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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/28822728/variability-of-lung-clearance-index-in-clinically-stable-cystic-fibrosis-lung-disease-in-school-age-children
#1
Marcus Svedberg, Per M Gustafsson, Paul D Robinson, Monica Rosberg, Anders Lindblad
BACKGROUND: Data on long term variability of Lung Clearance Index (LCI) in Cystic Fibrosis (CF) is urgently needed to guide test result interpretation. Our aim was to evaluate LCI variability in clinically stable CF lung disease in school age children. METHODS: Paediatric patients, aged 6 to 17years, attending the outpatient CF clinic performed Multiple Breath Nitrogen Washout (Exhalyzer® D) and spirometry every third month over a period of one year. Clinical stability was assessed by the Cystic Fibrosis Clinical Score (CFCS) at each visit...
August 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28822727/exploring-probiotic-use-in-a-regional-cystic-fibrosis-consortium
#2
LETTER
K D Gonzalez, J B Zuckerman, E H Sears, B S Prato, M Guill, W Craig, C Milliard, E Parker, T Lever, M M Griffin, L W Leclair
No abstract text is available yet for this article.
August 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28811149/three-center-feasibility-of-lung-clearance-index-in-infants-and-preschool-children-with-cystic-fibrosis-and-other-lung-diseases
#3
Mirjam Stahl, Simon Y Graeber, Cornelia Joachim, Sandra Barth, Isabell Ricklefs, Gesa Diekmann, Matthias V Kopp, Lutz Naehrlich, Marcus A Mall
BACKGROUND: Lung clearance index (LCI) detects early ventilation inhomogeneity and has been suggested as sensitive endpoint in multicenter intervention trials in infants and preschoolers with cystic fibrosis (CF). However, the feasibility of multicenter LCI in this age group has not been determined. We, therefore, investigated the feasibility of LCI in infants and preschoolers with and without CF in a three-center setting. METHODS: Following central training, standardized SF6-MBW measurements were performed in 73 sedated children (10 controls, 49 with CF and 14 with other lung diseases), mean age 2...
August 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28803050/the-arest-cf-experience-in-biobanking-more-than-just-tissues-tubes-and-time
#4
Luke W Garratt, Anthony Kicic, Colin Robertson, Sarath Ranganathan, Peter D Sly, Stephen M Stick
Research to further improve outcomes for people with CF is dependent upon well characterised, archived and accessible clinical specimens. The recent article by Beekman et al. published in Journal of Cystic Fibrosis summarised a scientific meeting at the 13th ECFS Basic Science Conference. This meeting discussed how well-annotated, clinical biobanks for CF could be established in Europe to meet the needs of therapeutic development. The Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) has conducted biobanking of CF research and clinical specimens since the late 1990s and is custodian of the most comprehensive paediatric CF biobank in the world that focuses on the first years of life...
August 9, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28784288/exercise-and-its-varied-impact-on-airway-dynamics
#5
LETTER
Daniel Stevens
No abstract text is available yet for this article.
August 4, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28781231/interpretation-of-cystic-fibrosis-centre-rankings-meaningful-comparisons-or-biased-statistics
#6
EDITORIAL
Sanja Stanojevic
No abstract text is available yet for this article.
August 4, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28774749/sexual-and-reproductive-health-behaviors-and-experiences-reported-by-young-women-with-cystic-fibrosis
#7
Traci M Kazmerski, Gregory S Sawicki, Elizabeth Miller, Kelley A Jones, Kaleab Z Abebe, Lisa K Tuchman, Sigrid Ladores, Ronald C Rubenstein, Scott D Sagel, Daniel J Weiner, Joseph M Pilewski, David M Orenstein, Sonya Borrero
BACKGROUND: The prevalence of general and disease-specific sexual and reproductive health (SRH) concerns is unknown in the United States (U.S.) female CF population. This study aimed to describe and compare the SRH experiences and behaviors of young women with CF with the general U.S. POPULATION: HASH(0x3c17c50) METHODS: Young women with CF ages 15-24years from five geographically diverse U.S. CF centers participated in a survey investigating SRH...
August 1, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28765072/poor-recovery-from-a-pulmonary-exacerbation-does-not-lead-to-accelerated-fev1-decline
#8
Don B Sanders, Zhanhai Li, Qianqian Zhao, Philip M Farrell
BACKGROUND: Patients with CF treated for pulmonary exacerbations (PEx) may experience faster subsequent declines in FEV1. Additionally, incomplete recovery to baseline FEV1 occurs frequently following PEx treatment. Whether accelerated declines in FEV1 are preceded by poor PEx recovery has not been studied. METHODS: Using 2004 to 2011 CF Foundation Patient Registry data, we randomly selected one PEx among patients ≥6years of age with no organ transplantations, ≥12months of data before and after the PEx, and ≥1 FEV1 recorded within the 6months before and 3months after the PEx...
July 29, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28757079/tracheal-diverticula-in-advanced-cystic-fibrosis-prevalence-features-and-outcomes-after-lung-transplantation
#9
Siddhartha G Kapnadak, Gregory A Kicska, Kathleen J Ramos, Desiree A Marshall, Tamara Y Carroll, Sudhakar N Pipavath, Michael S Mulligan, Christopher H Goss, Moira L Aitken
BACKGROUND: Tracheal diverticula (TD) are rare anomalies that may harbor infected secretions, posing potential risk to patients with lung disease. In an end-stage cystic fibrosis (CF) cohort, we describe the characteristics and associated post-lung transplant (LTx) outcomes of TD. METHODS: Pre-transplant computed tomography (CT)'s were reviewed in CF patients undergoing LTx. TD were characterized radiographically and on autopsy when available. Pre-transplant clinical variables and post-transplant outcomes were compared by TD status...
July 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28754328/age-related-levels-of-fecal-m2-pyruvate-kinase-in-children-with-cystic-fibrosis-and-healthy-children-0-to-10years-old
#10
Millie Garg, Steven T Leach, Tamara Pang, Bronwen Needham, Michael J Coffey, Tamarah Katz, Roxanne Strachan, John Widger, Penelope Field, Yvonne Belessis, Sandra Chuang, Andrew S Day, Adam Jaffe, Chee Y Ooi
BACKGROUND: The pathogenesis of gut inflammation, bacterial dysbiosis and increased rates of malignancy in CF is unclear. Fecal M2-pyruvate kinase (M2-PK) is a biomarker indicative of cellular proliferation that may be raised in intestinal malignancy and inflammation. Biomarkers, including M2-PK, may be useful in assessing effects of novel therapies on the gastrointestinal tract. METHODS: M2-PK was measured in stools collected from patients with CF and HC (0-10years)...
July 25, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28751115/comment-on-data-sparsity-diagnostic-and-prognostic-significance-of-systemic-alkyl-quinolones-for-p-aeruginosa-in-cystic-fibrosis-a-longitudinal-study
#11
LETTER
Erfan Ayubi, Jamal Hallajzadeh, Saeid Safiri
No abstract text is available yet for this article.
July 24, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28743561/increased-prevalence-of-colonic-adenomas-in-patients-with-cystic-fibrosis
#12
Mehdi Hegagi, Shawn D Aaron, Paul James, Rakesh Goel, Avijit Chatterjee
BACKGROUND: Cystic fibrosis (CF) is the most common lethal genetic illness in the Caucasian population. Studies have shown that CF patients are at an elevated risk of developing colon cancer. Colonic adenomas are the precursors of colon cancer. This study aims to determine the prevalence of adenomas in patients with cystic fibrosis. METHODS: All patients were recruited prospectively at The Ottawa Hospital Cystic Fibrosis Clinic from 2010 through 2015. Baseline demographic and cystic fibrosis disease characteristics were collected from the clinic's CF patient database...
July 22, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28739210/bile-salt-stimulated-lipase-inhibition-by-phospholipids-and-relief-by-phospholipase-a2
#13
Elena Venuti, Dmitry Shishmarev, Philip W Kuchel, Shoma Dutt, Caron S Blumenthal, Kevin J Gaskin
INTRODUCTION: Bile salt stimulated lipase (BSSL; Enzyme Commission (EC) number 3.1.1.13) has been a candidate triglyceridase for improving enzyme therapy for pancreatic insufficiency; however, its efficacy is near absent. We hypothesise that similarly to pancreatic lipase, BSSL is inhibited by phospholipids and this inhibition is relieved by Phospholipase A2 (PLA2; EC 3.1.1.4), and the present study was undertaken to explore this possibility. MATERIALS AND METHODS: Synthetic emulsions of triglyceride and phosphatidylcholine (PC) or lysophosphatidylcholine (LPC)/bile salt mixed micelles were used as a model of intestinal digestion-media...
July 21, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28739209/analytical-and-biological-variation-in-repeated-sweat-chloride-concentrations-in-clinical-trials-for-cftr-modulator-therapy
#14
V A LeGrys, T C Moon, J Laux, M J Rock, F Accurso
BACKGROUND: Using sweat chloride as a biomarker for CFTR modifying drugs requires knowledge of analytical and biological variation. METHODS: 979 sweat chloride concentrations from 128 subjects enrolled in the placebo arm of 2 multicenter, investigational drug trials were analyzed to determine coefficients of variation (CV) as well as reference change value (RCV) and index of individuality (II). RESULTS: For these populations, calculated values for the two studies were: analytical variation (3...
July 21, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28732801/bicarbonate-in-cystic-fibrosis
#15
REVIEW
Karl Kunzelmann, Rainer Schreiber, Hans Beat Hadorn
BACKGROUND: Cystic fibrosis (CF, mucoviscidosis) is caused by mutations in the gene encoding CF transmembrane conductance regulator (CFTR), which is a chloride and bicarbonate channel necessary for fluid secretion and extracellular alkalization. For a long time, research concentrated on abnormal Cl(-) and Na(+) transport, but neglected bicarbonate as a crucial factor in CF. METHODS: The present short review reports early findings as well as recent insights into the role of CFTR for bicarbonate transport and its defects in CF...
July 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28720345/the-potential-of-phage-therapy-in-cystic-fibrosis-essential-human-bacterial-phage-interactions-and-delivery-considerations-for-use-in-pseudomonas-aeruginosa-infected-airways
#16
REVIEW
Stephanie Trend, Angela M Fonceca, William G Ditcham, Anthony Kicic, Arest Cf
As antimicrobial-resistant microbes become increasingly common and a significant global issue, novel approaches to treating these infections particularly in those at high risk are required. This is evident in people with cystic fibrosis (CF), who suffer from chronic airway infection caused by antibiotic resistant bacteria, typically Pseudomonas aeruginosa. One option is bacteriophage (phage) therapy, which utilises the natural predation of phage viruses upon their host bacteria. This review summarises the essential and unique aspects of the phage-microbe-human lung interactions in CF that must be addressed to successfully develop and deliver phage to CF airways...
July 15, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28716479/lower-exhaled-nitric-oxide-in-infants-with-cystic-fibrosis-compared-to-healthy-controls
#17
Insa Korten, Margot Liechti, Florian Singer, Gaudenz Hafen, Isabelle Rochat, Pinelopi Anagnostopoulou, Dominik Müller-Suter, Jakob Usemann, Alexander Moeller, Urs Frey, Philipp Latzin, Carmen Casaulta
Exhaled nitric oxide (FENO) is a well-known, non-invasive airway biomarker. In patients with Cystic Fibrosis (CF) FENO is decreased. To understand if reduced FENO is primary related to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) dysfunction or an epiphenomenon of chronic inflammation, we measured FENO in 34 infants with CF prior to clinical symptoms and in 68 healthy controls. FENO was lower in CF compared to controls (p=0.0006) and the effect was more pronounced in CF infants without residual CFTR function (p<0...
July 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28712885/detection-of-cftr-function-and-modulation-in-primary-human-nasal-cell-spheroids
#18
John J Brewington, Erin T Filbrandt, F J LaRosa, Alicia J Ostmann, Lauren M Strecker, Rhonda D Szczesniak, John P Clancy
BACKGROUND: Expansion of CFTR modulators to patients with rare/undescribed mutations will be facilitated by patient-derived models quantifying CFTR function and restoration. We aimed to generate a personalized model system of CFTR function and modulation using non-surgically obtained nasal epithelial cells (NECs). METHODS: NECs obtained by curettage from healthy volunteers and CF patients were expanded and grown in 3-dimensional culture as spheroids, characterized, and stimulated with cAMP-inducing agents to activate CFTR...
July 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28711222/retrospective-observational-study-of-french-patients-with-cystic-fibrosis-and-a-gly551asp-cftr-mutation-after-1-and-2years-of-treatment-with-ivacaftor-in-a-real-world-setting
#19
Dominique Hubert, Clémence Dehillotte, Anne Munck, Valérie David, Jinmi Baek, Laurent Mely, Stéphane Dominique, Sophie Ramel, Isabelle Danner Boucher, Sylvaine Lefeuvre, Quitterie Reynaud, Virginie Colomb-Jung, Prissile Bakouboula, Lydie Lemonnier
BACKGROUND: Ivacaftor has been shown to improve lung function and body weight in patients with CF and a gating mutation. Real-world evaluation is warranted to examine its safety and effectiveness over the long term. METHODS: A retrospective observational multicentre study collected clinical data in the year before and the 2years after ivacaftor initiation in patients with CF and a Gly551Asp-CFTR mutation. RESULTS: Fifty-seven patients were included...
July 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28711221/the-evolution-of-lung-transplantation-for-cystic-fibrosis-a-2017-update
#20
REVIEW
Gregory Snell, Anna Reed, Marc Stern, Denis Hadjiliadis
Lung transplantation (LTx) is an established therapy for patients with end-stage cystic fibrosis (CF). Indeed, CF is the commonest indication for those aged<50years of age needing LTx. CF LTx is associated with a 45% 10year survival - according to the world's largest registry. It is important all otherwise suitable CF patients with severe lung disease have a timely referral for discussion and consideration of the possibility of LTx. LTx discussions must carefully consider colonisation or infection with Burkholderia cenocepacia, Mycobacterium abscessus and Scediosporium - as good LTx outcomes cannot be guaranteed...
July 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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