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
