Articles with "elexacaftor tezacaftor" as a keyword



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Elexacaftor‐tezacaftor‐ivacaftor overdose in an adolescent female with cystic fibrosis

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Published in 2022 at "Pediatric Pulmonology"

DOI: 10.1002/ppul.26116

Abstract: Cystic fibrosis (CF) represents a lifelong, chronic disease that requires intensive, regimented care for the entire duration of a patient's life This article is protected by copyright. All rights reserved. read more here.

Keywords: ivacaftor overdose; elexacaftor tezacaftor; tezacaftor ivacaftor; overdose adolescent ... See more keywords
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Outcome of elexacaftor/tezacaftor/ivacaftor therapy in patients with cystic fibrosis and solid organ transplantation

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Published in 2022 at "Pediatric Pulmonology"

DOI: 10.1002/ppul.26217

Abstract: We report a case series of four patients with cystic fibrosis (CF) and previous solid organ transplantation (SOT) receiving elexacaftor/tezacaftor/ivacaftor therapy for 6 months or more. Data was collected retrospectively. The treatment was well tolerated… read more here.

Keywords: organ transplantation; patients cystic; elexacaftor tezacaftor; tezacaftor ivacaftor ... See more keywords
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Impact of elexacaftor–tezacaftor–ivacaftor on bacterial colonization and inflammatory responses in cystic fibrosis

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Published in 2022 at "Pediatric Pulmonology"

DOI: 10.1002/ppul.26261

Abstract: Cystic fibrosis (CF) is a multisystem disease with progressive deterioration. Recently, CF transmembrane conductance regulator (CFTR) modulator therapies were introduced that repair underlying protein defects. Objective of this study was to determine the impact of… read more here.

Keywords: inflammatory responses; impact elexacaftor; elexacaftor tezacaftor; tezacaftor ivacaftor ... See more keywords
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Clinical change 2 years from start of elexacaftor‐tezacaftor‐ivacaftor in severe cystic fibrosis

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Published in 2023 at "Pediatric Pulmonology"

DOI: 10.1002/ppul.26318

Abstract: Limited published research is available on the impact of elexacaftor/tezacaftor/ivacaftor (ETI) beyond the initial few months postdrug initiation, especially for those who initiated therapy via individual investigational new drug application. The experiences of patients with… read more here.

Keywords: cystic fibrosis; elexacaftor tezacaftor; tezacaftor ivacaftor;
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Positive and negative impacts of elexacaftor/tezacaftor/ivacaftor: Healthcare providers' observations across US centers.

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Published in 2023 at "Pediatric pulmonology"

DOI: 10.1002/ppul.26527

Abstract: BACKGROUND Elexacaftor/tezacaftor/ivacaftor (ETI) has been associated with unprecedented clinical improvements, transforming the management of cystic fibrosis (CF). However, side effects with implications for safety and well-being have been reported, including neuropsychiatric changes. This study aimed… read more here.

Keywords: healthcare providers; across centers; positive negative; elexacaftor tezacaftor ... See more keywords
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Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease with the Phe508del/minimal function genotype.

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Published in 2021 at "Respiratory medicine"

DOI: 10.1016/j.rmed.2021.106646

Abstract: BACKGROUND Elexacaftor/tezacaftor/ivacaftor (E/T/I) is a cystic fibrosis transmembrane conductance regulator (CFTR) triple combination therapy used for the treatment of cystic fibrosis (CF) in patients aged ≥12 years who have at least one copy of the… read more here.

Keywords: cystic fibrosis; elexacaftor tezacaftor; treatment; safety ... See more keywords
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Managing cystic fibrosis in children aged 6-11yrs: a critical review of elexacaftor/tezacaftor/ivacaftor combination therapy

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Published in 2023 at "Expert Review of Respiratory Medicine"

DOI: 10.1080/17476348.2023.2179989

Abstract: ABSTRACT Introduction Cystic fibrosis is a life-limiting, autosomal recessive genetic disorder resulting in multi-organ disease due to CF transmembrane conductance regulator (CFTR) protein dysfunction. CF treatment previously focused on mitigation of disease signs and symptoms.… read more here.

Keywords: review; elexacaftor tezacaftor; tezacaftor ivacaftor; children aged ... See more keywords
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Impact of Elexacaftor/Tezacaftor/Ivacaftor Therapy on the Cystic Fibrosis Airway Microbial Metagenome

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Published in 2022 at "Microbiology Spectrum"

DOI: 10.1128/spectrum.01454-22

Abstract: Shotgun metagenome sequencing of respiratory secretions with spike-in controls for normalization demonstrated that 1 year of high-efficient CFTR modulation with elexacaftor/tezacaftor/ivacaftor extensively reduced the bacterial load. Longer observation periods will be necessary to resolve whether… read more here.

Keywords: tez iva; elx tez; elexacaftor tezacaftor; tezacaftor ivacaftor ... See more keywords
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Exploring the impact of elexacaftor-tezacaftor-ivacaftor treatment on opinions regarding airway clearance techniques and nebulisers: TEMPO a qualitative study in children with cystic fibrosis, their families and healthcare professionals

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Published in 2022 at "BMJ Open Respiratory Research"

DOI: 10.1136/bmjresp-2022-001420

Abstract: Background Cystic fibrosis (CF) is a genetic condition caused by variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that primarily impacts the lungs. Treatments historically have been symptomatic to improve airway clearance and… read more here.

Keywords: airway clearance; treatment; elexacaftor tezacaftor; cystic fibrosis ... See more keywords
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Effect of elexacaftor/tezacaftor/ivacaftor on airway and systemic inflammation in cystic fibrosis

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Published in 2023 at "Thorax"

DOI: 10.1136/thorax-2022-219943

Abstract: Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to improve lung function in people with cystic fibrosis (PWCF). However, its biological effects remain incompletely understood. Here we describe alterations in pulmonary and systemic inflammation in PWCF… read more here.

Keywords: cystic fibrosis; systemic inflammation; elexacaftor tezacaftor; tezacaftor ivacaftor ... See more keywords
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Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor

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Published in 2022 at "European Respiratory Journal"

DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a1172

Abstract: Background Abnormal macrophage function caused by dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR) is a critical contributor to chronic airway infections and inflammation in people with cystic fibrosis (PWCF). Elexacaftor/tezacaftor/ivacaftor (ETI) is a new CFTR… read more here.

Keywords: clinical outcomes; function; cftr; elexacaftor tezacaftor ... See more keywords