November 2016
Title: Multilocus amplicon sequencing of Pseudomonas aeruginosa cystic fibrosis airways isolates collected prior to and after early antipseudomonal chemotherapy
Authors Fischer S, Greipel L, Klockgether J, Dorda M, Wiehlmann L, Cramer N, Tümmler B
References J Cyst Fibros. 2016 Nov 8. pii: S1569-1993(16)30648-8
Keywords Amplicon sequencing, Early antipseudomonal treatment, Pseudomonas aeruginosa, Single nucleotide polymorphism
Title: Multilocus amplicon sequencing of Pseudomonas aeruginosa cystic fibrosis airways isolates collected prior to and after early antipseudomonal chemotherapy
Authors Fischer S, Greipel L, Klockgether J, Dorda M, Wiehlmann L, Cramer N, Tümmler B
References J Cyst Fibros. 2016 Nov 8. pii: S1569-1993(16)30648-8
Keywords Amplicon sequencing, Early antipseudomonal treatment, Pseudomonas aeruginosa, Single nucleotide polymorphism
November 2016
Title: Biomarkers for Cystic Fibrosis Drug Development
Authors Marianne S. Muhlebach, JP Clancy, Sonya L. Heltshe, Assem Ziady, Tom Kelley, Frank Accurso, Joseph Pilewski, Nicole Mayer-Hamblett, Elizabeth Joseloff, Scott D. Sagel
References dx.doi.org/10.1016/j.jcf.2016.10.009
Keywords Biomarker, Inflammation, Microbiome, Infection, Advanced technologies, Electrophysiology
October 2016
Title: Diagnostic and prognostic significance of systemic alkyl quinolones for P. aeruginosa in cystic fibrosis: a longitudinal study
Authors Helen L. Barr, Nigel Halliday, David A. Barrett, Paul Williams, Douglas L. Forrester, Daniel Peckham, Kate Williams, Alan R. Smyth, David Honeybourne, Joanna L.Whitehouse, Edward F. Nash, Jane Dewar, Andrew Clayton, Alan J. Knox , Miguel Cámara , and Andrew W. Fogarty
References J Cyst Fibros. 2016 Oct 20. pii: S1569-1993(16)30617-8
October 2016
Title: Coronary artery disease in Cystic Fibrosis: An Emerging Concern?
Authors Kate Skolnik, Robert D. Levy, Pearce G. Wilcox, Bradley S. Quon
References J Cyst Fibros. 2016 Oct 14. pii: S1569-1993(16)30612-9
Keywords Atherosclerosis, Coronary artery disease, Diabetes, Dyslipidemia, Heart disease
Title: Coronary artery disease in Cystic Fibrosis: An Emerging Concern?
Authors Kate Skolnik, Robert D. Levy, Pearce G. Wilcox, Bradley S. Quon
References J Cyst Fibros. 2016 Oct 14. pii: S1569-1993(16)30612-9
Keywords Atherosclerosis, Coronary artery disease, Diabetes, Dyslipidemia, Heart disease
October 2016
Title: Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes
Authors K De Boeck, A Zolin
References J Cyst Fibros. 2016 Oct 11. pii: S1569-1993(16)30611-7.
Keywords Age distribution, Lung function, Mutation class
Theme Epidemiology/Models of care
Title: Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes
Authors K De Boeck, A Zolin
References J Cyst Fibros. 2016 Oct 11. pii: S1569-1993(16)30611-7.
Keywords Age distribution, Lung function, Mutation class
Theme Epidemiology/Models of care
October 2016
Title: Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation
Authors Isabelle Sermet-Gaudelus, Martial Delion, Isabelle Durieu, Jacky Jacquot, Dominique Hubert
References J Cyst Fibros. 2016 Oct 10. pii: S1569-1993(16)30605-1
Keywords Bone density, CFTR corrector, RANKL
October 2016
Title: A cross-sectional analysis of daytime versus nocturnal polysomnographic respiratory parameters in cystic fibrosis during early adolescence.
Authors Waters KA, Lowe A, Cooper P, Vella S, Selvadurai H
References J Cyst Fibros. 2016 Oct 8. pii: S1569-1993(16)30609-9
Keywords Adolescent, Child, Exercise test, Pulmonary, Sleep study
October 2016
Title: Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation
Authors Venkateshwar Mutyam, Emily Falk Libby, Ning Peng, Denis Hadjiliadis, Michael Bonk, George M. Solomon, Steven M. Rowe
References J Cyst Fibros. 2016 Oct 1. pii: S1569-1993(16)30607-5
Keywords CFTR potentiator, Ivacaftor, PTC mutations, Readthrough, W1282X
September 2016
Title: Effect of probiotics on respiratory, gastrointestinal and nutritional outcomes in patients with cystic fibrosis: A systematic review
Authors Anderson JL, Miles C, Tierney AC
References J Cyst Fibros. 2016 Sep 29. pii: S1569-1993(16)30606-3
Keywords Probiotics, Systematic review
Theme Nutrition/Gastroenterology
September 2016
Title: Nasal potential difference outcomes support diagnostic decisions in cystic fibrosis
Authors Tridello G, Menin L, Pintani E, Bergamini G, Assael BM, Melotti P
References J Cyst Fibros. 2016 Sep;15(5):579-82.
Keywords CFTR genetic test, CFTR related disorders, Cystic fibrosis transmembrane conductance regulator, Diagnosis, Nasal potential difference measurements, Sweat test
Theme Diagnosis/Screening
Title: Potentiator synergy in rectal organoids carrying S1251N, G551D or F508del CFTR mutations
Authors Johanna F. Dekkers, Peter Van Mourik, Annelotte M. Vonk, Evelien Kruisselbrink, Gitte Berkers, Karin M. de Winter - de Groot, Hettie M. Janssens, Inez Bronsveld, Cornelis K. van der Ent, Hugo R. de Jonge, Jeffrey M.Beekman
References J Cyst Fibros. 2016 Sep;15(5):568-78
Keywords Curcumin, Genistein, Intestinal current measurements (ICM), Intestinal organoids, VX-770
September 2016
Title: Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines
Authors Sarah Jane Schwarzenberg, , Sarah E. Hempstead, , Catherine M. McDonald, , Scott W. Powers, , Jamie Wooldridge, Shaina Blair, Steven Freedman, Elaine Harrington, Peter J. Murphy, Lena Palmer, Amy E. Schrader, Kyle Shiel, Jillian Sullivan, Melissa Wallentinel, Bruce C. Marshall, , Amanda Radmer Leonard
References J Cyst Fibros. 2016 Sep 3. pii: S1569-1993(16)30594-X
Keywords Enteral nutrition, Feeding tube, Gastrostomy, Nasojejunal tube
Theme Nutrition/Gastroenterology
August 2016
Title: Cancer Risk among Lung Transplant Recipients with Cystic Fibrosis
Authors Aliza K. Fink a, Elizabeth L. Yanik b, Bruce C. Marshall, Michael Wilschanski, Charles F. Lynch, April A. Austin, Glenn Copeland, Mahboobeh Safaeian, Eric A. Engels
References J Cyst Fibros. 2016 Aug 15. pii: S1569-1993(16)30568-9
Keywords Cancer, Transplant
August 2016
Title: Reduced survival in adult cystic fibrosis despite attenuated lung function decline.
Authors Keating C, Poor AD, Liu X, Chiuzan C, Backenroth D, Zhang Y, DiMango E
References J Cyst Fibros. 2016 Aug 10. pii: S1569-1993(16)30569-0.
Keywords Adult diagnosis cystic fibrosis, Lung function, Survival
August 2016
Title: The changing prevalence of pulmonary infection in adults with cystic fibrosis: A longitudinal analysis
Authors Ramsay KA, Sandhu H, Geake JB, Ballard E, O'Rourke P, Wainwright CE, Reid DW, Kidd TJ, Bell SC
References J Cyst Fibros. 2016 Aug 8. pii: S1569-1993(16)30567-7
Keywords Lung function, Paediatric, Prevalence, Pseudomonas aeruginosa, Transition
August 2016
Title: Long-term work participation among cystic fibrosis patients undergoing lung transplantation
Authors Krivchenia K, Hayes D Jr, Tobias JD, Tumin D
References J Cyst Fibros. 2016 Aug 3. pii: S1569-1993(16)30564-1.
Keywords Employment, Lung transplantation
Theme Epidemiology/Models of care
July 2016
Title: Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients.
Authors Thomen RP, Walkup LL, Roach DJ, Cleveland ZI, Clancy JP, Woods JC
References J Cyst Fibros. 2016 Jul 28. pii: S1569-1993(16)30565-3.
Keywords Hyperpolarized, MRI, Pediatric
July 2016
Title: Ivacaftor and symptoms of extra-oesophageal reflux in patients with cystic fibrosis and G551D mutation
Authors Gemma L Zeybel, Jeffrey P Pearson, Amaran Krishnan, Stephen J Bourke, Simon Doe, Alan Anderson, Shoaib Faruqi, Alyn H Morice, Rhys Jones, Mellissa McDonnell, Mujdat Zeybel, Peter W Dettmar, Malcolm Brodlie, Chris Ward
References J Cyst Fibros. 2016 Jul 27. pii: S1569-1993(16)30561-6
Keywords CFTR potentiator, G551D, Gastrointestinal, Real life data
Theme Nutrition/Gastroenterology
July 2016
Title: The Effects of Ivacaftor on CF Fatty Acid Metabolism: An Analysis from the GOAL Study
Authors Michael Glenn O’Connor, Adam Seegmiller
References J Cyst Fibros. 2016 Jul 26. pii: S1569-1993(16)30563-X.
Keywords Fatty acids, Ivacaftor, Prostaglandin E
Theme Nutrition/Gastroenterology
July 2016
Title: Limitations to providing adult cystic fibrosis care in Europe: Results of a care centre survey
Authors Susan Madge, Scott C Bell, Pierre- Régis Burgel, Karleen De Rijcke, Francesco Blasi, J. Stuart Elborn
References J Cyst Fibros. 2016 Jul 25. pii: S1569-1993(16)30558-6
Keywords Adults, Eastern Europe, Service provision, Western Europe
Theme Epidemiology/Models of care
July 2016
Title: A Product of Immunoreactive Trypsinogen and Pancreatitis-Associated Protein as Second Tier Strategy in Cystic Fibrosis Newborn Screening
Authors Sophia Weidler, Konrad H. Stopsack, Jutta Hammermann, Olaf Sommerburg, Marcus A. Mall, Georg F. Hoffmann, Dirk Kohlmüller, Jürgen G. Okun, Milan Macek Jr., Felix Votava, Veronika Krulišová, Miroslava Balaščaková, Veronika Skalická, Min Ae Lee-Kirsch, Marina Stopsack
References J Cyst Fibros. 2016 Jul 22. pii: S1569-1993(16)30559-8
Keywords Cutoff, Immunoreactive trypsinogen, Newborn screening, Pancreatitis-associated protein
Theme Diagnosis/Screening
July 2016
Title: The Cystic Fibrosis Foundation Patient Registry. Design and Methods of a National Observational Disease Registry
Authors Knapp EA, Fink AK, Goss CH, Sewall A, Ostrenga J, Dowd C, Elbert A, Petren KM, Marshall BC
References Ann Am Thorac Soc. 2016 Jul;13(7):1173-9. doi: 10.1513/AnnalsATS.201511-781OC.
June 2016
Title: IL-22 exacerbates weight loss in a murine model of chronic pulmonary Pseudomonas aeruginosa infection
Authors Hannah K. Bayes, Neil D. Ritchie, Christopher Ward, Paul A. Corris, Malcolm Brodlie, Thomas J. Evans
References J Cyst Fibros. 2016 Jun 30. pii: S1569-1993(16)30553-7
Keywords Chronic infection, Interleukin-22, Mucosal immunity, Pseudomonas aeruginosa
Title: A Novel Guluronate Oligomer Improves Intestinal Transit and Survival in Cystic Fibrosis Mice
Authors Megan Vitko, Dana M. Valerio, Philip D. Rye, Edvar Onsøyen, Astrid H. Myrset, Arne Dessen, Mitchell L. Drumm, Craig A. Hodges
References J Cyst Fibros. 2016 Jun 30. pii: S1569-1993(16)30553-7
Keywords Chronic infection, Interleukin-22, Mucosal immunity, Pseudomonas aeruginosa
Theme Nutrition/Gastroenterology
June 2016
Title: Triazole resistance in Aspergillus fumigatus isolates from patients with cystic fibrosis in Italy.
Authors Prigitano A, Esposto MC, Biffi A, De Lorenzis G, Favuzzi V, Koncan R, Lo Cascio G, Barao Ocampo M, Colombo C, Pizzamiglio G, Romanò L, Tortorano AM
References J Cyst Fibros. 2016 Jun 26. pii: S1569-1993(16)30551-3.
Keywords Aspergillus fumigatus, Azole resistance, Italy
June 2016
Title: Longitudinal study of Stenotrophomonas maltophilia antibody levels and outcomes in cystic fibrosis patients.
Authors Wettlaufer J, Klingel M, Yau Y, Stanojevic S, Tullis E, Ratjen F, Waters V
References J Cyst Fibros. 2016 Jun 23. pii: S1569-1993(16)30552-5.
Keywords Antibodies, Pulmonary exacerbation, Stenotrophomonas maltophilia
June 2016
Title: Objective airway artery dimensions compared to CT scoring methods assessing structural cystic fibrosis lung disease
Authors W. Kuo, E. R. Andrinopoulou, A. Perez-Rovira, H. Ozturk, M. de Bruijne, H.A.W.M. Tiddens
References J Cyst Fibros. 2016 Jun 21. pii: S1569-1993(16)30522-7
Keywords Bronchiectasis, Chest CT, Pediatrics, Scoring
June 2016
Title: Risk of asthma in heterozygous carriers for cystic fibrosis: a meta-analysis
Authors Anne Orholm Nielsen, Sadaf Qayum, Pierre Nourdine Bouchelouche, Lars Christian Laursen, Ronald Dahl, Morten Dahl
References J Cyst Fibros. 2016 Jun 17. pii: S1569-1993(16)30524-0
Keywords Airway obstruction, Asthma, Epidemiology, Review
Theme Epidemiology/Models of care
Title: Multiple Prevalent Fractures in Relation to Macroscopic Bone Architecture in Patients with Cystic Fibrosis
Authors Mirjam Stahl, Christian Holfelder, Carolin Kneppo, Meinhard Kieser, Christian Kasperk, Eckhard Schoenau, Olaf Sommerburg, Burkhard Tönshoff
References J Cyst Fibros. 2016 Jun 17. pii: S1569-1993(16)30547-1
Keywords Bone densitometry, Bone disease, Cystic fibrosis, Fracture rate, Macroscopic bone architecture, Noninvasive monitoring
June 2016
Title: Airway inflammation in mild cystic fibrosis.
Authors Jonas Eckrich, Ulrich M. Zissler, Friederike Serve, Patricia Leutz, Christina Smaczny, Sabina Schmitt-Grohé, Daniela Fussbroich, Ralf Schubert, Stefan Zielen, Olaf Eickmeier
References J Cyst Fibros. 2016 Jun 10. pii: S1569-1993(16)30523-9
Keywords Airway inflammation, Gene expression, Induced sputum, Pro-inflammatory biomarkers, Small airway disease
Title: Respiratory Pathogens Mediate the Association between Lung Function and Temperature in Cystic Fibrosis
Authors Joseph M. Collaco, Karen S. Raraigh, Lawrence J. Appel, Garry R. Cutting
References J Cyst Fibros. 2016 Jun 10. pii: S1569-1993(16)30514-8.
Keywords Climate, FEV1, Lung function, MRSA, Pseudomonas aeruginosa, Temperature
June 2016
Title: Bioelectrical impedance in young patients with cystic fibrosis: validation of a specific equation and clinical relevance
Authors Charatsi AM, Dusser P, Freund R, Maruani G, Rossin H, Boulier A, Le Bourgeois M, Chedevergne F, de Blic J, Letourneur A, Casimir G, Jais JP, Sermet-Gaudelus, Charatsi AM, Dusser P
References J Cyst Fibros. 2016 Jun 8. pii: S1569-1993(16)30053-4
Keywords Bioelectrical impedance analysis, Body composition, Diaphragm, Fat-free mass, Lung function
June 2016
Title: Pancreatic Elastase-1 Quick Test for rapid assessment of pancreatic status in cystic fibrosis patients.
Authors Walkowiak J, Glapa A, Nowak JK, Bober L, Rohovyk N, Wenska-Chyży E, Sobkowiak P, Lisowska A
References J Cyst Fibros. 2016 Jun 7. pii: S1569-1993(16)30511-2.
Keywords Exocrine pancreatic insufficiency, Point-of-care, Qualitative test, Sensitivity, Specificity
June 2016
Title: Sino-nasal Inhalation of Isotonic versus Hypertonic Saline (6.0 %) in CF Patients with Chronic Rhinosinusitis – Results of a Multicentre, Prospective, Randomized, Double-blind, Controlled Trial
Authors Jochen G Mainz, Ulrike Schumacher, Katja Schädlich, Julia Hentschel, Christiane Koitschev, Assen Koitschev, Joachim Riethmüller, Freerk Prenzel, Olaf Sommerburg, Bärbel Wiedemann, Doris Staab, Wolfgang Gleiber, Rainald Fischer, James F Beck , Christin A
References J Cyst Fibros. 2016 Jun 4. pii: S1569-1993(16)30052-2
June 2016
Title: The Dynamics of Disease Progression in Cystic Fibrosis
Authors Adler FR, Liou TG
References PLoS One. 2016 Jun 1;11(6):e0156752. doi: 10.1371/journal.pone.0156752. eCollection 2016
Title: Cystic fibrosis in Latin America - Improving the awareness
Authors Luiz Vicente Ribeiro F. Silva Filho, Claudio Castaños, Héctor Hernán Ruíz
References J Cyst Fibros. 2016 Jun 1. pii: S1569-1993(16)30509-4.
Keywords Awareness, Diagnosis, Latin America, Management, Progress
May 2016
Title: Episodic oral antibiotic use in CF: discordance between the electronic medical record and self-report
Authors Lindsay J. Caverly, Tanner J. Caverly, Linda M. Kalikin, Bridget K. Foster, Richard H. Simon, John J. LiPuma
References J Cyst Fibros. 2016 May 4. pii: S1569-1993(16)30044-3
Keywords Bioelectrical impedance analysis, Body composition, Diaphragm, Fat-free mass, Lung function
Theme Epidemiology/Models of care
May 2016
Title: A false positive newborn screening result due to a complex allele carrying two frequent CF-causing variants.
Authors Bergougnoux A, Boureau-Wirth A, Rouzier C, Altieri JP, Verneau F, Larrieu L, Koenig M, Claustres M, Raynal C
References J Cyst Fibros. 2016 May;15(3):309-12
Keywords CFTR gene, Complex allele, False positive, Newborn screening
Title: A false positive newborn screening result due to a complex allele carrying two frequent CF-causing variants.
Authors Bergougnoux A, Boureau-Wirth A, Rouzier C, Altieri JP, Verneau F, Larrieu L, Koenig M, Claustres M, Raynal C
References J Cyst Fibros. 2016 May;15(3):309-12
Keywords CFTR gene, Complex allele, False positive, Newborn screening
April 2016
Title: Improving performance in the detection and management of cystic fibrosis-related diabetes in the Mountain West Cystic Fibrosis Consortium.
Authors Liou TG, Jensen JL, Allen SE, Brayshaw SJ, Brown MA, Chatfield B, Koenig J, McDonald C, Packer KA, Peet K, Radford P, Reineke LM, Otsuka K, Wagener JS, Young D, Marshall BC
References BMJ Open Diabetes Res Care. 2016 Apr 29;4(1):e000183. doi: 10.1136/bmjdrc-2015-000183.
Keywords Biomarker, Inflammation, Plasma, Pulmonary exacerbation
April 2016
Title: Improving performance in the detection and management of cystic fibrosis-related diabetes in the Mountain West Cystic Fibrosis Consortium
Authors Liou TG, Jensen JL, Allen SE, Brayshaw SJ, Brown MA, Chatfield B, Koenig J, McDonald C, Packer KA, Peet K, Radford P, Reineke LM, Otsuka K, Wagener JS, Young D, Marshall BC
References BMJ Open Diabetes Res Care. 2016 Apr 29;4(1):e000183. doi: 10.1136/bmjdrc-2015-000183. eCollection 2016.
April 2016
Title: Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene
Authors Dupuis A, Keenan K, Ooi CY, Dorfman R, Sontag MK, Naehrlich L, Castellani C, Strug, LJ Rommens JM, Gonska T
References Genet Med. 2016 Apr;18(4):333-40. doi: 10.1038/gim.2015.79
April 2016
Title: Controversies with Kalydeco: Newspaper Coverage in Canada and the Unites States of the Cystic Fibrosis “Wonder Drug”
Authors Christen Rachul, Maeghan Toews, Timothy Caulfield
References J Cyst Fibros. 2016 Apr 14. pii: S1569-1993(16)30015-7
Theme Epidemiology/Models of care
Title: Optimizing beta-lactam pharmacodynamics against Pseudomonas aeruginosa in adult cystic fibrosis patients.
Authors Thompson RZ, Martin CA, Burgess DR, Rutter WC, Burgess DS
References J Cyst Fibros. 2016 Apr 27. pii: S1569-1993(16)30019-4.
Keywords Beta-lactam, Continuous-infusion, Pharmacodynamics, Pharmacokinetics, Prolonged infusion, Pseudomonas aeruginosa
March 2016
Title: The Geographic Impact on Hospitalization in Patients with Cystic Fibrosis.
Authors Kopp BT, Nicholson L, Paul G, Tobias J, Ramanathan C, Hayes D Jr
References J Pediatr. 2016 Mar;170:246-52.e1-4. doi: 10.1016/j.jpeds.2015.11.012. Epub 2015 Dec 13.
March 2016
Title: Short-term and long-term response to pulmonary exacerbation treatment in cystic fibrosis.
Authors Heltshe SL, Goss CH, Thompson V, Sagel SD, Sanders DB, Marshall BC, Flume PA
References Thorax. 2016 Mar;71(3):223-9. doi: 10.1136/thoraxjnl-2014-206750. Epub 2015 Apr 24.
March 2016
Title: Comparison of two pancreatic enzyme products for exocrine insufficiency in patients with cystic fibrosis
Authors C.J. Taylor, R. Thieroff-Ekerdt, S. Shiff, L. Magnus, R. Fleming, C. Gommoll
References J Cyst Fibros. 2016 Mar 21. pii: S1569-1993(16)30001-7
Keywords Clinical trial, Enzepi, Exocrine pancreatic insufficiency (EPI), Pancreatic enzyme product (PEP), Zenpep
Theme Nutrition/Gastroenterology
Title: Extensive cultivation of soil and water samples yields various pathogens in patients with cystic fibrosis but not Burkholderia multivorans.
Authors Charlotte Peeters, Eliza Depoorter, Jessy Praet, Peter Vandamme
References J Cyst Fibros. 2016 Mar 17. pii: S1569-1993(16)30005-4
Keywords Burkholderia multivorans, Gram-negative non-fermenters, Isolation, Rapidly growing mycobacteria, Soil, Water
Title: Disease disclosure in individuals with cystic fibrosis: Association with psychosocial and health outcomes
Authors Adrienne P. Borschuk, Robin S. Everhart, Michelle N. Eakin, Devin Rand-Giovannetti, Belinda Borrelli, Kristin A. Riekert
References J Cyst Fibros. 2016 Mar 17. pii: S1569-1993(16)30002-9
Keywords Disclosure, Medication adherence, Self-efficacy, Social support
Title: Evaluation of a systems biology approach to identify pharmacological correctors of the mutant CFTR chloride channel
Authors Pesce E, Gorrieri G, Sirci F, Napolitano F, Carrella D, Caci E, Tomati V, Zegarra-Moran O, di Bernardo D, Galietta LJ
References J Cyst Fibros. 2016 Mar 10. pii: S1569-1993(16)00057-6
Keywords CFTR, Chloride channel, Corrector, F508del, Systems biology
February 2016
Title: Epidemiology of the Respiratory Bacteriology of Patients with Cystic Fibrosis
Authors Salsgiver EL, Fink AK, Knapp EA, LiPuma JJ, Olivier KN, Marshall BC, Saiman L. Changing
References Chest. 2016 Feb;149(2):390-400. doi: 10.1378/chest.15-0676.
Keywords Pseudomonas aeruginosa, Staphylococcus aureus, registry
Title: Assessing Differences in Mortality Rates and Risk Factors Between Hispanic and Non-Hispanic Patients With Cystic Fibrosis in California.
Authors Buu MC, Sanders LM, Mayo JA, Milla CE, Wise PH
References Chest. 2016 Feb;149(2):380-389. doi: 10.1378/chest.14-2189. Epub 2016 Jan 12.
Keywords ethnicity, health disparities, pediatric pulmonology
February 2016
Title: Clinical outcomes in cystic fibrosis patients with Trichosporon respiratory infection
Authors Charles R. Esther, Rongpong Plongla, Alan Kerr, Feng-Chang Lin, Peter Gilligan
References J Cyst Fibros. 2016 Feb 27. pii: S1569-1993(16)00037-0
Keywords Chryseobacterium, Lung function
Title: Safety and Efficacy of Prolonged Levofloxacin Inhalation Solution (1 APT-1026) Treatment for Cystic Fibrosis and Chronic Pseudomonas aeruginosa Airway Infection
Authors J. Stuart Elborn, Patrick A. Flume, Fredric Cohen, Jeffery Loutit, Donald R. VanDevanter
References J Cyst Fibros. 2016 Feb 26. pii: S1569-1993(16)00012-6
Title: The role of basophil activation test in allergic bronchopulmonary aspergillosis and Aspergillus fumigatus sensitization in cystic fibrosis patients
Authors Anna Katelari, Marianna Tzanoudaki, Maria Noni, Maria Kanariou, Maria Theodoridou, Emmanuel Kanavakis, Stavros-Eleftherios Doudounakis, Christina Kanaka-Gantenbein
References J Cyst Fibros. 2016 Feb 25. pii: S1569-1993(16)00016-3
Keywords Allergic bronchopulmonary aspergillosis, Aspergillus fumigatus, Aspergillus fumigatus sensitization, Basophil activation test
Theme Epidemiology/Models of care
Title: Glycated hemoglobin cannot yet be proposed as a screening tool for cystic fibrosis related diabetes
Authors Boudreau V, Coriati A, Desjardins K, Rabasa-Lhoret R
References J Cyst Fibros. 2016 Feb 19. pii: S1569-1993(16)00017-5
Keywords Adult, CFRD, Glycated hemoglobin, Oral glucose tolerance test
Theme Diagnosis/Screening
Title: International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: associated factors and outcome.
Authors Anne Munck, Corinne Alberti, Carla Colombo, Nataliya Kashirskaya, Helmut Ellemunter, Maria Fotoulaki, Roderick Houwen, Eddy Robberecht, Priscilla Boizeau, Michael Wilschanski on behalf of the CF/Pancreas ESPGHAN Working Group and DIOS Study Group
References J Cyst Fibros. 2016 Feb 23. pii: S1569-1993(16)00014-X.
Keywords Abdominal pain, Distal intestinal obstruction syndrome, Incidence
Theme Nutrition/Gastroenterology
Title: 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
Authors Patrick A. Flume, Donald R. VanDevanter, Elizabeth E. Morgan, Michael N. Dudley, Jeffery S. Loutit, Scott C. Bell, Eitan Kerem, Rainald Fischer, Alan R. Smyth, Shawn D. Aaron, Douglas Conrad, David E. Geller, J. Stuart Elborn on behalf of the APT investigators
References J Cyst Fibros. 2016 Feb 4. pii: S1569-1993(15)00276-3.
Keywords Aerosol, Antibiotics, Fluoroquinolone, Pseudomonas
January 2016
Title: Measurement of fecal elastase improves performance of newborn screening for cystic fibrosis.
Authors Barben J, Rueegg CS, Jurca M, Spalinger J, Kuehni CE, Swiss Cystic Fibrosis Screening Group
References J Cyst Fibros. 2016 Jan 27. pii: S1569-1993(16)00002-3
Keywords CFSPID, Fecal elastase, Newborn screening
January 2016
Title: Microbiological yield from induced sputum compared to oropharyngeal swab in young children with cystic fibrosis
Authors Marco Zampoli, Komala Pillay, Henri Carrara, Heather J Zar, Brenda Morrow
References J Cyst Fibros. 2016 Jan 26. pii: S1569-1993(16)00008-4.
Keywords Induced sputum, Oropharyngeal swab, Young children
January 2016
Title: Host Response to Staphylococcus aureus Cytotoxins In Children with Cystic
Authors Ashley D. Chadha, Isaac P. Thomsen, Natalia Jimenez-Truque, Nicole R. Soper, Lauren S. Jones, Andrew G. Sokolow, Victor J. Torres, C. Buddy Creech
References J Cyst Fibros. 2016 Jan 25. pii: S1569-1993(15)00318-5
Keywords Exotoxins, LukAB, Serology, Staphylococcus aureus
January 2016
Title: Ultrasound and magnetic resonance imaging assessment of joint disease in symptomatic patients with Cystic Fibrosis Arthropathy
Authors G Fitch, K Williams, JE Freeston, S Dass, A Grainger, R Hodgson, L Horton, P Whitaker, D Peckham
References J Cyst Fibros. 2016 Jan 19. pii: S1569-1993(15)00317-3
Keywords Arthropathy, Joint count, MRI, Ultrasound
January 2016
Title: Implementation of newborn screening for Cystic Fibrosis in Norway. Results from the first three years
Authors Emma Lundman, H. Junita Gaup, Egil Bakkeheim, Edda J. Olafsdottir, Terje Rootwelt, Olav Trond Storrøsten, Rolf D. Pettersen
References J Cyst Fibros. 2016 Jan 12. pii: S1569-1993(15)00312-4
Keywords Newborn screening
January 2016
Title: YKL-40 as marker of severe lung disease in cystic fibrosis patients.
Authors Leonardi S, Parisi GF, Capizzi A, Manti S, Cuppari C, Scuderi MG, Rotolo N, Lanzafame A, Musumeci M, Salpietro C
References J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00315-X.
Keywords Acute pulmonary exacerbation, Lung function decline, Pulmonary pseudomonas infection, YKL-40
Title: Distribution of the species of Achromobacter in a French Cystic Fibrosis centre and Multilocus Sequence Typing analysis reveal the predominance of A. xylosoxidans and clonal relationships between some clinical and environmental isolates
Authors Lucie Amoureux, Julien Bador, Fatma Bounoua Zouak, Angélique Chapuis, Claire deCurraize, Catherine Neuwirth
References J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00281-7
Keywords Achromobacter spp., Cystic fibrosis, Environment, Epidemiology, Genotyping, MLST, nNrdA
Title: Gastric emptying and gastro-oesophageal reflux in children with cystic fibrosis
Authors Hauser B, De Schepper J, Malfroot A, De Wachter E, De Schutter I, Keymolen K, Vandenplas Y
References J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00287-8
Keywords (13)C-gastric emptying breath test, Children, Gastric emptying, Gastro-oesophageal reflux, Multichannel intraluminal impedance-pH monitoring
Theme Nutrition/Gastroenterology
January 2016
Title: Low sodium status in cystic fibrosis-as assessed by calculating fractional Na+ excretion-is associated with decreased growth parameters.
Authors Knepper C, Ellemunter H, Eder J, Niedermayr K, Haerter B, Hofer P, Scholl-Bürgi S, Müller T, Heinz-Erian P
References J Cyst Fibros. 2016 Jan 6. pii: S1569-1993(15)00282-9
Keywords Fractional sodium excretion, Growth retardation, Normonatremic sodium depletion
Theme Nutrition/Gastroenterology
December 2015
Title: Preliminary Comparison of Normalized T1 and Non-Contrast Perfusion MRI Assessments of Regional Lung Disease in People with Cystic Fibrosis
Authors Shannon B. Donnola, Elliott C. Dasenbrook, David Weaver, Lan Lu, Karishma Gupta, Anjali Prabhakaran, Xin Yu, James F. Chmiel, Kimberly McBennett, Michael W. Konstan, Mitchell L. Drumm, Chris A. Flask
References J Cyst Fibros. 2015 Dec 21. pii: S1569-1993(15)00267-2
Keywords Imaging, Lung disease, Magnetic Resonance Imaging, Perfusion, Relaxometry
Title: Psychological resilience and intolerance of uncertainty in coping with cystic fibrosis
Authors Horst Mitmansgruber, Ulrike Smrekar, Bianca Rabanser, Thomas Beck, Johannes Eder, Helmut Ellemunter
References J Cyst Fibros. 2015 Dec 10. pii: S1569-1993(15)00269-6
Keywords Intolerance of uncertainty, Psychological well-being, Quality of life, Resilience
Title: Epidemic Pseudomonas aeruginosa Infection in Patients with Cystic Fibrosis is Not a Risk Factor for Poor Clinical Outcomes Following Lung Transplantation
Authors Julia Pritchard, Mitesh V. Thakrar, Ranjani Somayaji, Michael G. Surette, Harvey R. Rabin, Doug Helmersen, Dale Lien, Swathi Purighalla, Barbara Waddell, Michael D. Parkins
References J Cyst Fibros. 2015 Dec 2. pii: S1569-1993(15)00262-3
Keywords Bronchiectasis, Liverpool epidemic strain, Multi-drug resistant, Prairie Epidemic Strain, Transmissible, Transplant
Title: Bronchial epithelial cell lines and primary nasal epithelial cells from cystic fibrosis respond differently to cigarette smoke exposure
Authors Mark Thomas Shaw Williams, Francine de Courcey, David Comer, Joseph S Elborn, Madeleine Ennis
References J Cyst Fibros. 2015 Dec 1. pii: S1569-1993(15)00260-X
Keywords Airway, Bronchial, CSE, Cells, Cytokine, EGFR, Epithelial, F508del, Healthy, IL-8, Immortalised, Inflammation, Nasal, Patients, Primary, R117H, Release
November 2015
Title: Early Life Growth Trajectories in Cystic Fibrosis are Associated with Pulmonary Function at Age 6 Years.
Authors Sanders DB, Fink A, Mayer-Hamblett N, Schechter M, Sawicki GS, Rosenfeld M, Flume PA, Morgan WJ
References Journal of Pediatrics. 2015 Nov;167(5):1081-8.e1. PMID: 26340874
November 2015
Title: A Longitudinal Analysis of Chronic MRSA and Pseudomonas aeruginosa Co-infection in Cystic Fibrosis: A Single-Center Study
Authors Maret L. Maliniaka, Arlene A. Stecenkoa, Nael A. McCarty
References J Cyst Fibros. 2015 Nov 20. pii: S1569-1993(15)00258-1
Keywords Methicillin-resistant Staphylococcus aureus, Pseudomonas aeruginosa, Pulmonary function tests, Longitudinal studies, Epidemiology
October 2015
Title: Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis
Authors Leung DH, Ye W, Molleston JP, Weymann A, Ling S, Paranjape SM, Romero R, Schwarzenberg SJ, Palermo J, Alonso EM, Murray KF, Marshall BC, Sherker AH, Siegel MJ, Krishnamurthy R, Harned R, Karmazyn B, Magee JC, Narkewicz MR; Cystic Fibrosis Liver Disease Ne
References J Pediatr. 2015 Oct;167(4):862-868.e2. doi: 10.1016/j.jpeds.2015.06.062.
September 2015
Title: Genome-wide association meta-analysis identifies five modifier loci of lung disease severity in cystic fibrosis
Authors Corvol H, Blackman SM, Boëlle PY, Gallins PJ, Pace RG, Stonebraker JR, Accurso FJ, Clement A, Collaco JM, Dang H, Dang AT, Franca A, Gong J, Guillot L, Keenan K, Li W, Lin F, Patrone MV, Raraigh KS, Sun L, Zhou YH, O'Neal WK, Sontag MK, Levy H, Durie PR,
References Nat Commun. 2015 Sep 29;6:8382. doi: 10.1038/ncomms9382.
September 2015
Title: Outcomes and Treatment of Chronic Methicillin-Resistant Staphylococcus aureus Differs by Staphylococcal Cassette Chromosome mec (SCCmec) Type in Children With Cystic Fibrosis
Authors Heltshe SL, Saiman L, Popowitch EB, Miller MB, Kloster M, Thompson V, Ferkol TW, Hoover WC, Schechter MS, Muhlebach MS.
References J Pediatric Infect Dis Soc. 2015 Sep;4(3):225-31. doi: 10.1093/jpids/piu048. Epub 2014 Jun 9.
Keywords PVL, Panton-Valentine leukocidin, SCCmec type, methicillin-resistant Staphylococcus aureus; pulmonary exacerbation
August 2015
Title: Rate of Uptake of Ivacaftor Use after U.S. Food and Drug Administration Approval among Patients Enrolled in the U.S. Cystic Fibrosis Foundation Patient Registry
Authors Sawicki GS, Dasenbrook E, Fink AK, Schechter MS
References Ann Am Thorac Soc. 2015 Aug;12(8):1146-52. doi: 10.1513/AnnalsATS.201504-214OC
Keywords Ivacaftor, patient registry
June 2015
Title: Multicenter Observational Study on Factors and Outcomes Associated with Various Methicillin-Resistant Staphylococcus aureus Types in Children with Cystic Fibrosis
Authors Muhlebach MS, Heltshe SL, Popowitch EB, Miller MB, Thompson V, Kloster M, Ferkol T, Hoover WC, Schechter MS, Saiman L; STAR-CF Study Team
References Ann Am Thorac Soc. 2015 Jun;12(6):864-71.
Keywords Panton–Valentine leukocidin, SCCmec type, clinic visits, Methicillin-resistant Staphylococcus aureus, risk factor
May 2015
Title: Fine particulate matter exposure and initial Pseudomonas aeruginosa acquisition in cystic fibrosis.
Authors Psoter KJ, De Roos AJ, Mayer JD, Kaufman JD, Wakefield J, Rosenfeld M.
References Ann Am Thorac Soc. 2015 Mar;12(3):385-91. doi: 10.1513/AnnalsATS
Keywords Pseudomonas aeruginosa, ambient air pollution, Epidemiology, Fine particulate matter
Title: Effect of treatment of cystic fibrosis pulmonary exacerbations on systemic inflammation.
Authors Sagel SD, Thompson V, Chmiel JF, Montgomery GS, Nasr SZ, Perkett E, Saavedra MT, Slovis B, Anthony MM, Emmett P, Heltshe SL.
References Ann Am Thorac Soc. 2015 May;12(5):708-17
Keywords Biomarker, Plasma, Pulmonary exacerbation
May 2015
Title: Outcomes of infants with indeterminate diagnosis detected by cystic fibrosis newborn screening.
Authors Ren CL, Fink AK, Petren K, Borowitz DS, McColley SA, Sanders DB, Rosenfeld M, Marshall BC.
References Pediatrics. 2015 Jun;135(6):e1386-92. doi: 10.1542/peds.2014-3698. Epub 2015 May 11.
March 2015
Title: Comparison of WHO and CDC growth charts in predicting pulmonary outcomes in cystic fibrosis
Authors Machogu E, Cao Y, Miller T, Simpson P, Levy H, Quintero D, Goday PS.
References J Pediatr Gastroenterol Nutr. 2015 Mar;60(3):378-83. doi: 10.1097/MPG.0000000000000610.
January 2015
Title: Characterization of Missing Data in Clinical Registry Studies.
Authors Mendelsohn AB, Dreyer NA, Mattox PW, Su Z, Swenson A, Li R, Turner JR, Velentgas P.
References Ther Innov Regul Sci. 2015 Jan;49(1):146-154. doi: 10.1177/2168479014532259.
Keywords missing data, missingness, observational, registry, research, studies
December 2014
Title: Gender Differences in Outcomes of Patients with Cystic Fibrosis
Authors Harness-Brumley CL, Elliott AC, Rosenbluth DB, Raghavan D, Jain R.
References J Womens Health (Larchmt). 2014 Dec;23(12):1012-20. doi: 10.1089/jwh.2014.4985.
December 2014
Title: Improvements in lung function and height among cohorts of 6-year-olds with cystic fibrosis from 1994 to 2012
Authors VanDevanter DR, Pasta DJ, Konstan MW.
References J Pediatr. 2014 Dec;165(6):1091-1097.e2. doi: 10.1016/j.jpeds.2014.06.061
June 2014
Title: Differential geographical risk of initial Pseudomonas aeruginosa acquisition in young U.S. children with cystic fibrosis.
Authors Psoter KJ, Rosenfeld M, De Roos AJ, Mayer JD, Wakefield J.
References Am J Epidemiol. 2014 Jun 15;179(12):1503-13. doi: 10.1093/aje/kwu077.
Keywords Bayesian hierarchical spatial models, Pseudomonas aeruginosa, Weibull regression, interval censoring, intrinsic conditional autoregression
March 2014
Title: Antimicrobial susceptibility and molecular typing of MRSA in cystic fibrosis.
Authors Champion EA, Miller MB, Popowitch EB, Hobbs MM, Saiman L, Muhlebach MS; STAR-CF Study Team.
References Pediatr Pulmonol. 2014 Mar;49(3):230-7. doi: 10.1002/ppul.22815
Keywords Panton-Valentine leukocidin, community MRSA, hospital MRSA, multi-center study, spa-typing; staphylococcal cassette chromosome mec
March 2014
Title: Modeling the impact of antibiotic exposure on human microbiota
Authors Zhao J, Murray S, Lipuma JJ.
References Sci Rep. 2014 Mar 11;4:4345. doi: 10.1038/srep04345
January 2014
Title: Effects of puberty on cystic fibrosis related pulmonary exacerbations in women versus men
Authors Sutton S, Rosenbluth D, Raghavan D, Zheng J, Jain
References Pediatr Pulmonol. 2014 Jan;49(1):28-35. doi: 10.1002/ppul.22767
Keywords Exacerbation, gender disparity, puberty
December 2013
Title: A semiparametric approach to estimate rapid lung function decline in cystic fibrosis
Authors Szczesniak RD, McPhail GL, Duan LL, Macaluso M, Amin RS, Clancy JP
References Ann Epidemiol. 2013 Dec;23(12):771-7. doi: 10.1016/j.annepidem.2013.08.009. Epub 2013 Oct 5
Keywords Epidemiologic methods, Nonlinear models, Spline regression
October 2013
Title: Genetic modifiers of cystic fibrosis-related diabetes.
Authors Blackman SM, Commander CW, Watson C, Arcara KM, Strug LJ, Stonebraker JR, Wright FA, Rommens JM, Sun L, Pace RG, Norris SA, Durie PR, Drumm ML, Knowles MR, Cutting GR.
References Diabetes. 2013 Oct;62(10):3627-35. doi: 10.2337/db13-0510.
June 2013
Title: Season is associated with Pseudomonas aeruginosa acquisition in young children with cystic fibrosis.
Authors Psoter KJ, De Roos AJ, Wakefield J, Mayer J, Rosenfeld M.
References Clin Microbiol Infect. 2013 Nov;19(11):E483-9. doi: 10.1111/1469-0691.12272. Epub 2013 Jun 25.
Keywords Acquisition, Pseudomonas aeruginosa, Climate, Seasonality
November 2011
Title: Effect of temperature on cystic fibrosis lung disease and infections: a replicated cohort study.
Authors Collaco JM, McGready J, Green DM, Naughton KM, Watson CP, Shields T, Bell SC, Wainwright CE; ACFBAL Study Group, Cutting GR
References PLoS One. 2011;6(11):e27784. doi: 10.1371/journal.pone.0027784. Epub 2011 Nov 1
September 2011
Title: A Novel Lung Disease Phenotype Adjusted for Mortality Attrition for Cystic Fibrosis Genetic Modifier Studies
Authors Taylor C, Commander CW, Collaco JM, Strug LJ, Li W, Wright FA, Webel AD, Pace RG, Stonebraker JR, Naughton K, Dorfman R, Sandford A, Blackman SM, Berthiaume Y, Paré P, Drumm ML, Zielenski J, Durie P, Cutting GR, Knowles MR, Corey M.
References Pediatr Pulmonol. 2011 Sep;46(9):857-69. doi: 10.1002/ppul.21456
January 2006
Title: Clinical trial participants compared with nonparticipants in cystic fibrosis
Authors Goss CH, Rubenfeld GD, Ramsey BW, Aitken ML.
References Am J Respir Crit Care Med. 2006 Jan 1;173(1):98-104
January 2004
Title: Effect of ambient air pollution on pulmonary exacerbations and lung function in cystic fibrosis.
Authors Goss CH, Newsom SA, Schildcrout JS, Sheppard L, Kaufman JD
References Am J Respir Crit Care Med. 2004 Apr 1;169(7):816-21. Epub 2004 Jan 12.
