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
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: 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: 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
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: 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: 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
September 2017
Title: Blood flow regulation and oxidative stress during submaximal cycling exercise in patients with cystic fibrosis.
Authors Tucker MA, Berry B, Seigler N, Davison GW, Quindry JC, Eidson D, McKie KT, Harris RA.
References J Cyst Fibros. 2017 Sep 8. pii: S1569-1993(17)30878-0. doi: 10.1016/j.jcf.2017.08.015.
Keywords Electron paramagnetic resonance spectroscopy, Exercise intolerance, Reactive oxygen species, Retrograde velocity
September 2017
Title: Real life practice of sweat testing in Europe.
Authors Cirilli N, Southern KW, Buzzetti R, Barben J, Nährlich L, Munck A, Wilschanski M, De Boeck K, Derichs N, of the ECFS Diagnostic Network Working Group.
References J Cyst Fibros. 2017 Sep 27. pii: S1569-1993(17)30881-0. doi: 10.1016/j.jcf.2017.09.002.
September 2017
Title: End-of-life practice patterns at U.S. adult cystic fibrosis care centers: A national retrospective chart review.
Authors Chen E, Homa K, Goggin J, Sabadosa KA, Hempstead S, Marshall BC, Faro A, Dellon EP.
References J Cyst Fibros. 2017 Sep 13. pii: S1569-1993(17)30873-1. doi: 10.1016/j.jcf.2017.08.010
Keywords Advance care planning, End-of-life care, Hospice, Lung transplant, Palliative care
August 2017
Title: Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence.
Authors Anna M. Georgiopoulos, Deborah Friedman, Elizabeth A. Porter, Amy Krasner Sheetal P. Kakarala, Breanna K. Glaeser, Siena C.Napoleon, Janet Wozniak
References J Cyst Fibros. 2017 Aug 31. pii: S1569-1993(17)30874-3. doi: 10.1016/j.jcf.2017.08.011.
Keywords Adherence, Attention-deficit hyperactivity disorder, health-related quality of life
August 2017
Title: Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis
Authors Traci M. Kazmerski, Gregory S. Sawicki, Elizabeth Miller, Kelley A. Jones, Kaleab Z. Abebe, Lisa K. Tuchman, Sigrid Ladores, Ronald C. Rubenstein, Scott D. Sagel, Daniel J. Weine3, Joseph M. Pilewski, David M. Orenstein and Sonya Borrero
References J Cyst Fibros. 2017 Aug 30. pii: S1569-1993(17)30872-X. doi: 10.1016/j.jcf.2017.08.009
Keywords Sexual and reproductive health, Women's health
August 2017
Title: 16S rRNA gene sequencing reveals site-specific signatures of the upper and lower airways of cystic fibrosis patients.
Authors Lucas SK, Yang R, Dunitz JM, Boyer HC, Hunter RC
References J Cyst Fibros. 2017 Aug 18. pii: S1569-1993(17)30865-2. doi: 10.1016/j.jcf.2017.08.007.
Keywords Chronic sinusitis, Microbial ecology, Microbiome, Mucus
August 2017
Title: Variability of Lung Clearance Index in clinically stable Cystic Fibrosis Lung Disease in School Age Children
Authors Marcus Svedberg, Per M. Gustafsson, Paul D. Robinson, Monica Rosberg, Anders Lindblad
References J Cyst Fibros. 2017 Aug 16. pii: S1569-1993(17)30839-1. doi: 10.1016/j.jcf.2017.08.004
Keywords Lung clearance index, Multiple breath washout, Pulmonary exacerbation
July 2017
Title: Poor recovery from a pulmonary exacerbation does not lead to accelerated FEV1 decline
Authors Don B. Sanders, Zhanhai Li,c, Qianqian Zhao, Philip Farrell
References J Cyst Fibros. 2017 Jul 29. pii: S1569-1993(17)30818-4. doi: 10.1016/j.jcf.2017.07.003
Keywords Epidemiology, FEV1, Pulmonary exacerbation
July 2017
Title: Tracheal diverticula in advanced cystic fibrosis: Prevalence, features, and outcomes after lung transplantation.
Authors Siddhartha G. Kapnadak, Gregory A. Kicska, Kathleen A. Ramos, Desiree A. Marshall, Tamara Y. Carroll, Sudhakar N. Pipavath, Michael S. Mulligan, Christopher H. Goss, Moira L. Aitken
References J Cyst Fibros. 2017 Jul 27. pii: S1569-1993(17)30830-5. doi: 10.1016/j.jcf.2017.07.015.
Keywords Lung diseases, Lung transplantation, Pseudomonas infections, Tomography, x-ray computed, Tracheal diverticulum
July 2017
Title: Age-related levels of fecal M2-pyruvate kinase in children with cystic fibrosis and healthy children 0 to 10years old.
Authors Garg M, Leach ST, Pang T, Needham B, Coffey MJ, Katz T, Strachan R, Widger J, Field P, Belessis Y, Chuang S, Day AS, Jaffe A, Ooi CY.
References J Cyst Fibros. 2017 Jul 25. pii: S1569-1993(17)30826-3. doi: 10.1016/j.jcf.2017.07.011.
Keywords Biomarkers, Gastrointestinal inflammation, Intestinal neoplasms, Pediatrics, Pyruvate kinase
July 2017
Title: Increased Prevalence of Colonic Adenomas in Patients with Cystic Fibrosis
Authors Hegagi M, Aaron SD, James P, Goel R, Chatterjee A
References J Cyst Fibros. 2017 Jul 22. pii: S1569-1993(17)30824-X. doi: 10.1016/j.jcf.2017.07.009
Keywords adenoma, colonoscopy
July 2017
Title: Bile salt stimulated lipase, Cystic fibrosis, Enzyme therapy, NMR spectroscopy, Pancreatic insufficiency, Titrimetric enzyme assay
Authors Elena Venuti, Dmitry Shishmarev, Philip W. Kuchel, Shoma Dutt, Caron S. Blumenthal, and Kevin J. Gaskin1
References J Cyst Fibros. 2017 Jul 21. pii: S1569-1993(17)30820-2. doi: 10.1016/j.jcf.2017.07.005
Keywords Bile salt stimulated lipase, Enzyme therapy, NMR spectroscopy, pancreatic insufficiency, Titrimetric enzyme assay
Title: Analytical and Biological Variation in Repeated Sweat Chloride Concentrations in Clinical Trials for CFTR Modulator Therapy
Authors VA LeGrys, TC Moon, J Laux, MJ Rock, F Accurso
References J Cyst Fibros. 2017 Jul 21. pii: S1569-1993(17)30823-8. doi: 10.1016/j.jcf.2017.07.008
Keywords Analytical variation, Biological variation, Index of individuality, Reference change value, sweat chloride
July 2017
Title: Differences between WHO AND CDC early growth measurements in the assessment of Cystic Fibrosis clinical outcomes.
Authors Usatin D, Yen EH, McDonald C, Asfour F, Pohl J, Robson J
References J Cyst Fibros. 2017 Jul;16(4):503-509. doi: 10.1016/j.jcf.2016.12.001
Keywords Forced Expiratory Volume in 1second percent predicted (FEV1(pp)), Growth standards, Survival, Weight-for-length (WFL)
Title: Outcomes associated with antibiotic regimens for treatment of Mycobacterium abscessus in cystic fibrosis patients.
Authors DaCosta A, Jordan CL, Giddings O, Lin FC, Gilligan P, Esther CR Jr
References J Cyst Fibros. 2017 Jul;16(4):483-487. doi: 10.1016/j.jcf.2017.04.013. Epub 2017 May 8
Keywords Mycobacterium abscessus, Nontuberculous mycobacterium
Title: Macronutrient Intake in Preschoolers with Cystic Fibrosis and the Relationship between Macronutrients and Growth
Authors Stephanie S. Filigno, Shannon M. Robson, Rhonda D. Szczesniak, Leigh A. Chamberlin, Meredith A. Baker, Stephanie M. Sullivan, John Kroner, Scott W. Powers
References J Cyst Fibros. 2017 Jul;16(4):519-524. doi: 10.1016/j.jcf.2017.01.010. Epub 2017 Feb 6.
Keywords Behavioral intervention, Children, Nutrition
Title: Worsening anxiety and depression after initiation of lumacaftor/ivacaftor combination therapy in adolescent females with cystic fibrosis
Authors Cameron J. McKinzie, Jennifer L. Goralski, Terry L. Noah, George Z. Retsch-Bogart, Mary Beth Prieur
References J Cyst Fibros. 2017 Jul;16(4):525-527. doi: 10.1016/j.jcf.2017.05.008. Epub 2017 Jun 8.
July 2017
Title: Lower exhaled nitric oxide in infants with Cystic Fibrosis compared to healthy controls
Authors Insa Korten, Margot Liechti, Florian Singer, Gaudenz Hafen, Isabelle Rochat, Pinelopi Anagnostopoulou, Dominik Müller-Suter, Jakob Usemann, Alexander Moeller, Urs Frey, Philipp Latzin, Carmen Casaulta for the SCILD7 and BILD study group
July 2017
Title: Detection of CFTR Function and Modulation in Primary Human Nasal Cell Spheroids
Authors John J. Brewington, Erin T. Filbrandt, F. J. LaRosa III, Alicia J. Ostmann, Lauren M. Strecker, Rhonda D. Szczesniak, John P. Clancy
References J Cyst Fibros. 2017 Jul 13. pii: S1569-1993(17)30812-3. doi: 10.1016/j.jcf.2017.06.010.
Keywords CFTR, Modulator, Organoid, Personalized model system
July 2017
Title: Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2 years of treatment with ivacaftor in a real-world setting
Authors Hubert D, Dehillotte C, Munck A, David V, Baek J, Mely L, Dominique S, Ramel S, Danner Boucher I, Lefeuvre S, Reynaud Q, Colomb-Jung V, Bakouboula P, Lemonnier L
References J Cyst Fibros. 2017 Jul 12. pii: S1569-1993(17)30816-0. doi: 10.1016/j.jcf.2017.07.001
Keywords CFTR modulators, Lumacaftor
June 2017
Title: Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor.
Authors Flume PA, Wainwright CE, Tullis ED, Rodriguez S, Niknian M, Higgins M, Davies JC, Wagener JS
References J Cyst Fibros. 2017 Jun 24. pii: S1569-1993(17)30770-1. doi: 10.1016/j.jcf.2017.06.002
Keywords Cystic fibrosis transmembrane conductance regulator, Ivacaftor, Pulmonary exacerbations, Pulmonary function
June 2017
Title: A single bout of maximal exercise improves lung function in patients with cystic fibrosis.
Authors Matthew A. Tucker, Reva Crandall1, Nicole Seigler, Paula Rodriguez-Miguelez, Kathleen T. McKie, Caralee Forseen, Jeffrey Thomas, & Ryan A. Harris
Keywords Exercise capacity, Impulse oscillometry, Multi-breath washout; Spirometry
Theme Physiotherapy/Exercise testing
June 2017
Title: The use of fructosamine in cystic fibrosis-related diabetes (CFRD) screening.
Authors Lam GY, Doll-Shankaruk M, Dayton J, Rodriguez-Capote K, Higgins TN, Thomas D, Mulchey K, Smith MP, Brown NE, Leung WM, Estey MP
References J Cyst Fibros. 2017 Jun 22. pii: S1569-1993(17)30767-1. doi: 10.1016/j.jcf.2017.05.010
Keywords Cystic fibrosis-related diabetes, FEV(1), Oral glucose tolerance test, screening, Serum fructosamine
June 2017
Title: Pulmonary surfactant dysfunction in pediatric cystic fibrosis: Mechanisms and reversal with a lipid-sequestering drug.
Authors Gunasekara L, Al-Saiedy M, Green F, Pratt R, Bjornson C, Yang A, Michael Schoel W, Mitchell I, Brindle M, Montgomery M, Keys E, Dennis J, Shrestha G, Amrein M.
References J Cyst Fibros. 2017 Jun 7. pii: S1569-1993(17)30151-0.
Keywords Cholesterol, oxidative stress, Free fatty acids, Genotypes, Infection, Inflammation, Lung surfactant, Phospholipids, Surface tension
June 2017
Title: Complications of long and intermediate term venous catheters in cystic fibrosis patients: A multicenter study.
Authors May TL, Gifford AH, Lahiri T, Black A, Trang J, Cornell AG, Gonzalez K, Morin S, Napier M, Duarte CW, Zuckerman JB
References J Cyst Fibros. 2017 Jun 1. pii: S1569-1993(17)30150-9. doi: 10.1016/j.jcf.2017.04.014
Keywords CFF Patient Registry, Central line, Infectious complications, Pulmonary exacerbation
May 2017
Title: Development and Validation of CF-Medication Beliefs Questionnaire: A mixed-methods approach
Authors Michelle N. Eakin, Shang-En Chung, Jessica Hoehn, Belinda Borrelli, Devin Rand-Giovannetti, and Kristin A. Riekert
References J Cyst Fibros. 2017 May 23. pii: S1569-1993(17)30152-2
May 2017
Title: Inhaled ENaC Antisense Oligonucleotide Ameliorates Cystic Fibrosis-Like Lung Disease in Mice
Authors Jeff R. Crosby, Chenguang Zhao, Chong Jiang b Dong Bai, Melanie Katz, Sarah Greenlee, Hiroshi Kawabe , Michael McCaleb, Daniela Rotin, Shuling Guo, Brett P. Monia
References J Cyst Fibros. 2017 May 20. pii: S1569-1993(17)30154-6. doi: 10.1016/j.jcf.2017.05.003.
Keywords Aerosol, Antisense oligonucleotide, ENaC
May 2017
Title: Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis
Authors Aurélie Labaste, Camille Olhmann, Catherine Mainguy, Virginie Jubin, Marie Perceval, Laurianne Coutier, Philippe Reix
References J Cyst Fibros. 2017 May 18. pii: S1569-1993(17)30153-4. doi: 10.1016/j.jcf.2017.05.002.
Keywords Acute, Airway obstruction, Bronchoconstriction, Lumacaftor, Modulators, Pediatric
May 2017
Title: AP2 α modulates cystic fibrosis transmembrane conductance regulator function in the human intestine
Authors Kumari V, Desai S, Ameen NA.
References J Cyst Fibros. 2017 May;16(3):327-334.
Keywords AP2α, CFTR, Clathrin
May 2017
Title: Dietary intake and lipid profile in children and adolescents with cystic fibrosis.
Authors Woestenenk JW, Schulkes DA, Schipper HS, van der Ent CK, Houwen RHJ.
References J Cyst Fibros. 2017 May;16(3):410-417. doi: 10.1016/j.jcf.2017.02.010.
Keywords Cholesterol, Dietary intake, Fat intake, Lipid profile, Triglyceride
Title: Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR
Authors Scott H. Donaldson, George M. Solomon, Pamela L. Zeitlin, Patrick A. Flume, Alicia Casey, Karen McCoy, Edith T. Zemanick, Arun Mandagere, Janice M. Troha, Steven A. Shoemaker, James F. Chmiel, Jennifer L. Taylor-Cousa
References J Cyst Fibros. 2017 May;16(3):371-379. doi: 10.1016/j.jcf.2017.01.009. Epub 2017 Feb 13.
Keywords CFTR stabilizer, GSNOR, F508del-CFTR, Homozygous, Modulator
Title: Effectiveness of a stepwise Pseudomonas aeruginosa eradication protocol in children with cystic fibrosis
Authors Ana C. Blanchard, Eric Horton, Sanja Stanojevic, Louise Taylor, Valerie Waters, Felix Ratjen
References J Cyst Fibros. 2017 May;16(3):395-400. doi: 10.1016/j.jcf.2017.01.007. Epub 2017 Feb 9.
Keywords Antibiotic eradication therapy, Eradication failure, Pseudomonas aeruginosa infection
Title: Use of FEV1 in Cystic Fibrosis Epidemiologic Studies and Clinical Trials: A Statistical Perspective for the Clinical Researcher
Authors Rhonda Szczesniak, Sonya L. Heltshe, Sanja Stanojevic, Nicole Mayer-Hamblett
References J Cyst Fibros. 2017 May;16(3):318-326. doi: 10.1016/j.jcf.2017.01.002. Epub 2017 Jan 20.
Keywords Disease progression, FEV(1) endpoints, Longitudinal, Lung function, Spirometry
Title: Inhaled dry powder mannitol in children with cystic fibrosis: A randomised efficacy and safety trial.
Authors De Boeck K, Haarman E, Hull J, Lands LC, Moeller A, Munck A, Riethmüller J, Tiddens H, Volpi S, Leadbetter J, Charlton B, Malfroot A; DPM-CF-204 Study Group.
References J Cyst Fibros. 2017 May;16(3):380-387.
Keywords FEV1, Mannitol, Paediatric, Randomised
Title: Modelling Cystic Fibrosis Disease Progression in Patients with the Rare CFTR Mutation P67L
Authors Isobel E.R. MacKenzie, Valerie Paquette, Frances Gosse, Sheenagh George, Frederic Chappe and Valerie Chappe
References J Cyst Fibros. 2017 May;16(3):335-341.
Keywords Data Registry, Disease progression, Mild CF, P67L, Rare mutations
May 2017
Title: What Keeps Children with Cystic Fibrosis awake at night?
Authors Vandeleur M, Walter LM, Armstrong DS, Robinson P, Nixon GM, Horne RSC.
References J Cyst Fibros. 2017 May 4. pii: S1569-1993(17)30142-X.
Keywords Actigraphy, Oximetry, Sleep, Sleep hygiene
May 2017
Title: Is there an association between back pain and stress incontinence in adults with cystic fibrosis? A retrospective cross-sectional study.
Authors Ashbrook JE, Shacklady C, Johnson S, Yeowell G, Goodwin PC.
References J Cyst Fibros. 2017 May 2. pii: S1569-1993(17)30102-9
Keywords Back pain, Cross sectional study, Lung function, Stress urinary incontinence
April 2017
Title: A Multiple Reader Scoring System for Nasal Potential Difference Parameters
Authors George M. Solomon, Bo Liu, Isabelle Sermet-Gaudelus, Isabelle Fajac, Michael Wilschanski, Francois Vermeulen, Steven M. Rowe
References J Cyst Fibros. 2017 Apr 29. pii: S1569-1993(17)30141-8
Keywords CFTR, Clinical trial outcomes, Nasal potential difference
