Figure 2.

Axial plane high resolution CT images of proband A. Examples of enlargement of the airways (tram-tracking and signet rings) are indicated by arrows. This bronchiectasis is seen in the absence of mucus plugging, scar tissue, or surrounding inflammation. It is unknown as to whether this mild pulmonary phenotype would be more exacerbated had the proband not been undergoing daily preventative lung therapies (aerosolized albuterol, hypertonic saline, chest physiotherapy, etc.) due to her original CF diagnosis.