Abstract

To determine the extent and effects of increased metabolic demand represented by Pseudomonas colonization on body composition and resting energy expenditure in children with cystic fibrosis (CF). The study comprised 18 stable children with CF, of whom 10 (6 male/4 female) were colonized with Pseudomonas species and 8 (4 male/4 female) were not. The groups were of similar age range and genotype. Measured resting energy expenditure (REE) was performed by open circuit indirect calorimetry and compared with predicted REE calculated from standard equations. Body composition was determined by dual-energy x-ray absorptiometry, including lean body mass (LBM), fat mass (FM), bone mineral density (BMD), and anterior-posterior spine density (APS); these were compared using z-scores. Routine pulmonary function testing assessed forced vital capacity, forced expiratory volume in 1 second (FEV1), and forced expiratory flow over middle half of vital capacity (FEF25% to 75%); these were compared as percent predicted. As expected, results of pulmonary function testing showed significant deterioration among the children colonized with Pseudomonas species when compared with the children who were not, while standard anthropometry showed no differences in weight, height, or weight-for-height percentile and respective z-scores. Although a trend of lower LBM was noted among the children colonized with Pseudomonas species, no significant differences were found between these children and children who were not colonized with Pseudomonas species when z-scores for LBM, FM, BMD, or APS were compared during body composition analysis. In addition, neither REE as kilocalories per day (kcal/d) nor REE expressed as a percent predicted by standard equations discriminated between subgroups of children colonized with Pseudomonas species and children who were not. However, metabolic demand, expressed as resting energy expenditure in kilocalories per kilogram (kcal/kg) of LBM (REE/LBM), revealed significant differences between children colonized with Pseudomonas species and children who were not (75.4+/-4.4 vs 58.6+/-2.9 kcal/kg, p < .05). The effect of Pseudomonas colonization on metabolic demand in children with CF can be accurately assessed by expressing resting energy expenditure as kilocalorie per kilogram of LBM, the active metabolic component of the body. The 50% increase in REE/LBM seen in the children colonized with Pseudomonas species represents the metabolic demand from the inflammatory burden and work of breathing resulting from the effects of the Pseudomonas colonization. The trend of a lower LBM in the children colonized with Pseudomonas species makes this finding even more dramatic.

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