Elexacaftor‐tezacaftor‐ivacaftor overdose in an adolescent female with cystic fibrosis
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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
Outcome of elexacaftor/tezacaftor/ivacaftor therapy in patients with cystic fibrosis and solid organ transplantation
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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
Impact of elexacaftor–tezacaftor–ivacaftor on bacterial colonization and inflammatory responses in cystic fibrosis
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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
Clinical change 2 years from start of elexacaftor‐tezacaftor‐ivacaftor in severe cystic fibrosis
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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;
Positive and negative impacts of elexacaftor/tezacaftor/ivacaftor: Healthcare providers' observations across US centers.
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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
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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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
Managing cystic fibrosis in children aged 6-11yrs: a critical review of elexacaftor/tezacaftor/ivacaftor combination therapy
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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
Impact of Elexacaftor/Tezacaftor/Ivacaftor Therapy on the Cystic Fibrosis Airway Microbial Metagenome
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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
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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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
Effect of elexacaftor/tezacaftor/ivacaftor on airway and systemic inflammation in cystic fibrosis
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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
Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor
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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