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

March 2018

Title: Defining Research Priorities in Cystic Fibrosis. Can Existing Knowledge and Training in Biomedical Research affect the Choice?
References
J Cyst Fibros. 2018 Mar 15. pii: S1569-1993(18)30032-8. doi: 10.1016/j.jcf.2018.02.009.

March 2018

Title: Implementing the International Committee on Mental Health in Cystic Fibrosis (ICMH) Guidelines: Screening accuracy and referral-treatment pathways
References
J Cyst Fibros. 2018 Mar 1. pii: S1569-1993(18)30028-6. doi: 10.1016/j.jcf.2018.02.005.

February 2018

Title: Cystic fibrosis epithelial cells are primed for apoptosis as a result of increased Fas (CD95).
References
J Cyst Fibros. 2018 Feb 24. pii: S1569-1993(18)30013-4. doi: 10.1016/j.jcf.2018.01.010.

February 2018

Title: Molecular characterization of gene regulatory networks in primary human tracheal and bronchial epithelial cells.
References
J Cyst Fibros. 2018 Feb 17. pii: S1569-1993(18)30012-2. doi: 10.1016/j.jcf.2018.01.009

February 2018

Title: Mast cell tryptase changes with Aspergillus fumigatus - Host crosstalk in cystic fibrosis patients.
References
J Cyst Fibros. 2018 Feb 15. pii: S1569-1993(18)30015-8. doi: 10.1016/j.jcf.2018.01.012.

February 2018

Title: Risk factors for persistent Aspergillus respiratory isolation in cystic fibrosis.
References
J Cyst Fibros. 2018 Feb 12. pii: S1569-1993(18)30011-0. doi: 10.1016/j.jcf.2018.01.008

February 2018

Title: Early detection using qPCR of Pseudomonas aeruginosa infection in children with cystic fibrosis undergoing eradication treatment
References

February 2018

Title: Comparison of facemask and mouthpiece interfaces for Multiple Breath Washout measurements
References

January 2018

Title: Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis.
References
J Cyst Fibros. 2018 Jan 30. pii: S1569-1993(18)30009-2. doi: 10.1016/j.jcf.2018.01.006.

January 2018

Title: The effect of enteral tube feeding in cystic fibrosis: A registry based study.
References
J Cyst Fibros. 2018 Jan 21. pii: S1569-1993(18)30007-9. doi: 10.1016/j.jcf.2018.01.004.

January 2018

Title: Hypertonic saline has a prolonged effect on mucociliary clearance in adults with cystic fibrosis
References
J Cyst Fibros. 2018 Jan 19. pii: S1569-1993(18)30004-3. doi: 10.1016/j.jcf.2018.01.001

January 2018

Title: The relationship between sweat chloride levels and mortality in cystic fibrosis varies by individual genotype.
References
J Cyst Fibros. 2018 Jan;17(1):34-42. doi: 10.1016/j.jcf.2017.11.002. Epub 2017 Dec 6.
Title: Drugs during pregnancy and breast feeding in women diagnosed with Cystic Fibrosis – an update
References
J Cyst Fibros. 2018 Jan;17(1):17-25. doi: 10.1016/j.jcf.2017.11.009. Epub 2017 Dec 7

January 2018

Title: One time quantitative PCR detection of Pseudomonas aeruginosa to discriminate intermittent from chronic infection in cystic fibrosis
References
 2018 Jan 11. pii: S1569-1993(18)30001-8. doi: 10.1016/j.jcf.2017.12.007

January 2018

Title: Quantifying fluctuation in glucose levels to identify early changes in glucose homeostasis in cystic fibrosis.
References
J Cyst Fibros. 2018 Jan 9. pii: S1569-1993(17)30982-7. doi: 10.1016/j.jcf.2017.12.004

December 2017

Title: KB001-A, a novel anti-inflammatory, found to be safe and well-tolerated in cystic fibrosis patients infected with Pseudomonas aeruginosa.
References
J Cyst Fibros. 2017 Dec 29. pii: S1569-1993(17)30984-0. doi: 10.1016/j.jcf.2017.12.006

December 2017

Title: Quality of life and mood in children with cystic fibrosis: Associations with sleep quality.
References
J Cyst Fibros. 2017 Dec 24. pii: S1569-1993(17)30978-5. doi: 10.1016/j.jcf.2017.11.021.

December 2017

Title: The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosis
References
J Cyst Fibros. 2017 Dec 21. pii: S1569-1993(17)30980-3. doi: 10.1016/j.jcf.2017.12.002

December 2017

Title: Effect of Lumacaftor/Ivacaftor on glucose metabolism and insulin secretion in Phe508del homozygous cystic fibrosis patients
References
J Cyst Fibros. 2017 Dec 15. pii: S1569-1993(17)30973-6. doi: 10.1016/j.jcf.2017.11.016.
Title: Hypoglycaemia (low blood sugar) in cystic fibrosis: an analysis of single centre adult cystic fibrosis clinic
References
J Cyst Fibros. 2017 Dec 15. pii: S1569-1993(17)30972-4. doi: 10.1016/j.jcf.2017.11.015.

December 2017

Title: Resolvin D1 regulates epithelial ion transport and inflammation in cystic fibrosis airways
References
J Cyst Fibros. 2017 Dec 7. pii: S1569-1993(17)30974-8. doi: 10.1016/j.jcf.2017.11.017

November 2017

Title: FAM13A is a modifier gene of cystic fibrosis lung phenotype regulating rhoa activity, actin cytoskeleton dynamics and epithelial-mesenchymal transition
References

November 2017

Title: Development and electronic validation of the revised Cystic Fibrosis Questionnaire (CFQ-R Teen/Adult): New tool for monitoring psychosocial health in CF.
References
J Cyst Fibros. 2017 Nov 17. pii: S1569-1993(17)30954-2. doi: 10.1016/j.jcf.2017.10.015.
Theme
Clinical Trials, Diabetes, Diagnosis/Screening, Endocrinology, Epidemiology/Models of care, Exercise, False-positives, Imaging, Immunology, Lung Disease and Infection, Microbiology, Newborn screening, Nutrition/Gastroenterology, Parents, Physiotherapy/Exercise testing, Pregnancy with CF, Psychosocial

November 2017

Title: Caregiver burden of parents of young children with cystic fibrosis
References
J Cyst Fibros. 2017 Nov 14. pii: S1569-1993(17)30879-2. doi: 10.1016/j.jcf.2017.08.016

November 2017

Title: Lack of efficacy of Lactobacillus GG in reducing pulmonary exacerbations and hospital admissions in children with cystic fibrosis: A randomised placebo controlled trial.
References
J Cyst Fibros. 2017 Nov 8. pii: S1569-1993(17)30951-7.

November 2017

Title: Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR
References
J Cyst Fibros. 2017 Nov 7. pii: S1569-1993(17)30891-3. doi: 10.1016/j.jcf.2017.09.012

November 2017

Title: Ceftaroline Pharmacokinetics and Pharmacodynamics in Patients with Cystic Fibrosis
References
J Cyst Fibros. 2017 Nov 2. pii: S1569-1993(17)30947-5. doi: 10.1016/j.jcf.2017.10.010

October 2017

Title: Lung Function Decline is Delayed But Not Decreased in Patients With Cystic Fibrosis and the R117H Gene Mutation
References
J Cyst Fibros. 2017 Oct 31. pii: S1569-1993(17)30912-8. doi: 10.1016/j.jcf.2017.10.003

October 2017

Title: Defining Palliative Care in Cystic Fibrosis: A Delphi Study
References
J Cyst Fibros. 2017 Oct 30. pii: S1569-1993(17)30948-7. doi: 10.1016/j.jcf.2017.10.011.

October 2017

Title: Do cystic fibrosis centres with the lowest Fev1 still use the least amount of intravenous antibiotics? A registry-based comparison of intravenous antibiotic use among adult CF centres in the UK
References
J Cyst Fibros. 2017 Oct 23. pii: S1569-1993(17)30916-5. doi: 10.1016/j.jcf.2017.10.005

October 2017

Title: IgG avidity to Pseudomonas aeruginosa over the course of chronic lung biofilm infection in cystic fibrosis
References
J Cyst Fibros. 2017 Oct 11. pii: S1569-1993(17)30883-4. doi: 10.1016/j.jcf.2017.09.004

September 2017

Title: Blood flow regulation and oxidative stress during submaximal cycling exercise in patients with cystic fibrosis.
References
J Cyst Fibros. 2017 Sep 8. pii: S1569-1993(17)30878-0. doi: 10.1016/j.jcf.2017.08.015.

September 2017

Title: Real life practice of sweat testing in Europe.
References
J Cyst Fibros. 2017 Sep 27. pii: S1569-1993(17)30881-0. doi: 10.1016/j.jcf.2017.09.002.

September 2017

Title: End-of-life practice patterns at U.S. adult cystic fibrosis care centers: A national retrospective chart review.
References
J Cyst Fibros. 2017 Sep 13. pii: S1569-1993(17)30873-1. doi: 10.1016/j.jcf.2017.08.010

August 2017

Title: Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence.
References
J Cyst Fibros. 2017 Aug 31. pii: S1569-1993(17)30874-3. doi: 10.1016/j.jcf.2017.08.011. 

August 2017

Title: Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis
References
J Cyst Fibros. 2017 Aug 30. pii: S1569-1993(17)30872-X. doi: 10.1016/j.jcf.2017.08.009

August 2017

Title: 16S rRNA gene sequencing reveals site-specific signatures of the upper and lower airways of cystic fibrosis patients.
References
J Cyst Fibros. 2017 Aug 18. pii: S1569-1993(17)30865-2. doi: 10.1016/j.jcf.2017.08.007.

August 2017

Title: Variability of Lung Clearance Index in clinically stable Cystic Fibrosis Lung Disease in School Age Children
References
J Cyst Fibros. 2017 Aug 16. pii: S1569-1993(17)30839-1. doi: 10.1016/j.jcf.2017.08.004

July 2017

Title: Poor recovery from a pulmonary exacerbation does not lead to accelerated FEV1 decline
References
J Cyst Fibros. 2017 Jul 29. pii: S1569-1993(17)30818-4. doi: 10.1016/j.jcf.2017.07.003

July 2017

Title: Tracheal diverticula in advanced cystic fibrosis: Prevalence, features, and outcomes after lung transplantation.
References
J Cyst Fibros. 2017 Jul 27. pii: S1569-1993(17)30830-5. doi: 10.1016/j.jcf.2017.07.015. 

July 2017

Title: Age-related levels of fecal M2-pyruvate kinase in children with cystic fibrosis and healthy children 0 to 10years old.
References
J Cyst Fibros. 2017 Jul 25. pii: S1569-1993(17)30826-3. doi: 10.1016/j.jcf.2017.07.011.

July 2017

Title: Increased Prevalence of Colonic Adenomas in Patients with Cystic Fibrosis
References
J Cyst Fibros. 2017 Jul 22. pii: S1569-1993(17)30824-X. doi: 10.1016/j.jcf.2017.07.009
 

July 2017

Title: Bile salt stimulated lipase, Cystic fibrosis, Enzyme therapy, NMR spectroscopy, Pancreatic insufficiency, Titrimetric enzyme assay
References
J Cyst Fibros. 2017 Jul 21. pii: S1569-1993(17)30820-2. doi: 10.1016/j.jcf.2017.07.005
Title: Analytical and Biological Variation in Repeated Sweat Chloride Concentrations in Clinical Trials for CFTR Modulator Therapy
References
J Cyst Fibros. 2017 Jul 21. pii: S1569-1993(17)30823-8. doi: 10.1016/j.jcf.2017.07.008

July 2017

Title: Differences between WHO AND CDC early growth measurements in the assessment of Cystic Fibrosis clinical outcomes.
References
J Cyst Fibros. 2017 Jul;16(4):503-509. doi: 10.1016/j.jcf.2016.12.001
Title: Outcomes associated with antibiotic regimens for treatment of Mycobacterium abscessus in cystic fibrosis patients.
References
J Cyst Fibros. 2017 Jul;16(4):483-487. doi: 10.1016/j.jcf.2017.04.013. Epub 2017 May 8
Title: Macronutrient Intake in Preschoolers with Cystic Fibrosis and the Relationship between Macronutrients and Growth
References
J Cyst Fibros. 2017 Jul;16(4):519-524. doi: 10.1016/j.jcf.2017.01.010. Epub 2017 Feb 6.
Title: Worsening anxiety and depression after initiation of lumacaftor/ivacaftor combination therapy in adolescent females with cystic fibrosis
References
J Cyst Fibros. 2017 Jul;16(4):525-527. doi: 10.1016/j.jcf.2017.05.008. Epub 2017 Jun 8.

July 2017

Title: Lower exhaled nitric oxide in infants with Cystic Fibrosis compared to healthy controls
References

July 2017

Title: Detection of CFTR Function and Modulation in Primary Human Nasal Cell Spheroids
References
J Cyst Fibros. 2017 Jul 13. pii: S1569-1993(17)30812-3. doi: 10.1016/j.jcf.2017.06.010.

July 2017

Title: Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2 years of treatment with ivacaftor in a real-world setting
References
J Cyst Fibros. 2017 Jul 12. pii: S1569-1993(17)30816-0. doi: 10.1016/j.jcf.2017.07.001

June 2017

Title: Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor.
References
J Cyst Fibros. 2017 Jun 24. pii: S1569-1993(17)30770-1. doi: 10.1016/j.jcf.2017.06.002

June 2017

Title: A single bout of maximal exercise improves lung function in patients with cystic fibrosis.
References

2017 Jun 23. pii: S1569-1993(17)30768-3. doi: 10.1016/j.jcf.2017.05.011

Theme
Physiotherapy/Exercise testing

June 2017

Title: The use of fructosamine in cystic fibrosis-related diabetes (CFRD) screening.
References
J Cyst Fibros. 2017 Jun 22. pii: S1569-1993(17)30767-1. doi: 10.1016/j.jcf.2017.05.010
Theme
Diabetes

June 2017

Title: Pulmonary surfactant dysfunction in pediatric cystic fibrosis: Mechanisms and reversal with a lipid-sequestering drug.
References
J Cyst Fibros. 2017 Jun 7. pii: S1569-1993(17)30151-0.

June 2017

Title: Complications of long and intermediate term venous catheters in cystic fibrosis patients: A multicenter study.
References
J Cyst Fibros. 2017 Jun 1. pii: S1569-1993(17)30150-9. doi: 10.1016/j.jcf.2017.04.014

May 2017

Title: Development and Validation of CF-Medication Beliefs Questionnaire: A mixed-methods approach
References
J Cyst Fibros. 2017 May 23. pii: S1569-1993(17)30152-2

May 2017

Title: Inhaled ENaC Antisense Oligonucleotide Ameliorates Cystic Fibrosis-Like Lung Disease in Mice
References
J Cyst Fibros. 2017 May 20. pii: S1569-1993(17)30154-6. doi: 10.1016/j.jcf.2017.05.003.

May 2017

Title: Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis
References
J Cyst Fibros. 2017 May 18. pii: S1569-1993(17)30153-4. doi: 10.1016/j.jcf.2017.05.002. 

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: Dietary intake and lipid profile in children and adolescents with cystic fibrosis.
References
J Cyst Fibros. 2017 May;16(3):410-417. doi: 10.1016/j.jcf.2017.02.010. 
Title: Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR
References
J Cyst Fibros. 2017 May;16(3):371-379. doi: 10.1016/j.jcf.2017.01.009. Epub 2017 Feb 13.
Title: Effectiveness of a stepwise Pseudomonas aeruginosa eradication protocol in children with cystic fibrosis
References
J Cyst Fibros. 2017 May;16(3):395-400. doi: 10.1016/j.jcf.2017.01.007. Epub 2017 Feb 9.
Title: Use of FEV1 in Cystic Fibrosis Epidemiologic Studies and Clinical Trials: A Statistical Perspective for the Clinical Researcher
References
J Cyst Fibros. 2017 May;16(3):318-326. doi: 10.1016/j.jcf.2017.01.002. Epub 2017 Jan 20.
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: What Keeps Children with Cystic Fibrosis awake at night?
References
J Cyst Fibros. 2017 May 4. pii: S1569-1993(17)30142-X.

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: A Multiple Reader Scoring System for Nasal Potential Difference Parameters
References
J Cyst Fibros. 2017 Apr 29. pii: S1569-1993(17)30141-8

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: Increased Risk of PTLD in Lung Transplant Recipients with Cystic Fibrosis
References
J Cyst Fibros. 2017 Apr 26. pii: S1569-1993(17)30096-6. doi: 10.1016/j.jcf.2017.03.013.

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.