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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/28428011/cftr-dependent-defect-in-alternatively-activated-macrophages-in-cystic-fibrosis
#1
Abdullah A Tarique, Peter D Sly, Patrick G Holt, Anthony Bosco, Robert S Ware, Jayden Logan, Scott C Bell, Claire E Wainwright, Emmanuelle Fantino
BACKGROUND: The role of the macrophages in cystic fibrosis (CF) lung disease has been poorly studied. We hypothesized that alternatively activated M2 macrophages are abnormal in CF lung disease. METHODS: Blood samples were collected from adults (n=13) children (n=27) with CF on admission for acute pulmonary exacerbation and when clinically stable. Monocytes were differentiated into macrophages and polarized into classical (M1) and alternatively-activated (M2) phenotypes, function determined ex-vivo and compared with healthy controls...
April 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28416415/age-dependent-variation-of-fecal-calprotectin-in-cystic-fibrosis-and-healthy-children
#2
Millie Garg, Steven T Leach, Michael J Coffey, Tamarah Katz, Roxanne Strachan, Tamara Pang, Bronwen Needham, Kei Lui, Fathalla Ali, Andrew S Day, Laura Appleton, Vesal Moeeni, Adam Jaffe, Chee Y Ooi
BACKGROUND: Fecal calprotectin may be used as a non-invasive method to assess the effect of novel therapies on the gut in cystic fibrosis (CF). METHOD: Stools from CF patients and healthy controls (HC) (0-10years old) were prospectively collected for evaluation of temporal trends. RESULTS: 130 CF samples (64 subjects) and 114 HC samples (101 subjects) were collected. Overall, fecal calprotectin levels were different in CF patients and HC from 0 to 10years (P=0...
April 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28416146/pseudomonas-aeruginosa-eradication-finally-moving-the-needle
#3
EDITORIAL
Jonathan Cogen, Margaret Rosenfeld
No abstract text is available yet for this article.
April 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28392014/a-treatment-evaluator-tool-to-monitor-the-real-world-effectiveness-of-inhaled-aztreonam-lysine-in-cystic-fibrosis
#4
Barry J Plant, Damian G Downey, Joe A Eustace, Cedric Gunaratnam, Charles S Haworth, Andrew M Jones, Edward F McKone, Daniel G Peckham, R Ian Ketchell, Diana Bilton
BACKGROUND: Studies are required that evaluate real-world outcomes of inhaled aztreonam lysine in patients with cystic fibrosis (CF). METHODS: Our treatment-evaluator tool assessed the effectiveness of inhaled aztreonam in routine practice in 117 CF patients across four time periods (6-12 (P2) and 0-6months (P1) pre-initiation, and 0-6 (T1) and 6-12months (T2) post-initiation). Outcomes were: changes in %-predicted forced expiratory volume in 1s (FEV1), body-mass index (BMI), hospitalisation days and intravenous antibiotic usage...
April 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28392015/modeling-cystic-fibrosis-disease-progression-in-patients-with-the-rare-cftr-mutation-p67l
#5
Isobel E R MacKenzie, Valerie Paquette, Frances Gosse, Sheenagh George, Frederic Chappe, Valerie Chappe
BACKGROUND: The progression of cystic fibrosis (CF) in patients with the rare mutation P67L was examined to determine if it induced a milder form of CF compared to the common severe ΔF508 mutation. METHODS: Parameters of lung function, level of bacterial infection, nutritional status and hospitalization were used to represent CF progression. Age at diagnosis and pancreatic status were used to assess CF presentation. Analysis of data from the CF Canada Registry collected over a 15-year period included 266 ΔF508/ΔF508 homozygote patients from CF clinics in Atlantic Canada and 26 compound heterozygote patients with the rare P67L mutation from clinics across Canada...
April 5, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28377087/monocyte-derived-macrophages-from-cf-pigs-exhibit-increased-inflammatory-responses-at-birth
#6
Lily Paemka, Brian N McCullagh, Mahmoud H Abou Alaiwa, David A Stoltz, Qian Dong, Christoph O Randak, Robert D Gray, Paul B McCray
BACKGROUND: We sought to address whether CF macrophages have a primary functional defect as a consequence of CFTR loss and thus contribute to the onset of infection and inflammation observed in CF lung disease. METHODS: Monocyte derived macrophages (MDMs) were prepared from newborn CF and non-CF pigs. CFTR mRNA expression was quantified by rtPCR and anion channel function was determined using whole cell patch clamp analysis. IL8 and TNFα release from MDMs in response to lipopolysaccharide stimulation was measured by ELISA...
April 1, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28347603/liver-disease-in-cystic-fibrosis-presents-as-non-cirrhotic-portal-hypertension
#7
LETTER
Peter Witters, Louis Libbrecht, Tania Roskams, Kris De Boeck, Lieven Dupont, Marijke Proesmans, François Vermeulen, Geert Maleux, Diethard Monbaliu, Jacques Pirenne, David Cassiman
No abstract text is available yet for this article.
March 24, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28330773/a-3-year-prognostic-score-for-adults-with-cystic-fibrosis
#8
L Nkam, J Lambert, A Latouche, G Bellis, P R Burgel, M N Hocine
BACKGROUND: Therapeutic progress in patients with cystic fibrosis (CF) has resulted in improved prognosis over the past decades. We aim to reevaluate prognostic factors of CF and provide a prognostic score to predict the risk of death or lung transplantation (LT) within a 3-year period in adult patients. METHODS: We developed a logistic model using data from the French CF Registry and combined the coefficients into a prognostic score. The discriminative abilities of the model and the prognostic score were assessed by c-statistic...
March 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28320633/nutritional-status-nutrient-intake-and-use-of-enzyme-supplements-in-paediatric-patients-with-cystic-fibrosis-a-european-multicentre-study-with-reference-to-current-guidelines
#9
Joaquim Calvo-Lerma, Jessie M Hulst, Inês Asseiceira, Ine Claes, Maria Garriga, Carla Colombo, Victoria Fornés, Sandra Woodcock, Tiago Martins, Mieke Boon, Mar Ruperto, Sylvia Walet, Chiara Speziali, Peter Witters, Etna Masip, Celeste Barreto, Kris de Boeck, Carmen Ribes-Koninckx
BACKGROUND: The New European guidelines have established the most updated recommendations on nutrition and pancreatic enzyme replacement therapy (PERT) in CF. In the context of MyCyFAPP project - a European study in children with CF aimed at developing specific tools for improvement of self-management - the objective of the current study was to assess nutritional status, daily energy and macronutrient intake, and PERT dosing with reference to these new guidelines. METHODS: Cross sectional study in paediatric patients with CF from 6 European centres...
March 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28325531/real-life-initiation-of-lumacaftor-ivacaftor-combination-in-adults-with-cystic-fibrosis-homozygous-for-the-phe508del-cftr-mutation-and-severe-lung-disease
#10
Dominique Hubert, Raphaël Chiron, Boubou Camara, Dominique Grenet, Anne Prévotat, Laurence Bassinet, Stéphane Dominique, Gilles Rault, Julie Macey, Isabelle Honoré, Reem Kanaan, Sylvie Leroy, Nadine Desmazes Dufeu, Pierre-Régis Burgel
OBJECTIVE: To investigate the short-term adverse events and effectiveness of lumacaftor/ivacaftor combination treatment in adults with cystic fibrosis (CF) and severe lung disease in a real life setting. METHODS: A multicentre observational study investigated adverse events, treatment discontinuation, FEV1 and body mass index (BMI) one month and three months after lumacaftor/ivacaftor initiation in adults with CF and FEV1 below 40% predicted. RESULTS: Respiratory adverse events (AEs) were reported by 27 of 53 subjects (51%) and 16 (30%) discontinued treatment...
March 15, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28314540/technological-advances-shed-light-on-left-ventricular-cardiac-disturbances-in-cystic-fibrosis
#11
REVIEW
Zahra N Sayyid, Zachary M Sellers
Cystic fibrosis (CF), the most common autosomal recessive lethal disease in Caucasians, causes chronic pulmonary disease and can lead to cor pulmonale with right ventricular dysfunction. The presence of the cystic fibrosis transmembrane conductance regulator (CFTR) in cardiac myocardia has prompted debate regarding possible defective ion channel-induced cardiomyopathy. Clinical heart disease in CF is considered rare and is restricted to case reports. It has been unclear if this is due to the lack of physiological importance of CFTR in the heart, the relatively short lifespan of those with CF, or a technical inability to detect subclinical disease...
March 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28314539/immediate-effects-of-lumacaftor-ivacaftor-administration-on-lung-function-in-patients-with-severe-cystic-fibrosis-lung-disease
#12
Natalia Popowicz, Jamie Wood, Anna Tai, Sue Morey, Siobhain Mulrennan
Safety-data for lumacaftor/ivacaftor (LUM/IVA) combination therapy in patients with severe lung disease (percent predicted forced expiratory volume in 1s [ppFEV1] <40) remain limited. We report immediate post-dose respiratory-related adverse events in 12 patients with severe cystic fibrosis (CF) lung disease (median [IQR] ppFEV1: 34 [31-36]) prescribed LUM/IVA. All patients experienced a decline in ppFEV1 from baseline at 2-hours (median [IQR] relative change: -19 [-21 to -11]%, p<0.001) that persisted at 24-hours but recovered in most patients at 1-month...
March 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28302366/the-cf-cares-primary-palliative-care-model-a-cf-specific-structured-assessment-of-symptoms-distress-and-coping
#13
Deborah Friedman, Rachel W Linnemann, Lily L Altstein, Suhayla Islam, Kieu-Tram Bach, Chelsea Lamb, John Volpe, Caitlin Doolittle, Anita St John, Patricia J O'Malley, Gregory S Sawicki, Anna M Georgiopoulos, Lael M Yonker, Samuel M Moskowitz
BACKGROUND: Current palliative care tools do not address distressing chronic symptoms that are most relevant to cystic fibrosis. METHODS: A CF-specific structured assessment based on a primary palliative care framework was administered to 41 adolescents and adults with CF. Descriptive and correlational analyses were conducted. RESULTS: Patients reported numerous physical and psychological symptoms (mean of 10 per patient), with psychological symptoms rated as more distressing...
March 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28285933/the-patient-voice-in-research-supporting-actor-or-starring-role
#14
EDITORIAL
Nicola J Rowbotham, Alan R Smyth
No abstract text is available yet for this article.
March 9, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28284527/feasibility-of-placebo-controlled-trial-designs-for-new-cftr-modulator-evaluation
#15
Donald R VanDevanter, Nicole Mayer-Hamblett, Michael Boyle
BACKGROUND: New CFTR modulators are in development that sponsors anticipate will be comparable or superior to approved modulators. Testing these agents for efficacy will require either placebo-controlled or active-comparator trials. METHODS: We surveyed US CF physicians and their patients eligible to receive approved modulators or their families for willingness to participate in placebo-controlled modulator trials of varying duration. RESULTS: Interest in placebo-controlled trials of short duration (2-4weeks) was greatest, with few respondents, particularly among patient respondents, willing to consider 6month studies...
March 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28284526/association-between-glucose-intolerance-and-bacterial-colonisation-in-an-adult-population-with-cystic-fibrosis-emergence-of-stenotrophomonas-maltophilia
#16
C Lehoux Dubois, V Boudreau, F Tremblay, A Lavoie, Y Berthiaume, R Rabasa-Lhoret, A Coriati
BACKGROUND: Diabetes is common in cystic fibrosis (CF). Glucose can be detected in the airway when the blood glucose is elevated, which favours bacterial growth. We investigated the relationship between dysglycemia and lung pathogens in CF. METHODS: Cross-sectional and prospective analysis of CF patients (N=260) who underwent a 2h-oral glucose tolerance test. Clinical data was collected. RESULTS: Stenotrophomonas maltophilia (S. maltophilia) was the sole bacteria increased in dysglycemic (AGT: 20...
March 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28283399/dietary-intake-and-lipid-profile-in-children-and-adolescents-with-cystic-fibrosis
#17
Janna W Woestenenk, Dorothea A Schulkes, Henk S Schipper, Cornelis K van der Ent, Roderick H J Houwen
BACKGROUND: Cystic fibrosis (CF) patients are advised to derive 35% of their daily energy intake from dietary fat. Whether this high fat intake is associated with dyslipidaemia is unknown. We described the lipid profile and dietary intake in paediatric patients with CF. METHODS: 110 fasting lipid concentrations of 110 Dutch patients with CF were studied, along with 86 measurements of dietary intake. For the total group and for boys and girls separately, the lipid profile and the dietary intake were investigated...
March 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28262570/peripheral-muscle-abnormalities-in-cystic-fibrosis-etiology-clinical-implications-and-response-to-therapeutic-interventions
#18
Mathieu Gruet, Thierry Troosters, Samuel Verges
Peripheral muscle dysfunction is an important systemic consequence of cystic fibrosis (CF) with major clinical implications, such as exercise intolerance and reduced quality of life. Evidence is now accumulating that lack of physical activity is unlikely to be the sole explanation for peripheral muscle dysfunction of patients with CF. Particularly, the demonstration of CFTR expression in both murine and human skeletal muscle suggests the potential implication of intrinsic CF-related factors. By combining data from both human and animal models, this review describes CF peripheral muscle abnormalities and critically reviews the advances in understanding the impact of the underlying mechanisms...
March 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28262569/pilot-trial-of-tobramycin-inhalation-powder-in-cystic-fibrosis-patients-with-chronic-burkholderia-cepacia-complex-infection
#19
Valerie Waters, Yvonne Yau, Trevor Beaudoin, Jillian Wettlaufer, Sean Kevin Tom, Nancy McDonald, Leena Rizvi, Michelle Klingel, Felix Ratjen, Elizabeth Tullis
There is no effective chronic suppressive therapy Burkholderia cepacia complex infection in cystic fibrosis (CF) patients. This was a pilot, open-label clinical trial of tobramycin inhalation powder (TIP) delivered via Podhaler twice daily for 28days in adults and children with CF and chronic B. cepacia complex infection in Toronto, Canada. A total of 10 subjects (4 pediatric, 6 adult patients) were treated. There was a mean drop of 1.4 log (CFU/ml) in sputum bacterial density (p=0.01) and sputum IL-8 levels decreased significantly after 28days of TIP (p=0...
March 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28258928/inhaled-dry-powder-mannitol-in-children-with-cystic-fibrosis-a-randomised-efficacy-and-safety-trial
#20
K De Boeck, E Haarman, J Hull, L C Lands, A Moeller, A Munck, J Riethmüller, H Tiddens, S Volpi, J Leadbetter, B Charlton, A Malfroot
INTRODUCTION: Inhaled mannitol has beneficial effects on lung function, mucociliary clearance, quality of life and sputum properties. This trial examined the efficacy of inhaled mannitol in children with cystic fibrosis (CF). METHODS: The efficacy of inhaled mannitol in children with CF aged 6-17years was assessed in a phase 2, randomised, placebo-controlled crossover study. Subjects were randomly assigned to mannitol 400mg every 12h or matching placebo for 8weeks, followed by an 8week washout and an 8week period with the alternate treatment...
February 28, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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