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Increased energy expenditure in young children with cystic fibrosis
R W Shepherd1, T L Holt, L Vasques-Velasquez
1Department of Gastroenterology, Royal Children's Hospital, University of Queensland, Brisbane, Australia.
Insights
Infants with cystic fibrosis (CF) exhibit significantly higher total energy expenditure (TEE) than healthy infants. This increased energy need may contribute to undernutrition in CF, even without lung disease.
Area of Science:
- Pediatrics
- Metabolism
- Genetics
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Altered energy balance and undernutrition are common in CF patients.
- The precise drivers of increased energy needs in CF remain unclear, particularly in early stages.
Purpose of the Study:
- To quantify total energy expenditure (TEE) in infants with cystic fibrosis (CF) without chronic lung disease.
- To compare TEE in CF infants with age- and weight-matched healthy controls.
- To identify factors contributing to altered energy expenditure in early-stage CF.
Main Methods:
- Total energy expenditure (TEE) was measured using the doubly-labeled water method.
- The study included 9 clinically well infants with CF and 16 healthy controls.
- Infants were matched for age and body weight for comparative analysis.
Main Results:
- CF infants demonstrated 25% higher TEE compared to healthy controls.
- Mean TEE for CF infants was 950 (38) kcal, versus 876 (72) kcal for age-matched controls and 758 (46) kcal for weight-matched controls.
- Increased TEE was observed independently of body weight, underweight status, pancreatic insufficiency, or lung disease.
Conclusions:
- Infants with CF have elevated TEE, suggesting a potential energy-requiring basic defect.
- Increased TEE may be a significant factor contributing to undernutrition in CF, even in the absence of overt lung disease.
- Further research is needed to elucidate the mechanisms behind elevated energy expenditure in CF.
Abstract:
To investigate the role of energy expenditure in the altered energy balance in cystic fibrosis (CF), total energy expenditure (TEE) was measured by the doubly-labelled water method in 9 clinically well CF infants (body weight 7.3-10.9 kg) without chronic lung disease. CF infants had 25% higher rates of energy expenditure when compared with data derived from measurements of TEE obtained by the same method in 16 healthy infants, matched for age and body weight. Mean TEE (SEM) for CF was 950 (38) kcal, vs 876 (72) kcal for controls matched for age and 758 (46) kcal for controls matched for weight. Although subclinical disease activity cannot be excluded as a determinant of the excess TEE, the possibility of an energy-requiring basic defect is suggested, because further analysis indicated that factors other than body weight, degree of underweight, presence of pancreatic insufficiency, or presence of lung disease were important. Increased TEE may contribute to undernutrition in CF, even in the absence of chronic lung disease.