The dilemma of initiating ELX/TEZ/IVA in a CF patient recovering from acute‐on‐chronic liver failure
Sign Up to like & getrecommendations! Published in 2022 at "Pediatric Pulmonology"
DOI: 10.1002/ppul.25917
Abstract: Cystic fibrosis liver disease (CFLD) is the third leading cause of mortality in cystic fibrosis (CF), with a wide spectrum of manifestations This article is protected by copyright. All rights reserved. read more here.
Keywords: initiating elx; liver; iva patient; dilemma initiating ... 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
Elexacaftor, tezacaftor and ivacaftor: a case of severe rash and approach to desensitisation
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DOI: 10.1136/bcr-2021-247042
Abstract: We present a case of severe rash following induction of elexacaftor, tezacaftor and ivacaftor (ELX/TEZ/IVA) in a young adult male cystic fibrosis patient. While rash is a commonly reported side effect which resolves in 1–2… read more here.
Keywords: severe rash; rash; case severe; elx tez ... See more keywords
S67 Effects of tezacaftor/ivacaftor (TEZ/IVA) treatment in patients heterozygous for F508del-CFTR and a residual function mutation: patient-reported outcomes in a phase 3 randomized, controlled trial (EXPAND)
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DOI: 10.1136/thorax-2018-212555.73
Abstract: Introduction and objectives To examine the treatment effect of TEZ/IVA on disease-related symptoms, functioning, and well-being as measured by Cystic Fibrosis Questionnaire–Revised (CFQ-R) in patients heterozygous for F508del and a second allele with a CFTR… read more here.
Keywords: analysis; treatment; tez iva; iva treatment ... See more keywords
A Phase 3 Open-Label Study of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One F508del Allele
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DOI: 10.1164/rccm.202102-0509oc
Abstract: Rationale: Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to be efficacious and safe in patients ≥12 years of age with cystic fibrosis and at least one F508del-CFTR (cystic fibrosis transmembrane conductance regulator) allele, but it has not been… read more here.
Keywords: years age; f508del; cystic fibrosis; elx tez ... See more keywords
Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function Mutation: A Phase 3b, Randomized, Placebo-controlled Study
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DOI: 10.1164/rccm.202202-0392oc
Abstract: Rationale The triple-combination regimen elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to be safe and efficacious in children aged 6 through 11 years with cystic fibrosis and at least one F508del-CFTR allele in a phase 3, open-label, single-arm… read more here.
Keywords: tez iva; elx tez; cystic fibrosis;
Short-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor Combination on Glucose Tolerance in Young People With Cystic Fibrosis—An Observational Pilot Study
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DOI: 10.3389/fped.2022.852551
Abstract: Background The effect of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on glucose tolerance and/or cystic-fibrosis-related diabetes (CFRD) is not well understood. We performed an observational study on the short-term effects of ELX/TEZ/IVA on glucose tolerance. Methods Sixteen adolescents with… read more here.
Keywords: tez iva; study; glucose tolerance; elx tez ... See more keywords
Changes in cystic fibrosis transmembrane conductance regulator protein expression prior to and during elexacaftor-tezacaftor-ivacaftor therapy
Sign Up to like & getrecommendations! Published in 2023 at "Frontiers in Pharmacology"
DOI: 10.3389/fphar.2023.1114584
Abstract: Background: Defects in expression, maturation or function of the epithelial membrane glycoprotein CFTR are causative for the progressive disease cystic fibrosis. Recently, molecular therapeutics that improve CFTR maturation and functional defects have been approved. We… read more here.
Keywords: cftr; protein expression; elx tez; tez iva ... See more keywords