Date Published: July 10, 2019
Publisher: Public Library of Science
Author(s): Lidia Strzelczuk–Judka, Irena Wojsyk–Banaszak, Aleksandra Zakrzewska, Katarzyna Jończyk–Potoczna, Yan Li.
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.
Cystic fibrosis (CF) is one of the most common autosomal recessive hereditary life-shortening disorders among the White population [1,2]. The disease is caused by the mutation of gene coding CFTR protein (Cystic Fibrosis Transmembrane Conductance Regulator), leading to the production of dense mucus in the airways and exocrine glands and the impairment of their functioning. The main affected systems comprise respiratory and digestive systems, and the chronic pulmonary disease remains the main cause of morbidity and one of the most important prognostic factors in CF [1,3,4]. Chronic inflammation due to impaired mucocilliary clearance and mucus impaction in the airways results in bronchiectasis and progressive lung tissue destruction .
We enrolled 48 patients of European descent (24 males) aged 5 to 18 years diagnosed with CF who were admitted to the Pulmonology Department for a scheduled annual diagnostic check-up. The patients underwent a chest ultrasound and plain x-ray, and the time interval between the studies was not longer than 72 hours.
Cystic fibrosis is a life-shortening genetic disorder, involving the respiratory system and requiring chronic therapy. During the course of the disease patients suffer recurrent exacerbations, that affect patients quality of life and survival. Radiology plays a significant role in the patients’ follow-up, enabling monitoring of the disease, response to treatment as well as the diagnosis of exacerbations . Unfortunately conventional x-rays, especially when they are numerous and repeated over the course of disease, add up to a cumulative ionising radiation dose. Diminishing the radiation exposure, by looking for alternative diagnostic modalities, should be considered as one of the goals of contemporary medicine.