Energy expenditure in cystic fibrosis

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Resting energy expenditure (REE) in patients with cystic fibrosis (CF) has been studied many times. The initial studies concluded that REE was increased due to either the basic defect in CF or to the lung disease. Since that time, the details have continued to challenge investigators. First it appeared that this was an abnormality that affects older male patients with CF, possibly because of deterioration in lung function. Several studies have since shown that there is a sex difference, and larger studies have suggested that prepubertal and pubertal female patients have an increased REE compared with males. Whether or not this abnormality persisted was unknown until the recent study by Magoffin et al who studied whether females with CF have a persistently increased elevation in REE. The analysis showed that REE was increased in girls with CF compared with controls and with boys. The abnormality persisted for two years or more and through puberty, independent of pulmonary and liver disease. The mechanism remains to be fully elucidated. However, the results do emphasize the need for high energy diet in individuals affected by CF, particularly female adolescents with pancreatic insufficiency.