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Energy expenditure and genotype of children with cystic fibrosis.
Pediatr Res 1994; 35(4 Pt 1):451-60PR

Abstract

Increased energy expenditure, poor dietary intake, and fat malabsorption in patients with cystic fibrosis (CF) frequently lead to growth failure and malnutrition, which are associated with pulmonary failure and decreased survival. The study purpose was to understand better the energy expenditure and requirements in the mild pulmonary disease state in children. Resting and total energy expenditure were measured in 6- to 9-yr-old, pancreatic-insufficient children with CF (n = 25) and control children (n = 25) of similar age, gender, and weight. The effect of the most common genotype, homozygous delta F508, on energy expenditure was also investigated. Dietary intake, degree of fat malabsorption, body composition, physical activity, and clinical status were determined. The CF group had a 9% increase in resting energy expenditure, which was not related to genotype or severity of lung disease. Both CF genotype subgroups (delta F508 homozygous and all others) had a similar, modest resting energy expenditure increase. Total energy expenditure was increased by 12% in the entire CF group and by 23% in the delta F508 homozygous CF subgroup compared with controls. The total energy expenditure increase in delta F508 homozygous children may be related to increased voluntary physical activity, reflecting no activity reduction associated with lung disease, or to an unidentified genotype-related mechanism. The clinical implication is that a detailed physical activity assessment should be evaluated along with resting energy expenditure, either measured or estimated by equations, when daily energy needs are being determined for children with CF.

Authors+Show Affiliations

Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.
Review

Language

eng

PubMed ID

7519343

Citation

Tomezsko, J L., et al. "Energy Expenditure and Genotype of Children With Cystic Fibrosis." Pediatric Research, vol. 35, no. 4 Pt 1, 1994, pp. 451-60.
Tomezsko JL, Stallings VA, Kawchak DA, et al. Energy expenditure and genotype of children with cystic fibrosis. Pediatr Res. 1994;35(4 Pt 1):451-60.
Tomezsko, J. L., Stallings, V. A., Kawchak, D. A., Goin, J. E., Diamond, G., & Scanlin, T. F. (1994). Energy expenditure and genotype of children with cystic fibrosis. Pediatric Research, 35(4 Pt 1), pp. 451-60.
Tomezsko JL, et al. Energy Expenditure and Genotype of Children With Cystic Fibrosis. Pediatr Res. 1994;35(4 Pt 1):451-60. PubMed PMID: 7519343.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Energy expenditure and genotype of children with cystic fibrosis. AU - Tomezsko,J L, AU - Stallings,V A, AU - Kawchak,D A, AU - Goin,J E, AU - Diamond,G, AU - Scanlin,T F, PY - 1994/4/1/pubmed PY - 1994/4/1/medline PY - 1994/4/1/entrez SP - 451 EP - 60 JF - Pediatric research JO - Pediatr. Res. VL - 35 IS - 4 Pt 1 N2 - Increased energy expenditure, poor dietary intake, and fat malabsorption in patients with cystic fibrosis (CF) frequently lead to growth failure and malnutrition, which are associated with pulmonary failure and decreased survival. The study purpose was to understand better the energy expenditure and requirements in the mild pulmonary disease state in children. Resting and total energy expenditure were measured in 6- to 9-yr-old, pancreatic-insufficient children with CF (n = 25) and control children (n = 25) of similar age, gender, and weight. The effect of the most common genotype, homozygous delta F508, on energy expenditure was also investigated. Dietary intake, degree of fat malabsorption, body composition, physical activity, and clinical status were determined. The CF group had a 9% increase in resting energy expenditure, which was not related to genotype or severity of lung disease. Both CF genotype subgroups (delta F508 homozygous and all others) had a similar, modest resting energy expenditure increase. Total energy expenditure was increased by 12% in the entire CF group and by 23% in the delta F508 homozygous CF subgroup compared with controls. The total energy expenditure increase in delta F508 homozygous children may be related to increased voluntary physical activity, reflecting no activity reduction associated with lung disease, or to an unidentified genotype-related mechanism. The clinical implication is that a detailed physical activity assessment should be evaluated along with resting energy expenditure, either measured or estimated by equations, when daily energy needs are being determined for children with CF. SN - 0031-3998 UR - https://www.unboundmedicine.com/medline/citation/7519343/Energy_expenditure_and_genotype_of_children_with_cystic_fibrosis_ L2 - http://dx.doi.org/10.1203/00006450-199404000-00013 DB - PRIME DP - Unbound Medicine ER -