ECFS Newsletter Sep 2013 Issue 43

1. Letter from the President

Dear Friends,

I hope this finds you well and that you all have had a chance to enjoy some relaxing summer vacation.

For those of you who were able to participate in the June ECFS Conference in Lisbon, I am sure you will agree that it was a great success, and I would again like to thank the conference President, Margarida Amaral, and the Vice President, Celeste Barreto for the enormous amount of work they contributed to the event. I would also like to thank the Cystic Fibrosis Associations of Portugal for their considerable and enthusiastic support. My sincere thanks go to the scientific committee who produced an excellent programme, and to the speakers, moderators and presenters for their superb contribution.

The ECFS continued its tradition of awarding Travel Grants to young researchers based on the merit of their submitted abstracts, and were delighted to welcome the 3 Young Investigator Awardees to the Opening Ceremony.  All the award winners also received a 1 year free subscription to the ECFS membership.

2013 Young Investigator Awards:

Once again, speakers at the this year’s conference were asked to make their presentations (slides)  available on the ECFS website (for members only) and these can be viewed by signing in under the “MY ECFS” tab in the top banner of our website www.ecfs.eu.

We are well into the planning for the spring Basic Science Conference which will be held on Malta from 26-29 March, and the annual conference which will be held 11-14 June 2014 in Gothenburg, Sweden. Programmes for both conferences will be available soon and we hope that you will take an active part in these events and consider submitting your best work for presentation and discussion. More information about the conferences can be found on our website.

As you will all be aware, we very sadly reported the death of Gerd Döring in early July, and the number of tributes posted on the ECFS website has been reflective of the legacy of this great scientist, friend, and mentor to many. A special edition of the ECFS Newsletter has been compiled in memory of Gerd. 

The task force on adult care is making good progress.  We met in Barcelona at the ERS and had further productive discussion. The post graduate symposium and the CF sessions were well attended and there was excellent interchange of ideas.  Thanks to Anders Lindblad for all his hard work in promoting CF at the ERS as chair of the CF Special Interest Group.

Development of ECFS funding support applications.  The ECFS is being approached more frequently with applications for financial support for various activities.  We are developing a set of criteria and guidelines for such applications which we expect to be available within the coming months.

I would like to thank those members who participated in this year’s Annual General Meeting in Lisbon, and you will find the minutes from the meeting included in this Newsletter.

As always, I would like to remind you to please feel free to send us articles that you would like to have included in forthcoming newsletters as this provides a great vehicle for communication.

Best wishes,
Stuart Elborn (ECFS President)

2. Minutes of the Annual General Meeting of the European Cystic Fibrosis Society – 12 June 2013

Presiding Officer: Prof. S Elborn.
The meeting was opened at 17.00. Number of Participants: 62

President's Report
Secretary's Report
Treasurer's Report
Journal of Cystic Fibrosis - Update
ECFS Clinical Trials Network - Update
ECFS Patient Registry - Update
Reports from the ECFS Working Groups
Annual Conference 2014
Any Other Business

President’s Report
Prof. Stuart Elborn welcomed the ECFS members present and thanked them for their participation in the meeting. He reported that over 2200 delegates had pre-registered for the Lisbon conference and that 524 abstracts had been accepted. He took the opportunity to thank Prof. Amaral, Dr. Barreto, and all the other organizers for their hard work.

He went on to provide an overview of the ECFS activities over the last year, beginning with the highly successful Basic Science conference held in Malaga where a record number of attendees and abstract submissions were recorded. He encouraged physicians to attend this very important conference. He reported on the Standards of Care Consensus meeting held in Verona in April 2012, where excellent progress had been made towards compiling the manuscript which will be further developed over the summer months. The next meeting of the Joint ERS/ECFS Task Force on Adult CF Care will be held in conjunction with the ERS meeting in September. He presented a slide listing the current ECFS Projects and Working Groups, and noted that reports on these activities would be provided later in the meeting.  The President thanked the Editors of the 1st edition of the ECFS Book entitled Health Care Issues and Challenges in the Adolescent with Cystic Fibrosis, which had been distributed to all ECFS members in December 2012.

The President then presented the programme of activities for 2013 and 2014 to include:

•    Standards of Care
•    Joint ERS/ECFS Adult CF Task Force
•    ECFS Book – the next edition
•    CTN Steering Group Meeting 23-24 January 2014, Leuven, Belgium
•    Registry Steering Group Meeting 24 January 2014, Leuven, Belgium
•    ECFS Diagnostic Network WG Meeting 13-15 February 2014, Berlin, Germany
•    Basic Science Conference 26-29 March 2014, Sicily or Malta
•    Joint CFF/ECFS Guideline on Mental Health – Spring 2014
•    Young Investigators Meeting – Spring 2014
•    European CF Conference 11-14 June Gothenburg, Sweden

He took the opportunity of thanking Hanne Olesen, who is stepping down as the Director of the ECFS Patient Registry (ECFSPR), and praised the progress of this project during her stewardship.  He also sincerely thanked Michael Wilschanski who in early 2013 stepped down as the Coordinator of the Diagnostic Network Working Group (DNWG) after more than 10 years.  He welcomed Ed McKone as the new Director of the ECFSPR and Nico Derichs, the new coordinator of the DNWG.  He also thanked the ECFS Membership, the National Patient Organisations, the Pharmaceutical Industry, and International collaborators for their continuing support of ECFS activities.

The audience were asked for any questions: None received.

Secretary’s Report
As Prof. Milan Macek, the ECFS Secretary, was unable to attend the meeting, Prof. Elborn provided this report.

The preliminary membership numbers for 2013 (May) were 688, a decrease of 104 in comparison with the figures reported at the same time frame in 2012.  One of the reasons for this was that fewer conference registration/membership packages had been sold than in the two previous years and this could be expected as this option is only available for new members. However, the core number of members remains stable. In view of the healthy financial situation of the Society, he was happy to inform the membership that there would be no increase to the subscription fee in 2014; it will remain at Euro 120, and there will be a continued lower rate for Allied Health Professionals who do not wish to receive the Journal of Cystic Fibrosis (JCF). 
Following discussions with Elsevier, the JCF publishers, the ECFS was happy to announce that all articles included in the Journal which are more than 2 years old are now freely available for download.

The membership were reminded that there will be elections for Board positions that become vacant after June 2014.  Su Madge will complete her second term, and Benny Assael will complete his first term but will not be standing for a further term. Additionally, voting will be required for a President-Elect as Stuart Elborn completes his mandate in 2015.

To close the report, a slide was presented on the most viewed pages on the ECFS website with the annual conferences being the most frequently visited followed by the visits to the freely available publications and guidelines.

Questions/Comments received: 
Daniel Peckham asked if direct debit could be used for paying membership fees.  This is unfortunately not an available option in Denmark.

Anil Mehta proposed support (alternative membership fees) for lower income countries. This will be considered in a “think tank” meeting that the ECFS Board will hold later in 2013.

Treasurer’s Report
Prof. Tania Pressler presented the audited ECFS Financial statement for the year 2012. The result of the year was very favourable, with a surplus of € 424.499 resulting in a positive balance for the Society’s net assets of € 2.365.989 at 31st December 2012.  Prof. Pressler informed the AGM that the full audited accounts will be posted on the website (linked here)

Update on Journal of Cystic Fibrosis
On behalf of Prof. Gerd Döring, Dr. Harry Heijerman provided this report.

Dr. Heijerman first reported on the increased impact factor for the Journal at 3.190, which had been calculated on the number of citations in 2011 of items published in 2009 and 2010. A slide was shown listing the top 10 articles contributing the increased impact factor, and another demonstrating that the Journal was listed as 15th overall in the Respiratory Systems category.

He noted that the number of articles being submitted to the Journal continues to rise (319 in 2012), with a rejection rate of 61%.  A further slide was shown demonstrating the number of downloads from Science Direct which also continues to rise.  From a financial point of view, there was a clear correlation between the number of ECFS members and the corresponding JCF royalties generated.

Finally, Dr. Heijerman thanked Prof. Döring for his tireless work as Editor-in-Chief of the JCF, and noted that his term would finish at the end of 2013.  A new Editor-in-Chief will be announced shortly.

Questions/Comments received: 
Anil Mehta asked if JCF could be made available on Pubmed.  Harry Heijerman confirmed JCF was available on ScienceDirect but would investigate the availability on Pubmed with the publisher.

Report from ECFS CTN
Prof. Isabelle Fajac presented a slide showing the locations of the 30 active Clinical Trials Network sites in 11 countries with 15,000 patients.  A graph was shown demonstrating the progression of studies conducted by the CTN since 2009. There were currently 14 active studies within the network, 3 ended recently, 6 other studies have been approved and will begin before end 2013. Most are industry sponsored trials but 3 are investigator led trials.

Among the achievements and milestones:
- There has been an agreement on an Investigator-initiated study on MRSA eradication to compare 2 different MRSA eradication treatments.
- Many of the Standard Operating Procedures have been completed and are available upon request to the CTN office. 
- Certification of sites and the set-up of central laboratories.
- A manuscript of clinimetrics on CFTR biomarkers has been published in the European Regspiratory Journal in January.  More manuscripts on clinimetrics to review the evidence for the outcome measures used in the CF field are in the final stages before submission for publication. Inflammatory markers; Lung Clearance Index (LCI); Microbiological definitions.

- Work on quality within the CTN: evaluate and programme for improvement

Lastly Prof. Fajac presented a slide showing the CTN webpages at http://www.ecfs.eu/ctn where the following information was available:

•    Listing of CTN sites and the new map
•    Ongoing trials in the CTN sites (with results)
•    Brochures for patients in multiple languages
•    Brochures for companies
•    Newsletters and more!

Report from the ECFS Patient Registry
Dr. Hanne Olesen reported on the activities of the ECFS Patient Registry (ECFSPR).
She reported that 2010 and 2011 data had been received from 23 countries on more than 30,000 patients.
Activities:
- A new software is being developed and will be tailored to the needs of the ECFSPR – a demonstration of the software would be given in Lisbon.  Migration of 2008-2010 data is ongoing.
- The Project Data Quality Group, chaired by Vincent Gulmans, had almost completed their first task.
- The ECFSPR is actively engaged in the ERS/ECFS Adult CF Task Force.

Dr. Olesen reported the increase in data requests being received by the ECFSPR (10 already in 2013), and that 3 manuscripts were planned by the Registry Group.  Two publications were highlighted where data from the ECFS Patient Registry had been used.
For 2013 and 2014:
- Recruitment of new countries is to be discussed at a meeting in Slovakia in November
- Definition of new variables (LCI, CFRD, new therapies)
- A new business plan is to be developed for the ECFSPR
Finally, Dr. Olesen welcomed Dr. Ed McKone as the new ECFSPR Director, and thanked the project’s sponsors and collaborators.

Reports from the ECFS Working Groups
Prior to the Annual General Meeting, members had been sent the progress reports from the ECFS Working Groups.  The working group annual reports are available online at http://www.ecfs.eu/ecfs-working-groups/ecfs-working-groups. The audience were asked for any questions: None received.

Current Working Groups:
Diagnostics Network Working Group
Exercise Working Group
Gene Modifier Working Group
Lung Microbiome Working Group
Neonatal Screening Working Group
Non Tuberculosis Mycobacteria Working Group

Annual Conference 2014
Dr. Anders Lindblad, President of the 37th ECFS Conference, provided an introduction to the forthcoming event to be held in Gothenburg in June 2014.
Questions/Comments received: 
It was noted that the 2014 ECFS conference would be overlapping with the ESPGHN conference.  Decisions on conference days and locations are made many years in advance by independent bodies and this might lead to clashing dates.

Any Other Business
With no further items being raised, the meeting ended at 18.00.

3. ECFS CTN - Support from the Nation Associations

At the 2013 Lisbon conference, European national CF Associations present their continued support towards the ECFS Clinical Trials Network.

Representatives from the European CF National Associations presenting a cheque to the ECFS President Stuart Elborn, and the CTN Director, Isabelle Fajac.

4. ECFS CTN - Update

In September 2013, 16 trials are ongoing in ECFS-CTN sites. This means a continuous growth since the start of the network in 2009.

In addition 5 more studies have been reviewed, for which study start is pending, and 2 protocol reviews are currently ongoing. An overview of studies that are in the recruitment phase can be found here: http://www.ecfs.eu/ctn/clinical-trials

This public website also shows results for closed studies, as soon as they become available. This is currently the case for 6 studies.

5. Online survey evaluating the situation about stopping CF care because of a desire to stop fighting the disease 

As a reminder to the membership and in follow up to the ECFS Bulletin issued on 16 September, please find here the link to further information on this survey. 

6. International Study: “CFQ-R questionnaire and CF candidates for lung transplant”. First part of international project: A novel score for lung transplant indication in Cystic Fibrosis” 

Submitted by Amparo Solé

INTRODUCTION

Although there are international guidelines on when to transplant the Cystic Fibrosis (CF) population, the precise moment to place a CF patient on the list for lung transplantation (LT) remains uncertain. Nevertheless, hundreds of CF patients undergo LT every year. There are predictive models of survival that help determine the physiological readiness for transplantation in this population. Also, there is an international consensus, mainly based on respiratory functional parameters on when LT is indicated for patients with chronic respiratory diseases. In general, the main LT criteria for the CF population include: an FEV1 less than 30% predicted, a rapid decline in lung function, an increase in the number of exacerbations, progressive respiratory insufficiency, and/or life-threatening hemoptysis, among other factors. However, the FEV1 under 30% is not necessarily the best criterion for LT. In fact, there are CF patients with better lung mechanics that have other clinical parameters determining their need for lung transplantation. At the same time there are other CF patients with a lower FEV1 that have other stable clinical parameters whereby the risk of LT outweighs its benefit.

There are still patients who die on the waiting list which means, in part, that the indication for LT is not precisely defined. There is a need for precision in determining the proper time to list and more importantly when to refer a CF patient to a lung transplant center. In addition, there is a need to determine which factors will best predict mortality in this population.

According to the ISHLT 2006 guidelines, the decision for LT is derived from a comprehensive evaluation that takes into account several indicators of disease severity such as: FEV1, increases in oxygen need, hypercapnia, the need for non-invasive ventilation, functional status (e.g., 6-MWD), and pulmonary hypertension.

The current guidelines for referral are:
? FEV1 below 30% predicted or a rapid decline in FEV1, particularly in young female patients.
? Exacerbation of pulmonary disease requiring ICU stay.
? Increasing frequency of exacerbations requiring antibiotic therapy.
? Refractory and/or recurrent pneumothorax.
? Recurrent hemoptysis not controlled by embolization.

However, there is not an established score to predict the optimal moment to list a CF patient for LT. 

AIM:
Recently a specific questionnaire on quality of life (CFR-Q) has been demonstrated to accurately detect variations in lung function and changes in clinical performance of CF patients.  In fact, all recent trials in this population are including this questionnaire as an end point. The principal aim of this study is to apply this CFR-Q to all CF patients with an FEV1 below 40% to determine if this questionnaire can detect any significant changes in any of the domains that correlate with lung function and the variables that correlate with severe advanced disease.

Should you wish to participate to the international study, please contact:

Amparo Solé
Unidad de Trasplante Pulmonar y Fibrosis Quística Torre G, 4ª planta Hospital Universitario y Politécnico La Fe Bulevar Sur, s/n
46026 Valencia
Spain
sole_amp@gva.es
Tel +34 961244000 Ext 440459

7.  ECMM/ISHAM Working Group on "Filamentous fungi and chronic respiratory infections in cystic fibrosis

Submitted by Dr Laurance Delhaes

For an update on this international study with the purpose of standardization of the mycological examination of respiratory samples from CF patients (called MFIP for “MucoFong International Project”), please click on this link.

8. 2013 Lisbon Conference Survey – Free Registration Winners 

The ECFS would like to thank all those who took the time to answer the ECFS 2013 Survey after the conference in Lisbon.  As announced, three free registrations to the next ECFS Conference in Gothenburg were selected from the respondents.

We congratulate the winners:
Ingrid Demey (Belgium)
Aleksandra Norek (Poland)
Tom Matier (United Kingdom)

9. Vertex Announces Support for CF Research 

This month Vertex Pharmaceuticals has opened applications for the Vertex Innovation Awards (VIA) for research in cystic fibrosis (CF). The grant selection process will be performed by an independent Grant Committee of external CF experts.

The VIA programme is designed to support clinical research in CF and will focus on different areas each year. The specific areas of research will be announced at the start of each annual cycle, however, as the 2013–2014 VIA programme is its inaugural cycle, applications for clinical research projects will be considered across the entire field of CF.

Applications are invited from research centres with a demonstrated record of research and achievement in CF, for grants which will support innovative and collaborative clinical research that may impact the care of patients with CF. The independent Grant Committee will review and assess the submitted applications and award the research grants. For the 2013–2014 annual awards, the Grant Committee will select 1 to 3 projects for grants up to a total value of €500,000.

Professor J. Stuart Elborn, Director, Centre for Infection and Immunity, Queen’s University, Belfast, Northern Ireland, Chair of the Independent Grant Committee, believes that “The VIA programme is an opportunity to pump prime innovative research into the CF community.”

Professor Kris De Boeck, Pediatric Pulmonology Department, University Hospital Gasthuisberg, Leuven, Belgium is also a member of the Grant Committee and considers “The collaboration between centres is key to stimulate progress in CF research. The VIA grants address these needs.”

The VIA application period is from 2nd September to 18th November 2013. For further information about the awards, eligibility, selection criteria and an application form, please visit the VIA website: www.VertexInnovationAwards.com

10. Upcoming Events 

- ECFS Board Meeting 16 October 2013 – Salt Lake City, Utah, USA
- 27th North American Cystic Fibrosis Conference 17-19 October 2013 – Salt Lake City, USA
- ECFS Winter Board Meeting - 22 January 2014 – Leuven, Belgium
- ECFS CTN Steering Group Meeting - 23-24 January 2014 – Leuven, Belgium
- ECFS Patient Registry Steering Group Meeting - 24 January 2014 – Leuven, Belgium
- ECFS Diagnostic Network Working Group Meeting - 13-15 February 2014, Berlin, Germany
- 7th European Young Investigators Meeting – Spring 2014
- ECFS 11th Basic Science Conference - 26-29 March 2014 – St. Julians, Malta
- ECFS Board Meeting - 10 June 2014 – Gothenburg, Sweden
- 37th European CF Conference - 11-14 June 2014 – Gothenburg, Sweden

11. Current References in Cystic Fibrosis 

Adults & Adolescents

Ahmad A, Ahmed A, Patrizio P
Cystic fibrosis and fertility
Current Opinion in Obstetrics & Gynecology 2013; 25: 167 - 172

Alghanem S, Paterson I, Touw DJ, Thomson AH
Influence of multiple courses of therapy on aminoglycoside clearance in adult patients with cystic fibrosis
Journal of Antimicrobial Chemotherapy 2013; 68: 1338 - 1347

Chaudhry SR, Keaton M, Nasr SZ
Evaluation of a cystic fibrosis transition program from pediatric to adult care
Pediatric Pulmonology 2013; 48: 658 - 665

Chi DL
Dental caries prevalence in children and adolescents with cystic fibrosis: a qualitative systematic review and recommendations for future research
International Journal of Paediatric Dentistry 2013; 23: 376 - 386

Samoilenko VA, Krasovsky SA, Marchenkov YV, Chikina SY, Babadzhanova GY, Nagornyi AB, Chuchalin AG
The clinical features of cystic fibrosis in adult patients with carbohydrate metabolic disturbances
Terapevticheskii Arkhiv 2013; 85: 32 - 37

Staub MO, Leon K, Robin NH
Educating the Adolescent and Young Adult With Cystic Fibrosis About Their Reproductive Risks and Options
Chest 2013; 143: 580 - 581

Tierney S, Deaton C, Jones A, Oxley H, Biesty J, Kirk S
Liminality and transfer to adult services: A qualitative investigation involving young people with cystic fibrosis
International Journal of Nursing Studies 2013; 50: 738 - 746

Animal Model

Darrah RJ, Bederman IR, Mitchell AL, Hodges CA, Campanaro CAK, Drumm ML, Jacono FJ
Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice
Journal of Cystic Fibrosis 2013; 12: 345 - 351

Gavina M, Luciani A, Villella VR, Esposito S, Ferrari E, Bressani I, Casale A, Bruscia EM, Maiuri L, Raia V
Nebulized Hyaluronan Ameliorates Lung Inflammation in Cystic Fibrosis Mice
Pediatric Pulmonology 2013; 48: 761 - 771

Lazenby JJ, Griffin PE, Kyd J, Whitchurch CB, Cooley MA
A Quadruple Knockout of lasIR and rhlIR of Pseudomonas aeruginosa PAO1 That Retains Wild-Type Twitching Motility Has Equivalent Infectivity and Persistence to PAO1 in a Mouse Model of Lung Infection
Plos One 2013; 8: 4:e60973
Paroni M, Moalli F, Nebuloni M, Pasqualini F, Bonfield T, Nonis A, Mantovani A, Garlanda C, Bragonzi A
Response of CFTR-Deficient Mice to Long-Term chronic Pseudomonas aeruginosa Infection and PTX3 Therapy
Journal of Infectious Diseases 2013; 208: 130 - 138

Potash AE, Wallen TJ, Karp PH, Ernst S, Moninger TO, Gansemer ND, Stoltz DA, Zabner J, Chang EH
Adenoviral Gene Transfer Corrects the Ion Transport Defect in the Sinus Epithelia of a Porcine CF Model
Molecular Therapy 2013; 21: 947 - 953

Saussereau EL, Roussel D, Diallo S, Debarbieux L, Edelman A, Sermet-Gaudelus I
Characterization of Nasal Potential Difference in CFTR Knockout and F508del-CFTR Mice
Plos One 2013; 8: 3:e57317

Song PH, Song WF, Liu XS, Jin CH, Xie HY, Zhou L, Tuo BG, Zheng SD
Function and Expression of Cystic Fibrosis Transmembrane Conductance Regulator after Small Intestinal Transplantation in Mice
Plos One 2013; 8: 4:e62536

Stoltz DA, Rokhlina T, Ernst SE, Pezzulo AA, Ostedgaard LS, Karp PH, Samuel MS, Reznikov LR, Rector MV, Gansemer ND,
Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs
Journal of Clinical Investigation 2013; 123: 2685 - 2693

Antimicrobials

Agnello M, Wong-Beringer A
Differentiation in Quinolone Resistance by Virulence Genotype in Pseudomonas aeruginosa
Plos One 2012; 7: 8: e42973

Alhajlan M, Alhariri M, Omri A
Efficacy and Safety of Liposomal Clarithromycin and Its Effect on Pseudomonas aeruginosa Virulence Factors
Antimicrobial Agents and Chemotherapy 2013; 57: 2694 - 2704

Babu KS, Kastelik J, Morjaria JB
Role of long term antibiotics in chronic respiratory diseases
Respiratory Medicine 2013; 107: 800 - 815

Baxter CG, Rautemaa R, Jones AM, Webb AK, Bull M, Mahenthiralingam E, Denning DW
Intravenous antibiotics reduce the presence of Aspergillus in adult cystic fibrosis sputum
Thorax 2013; 68: 652 - 657

Bentley S, Gupta A, Balfour-Lynn IM
Subtherapeutic itraconazole and voriconazole levels in children with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 418 - 419

Catherinot E, Roux AL, Vibet MA, Bellis G, Lemonnier L, Le Roux E, Bernede-Bauduin C, Le Bourgeois M, Herrmann JL,
Inhaled therapies, azithromycin and Mycobacterium abscessus in cystic fibrosis patients
European Respiratory Journal 2013; 41: 1101 - 1106

Chiang WC, Nilsson M, Jensen PO, Høiby N, Nielsen TE, Givskov M, Tolker-Nielsen T
Extracellular DNA Shields against Aminoglycosides in Pseudomonas aeruginosa Biofilms
Antimicrobial Agents and Chemotherapy 2013; 57: 2352 - 2361
Cho HS, Lee JH, Ryu SY, Joo SW, Cho MH, Lee J
Inhibition of Pseudomonas aeruginosa and Escherichia coli O157:H7 Biofilm Formation by Plant Metabolite epsilon-Viniferin
Journal of Agricultural and Food Chemistry 2013; 61: 7120 - 7126

Clancy JP, Dupont L, Konstan MW, Billings J, Fustik S, Goss CH, Lymp J, Minic P, Quittner AL, Rubenstein RC, Young KR,
Phase II studies of nebulised Arikace in CF patients with Pseudomonas aeruginosa infection
Thorax 2013; 68: 818 - 825

Foweraker JE, Govan JRW
Antibiotic susceptibility testing in early and chronic respiratory infections with Pseudomonas aeruginosa
Journal of Cystic Fibrosis 2013; 12: 302

Galeva I, Konstan MW, Higgins M, Angyalosi G, Brockhaus F, Piggott S, Thomas K, Chuchalin AG
Tobramycin inhalation powder manufactured by improved process in cystic fibrosis: the randomized EDIT trial
Current Medical Research and Opinion 2013; 29: 947 - 956

Gilchrist FJ, Cox KJ, Rowe R, Horsley A, Webb AK, Jones AM, Bright-Thomas RJ
Itraconazole and inhaled fluticasone causing hypothalamic-pituitary-adrenal axis suppression in adults with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 399 - 402

Govoni M, Poli G, Acerbi D, Santoro D, Cicirello H, Annoni O, Ruzicka J
Pharmacokinetic and tolerability profiles of tobramycin nebuliser solution 300 mg/4 ml administered by PARI eFlow (R) rapid and PARI LC Plus (R) nebulisers in cystic fibrosis patients
Pulmonary Pharmacology & Therapeutics 2013; 26: 249 - 255

Grant SS, Hung DT
Persistent bacterial infections, antibiotic tolerance, and the oxidative stress response
Virulence 2013; 4: 273 - 283

Gutu AD, Sgambati N, Strasbourger P, Brannon MK, Jacobs MA, Haugen E, Kaul RK, Johansen HK, Hoiby N, Moskowitz SM
Polymyxin Resistance of Pseudomonas aeruginosa phoQ Mutants Is Dependent on Additional Two-Component Regulatory Systems
Antimicrobial Agents and Chemotherapy 2013; 57: 2204 - 2215

Hennig S, Standing JF, Staatz CE, Thomson AH
Population Pharmacokinetics of Tobramycin in Patients With and Without Cystic Fibrosis
Clinical Pharmacokinetics 2013; 52: 289 - 301

Imperi F, Massai F, Facchini M, Frangipani E, Visaggio D, Leoni L, Bragonzi A, Visca P
Repurposing the antimycotic drug flucytosine for suppression of Pseudomonas aeruginosa pathogenicity
Proceedings of the National Academy of Sciences of the United States of 2013; 110: 7458 - 7463

Jorgensen KM, Wassermann T, Jensen PO, Wang HZ, Molin S, Hoiby N, Ciofu O
Sublethal Ciprofloxacin Treatment Leads to Rapid Development of High-Level Ciprofloxacin Resistance during Long-Term Experimental Evolution of Pseudomonas aeruginosa
Antimicrobial Agents and Chemotherapy 2013; 57: 4215 - 4221
 
Lehievre L, Groh M, Angebault C, Maherault AC, Didier E, Bougnoux ME
Azole resistant Aspergillus fumigatus: An emerging problem
Medecine Et Maladies Infectieuses 2013; 43: 139 - 145

Llanes C, Pourcel C, Richardot C, Plesiat P, Fichant G, Cavallo JD, Merens A
Diversity of beta-lactam resistance mechanisms in cystic fibrosis isolates of Pseudomonas aeruginosa: a French multicentre study
Journal of Antimicrobial Chemotherapy 2013; 68: 1763 - 1771

Maiz L, Giron RM, Olveira C, Quintana E, Lamas A, Pastor D, Canton R, Mensa J
Inhaled antibiotics for the treatment of chronic bronchopulmonary Pseudomonas aeruginosa infection in cystic fibrosis: systematic review of randomised controlled trials
Expert Opinion On Pharmacotherapy 2013; 14: 1135 - 1149

Pan JC, Song FC, Ren DC
Controlling persister cells of Pseudomonas aeruginosa PDO300 by (Z)-4-bromo-5-(bromomethylene)-3-methylfuran-2(5H)-one
Bioorganic & Medicinal Chemistry Letters 2013; 23: 4648 - 4651

Pilcer G, De Bueger V, Traina K, Traore H, Sebti T, Vanderbist F, Amighi K
Carrier-free combination for dry powder inhalation of antibiotics in the treatment of lung infections in cystic fibrosis
International Journal of Pharmaceutics 2013; 451: 112 - 120

Pilcer G, Rosiere R, Traina K, Sebti T, Vanderbist F, Amighi K
New co-spray-dried tobramycin nanoparticles-clarithromycin inhaled powder systems for lung infection therapy in cystic fibrosis patients
Journal of Pharmaceutical Sciences 2013; 102: 1836 - 1846

Reymond JL, Bergmann M, Darbre T
Glycopeptide dendrimers as Pseudomonas aeruginosa biofilm inhibitors
Chemical Society Reviews 2013; 42: 4814 - 4822

Ruddy J, Emerson J, Moss R, Genatossio A, McNamara S, Burns JL, Anderson G, Rosenfeld M
Sputum Tobramycin Concentrations in Cystic Fibrosis Patients with Repeated Administration of Inhaled Tobramycin
Journal of Aerosol Medicine and Pulmonary Drug Delivery 2013; 26: 69 - 75

Schemuth H, Dittmer S, Lackner M, Sedlacek L, Hamprecht A, Steinmann E, Buer J, Rath PM, Steinmann J
In vitro activity of colistin as single agent and in combination with antifungals against filamentous fungi occurring in patients with cystic
fibrosis
Mycoses 2013; 56: 297 - 303

Stenbit AE, Bullington WM, Heh JL, Flume PA
Timing of inhaled tobramycin affects assessment of intravenous tobramycin pharmacokinetic monitoring
Journal of Cystic Fibrosis 2013; 12: 403 - 406
 
Stine AE, Nassar D, Miller JK, Clemons CB, Wilber JP, Young GW, Yun YH, Cannon CL, Leid JG, Youngs WJ, Milsted A
Modeling the response of a biofilm to silver-based antimicrobial
Mathematical Biosciences 2013; 244: 29 - 39

VanDevanter DR, Elkin EP, Pasta DJ, Morgan WJ, Konstan MW
Changing thresholds and incidence of antibiotic treatment of cystic fibrosis pulmonary exacerbations, 1995-2005
Journal of Cystic Fibrosis 2013; 12: 332 - 337

Wagener JS, Rasouliyan L, VanDevanter DR, Pasta DJ, Regelmann WE, Morgan WJ, Konstan MW
Oral, inhaled, and intravenous antibiotic choice for treating pulmonary exacerbations in cystic fibrosis
Pediatric Pulmonology 2013; 48: 666 - 673

Weidel E, de Jong JC, Brengel C, Storz MP, Braunshausen A, Negri M, Plaza A, Steinbach A, Muller R, Hartmann RW
Structure Optimization of 2-Benzamidobenzoic Acids as PqsD Inhibitors for Pseudomonas aeruginosa Infections and Elucidation of Binding Mode by SPR, STD NMR, and Molecular Docking
Journal of Medicinal Chemistry 2013; 56: 6146 - 6155

Winterstein AG, Eworuke E, Xu DD, Schuler P
Palivizumab immunoprophylaxis effectiveness in children with cystic fibrosis
Pediatric Pulmonology 2013; 48: 874 - 884

Zobell JT, Young DC, Waters CD, Ampofo K, Stockmann C, Sherwin CMT, Spigarelli MG
Optimization of anti-pseudomonal antibiotics for cystic fibrosis pulmonary exacerbations: VI. Executive summary
Pediatric Pulmonology 2013; 48: 525 - 537

 [Anonymous]
Tobramycin Inhalation Powder (Tobi Podhaler) for Cystic Fibrosis
Medical Letter On Drugs and Therapeutics 2013; 55: 51 - 52

Cardiology

Belle-van Meerkerk G, Cramer MJ, Kwakkel-van Erp JM, Nugroho MA, Tahri S, de Valk HW, van de Graaf EA
Pulmonary hypertension is a mild comorbidity in end-stage cystic fibrosis patients
Journal of Heart and Lung Transplantation 2013; 32: 609 - 614

Eaden J, Peckham D
Myocardial infarction in an adult with cystic fibrosis and heart and lung transplant
Multidisciplinary Respiratory Medicine 2013; 8: Art No 37

Koestenberger M, Ravekes W
Subclinical deterioration of systolic right ventricular function parameters in adolescents with mild cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 417

Poore S, Berry B, Eidson D, McKie KT, Harris RA
Evidence of Vascular Endothelial Dysfunction in Young Patients With Cystic Fibrosis
Chest 2013; 143: 939 - 945
 
Cell Biology

Bhattacharyya S, Kumar P, Tsuchiya M, Bhattacharyya A, Biswas R
Regulation of miR-155 biogenesis in cystic fibrosis lung epithelial cells: Antagonistic role of two mRNA-destabilizing proteins, KSRP and TTP
Biochemical and Biophysical Research Communications 2013; 433: 484 - 488

Ciavardelli D, D'Orazio M, Pieroni L, Consalvo A, Rossi C, Sacchetta P, Di Ilio C, Battistoni A, Urbani A
Proteomic and ionomic profiling reveals significant alterations of protein expression and calcium homeostasis in cystic fibrosis cells
Molecular Biosystems 2013; 9: 1117 - 1126

Kirk KL
Being positive: revisiting the elevated sodium permeability hypothesis in cystic fibrosis
Journal of Physiology-london 2013; 591: 3675 - 3676

O'Donoghue DL, Dua V, Moss GWJ, Vergani P
Increased apical Na+ permeability in cystic fibrosis is supported by a quantitative model of epithelial ion transport
Journal of Physiology-london 2013; 591: 3681 - 3692

Oliynyk I, Hussain R, Amin A, Johannesson M, Roomans GM
The effect of NO-donors on chloride efflux, intracellular Ca2+ concentration and mRNA expression of CFTR and ENaC in cystic fibrosis airway epithelial cells
Experimental and Molecular Pathology 2013; 94: 474 - 480

Soleti R, Porro C, Martinez MC
Apoptotic process in cystic fibrosis cells
Apoptosis 2013; 18: 1029 - 1038

Xu YQ, Krause A, Limberis M, Worgall TS, Worgall S
Low Sphingosine-1-Phosphate Impairs Lung Dendritic Cells in Cystic Fibrosis
American Journal of Respiratory Cell and Molecular Biology 2013; 48: 250 - 257

CFTR

Aeffner F, Abdulrahman B, Hickman-Davis JM, Janssen PM, Amer A, Bedwell DM, Sorscher EJ, Davis IC
Heterozygosity for the F508del Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Anion Channel Attenuates Influenza Severity
Journal of Infectious Diseases 2013; 208: 780 - 789

Ahner A, Gong XY, Schmidt BZ, Peters KW, Rabeh WM, Thibodeau PH, Lukacs GL, Frizzell RA
Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway
Molecular Biology of the Cell 2013; 24: 74 - 84

Aissat A, de Becdelievre A, Golmard L, Vasseur C, Costa C, Chaoui A, Martin N, Costes B, Goossens M, Girodon E, Fanen P,
Combined Computational-Experimental Analyses of CFTR Exon Strength Uncover Predictability of Exon-Skipping Level
Human Mutation 2013; 34: 873 - 881
 
Amato F, Seia M, Giordano S, Elce A, Zarrilli F, Castaldo G, Tomaiuolo R
Gene Mutation in MicroRNA Target Sites of CFTR Gene: A Novel Pathogenetic Mechanism in Cystic Fibrosis?
Plos One 2013; 8: 3:e60448

Ao M, Sarathy J, Domingue J, Alrefai WA, Rao MC
Chenodeoxycholic acid stimulates Cl- secretion via cAMP signaling and increases cystic fibrosis transmembrane conductance regulator phosphorylation in T84 cells
American Journal of Physiology-cell Physiology 2013; 305: C447 - C456

Averna M, Pedrazzi M, Minicucci L, De Tullio R, Cresta F, Salamino F, Pontremoli S, Melloni E
Calpain Inhibition Promotes the Rescue of F(508)del-CFTR in PBMC from Cystic Fibrosis Patients
Plos One 2013; 8: 6:e66089

Birault V, Solari R, Hanrahan J, Thomas DY
Correctors of the basic trafficking defect of the mutant F508del-CFTR that causes cystic fibrosis
Current Opinion in Chemical Biology 2013; 17: 353 - 360

Cebotaru L, Woodward O, Cebotaru V, Guggino WB
Transcomplementation by a Truncation Mutant of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Enhances Delta F508 Processing through a Biomolecular Interaction
Journal of Biological Chemistry 2013; 288: 10505 - 1051

Costantino L, Rusconi D, Solda G, Seia M, Paracchini V, Porcaro L, Asselta R, Colombo C, Duga S
Fine Characterization of the Recurrent c.1584+18672A > G Deep-Intronic Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Gene
American Journal of Respiratory Cell and Molecular Biology 2013; 48: 619 - 625

Csanady L, Mihalyi C, Szollosi A, Torocsik B, Vergani P
Conformational changes in the catalytically inactive nucleotide-binding site of CFTR
Journal of General Physiology 2013; 142: 61 - 73

Cui GY, Freeman CS, Knotts T, Prince CZ, Kuang C, McCarty NA
Two Salt Bridges Differentially Contribute to the Maintenance of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Channel Function
Journal of Biological Chemistry 2013; 288: 20758 - 2076

Dekkers JF, Wiegerinck CL, de Jonge HR, Bronsveld I, Janssens HM, de Winter-de Groot KM, Brandsma AM, de Jong NWM,
A functional CFTR assay using primary cystic fibrosis intestinal organoids
Nature Medicine 2013; 19: 939 - U184

El-Seedy A, Pasquet MC, Bienvenu T, Bieth E, Audrezet MP, Kitzis A, Ladeveze V
Consequences of partial duplications of the human CFTR gene on cf diagnosis: Mutations or ectopic variations
Journal of Cystic Fibrosis 2013; 12: 407 - 410

Goda K, Szaloki G
A sensitive tool to measure CFTR channel activity
Cytometry Part a 2013; 83A: 528 - 529

Holleran JP, Zeng JX, Frizzell RA, Watkins SC
Regulated recycling of mutant CFTR is partially restored by pharmacological treatment
Journal of Cell Science 2013; 126: 2692 - 2703
 
Jabr S, Gartner S, Milne GL, Roca-Ferrer J, Casas J, Moreno A, Gelpi E, Picado C
Quantification of major urinary metabolites of PGE(2) and PGD(2) in cystic fibrosis: Correlation with disease severity
Prostaglandins Leukotrienes and Essential Fatty Acids 2013; 89: 121 - 126

Jin WZ, Song YL, Bai CX, Jiang JJ
Novel role for cystic fibrosis transmembrane conductance regulator in alveolar fluid clearance in lipopolysaccharide-induced acute lung injury in mice
Respirology 2013; 18: 978 - 982

Kravtsov DV, Ameen NA
Molecular Motors and Apical CFTR Traffic in Epithelia
International Journal of Molecular Sciences 2013; 14: 9628 - 9642

Lee HW, Cheng J, Kovbasnjuk O, Donowitz M, Guggino WB
Insulin-Like Growth Factor 1 (IGF-1) Enhances the Protein Expression of CFTR
Plos One 2013; 8: 3:e59992

Londino JD, Lazrak A, Jurkuvenaite A, Collawn JF, Noah JW, Matalon S
Influenza matrix protein 2 alters CFTR expression and function through its ion channel activity
American Journal of Physiology-lung Cellular and Molecular Physiology 2013; 304: L582 - L592

Loo TW, Bartlett MC, Clarke DM
Corrector VX-809 stabilizes the first transmembrane domain of CFTR
Biochemical Pharmacology 2013; 86: 612 - 619

Merk D, Schubert-Zsilavecz M
Repairing mutated proteins - development of small molecules targeting defects in the cystic fibrosis transmembrane conductance regulator
Expert Opinion On Drug Discovery 2013; 8: 691 - 708

Montagnani M, Cazzato S, Mutignani M, Cevenini M, Guidetti E, Ben Zvi I, Aldini R, Saraceni G, Cavoli C, Garagnani P,
A Patient With Pancreas Divisum, Recurrent Acute Pancreatitis, and Homozygosity for the Cystic Fibrosis Transmembrane Regulator-Associated Protein 5T Allele
Clinical Gastroenterology and Hepatology 2013; 11: 579 - 581

Monterisi S, Casavola V, Zaccolo M
Local modulation of cystic fibrosis conductance regulator: cytoskeleton and compartmentalized cAMP signalling
British Journal of Pharmacology 2013; 169: 1 - 9

Namkung W, Park J, Seo Y, Verkman AS
Novel Amino-Carbonitrile-Pyrazole Identified in a Small Molecule Screen Activates Wild-Type and Delta F508 Cystic Fibrosis Transmembrane Conductance Regulator in the Absence of a cAMP Agonist
Molecular Pharmacology 2013; 84: 384 - 392

Nieddu E, Pollarolo B, Merello L, Schenone S, Mazzei M
F508del-CFTR Rescue: A Matter of Cell Stress Response
Current Pharmaceutical Design 2013; 19: 3476 - 3496

Okeyo G, Wang W, Wei SP, Kirk KL
Converting Nonhydrolyzable Nucleotides to Strong Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Agonists by Gain of
Function (GOF) Mutations
Journal of Biological Chemistry 2013; 288: 17122 - 1713
 
Okiyoneda T, Veit G, Dekkers JF, Bagdany M, Soya N, Xu HJ, Roldan A, Verkman AS, Kurth M, Simon A, Hegedus T,
Mechanism-based corrector combination restores Delta F508-CFTR folding and function
Nature Chemical Biology 2013; 9: 444 - U69

Pelaseyed T, Gustafsson JK, Gustafsson IJ, Ermund A, Hansson GC
Carbachol-induced MUC17 endocytosis is concomitant with NHE3 internalization and CFTR membrane recruitment in enterocytes
American Journal of Physiology-cell Physiology 2013; 305: C457 - C467

Raksaseri P, Chatsudthipong V, Muanprasat C, Soodvilai S
Activation of liver X receptors reduces CFTR-mediated Cl- transport in kidney collecting duct cells
American Journal of Physiology-renal Physiology 2013; 305: F583 - F591

Raynal C, Baux D, Theze C, Bareil C, Taulan M, Roux AF, Claustres M, Tuffery-Giraud S, Georges MD
A Classification Model Relative to Splicing for Variants of Unknown Clinical Significance: Application to the CFTR Gene
Human Mutation 2013; 34: 774 - 784

Ren AX, Zhang WQ, Yarlagadda S, Sinha C, Arora K, Moon CS, Naren AP
MAST205 Competes with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-associated Ligand for Binding to CFTR to Regulate CFTR-mediated Fluid Transport
Journal of Biological Chemistry 2013; 288: 12325 - 1233

Rowe SM, Verkman AS
Cystic Fibrosis Transmembrane Regulator Correctors and Potentiators
Cold Spring Harbor Perspectives in Biology 2013; 5: 8:a009761

Saranko H, Tordai H, Telbisz A, Ozvegy-Laczka C, Erdos G, Sarkadi B, Hegedus T
Effects of the gout-causing Q141K polymorphism and a CFTR Delta F508 mimicking mutation on the processing and stability of the ABCG2
protein
Biochemical and Biophysical Research Communications 2013; 437: 140 - 145

Schippa S, Iebba V, Santangelo F, Gagliardi A, De Biase RV, Stamato A, Bertasi S, Lucarelli M, Conte MP, Quattrucci S
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Allelic Variants Relate to Shifts in Faecal Microbiota of Cystic Fibrosis Patients
Plos One 2013; 8: 4:e61176

Sebastian A, Rishishwar L, Wang JR, Bernard KF, Conley AB, McCarty NA, Jordan IK
Origin and evolution of the cystic fibrosis transmembrane regulator protein R domain
Gene 2013; 523: 137 - 146

Smith L, Litman P, Kohli E, Amick J, Page RC, Misra S, Liedtke CM
RACK1 interacts with filamin-A to regulate plasma membrane levels of the cystic fibrosis transmembrane conductance regulator
American Journal of Physiology-cell Physiology 2013; 305: C111 - C120
 
Snodgrass SM, Cihil KM, Cornuet PK, Myerburg MM, Swiatecka-Urban A
Tgf-beta 1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of Delta F508-Cftr in Primary Differentiated Human Bronchial Epithelial
Cells
Plos One 2013; 8: 5:e63167

Snyder DS, Tradtrantip L, Battula S, Yao CJ, Phuan PW, Fettinger JC, Kurth MJ, Verkman AS
Absolute Configuration and Biological Properties of Enantiomers of CFTR Inhibitor BPO-27
Acs Medicinal Chemistry Letters 2013; 4: 456 - 459

Sohma Y, Yu YC, Hwang TC
Curcumin and Genistein: the Combined Effects on Disease-associated CFTR Mutants and their Clinical Implications
Current Pharmaceutical Design 2013; 19: 3521 - 3528

Tang S, Moonnumakal SP, Stevens B, Douglas G, Mason S, Schmitt ES, Eng CM, Katz M, Fang P
Characterization of a recurrent 3.8 kb deletion involving exons 17a and 17b within the CFTR gene
Journal of Cystic Fibrosis 2013; 12: 290 - 294

Tukaye DN, Kwon SH, Guggino WB
The GAP Portion of Pseudomonas Aeruginosa Type III Secreted Toxin ExoS Upregulates Total and Surface Levels of Wild Type CFTR
Cellular Physiology and Biochemistry 2013; 31: 153 - 165

van Meegen MA, Terheggen-Lagro SWJ, Koymans KJ, van der Ent CK, Beekman JM
Apical CFTR Expression in Human Nasal Epithelium Correlates with Lung Disease in Cystic Fibrosis
Plos One 2013; 8: 3:e57617

Verkman AS, Synder D, Tradtrantip L, Thiagarajah JR, Anderson MO
CFTR Inhibitors
Current Pharmaceutical Design 2013; 19: 3529 - 3541

Vijftigschild LAW, van der Ent CK, Beekman JM
A novel fluorescent sensor for measurement of CFTR function by flow cytometry
Cytometry Part a 2013; 83A: 576 - 584

Villella VR, Esposito S, Bruscia EM, Vicinanza M, Cenci S, Guido S, Pettoello-Mantovani M, Carnuccio R, De Matteis MA,
Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator
Cell Death and Differentiation 2013; 20: 1101 - 1115

Vohwinkel CU, Vadasz I
Influenza A matrix protein M2 downregulates CFTR: inhibition of chloride transport by a proton channel of the viral envelope
American Journal of Physiology-lung Cellular and Molecular Physiology 2013; 304: L813 - L816

Wang X, Zhang YF, Yu B, Yang S, Luan J, Liu X, Yang H
Dehydrocostuslactone, a sesquiterpene lactone activates wild-type and F508 mutant CFTR chloride channel
Journal of Asian Natural Products Research 2013; 15: 855 - 866

Xie C, Jiang XH, Zhang JT, Sun TT, Dong JD, Sanders AJ, Diao RY, Wang Y, Fok KL, Tsang LL, Yu MK, Zhang XH,
CFTR suppresses tumor progression through miR-193b targeting urokinase plasminogen activator (uPA) in prostate cancer
Oncogene 2013; 32: 2282 - 2291
 
Yang Y, Cheng Y, Lian QQ, Yang L, Qi W, Wu DR, Zheng X, Liu YJ, Li WJ, Jin SW, Smith FG
Contribution of CFTR to Alveolar Fluid Clearance by Lipoxin A(4) via PI3K/Akt Pathway in LPS-Induced Acute Lung Injury
Mediators of Inflammation 2013; 62: 19 - 29

Zhou LM, Palais RA, Ye FL, Chen J, Montgomery JL, Wittwer CT
Symmetric Snapback Primers for Scanning and Genotyping of the Cystic Fibrosis Transmembrane Conductance Regulator Gene
Clinical Chemistry 2013; 59: 1052 - 1061

Zhou Y, Song KJ, Painter RG, Aiken M, Reiser J, Stanton BA, Nauseef WM, Wang GS
Cystic Fibrosis Transmembrane Conductance Regulator Recruitment to Phagosomes in Neutrophils
Journal of Innate Immunity 2013; 5: 219 - 230

Clinical

Fainardi V, Koo SD, Padley SPG, Lam SHY, Bush A
Prevalence of scoliosis in cystic fibrosis
Pediatric Pulmonology 2013; 48: 553 - 555

Farjadian S, Moghtaderi M, Kashef S, Alyasin S, Najib K, Saki F
Clinical and Genetic Features in Patients with Cystic Fibrosis in Southwestern Iran
Iranian Journal of Pediatrics 2013; 23: 212 - 215

Goss CH, Ratjen F
Update in Cystic Fibrosis 2012
American Journal of Respiratory and Critical Care Medicine 2013; 187: 915 - 919

Jones A, Bilton D, Evans TW, Finney SJ
Predictors of outcome in patients with cystic fibrosis requiring endotracheal intubation
Respirology 2013; 18: 630 - 636

Lo C, Cooper-Knock J, Garrard K, Martindale J, Williams T, Shaw P
Concurrent amyotrophic lateral sclerosis and cystic fibrosis supports common pathways of pathogenesis
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 2013; 14: 473 - 475

Rechitsky S, Verlinsky O, Kuliev A
PGD for cystic fibrosis patients and couples at risk of an additional genetic disorder combined with 24-chromosome aneuploidy testing
Reproductive Biomedicine Online 2013; 26: 420 - 430

Tümmler B
Cystic fibrosis
Monatsschrift Kinderheilkunde 2013; 161: 399

Diabetes

Bellin MD, Laguna T, Leschyshyn J, Regelmann W, Dunitz J, Billings J, Moran A
Insulin secretion improves in cystic fibrosis following ivacaftor correction of CFTR: a small pilot study
Pediatric Diabetes 2013; 14: 417 - 421

Ishimo MC, Belson L, Ziai S, Levy E, Berthiaume Y, Coderre L, Rabasa-Lhoret R
Hypertriglyceridemia is associated with insulin levels in adult cystic fibrosis patients
Journal of Cystic Fibrosis 2013; 12: 271 - 276

Kelly A, Moran A
Update on cystic fibrosis-related diabetes
Journal of Cystic Fibrosis 2013; 12: 318 - 331
Redondo MJ, Rodriguez LM, Escalante M, Smith EO, Balasubramanyam A, Haymond MW
Types of pediatric diabetes mellitus defined by anti-islet autoimmunity and random C-peptide at diagnosis
Pediatric Diabetes 2013; 14: 333 - 340

Sunni M, Bellin MD, Moran A
Exogenous insulin requirements do not differ between youth and adults with cystic fibrosis related diabetes
Pediatric Diabetes 2013; 14: 295 - 298

Diagnosis

Deans Z, Fiorentino F, Biricik A, Traeger-Synodinos J, Moutou C, De Rycke M, Renwick P, SenGupta S, Goossens V, Harton G
The experience of 3 years of external quality assessment of preimplantation genetic diagnosis for cystic fibrosis
European Journal of Human Genetics 2013; 21: 800 - 806

Goncalves AC, Marson FAD, Mendonca RMD, Ribeiro JD, Ribeiro AF, Paschoal IA, Levy CE
Saliva as a potential tool for cystic fibrosis diagnosis
Diagnostic Pathology 2013; 8: Article 46

Lench N, Barrett A, Fielding S, McKay F, Hill M, Jenkins L, White H, Chitty LS
The clinical implementation of non-invasive prenatal diagnosis for single-gene disorders: challenges and progress made
Prenatal Diagnosis 2013; 33: 555 - 562

Pullan NJ, Thurston V, Barber S
Evaluation of an inductively coupled plasma mass spectrometry method for the analysis of sweat chloride and sodium for use in the diagnosis of cystic fibrosis
Annals of Clinical Biochemistry 2013; 50: 267 - 270

Rechitsky S, Verlinsky O, Kuliev A
PGD for cystic fibrosis patients and couples at risk of an additional genetic disorder combined with 24-chromosome aneuploidy testing
Reproductive Biomedicine Online 2013; 26: 420 - 430

Thao NK, Borgard JP, Miled R, Rota M
Sweat chloride measurement using direct potentiometry: Spotchem (R) (Elitech-Arkray) evaluation and comparison with coulometry and conductivity
Annales de Biologie Clinique 2013; 71: 443 - 448

Trujillano D, Ramos MD, Gonzalez J, Tornador C, Sotillo F, Escaramis G, Ossowski S, Armengol L, Casals T, Estivill X
Next generation diagnostics of cystic fibrosis and CFTR-related disorders by targeted multiplex high-coverage resequencing of CFTR
Journal of Medical Genetics 2013; 50: 455 - 462

Wujcik EK, Blasdel NJ, Trowbridge D, Monty CN
Ion Sensor for the Quantification of Sodium in Sweat Samples
IEEE Sensors Journal 2013; 13: 3430 - 3436

Endocrinology

Naehrlich L, Dorr HG, Bagheri-Behrouzi A, Rauh M
Iodine deficiency and subclinical hypothyroidism are common in cystic fibrosis patients
Journal of Trace Elements in Medicine and Biology 2013; 27: 122 - 125
 
Exercise

Burtin C, Van Remoortel H, Vrijsen B, Langer D, Colpaert K, Gosselink R, Decramer M, Dupont L, Troosters T
Impact of exacerbations of cystic fibrosis on muscle strength
Respiratory Research 2013; 14: Article 46

Dassios T, Katelari A, Doudounakis S, Dimitriou G
Aerobic exercise and respiratory muscle strength in patients with cystic fibrosis
Respiratory Medicine 2013; 107: 684 - 690

Gruet M, Brisswalter J, Vallier C, Mely L, Vallier JM
Strategies to enhance physical performance in cystic fibrosis patients
Science & Sports 2013; 28: 115 - 124

Higgins LW, Robertson RJ, Kelsey SF, Olson MB, Hoffman LA, Rebovich PJ, Haile L, Orenstein DM
Exercise intensity self-regulation using the OMNI scale in children with cystic fibrosis
Pediatric Pulmonology 2013; 48: 497 - 505

Moco VJR, Lopes AJ, Vigario PS, Almeida VP, Dias RF, Menezes SLS, Guimaraes FS
Pulmonary disease severity and peripheral muscle function as limiting factors for exercise capacity in adult patients with cystic fibrosis
Isokinetics and Exercise Science 2013; 21: 219 - 226

Rasekaba TM, Button BM, Wilson JW, Holland AE
Reduced physical activity associated with work and transport in adults with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 229 - 233

Vivodtzev I, Decorte N, Wuyam B, Gonnet N, Durieu I, Levy P, Cracowski JL, Cracowski C
Benefits of Neuromuscular Electrical Stimulation Prior to Endurance Training in Patients With Cystic Fibrosis and Severe Pulmonary Dysfunction
Chest 2013; 143: 485 - 493

Ward N, White D, Rowe H, Stiller K, Sullivan T
Physical activity levels of patients with cystic fibrosis hospitalised with an acute respiratory exacerbation
Respiratory Medicine 2013; 107: 1014 - 1020

Gastroenterology

Gelfond D, Borowitz D
Gastrointestinal Complications of Cystic Fibrosis
Clinical Gastroenterology and Hepatology 2013; 11: 333 - 342

Gelfond D, Ma CX, Semler J, Borowitz D
Intestinal pH and Gastrointestinal Transit Profiles in Cystic Fibrosis Patients Measured by Wireless Motility Capsule
Digestive Diseases and Sciences 2013; 58: 2275 - 2281

Kaunitz JD, Akiba Y
Wireless Telemetry and Cystic Fibrosis: Just the pHacts
Digestive Diseases and Sciences 2013; 58: 2129 - 2130

Kolbe EW, Tamm S, Hedtfeld S, Becker T, Tummler B, Stanke F
CLCA4 variants determine the manifestation of the cystic fibrosis basic defect in the intestine
European Journal of Human Genetics 2013; 21: 691 - 694

Ramos AFP, de Fuccio MB, Moretzsohn LD, Barbosa AJA, Passos MDF, Carvalho RS, Coelho LGV
Cystic fibrosis, gastroduodenal inflammation, duodenal ulcer, and H. pylori infection: The "cystic fibrosis paradox" revisited
Journal of Cystic Fibrosis 2013; 12: 377 - 383
Servidoni MF, Sousa M, Vinagre AM, Cardoso SR, Ribeiro MA, Meirelles LR, de Carvalho RB, Kunzelmann K, Ribeiro AF,
Rectal forceps biopsy procedure in cystic fibrosis: technical aspects and patients perspective for clinical trials feasibility
BMC Gastroenterology 2013; 13: Article No 9

Sheikh SI, Ryan-Wenger NA, McCoy KS
Outcomes of surgical management of severe GERD in patients with cystic fibrosis
Pediatric Pulmonology 2013; 48: 556 - 562

Stallings VA, Mondick JT, Schall JI, Barrett JS, Wilson M, Mascarenhas MR
Diagnosing malabsorption with systemic lipid profiling: pharmacokinetics of pentadecanoic acid and triheptadecanoic acid following oral administration in healthy subjects and subjects with cystic fibrosis
International Journal of Clinical Pharmacology and Therapeutics 2013; 51: 263 - 273

Wilschanski M, Novak I
The Cystic Fibrosis of Exocrine Pancreas
Cold Spring Harbor Perspectives in Medicine 2013; 3: 5:a009746

Genetics

Boudaya M, Fredj SH, Siala H, Bibi A, Messaoud T
Identification of a cystic fibrosis mutation W19X in Tunisia
Annales de Biologie Clinique 2013; 71: 223 - 226

Clarke LA, Sousa L, Barreto C, Amaral MD
Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies
Respiratory Research 2013; 14: Article 38

Coelho-Macias V, Fernandes S, Lamarao P, Assis-Pacheco F, Cardoso J
Aquagenic keratoderma associated with a mutation of the cystic fibrosis gene
Revista Portuguesa de Pneumologia 2013; 19: 125 - 128

Dal'Maso VB, Mallmann L, Siebert M, Simon L, Saraiva-Pereira ML, Dalcin PDR
Diagnostic contribution of molecular analysis of the cystic fibrosis transmembrane conductance regulator gene in patients suspected of having mild or atypical cystic fibrosis
Jornal Brasileiro de Pneumologia 2013; 39: 181 - 189

Davis SD, Ferkol T
Identifying the Origins of Cystic Fibrosis Lung Disease
New England Journal of Medicine 2013; 368: 2026 - 2028

Dharajiya N, Chisholm KM, Dietz L, Richards CS, Kharrazi M, Schrijver I
Identification of the CFTR p.Phe508Del founder mutation in the absence of a polythymidine 9T allele in a Hispanic patient
Clinical Genetics 2013; 83: 598 - 599

Fredj SH, Boudaya M, Oueslati S, Sahnoun S, Sahli C, Siala H, Boussetta K, Bibi A, Messaoud T
New frameshift CF mutation 3729delAinsTCT in a Tunisian cystic fibrosis patient
Journal of Genetics 2013; 92: 81 - 83

Giordano S, Amato F, Elce A, Monti M, Iannone C, Pucci P, Seia M, Angioni A, Zarrilli F, Castaldo G, Tomaiuolo R
Molecular and Functional Analysis of the Large 5 ' Promoter Region of CFTR Gene Revealed Pathogenic Mutations in CF and
CFTR-Related Disorders
Journal of Molecular Diagnostics 2013; 15: 331 - 340
Gisler FM, von Kanel T, Kraemer R, Schaller A, Gallati S
Identification of SNPs in the cystic fibrosis interactome influencing pulmonary progression in cystic fibrosis
European Journal of Human Genetics 2013; 21: 397 - 403

Jang MA, Kim SY, Jeong BH, Park HY, Jeon K, Kim JW, Ki CS, Koh WJ
Association of CFTR gene variants with nontuberculous mycobacterial lung disease in a Korean population with a low prevalence of cystic fibrosis
Journal of Human Genetics 2013; 58: 298 - 303

Kizil Y, Ergun MA, Aydil U, Uslu S
Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations in Patients with Massive Nasal Polyposis
Nobel Medicus 2013; 9: 17 - 20

Marson FAD, Bertuzzo CS, Secolin R, Ribeiro AF, Ribeiro JD
Genetic interaction of GSH metabolic pathway genes in cystic fibrosis
BMC Medical Genetics 2013; 14: Article No 6

Masekela R, Zampoli M, Westwood AT, White DA, Green RJ, Olorunju S, Kwofie-Mensah M
Phenotypic expression of the 3120+1G > A mutation in non-Caucasian children with cystic fibrosis in South Africa
Journal of Cystic Fibrosis 2013; 12: 363 - 366

Milla CE
Cystic fibrosis in the era of genomic medicine
Current Opinion in Pediatrics 2013; 25: 323 - 328

Montagnani M, Cazzato S, Mutignani M, Cevenini M, Guidetti E, Ben Zvi I, Aldini R, Saraceni G, Cavoli C, Garagnani P,
A Patient With Pancreas Divisum, Recurrent Acute Pancreatitis, and Homozygosity for the Cystic Fibrosis Transmembrane Regulator-Associated Protein 5T Allele
Clinical Gastroenterology and Hepatology 2013; 11: 579 - 581

Omran A, Elimam D, Yin F
MicroRNAs: New Insights into Chronic Childhood Diseases
Biomed Research International 2013; 197: 445 - 473

Stoerker J, Goodman TG, Walline HM, Sugalski J, Holland CA
Evaluation of a BeadXpress Assay for a 151-Mutation and Variant CFTR Screening Panel After 11,000 Samples: Implications for Practice
Diagnostic Molecular Pathology 2013; 22: 144 - 148

Growth & Development

Brookes I, Desai M, Duthie G, Lander A
Poor growth in an infant with cystic fibrosis due to an antenatal perforation and incomplete bowel obstruction
Journal of Cystic Fibrosis 2013; 12: 295 - 297

Donadio MVF, de Souza GC, Tiecher G, Heinzmann JP, Paim TF, Hommerding PX, Marostica PJC
Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis
Jornal de Pediatria 2013; 89: 151 - 157

Vieni G, Faraci S, Collura M, Lombardo M, Traverso G, Cristadoro S, Termini L, Lucanto MC, Furnari ML, Trimarchi G,
Stunting is an independent predictor of mortality in patients with cystic fibrosis
Clinical Nutrition 2013; 32: 382 - 385
 
Immunology & Inflammation

Baxter CG, Moore CB, Jones AM, Webb AK, Denning DW
IgE-Mediated Immune Responses and Airway Detection of Aspergillus and Candida in Adult Cystic Fibrosis
Chest 2013; 143: 1351 - 1357

Beaudoin T, LaFayette S, Roussel L, Berube J, Desrosiers M, Nguyen D, Rousseau S
The Level of p38 alpha Mitogen-Activated Protein Kinase Activation in Airway Epithelial Cells Determines the Onset of Innate Immune Responses to Planktonic and Biofilm Pseudomonas aeruginosa
Journal of Infectious Diseases 2013; 207: 1544 - 1555

Beiersdorf N, Schien M, Hentschel J, Pfister W, Markert UR, Mainz JG
Soluble inflammation markers in nasal lavage from CF patients and healthy controls
Journal of Cystic Fibrosis 2013; 12: 249 - 257

Bessich JL, Nymon AB, Moulton LA, Dorman D, Ashare A
Low Levels of Insulin-like Growth Factor-1 Contribute to Alveolar Macrophage Dysfunction in Cystic Fibrosis
Journal of Immunology 2013; 191: 378 - 385

Bickford JS, Mueller C, Newsom KJ, Barilovits SJ, Beachy DE, Herlihy JD, Keeler B, Flotte TR, Nick HS
Effect of allergy and inflammation on eicosanoid gene expression in CFTR deficiency
Journal of Cystic Fibrosis 2013; 12: 258 - 265

Chan YR, Chen K, Duncan SR, Lathrop KL, Latoche JD, Logar AJ, Pociask DA, Wahlberg BJ, Ray P, Ray A, Pilewski JM,
Patients with cystic fibrosis have inducible IL-17(+)IL-22(+) memory cells in lung draining lymph nodes
Journal of Allergy and Clinical Immunology 2013; 131: 1117

Hector A, Kormann M, Kammermeier J, Burdi S, Marcos V, Rieber N, Mays L, Illig T, Klopp N, Falkenstein F, Kappler M,
Expression and Regulation of Interferon-Related Development Regulator-1 in Cystic Fibrosis Neutrophils
American Journal of Respiratory Cell and Molecular Biology 2013; 48: 71 - 77

Houston N, Stewart N, Smith DS, Bell SC, Champion AC, Reid DW
Sputum neutrophils in cystic fibrosis patients display a reduced respiratory burst
Journal of Cystic Fibrosis 2013; 12: 352 - 362

Huaux F, Noel S, Dhooghe B, Panin N, Lo Re S, Lison D, Wallemacq P, Marbaix E, Scholte BJ, Lebecque P, Leal T
Dysregulated Proinflammatory and Fibrogenic Phenotype of Fibroblasts in Cystic Fibrosis
Plos One 2013; 8: 5: e64341

Kelly C, Williams MT, Elbom JS, Ennis M, Schock BC
Expression of the inflammatory regulator A20 correlates with lung function in patients with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 411 - 415

Kelly C, Williams MT, Mitchell K, Elborn JS, Ennis M, Schock BC
Expression of the nuclear factor-kappa B inhibitor A20 is altered in the cystic fibrosis epithelium
European Respiratory Journal 2013; 41: 1315 - 1323
 
Laval J, Touhami J, Herzenberg LA, Conrad C, Taylor N, Battini JL, Sitbon M, Tirouvanziam R
Metabolic Adaptation of Neutrophils in Cystic Fibrosis Airways Involves Distinct Shifts in Nutrient Transporter Expression
Journal of Immunology 2013; 190: 6043 - 6050

Mahmood F, Hakimiyan A, Jayaraman V, Wood S, Sivaramakrishnan G, Rehman T, Reuhs BL, Chubinskaya S, Shafikhani SH
A novel human antimicrobial factor targets Pseudomonas aeruginosa through its type III secretion system
Journal of Medical Microbiology 2013; 62: 531 - 539

Melvin TAN, Lane AP, Nguyen MT, Lin SY
Sinonasal epithelial cell expression of Toll-like receptor 9 is elevated in cystic fibrosis-associated chronic rhinosinusitis
American Journal of Rhinology & Allergy 2013; 27: 30 - 33

Patria MF, Longhi B, Esposito S
Influenza vaccination in children with cystic fibrosis
Expert Review of Vaccines 2013; 12: 415 - 420

Petrova G, Strateva T, Ulevinov N, Perenovska P
Immunoglobulin levels, cytology and microbiologic investigations of broncho-alveolar lavage in children with cystic fibrosis
Brazilian Journal of Infectious Diseases 2013; 17: 272 - 273

Rosario NA, Riedi CA
Cystic fibrosis and atopy
Allergologia Et Immunopathologia 2013; 41: 137 - 139

Stigliani M, Aquino RP, Del Gaudio P, Mencherini T, Sansone F, Russo P
Non-steroidal anti-inflammatory drug for pulmonary administration: Design and investigation of ketoprofen lysinate fine dry powders
International Journal of Pharmaceutics 2013; 448: 198 - 204

Tirelli AS, Colombo C, Torresani E, Fortunato F, Biffi A, Cariani L, Dacco V, Carbone A, Edefonti A, Paglialonga F, Conese
Effects of treatment in the levels of circulating cytokines and growth factors in cystic fibrosis and dialyzed patients by multi-analytical
determination with a biochip array platform
Cytokine 2013; 62: 413 - 420

Zemanick ET, Harris JK, Wagner BD, Robertson CE, Sagel SD, Stevens MJ, Accurso FJ, Laguna TA
Inflammation and Airway Microbiota during Cystic Fibrosis Pulmonary Exacerbations
Plos One 2013; 8: 4:e62917

Zhang PX, Murray TS, Villella VR, Ferrari E, Esposito S, D'Souza A, Raia V, Maiuri L, Krause DS, Egan ME, Bruscia EM
Reduced Caveolin-1 Promotes Hyperinflammation due to Abnormal Heme Oxygenase-1 Localization in Lipopolysaccharide-Challenged Macrophages with Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator
Journal of Immunology 2013; 190: 5196 - 5206

Liver Disease

Behrens CB, Langholz JH, Eiler J, Jenewein R, Naehrlich L, Fuchs K, Harth S, Krombach GA, Alzen GFP
A pilot study of the characterization of hepatic tissue strain in children with cystic-fibrosis-associated liver disease (CFLD) by acoustic
radiation force impulse imaging
Pediatric Radiology 2013; 43: 552 - 557

Eminoglu TF, Polat E, Gokce S, Ezgu FS, Senel S, Apaydin S
Cystic Fibrosis Presenting with Neonatal Cholestasis Simulating Biliary Atresia in a Patient with a Novel Mutation
Indian Journal of Pediatrics 2013; 80: 502 - 504

Kitson MT, Kemp WW, Iser DM, Paul E, Wilson JW, Roberts SK
Utility of transient elastography in the non-invasive evaluation of cystic fibrosis liver disease
Liver International 2013; 33: 698 - 705

Mayer-Hamblett N, Kloster M, Ramsey BW, Narkewicz MR, Saiman L, Goss CH
Incidence and clinical significance of elevated liver function tests in cystic fibrosis clinical trials
Contemporary Clinical Trials 2013; 34: 232 - 238

Microbiology

Agarwal R, Chakrabarti A, Shah A, Gupta D, Meis JF, Guleria R, Moss R, Denning DW
Allergic bronchopulmonary aspergillosis: review of literature and proposal of new diagnostic and classification criteria
Clinical and Experimental Allergy 2013; 43: 850 - 873

Bala A, Gupta RK, Chhibber S, Harjai K
Detection and quantification of quinolone signalling molecule: A third quorum sensing molecule of Pseudomonas aeruginosa by high
performance-thin layer chromatography
Journal of Chromatography B-analytical Technologies in the Biomedical 2013; 930: 30 - 35

Barrado L, Branas P, Orellana M, Martinez M, Garica G, Otero JR, Chaves F
Molecular Characterization of Achromobacter Isolates from Cystic Fibrosis and Non-Cystic Fibrosis Patients in Madrid, Spain
Journal of Clinical Microbiology 2013; 51: 1927 - 1930

Beaume M, Monina N, Schrenzel J, Francois P
Bacterial genome evolution within a clonal population: from in vitro investigations to in vivo observations
Future Microbiology 2013; 8: 661 - 674

Behrends V, Bell TJ, Liebeke M, Cordes-Blauert A, Ashraf SN, Nair C, Zlosnik JEA, Williams HD, Bundy JG
Metabolite Profiling to Characterize Disease-related Bacteria Gluconate Excretion by Pseudomonas Aeruginosa Mutants and Clinical Isolates from Cystic Fibrosis Patients
Journal of Biological Chemistry 2013; 288: 15098 - 1510

Bezuidt OKI, Klockgether J, Elsen S, Attree I, Davenport CF, Tümmler B
Intraclonal genome diversity of Pseudomonas aeruginosa clones CHA and TB
BMC Genomics 2013; 14: ArticleNo416
 
Bjarnsholt T
The role of bacterial biofilms in chronic infections
APMIS 2013; 121: 1 - 58

Bryant JM, Grogono DM, Greaves D, Foweraker J, Roddick I, Inns T, Reacher M, Haworth CS, Curran MD, Harris SR,
Whole-genome sequencing to identify transmission of Mycobacterium abscessus between patients with cystic fibrosis: a retrospective cohort study
Lancet 2013; 381: 1551 - 1560

Cescutti P, Cuzzi B, Herasimenka Y, Rizzo R
Structure of a novel exopolysaccharide produced by Burkholderia vietnamiensis, a cystic fibrosis opportunistic pathogen
Carbohydrate Polymers 2013; 94: 253 - 260

Charlson ES, Bittinger K, Chen J, Diamond JM, Li HZ, Collman RG, Bushman FD
Assessing Bacterial Populations in the Lung by Replicate Analysis of Samples from the Upper and Lower Respiratory Tracts
Plos One 2012; 7: 9:e42786

Choi HJ, Kim MH, Cho MS, Kim BK, Kim JY, Kim C, Park DS
Improved PCR for identification of Pseudomonas aeruginosa
Applied Microbiology and Biotechnology 2013; 97: 3643 - 3651

Choudhary KS, Hudaiberdiev S, Gelencser Z, Coutinho BG, Venturi V, Pongor S
The Organization of the Quorum Sensing luxI/R Family Genes in Burkholderia
International Journal of Molecular Sciences 2013; 14: 13727 - 1374

Christensen LD, van Gennip M, Rybtke MT, Wu H, Chiang WC, Alhede M, Høiby N, Nielsen TE, Givskov M, Tolker-Nielsen T
Clearance of Pseudomonas aeruginosa Foreign-Body Biofilm Infections through Reduction of the Cyclic Di-GMP Level in the Bacteria
Infection and Immunity 2013; 81: 2705 - 2713

Cremet L, Caroff N, Giraudeau C, Reynaud A, Caillon J, Corvec S
Detection of clonally related Escherichia coli isolates producing different CMY beta-lactamases from a cystic fibrosis patient
Journal of Antimicrobial Chemotherapy 2013; 68: 1032 - 1035

Denman CC, Brown AR
Mannitol promotes adherence of an outbreak strain of Burkholderia multivorans via an exopolysaccharide-independent mechanism that is associated with upregulation of newly identified fimbrial and afimbrial adhesins
Microbiology 2013; 159: 771 - 781

Do PCM, Nussbaum E, Moua J, Chin T, Randhawa I
Clinical significance of respiratory isolates for Mycobacterium abscessus complex from pediatric patients
Pediatric Pulmonology 2013; 48: 470 - 480

Duff RM, Simmonds NJ, Davies JC, Wilson R, Alton EW, Pantelidis P, Cox MJ, Cookson WOCM, Bilton D, Moffatt MF
A molecular comparison of microbial communities in bronchiectasis and cystic fibrosis
European Respiratory Journal 2013; 41: 991 - 993
 
Duus LM, Høiby N, Wang M, Schiotz O, Norskov-Lauritsen N
Bacteria of the genus Dyella can chronically colonise the airways of patients with cystic fibrosis and elicit a pronounced antibody response
International Journal of Medical Microbiology 2013; 303: 267 - 269

Duytschaever G, Huys G, Bekaert M, Boulanger L, De Boeck K, Vandamme P
Dysbiosis of bifidobacteria and Clostridium cluster XIVa in the cystic fibrosis fecal microbiota
Journal of Cystic Fibrosis 2013; 12: 206 - 215

Eickmeier O, Rieber N, Eckrich J, Hector A, Graeppler-Mainka U, Hartl D
Immune Response, Diagnosis and Treatment of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis Lung Disease
Current Pharmaceutical Design 2013; 19: 3669 - 3678

El-Kirat-Chatel S, Mil-Homens D, Beaussart A, Fialho AM, Dufrene YF
Single-molecule atomic force microscopy unravels the binding mechanism of a Burkholderia cenocepacia trimeric autotransporter adhesin
Molecular Microbiology 2013; 89: 649 - 659

Essoh C, Blouin Y, Loukou G, Cablanmian A, Lathro S, Kutter E, Thien HV, Vergnaud G, Pourcel C
The Susceptibility of Pseudomonas aeruginosa Strains from Cystic Fibrosis Patients to Bacteriophages
Plos One 2013; 8: 4:e60575

Estrada-de los Santos P, Vinuesa P, Martinez-Aguilar L, Hirsch AM, Caballero-Mellado J
Phylogenetic Analysis of Burkholderia Species by Multilocus Sequence Analysis
Current Microbiology 2013; 67: 51 - 60

Eusebio N, Coutinho CP, Sa-Correia I, Araujo R
SNaPBcen: a Novel and Practical Tool for Genotyping Burkholderia cenocepacia
Journal of Clinical Microbiology 2013; 51: 2646 - 2653

Eusebio N, Pinheiro T, Amorim AA, Gamboa F, Saraiva L, Gusmao L, Amorim A, Araujo R
SNaPaer. A Practical Single Nucleotide Polymorphism Multiplex Assay for Genotyping of Pseudomonas aeruginosa
Plos One 2013; 8: 6:e66083

Fernandez-Olmos A, Garcia-Castillo M, Alba JM, Morosini MI, Lamas A, Romero B, Galan JC, del Campo R, Canton R
Population Structure and Antimicrobial Susceptibility of Both Nonpersistent and Persistent Pseudomonas aeruginosa Isolates Recovered from Cystic Fibrosis Patients
Journal of Clinical Microbiology 2013; 51: 2761 - 2765

Ferreira AS, Silva IN, Oliveira VH, Becker JD, Givskov M, Ryan RP, Fernandes F, Moreira LM
Comparative Transcriptomic Analysis of the Burkholderia cepacia Tyrosine Kinase bceF Mutant Reveals a Role in Tolerance to Stress, Biofilm Formation, and Virulence
Applied and Environmental Microbiology 2013; 79: 3009 - 3020

Forier K, Messiaen AS, Raemdonck K, Deschout H, Rejman J, De Baets F, Nelis H, De Smedt SC, Demeester J, Coenye T,
Transport of nanoparticles in cystic fibrosis sputum and bacterial biofilms by single-particle tracking microscopy
Nanomedicine 2013; 8: 935 - 949
 
Gesualdo F, Bongiorno D, Rizzo C, Bella A, Menichella D, Stefani S, Tozzi AE
MRSA Nasal Colonization in Children: Prevalence Meta-analysis, Review of Risk Factors and Molecular Genetics
Pediatric Infectious Disease Journal 2013; 32: 479 - 485

Gustave JE, Jurcisek JA, McCoy KS, Goodman SD, Bakaletz LO
Targeting bacterial integration host factor to disrupt biofilms associated with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 384 - 389

Hayes G
Oestrogen induces mucoid conversion of Pseudomonas Aeruginosa in women with cystic fibrosis, and increases exacerbations
Thorax 2013; 68: 570

Hilmi D, Parcina M, Bode K, Ostrop J, Schuett S, Heeg K, Ziebuhr W, Sommerburg O, Bekeredjian-Ding I
Functional variation reflects intra-strain diversity of Staphylococcus aureus small colony variants in the host-pathogen interaction
International Journal of Medical Microbiology 2013; 303: 61 - 69

Hussain R, Oliynyk I, Roomans GM, Bjorkqvist M
Modulation of ENaC, CFTR, and iNOS expression in bronchial epithelial cells after stimulation with Staphylococcus epidermidis (94B080) and Staphylococcus aureus (90B083)
APMIS 2013; 121: 814 - 826

Jenkins RE, Yaseen FS, Monshi MM, Whitaker P, Meng XL, Farrell J, Hamlett J, Sanderson JP, El-Ghaiesh S, Peckham D,
beta-Lactam Antibiotics Form Distinct Haptenic Structures on Albumin and Activate Drug-Specific T-Lymphocyte Responses in Multiallergic Patients with Cystic Fibrosis
Chemical Research in Toxicology 2013; 26: 963 - 975

Karaba SM, White RC, Cianciotto NP
Stenotrophomonas maltophilia Encodes a Type II Protein Secretion System That Promotes Detrimental Effects on Lung Epithelial Cells
Infection and Immunity 2013; 81: 3210 - 3219

Keren I, Mulcahy LR, Lewis K
Persister Eradication: Lessons from the World of Natural Products
Natural Product Biosynthesis by Microorganisms and Plants, Pt C 2012; 517: 387 - 406

Kidd TJ, Ramsay KA, Hu HH, Marks GB, Wainwright CE, Bye PT, Elkins MR, Robinson PJ, Rose BR, Wilson JW,
Shared Pseudomonas aeruginosa genotypes are common in Australian cystic fibrosis centres
European Respiratory Journal 2013; 41: 1091 - 1100

Kieninger E, Singer F, Tapparel C, Alves MP, Latzin P, Tan HL, Bossley C, Casaulta C, Bush A, Davies JC, Kaiser L,
High Rhinovirus Burden in Lower Airways of Children With Cystic Fibrosis
Chest 2013; 143: 782 - 790

Konings AF, Martin LW, Sharples KJ, Roddam LF, Latham R, Reid DW, Lamont IL
Pseudomonas aeruginosa Uses Multiple Pathways To Acquire Iron during Chronic Infection in Cystic Fibrosis Lungs
Infection and Immunity 2013; 81: 2697 - 2704

Kreutzfeldt KM, McAdam PR, Claxton P, Holmes A, Seagar AL, Laurenson IF, Fitzgerald JR
Molecular Longitudinal Tracking of Mycobacterium abscessus spp. during Chronic Infection of the Human Lung
Plos One 2013; 8: 5:e63237

LaSarre B, Federle MJ
Exploiting Quorum Sensing To Confuse Bacterial Pathogens
Microbiology and Molecular Biology Reviews 2013; 77: 73 - 111

Lavin J, Bhushan B, Schroeder JW
Correlation between respiratory cultures and sinus cultures in children with cystic fibrosis
International Journal of Pediatric Otorhinolaryngology 2013; 77: 686 - 689

Le Gall F, Le Berre R, Rosec S, Hardy J, Gouriou S, Boisrame-Gastrin S, Vallet S, Rault G, Payan C, Hery-Arnaud G
Proposal of a quantitative PCR-based protocol for an optimal Pseudomonas aeruginosa detection in patients with cystic fibrosis
BMC Microbiology 2013; 13: Art icle No

Li XY, Hu Y, Gong J, Zhang LS, Wang GJ
Comparative genome characterization of Achromobacter members reveals potential genetic determinants facilitating the adaptation to a pathogenic lifestyle
Applied Microbiology and Biotechnology 2013; 97: 6413 - 6425

Liu JC, Modha DE, Gaillard EA
What is the clinical significance of filamentous fungi positive sputum cultures in patients with cystic fibrosis?
Journal of Cystic Fibrosis 2013; 12: 187 - 193

Lopez-Causape C, Rojo-Molinero E, Mulet X, Cabot G, Moya B, Figuerola J, Togores B, Perez JL, Oliver A
Clonal Dissemination, Emergence of Mutator Lineages and Antibiotic Resistance Evolution in Pseudomonas aeruginosa Cystic Fibrosis Chronic Lung Infection
Plos One 2013; 8: 8:e71001

Malcolm KC, Nichols EM, Caceres SM, Kret JE, Martiniano SL, Sagel SD, Chan ED, Caverly L, Solomon GM, Reynolds P,
Mycobacterium abscessus Induces a Limited Pattern of Neutrophil Activation That Promotes Pathogen Survival
Plos One 2013; 8: 2:e57402

Martin K, Baddal B, Mustafa N, Perry C, Underwood A, Constantidou C, Loman N, Kenna DT, Turton JF
Clusters of genetically similar isolates of Pseudomonas aeruginosa from multiple hospitals in the UK
Journal of Medical Microbiology 2013; 62: 988 - 1000

Novotny LA, Amer AO, Brockson ME, Goodman SD, Bakaletz LO
Structural Stability of Burkholderia cenocepacia Biofilms Is Reliant on eDNA Structure and Presence of a Bacterial Nucleic Acid Binding Protein
Plos One 2013; 8: 6:e67629

O'Sullivan BP, Sassetti CM
Infection control in cystic fibrosis: share and share alike
Lancet 2013; 381: 1517 - 1519

Palmer GC, Jorth PA, Whiteley M
The role of two Pseudomonas aeruginosa anthranilate synthases in tryptophan and quorum signal production
Microbiology-sgm 2013; 159: 959 - 969
 
Patankar YR, Lovewell RR, Poynter ME, Jyot J, Kazmierczak BI, Berwin B
Flagellar Motility Is a Key Determinant of the Magnitude of the Inflammasome Response to Pseudomonas aeruginosa
Infection and Immunity 2013; 81: 2043 - 2052

Pattison SH, Rogers GB, Crockard M, Elborn JS, Tunney MM
Molecular detection of CF lung pathogens: Current status and future potential
Journal of Cystic Fibrosis 2013; 12: 194 - 205

Ramos CG, Grilo AM, da Costa PJP, Feliciano JR, Leitao JH
MtvR Is a Global Small Noncoding Regulatory RNA in Burkholderia cenocepacia
Journal of Bacteriology 2013; 195: 3514 - 3523

Reymond JL, Bergmann M, Darbre T
Glycopeptide dendrimers as Pseudomonas aeruginosa biofilm inhibitors
Chemical Society Reviews 2013; 42: 4814 - 4822

Roman F, Roldan C, Trincado P, Ballesteros C, Carazo C, Vindel A
Detection of Linezolid-Resistant Staphylococcus aureus with 23S rRNA and Novel L4 Riboprotein Mutations in a Cystic Fibrosis Patient in Spain
Antimicrobial Agents and Chemotherapy 2013; 57: 2428 - 2429

Rusciano G, Capriglione P, Pesce G, Abete P, Carnovale V, Sasso A
Raman spectroscopy as a new tool for early detection of bacteria in patients with cystic fibrosis
Laser Physics Letters 2013; 10: UNSP 075603

Sales-Campos H, Tonani L, Cardoso CRB, Kress MRV
The Immune Interplay between the Host and the Pathogen in Aspergillus fumigatus Lung Infection
Biomed Research International 2013; N/K: 693023

Salvador-Garcia C, Yague-Guirao G, Pastor-Vivero MD, Saez-Nieto JA
Chronic colonization of Inquilinus limosus in a patient with cystic fibrosis: First report in Spain
Enfermedades Infecciosas Y Microbiologia Clinica 2013; 31: 414 - 415

Shanmugham B, Pan A
Identification and Characterization of Potential Therapeutic Candidates in Emerging Human Pathogen Mycobacterium abscessus: A Novel Hierarchical In Silico Approach
Plos One 2013; 8: 3:e59126

Sharma P, Diene SM, Thibeaut S, Bittar F, Roux V, Gomez C, Reynaud-Gaubert M, Rolain JM
Phenotypic and genotypic properties of Microbacterium yannicii, a recently described multidrug resistant bacterium isolated from a lung transplanted patient with cystic fibrosis in France
BMC Microbiology 2013; 13: Article 97

Spilker T, Vandamme P, LiPuma JJ
Identification and distribution of Achromobacter species in cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 298 - 301

Steiss JO, Lindemann H
Allergic bronchopulmonary aspergillosis in cystic fibrosis
Allergologie 2013; 36: 275 - 281

Suppiger A, Schmid N, Aguilar C, Pessi G, Eberl L
Two quorum sensing systems control biofilm formation and virulence in members of the Burkholderia cepacia complex
Virulence 2013; 4: 400 - 409
Syrmis MW, Moser RJ, Kidd TJ, Hunt P, Ramsay KA, Bell SC, Wainwright CE, Grimwood K, Nissen MD, Sloots TP, Whiley
High-throughput single-nucleotide polymorphism-based typing of shared Pseudomonas aeruginosa strains in cystic fibrosis patients using the Sequenom iPLEX platform
Journal of Medical Microbiology 2013; 62: 734 - 740

Valle J, Solano C, Garcia B, Toledo-Arana A, Lasa I
Biofilm switch and immune response determinants at early stages of infection
Trends in Microbiology 2013; 21: 364 - 371

Van Acker H, Sass A, Bazzini S, De Roy K, Udine C, Messiaen T, Riccardi G, Boon N, Nelis HJ, Mahenthiralingam E, Coenye
Biofilm-Grown Burkholderia cepacia Complex Cells Survive Antibiotic Treatment by Avoiding Production of Reactive Oxygen Species
Plos One 2013; 8: 4:e58943

van Ingen J, Griffith DE, Aksamit TR, Wagner D
Pulmonary diseases caused by non-tuberculous mycobacteria
Tuberculosis Book series :European Respiratory Monograph 2012; 56: 25-37

Vandamme P, Moore ERB, Cnockaert M, De Brandt E, Svensson-Stadler L, Houf K, Spilker T, LiPuma JJ
Achromobacter animicus sp nov., Achromobacter mucicolens sp nov., Achromobacter pulmonis sp nov and Achromobacter spiritinus sp nov., from human clinical samples
Systematic and Applied Microbiology 2013; 36: 1 - 10

Voronina OL, Chernukha MY, Shaginyan IA, Kunda MS, Avetisyan LR, Orlova AA, Lunin VG, Avakyan LV, Kapranov NI,
Characterization of genotypes for Burkholderia cepacia complex strains isolated from patients in hospitals of the Russian federation
Molecular Genetics Microbiology and Virology 2013; 28: 64 - 73

Wang SW, Parsek MR, Wozniak DJ, Ma LYZ
A spider web strategy of type IV pili-mediated migration to build a fibre-like Psl polysaccharide matrix in Pseudomonas aeruginosa biofilms
Environmental Microbiology 2013; 15: 2238 - 2253

Wolter DJ, Emerson JC, McNamara S, Buccat AM, Qin X, Cochrane E, Houston LS, Rogers GB, Marsh P, Prehar K, Pope
Staphylococcus aureus Small-Colony Variants Are Independently Associated With Worse Lung Disease in Children With Cystic Fibrosis
Clinical Infectious Diseases 2013; 57: 384 - 391

Workentine ML, Sibley CD, Glezerson B, Purighalla S, Norgaard-Gron JC, Parkins MD, Rabin HR, Surette MG
Phenotypic Heterogeneity of Pseudomonas aeruginosa Populations in a Cystic Fibrosis Patient
Plos One 2013; 8: 4:e60225

Wright EA, Fothergill JL, Paterson S, Brockhurst MA, Winstanley C
Sub-inhibitory concentrations of some antibiotics can drive diversification of Pseudomonas aeruginosa populations in artificial sputum medium
BMC Microbiology 2013; 13: Art No170

Zhao K, Tseng BS, Beckerman B, Jin F, Gibiansky ML, Harrison JJ, Luijten E, Parsek MR, Wong GCL
Psl trails guide exploration and microcolony formation in Pseudomonas aeruginosa biofilms
Nature 2013; 497: 388 -
Zouhair R, Rougeron A, Razafimandimby B, Kobi A, Bouchara JP, Giraud S
Distribution of the different species of the Pseudallescheria boydii/Scedosporium apiospermum complex in French patients with cystic
fibrosis
Medical Mycology 2013; 51: 603 - 613

Nutrition

Barja S, Capo E, Briceno L, Jakubson L, Mendez M, Becker A
Anemia and Iron Deficiency in Children with Chronic Respiratory Diseases
Nutricion Hospitalaria 2013; 28: 787 - 793

Gaskin KJ
Nutritional care in children with cystic fibrosis: are our patients becoming better?
European Journal of Clinical Nutrition 2013; 67: 558 - 564

Nicolo M, Stratton KW, Rooney W, Boullata J
Pancreatic Enzyme Replacement Therapy for Enterally Fed Patients With Cystic Fibrosis
Nutrition in Clinical Practice 2013; 28: 485 - 489

O'Brien CE, Harden H, Com G
A Survey of Nutrition Practices for Patients With Cystic Fibrosis
Nutrition in Clinical Practice 2013; 28: 237 - 241
Pandit C, Graham C, Selvadurai H, Gaskin K, Cooper P, van Asperen P
Festival food coma in cystic fibrosis
Pediatric Pulmonology 2013; 48: 725 - 727

Smith DJ, Anderson GJ, Lamont IL, Masel P, Bell SC, Reid DW
Accurate assessment of systemic iron status in cystic fibrosis will avoid the hazards of inappropriate iron supplementation
Journal of Cystic Fibrosis 2013; 12: 303 - 304

Physiotherapy

Holland AE, Button BM
Physiotherapy for cystic fibrosis in Australia: Knowledge and acceptance of the Consensus Statement recommendations
Respirology 2013; 18: 652 - 656

Psychosocial

Bodnar R, Holics K, Ujhelyi R, Kadar L, Kovacs L, Bolbas K, Szekely G, Gyurkovits K, Solyom E, Meszaros A
Quality of life in Hungarian patients with cystic fibrosis
Orvosi Hetilap 2013; 154: 784 - 791

Chudleigh J, Hoo AF, Ahmed D, Prasad A, Sheehan D, Francis J, Buckingham S, Cowlard J, Thia L, Nguyen TTD, Stocks J
Positive parental attitudes to participating in research involving newborn screened infants with CF
Journal of Cystic Fibrosis 2013; 12: 234 - 240

Higham L, Ahmed S, Ahmed M
Hoping to Live a "Normal" Life Whilst Living with Unpredictable Health and Fear of Death: Impact of Cystic Fibrosis on Young Adults

Kianifar HR, Bakhshoodeh B, Hebrani P, Behdani F
Qulaity of Life in Cystic Fibrosis Children
Iranian Journal of Pediatrics 2013; 23: 149 - 153
 
Pizzignacco TMP, Furtado MCC, Torres LAMM, Frizo AC, De Lima RAG
Lola had one thing: building an educational book for children with cystic fibrosis
Acta Paulista de Enfermagem 2012; 25: 319 - 322

Richards KM, Lester MK, Chin MJ, Marshall BC
A Preliminary Evaluation of the Effectiveness of the Cystic Fibrosis Foundation Mentoring Program for Respiratory Care
Respiratory Care 2013; 58: 764 - 769

Tluczek A, Becker T, Grieve A, Laxova A, Rock MJ, Gershan WM, Green CG, Farrell PM
Health-Related Quality of Life in Children and Adolescents with Cystic Fibrosis: Convergent Validity with Parent-Reports and Objective
Measures of Pulmonary Health
Journal of Developmental and Behavioral Pediatrics 2013; 34: 252 - 261

Uldall SW
Attitudes among Danes toward termination of pregnancy for social reasons and fetal abnormality
Prenatal Diagnosis 2013; 33: 716 - 721

Ullrich G
Employment in CF adults deserves much more attention
Journal of Cystic Fibrosis 2013; 12: 416

van Gool K, Norman R, Delatycki MB, Hall J, Massie J
Understanding the Costs of Care for Cystic Fibrosis: An Analysis by Age and Health State
Value in Health 2013; 16: 345 - 355

Pulmonology

Aanaes K, von Buchwald C, Hjuler T, Skov M, Alanin M, Johansen HK
The effect of sinus surgery with intensive follow-up on pathogenic sinus bacteria in patients with cystic fibrosis
American Journal of Rhinology & Allergy 2013; 27: E1 - E4

Byrnes CA, Vidmar S, Cheney JL, Carlin JB, Armstrong DS, Cooper PJ, Grimwood K, Moodie M, Robertson CF, Rosenfeld
Prospective evaluation of respiratory exacerbations in children with cystic fibrosis from newborn screening to 5 years of age
Thorax 2013; 68: 643 - 651

Collaco JM, Morrow CB, Green DM, Cutting GR, Mogayzel PJ
Environmental allergies and respiratory morbidities in cystic fibrosis
Pediatric Pulmonology 2013; 48: 857 - 864

Dassios T, Katelari A, Doudounakis S, Mantagos S, Dimitriou G
Respiratory muscle function in patients with cystic fibrosis
Pediatric Pulmonology 2013; 48: 865 - 873

Forrester DL, Knox AJ, Smyth AR, Fogarty AW
Measures of body habitus are associated with lung function in adults with cystic fibrosis: A population-based study
Journal of Cystic Fibrosis 2013; 12: 284 - 289

Fuchs SI, Junge S, Ellemunter H, Ballmann M, Gappa M
Calculation of the capnographic index based on expiratory molar mass-volume-curves - A suitable tool to screen for cystic fibrosis lung disease
Journal of Cystic Fibrosis 2013; 12: 277 - 283
 
Ghio AJ, Roggli VL, Soukup JM, Richards JH, Randell SH, Muhlebach MS
Iron accumulates in the lavage and explanted lungs of cystic fibrosis patients
Journal of Cystic Fibrosis 2013; 12: 390 - 398

Gilchrist FJ, Bright-Thomas RJ, Jones AM, Smith D, Spanel P, Webb AK, Lenney W
Hydrogen cyanide concentrations in the breath of adult cystic fibrosis patients with and without Pseudomonas aeruginosa infection
Journal of Breath Research 2013; 7: 2:026010

Goeminne PC, Kicinski M, Vermeulen F, Fierens F, De Boeck K, Nemery B, Nawrot TS, Dupont LJ
Impact of Air Pollution on Cystic Fibrosis Pulmonary Exacerbations A Case-Crossover Analysis
Chest 2013; 143: 946 - 954

Harris WT, Kelly DR, Zhou Y, Wang DZ, Macewen M, Hagood JS, Clancy JP, Ambalavanan N, Sorscher EJ
Myofibroblast Differentiation and Enhanced Tgf-B Signaling in Cystic Fibrosis Lung Disease
Plos One 2013; 8:

Horsley AR, Davies JC, Gray RD, Macleod KA, Donovan J, Aziz ZA, Bell NJ, Rainer M, Mt-Isa S, Voase N, Dewar MH,
Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation
Thorax 2013; 68: 532 - 539

Jaberoo MC, Pulido MA, Saleh HA
Modified Lothrop procedure in cystic fibrosis patients: does it have a role?
Journal of Laryngology and Otology 2013; 127: 666 - 669

Kallet RH, Volsko TA, Hess DR
Respiratory Care Year in Review 2012: Invasive Mechanical Ventilation, Noninvasive Ventilation, and Cystic Fibrosis
Respiratory Care 2013; 58: 702 - 711

Lim YW, Schmieder R, Haynes M, Furlan M, Matthews TD, Whiteson K, Poole SJ, Hayes CS, Low DA, Maughan H, Edwards
Mechanistic Model of Rothia mucilaginosa Adaptation toward Persistence in the CF Lung, Based on a Genome Reconstructed from
Metagenomic Data
Plos One 2013; 8: 5:e64285

McCarthy C, Dimitrov BD, Meurling IJ, Gunaratnam C, McElvaney NG
The CF-ABLE Score A Novel Clinical Prediction Rule for Prognosis in Patients With Cystic Fibrosis
Chest 2013; 143: 1358 - 1364

Mott LS, Park J, Gangell CL, de Klerk NH, Sly PD, Murray CP, Stick SM
Distribution of Early Structural Lung Changes due to Cystic Fibrosis Detected with Chest Computed Tomography
Journal of Pediatrics 2013; 163: 243 - U658

Muramatu LH, Stirbulov R, Forte WCN
Pulmonary function parameters and use of bronchodilators in patients with cystic fibrosis
Jornal Brasileiro de Pneumologia 2013; 39: 48 - 55

Quanjer PH, Bush A, Aurora P
Flow-to-Volume "Dysanapsis" in Cystic Fibrosis
American Journal of Respiratory and Critical Care Medicine 2013; 187: 891
 

Regelmann WE, Schechter MS, Wagener JS, Morgan WJ, Pasta DJ, Elkin EP, Konstan MW
Pulmonary exacerbations in cystic fibrosis: Young children with characteristic signs and symptoms
Pediatric Pulmonology 2013; 48: 649 - 657

Reid DW, Bell SC
ICU outcomes in cystic fibrosis following invasive ventilation
Respirology 2013; 18: 585 - 586

Rivas-Crespo MF, Jimenez DG, Quiros MDA, Aguirre AS, Gonzalez SH, Martin JJD, Otero JMG, Almarza AL,
High Serum Retinol and Lung Function in Young Patients With Cystic Fibrosis
Journal of Pediatric Gastroenterology and Nutrition 2013; 56: 657 - 662

Simanovsky N, Cohen-Cymberknoh M, Shoseyov D, Gileles-Hillel A, Wilschanski M, Kerem E, Hiller N
Differences in the Pattern of Structural Abnormalities on CT Scan in Patients With Cystic Fibrosis and Pancreatic Sufficiency or Insufficiency
Chest 2013; 144: 208 - 214

Singer F, Kieninger E, Abbas C, Yammine S, Fuchs O, Proietti E, Regamey N, Casaulta C, Frey U, Latzin P
Practicability of nitrogen multiple-breath washout measurements in a pediatric cystic fibrosis outpatient setting
Pediatric Pulmonology 2013; 48: 739 - 746

Sly PD, Gangell CL, Chen LP, Ware RS, Ranganathan S, Mott LS, Murray CP, Stick SM
Risk Factors for Bronchiectasis in Children with Cystic Fibrosis
New England Journal of Medicine 2013; 368: 1963 - 1970

Subbarao P, Stanojevic S, Brown M, Jensen R, Rosenfeld M, Davis S, Brumback L, Gustafsson P, Ratjen F
Lung Clearance Index as an Outcome Measure for Clinical Trials in Young Children with Cystic Fibrosis A Pilot Study Using Inhaled Hypertonic Saline
American Journal of Respiratory and Critical Care Medicine 2013; 188: 456 - 460

Verbanck S, Paiva M, Schuermans D, Malfroot A, Vincken W, Vanderhelst E
Acinar and conductive ventilation heterogeneity in severe CF lung disease: Back to the model
Respiratory Physiology & Neurobiology 2013; 188: 124 - 132

Vilozni D
Flow-to-Volume "Dysanapsis" in Cystic Fibrosis Reply
American Journal of Respiratory and Critical Care Medicine 2013; 187: 891 - 892

Widger J, Ranganathan S, Robinson PJ
Progression of structural lung disease on CT scans in children with cystic fibrosis related diabetes
Journal of Cystic Fibrosis 2013; 12: 216 - 221

Wielputz MO, Eichinger M, Puderbach M
Magnetic Resonance Imaging of Cystic Fibrosis Lung Disease
Journal of Thoracic Imaging 2013; 28: 151 - 159

Yalindag-Ozturk N, Vuran C, Karakoc F, Ersu R
Use of pumpless extracorporeal lung assist as rescue therapy in adolescent with cystic fibrosis
Pediatrics International 2013; 55: E83 - E85
 
Yamada T, Nakanishi Y, Homma T, Uehara K, Mizutani T, Hoshi E, Shimizu Y, Kawabata Y, Colby TV
Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema
Pathology International 2013; 63: 206 - 213

Zarei S, Mirtar A, Andresen B, Salamon P
Modeling the airflow in a lung with cystic fibrosis
Journal of Non-equilibrium Thermodynamics 2013; 38: 119 - 140

Ziobro R, Henry B, Edwards MJ, Lentsch AB, Gulbins E
Ceramide mediates lung fibrosis in cystic fibrosis
Biochemical and Biophysical Research Communications 2013; 434: 705 - 709

Radiology

de Freitas MR, Vasconcelos DN, Freitas AEDA, Maia JH, Silva CDE
Nasal endoscopic and CT scan alterations of the paranasal sinuses as predictors of severity in patients with cystic fibrosis
Brazilian Journal of Otorhinolaryngology 2013; 79: 480 - 486

de Jong PA, Owens CM
Radiation Dose for Pediatric Patients With Cystic Fibrosis A Continuous Adjustment Process and Remaining Concern
Chest 2012; 142: 1077

Fraioli F, Serra G, Ciarlo G, Massaccesi V, Liberali S, Fiorelli A, Macri F, Catalano C
Chest MR imaging in the follow-up of pulmonary alterations in paediatric patients with middle lobe syndrome: comparison with chest X-ray
Radiologia Medica 2013; 118: 444 - 455

Lopez-Rodriguez MJ, Lavado-Garcia JM, Canal-Macias ML, Calderon-Garcia JF, Moran JM, Pedrera-Zamorano JD
Quantitative Ultrasound in Spanish Children and Young Adults With Cystic Fibrosis
Biological Research for Nursing 2013; 15: 280 - 284

Mieville FA, Berteloot L, Grandjean A, Ayestaran P, Gudinchet F, Schmidt S, Brunelle F, Bochud FO, Verdun FR
Model-based iterative reconstruction in pediatric chest CT: assessment of image quality in a prospective study of children with cystic fibrosis
Pediatric Radiology 2013; 43: 558 - 567

O'Connell OJ, McGarrigle A, OConnor OJ, Maher MM, Plant BJ
Radiation Dose for Pediatric Patients With Cystic Fibrosis A Continuous Adjustment Process and Remaining Concern Response
Chest 2012; 142: 1078

Zeman KL, Wu JH, Donaldson SH, Bennett WD
Comparison of (133)Xenon Ventilation Equilibrium Scan (XV) and (99m)Technetium Transmission (TT) Scan for Use in Regional Lung Analysis by 2D Gamma Scintigraphy in Healthy and Cystic Fibrosis Lungs
Journal of Aerosol Medicine and Pulmonary Drug Delivery 2013; 26: 94 - 100
 
Screening

Beydon N, Robinson PD
Early Intervention for Newborns Screened for Cystic Fibrosis
American Journal of Respiratory and Critical Care Medicine 2013; 188: 409 - 410

Marson FAD, Bertuzzo CS, Ribeiro MAGD, Ribeiro AF, Ribeiro JD
Screening for F508del as a first step in the molecular diagnosis of cystic fibrosis
Jornal Brasileiro de Pneumologia 2013; 39: 306 - 316

McClaren BJ, Aitken M, Massie J, Amor D, Ukoumunne OC, Metcalfe SA
Cascade carrier testing after a child is diagnosed with cystic fibrosis through newborn screening: investigating why most relatives do not have testing
Genetics in Medicine 2013; 15: 533 - 540

Nahrlich L, Zimmer KP
Neonatal Cystic Fibrosis Screening-Time to Begin!
Deutsches Arzteblatt International 2013; 110: 354 - 355

Nunes AKC, Wachholz RG, Rover MRM, Souza LC
Prevalence of disorders detected by newborn screening in Santa Catarina
Arquivos Brasileiros de Endocrinologia E Metabologia 2013; 57: 360 - 367

Radivojevic D, Sovtic A, Minic P, Grkovic S, Guc-Scekic M, Lalic T, Miskovic M
Newborn screening for cystic fibrosis in Serbia: A pilot study
Pediatrics International 2013; 55: 181 - 184

Rueegg CS, Kuehni CE, Gallati S, Baumgartner M, Torresani T, Barben J
One-Year Evaluation of a Neonatal Screening Program for Cystic Fibrosis in Switzerland
Deutsches Arzteblatt International 2013; 110: 356 - U25

Sass AM, Schmerk C, Agnoli K, Norville PJ, Eberl L, Valvano MA, Mahenthiralingam E
The unexpected discovery of a novel low-oxygen-activated locus for the anoxic persistence of Burkholderia cenocepacia
ISME Journal 2013; 7: 1568 - 1581

Sobczynska-Tomaszewska A, Oltarzewski M, Czerska K, Wertheim-Tysarowska K, Sands D, Walkowiak J, Bal J, Mazurczak T
Newborn screening for cystic fibrosis: Polish 4 years' experience with CFTR sequencing strategy
European Journal of Human Genetics 2013; 21: 391 - 396

Therapy

Amaral MD, Farinha CM
Rescuing Mutant CFTR: A Multi-task Approach to a Better Outcome in Treating Cystic Fibrosis
Current Pharmaceutical Design 2013; 19: 3497 - 3508

Bilton D, Bellon G, Charlton B, Cooper P, De Boeck K, Flume PA, Fox HG, Gallagher CG, Geller DE, Haarman EG, Hebestreit
Pooled analysis of two large randomised phase III inhaled mannitol studies in cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 367 - 376

Conrad D, Haynes M, Salamon P, Rainey PB, Youle M, Rohwer F
Cystic Fibrosis Therapy: A Community Ecology Perspective
American Journal of Respiratory Cell and Molecular Biology 2013; 48: 150 - 156
Davies JC, Wainwright CE, Canny GJ, Chilvers MA, Howenstine MS, Munck A, Mainz JG, Rodriguez S, Li HH, Yen K,
Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation
American Journal of Respiratory and Critical Care Medicine 2013; 187: 1219 - 1225

Dentice R, Elkins M
Timing of dornase alfa inhalation for cystic fibrosis
Cochrane Database of Systematic Reviews 2013; 6: CD007923

Donaldson SH, Galietta L
New Pulmonary Therapies Directed at Targets Other than CFTR
Cold Spring Harbor Perspectives in Medicine 2013; 3: 6:a009787

Fajac I, Sermet-Gaudelus I
Cystic fibrosis: New treatments targeting the CFTR protein
Revue des Maladies Respiratoires 2013; 30: 255 - 261

Griese M, Kappler M, Eismann C, Ballmann M, Junge S, Rietschel E, van Koningsbruggen-Rietschel S, Staab D,
Inhalation Treatment with Glutathione in Patients with Cystic Fibrosis A Randomized Clinical Trial
American Journal of Respiratory and Critical Care Medicine 2013; 188: 83 - 89

McPhail GL, Clancy JP
IVACAFTOR: THE FIRST THERAPY ACTING ON THE PRIMARY CAUSE OF CYSTIC FIBROSIS
Drugs of Today 2013; 49: 253 - 260

Moss RB, Mistry SJ, Konstan MW, Pilewski JM, Kerem E, Tal-Singer R, Lazaar AL
Safety and early treatment effects of the CXCR2 antagonist SB-656933 in patients with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 241 - 248

Nahrlich L, Mainz JG, Adams C, Engel C, Herrmann G, Icheva V, Lauer J, Deppisch C, Wirth A, Unger K, Graepler-Mainka
Therapy of CF-Patients with Amitriptyline and Placebo - a Randomised, Double-Blind, Placebo-Controlled Phase IIb Multicenter, Cohort-Study
Cellular Physiology and Biochemistry 2013; 31: 505 - 512

Rowe SM, Liu B, Hill A, Hathorne H, Cohen M, Beamer JR, Accurso FJ, Dong QM, Ordonez CL, Stone AJ, Olson ER, Clancy
Optimizing Nasal Potential Difference Analysis for CFTR Modulator Development: Assessment of Ivacaftor in CF Subjects with the G551D-CFTR Mutation
Plos One 2013; 8: 7:e66955

Rowe SM, Reeves G, Hathorne H, Solomon GM, Abbi S, Renard D, Lock R, Zhou P, Danahay H, Clancy JP, Waltz DA
Reduced Sodium Transport With Nasal Administration of the Prostasin Inhibitor Camostat in Subjects With Cystic Fibrosis
Chest 2013; 144: 200 - 207

Wilschanski M
Novel Therapeutic Approaches for Cystic Fibrosis
Discovery Medicine 2013; 81: 127 - 133

Zaman K, Fraser-Butler M, Bennett D
Novel S-Nitrosothiols Have Potential Therapeutic Uses for Cystic Fibrosis
Current Pharmaceutical Design 2013; 19: 3509 - 3520
 
Transplantation

Borro JM, Rama P, Rey T, Fernandez-Rivera C
Long-Term Success of Combined Kidney-Lung Transplantation in a Patient With Cystic Fibrosis
Archivos de Bronconeumologia 2013; 49: 272 - 274

Corris PA
Lung Transplantation for Cystic Fibrosis and Bronchiectasis
Seminars in Respiratory and Critical Care Medicine 2013; 34: 297 - 304

Harring TR, Nguyen NTT, Liu H, Karpen SJ, Goss JA, O'Mahony CA
Liver transplantation in cystic fibrosis: A report from Baylor College of Medicine and the Texas Children's Hospital
Pediatric Transplantation 2013; 17: 271 - 277

Inci I, Schuurmans MM, Kestenholz P, Schneiter D, Hillinger S, Opitz I, Boehler A, Weder W
Long-term outcomes of bilateral lobar lung transplantation
European Journal of Cardio-thoracic Surgery 2013; 43: 1220 - 1225

Samano MN, Pego-Fernandes PM, Ribeiro AKF, Turaca K, Abdalla LG, Fernandes LM, Correia AT, Jatene FB
Lung Transplantation in Patients With Cystic Fibrosis
Transplantation Proceedings 2013; 45: 1137 - 1141

Thabut G, Christie JD, Mal H, Fournier M, Brugiere O, Leseche G, Castier Y, Rizopoulos D
Survival Benefit of Lung Transplant for Cystic Fibrosis since Lung Allocation Score Implementation
American Journal of Respiratory and Critical Care Medicine 2013; 187: 1335 - 1340

Vital D, Holzmann D, Boehler A, Hofer M
Nasal polyposis in lung transplant recipients with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 266 - 270

Witters P, Dupont L, Vermeulen F, Proesmans M, Cassiman D, Wallemacq P, De Boeck K
Lung transplantation in cystic fibrosis normalizes essential fatty acid profiles
Journal of Cystic Fibrosis 2013; 12: 222 - 228

Urology

Nazareth D, Walshaw M
A review of renal disease in cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 309 - 317