Elexacaftor/tezacaftor/ivacaftor improves lung imaging outcomes in cystic fibrosis

Early morphologic changes in the lungs of asymptomatic infants and young children with cystic fibrosis

This Year in Review examines the most relevant articles published on lung cancer, respiratory infections, tuberculosis (TB), cystic fibrosis (CF), pleural diseases, and interventional pulmonology and imaging in Respirology and other respiratory medicine journals during 2013. Important findings are highlighted with comments in the following sections, each of which was contributed by an author as named in the beginning of each section.

Effect of elexacaftor-tezacaftor-ivacaftor on bronchial dilatations in adolescents with cystic fibrosis: a multicentre prospective observational study.

Making Contrast Material Obsolete: Functional Lung Imaging with MRI.

To investigate the causal associations between family relationships, family functioning, social circumstances and health outcomes in young children with cystic fibrosis. The anticipated health gains for patients with cystic fibrosis, promised by early diagnosis through newborn screening, have yet to be fully realized, despite advances in cystic fibrosis health care with aggressive management in multidisciplinary clinics and the development of specific medications. Adverse psychosocial functioning may underpin the current lack of progress as it is well recognized that compromised early parent-child attachment relationship experiences and adverse social circumstances have negative impacts on lifelong health status and health resource use, even in healthy children. A cross-sectional (initial) and longitudinal (progressive), multicentre study of children aged 3 months-6 years with cystic fibrosis, who have been diagnosed by newborn screening. Questionnaire and observational measures of parent psychosocial functioning, parenting and parent-child attachment and social markers; and including clinical outcomes of regular health surveillance with clinical, lung imaging (computerized tomography) and bronchoalveolar lavage for airway microbiology and inflammation. This will be the first study to investigate the causal effect of psychosocial functioning, parenting and attachment on physical health outcome measures in children with cystic fibrosis.

Background In cystic fibrosis (CF), recurrent imaging and pulmonary function tests (PFTs) are needed for the assessment of lung function during disease management. Purpose To assess the clinical feasibility of pulmonary three-dimensional ultrashort echo time (UTE) MRI at breath holding for quantitative image analysis of ventilation inhomogeneity and hyperinflation in CF compared with PFT. Materials and Methods In this prospective study from May 2018 to June 2019, participants with CF and healthy control participants underwent PFTs and functional lung MRI by using a prototypical single breath-hold three-dimensional UTE sequence. Fractional ventilation (FV) was calculated from acquired data in normal inspiration and normal expiration. FV of each voxel was normalized to the whole lung mean (FVN), and interquartile range of normalized ventilation (IQRN; as a measure of ventilation heterogeneity) was calculated. UTE signal intensity (SI) was assessed in full expiration (SIN, normalized to aortic blood). Obtained metrics were compared between participants with CF and control participants. For participants with CF, MRI metrics were correlated with the standard lung clearance index (LCI) and PFT. Mann-Whitney U tests and Spearman correlation were used for statistical analysis. Results Twenty participants with CF (mean age, 17 years ± 9 [standard deviation]; 12 men) and 10 healthy control participants (24 years ± 8; five men) were included. IQRN was higher for participants with CF than for control participants (mean, 0.66 ± 0.16 vs 0.50 ± 0.04, respectively; P = .007). In the 20 participants with CF, IQRN correlated with obstruction markers forced expiratory volume in 1 second-to-forced vital capacity ratio (r = -0.70; 95% confidence interval [CI]: -0.92, -0.28; P < .001), mean expiratory flow 25% (r = 0.78; 95% CI: -0.95, -0.39; P < .001), and with the ventilation inhomogeneity parameter LCI (r = 0.90; 95% CI: 0.69, 0.96; P < .001). Mean SIN in full expiration was lower in participants with CF than in control participants (0.34 ± 0.08 vs 0.39 ± 0.03, respectively; P = .03). Conclusion Three-dimensional ultrashort echo time MRI in the lungs allowed for functional imaging of ventilation inhomogeneity within a few breath holds in patients with cystic fibrosis. © RSNA, 2020 Online supplemental material is available for this article. See also the editorial by Wielpütz in this issue.

Journal of Magnetic Resonance ImagingEarly View Editorial Editorial for “Non-Contrast-Enhanced Functional Lung MRI to Evaluate Treatment Response of Allergic Bronchopulmonary Aspergillosis in Patients With Cystic Fibrosis: A Pilot Study” Niranjan Balu PhD, Corresponding Author Niranjan Balu PhD [email protected] orcid.org/0000-0002-1489-6683 Department of Radiology, University of Washington, Seattle, Washington, USA[email protected]Search for more papers by this authorSudhakar Pipavath MD, Sudhakar Pipavath MD Department of Radiology, University of Washington, Seattle, Washington, USASearch for more papers by this author Niranjan Balu PhD, Corresponding Author Niranjan Balu PhD [email protected] orcid.org/0000-0002-1489-6683 Department of Radiology, University of Washington, Seattle, Washington, USA[email protected]Search for more papers by this authorSudhakar Pipavath MD, Sudhakar Pipavath MD Department of Radiology, University of Washington, Seattle, Washington, USASearch for more papers by this author First published: 07 June 2023 https://doi.org/10.1002/jmri.28845 Evidence Level: 5 Technical Efficacy: Stage 1 Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. References 1Ohno Y, Seo JB, Parraga G, et al. Pulmonary functional imaging: Part 1—State-of-the-art technical and physiologic underpinnings. Radiology 2021; 299: 508- 523. 2Bauman G, Puderbach M, Deimling M, et al. Non-contrast-enhanced perfusion and ventilation assessment of the human lung by means of Fourier decomposition in proton MRI: Non-CE functional lung imaging in 1H MRI. Magn Reson Med 2009; 62: 656- 664. 3Bondesson D, Schneider MJ, Gaass T, et al. Nonuniform Fourier-decomposition MRI for ventilation- and perfusion-weighted imaging of the lung. Magn Reson Med 2019; 82: 1312- 1321. 4Pusterla O, Bauman G, Wielpütz MO, et al. Rapid 3D in vivo 1H human lung respiratory imaging at 1.5 T using ultra-fast balanced steady-state free precession: Ultra-fast SSFP for 3D lung functional MRI. Magn Reson Med 2017; 78: 1059- 1069. 5Voskrebenzev A, Gutberlet M, Klimeš F, et al. Feasibility of quantitative regional ventilation and perfusion mapping with phase-resolved functional lung (PREFUL) MRI in healthy volunteers and COPD, CTEPH, and CF patients. Magn Reson Med 2018; 79: 2306- 2314. 6Fischer A, Weick S, Ritter CO, et al. SElf-gated Non-Contrast-Enhanced FUnctional Lung imaging (SENCEFUL) using a quasi-random fast low-angle shot (FLASH) sequence and proton MRI. NMR Biomed 2014; 27: 907- 917. 7Benlala I, Klaar R, Gaass T, et al. Non-contrast-enhanced functional lung MRI to evaluate treatment response of allergic bronchopulmonary aspergillosis in patients with cystic fibrosis: A pilot study. J Magn Reson Imaging 2023; Epub ahead of print. 8Kraemer R, Deloséa N, Ballinari P, Gallati S, Crameri R. Effect of allergic bronchopulmonary aspergillosis on lung function in children with cystic fibrosis. Am J Respir Crit Care Med 2006; 174: 1211- 1220. 9Mastella G, Rainisio M, Harms HK, et al. Allergic bronchopulmonary aspergillosis in cystic fibrosis. A European epidemiological study. Epidemiologic Registry of Cystic Fibrosis. Eur Respir J 2000; 16: 464- 471. 10Kaireit TF, Sorrentino SA, Renne J, et al. Functional lung MRI for regional monitoring of patients with cystic fibrosis. PLoS One 2017; 12:e0187483. Early ViewOnline Version of Record before inclusion in an issue ReferencesRelatedInformation

Protocolo de diagnóstico y seguimiento de los pacientes con fibrosis quística

Currently, the life expectancy for cystic fibrosis (CF) lung disease is less than 40 years due to decreasing lung function despite significant advances in the care and treatment of these...

Respiratory tract exacerbations play a crucial role in progressive lung damage of people with cystic fibrosis, representing a major determinant in the loss of functional lung tissue, quality of life and patient survival. Detection and monitoring of respiratory tract exacerbations are challenging for clinicians, since under- and over-treatment convey several risks for the patient. Although various diagnostic and monitoring tools are available, their implementation is hampered by the current definition of respiratory tract exacerbation, which lacks objective "cut-offs" for clinical and lung function parameters. In particular, the latter shows a large variability, making the current 10% change in spirometry outcomes an unreliable threshold to detect exacerbation. Moreover, spirometry cannot be reliably performed in preschool children and new emerging tools, such as the forced oscillation technique, are still complementary and need more validation. Therefore, lung imaging is a key in providing respiratory tract exacerbation-related structural and functional information. However, imaging encompasses several diagnostic options, each with different advantages and limitations; for instance, conventional chest radiography, the most used radiological technique, may lack sensitivity and specificity in respiratory tract exacerbations diagnosis. Other methods, including computed tomography, positron emission tomography and magnetic resonance imaging, are limited by either radiation safety issues or the need for anesthesia in uncooperative patients. Finally, lung ultrasound has been proposed as a safe bedside option but it is highly operator-dependent and there is no strong evidence of its possible use during respiratory tract exacerbation. This review summarizes the clinical challenges of respiratory tract exacerbations in patients with cystic fibrosis with a special focus on imaging. Firstly, the definition of respiratory tract exacerbation is examined, while diagnostic and monitoring tools are briefly described to set the scene. This is followed by advantages and disadvantages of each imaging technique, concluding with a diagnostic imaging algorithm for disease monitoring during respiratory tract exacerbation in the cystic fibrosis patient.

Insights into pediatric asthma with hyperpolarized magnetic resonance imaging of the lung

Cystic fibrosis (CF) lung disease is caused by mutations in the CFTR-gene and remains one of the most frequent lethal inherited diseases in the Caucasian population. Given the progress in CF therapy and the consecutive improvement in prognosis, monitoring of disease progression and effectiveness of therapeutic interventions with repeated imaging of the CF lung plays an increasingly important role. So far, the chest radiograph has been the most widely used imaging modality to monitor morphological changes in the CF lung. CT is the gold standard for assessment of morphological changes of airways and lung parenchyma. Considering the necessity of life-long repeated imaging studies, the cumulative radiation doses reached with CT is problematic for CF patients. A sensitive, non-invasive and quantitative technique without radiation exposure is warranted for monitoring of disease activity. In previous studies, MRI proved to be comparable to CT regarding the detection of morphological changes in the CF lung without using ionising radiation. Furthermore, MRI was shown to be superior to CT regarding assessment of functional changes of the lung. This review presents the typical morphological and functional MR imaging findings with respect to MR-based follow-up of CF lung disease. MRI offers a variety of techniques for morphological and functional imaging of the CF lung. Using this radiation free technique short- and long-term follow-up studies are possible enabling an individualised guidance of the therapy.

18F-Fluorodeoxyglucose-PET/CT Imaging of Lungs in Patients With Cystic Fibrosis

Imaging represents an important noninvasive means to assess cystic fibrosis (CF) lung disease, which remains the main cause of morbidity and mortality in CF patients. While the development of new imaging techniques has revolutionised clinical practice, advances have posed diagnostic and monitoring challenges. The authors aim to summarise these challenges and make evidence-based recommendations regarding imaging assessment for both clinicians and radiologists. A committee of 21 experts in CF from the 10 largest specialist centres in Italy was convened, including a radiologist and a pulmonologist from each centre, with the overall aim of developing clear and actionable recommendations for lung imaging in CF. An a priori threshold of at least 80% of the votes was required for acceptance of each statement of recommendation. After a systematic review of the relevant literature, the committee convened to evaluate 167 articles. Following five RAND conferences, consensus statements were developed by an executive subcommittee. The entire consensus committee voted and approved 28 main statements. There is a need for international guidelines regarding the appropriate timing and selection of imaging modality for patients with CF lung disease; timing and selection depends upon the clinical scenario, the patient's age, lung function and type of treatment. Despite its ubiquity, the use of the chest radiograph remains controversial. Both computed tomography and magnetic resonance imaging should be routinely used to monitor CF lung disease. Future studies should focus on imaging protocol harmonisation both for computed tomography and for magnetic resonance imaging. The introduction of artificial intelligence imaging analysis may further revolutionise clinical practice by providing fast and reliable quantitative outcomes to assess disease status. To date, there is no evidence supporting the use of lung ultrasound to monitor CF lung disease.

The potential of magnetic resonance imaging (MRI) in lung imaging appears to be increasing year-on-year;1 having previously been considered a tool unlikely suitable for an air-filled organ, both hyperpolarised gas...