November 2021
Title: An in-depth study of the CFTR gene enables selection of CFTR therapies for almost all individuals with CF
Authors Karen S Raraigh, Melis A Aksit, Kurt Hetrick, Rhonda G Pace, Hua Ling, Wanda O'Neal, Elizabeth Blue, Yi-Hui Zhou, Michael J Bamshad, Scott M Blackman, Ronald L Gibson, Michael R Knowles, Garry R Cutting, for the CF Genome Project
References J Cyst Fibros. 2021 Nov 12;S1569-1993(21)02113-5. doi: 10.1016/j.jcf.2021.10.011.
Keywords CFTR, Modulator therapy, Variant annotation, Variant identification, Whole genome sequencing
Theme Care of patients with CF, CFTR/Basic Science
November 2021
Title: What do we already know and what do we need to study next when looking at markers of inflammation in sputum in CF trials?
Authors Agathe Lepissier, Charlotte Addy, Kate Hayes, Sabrina Noel, Stéphanie Bui, Pierre-Régis Burgel, Lieven Dupont, Olaf Eickmeier, Michael Fayon, Teresinha Leal, Carlos Lopes, Damian G Downey, Isabelle Sermet-Gaudelus
References J Cyst Fibros. 2021 Nov 9;S1569-1993(21)02111-1. doi: 10.1016/j.jcf.2021.10.009.
Keywords Inflammation, sputum biomarkers
November 2021
Title: How effective are exercise interventions at increasing physical activity in people with Cystic Fibrosis?
Authors M Curran, A C Tierney, B Button, L Collins, L Kennedy, C McDonnell, B Casserly, R Cahalan
References J Cyst Fibros. 2021 Nov 6;S1569-1993(21)02110-X. doi: 10.1016/j.jcf.2021.10.008.
Keywords Exercise, Interventions, Physical activity promotion, Training
Theme Care of patients with CF, Exercise
October 2021
Title: Arm muscle area as an early indicator of worsening nutritional status in children - a single centre experience
Authors Helmut Ellemunter, Markus Dumke, Gratiana Steinkamp
References J Cyst Fibros. 2021 Oct 30;S1569-1993(21)02108-1. doi: 10.1016/j.jcf.2021.10.006.
Keywords Anthropometric parameters, Arm muscle area, Fat-free mass, nutritional status, Skinfold measurements
Theme Care of patients with CF, Nutrition/Gastroenterology
October 2021
Title: Clofazimine has no influence on the blood concentrations of tezacaftor-ivacaftor in a patient with cystic fibrosis.
Authors S E M Vonk, S W J Terheggen-Lagro, L M Mouissie, R A A Mathôt, E M Kemper, Amsterdam Mucociliary Clearance Disease (AMCD) research group
References J Cyst Fibros. 2021 Oct 29;S1569-1993(21)02105-6. doi: 10.1016/j.jcf.2021.10.003.
Keywords Clofazimine, Drug-drug interaction, Tezacaftor-ivacaftor
Theme Care of patients with CF, Lung Disease and Infection
October 2021
Title: The effect of CFTR modulation with elexacaftor-tezacaftor-ivacaftor on the anxiety sweat rate in people with cystic fibrosis
Authors Sophia Theres Pallenberg, Sibylle Junge, Felix C Ringshausen, Annette Sauer-Heilborn, Gesine Hansen, Anna Maria Dittrich, Burkhard Tümmler, Manuel Nietert
References J Cyst Fibros. 2021 Oct 28;S1569-1993(21)02107-X. doi: 10.1016/j.jcf.2021.10.005.
Keywords CFTR modulator, Sweat rate assay, Sweat test
Theme Diagnosis/Screening
October 2021
Title: Exploring new treatment options for people with cystic fibrosis with severe stop mutations
Authors E de Poel, S Spelier, S W F Suen, E Kruisselbrink, S Y Graeber, M A Mall, E J M Weersink, M M van der Eerden, G H Koppelman, C K van der Ent, J M Beekman
References J Cyst Fibros. 2021 Oct 16;S1569-1993(21)01425-9. doi: 10.1016/j.jcf.2021.09.020.
Keywords CFTR modulation, CFTR nonsense mutation, Intestinal organoids, nonsense mRNA mediated decay inhibition, Premature termination codon, Read-through
October 2021
Title: Damage in the cell lining of the airways in cystic fibrosis
Authors Amandine M Collin, Marylène Lecocq, Bruno Detry, François M Carlier, Caroline Bouzin, Philippe de Sany, Delphine Hoton, Stijn Verleden, Antoine Froidure, Charles Pilette, Sophie Gohy
References J Cyst Fibros. 2021 Oct 14;S1569-1993(21)01424-7. doi: 10.1016/j.jcf.2021.09.019.
Keywords Epithelial differentiation, Epithelial-to-mesenchymal transition, Respiratory epithelium
Theme CFTR/Basic Science, Lung Disease and Infection
October 2021
Title: Is it possible to monitor young people with cystic fibrosis at home? Results of the CLIMB-CF study
Authors C Edmondson, N Westrupp, P Seddon, C Olden, C Wallis, C Dawson, M Brodlie, F Baxter, J McCormick, S MacFarlane, D Rice, A Macleod, R Brooker, M Connon, S Ghayyda, L Blaikie, R Thursfield, L Brown, A Price, E Fleischer, J Itterman, D Hughes, P Barrett, , M Surette, C Donnelly, D Mateos-Corral, G Padley, J Wallenburg, K Brownlee, E W F W Alton, A Bush, J C Davies
References J Cyst Fibros. 2021 Oct 8;S1569-1993(21)01423-5. doi: 10.1016/j.jcf.2021.09.018.
Keywords anxiety, depression, Digital technology, Feasibility, Home monitoring
Theme Care of patients with CF, Clinical Trials
October 2021
Title: Lung infection by Pseudomonas aeruginosa in children with cystic fibrosis: risk factors and impact on lung function
Authors Julie Mésinèle, Manon Ruffin, Astrid Kemgang, Loïc Guillot, Pierre-Yves Boëlle, Harriet Corvol
References J Cyst Fibros. 2021 Oct 7;S1569-1993(21)01422-3. doi: 10.1016/j.jcf.2021.09.017.
Keywords Children, Genetic modifiers, Lung function, Pseudomonas aeruginosa, Risk factors
Theme Lung Disease and Infection
October 2021
Title: A review of sweat testing in Ireland in the era of modern CF care
Authors Barrie Maguire, Ophelia Blake, Gerard Boran, Ingrid Borovickova, Sabah Abdelfadil, Caroline Murray, Basil Elnazir, Barry Linnane
References J Cyst Fibros. 2021 Oct 4;S1569-1993(21)01417-X. doi: 10.1016/j.jcf.2021.09.012.
Keywords National survey, Newborn screening, Quantity not sufficient, sweat testing
Theme Diagnosis/Screening
October 2021
Title: Features of Salt Imbalance and Pseudo Barter Syndrome in hospitalized Children and Young Adults with Cystic Fibrosis.
Authors Danish Abdul Aziz, Fatima Siddiqui, Quratulain Abbasi, Haissan Iftikhar, Shahira Shahid, Fatima Mir
References J Cyst Fibros. 2021 Oct 2;S1569-1993(21)01418-1. doi: 10.1016/j.jcf.2021.09.013.
Keywords Hypokalemic alkalosis, Hyponatremia, Pseudo-Bartter syndrom
Theme Care of patients with CF, Endocrinology
September 2021
Title: Sense of Smell in People with Cystic Fibrosis: Impact of Modulator Therapy
Authors Daniel M Beswick, Stephen M Humphries, Connor D Balkissoon, Matthew Strand, Eszter K Vladar, Vijay R Ramakrishnan, Jennifer L Taylor-Cousar
References J Cyst Fibros. 2021 Sep 28;S1569-1993(21)01419-3. doi: 10.1016/j.jcf.2021.09.014.
Keywords CFTR modulator therapy, Olfaction, Olfactory cleft
Theme Care of patients with CF, Nutrition/Gastroenterology
September 2021
Title: A summary of previously-published research looking at the length of treatment of acute infections and exacerbations in people with cystic fibrosis
Authors Trevor Nicholson, Aisling Smith, Edward McKone, Charles Gallagher
References J Cyst Fibros. 2021 Sep 27;S1569-1993(21)01358-8. doi: 10.1016/j.jcf.2021.08.017.
Keywords antibiotic, Duration of treatment, acute exacerbations
Theme Care of patients with CF, Lung Disease and Infection
September 2021
Title: A serious consideration about the wise use of the new, highly efficient, very expensive, treatment for cystic fibrosis (Trikafta©/Kaftrio©)
Authors Donatello Salvatore, Angela Pepe, Vincenzo Carnovale, Fabio Majo, Rita Padoan, Serena Quattrucci, Marco Salvatore, Domenica Taruscio, Annalisa Amato, Gianluca Ferrari, Giuseppe Campagna
References J Cyst Fibros. 2021 Sep 25;S1569-1993(21)01416-8. doi: 10.1016/j.jcf.2021.09.011.
Keywords CFTR modulator, CFTR-related disorders
September 2021
Title: Using a digital system, CFHealthHub, to automatically record the use of preventative inhaled treatments as to compare the amount of medicines collected from pharmacy to the amount actually used.
Authors Amanda Bevan and Martin Wildman for CFDigicare (https://www.cfhealthhub.com/cfdigicare/)
References J Cyst Fibros. 2021 Sep 23;S1569-1993(21)01408-9. doi: 10.1016/j.jcf.2021.09.007.
Keywords Adherence to therapy, Cost analysis, Measurement systems
Theme Care of patients with CF
September 2021
Title: Prevalence, management and outcome of Italian infants with cystic fibrosis screening positive inconclusive diagnosis
Authors Vito Terlizzi, Laura Claut, Antonella Tosco, Carla Colombo, Valeria Raia, Benedetta Fabrizzi, Marco Lucarelli, Antonio Angeloni, Giuseppe Cimino, Alice Castaldo, Laura Marsiglio, Silviana Timpano, Natalia Cirilli, Laura Moroni, Filippo Festini, , Pietro Piccinini, Lucia Zavataro, Paolo Bonomi, Giovanni Taccetti, Kevin W Southern, Rita Padoan
References J Cyst Fibros. 2021 Sep;20(5):828-834. doi: 10.1016/j.jcf.2021.03.015.
Title: Drug levels and dosing recommendations for antibiotics used to treat Mycobacterium avium complex in people with cystic fibrosis.
Authors Stacey L. Martiniano, Brandie D. Wagner, Laney Brennan, Michael F. Wempe, Peter L. Anderson, Charles L. Daley, Meg Anthony, Jerry A. Nick, and Scott D. Sagel
References J Cyst Fibros. 2021 Sep;20(5):772-778. doi: 10.1016/j.jcf.2021.04.011. Epub 2021 May 21.
Keywords Mycobacterium avium, nontuberculous mycobacteria, Pharmacokinetics, Therapeutic drug monitoring
Theme Care of patients with CF, Lung Disease and Infection, Microbiology
Title: Drug development for Cystic Fibrosis patients carrying the 3849+10kb C-to-T splicing mutation
Authors Yifat S Oren, Michal Irony-Tur Sinai, Anita Golec, Ofra Barchad-Avitzur, Venkateshwar Mutyam, Yao Li, Jeong Hong, Efrat Ozeri-Galai, Aurélie Hatton, Chen Leibson, Liran Carmel, Joel Reiter, Eric J Sorscher, Steve D Wilton, Eitan Kerem, Steven M Rowe, Isab
References J Cyst Fibros. 2021 Sep;20(5):865-875. doi: 10.1016/j.jcf.2021.06.003. Epub 2021 Jul 2.
Keywords 3849+10 kb C-to-T mutation, Antisense oligonucleotides, drug development, Splicing modulation
September 2021
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 Sep;19(5):e39-e41. 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
Theme Care of patients with CF, Psychosocial
September 2021
Title: Can cough swabs be used to identify bacterial infections in patient with cystic fibrosis.
Authors Dominic Fenn, Mahmoud I Abdel-Aziz, Paul Brinkman, Renate Kos, Anne H Neerincx, Josje Altenburg, E Weersink, Eric G Haarman, Suzanne W J Terheggen-Lagro, Anke H Maitland-van der Zee, Lieuwe D J Bos, Amsterdam mucociliary clearance disease research group
References J Cyst Fibros. 2021 Sep 18;S1569-1993(21)01372-2. doi: 10.1016/j.jcf.2021.08.031.
Keywords Microbiome, Next generation sequencing, Pseudomonas aeruginosa, pulmonary infection
September 2021
Title: Study that tested the effects of a new drug called acebilustat in adultswith cystic fibrosis
Authors J. Stuart Elborn, Michael W. Konstan, Jennifer L. Taylor-Cousar, Isabelle Fajac, Alexander Horsley, Sivagurunathan Sutharsan, Shawn D. Aaron, Cori L. Daines, Ahmet Uluer, Damian G. Downey, Vincenzina V. Lucidi, Sanjeev Ahuja, Eric Springman, John Mershon, Ralph Grosswald, Steven M. Rowe, for the EMPIRE-CF study group.
References J Cyst Fibros. 2021 Sep 16;S1569-1993(21)01348-5. doi: 10.1016/j.jcf.2021.08.007.
Keywords CFTR, Inflammation, LTA4H, LTB(4), Neutrophils
September 2021
Title: The effect of ETI (Trikafta™) on blood sugars in adults with cystic fibrosis
Authors Kevin J Scully, Peter Marchetti, Gregory S Sawicki, Ahmet Uluer, Manuela Cernadas, Rebecca E Cagnina, John C Kennedy, Melissa S Putman
References J Cyst Fibros. 2021 Sep 13;S1569-1993(21)01377-1. doi: 10.1016/j.jcf.2021.09.001.
Keywords CFTR modulator, continuous glucose monitoring, Cystic fibrosis related diabetes, Elexacaftor-tezacaftor-ivacaftor
September 2021
Title: Defining constipation in patients with Cystic Fibrosis following criteria delineated by the European Society for Paediatric Gastroenterology Hepatology and Nutrition
Authors Marina Abib Stefano, Natascha Silva Sandy, Carlos Zagoya, Franziska Duckstein, Antonio Fernando Ribeiro, Jochen G Mainz, Elizete Aparecida Lomazi
References J Cyst Fibros. 2021 Sep 9;S1569-1993(21)01362-X. doi: 10.1016/j.jcf.2021.08.021.
Theme Care of patients with CF, Nutrition/Gastroenterology
Title: Defining constipation in patients with Cystic Fibrosis following criteria delineated by the European Society for Paediatric Gastroenterology Hepatology and Nutrition
Authors Marina Abib Stefano, Natascha Silva Sandy, Carlos Zagoya, Franziska Duckstein, Antonio Fernando Ribeiro, Jochen G Mainz, Elizete Aparecida Lomazi
References J Cyst Fibros. 2021 Sep 9;S1569-1993(21)01362-X. doi: 10.1016/j.jcf.2021.08.021.
September 2021
Title: A review of three studies examining different aspects of pulmonary exacerbation treatment
Authors Georgene E Hergenroeder, Jonathan D Cogen
References J Cyst Fibros. 2021 Sep 6;S1569-1993(21)01365-5. doi: 10.1016/j.jcf.2021.08.024.
Keywords Antimicrobial stewardship, Pulmonary exacerbation
Theme Care of patients with CF, Epidemiology/Models of care
September 2021
Title: Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?
Authors Emily Granger, Gwyneth Davies, Ruth H Keogh
References J Cyst Fibros. 2021 Sep 5;S1569-1993(21)01355-2. doi: 10.1016/j.jcf.2021.08.014.
Keywords Ivacaftor, Registry data, Treatment burden, Treatment patterns
Theme Care of patients with CF, Epidemiology/Models of care
September 2021
Title: New perspectives on physical exercise training in adults and children with cystic fibrosis
Authors Mathieu Gruet, Zoe L Saynor, Don S Urquhart, Thomas Radtke
References J Cyst Fibros. 2021 Sep 4;S1569-1993(21)01345-X. doi: 10.1016/j.jcf.2021.08.004
Keywords Aerobic training, CFTR modulator, Exercise preferences, Physical fitness, Resistance training
September 2021
Title: Needle-free bubble test
Authors Audrey Reynaerts, François Vermeulen, Angélique Mottais, Sophie Gohy, Patrick Lebecque, Raphaël Frédérick, Rita Vanbever, Teresinha Leal
References J Cyst Fibros. 2021 Sep 3;S1569-1993(21)01353-9. doi: 10.1016/j.jcf.2021.08.012.
Keywords CFTR, Iontophoresis, Nonlinear mixed-effects model, Sweat gland, Sweat test
Theme Care of patients with CF, Diagnosis/Screening
Title: Clinical presentation and basic defect of the CFTR genotype p.Phe508del / p.Arg117His in a mother and her monozygous twin daughters
Authors Christiane Lex, Rebecca Minso, Nadine Alfeis, Hendrik Rosewich, Sylvia Schucht, Burkhard Tümmler
References J Cyst Fibros. 2021 Sep 3;S1569-1993(21)01359-X. doi: 10.1016/j.jcf.2021.08.018.
Keywords Intestinal current measurement, Nasal potential difference, Twin research
August 2021
Title: Survival in cystic fibrosis after need for mechanical life support.
Authors Jenna M. Gibilaro, Claire Keating, Luke Benvenuto, Amanda Kramer, Ann Privorotskiy, Yuan Zheng, Cheng-Shiun Leu
References J Cyst Fibros. 2021 Aug 31;S1569-1993(21)01357-6. doi: 10.1016/j.jcf.2021.08.016.
Title: Clofazimine use for treatment of mycobacteria in children with and without cystic fibrosis
Authors L H Cameron, C A Peloquin, P Hiatt, M Mann, J R Starke, J Faircloth, J C McNeil, A Patel, F Ruiz
References J Cyst Fibros. 2021 Aug 31;S1569-1993(21)01351-5. doi: 10.1016/j.jcf.2021.08.010.
August 2021
Title: Comparing lung function testing at home vs in the clinic.
Authors Alex Paynter, Umer Khan, Sonya L Heltshe, Christopher H Goss, Noah Lechtzin, Nicole Mayer Hamblett
References J Cyst Fibros. 2021 Aug 30;S1569-1993(21)01354-0. doi: 10.1016/j.jcf.2021.08.013.
Keywords CF outcome measures, COVID-19, Coronavirus, Forced expiratory volume in one second (FEV1), Remote monitoring, Telehealth
August 2021
Title: The influence of cystic fibrosis on work ability of affected patients
Authors Veruscka Leso, Rosaria Romano, Carolina Santocono, Marilisa Caruso, Paola Iacotucci, Vincenzo Carnovale, Ivo Iavicoli
References J Cyst Fibros. 2021 Aug 29;S1569-1993(21)01352-7. doi: 10.1016/j.jcf.2021.08.011.
Keywords Employment, Occupation, Professional life, Risk assessment and management, Work ability
August 2021
Title: How did new infections with important bacteria change over time across U.S. CF care centers
Authors Marianne S Muhlebach, Xiaotong Jiang, Michael R Kosorok, Eili Y Klein, Lisa Saiman
References J Cyst Fibros. 2021 Aug 26;S1569-1993(21)01350-3. doi: 10.1016/j.jcf.2021.08.009.
Keywords CF center, Epidemiology, ethnicity, infection control, Methicillin resistant Staphylococcus aureus (MRSA), Pseudomonas aeruginosa
Title: Review of pregnancy in cystic fibrosis
Authors Raksha Jain, Traci M Kazmerski, Lisa C Zuckerwise, Natalie E West, Kristina Montemayor, Moira L Aitken, Edith Cheng, Andrea H Roe, Alexandra Wilson, Caitlin Mann, Sigrid Ladores, Jacqui Sjoberg, Madeline Poranski, Jennifer L Taylor-Cousar
References J Cyst Fibros. 2021 Aug 26;S1569-1993(21)01338-2. doi: 10.1016/j.jcf.2021.07.019.
Keywords Family planning, Fertility, Parenthood, pregnancy
August 2021
Title: Orkambi may improve liver disease in people with CF
Authors David Drummond, Jérémy Dana, Laureline Berteloot, Elena K. Schneider-Futschik, Frédérique Chedevergne, Céline Bailly-Botuha, Thao Nguyen-Khoa, Muriel Le Bourgeois, Dominique Debray, Muriel Girard, Isabelle Sermet-Gaudelus
References J Cyst Fibros. 2021 Aug 25;S1569-1993(21)01337-0. doi: 10.1016/j.jcf.2021.07.018.
Keywords CFTR modulator, Cystic-fibrosis liver disease, Cirrhosis, hepatobiliary, liver enzymes; pediatrics
August 2021
Title: A study of the drug riociguat as a possible treatment for people with cystic fibrosis
Authors Nico Derichs, Jennifer L Taylor-Cousar, Jane C Davies, Isabelle Fajac, Elizabeth Tullis, Dilip Nazareth, Damian G Downey, Daniel Rosenbluth, Anne Malfroot, Clare Saunders, Renee Jensen, George M Solomon, Francois Vermeulen, Andreas Kaiser, Stefan Willmann, Soundos Saleh, Karoline Droebner, Peter Sandner, Christine E. Bear, Anja Hoffmann, Felix Ratjen1, Steven M. Rowe
References J Cyst Fibros. 2021 Aug 19;S1569-1993(21)01329-1. doi: 10.1016/j.jcf.2021.07.015.
Keywords Phe508del, Riociguat
Title: Do cystic fibrosis patients have an abnormal innate immune response to pathogens?
Authors Isabelle Fleurot, Raquel López-Gálvez, Pascal Barbry, Antoine Guillon, Mustapha Si-Tahar, Andrea Bähr, Nikolai Klymiuk, Jean-Claude Sirard, Ignacio Caballero
References J Cyst Fibros. 2021 Aug 19;S1569-1993(21)01343-6. doi: 10.1016/j.jcf.2021.08.002.
August 2021
Title: Ivacaftor withdrawal syndrome: A rare but potentially life-threatening consequence from a life-saving medication
Authors James M Clegg, Kelsey W Malloy, Rebekah F Brown, Alison G Grisso, Andrew G Sokolow
References J Cyst Fibros. 2021 Aug 11;S1569-1993(21)01340-0. doi: 10.1016/j.jcf.2021.07.021.
Keywords Highly effective modulator therapy, Ivacaftor withdrawal syndrome
Theme Care of patients with CF, Pulmonology
August 2021
Title: Use of elexacaftor/tezacaftor/ivacaftor in liver transplant patients with cystic fibrosis
Authors Cameron J McKinzie, Christina T Doligalski, Steven J Lobritto, Raymond D Coakley, W Adam Gower
References J Cyst Fibros. 2021 Aug 9;S1569-1993(21)01336-9. doi: 10.1016/j.jcf.2021.07.017.
Keywords CFTR modulator; Cystic fibrosis; Liver transplantation
August 2021
Title: “Coping and Learning to Manage Stress with CF” a Telehealth Pilot Intervention
Authors Christina J Bathgate, Kristin M Kilbourn, Nora H Murphy, Frederick S Wamboldt, Kristen E Holm
References J Cyst Fibros. 2021 Aug 5;S1569-1993(21)01326-6. doi: 10.1016/j.jcf.2021.07.012.
Keywords anxiety, Cognitive behavioral therapy, Coping, depression, pilot study, RCT, Stress
Title: Do bone active therapy and lung disease influence the skeletal outcome after transplantation?
Authors Grassi Giorgia, Chiodini Iacopo, Cairoli Elisa, Morlacchi Letizia Corinna, Rossetti Valeria, Rosso Lorenzo, Righi Ilaria, Nosotti Mario, Arosio Maura, Blasi Francesco, Eller-Vainicher Cristina
References J Cyst Fibros. 2021 Aug 5;S1569-1993(21)01327-8. doi: 10.1016/j.jcf.2021.07.013
Keywords Lung transplantation, Trabecular bone score, Vertebral fractures
August 2021
Title: Cancer cases in the UK Cystic Fibrosis Population
Authors Olga Archangelidi, Paul Cullinan, Nicholas J Simmonds, Emmanouil Mentzakis, Daniel Peckham, Diana Bilton, Siobhán B Carr
References J Cyst Fibros. 2021 Aug 1;S1569-1993(21)01302-3. doi: 10.1016/j.jcf.2021.07.004.
Keywords Breast, Cancer, Gastro-intestinal, registry, Risk, skin
Theme Care of patients with CF, Diabetes, Epidemiology/Models of care, Nutrition/Gastroenterology, Transplantation
Title: Is elexacaftor/tezacaftor/ivacaftor always better than ezacaftor/ivacaftor?
Authors Eva Furstova, Tereza Dousova, Jakub Beranek, Malgorzata Libik, Libor Fila, Martin Modrak, Ondrej Cinek, Milan Macek Jr, Pavel Drevinek
References J Cyst Fibros. 2021 Aug 1;S1569-1993(21)01304-7. doi: 10.1016/j.jcf.2021.07.006.
Keywords CFTR sequence, Elexacaftor, Intestinal organoids, Ivacaftor, Tezacaftor
July 2021
Title: Different approaches to Aspergillus-related conditions in people with cystic fibrosis
Authors Gina Hong, Sameer Desai, Richard B Moss, Patience Eschenhagen, Bradley S Quon, Carsten Schwarz
References J Cyst Fibros. 2021 Jul 28;S1569-1993(21)01306-0. doi: 10.1016/j.jcf.2021.07.008.
Keywords Allergic bronchopulmonary aspergillosis, Aspergillus bronchitis, Aspergillus colonization, Fungi
July 2021
Title: Urinary tract infections in cystic fibrosis patients
Authors Seth A Reasoner, Kyle T Enriquez, Benjamin Abelson, Steven Scaglione, Bennett Schneier, Michael G O'Connor, Gerald Van Horn, Maria Hadjifrangiskou
References J Cyst Fibros. 2021 Jul 27;S1569-1993(21)01303-5. doi: 10.1016/j.jcf.2021.07.005
Keywords Antibiotic resistance, Cystitis, Urinary tract infection
July 2021
Title: Utilization of Electronic Patient-Reported Outcome Measures in Cystic Fibrosis Research
Authors Meghana Sathe, Baha Moshiree, Phuong T Vu, Umer Khan, Sonya L Heltshe, Melita Romasco, Steven D Freedman, Sarah Jane Schwarzenberg, Christopher H Goss, A Jay Freeman
References J Cyst Fibros. 2021 Jul 22;S1569-1993(21)01299-6. doi: 10.1016/j.jcf.2021.07.002.
Keywords Electronic PROMs, Patient reported outcomes measures, gastrointestinal manifestations of cystic fibrosis
July 2021
Title: 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. 2021 Jul;20(4):612-617. doi: 10.1016/j.jcf.2020.09.011. Epub 2020 Oct 3.
Theme Care of patients with CF, Pulmonology
Title: Review of abnormal Stomach and Intestinal Movement in Cystic Fibrosis
Authors Sara Henen, Christine Denton, Jeff Teckman, Drucy Borowitz, Dhiren Patel
References J Cyst Fibros. 2021 Jul;20(4):578-585. doi: 10.1016/j.jcf.2021.05.016. Epub 2021 Jun 17.
Keywords GI motility, dysmotility, Gastrointestinal, highly effective modulators, laxatives; motility
Title: Stopping exposure to smoke improves breathing and nutritional outcomes in children and adolescents with cystic fibrosis
Authors Gabriela R Oates, Elizabeth Baker, Joseph M Collaco, Steven M Rowe, Sarah B Rutland, Christopher M Fowler, William T Harris
References J Cyst Fibros. 2021 Jul;20(4):618-624. doi: 10.1016/j.jcf.2021.06.014. Epub 2021 Jul 17.
Keywords Secondhand smoke, Smoke exposure, Smoking cessation, Tobacco
July 2021
Title: Disease burden in people with cystic fibrosis heterozygous for F508del and a minimal function mutation
Authors Gregory S. Sawicki, Kate Van Brunt, Jason Booth, Evan Bailey, Stefanie J. Millar, Michael W. Konstan, Patrick A. Flume
References J Cyst Fibros. 2021 Jul 19;S1569-1993(21)01300-X. doi: 10.1016/j.jcf.2021.07.003.
July 2021
Title: Drug-Induced Liver Injury from Elexacaftor/Ivacaftor/Tezacaftor
Authors Sarah Lowry; Peter J. Mogayzel, Jr.; Kiyoko Oshima; Wikrom Karnsakul
References J Cyst Fibros. 2021 Jul 16;S1569-1993(21)01297-2. doi: 10.1016/j.jcf.2021.07.001.
Keywords CFTR modulator, Drug-induced liver injury
July 2021
Title: A Short extension to multiple breath washout provides additional indications of blocked and severely diseased airways in people with CF: a pilot study
Authors Christopher Short, Thomas Semple, Clare Saunders, Dominic Hughes, Samantha Irving, Laura Gardener, Mark Rosenthal, Paul D Robinson, Jane C Davies
References J Cyst Fibros. 2021 Jul 15;S1569-1993(21)01298-4. doi: 10.1016/j.jcf.2021.06.013.
Keywords Air Trapping, Biomarkers, Computed Tomography (CT), Lung clearance index (LCI), Lung Function Testing, Multiple breath washout (mbw), Pulmonary function testing (pft), Pulmonary physiology
July 2021
Title: There are two main types of problems with arteries in cystic fibrosis and existence of one or the other depends on pancreatic health
Authors Jan Krzysztof Nowak, Andrzej Wykrętowicz, Edyta Mądry, Tomasz Krauze, Sławomira Drzymała-Czyż, Patrycja Krzyżanowska-Jankowska, Paulina Sobkowia, Agata Schneider, Joanna Goździk-Spychalska, Szymon Kurek, Victoria Kononets, Nataliya Kashirskaya, Aleksandra Lisowska, Jarosław Walkowiak
References J Cyst Fibros. 2021 Jul 10;S1569-1993(21)01294-7. doi: 10.1016/j.jcf.2021.06.010.
Keywords Arterial stiffness, Atherosclerosis, Digital volume pulse, Intima-media, Lipids
Theme Care of patients with CF, Nutrition/Gastroenterology
Title: Opioid Pain Management Among Adults with CF: Survey Results
Authors Sarah Allgood, Jessica L Zemlak, Elisabeth Dellon, Siddhartha G Kapnadak, Jessica Goggin, Noah Lechtzin
References J Cyst Fibros. 2021 Jul 10;S1569-1993(21)01291-1. doi: 10.1016/j.jcf.2021.06.007.
Keywords Adults, Opioids, Pain
July 2021
Title: Genetics of immunity against the Pseudomonas aeruginosa bacteria in people with cystic fibrosis
Authors Janardan P Pandey, Aryan M Namboodiri, Paul J Nietert, Michael R Knowles, Rhonda G Pace, Gerald B Pier
References J Cyst Fibros. 2021 Jul 7;S1569-1993(21)01295-9. doi: 10.1016/j.jcf.2021.06.011.
Keywords GM/KM allotypes, IgG antibodies, Pseudomonas aeruginosa
June 2021
Title: How does the cystic fibrosis drug ivacaftor move in the body during pregnancy and breastfeeding?
Authors Fiona Qiu, Mark D Habgood, Yifan Huang, Katarzyna M Dziegielewska, Sam Toll, Elena K Schneider-Futschik
References J Cyst Fibros. 2021 Jun 27;S1569-1993(21)01289-3. doi: 10.1016/j.jcf.2021.06.005.
Keywords Breastfeeding, Cystic fibrosis transmembrane conductance regulator, Ivacaftor, pregnancy, blood brain barrier
June 2021
Title: Use of LCI to monitor CFTR Modulator effect in people with CF
Authors Philippe Reix, Aurélie Tatopoulos, Iulia Ioan, Muriel Le Bourgeois, Stéphanie Bui, Marie Luce Choukroun, Katia Bessaci-Kabouya, Michele Gerardin, Plamen Bokov, Jennifer Da Silva, Jean-Louis Paillasseur, Pierre Regis Burgel,, French Cystic Fibrosis Reference Network study group
References J Cyst Fibros. 2021 Jun 25;S1569-1993(21)01286-8. doi: 10.1016/j.jcf.2021.06.002.
Keywords CFTR modulators, Lumacaftor, Multiple breath washout
June 2021
Title: How changes in health insurance coverage for young children with CF can impact important health outcomes, such as lung function at 6 years of age
Authors Kimberly M Dickinson, Kevin J Psoter, Kristin A Riekert, Joseph M Collaco
References J Cyst Fibros. 2021 Jun 23;S1569-1993(21)01290-X. doi: 10.1016/j.jcf.2021.06.006.
Keywords BMI, Early childhood, FEV(1)pp, health insurance, registry
Theme Care of patients with CF
June 2021
Title: Effect of elexacaftor-texacaftor-ivacaftor in cystic fibrosis patients with advanced lung disease
Authors Bermingham, B.; Rueschhoff, A. Ratti, G. Nesmith, A., Goodwin D, Gray S, Flume P, Solomon, G. M., Cohen, L., Garcia, B.
References J Cyst Fibros. 2021 Jun 21;S1569-1993(21)00160-0. doi: 10.1016/j.jcf.2021.05.009
June 2021
Title: Using an Antibiotic Treatment Called Amikacin Liposome Inhalation Suspension (or ALIS) for Chronic Infection With Pseudomonas aeruginosa in People With Cystic Fibrosis for 2 years
Authors Diana Bilton, Isabelle Fajac, Tacjana Pressler, John Paul Clancy, Dorota Sands, Predrag Minic, Marco Cipolli, Ivanka Galeva, Amparo Solé, Alexandra L Quittner, Zhanna Jumadilova, Monika Ciesielska, Michael W Konstan, CLEAR-110 Study Group
References J Cyst Fibros. 2021 Jun 15;S1569-1993(21)00164-8. doi: 10.1016/j.jcf.2021.05.013.
Keywords ALIS, Amikacin, Liposomal, Pseudomonas aeruginosa
June 2021
Title: Complications relating to the heart and blood vessels in people with Cystic Fibrosis: A Review of the Literature
Authors T. Spencer Poore, Jennifer Taylor-Cousar, Edith Zemanick
References J Cyst Fibros. 2021 Jun 14;S1569-1993(21)00126-0. doi: 10.1016/j.jcf.2021.04.016.
Keywords CFTR, Cardiovascular disease, sildenafil
June 2021
Title: Cystic fibrosis-related diabetes (CFRD) and cognitive ability (memory, attention and task switching) in adults with cystic fibrosis
Authors Helen K Chadwick, Janice Abbott, Margaret Anne Hurley, Louise Dye, Clare L Lawton, Michael W Mansfield, Daniel Peckham
References J Cyst Fibros. 2021 Jun 13;S1569-1993(21)00124-7. doi: 10.1016/j.jcf.2021.04.014.
Keywords Cognition, Cognitive tests, Cystic fibrosis-related diabetes, Executive function
Title: Liver toxicity with triple therapy in cystic fibrosis: when it is worth taking the risk.
Authors Dimitri Stylemans, Silke François, Stefanie Vincken, Sylvia Verbanck, Eef Vanderhelst
References J Cyst Fibros. 2021 Jun 13;S1569-1993(21)00174-0. doi: 10.1016/j.jcf.2021.05.017.
June 2021
Title: New drug for cystic fibrosis does not significantly interfere with another drug that prevents organ rejection
Authors Megan Smith, Kevin J Ryan, Hector Gutierrez, Luz Helena Gutierrez Sanchez, Janaina Nogueira Anderson, Edward P Acosta, Kim W Benner, Jennifer S Guimbellot
References J Cyst Fibros. 2021 Jun 12;S1569-1993(21)00159-4. doi: 10.1016/j.jcf.2021.05.008.
Keywords CFTR, Cytochrome p450, Drug interaction, Elexacaftor, Ivacaftor, Liver transplantation, Tacrolimus, Tezacaftor
June 2021
Title: What do different bacteria together do to the lung epithelium in Cystic Fibrosis
Authors Bertelsen, J Stuart Elborn, Bettina C Schock
References J Cyst Fibros. 2021 Jun 7;S1569-1993(21)00122-3. doi: 10.1016/j.jcf.2021.04.012.
Keywords Airway microbiome, Anaerobic bacteria, Cystic Fibrosis Airways inflammation, Host-pathogen-interaction, Mixed infection, Prevotella spp., Signalling
June 2021
Title: Comparison of gene editing methods to correct the W1282X mutation
Authors Lúcia Santos, Karen Mention, Kader Cavusoglu-Doran, David J Sanz, Mafalda Bacalhau, Miquéias Lopes-Pacheco, Patrick T Harrison, Carlos M Farinha
References J Cyst Fibros. 2021 Jun 5;S1569-1993(21)00167-3. doi: 10.1016/j.jcf.2021.05.014.
Keywords CFTR, CRISPR/Cas12a, CRISPR/Cas9, human bronchial epithelial cells, W1282X
Title: Appearance and behaviour of methicillin resistant Staphylococcus aureus (MRSA) from early and chronic infection in cystic fibrosis (CF)
Authors Deirdre Gilpin, Lucas R Hoffman, Agathe Ceppe, Marianne S Muhlebach
References J Cyst Fibros. 2021 Jun 5;S1569-1993(21)00168-5. doi: 10.1016/j.jcf.2021.05.015.
Keywords Adaptation, Anaerobe, Antimicrobial resistance, Haemolysis, Outcome, Staphylococcus aureus
June 2021
Title: Effect of inhaled antibiotic therapy with levofloxacin in people with cystic fibrosis
Authors Carsten Schwarz, Claudia Grehn, Svenja Temming, Frederik Holz, Patience Ndidi Eschenhagen
References J Cyst Fibros. 2021 Jun 4;S1569-1993(21)00051-5. doi: 10.1016/j.jcf.2021.03.002.
Keywords chronic Pseudomonas aeruginosa infection, Inhaled antibiotics, Levofloxacin solution, Pulmonary function test, exacerbation rate
June 2021
Title: Can a new blood test be used to screen for CF-related diabetes ?
Authors Tom Toin, Quitterie Reynaud, Angélique Denis, Isabelle Durieu, Catherine Mainguy, Catherine Llerena, Isabelle Pin, Sandrine Touzet, Philippe Reix
References J Cyst Fibros. 2021 Jun 2;S1569-1993(21)00161-2. doi: 10.1016/j.jcf.2021.05.010.
Keywords CFRD, Diabetes, HOMA, Homeostasis, Indice
Title: Evaluating Pulmonary Exacerbation Treatment Differences in Women and Men with CF
Authors Kristina Montemayor, Kevin J Psoter, Noah Lechtzin, Sara W Carson, Christian A Merlo, Rebecca H Dezube, Kristin A Riekert, Sarah Allgood, Alexandra Toporek, Mark T Jennings, Natalie E West
References J Cyst Fibros. 2021 Jun 2;S1569-1993(21)00163-6. doi: 10.1016/j.jcf.2021.05.012.
Keywords Pulmonary exacerbations, Sex differences, Treatment patterns
June 2021
Title: Respiratory symptoms are not more frequent or severe in infants with CF compared to healthy infants.
Authors Insa Korten, Marc-Alexander Oestreich, Urs Frey, Alexander Moeller, Andreas Jung, Renate Spinas, Dominik Mueller-Suter, Daniel Trachsel, Isabelle Rochat, Ben Spycher, Philipp Latzin, Carmen Casaulta, Kathryn Ramsey
References J Cyst Fibros. 2021 Jun 1;S1569-1993(21)00116-8. doi: 10.1016/j.jcf.2021.04.006.
Keywords Cohort study, Healthy controls, Infancy, Respiratory symptoms
May 2021
Title: The effect of lumacaftor-ivacaftor on mucus clearance
Authors Scott H. Donaldson, Beth L. Laube, Peter Mogayzel, Timothy E. Corcoran, Joseph M. Pilewski, Agathe Ceppe, Jihong Wu, Pradeep G. Bhambhvani, Felix Ratjen, Scott D. Sagel, J.P. Clancy, Steven M. Rowe*, William D. Bennett* *on behalf of the PROSPECT Investig
References J Cyst Fibros. 2021 May 31;S1569-1993(21)00131-4. doi: 10.1016/j.jcf.2021.05.004.
May 2021
Title: How frequent is the condition of overweight among patients with cystic fibrosis in Italy?
Authors Andrea Gramegna, Stefano Aliberti, Martina Contarini, Daniela Savi, Giovanni Sotgiu, Fabio Majo, Laura Saderi, Vincenzina Lucidi, Francesco Amati, Maria Pappalettera, Paolo Palange, Francesco Blasi
References J Cyst Fibros. 2021 May 27;S1569-1993(21)00129-6. doi: 10.1016/j.jcf.2021.05.002.
Keywords Adults, Overweight
May 2021
Title: Sleep in children and youth with cystic fibrosis: A review of the research
Authors Jordana McMurray, Kimberley Widger, Anne L Stephenson, Robyn Stremler
References J Cyst Fibros. 2021 May 23;S1569-1993(21)00132-6. doi: 10.1016/j.jcf.2021.05.005.
May 2021
Title: Early signs of Cystic Fibrosis Related Diabetes (CFRD) and low amount of the neuropeptide VIP in ΔF508-CF mice.
Authors Anna Semaniakou, Frederic Chappe, Younes Anini, Valerie Chappe
References J Cyst Fibros. 2021 May 22;S1569-1993(21)00133-8. doi: 10.1016/j.jcf.2021.05.006.
Keywords Cystic fibrosis-related diabetes, Glucagon, Insulin, pancreas, Vasoactive Intestinal Peptide
May 2021
Title: Pseudomonas aeruginosa infection, but not CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis
Authors Dirk Westhölter, Hendrik Beckert, Svenja Straßburg, Matthias Welsner, Sivagurunathan Sutharsan, Christian Taube, Sebastian Reuter
References J Cyst Fibros. 2021 May 21;S1569-1993(21)00128-4. doi: 10.1016/j.jcf.2021.05.001.
Keywords CFTR modulator therapy, Pseudomonas aeruginosa, Cytokines, lymphocytes, non-CF bronchiectasis, regulatory T cells
May 2021
Title: 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. 2021 May;20(3):424-431. doi: 10.1016/j.jcf.2020.10.002. Epub 2020 Nov 5.
Keywords health disparities, Pseudomonas aeruginosa
Theme Care of patients with CF, Lung Disease and Infection, Psychosocial
Title: Mycobacterium abscessus smooth and rough colony variants in cystic fibrosis patients
Authors Ana Victoria Gutiérrez, Sophie Alexandra Baron, Feyrouz Sonia Sardi, Jamal Saad, Bérengère Coltey, Martine Reynaud-Gaubert, Michel Drancourt
References J Cyst Fibros. 2021 May;20(3):421-423. doi: 10.1016/j.jcf.2021.02.002.
Keywords Mycobacterium abscessus, Rough variant, Smooth variant, Whole genome sequencing
Theme Care of patients with CF, Microbiology
Title: SARS-CoV2 infection in CF patient colonized by Burkholderia cenocepacia
Authors C Olcese, R Casciaro, D Pirlo, C Debbia, E Castagnola, F Cresta, C Castellani
References J Cyst Fibros. 2021 May;20(3):e29-e31. doi: 10.1016/j.jcf.2021.03.024. Epub 2021 Apr 8.
Keywords Burkholderia cepacia, Pulmonary exacerbation, SARS-CoV-2 COVID-19
May 2021
Title: Quality of life and health outcomes in children with CF aged 5 years
Authors Joyce Cheney, Suzanna Vidmar, Nicholas Gailer, Claire Wainwright, Tonia A Douglas, Australasian Cystic Fibrosis Bronchoalveolar Lavage (ACFBAL) study group
References J Cyst Fibros. 2020 May;19(3):483-491. doi: 10.1016/j.jcf.2020.02.022. Epub 2020 Mar 9.
Keywords Children, Exacerbations, health-related quality of life, Lung function, Preschool, Pulmonary
May 2021
Title: Boosting the rescue of faulty CFTR with phosphate
Authors Vinciane Saint‐Criq, Yiting Wang, Livia Delpiano, JinHeng Lin, David N. Sheppard, Michael A. Gray
References J Cyst Fibros. 2021 May 18;S1569-1993(21)00123-5. doi: 10.1016/j.jcf.2021.04.013.
Keywords Airway epithelia, CFTR correctors, Elexacaftor-tezacaftor-ivacaftor (Trikafta), F508del-CFTR, Phosphate, SLC34A2
May 2021
Title: CF Pancreatic Duct Secretions Can Alter Islet Function
Authors Pavana G. Rotti, Idil A, Evans, Yulong Zhang, Bo Liang, Nathan Cunicelli, Yunxia O’Malley, Andrew W. Norris, Aliye Uc, John F. Engelhardt
References J Cyst Fibros. 2021 May 17;S1569-1993(21)00120-X. doi: 10.1016/j.jcf.2021.04.010.
Keywords Cystic fibrosis related diabetes, ductal cells, pancreas, proteome, secretome
Title: Focusing on tiny compounds in exhaled breath, can they help detect bacteria in people with cystic fibrosis?
Authors Renate Kos, Paul Brinkman, Anne H Neerincx, Tamara Paff, Marije G Gerritsen, Ariana Lammers, Aletta D Kraneveld, Harry G M Heijerman, Hettie M Janssens, Jane C Davies, Christof J Majoor, Els J Weersink, Peter J Sterk, Eric G Haarman, Lieuwe D Bos, Anke H Maitland-van der Zee, Amsterdam Mucociliary Clearance Disease (AMCD) Research Group and the Amsterdam UMC Breath Research Group
References J Cyst Fibros. 2021 May 17;S1569-1993(21)00125-9. doi: 10.1016/j.jcf.2021.04.015.
Keywords Exhaled biomarkers, Pseudomonas aeruginosa, VOC
Title: Aquagenic wrinkling in children under two years of age: Could this be a potential clinical referral tool for cystic fibrosis among non-screened populations?
Authors Grace R Paul, Shasha Bai, Kenneth Jackson, Karen S McCoy
References J Cyst Fibros. 2021 May 17;S1569-1993(21)00112-0. doi: 10.1016/j.jcf.2021.04.002.
Keywords Aquagenic wrinkling, CF early diagnosis, Nutrition, Time to wrinkle
May 2021
Title: How does infection with Pseudomonas and Aspergillus affect people with cystic fibrosis? A study using UK registry data.
Authors Dominic A Hughes, Olga Archangelidi, Matthew Coates, Darius Armstrong-James, Stuart J Elborn, Siobhán B Carr, Jane C Davies
References J Cyst Fibros. 2021 May 3;S1569-1993(21)00117-X. doi: 10.1016/j.jcf.2021.04.007.
Keywords Aspergillus, Infection, Pseudomonas, registry
April 2021
Title: Microbiota diversity in CF chronic sinusitis
Authors Sarah K Lucas, Erin Feddema, Holly C Boyer, Ryan C Hunter
References J Cyst Fibros. 2021 Apr 27;S1569-1993(21)00104-1. doi: 10.1016/j.jcf.2021.03.022.
Keywords Chronic sinusitis, Microbial ecology, Microbiome, Pseudomonas aeruginosa, Staphylococcus aureus
April 2021
Title: Risk factors for joint inflammation and joint pain in cystic fibrosis
Authors C. Grehn*, A.-M. Dittrich, J. Wosniok, F. Holz, S. Hafkemeyer, L. Naehrlich, C. Schwarz, Registry working group of the German CF Registry
References J Fibros . 2021 May 22;S1569-1993(21)00130-2. doi: 10.1016/j.jcf.2021.05.003.
April 2021
Title: A study to understand which factors are important to people with Cystic Fibrosis when deciding whether to take part in clinical trials
Authors R Dobra, JS Elborn, S Madge, L Allen, M Boeri, F Kee, S Goundry, T Purcell, C Saunders, JC Davies
References J Cyst Fibros. 2021 Apr 21;S1569-1993(21)00103-X. doi: 10.1016/j.jcf.2021.03.021.
April 2021
Title: Meconium Ileus due to GUCY2C gene mutations in three unrelated South Indian families
Authors Sneha Varkki, Antony Terance Benjamin, Rekha Athiyarath, Sumita Danda, Ravikumar Sowmya, Gary Connett
References J Cyst Fibros. 2021 Apr 18;S1569-1993(21)00105-3. doi: 10.1016/j.jcf.2021.03.023.
Keywords Gucy2c gene mutation, meconium ileus
April 2021
Title: Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020.
Authors Naehrlich Lutz1 for the European Cystic Fibrosis COVID project group
References Journal of Cystic Fibrosis 2021; April 17. DOI:
https://doi.org/10.1016/j.jcf.2021.03.017
Keywords COVID-19, SARS-CoV-2, Europe, Incidence, Epidemiology
April 2021
Title: Optimising equity of access: how should we allocate slots to the most competitive trials in Cystic Fibrosis (CF)?
Authors R Dobra, G Davies, K Pike, C Strassle, L Allen, R Brendell, K Brownlee, S B Carr, N J Simmonds, J C Davies, London Network of Clinical Trials Accelerator Platform sites and affiliates
References J Cyst Fibros. 2021 Apr 16;S1569-1993(21)00109-0. doi: 10.1016/j.jcf.2021.03.027.
April 2021
Title: Trends in the use of healthcare resources over time and the associated costs for people with cystic fibrosis?
Authors Isabelle Durieu, Faustine Dalon, Quitterie Reynaud, Lydie Lemonnier, Clémence Dehillotte, Marjorie Bérard, Déborah Walther, Marie Viprey, Eric Van Ganse, Manon Belhassen
References J Cyst Fibros. 2021 Apr 14;S1569-1993(21)00107-7. doi: 10.1016/j.jcf.2021.03.025.
Keywords Costs, Healthcare resource use, Observational study
April 2021
Title: Bio-informatic analyses clarify how blood vessel malfunction may happen in people with cystic fibrosis
Authors Lucas Treps, Mathias Declercq, Siham Bousfia, Peter Carmeliet, Peter Witters
References J Cyst Fibros. 2021 Apr 13;S1569-1993(21)00101-6. doi: 10.1016/j.jcf.2021.03.019.
April 2021
Title: Infant with cystic fibrosis has normal pancreas function and was missed by newborn screening after mom stayed on CFTR modulator therapy during pregnancy.
Authors Christopher N Fortner, Julie M Seguin, Denise M Kay
References J Cyst Fibros. 2021 Apr 9;S1569-1993(21)00100-4. doi: 10.1016/j.jcf.2021.03.018.
Keywords CFTR modulator, Elexacaftor, Fecal elastase, Immunoreactive trypsinogen, In utero exposure, Ivacaftor, Newborn screen, Pancreatic dysfunction, pregnancy, Tezacaftor
April 2021
Title: Peripheral lung effect of elexacaftor/tezacaftor/ivacaftor in adult cystic fibrosis
Authors Dimitri Stylemans, Chantal Darquenne, Daniël Schuermans, Sylvia Verbanck, Eef Vanderhelst
References J Cyst Fibros. 2021 Apr 6;S1569-1993(21)00098-9. doi: 10.1016/j.jcf.2021.03.016
April 2021
Title: The role of the “PTM-CODE” in restoring F50del-CFTR functionality
Authors Claudio D'Amore, Christian Borgo, Mauro Salvi
References J Cyst Fibros. 2021 Apr 1;S1569-1993(21)00086-2. doi: 10.1016/j.jcf.2021.03.010.
Keywords CFTR correctors, F508del-CFTR, Post-translational modifications, Regulatory insertion region, mutagenesis
Title: Nasal high-flow therapy as an adjunct to exercise in patients with cystic fibrosis: A pilot feasibility trial
Authors Giulia Spoletini, Ruth Watson, Wang Yng Lim, Kim Pollard, Christine Etherington, Ian J Clifton, Daniel G Peckham
References J Cyst Fibros. 2021 Apr 1;S1569-1993(21)00054-0. doi: 10.1016/j.jcf.2021.03.005.
Keywords 6-minute walking test, Dyspnoea, Exercise-induced desaturation, Nasal high-flow therapy
March 2021
Title: Elevated blood glucose during exacerbations of cystic fibrosis lung disease is associated with poor recovery of lung function.
Authors Michael Pallin, Shanal Kumar, Chris Daley, Shrinkhala Dawadi, Paul Leong, Erin Carr, Georgia Soldatos
References Cyst Fibros. 2021 Mar 26;S1569-1993(21)00053-9. doi: 10.1016/j.jcf.2021.03.004.
Keywords continuous glucose monitoring, FEV(1), Hyperglycaemia, Pulmonary exacerbation
