July 2020
Title: Clinical characteristics and outcomes associated with Inquilinus infection in cystic fibrosis
Authors Patricia M Lenhart-Pendergrass, Lindsay J Caverly, Brandie D Wagner, Scott D Sagel, Jerry A Nick, John J LiPuma, Stacey L Martiniano
References J Cyst Fibros. 2020 Jul 31;S1569-1993(20)30803-1. doi: 10.1016/j.jcf.2020.07.011. Online ahead of print.
Title: The impact of the COVID-19 pandemic on the emotional well-being and home treatment of Belgian patients with cystic fibrosis, including transplanted patients and children
Authors Trudy Havermans, Janne Houben, Francois Vermeulen, Mieke Boon, Marijke Proesmans, Natalie Lorent, Erik de Soir, Robin Vos, Lieven Dupont
References J Cyst Fibros. 2020 Jul 31;S1569-1993(20)30815-8. doi: 10.1016/j.jcf.2020.07.022. Online ahead of print.
Keywords COVID-19, Home treatment, Lung transplantation, Psychological impact
July 2020
Title: Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis
Authors Julie Safirstein; Jonathan J. Grant; Emily Clausen; Deepika Savant; Rebecca Dezube; Gina Hong
References J Cyst Fibros. 2020 Jul 29;S1569-1993(20)30806-7. doi: 10.1016/j.jcf.2020.07.014.
July 2020
Title: Developing Innovative GastroEnterology Specialty Training: the DIGEST model for expanding cystic fibrosis gastroenterologic expertise
Authors Sarah Shrager Lusman, Drucy Borowitz, Bruce C Marshall, Michael R Narkewicz, Tanja Gonska, Richard J Grand, Richard H Simon, Maria R Mascarenhas, Sarah Jane Schwarzenberg, Steven D Freedman
References J Cyst Fibros. 2020 Jul 28;S1569-1993(20)30805-5. doi: 10.1016/j.jcf.2020.07.012.
Keywords Gastroenterology, Hepatology, Nutrition, Training
July 2020
Title: Citrullination of extracellular histone H3.1 reduces antibacterial activity and exacerbates its proteolytic degradation
Authors Lloyd Tanner, Ravi K V Bhongir, Christofer A Q Karlsson, Sandy Le, Johanna K Ljungberg, Pia Andersson, Cecilia Andersson, Johan Malmström, Arne Egesten, Andrew B Single
References J Cyst Fibros. 2020 Jul 26;S1569-1993(20)30802-X. doi: 10.1016/j.jcf.2020.07.010. Online ahead of print.
Keywords AMPs, Citrullination, Innate immunity, PADI4, Pseudomonas aeruginosa
July 2020
Title: Prospective cohort study of ototoxicity in persons with cystic fibrosis following a single course of intravenous tobramycin
Authors Angela Garinis, Malcolm Gleser, Alexis Johns, Erik Larsen, Jay Vachhani
References J Cyst Fibros . 2020 Jul 23;S1569-1993(20)30793-1. doi: 10.1016/j.jcf.2020.07.001. Online ahead of print.
Keywords Aminoglycosides, Hearing loss, Ototoxicity, Tobramycin
July 2020
Title: Screening practices for nontuberculous mycobacteria at US cystic fibrosis centers
Authors Derek Low, Dulaney A Wilson, Patrick A Flume
References J Cyst Fibros. 2020 Jul;19(4):569-574. doi: 10.1016/j.jcf.2020.02.013. Epub 2020 Mar 13.
Keywords Epidemiology, nontuberculous mycobacteria
July 2020
Title: Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis
Authors Emily DiMango, Jonathan Overdevest, Claire Keating, Sarah Fracasso Francis, David Dansky, David Gudis
References J Cyst Fibros. 2020 Jul 18;S1569-1993(20)30794-3. doi: 10.1016/j.jcf.2020.07.002.
Keywords Highly effective modulator, Quality of life, SNOT-22, Sinonasal
July 2020
Title: Evaluating assumptions of definition-based pulmonary exacerbation endpoints in cystic fibrosis clinical trials
Authors Donald R VanDevanter, Nicole Mayer Hamblett, Noah Simon, Joseph McIntosh, Michael W Konstan
References J Cyst Fibros. 2020 Jul 15;S1569-1993(20)30800-6. doi: 10.1016/j.jcf.2020.07.008.
July 2020
Title: Efficacy and safety of ataluren in patients with nonsense-mutation cystic fibrosis not receiving chronic inhaled aminoglycosides: the international, randomized, double-blind, placebo-controlled Ataluren Confirmatory Trial in Cystic Fibrosis (ACT CF)
Authors MW Konstan, DR VanDevanter, SM Rowe, M Wilschanski, E Kerem, I Sermet-Gaudelus, E DiMango, P Melotti, J McIntosh, K De Boeck on behalf of the ACT CF Study Group
References J Cyst Fibros. 2020 Jul;19(4):595-601. doi: 10.1016/j.jcf.2020.01.007. Epub 2020 Jan 23.
Keywords Ataluren, Clinical trial
Title: CFTR modulator therapy for cystic fibrosis caused by the rare c.3700A>G mutation
Authors Puay-Wah Phuan, Peter M Haggie, Joseph A Tan, Amber A Rivera, Walter E Finkbeiner, Dennis W Nielson, Merlin M Thomas, Ibrahim A Janahi, Alan S Verkman
References J Cyst Fibros . 2020 Jul 14;S1569-1993(20)30795-5. doi: 10.1016/j.jcf.2020.07.003.
Keywords C.3700A>G, CFTR modulators, Co-potentiators, I1234V, N-of-1 clinical trials
July 2020
Title: A four week trial of hypertonic saline in children with mild cystic fibrosis lung disease: Effect on mucociliary clearance and clinical outcomes
Authors Scott H Donaldson, T Danielle Samulski, Caroline LaFave, Kirby Zeman, Jihong Wu, Aaron Trimble, Agathe Ceppe, William D Bennett, Stephanie D Davis
References J Cyst Fibros. 2020 Jul 12;S1569-1993(20)30801-8. doi: 10.1016/j.jcf.2020.07.009.
Keywords clinical trials, Clinicaltrials.gov registration number, NCT01031706, Hypertonic saline, Mucociliary clearance
July 2020
Title: Evaluation of airway and circulating inflammatory biomarkers for cystic fibrosis drug development
Authors Raksha Jain, Arthur Baines, Umer Khan, Brandie D Wagner, Scott D Sagel
References J Cyst Fibros . 2020 Jul 1;S1569-1993(20)30782-7. doi: 10.1016/j.jcf.2020.06.017.
Keywords Biomarkers, Inflammation, Lung function, Sputum