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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/28720345/the-potential-of-phage-therapy-in-cystic-fibrosis-essential-human-bacterial-phage-interactions-and-delivery-considerations-for-use-in-pseudomonas-aeruginosa-infected-airways
#1
REVIEW
Stephanie Trend, Angela M Fonceca, William G Ditcham, Anthony Kicic, Arest Cf
As antimicrobial-resistant microbes become increasingly common and a significant global issue, novel approaches to treating these infections particularly in those at high risk are required. This is evident in people with cystic fibrosis (CF), who suffer from chronic airway infection caused by antibiotic resistant bacteria, typically Pseudomonas aeruginosa. One option is bacteriophage (phage) therapy, which utilises the natural predation of phage viruses upon their host bacteria. This review summarises the essential and unique aspects of the phage-microbe-human lung interactions in CF that must be addressed to successfully develop and deliver phage to CF airways...
July 15, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28716479/lower-exhaled-nitric-oxide-in-infants-with-cystic-fibrosis-compared-to-healthy-controls
#2
Insa Korten, Margot Liechti, Florian Singer, Gaudenz Hafen, Isabelle Rochat, Pinelopi Anagnostopoulou, Dominik Müller-Suter, Jakob Usemann, Alexander Moeller, Urs Frey, Philipp Latzin, Carmen Casaulta
Exhaled nitric oxide (FENO) is a well-known, non-invasive airway biomarker. In patients with Cystic Fibrosis (CF) FENO is decreased. To understand if reduced FENO is primary related to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) dysfunction or an epiphenomenon of chronic inflammation, we measured FENO in 34 infants with CF prior to clinical symptoms and in 68 healthy controls. FENO was lower in CF compared to controls (p=0.0006) and the effect was more pronounced in CF infants without residual CFTR function (p<0...
July 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28712885/detection-of-cftr-function-and-modulation-in-primary-human-nasal-cell-spheroids
#3
John J Brewington, Erin T Filbrandt, F J LaRosa, Alicia J Ostmann, Lauren M Strecker, Rhonda D Szczesniak, John P Clancy
BACKGROUND: Expansion of CFTR modulators to patients with rare/undescribed mutations will be facilitated by patient-derived models quantifying CFTR function and restoration. We aimed to generate a personalized model system of CFTR function and modulation using non-surgically obtained nasal epithelial cells (NECs). METHODS: NECs obtained by curettage from healthy volunteers and CF patients were expanded and grown in 3-dimensional culture as spheroids, characterized, and stimulated with cAMP-inducing agents to activate CFTR...
July 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28711222/retrospective-observational-study-of-french-patients-with-cystic-fibrosis-and-a-gly551asp-cftr-mutation-after-1-and-2years-of-treatment-with-ivacaftor-in-a-real-world-setting
#4
Dominique Hubert, Clémence Dehillotte, Anne Munck, Valérie David, Jinmi Baek, Laurent Mely, Stéphane Dominique, Sophie Ramel, Isabelle Danner Boucher, Sylvaine Lefeuvre, Quitterie Reynaud, Virginie Colomb-Jung, Prissile Bakouboula, Lydie Lemonnier
BACKGROUND: Ivacaftor has been shown to improve lung function and body weight in patients with CF and a gating mutation. Real-world evaluation is warranted to examine its safety and effectiveness over the long term. METHODS: A retrospective observational multicentre study collected clinical data in the year before and the 2years after ivacaftor initiation in patients with CF and a Gly551Asp-CFTR mutation. RESULTS: Fifty-seven patients were included...
July 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28711221/the-evolution-of-lung-transplantation-for-cystic-fibrosis-a-2017-update
#5
REVIEW
Gregory Snell, Anna Reed, Marc Stern, Denis Hadjiliadis
Lung transplantation (LTx) is an established therapy for patients with end-stage cystic fibrosis (CF). Indeed, CF is the commonest indication for those aged<50years of age needing LTx. CF LTx is associated with a 45% 10year survival - according to the world's largest registry. It is important all otherwise suitable CF patients with severe lung disease have a timely referral for discussion and consideration of the possibility of LTx. LTx discussions must carefully consider colonisation or infection with Burkholderia cenocepacia, Mycobacterium abscessus and Scediosporium - as good LTx outcomes cannot be guaranteed...
July 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28690130/investigating-the-variation-in-the-incidence-of-new-pseudomonas-aeruginosa-infection-between-paediatric-cystic-fibrosis-centres
#6
LETTER
F J Gilchrist, A M Jones, A R Smyth, K W Southern, A K Webb, W Lenney
No abstract text is available yet for this article.
July 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28651844/recovery-of-lung-function-following-a-pulmonary-exacerbation-in-patients-with-cystic-fibrosis-and-the-g551d-cftr-mutation-treated-with-ivacaftor
#7
Patrick A Flume, Claire E Wainwright, D Elizabeth Tullis, Sally Rodriguez, Minoo Niknian, Mark Higgins, Jane C Davies, Jeffrey S Wagener
BACKGROUND: Pulmonary exacerbations (PEx) are associated with acute loss of lung function that is often not recovered after treatment. We investigated lung function recovery following PEx for ivacaftor- and placebo-treated subjects. METHODS: Short- and long-term pulmonary function recovery data after PEx were summarized from a placebo-controlled trial in 161 cystic fibrosis patients≥12years old with the G551D-CFTR mutation (NCT00909532). Short-term recovery was measured 2 to 8weeks after treatment, and long-term recovery was determined at the end-of-study, both compared with baseline measured just prior to the PEx...
June 24, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28651845/a-single-bout-of-maximal-exercise-improves-lung-function-in-patients-with-cystic-fibrosis
#8
Matthew A Tucker, Reva Crandall, Nichole Seigler, Paula Rodriguez-Miguelez, Kathleen T McKie, Caralee Forseen, Jeffrey Thomas, Ryan A Harris
BACKGROUND: Responses to a single bout of exercise may provide critical information for maximizing improvements in pulmonary function following exercise training in cystic fibrosis (CF). We sought to determine if acute maximal exercise improves pulmonary function in patients with CF. METHODS: Thirty-three patients with CF completed a comprehensive assessment of pulmonary function to determine forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and lung clearance index (LCI) prior to and immediately following maximal aerobic exercise on a cycle ergometer...
June 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28648493/the-use-of-fructosamine-in-cystic-fibrosis-related-diabetes-cfrd-screening
#9
Grace Y Lam, Michelle Doll-Shankaruk, Jan Dayton, Karina Rodriguez-Capote, Trefor N Higgins, Dylan Thomas, Kimberley Mulchey, Maeve P Smith, Neil E Brown, Winnie M Leung, Mathew P Estey
OBJECTIVE: To determine whether serum fructosamine correlates with glycemic control and clinical outcomes in patients being screened for cystic fibrosis-related diabetes (CFRD). METHODS: Fructosamine and percent predicted forced expiratory volume in 1s (FEV1) were measured in patients undergoing a 2h oral glucose tolerance test (OGTT) for CFRD screening. Fractional serum fructosamine (FSF) was calculated as fructosamine/total protein. RESULTS: FSF exhibited a positive correlation with 2h OGTT results (r(2)=0...
June 22, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28625799/continuous-glucose-monitoring-in-a-cystic-fibrosis-patient-to-predict-pulmonary-exacerbation
#10
T B Inman, J A Proudfoot, M Lim, C Demeterco-Berggren
Patients with cystic fibrosis (CF) experience a significant decline in pulmonary status before the diagnosis of cystic fibrosis related diabetes (CFRD). We hypothesized that hyperglycemia may be a factor in the decline of pulmonary function and increased frequency of pulmonary exacerbations. Long term continuous glucose monitoring (CGM) has not been reported in patients with CF and impaired glucose tolerance. We performed CGM for three months in a 17year old male with F508del and F553X CF mutations, baseline forced expiratory volume in 1s (FEV1) of 92% predicted, and impaired glucose tolerance to evaluate changes in glucose levels prior to the diagnosis of a pulmonary exacerbation...
June 15, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28599957/pulmonary-surfactant-dysfunction-in-pediatric-cystic-fibrosis-mechanisms-and-reversal-with-a-lipid-sequestering-drug
#11
Lasantha Gunasekara, Mustafa Al-Saiedy, Francis Green, Ryan Pratt, Candice Bjornson, Ailian Yang, W Michael Schoel, Ian Mitchell, Mary Brindle, Mark Montgomery, Elizabeth Keys, John Dennis, Grishma Shrestha, Matthias Amrein
BACKGROUND: Airway surfactant is impaired in cystic fibrosis (CF) and associated with declines in pulmonary function. We hypothesized that surfactant dysfunction in CF is due to an excess of cholesterol with an interaction with oxidation. METHODS: Surfactant was extracted from bronchial lavage fluid from children with CF and surface tension, and lipid content, inflammatory cells and microbial flora were determined. Dysfunctional surfactant samples were re-tested with a lipid-sequestering agent, methyl-β-cyclodextrin (MβCD)...
June 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28579360/complications-of-long-and-intermediate-term-venous-catheters-in-cystic-fibrosis-patients-a-multicenter-study
#12
Teresa L May, Alex H Gifford, Thomas Lahiri, Adam Black, Janet Trang, Alexandra G Cornell, Karyll Gonzalez, Scott Morin, Mark Napier, Christine W Duarte, Jonathan B Zuckerman
BACKGROUND: Totally implantable venous access devices (TIVADs) or peripherally inserted central venous catheters (PICCs) are commonly used in the care of patients with cystic fibrosis (CF), but they are associated with various complications, including thrombosis, infection, and insertion site symptoms. METHODS: We conducted a retrospective review of PICC and TIVAD use in adults and children with CF over an 8-year period at 3 accredited care centers. Patient attributes included CFTR genotype, comorbidities, lung function, body mass index, use of anticoagulation, and respiratory tract microbiology...
June 1, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28549610/combined-lung-liver-pancreas-transplantation-in-a-recipient-with-cystic-fibrosis
#13
A S Barbas, M J Dib, D P Al-Adra, N Goldaracena, G Sapisochin, T K Waddell, S Keshavjee, N Selzner, C Chaparro, M S Cattral
Cystic fibrosis (CF) affects multiple organs including the lung, liver, and pancreas. Lung transplant, liver transplant, and combined lung-liver transplant have become well-established therapies for CF patients with end-stage organ failure. Thus far, however, there has been limited experience with pancreas transplantation in CF. In this report, we detail the clinical history, transplant procedure, and post-operative recovery of a patient who underwent combined lung-liver-pancreas transplant for advanced CF.
May 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28549609/development-and-validation-of-cf-medication-beliefs-questionnaire-a-mixed-methods-approach
#14
Michelle N Eakin, Shang-En Chung, Jessica Hoehn, Belinda Borrelli, Devin Rand-Giovannetti, Kristin A Riekert
BACKGROUND: Beliefs about medication have been associated with adherence in other diseases but there are no existing disease-specific medication beliefs questionnaires for CF. This mixed-methods validated the Cystic Fibrosis Medication Belief Questionnaire (CF-MBQ), based on social cognitive theory. METHODS: Based on previous research, items were developed for five domains: motivation, self-efficacy, perceived importance, and decisional balance to take or miss medications...
May 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28539224/inhaled-enac-antisense-oligonucleotide-ameliorates-cystic-fibrosis-like-lung-disease-in-mice
#15
Jeff R Crosby, Chenguang Zhao, Chong Jiang, Dong Bai, Melanie Katz, Sarah Greenlee, Hiroshi Kawabe, Michael McCaleb, Daniela Rotin, Shuling Guo, Brett P Monia
BACKGROUND: Epithelial sodium channel (ENaC, Scnn1) hyperactivity in the lung leads to airway surface dehydration and mucus accumulation in cystic fibrosis (CF) patients and in mice with CF-like lung disease. METHODS: We identified several potent ENaC specific antisense oligonucleotides (ASOs) and tested them by inhalation in mouse models of CF-like lung disease. RESULTS: The inhaled ASOs distributed into lung airway epithelial cells and decreased ENaC expression by inducing RNase H1-dependent degradation of the targeted Scnn1a mRNA...
May 20, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28529073/real-life-acute-lung-function-changes-after-lumacaftor-ivacaftor-first-administration-in-pediatric-patients-with-cystic-fibrosis
#16
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/28479021/what-keeps-children-with-cystic-fibrosis-awake-at-night
#17
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
#18
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
#19
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
#20
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
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