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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/28625799/continuous-glucose-monitoring-in-a-cystic-fibrosis-patient-to-predict-pulmonary-exacerbation
#1
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/28610881/impaired-innate-immune-cells-in-cystic-fibrosis-is-it-really-a-surprise
#2
EDITORIAL
Tracey Bonfield, James F Chmiel
No abstract text is available yet for this article.
June 10, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28602538/worsening-anxiety-and-depression-after-initiation-of-lumacaftor-ivacaftor-combination-therapy-in-adolescent-females-with-cystic-fibrosis
#3
Cameron J McKinzie, Jennifer L Goralski, Terry L Noah, George Z Retsch-Bogart, Mary Beth Prieur
In both phase III studies of LUM/IVA, as well as an extension study, worsening of mental health was not reported as a common side effect. Here we describe five cases in adolescent female patients that suggest a worsening of anxiety or depression associated with its use. In these five patients, two experienced suicidal ideation and three made suicide attempts that resulted in psychiatric hospitalizations.
June 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28601425/the-changing-face-of-nutrition-in-cystic-fibrosis
#4
EDITORIAL
Susan P Wolfe, Clare Collins
No abstract text is available yet for this article.
June 7, 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
#5
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/28599956/editorial-to-don-t-judge-a-book-by-its-cover-the-emerging-challenge-of-diagnosing-cf-in-non-caucasians
#6
EDITORIAL
Melanie Chin, Lisa Strug, Anne L Stephenson
No abstract text is available yet for this article.
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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