July 2019
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: 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.
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: 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
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 Elbornb, Michae
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: 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; and Michael D. Par
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 J Cyst Fibros. 2019 Feb 27. pii: S1569-1993(18)30804-X. doi: 10.1016/j.jcf.2019.02.004.
References Deschamp AR, Hatch JE, Slaven JE, Gebregziabher N, Storch G, Hall GL, Stick S, Ranganathan S, Ferkol TW, Davis SD.
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: 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
November 2018
Title: Urinary Metabolomics Reveals Unique Metabolic Signatures in Infants with Cystic Fibrosis
Authors Kopp BT, Joseloff E, Goetz D, Ingram B, Heltshe SL, Leung DH, Ramsey BW, McCoy K, Borowitz D
References J Cyst Fibros. 2018 Nov 23. pii: S1569-1993(18)30916-0. doi: 10.1016/j.jcf.2018.10.016
Keywords Biomarkers, Metabolites
November 2018
Title: Cystic fibrosis is associated with an increased risk of Barrett’s esophagus
Authors Rita M. Knotts, Quinn S. Solfisburg, Claire Keating, Emily DiMango, Charles J. Lightdale, Julian A. Abrams
References J Cyst Fibros. 2018 Nov 22. pii: S1569-1993(18)30933-0. doi: 10.1016/j.jcf.2018.11.005
Keywords Barrett's esophagus, Esophageal adenocarcinoma (EAC), Gastroesophageal reflux disease (GERD), Malignancy
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: Clinical outcomes in U.S. infants with cystic fibrosis from 2001 to 2012
Authors Hoch H, Sontag MK, Scarbro S, Juarez-Colunga E, McLean C, Kempe A, Sagel SD
References Pediatr Pulmonol. 2018 Nov;53(11):1492-1497. doi: 10.1002/ppul.2416
Keywords Pseudomonas aeruginosa, Staphylococcus aureus, malnutrition, Newborn screening
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: Antibody response against Mycobacterium avium complex in cystic fibrosis patients measured by a novel IgG ELISA test
Authors Ravnholt C, Qvist T, Kolpen M, Pressler T, Skov M, Høiby N
References J Cyst Fibros. 2018 Nov 29. pii: S1569-1993(18)30938-X. doi: 10.1016/j.jcf.2018.11.011. [
Keywords Accuracy, diagnostic, Mycobacterium avium, Nontuberculous, Serology
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: SWATH label-free proteomics for cystic fibrosis research
Authors Braccia C, Tomati V, Caci E, Pedemonte N, Armirotti A
References J Cyst Fibros. 2018 Oct 19. pii: S1569-1993(18)30855-5. doi: 10.1016/j.jcf.2018.10.004.
Keywords Bronchial epithelial cells, Proteomics
Title: SWATH label-free proteomics for cystic fibrosis research
Authors Braccia C, Tomati V, Caci E, Pedemonte N, Armirotti A
References J Cyst Fibros. 2018 Oct 19. pii: S1569-1993(18)30855-5. doi: 10.1016/j.jcf.2018.10.004.
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: Disparities in Mortality of Hispanic Patients with Cystic Fibrosis in the United States. A National and Regional Cohort Study
Authors Rho J, Ahn C, Gao A, Sawicki GS, Keller A, Jain R.
References Am J Respir Crit Care Med. 2018 Oct 15;198(8):1055-1063. doi: 10.1164/rccm.201711-2357OC.
Keywords Hispanic, disparity, mortality, regional
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: Likelihood-based analysis of outcome-dependent sampling designs with longitudinal data.
Authors Zelnick LR, Schildcrout JS, Heagerty PJ
References Stat Med. 2018 Jun 15;37(13):2120-2133. doi: 10.1002/sim.7633.
Keywords biased sampling, epidemiological study design, longitudinal data analysis
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
