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Article Title |
Author(s) |
Type (Status) |
Available Online |
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PCR detection of Burkholderia cepacia complex as one of key factors to handle a long-term outbreak
DOI: 10.1016/j.jcf.2012.04.005
Abstract: Background: Once the outbreak with Burkholderia cenocepacia ST32 was identified in the Prague cystic fibrosis (CF) centre, molecular tools were implemented into diagnostic routine in order t...
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Klara Dedeckova,
Libor Fila,
Veronika Skalicka,
Jana Bartosova,
Tereza Kucerova,
Vera Vavrova,
Dana Zemkova,
Lucie Kalferstova,
Oto Melter,
Ondrej Cinek,
Pavel Drevinek
et al.
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Full-length article
(In Press Corrected Proof)
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24 May 2012 |
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Increase in interleukin-8 production from circulating neutrophils upon antibiotic therapy in cystic fibrosis patients
DOI: 10.1016/j.jcf.2012.04.010
Abstract: Background: It is not known whether antibiotic therapy for lung disease in cystic fibrosis (CF) has an influence on circulating polymorphonuclear neutrophil (PMN) function and apoptosis. Pat...
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Pasqualina Montemurro,
Maria A. Mariggiò,
Giovanna Barbuti,
Amalia Cassano,
Alessandra Vincenti,
Gabriella Serio,
Lorenzo Guerra,
Anna Diana,
Teresa Santostasi,
Angela Polizzi,
Ruggiero Fumarulo,
Valeria Casavola,
Antonio Manca,
Massimo Conese
et al.
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Full-length article
(In Press Corrected Proof)
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21 May 2012 |
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Blood basophils from cystic fibrosis patients with allergic bronchopulmonary aspergillosis are primed and hyper-responsive to stimulation by aspergillus allergens
DOI: 10.1016/j.jcf.2012.04.008
Abstract: Introduction: Fifteen to sixty percent of cystic fibrosis patients harbor Aspergillus fumigatus (Af) in their airways (CF-AC) and some will develop allergic bronchopulmonary aspergillosis (C...
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Yael Gernez,
Colleen E. Dunn,
Cassie Everson,
Erin Mitsunaga,
Lakshmi Gudiputi,
Karolina Krasinska,
Zoe A. Davies,
Leonore A. Herzenberg,
Rabindra Tirouvanziam,
Richard B. Moss
et al.
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Full-length article
(In Press Corrected Proof)
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18 May 2012 |
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Colonisation and infection of the paranasal sinuses in cystic fibrosis patients is accompanied by a reduced PMN response
DOI: 10.1016/j.jcf.2012.04.011
Abstract: Background: We studied whether the sinuses might be foci for Pseudomonas aeruginosa lung infection. Methods: Endoscopic Sinus Surgery was performed in 78 CF patients; PFGE was used for bacte...
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Helle Krogh Johansen,
Kasper Aanaes,
Tania Pressler,
Kim Gjerrum Nielsen,
Jacob Fisker,
Marianne Skov,
Niels Høiby,
Christian von Buchwald
et al.
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Full-length article
(In Press Corrected Proof)
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17 May 2012 |
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Functional analysis of synonymous substitutions predicted to affect splicing of the CFTR gene
DOI: 10.1016/j.jcf.2012.04.009
Abstract: Background: Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Over 1800 CFTR mutations have been reported, and about 12% of mutat...
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Alexandra Scott,
Hanna M. Petrykowska,
Timothy Hefferon,
Valer Gotea,
Laura Elnitski
et al.
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Full-length article
(In Press Corrected Proof)
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16 May 2012 |
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Role of respiratory viruses in pulmonary exacerbations in children with cystic fibrosis
DOI: 10.1016/j.jcf.2012.04.006
Abstract: Background: The role of respiratory viruses in cystic fibrosis (CF) exacerbations is incompletely understood. Methods: Cross-sectional study of CF children with a pulmonary exacerbation. Mid...
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Sandra Asner,
Valerie Waters,
Melinda Solomon,
Yvonne Yau,
Susan E. Richardson,
Hartmut Grasemann,
Farhad Gharabaghi,
Dat Tran
et al.
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Full-length article
(In Press Corrected Proof)
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14 May 2012 |
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Validation of the Treatment Satisfaction Questionnaire for Medication in patients with cystic fibrosis
DOI: 10.1016/j.jcf.2012.04.007
Abstract: Background: Our objective was to confirm the measurement properties of the Treatment Satisfaction Questionnaire with Medication (TQSM) in patients with cystic fibrosis (CF) receiving inhaled...
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Antoine Regnault,
Maria-Magdalena Balp,
Károly Kulich,
Muriel Viala-Danten
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Full-length article
(In Press Corrected Proof)
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14 May 2012 |
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Risk factors for rate of decline in FEV1 in adults with cystic fibrosis
DOI: 10.1016/j.jcf.2012.03.009
Abstract: Background: Previously we assessed risk factors for FEV1 decline in children and adolescents using the Epidemiologic Study of Cystic Fibrosis (J Pediatr 2007;151:134–139); the current study ...
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Michael W. Konstan,
Jeffrey S. Wagener,
Donald R. VanDevanter,
David J. Pasta,
Ashley Yegin,
Lawrence Rasouliyan,
Wayne J. Morgan
et al.
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Full-length article
(In Press Corrected Proof)
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07 May 2012 |
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Successful treatment of cepacia syndrome with a combination of intravenous cyclosporin, antibiotics and oral corticosteroids
DOI: 10.1016/j.jcf.2012.04.002
Abstract: Burkholderia cepacia complex (BCC) is a group of 17 closely related bacterial species that can cause pulmonary infection in patients with cystic fibrosis (CF). The clinical manifestations of...
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Francis J. Gilchrist,
A. Kevin Webb,
Rowland J. Bright-Thomas,
Andrew M. Jones
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Short communication
(In Press Corrected Proof)
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03 May 2012 |
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Risk factors for age at initial Pseudomonas acquisition in the cystic fibrosis epic observational cohort
DOI: 10.1016/j.jcf.2012.04.003
Abstract: Background: Risk factors for initial Pseudomonas aeruginosa (Pa) acquisition, particularly environmental exposures, are poorly understood. We aimed to identify such risk factors in order to ...
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Margaret Rosenfeld,
Julia Emerson,
Sharon McNamara,
Valeria Thompson,
Bonnie W. Ramsey,
Wayne Morgan,
Ronald L. Gibson,
for the EPIC Study Group
et al.
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Full-length article
(In Press Corrected Proof)
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03 May 2012 |
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Prevalence of cystic fibrosis pathogens in the oropharynx of healthy children and implications for cystic fibrosis care
DOI: 10.1016/j.jcf.2012.04.004
Abstract: Objective: To describe the prevalence of the CF pathogens Pseudomonas aeruginosa, Staphylococcus aureus and Haemophilus influenzae in OP cultures from healthy children. Methods: Oropharyngea...
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Margaret Rosenfeld,
Carmen Bernardo-Ocampo,
Julia Emerson,
Alan Genatossio,
Jane Burns,
Ron Gibson
et al.
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Short communication
(In Press Corrected Proof)
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01 May 2012 |
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Gut dysbiosis in cystic fibrosis
DOI: 10.1016/j.jcf.2012.03.007
In people with CF, intestinal exocrine malfunction, antibiotic usage and swallowing of infected respiratory mucus likely perturb the normal community of commensal bacteria in the gut. People with CF...
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Pauline D. Scanlan,
Angus Buckling,
Weidong Kong,
Yvette Wild,
Susan V. Lynch,
Freya Harrison
et al.
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Correspondence
(In Press Corrected Proof)
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26 April 2012 |
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Pilot study on the use of acoustic radiation force impulse imaging in the staging of cystic fibrosis associated liver disease
DOI: 10.1016/j.jcf.2012.04.001
Abstract: Background: Acoustic radiation force impulse (ARFI) is a novel technique for the measurement of hepatic stiffness, which could be valuable in clinical follow-up of patients affected by cysti...
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Melania Manco,
Cristina Lo Zupone,
Federico Alghisi,
Maria Luisa D'Andrea,
Vincenzina Lucidi,
Lidia Monti
et al.
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Full-length article
(In Press Corrected Proof)
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26 April 2012 |
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GUSB and ATP2B4 are suitable reference genes for CFTR gene expression data normalization in nasal epithelium cells
DOI: 10.1016/j.jcf.2012.03.008
Abstract: Background: CFTR expression studies contribute in understanding the relationship between CFTR transcripts and clinical outcomes. Normalization of qPCR data is an essential step to determine ...
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Laia Masvidal,
Antoni Alvarez,
Laura Ruano,
Javier de Gracia,
Sara Larriba,
Teresa Casals
et al.
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Full-length article
(In Press Corrected Proof)
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23 April 2012 |
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A phase I clinical study of inhaled nitric oxide in healthy adults
DOI: 10.1016/j.jcf.2012.01.003
Abstract: Background: Nitric oxide (NO) is an approved pulmonary vasodilator for neonates and full term infants up to a dose of 80ppm. At 100ppm to 200ppm, NO has potent antimicrobial activities in vi...
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Chris Miller,
Minna Miller,
Bevin McMullin,
Gilly Regev,
Lena Serghides,
Kevin Kain,
Jeremy Road,
Yossef Av-Gay
et al.
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Full-length article
(In Press Corrected Proof)
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20 April 2012 |
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Cystic fibrosis research in allied health and nursing professions
DOI: 10.1016/j.jcf.2012.03.004
Abstract: This report is the result of the “Allied Health and Nursing Professions Working Group” meeting which took place in Verona, Italy, November 2009, which was organised by the European Cystic Fi...
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Judy M. Bradley,
Susan Madge,
Alison M. Morton,
Alexandra L. Quittner,
J. Stuart Elborn,
For the Allied Health and Nursing Professions Working Group, European Cystic Fibrosis Society
et al.
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Full-length article
(In Press Corrected Proof)
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19 April 2012 |
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LDL-cholesterol and insulin are independently associated with body mass index in adult cystic fibrosis patients
DOI: 10.1016/j.jcf.2012.03.006
Abstract: Background: The median life expectancy of cystic fibrosis (CF) patients has increased dramatically over the last few years and we now observe a subset of patients with a body mass index (BMI...
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Lise Coderre,
Christophe Fadainia,
Linda Belson,
Virginie Belisle,
Sophie Ziai,
Geneviève Maillhot,
Yves Berthiaume,
Rémi Rabasa-Lhoret
et al.
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Full-length article
(In Press Corrected Proof)
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16 April 2012 |
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Immunomodulatory activity of vardenafil on induced lung inflammation in cystic fibrosis mice
DOI: 10.1016/j.jcf.2012.03.003
Abstract: Background: We tested the hypothesis that vardenafil, a common drug used for improving erectile dysfunction and able to partially normalize transepithelial chloride transport in cystic fibro...
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Bob Lubamba,
François Huaux,
Jean Lebacq,
Etienne Marbaix,
Barbara Dhooghe,
Nadtha Panin,
Pierre Wallemacq,
Teresinha Leal
et al.
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Full-length article
(In Press Corrected Proof)
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13 April 2012 |
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The ease of breathing test tracks clinical changes in cystic fibrosis
DOI: 10.1016/j.jcf.2012.03.005
Abstract: Introduction: The EOB is a measure of dyspnea that correlates with CF pulmonary function and exercise tolerance, but has not been shown to track clinical changes. Methods: The EOB was admini...
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Traci Kazmerski,
David M. Orenstein
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Full-length article
(In Press Corrected Proof)
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11 April 2012 |
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Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) in Chinese patients with congenital bilateral absence of vas deferens
DOI: 10.1016/j.jcf.2012.01.005
Abstract: Background: Genetic testing of the cystic fibrosis transmembrane conductance (CFTR) gene is currently performed in patients with congenital bilateral absence of vas deferens (CBAVD). This st...
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Hongjun Li,
Qiaolian Wen,
Hanzhong Li,
Lixi Zhao,
Xinyu Zhang,
Jing Wang,
Longfei Cheng,
Jingwen Yang,
Si Chen,
Xu Ma,
Binbin Wang
et al.
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Full-length article
(In Press Corrected Proof)
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09 April 2012 |
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Right ventricular dysfunction in adolescents with mild cystic fibrosis
DOI: 10.1016/j.jcf.2012.03.002
Abstract: Background: In cystic fibrosis (CF) patients the right ventricle (RV) suffers a progressive deterioration, but it is not clear when these changes begin. The aim of this study was to analyze ...
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Antonio Baño-Rodrigo,
Antonio Salcedo-Posadas,
Jose R. Villa-Asensi,
Amalia Tamariz-Martel,
Alejandro Lopez-Neyra,
Elena Blanco-Iglesias
et al.
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Full-length article
(In Press Corrected Proof)
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09 April 2012 |
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Multiple antibiotic-resistant Pseudomonas aeruginosa and lung function decline in patients with cystic fibrosis
DOI: 10.1016/j.jcf.2012.02.005
Abstract: Background: The goal of this study was to determine the association of multiple antibiotic-resistant Pseudomonas aeruginosa (MARPA) acquisition with lung function decline in patients with cy...
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Clement L. Ren,
Michael W. Konstan,
Ashley Yegin,
Lawrence Rasouliyan,
Benjamin Trzaskoma,
Wayne J. Morgan,
Warren Regelmann,
for The Scientific Advisory Group, Investigators, and Coordinators of the Epidemiologic Study of Cystic Fibrosis
et al.
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Full-length article
(In Press Corrected Proof)
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26 March 2012 |
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A heat-stable cytotoxic factor produced by Achromobacter xylosoxidans isolated from Brazilian patients with CF is associated with in vitro increased proinflammatory cytokines
DOI: 10.1016/j.jcf.2012.02.002
Abstract: Background: Recently, Achromobacter xylosoxidans has been related to chronic lung diseases in patients suffering from cystic fibrosis (CF), but its involvement has not been elucidated. Some ...
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Rebeca P. Mantovani,
Carlos E. Levy,
Tomomasa Yano
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Full-length article
(In Press Corrected Proof)
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22 March 2012 |
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Results of antibiotic susceptibility testing do not influence clinical outcome in children with cystic fibrosis
DOI: 10.1016/j.jcf.2012.02.006
Abstract: Introduction: Patients with CF experience pulmonary exacerbations. These are often initially empirically treated with intravenous antibiotics, with antibiotic choice refined after susceptibi...
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M.N. Hurley,
A.H. Amin Ariff,
C. Bertenshaw,
J. Bhatt,
A.R. Smyth
et al.
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Full-length article
(In Press Corrected Proof)
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21 March 2012 |
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Cost-effectiveness of carrier screening for cystic fibrosis in Australia
DOI: 10.1016/j.jcf.2012.02.007
Abstract: Background: Carrier screening for cystic fibrosis is not widely available in Australia, partly due to concerns regarding its cost-effectiveness. The benefit of information from pregnancy to ...
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Richard Norman,
Kees van Gool,
Jane Hall,
Martin Delatycki,
John Massie
et al.
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Full-length article
(In Press Corrected Proof)
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19 March 2012 |
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Bloodstream infections in cystic fibrosis: Nine years of experience in both adults and children
DOI: 10.1016/j.jcf.2012.02.004
Abstract: Background: We report the aetiology and outcome of bloodstream infections (BSI) occurring at two regional cystic fibrosis (CF) centres (one adult, one paediatric) between 1998 and 2006. Meth...
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James Cargill,
Christine Etherington,
Daniel Peckham,
Steven Conway,
Miles Denton
et al.
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Short communication
(In Press Corrected Proof)
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14 March 2012 |
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Anti-inflammatory effects of DX-890, a human neutrophil elastase inhibitor
DOI: 10.1016/j.jcf.2012.02.003
Abstract: Background: Neutrophil elastase (NE)-mediated inflammation contributes to lung damage in cystic fibrosis (CF). We investigated if DX-890, a small-protein NE inhibitor, could reduce neutrophi...
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Fiona K. Dunlevy,
S. Lorraine Martin,
Francine de Courcey,
J. Stuart Elborn,
Madeleine Ennis
et al.
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Full-length article
(In Press Corrected Proof)
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14 March 2012 |
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Clinical evidence that V456A is a Cystic Fibrosis causing mutation in South Asians
DOI: 10.1016/j.jcf.2012.02.001
Abstract: Background: Cystic Fibrosis (CF) genotypes in South Asians are variable with a decreased incidence of Delta F508 and an increased incidence of novel mutations. The objective of this study is...
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L. Uppaluri,
S.J. England,
T.F. Scanlin
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Full-length article
(In Press Corrected Proof)
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07 March 2012 |
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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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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 |