The aims of this article are to summarize existing knowledge regarding the pathophysiology
of small airways disease in cystic fibrosis (CF), to speculate about additional mechanisms …

Computed tomography (CT) is a sensitive technique to monitor structural changes related to
cystic fibrosis (CF) lung disease. It detects structural pulmonary abnormalities such as …

Respiratory muscle strength is a proven predictor of long-term outcome of neuromuscular
disease (NMD), including amyotrophic lateral sclerosis, Duchenne muscular dystrophy, and …

The aim of this exploratory study was to evaluate diaphragmatic function across various
neuromuscular diseases using spirometry-controlled MRI. We measured motion of the …

For effective clinical management of cystic fibrosis (CF) lung disease it is important to closely
monitor the start and progression of lung damage. The aim of this study was to investigate …

Background: A sensitive and valid non-invasive marker of early cystic fibrosis (CF) lung disease
is sought. The lung clearance index (LCI) from multiple-breath washout (MBW) is known …

Objective: Congenital airway malacia is one of the few causes of irreversible airways
obstruction in children, but the incidence in the general population is unknown. Severe airway …

Objective: Our objective was to determine whether long-term treatment of young patients with
cystic fibrosis (CF) with dornase alfa maintains lung function and reduces respiratory tract …

Background: A study was undertaken to compare the ability of computed tomographic (CT)
scores and pulmonary function tests to detect changes in lung disease in children and adults …

Rationale: Chest computed tomography (CT) is the gold standard for demonstrating cystic
fibrosis (CF) airway disease. However, there are no standardized outcome measures …