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 published in the Journal of Cystic Fibrosis (JCF).

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

March 2017

Title: Real-life initiation of lumacaftor/ivacaftor combination in adults with cystic fibrosis homozygous for the Phe508del CFTR mutation and severe lung disease
References
J Cyst Fibros. 2017 Mar 15. pii: S1569-1993(17)30086-3

March 2017

Title: The CF-CARES primary palliative care model: A CF-specific structured assessment of symptoms, distress, and coping. distress, and coping.
References
J Cyst Fibros. 2017 Mar 14. pii: S1569-1993(17)30077-2.
Title: Immediate Effects of Lumacaftor/Ivacaftor Administration on Lung Function in Patients with Severe Cystic Fibrosis Lung Disease
References
 2017 Mar 14. pii: S1569-1993(17)30075-9

March 2017

Title: Feasibility of placebo-controlled trial designs for new CFTR modulator evaluation
References
J Cyst Fibros. 2017 Mar 8. pii: S1569-1993(17)30078-4
Title: Association between glucose intolerance and bacterial colonisation in an adult population with cystic fibrosis, emergence of Stenotrophomonas maltophilia.
References
J Cyst Fibros. 2017 Mar 8. pii: S1569-1993(17)30025-5.

March 2017

Title: Pilot trial of tobramycin inhalation powder in cystic fibrosis patients with chronic Burkholderia cepacia complex infection
References
J Cyst Fibros. 2017 Mar 2. pii: S1569-1993(17)30074-7. 
Theme
CFTR/Basic Science
Title: Peripheral muscle abnormalities in cystic fibrosis: etiology, clinical implications and response to therapeutic interventions
References
J Cyst Fibros. 2017 Mar 2. pii: S1569-1993(17)30032-2.

February 2017

Title: The Cumulative Effects of Intravenous Antibiotic Treatments on Hearing in Patients with Cystic Fibrosis
References
J Cyst Fibros. 2017 Feb 23. pii: S1569-1993(17)30013-9.

February 2017

Title: Mycobacterium abscessus, Mycobacterium avium, Non-tuberculous mycobacteria, Pulmonary disease
References
J Cyst Fibros. 2017 Feb 12. pii: S1569-1993(17)30024-3

February 2017

Title: Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosis.
References
J Cyst Fibros. 2017 Feb 7. pii: S1569-1993(17)30020-6.

February 2017

Title: Fatal disseminated Rasamsonia infection in cystic fibrosis post-lung transplantation
References
J Cyst Fibros. 2017 Feb 6. pii: S1569-1993(17)30012-7.

February 2017

Title: Pregnancy among cystic fibrosis women in the area of CFTR modulators
References
Title: The 1-min sit-to-stand test in cystic fibrosis - Insights into cardiorespiratory responses.
References
J Cyst Fibros. 2017 Feb 7. pii: S1569-1993(17)30019-X

January 2017

Title: Effect of freezing sputum on Pseudomonas aeruginosa population heterogeneity
References
J Cyst Fibros. 2017 Jan 23. pii: S1569-1993(17)30011-5.

January 2017

Title: Correlation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor
References
J Cyst Fibros. 2017 Jan;16(1):41-44.

January 2017

Title: Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis.
References
J Cyst Fibros. 2017 Jan 26. pii: S1569-1993(16)30673-7.
Title: Changes of CFTR functional measurements and clinical improvements in Cystic Fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor
References
J Cyst Fibros. 2017 Jan;16(1):45-48

December 2016

Title: BMI Fails to Identify Poor Nutritional Status in Stunted Children with CF
References

J Cyst Fibros. 2016 Dec 1. pii: S1569-1993(16)30663-4

Theme
Nutrition/Gastroenterology
Title: Non-invasive prenatal diagnosis (NIPD) of Cystic Fibrosis: an optimized protocol using MEMO fluorescent PCR to detect the p.Phe508del mutation
References
J Cyst Fibros. 2016 Dec 28. pii: S1569-1993(16)30680-4
Title: Impact of azithromycin on the clinical and antimicrobial effectiveness of tobramycin in the treatment of cystic fibrosis
References
J Cyst Fibros. 2016 Dec 24. pii: S1569-1993(16)30672-5.
Title: Increasing sputum levels of gamma-glutamyltransferase may identify cystic fibrosis patients who do not benefit from inhaled glutathione.
References
J Cyst Fibros. 2016 Dec 14. pii: S1569-1993(16)30671-3.
Title: The psychometric properties of the Leicester Cough Questionnaire and Respiratory Symptoms in CF tool in cystic fibrosis.
References
 2016 Dec 13. pii: S1569-1993(16)30669-5
Title: Environmental Scan of Cystic Fibrosis Research Worldwide
References
J Cyst Fibros. 2016 Dec 1. pii: S1569-1993(16)30655-5

November 2016

Title: Oxidized glutathione and uric acid as biomarkers of early cystic fibrosis lung disease
References

J Cyst Fibros. 2016 Nov 24. pii: S1569-1993(16)30647-6

Title: Pleural Effusions in Non-Transplanted Cystic Fibrosis Patients
References
Title: Bacterial proteases and haemostasis dysregulation in the CF lung.
References

J Cyst Fibros. 2016 Nov 10.

Title: Continuous alternating inhaled antibiotic therapy in CF: a single center retrospective analysis.
References

J Cyst Fibros. 2016 Nov;15(6):802-808.

Title: Biomarkers for Cystic Fibrosis Drug Development
References

dx.doi.org/10.1016/j.jcf.2016.10.009

Title: The fate of inhaled antibiotics after deposition in cystic fibrosis: how to get drug to the bug?
References

dx.doi.org/10.1016/j.jcf.2016.10.001 show

Title: Multilocus amplicon sequencing of Pseudomonas aeruginosa cystic fibrosis airways isolates collected prior to and after early antipseudomonal chemotherapy
References

J Cyst Fibros. 2016 Nov 8. pii: S1569-1993(16)30648-8

Title: Pleural Effusions in Non-Transplanted Cystic Fibrosis Patients
References
Title: Biomarkers for Cystic Fibrosis Drug Development
References

dx.doi.org/10.1016/j.jcf.2016.10.009

Title: Continuous alternating inhaled antibiotic therapy in CF: a single center retrospective analysis.
References

J Cyst Fibros. 2016 Nov;15(6):802-808.

Title: The fate of inhaled antibiotics after deposition in cystic fibrosis: how to get drug to the bug?
References

dx.doi.org/10.1016/j.jcf.2016.10.001 show

Title: Multilocus amplicon sequencing of Pseudomonas aeruginosa cystic fibrosis airways isolates collected prior to and after early antipseudomonal chemotherapy
References

J Cyst Fibros. 2016 Nov 8. pii: S1569-1993(16)30648-8

Title: Bacterial proteases and haemostasis dysregulation in the CF lung.
References

J Cyst Fibros. 2016 Nov 10.

October 2016

Title: Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes
References

J Cyst Fibros. 2016 Oct 11. pii: S1569-1993(16)30611-7.

Theme
Epidemiology/Models of care
Title: Coronary artery disease in Cystic Fibrosis: An Emerging Concern?
References

J Cyst Fibros. 2016 Oct 14. pii: S1569-1993(16)30612-9

Title: Diagnostic and prognostic significance of systemic alkyl quinolones for P. aeruginosa in cystic fibrosis: a longitudinal study
References

J Cyst Fibros. 2016 Oct 20. pii: S1569-1993(16)30617-8

Title: Clostridium difficile carriage in adult cystic fibrosis (CF); implications for patients with CF and the potential for transmission of nosocomial infection.
References
Title: Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes
References

J Cyst Fibros. 2016 Oct 11. pii: S1569-1993(16)30611-7.

Theme
Epidemiology/Models of care
Title: Coronary artery disease in Cystic Fibrosis: An Emerging Concern?
References

J Cyst Fibros. 2016 Oct 14. pii: S1569-1993(16)30612-9

Title: A cross-sectional analysis of daytime versus nocturnal polysomnographic respiratory parameters in cystic fibrosis during early adolescence.
References

J Cyst Fibros. 2016 Oct 8. pii: S1569-1993(16)30609-9

Theme
Pulmonology
Title: Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation
References

J Cyst Fibros. 2016 Oct 10. pii: S1569-1993(16)30605-1

Theme
CFTR/Basic Science
Title: Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation
References

J Cyst Fibros. 2016 Oct 1. pii: S1569-1993(16)30607-5

September 2016

Title: Effect of probiotics on respiratory, gastrointestinal and nutritional outcomes in patients with cystic fibrosis: A systematic review
References

J Cyst Fibros. 2016 Sep 29. pii: S1569-1993(16)30606-3

Theme
Nutrition/Gastroenterology
Title: Adherence to infection prevention and control guidelines: A vignette-based study of decision-making and risk-taking in young adults with cystic fibrosis.
References
Title: Nasal potential difference outcomes support diagnostic decisions in cystic fibrosis
References

J Cyst Fibros. 2016 Sep;15(5):579-82.

Theme
Diagnosis/Screening
Title: Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines
References

J Cyst Fibros. 2016 Sep 3. pii: S1569-1993(16)30594-X

Theme
Nutrition/Gastroenterology
Title: Potentiator synergy in rectal organoids carrying S1251N, G551D or F508del CFTR mutations
References

J Cyst Fibros. 2016 Sep;15(5):568-78

Theme
CFTR/Basic Science
Title: Cancer Risk among Lung Transplant Recipients with Cystic Fibrosis
References

J Cyst Fibros. 2016 Aug 15. pii: S1569-1993(16)30568-9

August 2016

Title: Reduced survival in adult cystic fibrosis despite attenuated lung function decline.
References

J Cyst Fibros. 2016 Aug 10. pii: S1569-1993(16)30569-0.

Theme
Pulmonology
Title: The changing prevalence of pulmonary infection in adults with cystic fibrosis: A longitudinal analysis
References

J Cyst Fibros. 2016 Aug 8. pii: S1569-1993(16)30567-7

Title: Long-term work participation among cystic fibrosis patients undergoing lung transplantation
References

J Cyst Fibros. 2016 Aug 3. pii: S1569-1993(16)30564-1.

Theme
Epidemiology/Models of care

July 2016

Title: Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients.
References

J Cyst Fibros. 2016 Jul 28. pii: S1569-1993(16)30565-3.

Title: The Effects of Ivacaftor on CF Fatty Acid Metabolism: An Analysis from the GOAL Study
References

J Cyst Fibros. 2016 Jul 26. pii: S1569-1993(16)30563-X.

Theme
Nutrition/Gastroenterology
Title: Ivacaftor and symptoms of extra-oesophageal reflux in patients with cystic fibrosis and G551D mutation
References

J Cyst Fibros. 2016 Jul 27. pii: S1569-1993(16)30561-6

Theme
Nutrition/Gastroenterology
Title: Limitations to providing adult cystic fibrosis care in Europe: Results of a care centre survey
References

J Cyst Fibros. 2016 Jul 25. pii: S1569-1993(16)30558-6

Theme
Epidemiology/Models of care
Title: A Product of Immunoreactive Trypsinogen and Pancreatitis-Associated Protein as Second Tier Strategy in Cystic Fibrosis Newborn Screening
References

J Cyst Fibros. 2016 Jul 22. pii: S1569-1993(16)30559-8

Theme
Diagnosis/Screening

June 2016

Title: IL-22 exacerbates weight loss in a murine model of chronic pulmonary Pseudomonas aeruginosa infection
References

J Cyst Fibros. 2016 Jun 30. pii: S1569-1993(16)30553-7

Theme
Immunology
Title: A Novel Guluronate Oligomer Improves Intestinal Transit and Survival in Cystic Fibrosis Mice
References

J Cyst Fibros. 2016 Jun 30. pii: S1569-1993(16)30553-7

Theme
Nutrition/Gastroenterology
Title: Triazole resistance in Aspergillus fumigatus isolates from patients with cystic fibrosis in Italy.
References

J Cyst Fibros. 2016 Jun 26. pii: S1569-1993(16)30551-3.

Theme
Microbiology
Title: Objective airway artery dimensions compared to CT scoring methods assessing structural cystic fibrosis lung disease
References

J Cyst Fibros. 2016 Jun 21. pii: S1569-1993(16)30522-7

Title: Longitudinal study of Stenotrophomonas maltophilia antibody levels and outcomes in cystic fibrosis patients.
References

J Cyst Fibros. 2016 Jun 23. pii: S1569-1993(16)30552-5.

Title: Risk of asthma in heterozygous carriers for cystic fibrosis: a meta-analysis
References

J Cyst Fibros. 2016 Jun 17. pii: S1569-1993(16)30524-0

Theme
Epidemiology/Models of care
Title: Multiple Prevalent Fractures in Relation to Macroscopic Bone Architecture in Patients with Cystic Fibrosis
References

J Cyst Fibros. 2016 Jun 17. pii: S1569-1993(16)30547-1

Title: Airway inflammation in mild cystic fibrosis.
References

J Cyst Fibros. 2016 Jun 10. pii: S1569-1993(16)30523-9

Title: Respiratory Pathogens Mediate the Association between Lung Function and Temperature in Cystic Fibrosis
References

J Cyst Fibros. 2016 Jun 10. pii: S1569-1993(16)30514-8.

Title: Pancreatic Elastase-1 Quick Test for rapid assessment of pancreatic status in cystic fibrosis patients.
References

J Cyst Fibros. 2016 Jun 7. pii: S1569-1993(16)30511-2.

Title: Bioelectrical impedance in young patients with cystic fibrosis: validation of a specific equation and clinical relevance
References

J Cyst Fibros. 2016 Jun 8. pii: S1569-1993(16)30053-4

Title: Cystic fibrosis in Latin America - Improving the awareness
References

J Cyst Fibros. 2016 Jun 1. pii: S1569-1993(16)30509-4.

Title: Sino-nasal Inhalation of Isotonic versus Hypertonic Saline (6.0 %) in CF Patients with Chronic Rhinosinusitis – Results of a Multicentre, Prospective, Randomized, Double-blind, Controlled Trial
References

J Cyst Fibros. 2016 Jun 4. pii: S1569-1993(16)30052-2

Theme
Clinical Trials

May 2016

Title: A false positive newborn screening result due to a complex allele carrying two frequent CF-causing variants.
References

J Cyst Fibros. 2016 May;15(3):309-12

Theme
Newborn screening
Title: A false positive newborn screening result due to a complex allele carrying two frequent CF-causing variants.
References

J Cyst Fibros. 2016 May;15(3):309-12

Theme
Newborn screening
Title: Episodic oral antibiotic use in CF: discordance between the electronic medical record and self-report
References

J Cyst Fibros. 2016 May 4. pii: S1569-1993(16)30044-3

Theme
Epidemiology/Models of care

April 2016

Title: Controversies with Kalydeco: Newspaper Coverage in Canada and the Unites States of the Cystic Fibrosis “Wonder Drug”
References

J Cyst Fibros. 2016 Apr 14. pii: S1569-1993(16)30015-7

Theme
Epidemiology/Models of care
Title: Optimizing beta-lactam pharmacodynamics against Pseudomonas aeruginosa in adult cystic fibrosis patients.
References

J Cyst Fibros. 2016 Apr 27. pii: S1569-1993(16)30019-4.

Theme
Microbiology

March 2016

Title: Comparison of two pancreatic enzyme products for exocrine insufficiency in patients with cystic fibrosis
References

J Cyst Fibros. 2016 Mar 21. pii: S1569-1993(16)30001-7

Theme
Nutrition/Gastroenterology
Title: Extensive cultivation of soil and water samples yields various pathogens in patients with cystic fibrosis but not Burkholderia multivorans.
References

J Cyst Fibros. 2016 Mar 17. pii: S1569-1993(16)30005-4

Theme
Microbiology
Title: Disease disclosure in individuals with cystic fibrosis: Association with psychosocial and health outcomes
References

J Cyst Fibros. 2016 Mar 17. pii: S1569-1993(16)30002-9

Title: Evaluation of a systems biology approach to identify pharmacological correctors of the mutant CFTR chloride channel
References

J Cyst Fibros. 2016 Mar 10. pii: S1569-1993(16)00057-6

Theme
CFTR/Basic Science

February 2016

Title: Clinical outcomes in cystic fibrosis patients with Trichosporon respiratory infection
References

J Cyst Fibros. 2016 Feb 27. pii: S1569-1993(16)00037-0

Theme
Microbiology
Title: Safety and Efficacy of Prolonged Levofloxacin Inhalation Solution (1 APT-1026) Treatment for Cystic Fibrosis and Chronic Pseudomonas aeruginosa Airway Infection
References

J Cyst Fibros. 2016 Feb 26. pii: S1569-1993(16)00012-6

Theme
Clinical Trials
Title: The role of basophil activation test in allergic bronchopulmonary aspergillosis and Aspergillus fumigatus sensitization in cystic fibrosis patients
References

J Cyst Fibros. 2016 Feb 25. pii: S1569-1993(16)00016-3

Theme
Epidemiology/Models of care
Title: Glycated hemoglobin cannot yet be proposed as a screening tool for cystic fibrosis related diabetes
References

J Cyst Fibros. 2016 Feb 19. pii: S1569-1993(16)00017-5

Theme
Diagnosis/Screening
Title: International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: associated factors and outcome.
References

J Cyst Fibros. 2016 Feb 23. pii: S1569-1993(16)00014-X.

Theme
Nutrition/Gastroenterology
Title: A Phase 3, Multi-center, Multinational, Randomized, Double-blind, Placebo-controlled Study to Evaluate the Efficacy and Safety of Levofloxacin Inhalation Solution (APT-1026) in Stable Cystic Fibrosis Patients
References

J Cyst Fibros. 2016 Feb 4. pii: S1569-1993(15)00276-3.

Theme
Clinical Trials

January 2016

Title: Measurement of fecal elastase improves performance of newborn screening for cystic fibrosis.
References

J Cyst Fibros. 2016 Jan 27. pii: S1569-1993(16)00002-3

Theme
Newborn screening
Title: Host Response to Staphylococcus aureus Cytotoxins In Children with Cystic
References

J Cyst Fibros. 2016 Jan 25. pii: S1569-1993(15)00318-5

Theme
Microbiology
Title: YKL-40 as marker of severe lung disease in cystic fibrosis patients.
References

J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00315-X.

Theme
Immunology
Title: Low sodium status in cystic fibrosis-as assessed by calculating fractional Na+ excretion-is associated with decreased growth parameters.
References

J Cyst Fibros. 2016 Jan 6. pii: S1569-1993(15)00282-9

Theme
Nutrition/Gastroenterology
Title: Microbiological yield from induced sputum compared to oropharyngeal swab in young children with cystic fibrosis
References

J Cyst Fibros. 2016 Jan 26. pii: S1569-1993(16)00008-4.

Theme
Microbiology
Title: Ultrasound and magnetic resonance imaging assessment of joint disease in symptomatic patients with Cystic Fibrosis Arthropathy
References

J Cyst Fibros. 2016 Jan 19. pii: S1569-1993(15)00317-3

Theme
Imaging
Title: Implementation of newborn screening for Cystic Fibrosis in Norway. Results from the first three years
References

J Cyst Fibros. 2016 Jan 12. pii: S1569-1993(15)00312-4

Theme
Newborn screening
Title: Distribution of the species of Achromobacter in a French Cystic Fibrosis centre and Multilocus Sequence Typing analysis reveal the predominance of A. xylosoxidans and clonal relationships between some clinical and environmental isolates
References

J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00281-7

Theme
Microbiology
Title: Gastric emptying and gastro-oesophageal reflux in children with cystic fibrosis
References

J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00287-8

Theme
Nutrition/Gastroenterology

December 2015

Title: Preliminary Comparison of Normalized T1 and Non-Contrast Perfusion MRI Assessments of Regional Lung Disease in People with Cystic Fibrosis
References

J Cyst Fibros. 2015 Dec 21. pii: S1569-1993(15)00267-2

Theme
Imaging
Title: Psychological resilience and intolerance of uncertainty in coping with cystic fibrosis
References

J Cyst Fibros. 2015 Dec 10. pii: S1569-1993(15)00269-6

Title: Epidemic Pseudomonas aeruginosa Infection in Patients with Cystic Fibrosis is Not a Risk Factor for Poor Clinical Outcomes Following Lung Transplantation
References

J Cyst Fibros. 2015 Dec 2. pii: S1569-1993(15)00262-3

Title: Bronchial epithelial cell lines and primary nasal epithelial cells from cystic fibrosis respond differently to cigarette smoke exposure
References

J Cyst Fibros. 2015 Dec 1. pii: S1569-1993(15)00260-X

Theme
CFTR/Basic Science