May 2021
Title: Mycobacterium abscessus smooth and rough colony variants in cystic fibrosis patients
Authors Ana Victoria Gutiérrez, Sophie Alexandra Baron, Feyrouz Sonia Sardi, Jamal Saad, Bérengère Coltey, Martine Reynaud-Gaubert, Michel Drancourt
References J Cyst Fibros. 2021 May;20(3):421-423. doi: 10.1016/j.jcf.2021.02.002.
Keywords Mycobacterium abscessus, Rough variant, Smooth variant, Whole genome sequencing
Theme Care of patients with CF, Microbiology
Title: SARS-CoV2 infection in CF patient colonized by Burkholderia cenocepacia
Authors C Olcese, R Casciaro, D Pirlo, C Debbia, E Castagnola, F Cresta, C Castellani
References J Cyst Fibros. 2021 May;20(3):e29-e31. doi: 10.1016/j.jcf.2021.03.024. Epub 2021 Apr 8.
Keywords Burkholderia cepacia, Pulmonary exacerbation, SARS-CoV-2 COVID-19
May 2021
Title: Quality of life and health outcomes in children with CF aged 5 years
Authors Joyce Cheney, Suzanna Vidmar, Nicholas Gailer, Claire Wainwright, Tonia A Douglas, Australasian Cystic Fibrosis Bronchoalveolar Lavage (ACFBAL) study group
References J Cyst Fibros. 2020 May;19(3):483-491. doi: 10.1016/j.jcf.2020.02.022. Epub 2020 Mar 9.
Keywords Children, Exacerbations, health-related quality of life, Lung function, Preschool, Pulmonary
May 2021
Title: Boosting the rescue of faulty CFTR with phosphate
Authors Vinciane Saint‐Criq, Yiting Wang, Livia Delpiano, JinHeng Lin, David N. Sheppard, Michael A. Gray
References J Cyst Fibros. 2021 May 18;S1569-1993(21)00123-5. doi: 10.1016/j.jcf.2021.04.013.
Keywords Airway epithelia, CFTR correctors, Elexacaftor-tezacaftor-ivacaftor (Trikafta), F508del-CFTR, Phosphate, SLC34A2
May 2021
Title: CF Pancreatic Duct Secretions Can Alter Islet Function
Authors Pavana G. Rotti, Idil A, Evans, Yulong Zhang, Bo Liang, Nathan Cunicelli, Yunxia O’Malley, Andrew W. Norris, Aliye Uc, John F. Engelhardt
References J Cyst Fibros. 2021 May 17;S1569-1993(21)00120-X. doi: 10.1016/j.jcf.2021.04.010.
Keywords Cystic fibrosis related diabetes, ductal cells, pancreas, proteome, secretome
Title: Focusing on tiny compounds in exhaled breath, can they help detect bacteria in people with cystic fibrosis?
Authors Renate Kos, Paul Brinkman, Anne H Neerincx, Tamara Paff, Marije G Gerritsen, Ariana Lammers, Aletta D Kraneveld, Harry G M Heijerman, Hettie M Janssens, Jane C Davies, Christof J Majoor, Els J Weersink, Peter J Sterk, Eric G Haarman, Lieuwe D Bos, Anke H Maitland-van der Zee, Amsterdam Mucociliary Clearance Disease (AMCD) Research Group and the Amsterdam UMC Breath Research Group
References J Cyst Fibros. 2021 May 17;S1569-1993(21)00125-9. doi: 10.1016/j.jcf.2021.04.015.
Keywords Exhaled biomarkers, Pseudomonas aeruginosa, VOC
Title: Aquagenic wrinkling in children under two years of age: Could this be a potential clinical referral tool for cystic fibrosis among non-screened populations?
Authors Grace R Paul, Shasha Bai, Kenneth Jackson, Karen S McCoy
References J Cyst Fibros. 2021 May 17;S1569-1993(21)00112-0. doi: 10.1016/j.jcf.2021.04.002.
Keywords Aquagenic wrinkling, CF early diagnosis, Nutrition, Time to wrinkle
May 2021
Title: How does infection with Pseudomonas and Aspergillus affect people with cystic fibrosis? A study using UK registry data.
Authors Dominic A Hughes, Olga Archangelidi, Matthew Coates, Darius Armstrong-James, Stuart J Elborn, Siobhán B Carr, Jane C Davies
References J Cyst Fibros. 2021 May 3;S1569-1993(21)00117-X. doi: 10.1016/j.jcf.2021.04.007.
Keywords Aspergillus, Infection, Pseudomonas, registry
April 2021
Title: Microbiota diversity in CF chronic sinusitis
Authors Sarah K Lucas, Erin Feddema, Holly C Boyer, Ryan C Hunter
References J Cyst Fibros. 2021 Apr 27;S1569-1993(21)00104-1. doi: 10.1016/j.jcf.2021.03.022.
Keywords Chronic sinusitis, Microbial ecology, Microbiome, Pseudomonas aeruginosa, Staphylococcus aureus
April 2021
Title: Risk factors for joint inflammation and joint pain in cystic fibrosis
Authors C. Grehn*, A.-M. Dittrich, J. Wosniok, F. Holz, S. Hafkemeyer, L. Naehrlich, C. Schwarz, Registry working group of the German CF Registry
References J Fibros . 2021 May 22;S1569-1993(21)00130-2. doi: 10.1016/j.jcf.2021.05.003.
April 2021
Title: A study to understand which factors are important to people with Cystic Fibrosis when deciding whether to take part in clinical trials
Authors R Dobra, JS Elborn, S Madge, L Allen, M Boeri, F Kee, S Goundry, T Purcell, C Saunders, JC Davies
References J Cyst Fibros. 2021 Apr 21;S1569-1993(21)00103-X. doi: 10.1016/j.jcf.2021.03.021.
April 2021
Title: Meconium Ileus due to GUCY2C gene mutations in three unrelated South Indian families
Authors Sneha Varkki, Antony Terance Benjamin, Rekha Athiyarath, Sumita Danda, Ravikumar Sowmya, Gary Connett
References J Cyst Fibros. 2021 Apr 18;S1569-1993(21)00105-3. doi: 10.1016/j.jcf.2021.03.023.
Keywords Gucy2c gene mutation, meconium ileus
April 2021
Title: Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020.
Authors Naehrlich Lutz1 for the European Cystic Fibrosis COVID project group
References Journal of Cystic Fibrosis 2021; April 17. DOI:
https://doi.org/10.1016/j.jcf.2021.03.017
Keywords COVID-19, SARS-CoV-2, Europe, Incidence, Epidemiology
April 2021
Title: Optimising equity of access: how should we allocate slots to the most competitive trials in Cystic Fibrosis (CF)?
Authors R Dobra, G Davies, K Pike, C Strassle, L Allen, R Brendell, K Brownlee, S B Carr, N J Simmonds, J C Davies, London Network of Clinical Trials Accelerator Platform sites and affiliates
References J Cyst Fibros. 2021 Apr 16;S1569-1993(21)00109-0. doi: 10.1016/j.jcf.2021.03.027.
April 2021
Title: Trends in the use of healthcare resources over time and the associated costs for people with cystic fibrosis?
Authors Isabelle Durieu, Faustine Dalon, Quitterie Reynaud, Lydie Lemonnier, Clémence Dehillotte, Marjorie Bérard, Déborah Walther, Marie Viprey, Eric Van Ganse, Manon Belhassen
References J Cyst Fibros. 2021 Apr 14;S1569-1993(21)00107-7. doi: 10.1016/j.jcf.2021.03.025.
Keywords Costs, Healthcare resource use, Observational study
April 2021
Title: Bio-informatic analyses clarify how blood vessel malfunction may happen in people with cystic fibrosis
Authors Lucas Treps, Mathias Declercq, Siham Bousfia, Peter Carmeliet, Peter Witters
References J Cyst Fibros. 2021 Apr 13;S1569-1993(21)00101-6. doi: 10.1016/j.jcf.2021.03.019.
April 2021
Title: Infant with cystic fibrosis has normal pancreas function and was missed by newborn screening after mom stayed on CFTR modulator therapy during pregnancy.
Authors Christopher N Fortner, Julie M Seguin, Denise M Kay
References J Cyst Fibros. 2021 Apr 9;S1569-1993(21)00100-4. doi: 10.1016/j.jcf.2021.03.018.
Keywords CFTR modulator, Elexacaftor, Fecal elastase, Immunoreactive trypsinogen, In utero exposure, Ivacaftor, Newborn screen, Pancreatic dysfunction, pregnancy, Tezacaftor
April 2021
Title: Peripheral lung effect of elexacaftor/tezacaftor/ivacaftor in adult cystic fibrosis
Authors Dimitri Stylemans, Chantal Darquenne, Daniël Schuermans, Sylvia Verbanck, Eef Vanderhelst
References J Cyst Fibros. 2021 Apr 6;S1569-1993(21)00098-9. doi: 10.1016/j.jcf.2021.03.016
April 2021
Title: The role of the “PTM-CODE” in restoring F50del-CFTR functionality
Authors Claudio D'Amore, Christian Borgo, Mauro Salvi
References J Cyst Fibros. 2021 Apr 1;S1569-1993(21)00086-2. doi: 10.1016/j.jcf.2021.03.010.
Keywords CFTR correctors, F508del-CFTR, Post-translational modifications, Regulatory insertion region, mutagenesis
Title: Nasal high-flow therapy as an adjunct to exercise in patients with cystic fibrosis: A pilot feasibility trial
Authors Giulia Spoletini, Ruth Watson, Wang Yng Lim, Kim Pollard, Christine Etherington, Ian J Clifton, Daniel G Peckham
References J Cyst Fibros. 2021 Apr 1;S1569-1993(21)00054-0. doi: 10.1016/j.jcf.2021.03.005.
Keywords 6-minute walking test, Dyspnoea, Exercise-induced desaturation, Nasal high-flow therapy
March 2021
Title: Elevated blood glucose during exacerbations of cystic fibrosis lung disease is associated with poor recovery of lung function.
Authors Michael Pallin, Shanal Kumar, Chris Daley, Shrinkhala Dawadi, Paul Leong, Erin Carr, Georgia Soldatos
References Cyst Fibros. 2021 Mar 26;S1569-1993(21)00053-9. doi: 10.1016/j.jcf.2021.03.004.
Keywords continuous glucose monitoring, FEV(1), Hyperglycaemia, Pulmonary exacerbation
March 2021
Title: Does having Exophiala in sputum samples make a difference to health in CF?
Authors Rowena Mills, Riina Rautemaa-Richardson, Stuart Wilkinson, Latifa Patel, Anirban Maitra, Alex Horsley
References J Cyst Fibros. 2021 Mar 25;S1569-1993(21)00088-6. doi: 10.1016/j.jcf.2021.03.012.
Keywords Exophiala species, Fungal infection, Paediatric
Title: Emergence and impact of oprD mutations in Pseudomonas aeruginosa strains in cystic fibrosis
Authors Laura J Sherrard, Bryan A Wee, Christine Duplancic, Kay A Ramsay, Keyur A Dave, Emma Ballard, Claire E Wainwright, Keith Grimwood, Hanna E Sidjabat, David M Whiley, Scott A Beatson, Timothy J Kidd, Scott C Bell
References J Cyst Fibros. 2021 Mar 25;S1569-1993(21)00056-4. doi: 10.1016/j.jcf.2021.03.007.
Keywords Antimicrobial resistance, clinical outcomes, OPRD, Pseudomonas aeruginosa
March 2021
Title: Impact of Trikafta on mothers and babies during pregnancy and lactation
Authors Jennifer L Taylor-Cousar, R. Jain
References J Cyst Fibros. 2021 Mar 21;S1569-1993(21)00055-2. doi: 10.1016/j.jcf.2021.03.006.
Keywords CFTR, infant, lactation, Modulators, pregnancy
March 2021
Title: The Proteostatic Network Chaperome is Downregulated in F508del Homozygote Cystic Fibrosis
Authors Marc A Sala, Michael Alexander, Basil Khuder, Yuliya Politanska, Hiam Abdala-Valencia, G R Scott Budinger, Jing Liu, Manu Jain, Paul A Reyfman
References J Cyst Fibros. 2021 Mar;20(2):356-363. doi: 10.1016/j.jcf.2020.12.018. Epub 2021 Jan 23.
Keywords Chaperones, Ionocyte, nasal epithelium, Proteostasis, Transcriptomic
March 2021
Title: Adverse events in clinical trials in children with CF
Authors Jessica E Pittman, Umer Khan, Theresa A Laguna, Sonya Heltshe, Christopher H Goss, Don B Sanders
References J Cyst Fibros. 2021 Mar 18;S1569-1993(21)00048-5. doi: 10.1016/j.jcf.2021.02.013.
Keywords Clinical trial, Phase III, Study Design
March 2021
Title: Does Using Home Spirometry Lower Healthcare Costs of Care for people with cystic fibrosis?
Authors Natalie Franz, Hannah Rapp, Ryan N Hansen, Laura S Gold, Christopher H Goss, Noah Lechtzin, Larry G Kessler
References J Cyst Fibros. 2021 Mar 11;S1569-1993(21)00049-7. doi: 10.1016/j.jcf.2021.02.014.
Keywords Healthcare cost, Cost effectiveness, Home spirometry, Pulmonary exacerbations
Title: Efficacy and safety of inhaled dry-powder mannitol in adults with cystic fibrosis: An international, randomized controlled study
Authors Patrick A. Flume, Elena Amelina, Cori L. Daines, Brett Charlton, Joanna Leadbetter, Alessandro Guasconi, Moira L. Aitken
References J Cyst Fibros. 2021 Mar 11;S1569-1993(21)00046-1. doi: 10.1016/j.jcf.2021.02.011.
Keywords Mannitol, Respiratory function tests, Safety
March 2021
Title: The Effect of Oral and Intravenous Antimicrobials on Pulmonary Exacerbation Recovery in Cystic Fibrosis
Authors Eden J VanDevanter, Sonya L Heltshe, Michelle Skalland, Noah Lechtzin, Dave Nichols, Christopher H Goss
References J Cyst Fibros. 2021 Mar 5;S1569-1993(21)00047-3. doi: 10.1016/j.jcf.2021.02.012.
Keywords Exacerbations, Home spirometry, intravenous antibiotics, oral antibiotics
March 2021
Title: How important are different trials features when people decide whether to take part in cystic fibrosis clinical trials?
Authors Rebecca Anne Dobra, Marco Boeri, Stuart Elborn, Frank Kee, Susan Madge, Jane C Davies
References BMJ Open. 2021 Mar 2;11(3):e045803. doi: 10.1136/bmjopen-2020-045803.
Keywords clinical trials, statistics & research method
Title: Chronic incretin-based therapy in cystic fibrosis-related diabetes: a tale of 3 patients treated with sitagliptin for over 5 years
Authors Samuel T Olatunbosun
References J Cyst Fibros. 2021 Mar 2;S1569-1993(21)00040-0. doi: 10.1016/j.jcf.2021.02.005.
Keywords Chronic incretin-based therapy, Cystic-fibrosis related diabetes, Dipeptidyl peptidase-4 inhibitor, pancreatic insufficiency, Sitagliptin
Title: SLC26A9 SNP rs7512462 is not associated with lung disease severity or lung function response to ivacaftor in cystic fibrosis patients with G551D-CFTR
Authors Alice C Eastman, Rhonda G Pace, Hong Dang, Melis Atalar Aksit, Briana Vecchio-Pagán, Anh-Thu N Lam, Wanda K O'Neal, Scott M Blackman, Michael R Knowles, Garry R Cutting
References J Cyst Fibros. 2021 Mar 2;S1569-1993(21)00042-4. doi: 10.1016/j.jcf.2021.02.007.
Keywords G551D, Ivacaftor efficacy, Modifier genes, SLC26A9 rs7512462
February 2021
Title: “Il faut continuer à poser des questions” patient reported outcome measures in cystic fibrosis: An anthropological perspective
Authors Rosa Coucke, Audrey Chansard, Véronique Bontemps, Dominique Grenet, Dominique Hubert, Clémence Martin, Elise Lammertyn, Emmanuelle Bardin, Veerle Bulteel, Frédérique Chedevergne, Muriel Le Bourgeois, Pierre-Régis Burgel, Isabelle Honore, Hilde de Keyser, Maya Kirszenbaum, Paola de Carli, Isabelle Sermet-Gaudelus, Kate Hayes, European Cystic Fibrosis Society-Clinical Trials Network Patient Advisory Group
References J Cyst Fibros. 2021 Feb 26;S1569-1993(21)00044-8. doi: 10.1016/j.jcf.2021.02.009.
Keywords Patient reported outcome measures, Patient-led research
Title: Limb muscle size and contractile function in adults with cystic fibrosis: A systematic review and meta-analysis
Authors Kenneth Wu, Polyana L Mendes, Jenna Sykes, Anne L Stephenson, Sunita Mathur
References J Cyst Fibros. 2021 Feb 26;S1569-1993(21)00045-X. doi: 10.1016/j.jcf.2021.02.010
Keywords Adult, Muscle weakness, Muscular atrophy, Skeletal muscle
February 2021
Title: Defining key outcomes to evaluate performance of newborn screening programmes for cystic fibrosis
Authors Anne Munck, Kevin W Southern, Carlo Castellani, Karin M de Winter-de Groot, Silvia Gartner, Nataliya Kashirskaya, Barry Linnane, Sarah J Mayell, Marijke Proesmans, Dorota Sands, Olaf Sommerburg, Jürg Barben, European CF Society Neonatal Screening Working
References J Cyst Fibros. 2021 Feb 23;S1569-1993(21)00041-2. doi: 10.1016/j.jcf.2021.02.006.
Keywords CFSPID, Evaluation (3–6 words), Newborn screening, Outcome
Title: Performing an exercise capacity test, known as the modified shuttle test, can help to detect the risk of being hospitalized in youths with cystic fibrosis
Authors Márcio Vinícius Fagundes Donadio, Fernanda Maria Vendrusculo, Natália Evangelista Campos, Nicolas Acosta Becker, Ingrid Silveira de Almeida, Karen Caroline Vasconcelos Queiroz, Luanna Rodrigues Leite and Evanirso Silva Aquino
February 2021
Title: Outcomes of cystic fibrosis pulmonary exacerbations treated with antibiotics with activity against anaerobic bacteria
Authors Lauren M. Castner, Madsen Zimbric, Shannon Cahalan, Corey Powell, Lindsay J. Caverly
References J Cyst Fibros. 2021 Feb 18;S1569-1993(21)00036-9. doi: 10.1016/j.jcf.2021.02.001.
Keywords Anaerobes, Antibiotics, Exacerbations
February 2021
Title: Neutrophil Dysfunction in Cystic Fibrosis
Authors Lael M. Yonker, Anika Marand,, Sinan Muldur, Alex Hopke, Hui Min Leung, Denis De La Flor , Grace Park , Hanna Pinsky , Lauren B. Guthrie , Guillermo J. Tearney, Daniel Irimia, Bryan P. Hurley
References J Cyst Fibros. 2021 Feb 13;S1569-1993(21)00035-7. doi: 10.1016/j.jcf.2021.01.012.
Keywords Inflammation, Micro-fluidics, Micro-optical coherence tomography, Neutrophil
February 2021
Title: Airways glutathione S-transferase omega-1 and inflammation in cystic fibrosis
Authors Simona Piaggi, Elisabetta Marchi, Vittoria Carnicelli, Riccardo Zucchi, Matthias Griese, Andreas Hector, Claudio Sorio, Alfonso Pompella, Alessandro Corti
References J Cyst Fibros. 2021 Feb 11;S1569-1993(21)00033-3. doi: 10.1016/j.jcf.2021.01.010.
Keywords A140D polymorphism, FEV1, FEV1/FVC ratio, GSTO1-1, Glutathione S-transferase omega-1-1, Gamma-glutamyltransferase, lung inflammation
February 2021
Title: Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC Study
Authors Edith T. Zemanick, Michael W. Konstan, Donald R. VanDevanter, Steven M. Rowe, JP Clancy, Katherine Odem-Davis, Michelle Skalland, Nicole Mayer-Hamblett
References J Cyst Fibros. 2021 Feb 8;S1569-1993(21)00034-5. doi: 10.1016/j.jcf.2021.01.011.
Keywords CFTR modulator, Ivacaftor, Lumacaftor-ivacaftor, sweat chloride, Sweat test
February 2021
Title: Targeting G542X CFTR Nonsense Alleles With ELX-02 Restores CFTR Function in Human-Derived Intestinal Organoids
Authors Daniel K Crawford, Jasper Mullenders, Johanna Pott, Sylvia F Boj, Shira Landskroner-Eiger, Matthew M Goddeeris
References J Cyst Fibros. 2021 Feb 5;S1569-1993(21)00028-X. doi: 10.1016/j.jcf.2021.01.009.
Keywords ELX-02, NB124, Nonsense allele, Organoid, Read-through
January 2021
Title: Attitudes of Pain and Opioids Prescription Practices in U.S. Cystic Fibrosis Centers
Authors Yang Yaoli, Hollatz Trina, Mukadam Zubin, Laxova Anita, Decker Catherine, Braun Andrew T
References J Cyst Fibros. 2021 Jan;20(1):127-132. doi: 10.1016/j.jcf.2020.07.021. Epub 2020 Aug 11
Keywords Attitudes, Chronic pain, Opioid, Pain management
January 2021
Title: Experience of COVID-19 during the first wave in children with cystic fibrosis worldwide.
Authors Robert Bain, Rebecca Cosgriff, Marco Zampoli, Alexander Elbert, Pierre-Régis Burgel, Siobhán B Carr, Claudio Castaños, Carla Colombo, Harriet Corvol, Albert Faro, Christopher H Goss, Hector Gutierrez, Andreas Jung, Nataliya Kashirskaya, Bruce C Marshall, Joel Melo, Pedro Mondejar-Lopez, Isabelle de Monestrol, Lutz Naehrlich, Rita Padoan, Maria Dolores Pastor-Vivero, Samar Rizvi, Marco Salvatore, Luiz Vicente Ribeiro Ferreira da Silva Filho, Keith G Brownlee, Iram J Haq, Malcolm Brodlie
References J Cyst Fibros. 2021 Jan;20(1):25-30. doi: 10.1016/j.jcf.2020.11.021. Epub 2020 Dec 3.
Keywords COVID-19, Children
Title: Alpha-1 Antitrypsin for Cystic Fibrosis Complicated by Severe Cytokinemic COVID-19
Authors Oliver J McElvaney, Eoin O’Connor, Natalie L McEvoy, Daniel D Fraughan, Jennifer Clarke, Oisín F McElvaney, Cedric Gunaratnam, James O’Rourke, Gerard F Curley, Noel G McElvaney
References J Cyst Fibros. 2021 Jan;20(1):31-35. doi: 10.1016/j.jcf.2020.11.012.
Keywords Alpha-1 antitrypsin, Anti-inflammatory, COVID-19, Coronavirus, Cytokinemia, Inflammation, Interleukin-1β, Interleukin-6, Neutrophil elastase
Title: Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants
Authors Onofrio Laselva, Claire Bartlett, Alec Popa, Hong Ouyang, Tarini N A Gunawardena, Tanja Gonska, Theo J Moraes, Christine E Bear
References J Cyst Fibros. 2021 Jan;20(1):106-119. doi: 10.1016/j.jcf.2020.07.015. Epub 2020 Jul 30.
Keywords CFTR, Patient-derived nasal epithelial cultures, Personalised medicine, Rare mutations
Title: ACT with CF: A telehealth and in-person feasibility study to address anxiety and depressive symptoms among people with cystic fibrosis
Authors C Virginia O'Hayer, Caitlin M O'Loughlin, Chelsi N Nurse, Patrick J Smith, Michael J Stephen
References J Cyst Fibros. 2021 Jan;20(1):133-139. doi: 10.1016/j.jcf.2020.11.013. Epub 2020 Dec 2.
Keywords Lung function, acceptance and commitment therapy, anxiety, depressive symptoms, pilot study, psychotherapy
January 2021
Title: Comparing oral glucose tolerance test and continuous glucose monitoring
Authors H Elidottir, S Diemer, E Eklund, C R Hansen
References J Cyst Fibros. 2021 Jan 18;S1569-1993(21)00002-3. doi: 10.1016/j.jcf.2021.01.002.
Keywords Abnormal glucose tolerance, continuous glucose monitoring, Cystic fibrosis related diabetes, Lung clearance index (LCI), Lung function, Oral glucose tolerance test
January 2021
Title: Restoration of the chloride and bicarbonate (soda) transport function in cystic fibrosis by Kaftrio
Authors Marcel J C Bijvelds, Floris J M Roos, Kelly F Meijsen, Henk P Roest, Monique M A Verstegen, Hettie M Janssens, Luc J W van der Laan, Hugo R de Jonge
References J Cyst Fibros. 2021 Dec 15;S1569-1993(21)02165-2. doi: 10.1016/j.jcf.2021.12.006.
Keywords Bile ducts, CFTR modulators, Cholangiocytes, Intestine, Organoids
Theme Care of patients with CF, CFTR/Basic Science
Title: Changes in airway inflammation with pseudomonas eradication in early cystic fibrosis
Authors Luke W Garratt, Oded Breuer, Craig J Schofield, Samantha A McLean, Daniel R Laucirica, Rabindra Tirouvanziam, Barry S Clements, Anthony Kicic, Sarath Ranganathan, Stephen M Stick, On Behalf Of Arest CF
References J Cyst Fibros . 2021 Jan 15;S1569-1993(20)30947-4. doi: 10.1016/j.jcf.2020.12.015.
Keywords Bronchiectasis, CT, Inflammation, Neutrophil elastase, P. aeruginosa
January 2021
Title: Is hyperpolarised gas magnetic resonance imaging a valid and reliable tool to detect lung health in cystic fibrosis patients? A COSMIN systematic review.
Authors Fatmah Mallallah, Anna Packham, Ellen Lee, Daniel Hind
References J Cyst Fibros. 2021 Jan 13;S1569-1993(20)30952-8. doi: 10.1016/j.jcf.2020.12.020.
Keywords Construct validity, Content validity, FEV1, Helium, Hyperpolarised gas MRI, MRI; Reliability, Systematic review, Ventilation defect, Xenon
January 2021
Title: Designing the GALAXY study: Partnering with the cystic fibrosis community to optimize assessment of gastrointestinal symptoms
Authors A Jay Freeman, Meghana Sathe, Enid Aliaj, Drucy Borowitz, Barbra Fogarty, Christopher H Goss, Steven Freedman, Sonya L Heltshe, Umer Khan, Dara Riva, Christina Roman, Melita Romasco, Sarah Jane Schwarzenberg, Carmen A Ufret-Vincenty, Baha Moshiree
References J Cyst Fibros. 2021 Jan 12;S1569-1993(20)30953-X. doi: 10.1016/j.jcf.2020.12.021.
Keywords Community Involvement, Gastrointestinal, Patient reported outcome measures (proms)
January 2021
Title: Relationship between airway dysbiosis, inflammation and lung function in adults with cystic fibrosis
Authors Dario L Frey, Sébastien Boutin, Susanne A Dittrich, Simon Y Graeber, Mirjam Stahl, Sabine Wege, Felix J F Herth, Olaf Sommerburg, Carsten Schultz, Marcus A Mall, Alexander H Dalpke
References J Cyst Fibros. 2021 Jan 8;S1569-1993(20)30954-1. doi: 10.1016/j.jcf.2020.12.022.
Keywords Inflammation, Lung function, Microbiome
January 2021
Title: Significant functional differences in differentiated Conditionally Reprogrammed (CRC)- and Feeder-free Dual SMAD inhibited-expanded human nasal epithelial cells
Authors Nikhil T Awatade, Sharon L Wong, Alexander Capraro, Elvis Pandzic, Iveta Slapetova, Ling Zhong, Nihan Turgutoglu, Laura K Fawcett, Renee M Whan, Adam Jaffe, Shafagh A Waters
References J Cyst Fibros. 2021 Jan 4;S1569-1993(20)30951-6. doi: 10.1016/j.jcf.2020.12.019.
Keywords Significant functional differences in differentiated Conditionally Reprogrammed (CRC)- and Feeder-free Dual SMAD inhibited-expanded human nasal epithelial cells
December 2020
Title: Pilot study suggests that the gut bacteria in people with CF change with CFTR modulator therapy
Authors C E Pope, A T Vo, H S Hayden, E J Weiss, S Durfey, S McNamara, A Ratjen, B Grogan, S Carter, L Nay, M R Parsek, P K Singh, E F McKone, M L Aitken, M R Rosenfeld, L R Hoffman
References J Cyst Fibros. 2020 Dec 31;S1569-1993(20)30934-6. doi: 10.1016/j.jcf.2020.12.002.
Keywords Antibiotics, CFTR modulators, Dysbiosis, Fecal microbiome
December 2020
Title: Men's health in the modern era of cystic fibrosis
Authors F N Khan, V Tangpricha, K S Hughan, R Jain, S L Ladores, J L Taylor-Cousar, N E West, K Montemayor, G S Sawicki, A Uluer, M L Aitken, M Shnorhavorian, L A Bray, C Kvam, M Stalvey, T M Kazmerski
References J Cyst Fibros. 2020 Dec 26;S1569-1993(20)30945-0. doi: 10.1016/j.jcf.2020.12.013.
December 2020
Title: Psychological distress in adults with and without cystic fibrosis during the COVID-19 lockdown
Authors Riccardo Ciprandi, Maurizio Bonati, Rita Campi, Rita Pescini, Carlo Castellani
References J Cyst Fibros. 2020 Dec 23;S1569-1993(20)30948-6. doi: 10.1016/j.jcf.2020.12.016.
Keywords anxiety, COVID-19, depression, Pandemics, Psychosocial distress
December 2020
Title: Elexacafator/tezacaftor/ivacaftor Resolves Subfertility in Females with CF: A Two Center Case Series
Authors Kate E O'Connor, Dana L Goodwin, Andrew NeSmith, Bryan Garcia, Christina Mingora, Sigrid L Ladores, Steve M Rowe, Stefanie Krick, George M Solomon
References J Cyst Fibros. 2020 Dec 19;S1569-1993(20)30943-7. doi: 10.1016/j.jcf.2020.12.011.
Keywords CFTR modulators, Fertility
December 2020
Title: Vitamin D supplementation in patients with cystic fibrosis: A systematic review and meta-analysis
Authors Márk Félix Juhász, Orsolya Varannai, Dávid Németh, Zsolt Szakács, Szabolcs Kiss, Vera Dóra Izsák, Ágnes Rita Martonosi, Péter Hegyi, Andrea Párniczky
References J Cyst Fibros. 2020 Dec 18;S1569-1993(20)30940-1. doi: 10.1016/j.jcf.2020.12.008.
Keywords Meta-analysis, Nutrition, Systematic review, Vitamin D
Title: The association between the function of the cellular salt channel cystic fibrosis transmembrane conductance regulator and lung function in people with cystic fibrosis
Authors Nadine Bernasconi, Elisabeth Kieninger, Michelle Shaw, Johanna Kurz, Alexander Moeller, Felix Ratjen, Isabelle Rochat, Sanja Stanojevic, Florian Singer
References J Cyst Fibros. 2020 Dec 18;S1569-1993(20)30941-3. doi: 10.1016/j.jcf.2020.12.009.
Keywords Child, Pulmonary function, treatment
December 2020
Title: Association of Inhaled Antibiotics in Addition to Standard Intravenous Therapy and Outcomes of Pediatric Inpatient Pulmonary Exacerbations
Authors Jonathan D Cogen Anna V Faino, Frankline Onchiri, Lucas R Hoffman, Matthew P Kronman, Maria Nelson, David P Nichols, Margaret Rosenfeld, Donald R VanDevanter, Ronald L Gibson
References Ann Am Thorac Soc. 2020 Dec;17(12):1590-1598. doi: 10.1513/AnnalsATS.202002-179OC.
Keywords Antibiotics, Epidemiology, Pulmonary exacerbations
Theme Care of patients with CF, Lung Disease and Infection
December 2020
Title: Assessing the effect of adding chelating agent (Tris-CaEDTA) to nebulised tobramycin for improving bacterial killing and lung function in cystic fibrosis patients with Pseudomonas aeruginosa lung infection.
Authors Ramaa Puvvadi, Helga Mikkelsen, Lucy McCahon, Samantha Grogan, William Ditcham, David W Reid, Iain Lamont, Stephen M Stick, Barry Clements
References J Cyst Fibros. 2020 Dec 16;S1569-1993(20)30936-X. doi: 10.1016/j.jcf.2020.12.004.
Keywords EDTA, Pseudomonas aeruginosa, Tobramycin
Title: Ototoxicity in Cystic Fibrosis Patients Receiving Intravenous Tobramycin for Acute Pulmonary Exacerbation
Authors Emily Harruff, Jonathan Kil, Maria Gabriela Tupayachi Ortiz, Daniel Dorgan, Raksha Jain, Elizabeth A Poth, Robert C Fifer, Yun Jin M Kim, Angela G Shoup, Patrick A Flume
References J Cyst Fibros. 2020 Dec 16;S1569-1993(20)30930-9. doi: 10.1016/j.jcf.2020.11.020.
Keywords Aminoglycoside, Hearing loss, Ototoxicity, Tinnitus, Tobramycin
December 2020
Title: The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network
Authors Kelsie Pearson, Nicole Mayer-Hamblett, Christopher H Goss, George Z Retsch-Bogart, Jill M VanDalfsen, Patricia Burks, Daniel Rosenbluth, John Paul Clancy, Amy Hoffman, David P Nichols
References J Cyst Fibros. 2020 Dec 15;S1569-1993(20)30939-5. doi: 10.1016/j.jcf.2020.12.007.
Keywords Clinical research, Coronavirus, SARS-CoV-2
Title: A phase 3, randomized, double-blind, parallel-group study to evaluate tezacaftor/ivacaftor in people with cystic fibrosis heterozygous
Authors Edward F McKone, Emily A DiMango, Sivagurunathan Sutharsan, Tara Lynn Barto, Daniel Campbell, Neil Ahluwalia, Mark Higgins, Caroline A Owen, Elizabeth Tullis
References J Cyst Fibros. 2020 Dec 15;S1569-1993(20)30906-1. doi: 10.1016/j.jcf.2020.11.003.
Keywords CFTR gating mutation, Clinical trial, F508del-CFTR mutation, Ivacaftor, Tezacaftor
December 2020
Title: Impact of biofilm formation and azoles' susceptibility in Scedosporium/Lomentospora species using an in vitro model that mimics the cystic fibrosis patients' airway environment
Authors Thaís P Mello, Michaela Lackner, Marta H Branquinha, André L S Santos
References J Cyst Fibros. 2020 Dec 14;S1569-1993(20)30933-4. doi: 10.1016/j.jcf.2020.12.001.
Keywords Antifungal susceptibility, Azoles, Biofilm, Mucoviscidosis, Scedosporium/Lomentospora
December 2020
Title: Environmental risks of Pseudomonas aeruginosa-What to advise patients and parents
References J Cyst Fibros. 2020 Dec 12;S1569-1993(20)30937-1. doi: 10.1016/j.jcf.2020.12.005. Online ahead of print.
Keywords Environment, Infection, Pseudomonas, Soil, Water
December 2020
Title: Amphotericin B induces epithelial voltage responses in people with cystic fibrosis
Authors Rajeev S. Chorghade, Bo Ram Kim, Janice L. Launspach, Philip H. Karp, Michael J. Welsh, Martin D. Burke
References J Cyst Fibros. 2020 Dec 8;S1569-1993(20)30928-0. doi: 10.1016/j.jcf.2020.11.018.
December 2020
Title: Organoids for personalized treatment of Cystic Fibrosis: Professional perspectives on the ethics and governance of organoid biobanking
Authors Michael A Lensink, Sarah N Boers, Karin R Jongsma, Sarah E Carter, Cornelis K van der Ent, Annelien L Bredenoord
References J Cyst Fibros. 2020 Dec 7;S1569-1993(20)30921-8. doi: 10.1016/j.jcf.2020.11.015.
Keywords Biobanking, Ethics, Governance, Organoid technology, Precision medicine, Qualitative interview study
December 2020
Title: Phages as immunomodulators and their promising use as anti-inflammatory agents in a cftr loss-of-function zebrafish model
Authors Marco Cafora, Alessia Brix, Francesca Forti, Nicoletta Loberto, Massimo Aureli, Federica Briani, Anna Pistocchi
References J Cyst Fibros. 2020 Dec 6;S1569-1993(20)30927-9. doi: 10.1016/j.jcf.2020.11.017.
Keywords Inflammation; Innate immunity, Phages, Zebrafish
December 2020
Title: Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis
Authors Matthew M Willmering, David J Roach, Elizabeth L Kramer, Laura L Walkup, Zackary I Cleveland, Jason C Woods
References J Cyst Fibros. 2020 Dec 4;S1569-1993(20)30929-2. doi: 10.1016/j.jcf.2020.11.019.
Keywords Function, MRI, Multiple breath washout, Spirometry, Structure, Xenon
December 2020
Title: MyCyFapp clinical trial sub-study on change in PERT and fat absorption
Authors Joaquim Calvo-Lerma, Mieke Boon, Carla Colombo, Barbara de Koning, Inês Asseiceira, Maria Garriga, Maria Roca, Ine Claes, Anna Bulfamante, Sylvia Walet, Luisa Pereira, Mar Ruperto, Etna Masip, Andrea Asensio-Grau, Arianna Giana, Philine Affourtit, Ana Heredia, Saioa Vicente, Ana Andrés, Kris de Boeck, Jessie Hulst and Carmen Ribes Koninckx
References J Cyst Fibros. 2020 Dec 2;S1569-1993(20)30926-7. doi: 10.1016/j.jcf.2020.11.016.
Keywords Coefficient of fat absorption, Digestion, In vitro digestion, Paediatrics, Pancreatic enzyme replacement therapy, pancreatic insufficiency
Title: CFTR plays an important role in the regulation of vascular resistance and high-fructose/salt-diet induced hypertension in mice
Authors Ya-Ping Zhang, Lingyu Linda Ye, Hong Yuan, Dayue Darrel Duan
References J Cyst Fibros. 2020 Dec 2;S1569-1993(20)30920-6. doi: 10.1016/j.jcf.2020.11.014.
Keywords Cystic fibrosis transmembrane conductance regulator, Hypertension, Vascular tone, With-no-lysine kinase
December 2020
Title: YKL-40 as a clinical biomarker in adult patients with CF: Implications of a CHI3L1 single nucleotide polymorphism in disease severity.
Authors Adèle Coriati, Guillaume F Bouvet, Chantal Massé, Thierry Ducruet, Yves Berthiaume
References J Cyst Fibros. 2020 Dec 1;S1569-1993(20)30914-0. doi: 10.1016/j.jcf.2020.11.011.
Keywords CHI3L1, Disease severity, Single nucleotide polymorphism, YKL-40
November 2020
Title: Updated guidance on the care of children with an inconclusive diagnosis after newborn screening
Authors Jürg Barben, Carlo Castellani, Anne Munck, Jane C Davies, Karin M de Winter-de Groot, Silvia Gartner, Nataliya Kashirskaya, Barry Linnane, Sarah J Mayell, Susanna McColley, Chee Y Ooi, Marijke Proesmans, Clement L Ren, Danieli Salinas, Dorota Sands, Isabelle Sermet-Gaudelus, Olaf Sommerburg, Kevin W Southern. For the European CF Society Neonatal Screening Working Group (ECFS NSWG)
References J Cyst Fibros. 2020 Nov 27;S1569-1993(20)30909-7. doi: 10.1016/j.jcf.2020.11.006.
Keywords CFSPID, CRMS, Management, Newborn screening
November 2020
Title: The cystic fibrosis gut as a potential source of multidrug resistant pathogens
Authors Steven L Taylor, Lex E X Leong, Sarah K Sims, Rebecca L Keating, Lito E Papanicolas, Alyson Richard, Fredrick M Mobegi, Steve Wesselingh, Lucy D Burr, Geraint B Rogers
References J Cyst Fibros. 2020 Nov 26;S1569-1993(20)30912-7. doi: 10.1016/j.jcf.2020.11.009.
Keywords Anti-bacterial agents, Drug resistance, microbiota, Resistome
Title: Prostaglandin E2 and phagocytosis of inhaled particulate matter by airway macrophages in cystic fibrosis
Authors Norrice M Liu, Lisa Miyashita, Marek Sanak, Benjamin Barratt, Jonathan Grigg
References J Cyst Fibros. 2020 Nov 26;S1569-1993(20)30913-9. doi: 10.1016/j.jcf.2020.11.010.
Keywords Airway macrophage, Particulate matter, Phagocytosis, Prostaglandin E2
November 2020
Title: Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation
Authors J S Guimbellot, A Baines, A Paynter, S L Heltshe, J VanDalfsen, M Jain, S M Rowe, S D Sagel, GOAL-e2 Investigators
References J Cyst Fibros. 2020 Nov 25;S1569-1993(20)30911-5. doi: 10.1016/j.jcf.2020.11.008.
Keywords CFTR, Ivacaftor; Lung function, Pseudomonas aeruginosa, Quality of life, sweat chloride
Title: Lumacaftor/ivacaftor in people with cystic fibrosis with an A455E-CFTR mutation
Authors Gitte Berkers, Renske van der Meer, Harry Heijerman, Jeffrey M Beekman, Sylvia F Boj, Robert G J Vries, Peter van Mourik, Jamie R Doyle, Paul Audhya, Zheng Jason Yuan, Nils Kinnman, C Kors van der Ent
References J Cyst Fibros. 2020 Nov 25;S1569-1993(20)30910-3. doi: 10.1016/j.jcf.2020.11.007.
Keywords A455E–CFTR, Crossover study, Cystic fibrosis, Lumacaftor/ivacaftor
November 2020
Title: Long-term azithromycin use is not associated with QT prolongation in children with cystic fibrosis
Authors Amalia S Magaret, Jack Salerno, Jason F Deen, Margaret Kloster, Nicole Mayer-Hamblett, Bonnie W Ramsey, Dave P Nichols
References J Cyst Fibros. 2020 Nov 24;S1569-1993(20)30908-5. doi: 10.1016/j.jcf.2020.11.005.
Keywords Azithromycin, Pulmonary exacerbation, QT prolongation
November 2020
Title: Outcomes and endpoints reported in studies of pulmonary exacerbations in people with cystic fibrosis (CF): a systematic review
Authors Charlie McLeod, Jamie Wood, André Schultz, Richard Norman, Sherie Smith, Christopher C Blyth, Steve Webb, Alan R Smyth, Thomas L Snelling
References J Cyst Fibros. 2020 Nov;19(6):858-867. doi: 10.1016/j.jcf.2020.08.015. Epub 2020 Nov 13.
Keywords Endpoint measure, Outcome assessment, Outcome variables, Patient reported outcome measures, Pulmonary exacerbation
Title: Laboratory reporting on the clinical spectrum of CFTR p.Arg117His: still room for improvement
Authors Nele Laudus, Marie-Pierre Audrézet, Emmanuelle Girodon, Michael A Morris, Dragica Radojkovic, Caroline Raynal, Manuela Seia, Alexandra Štambergová, Heike Torkler, Raina Yamamoto, Elisabeth M C Dequeker
References J Cyst Fibros. 2020 Nov;19(6):969-974. doi: 10.1016/j.jcf.2020.05.005. Epub 2020 Jun 3
Keywords CFTR-related disorders, External quality assessment, P.Arg117His
Theme Care of patients with CF, Diagnosis/Screening
November 2020
Title: Impact of Socioeconomic Position on Access to the U.S. Lung Transplant Waiting List in a Matched Cystic Fibrosis Cohort
Authors Carli J Lehr, Aliza K Fink, Melissa Skeans, Albert Faro, Gabriela Fernandez, Elliott Dasenbrook, Maryam Valapour
References Ann Am Thorac Soc. 2020 Nov;17(11):1384-1392. doi: 10.1513/AnnalsATS.202001-030OC
Keywords Socioeconomic position, transplantation
Theme Care of patients with CF, Psychosocial, Transplantation
November 2020
Title: Predicting effects of mutations on CFTR’s mRNA
Authors Natacha Martin, Anne Bergougnoux, Nesrine Baatallah, Benoit Chevalier, Jessica Varilh, David Baux, Bruno Costes, Pascale Fanen, Caroline Raynal, Isabelle Sermet-Gaudelus, Emmanuelle Girodon, Magali Taulan-Cadars, Alexandre Hinzpeter
References J Cyst Fibros. 2020 Dec 16;S1569-1993(20)30935-8. doi: 10.1016/j.jcf.2020.12.003.
Keywords CFTR, Exon skipping, In silico predictions, Minigene, Splicing
November 2020
Title: Defective immunometabolism pathways in cystic fibrosis macrophages
Authors Kaitlin Hamilton, Kathrin Krause, Asmaa Badr, Kylene Daily, Shady Estfanous, Mostafa Eltobgy, Arwa Abu Khweek, Midhun N K Anne, Cierra Carafice, Daniel Baetzhold, Jeffrey R Tonniges, Xiaoli Zhang, Mikhail A Gavrilin, Narasimham L Parinandi, Amal O Amer
References J Cyst Fibros. 2020 Nov 15;S1569-1993(20)30880-8. doi: 10.1016/j.jcf.2020.10.006.
Keywords Bacterial infection, Immunometabolism, Macrophage, Mitochondria, Reactive oxygen species
November 2020
Title: Airway epithelial stem cell chimerism in cystic fibrosis lung transplant recipients
Authors Don Hayes J, Rachael E Rayner, Cynthia L Hill, Alfahdah Alsudayri, Mahelet Tadesse, Scott W Lallier, Hemant Parekh, Guy N Brock, Estelle Cormet-Boyaka, Susan D Reynolds
References J Cyst Fibros. 2020 Nov 10;S1569-1993(20)30881-X. doi: 10.1016/j.jcf.2020.09.013.
Keywords Airway epithelial stem cell, Basal cell, Chronic lung allograft dysfunction, Lung transplant
November 2020
Title: The virtual CF clinic – what this means for sputum microbiology
Authors John E Moore, B Cherie Millar, John McCaughan, Damian O'Neill, Jane Bell, Amanda Crossan, Jacqueline C Rendall
References J Cyst Fibros. 2020 Nov 6;S1569-1993(20)30879-1. doi: 10.1016/j.jcf.2020.10.005.
Keywords COVID19, Mail, Post, Sputum microbiology, Transportation, Virtual clinic
November 2020
Title: Early acquisition and conversion of Pseudomonas aeruginosa in Hispanic youth with cystic fibrosis in the United States
Authors Meghan E McGarry, Chiung-Yu Huang, Dennis W Nielson, Ngoc P Ly
References J Cyst Fibros. 2020 Nov 5;S1569-1993(20)30876-6. doi: 10.1016/j.jcf.2020.10.002.
Keywords health disparities, Pseudomonas aeruginosa
November 2020
Title: The global impact of SARS-CoV-2 in 181 people with cystic fibrosis
Authors Elliot McClenaghan, Rebecca Cosgriff, Keith Brownlee, Susannah Ahern, Pierre-Régis Burgel, Catherine A Byrnes, Carla Colombo, Harriet Corvol, Stephanie Y Cheng, Géraldine Daneau, Alexander Elbert, Albert Faro, Christopher H Goss, Vincent Gulmans, Hector Gutierrez, Isabelle de Monestrol, Andreas Jung, Lutz Nährlich, Nataliya Kashirskaya, Bruce C Marshall, Edward McKone, Peter G Middleton, Pedro Mondejar-Lopez, M Dolores Pastor-Vivero, Rita Padoan, Samar Rizvi, Rasa Ruseckaite, Marco Salvatore, Anne L Stephenson, Luiz Vicente R da Silva Filho, Joel Melo, Marco Zampoli, Siobhán B Carr, Global Registry Harmonization Group
References J Cyst Fibros. 2020 Nov 4;S1569-1993(20)30877-8. doi: 10.1016/j.jcf.2020.10.003
Keywords COVID-19, Coronavirus, SARS-CoV-2, Transplant
October 2020
Title: Long-term bone mineral density changes and fractures in lung transplant recipients with cystic fibrosis
Authors Gabrielle Durette, Valérie Jomphe, Nathalie J Bureau, Charles Poirier, Pasquale Ferraro, Larry C. Lands, Geneviève Mailhot
Keywords Lung transplant, bone mineral density, Vertebral fractures, Non-vertebral fractures
October 2020
Title: Prospective longitudinal association between repeated multiple breath washout measurements and computed tomography scores in children with cystic fibrosis
Authors Rikke Mulvad Sandvik, Thomas Kongstad, Kent Green, Christian Voldby, Frederik Buchvald, Marianne Skov, Tacjana Pressler, Kim Gjerum Nielsen
References J Cyst Fibros. 2020 Oct 4;2121. doi: 10.1016/j.jcf.2020.09.010.
Keywords Lung clearance index, Lung function, Multiple breath washout, PRAGMA-CF, Progression, Second moment ratio, Spirometry-controlled CT, Structural lung disease
October 2020
Title: Tobacco smoke exposure limits the therapeutic benefit of tezacaftor/ivacaftor in pediatric patients with cystic fibrosis
Authors Elizabeth Baker, William T Harris, Steven M Rowe, Sarah B Rutland, Gabriela R Oates
References J Cyst Fibros. 2020 Oct 3;S1569-1993(20)30870-5. doi: 10.1016/j.jcf.2020.09.011. Online ahead of print.
Keywords CFTR modulators, Secondhand smoke, Smoke exposure, Tezacaftor/ivacaftor, Tobacco
September 2020
Title: Safety and Efficacy of Lenabasum in a Phase 2 Randomized, Placebo-Controlled Trial in Adults with Cystic Fibrosis
Authors James F Chmiel, Patrick Flume, Damian G Downey, Allen J Dozor, Carla Colombo, Henryk Mazurek, Ewa Sapiejka, Marta Rachel, Scott Constantine, Brian Conley, Nancy Dgetluck, Quinn Dinh, Barbara White, J Stuart Elborn, Lenabasum JBT101-CF-001 Study Group
References J Cyst Fibros. 2020 Sep 30;S1569-1993(20)30873-0. doi: 10.1016/j.jcf.2020.09.008.
Keywords Lenabasum, Pulmonary exacerbations, Safety
September 2020
Title: Evidence of early increased sialylation of airway mucins and defective mucociliary clearance in CFTR-/- deficient piglets
Authors Ignacio Caballero, Bélinda Ringot-Destrez, Mustapha Si-Tahar, Pascal Barbry, Antoine Guillon, Isabelle Lantier, Mustapha Berri, Claire Chevaleyre, Isabelle Fleurot, Céline Barc, Reuben Ramphal, Nicolas Pons, Agnès Paquet, Kévin Lebrigand, Carole Baron, Andrea Bähr, Nikolai Klymiuk, Renaud Léonard, Catherine Robbe-Masselot
References J Cyst Fibros. 2020 Sep 23;S1569-1993(20)30868-7. doi: 10.1016/j.jcf.2020.09.009.
Keywords CFTR, Mucin glycosylation, Mucociliary transport, Sus scrofa
September 2020
Title: A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous for F508del or heterozygous for the F508del-CFTR mutati
Authors Jane C Davies, Isabelle Sermet-Gaudelus, Lutz Naehrlich, R Scott Harris, Daniel Campbell, Neil Ahluwalia, Christopher Short, Eric Haseltine, Paul Panorchan, Clare Saunders, Caroline A Owen, Claire E Wainwright, VX16-661-115 Investigator Group
References J Cyst Fibros . 2020 Sep 21;S1569-1993(20)30811-0. doi: 10.1016/j.jcf.2020.07.023.
Keywords Clinical trial, F508del-CFTR mutation, Ivacaftor, Lung clearance index, Residual function CFTR mutation, Tezacaftor
September 2020
Title: Tobacco Smoke Exposure and Socioeconomic Factors Are Independent Predictors of Pulmonary Decline in Pediatric Cystic Fibrosis
Authors Gabriela R Oates, Elizabeth Baker, Steven M Rowe, Hector H Gutierrez, Michael S Schechter, Wayne Morgan, William T Harris
References J Cyst Fibros. 2020 Sep;19(5):783-790. doi: 10.1016/j.jcf.2020.02.004. Epub 2020 Feb 17.
Theme Care of patients with CF, Psychosocial, Pulmonology
Title: Anti-inflammatory effects of lenabasum, a cannabinoid receptor type 2 agonist, on macrophages from cystic fibrosis
Authors Abdullah A. Tarique , Tama Evron, George Zhang, Mark A. Tepper, Mohammed M. Morshed, Isabella S.G. Andersen, Nelufa Begum, Peter D. Sly, Emmanuelle Fantino
References J Cyst Fibros. 2020 Sep;19(5):823-829. doi: 10.1016/j.jcf.2020.03.015. Epub 2020 May 6.
Keywords Anti-inflammatory, CB2 agonists, Cannabinoid 2 receptor (CB2), Endocytosis, Macrophage polarization, Macrophage responses, Phagocytosis
September 2020
Title: What is the value of lung function testing in infants with cystic fibrosis?
Authors M Pollak, M Shaw, S Balkovec, D Wilson, K Kowalik, P Subbarao, F Ratjen
References J Cyst Fibros. 2020 Sep 17;S1569-1993(20)30867-5. doi: 10.1016/j.jcf.2020.09.005. Online ahead of print.
September 2020
Title: Trends in nontuberculous mycobacteria infection in children and young people with cystic fibrosis
Authors Noreen Zainal Abidin, Aaron Ions Gardner, Hannah-Louise Robinson, Iram J Haq, Matthew F Thomas, Malcolm Brodlie
References J Cyst Fibros. 2020 Sep 16;S1569-1993(20)30872-9. doi: 10.1016/j.jcf.2020.09.007.
Keywords nontuberculous mycobacteria, Pediatrics
September 2020
Title: Sputum for viral diagnosis in cystic fibrosis patients
Authors Emilie Cardot-Martin, Hélène Le Guillou-Guillemette, Rozenn Le Berre, Sophie Ramel, Jean Le Bihan, Dominique Grenet, Eric Farfour, Françoise Troussier, Thierry Urban, Lisa Billard, Léa Pilorgé, Adissa Minoui-Tran, Christopher Payan, Marie-Reine Munck, Genevieve Héry-Arnaudak, and Sophie Valletai* on behalf of the MucoMicrobes study group
References J Cyst Fibros. 2020 Sep 14;S1569-1993(20)30865-1. doi: 10.1016/j.jcf.2020.09.003.
Keywords Influenza virus, Multiplex molecular diagnosis, Respiratory virus, Sputum, nasopharyngeal
September 2020
Title: Delayed glucose peak and elevated 1-hour glucose on the oral glucose tolerance test identify youth with cystic fibrosis with lower oral disposition index
Authors Kalie L Tommerdahl, John T Brinton, Tim Vigers, Melanie Cree-Green, Philip S Zeitler, Kristen J Nadeau, Christine L Chan
References J Cyst Fibros. 2020 Sep 11;2111. doi: 10.1016/j.jcf.2020.08.020. Online ahead of print.
Keywords Cystic fibrosis-related diabetes, Oral disposition index, Oral glucose tolerance test, Pediatrics, screening
