With the CF Research News, we aim to bridge the gap between people with CF and the researchers investigating CF and how best to treat those with the condition.
We want to provide access to patients, parents, relatives, friends and caregivers of patients with CF to all scientific work mainly published in the Journal of Cystic Fibrosis (JCF).

All PDF files published hereunder are lay version summaries of work published in the JCF.

April 2022

Title: Use of the CFTR modulator combination elexacaftor/tezacaftor/ivacaftor after lung transplant
Authors
Kathleen J. Ramos, Jennifer S. Guimbellot, Maryam Valapour, Lauren E. Bartlett, Travis Hee Wai, Joseph M. Pilewski, Albert Faro, Joshua M. Diamond for the CFLTC Study Group
References
J Cyst Fibros. 2022 Apr 23;S1569-1993(22)00098-4. doi: 10.1016/j.jcf.2022.04.009.
Keywords
Cystic fibrosis transmembrane conductance regulator, Lung transplantation, Trikafta
Theme
Care of patients with CF, Diabetes, Lung Disease and Infection, Nutrition/Gastroenterology, Transplantation
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Scientific Abstract

April 2022

Title: How the number of kinds of antibiotics chosen to treat Pseudomonas during an exacerbation affect recovery
Authors
D.R. VanDevanter, N.E. West, D.B. Sanders, M. Skalland, C.H. Goss, P.A. Flume, S.L. Heltshe
References
J Cyst Fibros. 2022 Apr 21;S1569-1993(22)00096-0. doi: 10.1016/j.jcf.2022.04.006.
Keywords
antipseudomonal classes, P. aeruginosa, Pulmonary exacerbation
Theme
Care of patients with CF, Lung Disease and Infection, Pulmonology
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Scientific Abstract

April 2022

Title: Coping and Length of Life in Cystic Fibrosis
Authors
Janice Abbott, Margaret A. Hurley, Helen Chadwick, Daniel Peckham
References
J Cyst Fibros. 2022 Apr 20;S1569-1993(22)00100-X. doi: 10.1016/j.jcf.2022.04.011.
Keywords
Cox model, Coping, optimism, Survival
Theme
Care of patients with CF, Psychosocial
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Scientific Abstract

April 2022

Title: Onset of high blood pressure might be possible after the start of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis.
Authors
Andrea Gramegna, Claudia De Petro, Gloria Leonardi, Martina Contarini, Francesco Amati, Roberto Meazza, Stefano Carugo, Francesco Blasi
References
J Cyst Fibros. 2022 Apr 18;S1569-1993(22)00099-6. doi: 10.1016/j.jcf.2022.04.010.
Keywords
adverse events, Elexacaftor/tezacaftor/ivacaftor, Systemic arterial hypertension
Theme
Care of patients with CF, Endocrinology
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Scientific Abstract

April 2022

Title: Current Prices versus Minimum Costs of Production for CFTR Modulators
Authors
Jonathan Guo, Junzheng Wang, Jingchun Zhang, Joseph Fortunak, Andrew Hill
References
J Cyst Fibros. 2022 Apr 16;S1569-1993(22)00090-X. doi: 10.1016/j.jcf.2022.04.007.
 
Keywords
CFTR modulator, ; Cystic fibrosis transmembrane conductance regulator, Elexacaftor/tezacaftor/ivacaftor, Kaftrio, Trikafta
Theme
Care of patients with CF, Psychosocial
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Scientific Abstract
Title: How ivacaftor changes inflammation and protein levels in the bloodstream of people with CF and the G551D mutation
Authors
Jordana E. Hoppe, Brandie D. Wagner, J. Kirk Harris, Steven M. Rowe, Sonya L Heltshe, Emily M. DeBoer, Scott D. Sagel
References
J Cyst Fibros. 2022 Apr 16;S1569-1993(22)00089-3. doi: 10.1016/j.jcf.2022.03.012.
Keywords
CFTR modulation, Extrapulmonary, Inflammation, Proteomics
Theme
Care of patients with CF, Endocrinology, Nutrition/Gastroenterology, Pulmonology
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Scientific Abstract

April 2022

Title: Inflammation and infection change what mucus is made of, how much gets made, and how much it can dissolve
Authors
Matthew R Markovetz, Ian C Garbarine, Cameron B Morrison, William J Kissner, Ian Seim, M Gregory Forest, Micah J Papanikolas, Ronit Freeman, Agathe Ceppe, Andrew Ghio, Neil E Alexis, Stephen M Stick, Camille Ehre, Richard C Boucher, Charles R Esther, Marianne S Muhlebach, David B Hill
References
J Cyst Fibros. 2022 Apr 15;S1569-1993(22)00097-2. doi: 10.1016/j.jcf.2022.04.008.
Keywords
Airway mucus, Gel-forming mucins, Infection, Microrheology, Mucus flakes
Theme
CFTR/Basic Science, Lung Disease and Infection
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Scientific Abstract

April 2022

Title: Immune response after COVID-19 vaccination in cystic fibrosis patients
Authors
Athanasios Michos, Filippos Filippatos, Elisabeth-Barbara Tatsi, Charilaos Dellis, Vasiliki Efthymiou, Ioanna Zarkada, Evgenia Troupi, Vasiliki Syriopoulou, Ioanna Loukou
References
J Cyst Fibros. 2022 Apr 14;S1569-1993(22)00094-7. doi: 10.1016/j.jcf.2022.04.004.
Keywords
Antibody, BNT162b2, COVID-19, Neutralizing, SARS-CoV-2, Vaccine
Theme
Care of patients with CF, Immunology
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Scientific Abstract

April 2022

Title: Maternal Trikafta use during pregnancy and breastfeeding improved outcomes for a baby with CF
Authors
Sylvia Szentpetery, Kimberly Foil, Sara Hendrix, Sue Gray, Christina Mingora, Barbara Head, Donna Johnson, Patrick A Flume
References
J Cyst Fibros. 2022 Apr 11;S1569-1993(22)00095-9. doi: 10.1016/j.jcf.2022.04.005.
Keywords
CFTR modulators, pregnancy
Theme
Care of patients with CF, Parents, Pregnancy with CF
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Scientific Abstract

April 2022

Title: Magnetic Resonance Imaging Detects Improvements of Pulmonary and Paranasal Sinus Abnormalities in Response to Elexacaftor/Tezacaftor/Ivacaftor Therapy in Adults with Cystic Fibrosis
Authors
Lena Wucherpfennig, Simon M. F. Triphan, Sabine Wege, Hans-Ulrich Kauczor, Claus P. Heussel, Niclas Schmitt, Felix Wuennemann, Victoria L. Mayer, Olaf Sommerburg, Marcus A. Mall, Monika Eichinger, Mark O. Wielpütz
References
J Cyst Fibros. 2022 Apr 7;S1569-1993(22)00088-1. doi: 10.1016/j.jcf.2022.03.011.
Keywords
Airway disease, Functional MRI, Lung perfusion, Mucus obstruction, CFTR-modulator
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Scientific Abstract