March 2018
Title: Defining Research Priorities in Cystic Fibrosis. Can Existing Knowledge and Training in Biomedical Research affect the Choice?
Authors Roberto Buzzetti, Valeria Galici, Natalia Cirilli, Fabio Majo, Luigi Graziano, Stefano Costa, Simona Bonacina, Marila Carrubba, Giuseppe Davì, Sabrina Gagliano, Clizia Cazzarolli, Francesca Ficili, Federico Alghisi, Michele Samaja, Giuseppe Magazzù, CF Italian Patient Centered Outcomes Research Working Group (IPaCOR)
References J Cyst Fibros. 2018 Mar 15. pii: S1569-1993(18)30032-8. doi: 10.1016/j.jcf.2018.02.009.
March 2018
Title: Implementing the International Committee on Mental Health in Cystic Fibrosis (ICMH) Guidelines: Screening accuracy and referral-treatment pathways
Authors Marieke Verkleij, Derek de Winter, Margaret Anne Hurley, Janice Abbott
References J Cyst Fibros. 2018 Mar 1. pii: S1569-1993(18)30028-6. doi: 10.1016/j.jcf.2018.02.005.
Keywords Adolescent, anxiety, Caregiver, depression
February 2018
Title: Cystic fibrosis epithelial cells are primed for apoptosis as a result of increased Fas (CD95).
Authors Chen Q, Pandi SPS, Kerrigan L, McElvaney NG, Greene CM, Elborn JS, Taggart CC, Weldon S.
References J Cyst Fibros. 2018 Feb 24. pii: S1569-1993(18)30013-4. doi: 10.1016/j.jcf.2018.01.010.
Keywords Apoptosis, Epithelial cells, Fas
February 2018
Title: Molecular characterization of gene regulatory networks in primary human tracheal and bronchial epithelial cells.
Authors Gillen AE, Yang R, Cotton CU, Perez A, Randell SH, Leir SH, Harris
References J Cyst Fibros. 2018 Feb 17. pii: S1569-1993(18)30012-2. doi: 10.1016/j.jcf.2018.01.009
Keywords Airway epithelium, DNase-seq, Molecular signature, Open chromatin, Primary human bronchial cells, Primary human tracheal cells, RNA-seq, Transcriptional networks
February 2018
Title: Mast cell tryptase changes with Aspergillus fumigatus - Host crosstalk in cystic fibrosis patients.
Authors Gomez C, Carsin A, Gouitaa M, Reynaud-Gaubert M, Dubus JC, Mège JL, Ranque S, Vitte J
References J Cyst Fibros. 2018 Feb 15. pii: S1569-1993(18)30015-8. doi: 10.1016/j.jcf.2018.01.012.
Keywords Aspergillus fumigatus, Immunoglobulin E, Lung transplantation, Mast cell tryptase
February 2018
Title: Risk factors for persistent Aspergillus respiratory isolation in cystic fibrosis.
Authors Hong G, Psoter KJ, Jennings MT, Merlo CA, Boyle MP, Hadjiliadis D, Kawut SM, Lechtzin N
References J Cyst Fibros. 2018 Feb 12. pii: S1569-1993(18)30011-0. doi: 10.1016/j.jcf.2018.01.008
Keywords Aspergillus, Epidemiology, Fungus, Inhaled antibiotics
February 2018
Title: Early detection using qPCR of Pseudomonas aeruginosa infection in children with cystic fibrosis undergoing eradication treatment
Authors Ana C. Blanchard, Ashley M. RooneyEmail the author Ashley M. Rooney, Yvonne YauEmail the author Yvonne Yau, Yu ZhangEmail the author Yu Zhang, Patrick J. StapletonEmail the author Patrick J. Stapleton, Eric HortonEmail the author Eric Horton, Michelle Kli
February 2018
Title: Comparison of facemask and mouthpiece interfaces for Multiple Breath Washout measurements
Authors Paul D Robinson, Sooky Lum, Courtney Moore, Kate M Hardaker, Nick Benseler, Paul Aurora, Peter Cooper, Dominic Fitzgerald, Renee Jensen, Reginald McDonald, Hiran Selvadurai, Felix Ratjen, Sanja Stanojevic
January 2018
Title: Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis.
Authors Sanders DB, Zhang Z, Farrell PM, Lai HJ; Wisconsin CF Neonatal Screening Group.
References J Cyst Fibros. 2018 Jan 30. pii: S1569-1993(18)30009-2. doi: 10.1016/j.jcf.2018.01.006.
January 2018
Title: The effect of enteral tube feeding in cystic fibrosis: A registry based study.
Authors Libeert D, Declercq D, Wanyama S, Thomas M, Van Daele S, De Baets, Van Biervliet S
References J Cyst Fibros. 2018 Jan 21. pii: S1569-1993(18)30007-9. doi: 10.1016/j.jcf.2018.01.004.
Keywords Gastrostom, ; Malnutrition, Pulmonary function, Tube feeding
January 2018
Title: Hypertonic saline has a prolonged effect on mucociliary clearance in adults with cystic fibrosis
Authors Trimble AT, Whitney Brown A, Laube BL, Lechtzin N, Zeman KL, Wu J, Ceppe A, Waltz D, Bennett WD, Donaldson SH
References J Cyst Fibros. 2018 Jan 19. pii: S1569-1993(18)30004-3. doi: 10.1016/j.jcf.2018.01.001
January 2018
Title: The relationship between sweat chloride levels and mortality in cystic fibrosis varies by individual genotype.
Authors Espel JC, Palac HL, Bharat A, Cullina J, Prickett M, Sala M, McColley SA, Jain M
References J Cyst Fibros. 2018 Jan;17(1):34-42. doi: 10.1016/j.jcf.2017.11.002. Epub 2017 Dec 6.
Keywords CFTR genotype, mortality, mutation, sweat chloride
Title: Drugs during pregnancy and breast feeding in women diagnosed with Cystic Fibrosis – an update
Authors M.A.G.M. Kroon, A.M. Akkerman-Nijland, B.L. Rottier, G.H. Koppelman, O.W. Akkerman, D.J. Touw
References J Cyst Fibros. 2018 Jan;17(1):17-25. doi: 10.1016/j.jcf.2017.11.009. Epub 2017 Dec 7
Keywords breast feeding, CFTR modulators, pregnancy
January 2018
Title: One time quantitative PCR detection of Pseudomonas aeruginosa to discriminate intermittent from chronic infection in cystic fibrosis
Authors Sébastien Boutin, Michael Weitnauer, Selina Hassel, Simon Y. Graeber, Mirjam Stahl, A. Susanne Dittrich, Marcus A. Mall, Alexander H. Dalpke
References J Cyst Fibros. 2018 Jan 11. pii: S1569-1993(18)30001-8. doi: 10.1016/j.jcf.2017.12.007
January 2018
Title: Quantifying fluctuation in glucose levels to identify early changes in glucose homeostasis in cystic fibrosis.
Authors Brugha R, Wright M, Nolan S, Bridges N, Carr SB
References J Cyst Fibros. 2018 Jan 9. pii: S1569-1993(17)30982-7. doi: 10.1016/j.jcf.2017.12.004
Keywords continuous glucose monitoring, Cystic fibrosis related diabetes
December 2017
Title: KB001-A, a novel anti-inflammatory, found to be safe and well-tolerated in cystic fibrosis patients infected with Pseudomonas aeruginosa.
Authors Jain R, Beckett VV, Konstan MW, Accurso FJ, Burns JL, Mayer-Hamblett N, Milla C, VanDevanter DR, Chmiel JF; KB001-A Study Group
References J Cyst Fibros. 2017 Dec 29. pii: S1569-1993(17)30984-0. doi: 10.1016/j.jcf.2017.12.006
December 2017
Title: Quality of life and mood in children with cystic fibrosis: Associations with sleep quality.
Authors Vandeleur M, Walter LM, Armstrong DS, Robinson P, Nixon GM, Horne RSC.
References J Cyst Fibros. 2017 Dec 24. pii: S1569-1993(17)30978-5. doi: 10.1016/j.jcf.2017.11.021.
Keywords Children, Mood, Quality of life, Sleep
December 2017
Title: The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosis
Authors Daan Caudri, Lidija Turkovic, Jolyn Ng, Nicholas H de Klerk,Tim Rosenow, Graham L Hall, Sarath C Ranganathan, Peter D Sly, Stephen M Stick, on behalf of AREST CF
References J Cyst Fibros. 2017 Dec 21. pii: S1569-1993(17)30980-3. doi: 10.1016/j.jcf.2017.12.002
Keywords CT scan, Infection and inflammation, Lung function, Structural lung damage
December 2017
Title: Effect of Lumacaftor/Ivacaftor on glucose metabolism and insulin secretion in Phe508del homozygous cystic fibrosis patients
Authors Jan C. Thomassen, Matthias I. Mueller, Miguel A. Alejandre Alcazar, Ernst Rietschel and Silke van Koningsbruggen-Rietschel
References J Cyst Fibros. 2017 Dec 15. pii: S1569-1993(17)30973-6. doi: 10.1016/j.jcf.2017.11.016.
Keywords glucose metabolism, homozygous Phe508del, IVGTT, insulin secretion, Lumacaftor/ivacaftor, OGTT
Title: Hypoglycaemia (low blood sugar) in cystic fibrosis: an analysis of single centre adult cystic fibrosis clinic
Authors Natasha Armaghanian, Tania P Markovic, Jennie C Brand-Miller, Peter TP Bye, Carmel P Moriarty, Kate S Steinbeck
References J Cyst Fibros. 2017 Dec 15. pii: S1569-1993(17)30972-4. doi: 10.1016/j.jcf.2017.11.015.
Keywords hypoglycaemia, Oral glucose tolerance test
December 2017
Title: Resolvin D1 regulates epithelial ion transport and inflammation in cystic fibrosis airways
Authors Fiona C Ringholz, Gerard Higgins, Aurélie Hatton, Ali Sassi, Ahmad Moukachar, Coral Fustero-Torre, Monika Hollenhorst, Isabelle Sermet-Gaudelus, Brian J. Harvey, Paul McNally, Valerie Urbach
References J Cyst Fibros. 2017 Dec 7. pii: S1569-1993(17)30974-8. doi: 10.1016/j.jcf.2017.11.017
Keywords Airway surface liquid layer, CF alveolar macrophages, CF mice, ENaC, F508del-CFTR, Resolvin D1
November 2017
Title: FAM13A is a modifier gene of cystic fibrosis lung phenotype regulating rhoa activity, actin cytoskeleton dynamics and epithelial-mesenchymal transition
Authors Harriet Corvol, Nathalie Rousselet, Kristin E. Thompson, Laura Berdah, Guillaume Cottin, Tobias Foussigniere, Elisabeth Longchampt, Laurence Fiette, Edouard Sage, Céline Prunier, Mitchell Drumm, Craig A. Hodges, Pierre-Yves Boëlle, Loic Guillot
Keywords Modifier genes, FAM13A
November 2017
Title: Development and electronic validation of the revised Cystic Fibrosis Questionnaire (CFQ-R Teen/Adult): New tool for monitoring psychosocial health in CF.
Authors Solé A, Olveira C, Pérez I, Hervás D, Valentine V, Baca Yepez AN, Olveira G, Quittner A
References J Cyst Fibros. 2017 Nov 17. pii: S1569-1993(17)30954-2. doi: 10.1016/j.jcf.2017.10.015.
Keywords Cystic Fibrosis Questionnaire Revised, Diagnostic tool, Patient reported outcomes
Theme Clinical Trials, Diabetes, Diagnosis/Screening, Endocrinology, Epidemiology/Models of care, Exercise, False-positives, Imaging, Immunology, Lung Disease and Infection, Microbiology, Newborn screening, Nutrition/Gastroenterology, Parents, Physiotherapy/Exercise testing, Pregnancy with CF, Psychosocial
November 2017
Title: Caregiver burden of parents of young children with cystic fibrosis
Authors Fitzgerald C, George S, Somerville R, Linnane B, Fitzpatrick P
References J Cyst Fibros. 2017 Nov 14. pii: S1569-1993(17)30879-2. doi: 10.1016/j.jcf.2017.08.016
Keywords Caregiver burden, CarerQol, Parents
November 2017
Title: Lack of efficacy of Lactobacillus GG in reducing pulmonary exacerbations and hospital admissions in children with cystic fibrosis: A randomised placebo controlled trial.
Authors Bruzzese E, Raia V, Ruberto E, Scotto R, Giannattasio A, Bruzzese D, Cavicchi MC, Francalanci M, Colombo C, Faelli N, Daccò V, Magazzù G, Costa S, Lucidi V, Majo F, Guarino A
References J Cyst Fibros. 2017 Nov 8. pii: S1569-1993(17)30951-7.
November 2017
Title: Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR
Authors Jennifer L. Taylor-Cousar, Manu Jain, Tara Lynn Barto, Tarik Haddad, Jeffrey Atkinson, Simon Tian, Rui Tang, Gautham Marigowda, David Waltz, Joseph Pilewski on behalf of the VX14-809-106 Investigator Group
References J Cyst Fibros. 2017 Nov 7. pii: S1569-1993(17)30891-3. doi: 10.1016/j.jcf.2017.09.012
Keywords Advanced lung disease, F508del, Ivacaftor, Lumacaftor, Severe lung dysfunction
November 2017
Title: Ceftaroline Pharmacokinetics and Pharmacodynamics in Patients with Cystic Fibrosis
Authors Emily E. Barsky, Luis M. Pereira, Keri J. Sullivan, Alanna Wong, Alexander J. McAdam, Gregory S. Sawicki, Gregory P. Priebe, Susan M. Goobie
References J Cyst Fibros. 2017 Nov 2. pii: S1569-1993(17)30947-5. doi: 10.1016/j.jcf.2017.10.010
Keywords Ceftaroline, Infection, MRSA, Pharmacodynamics, Pharmacokinetics
October 2017
Title: Lung Function Decline is Delayed But Not Decreased in Patients With Cystic Fibrosis and the R117H Gene Mutation
Authors Jeffrey S. Wagener, Stefanie J. Millar, Nicole Mayer-Hamblett, Gregory S. Sawicki, Edward F. McKone6, Christopher H. Goss, Michael W. Konstan, Wayne J. Morgan, David J. Pasta2, Richard B. Moss
References J Cyst Fibros. 2017 Oct 31. pii: S1569-1993(17)30912-8. doi: 10.1016/j.jcf.2017.10.003
Keywords F508del, Lung function, Lung function decline, R117H
October 2017
Title: Defining Palliative Care in Cystic Fibrosis: A Delphi Study
Authors Dellon EP, Goggin J, Chen E, Sabadosa K, Hempstead SE, Faro A, Homa K
References J Cyst Fibros. 2017 Oct 30. pii: S1569-1993(17)30948-7. doi: 10.1016/j.jcf.2017.10.011.
Keywords Palliative care, Quality of life
October 2017
Title: Do cystic fibrosis centres with the lowest Fev1 still use the least amount of intravenous antibiotics? A registry-based comparison of intravenous antibiotic use among adult CF centres in the UK
Authors Zhe Hui Hoo, Michael J. Campbell, Rachael Curley, Stephen J. Walters, Martin J. Wildman
References J Cyst Fibros. 2017 Oct 23. pii: S1569-1993(17)30916-5. doi: 10.1016/j.jcf.2017.10.005
Keywords Clinical epidemiology, Intravenous antibiotic, Pulmonary exacerbation, Registry analysis
October 2017
Title: IgG avidity to Pseudomonas aeruginosa over the course of chronic lung biofilm infection in cystic fibrosis
Authors Renan Marrichi Mauch, Lena Lingren Nørregaard, Oana Ciofu, Carlos Emilio Levy, Niels Høiby
References J Cyst Fibros. 2017 Oct 11. pii: S1569-1993(17)30883-4. doi: 10.1016/j.jcf.2017.09.004
Keywords Biofilm, Chronic infection, Humoral response, IgG avidity, Pseudomonas aeruginosa
September 2017
Title: Blood flow regulation and oxidative stress during submaximal cycling exercise in patients with cystic fibrosis.
Authors Tucker MA, Berry B, Seigler N, Davison GW, Quindry JC, Eidson D, McKie KT, Harris RA.
References J Cyst Fibros. 2017 Sep 8. pii: S1569-1993(17)30878-0. doi: 10.1016/j.jcf.2017.08.015.
Keywords Electron paramagnetic resonance spectroscopy, Exercise intolerance, Reactive oxygen species, Retrograde velocity
September 2017
Title: Real life practice of sweat testing in Europe.
Authors Cirilli N, Southern KW, Buzzetti R, Barben J, Nährlich L, Munck A, Wilschanski M, De Boeck K, Derichs N, of the ECFS Diagnostic Network Working Group.
References J Cyst Fibros. 2017 Sep 27. pii: S1569-1993(17)30881-0. doi: 10.1016/j.jcf.2017.09.002.
September 2017
Title: End-of-life practice patterns at U.S. adult cystic fibrosis care centers: A national retrospective chart review.
Authors Chen E, Homa K, Goggin J, Sabadosa KA, Hempstead S, Marshall BC, Faro A, Dellon EP.
References J Cyst Fibros. 2017 Sep 13. pii: S1569-1993(17)30873-1. doi: 10.1016/j.jcf.2017.08.010
Keywords Advance care planning, End-of-life care, Hospice, Lung transplant, Palliative care
August 2017
Title: Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence.
Authors Anna M. Georgiopoulos, Deborah Friedman, Elizabeth A. Porter, Amy Krasner Sheetal P. Kakarala, Breanna K. Glaeser, Siena C.Napoleon, Janet Wozniak
References J Cyst Fibros. 2017 Aug 31. pii: S1569-1993(17)30874-3. doi: 10.1016/j.jcf.2017.08.011.
Keywords Adherence, Attention-deficit hyperactivity disorder, health-related quality of life
August 2017
Title: Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis
Authors Traci M. Kazmerski, Gregory S. Sawicki, Elizabeth Miller, Kelley A. Jones, Kaleab Z. Abebe, Lisa K. Tuchman, Sigrid Ladores, Ronald C. Rubenstein, Scott D. Sagel, Daniel J. Weine3, Joseph M. Pilewski, David M. Orenstein and Sonya Borrero
References J Cyst Fibros. 2017 Aug 30. pii: S1569-1993(17)30872-X. doi: 10.1016/j.jcf.2017.08.009
Keywords Sexual and reproductive health, Women's health
August 2017
Title: 16S rRNA gene sequencing reveals site-specific signatures of the upper and lower airways of cystic fibrosis patients.
Authors Lucas SK, Yang R, Dunitz JM, Boyer HC, Hunter RC
References J Cyst Fibros. 2017 Aug 18. pii: S1569-1993(17)30865-2. doi: 10.1016/j.jcf.2017.08.007.
Keywords Chronic sinusitis, Microbial ecology, Microbiome, Mucus
August 2017
Title: Variability of Lung Clearance Index in clinically stable Cystic Fibrosis Lung Disease in School Age Children
Authors Marcus Svedberg, Per M. Gustafsson, Paul D. Robinson, Monica Rosberg, Anders Lindblad
References J Cyst Fibros. 2017 Aug 16. pii: S1569-1993(17)30839-1. doi: 10.1016/j.jcf.2017.08.004
Keywords Lung clearance index, Multiple breath washout, Pulmonary exacerbation
July 2017
Title: Poor recovery from a pulmonary exacerbation does not lead to accelerated FEV1 decline
Authors Don B. Sanders, Zhanhai Li,c, Qianqian Zhao, Philip Farrell
References J Cyst Fibros. 2017 Jul 29. pii: S1569-1993(17)30818-4. doi: 10.1016/j.jcf.2017.07.003
Keywords Epidemiology, FEV1, Pulmonary exacerbation
July 2017
Title: Tracheal diverticula in advanced cystic fibrosis: Prevalence, features, and outcomes after lung transplantation.
Authors Siddhartha G. Kapnadak, Gregory A. Kicska, Kathleen A. Ramos, Desiree A. Marshall, Tamara Y. Carroll, Sudhakar N. Pipavath, Michael S. Mulligan, Christopher H. Goss, Moira L. Aitken
References J Cyst Fibros. 2017 Jul 27. pii: S1569-1993(17)30830-5. doi: 10.1016/j.jcf.2017.07.015.
Keywords Lung diseases, Lung transplantation, Pseudomonas infections, Tomography, x-ray computed, Tracheal diverticulum
July 2017
Title: Age-related levels of fecal M2-pyruvate kinase in children with cystic fibrosis and healthy children 0 to 10years old.
Authors Garg M, Leach ST, Pang T, Needham B, Coffey MJ, Katz T, Strachan R, Widger J, Field P, Belessis Y, Chuang S, Day AS, Jaffe A, Ooi CY.
References J Cyst Fibros. 2017 Jul 25. pii: S1569-1993(17)30826-3. doi: 10.1016/j.jcf.2017.07.011.
Keywords Biomarkers, Gastrointestinal inflammation, Intestinal neoplasms, Pediatrics, Pyruvate kinase
July 2017
Title: Increased Prevalence of Colonic Adenomas in Patients with Cystic Fibrosis
Authors Hegagi M, Aaron SD, James P, Goel R, Chatterjee A
References J Cyst Fibros. 2017 Jul 22. pii: S1569-1993(17)30824-X. doi: 10.1016/j.jcf.2017.07.009
Keywords adenoma, colonoscopy
July 2017
Title: Bile salt stimulated lipase, Cystic fibrosis, Enzyme therapy, NMR spectroscopy, Pancreatic insufficiency, Titrimetric enzyme assay
Authors Elena Venuti, Dmitry Shishmarev, Philip W. Kuchel, Shoma Dutt, Caron S. Blumenthal, and Kevin J. Gaskin1
References J Cyst Fibros. 2017 Jul 21. pii: S1569-1993(17)30820-2. doi: 10.1016/j.jcf.2017.07.005
Keywords Bile salt stimulated lipase, Enzyme therapy, NMR spectroscopy, pancreatic insufficiency, Titrimetric enzyme assay
Title: Analytical and Biological Variation in Repeated Sweat Chloride Concentrations in Clinical Trials for CFTR Modulator Therapy
Authors VA LeGrys, TC Moon, J Laux, MJ Rock, F Accurso
References J Cyst Fibros. 2017 Jul 21. pii: S1569-1993(17)30823-8. doi: 10.1016/j.jcf.2017.07.008
Keywords Analytical variation, Biological variation, Index of individuality, Reference change value, sweat chloride
July 2017
Title: Differences between WHO AND CDC early growth measurements in the assessment of Cystic Fibrosis clinical outcomes.
Authors Usatin D, Yen EH, McDonald C, Asfour F, Pohl J, Robson J
References J Cyst Fibros. 2017 Jul;16(4):503-509. doi: 10.1016/j.jcf.2016.12.001
Keywords Forced Expiratory Volume in 1second percent predicted (FEV1(pp)), Growth standards, Survival, Weight-for-length (WFL)
Title: Outcomes associated with antibiotic regimens for treatment of Mycobacterium abscessus in cystic fibrosis patients.
Authors DaCosta A, Jordan CL, Giddings O, Lin FC, Gilligan P, Esther CR Jr
References J Cyst Fibros. 2017 Jul;16(4):483-487. doi: 10.1016/j.jcf.2017.04.013. Epub 2017 May 8
Keywords Mycobacterium abscessus, Nontuberculous mycobacterium
Title: Macronutrient Intake in Preschoolers with Cystic Fibrosis and the Relationship between Macronutrients and Growth
Authors Stephanie S. Filigno, Shannon M. Robson, Rhonda D. Szczesniak, Leigh A. Chamberlin, Meredith A. Baker, Stephanie M. Sullivan, John Kroner, Scott W. Powers
References J Cyst Fibros. 2017 Jul;16(4):519-524. doi: 10.1016/j.jcf.2017.01.010. Epub 2017 Feb 6.
Keywords Behavioral intervention, Children, Nutrition
Title: Worsening anxiety and depression after initiation of lumacaftor/ivacaftor combination therapy in adolescent females with cystic fibrosis
Authors Cameron J. McKinzie, Jennifer L. Goralski, Terry L. Noah, George Z. Retsch-Bogart, Mary Beth Prieur
References J Cyst Fibros. 2017 Jul;16(4):525-527. doi: 10.1016/j.jcf.2017.05.008. Epub 2017 Jun 8.
July 2017
Title: Lower exhaled nitric oxide in infants with Cystic Fibrosis compared to healthy controls
Authors Insa Korten, Margot Liechti, Florian Singer, Gaudenz Hafen, Isabelle Rochat, Pinelopi Anagnostopoulou, Dominik Müller-Suter, Jakob Usemann, Alexander Moeller, Urs Frey, Philipp Latzin, Carmen Casaulta for the SCILD7 and BILD study group
July 2017
Title: Detection of CFTR Function and Modulation in Primary Human Nasal Cell Spheroids
Authors John J. Brewington, Erin T. Filbrandt, F. J. LaRosa III, Alicia J. Ostmann, Lauren M. Strecker, Rhonda D. Szczesniak, John P. Clancy
References J Cyst Fibros. 2017 Jul 13. pii: S1569-1993(17)30812-3. doi: 10.1016/j.jcf.2017.06.010.
Keywords CFTR, Modulator, Organoid, Personalized model system
July 2017
Title: Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2 years of treatment with ivacaftor in a real-world setting
Authors Hubert D, Dehillotte C, Munck A, David V, Baek J, Mely L, Dominique S, Ramel S, Danner Boucher I, Lefeuvre S, Reynaud Q, Colomb-Jung V, Bakouboula P, Lemonnier L
References J Cyst Fibros. 2017 Jul 12. pii: S1569-1993(17)30816-0. doi: 10.1016/j.jcf.2017.07.001
Keywords CFTR modulators, Lumacaftor
June 2017
Title: Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor.
Authors Flume PA, Wainwright CE, Tullis ED, Rodriguez S, Niknian M, Higgins M, Davies JC, Wagener JS
References J Cyst Fibros. 2017 Jun 24. pii: S1569-1993(17)30770-1. doi: 10.1016/j.jcf.2017.06.002
Keywords Cystic fibrosis transmembrane conductance regulator, Ivacaftor, Pulmonary exacerbations, Pulmonary function
June 2017
Title: A single bout of maximal exercise improves lung function in patients with cystic fibrosis.
Authors Matthew A. Tucker, Reva Crandall1, Nicole Seigler, Paula Rodriguez-Miguelez, Kathleen T. McKie, Caralee Forseen, Jeffrey Thomas, & Ryan A. Harris
Keywords Exercise capacity, Impulse oscillometry, Multi-breath washout; Spirometry
Theme Physiotherapy/Exercise testing
June 2017
Title: The use of fructosamine in cystic fibrosis-related diabetes (CFRD) screening.
Authors Lam GY, Doll-Shankaruk M, Dayton J, Rodriguez-Capote K, Higgins TN, Thomas D, Mulchey K, Smith MP, Brown NE, Leung WM, Estey MP
References J Cyst Fibros. 2017 Jun 22. pii: S1569-1993(17)30767-1. doi: 10.1016/j.jcf.2017.05.010
Keywords Cystic fibrosis-related diabetes, FEV(1), Oral glucose tolerance test, screening, Serum fructosamine
June 2017
Title: Pulmonary surfactant dysfunction in pediatric cystic fibrosis: Mechanisms and reversal with a lipid-sequestering drug.
Authors Gunasekara L, Al-Saiedy M, Green F, Pratt R, Bjornson C, Yang A, Michael Schoel W, Mitchell I, Brindle M, Montgomery M, Keys E, Dennis J, Shrestha G, Amrein M.
References J Cyst Fibros. 2017 Jun 7. pii: S1569-1993(17)30151-0.
Keywords Cholesterol, oxidative stress, Free fatty acids, Genotypes, Infection, Inflammation, Lung surfactant, Phospholipids, Surface tension
June 2017
Title: Complications of long and intermediate term venous catheters in cystic fibrosis patients: A multicenter study.
Authors May TL, Gifford AH, Lahiri T, Black A, Trang J, Cornell AG, Gonzalez K, Morin S, Napier M, Duarte CW, Zuckerman JB
References J Cyst Fibros. 2017 Jun 1. pii: S1569-1993(17)30150-9. doi: 10.1016/j.jcf.2017.04.014
Keywords CFF Patient Registry, Central line, Infectious complications, Pulmonary exacerbation
May 2017
Title: Development and Validation of CF-Medication Beliefs Questionnaire: A mixed-methods approach
Authors Michelle N. Eakin, Shang-En Chung, Jessica Hoehn, Belinda Borrelli, Devin Rand-Giovannetti, and Kristin A. Riekert
References J Cyst Fibros. 2017 May 23. pii: S1569-1993(17)30152-2
May 2017
Title: Inhaled ENaC Antisense Oligonucleotide Ameliorates Cystic Fibrosis-Like Lung Disease in Mice
Authors Jeff R. Crosby, Chenguang Zhao, Chong Jiang b Dong Bai, Melanie Katz, Sarah Greenlee, Hiroshi Kawabe , Michael McCaleb, Daniela Rotin, Shuling Guo, Brett P. Monia
References J Cyst Fibros. 2017 May 20. pii: S1569-1993(17)30154-6. doi: 10.1016/j.jcf.2017.05.003.
Keywords Aerosol, Antisense oligonucleotide, ENaC
May 2017
Title: Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis
Authors Aurélie Labaste, Camille Olhmann, Catherine Mainguy, Virginie Jubin, Marie Perceval, Laurianne Coutier, Philippe Reix
References J Cyst Fibros. 2017 May 18. pii: S1569-1993(17)30153-4. doi: 10.1016/j.jcf.2017.05.002.
Keywords Acute, Airway obstruction, Bronchoconstriction, Lumacaftor, Modulators, Pediatric
May 2017
Title: AP2 α modulates cystic fibrosis transmembrane conductance regulator function in the human intestine
Authors Kumari V, Desai S, Ameen NA.
References J Cyst Fibros. 2017 May;16(3):327-334.
Keywords AP2α, CFTR, Clathrin
May 2017
Title: Dietary intake and lipid profile in children and adolescents with cystic fibrosis.
Authors Woestenenk JW, Schulkes DA, Schipper HS, van der Ent CK, Houwen RHJ.
References J Cyst Fibros. 2017 May;16(3):410-417. doi: 10.1016/j.jcf.2017.02.010.
Keywords Cholesterol, Dietary intake, Fat intake, Lipid profile, Triglyceride
Title: Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR
Authors 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-Cousa
References J Cyst Fibros. 2017 May;16(3):371-379. doi: 10.1016/j.jcf.2017.01.009. Epub 2017 Feb 13.
Keywords CFTR stabilizer, GSNOR, F508del-CFTR, Homozygous, Modulator
Title: Effectiveness of a stepwise Pseudomonas aeruginosa eradication protocol in children with cystic fibrosis
Authors Ana C. Blanchard, Eric Horton, Sanja Stanojevic, Louise Taylor, Valerie Waters, Felix Ratjen
References J Cyst Fibros. 2017 May;16(3):395-400. doi: 10.1016/j.jcf.2017.01.007. Epub 2017 Feb 9.
Keywords Antibiotic eradication therapy, Eradication failure, Pseudomonas aeruginosa infection
Title: Use of FEV1 in Cystic Fibrosis Epidemiologic Studies and Clinical Trials: A Statistical Perspective for the Clinical Researcher
Authors Rhonda Szczesniak, Sonya L. Heltshe, Sanja Stanojevic, Nicole Mayer-Hamblett
References J Cyst Fibros. 2017 May;16(3):318-326. doi: 10.1016/j.jcf.2017.01.002. Epub 2017 Jan 20.
Keywords Disease progression, FEV(1) endpoints, Longitudinal, Lung function, Spirometry
Title: Inhaled dry powder mannitol in children with cystic fibrosis: A randomised efficacy and safety trial.
Authors De Boeck K, Haarman E, Hull J, Lands LC, Moeller A, Munck A, Riethmüller J, Tiddens H, Volpi S, Leadbetter J, Charlton B, Malfroot A; DPM-CF-204 Study Group.
References J Cyst Fibros. 2017 May;16(3):380-387.
Keywords FEV1, Mannitol, Paediatric, Randomised
Title: Modelling Cystic Fibrosis Disease Progression in Patients with the Rare CFTR Mutation P67L
Authors Isobel E.R. MacKenzie, Valerie Paquette, Frances Gosse, Sheenagh George, Frederic Chappe and Valerie Chappe
References J Cyst Fibros. 2017 May;16(3):335-341.
Keywords Data Registry, Disease progression, Mild CF, P67L, Rare mutations
May 2017
Title: What Keeps Children with Cystic Fibrosis awake at night?
Authors Vandeleur M, Walter LM, Armstrong DS, Robinson P, Nixon GM, Horne RSC.
References J Cyst Fibros. 2017 May 4. pii: S1569-1993(17)30142-X.
Keywords Actigraphy, Oximetry, Sleep, Sleep hygiene
May 2017
Title: Is there an association between back pain and stress incontinence in adults with cystic fibrosis? A retrospective cross-sectional study.
Authors Ashbrook JE, Shacklady C, Johnson S, Yeowell G, Goodwin PC.
References J Cyst Fibros. 2017 May 2. pii: S1569-1993(17)30102-9
Keywords Back pain, Cross sectional study, Lung function, Stress urinary incontinence
April 2017
Title: A Multiple Reader Scoring System for Nasal Potential Difference Parameters
Authors George M. Solomon, Bo Liu, Isabelle Sermet-Gaudelus, Isabelle Fajac, Michael Wilschanski, Francois Vermeulen, Steven M. Rowe
References J Cyst Fibros. 2017 Apr 29. pii: S1569-1993(17)30141-8
Keywords CFTR, Clinical trial outcomes, Nasal potential difference
April 2017
Title: Standardized Treatment of Pulmonary Exacerbations (STOP) study: Observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations.
Authors Sanders DB, Solomon GM, Beckett VV, West NE, Daines CL, Heltshe SL, VanDevanter DR, Spahr JE, Gibson RL, Nick JA, Marshall BC, Flume PA, Goss CH; STOP Study Group.
References J Cyst Fibros. 2017 Apr 28. pii: S1569-1993(17)30101-7.
Keywords FEV(1), Pseudomonas aeruginosa, Symptoms
April 2017
Title: Standardized Treatment of Pulmonary exacerbations (STOP) study: Physician treatment practices and outcomes for individuals with cystic fibrosis with pulmonary exacerbations.
Authors West NE, Beckett VV, Jain R, Sanders DB, Nick JA, Heltshe SL, Dasenbrook EC, VanDevanter DR, Solomon GM, Goss CH, Flume PA; STOP investigators.
References J Cyst Fibros. 2017 Apr 27. pii: S1569-1993(17)30099-1.
Keywords antibiotic therapy, Physician treatment practices, Pulmonary exacerbations
April 2017
Title: Increased Risk of PTLD in Lung Transplant Recipients with Cystic Fibrosis
Authors Erin M. Lowery, William Adams, Shellee A. Grim, Nina M. Clark, Leah Edwards, Jennifer E. Layden
References J Cyst Fibros. 2017 Apr 26. pii: S1569-1993(17)30096-6. doi: 10.1016/j.jcf.2017.03.013.
Keywords Ebstein–Barr virus, Post transplant lymphoproliferative disorder
April 2017
Title: Does current reporting of lung function by the UK cystic fibrosis registry allow a fair comparison of adult centres?
Authors Nightingale JA, Osmond C
References J Cyst Fibros. 2017 Apr 24. pii: S1569-1993(17)30103-0.
Keywords Clinical epidemiology, Respiratory measurement
April 2017
Title: Risk Factors for Persistent Methicillin-resistant Staphylococcus aureus Infection in Cystic Fibrosis
Authors Jennings MT, Dasenbrook EC, Lechtzin N, Boyle MP, Merlo CA
References J Cyst Fibros. 2017 Apr 23. pii: S1569-1993(17)30106-6.
Keywords Epidemiology, MRSA
April 2017
Title: Feasibility and normal values of an integrated conductivity (Nanoduct) sweat test system in healthy newborns
Authors Kuehni CE, Schindler M, Mazur A, Malzacher A, Hornung R, Barben J.
References J Cyst Fibros. 2017 Apr 22. pii: S1569-1993(17)30098-X.
Keywords Conductivity, Newborn screening, Sweat test
April 2017
Title: Rationalizing endpoints for prospective studies of pulmonary exacerbation treatment response in cystic fibrosis
Authors VanDevanter DR, Heltshe SL, Spahr J, Beckett VV, Daines CL, Dasenbrook EC, Gibson RL, Jain R, Sanders DB, Goss CH, Flume PA; STOP Study Group.
References J Cyst Fibros. 2017 Apr 21. pii: S1569-1993(17)30100-5
Keywords Clinical trial, endpoints, Exacerbation, Sample size
April 2017
Title: CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis
Authors Abdullah Al Tarique, Peter D Sly, Patrick G Holt, Anthony Bosco, Robert S Ware, Jayden Logan, Scott C Bell, Claire E Wainwright, Emmanuelle Fantino.
References J Cyst Fibros. 2017 Apr 17. pii: S1569-1993(17)30094-2.
Keywords Alternatively activated macrophages, CFTR, CFTR inhibitors, Classically activated macrophages, Endocytosis, IL-13 receptor, Monocyte-derived macrophages, Phagocytosis
April 2017
Title: Age-dependent variation of fecal calprotectin in cystic fibrosis and healthy children.
Authors Garg M, Leach ST, Coffey MJ, Katz T, Strachan R, Pang T, Needham B, Lui K, Ali F, Day AS, Appleton L, Moeeni V, Jaffe A, Ooi CY
References J Cyst Fibros. 2017 Apr 14. pii: S1569-1993(17)30093-0
Keywords Calprotectin, Gastrointestinal inflammation, Leukocyte L1 antigen complex, Pediatrics, S100 proteins
April 2017
Title: A treatment evaluator tool to monitor the real-world effectiveness of inhaled aztreonam lysine in cystic fibrosis.
Authors Plant BJ, Downey DG, Eustace JA, Gunaratnam C, Haworth CS, Jones AM, McKone EF, Peckham DG, Ketchell RI, Bilton D.
References J Cyst Fibros. 2017 Apr 6. pii: S1569-1993(17)30031-0.
Keywords Aztreonam, Evaluation tool, forced expiratory volume, intravenous antibiotics
April 2017
Title: Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth
Authors Paemka L, McCullagh BN, Abou Alaiwa MH, Stoltz DA, Dong Q, Randak CO, Gray RD, McCray PB Jr.
References J Cyst Fibros. 2017 Apr 1. pii: S1569-1993(17)30090-5
Keywords CFTR, Inflammation, Lipopolysaccharide, Macrophage, Monocyte
March 2017
Title: A 3-year prognostic score for adults with cystic fibrosis.
Authors Nkam L, Lambert J, Latouche A, Bellis G, Burgel PR, Hocine MN
References J Cyst Fibros. 2017 Mar 18. pii: S1569-1993(17)30087-5
Keywords Logistic model, Prognostic factors, Prognostic score, Registry data
March 2017
Title: Nutritional status, nutrient intake and use of enzyme supplements in paediatric patients with Cystic Fibrosis; a European multicentre study with reference to current guidelines
Authors Calvo-Lerma J, Hulst JM, Asseiceira I, Claes I, Garriga M, Colombo C, Fornés V, Woodcock S, Martins T, Boon M, Ruperto M, Walet S, Speziali C, Witters P, Masip E, Barreto C, de Boeck K, Ribes-Koninckx C; MyCyFAPP Project.
References J Cyst Fibros. 2017 Mar 17. pii: S1569-1993(17)30088-7
Theme Nutrition/Gastroenterology
March 2017
Title: When is too little care, too much harm in cystic fibrosis? Psychological and ethical approaches to the problem.
Authors Massie J, Morgan A, Gillam L
References J Cyst Fibros. 2017 Mar;16(2):299-303
Title: Clostridium difficile carriage in adult cystic fibrosis (CF); implications for patients with CF and the potential for transmission of nosocomial infection.
Authors DG Burke, MJ Harrison, C Fleming, M McCarthy, C Shortt, I Sulaiman, DM Murphy, JA Eustace, F Shanahan, C Hill, C Stanton, MC Rea, RP Ross, BJ Plant
References J Cyst Fibros. 2017 Mar;16(2):291-298.
Keywords Clostridium difficile, Nosocomial infection, Transmission
March 2017
Title: Real-life initiation of lumacaftor/ivacaftor combination in adults with cystic fibrosis homozygous for the Phe508del CFTR mutation and severe lung disease
Authors Hubert D, Chiron R, Camara B, Grenet D, Prévotat A, Bassinet L, Dominique S, Rault G, Macey J, Honoré I, Kanaan R, Leroy S, Desmazes Dufeu N, Burgel PR.
References J Cyst Fibros. 2017 Mar 15. pii: S1569-1993(17)30086-3
Keywords CFTR correctors, Lumacaftor/ivacaftor
March 2017
Title: Technological advances shed light on left ventricular cardiac disturbances in cystic fibrosis
Authors Sayyid ZN, Sellers ZM
References J Cyst Fibros. 2017 Mar 14. pii: S1569-1993(17)30080-2
Keywords Echocardiography, Heart function, Left ventricle
Title: The CF-CARES primary palliative care model: A CF-specific structured assessment of symptoms, distress, and coping. distress, and coping.
Authors Friedman D, Linnemann RW, Altstein LL, Islam S, Bach KT, Lamb C, Volpe J, Doolittle C, St John A, O'Malley PJ, Sawicki GS, Georgiopoulos AM, Yonker LM, Moskowitz SM.
References J Cyst Fibros. 2017 Mar 14. pii: S1569-1993(17)30077-2.
Keywords anxiety, Chronic symptom burden, depression, Palliative care, Symptom prevalence
Title: Immediate Effects of Lumacaftor/Ivacaftor Administration on Lung Function in Patients with Severe Cystic Fibrosis Lung Disease
Authors Natalia Popowicz, Jamie Wood, Anna Tai, Sue Morey, Siobhain Mulrennan
References J Cyst Fibros. 2017 Mar 14. pii: S1569-1993(17)30075-9
Keywords Ivacaftor, Lumacaftor, Lung function, adverse events
March 2017
Title: Feasibility of placebo-controlled trial designs for new CFTR modulator evaluation
Authors Donald R. VanDevanter, Nicole Mayer-Hamblett, Michael Boyle
References J Cyst Fibros. 2017 Mar 8. pii: S1569-1993(17)30078-4
Keywords Enrollment, Modulator, Placebo, Trial design
Title: Association between glucose intolerance and bacterial colonisation in an adult population with cystic fibrosis, emergence of Stenotrophomonas maltophilia.
Authors Lehoux Dubois, C., Boudreau, V., Tremblay, F., Lavoie A., Berthiaume Y., Rabasa-Lhoret, R., Coriati, A.
References J Cyst Fibros. 2017 Mar 8. pii: S1569-1993(17)30025-5.
Keywords Cystic fibrosis related diabetes, Infections, Pathogens, Pulmonary exacerbations, Stenotrophomonas maltophilia
March 2017
Title: Pilot trial of tobramycin inhalation powder in cystic fibrosis patients with chronic Burkholderia cepacia complex infection
Authors Valerie Waters, Yvonne Yau, Trevor Beaudoin, Jillian Wettlaufer, Sean Kevin Tom, Nancy McDonald, Leena Rizvi, Michelle Klingel, Felix Ratjen, Elizabeth Tullis
References J Cyst Fibros. 2017 Mar 2. pii: S1569-1993(17)30074-7.
Keywords Burkholderia cepacia, Inhaled tobramycin
Title: Peripheral muscle abnormalities in cystic fibrosis: etiology, clinical implications and response to therapeutic interventions
Authors Mathieu Gruet, Thierry Troosters, Samuel Verges
References J Cyst Fibros. 2017 Mar 2. pii: S1569-1993(17)30032-2.
Keywords Aerobic function, CFTR, Exercise interventions, Muscle function, Muscle metabolism, nutritional status
February 2017
Title: The Cumulative Effects of Intravenous Antibiotic Treatments on Hearing in Patients with Cystic Fibrosis
Authors Angela C. Garinis, Campbell P. Cross1, 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
References J Cyst Fibros. 2017 Feb 23. pii: S1569-1993(17)30013-9.
February 2017
Title: Mycobacterium abscessus, Mycobacterium avium, Non-tuberculous mycobacteria, Pulmonary disease
Authors Zoé Cavalli1, Quitterie Reynaud1, Romain Bricca, Raphaële Nove-Josserand, Stéphane Durupt, Philippe Reix, Marie Perceval, Michèle Pérouse de Montclos, Gérard Lina, Isabelle Durieu
References J Cyst Fibros. 2017 Feb 12. pii: S1569-1993(17)30024-3
Keywords Mycobacterium abscessus, Mycobacterium avium, Non-tuberculous mycobacteria, Pulmonary disease
February 2017
Title: Pregnancy among cystic fibrosis women in the area of CFTR modulators
Authors Sonya L. Heltshe, Emily M. Godfrey, Tatiana Josephy, Moira L. Aitken, Jennifer L.Taylor-Cousar
References J Cyst Fibros. 2017 Feb 9. pii: S1569-1993(17)30015-2
Keywords G551D, Ivacaftor, Lumacaftor, pregnancy
February 2017
Title: Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosis.
Authors Ramsey KA, McGirr C, Stick SM, Hall GL, Simpson SJ; AREST CF.
References J Cyst Fibros. 2017 Feb 7. pii: S1569-1993(17)30020-6.
Keywords Child, Computed tomography, Cystic fibrosis, Lung clearance index, Multiple breath washout, Posture, Ventilation distribution
Title: The 1-min sit-to-stand test in cystic fibrosis - Insights into cardiorespiratory responses.
Authors Radtke T, Hebestreit H, Puhan MA, Kriemler S
References J Cyst Fibros. 2017 Feb 7. pii: S1569-1993(17)30019-X
Keywords Exercise testing, Functional capacity, Lung disease, Validity
February 2017
Title: Fatal disseminated Rasamsonia infection in cystic fibrosis post-lung transplantation
Authors Gina Hong*, Marissa White*, Noah Lechtzin, Natalie E. West, Robin Avery, Heather Miller, Richard Lee, Robert J. Lovari, Christian Massire, Lawrence B. Blyn, Xinglun Liang, Deanna A. Sutton, Jianmin Fu, Brian L. Wickes, Nathan P. Wiederhold, Sean X. Zhang,
References J Cyst Fibros. 2017 Feb 6. pii: S1569-1993(17)30012-7.
Keywords Fungal infection, Lung transplantation, Rasamsonia
January 2017
Title: Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis.
Authors Altes TA, Johnson M, Fidler M, Botfield M, Tustison NJ, Leiva-Salinas C, de Lange EE, Froh D, Mugler JP 3rd
References J Cyst Fibros. 2017 Jan 26. pii: S1569-1993(16)30673-7.
Keywords cystic fibrosis transmembrane conductance regulator modulator, forced expiratory volume, Pulmonary
January 2017
Title: Effect of freezing sputum on Pseudomonas aeruginosa population heterogeneity
Authors Poonja A, Heirali A, Workentine M, Storey DG, Somayaji R, Rabin HR, Surette MG, Parkins MD
References J Cyst Fibros. 2017 Jan 23. pii: S1569-1993(17)30011-5.
