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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/29221674/the-relationship-between-sweat-chloride-levels-and-mortality-in-cystic-fibrosis-varies-by-individual-genotype
#1
Julia C Espel, Hannah L Palac, Ankit Bharat, Joanne Cullina, Michelle Prickett, Marc Sala, Susanna A McColley, Manu Jain
RATIONALE: The association between CFTR genotype, sweat chloride and mortality has been inconsistent, but no previous analyses have examined the association stratified by individual genotypes. OBJECTIVES: To evaluate the genotype-specific association between sweat chloride and mortality. METHODS: The CFF Patient Registry was assessed and included all patients in the registry between 1996 and 2012 with at least one F508del allele. We excluded patients without a documented genotype or plausible sweat chloride level...
December 5, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29212596/rare-cases-of-blastobotrys-raffinosifermentans-as-cause-of-fev1-decline-in-two-cf-patients-whole-genome-sequencing-to-exclude-transmission
#2
K Jahn, V Baettig, H M B Seth-Smith, A Egli, M Tamm
No abstract text is available yet for this article.
December 3, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29208346/advancing-the-gi-frontier-for-patients-with-cf
#3
EDITORIAL
Steven D Freedman, Michael Wilschanski, Sarah Jane Schwarzenberg
No abstract text is available yet for this article.
December 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29198838/another-step-in-the-journey-from-cftr-mutation-to-sweat-chloride-concentration-to-survival
#4
EDITORIAL
D R VanDevanter, N Mayer-Hamblett
No abstract text is available yet for this article.
November 30, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29191659/let-s-talk-about-sex-behaviors-experience-and-health-care-utilization-in-young-women-with-cf
#5
EDITORIAL
Sonya L Heltshe, Jennifer L Taylor-Cousar
No abstract text is available yet for this article.
November 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29187303/prevalence-and-characteristics-of-chronic-kidney-disease-among-danish-adults-with-cystic-fibrosis
#6
Kristina H Berg, Lene Ryom, Daniel Faurholt-Jepsen, Tania Pressler, Terese L Katzenstein
BACKGROUND: With improved prognosis of CF, comorbidities including chronic kidney disease (CKD) are becoming increasingly important. Identification of those at highest CKD risk is hence a priority. METHODS: In this cross-sectional study, adults with CF attending the Copenhagen CF Centre at Rigshospitalet with ≥2 measurements of serum creatinine from 2013 to 2015 were included. Data was obtained from an electronic CF database, which contains anonymised clinical and laboratory data on all individuals attending the clinic...
November 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29174082/multiple-reaction-monitoring-mass-spectrometry-to-identify-novel-plasma-protein-biomarkers-of-treatment-response-in-cystic-fibrosis-pulmonary-exacerbations
#7
James M Roberts, Darlene L Y Dai, Zsuzsanna Hollander, Raymond T Ng, Scott J Tebbutt, Pearce G Wilcox, Don D Sin, Bradley S Quon
BACKGROUND: Systemic inflammation decreases with IV antibiotics during the treatment of CF pulmonary exacerbations (PEx). We used multiple reaction monitoring mass spectrometry and immunoassays to monitor blood proteins during PEx treatment to determine if early changes could be used to predict PEx outcomes following treatment. METHODS: Blood samples from 25 PEx (22 unique adults) were collected within 24h of admission, day 5, day 10, and at IV antibiotic completion...
November 21, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29157922/development-and-electronic-validation-of-the-revised-cystic-fibrosis-questionnaire-cfq-r-teen-adult-new-tool-for-monitoring-psychosocial-health-in-cf
#8
Amparo Solé, Casilda Olveira, Ines Pérez, David Hervás, Vincent Valentine, Anick N Baca Yepez, Gabriel Olveira, Alexandra L Quittner
BACKGROUND: The Cystic Fibrosis Questionnaire-Revised (CFQ-R+14) is a disease-specific, health-related quality of life instrument for cystic fibrosis (CF) patients ≥14years. We have developed a Spanish electronic version of the CFQ-R (e-CFQ-R+14 Spain). Our aim was to compare the paper and electronic versions and to validate the electronic version. METHODS: Fifty CF patients completed the study. All answered the paper and electronic versions on day 1 and repeated the e-CFQR version 15days later...
November 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29157921/pooling-of-bronchoalveolar-lavage-in-children-with-cystic-fibrosis-does-not-adversely-affect-the-microbiological-yield-or-sensitivity-in-detecting-pulmonary-inflammation
#9
Paul McNally, Jennifer O'Rourke, Emmanuelle Fantino, Archana Chacko, Rishi Pabary, Andrew Turnbull, Tim Grant, Niamh O'Sullivan, Claire Wainwright, Barry Linnane, Jane C Davies, Peter D Sly
BACKGROUND: Bronchoalveolar lavage (BAL) is a potentially useful outcome measure for clinical trials in children with CF but its use is limited by variations in approach internationally. We sought to determine if pooling adversely affected the diagnostic properties of BAL. METHODS: Children undergoing bronchoscopy for clinical reasons were included. A multi-step study protocol ensured BAL was collected and analysed both separately and as a pooled fluid. RESULTS: Eighty-five children (53 CF, 32 control) were recruited...
November 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29150357/caregiver-burden-of-parents-of-young-children-with-cystic-fibrosis
#10
C Fitzgerald, S George, R Somerville, B Linnane, P Fitzpatrick
BACKGROUND: There is a paucity of research examining the impact of informal caregiving on parents of young children with cystic fibrosis (CF). The aim of this study was to examine caregiver burden and identify risk factors associated with high caregiver burden in mothers and fathers of young children with CF. METHODS: This was a cross-sectional study of parents of young children with CF. A total of 213 families were invited to complete the CarerQoL questionnaire, a validated tool composed of two parts: (i) the CarerQol-7D which describes the care situation in terms of the negative and positive effects of caregiving and (ii) the visual analogue scale (VAS) which measures happiness on a scale from 0 to 10 (0=completely unhappy and 10=completely happy)...
November 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29128317/lack-of-efficacy-of-lactobacillus-gg-in-reducing-pulmonary-exacerbations-and-hospital-admissions-in-children-with-cystic-fibrosis-a-randomised-placebo-controlled-trial
#11
Eugenia Bruzzese, Valeria Raia, Eliana Ruberto, Riccardo Scotto, Antonietta Giannattasio, Dario Bruzzese, Maria Cristina Cavicchi, Michela Francalanci, Carla Colombo, Nadia Faelli, Valeria Daccò, Giuseppe Magazzù, Stefano Costa, Vincenzina Lucidi, Fabio Majo, Alfredo Guarino
BACKGROUND: Intestinal dysbiosis has been described in Cystic Fibrosis (CF) and probiotics have been proposed to restore microbial composition. Aim of the study was to investigate the effects of Lactobacillus rhamnosus GG (LGG) on clinical outcomes in children with cystic fibrosis (CF). METHODS: A multicentre, randomised double-blind, clinical trial was conducted in children with CF. After 6months of baseline assessment, enrolled children (2 to 16years of age) received Lactobacillus GG (6×109CFU/day) or placebo for 12months...
November 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29128316/fecal-calprotectin-concentrations-in-young-children-with-cystic-fibrosis-authors-response
#12
LETTER
Millie Garg, Steven T Leach, Andrew S Day, Chee Y Ooi
No abstract text is available yet for this article.
November 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29126871/lumacaftor-ivacaftor-in-patients-with-cystic-fibrosis-and-advanced-lung-disease-homozygous-for-f508del-cftr
#13
Jennifer L Taylor-Cousar, Manu Jain, Tara Lynn Barto, Tarik Haddad, Jeffrey Atkinson, Simon Tian, Rui Tang, Gautham Marigowda, David Waltz, Joseph Pilewski
OBJECTIVE: Evaluation of the safety, tolerability, and efficacy of lumacaftor/ivacaftor in patients with cystic fibrosis (CF) with severe lung disease. METHODS: Patients with CF 12 years of age and older, homozygous for F508del-CFTR, with percent predicted forced expiratory volume in 1 second (ppFEV1) <40 received lumacaftor 400 mg/ivacaftor 250mg every 12h (full dose) for 24weeks in an open-label, prospective study (NCT02390219). Dose modification to half dose for 1-2weeks (including at initiation) was permitted...
November 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29103924/ceftaroline-pharmacokinetics-and-pharmacodynamics-in-patients-with-cystic-fibrosis
#14
Emily E Barsky, Luis M Pereira, Keri J Sullivan, Alanna Wong, Alexander J McAdam, Gregory S Sawicki, Gregory P Priebe, Susan M Goobie
BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) is a prevalent pathogen in patients with cystic fibrosis (CF) associated with increased morbidity. Ceftaroline fosamil is an intravenous (IV) cephalosporin with activity against MRSA. There are minimal data regarding dosing in the CF population. The objective of this study was to determine the pharmacokinetic and pharmacodynamic profile of IV ceftaroline in patients with CF. METHODS: We conducted a single-center prospective study of children and young adults with CF receiving ceftaroline (15mg/kg IV up to 600mg every 8h) as part of treatment for a CF pulmonary exacerbation between June 2016 and April 2017...
November 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29100868/lung-function-decline-is-delayed-but-not-decreased-in-patients-with-cystic-fibrosis-and-the-r117h-gene-mutation
#15
Jeffrey S Wagener, Stefanie J Millar, Nicole Mayer-Hamblett, Gregory S Sawicki, Edward F McKone, Christopher H Goss, Michael W Konstan, Wayne J Morgan, David J Pasta, Richard B Moss
BACKGROUND: Patients with cystic fibrosis (CF) experience variable lung disease phenotypes. The R117H mutation is often associated with preserved lung function. Our objective was to compare the rate of lung function decline in patients with the R117H mutation and patients homozygous for the F508del mutation. METHODS: Rate of decline in percentage-of-predicted FEV1 (ppFEV1) was analyzed using the 2006-2010 US CF Foundation Patient Registry. RESULTS: 4-year rate of decline was slower in 156 R117H patients compared with 6251 F508del patients (-0...
October 31, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29097163/defining-palliative-care-in-cystic-fibrosis-a-delphi-study
#16
E P Dellon, J Goggin, E Chen, K Sabadosa, S E Hempstead, A Faro, K Homa
BACKGROUND: The goal of palliative care is to improve quality of life for people with serious illness. We aimed to create a cystic fibrosis (CF)-specific definition of palliative care. METHODS: A working group of 36 CF care providers, researchers, palliative care providers, quality improvement experts, individuals with CF, and CF caregivers completed a series of questionnaires to rate the value of each of 22 attributes of palliative care, rank top attributes to construct definitions of palliative care, and then rate proposed definitions...
October 30, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29110966/longitudinal-development-of-initial-chronic-and-mucoid-pseudomonas-aeruginosa-infection-in-young-children-with-cystic-fibrosis
#17
S L Heltshe, U Khan, V Beckett, A Baines, J Emerson, D B Sanders, R L Gibson, W Morgan, M Rosenfeld
BACKGROUND: While the emergence of chronic and mucoid Pseudomonas aeruginosa (Pa) infection are both associated with poorer outcomes among CF patients, their relationship is poorly understood. We examined the longitudinal relationship of incident, chronic and mucoid Pa in a contemporary, young CF cohort in the current era of Pa eradication therapy. METHODS: This retrospective cohort was comprised of patients in the U.S. CF Foundation Patient Registry born 2006-2015, diagnosed before age 2, and with at least 3 respiratory cultures annually...
October 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29107600/inflammation-and-host-pathogen-interaction-cause-and-consequence-in-cystic-fibrosis-lung-disease
#18
Alessandra Bragonzi, Hamed Horati, Lauren Kerrigan, Nicola Ivan Lorè, Bob J Scholte, Sinéad Weldon
Cystic Fibrosis (CF) lung disease is associated with dysregulation of host defence systems, which ultimately disrupts the balance between inflammation and resolution and leaves the host susceptible to repeated infection. However, the mechanisms underlying these defects are complex and continue to garner significant interest among the CF research community. This review explores emerging data on novel aspects of innate host defence with promising biomarker and therapeutic potential for CF lung disease. Improved understanding of inflammation and host defence against pathogens in patients and animal models during the progression of CF lung disease is pivotal for the discovery of new therapeutics that can limit and/or prevent damage from birth...
October 26, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29107599/faecal-calprotectin-concentrations-in-young-children-with-cystic-fibrosis
#19
LETTER
Helmut Ellemunter, Katharina Schüller, Gratiana Steinkamp
No abstract text is available yet for this article.
October 26, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29102290/ion-channels-as-targets-to-treat-cystic-fibrosis-lung-disease
#20
S Lorraine Martin, Vinciane Saint-Criq, Tzyh-Chang Hwang, László Csanády
Lung health relies on effective mucociliary clearance and innate immune defence mechanisms. In cystic fibrosis (CF), an imbalance in ion transport due to an absence of chloride ion secretion, caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) and a concomitant sodium hyperabsorption, caused by dyregulation of the epithelial sodium channel (ENaC), results in mucus stasis which predisposes the lungs to cycles of chronic infection and inflammation leading to lung function decline...
October 25, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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