To explore the hypothesis that there is an increased metabolic rate in cystic fibrosis, resting energy expenditure was measured by indirect calorimetry in 23 subjects with cystic fibrosis in a stable clinical state and in 42 normal control subjects. Resting energy expenditure was found to be elevated by an average of 0.45 MJ/24 h [95% confidence interval (CI) = 0.26-0.64, t = 4.91, P less than 0.001] (108 kcal/24 h), or 9.2% above expected values derived from the regression relating resting energy expenditure to whole body weight and sex in control subjects. When related to lean body mass, values were still elevated by 0.36 MJ/24 h (95% CI = 0.18-0.53, t = 4.15, P less than 0.001) (86 kcal/24 h), or 7.2%. The increased values were found to be independent of age, sex, or body size. There were significant correlations between increased values and poor pulmonary function as measured by the ratio of the forced expiratory volume in 1 s to forced vital capacity (r = -0.44, P less than 0.05) and subclinical infection as indicated by the blood leukocyte count (r = 0.40, P less than 0.05). However, the correlations were low, suggesting that other factors may contribute to the increased resting energy expenditure, possibly including the putative metabolic defect in cystic fibrosis.
SUMMARY The diets of 20 children with cystic fibrosis were analysed for energy and nutrient content with simultaneous measurement of energy losses in stools. Median energy intakes were in excess of the WHO estimated daily requirements (118.2%) when expressed as MJ/kg/24 hours, the excess almost accounted for by energy losses in the stools. When expressed as MJ/24 hours, however, median energy intakes were 98-7% of that estimated for normal children of median weight for age. Compared with recently published data for normal school children the fat content of the diet was reduced (30-0%) as were intakes of iron and zinc. Children whose whole milk intakes were high had the greatest amount of fat and energy in their diets and were able to absorb energy in excess of that recommended. We conclude that many children with cystic fibrosis are still on low fat diets and whole milk is the single most useful food for the provision of extra dietary fat and energy.
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