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/30318450/authors-response-letter-to-the-editor-anaemia-and-iron-deficiency-in-relation-to-fatigue-in-cystic-fibrosis
#1
LETTER
M M Nap-van der Vlist, M Burghard, H J Hulzebos, W R Doeleman, H G M Heijerman, C K van der Ent, S L Nijhof
No abstract text is available yet for this article.
October 12, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30314936/attenuation-of-exacerbation
#2
EDITORIAL
Sarath C Ranganathan
No abstract text is available yet for this article.
October 9, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30301615/dysglycaemia-in-cf-why-are-investigating-it-what-should-we-measure-and-how-should-we-treat-it
#3
EDITORIAL
Amanda Brennan, Jennifer Beynon
No abstract text is available yet for this article.
October 6, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30297214/combined-antifungal-therapy-is-superior-to-monotherapy-in-pulmonary-scedosporiosis-in-cystic-fibrosis
#4
Carsten Schwarz, Claudia Brandt, Volker Melichar, Christoph Runge, Eberhard Heuer, Hany Sahly, Martin Schebek, Holger Köster, Jean-Philippe Bouchara, Thomas Biedermann, Peter Meißner, Jörg Große-Onnebrink, Heino Skopnik, Dominik Hartl, Ludwig Sedlacek, Kathrin Tintelnot
Cystic fibrosis (CF) is characterised by chronic airway infection with bacteria and fungi. Infections caused by Scedosporium/Lomentospora species can occur and are difficult to treat. Moulds belonging to the genus Scedosporium/Lomentospora are detected most frequently in respiratory samples of patients with CF, next to Aspergillus spp. Our aim was to define pulmonary fungal infections due to Scedosporium/Lomentospora in CF and to study the antimycotic treatment. In this multicentre study (12 centres; duration January 2008 to December 2014) 31 patients with a lung infection caused by moulds of the genus Scedosporium/Lomentospora were included...
October 5, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30279125/ivacaftor-restores-fgf19-regulated-bile-acid-homeostasis-in-cystic-fibrosis-patients-with-an-s1251n-or-a-g551d-gating-mutation
#5
Ivo P van de Peppel, Marcela Doktorova, Gitte Berkers, Hugo R de Jonge, Roderick H J Houwen, Henkjan J Verkade, Johan W Jonker, Frank A J A Bodewes
OBJECTIVE: Disruption of the enterohepatic circulation of bile acids (BAs) is part of the gastrointestinal phenotype of cystic fibrosis (CF). Ivacaftor (VX-770), a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, improves pulmonary function in CF patients with class III gating mutations. We studied the effect of ivacaftor on the enterohepatic circulation by assessing markers of BA homeostasis and their changes in CF patients. METHODS: In CF patients with an S1251N mutation (N = 16; age 9-35 years S125N study/NTR4873) or a G551D mutation (N = 101; age 10-24 years; GOAL study/ NCT01521338) we analyzed plasma fibroblast growth factor 19 (FGF19) and 7α-hydroxy-4-cholesten-3-one (C4) levels, surrogate markers for intestinal BA absorption and hepatic synthesis, respectively, before and after treatment with ivacaftor...
September 29, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30279124/phenotypic-spectrum-of-patients-with-cystic-fibrosis-and-cystic-fibrosis-related-disease-carrying-p-arg117his
#6
Katherine Keenan, Annie Dupuis, Katherine Griffin, Carlo Castellani, Elizabeth Tullis, Tanja Gonska
BACKGROUND: The "mild" gene variant, p.Arg117His in cystic fibrosis (CF) results in highly variable phenotypes ranging from male infertility to severe lung disease. Due to current interest to include this group in CFTR-targeted therapies, this study aims to describe the disease spectrum. METHODS: Retrospective study of Toronto CF and CFTR-related p.Arg117His patients. Longitudinally captured clinical data were compared between patients with 5T/7T-variants and those with a CF or CFTR-related diagnosis...
September 29, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30270113/antimicrobial-resistance-in-cystic-fibrosis-does-it-matter
#7
EDITORIAL
Patrick A Flume, Valerie J Waters, Scott C Bell, Donald R Van Devanter, J Stuart Elborn
No abstract text is available yet for this article.
September 27, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30268371/human-epididymis-protein-4-he4-levels-inversely-correlate-with-lung-function-improvement-delta-fev-1-in-cystic-fibrosis-patients-receiving-ivacaftor-treatment
#8
Béla Nagy, Zsolt Bene, Zsolt Fejes, Sonya L Heltshe, David Reid, Nicola J Ronan, Yvonne McCarthy, Daniel Smith, Attila Nagy, Elizabeth Joseloff, György Balla, János Kappelmayer, Milan Macek, Scott C Bell, Barry J Plant, Margarida D Amaral, István Balogh
BACKGROUND: We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy. METHODS: In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: 12 and Irish: 19 cases) were enrolled carrying at least one Class III CFTR CF-causing mutation (p.Gly551Asp) and being treated with CFTR potentiator ivacaftor...
September 26, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30268370/mental-health-screening-in-cystic-fibrosis-centres-across-europe
#9
J Abbott, T Havermans, S Jarvholm, E Landau, Y Prins, U Smrekar, D Staab, L Verity, M Verkleij
OBJECTIVES: The Cystic Fibrosis (CF) International Mental Health Guidelines Committee published consensus statements for screening and treating depression and anxiety in individuals with CF and their caregivers. This work aimed to evaluate the dissemination and implementation of the guidelines in Europe two years following their publication. METHODS: A 28-item survey was developed by the multidisciplinary ECFS Mental Health Working Group and emailed to approximately 300 CF centres across Europe...
September 26, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30266518/defining-antimicrobial-resistance-in-cystic-fibrosis
#10
REVIEW
Timothy J Kidd, Rafael Canton, Miquel Ekkelenkamp, Helle Krogh Johansen, Peter Gilligan, John J LiPuma, Scott C Bell, J Stuart Elborn, Patrick A Flume, Donald R VanDevanter, Valerie J Waters
Antimicrobial resistance (AMR) can present significant challenges in the treatment of cystic fibrosis (CF) lung infections. In CF and other chronic diseases, AMR has a different profile and clinical consequences compared to acute infections and this requires different diagnostic and treatment approaches. This review defines AMR, explains how it occurs, describes the methods used to measure AMR as well as their limitations, and concludes with future directions for research and development in the area of AMR in CF...
September 25, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30245262/magnetic-resonance-elastography-demonstrates-elevated-liver-stiffness-in-cystic-fibrosis-patients
#11
LETTER
Don Hayes, Ramkumar Krishnamurthy, Houchun Harry Hu
No abstract text is available yet for this article.
September 20, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30224332/repeated-hot-water-and-steam-disinfection-of-pari-lc-plus%C3%A2-nebulizers-alter-nebulizer-output
#12
Melanie Sue Collins, Matthew O'Brien, Craig M Schramm, Thomas S Murray
Currently, cystic fibrosis patients require daily nebulized treatments to achieve optimal lung health. Growth of pathogenic bacteria in patient nebulizers is well known, and disinfection guidelines have been established. In this short communication, we sought to discover what effect, if any, repeated nebulization/disinfection cycles had on nebulizer output. We nebulized saline repeatedly after exposure to boiling water, steam, and alcohol disinfection methods. While alcohol disinfection did not affect nebulizer output, boiling water and steam significantly decreased nebulizer output from baseline, 74...
September 14, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30224331/successful-lung-re-transplant-in-a-patient-with-cepacia-syndrome-due-to-burkholderia-ambifaria
#13
Kellie J Goodlet, Michael D Nailor, Ashraf Omar, Jasmine L Huang, John J LiPuma, Rajat Walia, Sofya Tokman
Chronic airway inflammation and infection drive morbidity and mortality among patients with cystic fibrosis (CF). While Haemophilus influenzae and Staphylococcus aureus predominate in children, the prevalence of Pseudomonas aeruginosa increases as patients age. Other bacteria, including species within the Burkholderia cepacia complex (Bcc), are also more prevalent among adults with CF. Species within the Bcc accelerate lung function decline and can trigger development of "cepacia syndrome," both before and after lung transplantation...
September 14, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30217546/false-positive-cannabinoid-screens-in-adult-cystic-fibrosis-patients-treated-with-lumacaftor-ivacaftor
#14
LETTER
Dana Kissner, Yvette LeFlore, Srinivas B Narayan, Gautham Marigowda, Christopher Simard, Cecile Le Camus
No abstract text is available yet for this article.
September 11, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30201330/bile-acid-homeostasis-in-gastrointestinal-and-metabolic-complications-of-cystic-fibrosis
#15
REVIEW
Ivo P van de Peppel, Frank A J A Bodewes, Henkjan J Verkade, Johan W Jonker
With the improved treatment of the pulmonary complications of cystic fibrosis (CF), gastrointestinal problems have become more important in the morbidity in CF. A hallmark of the gastrointestinal phenotype of CF, apart from pancreatic insufficiency, is a disruption of bile acid homeostasis. Bile acid homeostasis is important for many gastrointestinal processes including fat absorption, inflammation, microbial composition, as well as regulation of whole body energy metabolism. This review describes the impairment of bile acid homeostasis in CF, its possible consequences for gastrointestinal and metabolic complications and its potential as a target for therapy...
September 7, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30201329/rate-and-predictors-of-prescription-of-lumacaftor-ivacaftor-in-the-18-months-following-approval-in-the-united-states
#16
Gregory S Sawicki, Aliza K Fink, Michael S Schechter, Deena R Loeffler, Nicole Mayer-Hamblett
RATIONALE: Lumacaftor-ivacaftor (LUM-IVA) was approved in the US in 2015 for patients with CF aged >12 homozygous for the delF508 mutation, and patients aged 6 to 12 in 2016. OBJECTIVES: To examine the rate of initial LUM-IVA prescriptions following approval. METHODS: We compared patients eligible for LUM-IVA in the CF Foundation Patient Registry with and without prescriptions in 2015-2016. RESULTS: 5534 (53%) eligible patients had reported prescriptions...
September 7, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30197031/breathlessness-catastrophizing-relates-to-poorer-quality-of-life-in-adults-with-cystic-fibrosis
#17
Danijela Maras, Louise Balfour, Giorgio A Tasca, Ena Gaudet, Shawn D Aaron, William D Cameron, Smita Pakhale
BACKGROUND: Cystic Fibrosis (CF) is the most common genetic disease affecting children and young adults in Canada. It is a multi-system disease, however lung disease is largely responsible for mortality. Treatment advances have resulted in increased life expectancy and a subsequent need to better understand psychosocial issues associated with quality of life in adults living with CF. Emerging research suggests that anxiety and depression are related to poorer health-related quality of life (HRQoL) in CF patients, but there is little research examining cognitive processes, such as breathlessness catastrophizing...
September 6, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30195412/anaemia-and-iron-deficiency-in-relation-to-fatigue-in-cystic-fibrosis
#18
LETTER
Talbot N P, Flight W G
No abstract text is available yet for this article.
September 5, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30177416/differential-expression-of-genes-and-receptors-in-monocytes-from-patients-with-cystic-fibrosis
#19
Abdullah A Tarique, Peter D Sly, Diana G Cardenas, Lin Luo, Jennifer L Stow, Scott C Bell, Claire E Wainwright, Emmanuelle Fantino
INTRODUCTION: We previously reported defective alternative polarization (M2) of macrophages and early expression of classically polarized (M1) macrophage markers in unpolarized monocyte-derived macrophages (MDMs) in patients with cystic fibrosis (CF). The present study assessed whether the mechanism(s) underlying defective macrophage polarization resided in circulating monocytes. METHODS: Monocyte subsets (classical, intermediate and non-classical), markers for monocyte activation (CD163) and recruitment (CD195), receptors/genes associated with macrophage differentiation and polarization were analyzed in CF and compared with healthy individuals...
August 31, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/30172681/investigating-the-effects-of-long-term-dornase-alfa-use-on-lung-function-using-registry-data
#20
S J Newsome, R M Daniel, S B Carr, D Bilton, R H Keogh
BACKGROUND: Dornase alfa (DNase) is one of the commonest cystic fibrosis (CF) treatments and is often used for many years. However, studies have not evaluated the effectiveness of its long-term use. We aimed to use UK CF Registry data to investigate the effects of one-, two-, three-, four- and five-years of DNase use on lung function to see if the benefits of short-term treatment use are sustained long term. METHODS: We analysed data from 4,198 people in the UK CF Registry from 2007 to 2015 using g-estimation...
August 29, 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"