Within the range of concentrations examined in this study, the effect of hypertonic saline appears to be dose dependent. Inhalation of hypertonic saline remains a potentially useful treatment for patients with cystic fibrosis.

In patients with cystic fibrosis (CF), dehydration of airway secretions leads to a decrease in mucociliary clearance (MCC). We examined the acute effect of MCC of a single administration by aerosolization of hypertonic saline (7%) (HS), amiloride (0.3% in 0.12% NaCl) (AML) and a combination of AML and HS (AML + HS) in 12 patients with CF using a radioaerosol technique. Isotonic saline [0.9%] (IS) was used as a control solution. As both the AML and HS solutions induced cough in some patients, the last nine patients studied also underwent a cough clearance day. This was to eliminate the possible confounding effect of cough on MCC measurement. Patients ranged from 18 to 28 yr (mean +/- SD, 22 +/- 3) with an FEV1 of 27 to 112% predicted (61 +/- 30%). Following deposition of the radioaerosol, baseline clearance was assessed for 30 min. This was followed by a 30-min intervention period. Assessment of post-intervention clearance for a further 30 min was then performed. Comparison of the amount of radioaerosol cleared from the right lung was made at 60 min (%C60) and 90 min (%C90) using repeated measures ANOVA. The percent cleared at 60 and 90 min was significantly increased with HS (%C60 = 26.5%, %C90 = 29.4%) and the combination of AML + HS (%C60 = 23.1%, %C90 = 27.4%) compared with both IS (%C60 = 14.7%, %C90 = 17.5%) and COUGH (%C60 = 18.0%, %C90 = 19.5%), p < 0.01. Inhalation of hypertonic saline is a potentially useful treatment in patients with cystic fibrosis.

It has been postulated that hypertonic saline (HS) might impair the antimicrobial effects of defensins within the airways. Alternative non‐ionic osmotic agents such as mannitol may thus be preferable to HS in promoting bronchial mucus clearance (BMC) in patients with cystic fibrosis (CF). This study reports the effect of inhalation of another osmotic agent, dry powder Mannitol (300 mg), compared with its control (empty capsules plus matched voluntary cough) and a 6% solution of HS on BMC in 12 patients with cystic fibrosis (CF). Mucus clearance was measured using a radioaerosol/gamma camera technique. Post‐intervention clearance was measured for 60 min, followed by cough clearance for 30 min. Neither mannitol nor HS improved BMC during the actual intervention period compared with their respective controls. However during the post‐intervention measurement there was a significant improvement in BMC for both the mannitol (8.7±3.3%versus 2.8±0.7%) and HS (10.0±2.3%versus 3.5±0.8%). There was also a significant improvement in cough clearance with the Mannitol (9.7±2.4%) compared with its control (2.5±0.8%). Despite premedication with a bronchodilator, a small fall in forced expiratory volume in one second (FEV1) was seen immediately after administration of both the mannitol (7.3±2.5%) and HS (5.8±1.2%). Values of FEV1 returned to baseline by the end of the study. Inhaled mannitol is a potential mucoactive agent in cystic fibrosis patients. Further studies are required to establish the optimal dose and the long‐term effectiveness of mannitol. Eur Respir J 1999; 14: 678–685.

Abnormal mucociliary clearance (MCC) is one of the central hypotheses for the development of lung disease in patients with cystic fibrosis (CF). However, attempts to demonstrate this decrease of MCC in vivo have proved to be somewhat less definitive, with the evidence barely favoring impaired clearance. Any apparent disparities are most likely due to the variety of methodologies used by different laboratories to measure MCC. The limitations of the various methodologies are examined in this review, in an attempt to better facilitate comparison of results. A number of physical and pharmacological therapies have been developed to promote mucus clearance from the CF airway. A summary of the results of interventional studies utilizing the measurement of MCC as an outcome measure is presented.

The aim of this study was to examine baseline mucociliary clearance (MCC) in patients with cystic fibrosis (n = 30; mean +/- SEM age, 23 +/- 1 yr; FEV1, 68 +/- 5% pred; range, 14 to 126%) and a group of normal subjects (n = 12; mean age, 27 +/- 1 yr) after an aerosol deposition of 99mTc-sulphur colloid (mass median diameter, 4.8 microns; geometric standard deviation, 1.6). Dynamic geometric mean images were formed from gamma camera data, and the percent clearance of activity after 60 min (%C60) was calculated for the whole right lung. Initial deposition of the aerosol was determined in terms of the penetration index, the ratio of peripheral to central activity. For normal subjects, an increase in mean inspiratory flow rate (MIFR) (49 +/- 5 versus 21 +/- 3 L/min, p < 0.05) resulted in an increase in whole right lung MCC (%C60, 31 +/- 4 versus 18 +/- 2%; p < 0.05). When aerosol delivery was controlled (MIFR, 34 +/- 5 versus 36 +/- 5 L/min), there was excellent reproducibility between studies (whole lung %C60, 34 +/- 8 versus 31 +/- 7; NS). The measurement of MCC was highly reproducible in six patients studied on four occasions with a mean coefficient of variation of 3.3 +/- 1%. A breathing pattern to accentuate central deposition was utilized in the patient studies (MIFR, 49 +/- 4 L/min).(ABSTRACT TRUNCATED AT 250 WORDS)