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Editorial
Eradication of Pseudomonas aeruginosa by early antibiotic treatment has been one of the major advances in the last decade for subjects with CF . This fantastic success has let many people to forget th...
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Gerd Döring
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1
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| Original Articles |
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Prevalence and impact on FEV1 decline of chronic methicillin-resistant Staphylococcus aureus (MRSA) colonization in patients with Cystic Fibrosis: A single-center, case control study of 165 patients
12 September 2011
Abstract: Background: Risk factors for methicillin-resistant Staphylococcus aureus (MRSA) in Cystic Fibrosis (CF) and the impact on CF disease progression are still under debate. The objectives of thi...
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E. Vanderhelst,
L. De Meirleir,
S. Verbanck,
D. Piérard,
W. Vincken,
A. Malfroot
et al.
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2-7
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Chronic Stenotrophomonas maltophilia infection and exacerbation outcomes in cystic fibrosis
22 August 2011
Abstract: Background: Chronic Stenotrophomonas maltophilia infection is a risk factor for pulmonary exacerbation in cystic fibrosis (CF) but its impact on subsequent clinical outcomes is unknown. The ...
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Valerie Waters,
Eshetu G. Atenafu,
Juliana Giraldo Salazar,
Annie Lu,
Yvonne Yau,
Larissa Matukas,
Elizabeth Tullis,
Felix Ratjen
et al.
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8-13
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Delayed publication of clinical trials in cystic fibrosis
05 September 2011
Abstract: Background: When the publication of important trial data is delayed, or data are never published, this will prevent the proper practice of evidence based medicine through robust systematic r...
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M.N. Hurley,
A.P. Prayle,
A.R. Smyth
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14-17
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Exercise improves lung function and habitual activity in children with cystic fibrosis
07 September 2011
Abstract: Background: Cystic fibrosis (CF) lung disease leads to progressive deterioration in exercise capacity. Because physical activity has been shown to improve lung function and quality of life (...
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Shruti M. Paranjape,
Laura A. Barnes,
Kathryn A. Carson,
Karen von Berg,
Holly Loosen,
Peter J. Mogayzel
et al.
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18-23
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Validation of a predictive survival model in Italian patients with cystic fibrosis
26 September 2011
Abstract: Background: In 2001 Liou published a 5-year survival model using CFF Registry data. Aims: To evaluate its validity in predicting survival in Italian CF patients. Methods: In a retrospective ...
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Roberto Buzzetti,
Gianfranco Alicandro,
Laura Minicucci,
Sara Notarnicola,
Maria Lucia Furnari,
Gabriella Giordano,
Vincenzina Lucidi,
Enza Montemitro,
Valeria Raia,
Giuseppe Magazzù,
Giuseppe Vieni,
Serena Quattrucci,
Alessandro Ferrazza,
Rolando Gagliardini,
Natalia Cirilli,
Donatello Salvatore,
Carla Colombo
et al.
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24-29
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Mild cystic fibrosis in patients with the rare P5L CFTR mutation
10 October 2011
Abstract: Over 1800 Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) mutations have been identified so far, determining different degrees of CFTR dysfunction and a range of different cy...
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Lucia Spicuzza,
Concetta Sciuto,
Lucia Di Dio,
Teresa Mattina,
Salvatore Leonardi,
Michele Miraglia del Giudice,
Mario La Rosa
et al.
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30-33
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Smoking prevention and cessation programme in cystic fibrosis: Integrating an environmental health approach
14 October 2011
Abstract: Background: There have been several studies assessing the epidemiology and effects of tobacco smoke in the cystic fibrosis (CF) population, but few address the efforts of smoking cessation i...
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Juan Antonio Ortega-García,
María Trinidad López-Fernández,
Rayden Llano,
María Dolores Pastor-Vivero,
Pedro Mondéjar-López,
Miguel Felipe Sánchez-Sauco,
Manuel Sánchez-Solís
et al.
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34-39
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| Case Study |
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Ambulatory venovenous extracorporeal respiratory support as a bridge for cystic fibrosis patients to emergent lung transplantation
31 October 2011
Abstract: Venovenous extracorporeal membrane oxygenation (VV ECMO) is a therapeutic option to bridge patients with advanced lung disease to lung transplantation. The use of VV ECMO avoids the use of m...
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Don Hayes,
Jasleen Kukreja,
Joseph D. Tobias,
Hubert O. Ballard,
Charles W. Hoopes
et al.
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40-45
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| Short Communications |
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Recurrent abdominal pain in children with cystic fibrosis: A pilot prospective longitudinal evaluation of characteristics and management
12 September 2011
Abstract: Children with cystic fibrosis commonly experience abdominal pain; however this remains poorly characterised. This prospective cross-sectional study with a longitudinal design, examined the p...
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Anne Munck,
Anne Pesle,
Cécile Cunin-Roy,
Michèle Gerardin,
Isabelle Ignace,
Bertrand Delaisi,
Chantal Wood
et al.
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46-48
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Australian epidemic strain pseudomonas (AES-1) declines further in a cohort segregated cystic fibrosis clinic
12 September 2011
Abstract: Aim: To evaluate changes in prevalence of an epidemic strain of Pseudomonas aeruginosa (AES-1, Australian epidemic strain, type 1) in a paediatric cystic fibrosis (CF) centre practising coho...
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Amanda L. Griffiths,
Danielle F. Wurzel,
Phil J. Robinson,
Rosemary Carzino,
John Massie
et al.
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49-52
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Frequency of the hyperactive W493R ENaC variant in carriers of a CFTR mutation
14 September 2011
Abstract: Background: The basic defect of the autosomal recessive disorder cystic fibrosis (CF) manifests in chloride hyposecretion and sodium hyperabsorption. CF-like disease has been reported in a h...
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Melanie Handschick,
Silke Hedtfeld,
Burkhard Tümmler
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53-55
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The prevalence of “risky behaviour” in adults with cystic fibrosis
04 October 2011
Abstract: Background: The prevalence of “risky-behaviour” including alcohol and illicit drug use, smoking and unprotected sexual intercourse, of adults with cystic fibrosis (CF) is unknown. We conduct...
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Felicity A. Mc Ewan,
Margaret E. Hodson,
Nicholas J. Simmonds
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56-58
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MALDI-TOF MS improves routine identification of non-fermenting Gram negative isolates from cystic fibrosis patients
04 October 2011
Abstract: Identification of non-fermenting Gram-negative bacteria (NFGNB) from cystic fibrosis (CF) patients is often limited. A collection of stored NFGNB isolates (n=182) recovered from CF patients ...
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Ana Fernández-Olmos,
María García-Castillo,
María-Isabel Morosini,
Adelaida Lamas,
Luis Máiz,
Rafael Cantón
et al.
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59-62
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Ancestral haplotype 8.1 and lung disease severity in European cystic fibrosis patients
12 October 2011
Abstract: Background: The clinical course of cystic fibrosis (CF) lung disease varies between patients bearing identical CFTR mutations. This suggests that additional genetic modifiers may contribute ...
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Harriet Corvol,
Julie Beucher,
Pierre-Yves Boëlle,
Pierre-François Busson,
Céline Muselet-Charlier,
Annick Clement,
Felix Ratjen,
Hartmut Grasemann,
Judith Laki,
Colin N.A. Palmer,
J. Stuart Elborn,
Anil Mehta
et al.
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63-67
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Hypothesis: Cystic fibrosis carrier geography reflects interactions of tuberculosis and hypertension with vitamin D deficiency, altitude and temperature. Vitamin D deficiency effects and cf carrier advantage
04 October 2011
Abstract: Interactions between selective factors (hypertension and tuberculosis) and environmental effects (vitamin D deficiency [VDD], temperature, and altitude) largely explain cystic fibrosis (CF) ...
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Mark Lubinsky
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68-70
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| Letters to the Editor |
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Staphylococcus aureus and cystic fibrosis
24 August 2011
I read with great interest the review entitled “Staphylococcus aureus and MRSA in cystic fibrosis” by Goss and Muhlebach . They included important information regarding the current knowledge on the ci...
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Marcelo J. Mimica
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71
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Ursodeoxycholic acid in cystic fibrosis-associated liver disease
24 August 2011
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Chee Y. Ooi,
Scott Nightingale,
Peter R. Durie,
Steven D. Freedman
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72-73
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Response to the letter by Ooi et al.
19 October 2011
We read with interest the letter from Dr. CY Ooi and Coll. pointing out that more clinical and experimental evidence is needed before firm recommendations concerning long-term use of UDCA in CFLD are ...
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Carla Colombo,
Dominique Debray,
Deirdre Kelly,
Roderick Houwen,
Pier Maria Battezzati,
Birgitta Strandvik
et al.
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74-75
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