August 2021
Title: Clofazimine use for treatment of mycobacteria in children with and without cystic fibrosis
Authors L H Cameron, C A Peloquin, P Hiatt, M Mann, J R Starke, J Faircloth, J C McNeil, A Patel, F Ruiz
References J Cyst Fibros. 2021 Aug 31;S1569-1993(21)01351-5. doi: 10.1016/j.jcf.2021.08.010.
August 2021
Title: The influence of cystic fibrosis on work ability of affected patients
Authors Veruscka Leso, Rosaria Romano, Carolina Santocono, Marilisa Caruso, Paola Iacotucci, Vincenzo Carnovale, Ivo Iavicoli
References J Cyst Fibros. 2021 Aug 29;S1569-1993(21)01352-7. doi: 10.1016/j.jcf.2021.08.011.
Keywords Employment, Occupation, Professional life, Risk assessment and management, Work ability
August 2021
Title: Review of pregnancy in cystic fibrosis
Authors Raksha Jain, Traci M Kazmerski, Lisa C Zuckerwise, Natalie E West, Kristina Montemayor, Moira L Aitken, Edith Cheng, Andrea H Roe, Alexandra Wilson, Caitlin Mann, Sigrid Ladores, Jacqui Sjoberg, Madeline Poranski, Jennifer L Taylor-Cousar
References J Cyst Fibros. 2021 Aug 26;S1569-1993(21)01338-2. doi: 10.1016/j.jcf.2021.07.019.
Keywords Family planning, Fertility, Parenthood, pregnancy
August 2021
Title: Orkambi may improve liver disease in people with CF
Authors David Drummond, Jérémy Dana, Laureline Berteloot, Elena K. Schneider-Futschik, Frédérique Chedevergne, Céline Bailly-Botuha, Thao Nguyen-Khoa, Muriel Le Bourgeois, Dominique Debray, Muriel Girard, Isabelle Sermet-Gaudelus
References J Cyst Fibros. 2021 Aug 25;S1569-1993(21)01337-0. doi: 10.1016/j.jcf.2021.07.018.
Keywords CFTR modulator, Cystic-fibrosis liver disease, Cirrhosis, hepatobiliary, liver enzymes; pediatrics
August 2021
Title: Use of elexacaftor/tezacaftor/ivacaftor in liver transplant patients with cystic fibrosis
Authors Cameron J McKinzie, Christina T Doligalski, Steven J Lobritto, Raymond D Coakley, W Adam Gower
References J Cyst Fibros. 2021 Aug 9;S1569-1993(21)01336-9. doi: 10.1016/j.jcf.2021.07.017.
Keywords CFTR modulator; Cystic fibrosis; Liver transplantation
August 2021
Title: “Coping and Learning to Manage Stress with CF” a Telehealth Pilot Intervention
Authors Christina J Bathgate, Kristin M Kilbourn, Nora H Murphy, Frederick S Wamboldt, Kristen E Holm
References J Cyst Fibros. 2021 Aug 5;S1569-1993(21)01326-6. doi: 10.1016/j.jcf.2021.07.012.
Keywords anxiety, Cognitive behavioral therapy, Coping, depression, pilot study, RCT, Stress
Title: Do bone active therapy and lung disease influence the skeletal outcome after transplantation?
Authors Grassi Giorgia, Chiodini Iacopo, Cairoli Elisa, Morlacchi Letizia Corinna, Rossetti Valeria, Rosso Lorenzo, Righi Ilaria, Nosotti Mario, Arosio Maura, Blasi Francesco, Eller-Vainicher Cristina
References J Cyst Fibros. 2021 Aug 5;S1569-1993(21)01327-8. doi: 10.1016/j.jcf.2021.07.013
Keywords Lung transplantation, Trabecular bone score, Vertebral fractures
August 2021
Title: Is elexacaftor/tezacaftor/ivacaftor always better than ezacaftor/ivacaftor?
Authors Eva Furstova, Tereza Dousova, Jakub Beranek, Malgorzata Libik, Libor Fila, Martin Modrak, Ondrej Cinek, Milan Macek Jr, Pavel Drevinek
References J Cyst Fibros. 2021 Aug 1;S1569-1993(21)01304-7. doi: 10.1016/j.jcf.2021.07.006.
Keywords CFTR sequence, Elexacaftor, Intestinal organoids, Ivacaftor, Tezacaftor
July 2021
Title: Different approaches to Aspergillus-related conditions in people with cystic fibrosis
Authors Gina Hong, Sameer Desai, Richard B Moss, Patience Eschenhagen, Bradley S Quon, Carsten Schwarz
References J Cyst Fibros. 2021 Jul 28;S1569-1993(21)01306-0. doi: 10.1016/j.jcf.2021.07.008.
Keywords Allergic bronchopulmonary aspergillosis, Aspergillus bronchitis, Aspergillus colonization, Fungi
July 2021
Title: Urinary tract infections in cystic fibrosis patients
Authors Seth A Reasoner, Kyle T Enriquez, Benjamin Abelson, Steven Scaglione, Bennett Schneier, Michael G O'Connor, Gerald Van Horn, Maria Hadjifrangiskou
References J Cyst Fibros. 2021 Jul 27;S1569-1993(21)01303-5. doi: 10.1016/j.jcf.2021.07.005
Keywords Antibiotic resistance, Cystitis, Urinary tract infection
July 2021
Title: Utilization of Electronic Patient-Reported Outcome Measures in Cystic Fibrosis Research
Authors Meghana Sathe, Baha Moshiree, Phuong T Vu, Umer Khan, Sonya L Heltshe, Melita Romasco, Steven D Freedman, Sarah Jane Schwarzenberg, Christopher H Goss, A Jay Freeman
References J Cyst Fibros. 2021 Jul 22;S1569-1993(21)01299-6. doi: 10.1016/j.jcf.2021.07.002.
Keywords Electronic PROMs, Patient reported outcomes measures, gastrointestinal manifestations of cystic fibrosis
July 2021
Title: Stopping exposure to smoke improves breathing and nutritional outcomes in children and adolescents with cystic fibrosis
Authors Gabriela R Oates, Elizabeth Baker, Joseph M Collaco, Steven M Rowe, Sarah B Rutland, Christopher M Fowler, William T Harris
References J Cyst Fibros. 2021 Jul;20(4):618-624. doi: 10.1016/j.jcf.2021.06.014. Epub 2021 Jul 17.
Keywords Secondhand smoke, Smoke exposure, Smoking cessation, Tobacco
July 2021
Title: Disease burden in people with cystic fibrosis heterozygous for F508del and a minimal function mutation
Authors Gregory S. Sawicki, Kate Van Brunt, Jason Booth, Evan Bailey, Stefanie J. Millar, Michael W. Konstan, Patrick A. Flume
References J Cyst Fibros. 2021 Jul 19;S1569-1993(21)01300-X. doi: 10.1016/j.jcf.2021.07.003.
Title: Disease burden in people with cystic fibrosis heterozygous for F508del and a minimal function mutation
Authors Gregory S. Sawicki, Kate Van Brunt, Jason Booth, Evan Bailey, Stefanie J. Millar, Michael W. Konstan, Patrick A. Flume
References J Cyst Fibros. 2021 Jul 19;S1569-1993(21)01300-X. doi: 10.1016/j.jcf.2021.07.003.
Keywords F508del-CFTR; Minimal function; Observational study
July 2021
Title: Drug-Induced Liver Injury from Elexacaftor/Ivacaftor/Tezacaftor
Authors Sarah Lowry; Peter J. Mogayzel, Jr.; Kiyoko Oshima; Wikrom Karnsakul
References J Cyst Fibros. 2021 Jul 16;S1569-1993(21)01297-2. doi: 10.1016/j.jcf.2021.07.001.
Keywords CFTR modulator, Drug-induced liver injury
July 2021
Title: A Short extension to multiple breath washout provides additional indications of blocked and severely diseased airways in people with CF: a pilot study
Authors Christopher Short, Thomas Semple, Clare Saunders, Dominic Hughes, Samantha Irving, Laura Gardener, Mark Rosenthal, Paul D Robinson, Jane C Davies
References J Cyst Fibros. 2021 Jul 15;S1569-1993(21)01298-4. doi: 10.1016/j.jcf.2021.06.013.
Keywords Air Trapping, Biomarkers, Computed Tomography (CT), Lung clearance index (LCI), Lung Function Testing, Multiple breath washout (mbw), Pulmonary function testing (pft), Pulmonary physiology
July 2021
Title: Opioid Pain Management Among Adults with CF: Survey Results
Authors Sarah Allgood, Jessica L Zemlak, Elisabeth Dellon, Siddhartha G Kapnadak, Jessica Goggin, Noah Lechtzin
References J Cyst Fibros. 2021 Jul 10;S1569-1993(21)01291-1. doi: 10.1016/j.jcf.2021.06.007.
Keywords Adults, Opioids, Pain
July 2021
Title: Genetics of immunity against the Pseudomonas aeruginosa bacteria in people with cystic fibrosis
Authors Janardan P Pandey, Aryan M Namboodiri, Paul J Nietert, Michael R Knowles, Rhonda G Pace, Gerald B Pier
References J Cyst Fibros. 2021 Jul 7;S1569-1993(21)01295-9. doi: 10.1016/j.jcf.2021.06.011.
Keywords GM/KM allotypes, IgG antibodies, Pseudomonas aeruginosa
June 2021
Title: How does the cystic fibrosis drug ivacaftor move in the body during pregnancy and breastfeeding?
Authors Fiona Qiu, Mark D Habgood, Yifan Huang, Katarzyna M Dziegielewska, Sam Toll, Elena K Schneider-Futschik
References J Cyst Fibros. 2021 Jun 27;S1569-1993(21)01289-3. doi: 10.1016/j.jcf.2021.06.005.
Keywords Breastfeeding, Cystic fibrosis transmembrane conductance regulator, Ivacaftor, pregnancy, blood brain barrier
June 2021
Title: Use of LCI to monitor CFTR Modulator effect in people with CF
Authors Philippe Reix, Aurélie Tatopoulos, Iulia Ioan, Muriel Le Bourgeois, Stéphanie Bui, Marie Luce Choukroun, Katia Bessaci-Kabouya, Michele Gerardin, Plamen Bokov, Jennifer Da Silva, Jean-Louis Paillasseur, Pierre Regis Burgel,, French Cystic Fibrosis Reference Network study group
References J Cyst Fibros. 2021 Jun 25;S1569-1993(21)01286-8. doi: 10.1016/j.jcf.2021.06.002.
Keywords CFTR modulators, Lumacaftor, Multiple breath washout
June 2021
Title: Effect of elexacaftor-texacaftor-ivacaftor in cystic fibrosis patients with advanced lung disease
Authors Bermingham, B.; Rueschhoff, A. Ratti, G. Nesmith, A., Goodwin D, Gray S, Flume P, Solomon, G. M., Cohen, L., Garcia, B.
References J Cyst Fibros. 2021 Jun 21;S1569-1993(21)00160-0. doi: 10.1016/j.jcf.2021.05.009
June 2021
Title: Using an Antibiotic Treatment Called Amikacin Liposome Inhalation Suspension (or ALIS) for Chronic Infection With Pseudomonas aeruginosa in People With Cystic Fibrosis for 2 years
Authors Diana Bilton, Isabelle Fajac, Tacjana Pressler, John Paul Clancy, Dorota Sands, Predrag Minic, Marco Cipolli, Ivanka Galeva, Amparo Solé, Alexandra L Quittner, Zhanna Jumadilova, Monika Ciesielska, Michael W Konstan, CLEAR-110 Study Group
References J Cyst Fibros. 2021 Jun 15;S1569-1993(21)00164-8. doi: 10.1016/j.jcf.2021.05.013.
Keywords ALIS, Amikacin, Liposomal, Pseudomonas aeruginosa
June 2021
Title: Complications relating to the heart and blood vessels in people with Cystic Fibrosis: A Review of the Literature
Authors T. Spencer Poore, Jennifer Taylor-Cousar, Edith Zemanick
References J Cyst Fibros. 2021 Jun 14;S1569-1993(21)00126-0. doi: 10.1016/j.jcf.2021.04.016.
Keywords CFTR, Cardiovascular disease, sildenafil
June 2021
Title: Cystic fibrosis-related diabetes (CFRD) and cognitive ability (memory, attention and task switching) in adults with cystic fibrosis
Authors Helen K Chadwick, Janice Abbott, Margaret Anne Hurley, Louise Dye, Clare L Lawton, Michael W Mansfield, Daniel Peckham
References J Cyst Fibros. 2021 Jun 13;S1569-1993(21)00124-7. doi: 10.1016/j.jcf.2021.04.014.
Keywords Cognition, Cognitive tests, Cystic fibrosis-related diabetes, Executive function
Title: Liver toxicity with triple therapy in cystic fibrosis: when it is worth taking the risk.
Authors Dimitri Stylemans, Silke François, Stefanie Vincken, Sylvia Verbanck, Eef Vanderhelst
References J Cyst Fibros. 2021 Jun 13;S1569-1993(21)00174-0. doi: 10.1016/j.jcf.2021.05.017.
June 2021
Title: New drug for cystic fibrosis does not significantly interfere with another drug that prevents organ rejection
Authors Megan Smith, Kevin J Ryan, Hector Gutierrez, Luz Helena Gutierrez Sanchez, Janaina Nogueira Anderson, Edward P Acosta, Kim W Benner, Jennifer S Guimbellot
References J Cyst Fibros. 2021 Jun 12;S1569-1993(21)00159-4. doi: 10.1016/j.jcf.2021.05.008.
Keywords CFTR, Cytochrome p450, Drug interaction, Elexacaftor, Ivacaftor, Liver transplantation, Tacrolimus, Tezacaftor
June 2021
Title: What do different bacteria together do to the lung epithelium in Cystic Fibrosis
Authors Bertelsen, J Stuart Elborn, Bettina C Schock
References J Cyst Fibros. 2021 Jun 7;S1569-1993(21)00122-3. doi: 10.1016/j.jcf.2021.04.012.
Keywords Airway microbiome, Anaerobic bacteria, Cystic Fibrosis Airways inflammation, Host-pathogen-interaction, Mixed infection, Prevotella spp., Signalling
June 2021
Title: Comparison of gene editing methods to correct the W1282X mutation
Authors Lúcia Santos, Karen Mention, Kader Cavusoglu-Doran, David J Sanz, Mafalda Bacalhau, Miquéias Lopes-Pacheco, Patrick T Harrison, Carlos M Farinha
References J Cyst Fibros. 2021 Jun 5;S1569-1993(21)00167-3. doi: 10.1016/j.jcf.2021.05.014.
Keywords CFTR, CRISPR/Cas12a, CRISPR/Cas9, human bronchial epithelial cells, W1282X
Title: Appearance and behaviour of methicillin resistant Staphylococcus aureus (MRSA) from early and chronic infection in cystic fibrosis (CF)
Authors Deirdre Gilpin, Lucas R Hoffman, Agathe Ceppe, Marianne S Muhlebach
References J Cyst Fibros. 2021 Jun 5;S1569-1993(21)00168-5. doi: 10.1016/j.jcf.2021.05.015.
Keywords Adaptation, Anaerobe, Antimicrobial resistance, Haemolysis, Outcome, Staphylococcus aureus
June 2021
Title: Effect of inhaled antibiotic therapy with levofloxacin in people with cystic fibrosis
Authors Carsten Schwarz, Claudia Grehn, Svenja Temming, Frederik Holz, Patience Ndidi Eschenhagen
References J Cyst Fibros. 2021 Jun 4;S1569-1993(21)00051-5. doi: 10.1016/j.jcf.2021.03.002.
Keywords chronic Pseudomonas aeruginosa infection, Inhaled antibiotics, Levofloxacin solution, Pulmonary function test, exacerbation rate
June 2021
Title: Can a new blood test be used to screen for CF-related diabetes ?
Authors Tom Toin, Quitterie Reynaud, Angélique Denis, Isabelle Durieu, Catherine Mainguy, Catherine Llerena, Isabelle Pin, Sandrine Touzet, Philippe Reix
References J Cyst Fibros. 2021 Jun 2;S1569-1993(21)00161-2. doi: 10.1016/j.jcf.2021.05.010.
Keywords CFRD, Diabetes, HOMA, Homeostasis, Indice
Title: Evaluating Pulmonary Exacerbation Treatment Differences in Women and Men with CF
Authors Kristina Montemayor, Kevin J Psoter, Noah Lechtzin, Sara W Carson, Christian A Merlo, Rebecca H Dezube, Kristin A Riekert, Sarah Allgood, Alexandra Toporek, Mark T Jennings, Natalie E West
References J Cyst Fibros. 2021 Jun 2;S1569-1993(21)00163-6. doi: 10.1016/j.jcf.2021.05.012.
Keywords Pulmonary exacerbations, Sex differences, Treatment patterns
June 2021
Title: Respiratory symptoms are not more frequent or severe in infants with CF compared to healthy infants.
Authors Insa Korten, Marc-Alexander Oestreich, Urs Frey, Alexander Moeller, Andreas Jung, Renate Spinas, Dominik Mueller-Suter, Daniel Trachsel, Isabelle Rochat, Ben Spycher, Philipp Latzin, Carmen Casaulta, Kathryn Ramsey
References J Cyst Fibros. 2021 Jun 1;S1569-1993(21)00116-8. doi: 10.1016/j.jcf.2021.04.006.
Keywords Cohort study, Healthy controls, Infancy, Respiratory symptoms
May 2021
Title: The effect of lumacaftor-ivacaftor on mucus clearance
Authors Scott H. Donaldson, Beth L. Laube, Peter Mogayzel, Timothy E. Corcoran, Joseph M. Pilewski, Agathe Ceppe, Jihong Wu, Pradeep G. Bhambhvani, Felix Ratjen, Scott D. Sagel, J.P. Clancy, Steven M. Rowe*, William D. Bennett* *on behalf of the PROSPECT Investig
References J Cyst Fibros. 2021 May 31;S1569-1993(21)00131-4. doi: 10.1016/j.jcf.2021.05.004.
May 2021
Title: How frequent is the condition of overweight among patients with cystic fibrosis in Italy?
Authors Andrea Gramegna, Stefano Aliberti, Martina Contarini, Daniela Savi, Giovanni Sotgiu, Fabio Majo, Laura Saderi, Vincenzina Lucidi, Francesco Amati, Maria Pappalettera, Paolo Palange, Francesco Blasi
References J Cyst Fibros. 2021 May 27;S1569-1993(21)00129-6. doi: 10.1016/j.jcf.2021.05.002.
Keywords Adults, Overweight
May 2021
Title: Sleep in children and youth with cystic fibrosis: A review of the research
Authors Jordana McMurray, Kimberley Widger, Anne L Stephenson, Robyn Stremler
References J Cyst Fibros. 2021 May 23;S1569-1993(21)00132-6. doi: 10.1016/j.jcf.2021.05.005.
May 2021
Title: Pseudomonas aeruginosa infection, but not CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis
Authors Dirk Westhölter, Hendrik Beckert, Svenja Straßburg, Matthias Welsner, Sivagurunathan Sutharsan, Christian Taube, Sebastian Reuter
References J Cyst Fibros. 2021 May 21;S1569-1993(21)00128-4. doi: 10.1016/j.jcf.2021.05.001.
Keywords CFTR modulator therapy, Pseudomonas aeruginosa, Cytokines, lymphocytes, non-CF bronchiectasis, regulatory T cells
May 2021
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
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: 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,
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,
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: 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: 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.
