December 2022
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
October 2022
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
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: 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: 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: 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: 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: 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: 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: 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: 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: 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 Her
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, Isab
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: 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 G
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: 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, An
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: 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, Gen
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
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
September 2020
Title: Baseline CF disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression
Authors Malena Cohen-Cymberknoh, Bar Gindi Reiss, Joel Reiter, Noah Lechtzin, Joel Melo, Gema Pérez, Hannah Blau, Huda Mussaffi, Hagit Levine, Lea Bentur, Michal Gur, Galit Livnat, Javier Perez Miranda, Eva Polverino, Francesco Blasi, Stefano Aliberti, Micha Avir
References J Cyst Fibros. 2020 Sep 9;S1569-1993(20)30864-X. doi: 10.1016/j.jcf.2020.09.002.
September 2020
Title: Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients
Authors Amir Moheet, Daniel Beisang, Lin Zhang, Scott D Sagel, Jill M VanDalfsen, Sonya L Heltshe, Carla Frederick, Michelle Mann, Nicholas Antos, Joanne Billings, Steven M Rowe, Antoinette Moran, PROSPECT Investigators of the Cystic Fibrosis Foundation Therapeut
References J Cyst Fibros. 2020 Sep 8;S1569-1993(20)30862-6. doi: 10.1016/j.jcf.2020.09.001.
Keywords CFRD, insulin secretion, Orkambi
September 2020
Title: Factors influencing clinical trial participation for adult and pediatric patients with cystic fibrosis
Authors Marissa Lee, Xun Yang Hu, Sameer Desai, Eugenie Kwong, Joey Fu, Eri Flores, Lynda Lazosky, Pearce G Wilcox, Maggie Mcllwaine, Mark Chilvers, Connie Yang, Jonathan H Rayment, Bradley S Quon
References J Cyst Fibros. 2020 Sep 5;S1569-1993(20)30857-2. doi: 10.1016/j.jcf.2020.08.019. Online ahead of print.
Keywords Barriers, clinical trials, Facilitators, research
September 2020
Title: Aerosolized lancovutide in adolescents (≥12 years) and adults with cystic fibrosis - a randomized trial
Authors Ernst Eber, Maria Trawinska-Bartnicka, Dorota Sands, Gabriel Bellon, Uwe Mellies, Katalin Bolbás, Serena Quattrucci, Henryk Mazurek, Rudolf Widmann, Christian Schoergenhofer, Bernd Jilma, Felix Ratjen
References J Cyst Fibros. 2020 Sep 1;S1569-1993(20)30830-4. doi: 10.1016/j.jcf.2020.08.014.
Keywords Clinical trial, Lancovutide, Lung function, Quality of life
August 2020
Title: Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus
Authors Aderonke Sofoluwe, Alice Zoso, Marc Bacchetta, Sylvain Lemeille, Marc Chanson
References J Cyst Fibros. 2020 Aug 29;S1569-1993(20)30827-4. doi: 10.1016/j.jcf.2020.08.012. Online ahead of print.
Keywords Airway epithelium, Immune response, Pathogens, Transcriptome
August 2020
Title: Recommended shielding against COVID-19 impacts physical activity levels in adults with cystic fibrosis
Authors Thomas Radtke, Sarah R Haile, Holger Dressel, Christian Benden
References J Cyst Fibros. 2020 Aug 28;S1569-1993(20)30828-6. doi: 10.1016/j.jcf.2020.08.013
Keywords COVID-19, Coronavirus, Lung disease, Lung transplantation, SARS-CoV-2
August 2020
Title: Extensive CFTR sequencing through NGS in Brazilian individuals with cystic fibrosis: unravelling regional discrepancies in the country
Authors Luiz Vicente Ribeiro Ferreira da Silva Filho, Paulo José Cauduro Maróstica, Rodrigo Abensur Athanazio, Francisco José Caldeira Reis, Neiva Damaceno, Angela Tavares Paes, Adilson Yuuji Hira, David Schlesinger, Fernando Kok, Margarida D Amaral, Brazilian Cy
References J Cyst Fibros. 2020 Aug 17;S1569-1993(20)30822-5. doi: 10.1016/j.jcf.2020.08.007.
Keywords Brazil, Diagnosis, Genetics, Newborn screening, sweat chloride
August 2020
Title: The implementation of an aminoglycoside induced ototoxicity algorithm for people with cystic fibrosis
Authors E. Claire Elson, Ellen Meier, Christopher M. Oermann
References J Cyst Fibros. 2020 Aug 15;S1569-1993(20)30817-1. doi: 10.1016/j.jcf.2020.08.002. Online ahead of print.
Keywords Aminoglycoside, Ototoxicity
August 2020
Title: Long-term safety study of colistimethate sodium (Colobreathe®): Findings from the UK Cystic Fibrosis Registry
Authors Sigal Kaplan, Andrew Lee, Noreen Caine, Susan C Charman, Diana Bilton
References J Cyst Fibros. 2020 Aug 14;S1569-1993(20)30816-X. doi: 10.1016/j.jcf.2020.08.001. Online ahead of print.
Keywords Colistimethate sodium dry powder for inhalation, Colobreathe, Long-term safety, UK Cystic Fibrosis Registry
August 2020
Title: Changes in symptom scores as a potential clinical endpoint for studies of cystic fibrosis pulmonary exacerbation treatment
Authors D R VanDevanter, S L Heltshe, D B Sanders, N E West, M Skalland5, P A Flume, C H Goss, STOP-OB Study
References J Cyst Fibros. 2020 Aug 13;S1569-1993(20)30820-1. doi: 10.1016/j.jcf.2020.08.006. Online ahead of print.
Keywords clinical trials, Exacerbations, Symptoms
August 2020
Title: Decreased survival in cystic fibrosis patients with a positive screen for depression
Authors Michael S Schechter, Joshua S Ostrenga, Aliza K Fink, David H Barker, Gregory S Sawicki, Alexandra L Quittner
References J Cyst Fibros. 2020 Aug 12;S1569-1993(20)30812-2. doi: 10.1016/j.jcf.2020.07.020.
Keywords anxiety, depression, mental health, mortality
August 2020
Title: SARS-CoV-2 seroprevalence in a Belgian cohort of patients with cystic fibrosis
Authors Berardis S, Verroken A, Vetillart A, Struyf C, Gilbert M, Gruson D, Gohy S
References J Cyst Fibros. 2020 Aug 11;S1569-1993(20)30821-3. doi: 10.1016/j.jcf.2020.08.005
Keywords COVID-19, SARS-CoV-2, Seroprevalence
August 2020
Title: Screening for Nontuberculous mycobacteria (NTM) in people with Cystic Fibrosis
Authors Jerry A. Nick, Prithwiraj De, Delphi Chatterjee
References J Cyst Fibros. 2020 Aug; 9
August 2020
Title: Projecting the impact of delayed access to elexacaftor/tezacaftor/ivacaftor for people with Cystic Fibrosis
Authors Sanja Stanojevic, Katarina Vukovojac, Jenna Sykes, Felix Ratjen, Elizabeth Tullis, Anne L Stephenson
References J Cyst Fibros. 2020 Aug 5;S1569-1993(20)30809-2. doi: 10.1016/j.jcf.2020.07.017. Online ahead of print.
Keywords forecasting, Microsimulation, Modulators
August 2020
Title: Azithromycin and Tezacaftor/Ivacaftor is associated with first-degree heart block in an adult with Cystic Fibrosis
Authors Yang Song, Alexandra Coronel Palacios, Aravinda Thiagalingam, Peter G Middleton
References J Cyst Fibros. 2020 Aug 4;S1569-1993(20)30808-0. doi: 10.1016/j.jcf.2020.07.016. Online ahead of print.
Keywords Azithromycin, First degree heart block, Ivacaftor, Tezacaftor
August 2020
Title: Heat acclimation improves sweat gland function and lowers sweat sodium concentration in an adult with cystic fibrosis
Authors Ashley G B Willmott, Robert Holliss, Zoe Saynor, Jo Corbett, Adam J Causer, Neil S Maxwell
References J Cyst Fibros. 2020 Aug 2;S1569-1993(20)30804-3. doi: 10.1016/j.jcf.2020.07.013.
Keywords Adaptation, Heat acclimation, Heat stress, Sweat sodium concentration
July 2020
Title: Clinical characteristics and outcomes associated with Inquilinus infection in cystic fibrosis
Authors Patricia M Lenhart-Pendergrass, Lindsay J Caverly, Brandie D Wagner, Scott D Sagel, Jerry A Nick, John J LiPuma, Stacey L Martiniano
References J Cyst Fibros. 2020 Jul 31;S1569-1993(20)30803-1. doi: 10.1016/j.jcf.2020.07.011. Online ahead of print.
Title: The impact of the COVID-19 pandemic on the emotional well-being and home treatment of Belgian patients with cystic fibrosis, including transplanted patients and children
Authors Trudy Havermans, Janne Houben, Francois Vermeulen, Mieke Boon, Marijke Proesmans, Natalie Lorent, Erik de Soir, Robin Vos, Lieven Dupont
References J Cyst Fibros. 2020 Jul 31;S1569-1993(20)30815-8. doi: 10.1016/j.jcf.2020.07.022. Online ahead of print.
Keywords COVID-19, Home treatment, Lung transplantation, Psychological impact
July 2020
Title: Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis
Authors Julie Safirstein; Jonathan J. Grant; Emily Clausen; Deepika Savant; Rebecca Dezube; Gina Hong
References J Cyst Fibros. 2020 Jul 29;S1569-1993(20)30806-7. doi: 10.1016/j.jcf.2020.07.014.
July 2020
Title: Developing Innovative GastroEnterology Specialty Training: the DIGEST model for expanding cystic fibrosis gastroenterologic expertise
Authors Sarah Shrager Lusman, Drucy Borowitz, Bruce C Marshall, Michael R Narkewicz, Tanja Gonska, Richard J Grand, Richard H Simon, Maria R Mascarenhas, Sarah Jane Schwarzenberg, Steven D Freedman
References J Cyst Fibros. 2020 Jul 28;S1569-1993(20)30805-5. doi: 10.1016/j.jcf.2020.07.012.
Keywords Gastroenterology, Hepatology, Nutrition, Training
July 2020
Title: Citrullination of extracellular histone H3.1 reduces antibacterial activity and exacerbates its proteolytic degradation
Authors Lloyd Tanner, Ravi K V Bhongir, Christofer A Q Karlsson, Sandy Le, Johanna K Ljungberg, Pia Andersson, Cecilia Andersson, Johan Malmström, Arne Egesten, Andrew B Single
References J Cyst Fibros. 2020 Jul 26;S1569-1993(20)30802-X. doi: 10.1016/j.jcf.2020.07.010. Online ahead of print.
Keywords AMPs, Citrullination, Innate immunity, PADI4, Pseudomonas aeruginosa
July 2020
Title: Prospective cohort study of ototoxicity in persons with cystic fibrosis following a single course of intravenous tobramycin
Authors Angela Garinis, Malcolm Gleser, Alexis Johns, Erik Larsen, Jay Vachhani
References J Cyst Fibros . 2020 Jul 23;S1569-1993(20)30793-1. doi: 10.1016/j.jcf.2020.07.001. Online ahead of print.
Keywords Aminoglycosides, Hearing loss, Ototoxicity, Tobramycin
July 2020
Title: Screening practices for nontuberculous mycobacteria at US cystic fibrosis centers
Authors Derek Low, Dulaney A Wilson, Patrick A Flume
References J Cyst Fibros. 2020 Jul;19(4):569-574. doi: 10.1016/j.jcf.2020.02.013. Epub 2020 Mar 13.
Keywords Epidemiology, nontuberculous mycobacteria
July 2020
Title: Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis
Authors Emily DiMango, Jonathan Overdevest, Claire Keating, Sarah Fracasso Francis, David Dansky, David Gudis
References J Cyst Fibros. 2020 Jul 18;S1569-1993(20)30794-3. doi: 10.1016/j.jcf.2020.07.002.
Keywords Highly effective modulator, Quality of life, SNOT-22, Sinonasal
July 2020
Title: Evaluating assumptions of definition-based pulmonary exacerbation endpoints in cystic fibrosis clinical trials
Authors Donald R VanDevanter, Nicole Mayer Hamblett, Noah Simon, Joseph McIntosh, Michael W Konstan
References J Cyst Fibros. 2020 Jul 15;S1569-1993(20)30800-6. doi: 10.1016/j.jcf.2020.07.008.
July 2020
Title: Efficacy and safety of ataluren in patients with nonsense-mutation cystic fibrosis not receiving chronic inhaled aminoglycosides: the international, randomized, double-blind, placebo-controlled Ataluren Confirmatory Trial in Cystic Fibrosis (ACT CF)
Authors MW Konstan, DR VanDevanter, SM Rowe, M Wilschanski, E Kerem, I Sermet-Gaudelus, E DiMango, P Melotti, J McIntosh, K De Boeck on behalf of the ACT CF Study Group
References J Cyst Fibros. 2020 Jul;19(4):595-601. doi: 10.1016/j.jcf.2020.01.007. Epub 2020 Jan 23.
Keywords Ataluren, Clinical trial
Title: CFTR modulator therapy for cystic fibrosis caused by the rare c.3700A>G mutation
Authors Puay-Wah Phuan, Peter M Haggie, Joseph A Tan, Amber A Rivera, Walter E Finkbeiner, Dennis W Nielson, Merlin M Thomas, Ibrahim A Janahi, Alan S Verkman
References J Cyst Fibros . 2020 Jul 14;S1569-1993(20)30795-5. doi: 10.1016/j.jcf.2020.07.003.
Keywords C.3700A>G, CFTR modulators, Co-potentiators, I1234V, N-of-1 clinical trials
July 2020
Title: A four week trial of hypertonic saline in children with mild cystic fibrosis lung disease: Effect on mucociliary clearance and clinical outcomes
Authors Scott H Donaldson, T Danielle Samulski, Caroline LaFave, Kirby Zeman, Jihong Wu, Aaron Trimble, Agathe Ceppe, William D Bennett, Stephanie D Davis
References J Cyst Fibros. 2020 Jul 12;S1569-1993(20)30801-8. doi: 10.1016/j.jcf.2020.07.009.
Keywords clinical trials, Clinicaltrials.gov registration number, NCT01031706, Hypertonic saline, Mucociliary clearance
July 2020
Title: Evaluation of airway and circulating inflammatory biomarkers for cystic fibrosis drug development
Authors Raksha Jain, Arthur Baines, Umer Khan, Brandie D Wagner, Scott D Sagel
References J Cyst Fibros . 2020 Jul 1;S1569-1993(20)30782-7. doi: 10.1016/j.jcf.2020.06.017.
Keywords Biomarkers, Inflammation, Lung function, Sputum
June 2020
Title: A disruption of innervation around CF tissues results in low amount of VIP before CF disease has produced damage to the lungs and small intestine.
Authors Anna Semaniakou, Sarah Brothers, Grayson Gould, Mehrsa Zahiremani, Jamie Paton, Frederic Chappe, Audrey Li, Younes Anini, Roger P Croll, Valerie Chappe
References J Cyst Fibros. 2020 Jun 26;S1569-1993(20)30777-3. doi: 10.1016/j.jcf.2020.06.013. Online ahead of print
Keywords Acetylcholine, Innervation, Intestine, Mice, VIP
June 2020
Title: Fibrocyte accumulation in the lungs of cystic fibrosis patients
Authors Rajesh K Kasam, Prathibha R Gajjala, Anil G Jegga Jennifer A Courtney, Scott H Randell, Elizabeth L Kramer, John P Clancy, Satish K Madala
References J Cyst Fibros. 2020 Jun 24;S1569-1993(20)30756-6. doi: 10.1016/j.jcf.2020.06.011. Online ahead of print.
Keywords CFTR, Fibrocytes, lung, Pulmonary fibrosis
June 2020
Title: Tezacaftor/ivacaftor in people with cystic fibrosis who stopped lumacaftor/ivacaftor due to respiratory adverse events
Authors Carsten Schwarz, Sivagurunathan Sutharsan, Ralph Epaud, Ross C Klingsberg, Rainald Fischer, Steven M Rowe, Paul K Audhya, Neil Ahluwalia, Xiaojun You, Thomas J Ferro, Margaret E Duncan, Bote G Bruinsma
References J Cyst Fibros. 2020 Jun 23;S1569-1993(20)30730-X. doi: 10.1016/j.jcf.2020.06.001.
Keywords Clinical trial, Phase 3, Phe508del-CFTR
Title: Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function
Authors Pierre-Régis Burgel, Isabelle Durieu, Raphaël Chiron, Laurent Mely, Anne Prevotat, Marlene Murris-Espin, Michele Porzio, Michel Abely, Philippe Reix9, Christophe Marguet, Julie Macey, Isabelle Sermet-Gaudelus, Harriet Corvol, Stéphanie Bui, Tiphaine Biouh
References J Cyst Fibros. 2020 Jun 23;S1569-1993(20)30752-9. doi: 10.1016/j.jcf.2020.06.012. Online ahead of print.
June 2020
Title: Lumacaftor/ivacaftor improves liver cholesterol metabolism but does not influence hypocholesterolemia in patients with cystic fibrosis
Authors Monica Gelzo, Paola Iacotucci, Mafalda Caputo, Gustavo Cernera, Marika Comegna, Vincenzo Carnovale, Gaetano Corso,, Giuseppe Castaldoa
References J Cyst Fibros. 2020 Jun 22;S1569-1993(20)30779-7. doi: 10.1016/j.jcf.2020.06.015. Online ahead of print
Keywords Cholesterol homeostasis, Gas chromatography, Lumacaftor/ivacaftor, pancreatic insufficiency
June 2020
Title: Influenza B outbreak at an adult cystic fibrosis centre - clinical impact and factors influencing spread
Authors Jordan B Dennis, Andrew M Jones, Emma A Davies, William Welfare, Peter J Barry, Lisa Collier, Andrew Turner, Rowland J Bright-Thomas
References J Cyst Fibros. 2020 Jun 19;S1569-1993(20)30124-7. doi: 10.1016/j.jcf.2020.04.011.
Keywords Influenza, Lung function, Outbreak, Ventilation
