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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/28529073/real-life-acute-lung-function-changes-after-lumacaftor-ivacaftor-first-administration-in-pediatric-patients-with-cystic-fibrosis
#1
Aurélie Labaste, Camille Ohlmann, Catherine Mainguy, Virginie Jubin, Marie Perceval, Laurianne Coutier, Philippe Reix
The combination of lumacaftor and ivacaftor (LUM/IVA) has been reported to induce a mean acute absolute drop of -4.1% predicted forced expiratory volume in 1s (FEV1) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV1 changes after the first dose of LUM/IVA in CF patients. A total of 32 pediatric patients were included. Respiratory manifestations occurred in only 3 patients (9.4%), but FEV1 consistently decreased (-10.4±4.6%, range: -1.5; -21.8%). FEV1 only partially resumed after salbutamol inhalation...
May 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28495380/outcomes-associated-with-antibiotic-regimens-for-treatment-of-mycobacterium-abscessus-in-cystic-fibrosis-patients
#2
Alison DaCosta, Cameron L Jordan, Olivia Giddings, Feng-Chang Lin, Peter Gilligan, Charles R Esther
BACKGROUND: Mycobacterium abscessus infection is associated with declining lung function in cystic fibrosis (CF), but there is little evidence on clinical efficacy to guide treatment. METHODS: Retrospective review of 37 CF patients treated for M. abscessus respiratory infection at a single center from 2006 to 2014. Outcomes included change in FEV1 at 30, 60, 90, 180, and 365days after treatment and clearance of M. abscessus from sputum cultures. RESULTS: Lung function was significantly improved after 30 and 60days of treatment, but not at later time points...
May 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28479021/what-keeps-children-with-cystic-fibrosis-awake-at-night
#3
Moya Vandeleur, Lisa M Walter, David S Armstrong, Philip Robinson, Gillian M Nixon, Rosemary S C Horne
BACKGROUND: Sleep disturbance is common in children with cystic fibrosis (CF) however there are limited studies investigating the causes for poor sleep quality. In a cross sectional observational study we aimed to evaluate the clinical correlates of sleep disturbance in this population. METHODS: Children with CF (7-18years) free from pulmonary exacerbation completed medical review, overnight oximetry, the OSA-18 and 14days of actigraphy recordings with a sleep diary...
May 4, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28478961/biobanking-towards-increased-access-of-biomaterials-in-cystic-fibrosis-report-on-the-pre-conference-meeting-to-the-13th-ecfs-basic-science-conference-pisa-30-march-2-april-2016
#4
Jeffrey M Beekman, Chiuhui Mary Wang, Sara Casati, Katherine L Tuggle, Vincent A M Gulmans, Margarida Amaral, Kris De Boeck
No abstract text is available yet for this article.
May 3, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28476560/is-there-an-association-between-back-pain-and-stress-incontinence-in-adults-with-cystic-fibrosis-a-retrospective-cross-sectional-study
#5
Jane E Ashbrook, Carol Shacklady, Sue Johnson, Gillian Yeowell, Peter Charles Goodwin
BACKGROUND: Back pain and stress urinary incontinence (SUI) are common in adults with cystic fibrosis (CF). This study aimed to establish whether there is an association between back pain, lung function and stress urinary incontinence and its relative risk. METHOD: This was a cross-sectional, retrospective analysis of the Manchester Musculoskeletal Screening Tool (MMST) data. It includes pain, (Short Form McGill Pain Questionnaire (SF-MPQ and VAS)) and International Consultation on Incontinence Short Form (ICIQ-UI-SF) measures...
May 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28465124/a-multiple-reader-scoring-system-for-nasal-potential-difference-parameters
#6
George M Solomon, Bo Liu, Isabelle Sermet-Gaudelus, Isabelle Fajac, Michael Wilschanski, Francois Vermeulen, Steven M Rowe
BACKGROUND: Nasal Potential Difference (NPD) is a biomarker of CFTR activity used to diagnose CF and monitor experimental therapies. Limited studies have been performed to assess agreement between expert readers of NPD interpretation using a scoring algorithm. METHODS: We developed a standardized scoring algorithm for "interpretability" and "confidence" for PD (potential difference) measures, and sought to determine the degree of agreement on NPD parameters between trained readers...
April 29, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28460885/standardized-treatment-of-pulmonary-exacerbations-stop-study-observations-at-the-initiation-of-intravenous-antibiotics-for-cystic-fibrosis-pulmonary-exacerbations
#7
Don B Sanders, George M Solomon, Valeria V Beckett, Natalie E West, Cori L Daines, Sonya L Heltshe, Donald R VanDevanter, Jonathan E Spahr, Ronald L Gibson, Jerry A Nick, Bruce C Marshall, Patrick A Flume, Christopher H Goss
BACKGROUND: The Standardized Treatment of Pulmonary Exacerbations (STOP) program has the intent of defining best practices in the treatment of pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF). The objective of this analysis was to describe the clinical presentations of patients admitted for intravenous (IV) antibiotics and enrolled in a prospective observational PEx study as well as to understand physician treatment goals at the start of the intervention. METHODS: We enrolled adolescents and adults admitted to the hospital for a PEx treated with IV antibiotics...
April 28, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28457954/standardized-treatment-of-pulmonary-exacerbations-stop-study-physician-treatment-practices-and-outcomes-for-individuals-with-cystic-fibrosis-with-pulmonary-exacerbations
#8
Natalie E West, Valeria V Beckett, Raksha Jain, Don B Sanders, Jerry A Nick, Sonya L Heltshe, Elliott C Dasenbrook, Donald R VanDevanter, George M Solomon, Christopher H Goss, Patrick A Flume
BACKGROUND: Pulmonary exacerbations (PEx) are associated with increased morbidity and mortality in individuals with CF. PEx management practices vary widely, and optimization through interventional trials could potentially improve outcomes. The object of this analysis was to evaluate current physician treatment practices and patient outcomes for PEx. METHODS: The Standardized Treatment of Pulmonary exacerbations (STOP) observational study enrolled 220 participants ≥12years old admitted to the hospital for PEx at 11 U...
April 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28456611/increased-risk-of-ptld-in-lung-transplant-recipients-with-cystic-fibrosis
#9
Erin M Lowery, William Adams, Shellee A Grim, Nina M Clark, Leah Edwards, Jennifer E Layden
BACKGROUND: Post-transplant lymphoproliferative disease (PTLD) is an important cause of morbidity and mortality following lung transplantation. Recipients with cystic fibrosis (CF) may have an increased risk of PTLD although the literature is limited to single center cohorts. Our primary aim is to examine PTLD in an adult lung transplant population by utilizing the International Society for Heart and Lung Transplantation Registry. METHODS: We studied 30,598 adult recipients of lung transplants performed between 1999 and 2011...
April 26, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28462874/does-current-reporting-of-lung-function-by-the-uk-cystic-fibrosis-registry-allow-a-fair-comparison-of-adult-centres
#10
Julia Anne Nightingale, Clive Osmond
BACKGROUND: Outcome data for UK cystic fibrosis centres are publicly available in an annual report, which ranks centres by median FEV1% predicted. We wished to assess whether there are differences in lung function outcomes between adult centres that might imply differing standards of care. METHODS: UK Registry data from 4761 subjects at 34 anonymised adult centres were used to calculate mean FEV1% and rate of change of lung function for 2007-13. These measures were used to rank centres and compare outcomes...
April 24, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28446387/risk-factors-for-persistent-methicillin-resistant-staphylococcus-aureus-infection-in-cystic-fibrosis
#11
Mark T Jennings, Elliot C Dasenbrook, Noah Lechtzin, Michael P Boyle, Christian A Merlo
BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) has emerged as an important pathogen in cystic fibrosis (CF). Over 25% of individuals in the United States with CF are found to have MRSA in respiratory culture specimens, and persistent MRSA infection has been associated with more rapid decline in lung function and increased mortality. The objective of this study was to investigate clinical and demographic characteristics that are associated with the development of persistent MRSA infection in a CF population...
April 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28442278/feasibility-and-normal-values-of-an-integrated-conductivity-nanoduct%C3%A2-sweat-test-system-in-healthy-newborns
#12
Claudia E Kuehni, Matthias Schindler, Agnieszka Mazur, Andreas Malzacher, René Hornung, Juerg Barben
BACKGROUND: Nanoduct™ is a simple and practical sweat analysis system measuring conductivity in situ. It requires only three microlitres of sweat, making it especially applicable to newborns. METHODS: We measured conductivity in 260 healthy term infants at the age of four days, and again at four weeks to determine the proportion of successful tests, test duration, and normal values for sweat conductivity in newborns. RESULTS: Sufficient sweat was collected in 159/260 of four-day olds (61%), and in 225/239 of four-week olds (94%)...
April 22, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28438499/rationalizing-endpoints-for-prospective-studies-of-pulmonary-exacerbation-treatment-response-in-cystic-fibrosis
#13
D R VanDevanter, S L Heltshe, J Spahr, V V Beckett, C L Daines, E C Dasenbrook, R L Gibson, R Jain, D B Sanders, C H Goss, P A Flume
BACKGROUND: Given the variability in pulmonary exacerbation (PEx) management within and between Cystic Fibrosis (CF) Care Centers, it is possible that some approaches may be superior to others. A challenge with comparing different PEx management approaches is lack of a community consensus with respect to treatment-response metrics. In this analysis, we assess the feasibility of using different response metrics in prospective randomized studies comparing PEx treatment protocols. METHODS: Response parameters were compiled from the recent STOP (Standardized Treatment of PEx) feasibility study...
April 21, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28428011/cftr-dependent-defect-in-alternatively-activated-macrophages-in-cystic-fibrosis
#14
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
#15
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/28392014/a-treatment-evaluator-tool-to-monitor-the-real-world-effectiveness-of-inhaled-aztreonam-lysine-in-cystic-fibrosis
#16
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/28377087/monocyte-derived-macrophages-from-cf-pigs-exhibit-increased-inflammatory-responses-at-birth
#17
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
#18
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
#19
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
#20
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
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