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Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two <i>F508del</i> Alleles.

Author: AllombaChristine, BarthSandra, BergesJulian, DittrichAnna-Maria, DoellingerFelix, DohnaMartha, GraeberSimon Y, HansenGesine, HämmerlingSusanne, MallMarcus A, NaehrlichLutz, PallenbergSophia T, RenzDiane M, RingshausenFelix C, RöhmelJobst, Rückes-NilgesClaudia, SommerburgOlaf, StahlMirjam, TümmlerBurkhard, VitzthumConstanze, Vogel-ClaussenJens, WielpützMark O

Journal ArticleAn article published in an academic or professional journal that reports original research or scholarly analysis.
Multicenter StudyResearch conducted in collaboration with multiple research institutions. It aims to collect a wide range of data and obtain more generalizable results.
Observational StudyResearch that observes and collects data on participants' behavior and outcomes in their natural state, without intervention.
Research Support, Non-U.S. Gov'tResearch funding provided by government agencies outside the United States.

Overview

Triple-combination CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improved lung ventilation and morphology in patients with cystic fibrosis (CF) and one or two F508del alleles. The study demonstrated improvements in lung clearance index (LCI) and magnetic resonance imaging (MRI) scores in these patients. The results suggest that ELX/TEZ/IVA can effectively improve lung function and address abnormalities in lung morphology in CF patients with F508del mutations.
Paper Details　
Original Abstract of the Article :
<b>Rationale:</b> We recently demonstrated that triple-combination CFTR (cystic fibrosis transmembrane conductance regulator) modulator therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improves CFTR function in airway and intestinal epithelia to 40-50% of normal in patients with cystic f...See full text at original site
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引用元：
https://doi.org/10.1164/rccm.202201-0219OC

データ提供：米国国立医学図書館（NLM）

A Beacon of Hope in the Desert of Cystic Fibrosis: Triple-Combination CFTR Modulator Therapy

The journey through the desert of [cystic fibrosis (CF)] can be arduous, but [triple-combination CFTR modulator therapy] offers a beacon of hope. This study, like a caravan navigating a challenging terrain, investigated the effects of [elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA)] therapy on [lung clearance index (LCI)] and [lung morphology and perfusion] in patients with CF. The researchers found that [ELX/TEZ/IVA] therapy significantly [improved LCI] and [reduced abnormalities in lung morphology] in patients with one or two [F508del] alleles.

A Glimmer of Light in the Desert: ELX/TEZ/IVA Improves Lung Function in Cystic Fibrosis

These findings highlight the potential of [ELX/TEZ/IVA] therapy to [improve lung function] and [reduce disease severity] in patients with CF, offering a much-needed oasis of relief in the desert of this challenging condition.

Navigating the Desert of Cystic Fibrosis: Seeking the Best Care

The journey through the desert of cystic fibrosis requires careful navigation and ongoing support. While this study offers promising results, it's crucial to [consult with a healthcare professional] for personalized advice and treatment options.

Dr.Camel's Conclusion

This study offers encouraging evidence of the benefits of triple-combination CFTR modulator therapy in improving lung function and reducing disease severity in cystic fibrosis. The findings highlight the potential of this therapy to provide a brighter future for patients with CF, offering hope in the desert of this challenging disease.

Date :
  1. Date Completed 2022-08-03
  2. Date Revised 2022-08-09
Further Info :

Pubmed ID

35536314

DOI: Digital Object Identifier

10.1164/rccm.202201-0219OC

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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