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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/28087241/chest-imaging-in-cf-studies-commentary
#1
EDITORIAL
Irmgard Eichler, Efthymios Manolis
No abstract text is available yet for this article.
January 10, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28043799/the-expansion-and-performance-of-national-newborn-screening-programmes-for-cystic-fibrosis-in-europe
#2
Jürg Barben, Carlo Castellani, Jeannette Dankert-Roelse, Silvia Gartner, Nataliya Kashirskaya, Barry Linnane, Sarah Mayell, Anne Munck, Dorota Sands, Olaf Sommerburg, Simon Pybus, Victoria Winters, Kevin W Southern
BACKGROUND: Newborn screening (NBS) for cystic fibrosis (CF) is a well-established public health strategy with international standards. The aim of this study was to provide an update on NBS for CF in Europe and assess performance against the standards. METHODS: Questionnaires were sent to key workers in each European country. RESULTS: In 2016, there were 17 national programmes, 4 countries with regional programmes and 25 countries not screening in Europe...
December 30, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28040480/non-invasive-prenatal-diagnosis-nipd-of-cystic-fibrosis-an-optimized-protocol-using-memo-fluorescent-pcr-to-detect-the-p-phe508del-mutation
#3
C Guissart, C Dubucs, C Raynal, A Girardet, F Tran Mau Them, V Debant, C Rouzier, A Boureau-Wirth, E Haquet, J Puechberty, E Bieth, D Dupin Deguine, P Khau Van Kien, M P Brechard, V Pritchard, M Koenig, M Claustres, M C Vincent
BACKGROUND: Analysis of cell-free foetal DNA (cff-DNA) in maternal plasma is very promising for early diagnosis of monogenic diseases; in particular, cystic fibrosis (CF). However, NIPD of single-gene disorders has been limited by the availability of suitable technical platforms and the need to set up patient or disease-specific custom-made approaches. METHODS: To make research applications more readily accessible to the clinic, we offer a simple assay combining two independent methods to determine the presence or absence of paternally inherited foetal allele p...
December 28, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28040479/chest-imaging-in-cystic-fibrosis-studies-what-counts-and-can-be-counted
#4
REVIEW
Rhonda Szczesniak, Lidija Turkovic, Eleni-Rosalina Andrinopoulou, Harm A W M Tiddens
BACKGROUND: The dawn of precision medicine and CFTR modulators require more detailed assessment of lung structure in cystic fibrosis (CF) clinical studies. Various imaging markers have emerged and are measurable, but clarity is needed to identify what markers should count for clinical studies. High-resolution chest computed tomography (CT) scoring has yielded sensitive markers for the study of CF disease progression. Once completed, CT scores from ongoing randomized controlled trials can be used to examine relationships between imaging endpoints and therapeutic effectiveness...
December 28, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28025037/impact-of-azithromycin-on-the-clinical-and-antimicrobial-effectiveness-of-tobramycin-in-the-treatment-of-cystic-fibrosis
#5
Dave P Nichols, Carrie L Happoldt, Preston E Bratcher, Silvia M Caceres, James F Chmiel, Kenneth C Malcolm, Milene T Saavedra, Lisa Saiman, Jennifer L Taylor-Cousar, Jerry A Nick
BACKGROUND: Concomitant use of oral azithromycin and inhaled tobramycin occurs in approximately half of US cystic fibrosis (CF) patients. Recent data suggest that this combination may be antagonistic. METHODS: Test the hypothesis that azithromycin reduces the clinical benefits of tobramycin by analyses of clinical trial data, in vitro modeling of P. aeruginosa antibiotic killing, and regulation of the MexXY efflux pump. RESULTS: Ongoing administration of azithromycin associates with reduced ability of inhaled tobramycin, as compared with aztreonam, to improve lung function and quality of life in a completed clinical trial...
December 23, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28017620/biological-variability-of-the-sweat-chloride-in-diagnostic-sweat-tests-a-retrospective-analysis
#6
F Vermeulen, P Lebecque, K De Boeck, T Leal
BACKGROUND: The sweat test is the current gold standard for the diagnosis of cystic fibrosis (CF). CF is unlikely when sweat chloride (Clsw) is lower than 30mmol/L, Clsw>60 is suggestive of CF, with intermediate values between 30 and 60mmol/L. To correctly interpret a sweat chloride value, the biological variability of the sweat chloride has to be known. METHODS: Sweat tests performed in two centers using the classic Gibson and Cooke method were retrospectively reviewed (n=5904)...
December 22, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28007460/mri-accelerating-progress-in-functional-assessment-of-cystic-fibrosis-lung-disease
#7
EDITORIAL
Mark O Wielpütz, Marcus A Mall
No abstract text is available yet for this article.
December 19, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27989470/differences-between-who-and-cdc-early-growth-measurements-in-the-assessment-of-cystic-fibrosis-clinical-outcomes
#8
Danielle Usatin, Elizabeth H Yen, Catherine McDonald, Fadi Asfour, John Pohl, Jacob Robson
BACKGROUND: Early childhood growth status has been used to predict long-term clinical outcomes in Cystic Fibrosis (CF) patients. Adulthood CF outcomes based on early weight-for-length (WFL) measurements, using either World Health Organization (WHO) or Centers for Disease Control (CDC) scales, have not been compared. METHODS: Cystic Fibrosis Foundation registry patients were studied (n=3014). Participants were categorized at age two years as WFL <50th percentile on both WHO and CDC scales, ≥50th percentile on WHO but not CDC, or ≥50th percentile on both...
December 15, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27988297/increasing-sputum-levels-of-gamma-glutamyltransferase-may-identify-cystic-fibrosis-patients-who-do-not-benefit-from-inhaled-glutathione
#9
Alessandro Corti, Matthias Griese, Andreas Hector, Alfonso Pompella
Glutathione (GSH) is decreased in cystic fibrosis (CF) airways, thus its resupply by inhalation has been employed to restore antioxidant defense. CF airways present however increased activity of gamma-glutamyltransferase (GGT), the enzyme specifically capable of degrading GSH, and thus inhaled GSH might be promptly catabolized. In addition, prooxidant reactions are known to originate during GGT-mediated GSH catabolism. We determined levels of GGT in the sputum samples obtained from a previously published trial of GSH inhalation treatment, and analyzed their correlations with inflammatory markers and FEV1% values...
December 14, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27986495/current-strategies-for-the-long-term-assessment-monitoring-and-management-of-cystic-fibrosis-patients-treated-with-cftr-modulator-therapy
#10
J Stuart Elborn, Jane Davies, Marcus A Mall, Patrick A Flume, Barry Plant
The content for this activity is based on the satellite symposium, "Current Strategies for the Long-term Assessment, Monitoring, and Management for Cystic Fibrosis Patients Treated with CFTR Modulator Therapy" that was presented at the 39th European Cystic Fibrosis Society Conference on June 10, 2016 (Online access: http://courses.elseviercme.com/ecfs2016e/619e). The emergence of novel targeted agents, that directly correct CFTR loss function alleles, has created new treatment opportunities for patients with cystic fibrosis with advanced disease...
December 13, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27986494/the-psychometric-properties-of-the-leicester-cough-questionnaire-and-respiratory-symptoms-in-cf-tool-in-cystic-fibrosis-a-preliminary-study
#11
Nathan Ward, Kathy Stiller, Hilary Rowe, Anne E Holland
BACKGROUND: There are few tools to quantify the impact of cough in cystic fibrosis (CF). The psychometric properties of the Leicester Cough Questionnaire (LCQ) and Respiratory Symptoms in CF (ReS-CF) tool were investigated in adults with CF. METHODS: Validity and reliability were assessed in clinically stable participants who completed the questionnaires twice, along with the Cystic Fibrosis Questionnaire - Revised (CFQ-R). Responsiveness was assessed by change in questionnaires following treatment for an acute respiratory exacerbation...
December 13, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27979723/pleural-effusions-in-non-transplanted-cystic-fibrosis-patients
#12
Adam R Belanger, Kimtuyen Nguyen, Umar Osman, Christopher R Gilbert, Katie Allen, Ahmad Farid Al Rais, Lonny Yarmus, Jason A Akulian
BACKGROUND: Pleural effusions are considered rare in cystic fibrosis (CF) patients. There is a paucity of available information in the literature concerning the nature and significance of pleural effusions in non-transplanted CF patients. METHODS: We conducted a multicenter retrospective evaluation of non-transplanted adult CF patients. Given the small sample size, only descriptive statistics were performed. RESULTS: A total of 17 CF patients with pleural effusion were identified, of whom 9 patients underwent thoracentesis...
December 12, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27965061/cftr-nonsense-mutations-therapeutic-benefits-from-clinically-approved-drugs
#13
EDITORIAL
J Kevin Foskett
No abstract text is available yet for this article.
December 10, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27964834/variability-of-sweat-chloride-a-never-ending-story
#14
EDITORIAL
Burkhard Tümmler
No abstract text is available yet for this article.
December 7, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27939048/stroke-and-myocardial-infarction-following-bronchial-artery-embolization-in-a-cystic-fibrosis-patient
#15
LETTER
Matt Hoffman, Joseph P Gerding, Jonathan B Zuckerman
No abstract text is available yet for this article.
December 6, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27919570/agreement-between-multiple-breath-nitrogen-washout-systems-in-children-and-adults
#16
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
#17
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
#18
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
#19
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
#20
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
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