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Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis.

Author: AddanteAnnalisa, BarduaMarkus, BoutinSébastien, DuerrJulia, FentkerKerstin, GradzielskiMichael, GraeberSimon Y, HaagRainer, KirchnerMarieluise, KuppeAditi, LausterDaniel, MallMarcus A, MertinsPhilipp, PiehlerLinus, RöhmelJobst, SchauppLaura, StahlMirjam, TheeStephanie, VöllerMirjam, ZiehmMatthias

Observational StudyResearch that observes and collects data on participants' behavior and outcomes in their natural state, without intervention.
Journal ArticleAn article published in an academic or professional journal that reports original research or scholarly analysis.
Research Support, Non-U.S. Gov'tResearch funding provided by government agencies outside the United States.

Overview

This study aimed to determine the longitudinal effects of elexacaftor/tezacaftor/ivacaftor (ETI) on airway mucus rheology, microbiome, and inflammation in cystic fibrosis (CF) patients with one or two F508del alleles throughout the first 12 months of therapy.
Paper Details　
Original Abstract of the Article :
Label="BACKGROUND">Recent studies demonstrated that the triple combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) improves lung function and reduces pulmonary exacerbations in cystic fibrosis (CF) patients with at least one...See full text at original site
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引用元：
https://doi.org/10.1183/13993003.02153-2022

データ提供：米国国立医学図書館（NLM）

A Triple Threat: New Hope for Cystic Fibrosis

Cystic fibrosis (CF), a genetic disease affecting the lungs and other organs, presents a significant challenge for patients and healthcare professionals. This longitudinal study examines the impact of elexacaftor/tezacaftor/ivacaftor (ETI), a triple combination CFTR modulator therapy, on sputum viscoelastic properties, airway infection, and inflammation in CF patients with at least one F508del allele. The study aims to understand the broader effects of this groundbreaking therapy beyond its known benefits in improving lung function and reducing exacerbations.

The Impact of ETI

The study found that ETI significantly improved sputum viscoelastic properties, a key indicator of mucus viscosity and its ability to clear from the airways. This suggests that ETI may not only improve lung function but also address the underlying mechanisms that contribute to chronic airway infection and inflammation. The study also observed improvements in microbiome composition and inflammatory markers, further highlighting the potential of ETI to address the multi-faceted challenges of CF.

A New Dawn for CF Treatment

The study underscores the promise of ETI as a transformative therapy for CF patients. The positive effects on sputum viscoelastic properties, airway infection, and inflammation suggest that ETI may be a powerful tool for addressing the underlying mechanisms that contribute to CF lung disease. This study opens new avenues for exploring the potential of ETI to improve long-term outcomes and enhance the quality of life for CF patients.

Dr. Camel's Conclusion

Cystic fibrosis is a harsh desert where patients often struggle to breathe. This study reveals that ETI, like a refreshing oasis, offers hope for a more comfortable and healthier life for CF patients. The positive effects on sputum, infection, and inflammation suggest that ETI may not only improve lung function but also help us navigate the complexities of this challenging disease.

Date :
  1. Date Completed 2023-08-07
  2. Date Revised 2023-08-09
Further Info :

Pubmed ID

37414422

DOI: Digital Object Identifier

10.1183/13993003.02153-2022

SNS
PICO Info
in preparation
Languages

English

Positive IndicatorAn AI analysis index that serves as a benchmark for how positive the results of the study are. Note that it is a benchmark and requires careful interpretation and consideration of different perspectives.

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