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 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: 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: 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: 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: 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: 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: 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
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 Fibros
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: 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
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 Beek
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: 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, I
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
April 2020
Title: A multinational report to characterise SARs-CoV-2 infection in people with cystic fibrosis
Authors Rebecca Cosgriff, Susannah Ahern, Scott C Bell, Keith Brownlee, Pierre-Régis Burgel, Cass Byrnes, Harriet Corvol, Stephanie Y Cheng, Alexander Elbert, Albert Faro, Christopher H Goss, Vincent Gulmans, Bruce C Marshall, Edward McKone, Peter G Middleton, , Rasa Ruseckaiteb, Anne L. Stephensonfm, Siobhán B Carr
References J Cyst Fibros. 2020 Apr 25;S1569-1993(20)30125-9. doi: 10.1016/j.jcf.2020.04.01
Keywords Coronavirus, SARS-CoV-2
April 2020
Title: Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis
Authors M. Boon, J. Calvo-Lerma, I Claes, T. Havermans, I. Asseiceira, A. Bulfamante, M. Garriga, E. Masip, B.A.M. van Schijndel, V. Fornes, C. Barreto, C. Colombo, P. Crespo, S. Vicente, H. Janssens, J. Hulst, P. Witters, R. Nobili, L. Pereira, M. Ruperto,, E Van der Wiel, J G Mainz, K De Boeck, C Ribes-Koninckx
References J Cyst Fibros . 2020 Jul;19(4):562-568. doi: 10.1016/j.jcf.2020.04.001. Epub 2020 Apr 22
Keywords CFAbd-Score, Mobile application, MyCyFAPP, PERT, pancreatic insufficiency, PedsQL GI, Quality of life
April 2020
Title: “Mild” CFTR genetic variants might be associated with a lower than expected probability of developing CF
Authors A Boussaroque, M-P Audrézet, C Raynal, I Sermet-Gaudelus, T Bienvenu, C Férec, A Bergougnoux, M Lopez, V Scotet, A Munck, E Girodon
References J Cyst Fibros . 2020 Apr 20;S1569-1993(20)30098-9. doi: 10.1016/j.jcf.2020.03.019.
Keywords CFSPID, CFTR, Inconclusive cases, Newborn screening, Penetrance
April 2020
Title: Resting energy expenditure in cystic fibrosis patients decreases after lung transplantation, which improves applicability of prediction equations for energy requirement
Authors Francis M Hollander-Kraaijeveld, Anne-Sophie van Lanen, Nicole M de Roos, Ed A van de Graaf, Harry GM Heijerman
References J Cyst Fibros. 2020 Apr 11. pii: S1569-1993(20)30092-8. doi: 10.1016/j.jcf.2020.03.013.
April 2020
Title: Caring for gender diverse youth with cystic fibrosis
Authors Kacie M. Kidd; Gina M. Sequeira; Raina V. Voss; Daniel Weiner; Bonnie W. Ramsey; Raksha Jain; Traci M. Kazmerski
References J Cyst Fibros. 2020 Apr 5;S1569-1993(20)30074-6. doi: 10.1016/j.jcf.2020.03.003.
March 2020
Title: Antibiofilm and mucolytic action of nitric oxide delivered as a gas or via macromolecular donor using in vitro and ex vivo models
Authors Kaitlyn R. Rouillard, David B. Hill, Mark H. Schoenfisch
References J Cyst Fibros. 2020 Mar 20;S1569-1993(20)30075-8. doi: 10.1016/j.jcf.2020.03.004
Keywords Biofilm, Mucolytic, Mucus, Nitric oxide, Pseudomonas aeruginosa
Title: Go for It, Dream Big, Work Hard and Persist': A Message to the Next Generation of CF Leaders in Recognition of International Women's Day 2020
Authors Mona Al Langawi, Cass Byrnes, Jane C Davies, Samia Hamouda, Madhulika Kabra, Samia Z Rached, Dorota Sands, Michal Shteinberg, Jennifer Taylor-Cousar, Elizabeth Tullis, Claire Wainwright
References J Cyst Fibros. 2020 Mar 7;S1569-1993(20)30070-9. doi: 10.1016/j.jcf.2020.02.021.
Keywords Career, Childcare, Gender, Leadership, Promotion, women
March 2020
Title: Total bacterial load, inflammation, and structural lung disease in paediatric cystic fibrosis
Authors Steven L. Taylor, Lex E.X. Leong, Kerry L. Ivey, Steve Wesselingh, Keith Grimwood, Claire E. Wainwright, Geraint B. Rogers, On behalf of the Australasian Cystic Fibrosis Bronchoalveolar Lavage (ACFBAL) study group
References J Cyst Fibros. 2020 Mar 18; - DOI: 10.1016/j.jcf.2020.03.008
Keywords Bronchoalveolar lavage, Computed tomography, Infection, microbiota, Paediatric, quantitative polymerase chain reaction
March 2020
Title: Intrinsic Alterations in Peripheral Neutrophils From Cystic Fibrosis Newborn Piglets
Authors Déborah Bréa, Laura Soler, Isabelle Fleurot, Sandrine Melo, Claire Chevaleyre, Mustapha Berri, Valérie Labas, Ana-Paula Teixeira-Gomes, Julien Pujo, Nicolas Cenac, Andrea Bähr, Nikolai Klymiuk, Antoine Guillon, Mustapha Si-Tahar, Ignacio Caballero
References J Cyst Fibros. 2020 Mar 9;S1569-1993(20)30065-5. doi: 10.1016/j.jcf.2020.02.016.
Keywords CFTR, ICM-MS, Neutrophils, Pig model
March 2020
Title: Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey
Authors Edward F Nash, Peter G Middleton, Jennifer L Taylor-Cousar
References J Cyst Fibros 2020 Mar 6 - doi: 10.1016/j.jcf.2020.02.018.
Keywords Breastfeeding, CFTR, infant, Modulators, pregnancy
March 2020
Title: Ebselen attenuates tobramycin-induced ototoxicity in mice
Authors Rende Gu, Ryan J Longenecker, Jennifer Homan, Jonathan Kil
References J Cyst Fibros. 2020 Mar 5;S1569-1993(20)30063-1. doi: 10.1016/j.jcf.2020.02.014.
Keywords Aminoglycoside, Auditory brainstem response, Ebselen, Hearing loss, Ototoxicity, Tobramycin
February 2020
Title: Finding the Relevance of Antimicrobial Stewardship for Cystic Fibrosis
Authors Jonathan D Cogen, Barbara C Kahl, Holly Maples, Susanna A McColley, Jason A Roberts, Kevin L Winthrop, Andrew M Morris, Alison Holmes, Patrick A Flume, Donald R VanDevanter, Valerie Waters, Marianne S Muhlebach, J Stuart Elborn, Lisa Saiman, Scott C Bell, on behalf of the Antimicrobial Resistance International Working Group in Cystic Fibrosis
References J Cyst Fibros. 2020 Feb 29;S1569-1993(20)30059-X. doi: 10.1016/j.jcf.2020.02.012.
Keywords Antimicrobials, Antimicrobial stewardship, Pulmonary exacerbations
February 2020
Title: Glucose abnormalities detected by continuous glucose monitoring are common in young children with Cystic Fibrosis
Authors Bernadette J Prentice, Chee Y Ooi, Charles F Verge, Shihab Hameed, John Widger
References J Cyst Fibros. 2020 Feb 25;S1569-1993(20)30057-6. doi: 10.1016/j.jcf.2020.02.009.
Keywords Child, Cystic fibrosis-related diabetes, Diabetes, glucose
Theme False-positives, Imaging, Immunology, Lung Disease and Infection, Microbiology, Newborn screening, Nutrition/Gastroenterology, Parents, Physiotherapy/Exercise testing, Pregnancy with CF, Psychosocial, Pulmonology
February 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 Feb 17;S1569-1993(20)30049-7. doi: 10.1016/j.jcf.2020.02.004.
February 2020
Title: Amplifiers co-translationally enhance CFTR biosynthesis via PCBP1-mediated regulation of CFTR mRNA
Authors Danijela Dukovski, Adriana Villella, Cecilia Bastos, Randall King, Daniel Finley, Jeffery W Kelly, Richard I Morimoto, F Ulrich Hartl, Benito Munoz, Po-Shun Lee, Marija Zecevic, John Preston Miller
References J Cyst Fibros . 2020 Feb 14;S1569-1993(20)30052-7. doi: 10.1016/j.jcf.2020.02.006.
Keywords Amplifiers, CFTR modulators, Co-translational regulation, MRNA stabilization, PCBP1
Title: In Vivo demonstration of Pseudomonas aeruginosa biofilms as independent pharmacological microcompartments
Authors Lars Christophersen, Franziska Angelika Schwartz, Christian Johann Lerche, Trine Svanekjær, Kasper Nørskov Kragh, Anne Sofie Laulund, Kim Thomsen , Kaj-Åge Henneberg, Thomas Sams, Niels Høiby, Claus Moser
References J Cyst Fibros . 2020 Feb 14;S1569-1993(20)30033-3. doi: 10.1016/j.jcf.2020.01.009.
Keywords Alginate beads, Biofilm model, Chronic infection, Independent pharmacological compartment, Pharmacodynamics, Pharmacokinetics
February 2020
Title: Predictive Effects of Low Birth Weight and Small for Gestational Age Status on Respiratory and Nutritional Outcomes in Cystic Fibrosis
Authors Samar E Atteih, Karen S Raraigh, Scott M Blackman, Garry R Cutting, Joseph M Collaco
References J Cyst Fibros 2020 Feb 12 - DOI: 10.1016/j.jcf.2020.02.003
Keywords BMI, FEV1, birth weight, gestational age, neonate
Title: Lymphocyte responses to Mycobacterium tuberculosis and Mycobacterium bovis are similar between BCG-vaccinated patients with cystic fibrosis and healthy controls
Authors Renan M Mauch, Paulo César M Alves, Carlos E Levy, José D Ribeiro, Antônio F Ribeiro, Niels Høiby, Marcos T Nolasco da Silva
References J Cyst Fibros 2020 Feb 12 DOI: 10.1016/j.jcf.2020.01.013
Keywords BCG, Flow cytometry, lung infection, Mycobacterium tuberculosis, nontuberculous mycobacteria
February 2020
Title: Minimal change in structural, functional and inflammatory markers of lung disease in newborn screened infants with cystic fibrosis at one year
Authors Gwyneth Davies], Lena P Thia], Janet Stocks, Andrew Bush, Ah-Fong Hoo, Angie Wade, The Thanh Diem Nguyen, Alan S Brody, Alistair Calde] , Nigel J Klein, Siobhán B Carr, Colin Wallis, Ranjan Suri, Caroline S Pao, Gary Ruiz, Ian M Balfour-Lynn, on behalf of London Cystic Fibrosis Collaboration (LCFC)
References J Cyst Fibros . 2020 Feb 7;S1569-1993(20)30031-X. doi: 10.1016/j.jcf.2020.01.006.
Keywords Airway inflammation, Chest computed tomography, Infant lung function, Neutrophil elastase; Newborn screening
Title: Pseudomonas Aeruginosa Antibody Response in Cystic Fibrosis Decreases Rapidly Following Lung Transplantation
Authors Hanna Ferløv Schwensen, Claus Moser, Michael Perch, Tacjana Pressler, Niels Høiby
References J Cyst Fibros 2020 Feb 7 - DOI: 10.1016/j.jcf.2020.01.012
Keywords Humoral response, Lung transplantation, Pseudomonas aeruginosa
January 2020
Title: Identification of Mycobacterium Porcinum in Patients With Cystic Fibrosis: Pathogen or Contaminant?
Authors Grace R Paul, Amy Leber, Christopher J Nemastil, Kimberly J Novak, Michael Brady, Stephanie Stack-Simone, Alexander L Greninger, Stella Antonara
References J Cyst Fibros 2020 Jan 22 - 10.1016/j.jcf.2020.01.004
Keywords Mycobacterium porcinum, Nontuberculous mycobacterium, Whole genome sequencing
January 2020
Title: Glycaemic control and FEV1 recovery during pulmonary exacerbations in paediatric cystic fibrosis-related diabetes
Authors William Okoniewski, Kara S Hughan, Gabriel A Weiner, Daniel J Weiner, Erick Forno
References J Cyst Fibros 2020 Jan 21 - DOI: 10.1016/j.jcf.2019.12.016
Keywords Cystic fibrosis-related diabetes, Glycemic control, Lung function
January 2020
Title: Cystic Fibrosis Related Diabetes; Glucose challenge test; Oral glucose tolerance test.
Authors Saba Sheikh, A Russell Localio, Andrea Kelly, Ronald C Rubenstein
References Saba Sheikh, A Russell Localio, Andrea Kelly, Ronald C Rubenstein
Keywords Cystic fibrosis related diabetes, Glucose challenge test, Oral glucose tolerance test
January 2020
Title: Pregnancy outcomes in women with cystic fibrosis and poor pulmonary function
Authors Quitterie Reynaud, Christine Rousset Jablonski, Stéphanie Poupon-Bourdy, Angélique Denis, Muriel Rabilloud, Lydie Lemonnier, Raphaële Nove-Josserand, Stéphane Durupt, Sandrine Touzet, Isabelle Durieu, participating centers of the French Cystic Fibrosis Re
References J Cyst Fibros. 2020 Jan;19(1):80-83. doi: 10.1016/j.jcf.2019.06.003. Epub 2019 Jul 1.
Keywords CFTR modulators, Maternal decline, pregnancy, Pregnancy outcomes, Pulmonary function
Title: Inactivation of CFTR by CRISPR/Cas9 alters transcriptional regulation of inflammatory pathways and other networks
Authors Shuyu Hao, Erica A Roesch, Aura Perez, Rebecca L Weiner, Leigh C Henderson, Linda Cummings, Paul Consiglio, Joseph Pajka, Amy Eisenberg, Lauren Yeh, Calvin U Cotton, Mitchell L Drumm
References J Cyst Fibros. 2020 Jan;19(1):34-39. doi: 10.1016/j.jcf.2019.05.003.
January 2020
Title: Sputum trypsin-like protease activity relates to clinical outcome in cystic fibrosis.
Authors James A. Reihill, Kelly L. Moffitt, Lisa E.J. Douglas, J. Stuart Elborn, Andrew M. Jones, S. Lorraine Martin
References J Cyst Fibros 2020 Jan 10 - DOI: 10.1016/j.jcf.2019.12.014
Keywords ENaC, Lung function, Patient survival, Trypsin-like proteases
January 2020
Title: Clinical effect of lumacaftor/ivacaftor in F508del homozygous CF patients with FEV1 ≥90% predicted at baseline
Authors BL Aalbers, KM de Winter-de Groot, HGM Arets, RW Hofland, AC de Kiviet, MMM van Oirschot-van de Ven, MA Kruijswijk, S Schotman, S Michel, CK van der Ent, HGM Heijerman
References J Cyst Fibros 2020 Jan 7 - DOI: 10.1016/j.jcf.2019.12.015
January 2020
Title: Developing a smartphone application to support social connectedness and wellbeing in young people with cystic fibrosis
Authors Jacinta Francis; Donna Cross; Andre Schultz; David Armstrong; Rebecca Nguyen; Cindy Branch-Smith
References J Cyst Fibros 2020 Jan 6 - DOI: 10.1016/j.jcf.2019.12.011
Keywords Adolescents, mobile applications, Smartphone, young people
January 2020
Title: Adherence to lumacaftor-ivacaftor therapy in patients with cystic fibrosis in France
Authors Lucie Olivereau, Viviane Nave, Stephan Garcia, Marie Perceval, Muriel Rabilloud, Isabelle Durieu, Quitterie Reynaud
References J Cyst Fibros. 2020 Jan 2 doi: 10.1016/j.jcf.2019.09.018. Online ahead of print.
Keywords Adherence, CFTR correctors, Lumacaftor-ivacaftor
December 2019
Title: Rapid lung function decline in adults with early-stage cystic fibrosis lung disease
Authors Elliott C Dasenbrook, Aliza K Fink, Michael S Schechter, Don B Sanders, Stefanie J Millar, David J Pasta, Nicole Mayer-Hamblett
References J Cyst Fibros 2019 Dec 20 - DOI: 10.1016/j.jcf.2019.12.005
Keywords Adult, cohort studies; respiratory function tests, Risk factors
Title: Utilizing centralized biorepository samples for biomarkers of cystic fibrosis lung disease severity
Authors Scott D Sagel, Brandie D Wagner, Assem Ziady, Tom Kelley, John P Clancy, Monica Narvaez-Rivas, Joseph Pilewski, Elizabeth Joseloff, Wei Sha, Leila Zelnick, Kenneth D R Setchell, Sonya L Heltshe, Marianne S Muhlebach
References J Cyst Fibros 2019 Dec 20 - DOI: 10.1016/j.jcf.2019.12.007
Keywords Biomarkers, Lipids, Metabolomics, Proteins
December 2019
Title: Prevalence of unmet palliative care needs in adults with cystic fibrosis
Authors Elizabeth T Trandel, Joseph M Pilewski, Elisabeth P Dellon, Kwonho Jeong, Jonathan G Yabes, Laura T Moreines, Robert M Arnold, Zachariah P Hoydich, Dio Kavalieratos
References J Cyst Fibros. 2019 Dec 18;S1569-1993(19)30978-6. doi: 10.1016/j.jcf.2019.11.010
Keywords Needs assessment, Palliative care, Patient-reported outcomes, Quality of life
December 2019
Title: Structured Surveillance of Achromobacter, Pandoraea and Ralstonia Species From Patients in England With Cystic Fibrosis
Authors Amy Coward, Dervla T D Kenna, Neil Woodford, Jane F Turton, and members of the UK CF Surveillance Working Group., The UK CF Surveillance Working Group comprised
References J Cyst Fibros 2019 Dec 17 - DOI: 10.1016/j.jcf.2019.11.005
Keywords Achromobacter, Pandoraea, Ralstonia, Survey
December 2019
Title: Lumacaftor/ivacaftor reduces exacerbations in adults homozygous for Phe508del mutation with severe lung disease
Authors Koliarne Tong, Daniel Barker, Megan France, Lucy Burr, Hugh Greville, Simone Visser, Peter Middleton, Claire Wainwright, Douglas Dorahy, Peter Wark
References J Cyst Fibros. 2019 Dec 15;S1569-1993(19)30985-3. doi: 10.1016/j.jcf.2019.12.006.
Keywords adverse events, CFTR modulator, Exacerbations, Ivacaftor, Lumacaftor, Lung function
November 2019
Title: Antimicrobial Susceptibility Testing in Cystic Fibrosis: A Delphi approach to defining best practices
Authors Edith Zemanick, Pierre–Régis Burgel, Giovanni Taccetti, Alison Holmes, Felix Ratjen, Catherine A Byrnes, Valerie J. Waters, Scott C. Bell, Donald R. VanDevanter, J. Stuart Elborn, Patrick A. Flume, on behalf of the Antimicrobial Resistance International Working Group in Cystic Fibrosis
References J Cyst Fibros. 2019 Oct 31. pii: S1569-1993(19)30919-1. doi: 10.1016/j.jcf.2019.10.006.
November 2019
Title: Ivacaftor in Cystic Fibrosis With Residual Function: Lung Function Results From an N-of-1 Study
Authors Jerry A. Nick, Connie St. Clair, Marion C. Jones, Lan Lan, Mark Higgins
References J Cyst Fibros. 2019 Nov 26. pii: S1569-1993(19)30897-5. doi: 10.1016/j.jcf.2019.09.013.
Keywords Bayesian hierarchical model, Cystic fibrosis transmembrane conductance regulator, forced expiratory volume, Ivacaftor, Residual CFTR function
