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Resting energy expenditure in infants with cystic fibrosis
J P Girardet1, P Tounian, A Sardet
1Department of Pediatric Gastroenterology and Nutrition, Armand-Trousseau Pediatric Teaching Hospital, Paris, France.
Insights
Infants with cystic fibrosis have higher resting energy expenditure, indicating a metabolic disorder. Early nutritional therapy is crucial for managing this condition in children with cystic fibrosis.
Area of Science:
- Pediatrics
- Metabolic Disorders
- Respiratory Medicine
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, often leading to energy imbalance.
- Understanding energy expenditure in infants with CF is critical for early intervention.
- Previous research has not fully elucidated the resting energy expenditure in asymptomatic infants with CF.
Purpose of the Study:
- To quantify resting energy expenditure (REE) in infants with cystic fibrosis.
- To compare REE in infants with CF to healthy controls.
- To investigate the relationship between REE and body composition in infants with CF.
Main Methods:
- Resting energy expenditure was measured using open-circuit indirect calorimetry.
- Eight infants with cystic fibrosis (age 2-7 months) without overt lung disease were studied.
- Ten healthy age-matched infants served as controls.
Main Results:
- Infants with cystic fibrosis showed a 26% higher resting energy expenditure per kilogram of fat-free mass compared to controls.
- A 32% increase in resting energy expenditure was observed in CF patients relative to predicted values for fat-free mass.
- Significant linear correlations between REE and body weight/fat-free mass were found in both groups.
Conclusions:
- The findings suggest a constitutional metabolic disorder in cystic fibrosis, even in the absence of clinical symptoms.
- Elevated resting energy expenditure in infants with CF highlights the need for early nutritional support.
- Early nutritional therapy is recommended for infants diagnosed with cystic fibrosis to address energy imbalance.
Abstract:
To evaluate the contribution of energy expenditure to the energy imbalance seen in cystic fibrosis patients, resting energy expenditure was measured using open-circuit indirect calorimetry in eight infants with cystic fibrosis, aged 2-7 months (mean, 4), without overt lung disease and in 10 healthy age-matched controls. In both groups, we found close, significant, linear correlations between resting energy expenditure and body weight and between resting energy expenditure and fat-free mass as measured by anthropometry. Cystic fibrosis patients had a 26% increase in resting energy expenditure per kilogram of fat-free mass as compared with controls and a 32% increase in resting energy expenditure as compared with predicted values for fat-free mass. These data from young infants free of clinical symptoms suggest a constitutional metabolic disorder in cystic fibrosis and support the need for early nutritional therapy in cystic fibrosis patients.