No abstract

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.

No abstract

We have found allergic bronchopulmonary aspergillosis in 8.6% of 105 children with cystic fibrosis (CF). Its development was significantly associated with colonisation of the respiratory tract by Pseudomonas aeroginosa but not necessarily by the use of nebulised antibiotics. At the same time, 30% of the 105 CF children had A. fumigatus growing in their sputum, 30% had positive prick skin tests to A. fumigatus, 23% had a positive IgE antibody to A. fumigatus and 19% positive A. fumigatus precipitins (IgG antibodies). All of these features were significantly associated with the use of nebulised antibiotics but more strongly associated with Pseudomonas colonisation. It is possible that the Pseudomonas facilitates sensitisation to A. fumigatus or, alternatively, that A. fumigatus facilitates colonisation by Pseudomonas. Both hypotheses require investigation.