The effects of chronic hyperinflation, nutritional status, and posture on respiratory muscle strength in cystic fibrosis.

We measured maximal static inspiratory and expiratory pressures (PImax and PEmax) in 25 patients with cystic fibrosis (CF) and 80 normal control subjects to determine whether chronic hyperinflation and malnutrition reduce PImax and PImax/PEmax, respectively. In addition, we examined the effect of posture on pressures generated. We used a diminution in PEmax as an index of a malnutrition effect on pressures generated. The patients with CF, although significantly hyperinflated (ratio of residual volume to total lung capacity, 0.49), generated PImax values similar to those of the control subjects (p greater than 0.05). Despite evidence of malnutrition (mean body mass percentile, 78%) the PEmax values of patients with CF and those of control subjects were comparable (p greater than 0.05). There was no postural effect on pressures generated in the normal subjects or the CF group as a whole. We conclude that respiratory muscle strength is normal or supranormal in CF, despite chronic hyperinflation and malnutrition.