Bronchiectases at early chest computed tomography in children with cystic fibrosis are associated with increased risk of subsequent pulmonary exacerbations and chronic pseudomonas infection

AbstractBackgroundChildren with cystic fibrosis (CF) are often Pseudomonas aeruginosa (PsA) free and exhibit normal spirometry between the ages of 5 and 7. It is reported that computed tomography (CT) is more sensitive than FEV1 as an instrument in the identification of pulmonary disease. It is not known whether CF-CT scores in childhood may be used to highlight children at risk of developing severe disease.Aims1 — To assess the number of respiratory exacerbations (RTEs) during a follow-up period of 6years and their correlation with the CF-CT scores in young CF children. 2 — To assess whether PsA-negative CF children with high chest CF-CT scores are more likely to develop chronic PsA lung infection.Methods68 chest CT performed in patients without chronic PsA infection were scored. All patients (median age 7.8years) had at least 4 clinical, functional and microbiologic assessments/year in the subsequent 6years. RTE was defined as hospitalization and IV antibiotic treatment for respiratory symptoms.Results86.8% patients had <3 RTEs in the 6year follow-up period. The number of RTEs in the 6years subsequent to the CT scan was correlated to the bronchiectasis CT score (BCTS) (r=0.612; p<0.001) and to FEV1 at baseline (r=−0.495, p<0.001). A BCTS ≥17.5 identified patients with >3 RTEs during follow-up (sensitivity: 100%, specificity: 85%), while FEV1 did not. Only BCTS was significant in a logistic multivariate model (RR 1.15). BCTS was significantly lower and FEV1 higher in patients who did not develop chronic PsA infection by the end of the study.ConclusionIn CF children free from chronic PsA, both CT scores and FEV1 values demonstrate significant correlation with disease severity in the subsequent 6years but CT score has higher predictive value in the identification of patients at risk

The effect of CFTR modulators on structural lung disease in cystic fibrosis

Background: Newly developed quantitative chest computed tomography (CT) outcomes designed specifically to assess structural abnormalities related to cystic fibrosis (CF) lung disease are now available. CFTR modulators potentially can reduce some structural lung abnormalities. We aimed to investigate the effect of CFTR modulators on structural lung disease progression using different quantitative CT analysis methods specific for people with CF (PwCF). Methods: PwCF with a gating mutation (Ivacaftor) or two Phe508del alleles (lumacaftor-ivacaftor) provided clinical data and underwent chest CT scans. Chest CTs were performed before and after initiation of CFTR modulator treatment. Structural lung abnormalities on CT were assessed using the Perth Rotterdam Annotated Grid Morphometric Analysis for CF (PRAGMA-CF), airway-artery dimensions (AA), and CF-CT methods. Lung disease progression (0–3 years) in exposed and matched unexposed subjects was compared using analysis of covariance. To investigate the effect of treatment in early lung disease, subgroup analyses were performed on data of children and adolescents aged &lt;18 years. Results: We included 16 modulator exposed PwCF and 25 unexposed PwCF. Median (range) age at the baseline visit was 12.55 (4.25–36.49) years and 8.34 (3.47–38.29) years, respectively. The change in PRAGMA-CF %Airway disease (-2.88 (−4.46, −1.30), p = 0.001) and %Bronchiectasis extent (-2.07 (−3.13, −1.02), p &lt; 0.001) improved in exposed PwCF compared to unexposed. Subgroup analysis of paediatric data showed that only PRAGMA-CF %Bronchiectasis (-0.88 (−1.70, −0.07), p = 0.035) improved in exposed PwCF compared to unexposed. Conclusion: In this preliminary real-life retrospective study CFTR modulators improve several quantitative CT outcomes. A follow-up study with a large cohort and standardization of CT scanning is needed to confirm our findings.</p

Paediatric lung imaging: the times they are a-changin'

Until recently, functional tests were the most important tools for the diagnosis and monitoring of lung diseases in the paediatric population. Chest imaging has gained considerable importance for paediatric pulmonology as a diagnostic and monitoring tool to evaluate lung structure over the past decade. Since January 2016, a large number of papers have been published on innovations in chest computed tomography (CT) and/or magnetic resonance imaging (MRI) technology, acquisition techniques, image analysis strategies and their application in different disease areas. Together, these papers underline the importance and potential of chest imaging and image analysis for today's paediatric pulmonology practice. The focus of this review is chest CT and MRI, as these are, and will be, the modalities that will be increasingly used by most practices. Special attention is given to standardisation of image acquisition, image analysis and novel applications in chest MRI. The publications discussed underline the need for the paediatric pulmonology community to implement and integrate state-of-the-art imaging and image analysis modalities into their structure–function laboratory for the benefit of their patients

Spectral Data Augmentation Techniques to Quantify Lung Pathology from CT-Images

Data augmentation is of paramount importance in biomedical image processing tasks, characterized by inadequate amounts of labelled data, to best use all of the data that is present. In-use techniques range from intensity transformations and elastic deformations, to linearly combining existing data points to make new ones. In this work, we propose the use of spectral techniques for data augmentation, using the discrete cosine and wavelet transforms. We empirically evaluate our approaches on a CT texture analysis task to detect abnormal lung-tissue in patients with cystic fibrosis. Empirical experiments show that the proposed spectral methods perform favourably as compared to the existing methods. When used in combination with existing methods, our proposed approach can increase the relative minor class segmentation performance by 44.1% over a simple replication baseline

Chest radiography and computed tomography imaging in cystic fibrosis: current challenges and new perspectives

Imaging plays a pivotal role in the noninvasive assessment of cystic fibrosis (CF)-related lung damage, which remains the main cause of morbidity and mortality in children with CF. The development of new imaging techniques has significantly changed clinical practice, and advances in therapies have posed diagnostic and monitoring challenges. The authors summarise these challenges and offer new perspectives in the use of imaging for children with CF for both clinicians and radiologists. This article focuses on chest radiography and CT, which are the two main radiologic techniques used in most cystic fibrosis centres. Advantages and disadvantages of radiography and CT for imaging in CF are described, with attention to new developments in these techniques, such as the use of artificial intelligence (AI) image analysis strategies to improve the sensitivity of radiography and CT and the introduction of the photon-counting detector CT scanner to increase spatial resolution at no dose expense

The evolving role of radiological imaging in cystic fibrosis

PURPOSE OF REVIEW: Radiological imaging has a crucial role in pulmonary evaluation in cystic fibrosis (CF), having been shown to be more sensitive than pulmonary function testing at detecting structural lung changes. The present review summarizes the latest published information on established and evolving pulmonary imaging techniques for assessing people with this potentially life-limiting disorder. RECENT FINDINGS: Chest computed tomography (CT) has taken over the predominant role of chest radiography in many centres for the initial assessment and surveillance of CF lung disease. However, several emerging techniques offer a promising means of pulmonary imaging using less ionizing radiation. This is of particular importance given these patients tend to require repeated imaging throughout their lives from a young age. Such techniques include ultra-low-dose CT, tomosynthesis, dynamic radiography and magnetic resonance imaging. In addition, deep-learning algorithms are anticipated to improve diagnostic accuracy. SUMMARY: The recent introduction of triple-combination CF transmembrane regulator therapy has put further emphasis on the need for sensitive methods of monitoring treatment response to allow for early adaptation of treatment regimens in order to limit irreversible lung damage. Further research is needed to establish how emerging imaging techniques can contribute to this safely and effectively