LONGITUDE: An observational study of the long-term effectiveness of elexacaftor/tezacaftor/ivacaftor in people aged ≥12 years with cystic fibrosis using data from the United Kingdom Cystic Fibrosis Registry - 2-year analysis - Articles - Pneumology

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Pulmonary Fibrosis

The expanded French compassionate programme for elexacaftor-tezacaftor-ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study

★ 2 Recommended by Dr Martin Frey, Faculty Member

Lancet Respir Med

Burgel PR, Sermet-Gaudelus I, Girodon E, Durieu I, Houdouin V, Audousset C, Macey J, Grenet D, Porzio M, Murris-Espin M, Reix P, Baravalle M, Belleguic C, Mely L, Verhille J, Weiss L, Reynaud-Gaubert M, Mittaine M, Hamidfar R, Ramel S, Cosson L, Douvry B, Danner-Boucher I, Foucaud P, Roy C, Burnet E, Raynal C, Audrezet MP, Da Silva J, Martin C, French Cystic Fibrosis Reference Network study group

doi: 10.1016/S2213-2600(24)00208-X

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Vanzacaftor-tezacaftor-deutivacaftor versus elexacaftor-tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years and older (SKYLINE Trials VX20-121-102 and VX20-121-103): results from two randomised, active-controlled, phase 3 trials

★ 2 Recommended by Dr Martin Frey, Faculty Member

Lancet Respir Med

Keating C, Yonker LM, Vermeulen F, Prais D, Linnemann RW, Trimble A, Kotsimbos T, Mermis J, Braun AT, O'Carroll M, Sutharsan S, Ramsey B, Mall MA, Taylor-Cousar JL, McKone EF, Tullis E, Floreth T, Michelson P, Sosnay PR, Nair N, Zahigian R, Martin H, Ahluwalia N, Lam A, Horsley A

doi: 10.1016/S2213-2600(24)00411-9

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Further Publications

Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Adults and Adolescents with Cystic Fibrosis and at Least One F508del Allele: A Phase 3, Open-Label Extension Study

★ 3 Recommended by Dr Martin Frey, Faculty Member

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Am J Respir Crit Care Med

Daines CL, Polineni D, Tullis E, Costa S, Linnemann RW, Mall MA, McKone EF, Quon BS, Ringshausen FC, Selvadurai H, Taylor-Cousar JL, Withers NJ, Sawicki GS, Lee T, Ahluwalia N, Morlando Geiger J, Jennings M, Tan YV, Waltz D, Ramsey B, Griese M, VX17-445-105 Study Group

doi: 10.1164/rccm.202411-2231OC

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Vanzacaftor-tezacaftor-deutivacaftor for children aged 6-11 years with cystic fibrosis (RIDGELINE Trial VX21-121-105): an analysis from a single-arm, phase 3 trial

★ 2 Recommended by Dr Martin Frey, Faculty Member

★ 2 Recommended by Dr Marcus Hesse, Faculty Member

Lancet Respir Med

Hoppe JE, Kasi AS, Pittman JE, Jensen R, Thia LP, Robinson P, Tirakitsoontorn P, Ramsey B, Mall MA, Taylor-Cousar JL, McKone EF, Tullis E, Salinas DB, Zhu J, Chen YC, Rodriguez-Romero V, Sosnay PR, Davies G

doi: 10.1016/S2213-2600(24)00407-7

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Long-term impact of ivacaftor on mortality rate and health outcomes in people with cystic fibrosis

★ 2 Recommended by Dr Martin Frey, Faculty Member

★ 2 Recommended by Dr Marcus Hesse, Faculty Member

Thorax

Merlo CA, Thorat T, DerSarkissian M, McGarry LJ, Nguyen C, Gu YM, Healy J, Rubin JL, Brookhart MA

doi: 10.1136/thorax-2023-220558

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Pulmonary Fibrosis

Longer term follow-up of abdominal symptoms (CFAbd-Score) after initiation of Elexacaftor / Tezacaftor / Ivacaftor in adults with cystic fibrosis

★ 1 Recommended by Dr Martin Frey, Faculty Member

★ 2 Recommended by Dr Marcus Hesse, Faculty Member

J Cyst Fibros

Caley LR, Gillgrass L, Zagoya C, Saumtally H, Duckstein F, H W, Mainz JG, Peckham DG

doi: 10.1016/j.jcf.2025.01.010

Pulmonary FibrosisFurther Publications

Elexacaftor/tezacaftor/ivacaftor and inflammation in children and adolescents with cystic fibrosis: a retrospective dual-center cohort study

Ther Adv Respir Dis

Pepe A, Fevola C, Dolce D, Campana S, Ravenni N, Taccetti G, Salvatore D, Terlizzi V

doi: 10.1177/17534666251314706

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Effect of elexacaftor/tezacaftor/ivacaftor on systemic inflammation in cystic fibrosis

★ 3 Recommended by Dr Martin Frey, Faculty Member

★ 2 Recommended by Dr Marcus Hesse, Faculty Member

Thorax

Maher RE, Cytlak-Chaudhuri UM, Aleem S, Barry P, Brice DP, Caamaño Gutiérrez E, Driver K, Emmott E, Rothwell A, Smith E, Travis M, Lee D, McNamara PS, Waller I, Smith JA, Jones A, Lord RW

doi: 10.1136/thorax-2024-222242

Pulmonary Fibrosis

Real-World Effectiveness and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients with Phe508del -Gating and -Residual Function Genotypes

Respir Med

Salvatore D, Iacotucci P, Pepe A, Terminiello A, Majo F, Carnovale V, Vitullo P, Messore B, Parisi GF, Calderazzo MA, Leonetti G, Terlizzi V

doi: 10.1016/j.rmed.2025.108221

Related content

The expanded French compassionate programme for elexacaftor-tezacaftor-ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study

Vanzacaftor-tezacaftor-deutivacaftor versus elexacaftor-tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years and older (SKYLINE Trials VX20-121-102 and VX20-121-103): results from two randomised, active-controlled, phase 3 trials

Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Adults and Adolescents with Cystic Fibrosis and at Least One F508del Allele: A Phase 3, Open-Label Extension Study

Vanzacaftor-tezacaftor-deutivacaftor for children aged 6-11 years with cystic fibrosis (RIDGELINE Trial VX21-121-105): an analysis from a single-arm, phase 3 trial

Long-term impact of ivacaftor on mortality rate and health outcomes in people with cystic fibrosis

Longer term follow-up of abdominal symptoms (CFAbd-Score) after initiation of Elexacaftor / Tezacaftor / Ivacaftor in adults with cystic fibrosis

Elexacaftor/tezacaftor/ivacaftor and inflammation in children and adolescents with cystic fibrosis: a retrospective dual-center cohort study

Effect of elexacaftor/tezacaftor/ivacaftor on systemic inflammation in cystic fibrosis

Real-World Effectiveness and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients with Phe508del -Gating and -Residual Function Genotypes