Update on the application of chest computed tomography scanning to cystic fibrosis.
OBJECTIVE
To summarize the role of chest computed tomography as a tool to evaluate cystic fibrosis lung disease, and to describe what abnormalities can be detected using computed tomography and how computed tomography can be used in clinical practice.


RESULTS
Pulmonary function tests are only an indirect measure of lung structure, and are insensitive to localized and early structural abnormalities. Computed tomography is able to detect small areas with localized severe damage. The differentiation between normal and abnormal on computed tomography is relatively easy. In half of cystic fibrosis patients, the information obtained from pulmonary function tests is discordant with the information obtained from computed tomography.


CONCLUSIONS
Cystic fibrosis patients show lung inflammation and infection starting early in life. This leads to irreversible structural lung damage such as bronchiectasis and air trapping. Cystic fibrosis lung disease varies widely between patients. The primary aim of therapy is to prevent structural damage and to conserve lung function. Adequate monitoring of cystic fibrosis lung disease is paramount to tailor treatment to a patient's need. Computed tomography is currently the best tool to monitor lung structure, and pulmonary function tests are the best to monitor lung function.
Tiddens
Harm A W M
HA
Erasmus Medical Centre Rotterdam Sophia Children's Hospital, Department of Pediatric Pulmonology and Allergology, Rotterdam, The Netherlands. h.tiddens@erasmusmc.nl
de Jong
Pim A
PA
D003550:Cystic Fibrosis / Q000150:complications*; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology; D008171:Lung Diseases / Q000000981:diagnostic imaging* / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D013902:Radiography, Thoracic; D018570:Risk Assessment; D014057:Tomography, X-Ray Computed