journal
MENU ▼
Read by QxMD icon Read
search

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

journal
https://www.readbyqxmd.com/read/29661511/developments-in-cystic-fibrosis-personalised-epithelial-assays-science-and-patient-perspectives
#1
EDITORIAL
Gerard E Kaiko, Peter A B Wark
No abstract text is available yet for this article.
April 13, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29661510/cftr-rescue-with-vx-809-and-vx-770-favors-the-repair-of-primary-airway-epithelial-cell-cultures-from-patients-with-class-ii-mutations-in-the-presence-of-pseudomonas-aeruginosa-exoproducts
#2
Damien Adam, Claudia Bilodeau, Laura Sognigbé, Émilie Maillé, Manon Ruffin, Emmanuelle Brochiero
BACKGROUND: Progressive airway damage due to bacterial infections, especially with Pseudomonas aeruginosa remains the first cause of morbidity and mortality in CF patients. Our previous work revealed a repair delay in CF airway epithelia compared to non-CF. This delay was partially prevented after CFTR correction (with VRT-325) in the absence of infection. Our goals were now to evaluate the effect of the Orkambi combination (CFTR VX-809 corrector + VX-770 potentiator) on the repair of CF primary airway epithelia, in infectious conditions...
April 13, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29655935/establishing-the-diagnosis-of-chronic-colonization-with-pseudomonas-aeruginosa-of-cystic-fibrosis-patients-comparison-of-the-european-consensus-criteria-with-genotyping-of-p-aeruginosa-isolates
#3
Leander Jonckheere, Petra Schelstraete, Leen Van Simaey, Eva Van Braeckel, Julie Willekens, Sabine Van Daele, Frans De Baets, Mario Vaneechoutte
After antibiotic eradication treatment for a first ever Pseudomonas aeruginosa isolation, the European consensus criteria (ECC) are widely used to assess colonization status with P. aeruginosa in CF-patients. We evaluated to what extent genotyping (GT) of subsequent P. aeruginosa isolates could predict/assess chronic colonization (CC), in comparison with the ECC. METHODS: Over a 14-year period, sputa were cultured from 80 CF-patients (age range: 2-51 years), from a first ever isolation of P...
April 11, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29631774/children-with-cystic-fibrosis-demonstrate-no-respiratory-immunological-infective-or-physiological-consequences-of-vitamin-d-deficiency
#4
Rebecca M Thursfield, Khayam Naderi, Neil Leaver, Mark Rosenthal, Eric W F W Alton, Andrew Bush, Jane C Davies
BACKGROUND: Vitamin D has health benefits in many respiratory diseases but the evidence in CF is unclear. Induction of the antimicrobial peptides cathelicidin (LL37) and human-beta-defensin-2 (HBD-2) may be the mechanism of any benefit. We hypothesised that antimicrobial peptide levels would be decreased, and airway infection and inflammation greater, in CF children with vitamin D deficiency. The objective of the study was to explore relationships between vitamin D, LL37 and HBD-2, and airway infection, inflammation and physiology in children with CF...
April 6, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29606526/prevalence-of-severe-fatigue-among-adults-with-cystic-fibrosis-a-single-center-study
#5
Merel M Nap-van der Vlist, Marcella Burghard, H J Hulzebos, Wytze R Doeleman, Harry G M Heijerman, Cornelis K van der Ent, Sanne L Nijhof
BACKGROUND: With life expectancy increasing among patients with cystic fibrosis (CF), the prevalence of complications such as fatigue is also expected to increase. Our aim was to investigate the prevalence of severe fatigue among adults with CF and to identify factors associated with fatigue. METHODS: Adult patients with CF receiving treatment at a single center were invited to complete three questionnaires. We then studied the associations between fatigue and clinically measured parameters and between fatigue and patient-reported outcomes...
March 29, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29602718/defining-chronic-pseudomonas-aeruginosa-infection-in-cystic-fibrosis
#6
EDITORIAL
Valerie Waters, Keith Grimwood
No abstract text is available yet for this article.
March 27, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29580829/sweat-test-for-cystic-fibrosis-wearable-sweat-sensor-vs-standard-laboratory-test
#7
Dong-Hoon Choi, Abigail Thaxton, In Cheol Jeong, Kain Kim, Patrick R Sosnay, Garry R Cutting, Peter C Searson
BACKGROUND: Sweat chloride testing for diagnosis of cystic fibrosis (CF) involves sweat induction, collection and handling, and measurement in an analytical lab. We have developed a wearable sensor with an integrated salt bridge for real-time measurement of sweat chloride concentration. Here, in a proof-of-concept study, we compare the performance of the sensor to current clinical practice in CF patients and healthy subjects. METHOD: Sweat was induced on both forearms of 10 individuals with CF and 10 healthy subjects using pilocarpine iontophoresis...
March 23, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29580828/continuous-glucose-monitoring-abnormalities-in-cystic-fibrosis-youth-correlate-with-pulmonary-function-decline
#8
Christine L Chan, Timothy Vigers, Laura Pyle, Philip S Zeitler, Scott D Sagel, Kristen J Nadeau
BACKGROUND: To characterize glucose patterns with continuous glucose monitoring (CGM) in cystic fibrosis (CF) and assess relationships between CGM and clinical outcomes. METHODS: 110 CF youth and healthy controls (HC), 10-18 years, wore CGM up to 7 days. Correlations between CGM and lung function and BMI z-score change over the prior year were determined. RESULTS: Multiple CGM measures were higher in CF Normal Glycemic (CFNG) youth versus HC (peak glucose, excursions >140 mg/dl/day, %time > 140 mg/dl, standard deviation (SD) and mean amplitude of glycemic excursions (MAGE))...
March 23, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29572018/strategies-for-newborn-screening-for-cystic-fibrosis-a-systematic-review-of-health-economic-evaluations
#9
REVIEW
Masja Schmidt, Amber Werbrouck, Nick Verhaeghe, Elke De Wachter, Steven Simoens, Lieven Annemans, Koen Putman
BACKGROUND: Early detection of cystic fibrosis through newborn screening has significant clinical benefits. Cost-effectiveness plays an important role in selecting the optimal screening strategy from the many available options. OBJECTIVES: The objectives of this study are (1) to summarize study estimates of cost-effectiveness of cystic fibrosis newborn screening (CFNBS) strategies as compared to other strategies, (2) to assess the quality of the studies identified, and (3) to identify determinants of cost-effectiveness...
March 20, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29555479/cystic-fibrosis-patient-registries-a-valuable-source-for-clinical-research
#10
REVIEW
Elliott C Dasenbrook, Gregory S Sawicki
Cystic Fibrosis (CF) patient registries are valuable data sources for researchers studying the natural history, treatment paradigms, and long-term health outcomes of individuals with CF. In this review, we discuss the role of CF patient registries in facilitating comparative effectiveness research, particularly evaluating therapies and variation in health care delivery. We also discuss the limitations of registry-based research, particularly indication bias, as well as statistical methods that can be used to address these issues...
March 16, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29551462/defining-research-priorities-in-cystic-fibrosis-can-existing-knowledge-and-training-in-biomedical-research-affect-the-choice
#11
Roberto Buzzetti, Valeria Galici, Natalia Cirilli, Fabio Majo, Luigi Graziano, Stefano Costa, Simona Bonacina, Marila Carrubba, Giuseppe Davì, Sabrina Gagliano, Clizia Cazzarolli, Francesca Ficili, Federico Alghisi, Michele Samaja, Giuseppe Magazzù
The aim of this report is to assess whether the research issues priorities are perceived differently according to the Stakeholders (SH)'s individual knowledge of research topics and degree of training in biomedical research. Four groups of SH were enrolled in this study: 1. Skilled SH, specifically trained in biomedicine; 2. Unskilled untrained SH who responded to a written questionnaire in 2015; 3. SH who were trained for one year in a course delivered by professionals; 4. Untrained SH who responded to an online questionnaire in 2017...
March 15, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29550263/understanding-of-safety-monitoring-in-clinical-trials-by-individuals-with-cf-or-their-parents-a-qualitative-analysis
#12
Andrew S Kern-Goldberger, Amanda J Hessels, Lisa Saiman, Lynne M Quittell
BACKGROUND: Recruiting both pediatric and adult participants for clinical trials in CF is currently of paramount importance as numerous new therapies are being developed. However, recruitment is challenging as parents of children with CF and adults with CF cite safety concerns as a principal barrier to enrollment. In conjunction with the CF Foundation (CFF) Data Safety Monitoring Board (DSMB), a pilot brochure was developed to inform patients and parents of the multiple levels of safety monitoring; the CFF simultaneously created an infographic representing the safety monitoring process...
March 14, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29544685/comparison-of-ex-vivo-and-in-vitro-intestinal-cystic-fibrosis-models-to-measure-cftr-dependent-ion-channel-activity
#13
Domenique D Zomer-van Ommen, Eyleen de Poel, Evelien Kruisselbrink, Hugo Oppelaar, Annelotte M Vonk, Hettie M Janssens, Cornelis K van der Ent, Marne C Hagemeijer, Jeffrey M Beekman
BACKGROUND: New functional assays using primary human intestinal adult stem cell cultures can be valuable tools to study epithelial defects in human diseases such as cystic fibrosis. METHODS: CFTR-mediated ion transport was measured in rectal organoid-derived monolayers grown from subjects with various CFTR mutations and compared to donor-matched intestinal current measurements (ICM) in rectal biopsies and forskolin-induced swelling of rectal organoids. RESULTS: Rectal organoid-derived monolayers were generated within four days...
March 13, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29525410/early-detection-using-qpcr-of-pseudomonas-aeruginosa-infection-in-children-with-cystic-fibrosis-undergoing-eradication-treatment
#14
Ana C Blanchard, Ashley M Rooney, Yvonne Yau, Yu Zhang, Patrick J Stapleton, Eric Horton, Michelle Klingel, Sanja Stanojevic, Felix Ratjen, Bryan Coburn, Valerie Waters
BACKGROUND: Infection with Pseudomonas aeruginosa (Pa) with a chronic phenotype is associated with antibiotic eradication therapy (AET) failure. Our objective was to determine whether higher levels of Pa (detected using qPCR) prior to culture positivity were associated with AET failure in pediatric CF patients. METHODS: Pa-specific qPCR was performed on stored sputa prior to culture positivity in pediatric CF patients with new-onset culture-positive Pa infections undergoing AET with a 28-day course of tobramycin-inhaled solution (TIS)...
March 7, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29523474/mini-guts-in-a-dish-perspectives-of-adult-cystic-fibrosis-cf-patients-and-parents-of-young-cf-patients-on-organoid-technology
#15
Sarah N Boers, Karin M de Winter-de Groot, Jacquelien Noordhoek, Vincent Gulmans, Cornelis K van der Ent, Johannes J M van Delden, Annelien L Bredenoord
BACKGROUND: Organoid technology enables the cultivation of human tissues in a dish. Its precision medicine potential could revolutionize the Cystic Fibrosis (CF) field. We provide a first thematic exploration of the patient perspective on organoid technology to set the further research agenda, which is necessary for responsible development of this ethically challenging technology. METHODS: 23 semi-structured qualitative interviews with 14 Dutch adult CF patients and 12 parents of young CF patients to examine their experiences, opinions, and attitudes regarding organoid technology...
March 6, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29506920/ecfs-best-practice-guidelines-the-2018-revision
#16
REVIEW
Carlo Castellani, Alistair J A Duff, Scott C Bell, Harry G M Heijerman, Anne Munck, Felix Ratjen, Isabelle Sermet-Gaudelus, Kevin W Southern, Jurg Barben, Patrick A Flume, Pavla Hodková, Nataliya Kashirskaya, Maya N Kirszenbaum, Sue Madge, Helen Oxley, Barry Plant, Sarah Jane Schwarzenberg, Alan R Smyth, Giovanni Taccetti, Thomas O F Wagner, Susan P Wolfe, Pavel Drevinek
Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe...
March 2, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29503039/implementing-the-international-committee-on-mental-health-in-cystic-fibrosis-icmh-guidelines-screening-accuracy-and-referral-treatment-pathways
#17
Marieke Verkleij, Derek de Winter, Margaret Anne Hurley, Janice Abbott
BACKGROUND: The International Committee on Mental Health (ICMH) published screening guidelines in Cystic Fibrosis (CF). This work 1) evaluated the sensitivity of the recommended screening tools against the 'gold standard' clinical psychological assessment and 2) investigated referral and treatment pathways. METHODS: Ninety-six participants (79 caregivers; 17 adolescents with CF) completed the screening tools prior to formal assessment. Agreement between screening data and psychological assessment was evaluated, sensitivity analyses performed and referral pathways tracked...
March 1, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29486923/cystic-fibrosis-epithelial-cells-are-primed-for-apoptosis-as-a-result-of-increased-fas-cd95
#18
Qiwei Chen, Sudha Priya Soundara Pandi, Lauren Kerrigan, Noel G McElvaney, Catherine M Greene, J Stuart Elborn, Clifford C Taggart, Sinéad Weldon
BACKGROUND: Previous work suggests that apoptosis is dysfunctional in cystic fibrosis (CF) airways with conflicting results. We evaluated the relationship between dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR) and apoptosis in CF airway epithelial cells. METHODS: Apoptosis and associated caspase activity were analysed in non-CF and CF tracheal and bronchial epithelial cell lines. RESULTS: Basal levels of apoptosis and activity of caspase-3 and caspase-8 were significantly increased in CF epithelial cells compared to controls, suggesting involvement of extrinsic apoptosis signalling, which is mediated by the activation of death receptors, such as Fas (CD95)...
February 24, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29483003/comparison-of-facemask-and-mouthpiece-interfaces-for-multiple-breath-washout-measurements
#19
Paul D Robinson, Sooky Lum, Courtney Moore, Kate M Hardaker, Nick Benseler, Paul Aurora, Peter Cooper, Dominic Fitzgerald, Renee Jensen, Reginald McDonald, Hiran Selvadurai, Felix Ratjen, Sanja Stanojevic
BACKGROUND: Different interfaces (mouthpiece/nose clip vs. facemask) are used during multiple breath washout (MBW) tests in young children. METHODS: We investigated the effect of interface choice and breathing modalities on MBW outcomes in healthy adults and preschool children. RESULTS: In adults (n = 26) facemask breathing significantly increased LCI, compared to mouthpiece use (mean difference (95% CI) 0.4 (0.2; 0.6)), with results generalizable across sites and different equipment...
February 23, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29459038/molecular-characterization-of-gene-regulatory-networks-in-primary-human-tracheal-and-bronchial-epithelial-cells
#20
Austin E Gillen, Rui Yang, Calvin U Cotton, Aura Perez, Scott H Randell, Shih-Hsing Leir, Ann Harris
BACKGROUND: Robust methods to culture primary airway epithelial cells were developed several decades ago and these cells provide the model of choice to investigate many diseases of the human lung. However, the molecular signature of cells from different regions of the airway epithelium has not been well characterized. METHODS: We utilize DNase-seq and RNA-seq to examine the molecular signatures of primary cells derived from human tracheal and bronchial tissues, as well as healthy and diseased (cystic fibrosis (CF)) donor lung tissue...
February 17, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
journal
journal
39873
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"