RESTING ENERGY-EXPENDITURE IN INFANTS WITH CYSTIC-FIBROSIS

To evaluate the contribution of energy expenditure to the energy imbal ance 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 diseas e and in 10 healthy age-matched controls. In both groups, we found clo se, significant, linear correlations between resting energy expenditur e and body weight and between resting energy expenditure and fat-mass as measured by anthropometry. Cystic fibrosis patients had a 26% incre ase in resting energy expenditure per kilogram of fat-free mass as com pared with controls and 32% increase in resting energy expenditure as compared with predicted values for fat-free mass. These data from youn g infants free of clinical symptoms suggest a constitutional metabolic disorder in cystic fibrosis and support the need for early nutritiona l therapy cystic fibrosis patients.