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Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society

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https://read.qxmd.com/read/30879989/effects-of-lumacaftor-ivacaftor-on-physical-activity-and-exercise-tolerance-in-three-adults-with-cystic-fibrosis
#1
Daniela Savi, Stefano Schiavetto, Nicholas J Simmonds, Dario Righelli, Paolo Palange
The combination of the corrector lumacaftor with the potentiator ivacaftor has been approved for treatment of cystic fibrosis (CF) patients homozygous for the Phe508del CFTR mutation. There are no reports detailing the effect of lumacaftor-ivacaftor on physical activity (PA) and exercise tolerance. We performed incremental cardiopulmonary exercise testing (CPET) and we assessed PA pre- and post 2 years initiation of lumacaftor-ivacaftor in three CF adults. PA of mild intensity improved by +13% in patient 1, + 84% in patients 2 and + 89% in patient 3...
March 14, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30857926/the-effects-of-cycled-inhaled-aztreonam-on-the-cystic-fibrosis-cf-lung-microbiome
#2
Alya A Heirali, Nicole Acosta, Douglas G Storey, Matthew L Workentine, Ranjani Somayaji, Isabelle Laforest-Lapointe, Winnie Leung, Bradley S Quon, Yves Berthiaume, Harvey R Rabin, Barbara J Waddell, Laura Rossi, Michael G Surette, Michael D Parkins
BACKGROUND: To improve clinical outcomes, cystic fibrosis (CF) patients with chronic Pseudomonas aeruginosa infections are prescribed inhaled anti-pseudomonal antibiotics. Although, a diverse microbial community exists within CF airways, little is known about how the CF microbiota influences patient outcomes. We hypothesized that organisms within the CF microbiota are affected by inhaled-antibiotics and baseline microbiome may be used to predict therapeutic response. METHODS: Adults with chronic P...
March 8, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30853372/investigating-transmission-of-mycobacterium-abscessus-amongst-children-in-an-australian-cystic-fibrosis-centre
#3
Jennifer Yan, Ajay Kevat, Elena Martinez, Nicky Teese, Kareena Johnson, Sarath Ranganathan, Jo Harrison, John Massie, Andrew Daley
BACKGROUND: Mycobacterium abscessus is an emerging pathogen in cystic fibrosis (CF) lung disease. Hospital transmission of M. abscessus has been described. This paper details the investigation into possible cross-transmission of M. abscessus locally at our paediatric hospital CF centre, and the subsequent infection control response. METHODS: Whole genome sequencing (WGS) of M. abscessus respiratory isolates with epidemiological linkage analysis using hospital electronic medical records...
March 7, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30833123/comment-on-comparison-of-lung-clearance-index-determined-by-washout-of-n2-and-sf6-in-infants-and-preschool-children-with-cystic-fibrosis
#4
LETTER
Alex Horsley, Claire Nissenbaum, Lokesh Guglani, Daniel Weiner
No abstract text is available yet for this article.
March 1, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30827846/abnormal-preschool-lung-clearance-index-lci-reflects-clinical-status-and-predicts-lower-spirometry-later-in-childhood-in-cystic-fibrosis
#5
K M Hardaker, H Panda, K Hulme, A Wong, E Coward, P Cooper, D A Fitzgerald, C Pandit, S Towns, H Selvadurai, P D Robinson
BACKGROUND: Clinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear. METHODS: Initial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2-6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8.0). RESULTS: LCI was abnormal in 56% (28/50), with mean (SD) LCI 8...
February 28, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30826285/early-respiratory-viral-infections-in-infants-with-cystic-fibrosis
#6
Ashley R Deschamp, Joseph E Hatch, James E Slaven, Netsanet Gebregziabher, Gregory Storch, Graham L Hall, Stephen Stick, Sarath Ranganathan, Thomas W Ferkol, Stephanie D Davis
BACKGROUND: Viral infections contribute to morbidity in cystic fibrosis (CF), but the impact of respiratory viruses on the development of airway disease is poorly understood. METHODS: Infants with CF identified by newborn screening were enrolled prior to 4 months of age to participate in a prospective observational study at 4 centers. Clinical data were collected at clinic visits and weekly phone calls. Multiplex PCR assays were performed on nasopharyngeal swabs to detect respiratory viruses during routine visits and when symptomatic...
February 27, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30824378/associating-antimicrobial-susceptibility-testing-with-clinical-outcomes-in-cystic-fibrosis-more-rigor-and-less-frequency
#7
EDITORIAL
Rhonda D Szczesniak, Jonathan D Cogen, Margaret Rosenfeld
No abstract text is available yet for this article.
February 26, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30803905/decreased-mrna-and-protein-stability-of-w1282x-limits-response-to-modulator-therapy
#8
M A Aksit, A D Bowling, T A Evans, A T Joynt, D Osorio, S Patel, N West, C Merlo, P R Sosnay, G R Cutting, N Sharma
BACKGROUND: Cell-based studies have shown that W1282X generates a truncated protein that can be functionally augmented by modulators. However, modulator treatment of primary cells from individuals who carry two copies of W1282X generates no functional CFTR. To understand the lack of response to modulators, we investigated the effect of W1282X on CFTR RNA transcript levels. METHODS: qRT-PCR and RNA-seq were performed on primary nasal epithelial (NE) cells of a previously studied individual who is homozygous for W1282X, her carrier parents and control individuals without nonsense variants in CFTR...
February 22, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30797725/biofabrication-of-personalised-anatomical-models-and-tools-for-the-clinic
#9
EDITORIAL
Mark C Allenby, Maria A Woodruff
No abstract text is available yet for this article.
February 20, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30797724/cystic-fibrosis-we-see-fungus-among-us-but-should-we-care
#10
EDITORIAL
Gina Hong, Noah Lechtzin
No abstract text is available yet for this article.
February 20, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30795973/interprofessional-provider-educational-needs-and-preferences-regarding-the-provision-of-sexual-and-reproductive-health-care-in-cystic-fibrosis
#11
Traci M Kazmerski, Eliza B Nelson, Lori R Newman, Miriam J Haviland, Donna Luff, Alan M Leichtner, Margaret M Hayes, Elizabeth Miller, S Jean Emans, Gregory S Sawicki
BACKGROUND: Sexual and reproductive health (SRH) is an important disease-specific concern for adolescent and young adult (AYA) women that is not consistently addressed in cystic fibrosis (CF) centers. This study identifies educational needs and preferences of interprofessional CF providers regarding SRH in AYA women with CF. METHODS: Interprofessional CF providers participated in an anonymous survey regarding general and CF-specific SRH knowledge and skills, factors for optimizing SRH care, and preferred approaches for SRH training...
February 19, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30792172/repeated-hot-water-and-steam-disinfection-of-pari-lc-plus%C3%A2-nebulizers-alters-nebulizer-output
#12
LETTER
Melanie Sue Collins, Thomas S Murray
No abstract text is available yet for this article.
February 18, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30772245/social-support-is-associated-with-fewer-reported-symptoms-and-decreased-treatment-burden-in-adults-with-cystic-fibrosis
#13
Kassie D Flewelling, Deborah E Sellers, Gregory S Sawicki, Walter M Robinson, Edward J Dill
BACKGROUND: Although social support has been linked to a variety of health outcomes in those with and without chronic illness, this construct has rarely been studied in adults with cystic fibrosis (CF), who may face a unique set of clinical and psychosocial barriers. The current study explored the relationships between social support, mental health, physical health, treatment activity, and disease-specific quality of life in a sample of adults with CF. METHODS: Participants in the study included 250 adults with CF who took part in a larger longitudinal study...
February 13, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30772244/do-patients-with-cystic-fibrosis-participating-in-clinical-trials-demonstrate-placebo-response-a-meta-analysis
#14
Julie Coton, Ha-Hai Le, Victor Veuillet, Perrine Janiaud, Michel Cucherat, Behrouz Kassai-Koupai, François Gueyffier, Philippe Reix
BACKGROUND: Patients' and families' expectation that a cure for cystic fibrosis (CF) will be found is high. In other debilitating conditions, high expectation has been shown to drive a strong placebo response (PR). Therefore, our goal was to evaluate PR on objective continuous outcomes (FEV1 , BMI) and the CF Questionnaire Revised-Respiratory Domain (CFQR-RD) monitored during randomised clinical trials (RCTs) for CF. METHODS: We conducted a meta-analysis after a systematic review of the literature carried out to identify RCTs with FEV1 , CFQR-RD and BMI as outcome measures...
February 13, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30765182/ct-evaluation-of-hyperattenuating-mucus-to-diagnose-allergic-bronchopulmonary-aspergillosis-in-the-special-condition-of-cystic-fibrosis
#15
John Refait, Julie Macey, Stephanie Bui, Michaël Fayon, Patrick Berger, Laurence Delhaes, François Laurent, Gaël Dournes
BACKGROUND: Mucus plugging (MP), central bronchiectasis (CB), and consolidation/atelectasia (CA) are conventional CT signs to diagnose allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF). Hyperattenuating mucus (HAM) has recently been described and may improve diagnostic accuracy. The goal of our study was to compare HAM versus conventional CT signs to diagnose ABPA in CF. Secondary objectives were to determine the optimal threshold of HAM quantitatively and to assess the diagnostic value of HAM using chest radiograph (CXR)...
February 12, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30745235/how-reliable-is-your-hba1c-test-revisiting-the-use-of-hba1c-in-cystic-fibrosis-related-diabetes-cfrd-screening
#16
LETTER
Grace Y Lam, Shelby Sissons, Maeve P Smith, Neil E Brown, Winnie M Leung, Mathew P Estey
No abstract text is available yet for this article.
February 8, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30738804/incretin-dysfunction-and-hyperglycemia-in-cystic-fibrosis-role-of-acyl-ghrelin
#17
Xingshen Sun, Yaling Yi, Bo Liang, Yu Yang, Nan He, Katie Larson Ode, Aliye Uc, Kai Wang, Katherine N Gibson-Corley, John F Engelhardt, Andrew W Norris
BACKGROUND: Insulin secretion is insufficient in cystic fibrosis (CF), even before diabetes is present, though the mechanisms involved remain unclear. Acyl-ghrelin (AG) can diminish insulin secretion and is elevated in humans with CF. METHODS: We tested the hypothesis that elevated AG contributes to reduced insulin secretion and hyperglycemia in CF ferrets. RESULTS: Fasting AG was elevated in CF versus non-CF ferrets. Similar to its effects in other species, AG administration in non-CF ferrets acutely reduced insulin, increased growth hormone, and induced hyperglycemia...
February 6, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30738803/authors-response-letter-to-the-editor-comparison-of-lung-clearance-index-determined-by-washout-of-n2-and-sf6-in-infants-and-preschool-children-with-cystic-fibrosis
#18
LETTER
Mirjam Stahl, Cornelia Joachim, Mark O Wielpütz, Marcus A Mall
No abstract text is available yet for this article.
February 6, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30738802/lumacaftor-rescued-f508del-cftr-has-a-modified-bicarbonate-permeability
#19
Loretta Ferrera, Debora Baroni, Oscar Moran
Deletion of phenylalanine at position 508, F508del, the most frequent mutation among Cystic fibrosis (CF) patients, destabilizes the protein, thus causing both a folding and a trafficking defect, resulting in a dramatic reduction in expression of CFTR. In vitro treatment with lumacaftor produces an enhancement of anion transport in cells. We studied the permeability properties of the CFTR mutant F508del treated with the corrector lumacaftor, showing that the rescued protein has selectivity properties different than the wild type CFTR, showing an augmented bicarbonate permeability...
February 6, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://read.qxmd.com/read/30738801/health-economic-modelling-in-cystic-fibrosis-a-systematic-review
#20
REVIEW
Bishal Mohindru, David Turner, Tracey Sach, Diana Bilton, Siobhan Carr, Olga Archangelidi, Arjun Bhadhuri, Jennifer A Whitty
INTRODUCTION: Cystic Fibrosis (CF) is a heritable chronic condition. Due to the genetic and progressive nature of CF, a number of interventions are available for the condition. In the United Kingdom (U.K.) average annual cost of CF treatment is between €49,000 to €76,000 (2012) per patient [1]. A review of health economic modelling studies is warranted to provide decision makers and researchers with an in depth understanding of modelling practices in CF and guidance for future research...
February 6, 2019: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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