Diagnostic value of chest ultrasound in children with cystic fibrosis – Pilot study

Cystic fibrosis (CF) is one of the most common genetic disorders among the White population. The disease has a progressive course and leads to a reduction in the quality of life and of life expectancy. Standard diagnostic procedures used in the monitoring of CF patients include methods which expose patients to ionizing radiation. With increasing life expectancy in CF the cumulative dose of ionising radiation increases, prompting clinicians’ search for safer imaging studies. Despite its safety and availability lung ultrasound (LUS) is not routinely used in the diagnostic evaluation of CF patients. The aim of the study was to evaluate the diagnostic value of LUS in children with CF compared to a chest X-ray, and to assess the diagnostic value of the recently developed LUS score—CF-USS (Cystic Fibrosis Ultrasound Score). LUS was performed in 48 CF children and adolescents aged from 5 to 18 years (24 girls and 24 boys). LUS consisted of the assessment of the pleura, lung sliding, A-line and B-line artefacts, "lung rockets", alveolar consolidations, air bronchogram and pleural effusion. Chest radiography was performed in all patients and analyzed according to the modified Chrispin-Norman score. LUS was analyzed according to CF-USS. The correlation between the CF-USS and the modified Chrispin-Norman scores was moderate (R = 0.52, p = 0.0002) and strong in control studies. In 75% of patients undergoing LUS, small areas of subpleural consolidations were observed, which were not visible on x-rays. At the same time, LUS was not sensitive enough to visualize bronchial pathology, which plays an important role in assessing the progression of the disease. Conclusions: LUS constitutes an invaluable tool for the diagnosis of subpleural consolidations. CF-USS results correlate with the conventional x-ray modified Chrispin–Norman score. LUS should be considered a supplementary radiographic examination in the monitoring of CF patients, and CF-USS may provide clinicians with valuable information concerning the progression of the disease.