With the increasing longevity of cystic fibrosis (CF), there is a growing need to minimise exposure to ionising radiation in patients who undergo regular imaging tests while monitoring the course of the lung disease. This study aimed to define the role of lung ultrasounds (LUS) in the evaluation of lung disease severity in children with clinically stable CF. LUS was performed on 131 patients aged 5 weeks to 18 years (study group) and in 32 healthy children of an equivalent age range (control group). Additionally, an interobserver study was performed on 38 patients from the study group. In CF patients, the following ultrasound signs were identified: I-lines; Z-lines; single, numerous and confluent B-lines; Am-lines; small and major consolidations; pleural line abnormalities and small amounts of pleural fluid. The obtained results were evaluated against an original ultrasound score. LUS results were correlated with the results of chest X-ray (CXR) [very high], pulmonary function tests (PFTs) [high] and microbiological status [significant]. The interobserver study showed very good agreement between investigators. We conclude that LUS is a useful test in the evaluation of CF lung disease severity compared to routinely used methods. With appropriate standardisation, LUS is highly reproducible.
Keywords: Am-lines; CF; CF LUS score; LUS; cystic fibrosis; interobserver agreement; lung ultrasound.