The Use of N-Acetylcysteine in the Treatment of Cystic Fibrosis

Abstract

Inhalation of N-acetylcysteine improves the pulmonary toilet in cystic fibrosis by decreasing the viscosity of tracheobronchial secretions. Functional pulmonary gains consist of improved vital capacity with diminished functional residual capacity, more even distribution of inspired air, increased respiratory flow rate, increased maximum voluntary ventilation, and decreased respiratory work load. The clinical benefit consists of increased exercise capacity, improved linear growth, and weight gain. Benefit is dependent upon delivery of the aerosol into the trachea in sufficient concentration and upon adequate bronchial drainage after therapy. Without adequate bronchial drainage, gas diffusion difficulties may be superimposed upon obstructive and restrictive lung disease already present.