Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Effects of Lumacaftor-Ivacaftor on Lung Clearance Index, Magnetic Resonance Imaging, and Airway Microbiome in Phe508del Homozygous Patients with Cystic Fibrosis.

Annals of the American Thoracic Society - 1 Jun 2021

Graeber Simon Y, Boutin Sébastien, Wielpütz Mark O, Joachim Cornelia, Frey Dario L, Wege Sabine, Sommerburg Olaf, Kauczor Hans-Ulrich, Stahl Mirjam, Dalpke Alexander H, Mall Marcus A

Abstract excerpt

Rationale: Previous studies showed that lumacaftor-ivacaftor therapy results in partial rescue of CFTR (cystic fibrosis [CF] transmembrane conductance regulator) activity and a moderate improvement of spirometry in Phe508del homozygous patients with CF. However, the effects of lumacaftor-ivacaftor on lung clearance index (LCI), lung morphology and perfusion detected by chest magnetic resonance imaging (MRI), and...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Aminophenols
Share to Aminopyridines

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Benzodioxoles

Share to Cystic Fibrosis

Share to Cystic Fibrosis Transmembrane Conductance Regulator

Share to Microbiota

Share to Quinolones

Source

Identifiers

PMID: 33600745
DOI: 10.1513/AnnalsATS.202008-1054OC

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Effects of Lumacaftor-Ivacaftor on Lung Clearance Index, Magnetic Resonance Imaging, and Airway Microbiome in Phe508del Homozygous Patients with Cystic Fibrosis.PMID 33600745Read ↗

Impact of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in children with cystic fibrosis and one or two F508del alleles.Semantic proximity Read ↗

Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR. A Clinical Trial.Semantic proximity Read ↗

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles.Semantic proximity Read ↗

Structural changes in lung morphology detected by MRI after modulating therapy with elexacaftor/tezacaftor/ivacaftor in adolescent and adult patients with cystic fibrosis.Semantic proximity Read ↗

Effects of elexacaftor/tezacaftor/ivacaftor therapy in children with cystic fibrosis - a comprehensive assessment using lung clearance index, spirometry, and functional and structural lung MRI.Semantic proximity Read ↗

Impact of Elexacaftor-Tezacaftor-Ivacaftor on lung disease in cystic fibrosis.Semantic proximity Read ↗

The clinical impact of Lumacaftor-Ivacaftor on structural lung disease and lung function in children aged 6-11 with cystic fibrosis in a real-world setting.Semantic proximity Read ↗

The clinical impact of Lumacaftor-Ivacaftor on structural lung disease and lung function in children aged 6-11 with Cystic Fibrosis in a real-world settingSemantic proximity Read ↗

Effects of Elexacaftor/Tezacaftor/Ivacaftor on Sputum Viscoelastic Properties, Airway Infection and Inflammation in Patients with Cystic FibrosisSemantic proximity Read ↗

Cystic fibrosis year in review 2020: Section 2 pulmonary disease, infections, and inflammation.Semantic proximity Read ↗

Effects of Lumacaftor/Ivacaftor on Cystic Fibrosis Disease Progression in Children 2 through 5 Years of Age Homozygous for F508del-CFTR: A Phase 2 Placebo-controlled Clinical Trial.Semantic proximity Read ↗

Real-World Clinical Outcomes of Lumacaftor-Ivacaftor or Tezacaftor-Ivacaftor Therapy in Children With Cystic Fibrosis Homozygous for Phe508del.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.