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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/28215621/acetic-acid-is-elevated-in-the-exhaled-breath-of-cystic-fibrosis-patients
#1
LETTER
Patrik Španěl, Kristýna Sovová, Kseniya Dryahina, Tereza Doušová, Pavel Dřevínek, David Smith
No abstract text is available yet for this article.
February 16, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28209466/pharmacokinetics-and-safety-of-cavosonstat-n91115-in-healthy-and-cystic-fibrosis-adults-homozygous-for-f508del-cftr
#2
Scott H Donaldson, George M Solomon, Pamela L Zeitlin, Patrick A Flume, Alicia Casey, Karen McCoy, Edith T Zemanick, Arun Mandagere, Janice M Troha, Steven A Shoemaker, James F Chmiel, Jennifer L Taylor-Cousar
BACKGROUND: Cavosonstat (N91115), an orally bioavailable inhibitor of S-nitrosoglutathione reductase, promotes cystic fibrosis transmembrane conductance regulator (CFTR) maturation and plasma membrane stability, with a mechanism of action complementary to CFTR correctors and potentiators. METHODS: A Phase I program evaluated pharmacokinetics, drug-drug interactions and safety of cavosonstat in healthy and cystic fibrosis (CF) subjects homozygous for F508del-CFTR...
February 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28202251/high-incidence-of-non-tuberculous-mycobacteria-positive-cultures-among-adolescent-with-cystic-fibrosis
#3
Zoé Cavalli, Quitterie Reynaud, Romain Bricca, Raphaële Nove-Josserand, Stéphane Durupt, Philippe Reix, Marie Perceval, Michèle Pérouse de Montclos, Gérard Lina, Isabelle Durieu
BACKGROUND: We evaluated the prevalence of non-tuberculous mycobacteria (NTM)-positive cultures among our cystic fibrosis (CF) center patients, reviewed risk factors for NTM positivity, and determined its impact on lung function evolution. METHODS: From 2009 to 2014, CF adults and children attending the CF center of Lyon (France) and having at least one positive NTM isolate were included. Each case was matched by age and gender with two CF patients with no NTM isolate (controls)...
February 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28190780/pregnancy-among-cystic-fibrosis-women-in-the-era-of-cftr-modulators
#4
Sonya L Heltshe, Emily M Godfrey, Tatiana Josephy, Moira L Aitken, Jennifer L Taylor-Cousar
BACKGROUND: Little is known about how new therapies that partially correct the basic cystic fibrosis (CF) defect (ivacaftor and lumacaftor) might alter hormonal contraceptive effectiveness, impact pregnancy outcomes, or affect pregnancy timing. Examination of pregnancy rates among CF women during periods of CFTR modulator therapy initiation will provide foundation for further research in this area. METHODS: The Cystic Fibrosis Foundation Patient Registry was used to examine pregnancy rates and outcomes by genotype class before, during, and after the introduction of CFTR modulator therapies between 2005 and 2014...
February 9, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28189634/effectiveness-of-a-stepwise-pseudomonas-aeruginosa-eradication-protocol-in-children-with-cystic-fibrosis
#5
Ana C Blanchard, Eric Horton, Sanja Stanojevic, Louise Taylor, Valerie Waters, Felix Ratjen
INTRODUCTION: Antibiotic eradication therapy (AET) for initial Pseudomonas aeruginosa (Pa) infection is standard of care in children with cystic fibrosis (CF), but information is limited on treatment for patients who fail initial AET. The aim of this study was to evaluate the effectiveness of a multi-step protocol for AET for new-onset Pa infections in children with CF. METHODS: A three-step AET protocol which includes: (step 1) 28days of tobramycin inhalation solution (TIS) for new-onset Pa infection; (step 2) a second course of TIS for patients with positive respiratory tract culture after step 1; (step 3) 14days of intravenous antibiotics followed by 28days of TIS for patients with a subsequent positive culture...
February 9, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28188011/effect-of-posture-on-lung-ventilation-distribution-and-associations-with-structure-in-children-with-cystic-fibrosis
#6
Kathryn A Ramsey, Caroline McGirr, Stephen M Stick, Graham L Hall, Shannon J Simpson
BACKGROUND: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease. METHODS: Multiple breath washout (MBW) was performed in seated and supine postures in 25 healthy children and 21 children with CF. Children with CF also underwent a chest CT scan. Functional residual capacity (FRC), lung clearance index (LCI) and moment ratios were calculated from the MBW test. CT scans were evaluated for CF-related structural lung disease...
February 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28188010/the-1-min-sit-to-stand-test-in-cystic-fibrosis-insights-into-cardiorespiratory-responses
#7
Thomas Radtke, Helge Hebestreit, Milo A Puhan, Susi Kriemler
BACKGROUND: We aimed to characterize the cardiopulmonary response during a 1-min sit-to-stand (STS) test and compare peak exercise cardiorespiratory variables to a maximal cardiopulmonary exercise test (CPET) in cystic fibrosis (CF). We further aimed to assess the validity of the STS power index (PowerSTS) as a measure of exercise capacity. METHODS: Fifteen adult CF patients performed spirometry, CPET and the 1-min STS test with respiratory gas analysis. RESULTS: Peak-exercise cardiorespiratory variables during the 1-min STS test correlated strongly (r=0...
February 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28185888/validation-of-multiple-breath-washout-devices
#8
LETTER
Lena Raaijmakers, Renee Jensen, Sanja Stanojevic, Felix Ratjen
No abstract text is available yet for this article.
February 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28185887/fatal-disseminated-rasamsonia-infection-in-cystic-fibrosis-post-lung-transplantation
#9
Gina Hong, Marissa White, Noah Lechtzin, Natalie E West, Robin Avery, Heather Miller, Richard Lee, Robert J Lovari, Christian Massire, Lawrence B Blyn, Xinglun Liang, Deanna A Sutton, Jianmin Fu, Brian L Wickes, Nathan P Wiederhold, Sean X Zhang
BACKGROUND: Disseminated fungal infections are a known serious complication in individuals with cystic fibrosis (CF) following orthotopic lung transplantation. Aspergillus fumigatus and Scedosporium species are among the more common causes of invasive fungal infection in this population. However, it is also important for clinicians to be aware of other emerging fungal species which may require markedly different antifungal therapies. CASE SUMMARY: We describe the first laboratory-documented case of a fatal disseminated fungal infection caused by Rasamsonia aegroticola in a 21-year-old female CF patient status post-bilateral lung transplantation, which was only identified post-mortem...
February 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28185886/macronutrient-intake-in-preschoolers-with-cystic-fibrosis-and-the-relationship-between-macronutrients-and-growth
#10
Stephanie S Filigno, Shannon M Robson, Rhonda D Szczesniak, Leigh A Chamberlin, Meredith A Baker, Stephanie M Sullivan, John Kroner, Scott W Powers
BACKGROUND: Adequate nutrition is essential for growth in children with cystic fibrosis (CF). The new CF Foundation Clinical Practice Guidelines bring attention to monitoring macronutrient intake as well as total energy. METHODS: Dietary intake of 75 preschool children with CF and pancreatic insufficiency was examined and compared to the Clinical Practice Guidelines. Regression analyses examined relationships between macronutrient intake and growth. RESULTS: Approximately 45% of children met the 110% minimum recommended dietary allowance (RDA) recommendation...
February 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28179070/the-challenges-of-maintaining-momentum-in-cf-drug-development-and-approval
#11
EDITORIAL
Patrick A Flume, Donald R VanDevanter
No abstract text is available yet for this article.
February 5, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28162955/cystic-fibrosis-radiographic-biomarkers
#12
EDITORIAL
Anthony G Durmowicz
No abstract text is available yet for this article.
February 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28132845/use-of-hyperpolarized-helium-3-mri-to-assess-response-to-ivacaftor-treatment-in-patients-with-cystic-fibrosis
#13
Talissa A Altes, Mac Johnson, Meredith Fidler, Martyn Botfield, Nicholas J Tustison, Carlos Leiva-Salinas, Eduard E de Lange, Deborah Froh, John P Mugler
BACKGROUND: This pilot study evaluated the effect of short- and long-term ivacaftor treatment on hyperpolarized (3)He-magnetic resonance imaging (MRI)-defined ventilation defects in patients with cystic fibrosis aged ≥12years with a G551D-CFTR mutation. METHODS: Part A (single-blind) comprised 4weeks of ivacaftor treatment; Part B (open-label) comprised 48weeks of treatment. The primary outcome was change from baseline in total ventilation defect (TVD; total defect volume:total lung volume ratio)...
January 26, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28126444/effect-of-freezing-sputum-on-pseudomonas-aeruginosa-population-heterogeneity
#14
Ali Poonja, Alya Heirali, Matthew Workentine, Douglas G Storey, Ranjani Somayaji, Harvey R Rabin, Michael G Surette, Michael D Parkins
Pseudomonas aeruginosa develops profound population heterogeneity in CF airways. How changes in these populations relate to clinical status is unknown. In order to facilitate this understanding, frequent sampling of this community is required. To determine if the collection and storage of sputum at home may pose a viable option, we collected sputum from ten patients. Sputum samples were partitioned in two, with half immediately processed on MacConkey agar and half assessed after freezing for one week in a home-freezer...
January 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28117136/use-of-fev1-in-cystic-fibrosis-epidemiologic-studies-and-clinical-trials-a-statistical-perspective-for-the-clinical-researcher
#15
EDITORIAL
Rhonda Szczesniak, Sonya L Heltshe, Sanja Stanojevic, Nicole Mayer-Hamblett
BACKGROUND: Forced expiratory volume in 1s (FEV1) is an established marker of cystic fibrosis (CF) disease progression that is used to capture clinical course and evaluate therapeutic efficacy. The research community has established FEV1 surveillance data through a variety of observational data sources such as patient registries, and there is a growing pipeline of new CF therapies demonstrated to be efficacious in clinical trials by establishing improvements in FEV1. RESULTS: In this review, we summarize from a statistical perspective the clinical relevance of FEV1 based on its association with morbidity and mortality in CF, its role in epidemiologic studies of disease progression and comparative effectiveness, and its utility in clinical trials...
January 20, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28109711/the-impact-of-cystic-fibrosis-on-work-attendance-and-performance-in-adults-living-in-rural-and-remote-western-australia
#16
LETTER
Jamie Wood, Sue Jenkins, Siobhain Mulrennan, Kylie Hill
No abstract text is available yet for this article.
January 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28087241/chest-imaging-in-cf-studies-commentary
#17
Irmgard Eichler, Efthymios Manolis
No abstract text is available yet for this article.
January 10, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28043799/the-expansion-and-performance-of-national-newborn-screening-programmes-for-cystic-fibrosis-in-europe
#18
Jürg Barben, Carlo Castellani, Jeannette Dankert-Roelse, Silvia Gartner, Nataliya Kashirskaya, Barry Linnane, Sarah Mayell, Anne Munck, Dorota Sands, Olaf Sommerburg, Simon Pybus, Victoria Winters, Kevin W Southern
BACKGROUND: Newborn screening (NBS) for cystic fibrosis (CF) is a well-established public health strategy with international standards. The aim of this study was to provide an update on NBS for CF in Europe and assess performance against the standards. METHODS: Questionnaires were sent to key workers in each European country. RESULTS: In 2016, there were 17 national programmes, 4 countries with regional programmes and 25 countries not screening in Europe...
December 30, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28040480/non-invasive-prenatal-diagnosis-nipd-of-cystic-fibrosis-an-optimized-protocol-using-memo-fluorescent-pcr-to-detect-the-p-phe508del-mutation
#19
C Guissart, C Dubucs, C Raynal, A Girardet, F Tran Mau Them, V Debant, C Rouzier, A Boureau-Wirth, E Haquet, J Puechberty, E Bieth, D Dupin Deguine, P Khau Van Kien, M P Brechard, V Pritchard, M Koenig, M Claustres, M C Vincent
BACKGROUND: Analysis of cell-free foetal DNA (cff-DNA) in maternal plasma is very promising for early diagnosis of monogenic diseases; in particular, cystic fibrosis (CF). However, NIPD of single-gene disorders has been limited by the availability of suitable technical platforms and the need to set up patient or disease-specific custom-made approaches. METHODS: To make research applications more readily accessible to the clinic, we offer a simple assay combining two independent methods to determine the presence or absence of paternally inherited foetal allele p...
December 28, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28040479/chest-imaging-in-cystic-fibrosis-studies-what-counts-and-can-be-counted
#20
REVIEW
Rhonda Szczesniak, Lidija Turkovic, Eleni-Rosalina Andrinopoulou, Harm A W M Tiddens
BACKGROUND: The dawn of precision medicine and CFTR modulators require more detailed assessment of lung structure in cystic fibrosis (CF) clinical studies. Various imaging markers have emerged and are measurable, but clarity is needed to identify what markers should count for clinical studies. High-resolution chest computed tomography (CT) scoring has yielded sensitive markers for the study of CF disease progression. Once completed, CT scores from ongoing randomized controlled trials can be used to examine relationships between imaging endpoints and therapeutic effectiveness...
December 28, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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