published data from the Dietary Reference Intakes (DRI). Measured REE and TEE were significantly lower among subjects with LC-FAODs compared to normal published energy expenditure data. Our results suggests patients with a LC-FAOD exhibit a lower REE and therefore actually have a slightly lower TEE than estimated. Current prediction equations may overestimate energy expenditure of patients with a LC-FAOD.
Identifiants
pubmed: 36696737
pii: S1096-7192(23)00149-X
doi: 10.1016/j.ymgme.2023.107519
pmc: PMC9992335
mid: NIHMS1869720
pii:
doi:
Substances chimiques
Fatty Acids
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, U.S. Gov't, P.H.S.
Langues
eng
Sous-ensembles de citation
IM
Pagination
107519Subventions
Organisme : NIDDK NIH HHS
ID : F32 DK065400
Pays : United States
Organisme : FDA HHS
ID : R01 FD003895
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR000128
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR001857
Pays : United States
Informations de copyright
Copyright © 2023 Elsevier Inc. All rights reserved.
Références
J Inherit Metab Dis. 2017 Nov;40(6):831-843
pubmed: 28871440
Br J Nutr. 2021 May 14;125(9):983-997
pubmed: 32718378
J Inherit Metab Dis. 1998 Aug;21(6):645-54
pubmed: 9762600
N Engl J Med. 1986 Jul 10;315(2):96-100
pubmed: 3724804
JAMA. 2002 Oct 9;288(14):1728-32
pubmed: 12365956
Pediatr Blood Cancer. 2012 Jan;58(1):129-34
pubmed: 22009670
J Appl Physiol Respir Environ Exerc Physiol. 1980 May;48(5):770-5
pubmed: 7451284
J Pediatr. 1997 Jan;130(1):67-76
pubmed: 9003853
Eur J Appl Physiol. 2017 Sep;117(9):1765-1785
pubmed: 28689303
J Am Diet Assoc. 2002 Nov;102(11):1621-30
pubmed: 12449285
Nutrients. 2016 Oct 25;8(11):
pubmed: 27792142
Int J Obes (Lond). 2013 Jul;37(7):1006-11
pubmed: 23090575
Obesity (Silver Spring). 2018 May;26(5):903-909
pubmed: 29687648
PLoS One. 2016 Feb 16;11(2):e0147818
pubmed: 26881790
J Inherit Metab Dis. 1999 Jun;22(4):488-502
pubmed: 10407781
Am J Clin Nutr. 2021 Nov 8;114(5):1583-1589
pubmed: 34477824
J Sports Sci. 2005 Jan;23(1):15-9
pubmed: 15841591
JAMA. 2016 Jun 7;315(21):2284-91
pubmed: 27272580
J Inherit Metab Dis. 1999 Apr;22(2):123-31
pubmed: 10234607
J Inherit Metab Dis. 2010 Oct;33(5):527-32
pubmed: 20449660
J Clin Endocrinol Metab. 2014 Nov;99(11):4307-14
pubmed: 25105736
Mol Genet Metab. 2020 Sep - Oct;131(1-2):90-97
pubmed: 32928639
J Inherit Metab Dis. 2009 Aug;32(4):498-505
pubmed: 19452263
Am J Physiol Endocrinol Metab. 2013 Nov 15;305(10):E1299-308
pubmed: 24064340
Mol Genet Metab. 2020 Sep - Oct;131(1-2):23-37
pubmed: 33093005
Metabolism. 1989 Apr;38(4):364-70
pubmed: 2657322
Mol Genet Metab. 2009 Mar;96(3):85-90
pubmed: 19157942
Mol Genet Metab. 2003 Jun;79(2):114-23
pubmed: 12809642
Hum Mol Genet. 2019 Mar 15;28(6):928-941
pubmed: 30445591