Radiol Clin North Am 1998 Jan;36(1):143-61
Cystic fibrosis. An overview.
Ruzal-Shapiro C
A single gene defect in cystic fibrosis results in a spectrum of clinical
presentations. Though expression is variable, the
radiographic appearance reflects the underlying pathophysiology. Abnormalities
of the small airways result in air-trapping;
bronchiectasis and mucus plugging reflect large airway disease. These
radiographic findings can be assessed and followed using
varying scoring systems.