Effect of Salbutamol on Lung Ventilation in Children with Cystic Fibrosis: Comprehensive Assessment Using Spirometry, Multiple-Breath Washout, and Functional Lung Magnetic Resonance Imaging

Respiration ◽  
2021 ◽  
pp. 1-10
Author(s):  
Elisabeth Kieninger ◽  
Corin Willers ◽  
Katrin Röthlisberger ◽  
Sophie Yammine ◽  
Orso Pusterla ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Magnetic Resonance ◽  
Pulmonary Function Tests ◽  
Lung Ventilation ◽  
Matrix Pencil ◽  
Forced Expiratory Volume ◽  
Inhalation Therapy ◽  
Resonance Imaging ◽  
Multiple Breath Washout

<b><i>Background:</i></b> Inhalation therapy is one of the cornerstones of the daily treatment regimen in patients with cystic fibrosis (CF). Recommendations regarding the addition of bronchodilators, especially salbutamol are conflicting due to the lack of evidence. New diagnostic measures such as multiple-breath washout (<underline>MBW)</underline> and functional magnetic resonance imaging (MRI) have the potential to reveal new insights into bronchodilator effects in patients with CF. <b><i>Objective:</i></b> The objective of the study was to comprehensively assess the functional response to nebulized inhalation with salbutamol in children with CF. <b><i>Methods:</i></b> Thirty children aged 6–18 years with stable CF performed pulmonary function tests, MBW, and matrix pencil-MRI before and after standardized nebulized inhalation of salbutamol. <b><i>Results:</i></b> Lung clearance index decreased (improved) by −0.24 turnover (95% confidence interval [CI]: −0.53 to 0.06; <i>p</i> = 0.111). Percentage of the lung volume with impaired fractional ventilation and relative perfusion decreased (improved) by −0.79% (CI: −1.99 to 0.42; <i>p</i> = 0.194) and −1.31% (CI: −2.28 to −0.35; <i>p</i> = 0.009), respectively. Forced expiratory volume (FEV<sub>1</sub>) increased (improved) by 0.41 z-score (CI: 0.24–0.58; <i>p</i> &#x3c; 0.0001). We could not identify specific clinical factors associated with a more pronounced effect of salbutamol. <b><i>Conclusions:</i></b> There is a positive short-term effect of bronchodilator inhalation on FEV<sub>1</sub> in patients with CF, which is independent of ventilation inhomogeneity. Heterogeneous response between patients suggests that for prediction of a therapeutic effect this should be tested by spirometry in every patient individually.

scholarly journals Hyperpolarised 129Xe magnetic resonance imaging to monitor treatment response in children with cystic fibrosis

2019 ◽  
Vol 53 (5) ◽  
pp. 1802188 ◽  
Author(s):  
Jonathan H. Rayment ◽  
Marcus J. Couch ◽  
Nancy McDonald ◽  
Nikhil Kanhere ◽  
David Manson ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Magnetic Resonance ◽  
Lung Disease ◽  
Treatment Response ◽  
Forced Expiratory Volume ◽  
Response To Therapy ◽  
Breath Hold ◽  
Pulmonary Exacerbation ◽  
Resonance Imaging

Pulmonary magnetic resonance imaging using hyperpolarised 129Xe gas (XeMRI) can quantify ventilation inhomogeneity by measuring the percentage of unventilated lung volume (ventilation defect per cent (VDP)). While previous studies have demonstrated its sensitivity for detecting early cystic fibrosis (CF) lung disease, the utility of XeMRI to monitor response to therapy in CF is unknown. The aim of this study was to assess the ability of XeMRI to capture treatment response in paediatric CF patients undergoing inpatient antibiotic treatment for a pulmonary exacerbation.15 CF patients aged 8–18 years underwent XeMRI, spirometry, plethysmography and multiple-breath nitrogen washout at the beginning and end of inpatient treatment of a pulmonary exacerbation. VDP was calculated from XeMRI images obtained during a static breath hold using semi-automated k-means clustering and linear binning approaches.XeMRI was well tolerated. VDP, lung clearance index and the forced expiratory volume in 1 s all improved with treatment; however, response was not uniform in individual patients. Of all outcome measures, VDP showed the largest relative improvement (−42.1%, 95% CI −52.1–−31.9%, p<0.0001).These data support further investigation of XeMRI as a tool to capture treatment response in CF lung disease.

scholarly journals Patterns of regional lung physiology in cystic fibrosis using ventilation magnetic resonance imaging and multiple-breath washout

2018 ◽  
Vol 52 (5) ◽  
pp. 1800821 ◽  
Author(s):  
Laurie J. Smith ◽  
Guilhem J. Collier ◽  
Helen Marshall ◽  
Paul J.C. Hughes ◽  
Alberto M. Biancardi ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Total Lung Capacity ◽  
Resonance Imaging ◽  
Ventilation Distribution ◽  
Lung Clearance ◽  
Lung Capacity ◽  
Lung Clearance Index ◽  
Ventilation Heterogeneity ◽  
Multiple Breath Washout

Hyperpolarised helium-3 (3He) ventilation magnetic resonance imaging (MRI) and multiple-breath washout (MBW) are sensitive methods for detecting lung disease in cystic fibrosis (CF). We aimed to explore their relationship across a broad range of CF disease severity and patient age, as well as assess the effect of inhaled lung volume on ventilation distribution.32 children and adults with CF underwent MBW and 3He-MRI at a lung volume of end-inspiratory tidal volume (EIVT). In addition, 28 patients performed 3He-MRI at total lung capacity. 3He-MRI scans were quantitatively analysed for ventilation defect percentage (VDP), ventilation heterogeneity index (VHI) and the number and size of individual contiguous ventilation defects. From MBW, the lung clearance index, convection-dependent ventilation heterogeneity (Scond) and convection–diffusion-dependent ventilation heterogeneity (Sacin) were calculated.VDP and VHI at EIVT strongly correlated with lung clearance index (r=0.89 and r=0.88, respectively), Sacin (r=0.84 and r=0.82, respectively) and forced expiratory volume in 1 s (FEV1) (r=−0.79 and r=−0.78, respectively). Two distinct 3He-MRI patterns were highlighted: patients with abnormal FEV1 had significantly (p<0.001) larger, but fewer, contiguous defects than those with normal FEV1, who tended to have numerous small volume defects. These two MRI patterns were delineated by a VDP of ∼10%. At total lung capacity, when compared to EIVT, VDP and VHI reduced in all subjects (p<0.001), demonstrating improved ventilation distribution and regions of volume-reversible and nonreversible ventilation abnormalities.

Hyperpolarized Gas Magnetic Resonance Imaging of Pediatric Cystic Fibrosis Lung Disease

2019 ◽  
Vol 26 (3) ◽  
pp. 344-354 ◽  
Author(s):  
Giles Santyr ◽  
Nikhil Kanhere ◽  
Felipe Morgado ◽  
Jonathan H. Rayment ◽  
Felix Ratjen ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Magnetic Resonance ◽  
Lung Disease ◽  
Cystic Fibrosis Lung ◽  
Resonance Imaging ◽  
Hyperpolarized Gas ◽  
Cystic Fibrosis Lung Disease

Magnetic Resonance Imaging of the Lung: Cystic Fibrosis

2017 ◽  
pp. 277-291 ◽  
Author(s):  
Scott K. Nagle ◽  
Michael Puderbach ◽  
Monika Eichinger ◽  
Talissa A. Altes
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Magnetic Resonance ◽  
Resonance Imaging

scholarly journals Quantitative multivolume proton-magnetic resonance imaging in patients with cystic fibrosis lung disease: comparison with clinical indicators

2019 ◽  
Vol 53 (5) ◽  
pp. 1702020 ◽  
Author(s):  
Francesca Pennati ◽  
Caterina Salito ◽  
Irene Borzani ◽  
Giulia Cervellin ◽  
Simone Gambazza ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Magnetic Resonance ◽  
Lung Disease ◽  
Proton Magnetic Resonance ◽  
Magnetic Resonance Images ◽  
Imaging Biomarkers ◽  
Resonance Imaging ◽  
Clinical Indicators ◽  
Proton Magnetic Resonance Imaging

This cross-sectional study aims to verify the relationship between quantitative multivolume proton-magnetic resonance imaging (1H-MRI) and clinical indicators of ventilatory abnormalities in cystic fibrosis (CF) lung disease.Non-enhanced chest MRI, spirometry and multiple breath washout was performed by 28 patients (10–27 years) with CF lung disease. Images acquired at end-inspiration and end-expiration were registered by optical flow to estimate expiratory–inspiratory proton-density change (Δ1H-MRI) as a measure of regional ventilation. Magnetic resonance images were also evaluated using a CF-specific scoring system.Biomarkers of CF ventilation impairment were defined from the Δ1H-MRI as follows: Δ1H-MRI median, Δ1H-MRI quartile coefficient of variation (QCV) and percentage of low-ventilation volume (%LVV). Imaging biomarkers correlated to all the clinical measures of ventilation abnormality, with the strongest correlation between Δ1H-MRI median and forced expiratory volume in 1 s (r2=0.44, p<0.001), Δ1H-MRI QCV and lung clearance index (LCI) (r2=0.51, p<0.001) and %LVV and LCI (r2=0.66, p<0.001). Correlations were also found between imaging biomarkers of ventilation and morphological scoring.The study showed a significant correlation between quantitative multivolume MRI and clinical indicators of CF lung disease. MRI, as a non-ionising imaging technique, may be particularly attractive in CF care for longitudinal evaluation, providing a new imaging biomarker to detect early ventilatory abnormalities.

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