Figure 1.

The steps hypothesized to be relevant to the progression from dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR) to initial infection and inflammation and eventual development of airway structural damage are described. At each step, genetic and environmental modifiers can operate to alter outcome. The micrograph displays a bronchial airway from an individual with CF with the hallmark pathological findings including airway obstruction with mucus and inflammatory cells and peribronchial inflammatory response.