December 2020
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
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 Aviram, Inbal Golan Tripto, Elie Picard, Michal Novoselsky, Hagai Amsalem, Drorit Ochner, Eitan Kerem, Michal Shteinberg
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, Therapeutics Development Network
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 Cystic Fibrosis Patient Registry Contributors Team
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 Biouhee, Dominique Hubert, Anne Munck, Lydie Lemonnier, Clémence Dehillotte, Jennifer Da Silva, Jean-Louis Paillasseur, Clémence Martin, French Cystic Fibrosis Reference Network study group
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
June 2020
Title: In vitro 3D culture lung model from expanded primary cystic fibrosis human airway cells
Authors Rachael E Rayner, Jack Wellmerling, Wissam Osman, Sean Honesty, Maria Alfaro, Mark E Peeples, Estelle Cormet-Boyaka
References J Cyst Fibros. 2020 Jun 18;S1569-1993(20)30151-X. doi: 10.1016/j.jcf.2020.05.007. Online ahead of print.
Keywords CFTR ion channel, Cell expansion, In vitro, Lung model
June 2020
Title: Antimicrobial resistance: concerns of healthcare providers and people with CF
Authors Wendy Bullington, Sarah Hempstead, Alan R Smyth, Pavel Drevinek, Lisa Saiman, Valerie J Waters, Scott C Bell, Donald R VanDevanter, Patrick A Flume, Stuart Elborn, Marianne S Muhlebach, Antimicrobial Resistance International Working Group in Cystic Fibrosis
References J Cyst Fibros . 2020 Jun 17;S1569-1993(20)30159-4. doi: 10.1016/j.jcf.2020.05.009
Keywords Antimicrobial resistance, Survey
June 2020
Title: Postprandial changes in gastrointestinal function and transit in cystic fibrosis assessed by Magnetic Resonance Imaging
Authors Christabella Ng, Neele S Dellschaft, Caroline L Hoad, Luca Marciani, Lu Ban, Andrew P Prayle, Helen L Barr, Anke Jaudszus, Jochen G Mainz, Robin C Spiller, Penny Gowland, Giles Major, Alan R Smyth
References J Cyst Fibros. 2020 Jun 16;S1569-1993(20)30733-5. doi: 10.1016/j.jcf.2020.06.004.
Keywords Gastrointestinal function, Gastrointestinal symptoms, MRI
June 2020
Title: Newborn screening alone insufficient to improve pulmonary outcomes for cystic fibrosis
Authors Christina B Barreda, Philip M Farrell, Anita Laxova, Jens C Eickhoff, Andrew T Braun, Ryan J Coller, Michael J Rock
References J Cyst Fibros . 2020 Jun 13;S1569-1993(20)30731-1. doi: 10.1016/j.jcf.2020.06.002.
Title: The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis
Authors Leah Cuthbertson, Imogen Felton, Phillip James, Michael J Cox, Diana Bilton, Silke Schelenz, Michael R Loebinger, William O C Cookson, Nicholas J Simmonds, Miriam F Moffatt
References J Cyst Fibros. 2020 Jun 13;S1569-1993(20)30163-6. doi: 10.1016/j.jcf.2020.05.013.
Keywords Chronic airway disease, Filamentous fungi, Fungal airway disease, Mycobiome
Title: Immunotyping of clinically divergent p.Phe508del homozygous monozygous cystic fibrosis twins
Authors Esther Schamschula, Wolfgang Hagmann, Yassen Assenov, Silke Hedtfeld, Ahmed K Farag, Lennart M Roesner, Lutz Wiehlmann, Frauke Stanke, Sebastian Fischer, Angela Risch, Burkhard Tümmler
References J Cyst Fibros. 2020 Jun 13;S1569-1993(20)30754-2. doi: 10.1016/j.jcf.2020.06.009.
Keywords DNA methylation, Epigenetics, T cell receptor repertoire, Twin research
Title: Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations
Authors Anne Munck, Eitan Kerem, Helmut Ellemunter, Daniel Campbell, Linda T Wang, Neil Ahluwalia, Caroline A Owen, Claire Wainwright
References J Cyst Fibros . 2020 Jun 13;S1569-1993(20)30128-4. doi: 10.1016/j.jcf.2020.04.015.
Keywords Ivacaftor; Tezacaftor
June 2020
Title: Defective BACH1/HO-1 regulatory circuits in cystic fibrosis bronchial epithelial cells
Authors Shashipavan Chillappagari, Virajith Garapati, Poornima Mahavadi, Lutz Naehrlich, Bernd T Schmeck, M Lienhard Schmitz, Andreas Guenther
References J Cyst Fibros . 2020 Jun 11;S1569-1993(20)30150-8. doi: 10.1016/j.jcf.2020.05.006. Online ahead of print.
Keywords ACH1, Hemeoxygenase-1, MicroRNAs, Regulatory circuits
Title: Accumulation and persistence of Ivacaftor in airway epithelia with prolonged treatment
Authors Tara N Guhr Lee, Deborah M Cholon, Nancy L Quinney, Martina Gentzsch, Charles R Esther Jr
References J Cyst Fibros . 2020 Jun 11;S1569-1993(20)30123-5. doi: 10.1016/j.jcf.2020.04.010.
Keywords CFTR modulator, F508del, Ivacaftor, Pharmacokinetics
June 2020
Title: Clinimetric evaluation of muscle function tests for individuals with cystic fibrosis: A systematic review
Authors Yann Combret, Clement Medrinal, Tristan Bonnevie, Francis-Edouard Gravier, Pascal Le Roux, Bouchra Lamia, Guillaume Prieur, Gregory Reychler
References J Cyst Fibros . 2020 Jun 10;S1569-1993(20)30164-8. doi: 10.1016/j.jcf.2020.05.014.
Keywords Clinimetric properties, Limb muscles, Muscle function, Respiratory muscles
June 2020
Title: Building global development strategies for cf therapeutics during a transitional cftr modulator era
Authors N Mayer-Hamblett, S van Koningsbruggen-Rietschel, D P Nichols, D R VanDevanter, J C Davies, T Lee, A G Durmowicz, F Ratjen, M W Konstan, K Pearson, S C Bell, J P Clancy, J L Taylor-Cousar, K De Boeck, S H Donaldson, D G Downey, P A Flume, P Drevinek, C H Goss, I Fajac, A S Magaret, B S Quon, S M Singleton, J M VanDalfsen, G Z Retsch-Bogart
References J Cyst Fibros . 2020 Jun 7;S1569-1993(20)30161-2. doi: 10.1016/j.jcf.2020.05.011. Online ahead of print.
Keywords CFTR modulators, clinical trials, drug development, Global perspective
June 2020
Title: Pseudomonas aeruginosa antimicrobial susceptibility test (AST) results and pulmonary exacerbation treatment responses in cystic fibrosis
Authors Donald R. VanDevanter, Sonya L. Heltshe, Jay B. Hilliard, Michael W. Konstan
References J Cyst Fibros . 2020 Jun 4;S1569-1993(20)30152-1. doi: 10.1016/j.jcf.2020.05.008
Keywords Pseudomonas aeruginosa, Pulmonary exacerbation, Susceptibility testing
June 2020
Title: Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation
Authors Gitte Berkers, Renske van der Meer, Peter van Mourik, Annelotte M Vonk, Evelien Kruisselbrink, Sylvia Wf Suen, Harry Gm Heijerman, Christof J Majoor, Gerard H Koppelman, Jolt Roukema, Hettie M Janssens, Yolanda B de Rijke, E Marleen Kemper, Jeffrey M Beekman, Cornelis K van der Ent, Hugo R de Jonge
References J Cyst Fibros. 2020 Jun 1;S1569-1993(20)30127-2. doi: 10.1016/j.jcf.2020.04.014. Online ahead of print.
Keywords CFTR, Curcumin, Genistein, Ivacaftor, Organoids
May 2020
Title: A longitudinal assessment of non-Invasive biomarkers to diagnose and predict Ccystic Fibrosis-Associated Liver Disease
Authors Wikrom Karnsakul, Paul Wasuwanich, Thammasin Ingviya, Alexandra Vasilescu, Kathryn A Carson, Peter J Mogayzel, Kathleen B Schwarz
References J Cyst Fibros . 2020 May 30;S1569-1993(20)30133-8. doi: 10.1016/j.jcf.2020.05.002.
Keywords blood chemical analysis, blood platelets, liver cirrhosis, respiratory tract infections, transferases
Title: Disruption of the extracellular polymeric network of Pseudomonas aeruginosa biofilms by alginate lyase enhances pathogen eradication by antibiotics
Authors Said Daboor, John Rohde, Zhenyu Cheng
References J Cyst Fibros . 2020 May 30;S1569-1993(20)30119-3. doi: 10.1016/j.jcf.2020.04.006.
Keywords Alginate lyase, Antibiotics, Biofilm, Pseudomonas aeruginosa
Title: Metabolomics profiling of tobacco exposure in children with cystic fibrosis
Authors Benjamin L. Wisniewski, Chandra L. Shrestha, Shuzhong Zhang, Rohan Thompson, Myron Gross, Judith A. Groner, Karan Uppal, Octavio Ramilo, Asuncion Mejias, and Benjamin T. Kopp
References J Cyst Fibros . 2020 May 30;S1569-1993(20)30134-X. doi: 10.1016/j.jcf.2020.05.003.
Keywords Metabolites, Tobacco
May 2020
Title: Circulating CRP and calprotectin to diagnose CF pulmonary exacerbations
Authors David Jung, Kang Dong, Jiah Jang, Grace Y. Lam, Pearce G. Wilcox, Bradley S. Quon
References J Cyst Fibros. 2020 May 28;S1569-1993(20)30129-6. doi: 10.1016/j.jcf.2020.04.016.
Keywords Biomarkers, Diagnosis, Exacerbations, Inflammation
May 2020
Title: Testicular Pain Following Initiation of Elexacaftor/Tezacaftor/Ivacaftor in Males With Cystic Fibrosis
Authors Shannon M Rotolo, Stephanie Duehlmeyer, Sarah M Slack, Hollyann R Jacobs, Brian Heckman
References J Cyst Fibros. 2020 May 26;S1569-1993(20)30130-2. doi: 10.1016/j.jcf.2020.04.017
Keywords Adverse reactions, Congenital bilateral absence of the vas deferens, Drug-related side effects, Elexacaftor, Infertility, Male
May 2020
Title: Global assessment of the integrated stress response in CF patient-derived airway and intestinal tissues
Authors Giovana B. Bampi, Robert Rauscher, Sebastian Kirchner, Kathryn E. Oliver, Marcel J.C. Bijvelds, Leonardo A. Santos, Johannes Wagner, Raymond A. Frizzell, Hugo R. de Jonge, Eric J. Sorscher, Zoya Ignatova
References J Cyst Fibros. 2020 May 22;S1569-1993(20)30118-1. doi: 10.1016/j.jcf.2020.04.005.
Keywords ER stress, F508del-CFTR, Inflammation, RNA-seq, Tissue remodeling, Unfolded protein response (UPR)
May 2020
Title: Females with Cystic Fibrosis have a larger decrease in sweat chloride in response to lumacaftor/ivacaftor compared to males
Authors B L Aalbers, R W Hofland, I Bronsveld, K M de Winter-de Groot, H G M Arets, A C de Kiviet, M M M van Oirschot-van de Ven, M A Kruijswijk, S Schotman, S Michel, C K van der Ent, H G M Heijerman
References J Cyst Fibros. 2020 May 21;S1569-1993(20)30135-1. doi: 10.1016/j.jcf.2020.05.004
Keywords BMI, CFTR modulators, Lumacaftor/ivacator, sweat chloride, Sweat test, Treatment response, ppFEV1
May 2020
Title: Distal Intestinal Obstruction Syndrome resolved by dissolution with Coca-Cola® via colonoscopy in a cystic fibrosis patient
Authors Carlos Ferre-Aracil, Mariano González-Haba, Beatriz Tormo-Lanseros, Luis Giménez-Alvira, Manuel Jiménez-Garrido
References J Cyst Fibros . 2020 May 20;S1569-1993(20)30132-6. doi: 10.1016/j.jcf.2020.05.001. Online ahead of print.
Keywords Coca-Cola®, Distal intestinal obstruction syndrome, Fecal impaction, therapy
May 2020
Title: Multivariate joint modeling to identify markers of growth and lung function decline that predict cystic fibrosis pulmonary exacerbation onset
Authors E R Andrinopoulou, J P Clancy, R D Szczesniak
References BMC Pulm Med. 2020 May 19;20(1):142. doi: 10.1186/s12890-020-1177-z.
Keywords Dynamic prediction, functional data analysis, Medical monitoring, Multivariate longitudinal data, Registry analysis, Time-to-event
Theme Care of patients with CF, Pulmonology
Title: Glucose ingestion in cystic fibrosis induces severe redox imbalance: a potential role in diabetes
Authors William R. Hunt, Jason M. Hansen, Arlene A. Stecenko
References J Cyst Fibros. 2020 May;19(3):476-482. doi: 10.1016/j.jcf.2020.02.010. Epub 2020 Feb 27.
Keywords Cystic fibrosis-related diabetes, Redox
Title: Effect of one-year lumacaftor-ivacaftor treatment on glucose tolerance abnormalities in cystic fibrosis patients
Authors Bastien Misgault, Eva Chatron, Quitterie Reynaud, Sandrine Touzet, Michel Abely, Laurent Melly, Stéphane Dominique, Françoise Troussier, Olivia Ronsin-Pradel, Michèle Gerardin, Julie Mankikian, Laure Cosson, Raphael Chiron, Leila Bounyar, Michel Porzio, Isabelle Durieu, Laurence Weiss, Romain Kessler, Laurence Kessler
References J Cyst Fibros . 2020 Mar 19;S1569-1993(20)30073-4. doi: 10.1016/j.jcf.2020.03.002.
Keywords CFTR corrector and potentiator, Glucose tolerance abnormalities, OGTT
May 2020
Title: Average Rate of Lung Function Decline in Adults with Cystic Fibrosis in the United Kingdom: Data from the UK CF Registry
Authors L Caley, L Smith, H White, D G Peckham
References J Cyst Fibros. 2020 May 4;S1569-1993(20)30121-1. doi: 10.1016/j.jcf.2020.04.008.
Keywords Lung function, Rate of decline
