scholarly journals Lung clearance index in CF: a sensitive marker of lung disease severity

Thorax ◽  
2007 ◽  
Vol 63 (2) ◽  
pp. 96-97 ◽  
Author(s):  
J. C Davies ◽  
S. Cunningham ◽  
E. W F W Alton ◽  
J A Innes
Thorax ◽  
2017 ◽  
Vol 73 (1) ◽  
pp. 82-84 ◽  
Author(s):  
Sylvia Verbanck ◽  
Gregory G King ◽  
Wenxiao Zhou ◽  
Anne Miller ◽  
Cindy Thamrin ◽  
...  

In adult patients with cystic fibrosis (CF), the lung clearance index (LCI) derived from the multiple breath washout relates to both acinar and conductive ventilation heterogeneity. The latter component predicts an association between LCI and the number of bronchial segments affected by bronchiectasis. Here, we experimentally demonstrated this association in patients with CF, and also examined an ancillary group of patients with non-CF bronchiectasis. We conclude that lung disease severity in terms of number of bronchial segments results in an associated LCI increase, likely constituting a portion of LCI that cannot be reversed by treatment in patients with CF lung disease.


Genes ◽  
2021 ◽  
Vol 12 (3) ◽  
pp. 441
Author(s):  
Fanny Pineau ◽  
Davide Caimmi ◽  
Sylvie Taviaux ◽  
Maurane Reveil ◽  
Laura Brosseau ◽  
...  

Cystic fibrosis (CF) is a chronic genetic disease that mainly affects the respiratory and gastrointestinal systems. No curative treatments are available, but the follow-up in specialized centers has greatly improved the patient life expectancy. Robust biomarkers are required to monitor the disease, guide treatments, stratify patients, and provide outcome measures in clinical trials. In the present study, we outline a strategy to select putative DNA methylation biomarkers of lung disease severity in cystic fibrosis patients. In the discovery step, we selected seven potential biomarkers using a genome-wide DNA methylation dataset that we generated in nasal epithelial samples from the MethylCF cohort. In the replication step, we assessed the same biomarkers using sputum cell samples from the MethylBiomark cohort. Of interest, DNA methylation at the cg11702988 site (ATP11A gene) positively correlated with lung function and BMI, and negatively correlated with lung disease severity, P. aeruginosa chronic infection, and the number of exacerbations. These results were replicated in prospective sputum samples collected at four time points within an 18-month period and longitudinally. To conclude, (i) we identified a DNA methylation biomarker that correlates with CF severity, (ii) we provided a method to easily assess this biomarker, and (iii) we carried out the first longitudinal analysis of DNA methylation in CF patients. This new epigenetic biomarker could be used to stratify CF patients in clinical trials.


2018 ◽  
Vol 53 (4) ◽  
pp. 426-430 ◽  
Author(s):  
Laura Berdah ◽  
Jessica Taytard ◽  
Sophie Leyronnas ◽  
Annick Clement ◽  
Pierre-Yves Boelle ◽  
...  

Immunity ◽  
2017 ◽  
Vol 46 (2) ◽  
pp. 301-314 ◽  
Author(s):  
Dania Zhivaki ◽  
Sébastien Lemoine ◽  
Annick Lim ◽  
Ahsen Morva ◽  
Pierre-Olivier Vidalain ◽  
...  

2012 ◽  
Vol 11 (1) ◽  
pp. 63-67 ◽  
Author(s):  
Harriet Corvol ◽  
Julie Beucher ◽  
Pierre-Yves Boëlle ◽  
Pierre-François Busson ◽  
Céline Muselet-Charlier ◽  
...  

2012 ◽  
Vol 40 (4) ◽  
pp. 1309-1316 ◽  
Author(s):  
Robinder G. Khemani ◽  
Neal J. Thomas ◽  
Vani Venkatachalam ◽  
Jason P. Scimeme ◽  
Ty Berutti ◽  
...  

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