Results 41 to 50 of about 3,103 (172)

Elexacaftor/tezacaftor/ivacaftor as rescue therapy in a patient with the cystic fibrosis genotype F508DEL/G1244E

Clinical Case Reports, 2021
Elexacaftor/tezacaftor/ivacaftor (ETI) is a cystic fibrosis (CF) transmembrane regulator (CFTR) modulator. It is known to be efficacious in stable patients with severe pneumopathy, but there are few data concerning its effectiveness during acute ...
Donatello Salvatore, Carmela Colangelo, Michele D’Andria, Giovanni Marsicovetere, Domenica Passarella   +4 more
doaj   +1 more source

Impact of Elexacaftor/Tezacaftor/Ivacaftor Therapy on the Cystic Fibrosis Airway Microbial Metagenome

Microbiology Spectrum, 2022
The introduction of mutation-specific combination therapy with the cystic fibrosis transmembrane conductance regulator (CFTR) modulators elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has substantially improved lung function and quality of life of people
Sophia T. Pallenberg, Marie-Madlen Pust, Ilona Rosenboom, Gesine Hansen, Lutz Wiehlmann, Anna-Maria Dittrich, Burkhard Tümmler   +6 more
doaj   +1 more source

Cystic Fibrosis Patients with F508del/Minimal Function Genotype: Laboratory and Nutritional Evaluations after One Year of Elexacaftor/Tezacaftor/Ivacaftor Treatment

, 2022
The last ten years have been characterized by an enormous step forward in the therapy and management of patients with Cystic Fibrosis (CF), thanks to the development and combination of Cystic Fibrosis Transmembrane Receptor (CFTR) correctors and ...
Scialo F., Gelzo M., Castaldo G., Zarrilli F., Carnovale V., Amato F., Celardo A., Corso G., Iacotucci P.   +8 more
core   +2 more sources

Efficacy of Trikafta (ELX/TEZ/IVA) & Symdeko (TEZ/IVA) in Treating Cystic Fibrosis with F508del Allele: A Systematic Review and Meta-analysis

Thoracic Research and Practice
The objective of the study was to assess and compare the efficacy of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) treatment with TEZ/IVA treatment in individuals diagnosed with cystic fibrosis (CF) and carrying the F508del allele.
Kainat Hussain, Sonali Karhana, Aakriti Garg, Mohd Ashif Khan   +3 more
doaj   +1 more source

Precision Medicine Based on CFTR Genotype for People with Cystic Fibrosis

Pharmacogenomics and Personalized Medicine, 2022
Iram Haq,1,2 Maryam Almulhem,1 Simone Soars,1 David Poulton,2,3 Malcolm Brodlie1,2 1Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK; 2Paediatric Respiratory Medicine, Great North ...
Haq I, Almulhem M, Soars S, Poulton D, Brodlie M   +4 more
doaj  

Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele

, 2019
Background Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, and nearly 90% of patients have at least one copy of the Phe508del CFTR mutation.
Mall, Marcus A., Moskowitz, Samuel M., Dřevínek, Pavel, Polineni, Deepika, Tullis, Elizabeth, Lands, Larry C., Tian, Simon, McKee, Charlotte M., Marigowda, Gautham, Nair, Nitin, Ramsey, Bonnie W., Van Braeckel, Eva, Middleton, Peter G., McKone, Edward F., Simard, Christopher, Waltz, David, Xuan, Fengjuan, Taylor-Cousar, Jennifer L., Savage, Jessica, Vermeulen, François, Jain, Raksha, Rowe, Steven M.   +21 more
core   +1 more source

USO DE EXACAFTOR/ TEZACAFTOR /IVACAFTOR EN PACIENTES CON FIBROSIS QUÍSTICA POST-TRASPLANTE PULMONAR

Neumología Pediátrica, 2023
El uso de moduladores de CFTR en pacientes con fibrosis quística post trasplante pulmonar es un tema todavía controversial. Varias publicaciones reportan los beneficios del modulador elexacaftor/tezacaftor/ivacaftor en los síntomas extrapulmonares de la ...
Pablo Jorquera Pinto
doaj   +1 more source

Determining the Minimal Clinically Important Difference of the 40‐Item Smell Identification Test in People With Cystic Fibrosis

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background Chronic rhinosinusitis (CRS) and olfactory dysfunction (OD) are highly prevalent among people with cystic fibrosis (PwCF) and negatively impact quality of life. The 40‐item Smell Identification Test (SIT) is widely used to assess psychophysical olfaction, but a CF‐specific minimal clinically important difference (MCID) has not been ...
Eugene Oh, Jessa E. Miller, Michelle J. Lee, Anna Zemke, David Baraghoshi, Matthew J. Strand, Jeremiah A. Alt, Todd E. Bodner, Michael Chang, Naweed I. Chowdhury, Patricia H. Eshaghian, Anne E. Getz, Jennifer L. Goralski, David A. Gudis, Peter H. Hwang, Ashoke Khanwalkar, Adam J. Kimple, Jivianne T. Lee, Douglas A. Li, Jess C. Mace, Jayakar V. Nayak, Jonathan B. Overdevest, Zara Patel, Daniella K. Safatian, Rodney J. Schlosser, Brent Senior, Amanda L. Stapleton, Timothy L. Smith, Zachary M. Soler, Jeffrey D. Suh, Grant A. Turner, Marilene B. Wang, Milene T. Saavedra, Jennifer L. Taylor‐Cousar, Daniel M. Beswick   +34 more
wiley   +1 more source

Pregnancy‐related effect on elexacaftor, tezacaftor and ivacaftor pharmacokinetics in women with cystic fibrosis

British Journal of Clinical Pharmacology, EarlyView.
Aim The number of pregnancies among women with cystic fibrosis (wwCF) has steadily increased over the past decade. However, the pharmacokinetics (PK) of elexacaftor–tezacaftor–ivacaftor (ETI) during gestation remains uncharacterized, despite its widespread use in this population.
Paulette Magnas, Naim Bouazza, Frantz Foissac, Saïk Urien, Léo Froelicher‐Bournaud, Gabrielle Lui, Victoria Guillemart, Nicolas Carlier, Jennifer Da Silva, Johanna Fesenbeckh, Reem Kanaan, Isabelle Honoré, Clémence Martin, Jean‐Marc Treluyer, Vassilis Tsatsaris, Pierre‐Régis Burgel, Sihem Benaboud   +16 more
wiley   +1 more source

The combination elexacaftor/tezacaftor/ivacaftor (ETI) modulates the de novo synthethic pathway of ceramides in a genotype-independent manner

, 2023
We report here how the triple combination of drugs elexacaftor/tezacaftor/ivacaftor (ETI) alters the balance of the de-novo synthethic pathway of sphingolipids in primary cells of human bronchial epithelium.
Franceschi, Pietro, Nicoletta Pedemonte, Liessi, Nara, Nara Liessi, Nicoletta Loberto, Tomati, Valeria, Valeria Tomati, Capurro, Valeria, Loberto, Nicoletta, Aureli, Massimo, Mar Garcia-Aloy, Massimo Aureli, Pedemonte, Nicoletta, Pietro Franceschi, Garcia-Aloy, Mar, Andrea Armirotti, Armirotti, Andrea, Valeria Capurro   +17 more
core   +1 more source