Abstract
Cystic fibrosis (CF) is one of the most common genetic disorders in the Caucasian population. The disease has a progressive course and leads to reduced life quality and life expectancy. Standard diagnostic procedures used in the monitoring of CF patients, include methods exposing patients to the 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 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 aged from 5 to 18 years (24 girls and 24 boys). LUS consisted in the assessment of the pleura, lung sliding, A-line and B-line artifacts, “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.
Correlation between the CF-USS and the modified Chrispin-Norman scores were 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, 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 disease progression.
Conclusions LUS constitutes an invaluable tool for the diagnosis of subpleural consolidations. CF-USS results correlate with conventional x-ray modified Chrispin–Norman score. LUS should be considered an accessory radiographic examination in the monitoring of CF patients, and CF-USS may provide clinicians with valuable information concerning the disease progression.