The effectiveness of maintenance albuterol aerosol therapy in cystic fibrosis (CF) was assessed by comparing spirometric measurements at the beginning and end of 1 year. Peak expiratory flow rates (PEFR) were measured twice daily to determine bronchodilator responsiveness and spontaneous diurnal variation (SDV), and results were compared with groups of normal and asthmatic children. CF patients not receiving regular albuterol therapy served as a control group. In the treatment group, forced vital capacity (FVC) and forced expiratory volume in the first second (FEV1) were significantly increased (12.2% and 18.4%, respectively) over the course of the treatment year, as contrasted with a significant decrease during the preceding year. During the study year the CF control group had a significant decrease in FEV1 and FEF25-75%, and the difference between treatment and control groups was significant for FVC, FEV1, and FEF25-75%. PEFR increased from the first to the last week of the year-long observation period (from 71.8% to 78.7% of predicted values, P < 0.01). Spontaneous diurnal variations were significantly greater in the CF study group than a group of normal children; SDV decreased significantly in the treatment group during the year of study. A bronchodilator response of > 15% was present in 25.8% of CF patient days, but there was considerable interpatient variability. Frequent bronchodilator responders were accurately predicted by their baseline bronchodilator responsiveness, but not by age or personal or family history of asthma or atopy. No difference in long-term pulmonary function improvements were noted between frequent and infrequent responders. The results suggest that maintenance albuterol aerosol treatments reversed the progressive downward course in lung function in the CF treatment group. A double-blind placebo-controlled study is required to confirm these preliminary findings.