June 2016
Title: Multiple Prevalent Fractures in Relation to Macroscopic Bone Architecture in Patients with Cystic Fibrosis
Authors Mirjam Stahl, Christian Holfelder, Carolin Kneppo, Meinhard Kieser, Christian Kasperk, Eckhard Schoenau, Olaf Sommerburg, Burkhard Tönshoff
References J Cyst Fibros. 2016 Jun 17. pii: S1569-1993(16)30547-1
Keywords Bone densitometry, Bone disease, Cystic fibrosis, Fracture rate, Macroscopic bone architecture, Noninvasive monitoring
June 2016
Title: Airway inflammation in mild cystic fibrosis.
Authors Jonas Eckrich, Ulrich M. Zissler, Friederike Serve, Patricia Leutz, Christina Smaczny, Sabina Schmitt-Grohé, Daniela Fussbroich, Ralf Schubert, Stefan Zielen, Olaf Eickmeier
References J Cyst Fibros. 2016 Jun 10. pii: S1569-1993(16)30523-9
Keywords Airway inflammation, Gene expression, Induced sputum, Pro-inflammatory biomarkers, Small airway disease
Title: Respiratory Pathogens Mediate the Association between Lung Function and Temperature in Cystic Fibrosis
Authors Joseph M. Collaco, Karen S. Raraigh, Lawrence J. Appel, Garry R. Cutting
References J Cyst Fibros. 2016 Jun 10. pii: S1569-1993(16)30514-8.
Keywords Climate, FEV1, Lung function, MRSA, Pseudomonas aeruginosa, Temperature
June 2016
Title: Bioelectrical impedance in young patients with cystic fibrosis: validation of a specific equation and clinical relevance
Authors Charatsi AM, Dusser P, Freund R, Maruani G, Rossin H, Boulier A, Le Bourgeois M, Chedevergne F, de Blic J, Letourneur A, Casimir G, Jais JP, Sermet-Gaudelus, Charatsi AM, Dusser P
References J Cyst Fibros. 2016 Jun 8. pii: S1569-1993(16)30053-4
Keywords Bioelectrical impedance analysis, Body composition, Diaphragm, Fat-free mass, Lung function
June 2016
Title: Pancreatic Elastase-1 Quick Test for rapid assessment of pancreatic status in cystic fibrosis patients.
Authors Walkowiak J, Glapa A, Nowak JK, Bober L, Rohovyk N, Wenska-Chyży E, Sobkowiak P, Lisowska A
References J Cyst Fibros. 2016 Jun 7. pii: S1569-1993(16)30511-2.
Keywords Exocrine pancreatic insufficiency, Point-of-care, Qualitative test, Sensitivity, Specificity
June 2016
Title: Sino-nasal Inhalation of Isotonic versus Hypertonic Saline (6.0 %) in CF Patients with Chronic Rhinosinusitis – Results of a Multicentre, Prospective, Randomized, Double-blind, Controlled Trial
Authors Jochen G Mainz, Ulrike Schumacher, Katja Schädlich, Julia Hentschel, Christiane Koitschev, Assen Koitschev, Joachim Riethmüller, Freerk Prenzel, Olaf Sommerburg, Bärbel Wiedemann, Doris Staab, Wolfgang Gleiber, Rainald Fischer, James F Beck , Christin A
References J Cyst Fibros. 2016 Jun 4. pii: S1569-1993(16)30052-2
June 2016
Title: The Dynamics of Disease Progression in Cystic Fibrosis
Authors Adler FR, Liou TG
References PLoS One. 2016 Jun 1;11(6):e0156752. doi: 10.1371/journal.pone.0156752. eCollection 2016
Title: Cystic fibrosis in Latin America - Improving the awareness
Authors Luiz Vicente Ribeiro F. Silva Filho, Claudio Castaños, Héctor Hernán Ruíz
References J Cyst Fibros. 2016 Jun 1. pii: S1569-1993(16)30509-4.
Keywords Awareness, Diagnosis, Latin America, Management, Progress
May 2016
Title: Episodic oral antibiotic use in CF: discordance between the electronic medical record and self-report
Authors Lindsay J. Caverly, Tanner J. Caverly, Linda M. Kalikin, Bridget K. Foster, Richard H. Simon, John J. LiPuma
References J Cyst Fibros. 2016 May 4. pii: S1569-1993(16)30044-3
Keywords Bioelectrical impedance analysis, Body composition, Diaphragm, Fat-free mass, Lung function
Theme Epidemiology/Models of care
May 2016
Title: A false positive newborn screening result due to a complex allele carrying two frequent CF-causing variants.
Authors Bergougnoux A, Boureau-Wirth A, Rouzier C, Altieri JP, Verneau F, Larrieu L, Koenig M, Claustres M, Raynal C
References J Cyst Fibros. 2016 May;15(3):309-12
Keywords CFTR gene, Complex allele, False positive, Newborn screening
Title: A false positive newborn screening result due to a complex allele carrying two frequent CF-causing variants.
Authors Bergougnoux A, Boureau-Wirth A, Rouzier C, Altieri JP, Verneau F, Larrieu L, Koenig M, Claustres M, Raynal C
References J Cyst Fibros. 2016 May;15(3):309-12
Keywords CFTR gene, Complex allele, False positive, Newborn screening
April 2016
Title: Improving performance in the detection and management of cystic fibrosis-related diabetes in the Mountain West Cystic Fibrosis Consortium.
Authors Liou TG, Jensen JL, Allen SE, Brayshaw SJ, Brown MA, Chatfield B, Koenig J, McDonald C, Packer KA, Peet K, Radford P, Reineke LM, Otsuka K, Wagener JS, Young D, Marshall BC
References BMJ Open Diabetes Res Care. 2016 Apr 29;4(1):e000183. doi: 10.1136/bmjdrc-2015-000183.
Keywords Biomarker, Inflammation, Plasma, Pulmonary exacerbation
April 2016
Title: Improving performance in the detection and management of cystic fibrosis-related diabetes in the Mountain West Cystic Fibrosis Consortium
Authors Liou TG, Jensen JL, Allen SE, Brayshaw SJ, Brown MA, Chatfield B, Koenig J, McDonald C, Packer KA, Peet K, Radford P, Reineke LM, Otsuka K, Wagener JS, Young D, Marshall BC
References BMJ Open Diabetes Res Care. 2016 Apr 29;4(1):e000183. doi: 10.1136/bmjdrc-2015-000183. eCollection 2016.
April 2016
Title: Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene
Authors Dupuis A, Keenan K, Ooi CY, Dorfman R, Sontag MK, Naehrlich L, Castellani C, Strug, LJ Rommens JM, Gonska T
References Genet Med. 2016 Apr;18(4):333-40. doi: 10.1038/gim.2015.79
April 2016
Title: Clinical Practice Guidelines from the Cystic Fibrosis Foundation for Preschoolers with Cystic Fibrosis
References Pediatrics. 2016 April; 137(4), DOI:10.1542/peds.2015-1784
Keywords clinical practice, guidelines, Cystic Fibrosis Foundation, CFF, preschooler, infant
Theme Care of patients with CF, Clinical Trials, Pregnancy with CF
Title: Controversies with Kalydeco: Newspaper Coverage in Canada and the Unites States of the Cystic Fibrosis “Wonder Drug”
Authors Christen Rachul, Maeghan Toews, Timothy Caulfield
References J Cyst Fibros. 2016 Apr 14. pii: S1569-1993(16)30015-7
Theme Epidemiology/Models of care
Title: Optimizing beta-lactam pharmacodynamics against Pseudomonas aeruginosa in adult cystic fibrosis patients.
Authors Thompson RZ, Martin CA, Burgess DR, Rutter WC, Burgess DS
References J Cyst Fibros. 2016 Apr 27. pii: S1569-1993(16)30019-4.
Keywords Beta-lactam, Continuous-infusion, Pharmacodynamics, Pharmacokinetics, Prolonged infusion, Pseudomonas aeruginosa
March 2016
Title: The Geographic Impact on Hospitalization in Patients with Cystic Fibrosis.
Authors Kopp BT, Nicholson L, Paul G, Tobias J, Ramanathan C, Hayes D Jr
References J Pediatr. 2016 Mar;170:246-52.e1-4. doi: 10.1016/j.jpeds.2015.11.012. Epub 2015 Dec 13.
March 2016
Title: Short-term and long-term response to pulmonary exacerbation treatment in cystic fibrosis.
Authors Heltshe SL, Goss CH, Thompson V, Sagel SD, Sanders DB, Marshall BC, Flume PA
References Thorax. 2016 Mar;71(3):223-9. doi: 10.1136/thoraxjnl-2014-206750. Epub 2015 Apr 24.
March 2016
Title: Comparison of two pancreatic enzyme products for exocrine insufficiency in patients with cystic fibrosis
Authors C.J. Taylor, R. Thieroff-Ekerdt, S. Shiff, L. Magnus, R. Fleming, C. Gommoll
References J Cyst Fibros. 2016 Mar 21. pii: S1569-1993(16)30001-7
Keywords Clinical trial, Enzepi, Exocrine pancreatic insufficiency (EPI), Pancreatic enzyme product (PEP), Zenpep
Theme Nutrition/Gastroenterology
Title: Extensive cultivation of soil and water samples yields various pathogens in patients with cystic fibrosis but not Burkholderia multivorans.
Authors Charlotte Peeters, Eliza Depoorter, Jessy Praet, Peter Vandamme
References J Cyst Fibros. 2016 Mar 17. pii: S1569-1993(16)30005-4
Keywords Burkholderia multivorans, Gram-negative non-fermenters, Isolation, Rapidly growing mycobacteria, Soil, Water
Title: Disease disclosure in individuals with cystic fibrosis: Association with psychosocial and health outcomes
Authors Adrienne P. Borschuk, Robin S. Everhart, Michelle N. Eakin, Devin Rand-Giovannetti, Belinda Borrelli, Kristin A. Riekert
References J Cyst Fibros. 2016 Mar 17. pii: S1569-1993(16)30002-9
Keywords Disclosure, Medication adherence, Self-efficacy, Social support
Title: Evaluation of a systems biology approach to identify pharmacological correctors of the mutant CFTR chloride channel
Authors Pesce E, Gorrieri G, Sirci F, Napolitano F, Carrella D, Caci E, Tomati V, Zegarra-Moran O, di Bernardo D, Galietta LJ
References J Cyst Fibros. 2016 Mar 10. pii: S1569-1993(16)00057-6
Keywords CFTR, Chloride channel, Corrector, F508del, Systems biology
February 2016
Title: Epidemiology of the Respiratory Bacteriology of Patients with Cystic Fibrosis
Authors Salsgiver EL, Fink AK, Knapp EA, LiPuma JJ, Olivier KN, Marshall BC, Saiman L. Changing
References Chest. 2016 Feb;149(2):390-400. doi: 10.1378/chest.15-0676.
Keywords Pseudomonas aeruginosa, Staphylococcus aureus, registry
Title: Assessing Differences in Mortality Rates and Risk Factors Between Hispanic and Non-Hispanic Patients With Cystic Fibrosis in California.
Authors Buu MC, Sanders LM, Mayo JA, Milla CE, Wise PH
References Chest. 2016 Feb;149(2):380-389. doi: 10.1378/chest.14-2189. Epub 2016 Jan 12.
Keywords ethnicity, health disparities, pediatric pulmonology
February 2016
Title: Clinical outcomes in cystic fibrosis patients with Trichosporon respiratory infection
Authors Charles R. Esther, Rongpong Plongla, Alan Kerr, Feng-Chang Lin, Peter Gilligan
References J Cyst Fibros. 2016 Feb 27. pii: S1569-1993(16)00037-0
Keywords Chryseobacterium, Lung function
Title: Safety and Efficacy of Prolonged Levofloxacin Inhalation Solution (1 APT-1026) Treatment for Cystic Fibrosis and Chronic Pseudomonas aeruginosa Airway Infection
Authors J. Stuart Elborn, Patrick A. Flume, Fredric Cohen, Jeffery Loutit, Donald R. VanDevanter
References J Cyst Fibros. 2016 Feb 26. pii: S1569-1993(16)00012-6
Title: The role of basophil activation test in allergic bronchopulmonary aspergillosis and Aspergillus fumigatus sensitization in cystic fibrosis patients
Authors Anna Katelari, Marianna Tzanoudaki, Maria Noni, Maria Kanariou, Maria Theodoridou, Emmanuel Kanavakis, Stavros-Eleftherios Doudounakis, Christina Kanaka-Gantenbein
References J Cyst Fibros. 2016 Feb 25. pii: S1569-1993(16)00016-3
Keywords Allergic bronchopulmonary aspergillosis, Aspergillus fumigatus, Aspergillus fumigatus sensitization, Basophil activation test
Theme Epidemiology/Models of care
Title: Glycated hemoglobin cannot yet be proposed as a screening tool for cystic fibrosis related diabetes
Authors Boudreau V, Coriati A, Desjardins K, Rabasa-Lhoret R
References J Cyst Fibros. 2016 Feb 19. pii: S1569-1993(16)00017-5
Keywords Adult, CFRD, Glycated hemoglobin, Oral glucose tolerance test
Theme Diagnosis/Screening
Title: International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: associated factors and outcome.
Authors Anne Munck, Corinne Alberti, Carla Colombo, Nataliya Kashirskaya, Helmut Ellemunter, Maria Fotoulaki, Roderick Houwen, Eddy Robberecht, Priscilla Boizeau, Michael Wilschanski on behalf of the CF/Pancreas ESPGHAN Working Group and DIOS Study Group
References J Cyst Fibros. 2016 Feb 23. pii: S1569-1993(16)00014-X.
Keywords Abdominal pain, Distal intestinal obstruction syndrome, Incidence
Theme Nutrition/Gastroenterology
Title: A Phase 3, Multi-center, Multinational, Randomized, Double-blind, Placebo-controlled Study to Evaluate the Efficacy and Safety of Levofloxacin Inhalation Solution (APT-1026) in Stable Cystic Fibrosis Patients
Authors Patrick A. Flume, Donald R. VanDevanter, Elizabeth E. Morgan, Michael N. Dudley, Jeffery S. Loutit, Scott C. Bell, Eitan Kerem, Rainald Fischer, Alan R. Smyth, Shawn D. Aaron, Douglas Conrad, David E. Geller, J. Stuart Elborn on behalf of the APT investigators
References J Cyst Fibros. 2016 Feb 4. pii: S1569-1993(15)00276-3.
Keywords Aerosol, Antibiotics, Fluoroquinolone, Pseudomonas
January 2016
Title: Measurement of fecal elastase improves performance of newborn screening for cystic fibrosis.
Authors Barben J, Rueegg CS, Jurca M, Spalinger J, Kuehni CE, Swiss Cystic Fibrosis Screening Group
References J Cyst Fibros. 2016 Jan 27. pii: S1569-1993(16)00002-3
Keywords CFSPID, Fecal elastase, Newborn screening
January 2016
Title: Microbiological yield from induced sputum compared to oropharyngeal swab in young children with cystic fibrosis
Authors Marco Zampoli, Komala Pillay, Henri Carrara, Heather J Zar, Brenda Morrow
References J Cyst Fibros. 2016 Jan 26. pii: S1569-1993(16)00008-4.
Keywords Induced sputum, Oropharyngeal swab, Young children
January 2016
Title: Host Response to Staphylococcus aureus Cytotoxins In Children with Cystic
Authors Ashley D. Chadha, Isaac P. Thomsen, Natalia Jimenez-Truque, Nicole R. Soper, Lauren S. Jones, Andrew G. Sokolow, Victor J. Torres, C. Buddy Creech
References J Cyst Fibros. 2016 Jan 25. pii: S1569-1993(15)00318-5
Keywords Exotoxins, LukAB, Serology, Staphylococcus aureus
January 2016
Title: Ultrasound and magnetic resonance imaging assessment of joint disease in symptomatic patients with Cystic Fibrosis Arthropathy
Authors G Fitch, K Williams, JE Freeston, S Dass, A Grainger, R Hodgson, L Horton, P Whitaker, D Peckham
References J Cyst Fibros. 2016 Jan 19. pii: S1569-1993(15)00317-3
Keywords Arthropathy, Joint count, MRI, Ultrasound
January 2016
Title: Implementation of newborn screening for Cystic Fibrosis in Norway. Results from the first three years
Authors Emma Lundman, H. Junita Gaup, Egil Bakkeheim, Edda J. Olafsdottir, Terje Rootwelt, Olav Trond Storrøsten, Rolf D. Pettersen
References J Cyst Fibros. 2016 Jan 12. pii: S1569-1993(15)00312-4
Keywords Newborn screening
January 2016
Title: YKL-40 as marker of severe lung disease in cystic fibrosis patients.
Authors Leonardi S, Parisi GF, Capizzi A, Manti S, Cuppari C, Scuderi MG, Rotolo N, Lanzafame A, Musumeci M, Salpietro C
References J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00315-X.
Keywords Acute pulmonary exacerbation, Lung function decline, Pulmonary pseudomonas infection, YKL-40
Title: Distribution of the species of Achromobacter in a French Cystic Fibrosis centre and Multilocus Sequence Typing analysis reveal the predominance of A. xylosoxidans and clonal relationships between some clinical and environmental isolates
Authors Lucie Amoureux, Julien Bador, Fatma Bounoua Zouak, Angélique Chapuis, Claire deCurraize, Catherine Neuwirth
References J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00281-7
Keywords Achromobacter spp., Cystic fibrosis, Environment, Epidemiology, Genotyping, MLST, nNrdA
Title: Gastric emptying and gastro-oesophageal reflux in children with cystic fibrosis
Authors Hauser B, De Schepper J, Malfroot A, De Wachter E, De Schutter I, Keymolen K, Vandenplas Y
References J Cyst Fibros. 2016 Jan 8. pii: S1569-1993(15)00287-8
Keywords (13)C-gastric emptying breath test, Children, Gastric emptying, Gastro-oesophageal reflux, Multichannel intraluminal impedance-pH monitoring
Theme Nutrition/Gastroenterology
January 2016
Title: Low sodium status in cystic fibrosis-as assessed by calculating fractional Na+ excretion-is associated with decreased growth parameters.
Authors Knepper C, Ellemunter H, Eder J, Niedermayr K, Haerter B, Hofer P, Scholl-Bürgi S, Müller T, Heinz-Erian P
References J Cyst Fibros. 2016 Jan 6. pii: S1569-1993(15)00282-9
Keywords Fractional sodium excretion, Growth retardation, Normonatremic sodium depletion
Theme Nutrition/Gastroenterology
January 2016
Title: International Committee on Mental Health in Cystic Fibrosis: CFF and ECFS consensus statements for screening and treating depression and anxiety
Authors Quittner AL, et al.
References Thorax.2016; 71(1):26-34
Keywords International Committee on Mental Health in Cystic Fibrosis, CFF, ECFS, Cystic Fibrosis Foundation, consensus statement, consensus, screening, depression, treatment, anxiety
Title: Antibiotic treatment for Burkholderia cepacia complex in people with cystic fibrosis experiencing a pulmonary exacerbation
Authors Horsley A, Jones AM, Lord R
References Horsley A, Jones AM, Lord R. Antibiotic treatment for Burkholderia cepacia complex in people with cystic fibrosis experiencing a pulmonary exacerbation. Cochrane Database of Systematic Reviews 2016, Issue 1. Art. No.: CD009529. DOI: 10.1002/14651858.CD009529.pub3
Keywords antibiotic treatment, treatment, antibiotic, Burkholderia cepacia, Burkholderia cepacia complex, Pulmonary exacerbation
Theme Lung Disease and Infection
Title: US CFF and ECFS consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary
References Thorax. 2016; 71(1):88-90
Keywords US CFF, CFF, consensus, recommendation, Management, Non-tuberculous mycobacteria
Theme Lung Disease and Infection
Title: US CFF and ECFS consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis
References Thorax. 2016; 71:i1-i22
Keywords US CFF, CFF, consensus, recommendation, Management, Non-tuberculous mycobacteria
Theme Lung Disease and Infection
December 2015
Title: Preliminary Comparison of Normalized T1 and Non-Contrast Perfusion MRI Assessments of Regional Lung Disease in People with Cystic Fibrosis
Authors Shannon B. Donnola, Elliott C. Dasenbrook, David Weaver, Lan Lu, Karishma Gupta, Anjali Prabhakaran, Xin Yu, James F. Chmiel, Kimberly McBennett, Michael W. Konstan, Mitchell L. Drumm, Chris A. Flask
References J Cyst Fibros. 2015 Dec 21. pii: S1569-1993(15)00267-2
Keywords Imaging, Lung disease, Magnetic Resonance Imaging, Perfusion, Relaxometry
Title: Psychological resilience and intolerance of uncertainty in coping with cystic fibrosis
Authors Horst Mitmansgruber, Ulrike Smrekar, Bianca Rabanser, Thomas Beck, Johannes Eder, Helmut Ellemunter
References J Cyst Fibros. 2015 Dec 10. pii: S1569-1993(15)00269-6
Keywords Intolerance of uncertainty, Psychological well-being, Quality of life, Resilience
Title: Epidemic Pseudomonas aeruginosa Infection in Patients with Cystic Fibrosis is Not a Risk Factor for Poor Clinical Outcomes Following Lung Transplantation
Authors Julia Pritchard, Mitesh V. Thakrar, Ranjani Somayaji, Michael G. Surette, Harvey R. Rabin, Doug Helmersen, Dale Lien, Swathi Purighalla, Barbara Waddell, Michael D. Parkins
References J Cyst Fibros. 2015 Dec 2. pii: S1569-1993(15)00262-3
Keywords Bronchiectasis, Liverpool epidemic strain, Multi-drug resistant, Prairie Epidemic Strain, Transmissible, Transplant
Title: Bronchial epithelial cell lines and primary nasal epithelial cells from cystic fibrosis respond differently to cigarette smoke exposure
Authors Mark Thomas Shaw Williams, Francine de Courcey, David Comer, Joseph S Elborn, Madeleine Ennis
References J Cyst Fibros. 2015 Dec 1. pii: S1569-1993(15)00260-X
Keywords Airway, Bronchial, CSE, Cells, Cytokine, EGFR, Epithelial, F508del, Healthy, IL-8, Immortalised, Inflammation, Nasal, Patients, Primary, R117H, Release
November 2015
Title: Early Life Growth Trajectories in Cystic Fibrosis are Associated with Pulmonary Function at Age 6 Years.
Authors Sanders DB, Fink A, Mayer-Hamblett N, Schechter M, Sawicki GS, Rosenfeld M, Flume PA, Morgan WJ
References Journal of Pediatrics. 2015 Nov;167(5):1081-8.e1. PMID: 26340874
November 2015
Title: Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID): a new designation and management recommendations for infants with an inconclusive diagnosis following newborn screening
References Journal of Cystic Fibrosis. 2015 Nov;14(6):706-713
Keywords cystic fibrosis screen positive inconclusive diagnosis, CFSPID, screen positive, inconclusive diagnosis, designation, Management, recommendation, infant, Newborn screening
Theme Diagnosis/Screening
Title: A Longitudinal Analysis of Chronic MRSA and Pseudomonas aeruginosa Co-infection in Cystic Fibrosis: A Single-Center Study
Authors Maret L. Maliniaka, Arlene A. Stecenkoa, Nael A. McCarty
References J Cyst Fibros. 2015 Nov 20. pii: S1569-1993(15)00258-1
Keywords Methicillin-resistant Staphylococcus aureus, Pseudomonas aeruginosa, Pulmonary function tests, Longitudinal studies, Epidemiology
October 2015
Title: Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis
Authors Leung DH, Ye W, Molleston JP, Weymann A, Ling S, Paranjape SM, Romero R, Schwarzenberg SJ, Palermo J, Alonso EM, Murray KF, Marshall BC, Sherker AH, Siegel MJ, Krishnamurthy R, Harned R, Karmazyn B, Magee JC, Narkewicz MR; Cystic Fibrosis Liver Disease Ne
References J Pediatr. 2015 Oct;167(4):862-868.e2. doi: 10.1016/j.jpeds.2015.06.062.
October 2015
Title: Statement on Exercise Testing in Cystic Fibrosis
Authors Hebestreit H, et al.
References Respiration. 2015;90:332-351
Keywords statement, Exercise testing
Theme Physiotherapy/Exercise testing
Title: International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety
Authors Alexandra L Quittner, Janice Abbott, Anna M Georgiopoulos, Lutz Goldbeck, Beth Smith, Sarah E Hempstead, Bruce Marshall, Kathryn A Sabadosa, Stuart Elborn, The International Committee on Mental Health Thorax
References doi:10.1136/thoraxjnl-2015-207488
Keywords mental health, screening, depression, anxiety
September 2015
Title: Genome-wide association meta-analysis identifies five modifier loci of lung disease severity in cystic fibrosis
Authors Corvol H, Blackman SM, Boëlle PY, Gallins PJ, Pace RG, Stonebraker JR, Accurso FJ, Clement A, Collaco JM, Dang H, Dang AT, Franca A, Gong J, Guillot L, Keenan K, Li W, Lin F, Patrone MV, Raraigh KS, Sun L, Zhou YH, O'Neal WK, Sontag MK, Levy H, Durie PR,
References Nat Commun. 2015 Sep 29;6:8382. doi: 10.1038/ncomms9382.
September 2015
Title: Outcomes and Treatment of Chronic Methicillin-Resistant Staphylococcus aureus Differs by Staphylococcal Cassette Chromosome mec (SCCmec) Type in Children With Cystic Fibrosis
Authors Heltshe SL, Saiman L, Popowitch EB, Miller MB, Kloster M, Thompson V, Ferkol TW, Hoover WC, Schechter MS, Muhlebach MS.
References J Pediatric Infect Dis Soc. 2015 Sep;4(3):225-31. doi: 10.1093/jpids/piu048. Epub 2014 Jun 9.
Keywords PVL, Panton-Valentine leukocidin, SCCmec type, methicillin-resistant Staphylococcus aureus; pulmonary exacerbation
August 2015
Title: Rate of Uptake of Ivacaftor Use after U.S. Food and Drug Administration Approval among Patients Enrolled in the U.S. Cystic Fibrosis Foundation Patient Registry
Authors Sawicki GS, Dasenbrook E, Fink AK, Schechter MS
References Ann Am Thorac Soc. 2015 Aug;12(8):1146-52. doi: 10.1513/AnnalsATS.201504-214OC
Keywords Ivacaftor, patient registry
June 2015
Title: Multicenter Observational Study on Factors and Outcomes Associated with Various Methicillin-Resistant Staphylococcus aureus Types in Children with Cystic Fibrosis
Authors Muhlebach MS, Heltshe SL, Popowitch EB, Miller MB, Thompson V, Kloster M, Ferkol T, Hoover WC, Schechter MS, Saiman L; STAR-CF Study Team
References Ann Am Thorac Soc. 2015 Jun;12(6):864-71.
Keywords Panton–Valentine leukocidin, SCCmec type, clinic visits, Methicillin-resistant Staphylococcus aureus, risk factor
May 2015
Title: Fine particulate matter exposure and initial Pseudomonas aeruginosa acquisition in cystic fibrosis.
Authors Psoter KJ, De Roos AJ, Mayer JD, Kaufman JD, Wakefield J, Rosenfeld M.
References Ann Am Thorac Soc. 2015 Mar;12(3):385-91. doi: 10.1513/AnnalsATS
Keywords Pseudomonas aeruginosa, ambient air pollution, Epidemiology, Fine particulate matter
Title: Effect of treatment of cystic fibrosis pulmonary exacerbations on systemic inflammation.
Authors Sagel SD, Thompson V, Chmiel JF, Montgomery GS, Nasr SZ, Perkett E, Saavedra MT, Slovis B, Anthony MM, Emmett P, Heltshe SL.
References Ann Am Thorac Soc. 2015 May;12(5):708-17
Keywords Biomarker, Plasma, Pulmonary exacerbation
May 2015
Title: Outcomes of infants with indeterminate diagnosis detected by cystic fibrosis newborn screening.
Authors Ren CL, Fink AK, Petren K, Borowitz DS, McColley SA, Sanders DB, Rosenfeld M, Marshall BC.
References Pediatrics. 2015 Jun;135(6):e1386-92. doi: 10.1542/peds.2014-3698. Epub 2015 May 11.
March 2015
Title: Comparison of WHO and CDC growth charts in predicting pulmonary outcomes in cystic fibrosis
Authors Machogu E, Cao Y, Miller T, Simpson P, Levy H, Quintero D, Goday PS.
References J Pediatr Gastroenterol Nutr. 2015 Mar;60(3):378-83. doi: 10.1097/MPG.0000000000000610.
January 2015
Title: Characterization of Missing Data in Clinical Registry Studies.
Authors Mendelsohn AB, Dreyer NA, Mattox PW, Su Z, Swenson A, Li R, Turner JR, Velentgas P.
References Ther Innov Regul Sci. 2015 Jan;49(1):146-154. doi: 10.1177/2168479014532259.
Keywords missing data, missingness, observational, registry, research, studies
December 2014
Title: Gender Differences in Outcomes of Patients with Cystic Fibrosis
Authors Harness-Brumley CL, Elliott AC, Rosenbluth DB, Raghavan D, Jain R.
References J Womens Health (Larchmt). 2014 Dec;23(12):1012-20. doi: 10.1089/jwh.2014.4985.
December 2014
Title: Improvements in lung function and height among cohorts of 6-year-olds with cystic fibrosis from 1994 to 2012
Authors VanDevanter DR, Pasta DJ, Konstan MW.
References J Pediatr. 2014 Dec;165(6):1091-1097.e2. doi: 10.1016/j.jpeds.2014.06.061
December 2014
Title: CFF pulmonary guideline. Pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection
Authors Mogayzel PJ, et al.
References Annals of the American Thoracic Society. 2014; 11(10):1640-1650
Keywords CFF, Pulmonary, guideline, pharmacologic, prevention, eradication, pseudonomas aeruginosa, Infection
Theme Lung Disease and Infection
November 2014
Title: Aspergillus and cystic fibrosis: old disease – new classifications
Authors Felton IC, Simmonds J
References Current Opinion in Pulmonary Medicine. 2014; 20:632-638
Keywords Aspergillus, disease, classification
Theme Lung Disease and Infection
September 2014
Title: Management of cystic fibrosis-related diabetes in children and adolescents ISAPD Clinical Practice Consensus Guidelines 2014 Compendium)
References Pediatric Diabetes. 2014: 15 (Suppl.20): 65-76
Keywords Management, cf-related, Diabetes, Child, Children, Adolescent, ISPAD, ISPAD Clinical Practice Consensus Guidelines, compendium
August 2014
Title: Infection prevention and control guideline for cystic fibrosis: 2013 update.
References Infection Control and Hospital Epidemilogy
Keywords infection prevention, infection control, guideline
Theme Lung Disease and Infection
June 2014
Title: Differential geographical risk of initial Pseudomonas aeruginosa acquisition in young U.S. children with cystic fibrosis.
Authors Psoter KJ, Rosenfeld M, De Roos AJ, Mayer JD, Wakefield J.
References Am J Epidemiol. 2014 Jun 15;179(12):1503-13. doi: 10.1093/aje/kwu077.
Keywords Bayesian hierarchical spatial models, Pseudomonas aeruginosa, Weibull regression, interval censoring, intrinsic conditional autoregression
May 2014
Title: Vitamin A supplementation for cystic fibrosis
Authors Bonifant CM, et al.
References Bonifant CM, Shevill E, Chang AB. Vitamin A supplementation for cystic fibrosis. Cochrane Database of Systematic Reviews 2014, Issue 5. Art. No.: CD006751. DOI: 10.1002/14651858.CD006751.pub4
Keywords vitamin A, pancreatic insufficiency, fat soluble vitamin deficiency, vitamin deficiency, deficiency, co-absorbed, fat, supplement, eye, skin, respiratory, skeletal, Child
Theme Nutrition/Gastroenterology
Title: Vitamin D supplementation
Authors Ferguson JH, Chang AB
References Ferguson JH, Chang AB. Vitamin D supplementation for cystic fibrosis. Cochrane Database of Systematic Reviews 2014, Issue 5. Art. No.: CD007298. DOI: 10.1002/14651858.CD007298.pub4
Keywords Vitamin D, pancreatic insufficiency malabsorption, fat soluble vitamin, calcium homeostasis, bone mineralisation, extraskeletal effect, supplementation
Theme Nutrition/Gastroenterology
Title: European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis
References Journal of Cystic Fibrosis. 2014 May;13 Suppl 1:S43-S59
Keywords standards of care, quality management
Theme Care of patients with CF, Clinical Trials, Pregnancy with CF
Title: European Cystic Fibrosis Society Standards of Care: Best Practice guidelines
References Journal of Cystic Fibrosis. 2014 May;13 Suppl 1:S23-S42
Theme Care of patients with CF, Clinical Trials, Pregnancy with CF
Title: European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre
References Journal of Cystic Fibrosis. 2014 May;13 Suppl 1:S3-S22
Keywords standards of care, framework, Cystic Fibrosis Centre, CFC
Theme Care of patients with CF, Clinical Trials, Pregnancy with CF
March 2014
Title: Antimicrobial susceptibility and molecular typing of MRSA in cystic fibrosis.
Authors Champion EA, Miller MB, Popowitch EB, Hobbs MM, Saiman L, Muhlebach MS; STAR-CF Study Team.
References Pediatr Pulmonol. 2014 Mar;49(3):230-7. doi: 10.1002/ppul.22815
Keywords Panton-Valentine leukocidin, community MRSA, hospital MRSA, multi-center study, spa-typing; staphylococcal cassette chromosome mec
March 2014
Title: Modeling the impact of antibiotic exposure on human microbiota
Authors Zhao J, Murray S, Lipuma JJ.
References Sci Rep. 2014 Mar 11;4:4345. doi: 10.1038/srep04345
March 2014
Title: Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis
Authors Hirche TO, et al.
References Pulmonary Medicine. 2014:621342. doi: 10.1155/2014/621342. Epub 2014 Mar 30. Review
Keywords guideline, practical guideline, Lung transplant, Lung transplantation
January 2014
Title: Effects of puberty on cystic fibrosis related pulmonary exacerbations in women versus men
Authors Sutton S, Rosenbluth D, Raghavan D, Zheng J, Jain
References Pediatr Pulmonol. 2014 Jan;49(1):28-35. doi: 10.1002/ppul.22767
Keywords Exacerbation, gender disparity, puberty
December 2013
Title: A semiparametric approach to estimate rapid lung function decline in cystic fibrosis
Authors Szczesniak RD, McPhail GL, Duan LL, Macaluso M, Amin RS, Clancy JP
References Ann Epidemiol. 2013 Dec;23(12):771-7. doi: 10.1016/j.annepidem.2013.08.009. Epub 2013 Oct 5
Keywords Epidemiologic methods, Nonlinear models, Spline regression
October 2013
Title: Genetic modifiers of cystic fibrosis-related diabetes.
Authors Blackman SM, Commander CW, Watson C, Arcara KM, Strug LJ, Stonebraker JR, Wright FA, Rommens JM, Sun L, Pace RG, Norris SA, Durie PR, Drumm ML, Knowles MR, Cutting GR.
References Diabetes. 2013 Oct;62(10):3627-35. doi: 10.2337/db13-0510.
October 2013
Title: Culture-based diagnostic microbiology in cystic fibrosis: Can we simplify the complexity?
Keywords microbiology, diagnostic microbiology, culture-based, complexity, simplification
September 2013
Title: Treatment of pulmonary exacerbations in cystic fibrosis
References European Respiratory Review. 2013 Sep;22:205-216
Keywords treatment, Pulmonary exacerbation
Theme Lung Disease and Infection
June 2013
Title: Season is associated with Pseudomonas aeruginosa acquisition in young children with cystic fibrosis.
Authors Psoter KJ, De Roos AJ, Wakefield J, Mayer J, Rosenfeld M.
References Clin Microbiol Infect. 2013 Nov;19(11):E483-9. doi: 10.1111/1469-0691.12272. Epub 2013 Jun 25.
Keywords Acquisition, Pseudomonas aeruginosa, Climate, Seasonality
June 2013
Title: Drug induced complications; can we do more?
Keywords drug induced complications
April 2013
Title: Cystic Fibrosis Pulmonary Guidelines chronic medications for maintenance of lung health
Authors Mogayzel PJ, et al.
References American Journal of Respiratory and Critical Care Medicine. 2013; 187:680-689
Keywords Pulmonary, guideline, chronic medication, maintenance, lung health
Theme Lung Disease and Infection
Title: Update on cystic fibrosis-related diabetes
Keywords Diabetes, diabetes mellitus, CFRD
Title: A review of renal disease in cystic fibrosis
Keywords renal disease, Review, kidney
February 2013
Title: Molecular detection of CF lung pathogens: Current status and future potential
Keywords molecular detection, lung pathogens, nucleic acids, polymicrobial, misidentification, microbiology
December 2012
Title: Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis
Authors Waters V, Ratjen F
References Waters V, Ratjen F. Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2012, Issue 12. Art. No.: CD010004. DOI: 10.1002/14651858.CD010004.pub2
Keywords antibiotic treatment, antibiotic, treatment, nontuberculous mycobacteria, lung infection
Theme Lung Disease and Infection
Title: Treatment of lung infection in patients with cystic fibrosis: Current and future strategies
References Journal of Cystic Fibrosis. 2012 Dec;11(6):461-479
Keywords treatment, lung infection, strategy, future
Theme Lung Disease and Infection
Title: Nutritional intervention in patients with Cystic Fibrosis: A systematic review
Keywords nutritional intervention, Systematic review
November 2012
Title: Report of the workshop on endpoints for cystic fibrosis clinical trials
Keywords endpoints, clinical trials, report
Title: Treatment of lung infection in patients with cystic fibrosis:Current and future strategies
Authors Döring G, Flume P, Heijerman H, Elborn JS for the consensus study group
References Journal of Cystic Fibrosis 2012; 11 : 461-79
Keywords lung, lung infection, strategy
September 2012
Title: Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening
Keywords Lung function, abnormality, infants, Diagnosis, newborn, screening, nutritional status, pulmonary health
Title: Measures of body habitus are associated with lung function in adults with cystic fibrosis: A population-based study
Keywords body habitus, Lung function, population-based study, lean muscle mass, obesity
June 2012
Title: Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis
Authors Elphick HE, Southern KW
References Elphick HE, Southern KW. Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2012, Issue 6. Art. No.: CD002204. DOI: 10.1002/14651858.CD002204.pub2
Keywords antifungal therapy, therapy, allergic brochopulmonary aspergillosis, allergy
Theme Lung Disease and Infection
April 2012
Title: Vitamin D supplementation for cystic fibrosis
Authors Ferguson JH, Chang AB
References Ferguson JH, Chang AB. Vitamin D supplementation for cystic fibrosis. Cochrane Database of Systematic Reviews 2012, Issue 4. Art. No.: CD007298. DOI: 10.1002/14651858.CD007298.pub3
Keywords Vitamin D, supplement, supplementation, pancreatic insufficiency, pancreatic insufficiency malabsorption, fat soluble, fat soluble vitamin, calcium homeostasis, bone mineralisation, extraskeletal effect
Theme Nutrition/Gastroenterology
Title: An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation
Authors Tangpricha V, et al.
References The Journal of Clinical Endocrinology and Metabolism. 2012 Apr; 97(4):1082-93.
Keywords Vitamin D, vitamin D deficiency, Cystic Fibrosis Foundation, CFF
Title: Cystic fibrosis research in allied health and nursing professions
Keywords allied health, nursing, professions, research, roadmap
