elexacaftor

cystic fibrosis transmembrane conductance regulator (CFTR) corrector designed to restore Phe508del CFTR protein function in patients with cystic fibrosis when administered with tezacaftor and ivacaftor

Also Known As:

Networked: 285 relevant articles (42 outcomes, 39 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Mall, Marcus A: 11 articles (12/2022 - 10/2018)
2.	Moskowitz, Samuel M: 10 articles (12/2022 - 10/2018)
3.	Taylor-Cousar, Jennifer L: 9 articles (01/2022 - 10/2018)
4.	McKone, Edward F: 8 articles (01/2022 - 10/2018)
5.	Tullis, Elizabeth: 7 articles (01/2022 - 10/2018)
6.	Salvatore, Donatello: 6 articles (02/2022 - 01/2021)
7.	Rowe, Steven M: 6 articles (01/2022 - 10/2018)
8.	Waltz, David: 6 articles (01/2022 - 10/2018)
9.	Ahluwalia, Neil: 5 articles (12/2022 - 11/2019)
10.	Tian, Simon: 5 articles (12/2022 - 11/2019)

Related Diseases

1.	Cystic Fibrosis (Mucoviscidosis) 01/01/2022 - "Rationale: The cystic fibrosis (CF) modulator drug, elexacaftor/tezacaftor/ivacaftor (ETI), proved highly effective in controlled clinical trials for individuals with at least one F508del allele, which occurs in at least 85% of people with CF. " 08/29/2023 - "The most recent modulator combination, elexacaftor/tezacaftor/ivacaftor (Trikafta®), has been shown to improve clinical outcomes in most patients with cystic fibrosis (PwCF). " 11/01/2022 - "Elexacaftor/tezacaftor/ivacaftor is highly effective in treating people with cystic fibrosis (pwCF) who have ≥ 1 responsive mutation. " 10/01/2023 - "Rapid desensitization to elexacaftor/tezacaftor/ivacaftor in a cystic fibrosis patient using fatty snack for improved absorption." 01/01/2023 - "Elexacaftor/tezacaftor/ivacaftor (ETI) treatment is associated with significant improvement in lung function in people with cystic fibrosis (pwCF); however, some patients experience adverse effects (AEs) including hepatotoxicity. "
2.	Lung Diseases (Lung Disease) 01/01/2023 - "Spirometric and anthropometric improvements in response to elexacaftor/tezacaftor/ivacaftor depending on age and lung disease severity." 01/01/2022 - "In a longitudinal cohort study of 32 patients (23 men; median age 32.5 years) with advanced CF lung disease (median FEV1 24.8% predicted) starting elexacaftor-tezacaftor-ivacaftor, the reflux symptom index score fell from a pre-treatment median (IQR) of 15 (11-23) to 5 (2.8-7.3) (p<0.001), the Hull airway reflux score fell from a median of 26.5 (16.3-39) to 7.5 (4-12) (p<0.001), and the sinonasal outcome score from a median of 36.5 (22-24) to 20 (10-32) (p<0.001) at 6 months on treatment. " 01/01/2021 - "Optimism with Caution: Elexacaftor-Tezacaftor-Ivacaftor in Patients with Advanced Pulmonary Disease." 01/01/2021 - "Reply to Kuek et al.: Optimism with Caution: Elexacaftor-Tezacaftor-Ivacaftor in Patients with Advanced Pulmonary Disease." 01/01/2021 - "Under specific assumptions regarding disease state and treatment effect applied to the Canadian CF population it is projected that by 2030, early introduction of elexacaftor/tezacaftor/ivacaftor is expected to reduce the number of individuals with severe lung disease by 60% (95% CI 55.3; 63.9), increase the number of individuals with mild lung disease by 18% (95%CI 18.2; 19.0) and reduce the number of pulmonary exacerbations by 19% (95%CI 18.9; 19.5). "
3.	Infections 01/01/2023 - "However, it is unclear how improved CFTR activity affects CF lung infections.MethodsWe performed a prospective, multicenter, observational study to measure the effect of the newest and most effective CFTR modulator, elexacaftor/tezacaftor/ivacaftor (ETI), on CF lung infections. " 01/01/2022 - "The goal of this study was to identify real-world evidence for the effectiveness of elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) in decreasing infection-related visits and antimicrobial use in people with CF. " 09/25/2023 - "The recently approved triple therapy Elexacaftor-Tezacaftor-Ivacaftor (ETI) has significantly improved the lung function and decreased airway infection of persons with CF (pwCF). " 05/01/2023 - "Elexacaftor - Tezacaftor - Ivacaftor treatment improves systemic infection parameters and Pseudomonas aeruginosa colonization rate in patients with cystic fibrosis a monocentric observational study." 01/01/2021 - "Highly effective modulator therapies, such as elexacaftor/tezacaftor/ivacaftor, do not substantially reduce infection and inflammation in vivo in CF patients, and thus these three case reports are of special note in terms of suggesting adjunct therapeutic approaches. "
4.	Body Weight (Weight, Body) 01/01/2022 - "Body weight, BMI, and blood pressure were extracted from outpatient clinic visits for the year preceding and the period following the initiation of elexacaftor-tezacaftor-ivacaftor. " 01/01/2021 - "Elexacaftor-tezacaftor-ivacaftor increased BMI and body weight in individuals 12 years of age and older who were hetero- or homozygous for the F508del mutation. " 02/01/2023 - "In children aged ≥ 6 y old and adolescents with F508del_CFTR mutation, Elexacaftor-Tezacaftor-Ivacaftor tend to be more effective than first-generation therapy, demonstrating promising results by exhibiting significant improvement in lung function, body weight, and respiratory-related quality of life." 01/01/2022 - "Background: The novel and highly effective CFTR modulator combination of elexacaftor-tezacaftor-ivacaftor (ETI) has been shown to improve lung function and body weight in people with Cystic Fibrosis (pwCF) carrying a F508del mutation. " 01/01/2023 - "Background: Elexacaftor-tezacaftor-ivacaftor (ETI) is a novel, highly effective CFTR modulator combination proven to enhance lung function and body weight in people with cystic fibrosis (pwCF) carrying a F508del mutation. "
5.	Weight Gain 06/17/2023 - "The introduction of the novel therapy, Elexacaftor/Tezacaftor/Ivacaftor (ETI) has been effective in improving weight gain in both clinical trials and real-world studies. " 06/17/2023 - "Heterogeneity of weight gain after initiation of Elexacaftor/Tezacaftor/Ivacaftor in people with cystic fibrosis." 01/01/2022 - "Though weight gain has been reported in some clinical trials of CFTR modulators, the effect of elexacaftor-tezacaftor-ivacaftor on body weight, body mass index (BMI), blood pressure, lipids and glycemic control in the real-world setting remains incompletely described. "

Related Drugs and Biologics

1.	ivacaftor drug combination tezacaftor
2.	Cystic Fibrosis Transmembrane Conductance Regulator (Protein, CFTR)
3.	ivacaftor
4.	tezacaftor
5.	Chlorides
6.	Glucose (Dextrose)
7.	Drug Combinations
8.	Vitamins
9.	lumacaftor
10.	Lipids

Related Therapies and Procedures

1.	Therapeutics
2.	Lung Transplantation
3.	Glycemic Control
4.	Liver Transplantation
5.	Precision Medicine