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 …

MRI of the lung (3/3)—current applications and future perspectives

J Biederer, S Mirsadraee, M Beer, F Molinari… - Insights into …, 2012 - Springer
Background MRI of the lung is recommended in a number of clinical indications. Having a
non-radiation alternative is particularly attractive in children and young subjects, or pregnant …

Effects of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in patients with cystic fibrosis and one or two F508del …

SY Graeber, DM Renz, M Stahl… - American journal of …, 2022 - atsjournals.org
Rationale: We recently demonstrated that triple-combination CFTR (cystic fibrosis
transmembrane conductance regulator) modulator therapy with elexacaftor/tezacaftor …

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 …

Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults …

L Wucherpfennig, SMF Triphan, S Wege… - Journal of Cystic …, 2022 - Elsevier
Abstract Background Therapy with Elexacaftor/Tezacaftor/Ivacaftor (ETI) was recently
approved for adult cystic fibrosis (CF) patients with at least one F508del mutation. However …

CFTR: cystic fibrosis and beyond

MA Mall, D Hartl - 2014 - Eur Respiratory Soc
Cystic fibrosis (CF) remains the most common fatal hereditary lung disease. The discovery of
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …

Comparison of lung clearance index and magnetic resonance imaging for assessment of lung disease in children with cystic fibrosis

M Stahl, MO Wielpütz, SY Graeber… - American journal of …, 2017 - atsjournals.org
Rationale: Early onset and progression of lung disease in children with cystic fibrosis (CF)
indicates that sensitive noninvasive outcome measures are needed for diagnostic …

Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease

MO Wielpütz, M Puderbach… - American journal of …, 2014 - atsjournals.org
Rationale: Studies demonstrating early structural lung damage in infants and preschool
children with cystic fibrosis (CF) suggest that noninvasive monitoring will be important to …

Effects of lumacaftor–ivacaftor on lung clearance index, magnetic resonance imaging, and airway microbiome in Phe508del homozygous patients with cystic fibrosis

SY Graeber, S Boutin, MO Wielpütz… - Annals of the …, 2021 - atsjournals.org
Rationale: Previous studies showed that lumacaftor–ivacaftor therapy results in partial
rescue of CFTR (cystic fibrosis [CF] transmembrane conductance regulator) activity and a …

MRI of the lung (2/3). Why… when… how?

J Biederer, M Beer, W Hirsch, J Wild, M Fabel… - Insights into …, 2012 - Springer
Background Among the modalities for lung imaging, proton magnetic resonance imaging
(MRI) has been the latest to be introduced into clinical practice. Its value to replace X-ray …