Whole-body protein metabolism was studied in 11 undernourished cystic fibrosis (CF) patient (7 female), 12 normally nourished CF patients (3 female), 7 anorexia nervosa (AN) patients (all female), and 15 normal control subjects (9 female). Protein turnover was studied by the single dose [15N]glycine method and the cumulative excretion of labeled urinary urea and ammonia. Energy metabolism was studied by open-circuit indirect calorimetry. Contrary to previous reports, no differences were found between the protein turnover of CF groups and the normal control group. However, patients with AN had a negative net protein deposition. Resting energy expenditure was significantly reduced in AN patients and increased in CF patients. The gender of CF patients did not affect protein and energy metabolism but fat mass was higher and fat-free mass was lower in CF females.