Chest imaging in cystic fibrosis studies: What counts, and can be counted?

Szczesniak, Rhonda; Turkovic, Lidija; Andrinopoulou, Eleni‐Rosalina; Tiddens, Harm A.W.M.

doi:10.1016/j.jcf.2016.12.008

Cited by 48 publications

(52 citation statements)

References 52 publications

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“…Second, this study is limited by the lack of chest imaging studies. Future studies including chest CT or magnetic resonance imaging outcome measures [39,40] will be critical to specify and localise the structural abnormalities associated with surface-bound NE that determine lung function decline and pulmonary hyperinflation. Third, established surveillance programmes in infants and young children with CF that include annual BAL will provide opportunities to determine the role of this cell-bound pool of NE in early CF lung disease, where structural lung disease can occur even in the absence of free NE activity in BAL fluid [1,2].…”

Section: Discussionmentioning

confidence: 99%

Elastase activity on sputum neutrophils correlates with severity of lung disease in cystic fibrosis

et al. 2018

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Neutrophil elastase (NE) is a key risk factor for severity of cystic fibrosis (CF) lung disease. Recent studies identified increased NE activity on the surface of airway neutrophils from CF-like mice and patients with CF. However, the role of surface-bound NE in CF lung disease remains unknown. We determined the relationship between surface-bound NE activity and severity of lung disease in CF.Surface-bound NE activity was measured on sputum neutrophils from 35 CF patients and eight healthy controls using novel lipidated Förster resonance energy transfer reporters and correlated with free NE activity, neutrophil counts, interleukin-8, myeloperoxidase and antiproteases in sputum supernatant, and with lung function parameters.Surface-bound NE activity was increased in CF compared to healthy controls (p<0.01) and correlated with free NE activity (p<0.05) and other inflammation markers (p<0.001). Surface-bound and free NE activity correlated with forced expiratory volume in 1 s % predicted (p<0.01 and p<0.05), but only surface-bound NE activity correlated with plethysmographic functional residual capacity % pred (p<0.01) in patients with CF.We demonstrate that surface-bound NE activity on airway neutrophils correlates with severity of lung disease in patients with CF. Our results suggest that surface-bound NE activity may play an important role in the pathogenesis and serve as novel biomarker in CF lung disease.

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Section: Discussionmentioning

confidence: 99%

Elastase activity on sputum neutrophils correlates with severity of lung disease in cystic fibrosis

et al. 2018

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“…Despite this, FEV 1 presently is used to guide therapy . While other modalities to monitor CF lung disease are emerging, further optimization is required for these complex approaches …”

Section: Introductionmentioning

confidence: 99%

“…13 Despite this, FEV 1 presently is used to guide therapy. 9 While other modalities to monitor CF lung disease 14 are emerging, further optimization is required for these complex approaches. 15 It is thus evident that rapid, cost-effective, and informative strategies for lung ventilation evaluation in CF patients are urgently required.…”

Section: Introductionmentioning

confidence: 99%

Use of low field nuclear magnetic resonance to monitor lung inflammation and the amount of pathological components in the sputum of cystic fibrosis patients

Abrami

Maschio

Conese

et al. 2019

Magnetic Resonance in Med

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Purpose To develop a novel approach to monitor lung ventilation/inflammation in cystic fibrosis (CF) patients. Lung assessment in CF patients is relevant given that most patients succumb to respiratory failure. Respiratory functional tests (forced expiratory volume in the first second; FEV1) and inflammatory markers are used to test pulmonary ventilation/inflammation, respectively. However, FEV1 is effort dependent and might be uncomfortable for CF patients. Furthermore, inflammatory marker detection is costly and not rapid. To overcome these limitations, we propose the measurement, by means of low field nuclear magnetic resonance, of the spin‐spin relaxation time (T2m) of water hydrogens present in CF patient sputum. In CF sputum, different biological components are pathologically increased and inversely related to lung functionality. Moreover, we showed that these components alter in a dose‐dependent manner the T2m in synthetic CF sputum. Methods Sputum samples were obtained from 42 CF subjects by voluntary expectoration; FEV1, C‐reactive protein (CRP), blood neutrophil counts together with cytokine (tumor necrosis factor alpha [TNFα], interleukin [IL]‐1β, IL‐4, and vascular endothelial growth factor) quantifications were then evaluated. Results In sputum samples, we observe that T2m directly correlates (rFEV1 = 0.44; P < 10−4; 169 samples) with FEV1. Moreover, T2m inversely correlates with the circulating inflammation markers CRP/neutrophil number (rCRP = −0.44, P < 10−4; rNC = −0.37, P < 2 * 10−4; 103 and 86 samples, respectively) and with the sputum inflammatory cytokines TNFα/IL‐β1 (rTNFα = −0.72, P < 10−4; rIL‐1β = −0.685, P < 10−4; 27 samples). T2m variations also correspond to FEV1 values over time in defined patients. Conclusion These findings, together with the fast, reliable, and simple determination of T2m, make our approach a novel tool potentially usable in the real world of CF patients.

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“…Chest computed tomography (CT) scans are the most sensitive marker of lung disease in infancy . However, routine surveillance CT scans are currently used largely in research only.…”

Section: Introductionmentioning

confidence: 99%

Clinical utility of surveillance computed tomography scans in infants with cystic fibrosis

Newbegin

Pilkington

Shanthikumar

et al. 2018

Pediatric Pulmonology

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Chest imaging in cystic fibrosis studies: What counts, and can be counted?

Cited by 48 publications

References 52 publications

Elastase activity on sputum neutrophils correlates with severity of lung disease in cystic fibrosis

Elastase activity on sputum neutrophils correlates with severity of lung disease in cystic fibrosis

Use of low field nuclear magnetic resonance to monitor lung inflammation and the amount of pathological components in the sputum of cystic fibrosis patients

Clinical utility of surveillance computed tomography scans in infants with cystic fibrosis

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