[HTML][HTML] Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease with the Phe508del/minimal function …

V Carnovale, P Iacotucci, V Terlizzi, C Colangelo… - Respiratory …, 2021 - Elsevier
cystic fibrosis transmembrane conductance regulator (CFTR) triple combination therapy used
for the treatment of cystic fibrosis … in cystic fibrosis adults with mild to moderate lung disease

A review of cystic fibrosis: Basic and clinical aspects

Q Chen, Y Shen, J Zheng - Animal models and experimental …, 2021 - Wiley Online Library
… In addition, we describe animal models used to study human cystic fibrosis disease and …
lung disease is the main cause of CF complications and patient mortality. The course of disease

[HTML][HTML] Efficacy of elexacaftor/tezacaftor/ivacaftor in advanced cystic fibrosis lung disease

AR Djavid, AE Thompson, AL Irace… - Annals of the …, 2021 - atsjournals.org
… that gains in lung function occur more slowly in our population with advanced lung disease,
and longer follow up may reveal a more substantial response (6). Most of the lung function …

[HTML][HTML] Cystic fibrosis: current concepts

JA López-Valdez, LA Aguilar-Alonso… - Boletín médico del …, 2021 - scielo.org.mx
… Moreover, the critical pathogen in chronic lung disease is an unusual morphotype of P. …
Progressive lung disease remains the leading cause of morbidity and mortality in these …

[HTML][HTML] The microbiome in cystic fibrosis pulmonary disease

A Françoise, G Héry-Arnaud - Genes, 2020 - mdpi.com
… and the progression of lung disease do not fully explain the lung phenotypes of CF patients.
For … Until recently, CF-related lung disease research focused on major pathogens such as P. …

[HTML][HTML] Utilizing centralized biorepository samples for biomarkers of cystic fibrosis lung disease severity

SD Sagel, BD Wagner, A Ziady, T Kelley… - … of Cystic Fibrosis, 2020 - Elsevier
… with cystic fibrosis (CF), progressive lung diseaselung disease relies primarily on spirometry
to measure lung function which is relatively insensitive in individuals with mild lung disease

Lung transplantation for cystic fibrosis

JC Yeung, TN Machuca, C Chaparro, M Cypel… - … of Heart and Lung …, 2020 - Elsevier
… The current study reinforces the role of LTx in the treatment of end-stage lung disease
secondary to CF. Clearly, outcomes have continued to improve, and the 50% 5-year survival rule …

[HTML][HTML] The clinical use of lung MRI in cystic fibrosis: what, now, how?

G Dournes, LL Walkup, I Benlala, MM Willmering… - Chest, 2021 - Elsevier
… -related lung disease to be effective, the onset and progression of the lung disease should
be … either indirectly by monitoring lung function or more directly by monitoring lung structure. …

Hyperpolarized gas magnetic resonance imaging of pediatric cystic fibrosis lung disease

G Santyr, N Kanhere, F Morgado, JH Rayment… - Academic radiology, 2019 - Elsevier
… Conventional pulmonary function tests appear normal in early cystic fibrosis (CF) lung
disease. Therefore, new diagnostic approaches are required that can detect CF lung disease in …

[HTML][HTML] CFTR modulator therapy and its impact on lung transplantation in cystic fibrosis

C Benden, C Schwarz - Pulmonary Therapy, 2021 - Springer
… For individuals with progressive advanced CF lung disease (ACFLD), lung transplantation
… , and their impact on lung disease progression and the need for lung transplantation as the …