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: 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: 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
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: 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: 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.
January 2017
Title: Correlation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor
Authors Fidler MC, Beusmans J, Panorchan P, Van Goor F.
References J Cyst Fibros. 2017 Jan;16(1):41-44.
Keywords FEV(1), Ivacaftor, sweat chloride
Title: The fate of inhaled antibiotics after deposition in cystic fibrosis: how to get drug to the bug?
Authors Aukje C. Bos, Kimberly M. Passé, Johan W. Mouton, Hettie M. Janssens, Harm A.W.M. Tiddens
References J Cyst Fibros. 2017 Jan;16(1)
Keywords Inhaled antibiotics, Sputum binding, Alginate, Pseudomonas aeruginosa
Title: The fate of inhaled antibiotics after deposition in cystic fibrosis: how to get drug to the bug?
Authors Aukje C. Bos, Kimberly M. Passé, Johan W. Mouton, Hettie M. Janssens, Harm A.W.M. Tiddens
References J Cyst Fibros. 2017 Jan;16(1):13-23.
Keywords Inhaled antibiotics, Sputum binding, Alginate, Pseudomonas aeruginosa
Title: Adherence to infection prevention and control guidelines: A vignette-based study of decision-making and risk-taking in young adults with cystic fibrosis.
Authors Bowmer G, Latchford G, Duff A, Denton M, Dye L, Lawton C, Lee T
Keywords Adherence, Cross-infection, Cystic fibrosis, Decision-making, Infection prevention and control
January 2017
Title: Changes of CFTR functional measurements and clinical improvements in Cystic Fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor
Authors Myriam Mesbahi* and Michal Shteinberg*; Michael Wilschanski, Aurelie Hatton, Thao Nguyen–Khoa, Hannah Friedman, Michael Cohen, Virginie Escabasse, Muriel Le Bourgeois, VicenzinaLucidi, Isabelle Sermet-Gaudelus, Laurence Bassinet** and Galit Livnat**, *,**
References J Cyst Fibros. 2017 Jan;16(1):45-48
Keywords CFTR modulators, Evaporimetry; Ivacaftor, Nasal potential difference, Short circuit current
December 2016
Title: Non-invasive prenatal diagnosis (NIPD) of Cystic Fibrosis: an optimized protocol using MEMO fluorescent PCR to detect the p.Phe508del mutation
Authors C. Guissart, C. Dubucs, C. Raynal, A. Girardet, F. Tran Mau Them, V. Debant, C. Rouzier, A. Boureau-Wirth, E. Haquet, J. Puechberty, E. Bieth, D. Dupin Deguine, P. Khau Van Kien, M.P. Brechard, V. Pritchard, M. Koenig, M.Claustres, M.C. Vincent
References J Cyst Fibros. 2016 Dec 28. pii: S1569-1993(16)30680-4
Keywords https://www.ncbi.nlm.nih.gov/pubmed/?term=Non-invasive+prenatal+diagnosis+(NIPD)+of+Cystic+Fibrosis%3A+an+optimized+protocol+using+MEMO+fluorescent+PCR+to+detect+the+p.Phe508del+mutation
December 2016
Title: Impact of azithromycin on the clinical and antimicrobial effectiveness of tobramycin in the treatment of cystic fibrosis
Authors Nichols DP, Happoldt CL, Bratcher PE, Caceres SM, Chmiel JF, Malcolm KC, Saavedra MT, Saiman L, Taylor-Cousar JL, Nick JA.
References J Cyst Fibros. 2016 Dec 24. pii: S1569-1993(16)30672-5.
Keywords Azithromycin, Clinical trial, Drug interaction, Inhaled antibiotics, MexXY, Pseudomonas aeruginosa, Tobramycin
December 2016
Title: Increasing sputum levels of gamma-glutamyltransferase may identify cystic fibrosis patients who do not benefit from inhaled glutathione.
Authors Corti A, Griese M, Hector A, Pompella A
References J Cyst Fibros. 2016 Dec 14. pii: S1569-1993(16)30671-3.
Keywords Gamma-glutamyltransferase, Glutathione, Inflammation
December 2016
Title: The psychometric properties of the Leicester Cough Questionnaire and Respiratory Symptoms in CF tool in cystic fibrosis.
Authors Nathan Ward, Kathy Stiller, Hilary Rowe, Anne E Holland
References J Cyst Fibros. 2016 Dec 13. pii: S1569-1993(16)30669-5
Keywords Cough, Leicester Cough Questionnaire, Signs and symptoms, respiratory, Surveys and questionnaires
December 2016
Title: Pleural Effusions in Non-Transplanted Cystic Fibrosis Patients
Authors Adam R. Belanger, Kimtuyen Nguyen, Umar Osman, Christopher R. Gilbert, Katie Allen, Ahmad Farid Al Rais, Lonny Yarmus, Jason A. Akulian
References J Cyst Fibros. 2016 Dec 12. pii: S1569-1993(16)30664-6.
Keywords Pleural effusion, Patient outcome assessment
Title: Pleural Effusions in Non-Transplanted Cystic Fibrosis Patients
Authors Adam R. Belanger, Kimtuyen Nguyen, Umar Osman, Christopher R. Gilbert, Katie Allen, Ahmad Farid Al Rais, Lonny Yarmus, Jason A. Akulian
Keywords Alginate, Inhaled antibiotics, Pseudomonas aeruginosa, Sputum binding
December 2016
Title: Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis
Authors Waters V, Ratjen F
References Waters V, Ratjen F. Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2016, Issue 12. Art. No.: CD010004. DOI: 10.1002/14651858.CD010004.pub4
Keywords antibiotic treatment, antibiotic, nontuberculous mycobacteria, lung infection
Theme Lung Disease and Infection
Title: BMI Fails to Identify Poor Nutritional Status in Stunted Children with CF
Authors Michael W. Konstan, David J. Pasta, Jeffrey S. Wagener, Donald R. VanDevanter, Wayne J. Morgan
References J Cyst Fibros. 2016 Dec 1. pii: S1569-1993(16)30663-4
Keywords Body mass index, Growth, Height for age, Weight for age
Theme Nutrition/Gastroenterology
Title: Environmental Scan of Cystic Fibrosis Research Worldwide
References J Cyst Fibros. 2016 Dec 1. pii: S1569-1993(16)30655-5
Keywords Charitable organizations, Environmental scan, Rare disease, Research funding
November 2016
Title: Oxidized glutathione and uric acid as biomarkers of early cystic fibrosis lung disease
Authors Nina Dickerhof, Rufus Turner, Irada Khalilova, Emmanuelle Fantino, Peter D Sly, Anthony J Kettle
References J Cyst Fibros. 2016 Nov 24. pii: S1569-1993(16)30647-6
Keywords Allantoin, Glutathione sulfonamide, Myeloperoxidase, Neutrophil, Neutrophil elastase, Pseudomonas aeruginosa
November 2016
Title: Continuous alternating inhaled antibiotic therapy in CF: a single center retrospective analysis.
Authors Van de Kerkhove C, Goeminne PC, Kicinski M, Nawrot TS, Lorent N, Van Bleyenbergh P, De Boeck K, Dupont LJ
References J Cyst Fibros. 2016 Nov;15(6):802-808.
Keywords Continuous alternating inhaled antibiotic therapy, FEV(1), Inhaled antibiotics, Pseudomonas aeruginosa
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 J Cyst Fibros. 2016 Nov;15(6):714-723
Keywords Biomarker, Inflammation, Microbiome, Infection, Advanced technologies, Electrophysiology
Title: Continuous alternating inhaled antibiotic therapy in CF: a single center retrospective analysis.
Authors Van de Kerkhove C, Goeminne PC, Kicinski M, Nawrot TS, Lorent N, Van Bleyenbergh P, De Boeck K, Dupont LJ
References J Cyst Fibros. 2016 Nov;15(6):802-808.
Keywords Continuous alternating inhaled antibiotic therapy, FEV(1), Inhaled antibiotics, Pseudomonas aeruginosa
November 2016
Title: Bacterial proteases and haemostasis dysregulation in the CF lung.
Authors Reihill JA, Moreland M, Jarvis GE, McDowell A, Einarsson GG, Elborn JS, Martin SL
References J Cyst Fibros. 2016 Nov 10.
Keywords Burkholderia cenocepacia, Burkholderia multivorans, Coagulation, Haemoptysis, Haemostasis, Platelet aggregation, Pseudomonas aeruginosa
Title: Bacterial proteases and haemostasis dysregulation in the CF lung.
Authors Reihill JA, Moreland M, Jarvis GE, McDowell A, Einarsson GG, Elborn JS, Martin SL
References J Cyst Fibros. 2016 Nov 10.
Keywords Burkholderia cenocepacia, Burkholderia multivorans, Coagulation, Haemoptysis, Haemostasis, Platelet aggregation, Pseudomonas aeruginosa
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: Eradication therapy for Burkholderia cepacia complex in people with cystic fibrosis
References Regan KH, Bhatt J. Eradication therapy for Burkholderia cepacia complex in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2016, Issue 11. Art. No.: CD009876. DOI: 10.1002/14651858.CD009876.pub3
Keywords eradication therapy, therapy, Burkholderia cepacia, Burkholderia cepacia complex
Theme Lung Disease and Infection
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, Andrew W
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 MD, PhDg, Elaine Harrington RN, BSN, AE-Ch, Peter J. Murphy, MDi, Lena Palmer MD, MSCRj, Amy E. Schrader RDN, LDk, Kyle S
References J Cyst Fibros. 2016 Sep 3. pii: S1569-1993(16)30594-X
Keywords Enteral nutrition, Feeding tube, Gastrostomy, Nasojejunal tube
Theme Nutrition/Gastroenterology
September 2016
Title: Duration of intravenous antibiotic therapy in people with cystic fibrosis
Authors Plummer A, Wildman M, Gleeson T
References Plummer A, Wildman M, Gleeson T. Duration of intravenous antibiotic therapy in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2016, Issue 9. Art. No.: CD006682. DOI: 10.1002/14651858.CD006682.pub5
Keywords duration, intravenous antibiotic therapy, antibiotic, intravenous, therapy
Theme Lung Disease and Infection
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
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, Mari
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
