Physiologically Based Pharmacokinetic Modeling of Elexacaftor/Tezacaftor/Ivacaftor in Infants With Cystic Fibrosis [PDF]
CPT: Pharmacometrics &Systems Pharmacology, Volume 15, Issue 6, June 2026.ABSTRACT Ivacaftor is the only cystic fibrosis transmembrane conductance regulator modulator approved for infants ≥ 1 month. The elexacaftor/tezacaftor/ivacaftor combination, approved for children aged ≥ 2 years, has been shown to significantly slow CF progression.
Ngoc Hoa Truong +71 more
wiley +3 more sources
Management of Infants Exposed to Prenatal and Postnatal Elexacaftor/Tezacaftor/Ivacaftor: A Cross‐Sectional Survey [PDF]
Health Sci RepHealth Science Reports, Volume 9, Issue 5, May 2026.
Haley Haskett, Clement L. Ren
wiley +2 more sources
Elexacaftor–Tezacaftor–Ivacaftor Reduces Revision Sinus Surgery in People With Cystic Fibrosis [PDF]
The Laryngoscope, Volume 136, Issue 5, Page 2048-2053, May 2026.Elexacaftor–tezacaftor–ivacaftor (ETI) was associated with a significant decrease in the frequency and rate of endoscopic sinus surgeries in patients with cystic fibrosis. These findings suggest an improvement in CF‐related chronic rhinosinusitis outcomes following ETI and may influence future CF treatment decisions.
Amy Lin +6 more
wiley +2 more sources
Long‐term impact of Elexacaftor/Tezacaftor/ivacaftor on pulmonary, nutritional and metabolic outcomes in homozygous F508del cystic fibrosis patients: A real‐world cohort study [PDF]
British Journal of Clinical Pharmacology, Volume 92, Issue 6, Page 1822-1832, June 2026.Aim Elexacaftor/tezacaftor/ivacaftor (ETI) has markedly improved cystic fibrosis (CF) outcomes. However, its long‐term impact on nutrition, metabolism and liver health remains underexplored. We assessed 30‐month changes in pulmonary, nutritional, metabolic and inflammatory markers in people with CF (PwCF) homozygous for F508del.
Nicola Perrotta +5 more
wiley +2 more sources
Post-approval studies with the CFTR modulators Elexacaftor-Tezacaftor—Ivacaftor
Frontiers in Pharmacology, 2023 Triple combination therapy with the CFTR modulators elexacaftor (ELX), tezacaftor (TEZ) and ivacaftor (IVA) has been qualified as a game changer in cystic fibrosis (CF). We provide an overview of the body of literature on ELX/TEZ/IVA published between November 2019 and February 2023 after approval by the regulators.
Burkhard Tümmler, Burkhard Tümmler
openaire +4 more sources
Real-life impact of highly effective CFTR modulator therapy in children with cystic fibrosis
Frontiers in Pharmacology, 2023 Introduction: Recently, cystic fibrosis transmembrane regulator modulator therapy with elexacaftor/tezacaftor/ivacaftor has become available for children with cystic fibrosis (CF) carrying at least one F508del mutation.Objective: To assess the ...
Margarete Olivier +7 more
doaj +1 more source
Impact of Pharmacy Services on Time to Elexacaftor-Tezacaftor-Ivacaftor Initiation [PDF]
Journal of Managed Care & Specialty Pharmacy, 2021 The approval of elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) expanded highly effective cystic fibrosis transmembrane receptor modulator therapy to approximately 90% of persons age 12 and older with cystic fibrosis. Clinical pharmacists and pharmacy technicians played a key role in planning for ELX/TEZ/IVA initiation prior to FDA approval as well as ...
Lauren Roder +4 more
openaire +2 more sources
Frontiers in Immunology, 2023 IntroductionCystic fibrosis (CF), especially CF lung disease, is characterized by chronic infection, immune dysfunction including impairment of regulatory T cells (Tregs) and an exaggerated inflammatory response.
Dirk Westhölter +9 more
doaj +1 more source
Effects of CFTR modulators on serum biomarkers of liver fibrosis in children with cystic fibrosis
Hepatology Communications, 2023 The cystic fibrosis (CF) transmembrane conductance regulator corrector/potentiator combinations lumacaftor/ivacaftor and elexacaftor/tezacaftor/ivacaftor improve sweat chloride, pulmonary function, and nutrition.
Steven Levitte +3 more
doaj +1 more source
Personalized Selection of a CFTR Modulator for a Patient with a Complex Allele [L467F;F508del]
Current Issues in Molecular Biology, 2022 The presence of complex alleles in the CFTR gene can lead to difficulties in diagnosing cystic fibrosis and cause resistance to therapy with CFTR modulators.
Elena Kondratyeva +13 more
doaj +1 more source