Sunday, January 19, 2014

For the latest news on the ECFS activities, and current references in cystic fibrosis

January 2014

1. Letter from the President - Stuart Elborn

Dear Friends,

A very Happy New Year to you all.

2013 was another very busy year for the European Cystic Fibrosis Society, with not only the Basic Science Conference in Malaga and the Annual conference in Lisbon, but a number of special projects and Working Group meetings were held with some selected updates given below. One event not previously reported on was the ECFS Board’s strategy meeting held in November, where a critical review was undertaken on our priorities for the next few years and how we are to achieve the goals set. Our new mission statement reflects these goals:

The ECFS is an international community of scientific and clinical professionals committed to improving survival and quality of life for people with CF by promoting high quality research, education and care.



Stuart Elborn, ECFS President

Later in this Newsletter you will see some new initiatives introduced for 2014 which comprise new sessions for the annual conference, ECFS Short-Term Fellowships, and a new method for membership renewal.

There will be Board elections in 2014 as Su Madge and Benny Assael will be stepping down. I would like to encourage you all to consider your own nomination or to nominate an ECFS member you believe would benefit the ECFS community by being actively engaged in the Board. Please consider how important these elections are for the future of the Society. Further information about the elections is included later in the Newsletter.

Planning for the 2014 conferences is well underway with programmes and registration information available on the ECFS website. I would like to thank all those who have submitted abstracts towards these events, and also to the invited speakers who have accepted to contribute presentations. We have excellent programmes planned and we look forward to your participation.

In December 2013, the ECFS issued a Bulletin to the membership with a link to the core content of the 2013 conference held in Lisbon with special video reports, including interviews with international experts and slides with comments. We hope you may have found this both educational and enjoyable, but also perhaps an incentive to join us in Gothenburg in June 2014. The link is also available under your “MY ECFS” tab on the ECFS website (applies to current members).
logo-Lisbon Core Content

Journal of Cystic Fibrosis

We are delighted to welcome Scott Bell as the new Editor-in-Chief of the Journal of Cystic Fibrosis (JCF). Scott, as many of you will know, is based at The Prince Charles Hospital in Brisbane, and took over the helm of the JCF on 1 December 2013. Scott is being supported by Dominik Hartl as Deputy Editor.

Our thanks go to Harry Heijerman for taking on the editorship of the Journal since the untimely death of Gerd Döring last summer.

Scott Bell, Editor-in-Chief JCF

Scott Bell

Standards of Care: The special project group set up to review the original 2005 paper will be publishing their report covering Best Practice, Centre Framework and Quality Monitoring in 2014 in a supplement to the Journal of Cystic Fibrosis. There will be a dedicated symposium at the Gothenburg conference in June.

ERS/ECFS Joint Task Force: Following the preparatory meetings on the provision of care for adults with CF in Europe – a consensus meeting will be held in Lausanne, Switzerland on 26 February 2014.

Joint CFF/ECFS Mental Health Guidelines. Following the initial meeting of the joint group in May 2013, a survey has been undertaken to collate data from both US and European centres and a consensus meeting is to be held 11-12 April 2014.

ECFS Book: The 2nd Edition of the ECFS book has been initiated with the title of “Ageing with CF”. We hope to be able to send the book to our members during 2014.

We have a lot to do over the coming months and I hope you will join us for some of these activities.

My thanks, as always, to Dr. Henry Ryley for compiling the current references in Cystic Fibrosis contained at the end of this Newsletter.

Please contact us if you have news items you would like to have included in future Newsletters or published on our website.

Yours sincerely,
Stuart Elborn, ECFS President

2. ECFS Award – Call for Nominations

The ECFS Award is given annually to honour a person who has made an outstanding contribution to our basic understanding of cystic fibrosis or to the treatment or care of patients with cystic fibrosis. The winner of the award will be invited to present a lecture at the Opening Plenary on 11 June 2014 of the annual conference in Gothenburg.

You are cordially invited to nominate a candidate for this award. The deadline for proposals is 15 February 2014. Please mail your proposal, accompanied by a detailed motivation and curriculum vitae of the candidate to the ECFS Executive Director Christine Dubois (

3. ECFS Board Elections – Call for Nominations

Vote image

The ECFS cordially invites nominations for the following Board positions:
President Elect: Stuart Elborn will finish his term as President in 2015.
2 Board Positions: Su Madge will finish her two terms on the Board in 2014, and Benny Assael is stepping down from the Board after completing one term.

Job Descriptions and person specifics are available for both the President and Board members. President’s Job Description | Board Member’s Job Description.

Nominations should be sent to the ECFS Executive Director Christine Dubois ( by 4 March 2014 together with a motivational statement, confirmation that the candidate has agreed to the nomination, and a curriculum vitae. All nominated candidates must be current members of the ECFS. Information about the nominated candidates will be sent to the membership in April for an online vote prior to the Annual General Meeting in June where the results will be announced.

4. New initiatives for 2014

Annual Conference:

Two new sessions have been introduced for the Gothenburg conference that we hope will generate much interest.

- Breakfast sessions on the Thursday and Friday: “Meet the Experts”. These are small interactive sessions led by experts to stimulate discussion, answer questions and offer advice. The format encourages a more personal approach to learning.
- Interactive Case Studies session on the Friday using audience response system technology. This feature will allow the audience to answer questions asked by the presenter during the talk. Each interactive case presents an evolving patient history and a series of questions designed to test the diagnostic and/or therapeutic skills of the audience. The audience will receive immediate feedback on the answers and treatment choices, along with the opportunity to compare their final score with their peers. A panel of experts in the field will facilitate the discussion and foster exchange of information or discuss treatment possibilities based on their expertise and knowledge of the latest scientific publications.

Further details about both sessions can be found on the conference website.

ECFS Short-Term Fellowships:

The ECFS is happy to announce the introduction of Short-Term Fellowships for young researchers (under the age of 35).

The grants, up to 750 Euro, are specifically for travel/accommodation support to visit a CF Centre or CF laboratory in Europe to learn a specific technique. Further information can be found on the ECFS website at


In addition to the regular methods of renewing membership subscriptions, it is now possible for prior members of the Society to renew their membership in conjunction with registration to the annual conference. Previously it was only possible to combine the registration with NEW membership – so we hope this new facility will be of interest to members who prefer to complete both procedures in one action. The ECFS Office will happily help with any membership enquiries.


5. ECORN-CF – 2013 Update

The main aim is to build a model of a "European Centres of Reference Network for Cystic Fibrosis" (ECORN-CF). Patients, doctors, as well as other care team members should have easy access to expert knowledge and advice for cystic fibrosis.


ECORN-CF was originally a pilot project that was co-funded by the EU for three years until 2010. For the last three years (2011–2013), the day-to-day management of the several local expert advice forums and the quality control necessary for the quality-assured central English archive of ECORN-CF has been enabled with the infrastructure support of several partners from different countries (Mukoviszidose e.V., Christiane Herzog Stiftung, ECFS, CFE, Vaincre la Mucoviscidose, Association Belge de Lutte contre la Mucoviscidose)

Activities in 2013
Questions asked on the local platforms:

437 questions have been asked in the seven expert advice forums from Jan 1 until Dec 12, 2013 (some of them are still in the answering process). Most questions were asked in French (164), followed by German (141), English (53), Greek (24), Dutch (23), Romanian (18) and Lithuanian (14).

Compared to the reference period of last years’ reports (reference period was Jan 1 - Nov 1), there is a decrease of questions.

In 2011, a total number of 445 questions had been asked from Jan 1 - Nov 1 (10 languages)
In 2012, a total number of 467 questions had been asked from Jan 1 - Nov 1 (9 languages)
In 2013, a total number of 378 questions had been asked from Jan 1 - Nov 1 (7 languages)

Question & answer pairs (QA pairs) sent to the central English archive and published after quality check: 423 QA pairs being of cross-national relevance were translated from the respective language teams into English and sent to the central English archive for quality check via the system from Jan 1 until Dec 12, 2013. Only if the answer is in accordance with European consensus statements, guidelines and – in the absence of these – consented local and foreign expert opinion, the QA pair is published in the English central archive. If the answer is only satisfactory or with flaws, the English answer is amended and a feedback loop ensures that the answer in the original language is also updated in the local expert advice. Thanks to this input experts have been able to expand their knowledge.

Utilization of synergies: The proof editor taking care of the English archive receives the English versions of all language platforms. Sometimes, team A gives a very extensive answer about a topic that team B answered before but not as extensively. By additional comments in the data record, the two answers can be related to each other. If there are new aspects, then the original language team is also asked to write an amendment in the original language data record.

The future of the ECORN-CF project is under discussion.

6. Upcoming Events

- ECFS Diagnostic Network Working Group Meeting - 13-15 February, Berlin, Germany
- ECFS 11th Basic Science Conference - 26-29 March 2014 - Malta
- ECFS Board Meeting - 10 June 2014 – Gothenburg, Sweden
- 37th European CF Conference - 11-14 June 2014 – Gothenburg, Sweden

7. Current References in Cystic Fibrosis

Adults & Adolescents

Ball R, Southern KW, McCormack P, Duff AJA, Brownlee KG, McNamara PS
Adherence to nebulised therapies in adolescents with cystic fibrosis is best on week-days during school term-time
Journal of Cystic Fibrosis 2013; 12: 440 - 444

Jones AM
Adults with Cystic Fibrosis Should be Treated at a Specialist Centre
Paediatric Respiratory Reviews 2013; 14:Supl1: 13 - 15

Kopp BT, Hayes D, Ratkiewicz M, Baron N, Splaingard M
Light exposure and depression in hospitalized adult patients with cystic fibrosis
Journal of Affective Disorders 2013; 150: 585 - 589

Kreindler James L., Miller Victoria A.
Cystic fibrosis: addressing the transition from pediatric to adult-oriented health care
Patient Preference and Adherence 2013; 6: 2121 - 2128

Landaburu I, Gonzalvo MC, Clavero A, Ramirez JP, Yoldi A, Mozas J, Zamora S, Martinez L, Castilla JA
Genetic testing of sperm donors for cystic fibrosis and spinal muscular atrophy: evaluation of clinical utility
European Journal of Obstetrics & Gynecology and Reproductive Biology 2013; 170: 183 - 187

McIntyre K
Gender and survival in cystic fibrosis
Current Opinion in Pulmonary Medicine 2013; 19: 692 - 697

Nazareth D, Walshaw M
Coming of age in cystic fibrosis - transition from paediatric to adult care
Clinical Medicine 2013; 13: 482 - 486

Simmonds NJ
Ageing in Cystic Fibrosis and Long-term Survival
Paediatric Respiratory Reviews 2013; 14:Supl1: 6 - 9

Verbanck S, Paiva M, Paeps E, Schuermans D, Malfroot A, Vincken W, Vanderhelst E
Lung clearance index in adult cystic fibrosis patients: the role of convection-dependent lung units
European Respiratory Journal 2013; 42: 380 - 388

Zhang ZM, Lindstrom MJ, Lai HCJ
Pubertal Height Velocity and Associations with Prepubertal and Adult Heights in Cystic Fibrosis
Journal of Pediatrics 2013; 163: 376 -

Animal Model

Dhooghe B, Noel S, Bouzin C, Behets-Wydemans G, Leal T
Correction of Chloride Transport and Mislocalization of CFTR Protein by Vardenafil in the Gastrointestinal Tract of Cystic Fibrosis Mice
Plos One 2013; 8: 10:e77314

Sellers ZM, Kovacs A, Weinheimer CJ, Best PM
Left ventricular and aortic dysfunction in cystic fibrosis mice
Journal of Cystic Fibrosis 2013; 12: 517 - 524

Stalvey MS., Clines KL., Havasi V, McKibbin CR., Dunn L K., Chung W. Joon, C
Osteoblast CFTR Inactivation Reduces Differentiation and Osteoprotegerin Expression in a Mouse Model of Cystic Fibrosis-Related Bone Disease
Plos One 2013; 8:11.e80098

Teague W. Gerald
Trouble from the Start: Airway Structural Anomalies Present at Birth in the Cystic Fibrosis Piglet
American Journal of Respiratory and Critical Care Medicine 2013; 188: 1434 - 1441


Biswas S, Dubus JC, Reynaud-Gaubert M, Stremler N, Rolain JM
Evaluation of colistin susceptibility in multidrug-resistant clinical isolates from cystic fibrosis, France
European Journal of Clinical Microbiology & Infectious Diseases 2013; 32: 1461 - 1464

Blackledge MS, Worthington RJ, Melander C
Biologically inspired strategies for combating bacterial biofilms
Current Opinion in Pharmacology 2013; 13: 699 - 706

Hirt H, Gorr SU
Antimicrobial Peptide GL13K Is Effective in Reducing Biofilms of Pseudomonas aeruginosa
Antimicrobial Agents and Chemotherapy 2013; 57: 4903 - 4910

Hurley MN, Forrester DL, Smyth AR
Antibiotic adjuvant therapy for pulmonary infection in cystic fibrosis
Cochrane Database of Systematic Reviews 2013; 49: 49 - 56

Knapp L., Rushton L., Stapleton H., Sass A., Stewart S., Amezquita A., McClure P., Mahenthiralingam E., Maillard J. -Y.
The effect of cationic microbicide exposure against Burkholderia cepacia complex (Bcc); the use of Burkholderia lata strain 383 as a model bacterium
Journal of Applied Microbiology 2013; 7: 888 - 891

Mayer-Hamblett N, Saiman L, Lands LC, Anstead M, Rosenfeld M, Kloster M, Fisher L, Ratjen F
Impact of acute antibiotic therapy on the pulmonary exacerbation endpoint in cystic fibrosis clinical trials
Contemporary Clinical Trials 2013; 36: 99 - 105

McCaughey G, Diamond P, Elborn JS, McKevitt M, Tunney MM
Resistance Development of Cystic Fibrosis Respiratory Pathogens When Exposed to Fosfomycin and Tobramycin Alone and in Combination under Aerobic and Anaerobic Conditions
Plos One 2013; 8: 7:e69763

Murugan K., Sangeetha S., Kalyanasundaram V. B., Al-Sohaibani Saleh
In vitro and in silico screening for Andrographis paniculata quorum sensing mimics: new therapeutic leads for cystic fibrosis Pseudomonas aeruginosa biofilms
Plant Omics 2013; 9: 1431 - 1434

Ong HX, Traini D, Salama R, Anderson SD, Daviskas E, Young PM
The Effects of Mannitol on the Transport of Ciprofloxacin across Respiratory Epithelia
Molecular Pharmaceutics 2013; 10: 2915 - 2924

Pessi G, Braunwalder R, Grunau A, Omasits U, Ahrens CH, Eberl L
Response of Burkholderia cenocepacia H111 to Micro-Oxia
Plos One 2013; 8: 9:e72939

Stass H, Weimann B, Nagelschmitz J, Rolinck-Werninghaus C, Staab D
Tolerability and Pharmacokinetic Properties of Ciprofloxacin Dry Powder for Inhalation in Patients With Cystic Fibrosis: A Phase I,Randomized, Dose-Escalation Study
Clinical Therapeutics 2013; 35: 1571 - 1581

Tazi A, Chapron J, Touak G, Longo M, Hubert D, Collobert G, Dusser D, Poyart C, Morand PC
Rapid Emergence of Resistance to Linezolid and Mutator Phenotypes in Staphylococcus aureus Isolates from an Adult Cystic Fibrosis Patient
Antimicrobial Agents and Chemotherapy 2013; 57: 5186 - 5188

Young DC, Zobell JT, Stockmann C, Waters CD, Ampofo K, Sherwin CMT, Spigarelli MG
Optimization of Anti-Pseudomonal Antibiotics for Cystic Fibrosis Pulmonary Exacerbations: V. Aminoglycosides
Pediatric Pulmonology 2013; 48: 1047 - 1061


Tonelli AR
Pulmonary hypertension survival effects and treatment options in cystic fibrosis
Current Opinion in Pulmonary Medicine 2013; 19: 652 - 661

Cell Biology

Almaca J, Faria D, Sousa M, Uliyakina I, Conrad C, Sirianant L, Clarke LA, Martins JP, Santos M, Heriche JK, Huber W,
High-Content siRNA Screen Reveals Global ENaC Regulators and Potential Cystic Fibrosis Therapy Targets
Cell 2013; 154: 1390 - 1400

Buchanan PJ, McNally P, Harvey BJ, Urbach V
Lipoxin A(4)-mediated K-ATP potassium channel activation results in cystic fibrosis airway epithelial repair
American Journal of Physiology-lung Cellular and Molecular Physiology 2013; 305: L193 - L201

Gianotti A, Melani R, Caci E, Sondo E, Ravazzolo R, Galietta LJV, Zegarra-Moran O
Epithelial Sodium Channel Silencing as a Strategy to Correct the Airway Surface Fluid Deficit in Cystic Fibrosis
American Journal of Respiratory Cell and Molecular Biology 2013; 49: 445 - 452

Hobbs CA, Tan CD, Tarran R
Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease?
Journal of Physiology-london 2013; 591: 4377 - 4387

Rymut SM, Harker A, Corey DA, Burgess JD, Sun HT, Clancy JP, Kelley TJ
Reduced microtubule acetylation in cystic fibrosis epithelial cells
American Journal of Physiology-lung Cellular and Molecular Physiology 2013; 305: L419 - L431

Varelogianni G, Hussain R, Strid H, Oliynyk I, Roomans GM, Johannesson M
The effect of ambroxol on chloride transport, CFTR and ENaC in cystic fibrosis airway epithelial cells
Cell Biology International 2013; 37: 1149 - 1156


Billet A, Hanrahan JW
The secret life of CFTR as a calcium-activated chloride channel
Journal of Physiology-london 2013; 591: 5273 - 5278

Cai ZW, Li HY, Chen JH, Sheppard DN
Acute inhibition of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel by thyroid hormones involves multiple mechanisms
American Journal of Physiology-cell Physiology 2013; 305: C817 - C828

Cesaro L, Marin O, Venerando A, Donella-Deane.A, Pinna L A.
Phosphorylation of cystic fibrosis transmembrane conductance regulator (CFTR) serine-511 by the combined action of tyrosine kinases and CK2: the implication of tyrosine-512 and phenylalanine-508
Amino Acids 2013; 45: 1423 - 1429

Cheng J, Guggino W
Ubiquitination and Degradation of CFTR by the E3 Ubiquitin Ligase MARCH2 through Its Association with Adaptor Proteins CAL and STX6
Plos One 2013; 8: 6:e68001

Compain P, Decroocq C, Joosten A, de Sousa J, Rodriguez-Lucena D, Butters TD, Bertrand J, Clement R, Boinot C, Becq F,
Rescue of Functional CFTR Channels in Cystic Fibrosis: A Dramatic Multivalent Effect Using Iminosugar Cluster-Based Correctors
Chembiochem 2013; 14: 2050 - 2058

Conger BT, Zhang SY, Skinner D, Hicks SB, Sorscher EJ, Rowe SM, Woodworth BA
Comparison of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and Ciliary Beat Frequency Activation by the CFTR Modulators Genistein, VRT-532, and UCCF-152 in Primary Sinonasal Epithelial Cultures
Jama Otolaryngology-head & Neck Surgery 2013; 139: 822 - 827

Conner GE, Ivonnet P, Gelin M, Whitney P, Salathe M
H2O2 Stimulates Cystic Fibrosis Transmembrane Conductance Regulator through an Autocrine Prostaglandin Pathway, Using Multidrug-Resistant Protein-4
American Journal of Respiratory Cell and Molecular Biology 2013; 49: 672 - 679

Dawson JE, Farber PJ, Forman-Kay JD
Allosteric Coupling between the Intracellular Coupling Helix 4 and Regulatory Sites of the First Nucleotide-binding Domain of CFTR
Plos One 2013; 8: 9:e74347

Farinha CM, King-Underwood J, Sousa M, Correia AR, Henriques BJ, Roxo-Rosa M, Da Paula AC, Williams J, Hirst S, Gomes
Revertants, Low Temperature, and Correctors Reveal the Mechanism of F508del-CFTR Rescue by VX-809 and Suggest Multiple Agents for Full Correction
Chemistry & Biology 2013; 20: 943 - 955

Gholami K, Muniandy S, Salleh N
In-Vivo Functional Study on the Involvement of CFTR, SLC26A6, NHE-1 and CA Isoenzymes II and XII in Uterine Fluid pH, Volume and Electrolyte Regulation in Rats under Different Sex-Steroid Influence
International Journal of Medical Sciences 2013; 10: 1121 - 1134

Jakab RL, Collaco AM, Ameen NA
Characterization of CFTR High Expresser cells in the intestine
American Journal of Physiology-gastrointestinal and Liver Physiology 2013; 305: G453 - G465

Kim S, Beyer BA, Lewis C, Nadel JA
Normal CFTR Inhibits Epidermal Growth Factor Receptor-Dependent Pro-Inflammatory Chemokine Production in Human Airway Epithelial Cells
Plos One 2013; 8: 8:e72981

LeSimple P, Goepp J, Palmer ML, Fahrenkrug SC, O'Grady SM, Ferraro P, Robert R, Hanrahan JW
Cystic Fibrosis Transmembrane Conductance Regulator Is Expressed in Mucin Granules from Calu-3 and Primary Human Airway Epithelial Cells
American Journal of Respiratory Cell and Molecular Biology 2013; 49: 511 - 516

Loo TW, Bartlett MC, Clarke DM
Bithiazole Correctors Rescue CFTR Mutants by Two Different Mechanisms
Biochemistry 2013; 52: 5161 - 5163

Maitra R, Sivashanmugam P, Warner K
A Rapid Membrane Potential Assay to Monitor CFTR Function and Inhibition
Journal of Biomolecular Screening 2013; 18: 1132 - 1137

Martin C, Coolen N, Wu Y, Thevenot G, Touqui L, Pruliere-Escabasse V, Papon J-F, Coste, A, Escudier E, Dusser DJ, Fajac I, Burgel, P-R
CFTR dysfunction induces vascular endothelial growth factor synthesis in airway epithelium
European Respiratory Journal 2013; 42: 1553 - 1562

Montiel-Gonzalez MF, Vallecillo-Viejo I, Yudowski GA, Rosenthal JJC
Correction of mutations within the cystic fibrosis transmembrane conductance regulator by site-directed RNA editing
Proceedings of the National Academy of Sciences of the United States 2013; 110: 18285 - 1829

Nadel Jay A.
The CFTR and EGFR relationship in airway vascular growth, and its importance in cystic fibrosis
European Respiratory Journal 2013; 42: 1545 - 1552

Odolczyk N, Fritsch J, Norez C, Servel N, da Cunha MF, Bitam S, Kupniewska A, Wiszniewski L, Colas J, Tarnowski K,
Discovery of novel potent Delta F508-CFTR correctors that target the nucleotide binding domain
Embo Molecular Medicine 2013; 5: 1484 - 1501

Rahman KS, Cui GY, Harvey SC, McCarty NA
Modeling the Conformational Changes Underlying Channel Opening in CFTR
Plos One 2013; 8: 9:e74574

Raju S. V, Jackson P L., Courville C A., McNicholas C M., Sloane PA., Sabbatini G, Tidwell S, Tang LP, Liu B, Fortenberry JA., Jones CW.,Boydston JA., Clancy JP., Bowen LE., Accurso FJ., Blalock J E, Dransfield MT.,Rowe, SM.
Cigarette Smoke Induces Systemic Defects in Cystic Fibrosis Transmembrane Conductance Regulator Function
American Journal of Respiratory and Critical Care Medicine 2013; 188: 1321 - 1330

Ramachandran S, Karp PH, Osterhaus SR, Jiang P, Wohlford-Lenane C, Lennox KA, Jacobi AM, Praekh K, Rose SD, Behlke
Post-Transcriptional Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Expression and Function by MicroRNAs
American Journal of Respiratory Cell and Molecular Biology 2013; 49: 544 - 551

Sosnay PR, Siklosi KR, Van Goor F, Kaniecki K, Yu HH, Sharma N, Ramalho AS, Amaral MD, Dorfman R, Zielenski J,
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene
Nature Genetics 2013; 45: 1160 - U292

Venerando A, Franchin C, Cant N, Cozza G, Pagano MA, Tosoni K, Al-Zahrani A, Arrigoni G, Ford RC, Mehta A, Pinna LA
Detection of Phospho- Sites Generated by Protein Kinase CK2 in CFTR: Mechanistic Aspects of Thr1471 Phosphorylation
Plos One 2013; 8: 9:e74232

Wine JJ, Char JE, Chen J, Cho HJ, Dunn C, Frisbee E, Joo NS, Milla C, Modlin SE, Park IH, Thomas EAC, Tran KV, Verma
In Vivo Readout of CFTR Function: Ratiometric Measurement of CFTR-Dependent Secretion by Individual, Identifiable Human Sweat
Plos One 2013; 8: 10:e77114

Yi S, Pierucci-Alves F, Schultz BD
Transforming growth factor-beta 1 impairs CFTR-mediated anion secretion across cultured porcine vas deferens epithelial monolayer via the p38 MAPK pathway
American Journal of Physiology-cell Physiology 2013; 305: C867 - C876


Doorn CS, De Boo DW, Weersink EJM, Delden OM, Reekers JA, van Lienden KP
Permanent Cortical Blindness After Bronchial Artery Embolization
Cardiovascular and Interventional Radiology 2013; 36: 1686 - 1689

Doull I
Cystic Fibrosis Papers of the Year 2012
Paediatric Respiratory Reviews 2013; 14:Supl1: 28 - 30

Galietta LJV
Managing the Underlying Cause of Cystic Fibrosis: A Future Role for Potentiators and Correctors
Pediatric Drugs 2013; 15: 393 - 402

Havermans T, Colpaert K, De Boeck K, Dupont L, Abbott J
Pain in CF: Review of the literature
Journal of Cystic Fibrosis 2013; 12: 423 - 430

Michl RK, Mentzel HJ, Gerber A, Beck JF, Mainz JG
A Patient Nicknamed 'Saline': Atypical Course with Cystic Fibrosis
Klinische Padiatrie 2013; 225: 288 - 289

Towle D, Callan DA, Farrel PA, Egan ME, Murray TS
Baby bottle steam sterilizers disinfect home nebulizers inoculated with bacterial respiratory pathogens
Journal of Cystic Fibrosis 2013; 12: 512 - 516

Wilder-Smith E. P.
Aquagenic wrinkling of the palms is due to vasoconstriction of palmar skin vasculature
Medical Hypotheses 2013; 81: 963 - 965


Blackman SM, Commander CW, Watson C, Arcara KM, Strug LJ, Stonebraker JR, Wright FA, Rommens JM, Sun L, Pace
Genetic Modifiers of Cystic Fibrosis-Related Diabetes
Diabetes 2013; 62: 3627 - 3635

Bridges N
Diabetes in Cystic Fibrosis
Paediatric Respiratory Reviews 2013; 14:Supl1: 16 - 18

Hirsch Irl B., Janci Mary M., Goss Christopher H., Aitken Moira L.
Hypoglycemia in Adults With Cystic Fibrosis During Oral Glucose Tolerance Testing
Diabetes Care 2013; 20: 99A - 107A

Kern AS, Prestridge AL
Improving Screening for Cystic Fibrosis-Related Diabetes at a Pediatric Cystic Fibrosis Program
Pediatrics 2013; 132: E512 - E518

Konrad K, Scheuing N, Badenhoop K, Borkenstein MH, Gohlke B, Schofl C, Seufert J, Thon A, Holl RW
Cystic fibrosis-related diabetes compared with type 1 and type 2 diabetes in adults
Diabetes-metabolism Research and Reviews 2013; 29: 568 - 575

Monge ME, Perez JJ, Dwivedi P, Zhou MS, McCarty NA, Stecenko AA, Fernandez FM
Ion mobility and liquid chromatography/mass spectrometry strategies for exhaled breath condensate glucose quantitation in cystic fibrosis
Rapid Communications in Mass Spectrometry 2013; 27: 2263 - 2271

Neu A., Beyer P., Buerger-Buesing J., Danne T., Etspueler J., Heidtmann B., Holl R. W., Karges B., Kiess W., Knerr I., Kordonouri O. Lange, K.,Lepler R., Marg W.,Naeke A., Petersen M.,Podeswik, A.,Stachow, R.,von Sengbusch, S.,Wagner V.,Ziegler R.,Holterhus P.M.
Diagnosis, Treatment and Monitoring of Diabetes mellitus in Childhood and Adolescence
Diabetologie Und Stoffwechsel 2013; 8: S189 – S199

Valour F, Brault C, Abbas-Chorfa F, Martin C, Kessler L, Kanaan R, Mosnier-Pudar H, Coltey B, Nove-Josserand R, Durupt
Outcome of Cystic Fibrosis-Related Diabetes Two Years after Lung Transplantation
Respiration 2013; 86: 32 - 38


Bronsveld I, Vermeulen F, Sands D, Leal T, Leonard A, Melotti P, Yaakov Y, de Nooijer R, De Boeck K, Sermet I, Wilschanski
Influence of perfusate temperature on nasal potential difference
European Respiratory Journal 2013; 42: 389 - 393

Paff T, van der Schee MP, Daniels JMA, Pals G, Postmus PE, Sterk PJ, Haarman EG
Exhaled molecular profiles in the assessment of cystic fibrosis and primary ciliary dyskinesia
Journal of Cystic Fibrosis 2013; 12: 454 - 460

Valiulis A, Skurvydiene I, Miseviciene V, Kasnauskiene J, Vaideliene L, Utkus A
Relevance of Nasal Potential Difference in Diagnosis of Cystic Fibrosis Among Children
Medicina-Lithuania 2013; 49: 185 - 190

Wing D, Prausnitz MR, Buono MJ
Skin pretreatment with microneedles prior to pilocarpine iontophoresis increases sweat production
Clinical Physiology and Functional Imaging 2013; 33: 436 - 440


Frentescu L, Budisan L, Benga G
Acta Endocrinologica-bucharest 2013; 9: 349 - 359

Krenkova P, Piskackova T, Holubova A, Balascakova M, Krulisova V, Camajova J, Turnovec M, Libik M, Norambuena P,
Distribution of CFTR mutations in the Czech population: Positive impact of integrated clinical and laboratory expertise, detection of novel/de novo alleles and relevance for related/derived populations
Journal of Cystic Fibrosis 2013; 12: 532 - 537


Fitzgerald NM, Fitzgerald DA, Lands L, Selvadurai H
Diffusion capacity in children: what happens with exercise?
Paediatric Respiratory Reviews 2013; 14:Supl1: 190 - 194

Happ MB, Hoffman LA, DiVirgilio D, Higgins LW, Orenstein DM
Parent and Child Perceptions of a Self-Regulated, Home-Based Exercise Program for Children With Cystic Fibrosis
Nursing Research 2013; 62: 305 - 314

Houston Brian W., Mills Nicola, Solis-Moya Arturo
Inspiratory muscle training for cystic fibrosis
Cochrane Database of Systematic Reviews 2013; 13:

Martin Clemence, Chapron Jeanne, Hubert Dominique, Kanaan Reem, Honore Isabelle, Paillasseur Jean-Louis, Aubourg
Prognostic value of six minute walk test in cystic fibrosis adults.
Respiratory Medicine 2013; 107: 1888 - 94

Ziegler B, Fernandes AK, Sanches PRS, Silva DP, Thome PRO, Dalcin PTR
Dyspnea perception in cystic fibrosis patients
Brazilian Journal of Medical and Biological Research 2013; 46: 897 - 903


Borowitz D, Gelfond D
Intestinal complications of cystic fibrosis
Current Opinion in Pulmonary Medicine 2013; 19: 676 - 680

De Lisle RC, Borowitz D
The Cystic Fibrosis Intestine
Cold Spring Harbor Perspectives in Medicine 2013; 3: 9;a009753

Friedrich-Rust M, Schlueter N, Smaczny C, Eickmeier O, Rosewich M, Feifel K, Herrmann E, Poynard T, Gleiber W, Lais C,
Non-invasive measurement of liver and pancreas fibrosis in patients with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 431 - 439

Nagakumar P
Pseudomembranous Colitis in Cystic Fibrosis
Paediatric Respiratory Reviews 2013; 14:Supl1: 26 - 27

Shah N, Tan HL, Sebire N, Suri R, Leuven K
The Role of Endoscopy and Biopsy in the Management of Severe Gastrointestinal Disease in Cystic Fibrosis Patients
Pediatric Pulmonology 2013; 48: 1181 - 1189

Gene Therapy

Alton EWFW, Boyd AC, Cheng SH, Cunningham S, Davies JC, Gill DR, Griesenbach U, Higgins T, Hyde SC, Innes JA,
A randomised, double-blind, placebo-controlled phase IIB clinical trial of repeated application of gene therapy in patients with cystic fibrosis
Thorax 2013; 68: 1075 - 1077

Griesenbach U, Alton EWFW
Moving forward: cystic fibrosis gene therapy
Human Molecular Genetics 2013; 22: R52 - R58

Manunta MDI, McAnulty RJ, McDowell A, Jin J, Ridout D, Fleming J, Bottoms SE, Tossici-Bolt L, Laurent GJ, Biassoni L,
Airway Deposition of Nebulized Gene Delivery Nanocomplexes Monitored by Radioimaging Agents
American Journal of Respiratory Cell and Molecular Biology 2013; 49: 471 - 480


Abou Tayoun AN, Tunkey CD, Pugh TJ, Ross T, Shah M, Lee CC, Harkins TT, Wells WA, Tafe LJ, Amos CI, Tsongalis GJ
A Comprehensive Assay for CFTR Mutational Analysis Using Next-Generation Sequencing
Clinical Chemistry 2013; 59: 1481 - 1488

Castellani C
CFTR2: How will it help care?
Paediatric Respiratory Reviews 2013; 14:Supl1: 2 - 5

Ong T, Ramsey BW
Modifying disease in cystic fibrosis: current and future therapies on the horizon
Current Opinion in Pulmonary Medicine 2013; 19: 645 - 651

Prach L, Koepke R, Kharrazi M, Keiles S, Salinas DB, Reyes MC, Pian M, Opsimos H, Otsuka KN, Hardy KA, Milla CE,
Novel CFTR Variants Identified during the First 3 Years of Cystic Fibrosis Newborn Screening in California
Journal of Molecular Diagnostics 2013; 15: 710 - 722

van Meegen MA, Terheggen SWJ, Koymans KJ, Vijftigschild LAW, Dekkers JF, van der Ent CK, Beekman JM
CFTR-mutation Specific applications of CFTR-directed monoclonal antibodies
Journal of Cystic Fibrosis 2013; 12: 487 - 496

Growth & Development

Paccou J, Fardellone P, Cortet B
Cystic fibrosis-related bone disease
Current Opinion in Pulmonary Medicine 2013; 19: 681 - 686

Immunology & Inflammation

Amali M., Sullivan A., Yaseen F. S., Farrell J., Faulkner L., Whitaker P., Peckham D., Park B. K., Naisbitt D. J.
Characterization of the B cell response in piperacillin hypersensitive patients with cystic fibrosis
Immunology 2013; 140: 192

Choh LC, Ong GH, Vellasamy KM, Kalaiselvam K, Kang WT, Al-Maleki AR, Mariappan V, Vadivelu J
Burkholderia vaccines: are we moving forward?
Frontiers in Cellular and Infection Microbiology 2013; 3: Article ID: UNSP

Cifani N, Pompili B, Anile M, Patella M, Diso D, Venuta F, Cimino G, Quattrucci S, Di Domenico EG, Ascenzioni F, Del Porto
Reactive-Oxygen-Species-Mediated P. aeruginosa Killing Is Functional in Human Cystic Fibrosis Macrophages
Plos One 2013; 8: 8:e71717

Engelen MPKJ, Com G, Luiking YC, Deutz NEP
Stimulated Nitric Oxide Production and Arginine Deficiency in Children with Cystic Fibrosis with Nutritional Failure
Journal of Pediatrics 2013; 163: 369 - +

Gifford AM, Chalmers J D
The role of neutrophils in cystic fibrosis.
Current Opinion in Hematology 2014; 21: 16 - 22

Grasemann H, Pencharz PB
Arginine Metabolism in Patients with Cystic Fibrosis
Journal of Pediatrics 2013; 163: 317 - 319

McGivern TJP, Molloy K, Bahar M, McElvaney NG, Moran N, Kerrigan SW
A platelet dense-granule secretion defect may lead to a muted inflammatory cell mobilization response in cystic fibrosis patients
Journal of Thrombosis and Haemostasis 2013; 11: 1939 - 1942

O'Brien CE, Price ET
The Blood Neutrophil to Lymphocyte Ratio Correlates with Clinical Status in Children with Cystic Fibrosis: A Retrospective Study
Plos One 2013; 8: 10:e77420

Reeves EP, Banville N, Ryan DM, O'Reilly N, Bergin DA, Pohl K, Molloy K, McElvaney OJ, Alsaleh K, Aljorfi A, Kandalaft
Intracellular Secretory Leukoprotease Inhibitor Modulates Inositol 1,4,5-Triphosphate Generation and Exerts an Anti-Inflammatory Effect onNeutrophils of Individuals with Cystic Fibrosis and Chronic Obstructive Pulmonary Disease
Biomed Research International 2013; 37: 433 - 442

Simonin-Le Jeune K, Le Jeune A, Jouneau S, Belleguic C, Roux PF, Jaguin M, Dimanche-Boitre MT, Lecureur V, Leclercq C,
Impaired Functions of Macrophage from Cystic Fibrosis Patients: CD11b, TLR-5 Decrease and sCD14, Inflammatory Cytokines Increase
Plos One 2013; 8: 9e75667

Van de Weert-van Leeuwen PB, Van Meegen MA, Speirs JJ, Pals DJ, Rooijakkers SHM, Van der Ent CK, Terheggen-Lagro
Optimal Complement-Mediated Phagocytosis of Pseudomonas aeruginosa by Monocytes Is Cystic Fibrosis Transmembrane Conductance Regulator-Dependent
American Journal of Respiratory Cell and Molecular Biology 2013; 49: 463 - 470


Alby K, Gilligan PH, Miller MB
Comparison of Matrix-Assisted Laser Desorption Ionization-Time of Flight (MALDI-TOF) Mass Spectrometry Platforms for the Identification of Gram-Negative Rods from Patients with Cystic Fibrosis
Journal of Clinical Microbiology 2013; 51: 3852 - 3854

Ballok AE, O'Toole GA
Pouring Salt on a Wound: Pseudomonas aeruginosa Virulence Factors Alter Na+ and Cl- Flux in the Lung
Journal of Bacteriology 2013; 195: 4013 - 4019

Baxter CG, Dunn G, Jones AM, Webb K, Gore R, Richardson MD, Denning DW
Novel immunologic classification of aspergillosis in adult cystic fibrosis
Journal of Allergy and Clinical Immunology 2013; 132: 560 - +

Berkhout MC, Rijntjes E, el Bouazzaoui LH, Fokkens WJ, Brimicombe RW, Heijerman HGM
Importance of bacteriology in upper airways of patients with Cystic Fibrosis
Journal of Cystic Fibrosis 2013; 12: 525 - 529

Billings N, Millan MR, Caldara M, Rusconi R, Tarasova Y, Stocker R, Ribbeck K
The Extracellular Matrix Component Psl Provides Fast-Acting Antibiotic Defense in Pseudomonas aeruginosa Biofilms
Plos Pathogens 2013; 9: 8:e009720

Binder AM, Adjemian J, Olivier KN, Prevots DR
Epidemiology of Nontuberculous Mycobacterial Infections and Associated Chronic Macrolide Use among Persons with Cystic Fibrosis
American Journal of Respiratory and Critical Care Medicine 2013; 188: 807 - 812

Blus-Kadosh I, Zilka A, Yerushalmi G, Banin E
The Effect of pstS and phoB on Quorum Sensing and Swarming Motility in Pseudomonas aeruginosa
Plos One 2013; 8: 9:e74444

Bottger EC
Transmission of M abscessus in patients with cystic fibrosis
Lancet 2013; 382: 503 - 504

Bryant JM, Grogono DM, Parkhill J, Floto RA
Transmission of M abscessus in patients with cystic fibrosis reply
Lancet 2013; 382: 504

Costaglioli P, Barthe C, Fayon M, Christoflour N, Bui S, Derlich L, Domblides P,
Selection of Pseudomonas aeruginosa reference genes for RT-qPCR analysis from sputum of cystic fibrosis patients
Molecular and Cellular Probes 2014; 28: 10 - 12

da Silva LVRF, Ferreira FD, Reis FJC, de Britto MCA, Levy CE, Clark O, Ribeiro JD
Pseudomonas aeruginosa infection in patients with cystic fibrosis: scientific evidence regarding clinical impact, diagnosis, and treatment
Jornal Brasileiro de Pneumologia 2013; 39: 495 - 512

Damron FH, Barbier M, McKenney ES, Schurr MJ, Goldberg JB
Genes Required for and Effects of Alginate Overproduction Induced by Growth of Pseudomonas aeruginosa on Pseudomonas Isolation Agar Supplemented with Ammonium Metavanadate
Journal of Bacteriology 2013; 195: 4020 - 4036

Dedeckova K, Kalferstova L, Strnad H, Vavrova J, Drevinek P
Novel diagnostic PCR assay for Burkholderia cenocepacia epidemic strain ST32 and its utility in monitoring infection in cystic fibrosis patients
Journal of Cystic Fibrosis 2013; 12: 475 - 481

Di Lorenzo F, Sturiale L, Palmigiano A, Lembo-Fazio L, Paciello I, Coutinho CP, Sa-Correia I, Bernardini M, Lanzetta R,
Chemistry and Biology of the Potent Endotoxin from a Burkholderia dolosa Clinical Isolate from a Cystic Fibrosis Patient
Chembiochem 2013; 14: 1105 - 1115

Dias MBS, Cavassin LGT, Stempliuk V, Xavier LS, Lobo RD, Sampaio JLM, Pignatari AC, Borrasca VL, Bierrenbach AL,
Multi-institutional outbreak of Burkholderia cepacia complex associated with contaminated mannitol solution prepared in compounding pharmacy
American Journal of Infection Control 2013; 41: 1038 - 1042

Diene SM, L'homme T, Bellulo S, Stremler N, Dubus JC, Mely L, Leroy S, Degand N, Rolain JM
ISPa46, a novel insertion sequence in the oprD porin gene of an imipenem-resistant Pseudomonas aeruginosa isolate from a cystic fibrosis patient in Marseille, France
International Journal of Antimicrobial Agents 2013; 42: 268 - 271

Fang H, Toyofuku M, Kiyokawa T, Ichihashi A, Tateda K, Nomura N
The Impact of Anaerobiosis on Strain-Dependent Phenotypic Variations in Pseudomonas aeruginosa
Bioscience Biotechnology and Biochemistry 2013; 77: 1747 - 1752

Fehlberg LCC, Andrade LHS, Assis DM, Pereira RHV, Gales AC, Marques EA
Performance of MALDI-ToF MS for species identification of Burkholderia cepacia complex clinical isolates
Diagnostic Microbiology and Infectious Disease 2013; 77: 126 - 128

Flight WG, Bright-Thomas RJ, Tilston P, Mutton KJ, Guiver M, Webb AK, Jones AM
Chronic Rhinovirus Infection in an Adult with Cystic Fibrosis
Journal of Clinical Microbiology 2013; 51: 3893 - 3896

Friman VP, Ghoul M, Molin S, Johansen HK, Buckling A
Pseudomonas aeruginosa Adaptation to Lungs of Cystic Fibrosis Patients Leads to Lowered Resistance to Phage and Protist Enemies
Plos One 2013; 8: 9:e75380

Ganesan S, Sajjan US
Host evasion by Burkholderia cenocepacia
Frontiers in Cellular and Infection Microbiology 2012; 1: Article ID:UNSP 2

Garnett JP, Gray MA, Tarran R, Brodlie M, Ward C, Baker EH, Baines DL
Elevated Paracellular Glucose Flux across Cystic Fibrosis Airway Epithelial Monolayers Is an Important Factor for Pseudomonas aeruginosa
Plos One 2013; 8: 10:e76283

Gaspar MC, Couet W, Olivier JC, Pais AACC, Sousa JJS
Pseudomonas aeruginosa infection in cystic fibrosis lung disease and new perspectives of treatment: a review
European Journal of Clinical Microbiology & Infectious Diseases 2013; 32: 1231 - 1252

Gilchrist FJ, Sims H, Alcock A, Jones AM, Bright-Thomas RJ, Smith D, Spanel P, Webb AK, Lenney W
Is Hydrogen Cyanide a Marker of Burkholderia cepacia Complex?
Journal of Clinical Microbiology 2013; 51: 3849 - 3851

Hao YH, Kuang ZH, Xu Y, Walling BE, Lau GW
Pyocyanin-induced mucin production is associated with redox modification of FOXA2
Respiratory Research 2013; 14: Arti No 84

Hubert D, Reglier-Poupet H, Sermet-Gaudelus I, Ferroni A, Le Bourgeois M, Burgel PR, Serreau R, Dusser D, Poyart C, Coste
Association between Staphylococcus aureus alone or combined with Pseudomonas aeruginosa and the clinical condition of patients withcystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 497 - 503

Huse HK, Kwon T, Zlosnik JEA, Speert DP, Marcotte EM, Whiteley M
Pseudomonas aeruginosa Enhances Production of a Non-Alginate Exopolysaccharide during Long-Term Colonization of the Cystic Fibrosis Lung.
Plos One 2013; 8: 12:e82621

Jakobsen TH, Bjarnsholt T, Jensen PO, Givskov M, Hoiby N
Targeting quorum sensing in Pseudomonas aeruginosa biofilms: current and emerging inhibitors
Future Microbiology 2013; 8: 901 - 921

Jakobsen TH, Hansen MA, Jensen PO, Hansen L, Riber L, Cockburn A, Kolpen M, Hansen CR, Ridderberg W, Eickhardt S,
Complete Genome Sequence of the Cystic Fibrosis Pathogen Achromobacter xylosoxidans NH44784-1996 Complies with Important Pathogenic Phenotypes
Plos One 2013; 8: 7:e68484

Junkins RD, Shen A, Rosen K, McCormick C, Lin TJ
Autophagy Enhances Bacterial Clearance during P. aeruginosa Lung Infection
Plos One 2013; 8: 8:e72263
Karaman M, Firinci F, Karaman O, Uzuner N, Bahar IH
Long-term oropharyngeal colonization by C. albicans in children with cystic fibrosis
Yeast 2013; 30: 429 - 436

Krasny L, Hynek R, Hochel I
Identification of bacteria using mass spectrometry techniques
International Journal of Mass Spectrometry 2013; 353: 67 - 79

Labbe F, Babchia S, Evreux F, Chenal P
Isolation of Geosmithia argillacea in a cystic fibrosis patient
Journal de Mycologie Medicale 2013; 23: 176 - 178

Leung JM, Olivier KN
Nontuberculous mycobacteria: the changing epidemiology and treatment challenges in cystic fibrosis
Current Opinion in Pulmonary Medicine 2013; 19: 662 - 669

Mayer-Hamblett N, Rosenfeld M, Treggiari MM, Konstan MW, Retsch-Bogart G, Morgan W, Wagener J, Gibson RL, Khan U,
Standard care versus protocol based therapy for new onset Pseudomonas aeruginosa in cystic fibrosis
Pediatric Pulmonology 2013; 48: 943 - 953

Middleton PG, Chen SCA, Meyer W
Fungal infections and treatment in cystic fibrosis
Current Opinion in Pulmonary Medicine 2013; 19: 670 - 675

Oliver KE, Silo-Suh L
Impact of D-amino acid dehydrogenase on virulence factor production by a Pseudomonas aeruginosa
Canadian Journal of Microbiology 2013; 59: 598 - 603

Perez LRR, Dias CAG
Evaluation of the Gram stain of sputa from cystic fibrosis patients to predict the presence of Staphylococcus aureus
Diagnostic Microbiology and Infectious Disease 2013; 77: 99 - 105

Psoter KJ, De Roos AJ, Wakefield J, Mayer J, Rosenfeld M
Season is associated with Pseudomonas aeruginosa acquisition in young children with cystic fibrosis
Clinical Microbiology and Infection 2013; 19: E483 - E489

Russell GK, Gadhok R, Simmonds NJ
The destructive combination of Scediosporium apiosperum lung disease and exuberant inflammation in cystic fibrosis
Paediatric Respiratory Reviews 2013; 14: 22 - 25

Scott Nichollas E., Hare Nathan J., White Melanie Y., Manos Jim, Cordwell Stuart J.
Secretome of Transmissible Pseudomonas aeruginosa AES-1R Grown in a Cystic Fibrosis Lung-Like Environment
Journal of Proteome Research 2013; 26: 700 - 709

Seddon P, Fidler K, Raman S, Wyatt H, Ruiz G, Elston C, Perrin F, Gyi K, Bilton D, Drobniewski F., Newport M.
Prevalence of Nontuberculous Mycobacteria in Cystic Fibrosis Clinics, United Kingdom, 2009
Emerging Infectious Diseases 2013; 19: 1128-1130

Semler DD, Lyunch KH, Dennis JJ
The promise of bacteriophage therapy for Burkholderia cepacia complex respiratory infections
Frontiers in Cellular and Infection Microbiology 2012; 1: Article ID:UNSP 2

Shin HS, Lee JH, Paek SH, Jung YW, Ha UH
Pseudomonas aeruginosa-dependent upregulation of TLR2 influences host responses to a secondary Staphylococcus aureus infection
Pathogens and Disease 2013; 69: 149 - 156
Skurnik D, Roux D, Cattoir V, Danilchanka O, Lu X, Yoder-Himes DR., Han K, Guillard T, Jiang D, Gaultier C.,Guerin, F., Aschard, H., Leclercq R. Mekalanos JJ., Lory S., Pier GB
Enhanced in vivo fitness of carbapenem-resistant oprD mutants of Pseudomonas aeruginosa revealed through high-throughput sequencing
Proceedings of the National Academy of Sciences of the United States 2013; 110: 20747 - 20752

Smith David, Spanel Patrik, Gilchrist Francis J., Lenney Warren
Hydrogen cyanide, a volatile biomarker of Pseudomonas aeruginosa infection
Journal of Breath Research 2013; 46: 859 - 873

Strateva T, Petrova G, Stratev A, Perenovska P, Mitov I
Etiology of bronchopulmonary infections in Bulgarian cystic fibrosis patients
Brazilian Journal of Infectious Diseases 2013; 17: 617 - 618

Thogersen JCh, Morup M, Damkiaer S, Molin S, Jelsbak L
Archetypal analysis of diverse Pseudomonas aeruginosa transcriptomes reveals adaptation in cystic fibrosis airways
Bmc Bioinformatics 2013; 14: Art No 279

Valentine CD, Anderson MO, Papa FR, Haggie PM
X-Box Binding Protein 1 (XBP1s) Is a Critical Determinant of Pseudomonas aeruginosa Homoserine Lactone-Mediated Apoptosis
Plos Pathogens 2013; 9: 8:e1003576

Waters V, Atenafu EG, Lu A, Yau Y, Tullis E, Ratjen F
Chronic Stenotrophomonas maltophilia infection and mortality or lung transplantation in cystic fibrosis patients
Journal of Cystic Fibrosis 2013; 12: 482 - 486

Wong JK, Ranganathan SC, Hart E
Staphylococcus aureus in Early Cystic Fibrosis Lung Disease
Pediatric Pulmonology 2013; 48: 1151 - 1159

Xu Y, Duan CH, Kuang ZZ, Hao YH, Jeffries JL, Lau GW
Pseudomonas aeruginosa Pyocyanin Activates NRF2-ARE-Mediated Transcriptional Response via the ROS-EGFR-PI3K-AKT/MEK-ERK MAP Kinase Signaling in Pulmonary Epithelial Cells
Plos One 2013; 8: 8:e1003576

Yin YS, Withers TR, Wang X, Yu HWD
Evidence for Sigma Factor Competition in the Regulation of Alginate Production by Pseudomonas aeruginosa
Plos One 2013; 8:

Zhu JJ, Jimenez-Diaz J, Bean HD, Daphtary NA, Aliyeva MI, Lundblad LKA, Hill JE
Robust detection of P. aeruginosa and S. aureus acute lung infections by secondary electrospray ionization-mass spectrometry (SESI-MS) breathprinting: from initial infection to clearance
Journal of Breath Research 2013; 7:


Brei C, Simon A, Krawinkel MB, Naehrlich L
Individualized vitamin A supplementation for patients with cystic fibrosis
Clinical Nutrition 2013; 32: 805 - 810

Culhane S, George C, Pearo B, Spoede E
Malnutrition in Cystic Fibrosis: A Review
Nutrition in Clinical Practice 2013; 28: 676 - 683

Del Ciampo IRL, Sawamura R, Fernandes MIM
Cystic Fibrosis: From Protein-Energy Malnutrition to Obesity with Dyslipidemia
Iranian Journal of Pediatrics 2013; 23: 605 - 606
Engelen MPKJ, Com G, Wolfe RR, Deutz NEP
Dietary essential amino acids are highly anabolic in pediatric patients with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 445 - 453

Leggieri E, De Biase RV, Savi D, Zullo S, Halili I, Quattrucci S
Clinical effects of DHA supplementation in pediatric patients with cystic fibrosis
Minerva Pediatrica 2013; 65: 389 - 398


Button BM, Button B
Structure and Function of the Mucus Clearance System of the Lung
Cold Spring Harbor Perspectives in Medicine 2013; 3: 8:a009720

Hafen GM, Kernen Y, De Halleux QM
Time invested in the global respiratory care of cystic fibrosis paediatrics patients
Clinical Respiratory Journal 2013; 7: 338 - 341

Main E
What is the best airway clearance technique in cystic fibrosis?
Paediatric Respiratory Reviews 2013; 14:Supl1: 10 - 12

Volsko TA
Airway Clearance Therapy: Finding the Evidence
Respiratory Care 2013; 58: 1669 - 1678

Warnock L, Gates A, van der Schans CP
Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis
Cochrane Database of Systematic Reviews 2013; 164: E313 - E317


Afonso SBC, Mitre RMD
Difficult news: meanings attributed by family members of children with cystic fibrosis
Ciencia & Saude Coletiva 2013; 18: 2605 - 2613

Baker HM, Brown RL, Tluczek A
Development and validation of a cystic fibrosis genetic knowledge questionnaire within the general population of the United States
Journal of Cystic Fibrosis 2013; 12: 504 - 511

Cies JJ, Varlotta L
Clinical Pharmacist Impact on Care, Length of Stay, and Cost in Pediatric Cystic Fibrosis (CF) Patients
Pediatric Pulmonology 2013; 48: 1190 - 1194

Clisby N, Shaw S, Cormack M
Psychological impact of working with patients with cystic fibrosis at end-of-life, pre-transplant stage
Palliative & Supportive Care 2013; 11: 111 - 121

Dill EJ, Dawson R, Sellers DE, Robinson WM, Sawicki GS
Longitudinal Trends in Health-Related Quality of Life in Adults With Cystic Fibrosis
Chest 2013; 144: 981 - 989

Maruotti GM, Sarno L, Simioli S, Castaldo G, Martinelli P
Prenatal screening and counseling for genetic disorders
Journal of Maternal-fetal & Neonatal Medicine 2013; 26: 68 - 71

McDonald CM, Haberman D, Brown N
Self-efficacy: Empowering parents of children with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 538 - 543

Platten MJ, Newman E, Quayle E
Self-Esteem and Its Relationship to Mental Health and Quality of Life in Adults with Cystic Fibrosis
Journal of Clinical Psychology in Medical Settings 2013; 20: 392 - 399

Stick S, Tiddens H, Aurora P, Gustafsson P, Ranganathan S, Robinson P, Rosenfeld M, Sly P, Ratjen F
Early intervention studies in infants and preschool children with cystic fibrosis: are we ready?
European Respiratory Journal 2013; 42: 527 - 538

Taylor-Robinson DC, Smyth R, Diggle PJ, Whitehead M
A Longitudinal Study of the Impact of Social Deprivation and Disease Severity on Employment Status in the UK Cystic Fibrosis Population
Plos One 2013; 8: 8: e73322

Tepper LA, Utens EMWJ, Caudri D, Bos AC, Gonzalez-Graniel K, Duivenvoorden HJ, van der Weil ECW, Quittner AL,
Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis
European Respiratory Journal 2013; 42: 371 - 379


Armstrong D
The use of continuous positive airway pressure or non-invasive ventilation as forms of respiratory support in children with cystic fibrosis
Paediatric Respiratory Reviews 2013; 14: 19 - 21

Bakker EM, Borsboom GJJM, van der Wiel-Kooij EC, Caudri D, Rosenfeld M, Tiddens HAWM
Small Airway Involvement in Cystic Fibrosis Lung Disease: Routine Spirometry as an Early and Sensitive Marker
Pediatric Pulmonology 2013; 48: 1081 - 1088

Beer H, Southern KW, Swift AC
Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis
Cochrane Database of Systematic Reviews 2013; 3: 19515 - 1952

Castanos C
Risk factors for bronchiectasis in children with cystic fibrosis
Archivos Argentinos de Pediatria 2013; 111: 561 - 562

de Winter-de Groot KM, Noman SV, Speleman L, Schilder AGM, van der Ent CK
Nasal Nitric Oxide Levels and Nasal Polyposis in Children and Adolescents With Cystic Fibrosis
Jama Otolaryngology-head & Neck Surgery 2013; 139: 931 - 936

Deschaght P, Schelstraete P, Van Simaey L, Vanderkercken M, Raman A, Mahieu L, Van daele S,
Is the Improvement of CF Patients, Hospitalized for Pulmonary Exacerbation, Correlated to a Decrease in Bacterial Load?
Plos One 2013; 8:11.e79010

Eakin MN, Riekert KA
The impact of medication adherence on lung health outcomes in cystic fibrosis
Current Opinion in Pulmonary Medicine 2013; 19: 687 - 691

Fleck R, McPhail G, Szczesniak R, Knowlton J, Radhakrishnan R, Clancy J, Amin R
Aortopulmonary collateral flow in cystic fibrosis assessed with phase-contrast MRI
Pediatric Radiology 2013; 43: 1279 - 1286

Garland AL, Walton WG, Coakley RD, Tan CD, Gilmore RC, Hobbs CA, Tripathy A, Clunes LA, Bencharit S, Stutts MJ, Betts
Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways
Proceedings of the National Academy of Sciences of the United States of 2013; 110: 15973 - 1597

Gilchrist FJ., Samuels M, Klafkowski G, Watson NA., Lenney W
Pneumomediastinum and hyponatraemic dehydration as presenting features of cystic fibrosis
European Respiratory Journal 2013; 42: 1760-1762

Morgan WJ, Wagener JS, Yegin A, Pasta DJ, Millar SJ, Konstan MW
Probability of Treatment Following Acute Decline in Lung Function in Children with Cystic Fibrosis is Related to Baseline Pulmonary Function
Journal of Pediatrics 2013; 163: 1152 - +

Nick JA, Sanders LA, Ickes B, Briones NJ, Caceres SM, Malcolm KC, Brayshaw SJ, Chacon CS, Barboa CM, Jones MC, St
Blood mRNA biomarkers for detection of treatment response in acute pulmonary exacerbations of cystic fibrosis
Thorax 2013; 68: 929 - 937

Prioli M. A., Scarsini R., Pesarini G., Cristofaletti A., Castellani C., Assael B., Tridello G., Milano E. G., Dolci G., Vassanelli C.
The impairment of pulmonary function is related to the development of pulmonary hypertension in patients with cystic fibrosis?
European Heart Journal 2013; 34: 43

Sawicki GS, Ayyagari R, Zhang J, Signorovitch JE, Fan LY, Swallow E, Latremouille-Viau D, Wu EQ, Shi LZ
A pulmonary exacerbation risk score among cystic fibrosis patients not receiving recommended care
Pediatric Pulmonology 2013; 48: 954 - 961

Shoki AH, Mayer-HNicole, Wilcox PG., Sin DD., Quon BS.
Systematic Review of Blood Biomarkers in Cystic Fibrosis Pulmonary Exacerbations
Chest 2013; 144: 1659 - 1670

Solomon GM, Frederick C, Zhang SY, Gaggar A, Harris T, Woodworth BA, Steele C, Rowe SM
IP-10 Is a Potential Biomarker of Cystic Fibrosis Acute Pulmonary Exacerbations
Plos One 2013; 8: 8:e72398

Szczesniak RD., McPhail GL., Duan LL., Macaluso M, Amin RS., Clancy JP.
A semiparametric approach to estimate rapid lung function decline in cystic fibrosis
Annals of Epidemiology 2013; 23: 771 - 777

Vermeulen F, Ophoff J, Proesmans M, De Boeck K
Comparison of lung clearance index measured during helium washin and washout in children with cystic fibrosis
Pediatric Pulmonology 2013; 48: 962 - 969

Wielputz MO, Weinheimer O, Eichinger M, Wiebel M, Biederer J, Kauczor HU, Heussel CP, Mall MA, Puderbach M
Pulmonary Emphysema in Cystic Fibrosis Detected by Densitometry on Chest Multidetector Computed Tomography
Plos One 2013; 8: 8:e73142


Chen DL, Atkinson JJ, Ferkol TW
FDG PET Imaging in Cystic Fibrosis
Seminars in Nuclear Medicine 2013; 43: 412 - 419

Dasenbrook EC, Lu L, Donnola S, Weaver DE, Gulani V, Jakob PM, Konstan MW, Flask CA
Normalized T1 Magnetic Resonance Imaging for Assessment of Regional Lung Function in Adult Cystic Fibrosis Patients – A Cross-Sectional Study
Plos One 2013; 8: 9:e73286

Loeve M, Krestin GP, Rosenfeld M, de Bruijne M, Stick SM, Tiddens HA
Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease?
European Respiratory Journal 2013; 42: 844 - 857

Simanovsky N, Gileles-Hillel A, Frenkel R, Shosayov D, Hiller N
Correlation Between Computed Tomography Expression of Pulmonary Hypertension and Severity of Lung Disease in Cystic Fibrosis Patients
Journal of Thoracic Imaging 2013; 28: 383 - 387


Vernooij-van Langen AMM, Reijntjens S, van der Pal SM, Loeber JG, Dompeling E, Dankert-Roelse JE
To know or not to know, disclosure of a newborn carrier screening test result for cystic fibrosis
European Journal of Medical Genetics 2013; 56: 192 - 196


Chmiel JF., Konstan MW., Elborn J.S
Antibiotic and Anti-Inflammatory Therapies for Cystic Fibrosis
Cold Spring Harbor Perspectives in Medicine 2013; 3: 10:a009779

De Boeck K
Trying to find a cure for cystic fibrosis: CFTR biomarkers as outcomes
European Respiratory Journal 2013; 42: 1156 - 1157

Deeks ED
Ivacaftor: A Review of Its Use in Patients with Cystic Fibrosis
Drugs 2013; 73: 1595 - 1604

Devesa I, Fernandez-Ballester G, Ferrer-Montiel A
Targeting protein-protein interactions to rescue Delta f508-cftr: a novel corrector approach to treat cystic fibrosis
Embo Molecular Medicine 2013; 5: 1462 - 1464

Dubois AV, Midoux P, Gras D, Si-Tahar M, Brea D, Attucci S, Khelloufi MK, Ramphal R, Diot P, Gauthier F, Herve V
Poly-L-Lysine Compacts DNA, Kills Bacteria, and Improves Protease Inhibition in Cystic Fibrosis Sputum
American Journal of Respiratory and Critical Care Medicine 2013; 188: 703 - 709

Grasemann H, Tullis E, Ratjen F
A randomized controlled trial of inhaled L-Arginine in patients with cystic fibrosis
Journal of Cystic Fibrosis 2013; 12: 468 - 474

Harrison MJ, Murphy DM, Plant BJ
Ivacaftor in a G551D Homozygote with Cystic Fibrosis
New England Journal of Medicine 2013; 369: 1280 - 1282

Lebecque P
Trying to find a cure for cystic fibrosis: CFTR biomarkers as outcomes
European Respiratory Journal 2013; 42: 1155

Liedtke CM.
Understanding the cellular mechanism for inhaled hyperosmotic saline therapy for patients with cystic fibrosis. Focus on "Effect of apical hyperosmotic sodium challenge and amiloride on sodium transport in human bronchial epithelial cells from cystic
American Journal of Physiology-cell Physiology 2013; 305: C1096 – C1097

McKiernan PJ, Cunningham O, Greene CM, Cryan SA
Targeting miRNA-based medicines to cystic fibrosis airway epithelial cells using nanotechnology
International Journal of Nanomedicine 2013; 8: 3907 - 3915

O'Reilly R, Elphick HE
Development, clinical utility, and place of ivacaftor in the treatment of cystic fibrosis
Drug Design Development and Therapy 2013; 7: 929 - 937

Polenakovik HM, Sanville B
The use of ivacaftor in an adult with severe lung disease due to cystic fibrosis (Delta F508/G551D)
Journal of Cystic Fibrosis 2013; 12: 530 - 531

Savla R, Minko T
Nanotechnology approaches for inhalation treatment of fibrosis
Journal of Drug Targeting 2013; 21: 914 - 925

Sawicki GS, Ren CL, Konstan MW, Millar SJ, Pasta DJ, Quittner AL
Treatment complexity in cystic fibrosis: Trends over time and associations with site-specific outcomes
Journal of Cystic Fibrosis 2013; 12: 461 - 467

Schoenberger M, Althaus M
Novel small molecule epithelial sodium channel inhibitors as potential therapeutics in cystic fibrosis - a patent evaluation
Expert Opinion On Therapeutic Patents 2013; 23: 1383 - 1389


Casswell GK, Pilcher DV, Martin RS, Pellegrino VA, Marasco SF, Robertson C, Butt W, Buckland M, Gooi J, Snell GI, Westall
Buying time: The use of extracorporeal membrane oxygenation as a bridge to lung transplantation in pediatric patients
Pediatric Transplantation 2013; 17: E182 - E188

Lobo LJ, Chang LC, Esther CR, Gilligan PH, Tulu Z, Noone PG
Lung transplant outcomes in cystic fibrosis patients with pre-operative Mycobacterium abscessus respiratory infections
Clinical Transplantation 2013; 27: 523 - 529

Suhling H, Rademacher J, Greer M, Haverich A, Warnecke G, Gottlieb J, Welte T
Inhaled colistin following lung transplantation in colonised cystic fibrosis patients
European Respiratory Journal 2013; 42: 542 - 544