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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/29459038/molecular-characterization-of-gene-regulatory-networks-in-primary-human-tracheal-and-bronchial-epithelial-cells
#1
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
https://www.readbyqxmd.com/read/29456196/improvement-in-exophiala-dermatitidis-airway-persistence-and-respiratory-decline-in-response-to-interferon-gamma-therapy-in-a-patient-with-cystic-fibrosis
#2
Christopher P Eades, Darius P H Armstrong-James, Jimstan Periselneris, Andrew Jones, Nicholas Simmonds, Peter Kelleher, Anand Shah
No abstract text is available yet for this article.
February 15, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29456195/mast-cell-tryptase-changes-with-aspergillus-fumigatus-host-crosstalk-in-cystic-fibrosis-patients
#3
Carine Gomez, Ania Carsin, Marion Gouitaa, Martine Reynaud-Gaubert, Jean-Christophe Dubus, Jean-Louis Mège, Stéphane Ranque, Joana Vitte
Pulmonary and systemic antifungal immunity influences quality of life and survival of people with cystic fibrosis. Aspergillus fumigatus (Af) induces specific IgG and IgE. Mast cells respond to IgE, IgG and direct interactions with Af. Mast cells are the source of the protease tryptase. We aimed at evaluating serum baseline tryptase as a potential biomarker of the Af-host interaction in cystic fibrosis patients. Serum baseline tryptase, IgE and IgG directed to Af extract and Af molecular allergens were measured in 76 cystic fibrosis patients...
February 15, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29454878/a-comorbidity-of-cf-in-need-of-our-attention-and-activity-attention-deficit-hyperactivity-disorder
#4
EDITORIAL
Jurgen Lemiere, Trudy Havermans
No abstract text is available yet for this article.
February 14, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29444761/chronic-infection-sustained-by-a-pseudomonas-aeruginosa-high-risk-clone-producing-the-vim-1-metallo-%C3%AE-lactamase-in-a-cystic-fibrosis-patient-after-lung-transplantation
#5
Simona Pollini, Claudia Mugnaioli, Daniela Dolce, Silvia Campana, Anna Silvia Neri, Giovanni Taccetti, Gian Maria Rossolini
BACKGROUND: The significance of chronic lung infection by multidrug-resistant (MDR) pathogens in Cystic Fibrosis (CF) transplanted patients remains controversial, and the available information is overall limited. Here we describe the case of a chronic infection, sustained by a metallo-β-lactamase (MBL)-producing P. aeruginosa strain, in a CF patient following lung transplantation. METHODS: Twelve P. aeruginosa isolates collected from a CF patient over a 15-years follow-up period after lung transplantation were analysed for their antibiotic susceptibility profile, MBL production and clonal relatedness...
February 12, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29444760/risk-factors-for-persistent-aspergillus-respiratory-isolation-in-cystic-fibrosis
#6
Gina Hong, Kevin J Psoter, Mark T Jennings, Christian A Merlo, Michael P Boyle, Denis Hadjiliadis, Steven M Kawut, Noah Lechtzin
BACKGROUND: Aspergillus species are increasingly detected in the respiratory tracts of individuals with cystic fibrosis (CF), and chronic Aspergillus fumigatus is associated with more frequent hospitalizations for pulmonary exacerbations. However, patient and clinical factors that may contribute to the acquisition of persistent Aspergillus infection have yet to be identified. The objective of this study was to identify risk factors for development of Aspergillus respiratory isolation in CF...
February 12, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29398489/young-patients-with-cystic-fibrosis-demonstrate-subtle-alterations-of-the-cardiovascular-system
#7
Jacobien B Eising, Cornelis K van der Ent, Arco J Teske, Maaike M Vanderschuren, Cuno S P M Uiterwaal, Folkert J Meijboom
BACKGROUND: As life expectancy increases in patients with cystic fibrosis, it is important to pay attention to extra-pulmonary comorbidities. Several studies have shown signs of myocardial dysfunction in adult patients, but little is known about onset and development of these changes over time. In this prospective study, cardiac function in children with cystic fibrosis was compared to that of healthy children. METHODS: 33 children, aged 3-12years, with cystic fibrosis were recruited from the Wilhelmina Children's hospital and 33 age-matched healthy children were selected from the WHISTLER study, a population-based cohort study...
February 2, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29398488/a-guide-to-interpreting-estimated-median-age-of-survival-in-cystic-fibrosis-patient-registry-reports
#8
Ruth H Keogh, Sanja Stanojevic
Survival statistics, estimated using data collected by national cystic fibrosis (CF) patient registries, are used to inform the CF community and monitor survival of CF populations. Annual registry reports typically give the median age of survival, though different registries use different estimation approaches and terminology, which has created confusion for the community. In this article we explain how median age of survival is estimated, what its interpretation is, and what assumptions and limitations are involved...
February 2, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29396025/early-life-growth-patterns-persist-for-12%C3%A2-years-and-impact-pulmonary-outcomes-in-cystic-fibrosis
#9
Don B Sanders, Zhumin Zhang, Philip M Farrell, HuiChuan J Lai
BACKGROUND: In children with cystic fibrosis (CF), recovery from growth faltering within 2 years of diagnosis (Responders) is associated with better growth and less lung disease at age 6 years. This study examined whether these benefits are sustained through 12 years of age. METHODS: Longitudinal growth from 76 children with CF enrolled in the Wisconsin CF Neonatal Screening Project was examined and categorized into 5 groups: R12, R6, and R2, representing Responders who maintained growth improvement to age 12, 6, and 2 years, respectively, and I6 and N6, representing Non-responders whose growth did and did not improve during ages 2-6 years, respectively...
January 30, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29396024/in-statistics-we-trust-towards-the-careful-derivation-and-interpretation-of-meaningful-survival-estimates-in-cystic-fibrosis
#10
EDITORIAL
N Mayer-Hamblett, D Polineni, S L Heltshe
No abstract text is available yet for this article.
January 25, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29396026/the-effect-of-enteral-tube-feeding-in-cystic-fibrosis-a-registry-based-study
#11
Denis Libeert, Dimitri Declercq, Simeon Wanyama, Muriel Thomas, Sabine Van Daele, Frans De Baets, Stephanie Van Biervliet
BACKGROUND: Long-term effect of enteral tube feeding (ETF) in cystic fibrosis (CF) remains equivocal. METHODS: A Belgian CF registry based, retrospective, longitudinal study, evaluated the pre- and post- ETF (n = 113) clinical evolution and compared each patient with 2 age, gender, pancreatic status and genotype class-matched controls. RESULTS: At baseline ETF had a worse BMI z-score (p < 0.0001) and FEV1% (p < 0.0001) compared to controls...
January 21, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29371133/hypertonic-saline-has-a-prolonged-effect-on-mucociliary-clearance-in-adults-with-cystic-fibrosis
#12
Aaron T Trimble, A Whitney Brown, Beth L Laube, Noah Lechtzin, Kirby L Zeman, Jihong Wu, Agathe Ceppe, David Waltz, William D Bennett, Scott H Donaldson
BACKGROUND: Inhaled hypertonic saline (HS) has been shown to increase mucociliary clearance (MCC) and improve clinical outcomes in adults and adolescents with cystic fibrosis (CF). However, in younger children with CF, a large study failed to demonstrate clinical benefits. This discrepancy could reflect pharmacodynamic differences in the MCC response to HS in different populations. We previously demonstrated the absence of a sustained effect of HS on MCC in healthy adults and in this study sought to characterize the durability of the MCC response to HS in adults with CF...
January 19, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29358075/initial-development-and-pilot-testing-of-observer-reported-outcomes-obsros-for-children-with-cystic-fibrosis-ages-0-11years
#13
T C Edwards, J Emerson, A Genatossio, S McNamara, C Goss, D L Patrick, F Onchiri, M Rosenfeld
PURPOSE: Patient-reported outcomes are important clinical trial endpoints. Young children may not be able to reliably report on how they feel or function, so observer-reported outcomes (ObsROs) may be more appropriate for them. The purpose of this study was to develop and pilot field test electronic parent-reported observational instruments for children with cystic fibrosis (CF) 0-6 and 7-11years of age. METHODS: We performed concept elicitation interviews with parents of children with CF ≤11years of age to elicit the respiratory signs they could observe at baseline and during an acute respiratory illness...
January 18, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29336943/one-time-quantitative-pcr-detection-of-pseudomonas-aeruginosa-to-discriminate-intermittent-from-chronic-infection-in-cystic-fibrosis
#14
Sébastien Boutin, Michael Weitnauer, Selina Hassel, Simon Y Graeber, Mirjam Stahl, A Susanne Dittrich, Marcus A Mall, Alexander H Dalpke
BACKGROUND: Chronic airway infection with Pseudomonas aeruginosa is a major risk factor of progression of lung disease in patients with cystic fibrosis (CF). Chronic P. aeruginosa infection evolves from intermittent infection that is amenable to antibiotic eradication, whereas chronically adapted P. aeruginosa becomes resistant to antibiotic therapy. Discrimination of intermittent versus chronic infection is therefore of high therapeutic relevance, yet the available diagnostic methods are only partly satisfactory...
January 11, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29329721/quantifying-fluctuation-in-glucose-levels-to-identify-early-changes-in-glucose-homeostasis-in-cystic-fibrosis
#15
Rossa Brugha, Marie Wright, Suzie Nolan, Nicola Bridges, Siobhán B Carr
BACKGROUND: Cystic fibrosis related diabetes (CFRD) is associated with increased morbidity in CF. Variability in physiological systems is associated with dysfunctional homeostasis. We examined whether fluctuation in glucose is a marker of CFRD or "pre-diabetes". METHODS: Using a machine learning approach, we compared glucose IQR to current diagnostic criteria in a review of continuous glucose monitoring data. RESULTS: Analysis was performed on 248 studies from 142 children...
January 9, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29311001/up-to-date-and-projected-estimates-of-survival-for-people-with-cystic-fibrosis-using-baseline-characteristics-a-longitudinal-study-using-uk-patient-registry-data
#16
Ruth H Keogh, Rhonda Szczesniak, David Taylor-Robinson, Diana Bilton
BACKGROUND: Cystic fibrosis (CF) is the most common inherited disease in Caucasians, affecting around 10,000 individuals in the UK today. Prognosis has improved considerably over recent decades with ongoing improvements in treatment and care. Providing up-to-date survival predictions is important for patients, clinicians and health services planning. METHODS: Flexible parametric survival modelling of UK CF Registry data from 2011 to 2015, capturing 602 deaths in 10,428 individuals...
January 5, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29292092/kb001-a-a-novel-anti-inflammatory-found-to-be-safe-and-well-tolerated-in-cystic-fibrosis-patients-infected-with-pseudomonas-aeruginosa
#17
R Jain, V V Beckett, M W Konstan, F J Accurso, J L Burns, N Mayer-Hamblett, Carlos Milla, D R VanDevanter, J F Chmiel
BACKGROUND: Chronic Pseudomonas aeruginosa (Pa) airways infection, exuberant local inflammation, and progressive lung function loss are hallmarks of cystic fibrosis (CF). KB001-A is an anti-PcrV PEGylated monoclonal antibody fragment to the Type III secretion system of Pa. This 16-week study evaluated KB001-A associated effect on time-to-need for antibiotics for worsening respiratory signs and symptoms, as well as safety, and treatment-associated changes in symptom scores, inflammatory markers, and spirometry...
December 29, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29292091/ratiometric-sweat-secretion-optical-test-in-cystic-fibrosis-carriers-and-healthy-subjects
#18
Gabriella Bergamini, Gloria Tridello, Elisa Calcaterra, Stefano Ceri, Marco Tagliasacchi, Federico Bianchi, Federico Monti, Andrea Masciadri, Eugenia Laudanna, Denise Peserico, Elena Sorio, Valeria Esposito, Teresinha Leal, Baroukh Maurice Assael, Claudio Sorio, Paola Melotti
We have simplified the published procedure (5) for measuring sweat rates in individual human sweat glands. Sweat secretion rates were obtained from sweat drops secreted on the forearm by multiple individual glands. We computed a ratio between CFTR-dependent (by intradermal microinjection of a β adrenergic cocktail) and CFTR-independent (by methacoline as cholinergic stimulus) sweat secretion rates. We obtained a reproducible, approximately linear readout of CFTR function with measurements performed by two different independent teams...
December 29, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29289454/audiometric-assessment-of-pediatric-patients-with-cystic-fibrosis
#19
Kathryn L Kreicher, Michael J Bauschard, Clarice S Clemmens, Concetta Maria Riva, Ted A Meyer
BACKGROUND: The purpose of this study was to evaluate hearing impairment in pediatric patients with cystic fibrosis (CF). METHODS: This is a retrospective analysis of the AudGen database generated by Children's Hospital of Philadelphia. Audiograms were analyzed for type of hearing loss (HL), pure-tone-average (PTA), laterality, and change in hearing over time. Medical charts were reviewed to identify factors that influence development and progression of hearing loss...
December 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29277313/quality-of-life-and-mood-in-children-with-cystic-fibrosis-associations-with-sleep-quality
#20
Moya Vandeleur, Lisa M Walter, David S Armstrong, Philip Robinson, Gillian M Nixon, Rosemary S C Horne
BACKGROUND: We aimed to investigate the relationship between sleep quality, mood and health-related quality of life (HRQOL) in children with CF and controls. METHODS: Children (7-12years) and adolescents (13-18years) with CF and controls completed sleep evaluation: overnight oximetry and 14days of actigraphy. Age-appropriate questionnaires assessed mood (Children's Depression Inventory; CDI or Beck's Depression Inventory), HRQOL (CF Questionnaire-Revised; CFQ-R or PedsQL), and sleepiness (Pediatric Daytime Sleepiness Scale)...
December 24, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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