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Article Title |
Author(s) |
Type (Status) |
Available Online |
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The use of nebulised aztreonam lysine (AZLI) in aztreonam hypersensitive patients
DOI: 10.1016/j.jcf.2012.01.007
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P. Whitaker,
C. Etherington,
K. Williams,
S. Conway,
D. Peckham
et al.
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Correspondence
(In Press Corrected Proof)
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20 February 2012 |
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Risk factors for Mycobacterium abscessus infection in cystic fibrosis patients; a case–control study
DOI: 10.1016/j.jcf.2012.01.006
Abstract: Mycobacterium abscessus is a nontuberculous mycobacterium that is increasingly recognized as an opportunistic pathogen in cystic fibrosis (CF) patients. Factors that predispose CF patients t...
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Maarten Verregghen,
Harry G. Heijerman,
Monique Reijers,
Jakko van Ingen,
Cornelis K. van der Ent
et al.
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Short communication
(In Press Corrected Proof)
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20 February 2012 |
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Acknowledgment of referees
DOI: 10.1016/j.jcf.2011.11.002
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Miscellaneous
(In Press Corrected Proof)
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13 February 2012 |
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Comparison of antibiotic susceptibility patterns in Pseudomonas aeruginosa isolated from adult patients with cystic fibrosis (CF) with invasive Pseudomonas aeruginosa from non-CF patients
DOI: 10.1016/j.jcf.2012.01.002
The role of bacterial pathogens in CF pulmonary disease contributes greatly to the morbidity and mortality in patients with CF. CF patients have recurrent and chronic respiratory tract infections and ...
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Priyanka Rao,
John McCaughan,
Mark McCalmont,
Colin.E. Goldsmith,
Valerie Hall,
B. Cherie Millar,
Mary-Ann McCann,
Damian G. Downey,
Jacqueline C. Rendall,
J. Stuart Elborn,
John E. Moore
et al.
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Correspondence
(In Press Corrected Proof)
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13 February 2012 |
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Cystic fibrosis genetic counseling difficulties due to the identification of novel mutations in the CFTR gene
DOI: 10.1016/j.jcf.2012.01.004
Abstract: Background: The Cystic Fibrosis database includes amongst the 1893 gene mutations and polymorphisms a lot of missense mutations, the disease status of which still remains unproven. In popula...
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Myrto Poulou,
Irini Fylaktou,
Maria Fotoulaki,
Emmanuel Kanavakis,
Maria Tzetis
et al.
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Short communication
(In Press Corrected Proof)
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13 February 2012 |
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Retrospective analysis of stored dried blood spots from children with cystic fibrosis and matched controls to assess the performance of a proposed newborn screening protocol in Switzerland
DOI: 10.1016/j.jcf.2012.01.001
Abstract: Background: Newborn screening (NBS) for Cystic Fibrosis (CF) has been introduced in many countries, but there is no ideal protocol suitable for all countries. This retrospective study was co...
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Juerg Barben,
Sabina Gallati,
Ralph Fingerhut,
Martin H. Schoeni,
Matthias R. Baumgartner,
Toni Torresani,
SWISS CF SCREENING GROUP
et al.
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Full-length article
(In Press Corrected Proof)
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02 February 2012 |
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Ivacaftor potentiation of multiple CFTR channels with gating mutations
DOI: 10.1016/j.jcf.2011.12.005
Abstract: Background: The investigational CFTR potentiator ivacaftor (VX-770) increased CFTR channel activity and improved lung function in subjects with CF who have the G551D CFTR gating mutation. Th...
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Haihui Yu,
Bill Burton,
Chien-Jung Huang,
Jennings Worley,
Dong Cao,
James P. Johnson,
Art Urrutia,
John Joubran,
Sheila Seepersaud,
Katherine Sussky,
Beth J. Hoffman,
Fredrick Van Goor
et al.
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Full-length article
(In Press Corrected Proof)
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01 February 2012 |
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Voriconazole pharmacokinetics and photosensitivity in children with cystic fibrosis
DOI: 10.1016/j.jcf.2011.12.006
Abstract: Background: A high incidence of adverse skin reactions following long-term oral administration of voriconazole in children with cystic fibrosis and allergic bronchopulmonary aspergillosis (A...
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Sophia L. Markantonis,
Anna Katelari,
Eleni Pappa,
Stavros Doudounakis
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Full-length article
(In Press Corrected Proof)
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27 January 2012 |
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A randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference
DOI: 10.1016/j.jcf.2011.12.004
Abstract: Background: Preclinical data suggest that miglustat could restore the function of the cystic fibrosis transmembrane conductance regulator gene in cystic fibrosis cells. Methods: Single-cente...
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Anissa Leonard,
Patrick Lebecque,
Jasper Dingemanse,
Teresinha Leal
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Full-length article
(In Press Corrected Proof)
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27 January 2012 |
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Determining presence of lung disease in young children with cystic fibrosis: Lung clearance index, oxygen saturation and cough frequency
DOI: 10.1016/j.jcf.2011.12.003
Abstract: Background: Accurate assessment of pulmonary status in young children with cystic fibrosis (CF) requires sensitive and objective monitoring techniques. Objectives: This study aimed to evalua...
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E.M. Bakker,
J.C. van der Meijden,
E.M. Nieuwhof,
W.C.J. Hop,
H.A.W.M. Tiddens
et al.
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Full-length article
(In Press Corrected Proof)
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24 January 2012 |
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Feasibility of using pedometers to measure daily step counts in cystic fibrosis and an assessment of its responsiveness to changes in health state
DOI: 10.1016/j.jcf.2011.12.002
Abstract: Background: Evaluation of physical activity is integral to the assessment of daily physical function and a potential objective outcome measure for clinical trials. We evaluated the feasibili...
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Bradley S. Quon,
Donald L. Patrick,
Todd C. Edwards,
Moira L. Aitken,
Ronald L. Gibson,
Alan Genatossio,
Sharon McNamara,
Christopher H. Goss
et al.
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Full-length article
(In Press Corrected Proof)
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06 January 2012 |
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Effect of acute exacerbations on skeletal muscle strength and physical activity in cystic fibrosis
DOI: 10.1016/j.jcf.2011.12.001
Abstract: Background: Skeletal muscle weakness is an important complication of chronic respiratory disease. The effect of acute exacerbations on strength in patients with cystic fibrosis is not known....
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Jason Wieboldt,
Louis Atallah,
Julia L. Kelly,
Dinesh Shrikrishna,
Khin M. Gyi,
Benny Lo,
Guang Zhong Yang,
Diana Bilton,
Michael I. Polkey,
Nicholas S. Hopkinson
et al.
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Full-length article
(In Press Corrected Proof)
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06 January 2012 |
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Impact of IVS8-(TG)m(T)n on IRT and sweat chloride levels in newborns identified by California CF newborn screening
DOI: 10.1016/j.jcf.2011.11.010
Abstract: We examined the relation between the number of (TG) repeats at the (IVS8)-(TG)m(T)5 locus of the CFTR gene with neonatal serum immunoreactive trypsinogen (IRT) and sweat chloride (SC) concen...
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Steven Keiles,
Ruth Koepke,
Richard Parad,
Martin Kharrazi,
California Cystic Fibrosis Newborn Screening Consortium
et al.
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Short communication
(In Press Corrected Proof)
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03 January 2012 |
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Six minute walk test Z score: Correlations with cystic fibrosis severity markers
DOI: 10.1016/j.jcf.2011.11.009
Abstract: Background: The six-minute-walk-test (6MWT) has been increasingly used in cystic fibrosis (CF) patients. However, few studies in children have correlated 6MWT with current parameters used to...
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Fabíola Stollar,
Joaquim C. Rodrigues,
Maristela T. Cunha,
Claudio Leone,
Fabíola Villac Adde
et al.
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Short communication
(In Press Corrected Proof)
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22 December 2011 |
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Small macrophages are present in early childhood respiratory disease
DOI: 10.1016/j.jcf.2011.11.008
Abstract: Background: Recently, an established “small macrophage” phenotype has been observed in the sputum of patients with CF and COPD. However, little is known about the prevalence of this phenotyp...
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Luke W. Garratt,
Adam K.A. Wright,
Sarath C. Ranganathan,
Jonathan Grigg,
Peter D. Sly,
on behalf of AREST CF
et al.
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Full-length article
(In Press Corrected Proof)
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21 December 2011 |
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Long-term non-invasive ventilation in cystic fibrosis — Experience over two decades
DOI: 10.1016/j.jcf.2011.11.006
Abstract: Background: Non-invasive ventilation (NIV) is accepted as a bridge to lung transplantation in cystic fibrosis (CF) but there is little evidence to support its use outside this setting. Metho...
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William G. Flight,
Jonathan Shaw,
Susan Johnson,
A. Kevin Webb,
Andrew M. Jones,
Andrew M. Bentley,
Rowland J. Bright-Thomas
et al.
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Full-length article
(In Press Corrected Proof)
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19 December 2011 |
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IL-17A as a regulator of neutrophil survival in nasal polyp disease of patients with and without cystic fibrosis
DOI: 10.1016/j.jcf.2011.11.007
Abstract: Nasal polyps in adults are characterized by a chronic inflammation of the upper airways and by the preferential activation of Th2 cells. In contrast, IL-17 producing Th17 cells dominate the ...
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L. Derycke,
N. Zhang,
G. Holtappels,
T. Dutré,
C. Bachert
et al.
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Full-length article
(In Press Corrected Proof)
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19 December 2011 |
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Patient indicators of a pulmonary exacerbation: Preliminary reports from school aged children map onto those of adults
DOI: 10.1016/j.jcf.2011.11.005
Abstract: Background: Despite the importance of identifying and managing a pulmonary exacerbation, and its use as an outcome measure in interventions, there is no standardised definition in cystic fib...
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J. Abbott,
A. Holt,
A.M. Morton,
A. Hart,
G. Milne,
S.P. Wolfe,
S.P. Conway
et al.
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Full-length article
(In Press Corrected Proof)
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15 December 2011 |
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Increasing resistance of the Liverpool Epidemic Strain (LES) of Pseudomonas aeruginosa (Psa) to antibiotics in cystic fibrosis (CF)—A cause for concern?
DOI: 10.1016/j.jcf.2011.11.004
Abstract: Background: Transmissible Pseudomonas aeruginosa (Psa) strains such as the Liverpool Epidemic Strain (LES) are now widespread throughout UK CF clinics: their susceptibility to antibiotics is...
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Abdul Ashish,
Matthew Shaw,
C. Winstanley,
Martin J. Ledson,
Martin J. Walshaw
et al.
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Full-length article
(In Press Corrected Proof)
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07 December 2011 |
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Reduced levels of active GLP-1 in patients with cystic fibrosis with and without diabetes mellitus
DOI: 10.1016/j.jcf.2011.11.001
Abstract: Glucagon like peptide 1 (GLP-1) is an incretin hormone released as a bioactive peptide from intestinal L-cells in response to eating. It acts on target cells and exerts several functions as ...
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Magnus Hillman,
Leif Eriksson,
Lena Mared,
Karin Helgesson,
Mona Landin-Olsson
et al.
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Full-length article
(In Press Corrected Proof)
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05 December 2011 |
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Antimicrobial activity of fosfomycin and tobramycin in combination against cystic fibrosis pathogens under aerobic and anaerobic conditions
DOI: 10.1016/j.jcf.2011.11.003
Abstract: Background: There is a need for new antibiotics or combination of antibiotics that possess activity against increasingly resistant cystic fibrosis (CF) respiratory pathogens such as Pseudomo...
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Gerard McCaughey,
Matt McKevitt,
J. Stuart Elborn,
Michael M. Tunney
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Full-length article
(In Press Corrected Proof)
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05 December 2011 |
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Sinonasal persistence of Pseudomonas aeruginosa after lung transplantation
DOI: 10.1016/j.jcf.2011.10.009
ABSTRACT: We report on two CF patients who received double lung transplantation (LTX) due to Pseudomonas aeruginosa related pulmonary destruction. Prior to LTX we detected P. aeruginosa in nasal lavag...
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J.G. Mainz,
J. Hentschel,
C. Schien,
N. Cramer,
W. Pfister,
J.F. Beck,
B. Tümmler
et al.
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Short communication
(In Press Corrected Proof)
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02 December 2011 |
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A fat lot of good: Balance and trends in fat intake in children with cystic fibrosis
DOI: 10.1016/j.jcf.2011.10.007
Abstract: Background: The fundamental nutritional treatment of a high fat diet for cystic fibrosis (CF) is established and essentially unchanged in the last 25years. However, recent concerns have emer...
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C. Smith,
A. Winn,
P. Seddon,
S. Ranganathan
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Short communication
(In Press Corrected Proof)
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28 November 2011 |
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Comparative bone status assessment by dual energy X-ray absorptiometry, peripheral quantitative computed tomography and quantitative ultrasound in adolescents and young adults with cystic fibrosis
DOI: 10.1016/j.jcf.2011.10.004
Abstract: Purpose: Quantitative ultrasound bone sonometry (QUS) might be a promising screening method for cystic fibrosis (CF)-related bone disease, given its absence of radiation exposure, portabilit...
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Jean De Schepper,
Inge Roggen,
Stephanie Van Biervliet,
Eddy Robberecht,
Inge Gies,
Kathleen De Waele,
Elke De Wachter,
Anne Malfroot,
Frans De Baets,
Kaatje Toye,
Stefan Goemaere,
Olivia Louis
et al.
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Full-length article
(In Press Corrected Proof)
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28 November 2011 |
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Qualitative analysis of parent experiences with achieving cystic fibrosis nutrition recommendations
DOI: 10.1016/j.jcf.2011.10.006
Abstract: Background: A combined behavior–nutrition approach is recommended for children with CF with growth deficits. The present study aimed to use a qualitative approach to 1) understand families' ...
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Stephanie S. Filigno,
Erin E. Brannon,
Leigh Ann Chamberlin,
Stephanie M. Sullivan,
Kimberly A. Barnett,
Scott W. Powers
et al.
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Full-length article
(In Press Corrected Proof)
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23 November 2011 |
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Diabetes mellitus and survival in cystic fibrosis patients after lung transplantation
DOI: 10.1016/j.jcf.2011.10.005
Abstract: Background: Diabetes mellitus (DM) is common in CF and associated with more severe disease. It is unclear whether DM influences outcome of lung transplantation (LTx). Methods: One hundred tw...
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Markus Hofer,
Christoph Schmid,
Christian Benden,
Rudolf Speich,
Ilhan Inci,
Walter Weder,
Annette Boehler
et al.
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Full-length article
(In Press Corrected Proof)
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23 November 2011 |
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Comparing age of cystic fibrosis diagnosis and treatment initiation after newborn screening with two common strategies
DOI: 10.1016/j.jcf.2011.10.002
Abstract: Background: Newborn screening (NBS) for CF has become widespread, although there are multiple strategies. Little is known about outcomes such as age of diagnosis after different NBS methods....
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Don B. Sanders,
Huichuan J. Lai,
Michael J. Rock,
Philip M. Farrell
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Short communication
(In Press Corrected Proof)
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21 November 2011 |
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Employment and work disability in adults with cystic fibrosis
DOI: 10.1016/j.jcf.2011.10.008
Abstract: Background: As a result of prolonged survival, more patients with cystic fibrosis (CF) participate in the labour force. The aim of this study was to evaluate their education, occupation leve...
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Hervé Laborde-Castérot,
Carole Donnay,
Jeanne Chapron,
Pierre-Régis Burgel,
Reem Kanaan,
Isabelle Honoré,
Daniel Dusser,
Dominique Choudat,
Dominique Hubert
et al.
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Full-length article
(In Press Corrected Proof)
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21 November 2011 |
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Effect of dornase alfa on inflammation and lung function: Potential role in the early treatment of cystic fibrosis
DOI: 10.1016/j.jcf.2011.10.003
Abstract: Dornase alfa has been shown to reduce markers of inflammation and neutrophil-associated metalloproteinases in cystic fibrosis (CF), suggesting a potential benefit from use of this therapy ea...
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Michael W. Konstan,
Felix Ratjen
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Review article
(In Press Corrected Proof)
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17 November 2011 |
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Inflammatory and growth factor response to continuous and intermittent exercise in youth with cystic fibrosis
DOI: 10.1016/j.jcf.2011.10.001
Abstract: Background: Children with cystic fibrosis (CF) tend to suffer from chronic systemic inflammation and may have impaired growth associated with muscle catabolism. Therefore, investigating whic...
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Thanh Nguyen,
Joyce Obeid,
Hilde E. Ploeger,
Tim Takken,
Linda Pedder,
Brian W. Timmons
et al.
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Full-length article
(In Press Corrected Proof)
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09 November 2011 |
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A novel neutrophil derived inflammatory biomarker of pulmonary exacerbation in cystic fibrosis
DOI: 10.1016/j.jcf.2011.09.010
Abstract: Background: The focus of this study was to characterize a novel biomarker for cystic fibrosis (CF) that could reflect exacerbations of the disease and could be useful for therapeutic stratif...
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Emer P. Reeves,
David A. Bergin,
Sean Fitzgerald,
Elaine Hayes,
Joanne Keenan,
Michael Henry,
Paula Meleady,
Isabel Vega-Carrascal,
Michelle A. Murray,
Teck Boon Low,
Cormac McCarthy,
Emmet O'Brien,
Martin Clynes,
Cedric Gunaratnam,
Noel G. McElvaney
et al.
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Full-length article
(In Press Corrected Proof)
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31 October 2011 |
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CT of the paranasal sinuses is not a valid indicator for sinus surgery in CF patients
DOI: 10.1016/j.jcf.2011.09.009
Abstract: Background: No guidelines comprise when or to what extent sinus surgery should be done in patients with cystic fibrosis (CF) or how a CT scan of the paranasal sinuses should influence the de...
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Jacob Rasmussen,
Kasper Aanæs,
Rikke Norling,
Kim G. Nielsen,
Helle Krogh Johansen,
Christian von Buchwald
et al.
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Full-length article
(In Press Corrected Proof)
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24 October 2011 |
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Ultrastructural characterization of cystic fibrosis sputum using atomic force and scanning electron microscopy
DOI: 10.1016/j.jcf.2011.09.008
Abstract: Background: Cystic fibrosis (CF) lung disease is characterized by perpetuated neutrophilic inflammation with progressive tissue destruction. Neutrophils represent the major cellular fraction...
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Reinhard Manzenreiter,
Ferry Kienberger,
Veronica Marcos,
Kurt Schilcher,
Wolf D. Krautgartner,
Astrid Obermayer,
Marlene Huml,
Walter Stoiber,
Andreas Hector,
Matthias Griese,
Matthias Hannig,
Michael Studnicka,
Ljubomir Vitkov,
Dominik Hartl
et al.
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Full-length article
(In Press Corrected Proof)
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13 October 2011 |