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
Tezacaftor and ivacaftor for the treatment of cystic fibrosis
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DOI: 10.1080/17476348.2020.1682998
Abstract: ABSTRACT Introduction: Cystic fibrosis (CF) is a complex, multi-system, genetic disease affecting over 70,000 people worldwide. The underlying defect is a mutation in the CFTR gene. Dysfunctional CFTR protein results in abnormal anion movement across… read more here.
Keywords: ivacaftor; cystic fibrosis; tezacaftor; cftr ... See more keywords
Managing cystic fibrosis in children aged 6-11yrs: a critical review of elexacaftor/tezacaftor/ivacaftor combination therapy
Sign Up to like & getrecommendations! 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
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
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
Reply to Kuek et al.: Optimism with Caution: Elexacaftor–Tezacaftor–Ivacaftor in Patients with Advanced Pulmonary Disease
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DOI: 10.1164/rccm.202103-0796le
Abstract: elexacaftor–tezacaftor–ivacaftor (1). We congratulate the authors on capturing real-world population data in this key group of patients with FEV1,40% who have significant potential to benefit from these treatmentsbutwereexcludedfromthepivotalphase3trials.Theauthors demonstrated significant and rapid improvements in lung… read more here.
Keywords: lung transplantation; tezacaftor ivacaftor; lung; elexacaftor tezacaftor ... See more keywords