Following discovery of the cystic fibrosis transmembrane conductance regulator (CFTR)
gene in 1989 and subsequent elucidation of the varied CFTR protein abnormalities that …

This is the third in a series of four papers updating the European Cystic Fibrosis Society
(ECFS) standards for the care of people with CF. This paper focuses on recognising and …

During the past quarter century, the diagnosis and treatment of cystic fibrosis (CF) have
been transformed by molecular sciences that initiated a new era with discovery of the cystic …

Rationale: Previous cross-sectional studies have demonstrated that chest magnetic
resonance imaging (MRI) is sensitive to detect early lung disease in infants and preschool …

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 …

Rationale: Lumacaftor/ivacaftor (LUM/IVA) was shown to be safe and well tolerated in
children 2 through 5 years of age with cystic fibrosis (CF) homozygous for F508del-CFTR in …

Imaging plays a pivotal role in the noninvasive assessment of cystic fibrosis (CF)-related
lung damage, which remains the main cause of morbidity and mortality in children with CF …

Specialized care is provided to people with cystic fibrosis (pwCF) by interdisciplinary teams
nested within the CF Foundation's accredited care center network. This network allows for …

The functioning of the respiratory system can be interfered with by exposure to mixtures of
environmental chemicals, however, the evidence is still ambiguous. We evaluated the …

Background: Properly evaluating respiratory system dysfunction is essential in children with
cystic fibrosis (CF). This prospective study aimed to assess the course of early lung disease …