ABSTRACT
I. Introduction Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians caused by mutation in the CF transmembrane conductance regulator gene (1-3). The spectrum of clinical presentation in CF is vast and depends on which organs are affected. Since the discovery of the CFTR gene in 1989, a broad spectrum of CF phenotypes has been associated with specific CFTR gene mutations (1). CF is characterized by chronic lung infection and inflammation, with periods of acute exacerbation causing severe and irreversible lung tissue damage. Although our understanding of the pathophysiology of CF has increased, pulmonary infections remain the major cause of morbidity and mortality in patients with CF.
