With the CF Research News, we aim to bridge the gap between people with CF and the researchers investigating CF and how best to treat those with the condition.
We want to provide access to patients, parents, relatives, friends and caregivers of patients with CF to all scientific work mainly published in the Journal of Cystic Fibrosis (JCF).

All PDF files published hereunder are lay version summaries of work published in the JCF.

June 2018

Title: A Specialized Method of Sputum Collection and Processing for Therapeutic Interventions in Cystic Fibrosis
Authors
McElvaney OJ, Gunaratnam C, Reeves EP, McElvaney NG
References
J Cyst Fibros. 2018 Jun 27. pii: S1569-1993(18)30625-8. doi: 10.1016/j.jcf.2018.06.001. 
Keywords
clinical trials, Cytokines, Induced sputum, Inflammation, Interleukin-1β, Neutrophil elastase
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June 2018

Title: RNA sequencing data from neutrophils of patients with cystic fibrosis reveals potential for developing biomarkers for pulmonary exacerbations
Authors
Kaiyu Jiang , Kerry E. Poppenberg, Laiping Wong, Yanmin Chen, Drucy Borowitz, Danielle Goetz, Daniel Sheehan, Carla Frederick , Vincent M. Tutino, Hui Meng, James N. Jarvis
References
https://doi.org/10.1016/j.jcf.2018.05.014
Keywords
Biomarkers, Transcriptome, Neutrophils, RNA, Sequencing
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June 2018

Title: Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent
Authors
Binyu Lu, Li Li Molly Schneider, Craig A. Hodges, James A. Burgess, Thomas J. Kelley
References
J Cyst Fibros. 2018 Jun 22. pii: S1569-1993(18)30630-1. doi: 10.1016/j.jcf.2018.06.005
Keywords
Cholesterol, Microtubule
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Scientific Abstract

June 2018

Title: Resistin is elevated in cystic fibrosis sputum and correlates negatively with lung function.
Authors
Forrest OA, Chopyk DM, Gernez Y, Brown MR, Conrad CK, Moss RB, Tangpricha V, Peng L, Tirouvanziam R.
References
J Cyst Fibros. 2018 Jun 21. pii: S1569-1993(18)30623-4. doi: 10.1016/j.jcf.2018.05.018.
Keywords
Biomarker, Inflammation, Neutrophils, Plasma, Sputum
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Title: AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations
Authors
Rebecca J. Darrah, Frank J. Jacono, Neha Joshi, Anna L. Mitchell, Abdus Sattar, Cara K. Campanaro, Paul Litman, Jennifer Frey, David E. Nethery, Eric S. Barbato, Craig A. Hodges, Harriet Corvol, Garry R. Cutting, Michael R. Knowles, Lisa J. Strug, Mitchell L Drumm
References
J Cyst Fibros. 2018 Jun 21. pii: S1569-1993(18)30618-0. doi: 10.1016/j.jcf.2018.05.013.
Keywords
Angiotensin signaling, GWAS follow up, Mouse models of airway disease, Pulmonary function
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June 2018

Title: SPX-101 is stable in and retains function after exposure to cystic fibrosis sputum.
Authors
Sesma JI, Wu B, Stuhlmiller TJ, Scott DW
References
J Cyst Fibros. 2018 Jun 20. pii: S1569-1993(18)30627-1. doi: 10.1016/j.jcf.2018.06.002
Keywords
Airway hydration, ENaC, Mucus, SPX-101
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June 2018

Title: Characteristics and outcomes of oral antibiotic treated pulmonary exacerbations in children with cystic fibrosis.
Authors
Hoppe JE, Wagner BD, Accurso FJ, Zemanick ET, Sagel SD
References
J Cyst Fibros. 2018 Jun 16. pii: S1569-1993(18)30620-9. doi: 10.1016/j.jcf.2018.05.015. 
Keywords
Infection, Inflammation, Lung function, Pulmonary exacerbation
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Scientific Abstract

June 2018

Title: Chronic Azithromycin Use in Cystic Fibrosis and Concern for Treatment-Emergent Pathogens
Authors
Cogen JD, Onchiri F, Emerson J, et al.
References
Ann Am Thorac Soc. 2018 Jun; 15(6): 702709 
Keywords
macrolides, Methicillin-resistant Staphylococcus aureus, microbiology
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Scientific Abstract
Title: Likelihood-based analysis of outcome-dependent sampling designs with longitudinal data.
Authors
Zelnick LR, Schildcrout JS, Heagerty PJ
References
Stat Med. 2018 Jun 15;37(13):2120-2133. doi: 10.1002/sim.7633.
Keywords
biased sampling, epidemiological study design, longitudinal data analysis
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Scientific Abstract

June 2018

Title: Combination potentiator ('co-potentiator') therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators.
Authors
Phuan PW, Son JH, Tan JA, Li C, Musante I, Zlock L, Nielson DW, Finkbeiner WE, Kurth MJ, Galietta LJ, Haggie PM, Verkman AS
References
J Cyst Fibros. 2018 Jun 11. pii: S1569-1993(18)30592-7. doi: 10.1016/j.jcf.2018.05.010.
Keywords
CFTR, High-throughput screen, N1303K, Potentiator
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June 2018

Title: Physiological and pharmacological characterization of the N1303K mutant CFTR.
Authors
DeStefano S, Gees M, Hwang TC
References
J Cyst Fibros. 2018 Jun 7. pii: S1569-1993(18)30593-9. doi: 10.1016/j.jcf.2018.05.011. 
Keywords
GLPG1837, Gating; N1303K, VX-661, VX-770, VX-809
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Scientific Abstract

June 2018

Title: Continuous glucose monitoring guided insulin therapy is associated with improved clinical outcomes in cystic fibrosis-related diabetes.
Authors
Freddy Frost, Dilip Nazareth, Paula Dyce, Victoria Malone, Martin J Walshaw
References
J Cyst Fibros. 2018 Jun 6. pii: S1569-1993(18)30587-3. doi: 10.1016/j.jcf.2018.05.005. 
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June 2018

Title: Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosis
Authors
Montgomery ST, Dittrich AS, Garratt LW, Turkovic L, Frey DL, Stick SM, Mall MA, Kicic A AREST CF
References
J Cyst Fibros. 2018 Jun 5. pii: S1569-1993(18)30588-5. doi: 10.1016/j.jcf.2018.05.006
Keywords
Airway epithelium, Hypoxia, Interleukin (IL)-1, Mucus, Neutrophilic inflammation
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Scientific Abstract

June 2018

Title: Measuring Knowledge of Disease  Management in Adolescents with Cystic Fibrosis: Initial Psychometric  Evaluation.
Authors
Bernstein RM, Riekert KA, Quittner AL
References
Pediatr Allergy Immunol Pulmonol. 2018;31(2):56‐65
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Scientific Abstract
Title: Measured fetal and neonatal exposure to Lumacaftor and Ivacaftor during pregnancy and while breastfeeding
Authors
Trimble A, McKinzie C, Terrell M, Stringer E, Esther CR Jr.
References
J Cyst Fibros. 2018 Jun 1. pii: S1569-1993(18)30591-5. doi: 10.1016/j.jcf.2018.05.009. 
Keywords
CFTR modulators, Ivacaftor, Lumacaftor, pregnancy
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Scientific Abstract