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Antimicrobial activity of fosfomycin and tobramycin in combination against cystic fibrosis pathogens under aerobic and anaerobic conditions
05 December 2011
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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163-172
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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?
07 December 2011
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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173-179
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Patient indicators of a pulmonary exacerbation: Preliminary reports from school aged children map onto those of adults
15 December 2011
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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180-186
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Long-term non-invasive ventilation in cystic fibrosis — Experience over two decades
19 December 2011
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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187-192
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IL-17A as a regulator of neutrophil survival in nasal polyp disease of patients with and without cystic fibrosis
19 December 2011
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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193-200
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Small macrophages are present in early childhood respiratory disease
21 December 2011
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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201-208
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Effect of acute exacerbations on skeletal muscle strength and physical activity in cystic fibrosis
06 January 2012
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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209-215
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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
06 January 2012
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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216-222
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Determining presence of lung disease in young children with cystic fibrosis: Lung clearance index, oxygen saturation and cough frequency
24 January 2012
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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223-230
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A randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference
27 January 2012
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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231-236
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Ivacaftor potentiation of multiple CFTR channels with gating mutations
01 February 2012
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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237-245
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Voriconazole pharmacokinetics and photosensitivity in children with cystic fibrosis
27 January 2012
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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246-252
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| Short Communications |
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Six minute walk test Z score: Correlations with cystic fibrosis severity markers
22 December 2011
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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253-256
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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
03 January 2012
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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257-260
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Acknowledgment of referees
13 February 2012
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261-263
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