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Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society

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https://www.readbyqxmd.com/read/27919570/agreement-between-multiple-breath-nitrogen-washout-systems-in-children-and-adults
#1
William Poncin, Florian Singer, Anne-Sophie Aubriot, Patrick Lebecque
BACKGROUND: Comparability of multiple breath washout (MBW) systems has been little explored. We assessed agreement in lung clearance index (LCI) from two similar, commercial nitrogen MBW setups in patients with Cystic Fibrosis (CF) and controls. METHODS: The EasyOne Pro (NDD) and Exhalyzer D (EM) were randomly applied in 85 adults (34 with CF) and 97 children (47 with CF and normal forced expiratory volume in one second). We assessed differences between setups in LCI, lung volumes and breathing pattern and diagnostic performance for detecting abnormal lung function...
December 2, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27916551/environmental-scan-of-cystic-fibrosis-research-worldwide
#2
Janice Kelly
BACKGROUND: Cystic fibrosis (CF) is a rare fatal genetic disease, affecting 70,000 to 100,000 people worldwide [1]. Numerous countries have specific charitable organizations dedicated to CF, with many funding research to find a cure or effective control for the disease. Cystic Fibrosis Canada, the largest charity in Canada dedicated to funding research and care in CF, conducted an environmental scan of these organizations to learn and better understand their research goals and funding process...
December 1, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27916550/bmi-fails-to-identify-poor-nutritional-status-in-stunted-children-with-cf
#3
Michael W Konstan, David J Pasta, Jeffrey S Wagener, Donald R VanDevanter, Wayne J Morgan
BACKGROUND: Body mass index (BMI) is currently emphasized for evaluating nutritional status in children with cystic fibrosis (CF). Weight for age (WFA) and height for age (HFA) may get less attention. METHODS: Data from the Epidemiologic Study of Cystic Fibrosis were used to compare patient WFA, HFA, and BMI percentiles for children age 2 to 18years. RESULTS: For children with BMI between the 25th and 50th percentiles, 16.8% had WFA <10th percentile and 26...
December 1, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27908697/clostridium-difficile-carriage-in-adult-cystic-fibrosis-cf-implications-for-patients-with-cf-and-the-potential-for-transmission-of-nosocomial-infection
#4
D G Burke, M J Harrison, C Fleming, M McCarthy, C Shortt, I Sulaiman, D M Murphy, J A Eustace, F Shanahan, C Hill, C Stanton, M C Rea, R P Ross, B J Plant
Clostridium difficile is an anaerobic Gram-positive, spore-forming, toxin-producing bacillus transmitted among humans through the faecal-oral route. Despite increasing carriage rates and the presence of C. difficile toxin in stool, patients with CF rarely appear to develop typical manifestations of C. difficile infection (CDI). In this study, we examined the carriage, toxin production, ribotype distribution and antibiotic susceptibility of C. difficile in a cohort of 60 adult patients with CF who were pre-lung transplant...
November 29, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27894875/effect-of-bronchodilators-in-healthy-individuals-receiving-lumacaftor-ivacaftor-combination-therapy
#5
Gautham Marigowda, Fang Liu, David Waltz
In an open-label, single-center phase 1 pharmacokinetic study in healthy subjects who received lumacaftor (LUM) in combination with ivacaftor (IVA), review of spirometry data showed a transient decline in percent predicted forced expiratory volume in 1s (ppFEV1) within 4h of drug administration. An additional cohort of healthy subjects with normal baseline ppFEV1 values was studied to evaluate the ppFEV1 response to LUM/IVA administration and assess the effect of long-acting bronchodilators (LABDs) and short-acting bronchodilators (SABDs) on ppFEV1 response...
November 25, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27889352/oxidized-glutathione-and-uric-acid-as-biomarkers-of-early-cystic-fibrosis-lung-disease
#6
Nina Dickerhof, Rufus Turner, Irada Khalilova, Emmanuelle Fantino, Peter D Sly, Anthony J Kettle
BACKGROUND: In cystic fibrosis (CF) there is an urgent need for earlier diagnosis of pulmonary infections and inflammation using blood- and urine-based biomarkers. METHODS: Using mass spectrometry, oxidation products of glutathione and uric acid were measured in matched samples of bronchoalveolar lavage (BAL), serum and urine from 36 infants and children with CF, and related to markers of neutrophilic inflammation and infection in BAL. RESULTS: Oxidation products of glutathione (glutathione sulfonamide, GSA) and uric acid (allantoin), were elevated in BAL of children with pulmonary infections with Pseudomonas aeruginosa (PsA) compared to those without (p<0...
November 24, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27888005/a-simplified-semi-quantitative-structural-lung-disease-computed-tomography-outcome-during-quiet-breathing-in-infants-with-cystic-fibrosis
#7
Magali Saguintaah, Yann Cabon, Rémi Gauthier, Cécile Duboibaudry, Laure Couderc, Muriel Le Bourgeois, Philippe Reix, Raphael Chiron, Ikram Taleb Arrada, Nicolas Molinari, Stefan Matecki
Chest tomography (CT) using the controlled ventilation technique (CTCV) is a sensitive method to detect features of lung cystic fibrosis (CF) disease in infants with CF. However, this technique needs sedation and is not easily applied for the clinician who may need, in the follow-up, to evaluate more precisely lung disease in infants with CF. Thus, our study aims to evaluate if CT assessment of lung disease, without the need of sedation, during quiet breathing, using a semi-quantitative scoring system, is reproducible and may discriminate infants with CF from control infants at an early stage of the lung disease...
November 22, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27856165/the-impact-of-impaired-macrophage-functions-in-cystic-fibrosis-disease-progression
#8
REVIEW
Manuella Lévêque, Sophie Le Trionnaire, Paola Del Porto, Corinne Martin-Chouly
The underlying cause of morbidity in cystic fibrosis (CF) is the decline in lung function, which results in part from chronic inflammation. Inflammation and infection occur early in infancy in CF and the role of innate immune defense in CF has been highlighted in the last years. Once thought simply to be consumers of bacteria, macrophages have emerged as highly sensitive immune cells that are located at the balance point between inflammation and resolution of this inflammation in CF pathophysiology. In order to assess the potential role of macrophage in CF, we review the evidence that: (1) CF macrophage has a dysregulated inflammatory phenotype; (2) CF macrophage presents altered phagocytosis capacity and bacterial killing; and (3) lipid disorders in CF macrophage affect its function...
November 14, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27842897/cystic-fibrosis-biomarker-commentary
#9
Anthony G Durmowicz
No abstract text is available yet for this article.
November 11, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27839953/bacterial-proteases-and-haemostasis-dysregulation-in-the-cf-lung
#10
James A Reihill, Michelle Moreland, Gavin E Jarvis, Andrew McDowell, Gisli G Einarsson, J Stuart Elborn, S Lorraine Martin
BACKGROUND: Pathogenic bacteria which chronically colonise the cystic fibrosis (CF) lung produce a number of virulence determinants, including distinct proteolytic activities. The potential role bacterial proteases play on haemostatic dysregulation within the CF lung is, however, poorly defined, despite haemoptysis being a common complication in CF. METHODS: The potential impact of known CF pathogens (Pseudomonas aeruginosa and Burkholderia cepacia complex spp.) on haemostasis was examined for their ability to degrade fibrinogen and dysregulate fibrin clot formation and platelet aggregation...
November 10, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27836448/multilocus-amplicon-sequencing-of-pseudomonas-aeruginosa-cystic-fibrosis-airways-isolates-collected-prior-to-and-after-early-antipseudomonal-chemotherapy
#11
Sebastian Fischer, Leonie Greipel, Jens Klockgether, Marie Dorda, Lutz Wiehlmann, Nina Cramer, Burkhard Tümmler
BACKGROUND: Early antimicrobial chemotherapy can prevent or at least delay chronic cystic fibrosis (CF) airways infections with Pseudomonas aeruginosa. METHODS: During a 10-year study period P. aeruginosa was detected for the first time in 54 CF patients regularly seen at the CF centre Hannover. Amplicon sequencing of 34 loci of the P. aeruginosa core genome was performed in baseline and post-treatment isolates of the 15 CF patients who had remained P. aeruginosa - positive after the first round of antipseudomonal chemotherapy...
November 8, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27810230/therapeutic-benefit-of-ivacaftor-in-late-cystic-fibrosis-caused-by-homozygous-ivs8-5t-cftr-polymorphism
#12
LETTER
F Magne, S Durupt, R Nove-Josserand, F Bey-Omar, L Laoust, V Cottin, I Durieu, Q Reynaud
No abstract text is available yet for this article.
October 31, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27773592/correlation-of-sweat-chloride-and-percent-predicted-fev1-in-cystic-fibrosis-patients-treated-with-ivacaftor
#13
Meredith C Fidler, Jack Beusmans, Paul Panorchan, Frederick Van Goor
Ivacaftor, a CFTR potentiator that enhances chloride transport by acting directly on CFTR to increase its channel gating activity, has been evaluated in patients with different CFTR mutations. Several previous analyses have reported no statistical correlation between change from baseline in ppFEV1 and reduction in sweat chloride levels for individuals treated with ivacaftor. The objective of the post hoc analysis described here was to expand upon previous analyses and evaluate the correlation between sweat chloride levels and absolute ppFEV1 changes across multiple cohorts of patients with different CF-causing mutations who were treated with ivacaftor...
October 20, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27773591/diagnostic-and-prognostic-significance-of-systemic-alkyl-quinolones-for-p-aeruginosa-in-cystic-fibrosis-a-longitudinal-study
#14
Helen L Barr, Nigel Halliday, David A Barrett, Paul Williams, Douglas L Forrester, Daniel Peckham, Kate Williams, Alan R Smyth, David Honeybourne, Joanna L Whitehouse, Edward F Nash, Jane Dewar, Andrew Clayton, Alan J Knox, Miguel Cámara, Andrew W Fogarty
BACKGROUND: Pulmonary P. aeruginosa infection is associated with poor outcomes in cystic fibrosis (CF) and early diagnosis is challenging, particularly in those who are unable to expectorate sputum. Specific P. aeruginosa 2-alkyl-4-quinolones are detectable in the sputum, plasma and urine of adults with CF, suggesting that they have potential as biomarkers for P. aeruginosa infection. AIM: To investigate systemic 2-alkyl-4-quinolones as potential biomarkers for pulmonary P...
October 20, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27771239/nutrition-in-cf-two-new-important-guidelines
#15
EDITORIAL
Susan P Wolfe, Michael Wilschanski
No abstract text is available yet for this article.
October 19, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27751793/equitable-cf-care-as-a-basic-human-right
#16
EDITORIAL
Kris De Boeck, Eitan Kerem
No abstract text is available yet for this article.
October 14, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27751792/coronary-artery-disease-in-cystic-fibrosis-an-emerging-concern
#17
LETTER
Kate Skolnik, Robert D Levy, Pearce G Wilcox, Bradley S Quon
No abstract text is available yet for this article.
October 14, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27745801/year-to-year-change-in-fev1-in-patients-with-cystic-fibrosis-and-different-mutation-classes
#18
K De Boeck, A Zolin
In patients with cystic fibrosis, most treatments addressing the underlying basic defect are mutation or mutation class specific. These treatments are disease modifying if they lower the year to year change in lung function. We therefore calculated the current loss of lung function, measured by year to year change in forced expired volume in 1s in 11,417 patients included in the European Cystic Fibrosis Society Patient Registry. Whereas patients with at least one mutation of class IV or V have on average a lower year to year change, we did not find a difference between patients with a stop codon mutation, homozygous for F508del or at least one class III mutation...
October 11, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27745802/bone-demineralization-is-improved-by-ivacaftor-in-patients-with-cystic-fibrosis-carrying-the-p-gly551asp-mutation
#19
Isabelle Sermet-Gaudelus, Martial Delion, Isabelle Durieu, Jacky Jacquot, Dominique Hubert
Low bone mineral density (BMD) is a common problem in adults with cystic fibrosis (CF), the etiology of which is multifactorial. In this study, we provide the first evidence that ivacaftor improves BMD in CF patients carrying the p.Gly551Asp mutation. Consistently, in vitro experiments with TNF-α-stimulated primary F508del-CFTR osteoblasts demonstrated that correction of p.Phe508del-CFTR markedly decreased RANKL protein production, a major factor of bone resorption. These clinical and fundamental observations suggest that rescue of mutated CFTR protein improves bone remodeling and support the link between CFTR and bone cell physiology...
October 10, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27727099/a-cross-sectional-analysis-of-daytime-versus-nocturnal-polysomnographic-respiratory-parameters-in-cystic-fibrosis-during-early-adolescence
#20
K A Waters, A Lowe, P Cooper, S Vella, Hiran Selvadurai
BACKGROUND: In Cystic Fibrosis (CF), early detection and treatment of respiratory disease is considered the standard for respiratory care. Overnight polysomnography (PSG) may help identify respiratory deterioration in young patients with CF. METHODS: A prospective cohort study of 46 patients with CF, aged 8-12years, from a specialist clinic in a tertiary paediatric hospital. Daytime pulmonary function, shuttle test exercise testing and overnight PSG were studied...
October 8, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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