All Resources

This page provides filters and search facilities across all types of CF resources contained within the ECFS website.

Additionally, you can visit the specific resource type pages as follows:

More information on CF publications can be found here: Publications

May 2017

Title: AP2 α modulates cystic fibrosis transmembrane conductance regulator function in the human intestine
References
J Cyst Fibros. 2017 May;16(3):327-334.

May 2017

Title: Inhaled dry powder mannitol in children with cystic fibrosis: A randomised efficacy and safety trial.
References
J Cyst Fibros. 2017 May;16(3):380-387.
Title: Modelling Cystic Fibrosis Disease Progression in Patients with the Rare CFTR Mutation P67L
References
J Cyst Fibros. 2017 May;16(3):335-341.

May 2017

Title: Is there an association between back pain and stress incontinence in adults with cystic fibrosis? A retrospective cross-sectional study.
References
J Cyst Fibros. 2017 May 2. pii: S1569-1993(17)30102-9

April 2017

Title: Standardized Treatment of Pulmonary Exacerbations (STOP) study: Observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations.
References
J Cyst Fibros. 2017 Apr 28. pii: S1569-1993(17)30101-7.

April 2017

Title: Standardized Treatment of Pulmonary exacerbations (STOP) study: Physician treatment practices and outcomes for individuals with cystic fibrosis with pulmonary exacerbations.
References
J Cyst Fibros. 2017 Apr 27. pii: S1569-1993(17)30099-1.

April 2017

Title: Does current reporting of lung function by the UK cystic fibrosis registry allow a fair comparison of adult centres?
References
J Cyst Fibros. 2017 Apr 24. pii: S1569-1993(17)30103-0.

April 2017

Title: Risk Factors for Persistent Methicillin-resistant Staphylococcus aureus Infection in Cystic Fibrosis
References
J Cyst Fibros. 2017 Apr 23. pii: S1569-1993(17)30106-6.

April 2017

Title: Feasibility and normal values of an integrated conductivity (Nanoduct) sweat test system in healthy newborns
References
J Cyst Fibros. 2017 Apr 22. pii: S1569-1993(17)30098-X.

April 2017

Title: Rationalizing endpoints for prospective studies of pulmonary exacerbation treatment response in cystic fibrosis
References
J Cyst Fibros. 2017 Apr 21. pii: S1569-1993(17)30100-5
 

April 2017

Title: CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis
References
J Cyst Fibros. 2017 Apr 17. pii: S1569-1993(17)30094-2.

April 2017

Title: Age-dependent variation of fecal calprotectin in cystic fibrosis and healthy children.
References
J Cyst Fibros. 2017 Apr 14. pii: S1569-1993(17)30093-0

April 2017

Title: A treatment evaluator tool to monitor the real-world effectiveness of inhaled aztreonam lysine in cystic fibrosis.
References
J Cyst Fibros. 2017 Apr 6. pii: S1569-1993(17)30031-0. 

April 2017

Title: Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth
References
J Cyst Fibros. 2017 Apr 1. pii: S1569-1993(17)30090-5

March 2017

Title: A 3-year prognostic score for adults with cystic fibrosis.
References
J Cyst Fibros. 2017 Mar 18. pii: S1569-1993(17)30087-5

March 2017

Title: Nutritional status, nutrient intake and use of enzyme supplements in paediatric patients with Cystic Fibrosis; a European multicentre study with reference to current guidelines
References
J Cyst Fibros. 2017 Mar 17. pii: S1569-1993(17)30088-7
Theme
Nutrition/Gastroenterology

March 2017

Title: When is too little care, too much harm in cystic fibrosis? Psychological and ethical approaches to the problem.
References
J Cyst Fibros. 2017 Mar;16(2):299-303
Title: Clostridium difficile carriage in adult cystic fibrosis (CF); implications for patients with CF and the potential for transmission of nosocomial infection.
References
 2017 Mar;16(2):291-298.

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: Technological advances shed light on left ventricular cardiac disturbances in cystic fibrosis
References
J Cyst Fibros. 2017 Mar 14. pii: S1569-1993(17)30080-2
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: Pregnancy among cystic fibrosis women in the area of CFTR modulators
References
 2017 Feb 9. pii: S1569-1993(17)30015-2

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.
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

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.

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.

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.
Title: The fate of inhaled antibiotics after deposition in cystic fibrosis: how to get drug to the bug?
References
 2017 Jan;16(1)
Title: The fate of inhaled antibiotics after deposition in cystic fibrosis: how to get drug to the bug?
References
 2017 Jan;16(1):13-23.
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
 2017 Jan;16

January 2017

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: 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

December 2016

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.

December 2016

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.

December 2016

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

December 2016

Title: Pleural Effusions in Non-Transplanted Cystic Fibrosis Patients
References
 2016 Dec 12. pii: S1569-1993(16)30664-6.
Title: Pleural Effusions in Non-Transplanted Cystic Fibrosis Patients
References
 2016 Dec 12. 

December 2016

Title: Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis
References
Waters V, Ratjen F. Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2016, Issue 12. Art. No.: CD010004. DOI: 10.1002/14651858.CD010004.pub4
Theme
Lung Disease and Infection
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: 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

November 2016

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
 2016 Nov;15(6):714-723
Title: Continuous alternating inhaled antibiotic therapy in CF: a single center retrospective analysis.
References
J Cyst Fibros. 2016 Nov;15(6):802-808.

November 2016

Title: Bacterial proteases and haemostasis dysregulation in the CF lung.
References
J Cyst Fibros. 2016 Nov 10.
Title: Bacterial proteases and haemostasis dysregulation in the CF lung.
References
J Cyst Fibros. 2016 Nov 10.

November 2016

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: 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

November 2016

Title: Eradication therapy for Burkholderia cepacia complex in people with cystic fibrosis
References
Regan KH, Bhatt J. Eradication therapy for Burkholderia cepacia complex in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2016, Issue 11. Art. No.: CD009876. DOI: 10.1002/14651858.CD009876.pub3
Theme
Lung Disease and Infection
Title: Biomarkers for Cystic Fibrosis Drug Development
References

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

October 2016

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

October 2016

Title: Coronary artery disease in Cystic Fibrosis: An Emerging Concern?
References
J Cyst Fibros. 2016 Oct 14. pii: S1569-1993(16)30612-9
Title: Coronary artery disease in Cystic Fibrosis: An Emerging Concern?
References
J Cyst Fibros. 2016 Oct 14. pii: S1569-1993(16)30612-9

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: 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

October 2016

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

October 2016

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

October 2016

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

September 2016

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: 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

September 2016

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

September 2016

Title: Duration of intravenous antibiotic therapy in people with cystic fibrosis
References
Plummer A, Wildman M, Gleeson T. Duration of intravenous antibiotic therapy in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2016, Issue 9. Art. No.: CD006682. DOI: 10.1002/14651858.CD006682.pub5
Theme
Lung Disease and Infection

August 2016

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

August 2016

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

August 2016

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.

July 2016

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

July 2016

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

July 2016

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

July 2016

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

June 2016

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

June 2016

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.

June 2016

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

June 2016

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

June 2016

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.

June 2016

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

June 2016

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.

June 2016

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

June 2016

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

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

May 2016

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

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

April 2016

Title: Clinical Practice Guidelines from the Cystic Fibrosis Foundation for Preschoolers with Cystic Fibrosis
References
Pediatrics. 2016 April; 137(4), DOI:10.1542/peds.2015-1784
Theme
Care of patients with CF, Clinical Trials, Pregnancy with CF
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