November 2019
Title: Can Exercise Replace Airway Clearance Techniques in Cystic Fibrosis? A Survey of Patients and Healthcare Professionals
Authors N J Rowbotham, S J Smith, G Davies, T Daniels, Z C Elliott, K Gathercole, O C Rayner, A R Smyth
References J Cyst Fibros 2019 Nov 15 - DOI: 10.1016/j.jcf.2019.10.026
Keywords Airway clearance techniques, Co-production, Exercise, Priority setting
November 2019
Title: Characterising burden of treatment in cystic fibrosis to identify priority areas for clinical trials
Authors Gwyneth Davies, Nicola J Rowbotham, Sherie Smith, Zoe C Elliot, Katie Gathercole, Oli Rayner, Paul A Leighton, Sophie Herbert, Alistair JA Duff, Suja Chandran, Tracey Daniels, Edward F Nash, Alan R Smyth
References J Cyst Fibros. 2019 Nov 14. pii: S1569-1993(19)30961-0. doi: 10.1016/j.jcf.2019.10.025
Keywords Clinical trial, Co-production, Priority setting, Treatment burden
November 2019
Title: Prevalence and factors associated with overweight and obesity in adults with cystic fibrosis: A single-center analysis
Authors Harindhanavudhi T, Wang Q, Dunitz J, Moran A, Moheet A
References J Cyst Fibros. 2019 Nov 11. pii: S1569-1993(19)30922-1. doi: 10.1016/j.jcf.2019.10.004.
Keywords obesity, Prevalence
November 2019
Title: Regional differences in infection and structural lung disease in infants and young children with cystic fibrosis
Authors Regional differences in infection and structural lung disease in infants and young children with cystic fibrosis. Carzino R, Frayman KB, King L, Vidmar S, Ranganathan S; AREST CF.
References J Cyst Fibros. 2019 Nov 6. pii: S1569-1993(19)30933-6. doi: 10.1016/j.jcf.2019.10.018.
Keywords Bronchiectasis, Bronchoalveolar lavage, Chest computed tomography, Pro-inflammatory microbes, Right middle lobe and lingula
November 2019
Title: Insights into the variability of Nasal Potential Difference, a biomarker of CFTR activity
Authors Spyridoula Kyrilli, Theophraste Henry, Michael Wilschanski, Isabelle Fajac, Jane C Davies, Jean-Philippe Jais, Isabelle Sermet-Gaudelus
References J Cyst Fibros. 2019 Nov 4. pii: S1569-1993(19)30899-9. doi: 10.1016/j.jcf.2019.09.015.
Keywords Biomarker, Nasal potential difference
November 2019
Title: The implications of dysglycaemia on aerobic exercise and ventilatory function in cystic fibrosis
Authors Adam J. Causer, Janis K. Shute, Michael H. Cummings, Anthony I. Shepherd, Samuel R. Wallbanks, Mark I. Allenby, Irantzu Arregui-Fresneda, Victoria Bright, Mary P. Carroll, Gary Connett, Thomas Daniels, Tom Meredith, and Zoe L. Saynor
References J Cyst Fibros . 2019 Nov 2;S1569-1993(19)30895-1. doi: 10.1016/j.jcf.2019.09.014.
Keywords Cardiorespiratory fitness, Cystic fibrosis-related diabetes, Maximal oxygen uptake, Respiratory disease
October 2019
Title: Infection Prevention and Control in Patients With Cystic Fibrosis (CF): Results From a Survey in 35 German CF Treatment Centers
Authors Meyer S, Nüßlein T, Nährlich L, Bend J, Gärtner B, Becker SL, Simon A
References J Cyst Fibros 2019 Oct 31 pii: S1569-1993(19)30925-7. DOI: 10.1016/j.jcf.2019.10.012
Keywords Commission for hygiene and infection control, Infection prevention and control, Pseudomonas aeruginosa, Segregation, Survey
Title: Cystic fibrosis related diabetes in Europe: Prevalence, risk factors and outcome
Authors Olesen, HV; Drevinek P; Gulmans VAM; Hatziagorou E; Jung A; Mei-Zahav M; Stojnic N; Thomas M; Zolin A
References J Cyst Fibros. 2019 Oct 31. pii: S1569-1993(19)30916-6. doi: 10.1016/j.jcf.2019.10.009
Keywords Body mass index, Diabetes, Gender, Lung function
October 2019
Title: Minimal detectable change and minimal clinically important differences in modified shuttle walk test in children and adolescents with cystic fibrosis
Authors Tamara del Corral; Álvaro Gómez Sánchez; Ibai López de Uralde Villanueva
References J Cyst Fibros. 2019 Oct 30. pii: S1569-1993(19)30915-4. doi: 10.1016/j.jcf.2019.10.007.
Keywords Children and adolescents, Physical activity, Pulmonary rehabilitation, Shuttle walk test, Submaximal exercise testing
Title: Cystic Fibrosis in Black African Children
Authors Owusu SK, Morrow BM, White D, Klugman S, Vanker A, Gray D, Zampoli M
References J Cyst Fibros. 2019 Oct 30. pii: S1569-1993(19)30891-4. doi: 10.1016/j.jcf.2019.09.007.
Keywords Black African children, South Africa
Title: Occurrence, outcomes and predictors of portal hypertension in cystic fibrosis: a longitudinal prospective birth cohort study.
Authors Cipolli M, Fethney J, Waters D, Zanolla L, Menghelli I, Dutt S, Assael BM, Gaskin KJ
References J Cyst Fibros. 2019 Oct 30. pii: S1569-1993(19)30900-2. doi: 10.1016/j.jcf.2019.09.016
Keywords Incidence/Prevalence, Liver enzymes, portal hypertension
October 2019
Title: Intraoperative extracorporeal membrane oxygenation for lung transplantation in cystic fibrosis patients: predictors and impact on outcome
Authors Scaravilli V, Morlacchi LC, Merrino A, Piacentino E, Marasco D, Zanella A, Nosotti M, Rosso L, Polli F, Blasi F, Pesenti A, Grasselli G
References J Cyst Fibros. 2019 Oct 29. pii: S1569-1993(19)30930-0. doi: 10.1016/j.jcf.2019.10.016
Keywords Extracorporeal membrane oxygenation, Lung transplantation, Retrospective studies, Risk factors
October 2019
Title: Risk factors for progression of structural lung disease in school-age children with cystic fibrosis
Authors Marcus Svedberg, Per Gustafsson, Harm Tiddens, Henrik Imberg, Aldina Pivodic, Anders Lindblad
References J Cyst Fibros 2019 Oct 28 - DOI: 10.1016/j.jcf.2019.10.014
Keywords Chest CT, FEV(1), Pseudomonas aeruginosa, Structural lung disease
Title: Differences in clinical outcomes of paediatric cystic fibrosis patients with and without meconium ileus.
Authors Su Min Joyce Tan, Michael J. Coffey, Chee Y. Ooi
References J Cyst Fibros. 2019 Oct 28. pii: S1569-1993(19)30892-6. doi: 10.1016/j.jcf.2019.09.008.
Keywords clinical outcomes, Growth, Lung function, meconium ileus, Nutrition
October 2019
Title: Infection with Prevotella nigrescens induces Toll-like Receptor 2 signalling and low levels of p65 mediated inflammation in Cystic Fibrosis bronchial epithelial cells
Authors Bertelsen A, Elborn JS, Schock BC
References J Cyst Fibros. 2019 Oct 10. pii: S1569-1993(19)30889-6. doi: 10.1016/j.jcf.2019.09.005.
Keywords Anaerobic bacteria, Cystic Fibrosis Airways inflammation, Host-pathogen-interaction, Prevotella nigrescens, Signalling
October 2019
Title: GLPG2737 in lumacaftor/ivacaftor-treated CF subjects homozygous for the F508del mutation: A randomized phase 2A trial (PELICAN).
Authors van Koningsbruggen-Rietschel S, Conrath K, Fischer R, Sutharsan S, Kempa A, Gleiber W, Schwarz C, Hector A, Van Osselaer N, Pano A, Corveleyn S, Bwirire D, Santermans E, Muller K, Bellaire S, Van de Steen O
References J Cyst Fibros. 2019 Oct 5. pii: S1569-1993(19)30890-2. doi: 10.1016/j.jcf.2019.09.006.
Keywords CFTR corrector, Combination therapy, Efficacy, GLPG2737, Ivacaftor, Lumacaftor
September 2019
Title: Decreased mRNA and protein stability of W1282X limits response to modulator therapy
Authors Aksit MA, Bowling AD, Evans TA, Joynt AT, Osorio D, Patel S, West N, Merlo C, Sosnay PR, Cutting GR, Sharma N
References J Cyst Fibros. 2019 Sep;18(5):606-613. doi: 10.1016/j.jcf.2019.02.009. Epub 2019 Feb 23.
Title: CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials
Authors Bell SC, Barry PJ, De Boeck K, Drevinek P, Elborn JS, Plant BJ, Minić P, Van Braeckel E, Verhulst S, Muller K, Kanters D, Bellaire S, de Kock H, Geller DE, Conrath K, Van de Steen O, van der Ent K
References J Cyst Fibros. 2019 Sep;18(5):700-707. doi: 10.1016/j.jcf.2019.04.014.
Keywords CFTR modulator, F508del, GLPG2222, Gating mutation, Ivacaftor
Title: A two-center analysis of hyperpolarized 129Xe lung MRI in stable pediatric cystic fibrosis: Potential as a biomarker for multi-site trials.
Authors Couch MJ, Thomen R, Kanhere N, Hu R, Ratjen F, Woods J, Santyr G
References J Cyst Fibros. 2019 Sep;18(5):728-733. doi: 10.1016/j.jcf.2019.03.005. Epub 2019 Mar 25.
Keywords Hyperpolarized (129)Xe, Magnetic Resonance Imaging, Ventilation defect percent
Title: International approaches for delivery of positive newborn bloodspot screening results for CF
Authors Chudleigh J, Ren CL, Barben J, Southern KW.
References J Cyst Fibros. 2019 Sep;18(5):614-621. doi: 10.1016/j.jcf.2019.04.004. Epub 2019 Apr 30.
Keywords Communication, Newborn bloodspot screening
September 2019
Title: Micronutrient intake in children with cystic fibrosis in Sydney, Australia
Authors Adrienne Tham, Tamarah E Katz, Rosie E. Sutherland, Millie Garg, Victoria Liu, Chai Wei Tong, Rebecca Brunner, Justine Quintano, Clare Collins, Chee Y Ooi
References J Cyst Fibros. 2019 Sep 14. pii: S1569-1993(19)30885-9. doi: 10.1016/j.jcf.2019.08.028.
Keywords Diet quality, Dietary intake, Micronutrient, Nutrition
September 2019
Title: A critical review of definitions used to describe Pseudomonas aeruginosa microbiological status in patients with Cystic Fibrosis for application in clinical trials
Authors Taccetti G; Denton M; Hayes K, ECFS-CTN Microbiology Group; Drevinek P and Sermet-Gaudelus I.
References J Cyst Fibros. 2019 Sep 13. pii: S1569-1993(19)30867-7. doi: 10.1016/j.jcf.2019.08.014.
Keywords Microbiological status, Pseudomonas aeruginosa, Serology, anti pa-antibodies
September 2019
Title: A smartphone application for reporting symptoms in adults with cystic fibrosis improves the detection of exacerbations: results of a randomised controlled trial
Authors Wood J, Jenkins S, Putrino D, Mulrennan S, Morey S, Cecins N, Bear N, Hill K.
References J Cyst Fibros. 2019 Sep 12. pii: S1569-1993(19)30887-2. doi: 10.1016/j.jcf.2019.09.002.
Keywords Exacerbation, Smartphone, Telehealth, Telemedicine
September 2019
Title: The impact of chest computed tomography and chest radiography on clinical management of cystic fibrosis lung disease
Authors Carla F. Bortoluzzi, Eleonora Pontello, Emily Pintani, Karin M. de Winter-de Groot, Ciro D’Orazio, Baroukh M. Assael, M.G. Myriam Hunink, Harm A.W.M. Tiddens, Daan Caudri
References J Cyst Fibros. 2019 Sep 4. pii: S1569-1993(19)30837-9. doi: 10.1016/j.jcf.2019.08.005.
Keywords Chest radiography, Clinical management, Computed tomography
September 2019
Title: Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis-data from the European cystic fibrosis society patient registry.
Authors Hatziagorou E, Orenti A, Drevinek P, Kashirskaya N, Mei-Zahav M, De Boeck K.
References J Cyst Fibros. 2019 Sep 3;S1569-1993(19)30838-0. doi: 10.1016/j.jcf.2019.08.006.
Keywords Epidemiology, Pseudomonas aeruginosa, registry, Staphylococcus aureus
August 2019
Title: Whole-blood transcriptomic responses to lumacaftor/ivacaftor therapy in cystic fibrosis.
Authors Kopp BT, Fitch J, Jaramillo L, Shrestha CL, Robledo-Avila F, Zhang S, Palacios S, Woodley F, Hayes D Jr, Partida-Sanchez S, Ramilo O, White P., Meijas A.
References J Cyst Fibros. 2019 Aug 29. pii: S1569-1993(19)30868-9. doi: 10.1016/j.jcf.2019.08.021.
Keywords CFTR, RNA-seq, Transcriptomics
August 2019
Title: Addressing the Burden of Illness in Adults with Cystic Fibrosis with Screening and Triage: An Early Intervention Model of Palliative Care
Authors Dhingra L, Walker P, Berdella M, Plachta A, Chen J, Fresenius A, Balzano J, Barrett M, Bookbinder M, Wilder K, Glajchen M, Langfelder-Schwind E, Portenoy RK
References J Cyst Fibros. 2019 Aug 27. pii: S1569-1993(19)30841-0. doi: 10.1016/j.jcf.2019.08.009
Keywords Generalist-level palliative care, Models of care, screening, Telehealth
August 2019
Title: A Study to Test the Effects of a Treatment Called Amikacin Liposome Inhalation Suspension on Chronic Pseudomonas aeruginosa Infection in People with Cystic Fibrosis
Authors Bilton D, Pressler T, Fajac I, Clancy JP, Sands D, Minic P, Cipolli M, Galeva I, Sole A, Quittner AL, Liu K, McGinnis JP, Eagle G, Gupta R, Konstan MW; CLEAR-108 Study Group.
References J Cyst Fibros. 2019 Aug 23. pii: S1569-1993(19)30833-1. doi: 10.1016/j.jcf.2019.08.001.
Keywords ALIS, Amikacin liposome inhalation suspension, CFQ-R, LAI, Liposomal amikacin for inhalation, Pseudomonas aeruginosa
August 2019
Title: Dysregulated insulin in pancreatic insufficient cystic fibrosis with post-prandial hypoglycemia.
Authors Kilberg MJ, Sheikh S, Stefanovski D, Kubrak C, De Leon DD, Hadjiliadis D, Rubenstein RC, Rickels MR, Kelly A
References J Cyst Fibros. 2019 Aug 8. pii: S1569-1993(19)30827-6. doi: 10.1016/j.jcf.2019.07.006
Keywords Glucose tolerance, Hypoglycemia, insulin secretion, pancreatic insufficiency
July 2019
Title: Nasal Potential Difference in suspected Cystic Fibrosis patients with 5T: A step towards better characterization of a CFTR variant with varying clinical consequences
Authors Aalbers BL, Yaakov Y, Derichs N, Simmonds NJ, De Wachter E, Melotti P, De Boeck K, Leal T, Tümmler B, Wilschanski M, Bronsveld I
References J Cyst Fibros. 2019 Jul 19. pii: S1569-1993(19)30822-7. doi: 10.1016/j.jcf.2019.07.001
Keywords CFTR function, CFTR-related disorder, IVS8-5T, Nasal potential difference, sweat chloride
July 2019
Title: Incretin dysfunction and hyperglycemia in cystic fibrosis: role of acyl-ghrelin
Authors Xingshen Sun, Yaling Yi, Bo Liang, Yu Yang, Nan He, Katie Larson Ode, Aliye U, Kai Wang, Katherine N. Gibson-Corley, John F. Engelhardt, Andrew W. Norris
References J Cyst Fibros. 2019 Jul;18(4):557-565. doi: 10.1016/j.jcf.2019.01.010.
Keywords Cystic fibrosis related diabetes, Glucagon-like peptide-1, Glucose-dependent insulinotropic peptide, Growth hormone secretagogue receptor, [D-Lys3]-GHRP-6
Title: Randomized Controlled Study of Aerosolized Hypertonic Xylitol Versus Hypertonic Saline in Hospitalized Patients with Pulmonary Exacerbation of Cystic Fibrosis
Authors Sachinkumar Singh, Douglas Hornick, Janel Fedler, Janice L. Launspach, Mary E. Teresi, Thomas R. Santacroce, Joseph E. Cavanaugh, Rebecca Horan, George Nelson, Timothy D. Starner, Joseph Zabner, Lakshmi Durairaj
References J Cyst Fibros. 2019 Jul 18. pii: S1569-1993(19)30821-5. doi: 10.1016/j.jcf.2019.06.016
Keywords Exacerbations, Lung function, Quality of life, Respiratory symptom score, Sputum microbial density
Title: Ventilation and perfusion assessed by functional MRI in children with CF: reproducibility in comparison to lung function
Authors Sylvia Nyilas, Grzegorz Bauman, Orso Pusterla, Kathryn Ramsey, Florian Singer, Enno Stranzinger, Sophie Yammine, Carmen Casaulta, Oliver Bieri, Philipp Latzin
References J Cyst Fibros. 2019 Jul;18(4):543-550. doi: 10.1016/j.jcf.2018.10.003. Epub 2018 Oct 20.
July 2019
Title: Theranostics by testing CFTR modulators in patient-derived materials: the current status and a proposal for subjects with rare CFTR mutations
Authors Amaral MD, de Boeck K; ECFS Strategic Planning Task Force on ‘Speeding up access to new drugs for CF
References J Cyst Fibros. 2019 Jul 17. pii: S1569-1993(19)30814-8. doi: 10.1016/j.jcf.2019.06.010.
July 2019
Title: Speeding up access to new drugs for Cystic Fibrosis: Considerations for clinical trial design and delivery
Authors On behalf of the European CF Society (ECFS) Strategic Planning Task Force on ‘Speeding up access to new drugs for CF’: Margarida Amaral, Kris de Boeck, Jane C Davies, Pavel Drevinek, J Stuart Elborn, Eitan Kerem, Tim Lee
References J Cyst Fibros. 2019 Jul 11. pii: S1569-1993(19)30815-X. doi: 10.1016/j.jcf.2019.06.011.
Keywords CFTR modulators, Clinical trial design
Title: Supersonic Shear-Wave Elastography and APRI for the detection and staging of liver disease in paediatric cystic fibrosis
Authors Calvopina DA, Noble C, Weis A, Hartel GF, Ramm LE, Balouch F, Fernandez-Rojo MA, Coleman MA, Lewindon PJ, Ramm GA
References J Cyst Fibros. 2019 Jul 11. pii: S1569-1993(19)30820-3. doi: 10.1016/j.jcf.2019.06.017.
Keywords Cirrhosis, Hepatic fibrosis, Non-invasive assessment of liver disease, children's cholestatic liver disease
June 2019
Title: Dissociation of Systemic and Mucosal Autoimmunity in Cystic Fibrosis
Authors Theprungsirikul, J, Skopelja-Gardner, S, Meagher, R. E, Clancy, J. P, Zemanick, E.T, Ashare, A, Rigby, W. F. C
References J Cyst Fibros. 2019 Jun 28. pii: S1569-1993(19)30807-0. doi: 10.1016/j.jcf.2019.06.006.
Title: Dissociation of Systemic and Mucosal Autoimmunity in Cystic Fibrosis
Authors Theprungsirikul, J, Skopelja-Gardner, S, Meagher, R. E, Clancy, J. P, Zemanick, E.T, Ashare, A, Rigby, W. F. C
References J Cyst Fibros. 2019 Jun 28. pii: S1569-1993(19)30807-0. doi: 10.1016/j.jcf.2019.06.006.
Keywords autoimmunity, Bactericidal/permeability-increasing protein (BPI), Pseudomonas aeruginosa
June 2019
Title: Predictors of Pulmonary Exacerbation Treatment in Cystic Fibrosis
Authors Don B Sanders, Joshua S Ostrenga, Margaret Rosenfeld, Aliza K Fink, Michael S Schechter, Gregory S Sawicki, Patrick A Flume, Wayne J Morgan
References J Cyst Fibros 2019 Jun 27 - DOI: 10.1016/j.jcf.2019.06.008
June 2019
Title: Variation in lung function and nutritional decline in cystic fibrosis by genotype: An analysis of the Canadian Cystic Fibrosis Registry
Authors Garvin J. Leung, Ting J. Cho, Thomas Kovesi, Jemila S. Hamid, Dhenuka Radhakrishnan
References J Cyst Fibros. 2019 Jun 25. pii: S1569-1993(19)30812-4. doi: 10.1016/j.jcf.2019.06.007
Title: Variation in lung function and nutritional decline in cystic fibrosis by genotype: An analysis of the Canadian Cystic Fibrosis Registry
Authors Garvin J. Leung, Ting J. Cho, Thomas Kovesi, Jemila S. Hamid, Dhenuka Radhakrishnan
References J Cyst Fibros. 2019 Jun 25. pii: S1569-1993(19)30812-4. doi: 10.1016/j.jcf.2019.06.007
Keywords Body mass index, Epidemiology, Lung function
June 2019
Title: Do current clinical trials in cystic fibrosis match the priorities of patients and clinicans? A systematic review.
Authors Kalaitzis IS, Rowbotham NJ, Smith SJ, Smyth AR
References J Cyst Fibros. 2019 Jun 21. pii: S1569-1993(19)30806-9. doi: 10.1016/j.jcf.2019.06.005.
Keywords Clinical trial, Research priority
June 2019
Title: Disease Progression in Patients with Cystic Fibrosis Treated with Ivacaftor: Data From National US and UK Registries
Authors Volkova N, Moy K, Evans J, Campbell D, Tian S, Simard C, Higgins M, Konstan MW, Sawicki GS, Elbert A, Charman SC, Marshall BC, Bilton D
References J Cyst Fibros. 2019 Jun 10. pii: S1569-1993(19)30767-2. doi: 10.1016/j.jcf.2019.05.015.
Keywords CF complications, CF disease progression, CFTR, CFTR modulator, Ivacaftor
June 2019
Title: Antisense oligonucleotide eluforsen is safe and improves respiratory symptoms in F508DEL cystic fibrosis.
Authors Drevinek P, Pressler T, Cipolli M, De Boeck K, Schwarz C, Bouisset F, Boff M, Henig N, Paquette-Lamontagne N, Montgomery S, Perquin J, Tomkinson N, den Hollander W, Elborn JS
References J Cyst Fibros. 2019 Jun 7. pii: S1569-1993(19)30766-0. doi: 10.1016/j.jcf.2019.05.014
Keywords Antisense oligonucleotide, CFQ-R RSS, Clinical trial, Delta F508, Pulmonary Medicine
June 2019
Title: Non-fasting bioelectrical impedance analysis in cystic fibrosis: Implications for clinical practice and research
Authors Hollander-Kraaijeveld FM, Lindeman Y, de Roos NM, Burghard M, van de Graaf EA, Heijerman HGM
References J Cyst Fibros. 2019 Jun 5. pii: S1569-1993(19)30772-6. doi: 10.1016/j.jcf.2019.05.018
Keywords Anthropometry, Body composition, FEV1%pred, Non-fasting, Single frequency bioelectrical impedance analysis
Title: Blood Biomarkers to Predict Short-term Pulmonary Exacerbation Risk in Children and Adolescents with CF: A Pilot Study
Authors Amrit Singh, Mary He, Virginia Chen, Zsuzsanna Hollander, Scott J. Tebbutt, Raymond T. Ng, Bruce M. McManus, Don D. Sin, Felix Ratjen, Bradley S. Quon
References J Cyst Fibros. 2019 Jun 5. pii: S1569-1993(19)30774-X. doi: 10.1016/j.jcf.2019.05.020.
Keywords Biomarkers, Proteomics, Pulmonary exacerbations
Title: Abnormal Pro-Gly-Pro pathway and airway neutrophilia in pediatric cystic fibrosis
Authors Turnbull AR, Pyle CJ, Patel DF, Jackson PL, Hilliard TN, Regamey N, Tan HL, Brown S, Thursfield R, Short C, Mc Fie M, Alton EWFW, Gaggar A, Blalock JE, Lloyd CM, Bush A, Davies JC, Snelgrove RJ
References J Cyst Fibros. 2019 Jun 5. pii: S1569-1993(19)30769-6. doi: 10.1016/j.jcf.2019.05.017
Keywords matrikine, Neutrophil, protease
May 2019
Title: The use of antimicrobial susceptibility testing in pediatric cystic fibrosis pulmonary exacerbations
Authors Cogen JD, Whitlock KB, Gibson RL, Hoffman LR, VanDevanter DR
References J Cyst Fibros. 2019 May 28. pii: S1569-1993(19)30764-7. doi: 10.1016/j.jcf.2019.05.012.
Keywords Antimicrobial susceptibility testing, Antimicrobial switching, Pediatrics, Pulmonary exacerbations
May 2019
Title: GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1)
Authors Davies JC, Van de Steen O, van Koningsbruggen-Rietschel S, Drevinek P, Derichs N, McKone EF, Kanters D, Allamassey L, Namour F, de Kock H, Conrath K.
References J Cyst Fibros. 2019 May 27. pii: S1569-1993(19)30111-0. doi: 10.1016/j.jcf.2019.05.006.
Keywords CFTR modulator, G551D, GLPG1837, Gating mutation, Ivacaftor
Title: GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1)
Authors Davies JC, Van de Steen O, van Koningsbruggen-Rietschel S, Drevinek P, Derichs N, McKone EF, Kanters D, Allamassey L, Namour F, de Kock H, Conrath K
References J Cyst Fibros. 2019 May 27. pii: S1569-1993(19)30111-0. doi: 10.1016/j.jcf.2019.05.006.
May 2019
Title: Pilot study to test inhaled nitric oxide in cystic fibrosis patients with refractory Mycobacterium abscessus lung infection.
Authors Bentur L, Gur M, Ashkenazi M, Livnat-Levanon G, Mizrahi M, Tal A, Ghaffari A, Geffen Y, Aviram M, Efrati O
References J Cyst Fibros. 2019 May 22. pii: S1569-1993(19)30104-3. doi: 10.1016/j.jcf.2019.05.002.
Keywords Inhaled nitric oxide, Mycobacterium abscessus
May 2019
Title: Peak OGTT glucose is associated with lower lung function in young children with cystic fibrosis.
Authors Prentice BJ, Chelliah A, Ooi CY, Hameed S, Verge CF, Plush L, Widger J.
References J Cyst Fibros. 2019 May 21. pii: S1569-1993(18)30791-4. doi: 10.1016/j.jcf.2019.05.005.
Keywords Abnormal glucose tolerance, CFRD, CGM, Child, Cystic fibrosis-related diabetes, Growth
Title: Peak OGTT glucose is associated with lower lung function in young children with cystic fibrosis.
Authors Prentice BJ, Chelliah A, Ooi CY, Hameed S, Verge CF, Plush L, Widger J.
References J Cyst Fibros. 2019 May 21. pii: S1569-1993(18)30791-4. doi: 10.1016/j.jcf.2019.05.005.
Keywords Abnormal glucose tolerance, CFRD, CGM, Child, Cystic fibrosis-related diabetes, Growth
Title: Peak OGTT glucose is associated with lower lung function in young children with cystic fibrosis.
Authors Prentice BJ, Chelliah A, Ooi CY, Hameed S, Verge CF, Plush L, Widger J.
References J Cyst Fibros. 2019 May 21. pii: S1569-1993(18)30791-4. doi: 10.1016/j.jcf.2019.05.005.
Keywords Abnormal glucose tolerance, CFRD, CGM, Child, Cystic fibrosis-related diabetes, Growth
Title: Correspondence between Lung Function and Symptom Measures from the Cystic Fibrosis Respiratory Symptom Diary–Chronic Respiratory Infection Symptom Score (CFRSD-CRISS)
Authors Laura S. Gold, Donald L. Patrick, Ryan N. Hansen, Christopher H. Goss, and Larry Kessler
References J Cyst Fibros. 2019 May 21. pii: S1569-1993(19)30113-4. doi: 10.1016/j.jcf.2019.05.009.
Keywords Cystic Fibrosis Respiratory Symptom Diary - Chronic Respiratory Infection Symptom Score, Lung function, Pulmonary exacerbations
May 2019
Title: Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines
Authors Ramos KJ, Smith PJ, McKone EF, Pilewski JM, Lucy A, Hempstead SE, Tallarico E, Faro A, Rosenbluth DB, Gray AL, Dunitz JM; CF Lung Transplant Referral Guidelines Committee.
References J Cyst Fibros. 2019 May;18(3):321-333. doi: 10.1016/j.jcf.2019.03.002. Epub 2019 Mar 27.
Keywords Advanced lung disease, Communication, Lung transplant, Modifiable barriers, Referral, Transplant
Title: High airborne level of Aspergillus fumigatus and presence of azole-resistant TR34/L98H isolates in the home of a cystic fibrosis patient harbouring chronic colonisation with azole-resistant H285Y A. fumigatus.
Authors J Cyst Fibros. 2019 May;18(3):364-367. doi: 10.1016/j.jcf.2019.01.001. Epub 2019 Jan 9. Paluch M, Lejeune S, Hecquet E, Prévotat A, Deschildre A, Fréalle E
References J Cyst Fibros. 2019 May;18(3):364-367. doi: 10.1016/j.jcf.2019.01.001. Epub 2019 Jan 9.
Keywords Aspergillus fumigatus, Azole-resistance, Indoor mould exposure
Title: Predictive factors for lumacaftor/ivacaftor clinical response.
Authors Masson A, Schneider-Futschik EK, Baatallah N, Nguyen-Khoa T, Girodon E, Hatton A, Flament T, Le Bourgeois M, Chedevergne F, Bailly C, Kyrilli S, Achimastos D, Hinzpeter A, Edelman A, Sermet-Gaudelus I.
References J Cyst Fibros. 2019 May;18(3):368-374. doi: 10.1016/j.jcf.2018.12.011. Epub 2018 Dec 28.
Title: Differential Expression of Genes and Receptors in Monocytes from Patients with Cystic Fibrosis.
Authors Abdullah A Tarique, Peter D Sly, Diana G Cardenas, Lin Luo, Jennifer L Stow, Scott C Bell, Claire E Wainwright, Emmanuelle Fantino
References J Cyst Fibros. 2019 May;18(3):342-348. doi: 10.1016/j.jcf.2018.07.012. Epub 2018 Sep 1.
Keywords Cox-2, Gamma-chain receptor, IL-13/IL-4 receptor, IRF4, LRP1, MMP28, MMP9, Macrophage polarization, Monocyte-derived macrophages (MDMs), Monocytes, TIMP-1, TLR4
May 2019
Title: Study of 109 Achromobacter spp. isolates from 9 French CF centres reveals the circulation of a multiresistant clone of A. xylosoxidans belonging to ST 137
Authors Lucie Amoureux, Juliette Sauge, Benoit Sarret, Matthieu Lhoumeau, Audrey Bajard, Jennifer Tetu, Julien Bador, Catherine Neuwirth; MucoMicrobes group
References J Cyst Fibros. 2019 May 16. pii: S1569-1993(19)30065-7. doi: 10.1016/j.jcf.2019.04.005
Keywords Achromobacter, Identification, MLST, Resistance, ST 137, nrdA
May 2019
Title: Deep Learning to Automate Brasfield Chest Radiographic Scoring for Cystic Fibrosis
Authors Evan J. Zucker, Zachary A. Barnes, Matthew P. Lungren, Yekaterina Shpanskaya, Jayne M. Seekins, Safwan S. Halabi, David B. Larson
References J Cyst Fibros. 2019 May 2. pii: S1569-1993(19)30075-X. doi: 10.1016/j.jcf.2019.04.016.
Keywords Brasfield, Chest, Deep convolutional neural network, Deep learning, Radiograph
April 2019
Title: An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5 years (KLIMB).
Authors Rosenfeld M, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, Southern KW, Chilvers M, Higgins M, Tian S, Cooke J, Davies JC; KLIMB study group.
References J Cyst Fibros. 2019 Apr 30. pii: S1569-1993(19)30061-X. doi: 10.1016/j.jcf.2019.03.009.
Keywords CFTR potentiator, Ivacaftor, KLIMB, Pediatrics, Safety
April 2019
Title: Early glucose abnormalities are associated with pulmonary inflammation in young children with cystic fibrosis
Authors Prentice BJ, Ooi CY, Strachan RE, Hameed S, Ebrahimkhani S, Waters SA, Verge CF, Widger J
References J Cyst Fibros. 2019 Apr 26. pii: S1569-1993(18)30897-X. doi: 10.1016/j.jcf.2019.03.010.
Keywords CGM, continuous glucose monitoring, Cystic fibrosis-related diabetes, Inflammation
Title: Early glucose abnormalities are associated with pulmonary inflammation in young children with cystic fibrosis
Authors Prentice BJ, Ooi CY, Strachan RE, Hameed S, Ebrahimkhani S, Waters SA, Verge CF, Widger J
References J Cyst Fibros. 2019 Apr 26. pii: S1569-1993(18)30897-X. doi: 10.1016/j.jcf.2019.03.010.
Keywords CGM, continuous glucose monitoring, Cystic fibrosis-related diabetes, Inflammation
Title: Early glucose abnormalities are associated with pulmonary inflammation in young children with cystic fibrosis
Authors Prentice BJ, Ooi CY, Strachan RE, Hameed S, Ebrahimkhani S, Waters SA, Verge CF, Widger J
References J Cyst Fibros. 2019 Apr 26. pii: S1569-1993(18)30897-X. doi: 10.1016/j.jcf.2019.03.010.
Keywords CGM, continuous glucose monitoring, Cystic fibrosis-related diabetes, Inflammation
Title: Mucoid Pseudomonas aeruginosa and regional inflammation in the cystic fibrosis lung
Authors Malhotra S, Hayes D Jr, Wozniak DJ
References J Cyst Fibros. 2019 Apr 26. pii: S1569-1993(19)30069-4. doi: 10.1016/j.jcf.2019.04.009.
Keywords Airway, Cytokines, Inflammation, Lung; Mucoid, Pseudomonas aeruginosa
Title: Mucoid Pseudomonas aeruginosa and regional inflammation in the cystic fibrosis lung
Authors Malhotra S, Hayes D Jr, Wozniak DJ
References J Cyst Fibros. 2019 Apr 26. pii: S1569-1993(19)30069-4. doi: 10.1016/j.jcf.2019.04.009.
Keywords Airway, Cytokines, Inflammation, Lung; Mucoid, Pseudomonas aeruginosa
April 2019
Title: Oxidative Stress and Abnormal Bioactive Lipids in Early Cystic Fibrosis Lung Disease.
Authors Bob J Scholte, Hamed Horati, Mieke Veltman, Rob J Vreeken, Luke W Garratt, Harm A.W.M Tiddens, Hettie M. Janssens, Stephen M Stick, on behalf of the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF)
References J Cyst Fibros. 2019 Apr 25. pii: S1569-1993(19)30071-2. doi: 10.1016/j.jcf.2019.04.011.
Keywords Bronchoalveolar lavage, Isoprostanes, Lysolipids, Mass spectrometry (5), Oxylipins
April 2019
Title: Assessment of endothelial function is reproducible in patients with cystic fibrosis.
Authors Derella CC, Lee N, Crandall R, Blackburn M, Looney J, Mangieri A, Rodriguez-Miguelez P, Tucker MA, Harris RA
References J Cyst Fibros. 2019 Apr 4. pii: S1569-1993(19)30062-1. doi: 10.1016/j.jcf.2019.03.011
Keywords CVD risk, Flow-mediated dilation, Reproducibility, Vascular function
March 2019
Title: The impact of cystic fibrosis-related diabetes on health-related quality of life
Authors Eugenie Kwong, Sameer Desai, Louise Chong, Kathleen Lee, Jie Zheng, Pearce G. Wilcox, Bradley S. Quon
References J Cyst Fibros. 2019 Mar 29. pii: S1569-1993(19)30060-8. doi: 10.1016/j.jcf.2019.03.007.
Keywords CFQ-R teen/adult version, Cystic fibrosis-related diabetes, health-related quality of life, Insulin
March 2019
Title: Markers of bone turnover are reduced in patients with CF related diabetes; the role of glucose.
Authors Inger Hee Mathiesen, Mette Friberg Hitz, Terese Lea Katzenstein, Peter Oturai, Marianne Skov, Niklas Rye Jørgensen, Peter Oestrup Jensen, Christine Raaberg Mikkelsen, Rikke Krogh-Madsen, Tacjana Pressler, Daniel Faurholt-Jepsen.
References J Cyst Fibros. 2019 Mar 27. pii: S1569-1993(18)30809-9. doi: 10.1016/j.jcf.2019.01.004
Keywords Bone turnover, CF related diabetes, glucose
March 2019
Title: Aligning patients’ needs towards a comprehensive CF research program
Authors Noordhoek JJ, Gulmans VAM, Heijerman HGM, van der Ent CK
References J Cyst Fibros. 2019 Mar 26. pii: S1569-1993(18)30857-9. doi: 10.1016/j.jcf.2019.03.008.
Keywords Patient participation, Patient perspective, Research priorities
Title: Physical activity and associations with clinical outcome measures in adults with cystic fibrosis; a systematic review.
Authors Shelley J, Boddy LM, Knowles ZR, Stewart CE, Dawson EA.
References J Cyst Fibros. 2019 Mar 26. pii: S1569-1993(18)30898-1. doi: 10.1016/j.jcf.2019.03.003.
Keywords Exercise, PRISMA, Quality of life, Respiratory disease
March 2019
Title: Assessment of stability and fluctuations of cultured lower airway bacterial communities in people with cystic fibrosis
Authors Laura J. Sherrard, Gisli G. Einarsson, Elinor Johnston, Katherine O’Neill, Leanne McIlreavey, Stephanie J. McGrath, Deirdre F. Gilpin, Damian G. Downey, Alastair Reid, Noel G. McElvaney, Richard C. Boucher, Marianne S. Muhlebach, J. Stuart Elborn, Michael M. Tunney
References J Cyst Fibros. 2019 Mar 21. pii: S1569-1993(19)30052-9. doi: 10.1016/j.jcf.2019.02.012.
Keywords Bacterial density, Bacterial ecology, Extended-quantitative culture, Sputum
March 2019
Title: 3D Printing and the Cystic Fibrosis Lung
Authors Alicia A Mirza, Terry E Robinson, Kyle Gifford, Haiwei Henry Guo
References J Cyst Fibros. 2019 Mar;18(2):278-279. doi: 10.1016/j.jcf.2018.09.004.
Title: Cystic fibrosis related diabetes is not independently associated with increased Stenotrophomonas maltophilia infection: longitudinal data from the UK CF Registry
Authors Freddy Frost, Dilip Nazareth, Matt Shaw, Martin Walshaw
References J Cyst Fibros. 2019 Mar;18(2):294-298. doi: 10.1016/j.jcf.2018.10.011. Epub 2018 Oct 25
March 2019
Title: Use is Associated with Higher Hemoglobin Levels in Individuals with Cystic Fibrosis
Authors Gifford AH, Heltshe SL, Goss CH
References Ann Am Thorac Soc. 2019 Mar;16(3):331-340
Keywords anemia, hemoglobin, Ivacaftor, Lumacaftor
March 2019
Title: The Effects of Cycled Inhaled Aztreonam on the Cystic Fibrosis (CF) Lung Microbiome
Authors Alya A. Heirali; Nicole Acosta; Douglas G. Storey; Matthew L. Workentine; Ranjani Somayaji; Isabelle Laforest-Lapointe, Winnie Leung; Bradley S. Quon; Yves Berthiaum; Harvey R. Rabin; Barbara J. Waddell, Laura Rossi; Michael G. Surette; Michael D. Parkins
References J Cyst Fibros. 2019 Mar 8. pii: S1569-1993(19)30028-1. doi: 10.1016/j.jcf.2019.02.010
Keywords AZLI, Cycled therapy, Inhaled antibiotics, Nebulized, Pseudomonas aeruginosa, Staphylococcus aureus, Streptococcus
February 2019
Title: Abnormal Preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in Cystic Fibrosis.
Authors Hardaker KM, Panda H, Hulme K, Wong A, Coward E, Cooper P, Fitzgerald DA, Pandit C, Towns S, Selvadurai H, Robinson PD.
References J Cyst Fibros. 2019 Feb 28. pii: S1569-1993(19)30025-6. doi: 10.1016/j.jcf.2019.02.007.
Keywords Multiple breath washout, Preschool, Spirometry
February 2019
Title: Early respiratory viral infections in infants with cystic fibrosis
Authors Deschamp AR, Hatch JE, Slaven JE, Gebregziabher N, Storch G, Hall GL, Stick S, Ranganathan S, Ferkol TW, Davis SD.
References J Cyst Fibros. 2019 Feb 27. pii: S1569-1993(18)30804-X. doi: 10.1016/j.jcf.2019.02.004.
Keywords Bacteria, Inflammation, Pathogens, Viruses
February 2019
Title: Inhaled antibiotic use is associated with Scedosporium/Lomentospora species isolation in cystic fibrosis
Authors Hong G, Lechtzin N, Hadjiliadis D, Kawut S
References Pediatr Pulmonol. 2019 Feb;54(2):133-140
Keywords colonization, Epidemiology, Fungi, Infection
February 2019
Title: Social Support is Associated with Fewer Reported Symptoms and Decreased Treatment Burden in Adults with Cystic Fibrosis
Authors Kassie Flewelling; Deborah Sellers; Gregory Sawicki; Walter Robinson; Edward Dill
References J Cyst Fibros. 2019 Feb 13. pii: S1569-1993(19)30017-7. doi: 10.1016/j.jcf.2019.01.013
Keywords Health; Quality of life, Social support, Treatment activity
Title: Do patients with cystic fibrosis participating in clinical trials demonstrate placebo response? A meta-analysis.
Authors Coton J, Le HH, Veuillet V, Janiaud P, Cucherat M, Kassai-Koupai B, Gueyffier F, Reix P.
References J Cyst Fibros. 2019 Feb 13. pii: S1569-1993(18)30833-6. doi: 10.1016/j.jcf.2019.02.003
Keywords clinical trials, Meta-analysis, Placebo response, Randomised
February 2019
Title: CT evaluation of hyperattenuating mucus to diagnose allergic bronchopulmonary aspergillosis in the special condition of cystic fibrosis
Authors John Refait, Julie Macey, Stephanie Bui, Michaël Fayon, Patrick Berger, Laurence Delhaes, François Laurent, Gaël Dournes
References J Cyst Fibros. 2019 Feb 12. pii: S1569-1993(18)30902-0. doi: 10.1016/j.jcf.2019.02.002.
Keywords ABPA, Aspergillus, CT, HAM
February 2019
Title: Lumacaftor-rescued F508del-CFTR has a modified bicarbonate permeability
Authors Loretta Ferrera, Debora Baroni and Oscar Moran
References J Cyst Fibros. 2019 Feb 6. pii: S1569-1993(18)30927-5. doi: 10.1016/j.jcf.2019.01.012.
Keywords CFTR-Corrector, Ion permeability, Patch-clamp
February 2019
Title: Methylomic correlates of autophagy activity in cystic fibrosis
Authors Caution K, Pan A, Krause K, Badr A, Hamilton K, Vaidya A, Gosu H, Daily K, Estfanous S, Gavrilin MA, Drew ME, Cormet-Boyaka E, Chen X, Frankhouser DE, Bundschuh R, Yan P, Dakhlallah D, Amer AO.
References J Cyst Fibros. 2019 Feb 5. pii: S1569-1993(18)30824-5. doi: 10.1016/j.jcf.2019.01.011
January 2019
Title: Exploring the implications of different approaches to estimate centre-level adherence using objective adherence data in an adult cystic fibrosis centre – a retrospective observational study
Authors Hoo ZH, Curley R, Walters SJ, Campbell MJ, Wildman MJ.
References J Cyst Fibros 2019 Oct 31 DOI: 10.1016/j.jcf.2019.10.008
January 2019
Title: The main mechanism associated with progression of glucose intolerance in older patients with cystic fibrosis is insulin resistance and not reduced insulin secretion capacity.
Authors Johann Colomba, Valérie Boudreau, Catherine Lehoux-Dubois, Katherine Desjardins, Adèle Coriati, François Tremblay, Rémi Rabasa-Lhoret
References J Cyst Fibros. 2019 Jan 30. pii: S1569-1993(19)30015-3. doi: 10.1016/j.jcf.2019.01.009
Keywords CFRD, Diabetes, Glycaemia, insulin secretion
Title: Hyperbaric oxygen treatment increases killing of aggregating Pseudomonas aeruginosa isolates from cystic fibrosis patients
Authors Møller, Signe Agnete, Jensen, Peter Østrup, Høiby, Niels, Ciofu, Oana, Kragh, Kasper Nørskov, Bjarnsholt, Thomas, Kolpen, Mette
References J Cyst Fibros. 2019 Jan 30. pii: S1569-1993(19)30011-6. doi: 10.1016/j.jcf.2019.01.005.
Keywords Antibiotic tolerance, Hyperbaric oxygen, Lung infections, Pseudomonas aeruginosa
Title: Antimicrobial susceptibility testing (AST) and associated clinical outcomes in individuals with cystic fibrosis: A systematic review.
Authors Somayaji R, Parkins MD, Shah A, Martiniano SL, Tunney MM, Kahle JS,6 Waters VJ, Elborn JS, Bell SC, Flume PA, VanDevanter DR; Antimicrobial Resistance in Cystic Fibrosis InternationalWorking Group.
References J Cyst Fibros. 2019 Jan 30. pii: S1569-1993(19)30014-1. doi: 10.1016/j.jcf.2019.01.008.
January 2019
Title: Visceral Adipose Tissue is Associated with Poor Diet Quality and Higher Fasting Glucose in Adults with Cystic Fibrosis
Authors Moriah P. Bellissimo, Ivana Zhang, Elizabeth A. Ivie, Phong H. Tran, Vin Tangpricha, William R. Hunt, Arlene A. Stecenko, Thomas R. Ziegler, Jessica A. Alvarez
References J Cyst Fibros. 2019 Jan 18. pii: S1569-1993(18)30812-9. doi: 10.1016/j.jcf.2019.01.002
Keywords Body composition, Diet quality, Fat distribution, Healthy eating index, Nutrition
January 2019
Title: Application of Multiple Event Analysis as an alternative approach to studying pulmonary exacerbations as an outcome measure
Authors Elizabeth Juarez-Colunga, Rosenfeld Margaret, Edith T. Zemanick, Brandie Wagner
References J Cyst Fibros. 2019 Jan 11. pii: S1569-1993(18)30943-3. doi: 10.1016/j.jcf.2018.12.005
Keywords Andersen-Gill model, Cox model, Recurrent event analysis, Time-varying covariates
January 2019
Title: Systemic levels of anti-PAD4 autoantibodies correlate with airway obstruction in cystic fibrosis
Authors Ruchi Yadav, Dae-goon Yoo, J. Michelle Kahlenberg, S. Louis Bridges Jr, Oluwadamilola Oni, Hanwen Huang, Arlene Stecenko and Balázs Rada
References J Cyst Fibros. 2019 Jan 10. pii: S1569-1993(18)30971-8. doi: 10.1016/j.jcf.2018.12.010
Keywords Autoantibody, Lung disease, autoimmunity, Neutrophil extracellular traps, Neutrophils, PAD4
December 2018
Title: Measuring Recovery in Health-Related Quality of Life During and After Pulmonary Exacerbations in Patients with Cystic Fibrosis
Authors Patrick A. Flume, Ellison D. Suthoff, Mark Kosinski, Gautham Marigowda, Alexandra L. Quittner
References J Cyst Fibros. 2018 Dec 23. pii: S1569-1993(18)30942-1. doi: 10.1016/j.jcf.2018.12.004.
Keywords ClinicalTrials.gov identifiers, NCT01807923 and NCT01807949
December 2018
Title: Isogenic cell models of cystic fibrosis-causing variants in natively expressing pulmonary epithelial cells.
Authors Valley HC, Bukis KM, Bell A, Cheng Y, Wong E, Jordan NJ, Allaire NE, Sivachenko A, Liang F, Bihler H, Thomas PJ, Mahiou J, Mense
References J Cyst Fibros. 2018 Dec 15. pii: S1569-1993(18)30940-8. doi: 10.1016/j.jcf.2018.12.001.
Keywords CFTR, CRISPR/Cas9, Model system, Nonsense mutation, Premature termination codon
December 2018
Title: Cirrhosis associated with decreased survival and a 10-year lower median age at death of cystic fibrosis patients in the Netherlands
Authors Pals, FH, Verkade HJ, Gulmans VAM, de Koning BAE, Koot BGP, de Meij TGJ, Hendriks DM, Gierenz N, Vreugdenhil ACE, Houwen RHJ, Bodewes FAJA
References J Cyst Fibros. 2018 Dec 14. pii: S1569-1993(18)30937-8. doi: 10.1016/j.jcf.2018.11.009.
Keywords Cause of deathc Cirrhosis, nutritional status, Pulmonary function, Survival rate
December 2018
Title: Depletion of BAFF cytokine exacerbates infection in Pseudomonas aeruginosa infected mice
Authors Dušan Garić, Shao Tao,Eisha Ahmed, Mina Youssef, Cynthia Kanagaratham,Juhi Shah, Bruce Mazer, Danuta Radzioch
References J Cyst Fibros. 2018 Dec 7. pii: S1569-1993(18)30864-6. doi: 10.1016/j.jcf.2018.11.015.
December 2018
Title: Prevalence and diversity of filamentous fungi in the airways of cystic fibrosis patients - A Dutch, multicentre study.
Authors Engel TGP, Slabbers L, de Jong C, Melchers WJG, Hagen F, Verweij PE, Merkus P, Meis JF; Dutch Cystic Fibrosis Fungal Collection Consortium
References J Cyst Fibros. 2018 Dec 1. pii: S1569-1993(18)30939-1. doi: 10.1016/j.jcf.2018.11.012.
Keywords Aspergillus, Azole resistance, Cystic fibrosis, Epidemiology, Filamentous fungi, Penicillium
Theme Epidemiology/Models of care
November 2018
Title: Non-Invasive Ventilation and Clinical Outcomes in Cystic Fibrosis: findings from the UK CF Registry
Authors Olga Archangelidi, Siobhán B. Carr, Nicholas J. Simmonds, Diana Bilton, Winston Banya, Paul Cullinan on behalf of CF-EpiNet
References J Cyst Fibros. 2018 Nov 28. pii: S1569-1993(18)30934-2. doi: 10.1016/j.jcf.2018.11.006.
Keywords Non-invasive ventilation, Survival
November 2018
Title: Pre-transplant factors associated with mortality after lung transplantation in cystic fibrosis: A systematic review and meta-analysis
Authors Angela Koutsokera, Rhea A. Varughese, Jenna Sykes, Ani Orchanian-Cheff, Prakesh S. Shah, Cecilia Chaparro, Elizabeth Tullis, Lianne G. Singer, Anne L. Stephenson
Keywords Lung transplantation, Predictors, mortality
