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
November 2018
Title: Dental and periodontal manifestations in patients with cystic fibrosis - A systematic review
Authors Pawlaczyk-Kamieńska T, Borysewicz-Lewicka M, Śniatała R, Batura-Gabryel H, Cofta S.
References J Cyst Fibros. 2018 Nov 22. pii: S1569-1993(18)30935-4. doi: 10.1016/j.jcf.2018.11.007.
Keywords Dental caries, Enamel defects, Periodontal status
November 2018
Title: Eluforsen: a potential new treatment option for people with cystic fibrosis (CF) due to the F508del gene mutation?
Authors Isabelle Sermet-Gaudelus, John P. Clancy, David P. Nichols, Jerry A. Nick, Kris De Boeck, George M. Solomon, Marcus A. Mall,h,i, James Bolognese, Florilene Bouisset, Wilhelmina den Hollander, Nicolas Paquette-Lamontagne, Nigel Tomkinson, Noreen Henig, J.
References J Cyst Fibros. 2018 Nov 19. pii: S1569-1993(18)30914-7. doi: 10.1016/j.jcf.2018.10.015
Keywords Antisense oligonucleotide, Clinical trial, Cystic Fibrosis Transmembrane Conductance Regulator delta F508, Nasal potential difference, Pulmonary Medicine
November 2018
Title: Use of telavancin in adolescent patients with cystic fibrosis and prior intolerance to vancomycin: A case series.
Authors Bernstein AT, Leigh MW, Goralski JL, Esther CR Jr, McKinzie CJ.
References J Cyst Fibros. 2018 Nov;17(6):e48-e50. doi: 10.1016/j.jcf.2018.08.003.
Keywords Child, MRSA, Pediatric, Telavancin, Vancomycin
November 2018
Title: Comparison of Lung Clearance Index Determined by Washout of N2 and SF6 in Infants and Preschool Children with Cystic Fibrosis
Authors Stahl M, Joachim C, Wielpütz MO, Mall MA
References J Cyst Fibros. 2018 Nov 9. pii: S1569-1993(18)30929-9. doi: 10.1016/j.jcf.2018.11.001
Keywords Clinical trial endpoint, Early CF lung disease, Infant lung function test, Lung clearance index, Magnetic Resonance Imaging, Multiple breath washout
November 2018
Title: Glycosaminoglycans are differentially involved in bacterial binding to healthy and cystic fibrosis lung cells.
Authors Martin C, Lozano-Iturbe V, Girón RM, Vazquez-Espinosa E, Rodriguez D, Merayo-Lloves J, Vazquez F, Quirós LM, García B.
References J Cyst Fibros. 2018 Nov 8. pii: S1569-1993(18)30920-2. doi: 10.1016/j.jcf.2018.10.017.
Keywords Bacteria, Glycosaminoglycans, Host interaction, Infection, Lung cells
November 2018
Title: Validation of the French 3-year prognostic score using the Canadian Cystic Fibrosis registry.
Authors Adele Coriati, Jenna Sykes, Lionelle Nkam, Mounia N. Hocine, Pierre-Regis Burgel, Anne L. Stephenson
References J Cyst Fibros. 2018 Nov 2. pii: S1569-1993(18)30911-1. doi: 10.1016/j.jcf.2018.10.014
Keywords Data Registry, Death, Lung transplantation, Predictive score
October 2018
Title: Failure to conceive in women with CF is associated with pancreatic insufficiency and advancing age
Authors Shteinberg M, Lulu AB, Downey DG, Blumenfeld Z, Rousset-Jablonski C, Perceval M, Colombo A, Stein N, Livnat G, Gur M, Bentur L, Mussaffi H, Blau H, Sarouk I, Dagan A, Kerem E, Aviram M, Picard E, Aliberti S, Álvarez A, Miranda JP, Polverino E, Durieu I, S
References J Cyst Fibros. 2018 Oct 23. pii: S1569-1993(18)30867-1. doi: 10.1016/j.jcf.2018.10.009.
Keywords Adult, Fertility
October 2018
Title: Prevalence, geographic risk factor, and development of a standardized protocol for fungal isolation in cystic fibrosis: Results from the international prospective study “MFIP”
Authors Delhaes L, Touati K, Faure-Cognet O, Cornet M, Botterel F, DannaouiI E, Morio F, Le Pape P, Grenouillet F, Favennec L, Le Gal S, Nevez G, Duhamel A, Borman A, Saegeman V, Lagrou K, Gomez E, Carro ML, Canton R, Campana S, Buzina W, Chen S, Meyer W, Roilide
References J Cyst Fibros. 2018 Oct 19. pii: S1569-1993(18)30839-7. doi: 10.1016/j.jcf.2018.10.001
Keywords Aspergillus, Filamentous fungi, Lung mycobiota, Molds, Mycological examination
October 2018
Title: Seasonal fluctuation of lung function in cystic fibrosis: A national register-based study in two northern European populations
Authors Tavs Qvist, Daniela K. Schlüter, Vian Rajabzadeh, Peter J. Diggle, Tania Pressler, Siobhán B. Carr, David Taylor-Robinson
References J Cyst Fibros. 2018 Oct 18. pii: S1569-1993(18)30858-0. doi: 10.1016/j.jcf.2018.10.006.
Keywords Fluctuation, Percent predicted FEV1, Season, Seasonality
October 2018
Title: Combined antifungal therapy is superior to monotherapy in pulmonary scedosporiosis in cystic fibrosis
Authors Carsten Schwarz, Claudia Brandt, Volker Melicha, Christoph Runge, Eberhard Heuer, Hany Sahly, Martin Schebek, Holger Köster, Jean-Philippe Bouchara, Thomas Biedermann, Peter Meißner, Jörg Große-Onnebrink, Heino Skopnik, Dominik Hartl, Ludwig Sedlacek, Kat
References J Cyst Fibros. 2018 Oct 5. pii: S1569-1993(18)30794-X. doi: 10.1016/j.jcf.2018.08.012
Keywords Anti-fungal treatment, Mycosis, Pneumonia
September 2018
Title: Phenotypic spectrum of patients with cystic fibrosis and cystic fibrosis-related disease carrying p.Arg117His
Authors Katherine Keenan; Annie Dupuis; Katherine Griffin; Carlo Castellani; Elizabeth Tullis; Tanja Gonska
References J Cyst Fibros. 2018 Sep 29. pii: S1569-1993(18)30800-2. doi: 10.1016/j.jcf.2018.09.002
Keywords CFTR-related disease, Cystic fibrosis newborn screening, Diagnosis, P.Arg117His
September 2018
Title: Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV1) in cystic fibrosis patients receiving ivacaftor treatment.
Authors Nagy B Jr, Bene Z, Fejes Z, Heltshe SL, Reid D, Ronan NJ, McCarthy Y, Smith D, Nagy A, Joseloff E, Balla G, Kappelmayer J, Macek M Jr, Bell SC, Plant BJ, Amaral MD, Balogh I.
References J Cyst Fibros. 2018 Sep 26. pii: S1569-1993(18)30795-1. doi: 10.1016/j.jcf.2018.08.013.
Keywords BMI, Biomarker, FEV(1), HE4, Ivacaftor, sweat chloride
Title: Mental Health screening in cystic fibrosis centres across Europe
Authors J. Abbott, T. Havermans, S. Jarvholm, E. Landau, Y. Prins, U. Smrekar, D. Staab, L. Verity, M. Verkleij on behalf of the ECFS Mental Health Working Group
References J Cyst Fibros. 2018 Sep 26. pii: S1569-1993(18)30801-4. doi: 10.1016/j.jcf.2018.09.003
September 2018
Title: The Q359K/T360K mutation causes cystic fibrosis in Georgian Jews.
Authors Mei-Zahav M, Stafler P, Senderowitz H, Bentur L, Livnat G, Shteinberg M, Orenstein N, Bazak L, Prais D, Levine H, Gur M, Khazanov N, Simhaev L, Eliyahu H, Cohen M, Wilschanski M, Blau H, Mussaffi H.
References J Cyst Fibros. 2018 Sep;17(5):e41-e45. doi: 10.1016/j.jcf.2018.06.008. Epub 2018 Jul 20.
Keywords Georgian Jews, Q359K/T360K mutation, Structural models of CFTR
September 2018
Title: Repeated Hot Water and Steam Disinfection of Pari LC Plus® Nebulizers Alters Nebulizer Output
Authors Melanie Sue Collins, Matthew O’Brien, Craig M. Schramm, Thomas S Murray
References J Cyst Fibros. 2018 Sep 14. pii: S1569-1993(18)30753-7. doi: 10.1016/j.jcf.2018.08.005.
Keywords Cleaning, Disinfection, Home nebulizer, Steam sterilization
September 2018
Title: Higher Interleukin-7 serum concentrations in patients with cystic fibrosis correlate with impaired lung function
Authors Julia Seyfarth1, Sutharsan Sivagurunathan1, Sarah Ricken, Gerhard Weinreich, Laura Olbrich, Christian Taube, Ertan Mayatepek, Dirk Schramm, Marc Jacobsen
Keywords Interleukin-7, BMI, FEV1, Staphylococcus aureus
September 2018
Title: Rate and Predictors of Prescription of Lumacaftor – Ivacaftor in the 18 Months Following Approval in the United States
Authors Gregory S. Sawicki, Aliza K. Fink, Michael S. Schechter, Deena R. Loeffler, Nicole Mayer-Hamblett
References J Cyst Fibros. 2018 Sep 7. pii: S1569-1993(18)30755-0. doi: 10.1016/j.jcf.2018.08.007.
September 2018
Title: Breathlessness catastrophizing relates to poorer quality of life in adults with cystic fibrosis
Authors Maras D, Balfour L, Tasca GA, Gaudet E, Aaron SD, Cameron WD, Pakhale S
References J Cyst Fibros. 2018 Sep 6. pii: S1569-1993(18)30756-2. doi: 10.1016/j.jcf.2018.08.008
Keywords anxiety, Breathlessness Catastrophizing, depression, Quality of life
August 2018
Title: Investigating the effects of long-term dornase alfa use on lung function using registry data.
Authors Newsome SJ, Daniel RM, Carr SB, Bilton D, Keogh RH.
References J Cyst Fibros. 2018 Aug 29. pii: S1569-1993(18)30747-1. doi: 10.1016/j.jcf.2018.08.004
Keywords Long-term treatment effect; Patient registry; UK Cystic Fibrosis Registry
August 2018
Title: Correspondence between symptoms and preference-based health status measures in the STOP study.
Authors Gold LS, Patrick DL, Hansen RN, Beckett V, Goss CH, Kessler L.
References J Cyst Fibros. 2018 Aug 28. pii: S1569-1993(18)30743-4. doi: 10.1016/j.jcf.2018.08.001.
Keywords 5-level EuroQOL-5 dimensions, Cost effectiveness analysis, Cystic Fibrosis Respiratory Symptom Diary - Chronic Respiratory Infection Symptom Score, Lung function, Pulmonary exacerbations
August 2018
Title: DNA-analysis, Extended CFTR-gene analysis, Immuno-reactive trypsin, Newborn screening, Pancreatitis-associated protein,Validity
Authors Dankert-Roelse JE, Bouva MJ, Jakobs BS, Janssens HM, de Winter-de Groot KM, Schönbeck Y, Gille JJP, Gulmans VAM, Verschoof-Puite RK, Schielen PCJI, Verkerk PH
References J Cyst Fibros. 2018 Aug 23. pii: S1569-1993(18)30705-7. doi: 10.1016/j.jcf.2018.07.008
Keywords DNA-analysis, Extended CFTR-gene analysis, Immuno-reactive trypsin, Newborn screening, Pancreatitis-associated protein, Validity
Title: Lumacaftor/Ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV1.
Authors McColley SA, Konstan MW, Ramsey BW, Stuart Elborn J, Boyle MP, Wainwright CE, Waltz D, Vera-Llonch M, Marigowda G, Jiang JG, Rubin JL
References J Cyst Fibros. 2018 Aug 23. pii: S1569-1993(18)30719-7. doi: 10.1016/j.jcf.2018.07.011.
Keywords Ivacaftor, Lumacaftor, Percent predicted forced expiratory volume in 1 s, Pulmonary exacerbations
August 2018
Title: Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome
Authors Liu Q, Sabirzhanova I, Yanda MK, Bergbower EAS, Boinot C, Guggino WB, Cebotaru L
References J Cyst Fibros. 2018 Jun 20. pii: S1569-1993(18)30621-0. doi: 10.1016/j.jcf.2018.05.016.
Keywords Autophagy, CFTR, Correctors, Degradation, Mutations, Short circuit current, Western blot
August 2018
Title: Eradication of Persistent Methicillin-resistant Staphylococcus aureus Infection in Cystic Fibrosis
Authors Rebecca Dezube*, Mark T. Jennings*, Mary Rykiel, Marie Diener-West, Michael P. Boyle, James F. Chmiel, Elliott C. Dasenbrook - *Co-First Authors
References J Cyst Fibros. 2018 Aug 18. pii: S1569-1993(18)30702-1. doi: 10.1016/j.jcf.2018.07.005
Keywords Clinical trial, MRSA
August 2018
Title: Antisense oligonucleotide targeting of mRNAs encoding ENaC subunits α, β, and ɣ improves cystic fibrosis-like disease in mice
Authors Zhao C, Crosby J, Lv T, Bai D, Monia BP, Guo S.
References J Cyst Fibros. 2018 Aug 10. pii: S1569-1993(18)30703-3. doi: 10.1016/j.jcf.2018.07.006
Keywords Antisense oligonucleotide, ENaC, Subunit
Title: Continuous glucose monitoring in youth with cystic fibrosis treated with lumacaftor-ivacaftor
Authors Angel Li, TimVigers, Laura Pyle, Edith Zemanick, Kristen Nadeau, Scott D. Sagel, Christine L. Chan
References J Cyst Fibros. 2018 Aug 10. pii: S1569-1993(18)30718-5. doi: 10.1016/j.jcf.2018.07.010
Keywords continuous glucose monitoring, Cystic fibrosis related diabetes, Cystic fibrosis transmembrane conductance regulator protein modulator
July 2018
Title: Respiratory rate in infants with cystic fibrosis throughout the first year of life and association with lung clearance index measured shortly after birth
Authors Korten I, Kieninger E, Yammine S, Cangiano G, Nyilas S, Anagnostopoulou P, Singer F, Kuehni CE, Regamey N, Frey U, Casaulta C, Spycher BD, Latzin P; SCILD; BILD study group.
References J Cyst Fibros. 2018 Jul 27. pii: S1569-1993(18)30696-9. doi: 10.1016/j.jcf.2018.07.002.
Keywords Infant lung function, Lung clearance index, Respiratory rate
July 2018
Title: R560S: a class II CFTR mutation that is not rescued by current modulators
Authors Awatade NT, Ramalho S, Silva IAL, Felício V, Botelho HM, de Poel E, Vonk A, Beekman JM, Farinha CM, Amaral MD
References J Cyst Fibros. 2018 Jul 18. pii: S1569-1993(18)30643-X. doi: 10.1016/j.jcf.2018.07.001
Keywords CFTR, CFTR modulators, Ex vivo biomarkers, Intestinal organoids, R560S, Rare mutations
July 2018
Title: Prevalence of hypoglycemia during oral glucose tolerance testing in adults with cystic fibrosis and risk of developing cystic fibrosis-related diabetes.
Authors Mannik LA, Chang KA, Annoh PQK, Sykes J, Gilmour J, Robert R, Stephenson A
References J Cyst Fibros. 2018 Jul;17(4):536-541. doi: 10.1016/j.jcf.2018.03.009. Epub 2018 Apr 19.
Keywords diabetes mellitus, Glucose tolerance test, Hypoglycemia
July 2018
Title: Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis.
Authors Schechter MS, Schmidt HJ, Williams R, Norton R, Taylor D, Molzhon A
References J Cyst Fibros. 2018 Jul 13. pii: S1569-1993(18)30628-3. doi: 10.1016/j.jcf.2018.06.003.
Keywords Algorithm, FEV1, Lung function, Pulmonary exacerbation, quality improvement
July 2018
Title: Age-Related differences in Dental Caries and Associated Risk Factors in Individuals with Cystic Fibrosis Ages 6-20 years: A Pilot Study
Authors Donald L. Chi, Margaret Rosenfeld, Lloyd Mancl, Whasun O. Chung, Richard B. Presland, Elise Sarvas, Marilynn Rothen, Alaa Alkhateeba, Sharon McNamara, Alan Genatassio, Isabel Virella-Lowell, Carlos Milla, JoAnna Scott
References J Cyst Fibros. 2018 Jul 10. pii: S1569-1993(18)30642-8. doi: 10.1016/j.jcf.2018.06.009.
Keywords Caries risk factors, Dental caries, Pediatric dentistry
July 2018
Title: Establishment of a ΔF508-CF promyelocytic cell line for cystic fibrosis research and drug screening
Authors Scott Jennings, Hang Pong Ng, Guoshun Wang
Keywords ΔF508-CF, Neutrophils, HL-60 cells, Promyelocytic cell line
July 2018
Title: Italian and North American dietary intake after ivacaftor treatment for Cystic Fibrosis Gating Mutations.
Authors Sainath NN, Schall J, Bertolaso C, McAnlis C, Stallings VA
References J Cyst Fibros. 2018 Jul 5. pii: S1569-1993(18)30629-5. doi: 10.1016/j.jcf.2018.06.004
June 2018
Title: A Specialized Method of Sputum Collection and Processing for Therapeutic Interventions in Cystic Fibrosis
Authors McElvaney OJ, Gunaratnam C, Reeves EP, McElvaney NG
References J Cyst Fibros. 2018 Jun 27. pii: S1569-1993(18)30625-8. doi: 10.1016/j.jcf.2018.06.001.
Keywords clinical trials, Cytokines, Induced sputum, Inflammation, Interleukin-1β, Neutrophil elastase
June 2018
Title: RNA sequencing data from neutrophils of patients with cystic fibrosis reveals potential for developing biomarkers for pulmonary exacerbations
Authors Kaiyu Jiang , Kerry E. Poppenberg, Laiping Wong, Yanmin Chen, Drucy Borowitz, Danielle Goetz, Daniel Sheehan, Carla Frederick , Vincent M. Tutino, Hui Meng, James N. Jarvis
Keywords Biomarkers, Transcriptome, Neutrophils, RNA, Sequencing
June 2018
Title: Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent
Authors Binyu Lu, Li Li Molly Schneider, Craig A. Hodges, James A. Burgess, Thomas J. Kelley
References J Cyst Fibros. 2018 Jun 22. pii: S1569-1993(18)30630-1. doi: 10.1016/j.jcf.2018.06.005
Keywords Cholesterol, Microtubule
June 2018
Title: Resistin is elevated in cystic fibrosis sputum and correlates negatively with lung function.
Authors Forrest OA, Chopyk DM, Gernez Y, Brown MR, Conrad CK, Moss RB, Tangpricha V, Peng L, Tirouvanziam R.
References J Cyst Fibros. 2018 Jun 21. pii: S1569-1993(18)30623-4. doi: 10.1016/j.jcf.2018.05.018.
Keywords Biomarker, Inflammation, Neutrophils, Plasma, Sputum
Title: AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations
Authors Rebecca J. Darrah, Frank J. Jacono, Neha Joshi, Anna L. Mitchell, Abdus Sattar, Cara K. Campanaro, Paul Litman, Jennifer Frey, David E. Nethery, Eric S. Barbato, Craig A. Hodges, Harriet Corvol, Garry R. Cutting, Michael R. Knowles, Lisa J. Strug, Mitchel
References J Cyst Fibros. 2018 Jun 21. pii: S1569-1993(18)30618-0. doi: 10.1016/j.jcf.2018.05.013.
Keywords Angiotensin signaling, GWAS follow up, Mouse models of airway disease, Pulmonary function
June 2018
Title: SPX-101 is stable in and retains function after exposure to cystic fibrosis sputum.
Authors Sesma JI, Wu B, Stuhlmiller TJ, Scott DW
References J Cyst Fibros. 2018 Jun 20. pii: S1569-1993(18)30627-1. doi: 10.1016/j.jcf.2018.06.002
Keywords Airway hydration, ENaC, Mucus, SPX-101
June 2018
Title: Characteristics and outcomes of oral antibiotic treated pulmonary exacerbations in children with cystic fibrosis.
Authors Hoppe JE, Wagner BD, Accurso FJ, Zemanick ET, Sagel SD
References J Cyst Fibros. 2018 Jun 16. pii: S1569-1993(18)30620-9. doi: 10.1016/j.jcf.2018.05.015.
Keywords Infection, Inflammation, Lung function, Pulmonary exacerbation
June 2018
Title: Combination potentiator ('co-potentiator') therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators.
Authors Phuan PW, Son JH, Tan JA, Li C, Musante I, Zlock L, Nielson DW, Finkbeiner WE, Kurth MJ, Galietta LJ, Haggie PM, Verkman AS
References J Cyst Fibros. 2018 Jun 11. pii: S1569-1993(18)30592-7. doi: 10.1016/j.jcf.2018.05.010.
Keywords CFTR, High-throughput screen, N1303K, Potentiator
June 2018
Title: Physiological and pharmacological characterization of the N1303K mutant CFTR.
Authors DeStefano S, Gees M, Hwang TC
References J Cyst Fibros. 2018 Jun 7. pii: S1569-1993(18)30593-9. doi: 10.1016/j.jcf.2018.05.011.
Keywords GLPG1837, Gating; N1303K, VX-661, VX-770, VX-809
June 2018
Title: Continuous glucose monitoring guided insulin therapy is associated with improved clinical outcomes in cystic fibrosis-related diabetes.
Authors Freddy Frost, Dilip Nazareth, Paula Dyce, Victoria Malone, Martin J Walshaw
References J Cyst Fibros. 2018 Jun 6. pii: S1569-1993(18)30587-3. doi: 10.1016/j.jcf.2018.05.005.
June 2018
Title: Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosis
Authors Montgomery ST, Dittrich AS, Garratt LW, Turkovic L, Frey DL, Stick SM, Mall MA, Kicic A AREST CF
References J Cyst Fibros. 2018 Jun 5. pii: S1569-1993(18)30588-5. doi: 10.1016/j.jcf.2018.05.006
Keywords Airway epithelium, Hypoxia, Interleukin (IL)-1, Mucus, Neutrophilic inflammation
June 2018
Title: Measured fetal and neonatal exposure to Lumacaftor and Ivacaftor during pregnancy and while breastfeeding
Authors Trimble A, McKinzie C, Terrell M, Stringer E, Esther CR Jr.
References J Cyst Fibros. 2018 Jun 1. pii: S1569-1993(18)30591-5. doi: 10.1016/j.jcf.2018.05.009.
Keywords CFTR modulators, Ivacaftor, Lumacaftor, pregnancy
May 2018
Title: Primary sclerosing cholangitis is associated with abnormalities in CFTR.
Authors Werlin S, Scotet V, Uguen K, Audrezet MP, Cohen M, Yaakov Y, Safadi R, Ilan Y, Konikoff F, Galun E, Mizrahi M, Slae M, Sayag S, Cohen-Cymberknoh M, Wilschanski M, Ferec C
References J Cyst Fibros. 2018 May 26. pii: S1569-1993(18)30115-2. doi: 10.1016/j.jcf.2018.04.005.
Keywords CFTR-related disorder, Nasal potential difference, New generation sequencing, Primary sclerosing cholangitis
May 2018
Title: Emergence of livestock-associated MRSA isolated from cystic fibrosis patients: result of a Belgian national survey
Authors Dodémont M, Argudín MA, Willekens J, Vanderhelst E, Pierard D, Miendje Deyi VY, Hanssens L, Franckx H, Schelstraete P, Leroux-Roels I, Nonhoff C, Deplano A, Knoop C, Malfroot A, Denis O
References J Cyst Fibros. 2018 May 24. pii: S1569-1993(18)30577-0. doi: 10.1016/j.jcf.2018.04.008.
Keywords CC398, LA-MRSA, Small colony variant, spa-typing
Title: Multicentre Standardisation of Chest MRI as Radiation-Free Outcome Measure of Lung Disease in Young Children with Cystic Fibrosis
Authors Mark O. Wielpütz, Oyunbileg von Stackelberg, Mirjam Stahl, Bertram J. Jobst, Monika Eichinger, Michael U. Puderbach, Lutz Nährlich, Sandra Barth, Christian Schneider, Matthias V. Kopp, Isabell Ricklefs, Michael Buchholz, Burkhard Tümmler, Christian Dopfer
References J Cyst Fibros. 2018 May 24. pii: S1569-1993(18)30581-2. doi: 10.1016/j.jcf.2018.05.003.
Keywords Airway disease, Functional lung imaging, Lung perfusion, Magnetic Resonance Imaging
May 2018
Title: Strategies for newborn screening for cystic fibrosis: A systematic review of health economic evaluations.
Authors Schmidt M, Werbrouck A, Verhaeghe N, De Wachter E, Simoens S, Annemans L, Putman K
References J Cyst Fibros. 2018 May;17(3):306-315. doi: 10.1016/j.jcf.2018.03.002
Keywords Health economic evaluation, Newborn screening, Systematic review
Title: Audiometric assessment of paediatric patients with cystic fibrosis
Authors Kreicher KL, Bauschard MJ, Clemmens CS, Riva CM, Meyer TA.
References J Cyst Fibros. 2018 May;17(3):383-390. doi: 10.1016/j.jcf.2017.10.007. Epub 2017 Dec 27
Keywords Audiogram, Conductive hearing loss, Hearing loss, Otitis media, Sensorineural hearing loss
Title: Comparison of ex vivo and in vitro intestinal cystic fibrosis models to measure CFTR-dependent ion channel activity.
Authors Zomer-van Ommen DD, de Poel E, Kruisselbrink E, Oppelaar H, Vonk AM, Janssens HM, van der Ent CK, Hagemeijer MC, Beekman JM
References J Cyst Fibros. 2018 May;17(3):316-324. doi: 10.1016/j.jcf.2018.02.007.
Keywords Biopsies, CFTR, Differentiation, Donor-matched, Electrophysiology, Monolayers, Organoids
May 2018
Title: A prospective analysis of unplanned patient-initiated contacts in an adult cystic fibrosis centre.
Authors Burnet E, Hubert D, Champreux J, Honoré I, Kanaan R, Panzo R, Burgel PR
References J Cyst Fibros. 2018 May 7. pii: S1569-1993(18)30116-4. doi: 10.1016/j.jcf.2018.04.006
Keywords Ambulatory care, Contact frequency, Continuity of care, Outpatient, Patient-initiated contact, Telephone triage
April 2018
Title: Eradication of early P. aeruginosa infection in children <7 years of age with cystic fibrosis: The early study
Authors Felix Ratjen, Alexander Moeller, Martha L.McKinney, Irina Asherova, Nipa Alon, Robert Maykutn, Gerhild Angyalosi
Keywords Early eradication, Paediatric patient, Placebo-controlled, Recurrence, Tobramycin inhalation solution
April 2018
Title: Pulmonary exacerbations and acute declines in lung function in patients with cystic fibrosis
Authors Wagener JS, Williams MJ, Millar SJ, Morgan WJ, Pasta DJ, Konstan MW
References J Cyst Fibros. 2018 Apr 20. pii: S1569-1993(18)30026-2. doi: 10.1016/j.jcf.2018.02.003.
Keywords Lung function, Pulmonary exacerbations
Title: Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.
Authors Guimbellot J, Solomon GM, Baines A, Heltshe SL, VanDalfsen J, Joseloff E, Sagel SD, Rowe SM; GOALe(2) Investigators.
References J Cyst Fibros. 2018 Apr 20. pii: S1569-1993(18)30090-0. doi: 10.1016/j.jcf.2018.04.004.
Keywords CFTR potentiator, clinical trials, Gating mutation, Ivacaftor
Title: The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.
Authors Eckford PDW, McCormack J, Munsie L, He G, Stanojevic S, Pereira SL, Ho K, Avolio J, Bartlett C, Yang JY, Wong AP, Wellhauser L, Huan LJ, Jiang JX, Ouyang H, Du K, Klingel M, Kyriakopoulou L, Gonska T, Moraes TJ, Strug LJ, Rossant J, Ratjen F, Bear CE
References J Cyst Fibros. 2018 Apr 20. pii: S1569-1993(18)30086-9. doi: 10.1016/j.jcf.2018.03.013.
Keywords Clinical outcome data, Cultures, Drug responses, Nasal, Patient-specific primary tissues, Pluripotent stem cells, Precision medicine, RNAseq, Resource for discovery, Whole genome sequencing
April 2018
Title: Insights into the cystic fibrosis care in Eastern Europe: results of survey
Authors Walicka-Serzysko K, Peckova M, Noordhoek JJ, Sands D, Drevinek P
References J Cyst Fibros. 2018 Apr 19. pii: S1569-1993(18)30088-2. doi: 10.1016/j.jcf.2018.04.003.
Keywords Eastern Europe; Standards of care
April 2018
Title: CFTR rescue with VX-809 and VX-770 favors the repair of primary airway epithelial cell cultures from patients with class II mutations in the presence of Pseudomonas aeruginosa exoproducts.
Authors Adam D, Bilodeau C, Sognigbé L, Maillé É, Ruffin M, Brochiero E.
References J Cyst Fibros. 2018 Apr 13. pii: S1569-1993(18)30082-1. doi: 10.1016/j.jcf.2018.03.010.
Keywords J Cyst Fibros. 2018 Apr 13. pii: S1569-1993(18)30082-1. doi: 10.1016/j.jcf.2018.03.010.
April 2018
Title: Establishing the diagnosis of chronic colonization with Pseudomonas aeruginosa of cystic fibrosis patients: Comparison of the European consensus criteria with genotyping of P. aeruginosa isolates
Authors Jonckheere L, Schelstraete P, Van Simaey L, Van Braeckel E, Willekens J, Van Daele S, De Baets F, Vaneechoutte M
References J Cyst Fibros. 2018 Apr 11. pii: S1569-1993(18)30074-2. doi: 10.1016/j.jcf.2018.03.004.
Keywords European consensus criteria (ECC), Genotyping (GT), McRAPD, Pseudomonas aeruginosa
March 2018
Title: Prevalence of severe fatigue among adults with cystic fibrosis: a single center study
Authors Merel M. Nap-van der Vlist, Marcella Burghard, Erik J. Hulzebos, Wytze R. Doeleman, Harry G. Heijerman, C. Kors van der Ent, Sanne L. Nijhof
References J Cyst Fibros. 2018 Mar 29. pii: S1569-1993(18)30073-0. doi: 10.1016/j.jcf.2018.03.003.
Keywords Exercise capacity, FEV(1), Fatigue, Quality of life
March 2018
Title: Continuous glucose monitoring abnormalities in cystic fibrosis youth correlate with pulmonary function decline.
Authors Chan CL, Vigers T, Pyle L, Zeitler PS, Sagel SD, Nadeau KJ.
References J Cyst Fibros. 2018 Mar 23. pii: S1569-1993(18)30080-8. doi: 10.1016/j.jcf.2018.03.008.
Keywords Continuous glucose monitoring; Cystic fibrosis related diabetes; Pediatrics
Title: Sweat Test for Cystic Fibrosis: Wearable Sweat Sensor vs. Standard Laboratory Test
Authors Dong-Hoon Choi, Abigail Thaxton, In cheol Jeong, Kain Kim, Patrick R. Sosnay, Garry R. Cutting, Peter C. Searsona
References J Cyst Fibros. 2018 Mar 23. pii: S1569-1993(18)30075-4. doi: 10.1016/j.jcf.2018.03.005.
March 2018
Title: Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data
Authors Ruth Keogh, Rhonda Szczesniak, David Taylor-Robinson, Diana Bilton
References J Cyst Fibros. 2018 Mar;17(2):218-227. doi: 10.1016/j.jcf.2017.11.019.
Keywords CFTR genotype, Flexible parametric survival model, Longitudinal study, patient registry, Survival
Title: A guide to interpreting estimated median age of survival in cystic fibrosis patient registry reports
Authors Ruth H. Keogh, Sanja Stanojevic
References J Cyst Fibros. 2018 Mar;17(2):213-217. doi: 10.1016/j.jcf.2017.11.014
Keywords Annual report, Birth cohort, Conditional survival, Median survival, patient registry, Period estimates, UK Cystic Fibrosis Registry
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: Mini-guts in a dish: Perspectives of adult Cystic Fibrosis (CF) patients and parents of young CF patients on organoid technology
Authors Sarah N Boers, Karin M. de Winter- de Groot, Jacquelien Noordhoek, Vincent Gulmans, Cornelis K van der Ent, Johannes JM van Delden, and Annelien L Bredenoord
References J Cyst Fibros. 2018 Mar 6. pii: S1569-1993(18)30027-4. doi: 10.1016/j.jcf.2018.02.004
Keywords Biobanks, Ethics, Organoid technology, Participatory medicine, Qualitative interview study
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: The expression of Mirc1/Mir17-92 cluster in sputum samples correlates with pulmonary exacerbations in cystic fibrosis patients
Authors Kathrin Krause, Benjamin T. Kopp, Mia F. Tazi, Kyle Caution, Kaitlin Hamilton, Asmaa Badr, Chandra Shrestha, Dmitry Tumin, Don Hayes Jr., Frank Robledo, Luanne Hall-Stoodley, Brett G. Klamer, Xiaoli Zhang, Santiago Partida-Sanchez, Narasimham L. Parinandi
References J Cyst Fibros. 2017 Dec 11. pii: S1569-1993(17)30962-1. doi: 10.1016/j.jcf.2017.11.005
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
