Background Lung disease is the most frequent cause of morbidity and mortality in patients
with cystic fibrosis (CF), and there is a shortage of sensitive biomarkers able to regionally …

Background Multiple-breath washout (MBW) has been shown to detect early impairment of
lung function in children with cystic fibrosis (CF). Nitrogen (N 2) or sulfur hexafluoride (SF 6) …

Background Lung perfusion is one of the key components of oxygenation. It is hampered in
pulmonary arterial diseases and secondary due to parenchymal diseases. Methods …

MRI is sensitive to detect hallmarks of cystic fibrosis lung disease such as bronchial wall
thickening, bronchiectasis, mucus plugging and abnormal lung perfusion. A morpho …

Purpose Lung impairment from functional MRI is frequently assessed as defect percentage.
The defect distribution, however, is currently not quantified. The purpose of this work was to …

Background Bronchial artery dilatation (BAD) is associated with haemoptysis in advanced
cystic fibrosis (CF) lung disease. Our aim was to evaluate BAD onset and its association with …

Background: Lumacaftor/ivacaftor (LUM/IVA) has shown modest benefits in previous
research, but the exact effects in the cystic fibrosis (CF) lung remain unclear. This study aims …

Cystic fibrosis (CF) lung disease has the greatest impact on the morbidity and mortality of
patients suffering from this autosomal-recessive multiorgan disorder. Although CF is a …

Cystic fibrosis (CF) is one of the most common inherited and life-shortening pulmonary
diseases in the Caucasian population. With the widespread introduction of newborn …

Background Recent studies support magnetic resonance angiography (MRA) as a
diagnostic tool for pulmonary arterial disease. Purpose To determine MRA image quality …