Journal of Cystic Fibrosis
Journal Impact IF - Analysis · Trend · Prediction · Ranking


New

Journal Impact IF

2019-2020

4.759

10.9%

Journal Impact IF Trend

Related Journals

Popular Journals

Journal of Cystic Fibrosis

The 2019-2020 Journal Impact IF of Journal of Cystic Fibrosis is 4.759, which is just updated in 2020.

Journal of Cystic Fibrosis Impact Factor
Highest IF
4.759
Highest Journal Impact IF

The highest Journal Impact IF of Journal of Cystic Fibrosis is 4.759.

Lowest IF
2.873
Lowest Journal Impact IF

The lowest Journal Impact IF of Journal of Cystic Fibrosis is 2.873.

Total Growth Rate
49.2%
IF Total Growth Rate

The total growth rate of Journal of Cystic Fibrosis IF is 49.2%.

Annual Growth Rate
5.5%
IF Annual Growth Rate

The annual growth rate of Journal of Cystic Fibrosis IF is 5.5%.

Journal Impact IF Ranking

Subcategory Quartile Rank Percentile
Pulmonary and Respiratory Medicine Q1 19/131

Pulmonary and Respiratory Medicine 85%

Pediatrics, Perinatology and Child Health Q1 12/286

Pediatrics, Perinatology and Child Health 95%

Journal Impact IF Ranking

· In the Pulmonary and Respiratory Medicine research field, the Quartile of Journal of Cystic Fibrosis is Q1. Journal of Cystic Fibrosis has been ranked #19 over 131 related journals in the Pulmonary and Respiratory Medicine research category. The ranking percentile of Journal of Cystic Fibrosis is around 85% in the field of Pulmonary and Respiratory Medicine.
· In the Pediatrics, Perinatology and Child Health research field, the Quartile of Journal of Cystic Fibrosis is Q1. Journal of Cystic Fibrosis has been ranked #12 over 286 related journals in the Pediatrics, Perinatology and Child Health research category. The ranking percentile of Journal of Cystic Fibrosis is around 95% in the field of Pediatrics, Perinatology and Child Health.

Journal of Cystic Fibrosis Impact Factor 2020-2021 Prediction

Journal of Cystic Fibrosis Impact Factor Predition System

Journal of Cystic Fibrosis Impact Factor Prediction System is now online. You can start share your valuable insights with the community.

Predict Check All Preditions
Total Publications
9410
Total Citations
55862

Annual Publication Volume

Annual Citation Record

International Collaboration Trend

Cited Documents Trend

Journal Impact IF History

Year Journal Impact IF
Year Journal Impact IF
2019-2020 4.759
2018-2019 4.29
2017-2018 3.858
2016-2017 4.727
2015-2016 3.853
2014-2015 3.475
2013-2014 3.82
2012-2013 2.873
2011-2012 3.19
Journal Impact IF History

· The 2019-2020 Journal Impact IF of Journal of Cystic Fibrosis is 4.759
· The 2018-2019 Journal Impact IF of Journal of Cystic Fibrosis is 4.29
· The 2017-2018 Journal Impact IF of Journal of Cystic Fibrosis is 3.858
· The 2016-2017 Journal Impact IF of Journal of Cystic Fibrosis is 4.727
· The 2015-2016 Journal Impact IF of Journal of Cystic Fibrosis is 3.853
· The 2014-2015 Journal Impact IF of Journal of Cystic Fibrosis is 3.475
· The 2013-2014 Journal Impact IF of Journal of Cystic Fibrosis is 3.82
· The 2012-2013 Journal Impact IF of Journal of Cystic Fibrosis is 2.873
· The 2011-2012 Journal Impact IF of Journal of Cystic Fibrosis is 3.19

Publications Cites Dataset

Year Publications Citations
Year Publications Citations
2001 0 2
2002 38 10
2003 48 48
2004 92 178
2005 63 340
2006 482 641
2007 440 1070
2008 665 1222
2009 494 1713
2010 537 2164
2011 486 2609
2012 616 3071
2013 638 3545
2014 564 4235
2015 631 5324
2016 598 5554
2017 790 5667
2018 549 4796
2019 819 5589
2020 851 7645
2021 9 439
Publications Cites Dataset

· The Journal of Cystic Fibrosis has published 0 reports and received 2 citations in 2001.
· The Journal of Cystic Fibrosis has published 38 reports and received 10 citations in 2002.
· The Journal of Cystic Fibrosis has published 48 reports and received 48 citations in 2003.
· The Journal of Cystic Fibrosis has published 92 reports and received 178 citations in 2004.
· The Journal of Cystic Fibrosis has published 63 reports and received 340 citations in 2005.
· The Journal of Cystic Fibrosis has published 482 reports and received 641 citations in 2006.
· The Journal of Cystic Fibrosis has published 440 reports and received 1070 citations in 2007.
· The Journal of Cystic Fibrosis has published 665 reports and received 1222 citations in 2008.
· The Journal of Cystic Fibrosis has published 494 reports and received 1713 citations in 2009.
· The Journal of Cystic Fibrosis has published 537 reports and received 2164 citations in 2010.
· The Journal of Cystic Fibrosis has published 486 reports and received 2609 citations in 2011.
· The Journal of Cystic Fibrosis has published 616 reports and received 3071 citations in 2012.
· The Journal of Cystic Fibrosis has published 638 reports and received 3545 citations in 2013.
· The Journal of Cystic Fibrosis has published 564 reports and received 4235 citations in 2014.
· The Journal of Cystic Fibrosis has published 631 reports and received 5324 citations in 2015.
· The Journal of Cystic Fibrosis has published 598 reports and received 5554 citations in 2016.
· The Journal of Cystic Fibrosis has published 790 reports and received 5667 citations in 2017.
· The Journal of Cystic Fibrosis has published 549 reports and received 4796 citations in 2018.
· The Journal of Cystic Fibrosis has published 819 reports and received 5589 citations in 2019.
· The Journal of Cystic Fibrosis has published 851 reports and received 7645 citations in 2020.
· The Journal of Cystic Fibrosis has published 9 reports and received 439 citations in 2021.
· The total publications of Journal of Cystic Fibrosis is 9410.
· The total citations of Journal of Cystic Fibrosis is 55862.

What is Impact Factor?

The impact factor (IF) or journal impact factor (JIF) of an academic journal is a scientometric index calculated by Clarivate that reflects the yearly average number of citations of articles published in the last two years in a given journal. It is frequently used as a proxy for the relative importance of a journal within its field; journals with higher impact factor values are often deemed to be more important, or carry more intrinsic prestige in their respective fields, than those with lower values.

Journal of Cystic Fibrosis | Academic Accelerator - About the Impact Factor

Impact factor is commonly used to evaluate the relative importance of a journal within its field and to measure the frequency with which the “average article” in a journal has been cited in a particular time period. Journal which publishes more review articles will get highest IFs. Journals with higher IFs believed to be more important than those with lower ones. According to Eugene Garfield “impact simply reflects the ability of the journals and editors to attract the best paper available.” Journal which publishes more review articles will get maximum IFs. The Impact Factor of an academic journal is a scientometric Metric that reflects the yearly average number of citations that recent articles published in a given journal received. It is frequently used as a Metric for the relative importance of a journal within its field; journals with higher Impact Factor are often deemed to be more important than those with lower ones. The Journal of Cystic Fibrosis Impact Factor IF measures the average number of citations received in a particular year (2020) by papers published in the Journal of Cystic Fibrosis during the two preceding years (2018-2019). Note that 2020 Impact Factor are reported in 2021; they cannot be calculated until all of the 2020 publications have been processed by the indexing agency. New journals, which are indexed from their first published issue, will receive an impact factor after two years of indexing; in this case, the citations to the year prior to Volume 1, and the number of articles published in the year prior to Volume 1, are known zero values. Journals that are indexed starting with a volume other than the first volume will not get an impact factor until they have been indexed for three years. Occasionally, Journal Citation Reports assigns an impact factor to new journals with less than two years of indexing, based on partial citation data. The calculation always uses two complete and known years of item counts, but for new titles one of the known counts is zero. Annuals and other irregular publications sometimes publish no items in a particular year, affecting the count. The impact factor relates to a specific time period; it is possible to calculate it for any desired period. In addition to the 2-year Impact Factor, the 3-year Impact Factor, 4-year Impact Factor, 5-year Impact Factor, Real-Time Impact Factor can provide further insights and factors into the impact of Journal of Cystic Fibrosis.

History

The impact factor was devised by Eugene Garfield, the founder of the Institute for Scientific Information (ISI). Impact factors are calculated yearly starting from 1975 for journals listed in the Journal Citation Reports (JCR). ISI was acquired by Thomson Scientific & Healthcare in 1992, and became known as Thomson ISI. In 2018, Thomson ISI was sold to Onex Corporation and Baring Private Equity Asia. They founded a new corporation, Clarivate, which is now the publisher of the JCR.

Use

The impact factor is used to compare different journals within a certain field. The Web of Science indexes more than 11,500 science and social science journals. Journal impact factors are often used to evaluate the merit of individual articles and individual researchers. This use of impact factors was summarised by Hoeffel:

Impact Factor is not a perfect tool to measure the quality of articles but there is nothing better and it has the advantage of already being in existence and is, therefore, a good technique for scientific evaluation. Experience has shown that in each specialty the best journals are those in which it is most difficult to have an article accepted, and these are the journals that have a high impact factor. Most of these journals existed long before the impact factor was devised. The use of impact factor as a measure of quality is widespread because it fits well with the opinion we have in each field of the best journals in our specialty....In conclusion, prestigious journals publish papers of high level. Therefore, their impact factor is high, and not the contrary.

Eugene Garfield

In brief, Impact factors may be used by:
  • Authors to decide where to submit an article for publication.
  • Libraries to make collection development decisions
  • Academic departments to assess academic productivity
  • Academic departments to make decisions on promotion and tenure.
As impact factors are a journal-level metric, rather than an article- or individual-level metric, this use is controversial. Garfield agrees with Hoeffel,but warns about the "misuse in evaluating individuals" because there is "a wide variation [of citations] from article to article within a single journal". Other things to consider about Impact Factors:
  • Many journals do not have an impact factor.
  • The impact factor cannot assess the quality of individual articles. Even if citations were evenly distributed among articles, the impact factor would only measure the interests of other researchers in an article, not its importance and usefulness.
  • Only research articles, technical notes and reviews are “citable” items. Editorials, letters, news items and meeting abstracts are “non-citable items”.
  • Only a small percentage of articles are highly cited and they are found in a small subset of journals. This small proportion accounts for a large percentage of citations.
  • Controversial papers, such as those based on fraudulent data, may be highly cited, distorting the impact factor of a journal.
  • Citation bias may exist. For example, English language resources may be favoured. Authors may cite their own work.
Moreover, informed and careful use of these impact data is essential, and should be based on a thorough understanding of the methodology used to generate impact factors. There are controversial aspects of using impact factors:
  • It is not clear whether the number of times a paper is cited measures its actual quality.
  • Some databases that calculate impact factors fail to incorporate publications including textbooks, handbooks and reference books.
  • Certain disciplines have low numbers of journals and usage. Therefore, one should only compare journals or researchers within the same discipline.
  • Review articles normally are cited more often and therefore can skew results.
  • Self-citing may also skew results.
  • Some resources used to calculate impact factors have inadequate international coverage.
  • Editorial policies can artificially inflate an impact factor.
Impact factors have often been used in advancement and tenure decision-making. Many recognize that this is a coarse tool for such important decisions, and that a multitude of factors should be taken into account in these deliberations. When considering the use of the impact factor (IF), keep these aspects in mind:
  • IF analysis is limited to citations from the journals indexed by the Web of Science/Web of Knowledge. Currently, the Web of Science indexes only 8621 journals across the full breadth of the sciences, and just 3121 in the social sciences.
  • A high IF/citation rate says nothing about the quality -- or even, validity -- of the references being cited. Notorious or even retracted articles often attract a lot of attention, hence a high number of citations. The notority related to the first publication on "cold fusion" is one such example.
  • Journals that publish more "review articles" are often found near the top of the rankings. While not known for publishing new, creative findings, these individual articles tend to be heavily cited.
  • The IF measures the average number of citations to articles in the journal -- given this, a small number of highly-cited articles will skew the figure.
  • It takes several years for new journals to be added to the list of titles indexed by the Web of Science/Web of Knowledge, so these newer titles will be under-represented.
  • It's alleged that journal editors have learned to "game" the system, encouraging authors to cite their works previously published in the same journal.
Comparing Journals Across Disciplines? Not a good idea! Using Impact Factors within a given discipline should only be done with great care, as described above. Using impact factor data to compare journals across disciplines is even more problematic. Here are some of the reasons:
  • Disciplines where older literature is still referenced, such as Chemistry and Mathematics, offer challenges to the methodolgy since older citations (older than two years) are not used to calculate the impact factor for a given journal. (Five-year impact factor analysis, which can be calculated using the Journal Citation Index database, helps smooth out this problem only to some degree.)
  • Different disciplines have different practices regarding tendency to cite larger numbers of references. Higher overall citation rates will bump upward impact factor measurements.
  • Where it's common for large numbers of authors to collaborate on a single paper, such as in Physics, the tendency of authors to cite themselves (and in this case, more authors) will result in increased citation rates.

Pros and Cons of the Impact Factor

Pros:

  • A vetted, established metric for measuring journal impact within a discipline.
  • Designed to eliminate bias based on journal size and frequency.
Cons:
  • Individual articles makes an uneven contribution to overall Impact Factor.
  • Impact Factor does not account for certain things, things like context (postive or negative citaion) and intentionality (self-citation).
  • The metric is proprietary to and bound by the contents of the Thomson Reuters database.
  • Citations, on which the Impact Factor is based, count for less than 1% of an article's overall use.

Criticism

Numerous critiques have been made regarding the use of impact factors. A 2007 study noted that the most fundamental flaw is that impact factors present the mean of data that are not normally distributed, and suggested that it would be more appropriate to present the median of these data. There is also a more general debate on the validity of the impact factor as a measure of journal importance and the effect of policies that editors may adopt to boost their impact factor (perhaps to the detriment of readers and writers). Other criticism focuses on the effect of the impact factor on behavior of scholars, editors and other stakeholders. Others have made more general criticisms, arguing that emphasis on impact factor results from negative influence of neoliberal policies on academia claiming that what is needed is not just replacement of the impact factor with more sophisticated metrics for science publications but also discussion on the social value of research assessment and the growing precariousness of scientific careers in higher education.
Experts stress that there are limitations in using impact factors to evaluate a scholar's work. There are many reasons cited for not relying on impact factor alone to evaluate the output of a particular individual. Among these are the following:

  • A single factor is not sufficient for evaluating an author's work.
  • Journal values are meaningless unless compared within the same discipline. Impact factors vary among disciplines.
  • The impact factor was originally devised to show the impact of a specific journal, not a specific scholar. The quality and impact of the author's work may extend beyond the impact of a particular journal.
According to Jim Testa, a researcher for ThomsonReuters Scientific, the most widespread misuse of the Impact Factor is to evaluate the work of an individual author (instead of a journal). "To say that because a researcher is publishing in a certain journal, he or she is more influential or deserves more credit is not necessarily true. There are many other variables to consider." (interview 6/26/2008 in Thomson Reuters blog entry)

Journal of Cystic Fibrosis
Journal Profile

About

The Journal of Cystic Fibrosis is the official journal of the European Cystic Fibrosis Society. The journal is devoted to promoting the research and treatment of cystic fibrosis. To this end the journal publishes original scientific articles, editorials, case reports, short communications and other information relevant to cystic fibrosis. The journal also publishes news and articles concerning the activities and policies of the ECFS as well as those of other societies related the ECFS. None

Highly Cited Keywords

ISSN
1569-1993
ISSN

The ISSN of Journal of Cystic Fibrosis is 1569-1993 . An ISSN is an 8-digit code used to identify newspapers, journals, magazines and periodicals of all kinds and on all media–print and electronic.

ISSN (Online)
1873-5010
ISSN (Online)

The ISSN (Online) of Journal of Cystic Fibrosis is 1873-5010 . An ISSN is an 8-digit code used to identify newspapers, journals, magazines and periodicals of all kinds and on all media–print and electronic.

Publisher
Elsevier
Publisher

Journal of Cystic Fibrosis is published by Elsevier .

Publication Frequency
-
Publication Frequency

Journal of Cystic Fibrosis publishes reports - .

Coverage
2002 - Present
Coverage

The Publication History of Journal of Cystic Fibrosis covers 2002 - Present .

Open Access
NO
Open Access

Journal of Cystic Fibrosis is Subscription-based (non-OA) Journal. Publishers own the rights to the articles in their journals. Anyone who wants to read the articles should pay by individual or institution to access the articles. Anyone who wants to use the articles in any way must obtain permission from the publishers.

Publication Fee
Publication Fee

There is no publication fee for submiting manuscript to Journal of Cystic Fibrosis. Journal of Cystic Fibrosis is Subscription-based (non-OA) Journal. Publishers own the rights to the articles in their journals. Anyone who wants to read the articles should pay by individual or institution to access the articles.

Language
-
Language

The language of Journal of Cystic Fibrosis is - .

Country/Region
Netherlands
Country/Region

The publisher of Journal of Cystic Fibrosis is Elsevier , which locates in Netherlands .

Selected Articles

Full Title Authors
Full Title Authors
Cystic fibrosis related diabetes is not independently associated with increased Stenotrophomonas maltophilia infection: Longitudinal data from the UK CF Registry Mathieu Gruet · Nicolas Decorte · Laurent Mely · Jean-Marc Vallier · Boubou Camara · S. Quetant · Bernard Wuyam · Samuel Verges · Samuel Verges
P224 Aseptic centralised versus home extemporaneous preparation for cystic fibrosis outpatients parenteral antibiotic therapy: a survey on nurses satisfaction I.J. Haq · B. Verdon · K. Jiwa · Vinciane Saint-Criq · A.I. Gardner · Christopher Ward · Michael A. Gray · Malcolm Brodlie · Malcolm Brodlie
P260 Searching for CFTR mutations in CF patients from North Ossetia-Alania Republic, Russian Federation Alejandro Teper · J. Balinotti · V. Rodriguez · S. Lubovich · S. Zaragoza · D. Chang · N. Escobar · L. Galanternik · L. Galanternik
P095 Intravenous fosfomycin for treatment of pulmonary exacerbations in adult cystic fibrosis patients with chronic infection by Pseudomonas aeruginosa and other Gram-negative bacteria T.A. Vasilyeva · N.V. Petrova · N.Y. Kashirskaya · Z.K. Getoeva · M.Y. Dzhadzhieva · O.G. Novoselova · E. Kondratyeva · E.K. Ginter · R.A. Zinchenko · R.A. Zinchenko
WS03.3 Childhood nutritional status is a major factor determining lung function in adults with cystic fibrosis B. Messore · M. Bellocchia · C. Bena · C. Biglia · G.P. Bandelli · E. Clivati · G. Bonizzoni · S. Demichelis · F. Traversa · C. Albera · C. Albera
P223 Totally implantable venous access device tip position and risk of venous complications in cystic fibrosis Moshe Ashkenazi · N. Nathan · Ifat Sarouk · B.E. Bar Aluma · Adi Dagan · Yael Bezalel · Daphna Vilozni · Daphna Vilozni
P246 Qualityof life and influencing factors in children and young adults with cystic fibrosis A.O. Hart · K.M. Webb · W.G. Flight · W.G. Flight
P035 Differential gene expression by Pseudomonas aeruginosa in response to bacteriophage infection Emine Atag · N. Bas Ikizoglu · P. Ergenekon · E. Erdem Eralp · Yasemin Gokdemir · Bulent Karadag · Bulent Karadag
WS19.1 Glucose abnormalities in Canadian and French cystic fibrosis patients: a Glyc-one database analysis R. Brugha · I. Martin · S. Morales · S. Lehman · Ewfw Alton · Jane C. Davies · Jane C. Davies
P177 Is APRI index evaluation useful in diagnosing hepatic changes in the course of cystic fibrosis Q. Reynaud · V. Boudreau · Sandrine Touzet · K. Desjardins · S. Poupon Bourdy · Y. Berthiaume · R. Rabasa Lhoret · I. Durieu · I. Durieu
EPS6.06 Influence of a supervised exercise program ( CFmobil ) on motor ability in adult cystic fibrosis patients Sabina Więcek · Halina Wos · A. Pogorzelski · B. Kordys-Darmolinska · H. Mazurek · Urszula Grzybowska-Chlebowczyk · Urszula Grzybowska-Chlebowczyk
WS08.4 Feasibility of an incremental step test to assess aerobic fitness in adult cystic fibrosis M. Welsner · S. Sutharsan · F. Stehling · T. Tas · D. Thra · C. Koerner-Rettberg · S. Benzrath · W. Gruber · W. Gruber
WS15.3 Patterns of response to lumacaftor and ivacaftor in rectal organoids Z. Beverley · H. Parrott · C. Gray · A.L. Jones · A.L. Jones
WS17.2 Using a highly parallel sequencing assay for CFTR genotyping in ethnically diverse European patients with CF A.S. Ramalho · P. Peetermans · M. Proesmans · L. Dupont · F. Vermeulen · K. De Boeck · K. De Boeck
IPD2.04 Long-term effects of ivacaftor in patients with G551D mutation and mild lung disease S. Cuyx · E. Aertgeerts · F. Vermeulen · K. De Boeck · Harry Cuppens · Harry Cuppens
P074 Information value of non-invasive specimens for airway bacteriome research H. Ellemunter · C. Hindinger · G. Steinkamp · G. Steinkamp
WS15.2 R334W CFTR, a severely compromised chloride conductance mutant, retains its bicarbonate conductance and responds to the corrector combination, C4 + C18 O. Voronina · N. Ryzhova · M. Kunda · E. Aksenova · N. Sharapova · V. Sherman · A. Voronkova · E. Kondratyeva · A. Gintsburg · A. Gintsburg
P248 Factors related to anxiety and depression in adult cystic fibrosis patients Clément Boinot · H. Wang · Inna Sabirzhanova · C. Cebotaru · William B. Guggino · Liudmila Cebotaru · Liudmila Cebotaru
Continuous glucose monitoring guided insulin therapy is associated with improved clinical outcomes in cystic fibrosis-related diabetes N.Z. Uslu · Derya Kocakaya · S. Olgun Yildizeli · Berrin Ceyhan · Berrin Ceyhan
P164 How can continuous glucose monitoring system impact on therapy of Cystic Fibrosis-Related Diabetes (CFRD) in youth F. Frost · P. Dyce · D. Nazareth · Victoria Malone · M.J. Walshaw · M.J. Walshaw
P034 Are patients with cystic fibrosis in clinical trials sensitive to the placebo effect? A metanalysis S. Todorovic · T. Milenkovic · Predrag Minic · K. Mitrovic · A. Sovtic · M. Rodic · V. Rade · V. Rade
WS14.4 Use of computational fluid dynamics (CFD) to model aerosol deposition in the lungs of patients with cystic fibrosis J. Coton · H.-H. Le · V. Veuillet · P. Janiaud · M. Cucherat · B. Kassai-Koupai · F. Gueyffier · P. Reix · P. Reix
WS12.4 Are goal-focused, motivational text messages effective at improving patients belief in their own ability (self-efficacy) to complete inhaled therapies in adults with cystic fibrosis D. Hull · A. Black · W. Vos · W. Vos
P115 Risk factors for early lung function decline in young children with cystic fibrosis S. Garcia · L. Margueritte · I. Carpentier · V. Nave · V. Nave
EPS1.04 Use of ex vivo paediatric primary nasal epithelial cultures to investigate TMEM16A as a potential therapeutic target in children with cystic fibrosis A. Kotnik Pirs · M. Žolnir Dovč · Uros Krivec · Malena Aldeco · Dusanka Lepej · A. Zver · Marina Praprotnik · Marina Praprotnik
P240 Association between adherence to azithromycin and dornase alpha and lung function decline in adult cystic fibrosis (CF) patients: a two-year analysis D. Salvatore · Rita Padoan · Roberto Buzzetti · A. Amato · B. Giordani · G. Ferrari · F. Majo · F. Majo
P060 The epidemiology of nontuberculous mycobacteria in a paediatric cystic fibrosis centre Boubou Camara · A. Fuchs · J.-C. Benitez · S. Quetant · S. Chanoine · C. Llerena · A. Herbinet · Bernard Wuyam · V. Vion · Isabelle Pin · Isabelle Pin
Fecal calprotectin concentrations in young children with cystic fibrosis: Authors response Eugenia Bruzzese · Valeria Raia · Eliana Ruberto · Riccardo Scotto · Antonietta Giannattasio · Dario Bruzzese · Maria Cristina Cavicchi · Michela Francalanci · Carla Colombo · Nadia Faelli · Valeria Daccò · Giuseppe Magazzù · Stefano Costa · Vincenzina Lucidi · Fabio Majo · Alfredo Guarino · Alfredo Guarino
Faecal calprotectin concentrations in young children with cystic fibrosis Meghana Sathe · Roderick H. J. Houwen · Roderick H. J. Houwen
Antibiotic exposure and interpersonal variance mask the effect of ivacaftor on respiratory microbiota composition Narelle S. Cox · Narelle S. Cox · Anne E. Holland · Anne E. Holland
Multiple prevalent fractures in relation to macroscopic bone architecture in patients with cystic fibrosis Mirjam Stahl · Christian Holfelder · Carolin Kneppo · Meinhard Kieser · Christian Kasperk · Eckhard Schoenau · Olaf Sommerburg · Burkhard Tönshoff · Burkhard Tönshoff
Comparing the management of constipation and distal intestinal obstruction syndrome between paediatricians and adult physicians Deborah M. Cholon · Martina Gentzsch · Martina Gentzsch
Hypoglycaemia in patients with cystic fibrosis- harbinger of poor outcomes or innocent bystander? Hongyu Li · Emanuela Pesce · David N. Sheppard · Ashvani K. Singh · Nicoletta Pedemonte · Nicoletta Pedemonte
A specialized method of sputum collection and processing for therapeutic interventions in cystic fibrosis Amir Moheet · Katie Larson Ode · Katie Larson Ode · Katie Larson Ode
RNA sequencing data from neutrophils of patients with cystic fibrosis reveals potential for developing biomarkers for pulmonary exacerbations Oliver J. McElvaney · Cedric Gunaratnam · Emer P. Reeves · Noel G. McElvaney · Noel G. McElvaney
Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome Kaiyu Jiang · Kerry E. Poppenberg · Laiping Wong · Yanmin Chen · Drucy Borowitz · Danielle M. Goetz · Daniel W. Sheehan · Carla Frederick · Vincent M. Tutino · Hui Meng · James N. Jarvis · James N. Jarvis
The choice of lung function reference equation affects clinical trial eligibility: Results from a cystic fibrosis cohort Qiangni Liu · Inna Sabirzhanova · Murali K. Yanda · Emily Bergbower · Clément Boinot · William B. Guggino · Liudmila Cebotaru · Liudmila Cebotaru
AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations Inger Hee Mathiesen · Andreas Ronit · Tacjana Pressler · Tacjana Pressler
Resistin is elevated in cystic fibrosis sputum and correlates negatively with lung function Rebecca Darrah · Frank J. Jacono · Neha Joshi · Anna L. Mitchell · Abdus Sattar · Cara K. Campanaro · Paul Litman · Jennifer Frey · David Nethery · Eric S. Barbato · Craig A. Hodges · Harriet Corvol · Garry R. Cutting · Lisa J. Strug · Mitchell L. Drumm · Mitchell L. Drumm
CFTR modulator theratyping: Current status, gaps and future directions Osric Forrest · Daniel M. Chopyk · Yael Gernez · Milton R. Brown · Carol Conrad · Richard B. Moss · Vin Tangpricha · Limin Peng · Rabindra Tirouvanziam · Rabindra Tirouvanziam
Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent John P. Clancy · Calvin U. Cotton · Scott H. Donaldson · George M. Solomon · Donald R. VanDevanter · Michael P. Boyle · Martina Gentzsch · Jerry A. Nick · Beate Illek · John C. Wallenburg · Eric J. Sorscher · Margarida D. Amaral · Jeffrey M. Beekman · Anjaparavanda P. Naren · Robert J. Bridges · Philip J. Thomas · Garry R. Cutting · Steven M. Rowe · Anthony G. Durmowicz · Kris De Boeck · William Skach · Christopher M. Penland · Elizabeth Joseloff · Hermann Bihler · John Mahoney · Drucy Borowitz · Katherine L. Tuggle · Katherine L. Tuggle
SPX-101 is stable in and retains function after exposure to cystic fibrosis sputum Binyu Lu · Li Li · Molly Schneider · Craig A. Hodges · Calvin U. Cotton · James D. Burgess · Thomas J. Kelley · Thomas J. Kelley
Combination potentiator (co-potentiator) therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators Juliana Sesma · Bryant Wu · Timothy J. Stuhlmiller · David W. Scott · David W. Scott
Characteristics and outcomes of oral antibiotic treated pulmonary exacerbations in children with cystic fibrosis Puay-Wah Phuan · Jung-Ho Son · Joseph-Anthony Tan · Clarabella Li · Ilaria Musante · Lorna Zlock · Dennis W. Nielson · Walter E. Finkbeiner · Mark J. Kurth · Luis J. V. Galietta · Peter M. Haggie · A. S. Verkman · A. S. Verkman
P160 Striking the balance between cystic fibrosis and Cystic Fibrosis-Related Diabetes (CFRD) - a meta-ethnography Jordana E. Hoppe · Brandie D. Wagner · Brandie D. Wagner · Frank J. Accurso · Edith T. Zemanick · Scott D. Sagel · Scott D. Sagel
P146 Impact of the optimal duration of intensive rehabilitation training on patients with moderate to severe cystic fibrosis S. Collins · A. Jones · S. Woodward · J. Sturt · J. Sturt
EPS6.01 Shaking it up: a look at our centre experience with high frequency chest wall oscillation (HFCWO) H. Gauchez · A. Chaabi · A. Chaabi
WS08.6 Effects of home-based adapted physical activity in patients with cystic fibrosis: an interventional study C. Bridges · H. Carter · R.I. Ketchell · David C.W. Lau · J. Duckers · J. Duckers
EPS5.05 Epidemiology and phenotype of cystic fibrosis with residual function mutations in Italy K. Channon · C.C. Reilly · R.A. Massey-Chase · Caroline Elston · D. Nikoletou · D. Nikoletou
WS18.4 Evaluation of transplantation information delivered to patients and their relatives by the professionals from cystic fibrosis centres and transplant centres and from transplanted peer-patients in France Natalia Popowicz · Jamie Wood · Siobhain Mulrennan · C. Budgeon · G. Ryan · G. Ryan
Advancing the GI frontier for patients with CF Fiona Ringholz · Fiona Ringholz · Gerard Higgins · Aurélie Hatton · Ali Sassi · Ahmad Moukachar · Coral Fustero-Torre · Monika Hollenhorst · Monika Hollenhorst · I. Sermet-Gaudelus · Brian J. Harvey · Paul McNally · Paul McNally · V. Urbach · V. Urbach · V. Urbach · V. Urbach
The impact of National Cystic Fibrosis Registries: A review series Paul D. W. Eckford · Jacqueline McCormack · Lise Munsie · Gengming He · Sanja Stanojevic · Sergio L. Pereira · Karen Ho · Julie Avolio · Claire Bartlett · Jin Ye Yang · Amy P. Wong · Leigh Wellhauser · Ling Jun Huan · Jia Xin Jiang · Hong Ouyang · Kai Du · Michelle Klingel · Lianna Kyriakopoulou · Tanja Gonska · Theo J. Moraes · Lisa J. Strug · Lisa J. Strug · Janet Rossant · Felix Ratjen · Christine E. Bear · Christine E. Bear
Prevalence of hypoglycemia during oral glucose tolerance testing in adults with cystic fibrosis and risk of developing cystic fibrosis-related diabetes Rosie Sutherland · Tamarah Katz · Victoria Liu · Justine Quintano · Rebecca Brunner · Chai Wei Tong · Clare E. Collins · Chee Y. Ooi · Chee Y. Ooi · Chee Y. Ooi
Establishing the diagnosis of chronic colonization with Pseudomonas aeruginosa of cystic fibrosis patients: Comparison of the European consensus criteria with genotyping of P. aeruginosa isolates Lisa A. Mannik · Kristy A. Chang · Pascalyn Q.K. Annoh · Jenna Sykes · Julie Gilmour · Ronalee Robert · Anne L. Stephenson · Anne L. Stephenson · Anne L. Stephenson
CFTR rescue with VX-809 and VX-770 favors the repair of primary airway epithelial cell cultures from patients with class II mutations in the presence of Pseudomonas aeruginosa exoproducts Leander Jonckheere · Petra Schelstraete · Leen Van Simaey · Eva Van Braeckel · Julie Willekens · Sabine Van daele · Frans De Baets · Mario Vaneechoutte · Mario Vaneechoutte
Developments in cystic fibrosis personalised epithelial assays: Science and patient perspectives Damien Adam · Claudia Bilodeau · Laura Sognigbé · Émilie Maillé · Manon Ruffin · Emmanuelle Brochiero · Emmanuelle Brochiero
Children with cystic fibrosis demonstrate no respiratory immunological, infective or physiological, consequences of vitamin D deficiency Gerard E. Kaiko · Peter Wark · Peter Wark · Peter Wark
Defining chronic Pseudomonas aeruginosa infection in cystic fibrosis Rebecca M. Thursfield · Rebecca M. Thursfield · Khayam Naderi · Neil Leaver · Mark Rosenthal · Eric W. F. W. Alton · Andrew Bush · Andrew Bush · Jane C. Davies · Jane C. Davies · Jane C. Davies
Prevalence of severe fatigue among adults with cystic fibrosis: A single center study Valerie Waters · Keith Grimwood · Keith Grimwood
Comparison of ex vivo and in vitro intestinal cystic fibrosis models to measure CFTR-dependent ion channel activity Merel M. Nap-van der Vlist · Marcella Burghard · H.J. Hulzebos · W.R. Doeleman · Harry G.M. Heijerman · Cornelis K. van der Ent · S.L. Nijhof · S.L. Nijhof
Mini-guts in a dish: Perspectives of adult Cystic Fibrosis (CF) patients and parents of young CF patients on organoid technology Domenique D. Zomer-van Ommen · Eyleen de Poel · Evelien Kruisselbrink · Hugo Oppelaar · Annelotte Vonk · Hettie M. Janssens · Cornelis K. van der Ent · Marne C. Hagemeijer · Jeffrey M. Beekman · Jeffrey M. Beekman
Defining research priorities in cystic fibrosis. Can existing knowledge and training in biomedical research affect the choice Sarah N. Boers · Karin M. de Winter-de Groot · Jacquelien Noordhoek · Vincent Gulmans · Cornelis K. van der Ent · Johannes J. M. van Delden · Annelien L. Bredenoord · Annelien L. Bredenoord
Continuous glucose monitoring abnormalities in cystic fibrosis youth correlate with pulmonary function decline Roberto Buzzetti · Valeria Galici · Natalia Cirilli · Fabio Majo · L. Graziano · Stefano Costa · Simona Bonacina · Marila Carrubba · Giuseppe Davì · Sabrina Gagliano · Clizia Cazzarolli · Francesca Ficili · Federico Alghisi · Michele Samaja · Giuseppe Magazzù · Giuseppe Magazzù
ECFS best practice guidelines: the 2018 revision Christine L. Chan · Timothy Vigers · Laura Pyle · Philip Zeitler · Scott D. Sagel · Kristen J. Nadeau · Kristen J. Nadeau
The use of fructosamine in cystic fibrosis-related diabetes (CFRD) screening Matthew A. Tucker · Reva Crandall · Nichole Seigler · Paula Rodriguez-Miguelez · Kathleen T. McKie · Caralee Forseen · Jeffrey Thomas · Ryan A. Harris · Ryan A. Harris · Ryan A. Harris
Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor☆ C. Kors Van Der Ent · J. Stuart Elborn · J. Stuart Elborn · J. Stuart Elborn
Detection of CFTR function and modulation in primary human nasal cell spheroids Stephanie Trend · Angela Fonceca · William Graham Fox Ditcham · Anthony Kicic · Arest Cf · Arest Cf
Lower exhaled nitric oxide in infants with Cystic Fibrosis compared to healthy controls Dominique Hubert · Clémence Dehillotte · Anne Munck · V. David · Jinmi Baek · Laurent Mely · Stéphane Dominique · Sophie Ramel · Isabelle Danner Boucher · Sylvaine Lefeuvre · Quitterie Reynaud · Virginie Colomb-Jung · Prissile Bakouboula · L. Lemonnier · L. Lemonnier
Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2 years of treatment with ivacaftor in a real-world setting Gregory I. Snell · Anna Reed · Marc Stern · Denis Hadjiliadis · Denis Hadjiliadis
Analytical and biological variation in repeated sweat chloride concentrations in clinical trials for CFTR modulator therapy F. Magne · S. Durupt · R. Nove-Josserand · F. Bey-Omar · L. Laoust · V. Cottin · I. Durieu · Quitterie Reynaud · Quitterie Reynaud
Age-related levels of fecal M2-pyruvate kinase in children with cystic fibrosis and healthy children 0 to 10 years old Elena Venuti · Dmitry Shishmarev · Philip W. Kuchel · Shoma Dutt · Caron Blumenthal · Kevin J. Gaskin · Kevin J. Gaskin
Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis Don B. Sanders · Zhanhai Li · Qianqian Zhao · Philip M. Farrell · Philip M. Farrell
Exercise and its varied impact on airway dynamics K.D. Gonzalez · Jonathan B. Zuckerman · Edmund H. Sears · B.S. Prato · M. Guill · Wendy Y. Craig · C. Milliard · E. Parker · T. Lever · M.M. Griffin · L.W. Leclair · L.W. Leclair
Dietary intake of energy-dense, nutrient-poor and nutrient-dense food sources in children with cystic fibrosis Anne Stephenson · Diana Bilton · Diana Bilton
The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF Patrycja Krzyżanowska-Jankowska · Paulina Sobkowiak · Olaf Thalmann · Sławomira Drzymała-Czyż · Aleksandra Glapa · Marcin Hołysz · Dariusz Walkowiak · Nataliya Rohovyk · L. Bober · Andrzej Pogorzelski · Jarosław Walkowiak · Jarosław Walkowiak
WS11.6 The effect of 2-thiopyridine derivative 11026103, a novel antibacterial compound, on Burkholderia cenocepacia: transcriptomic response and resistance mechanisms V. David · A. Perrin · D. Pougneon-Bertrand · R. Kanaan · M. Kerbrat · A. Le Rhun · A. Le Rhun
Apolipoprotein E polymorphism determines vitamin K supplementation effectiveness in cystic fibrosis patients Jennifer S. Guimbellot · George M. Solomon · Arthur Baines · Sonya L. Heltshe · Sonya L. Heltshe · Jill VanDalfsen · Elizabeth Joseloff · Scott D. Sagel · Steven M. Rowe · Steven M. Rowe
WS12.5 A smartphone application for reporting symptoms in adults with cystic fibrosis: a randomised controlled trial J. Nunvar · S. Buroni · V. Makarov · S. Cardona · Pavel Drevinek · Pavel Drevinek
P218 Partnering with patients and parents of children with CF in the French care quality improvement program Jamie Wood · Sue Jenkins · D. Putrino · Siobhain Mulrennan · Sue Morey · N. Cecins · N. Bear · Kylie Hill · Kylie Hill
P017 A comparative study between clinically diagnosed and screen-detected children with cystic fibrosis: parents economic costs prior to diagnosis Dominique Pougheon Bertrand · G. Rault · V. David · Pierre Lombrail · Pierre Lombrail
P153 Service evaluation of an out-patient exercise programme for CF patients Patricia Fitzpatrick · C. Fitzgerald · A.Ó. Ceilleachair · K. Kapur · A. Staines · Barry Linnane · S. George · S. George
P192 To be or not to be (happy): fat is the question? F. Haynes · K. Mosley · K. Wheedon · J. Dewar · J. Dewar
IPD2.10 Observational study of glucose tolerance abnormalities in patients with cystic fibrosis homozygous for Phe508del CFTR treated by lumacaftor-ivacaftor D. Proud · R.I. Ketchell · David C.W. Lau · M. Rezaie · L. Speight · J. Duckers · J. Duckers
EPS5.06 Demography and clinical outcomes in cystic fibrosis lung transplant recipients in Belgium B. Misgault · E. Chatron · I. Durieu · Laurent Mely · M. Abely · L. Bounyar · M. Porzio · Laurence Weiss · R. Kessler · L. Kessler · L. Kessler
P045 Strain and antibiogram changes, over time, in paediatric cystic fibrosis patients colonised with Pseudomonas aeruginosa M. Thomas · S. Wanyama · L. Dupont · I. Etienne · Patrick Evrard · Benoît Rondelet · Youri Sokolow · D. Van Raemdonck · D. Van Raemdonck
P179 A retrospective audit of sodium chloride supplementation in infants with cystic fibrosis S. Mushtaq · A. Vickers · Z. Payne · D. Kenna · Jane F. Turton · N. Woodford · D.M. Livermore · D.M. Livermore
P172 Colorectal cancer in adults with cystic fibrosis: our experience at the All Wales Adult Cystic Fibrosis Centre K. Stead · M. Desai · C. Patchell · C. Patchell
P222 Home delivery influences medicines possession ratio in adult cystic fibrosis A. Sayers · J. Turner · L. Speight · R.I. Ketchell · J. Duckers · J. Duckers
P117 Time course of pulmonary hyperinflation in adult cystic fibrosis: clinical and lung function correlates M.K. Dooney · K.J. Martin · N. Iqbal · Andrew M. Jones · Peter J. Barry · Peter J. Barry
EPS5.07 Evaluating the impact of 2006 clinical practice guidelines for nutrition in children with cystic fibrosis in Australia C. Audousset · A. Lohbrunner · A. Prevotat · X. Dhalluin · O. Le Rouzic · Benoit Wallaert · Thierry Perez · Thierry Perez
Emergence of livestock-associated MRSA isolated from cystic fibrosis patients: Result of a Belgian national survey R. Ruseckaite · N. Pekin · S. King · E. Carr · S. Ahern · J. Oldroyd · A. Earnest · G. Sims · Claire Wainwright · David S. Armstrong · David S. Armstrong
Multicentre standardisation of chest MRI as radiation-free outcome measure of lung disease in young children with cystic fibrosis Bradley H. Rosen · Marc Chanson · Lara R. Gawenis · Jinghua Liu · Aderonke Sofoluwe · Alice Zoso · John F. Engelhardt · John F. Engelhardt
Primary sclerosing cholangitis is associated with abnormalities in CFTR Mark O. Wielpütz · Oyunbileg von Stackelberg · Mirjam Stahl · Bertram J. Jobst · Monika Eichinger · Michael Puderbach · Lutz Nährlich · Sandra Barth · Christian Schneider · Matthias V. Kopp · Isabell Ricklefs · Michael Buchholz · Burkhard Tümmler · Burkhard Tümmler · Christian Dopfer · Christian Dopfer · Jens Vogel-Claussen · Jens Vogel-Claussen · Hans-Ulrich Kauczor · Marcus A. Mall · Marcus A. Mall
A prospective analysis of unplanned patient-initiated contacts in an adult cystic fibrosis centre Steven L. Werlin · Virginie Scotet · Kevin Uguen · Marie-Pierre Audrézet · Michael Cohen · Yasmin Yaakov · Rifaat Safadi · Yaron Ilan · Fred M. Konikoff · Eitan Galun · Meir Mizrahi · Mordechai Slae · Shirley Sayag · Malena Cohen-Cymberknoh · Michael Wilschanski · Claude Férec · Claude Férec
Eradication of early P. aeruginosa infection in children <7 years of age with cystic fibrosis: The early study Espérie Burnet · Dominique Hubert · Juliette Champreux · Isabelle Honoré · R. Kanaan · Rosewilta Panzo · Pierre-Régis Burgel · Pierre-Régis Burgel
Pulmonary exacerbations and acute declines in lung function in patients with cystic fibrosis Felix Ratjen · Alexander Moeller · Martha L. McKinney · Irina Asherova · Nipa Alon · Robert Maykut · G. Angyalosi · Olga Bede · Katalin Bolbás · Vladimir Bulatov · Elena Chertok · Carla Colombo · Michael Fayon · Ferenc Gonczi · Essam Gouda · Jutta Hammermann · Anne Munck · Melinda Solomon · John Tsanakas · John Tsanakas
Insights into the cystic fibrosis care in Eastern Europe: Results of survey Jeffrey S. Wagener · Michael J. Williams · Stefanie J. Millar · Wayne J. Morgan · David J. Pasta · Michael W. Konstan · Michael W. Konstan
Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations Katarzyna Walicka-Serzysko · Monika Peckova · Jacquelien Noordhoek · Dorota Sands · Pavel Drevinek · Pavel Drevinek
Caregiver burden of parents of young children with cystic fibrosis James M. Roberts · Darlene L.Y. Dai · Zsuzsanna Hollander · Raymond T. Ng · Scott J. Tebbutt · Pearce G. Wilcox · Don D. Sin · Bradley S. Quon · Bradley S. Quon
Italian and North American dietary intake after ivacaftor treatment for Cystic Fibrosis Gating Mutations Jeffrey J. Wine · Gunnar C. Hansson · Peter König · Nam Soo Joo · Anna Ermund · Mario Pieper · Mario Pieper
Continuous glucose monitoring in youth with cystic fibrosis treated with lumacaftor-ivacaftor Chenguang Zhao · Jeff Crosby · Tinghong Lv · Dong Bai · Brett P. Monia · Shuling Guo · Shuling Guo
The Q359K/T360K mutation causes cystic fibrosis in Georgian Jews Rebecca Dezube · Mark T. Jennings · Rykiel Mf · Marie Diener-West · Michael P. Boyle · Michael P. Boyle · James F. Chmiel · Elliott C. Dasenbrook · Elliott C. Dasenbrook
R560S: A class II CFTR mutation that is not rescued by current modulators Insa Korten · Insa Korten · Elisabeth Kieninger · Sophie Yammine · Giulia Cangiano · Sylvia Nyilas · Pinelopi Anagnostopoulou · Florian Singer · Claudia E. Kuehni · Nicolas Regamey · Nicolas Regamey · Urs Frey · Carmen Casaulta · Ben D. Spycher · Philipp Latzin · Philipp Latzin
Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis Nikhil T. Awatade · Sofia Ramalho · Iris A.L. Silva · Verónica Felício · Hugo M. Botelho · Eyleen de Poel · Annelotte Vonk · Jeffrey M. Beekman · Carlos M. Farinha · Margarida D. Amaral · Margarida D. Amaral
Establishment of a ΔF508-CF promyelocytic cell line for cystic fibrosis research and drug screening Michael S. Schechter · H. Joel Schmidt · Ron Williams · Robert Norton · Deanna Taylor · Andrea Molzhon · Andrea Molzhon
Age-related heterogeneity in dental caries and associated risk factors in individuals with cystic fibrosis ages 6–20 years: A pilot study Scott Jennings · Hang Pong Ng · Guoshun Wang · Guoshun Wang
Using registry data to improve quality of care Donald L. Chi · Margaret Rosenfeld · Lloyd Mancl · Whasun O. Chung · Richard B. Presland · Elise W. Sarvas · Marilynn Rothen · Alaa Alkhateeb · Sharon McNamara · Alan Genatossio · Isabel Virella-Lowell · Carlos Milla · JoAnna Scott · JoAnna Scott
In statistics we trust: Towards the careful derivation and interpretation of meaningful survival estimates in cystic fibrosis Kieran McIntyre · Dominique Pougheon Bertrand · Gilles Rault · Gilles Rault
Understanding of safety monitoring in clinical trials by individuals with CF or their parents: A qualitative analysis Nicole Mayer-Hamblett · D. Polineni · Sonya L. Heltshe · Sonya L. Heltshe
Cystic fibrosis patient registries: A valuable source for clinical research Andrew S. Kern-Goldberger · Amanda J. Hessels · Lisa Saiman · Lynne M. Quittell · Lynne M. Quittell
Strategies for newborn screening for cystic fibrosis: A systematic review of health economic evaluations Elliott C. Dasenbrook · Gregory S. Sawicki · Gregory S. Sawicki
Sweat test for cystic fibrosis: Wearable sweat sensor vs. standard laboratory test Masja Schmidt · Amber Werbrouck · Amber Werbrouck · Nick Verhaeghe · Nick Verhaeghe · Elke De Wachter · Steven Simoens · Lieven Annemans · Koen Putman · Koen Putman
Early detection using qPCR of Pseudomonas aeruginosa infection in children with cystic fibrosis undergoing eradication treatment Dong Hoon Choi · Abigail Thaxton · In cheol Jeong · Kain Kim · Patrick R. Sosnay · Garry R. Cutting · Peter C. Searson · Peter C. Searson
New directions on lung clearance index variability and feasibility Ana C. Blanchard · Ashley Rooney · Yvonne Yau · Yu Zhang · Patrick Stapleton · Eric Horton · Michelle Klingel · Sanja Stanojevic · Felix Ratjen · Bryan Coburn · Valerie Waters · Valerie Waters
Cystic fibrosis epithelial cells are primed for apoptosis as a result of increased Fas (CD95) Katherine O’Neill · Clare Saunders · Clare Saunders
Comparison of facemask and mouthpiece interfaces for multiple breath washout measurements Qiwei Chen · Sudha Priya Soundara Pandi · Lauren Kerrigan · Noel G. McElvaney · Catherine M. Greene · J. Stuart Elborn · Clifford C. Taggart · Sinéad Weldon · Sinéad Weldon
Molecular characterization of gene regulatory networks in primary human tracheal and bronchial epithelial cells Paul Robinson · Paul Robinson · Sooky Lum · Courtney Moore · Kate M. Hardaker · Kate M. Hardaker · Nick Benseler · Paul Aurora · Paul Aurora · Peter Cooper · Dominic A. Fitzgerald · Dominic A. Fitzgerald · Renee Jensen · Reginald McDonald · Hiran Selvadurai · Hiran Selvadurai · Felix Ratjen · Sanja Stanojevic · Sanja Stanojevic
A comorbidity of CF in need of our attention and activity: Attention Deficit Hyperactivity Disorder! Austin E. Gillen · Rui Yang · Calvin U. Cotton · Aura Perez · Scott H. Randell · Shih Hsing Leir · Shih Hsing Leir · Ann Harris · Ann Harris · Ann Harris
Improvement in Exophiala dermatitidis airway persistence and respiratory decline in response to interferon-gamma therapy in a patient with cystic fibrosis Jurgen Lemiere · Trudy Havermans · Trudy Havermans
Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis Christopher P. Eades · Darius Armstrong-James · Jimstan Periselneris · Andrew Jones · N.J. Simmonds · Peter Kelleher · Anand Shah · Anand Shah
A guide to interpreting estimated median age of survival in cystic fibrosis patient registry reports Don B. Sanders · Zhumin Zhang · Philip M. Farrell · HuiChuan J. Lai · HuiChuan J. Lai
Risk factors for persistent Aspergillus respiratory isolation in cystic fibrosis Ruth H. Keogh · Sanja Stanojevic · Sanja Stanojevic
Chronic infection sustained by a Pseudomonas aeruginosa High-Risk clone producing the VIM-1 metallo-β-lactamase in a cystic fibrosis patient after lung transplantation Gina Hong · Kevin J. Psoter · Mark T. Jennings · Christian A. Merlo · Michael P. Boyle · Michael P. Boyle · Denis Hadjiliadis · Steven M. Kawut · Noah Lechtzin · Noah Lechtzin
Implementing the International Committee on Mental Health in Cystic Fibrosis (ICMH) guidelines: Screening accuracy and referral-treatment pathways Simona Pollini · Claudia Mugnaioli · D. Dolce · S. Campana · A.S. Neri · G. Taccetti · Gian Maria Rossolini · Gian Maria Rossolini
Mast cell tryptase changes with Aspergillus fumigatus – Host crosstalk in cystic fibrosis patients Marieke Verkleij · Derek de Winter · Margaret Anne Hurley · Janice Abbott · Janice Abbott
Young patients with cystic fibrosis demonstrate subtle alterations of the cardiovascular system Carine Gomez · Ania Carsin · Marion Gouitaa · Martine Reynaud-Gaubert · Jean-Christophe Dubus · Jean-Louis Mege · Stéphane Ranque · Joana Vitte · Joana Vitte
The effect of enteral tube feeding in cystic fibrosis: A registry based study Jacobien B. Eising · Cornelis K. van der Ent · Arco J. Teske · Maaike M. Vanderschuren · Cuno S.P.M. Uiterwaal · Folkert J. Meijboom · Folkert J. Meijboom
Positive clinical response to ivacaftor treatment in an individual with the CFTR genotype F508del/V456A Laurence Delhaes · Kada Touati · Odile Faure-Cognet · Muriel Cornet · Françoise Botterel · Eric DannaouiI · F. Morio · Patrice Le Pape · Frédéric Grenouillet · Loïc Favennec · Solène Le Gal · Gilles Nevez · Alain Duhamel · Andrew M. Borman · Veroniek Saegeman · Katrien Lagrou · Elia Gomez · Maiz-Luis Carro · Rafael Cantón · S. Campana · Walter Buzina · Sharon C.-A. Chen · Wieland Meyer · Emmanuel Roilides · Maria Simitsopoulou · Esther Manso · Lisa Cariani · A. Biffi · Ersilia Fiscarelli · Gabriella Ricciotti · Marc Pihet · Jean-Philippe Bouchara · Jean-Philippe Bouchara
Quantifying fluctuation in glucose levels to identify early changes in glucose homeostasis in cystic fibrosis Aaron Trimble · A. Whitney Brown · Beth L. Laube · Noah Lechtzin · Kirby L. Zeman · Jihong Wu · Agathe Ceppe · David A. Waltz · William D. Bennett · Scott H. Donaldson · Scott H. Donaldson
Respiratory rate in infants with cystic fibrosis throughout the first year of life and association with lung clearance index measured shortly after birth Meir Mei-Zahav · Patrick Stafler · H. Senderowitz · L. Bentur · L. Bentur · G. Livnat · Michal Shteinberg · N. Orenstein · N. Orenstein · L. Bazak · L. Bazak · Dario Prais · Hagit Levine · Michal Gur · N. Khazanov · L. Simhaev · H. Eliyahu · Michael Cohen · Michael Wilschanski · Hannah Blau · Huda Mussaffi · Huda Mussaffi
Eradication of persistent methicillin-resistant Staphylococcus aureus infection in cystic fibrosis Anna Sze Tai · Anna Sze Tai · Sue Morey · Sue Morey
One time quantitative PCR detection of Pseudomonas aeruginosa to discriminate intermittent from chronic infection in cystic fibrosis Traci M. Kazmerski · Gregory S. Sawicki · Elizabeth Miller · Kelley A. Jones · Kaleab Z. Abebe · Lisa Tuchman · Sigrid Ladores · Ronald C. Rubenstein · Scott D. Sagel · Daniel J. Weiner · Joseph M. Pilewski · David M. Orenstein · Sonya Borrero · Sonya Borrero
The open door policy - An important and under-recognised activity of the adult CF centre Angel Li · Tim Vigers · Laura Pyle · Edith T. Zemanick · Kristen J. Nadeau · Scott D. Sagel · Christine L. Chan · Christine L. Chan
AR-13 reduces antibiotic-resistant bacterial burden in cystic fibrosis phagocytes and improves cystic fibrosis transmembrane conductance regulator function Michal Shteinberg · Adi Ben Lulu · D.G. Downey · D.G. Downey · Zeev Blumenfeld · Christine Rousset-Jablonski · M. Perceval · Alessandra Colombo · Nili Stein · G. Livnat · Michal Gur · L. Bentur · Huda Mussaffi · Hannah Blau · Ifat Sarouk · Ifat Sarouk · Adi Dagan · Adi Dagan · Eitan Kerem · Micha Aviram · Elie Picard · Stefano Aliberti · Antonio Alvarez · Javier Perez Miranda · Eva Polverino · I. Durieu · J. Stuart Elborn · J. Stuart Elborn · Malena Cohen-Cymberknoh · Malena Cohen-Cymberknoh
Dysglycaemia in CF – Why are investigating it, what should we measure and how should we treat it? Kaivon Assani · Chandra L. Shrestha · Hannah Rinehardt · Shuzhong Zhang · Frank Robledo-Avila · Jack Wellmerling · Santiago Partida-Sanchez · Estelle Cormet-Boyaka · Susan D. Reynolds · Larry S. Schlesinger · Benjamin T. Kopp · Benjamin T. Kopp · Benjamin T. Kopp
Combined antifungal therapy is superior to monotherapy in pulmonary scedosporiosis in cystic fibrosis Amanda L. Brennan · Jennifer Beynon · Jennifer Beynon
IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation Carsten Schwarz · Claudia Brandt · Volker Melichar · Christoph Runge · Eberhard Heuer · Hany Sahly · Martin Schebek · Holger Köster · Jean-Philippe Bouchara · Thomas Biedermann · Peter Meißner · Jörg Große-Onnebrink · Heino Skopnik · Dominik Hartl · Ludwig Sedlacek · Kathrin Tintelnot · Kathrin Tintelnot
Mental Health screening in cystic fibrosis centres across Europe Ivo P. van de Peppel · Marcela Doktorova · G. Berkers · Hugo R. de Jonge · Roderick H. J. Houwen · Henkjan J. Verkade · Johan W. Jonker · Frank Bodewes · Frank Bodewes
Antimicrobial resistance in cystic fibrosis: Does it matter? Janice Abbott · Trudy Havermans · S. Jarvholm · E. Landau · Y. Prins · U. Smrekar · Doris Staab · L. Verity · M. Verkleij · M. Verkleij
Defining antimicrobial resistance in cystic fibrosis Patrick A. Flume · Valerie Waters · Scott C. Bell · Donald R. Van Devanter · J. Stuart Elborn · J. Stuart Elborn
Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV1) in cystic fibrosis patients receiving ivacaftor treatment T.J. Kidd · Rafael Cantón · Miquel B. Ekkelenkamp · Helle Krogh Johansen · John J. LiPuma · Scott C. Bell · J. Stuart Elborn · Patrick A. Flume · Donald R. VanDevanter · Valerie Waters · Valerie Waters
Phenotypic spectrum of patients with cystic fibrosis and cystic fibrosis-related disease carrying p.Arg117His Béla Nagy · Zsolt Bene · Zsolt Fejes · Sonya L. Heltshe · Dw Reid · N.J. Ronan · Yvonne McCarthy · Daniel Smith · Attila Nagy · Elizabeth Joseloff · György Balla · György Balla · János Kappelmayer · Milan Macek · Scott C. Bell · B.J. Plant · Margarida D. Amaral · Istvan Balogh · Istvan Balogh
False-positive cannabinoid screens in adult cystic fibrosis patients treated with lumacaftor/ivacaftor Katherine Keenan · Annie Dupuis · Katherine Griffin · Carlo Castellani · Elizabeth Tullis · Elizabeth Tullis · Tanja Gonska · Tanja Gonska
Successful Lung Re-transplant in a Patient with Cepacia Syndrome due to Burkholderia ambifaria Dana G. Kissner · Yvette LeFlore · Srinivas B. Narayan · Gautham Marigowda · Christopher Simard · Cecile Le Camus · Cecile Le Camus
Magnetic resonance elastography demonstrates elevated liver stiffness in cystic fibrosis patients Kellie J. Goodlet · Michael D. Nailor · Ashraf Omar · Jasmine Huang · John J. LiPuma · Rajat Walia · Sofya Tokman · Sofya Tokman
Breathlessness catastrophizing relates to poorer quality of life in adults with cystic fibrosis Don Hayes · Don Hayes · Ramkumar Krishnamurthy · Houchun Harry Hu · Houchun Harry Hu
Bile acid homeostasis in gastrointestinal and metabolic complications of cystic fibrosis Danijela Maras · Danijela Maras · Louise Balfour · Giorgio A. Tasca · Giorgio A. Tasca · Ena Gaudet · Shawn D. Aaron · Shawn D. Aaron · Shawn D. Aaron · William Cameron · William Cameron · William Cameron · Smita Pakhale · Smita Pakhale · Smita Pakhale · Smita Pakhale
Anaemia and iron deficiency in relation to fatigue in cystic fibrosis Ivo P. van de Peppel · Frank Bodewes · Henkjan J. Verkade · Johan W. Jonker · Johan W. Jonker
Rate and predictors of prescription of lumacaftor – Ivacaftor in the 18 months following approval in the United States N P Talbot · W G Flight · W G Flight
Correspondence between symptoms and preference-based health status measures in the STOP study Gregory S. Sawicki · Aliza K. Fink · Michael S. Schechter · Deena R. Loeffler · Nicole Mayer-Hamblett · Nicole Mayer-Hamblett
Use of telavancin in adolescent patients with cystic fibrosis and prior intolerance to vancomycin: A case series Laura S. Gold · Donald L. Patrick · Ryan N. Hansen · Valeria V. Beckett · Christopher H. Goss · Larry Kessler · Larry Kessler
Prevalence, geographic risk factor, and development of a standardized protocol for fungal isolation in cystic fibrosis: Results from the international prospective studyMFIP Sylvia Nyilas · Grzegorz Bauman · Orso Pusterla · Kathryn Angela Ramsey · Florian Singer · Enno Stranzinger · Sophie Yammine · Carmen Casaulta · Oliver Bieri · Philipp Latzin · Philipp Latzin
Differential expression of genes and receptors in monocytes from patients with cystic fibrosis C. Vasquez · J. Jurado · A. Rincon · N. Lung · A. Ovalle · M. Gantivar · D. Rivera · R. Casanova · R. Casanova
Is there an association between back pain and stress incontinence in adults with cystic fibrosis? A retrospective cross-sectional study Moya Vandeleur · Moya Vandeleur · Lisa M. Walter · David S. Armstrong · Philip J. Robinson · Gillian M. Nixon · Gillian M. Nixon · Rosemary S.C. Horne · Rosemary S.C. Horne
Investigating the effects of long-term dornase alfa use on lung function using registry data D.R. VanDevanter · J.S. Elborn · R.W. van Olden · P.A. Flume · J. Szwarcberg · J. Szwarcberg
Lumacaftor/Ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV1 Mette Kolpen · T. Qvist · Niels Høiby · Peter Østrup Jensen · Peter Østrup Jensen
Newborn blood spot screening for cystic fibrosis with a four-step screening strategy in the Netherlands Susanna A. McColley · Michael W. Konstan · Bonnie W. Ramsey · J. Stuart Elborn · Michael P. Boyle · Claire Wainwright · David A. Waltz · Montserrat Vera-Llonch · Gautham Marigowda · John G. Jiang · Jaime Rubin · Jaime Rubin
N1303K: Leaving no stone unturned in the search for transformational therapeutics J.E. Dankert-Roelse · Marelle J. Bouva · Bernadette S. Jakobs · Hettie M. Janssens · Karin M. de Winter-de Groot · Yvonne Schönbeck · Johan J. P. Gille · Vincent Gulmans · Rendelien K. Verschoof-Puite · Peter Schielen · P.H. Verkerk · P.H. Verkerk
Patient-reported outcomes: Time for a new approach? Sabrina Noel · Sabrina Noel · I. Sermet-Gaudelus · I. Sermet-Gaudelus · David N. Sheppard · David N. Sheppard
Antisense oligonucleotide targeting of mRNAs encoding ENaC subunits α, β, and γ improves cystic fibrosis-like disease in mice Janice Abbott · Janice Abbott
Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis Rossa Brugha · Marie Wright · Suzie Nolan · Nicola Bridges · Siobhán B. Carr · Siobhán B. Carr
Hypertonic saline has a prolonged effect on mucociliary clearance in adults with cystic fibrosis Denis Libeert · Dimitri Declercq · S. Wanyama · M. Thomas · Sabine Van daele · Frans De Baets · Stephanie Van Biervliet · Stephanie Van Biervliet
Initial development and pilot testing of observer-reported outcomes (ObsROs) for children with cystic fibrosis ages 0–11 years Sébastien Boutin · Sébastien Boutin · Michael Weitnauer · Selina Hassel · Selina Hassel · Simon Y. Graeber · Mirjam Stahl · A. Susanne Dittrich · A. Susanne Dittrich · Marcus A. Mall · Alexander H. Dalpke · Alexander H. Dalpke · Alexander H. Dalpke
Another step in the journey: From CFTR mutation to sweat chloride concentration to survival Jan C. Thomassen · Matthias I. Mueller · Miguel A. Alejandre Alcazar · Ernst Rietschel · Silke van Koningsbruggen-Rietschel · Silke van Koningsbruggen-Rietschel
The relationship between sweat chloride levels and mortality in cystic fibrosis varies by individual genotype Natasha Armaghanian · Natasha Armaghanian · Tania P. Markovic · Tania P. Markovic · Jennie Brand-Miller · Peter Bye · Carmel Moriarty · Kate Steinbeck · Kate Steinbeck · Kate Steinbeck · Kate Steinbeck
Pre-transplant factors associated with mortality after lung transplantation in cystic fibrosis: A systematic review and meta-analysis F. Frost · D. Nazareth · Matthew Shaw · M.J. Walshaw · M.J. Walshaw
Authors response: Letter to the Editor Anaemia and iron deficiency in relation to fatigue in cystic fibrosis Angela Koutsokera · Angela Koutsokera · Angela Koutsokera · Rhea Varughese · Rhea Varughese · Jenna Sykes · Ani Orchanian-Cheff · Prakesh S. Shah · C. Chaparro · C. Chaparro · Elizabeth Tullis · Lianne G. Singer · Anne Stephenson · Anne Stephenson
How to live a long and healthy life with cystic fibrosis: Lessons from the CF ferret M.M. Nap-van der Vlist · M. Burghard · H.J. Hulzebos · W.R. Doeleman · Harry Heijerman · C.K. van der Ent · S.L. Nijhof · S.L. Nijhof
Seasonal fluctuation of lung function in cystic fibrosis: A national register-based study in two northern European populations Jeffrey J. Wine · Jeffrey J. Wine
3D Printing and the Cystic Fibrosis Lung T. Qvist · Daniela K. Schlüter · Vian Rajabzadeh · Peter J. Diggle · Tania Pressler · Siobhán B. Carr · David Taylor-Robinson · David Taylor-Robinson
The prevalence, clinical status and genotype of cystic fibrosis patients living in Cuba using national registry data Ute Oltmanns · Karin Palmowski · Mark O. Wielpütz · Nicolas Kahn · Eva Baroke · Ralf Eberhardt · Sabine Wege · Matthias Wiebel · Michael Kreuter · Felix Herth · Marcus A. Mall · Marcus A. Mall
SWATH label-free proteomics for cystic fibrosis research Fidel Rodríguez-Calá · Ramón Suárez-Medina · Silvia Josefina Venero-Fernández · Alan Smyth · Siobhán B. Carr · Andrew W. Fogarty · Andrew W. Fogarty
Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data Todd C. Edwards · Julia Emerson · Alan Genatossio · Sharon McNamara · C.H. Goss · Donald L. Patrick · F. Onchiri · Margaret Rosenfeld · Margaret Rosenfeld
Ventilation and perfusion assessed by functional MRI in children with CF: reproducibility in comparison to lung function Sarath Ranganathan · Sarath Ranganathan · Sarath Ranganathan
Gene editing & stem cells M.A.G.M. Kroon · A.M. Akkerman-Nijland · Bart L. Rottier · Gerard H. Koppelman · Onno W. Akkerman · Daan Touw · Daan Touw
Drugs during pregnancy and breast feeding in women diagnosed with Cystic Fibrosis - An update Donald R. VanDevanter · Nicole Mayer-Hamblett · Nicole Mayer-Hamblett
Attenuation of exacerbation Susanna Esposito · Carla Colombo · A. Tosco · Sonia Volpi · Luca Ruggiero · Mara Lelii · Arianna Bisogno · Claudio Pelucchi · Nicola Principi · Nicola Principi
Failure to conceive in women with CF is associated with pancreatic insufficiency and advancing age Preston E. Bratcher · Kenneth C. Hunt · Kyle Pickard · Jennifer L. Taylor-Cousar · Jennifer L. Taylor-Cousar
Combined lung-liver-pancreas transplantation in a recipient with cystic fibrosis S. Evans · H. Bae · A. Sayers · P. Crighton · D. Lau · R.I. Ketchell · L. Speight · J. Duckers · J. Duckers
383 Blood sampling for children with CF: buzzy or not? Helge Hebestreit · E. Hulzebos · Jane E. Schneiderman · C. Karila · Steven R. Boas · Susi Kriemler · Tiffany Dwyer · M. Sahlberg · Don S. Urquhart · Larry C. Lands · Felix Ratjen · Tim Takken · L. Varanistkaya · V. Rücker · Alexandra Hebestreit · Thomas Radtke · Thomas Radtke
135 Is CF related diabetes associated with increased prevalence of Stenotrophomonas maltophilia I. Heeres · I. Heeres
EPS5.6 Temporal trends in key outcome measures in cystic fibrosis patients treated with ivacaftor: real-world data from the Irish CF registry F. Frost · J.L. Fothergill · Matthew Shaw · M.J. Ledson · J. Greenwood · Craig Winstanley · M.J. Walshaw · M.J. Walshaw
EPS5.3 Age and race disparities among pediatric and adult cystic fibrosis lung transplant recipients L. Kirwan · G. Fletcher · M. Harrington · S. Zhou · P. Jeleniewska · Edward F. McKone · Abaigeal D. Jackson · Abaigeal D. Jackson
Therapeutic benefit of ivacaftor in late cystic fibrosis caused by homozygous IVS8-5T CFTR polymorphism Karl Kunzelmann · Rainer Schreiber · Hans Beat Hadorn · Hans Beat Hadorn
Bicarbonate in cystic fibrosis Mehdi Hegagi · Shawn D. Aaron · Paul D. James · Rakesh Goel · Avijit Chatterjee · Avijit Chatterjee
Increased prevalence of colonic adenomas in patients with cystic fibrosis Erfan Ayubi · Jamal Hallajzadeh · Saeid Safiri · Saeid Safiri · Saeid Safiri
Comment on data sparsity - Diagnostic and prognostic significance of systemic alkyl quinolones for P. aeruginosa in cystic fibrosis: A longitudinal study Millie Garg · Steven T. Leach · Tamara Pang · Bronwen Needham · Michael J. Coffey · Tamarah Katz · Roxanne Strachan · John Widger · John Widger · Penelope Field · Penelope Field · Yvonne Belessis · Yvonne Belessis · Sandra Chuang · Sandra Chuang · Andrew S. Day · Adam Jaffe · Adam Jaffe · Chee Y. Ooi · Chee Y. Ooi · Chee Y. Ooi
408 Moving the UK CF registry from annual review data collection to encounter-based data collection: cost estimation to understand feasibility Carsten Schwarz · Claudia Brandt · Doris Staab · Doris Staab
Bile salt stimulated lipase: Inhibition by phospholipids and relief by phospholipase A2 Siddhartha G. Kapnadak · Gregory Kicska · Kathleen J. Ramos · Desiree A. Marshall · Tamara Y. Carroll · Sudhakar Pipavath · Michael S. Mulligan · Christopher H. Goss · Moira L. Aitken · Moira L. Aitken
Tracheal diverticula in advanced cystic fibrosis: Prevalence, features, and outcomes after lung transplantation Traci M. Kazmerski · Gregory S. Sawicki · Elizabeth Miller · Kelley A. Jones · Kaleab Z. Abebe · Lisa Tuchman · Sigrid Ladores · Ronald C. Rubenstein · Ronald C. Rubenstein · Scott D. Sagel · Daniel J. Weiner · Joseph M. Pilewski · David M. Orenstein · Sonya Borrero · Sonya Borrero
189 Lung function improvement in children with cystic fibrosis admitted for intravenous antibiotics S. Forshaw · Z.H. Hoo · Rachael Curley · M. Wildman · M. Wildman
Poor recovery from a pulmonary exacerbation does not lead to accelerated FEV1 decline Sabrina Noël · Nadtha Panin · Mathilde Beka · Amandine Collin · Sophie Gohy · Marcus A. Mall · Teresinha Leal · Teresinha Leal
38 Relationship between planar cell polarity protein network signaling and airway remodeling in CF Luke W. Garratt · Anthony Kicic · C. F. Robertson · C. F. Robertson · Sarath Ranganathan · Sarath Ranganathan · Peter D. Sly · Stephen M. Stick · Stephen M. Stick
The AREST CF experience in biobanking — More than just tissues, tubes and time Sanja Stanojevic · Sanja Stanojevic
Interpretation of Cystic Fibrosis Centre rankings: Meaningful comparisons or biased statistics? Daniel Stevens · Daniel Stevens
WS02.4 A peripheral blood transcriptional profile predicts disease severity and the risk of future exacerbations in adults with cystic fibrosis (CF) D. Gomez-Pastrana · C. Nwokoro · I. Delgado · C. Aragon · V. Chorro-Mari · M.E. Quintana-Gallego · C. Pao · C. Pao
Exploring probiotic use in a regional cystic fibrosis consortium Gautham Marigowda · Fang Liu · David A. Waltz · David A. Waltz
Effect of bronchodilators in healthy individuals receiving lumacaftor/ivacaftor combination therapy Marcus Svedberg · Per M. Gustafsson · Paul Robinson · Monica Rosberg · Anders Lindblad · Anders Lindblad
Variability of lung clearance index in clinically stable cystic fibrosis lung disease in school age children Mirjam Stahl · Simon Y. Graeber · Cornelia Joachim · Sandra Barth · Isabell Ricklefs · Gesa Diekmann · Matthias V. Kopp · Lutz Naehrlich · Marcus A. Mall · Marcus A. Mall · Marcus A. Mall
Three-center feasibility of lung clearance index in infants and preschool children with cystic fibrosis and other lung diseases Manuella Lévêque · Manuella Lévêque · Sophie Le Trionnaire · Sophie Le Trionnaire · Paola Del Porto · Corinne Martin-Chouly · Corinne Martin-Chouly · Corinne Martin-Chouly
The impact of impaired macrophage functions in cystic fibrosis disease progression Sebastian Fischer · Leonie Greipel · Jens Klockgether · Marie Dorda · Lutz Wiehlmann · Nina Cramer · Burkhard Tümmler · Burkhard Tümmler
WS05.3 Persistent early life growth patterns through age 12 years are associated with pulmonary outcomes P. Wark · K.A. Goon · D. Dorahy · R. Baran · T. Coleman · K. Baines · K. Baines
186 First experience with a new inhaled antibiotic treatment R.E. Sutherland · Tamarah Katz · V. Liu · J. Quintano · Rebecca Brunner · C. Tong · Clare E. Collins · Chee Y. Ooi · Chee Y. Ooi
29 Validating the organoid model across European laboratories A. Cowley · Alex Horsley · D. Denning · David J. Thornton · David J. Thornton
141 Aspergillus fumigatus degrades the respiratory mucins MUC5B and MUC5AC Michal Shteinberg · D.G. Downey · D. Beattie · J. McCaughan · A. Reid · Nili Stein · J.S. Elborn · J.S. Elborn
WS08.6 Cardiopulmonary exercise testing provides additional prognostic information in people with cystic fibrosis J. Roehmel · P. Specht · L. Lange · C. Schwarz · A. Salama · B. Mayer · B. Mayer
21 Lung function and disease severity in CF patients heterozygous for p. Arg117His E. Gudumac · S. Sciuca · L. Balanetchi · R. Selevestru · R. Selevestru
242 Pneumothorax in patients with cystic fibrosis Sophie Gohy · A. Scohy · S. Berardis · F. Bressant · A. Zitouni · Anne-Sophie Aubriot · N. Bauwens · E. Andre · Patrick Lebecque · Patrick Lebecque
107 RGM medium versus MGIT™ for isolation of nontuberculous mycobacteria from sputum of patients with cystic fibrosis T. Pressler · S. Donaldson · K.T. Smerud · A.H. Myrset · A.H. Myrset
54 Relationship between rate of percent predicted FEV1 (ppFEV1) decline and baseline and acute change in ppFEV1 in patients (pts) with cystic fibrosis (CF) treated with lumacaftor/ivacaftor (LUM/IVA) P. van Mourik · G. Berkers · A.M. Vonk · A.S. Ramalho · N.T. Awatade · C.K. van der Ent · K. De Boeck · Margarida D. Amaral · Jeffrey M. Beekman · Jeffrey M. Beekman
WS01.3 A double blind, randomised, placebo-controlled cross over study of inhaled alginate oligosaccharide (OligoG) administered for 28 days in subjects with cystic fibrosis Cameron J. McKinzie · Jennifer L. Goralski · Terry L. Noah · George Z. Retsch-Bogart · Mary Beth Prieur · Mary Beth Prieur
Worsening anxiety and depression after initiation of lumacaftor/ivacaftor combination therapy in adolescent females with cystic fibrosis Lasantha Gunasekara · Mustafa Al-Saiedy · Francis H. Y. Green · Ryan Pratt · Candice Bjornson · Ailian Yang · W. Michael Schoel · Ian Mitchell · Mary Brindle · Mark Montgomery · Elizabeth Keys · John Dennis · Grishma Shrestha · Matthias Amrein · Matthias Amrein
WS12.3 End-of-life-issues – a single-center retrospective analysis Michael W. Konstan · Edward F. McKone · Richard B. Moss · B. Lubarsky · Jaime Rubin · Stefanie J. Millar · David J. Pasta · Nicole Mayer-Hamblett · Christopher H. Goss · Wayne J. Morgan · Gregory S. Sawicki · Gregory S. Sawicki
404 SPARC questionnaire: identifying needs in people with cystic fibrosis (CF) attending the All Wales Adult CF Centre (AWACFC) P. Eschenhagen · N. Albrecht · D. Staab · C. Schwarz · C. Schwarz
Pulmonary surfactant dysfunction in pediatric cystic fibrosis: Mechanisms and reversal with a lipid-sequestering drug Tracey Bonfield · James F. Chmiel · James F. Chmiel
Impaired innate immune cells in cystic fibrosis: Is it really a surprise? Susan P. Wolfe · Clare E. Collins · Clare E. Collins
The changing face of nutrition in cystic fibrosis T.B. Inman · J.A. Proudfoot · M. Lim · M. Lim · C. Demeterco-Berggren · C. Demeterco-Berggren · C. Demeterco-Berggren
Continuous glucose monitoring in a cystic fibrosis patient to predict pulmonary exacerbation Marianne S. Muhlebach · John P. Clancy · Sonya L. Heltshe · Assem Ziady · Tom Kelley · Frank J. Accurso · Joseph M. Pilewski · Nicole Mayer-Hamblett · Nicole Mayer-Hamblett · Elizabeth Joseloff · Scott D. Sagel · Scott D. Sagel
EPS1.2 Transduction of rhesus macaque lung by AAV1 O. Gwilym · A. Sayers · N. Hassan · A. Prosser · L. Speight · R.I. Ketchell · D. Lau · A. Byrne · J. Duckers · J. Duckers
A single bout of maximal exercise improves lung function in patients with cystic fibrosis Aukje C. Bos · Kimberly M. Passé · Johan W. Mouton · Hettie M. Janssens · Harm A.W.M. Tiddens · Harm A.W.M. Tiddens
The fate of inhaled antibiotics after deposition in cystic fibrosis: How to get drug to the bug? Patrick A. Flume · Claire Wainwright · Claire Wainwright · D. Elizabeth Tullis · Sally Rodriguez · Minoo Niknian · M. Higgins · Jane C. Davies · Jane C. Davies · Jeffrey S. Wagener · Jeffrey S. Wagener
178 Piperacillin-induced hemolytic anemia – a frequent complication of treatment in patients with cystic fibrosis Philip M. Farrell · Don B. Sanders · Zhumin Zhang · HuiChuan J. Lai · HuiChuan J. Lai
150 Use of pseudomonas serum antibodies in a tertiary cystic fibrosis centre William B. Guggino · John F. Engelhardt · Z. Yan · Liudmila Cebotaru · Liudmila Cebotaru
Investigating the variation in the incidence of new Pseudomonas aeruginosa infection between paediatric cystic fibrosis centres John J. Brewington · Erin T. Filbrandt · F.J. LaRosa · Alicia J. Ostmann · Lauren M. Strecker · Rhonda D. Szczesniak · John P. Clancy · John P. Clancy
81 Specific IgE production against Staphylococcus aureus, Escherichia coli, Hemophilus influenzae and pneumococci in cystic fibrosis patients N.R. Lawrence · F.P. Edenborough · F.P. Edenborough
The potential of phage therapy in cystic fibrosis: Essential human-bacterial-phage interactions and delivery considerations for use in Pseudomonas aeruginosa-infected airways Insa Korten · Insa Korten · Margot Liechti · Florian Singer · Florian Singer · Gaudenz Hafen · Isabelle Rochat · Pinelopi Anagnostopoulou · Dominik Müller-Suter · Jakob Usemann · Jakob Usemann · Alexander Moeller · Urs Frey · Philipp Latzin · Philipp Latzin · Carmen Casaulta · Carmen Casaulta
322 Dietary intake of energy-dense, nutrient-poor, and nutrient-dense food sources in Australian children with cystic fibrosis A.-M. Dittrich · S. Dzoro · M. Albrecht · C. Dopfer · A. Sauer-Heilborn · F. Ringshausen · B. Tümmler · R. Valenta · B.J. Hales · I. Mitterman · I. Mitterman
The evolution of lung transplantation for cystic fibrosis: A 2017 update Vicky A. LeGrys · T.C. Moon · J. Laux · Michael J. Rock · Frank J. Accurso · Frank J. Accurso
377 Bridging the educational gap between specialist nurse and ward nurse K.J. Bayfield · Ewfw Alton · S. Irving · Andrew Bush · Alex Horsley · Jane C. Davies · Jane C. Davies
Increased risk of PTLD in lung transplant recipients with cystic fibrosis Don B. Sanders · George M. Solomon · Valeria V. Beckett · Natalie E. West · Cori L. Daines · Sonya L. Heltshe · Donald R. VanDevanter · Jonathan E. Spahr · Ronald L. Gibson · Jerry A. Nick · Bruce C. Marshall · Patrick A. Flume · Christopher H. Goss · Christopher H. Goss
Standardized Treatment of Pulmonary Exacerbations (STOP) study: Observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations George M. Solomon · Bo Liu · Isabelle Sermet-Gaudelus · Isabelle Sermet-Gaudelus · Isabelle Fajac · Michael Wilschanski · F. Vermeulen · Steven M. Rowe · Steven M. Rowe
277 Hypoglycaemia in cystic fibrosis in the absence of diabetes at the All Wales Adult CF Centre (AWACFC) F. Frost · R. Trafford · J. Greenwood · M.J. Ledson · D. Nazareth · M.J. Walshaw · M.J. Walshaw
EPS7.10 Clinical benefit of continuous nebulised Aztreonam Lysine for Inhalation (AZLI) in adults with cystic fibrosis – a retrospective cohort study J. Lee · L. Speight · L. George · J. Duckers · David C.W. Lau · R.I. Ketchell · F.S. Wong · F.S. Wong
EPS1.3 Safety, tolerability and early signs of efficacy with riociguat for the treatment of adult Phe508del homozygous cystic fibrosis patients: study design and rationale for the Rio-CF study R. Morton · S. Doe · W. Banya · N.J. Simmonds · N.J. Simmonds
323 Evaluation of nutritional status in cystic fibrosis (CF) patients with percutaneous endoscopic gastrostomy (PEG) followed up at Tuscan center N. Derichs · J.L. Taylor-Cousar · E. Tullis · J.C. Davies · D. Nazareth · D.G. Downey · D. Rosenbluth · I. Fajac · A. Malfroot · M. Bakker · J.P. Clancy · A. Uluer · C. Castellani · I. Sermet-Gaudelus · R.C. Ahrens · S. Sutharsan · T. Welte · T. Gust · A. Kaiser · A. Hoffmann · F. Ratjen · S.M. Rowe · S.M. Rowe
430 Transition in CF integrated teams working together E. Roselli · M.L. Guidotti · V. Galici · C. Braggion · C. Braggion
70 Making clinical research a priority. The development of a research team within the adult cystic fibrosis service: A four year review of research activity and future prospects N. West · A. Thaxton · E. Tallarico · E. Bush · P. Shah · Christian A. Merlo · Christian A. Merlo
165 Modeling polymicrobial infections in the pathogenesis of cystic fibrosis lung disease G. Parallada · A. Rey · G. Casavieja · D. Cosentino · D. Montero · P. Ramirez · S. Guillen · G. Rutz · M.N. Fontan · A. Ramos · R. Mendez · R. Mendez
EPS1.6 Safety and pharmacokinetics of 14-day dosing with SPX-101 in healthy human subjects Cristina Cigana · Irene Bianconi · M. De Simone · Camilla Riva · Rossella Baldan · Barbara Sipione · Daniela M. Cirillo · Alessandra Bragonzi · Alessandra Bragonzi
357 Factors associated with changes in health-related quality of life in children with cystic fibrosis during one-year follow-up A. Wheeler · D. Scott · T. Crowder · P. Couroux · P. Couroux
EPS3.10 Pregnancy in women with CF: Improved clinical outcomes 1990–2016 M. Van Horck · Bjorn Winkens · Geertjan Wesseling · K.M. de Winter-de Groot · I. de Vreede · Rijn Jobsis · Edward Dompeling · Edward Dompeling
IPD1.01 Non-tuberculous mycobacteria infection in children with cystic fibrosis – experience from a large tertiary centre in United Kingdom C. Etherington · A.M. Morton · I. Clifton · P. Whitaker · D. Peckham · D. Peckham
400 Looking at sexuality aspects after lung transplantation J. Gooch · L. Turnbull · C. Fullwood · A. Maitra · A. Maitra
320 Changes in anthropometrical parameters in infants with CF diagnosed through newborn screening (NBS) P. Catastini · S. De Masi · C. Braggion · C. Braggion
218 Lack of T cells reduces structural lung damage in mice with cystic fibrosis-like lung disease A. Jokic Miletic · G. Vilotijevic-Dautovic · S. Cegar · S. Petrovic · S. Ilic · D. Tenjovic · D. Tenjovic
WS06.5 Evaluation of the physical, emotional and social burden of the caregiver of the child with cystic fibrosis Matthias Hagner · Simone Schmidt · S Christochowitz · Marcus A. Mall · Marcus A. Mall
WS13.4 Efficacy and safety of lumacaftor/ivacaftor (LUM/IVA) in patients (pts) aged 6–11 years (yrs) with cystic fibrosis (CF) homozygous for F508del-CFTR: a randomized placebo (PBO)-controlled phase 3 trial M.A. Rangel · C. Ferraz · L.G. Vaz · L.G. Vaz
10 Detection of false negative cases by cystic fibrosis screening a brief review of our experience Felix Ratjen · S. Tian · Gautham Marigowda · C. Hug · Xiaohong Huang · S. Stanojevic · Carlos Milla · P. Robinson · David A. Waltz · Jane C. Davies · Jane C. Davies
426 The study of CFTR mutations in healthy populations of Karachay-Cherkessia Republic M. Ferro Conde · L. González García · M.E. Quintana-Gallego · J. Mora Vallellano · C. Delgado-Pecellín · A.I.Álvarez Rios · I. Delgado-Pecellín · I. Delgado-Pecellín
WS08.1 How do we navigate the transition from cystic fibrosis to lung transplant A.-B. Thinggaard · D. Damar · W. Ridderberg · N. Nørskov-Lauritsen · N. Nørskov-Lauritsen
WS01.4 A randomised, double-blind, placebo-controlled, parallel-group, dose-escalation study of inhaled single doses of POL6014, a potent and selective reversible inhibitor of human neutrophil elastase (NE), in cystic fibrosis (CF) patients T.A. Vasilyeva · N.V. Petrova · N.Y. Kashirskaya · A.K.-M. Makaov · R.A. Bikanov · E.K. Ginter · R.A. Zinchenko · R.A. Zinchenko
291 Colon cancer screening program in adult CF patients: an Italian cross-sectional pilot study L. Hooftman · E. Chevalier · A. Wach · J. Zimmermann · P. Bruijnzeel · N. Naue · I. Heimbeck · D. Kappeler · P. Barth · P. Barth
130 Mechanism of β-lactam resistance in Achromobacter xylosoxidans L.M. MacDougal · G. Brady · G. Brady
232 Lumacaftor/Ivacaftor is associated with a significant improvement in walk test and reduction in sweat chloride in a cohort of homozygous F508del CF patients with severe disease – a single centre experience S. Tedesco · B. Fabrizzi · N. Caporelli · M. Marzioni · P. Moretti · L. Rodella · C. Castellani · M. Cipolli · M. Cipolli
240 Descriptive analysis of atelectasis as a pulmonary complication in cystic fibrosis N.J. Ronan · Y. McCarthy · K. James · P. Arooj · E.B. Hunt · C. Shortt · C. Fleming · M. McCarthy · C. Howlett · C. Hickey · E.T. Flanagan · M. Daly · Joseph A. Eustace · Michael M. Maher · B.J. Plant · B.J. Plant
235 Association of the first phase xenobiotic biotransformation system genes with the risk of undesirable side effects and efficacy of antibiotic therapy in Russian CF children M. Martínez-Redondo · C. Prados Sánchez · Francisco García-Río · M.E. Quintana-Gallego · Silvia Castillo · A. Salcedo Posadas · Rosa María Girón · M.T. Martínez Martínez · Luis Máiz · Cosette Martin · J. Costa · J. Costa
72 Neutrophil extracellular trap formation triggered by Pseudomonas aeruginosa: relevance to cystic fibrosis lung disease O.G. Novoselova · N. Petrova · E. Kondratyeva · V. Sherman · R.A. Bikanov · R.A. Zinchenko · R.A. Zinchenko
237 Multidisciplinary treatment with ECMO in cystic fibrosis – RPAH experience B. Rada · M. Floyd · M. Winn · B. Chassaing · A.T. Gewirtz · J.B. Goldberg · L.L. McCarter · L.L. McCarter
EPS6.5 Detecting changes in health in cystic fibrosis – a role for smartphones? F. Finlayson · John Wilson · Michelle Gold · Michelle Gold
356 Potential utility of the Supportive and Palliative Care Indicators Tool (SPICT™) in end-of-life care for non-transplanted adults (NTxA) with cystic fibrosis (CF) E. Glennon · C. Roden · J. Barrett · T. Osborne · H. Sunsoa · R. Rashid · J.L. Whitehouse · E.F. Nash · E.F. Nash
328 Going the distance – patient opinion on an adult CF centre dietetic and diabetes home visit service S. Martinez-Barona · E. Masip · B. Polo · E. Donat · Carmen Ribes-Koninckx · Carmen Ribes-Koninckx
261 The prevalence of severe fatigue in cystic fibrosis Mirjam Stahl · Cornelia Joachim · Mark O. Wielpütz · Marcus A. Mall · Marcus A. Mall
IPD2.15 Self-reported motivators to treatment adherence in adults with cystic fibrosis in the UK H. Maredia · M.G. Bowring · A. Massie · S. Oyetunji · Christian A. Merlo · Robert S.D. Higgins · D. Segev · E. Bush · E. Bush
A multiple reader scoring system for Nasal Potential Difference parameters Natalie E. West · Valeria V. Beckett · Raksha Jain · Don B. Sanders · Jerry A. Nick · Sonya L. Heltshe · Sonya L. Heltshe · Elliott C. Dasenbrook · Donald R. VanDevanter · George M. Solomon · Christopher H. Goss · Christopher H. Goss · Patrick A. Flume · Patrick A. Flume
Standardized Treatment of Pulmonary exacerbations (STOP) study: Physician treatment practices and outcomes for individuals with cystic fibrosis with pulmonary exacerbations Julia A. Nightingale · Clive Osmond · Clive Osmond
What keeps children with cystic fibrosis awake at night Jeffrey M. Beekman · Chiuhui Mary Wang · Sara Casati · Katherine L. Tuggle · Vincent Gulmans · Margarida D. Amaral · Kris De Boeck · Kris De Boeck
EPS4.6 Inherent differences in multiple breath washout (MBW) using N 2 and SF 6 demonstrated by simultaneous analysis with respiratory mass spectrometry (RMS) Sheila Sivam · Ruth Dentice · Carmel Moriarty · V. Yozghatlian · C. Mellis · A. Glanville · Paul J. Torzillo · D. Gattas · Peter Bye · Peter Bye
Biobanking: towards increased access of biomaterials in cystic fibrosis. Report on the pre-conference meeting to the 13th ECFS Basic Science Conference, Pisa, 30 March-2 April, 2016 Alison DaCosta · Cameron L. Jordan · Olivia Giddings · Feng Chang Lin · Charles R. Esther · Charles R. Esther
Outcomes associated with antibiotic regimens for treatment of Mycobacterium abscessus in cystic fibrosis patients Aurélie Labaste · Camille Ohlmann · Catherine Mainguy · Virginie Jubin · M. Perceval · Laurianne Coutier · Laurianne Coutier
Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis Jeff Crosby · Chenguang Zhao · Chong Jiang · Dong Bai · Melanie Katz · Sarah Greenlee · Hiroshi Kawabe · Michael L. McCaleb · Daniela Rotin · Shuling Guo · Brett P. Monia · Brett P. Monia
Inhaled ENaC antisense oligonucleotide ameliorates cystic fibrosis-like lung disease in mice Michelle N. Eakin · Shang En Chung · Jessica L. Hoehn · Belinda Borrelli · Devin Rand-Giovannetti · Kristin A. Riekert · Kristin A. Riekert
Development and validation of CF-Medication Beliefs Questionnaire: A mixed-methods approach Andrew S. Barbas · M.J. Dib · David P. Al-Adra · Nicolas Goldaracena · Gonzalo Sapisochin · T.K. Waddell · Shaf Keshavjee · Nazia Selzner · C. Chaparro · Mark S. Cattral · Mark S. Cattral
375 Staff knowledge at the All Wales Adult CF CENTRE (AWACFC) – do we know what our patients know? R. Rebel · V.A.M. Gulmans · V.A.M. Gulmans
389 The current problems and needs in the medication chain of Dutch patients with cystic fibrosis M.M. Nap-van der Vlist · M. Burghard · H.J. Hulzebos · W.R. Doeleman · C.K. van der Ent · E.A. van de Graaf · S.L. Nijhof · S.L. Nijhof
WS19.1 LCI in young children with CF – N2 or SF6? M. van Eijk · C.K. van der Ent · H.G.M. Arets · Mi Kristensen · H. de Cock · A. van Dijk · B.J. Keijser · H.P. Haagsman · H.P. Haagsman
297 Liver steatosis in pediatric cystic fibrosis patients Elpis Hatziagorou · S. Giannakoulakos · E. Kouidi · A. Kampouras · M. Anifanti · A. Deligiannis · John Tsanakas · John Tsanakas
WS07.5 Novel antimicrobial peptides to fight infections in patients with cystic fibrosis G. Herrmann · E. Freitag · L. Kiefer · V. Bender · C. Adams · Ute Graepler-Mainka · J. Riethmüller · J. Riethmüller
EPS7.8 Combined dry powder tobramycin and nebulized colistin versus colistin inhalation in CF patients – a randomised, open label phase III clinical study I. Anastasiou · N. Tucker · T. Daniels · Julia A. Nightingale · Mary P. Carroll · Mary P. Carroll
99 Pf bacteriophage (Pf) in Pseudomonas aeruginosa (Pa) biofilms is associated with increased elastase in the sputum of patients with cystic fibrosis (CF) R. Keyte · L. Beswick · O. Rayner · M. Bryon · S.B. Carr · N. Medhurst · N. Medhurst
WS14.6 Effects of aspergillus colonization on pulmonary function in cystic fibrosis patients with lung transplant E.B. Burgener · A.A. Yacob · P. Bollyky · C.E. Milla · C.E. Milla
Oxidized glutathione and uric acid as biomarkers of early cystic fibrosis lung disease Anthony G. Durmowicz · Anthony G. Durmowicz
252 Physical activity/exercise is the most used airway clearance technique in children with cystic fibrosis in Sweden M. Masini · F. De Luca · E. Dei Rossi · E. Romeo · S. Veraldi · C. Imondi · Giuseppe Cimino · Giuseppe Cimino
WS06.3 Psychological aspects of newborn screening for cystic fibrosis in Italy: risks or benefits? S. Margareta · K. Ersson · S. Arnham · M. Mårtensson · A. Törnberg · U. Dennersten · U. Dennersten
58 Pharmacokinetics and safety of a novel CFTR corrector molecule GLPG2222 in subjects with cystic fibrosis (CF): results from a phase Ib study S. Oteri · D.R. Iusco · A. Manca · Stefano Costa · S. Pellegrino · A. Saja · Giuseppe Magazzù · M.C. Lucanto · M.C. Lucanto
174 Dynamic of microbial flora airway colonization in patients with cystic fibrosis after liver transplantation O. Van de Steen · K. De Boeck · F. Vermeulen · David E. Geller · H. de Kock · D. Kanters · C. Gesson · F. Namour · F. Namour
223 Iron status, disease severity and sputum microbiology in adults with cystic fibrosis K. Semczuk · H. Dmenska · A. Wojcieszek · B. Frone · E. Romanowska · S. Wiçckowski · P. Czubkowski · K. Dzierżanowska-Fangrat · D. Dzierzanowska · D. Dzierzanowska
176 The effect of treatment with intermittent inhaled tobramycin powder on systemic cytokines response in CF patients colonized with Pseudomonas aeruginosa N.P. Talbot · S.J. Chapman · W.G. Flight · W.G. Flight
WS08.4 Is there cardiac autonomic dysfunction among patients with cystic fibrosis M. Gur · Y. Toukan · F. Hakim · Y. Geffen · R. Bar-Yoseph · V. Nir · Lea Bentur · Lea Bentur
Multilocus amplicon sequencing of Pseudomonas aeruginosa cystic fibrosis airways isolates collected prior to and after early antipseudomonal chemotherapy Nina Dickerhof · Rufus Turner · Irada Khalilova · Emmanuelle Fantino · Peter D. Sly · Anthony J. Kettle · Anthony J. Kettle
Animal and model systems for studying cystic fibrosis M. Dodémont · M.A. Argudín · J. Willekens · Eef Vanderhelst · Denis Piérard · V. Y. Miendje Deyi · L. Hanssens · H. Franckx · P. Schelstraete · I. Leroux-Roels · C. Nonhoff · A. Deplano · Christiane Knoop · Anne Malfroot · Olivier Denis · Olivier Denis
A simplified, semi-quantitative structural lung disease computed tomography outcome during quiet breathing in infants with cystic fibrosis James Reihill · Michelle Moreland · Gavin E. Jarvis · Gavin E. Jarvis · Andrew McDowell · Andrew McDowell · G.G. Einarsson · J. Stewart Elborn · S. Lorraine Martin · S. Lorraine Martin
Lack of efficacy of Lactobacillus GG in reducing pulmonary exacerbations and hospital admissions in children with cystic fibrosis: A randomised placebo controlled trial Jennifer L. Taylor-Cousar · Manu Jain · Tara Lynn Barto · Tarik Haddad · Jeffrey J. Atkinson · S. Tian · Rui Tang · Gautham Marigowda · David A. Waltz · Joseph M. Pilewski · Joseph M. Pilewski
Pancreatitis and pancreatic cystosis in Cystic Fibrosis James M. Abraham · Christopher J. Taylor · Christopher J. Taylor
Nutrition: Prevention and management of nutritional failure in Cystic Fibrosis A. Jay Freeman · Chee Y. Ooi · Chee Y. Ooi
Gallbladder and bile duct disease in Cystic Fibrosis Jillian Sullivan · Maria R. Mascarenhas · Maria R. Mascarenhas
Inflammation and host-pathogen interaction: Cause and consequence in cystic fibrosis lung disease David N. Assis · Dominique Debray · Dominique Debray
Pancreatic insufficiency in Cystic Fibrosis Alessandra Bragonzi · Hamed Horati · Lauren Kerrigan · Nicola Ivan Lorè · Bob J. Scholte · Sinéad Weldon · Sinéad Weldon
Cystic Fibrosis and gastroesophageal reflux disease Vikesh K. Singh · Sarah Jane Schwarzenberg · Sarah Jane Schwarzenberg
Ion channels as targets to treat cystic fibrosis lung disease Asim Maqbool · Ans Pauwels · Ans Pauwels
Mechanisms of humoral immune response against Pseudomonas aeruginosa biofilm infection in cystic fibrosis S. Lorraine Martin · Vinciane Saint-Criq · Tzyh-Chang Hwang · László Csanády · László Csanády
Lung function decline is delayed but not decreased in patients with cystic fibrosis and the R117H gene mutation Peter Østrup Jensen · Carlos Emílio Levy · Niels Høiby · Niels Høiby · Niels Høiby
Approach to chronic abdominal pain in Cystic Fibrosis Jeffrey S. Wagener · Stefanie J. Millar · Nicole Mayer-Hamblett · Gregory S. Sawicki · Edward F. McKone · Christopher H. Goss · Michael W. Konstan · Wayne J. Morgan · David J. Pasta · Richard B. Moss · Richard B. Moss
Meconium ileus in Cystic Fibrosis Sarah Shrager Lusman · Richard J. Grand · Richard J. Grand
Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine Helmut Ellemunter · Katharina Schüller · G. Steinkamp · G. Steinkamp
Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation Jill Dorsey · Tanja Gonska · Tanja Gonska
Defining palliative care in cystic fibrosis: A Delphi study Aaron Trimble · Scott H. Donaldson · Scott H. Donaldson
Diagnostic and prognostic significance of systemic alkyl quinolones for P. aeruginosa in cystic fibrosis: A longitudinal study; response to comments Elisabeth P. Dellon · Jessica Goggin · Elaine Chen · Kathryn A. Sabadosa · Sarah E. Hempstead · Albert Faro · Karen Homa · Karen Homa
Ceftaroline pharmacokinetics and pharmacodynamics in patients with cystic fibrosis Helen L. Barr · Nigel Halliday · David A. Barrett · Paul Williams · Douglas L. Forrester · D. Peckham · K. Williams · Alan Smyth · D. Honeybourne · J.L. Whitehouse · E.F. Nash · Jane Dewar · Andrew Clayton · Alan J. Knox · Miguel Cámara · A. Fogarty · A. Fogarty
Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis Emily E. Barsky · Luis M. Pereira · Keri J. Sullivan · Alanna Wong · Alexander J. McAdam · Gregory S. Sawicki · Gregory P. Priebe · Susan M. Goobie · Susan M. Goobie
Do cystic fibrosis centres with the lowest FEV1 still use the least amount of intravenous antibiotics? A registry-based comparison of intravenous antibiotic use among adult CF centres in the UK Sonya L. Heltshe · Sonya L. Heltshe · Umer Khan · Valeria V. Beckett · Arthur Baines · Julia Emerson · Don B. Sanders · Ronald L. Gibson · Wayne J. Morgan · Margaret Rosenfeld · Margaret Rosenfeld
Cystic Fibrosis-related cirrhosis Z.H. Hoo · Z.H. Hoo · Michael J. Campbell · Rachael Curley · Rachael Curley · Stephen J. Walters · M. Wildman · M. Wildman · M. Wildman
Glucose trajectories in cystic fibrosis and their association with pulmonary function Daniel H. Leung · Michael R. Narkewicz · Michael R. Narkewicz
Corrigendum toDoes current reporting of lung function by the UK cystic fibrosis registry allow a fair comparison of adult centres?[J Cyst Fibros (2017) 585–591] Quitterie Reynaud · Muriel Rabilloud · Muriel Rabilloud · Sylvain Roche · Sylvain Roche · S. Poupon-Bourdy · Jean Iwaz · Jean Iwaz · R. Nove-Josserand · Emilie Blond · M. Laville · C. Llerena · S. Quetant · Sandrine Touzet · I. Durieu · I. Durieu
Exercise assessment and training in cystic fibrosis: Can less achieve more? Julia A. Nightingale · Clive Osmond · Clive Osmond
IgG avidity to Pseudomonas aeruginosa over the course of chronic lung biofilm infection in cystic fibrosis Anton Y. Peleg · Jocelyn M. Choo · Katherine M. Langan · D. Edgeworth · D. Keating · John Wilson · Geraint B. Rogers · Tom Kotsimbos · Tom Kotsimbos
Recent progress in translational cystic fibrosis research using precision medicine strategies Lena Nørregaard · Oana Ciofu · Carlos Emílio Levy · Niels Høiby · Niels Høiby
Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence J.A. Green · William D. Carroll · William D. Carroll · Francis J. Gilchrist · Francis J. Gilchrist · Francis J. Gilchrist
Real life practice of sweat testing in Europe Anna M. Georgiopoulos · Deborah Friedman · Elizabeth A. Porter · Amy Krasner · Sheetal P. Kakarala · Breanna Glaeser · Siena C. Napoleon · Janet Wozniak · Janet Wozniak
Progress in understanding mucus abnormalities in cystic fibrosis airways Natalia Cirilli · K.W. Southern · R. Buzzetti · Jürg Barben · L. Nährlich · Anne Munck · Michael Wilschanski · K. De Boeck · Nico Derichs · Nico Derichs
Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer Millie Garg · Steven T. Leach · Andrew S. Day · Chee Y. Ooi · Chee Y. Ooi · Chee Y. Ooi
Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR C. Fitzgerald · S. George · R. Somerville · Barry Linnane · Patricia Fitzpatrick · Patricia Fitzpatrick
Bacterial proteases and haemostasis dysregulation in the CF lung D.G. Burke · M.J. Harrison · C. Fleming · M. McCarthy · C. Shortt · I. Sulaiman · Desmond M. Murphy · Joseph A. Eustace · Fergus Shanahan · Colin Hill · Catherine Stanton · Mary C. Rea · R.P. Ross · B.J. Plant · B.J. Plant
Multiple reaction monitoring mass spectrometry to identify novel plasma protein biomarkers of treatment response in cystic fibrosis pulmonary exacerbations Michael P. Bonk · Michael M. Rey · Denis Hadjiliadis · Denis Hadjiliadis
429 Insight into the outcomes of Croatian patients with cystic fibrosis Adam T. Bernstein · Margaret W. Leigh · Jennifer L. Goralski · Charles R. Esther · Cameron J. McKinzie · Cameron J. McKinzie
415 Do females have worse outcomes? Cross-sectional analysis using the UK CF Registry 2013 M. Brajcinović · D. T-Drinković · A. Vukić-Dugac · D. Tjesić-D · D. Tjesić-D
422 Clinical and functional characteristics of cystic fibrosis patients in a multidisciplinary care program in a hospital in Bogotá, Colombia S.S. Hippolyte · N.J. Simmonds · Diana Bilton · Uta Griesenbach · R. Keogh · R. Keogh
388 High frequency of unscheduled patient-initiated contacts with CF care centre in adults with low lung function Abdullah A. Tarique · Peter D. Sly · Diana G. Cardenas · Lin Luo · Jennifer L. Stow · Scott C. Bell · Claire Wainwright · Emmanuelle Fantino · Emmanuelle Fantino
EPS7.7 Microbiologic changes in CF patients with chronic P. aeruginosa airway infection after 6 cycles of inhaled levofloxacin E. Burnet · Dominique Hubert · J. Champreux · R. Panzo · Pierre-Régis Burgel · Pierre-Régis Burgel
EPS7.3 Oxygen is necessary for the bactericidal activity of amikacin on Mycobacterium abscessus Simon Newsome · Rhian Daniel · Siobhán B. Carr · Diana Bilton · Ruth H. Keogh · Ruth H. Keogh
Translational research to enable personalized treatment of cystic fibrosis Ruth H. Keogh · Rhonda D. Szczesniak · David Taylor-Robinson · Diana Bilton · Diana Bilton
Epidemiology of CF: How registries can be used to advance our understanding of the CF population Marne C. Hagemeijer · Daniel J. Siegwart · Lisa J. Strug · Liudmila Cebotaru · Michael J. Torres · Aderonke Sofoluwe · Jeffrey M. Beekman · Jeffrey M. Beekman
Ratiometric sweat secretion optical test in cystic fibrosis, carriers and healthy subjects Abaigeal D. Jackson · Christopher H. Goss · Christopher H. Goss
The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosis Gabriella Bergamini · Gloria Tridello · Elisa Calcaterra · Stefano Ceri · Marco Tagliasacchi · Federico Bianchi · Federico Monti · Andrea Masciadri · Eugenia Laudanna · Denise Peserico · Elena Sorio · Valeria Esposito · Teresinha Leal · Baroukh M. Assael · Claudio Sorio · Paola Melotti · Paola Melotti
Attention deficit hyperactivity disorder symptoms in patients with cystic fibrosis Daan Caudri · Lidija Turkovic · Jolyn Ng · Nicholas de Klerk · Tim Rosenow · Graham L. Hall · Graham L. Hall · Sarath Ranganathan · Sarath Ranganathan · Peter D. Sly · Stephen M. Stick · Stephen M. Stick
Protein and lipid interactions – Modulating CFTR trafficking and rescue Malena Cohen-Cymberknoh · Tzlil Tanny · Oded Breuer · Hannah Blau · Huda Mussaffi · Diana Kadosh · Silvia Gartner · Alma Salinas · Lea Bentur · Vered Nir · M. Gur · Joel Reiter · David Shoseyov · Eitan Kerem · Itai Berger · Itai Berger
KB001-A, a novel anti-inflammatory, found to be safe and well-tolerated in cystic fibrosis patients infected with Pseudomonas aeruginosa Carlos M. Farinha · Elizabeth A. Miller · Nael A. McCarty · Nael A. McCarty
Variability of monthly nitrogen multiple-breath washout during one year in children with cystic fibrosis Raksha Jain · V.V. Beckett · Michael W. Konstan · Michael W. Konstan · Frank J. Accurso · Jane L. Burns · Nicole Mayer-Hamblett · Carlos Milla · Donald R. VanDevanter · James F. Chmiel · James F. Chmiel · James F. Chmiel
Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles Kent Green · Thomas Kongstad · M. Skov · Frederik Buchvald · Susanne Rosthøj · Jacob Louis Marott · Per Gustafsson · Tacjana Pressler · Kim G. Nielsen · Kim G. Nielsen
Quality of life and mood in children with cystic fibrosis: Associations with sleep quality Jeeyeon Kim · Zoe Davies · Colleen Dunn · Jeffrey J. Wine · Carlos Milla · Carlos Milla
Audiometric assessment of pediatric patients with cystic fibrosis Moya Vandeleur · Moya Vandeleur · Lisa M. Walter · David S. Armstrong · David S. Armstrong · Philip J. Robinson · Gillian M. Nixon · Gillian M. Nixon · Rosemary S.C. Horne · Rosemary S.C. Horne
Effect of Lumacaftor/Ivacaftor on glucose metabolism and insulin secretion in Phe508del homozygous cystic fibrosis patients Kathryn L. Kreicher · Kathryn L. Kreicher · Michael J. Bauschard · Clarice S. Clemmens · Concetta Maria Riva · Ted A. Meyer · Ted A. Meyer
Blood flow regulation and oxidative stress during submaximal cycling exercise in patients with cystic fibrosis Patrick T. Harrison · Nils Hoppe · Nils Hoppe · Ulrich Martin · Ulrich Martin
Rare cases of Blastobotrys raffinosifermentans as cause of FEV1 decline in two CF patients – Whole genome sequencing to exclude transmission Matthew A. Tucker · Breana Berry · Nichole Seigler · Gareth W. Davison · John C. Quindry · Dabney Eidson · Kathleen T. McKie · Ryan A. Harris · Ryan A. Harris · Ryan A. Harris
Hypoglycaemia in cystic fibrosis: An analysis of a single centre adult cystic fibrosis clinic K. Jahn · V. Baettig · Helena Seth-Smith · Adrian Egli · M. Tamm · M. Tamm
Colistin resistance in Pseudomonas aeruginosa and Achromobacter spp. cultured from Danish cystic fibrosis patients is not related to plasmid-mediated expression of mcr-1 Julia C. Espel · Hannah L. Palac · Ankit Bharat · Joanne Cullina · Michelle Prickett · Marc Sala · Susanna A. McColley · Manu Jain · Manu Jain
Resolvin D1 regulates epithelial ion transport and inflammation in cystic fibrosis airways M.G. Pedersen · H.V. Olesen · Søren Jensen-Fangel · Niels Nørskov-Lauritsen · M. Wang · M. Wang
Take it to the Bank Steven D. Freedman · Steven D. Freedman · Steven D. Freedman · Michael Wilschanski · Sarah Jane Schwarzenberg · Sarah Jane Schwarzenberg · Sarah Jane Schwarzenberg
Learnings from the Editors Desk – 2017 John P. Clancy · Elizabeth Joseloff · Elizabeth Joseloff
FAM13A is a modifier gene of cystic fibrosis lung phenotype regulating rhoa activity, actin cytoskeleton dynamics and epithelial-mesenchymal transition Scott C. Bell · Carlo Castellani · Patrick A. Flume · Patrick A. Flume
New Comorbidities in the Changing Face of Cystic Fibrosis Care Harriet Corvol · Nathalie Rousselet · Kristin E. Thompson · Laura Berdah · Guillaume Cottin · Tobias Foussigniere · Elisabeth Longchampt · Laurence Fiette · Edouard Sage · Céline Prunier · Mitchell L. Drumm · Craig A. Hodges · Pierre-Yves Boëlle · Loïc Guillot · Loïc Guillot
End-of-life practice patterns at U.S. adult cystic fibrosis care centers: A national retrospective chart review Nina Sainath · Joan I. Schall · Chiara Bertolaso · Carolyn McAnlis · Virginia A. Stallings · Virginia A. Stallings · Virginia A. Stallings
Therapeutic approaches to CFTR dysfunction: from discovery to drug development Elaine Chen · Karen Homa · Jessica Goggin · Kathryn A Sabadosa · Sarah E. Hempstead · Bruce C. Marshall · Albert Faro · Elisabeth P. Dellon · Elisabeth P. Dellon
Pseudomonas aeruginosa eradication: Finally moving the needle? Sarah K. Lucas · Robert Z. Yang · Jordan M. Dunitz · Holly Boyer · Ryan C. Hunter · Ryan C. Hunter
A first step to STOP cystic fibrosis exacerbations Carlo Castellani · Alistair Duff · Scott C. Bell · Harry G.M. Heijerman · Anne Munck · Felix Ratjen · Isabelle Sermet-Gaudelus · K.W. Southern · Jürg Barben · Patrick A. Flume · Pavla Hodková · Nataliya Kashirskaya · Maya N. Kirszenbaum · Sue Madge · H. Oxley · B.J. Plant · Sarah Jane Schwarzenberg · Alan Smyth · G. Taccetti · Thomas O. F. Wagner · Susan P. Wolfe · Pavel Drevinek · Pavel Drevinek
Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosis Gina Hong · Marissa White · Noah Lechtzin · Natalie E. West · Robin Avery · Heather Miller · Richard S. Lee · Robert Lovari · Christian Massire · Lawrence B. Blyn · Xinglun Liang · Deanna A. Sutton · Jianmin Fu · Brian L. Wickes · Nathan P. Wiederhold · Sean X. Zhang · Sean X. Zhang · Sean X. Zhang
The challenges of maintaining momentum in CF drug development and approval - Commentary Kathryn A. Ramsey · Kathryn A. Ramsey · Caroline McGirr · Stephen M. Stick · Stephen M. Stick · Graham L. Hall · Graham L. Hall · Shannon J. Simpson · Shannon J. Simpson
Cystic fibrosis radiographic biomarkers - Commentary Isabelle Sermet-Gaudelus · M. Delion · I. Durieu · Jacky Jacquot · Dominique Hubert · Dominique Hubert
Effect of freezing sputum on Pseudomonas aeruginosa population heterogeneity Anthony G. Durmowicz · Anthony G. Durmowicz
Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis Ali Poonja · Alya Heirali · Matthew L. Workentine · Douglas G. Storey · Ranjani Somayaji · Harvey R. Rabin · Michael G. Surette · Michael G. Surette · Michael D. Parkins · Michael D. Parkins
Use of FEV1 in cystic fibrosis epidemiologic studies and clinical trials: A statistical perspective for the clinical researcher Talissa A. Altes · Mac Johnson · Meredith Fidler · Martyn Botfield · Nicholas J. Tustison · Carlos Leiva-Salinas · Eduard E. de Lange · Deborah K. Froh · John P. Mugler · John P. Mugler
The impact of cystic fibrosis on work attendance and performance in adults living in rural and remote Western Australia Rhonda D. Szczesniak · Sonya L. Heltshe · Sonya L. Heltshe · Sanja Stanojevic · Nicole Mayer-Hamblett · Nicole Mayer-Hamblett · Nicole Mayer-Hamblett
Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes Jamie Wood · Jamie Wood · Sue Jenkins · Sue Jenkins · Siobhain Mulrennan · Kylie Hill · Kylie Hill · Kylie Hill
A cross-sectional analysis of daytime versus nocturnal polysomnographic respiratory parameters in cystic fibrosis during early adolescence☆ K. De Boeck · K. De Boeck · A Zolin · A Zolin · A Zolin
Chest imaging in CF studies - Commentary Karen A. Waters · A. Lowe · Peter Cooper · Silvano Vella · Hiran Selvadurai · Hiran Selvadurai
The psychometric properties of the Leicester Cough Questionnaire and Respiratory Symptoms in CF tool in cystic fibrosis: A preliminary study ☆ ☆☆ ★ Irmgard Eichler · Efthymios Manolis · Efthymios Manolis
Pleural effusions in non-transplanted cystic fibrosis patients Nathan Ward · Nathan Ward · Kathy Stiller · Hilary Rowe · Anne E. Holland · Anne E. Holland
Chest imaging in cystic fibrosis studies: What counts, and can be counted? Adam R. Belanger · Kimtuyen Nguyen · Umar Osman · Christopher R. Gilbert · Katie Allen · Ahmad Farid Al Rais · Lonny Yarmus · Jason Akulian · Jason Akulian
Current strategies for the long-term assessment, monitoring, and management of cystic fibrosis patients treated with CFTR modulator therapy C. Van de Kerkhove · Pieter Goeminne · Michal Kicinski · Tim S. Nawrot · N. Lorent · P. Van Bleyenbergh · K. De Boeck · Lieven Dupont · Lieven Dupont
MRI accelerating progress in functional assessment of cystic fibrosis lung disease J. Stuart Elborn · Jane C. Davies · Marcus A. Mall · Patrick A. Flume · B.J. Plant · B.J. Plant
Impact of azithromycin on the clinical and antimicrobial effectiveness of tobramycin in the treatment of cystic fibrosis Mark O. Wielpütz · Marcus A. Mall · Marcus A. Mall
Differences between WHO AND CDC early growth measurements in the assessment of Cystic Fibrosis clinical outcomes Dave P. Nichols · Carrie Happoldt · Preston E. Bratcher · Silvia M. Caceres · James F. Chmiel · Kenneth C. Malcolm · Milene T. Saavedra · Lisa Saiman · Jennifer L. Taylor-Cousar · Jerry A. Nick · Jerry A. Nick
Non-invasive prenatal diagnosis (NIPD) of cystic fibrosis: an optimized protocol using MEMO fluorescent PCR to detect the p.Phe508del mutation Danielle Usatin · Elizabeth H. Yen · Catherine M. McDonald · Fadi Asfour · John F. Pohl · Jacob Robson · Jacob Robson
Increasing sputum levels of gamma-glutamyltransferase may identify cystic fibrosis patients who do not benefit from inhaled glutathione Claire Guissart · C. Dubucs · Caroline Raynal · A. Girardet · F. Tran Mau Them · Vanessa Debant · Cécile Rouzier · Amandine Boureau-Wirth · Emmanuelle Haquet · Jacques Puechberty · Eric Bieth · D. Dupin Deguine · P. Khau Van Kien · M.P. Brechard · Victoria Pritchard · Michel Koenig · Mireille Claustres · M.C. Vincent · M.C. Vincent
The expansion and performance of national newborn screening programmes for cystic fibrosis in Europe Alessandro Corti · Matthias Griese · Andreas Hector · Alfonso Pompella · Alfonso Pompella
Stroke and myocardial infarction following bronchial artery embolization in a cystic fibrosis patient Jürg Barben · Carlo Castellani · J.E. Dankert-Roelse · Silvia Gartner · Nataliya Kashirskaya · Barry Linnane · Anne Munck · Dorota Sands · Olaf Sommerburg · Simon Pybus · Victoria Winters · K.W. Southern · K.W. Southern
BMI fails to identify poor nutritional status in stunted children with CF Matt Hoffman · Joseph P. Gerding · Jonathan B. Zuckerman · Jonathan B. Zuckerman
CFTR nonsense mutations: Therapeutic benefits from clinically approved drugs? Michael W. Konstan · David J. Pasta · Jeffrey S. Wagener · Donald R. VanDevanter · Wayne J. Morgan · Wayne J. Morgan
Variability of sweat chloride — A never ending story J. Kevin Foskett · J. Kevin Foskett
Environmental scan of cystic fibrosis research worldwide Burkhard Tümmler · Burkhard Tümmler
Agreement between multiple-breath nitrogen washout systems in children and adults Janice Kelly · Janice Kelly
Clostridium difficile carriage in adult cystic fibrosis (CF); implications for patients with CF and the potential for transmission of nosocomial infection William Poncin · Florian Singer · Anne-Sophie Aubriot · Patrick Lebecque · Patrick Lebecque
Fatal disseminated Rasamsonia infection in cystic fibrosis post-lung transplantation Thomas Radtke · Helge Hebestreit · Milo A. Puhan · Susi Kriemler · Susi Kriemler
The 1-min sit-to-stand test in cystic fibrosis — Insights into cardiorespiratory responses Ana C. Blanchard · Eric Horton · Sanja Stanojevic · Louise Taylor · Valerie Waters · Felix Ratjen · Felix Ratjen
Effectiveness of a stepwise Pseudomonas aeruginosa eradication protocol in children with cystic fibrosis Sonya L. Heltshe · Sonya L. Heltshe · Emily M. Godfrey · Tatiana Josephy · Moira L. Aitken · Jennifer L. Taylor-Cousar · Jennifer L. Taylor-Cousar
When is too little care, too much harm in cystic fibrosis? Psychological and ethical approaches to the problem Donald R. VanDevanter · Nicole Mayer-Hamblett · Michael P. Boyle · Michael P. Boyle · Michael P. Boyle
Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth Pierre-Régis Burgel · Dw Reid · Shawn D. Aaron · Shawn D. Aaron
Applying recent advances in the science of CFTR-based therapeutics to improve outcomes in patients with cystic fibrosis Lily Paemka · Brian N. McCullagh · Mahmoud H. Abou Alaiwa · David A. Stoltz · Qian Dong · Christoph O. Randak · Robert D. Gray · Robert D. Gray · Paul B. McCray · Paul B. McCray
Liver disease in cystic fibrosis presents as non-cirrhotic portal hypertension John P. Clancy · Debbie Benitez · Isabelle Fajac · Manu Jain · Steven M. Rowe · Gregory S. Sawicki · Gregory S. Sawicki
Technological advances shed light on left ventricular cardiac disturbances in cystic fibrosis Peter Witters · Louis Libbrecht · Tania Roskams · Kris De Boeck · Lieven Dupont · Marijke Proesmans · F. Vermeulen · Geert Maleux · Diethard Monbaliu · Jacques Pirenne · David Cassiman · David Cassiman
Correlation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor Zahra N. Sayyid · Zachary M. Sellers · Zachary M. Sellers
Diagnostic and prognostic significance of systemic alkyl quinolones for P. aeruginosa in cystic fibrosis: a longitudinal study Meredith Fidler · Jack Beusmans · Paul Panorchan · Fredrick Van Goor · Fredrick Van Goor
Immediate effects of lumacaftor/ivacaftor administration on lung function in patients with severe cystic fibrosis lung disease Helen L. Barr · Nigel Halliday · David A. Barrett · Paul Williams · Douglas L. Forrester · D. Peckham · K. Williams · Alan Smyth · D. Honeybourne · J.L. Whitehouse · E.F. Nash · Jane Dewar · Andrew Clayton · Alan J. Knox · Miguel Cámara · Andrew W. Fogarty · Andrew W. Fogarty
A 3-year prognostic score for adults with cystic fibrosis Natalia Popowicz · Natalia Popowicz · Jamie Wood · Anna Tai · Sue Morey · Siobhain Mulrennan · Siobhain Mulrennan · Siobhain Mulrennan
Real-life initiation of lumacaftor/ivacaftor combination in adults with cystic fibrosis homozygous for the Phe508del CFTR mutation and severe lung disease L. Nkam · J. Lambert · A. Latouche · Gil Bellis · Pr. Burgel · M.N. Hocine · M.N. Hocine
Nutritional status, nutrient intake and use of enzyme supplements in paediatric patients with Cystic Fibrosis; a European multicentre study with reference to current guidelines Dominique Hubert · R. Chiron · Boubou Camara · D. Grenet · A. Prevotat · Laurence Bassinet · Stéphane Dominique · Gilles Rault · Julie Macey · Isabelle Honoré · R. Kanaan · Sylvie Leroy · Nadine Dufeu · Pierre-Régis Burgel · Pierre-Régis Burgel
Association between glucose intolerance and bacterial colonisation in an adult population with cystic fibrosis, emergence of Stenotrophomonas maltophilia Kate Skolnik · Robert D. Levy · Pearce G. Wilcox · Pearce G. Wilcox · Bradley S. Quon · Bradley S. Quon · Bradley S. Quon
Feasibility of placebo-controlled trial designs for new CFTR modulator evaluation C. Lehoux Dubois · V. Boudreau · François Tremblay · Annick Lavoie · Yves Berthiaume · Rémi Rabasa-Lhoret · Adèle Coriati · Adèle Coriati
The patient voice in research — Supporting actor or starring role? John Massie · Alice Morgan · Lynn Gillam · Lynn Gillam · Lynn Gillam
Pregnancy among cystic fibrosis women in the era of CFTR modulators Lena Raaijmakers · Renee Jensen · Sanja Stanojevic · Felix Ratjen · Felix Ratjen
Pilot trial of tobramycin inhalation powder in cystic fibrosis patients with chronic Burkholderia cepacia complex infection Nicola J. Rowbotham · Alan Smyth · Alan Smyth
Inhaled dry powder mannitol in children with cystic fibrosis: A randomised efficacy and safety trial Valerie Waters · Yvonne Yau · Trevor Beaudoin · Jillian Wettlaufer · Sean Kevin Tom · Nancy McDonald · Leena Rizvi · Michelle Klingel · Felix Ratjen · Elizabeth Tullis · Elizabeth Tullis
Peripheral muscle abnormalities in cystic fibrosis: Etiology, clinical implications and response to therapeutic interventions K. De Boeck · Eric G. Haarman · J. Hull · Larry C. Lands · Alexander Moeller · Anne Munck · J. Riethmüller · Harm A.W.M. Tiddens · S. Volpi · J. Leadbetter · Brett Charlton · Anne Malfroot · Anne Malfroot
Dietary intake and lipid profile in children and adolescents with cystic fibrosis Mathieu Gruet · Thierry Troosters · Samuel Verges · Samuel Verges
Ethnicity impacts the cystic fibrosis diagnosis: A note of caution J.W. Woestenenk · Dorothea A. Schulkes · Henk S. Schipper · Cornelis K. van der Ent · Roderick H. J. Houwen · Roderick H. J. Houwen
The cumulative effects of intravenous antibiotic treatments on hearing in patients with cystic fibrosis Barbara Bosch · Diana Bilton · Patrick R. Sosnay · Karen S. Raraigh · Denise Y.F. Mak · Hiroshi Ishiguro · Vincent Gulmans · M. Thomas · Harry Cuppens · Margarida D. Amaral · Kris De Boeck · Kris De Boeck
Counterpoint: Too little care or too little collaboration: Approaches to treatment refusal in CF Angela C. Garinis · Campbell P. Cross · Priya Srikanth · Kelly Carroll · M. Patrick Feeney · Douglas H. Keefe · Lisa L. Hunter · Daniel B. Putterman · David M. Cohen · Jeffrey A. Gold · Peter S. Steyger · Peter S. Steyger
Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR Gregory S. Sawicki · Kristin A. Riekert · Kristin A. Riekert
Acetic acid is elevated in the exhaled breath of cystic fibrosis patients Scott H. Donaldson · George M. Solomon · Pamela L. Zeitlin · Patrick A. Flume · Alicia Casey · Karen McCoy · Edith T. Zemanick · Arun Mandagere · Janice M. Troha · Steven A. Shoemaker · James F. Chmiel · Jennifer L. Taylor-Cousar · Jennifer L. Taylor-Cousar
High incidence of non-tuberculous mycobacteria-positive cultures among adolescent with cystic fibrosis Patrik Španěl · Kristýna Sovová · Kseniya Dryahina · Tereza Doušová · Pavel Dřevínek · David Smith · David Smith
Macronutrient intake in preschoolers with cystic fibrosis and the relationship between macronutrients and growth Zoé Cavalli · Quitterie Reynaud · Romain Bricca · R. Nove-Josserand · S. Durupt · M. Perceval · Michèle Pérouse de Montclos · Gérard Lina · I. Durieu · I. Durieu
Validation of multiple breath washout devices Stephanie S. Filigno · Stephanie S. Filigno · Shannon M. Robson · Shannon M. Robson · Rhonda D. Szczesniak · Leigh A. Chamberlin · Meredith A. Baker · Stephanie M. Sullivan · John W. Kroner · Scott W. Powers · Scott W. Powers · Scott W. Powers
16S rRNA gene sequencing reveals site-specific signatures of the upper and lower airways of cystic fibrosis patients Erin M. Lowery · Erin M. Lowery · William Adams · Shellee A. Grim · Nina M. Clark · Leah Edwards · Jennifer E. Layden · Jennifer E. Layden · Jennifer E. Layden
419 Does current reporting of lung function by the UK cystic fibrosis registry allow comparison of adult centres Jane E. Ashbrook · Carol Shacklady · S.C. Johnson · Gillian Yeowell · Peter C. Goodwin · Peter C. Goodwin
Rationalizing endpoints for prospective studies of pulmonary exacerbation treatment response in cystic fibrosis Jonathan Cogen · Margaret Rosenfeld · Margaret Rosenfeld
Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients Robert P. Thomen · Robert P. Thomen · Laura L. Walkup · David Roach · Zackary I. Cleveland · John P. Clancy · Jason C. Woods · Jason C. Woods · Jason C. Woods
Longitudinal study of Stenotrophomonas maltophilia antibody levels and outcomes in cystic fibrosis patients Jillian Wettlaufer · Michelle Klingel · Yvonne Yau · Sanja Stanojevic · Elizabeth Tullis · Felix Ratjen · Valerie Waters · Valerie Waters
Absence of calf muscle metabolism alterations in active cystic fibrosis adults with mild to moderate lung disease Nicolas Decorte · Mathieu Gruet · Boubou Camara · S. Quetant · Laurent Mely · Jean-Marc Vallier · Samuel Verges · Bernard Wuyam · Bernard Wuyam
Effect of probiotics on respiratory, gastrointestinal and nutritional outcomes in patients with cystic fibrosis: A systematic review Jacqueline L. Anderson · Caitlin Miles · Audrey C. Tierney · Audrey C. Tierney
Airway inflammation in mild cystic fibrosis T. Jakovska-Maretti · T. Jakovska-Maretti
Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor Myriam Mesbahi · Michal Shteinberg · Michael Wilschanski · Aurélie Hatton · Thao Nguyen–Khoa · Hannah Friedman · Michael Cohen · Virginie Escabasse · Muriel Le Bourgeois · Vicenzina Lucidi · Isabelle Sermet-Gaudelus · Laurence Bassinet · G. Livnat · G. Livnat
Adherence to infection prevention and control guidelines: A vignette-based study of decision-making and risk-taking in young adults with cystic fibrosis G. Bowmer · G. Bowmer · Gary Latchford · Alistair Duff · Alistair Duff · M. Denton · Louise Dye · Clare L. Lawton · T. Lee · T. Lee
Phenotypic shift in Pseudomonas aeruginosa populations from cystic fibrosis lungs after 2-week antipseudomonal treatment Laia Fernández-Barat · Oana Ciofu · Kasper Nørskov Kragh · Tania Pressler · Ulla Johansen · Anna Motos · Antoni Torres · Niels Høiby · Niels Høiby
Cancer risk among lung transplant recipients with cystic fibrosis Aliza K. Fink · Elizabeth L. Yanik · Bruce C. Marshall · Michael Wilschanski · Charles F. Lynch · April A. Austin · Glenn Copeland · Mahboobeh Safaeian · Eric A. Engels · Eric A. Engels
Limitations to providing adult cystic fibrosis care in Europe: Results of a care centre survey Susan Madge · Scott C. Bell · Scott C. Bell · Pierre Régis Burgel · Karleen De Rijcke · Francesco Blasi · J. Stuart Elborn · J. Stuart Elborn
Triazole resistance in Aspergillus fumigatus isolates from patients with cystic fibrosis in Italy Anna Prigitano · Maria Carmela Esposto · A. Biffi · Gabriella De Lorenzis · Vincenza Favuzzi · Raffaella Koncan · Giuliana Lo Cascio · Marisol Barao Ocampo · Carla Colombo · Giovanna Pizzamiglio · Luisa Romanò · Anna Maria Tortorano · Anna Maria Tortorano
Preliminary comparison of normalized T1 and non-contrast perfusion MRI assessments of regional lung disease in cystic fibrosis patients Shannon B. Donnola · Elliott C. Dasenbrook · Elliott C. Dasenbrook · David Weaver · David Weaver · Lan Lu · Karishma Gupta · Anjali Prabhakaran · Xin Yu · James F. Chmiel · James F. Chmiel · Kimberly McBennett · Kimberly McBennett · Michael W. Konstan · Michael W. Konstan · Mitchell L. Drumm · Chris A. Flask · Chris A. Flask
Prevalence of elevated liver enzymes in children with cystic fibrosis diagnosed by newborn screen Samantha A. Woodruff · Marci K. Sontag · Frank J. Accurso · Ronald J. Sokol · Michael R. Narkewicz · Michael R. Narkewicz
Variability of sweat chloride concentration in subjects with cystic fibrosis and G551D mutations F. Vermeulen · C. Le Camus · Jane C. Davies · Jane C. Davies · Diana Bilton · Diana Bilton · D. Milenković · K. De Boeck · K. De Boeck
Modeling cystic fibrosis disease progression in patients with the rare CFTR mutation P67L Mark T. Jennings · Elliot C. Dasenbrook · Noah Lechtzin · Michael P. Boyle · Michael P. Boyle · Christian A. Merlo · Christian A. Merlo
CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis Vandana Kumari · Shruti Desai · Nadia A. Ameen · Nadia A. Ameen
Age-dependent variation of fecal calprotectin in cystic fibrosis and healthy children Abdullah A. Tarique · Peter D. Sly · Patrick G. Holt · Patrick G. Holt · Anthony Bosco · Robert S. Ware · Jayden Logan · Scott C. Bell · Scott C. Bell · Claire E. Wainwright · Emmanuelle Fantino · Emmanuelle Fantino
Orkambi in patients with severe disease — Bumps in the road to CFTR modulation Millie Garg · Steven T. Leach · Michael J. Coffey · Tamarah Katz · Roxanne Strachan · Tamara Pang · Bronwen Needham · Kei Lui · Kei Lui · Fathalla Ali · Andrew S. Day · Laura Appleton · Vesal Moeeni · Adam Jaffe · Adam Jaffe · Chee Y. Ooi · Chee Y. Ooi · Chee Y. Ooi
Feasibility and normal values of an integrated conductivity (Nanoduct™) sweat test system in healthy newborns Alex Horsley · Peter J. Barry · Peter J. Barry
AP2 α modulates cystic fibrosis transmembrane conductance regulator function in the human intestine Donald R. VanDevanter · Sonya L. Heltshe · Sonya L. Heltshe · Jonathan E. Spahr · Valeria V. Beckett · Cori L. Daines · Elliott C. Dasenbrook · R.L. Gibson · Raksha Jain · Don B. Sanders · C.H. Goss · C.H. Goss · Patrick A. Flume · Patrick A. Flume
Risk factors for persistent methicillin-resistant Staphylococcus aureus infection in cystic fibrosis Claudia E. Kuehni · Matthias Schindler · Agnieszka Mazur · Andreas Malzacher · René Hornung · Juerg Barben · Juerg Barben
Ivacaftor and symptoms of extra-oesophageal reflux in patients with cystic fibrosis and G551D mutation Gemma Louise Zeybel · Jeffrey P. Pearson · A. Krishnan · Stephen Bourke · S. Doe · A. Anderson · Shoaib Faruqi · Alyn H. Morice · Rhys Jones · Melissa J. McDonnell · Müjdat Zeybel · Müjdat Zeybel · Peter W. Dettmar · Malcolm Brodlie · Malcolm Brodlie · Christopher Ward · Christopher Ward · Christopher Ward
A treatment evaluator tool to monitor the real-world effectiveness of inhaled aztreonam lysine in cystic fibrosis Isobel E.R. MacKenzie · Valerie Paquette · Frances Gosse · Sheenagh George · Frédéric Chappe · Valerie Chappe · Valerie Chappe
Objective airway artery dimensions compared to CT scoring methods assessing structural cystic fibrosis lung disease Wieying Kuo · Wieying Kuo · Eleni-Rosalina Andrinopoulou · Adria Perez-Rovira · Adria Perez-Rovira · Hadiye Ozturk · Marleen de Bruijne · Marleen de Bruijne · Harm A.W.M. Tiddens · Harm A.W.M. Tiddens · Harm A.W.M. Tiddens
Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation Susan P. Wolfe · Michael Wilschanski · Michael Wilschanski
Risk of asthma in heterozygous carriers for cystic fibrosis: A meta-analysis Anne Orholm Nielsen · Sadaf Qayum · Pierre Bouchelouche · Lars Christian Laursen · Ronald Dahl · Morten Dahl · Morten Dahl
Efficacy measures for clinical trials: A review series Patrick A. Flume · Donald R. VanDevanter · Donald R. VanDevanter
CF end point commentary Anthony G. Durmowicz · Anthony G. Durmowicz
Aminoglycosides: old friend…new foe? N.J. Simmonds · Alison H. Thomson · Alison H. Thomson
A novel guluronate oligomer improves intestinal transit and survival in cystic fibrosis mice Megan Vitko · Dana M. Valerio · Philip D. Rye · Edvar Onsøyen · Astrid Hilde Myrset · Arne Dessen · Mitchell L. Drumm · Craig A. Hodges · Craig A. Hodges
Physiologic endpoints for clinical studies for cystic fibrosis Sanja Stanojevic · Felix Ratjen · Felix Ratjen
189 Improving vitamin D status at Bristol Adult Cystic Fibrosis Centre (BACFC) A. Keele · J. Al-Siaidi · K. Bateman · K. Bateman
173 Diurnal variation of glucose in CFRD: implications for management L. Birch · J. Robinson · R.M. Kaminski · D. Nazareth · D. Nazareth
109 An evaluation of the performance of a galactomannan (GM) assay in aiding the diagnosis of allergic broncho-pulmonary aspergillosis (AβPA) in cystic fibrosis (CF) patients R. Dhillon · L. Speight · J. Duckers · P.L. White · P.L. White
WS10.4 Disease-related pain in children and adolescents with cystic fibrosis (CF): effect on the health-related quality of life Elpis Hatziagorou · M. Pasapnoi · L. Nousia · D. Damigos · John Tsanakas · John Tsanakas
166 CF liver disease and lung function Ioana Ciuca · L. Pop · Z. Popa · L. Tamas · L. Tamas
56 Prevalence of hypermutator strains among clinical isolates of Achromobacter sp W. Ridderberg · N. Nørskov-Lauritsen · N. Nørskov-Lauritsen
232 Consulting the experts: engaging pre-adolescent children and their parents in the development of a study proposal L. Cave · L. Milnes · R. Carroll · K. Turner · T. Lee · T. Lee
269 Tooth decay risk factors for individuals with cystic fibrosis Donald L. Chi · Margaret Rosenfeld · Elise W. Sarvas · Sharon McNamara · Alan Genatossio · Marilynn Rothen · A. Kim · A. Kim
44 Upper airway sampling in preschool CF patients to assess pathogen colonization and inflammation Julia Hentschel · K. Wullenkord · T. Schilling · Wibke K. Janhsen · Thomas Lehmann · J. Rödel · Wolfgang Pfister · Uta-Christina Hipler · Jochen G. Mainz · Jochen G. Mainz
22 Testing flow cytometry to detect CFTR expression recovery after drug treatment in epithelial cell lines S. Vercellone · Sara Caldrer · Jan Johansson · Mario Buffelli · Paola Melotti · Claudio Sorio · Claudio Sorio
A phase 3, multi-center, multinational, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of levofloxacin inhalation solution (APT-1026) in stable cystic fibrosis patients Patrick A. Flume · Donald R. VanDevanter · Elizabeth E. Morgan · Michael N. Dudley · Jeffery S. Loutit · Scott C. Bell · E. Kerem · Rainald Fischer · Alan Smyth · Shawn D. Aaron · Douglas Conrad · David E. Geller · J. Stuart Elborn · J. Stuart Elborn
Evaluation of a systems biology approach to identify pharmacological correctors of the mutant CFTR chloride channel Emanuela Pesce · Giulia Gorrieri · Francesco Sirci · Francesco Napolitano · Diego Carrella · Emanuela Caci · Valeria Tomati · Olga Zegarra-Moran · Diego di Bernardo · Luis J. V. Galietta · Luis J. V. Galietta
Extensive cultivation of soil and water samples yields various pathogens in patients with cystic fibrosis but not Burkholderia multivorans Charlotte Peeters · Eliza Depoorter · Jessy Praet · Peter Vandamme · Peter Vandamme
Disease disclosure in individuals with cystic fibrosis: Association with psychosocial and health outcomes Adrienne P. Borschuk · Robin S. Everhart · Michelle N. Eakin · Devin Rand-Giovannetti · Belinda Borrelli · Kristin A. Riekert · Kristin A. Riekert
Daily Observations of Nebuliser Use and Technique (DONUT) in children with cystic fibrosis Aukje C. Bos · Harm A.W.M. Tiddens · Kirby Tong Minh · Inge Heeres · Joke L. Overweel-Uijterlinde · Annelies E. Kok · Eleni-Rosalina Andrinopoulou · Hettie M. Janssens · Hettie M. Janssens
Comparison of two pancreatic enzyme products for exocrine insufficiency in patients with cystic fibrosis Christopher J. Taylor · R. Thieroff-Ekerdt · S. Shiff · L. Magnus · R. Fleming · C. Gommoll · C. Gommoll
Hypoglycaemia in cystic fibrosis in the absence of diabetes: A systematic review N. Armaghanian · N. Armaghanian · Jennie Brand-Miller · Tania P. Markovic · Tania P. Markovic · Katharine Steinbeck · Katharine Steinbeck · Katharine Steinbeck · Katharine Steinbeck
WS21.1 When should chest physiotherapy (CPT) be started in children with atypical cystic fibrosis C. Thornton · N. Moss · O. Lamptey · S. Wilkinson · S. Wilkinson
208 A concern and unpleasant reminder of my serious illness. Cystic fibrosis patients with experience of intravenous antibiotic home treatment – a Scandinavian Nurse Specialist Group/Cystic Fibrosis (SNSG/CF) study E.J. Hunstad · A. Nyberg · U. Skogeland · G. Haugen · K. Strandner · I.E. Grandinsson · H. Jacobsen · M. Presfeldt · M. Pehn · B.B. Akselsen · B.B. Akselsen
WS03.4 Angiotensin receptor blockers reverse cystic fibrosis (CF)-related mucociliary dysfunction in vitro and in a novel CF sheep model in vivo Matthias Salathe · N. Baumlin · A. Kis · S. Krick · A. Schmid · J. Sabater · W. Abraham · W. Abraham
ePS02.5 Striving to improve the outpatient journey for adults with cystic fibrosis – a quality improvement project S. Talbot · H. Parrott · S. Madge · K. Gyi · A.M. Jones · N.J. Simmonds · N.J. Simmonds
50 Role of reaginic IgE phenomena in Pseudomonas aeruginosa infection in patients with cystic fibrosis S. Sciuca · L. Balanetchi · C. Grigore · A. Cotoman · A. Cotoman
231 Are adult cystic fibrosis patients aware of the importance of skin health and sun protection D. Office · P. Whitaker · R. Metcalfe · D. Peckham · D. Peckham
ePS04.5 INSPIRE-CF: levels of participation of children with cystic fibrosis randomised to a 24-month weekly supervised exercise intervention S.J. Ledger · H. Douglas · L. Sarria Jaramillo · Paul Aurora · E. Main · E. Main
WS07.4 Effect of ivacaftor on airway bacterial community composition in CF patients with the G551D mutation D. Mooney · N.J. Ronan · G.G. Einarsson · Et Flanagan · Joe Eustace · Michael M. Tunney · J.S. Elborn · B.J. Plant · B.J. Plant
ePS04.4 Efficacy of osteopathic manipulative treatment on pain in patients with cystic fibrosis during pulmonary exacerbation: a randomized controlled study L. Graziano · T. Perelli · B. Giacomodonato · M. Varchetta · S. De Sanctis · L. Necci · F. Alatri · F. Alatri
WS18.3 A combination of CFTR functional tests supporting drug development and diagnosis: the contribution of intestinal epithelial organoids Sara Caldrer · S. Vercellone · Angela Sandri · Claudio Sorio · L. Rodella · Filippo Catalano · Angelo Cerofolini · Francesco Lombardo · Mario Buffelli · H. R. De Jonge · Baroukhmaurice Assael · Paola Melotti · Paola Melotti
ePS05.3 Effect of allergic bronchopulmonary aspergillosis on FEV1 in children and adolescents with cystic fibrosis: an ECFS patient registry analysis Athanasios G. Kaditis · Michael Miligkos · Anna Bossi · Carla Colombo · Elpis Hatziagorou · Nataliya Kashirskaya · I. de Monestrol · M. Thomas · Meir Mei-Zahav · George P. Chrousos · Anna Zolin · Anna Zolin
26 Retrospective observational study of French patients with cystic fibrosis and a G551D mutation after 1 and 2 years of treatment with ivacaftor Dominique Hubert · C. Dehillotte · J. Baek · P. Bakouboula · V. Colomb-Jung · L. Lemonnier · L. Lemonnier
ePS01.8 Inorganic ion concentrations in the saliva of cystic fibrosis adults differ from controls and may assist in diagnosis J.P. Lennon · H.H. House · B. Smith · P. Gounder · Peter G. Middleton · Peter G. Middleton
220 Does cystic fibrosis have an impact on school absenteeism C. Vigier · S. Lefeuvre · A. Dabadie · J. Beucher · M. Dagorne · G. Rault · V. Storni · P. Vigneron · P. Pladys · E. Deneuville · E. Deneuville
WS20.2 High level of β-(1,3)-D-glucan antigenemia in cystic fibrosis in the absence of systemic fungal infection V. Rautemaa · H. Green · R. Richardson · Andrew M. Jones · Andrew M. Jones
117 The fate of inhaled antibiotics after deposition in patients with cystic fibrosis: how to get drug to the bug? A.C. Bos · K.M. Passé · Johan W. Mouton · H.M. Janssens · Harm A.W.M. Tiddens · Harm A.W.M. Tiddens
IL-22 exacerbates weight loss in a murine model of chronic pulmonary Pseudomonas aeruginosa infection Hannah K. Bayes · Neil D. Ritchie · Christopher Ward · Paul Corris · Paul Corris · Malcolm Brodlie · Malcolm Brodlie · Thomas J. Evans · Thomas J. Evans
Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines Sarah Jane Schwarzenberg · Sarah E. Hempstead · Catherine M. McDonald · Scott W. Powers · Jamie L. Wooldridge · Shaina Blair · Steven D. Freedman · Elaine Harrington · Peter J. Murphy · Lena B. Palmer · Amy E. Schrader · Kyle Shiel · Jillian Sullivan · Melissa Wallentine · Bruce C. Marshall · Amanda Leonard · Amanda Leonard
Nasal potential difference outcomes support diagnostic decisions in cystic fibrosis Gloria Tridello · Laura Menin · Emily Pintani · Gabriella Bergamini · Baroukh M. Assael · Paola Melotti · Paola Melotti
ePS02.8 Unscheduled contacts initiated by patients in an adult CF centre: a twelve-month prospective study Jarosław Walkowiak · Aleksandra Glapa · Jan K. Nowak · L. Bober · Nataliya Rohovyk · Ewa Wenska-Chyży · Paulina Sobkowiak · Aleksandra Lisowska · Aleksandra Lisowska
Insulin secretion abnormalities in patients with cystic fibrosis C. Sellar · R.M. Kaminski · D. Nazareth · D. Nazareth
177 Screening practices for cystic fibrosis related diabetes (CFRD) in the UK J.P. Miller · L. Drew · O. Green · D. Dukovski · B. McEwan · A. Villella · N. Patel · C.M. Bastos · M.D. Cullen · H. Danh · S. Wachi · K.A. Giuliano · K.A. Longo · A. Bhalla · D. Qiu · C. Zou · M. Ivarsson · B. Munoz · H. Mehmet · H. Mehmet
WS13.5 CFTR amplifiers are mutation-agnostic modulators that increase CFTR protein levels and complement other CF therapeutic modalities Jane C. Davies · Harm A.W.M. Tiddens · Anne Malfroot · H.G.M. Heijerman · Eitan Kerem · Lena Hjelte · J. Sun · J. Mcintosh · Isabelle Sermet-Gaudelus · K. De Boeck · K. De Boeck
WS13.1.1 Ataluren in nonsense mutation cystic fibrosis patients not receiving tobramycin: significant lung function benefits in the paediatric age range Jonas Eckrich · U. Zissler · U. Zissler · Friederike Serve · Patricia Leutz · Christina Smaczny · Sabina Schmitt-Grohé · Daniela Fussbroich · Ralf Schubert · Stefan Zielen · Olaf Eickmeier · Olaf Eickmeier
183 Prevalence of osteoporosis in patients with cystic fibrosis in Republic of Macedonia C. Dupont · R. Panzo · Isabelle Honoré · Dominique Hubert · R. Kanaan · E. Burnet · V. Colomb-Jung · J. Champreux · Pierre-Régis Burgel · Daniel Dusser · A.-S. Duflos · A.-S. Duflos
207 Is the provision of care for I.V. antibiotic courses similar throughout French CF centers D.J. Waine · D.D. Derry · D.D. Derry
131 Pulmonary function test measurement contributes more information than clinic spirometry M. Scalbert-Dujardin · E. Leclercq · A. Boldron · E. Leroy · J. Bazin · J. Bazin
114 Realization and evaluation of the effectiveness of preventive actions against influenza for patients with cystic fibrosis followed in the Dunkerque Cystic Fibrosis Treatment Centre and for their healthcare workers M. Hassan · M.M. Bonafede · B.L. Limone · Paul Hodgkins · E.D. Suthoff · Gregory S. Sawicki · Gregory S. Sawicki
28 Reduction in pulmonary exacerbations (PEx) after initiation of ivacaftor: a retrospective cohort study among patients with cystic fibrosis (CF) treated in real-world settings E. Burnet · Pierre-Régis Burgel · J. Champreux · Isabelle Honoré · R. Kanaan · R. Panzo · C. Dupont · Dominique Hubert · Dominique Hubert
Pancreatic Elastase-1 Quick Test for rapid assessment of pancreatic status in cystic fibrosis patients A.C. Bos · C. Van Holsbeke · J. De Backer · H.M. Janssens · W. Vos · Harm A.W.M. Tiddens · Harm A.W.M. Tiddens
A product of immunoreactive trypsinogen and pancreatitis-associated protein as second-tier strategy in cystic fibrosis newborn screening Sophia Weidler · Konrad H. Stopsack · Jutta Hammermann · Olaf Sommerburg · Marcus A. Mall · Georg F. Hoffmann · Dirk Kohlmüller · Jürgen G. Okun · Milan Macek · Felix Votava · Veronika Krulisova · Miroslava Balascakova · V. Skalicka · Min Ae Lee-Kirsch · Marina Stopsack · Marina Stopsack
ePS06.4 Patient-specific modeling of regional tobramycin concentration levels in airways of patients with cystic fibrosis: can we dose once daily? K.M. de Winter-de Groot · H.M. Janssens · R.T. van Uum · J.F. Dekkers · Harm A.W.M. Tiddens · G. Berkers · A.M. Vonk · E. Kruisselbrink · Robert G.J. Vries · Hans Clevers · R. H. J. Houwen · Johanna C. Escher · Jeffrey M. Beekman · C.K. van der Ent · C.K. van der Ent
23 Stratifying young children with cystic fibrosis for disease severity using intestinal organoid swelling, intestinal current measurements or sweat chloride concentration as CFTR-dependent biomarker A.S. Ramalho · F. Vermeulen · M. Proesmans · K. De Boeck · K. De Boeck
WS18.1 Using CF colon organoids to study CFTR function of uncharacterized mutations and test potential therapies R. a Rogvi · T.K. Hansen · H.V. Olesen · H.V. Olesen
WS12.6 How does transferral from CF doctors to endocrinologists affect blood glucose control in patients with CFRD A.M. Iones · I. Felton · M.J. Harrison · E. Bowman · D. Bilton · N.J. Simmonds · N.J. Simmonds
116 The use of nebulised voriconazole in CF-related fungal infections: a case series T. Pugatsch · Malena Cohen-Cymberknoh · H. Elyashar-Earon · I. Eisenstadt · Michael Wilschanski · O. Breuer · S. Kurz · S. Palmor · S. Armoni · E. Kerem · E. Kerem
WS16.5 Website based educational program on cystic fibrosis (CF) for community physicians S. Fustik · T. Jakovska-Maretti · L. Spirevska · L. Spirevska
164 Evaluation of liver disease in cystic fibrosis: clinical and genetic characteristics of patients Laurent Meijer · G. Héry-Arnaud · R. Le Berre · Emmanuel Nowak · L. Le Roux · L. Guéganton · G. Rault · Dominique Mottier · Dominique Mottier

Cystic fibrosis related diabetes is not independently associated with increased Stenotrophomonas maltophilia infection: Longitudinal data from the UK CF Registry
Journal of Cystic Fibrosis | 2018
Mathieu Gruet · Nicolas Decorte · Laurent Mely · Jean-Marc Vallier · Boubou Camara · S. Quetant · Bernard Wuyam · Samuel Verges · Samuel Verges
P224 Aseptic centralised versus home extemporaneous preparation for cystic fibrosis outpatients parenteral antibiotic therapy: a survey on nurses satisfaction
Journal of Cystic Fibrosis | 2018
I.J. Haq · B. Verdon · K. Jiwa · Vinciane Saint-Criq · A.I. Gardner · Christopher Ward · Michael A. Gray · Malcolm Brodlie · Malcolm Brodlie
P260 Searching for CFTR mutations in CF patients from North Ossetia-Alania Republic, Russian Federation
Journal of Cystic Fibrosis | 2018
Alejandro Teper · J. Balinotti · V. Rodriguez · S. Lubovich · S. Zaragoza · D. Chang · N. Escobar · L. Galanternik · L. Galanternik
P095 Intravenous fosfomycin for treatment of pulmonary exacerbations in adult cystic fibrosis patients with chronic infection by Pseudomonas aeruginosa and other Gram-negative bacteria
Journal of Cystic Fibrosis | 2018
T.A. Vasilyeva · N.V. Petrova · N.Y. Kashirskaya · Z.K. Getoeva · M.Y. Dzhadzhieva · O.G. Novoselova · E. Kondratyeva · E.K. Ginter · R.A. Zinchenko · R.A. Zinchenko
WS03.3 Childhood nutritional status is a major factor determining lung function in adults with cystic fibrosis
Journal of Cystic Fibrosis | 2018
B. Messore · M. Bellocchia · C. Bena · C. Biglia · G.P. Bandelli · E. Clivati · G. Bonizzoni · S. Demichelis · F. Traversa · C. Albera · C. Albera