The growing use of modulator therapies aimed at restoring cystic fibrosis transmembrane
conductance regulator (CFTR) protein function in people with cystic fibrosis has …

Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding for the
ion channel cystic fibrosis transmembrane conductance regulator (CFTR). The management …

Background We recently demonstrated that elexacaftor/tezacaftor/ivacaftor (ETI) improves
the lung clearance index (LCI) and abnormalities in lung morphology detected by magnetic …

Rationale: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are characterized by
inherited impaired mucociliary clearance leading to chronic progressive lung disease as …

Rationale: Clinical trials show that lumacaftor/ivacaftor (LUM/IVA) treatment has the potential
to modify early cystic fibrosis (CF) disease progression in children as young as 2 years of …

Background Previous studies showed that contrast-enhanced (CE) morpho-functional
magnetic resonance imaging (MRI) detects abnormalities in lung morphology and perfusion …

Asthma is characterized by inflammation of airways and bronchial hyperresponsiveness that
leads to variable airflow obstruction. 1–3 It is a heterogeneous disease that is diagnosed …

The major cause of mortality in people with cystic fibrosis (pwCF) is progressive lung
disease characterised by acute and chronic infections, the accumulation of mucus, airway …

Cystic fibrosis (CF) is a well-characterised genetic disease caused by reduced function or
absence of cystic fibrosis transmembrane regulator (CFTR) protein. In the 1930s–1950s, the …

Objetivos: Este estudio buscó evaluar la efectividad del tratamiento con ETI, a partir de la
recuperación de la función pulmonar (FP), en pacientes con fibrosis quística (FQ) avanzada …