Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis

KJ Bayfield, TA Douglas, T Rosenow, JC Davies… - Thorax, 2021 - thorax.bmj.com
Structural and functional defects within the lungs of children with cystic fibrosis (CF) are
detectable soon after birth and progress throughout preschool years often without overt …

[HTML][HTML] Dornase alfa in Cystic Fibrosis: Indications, comparative studies and effects on lung clearance index

V Terlizzi, C Castellani, G Taccetti, B Ferrari - Italian Journal of Pediatrics, 2022 - Springer
Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations,
affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF …

Lung clearance index to track acute respiratory events in school-age children with cystic fibrosis

L Perrem, S Stanojevic, M Shaw, R Jensen… - American Journal of …, 2021 - atsjournals.org
Rationale: The lung clearance index (LCI) is responsive to acute respiratory events in
preschool children with cystic fibrosis (CF), but its utility to identify and manage these events …

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

JH Rayment, MJ Couch, N McDonald… - European …, 2019 - Eur Respiratory Soc
Pulmonary magnetic resonance imaging using hyperpolarised 129Xe gas (XeMRI) can
quantify ventilation inhomogeneity by measuring the percentage of unventilated lung …

Lung clearance index to monitor treatment response in pulmonary exacerbations in preschool children with cystic fibrosis

JH Rayment, S Stanojevic, SD Davis, G Retsch-Bogart… - Thorax, 2018 - thorax.bmj.com
Background Antibiotic treatment for pulmonary symptoms in preschool children with cystic
fibrosis (CF) varies among clinicians. The lung clearance index (LCI) is sensitive to early CF …

Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis

S Stanojevic, SD Davis, L Perrem… - European …, 2021 - Eur Respiratory Soc
The lung clearance index (LCI) measured by the multiple breath washout (MBW) test is
sensitive to early lung disease in children with cystic fibrosis. While LCI worsens during the …

Ex vivo model predicted in vivo efficacy of CFTR modulator therapy in a child with rare genotype

V Terlizzi, F Amato, C Castellani… - … Genetics & Genomic …, 2021 - Wiley Online Library
Background New drugs that target the basic defect in cystic fibrosis (CF) patients may now
be used in a large number of patients carrying responsive mutations. Nevertheless, further …

The assessment of short-and long-term changes in lung function in cystic fibrosis using 129Xe MRI

LJ Smith, A Horsley, J Bray, PJC Hughes… - European …, 2020 - Eur Respiratory Soc
Introduction Xenon-129 (129Xe) ventilation magnetic resonance imaging (MRI) is sensitive
to detect early cystic fibrosis (CF) lung disease and response to treatment. 129Xe-MRI could …

[HTML][HTML] Ventilation and perfusion assessed by functional MRI in children with CF: reproducibility in comparison to lung function

S Nyilas, G Bauman, O Pusterla, K Ramsey… - Journal of Cystic …, 2019 - Elsevier
Background Chronic lung diseases such as cystic fibrosis (CF) can be monitored by imaging
and lung function modalities. Magnetic resonance imaging (MRI) techniques such as matrix …

[HTML][HTML] Impact of cross-sensitivity error correction on representative nitrogen-based multiple breath washout data from clinical trials

PD Robinson, R Jensen, RA Seeto… - Journal of Cystic …, 2022 - cysticfibrosisjournal.com
The past decade has been one of rapid advance for Multiple Breath Washout (MBW) as a
tool to detect early obstructive lung disease. Consensus recommendations for both …