Future therapies for cystic fibrosis

L Allen, L Allen, SB Carr, G Davies, D Downey… - Nature …, 2023 - nature.com
We are currently witnessing transformative change for people with cystic fibrosis with the
introduction of small molecule, mutation-specific drugs capable of restoring function of the …

Current state of the art MRI for the longitudinal assessment of cystic fibrosis

JC Woods, JM Wild, MO Wielpütz… - Journal of Magnetic …, 2020 - Wiley Online Library
Pulmonary MRI can now provide high‐resolution images that are sensitive to early disease
and specific to inflammation in cystic fibrosis (CF) lung disease. With specificity and function …

Efficacy and safety of lumacaftor and ivacaftor in patients aged 6–11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled …

F Ratjen, C Hug, G Marigowda, S Tian… - The lancet Respiratory …, 2017 - thelancet.com
Background Lumacaftor and ivacaftor combination treatment showed efficacy in patients
aged 12 years or older with cystic fibrosis homozygous for F508del-cystic fibrosis …

Expanding applications of pulmonary MRI in the clinical evaluation of lung disorders: Fleischner Society position paper

H Hatabu, Y Ohno, WB Gefter, G Parraga, B Madore… - Radiology, 2020 - pubs.rsna.org
Pulmonary MRI provides structural and quantitative functional images of the lungs without
ionizing radiation, but it has had limited clinical use due to low signal intensity from the lung …

[HTML][HTML] In vivo methods and applications of xenon-129 magnetic resonance

H Marshall, NJ Stewart, HF Chan, M Rao… - Progress in Nuclear …, 2021 - Elsevier
Hyperpolarised gas lung MRI using xenon-129 can provide detailed 3D images of the
ventilated lung airspaces, and can be applied to quantify lung microstructure and detailed …

Postinfectious bronchiolitis obliterans in children: diagnostic workup and therapeutic options: a workshop report

SP Jerkic, F Brinkmann, A Calder… - Canadian respiratory …, 2020 - Wiley Online Library
Bronchiolitis obliterans (BO) is a rare, chronic form of obstructive lung disease, often initiated
with injury of the bronchiolar epithelium followed by an inflammatory response and …

Lung MRI with hyperpolarised gases: current & future clinical perspectives

NJ Stewart, LJ Smith, HF Chan… - The British Journal of …, 2022 - academic.oup.com
The use of pulmonary MRI in a clinical setting has historically been limited. Whilst CT
remains the gold-standard for structural lung imaging in many clinical indications, technical …

State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the “iMAging managEment of cySTic fibROsis” …

P Ciet, S Bertolo, M Ros, R Casciaro… - European …, 2022 - Eur Respiratory Soc
Objective Imaging represents an important noninvasive means to assess cystic fibrosis (CF)
lung disease, which remains the main cause of morbidity and mortality in CF patients. While …

[HTML][HTML] Multicentre standardisation of chest MRI as radiation-free outcome measure of lung disease in young children with cystic fibrosis

MO Wielpütz, O von Stackelberg, M Stahl… - Journal of Cystic …, 2018 - Elsevier
Background A recent single-centre study demonstrated that MRI is sensitive to detect early
abnormalities in the lung and response to therapy in infants and preschool children with …

129Xe and Free‐Breathing 1H Ventilation MRI in Patients With Cystic Fibrosis: A Dual‐Center Study

H Marshall, A Voskrebenzev, LJ Smith… - Journal of Magnetic …, 2023 - Wiley Online Library
Background Free‐breathing 1H ventilation MRI shows promise but only single‐center
validation has yet been performed against methods which directly image lung ventilation in …