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lung function tests in cystic fibrosis

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https://www.readbyqxmd.com/read/29140739/cftr-genotype-and-maximal-exercise-capacity-in-cystic-fibrosis-a-cross-sectional-study
#1
Thomas Radtke, Helge Hebestreit, Sabina Gallati, Jane E Schneiderman, Julia Braun, Daniel Stevens, Erik Hj Hulzebos, Tim Takken, Steven R Boas, Don S Urquhart, Larry C Lands, Sergio Tejero, Aleksandar Sovtic, Tiffany Dwyer, Milos Petrovic, Ryan A Harris, Chantal Karila, Daniela Savi, Jakob Usemann, Meir Mei-Zahav, Elpis Hatziagorou, Felix Ratjen, Susi Kriemler
RATIONALE: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with CF may present an important determinant of aerobic exercise capacity in CF. Previous studies on the relationship between CFTR genotype and aerobic exercise capacity are scarce and contradictory. OBJECTIVES: This study was designed to explore factors influencing aerobic exercise capacity, expressed as peak oxygen consumption (VO2peak) with a specific focus on CFTR genotype in children and adults with CF...
November 15, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29136345/regional-lung-function-testing-in-children-using-electrical-impedance-tomography
#2
Barbara Vogt, Sarah Löhr, Zhanqi Zhao, Christian Falkenberg, Tobias Ankermann, Norbert Weiler, Inéz Frerichs
OBJECTIVE: To evaluate regional lung function in lung-healthy children before and after exercise challenge using electrical impedance tomography (EIT). METHODS: Regional lung function was examined using EIT in 100 lung-healthy children (three age subgroups: 74-121, 122-155, 156-195 months) at baseline and 10 min after exercise. Global lung function was assessed by spirometry using Z-Scores of FEV1 , FVC, FEV1 /FVC, and FEF75 . The same lung function measures were determined in 912 EIT image pixels to enable the spatial and temporal ventilation distribution analysis...
November 14, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29122914/pulmonary-function-deficits-in-newborn-screened-infants-with-cystic-fibrosis-managed-with-standard-uk-care-are-mild-and-transient
#3
Gwyneth Davies, Janet Stocks, Lena P Thia, Ah-Fong Hoo, Andrew Bush, Paul Aurora, Lucy Brennan, Simon Lee, Sooky Lum, Philippa Cottam, Joanne Miles, Jane Chudleigh, Jane Kirkby, Ian M Balfour-Lynn, Siobhán B Carr, Colin Wallis, Hilary Wyatt, Angie Wade
With the advent of novel designer molecules for cystic fibrosis (CF) treatment, there is huge need for early-life clinical trial outcomes, such as infant lung function (ILF). We investigated the degree and tracking of ILF abnormality during the first 2 years of life in CF newborn screened infants.Forced expiratory volume in 0.5 s (FEV0.5), lung clearance index (LCI) and plethysmographic functional residual capacity were measured at ∼3 months, 1 year and 2 years in 62 infants with CF and 34 controls...
November 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29115018/relationship-between-exercise-capacity-and-glucose-tolerance-in-cystic-fibrosis
#4
Karla Foster, Guixia Huang, Nanhua Zhang, Joseph Crisalli, Barbara Chini, Raouf Amin, Deborah Elder
BACKGROUND: Improved exercise capacity (EC) and normal glucose tolerance (NGT) are independently associated with favorable outcomes in CF, however, little information on this relationship exists in patients with CF. METHODS: Cardiopulmonary exercise tests, oral glucose tolerance tests (OGTT), and HbA1c values measured within a 12-month period were evaluated on 83 pediatric patients diagnosed with CF. Patients were categorized as having NGT, abnormal glucose tolerance (AGT), or CF-related diabetes (CFRD)...
November 8, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29101284/genetic-mannose-binding-lectin-deficiency-is-associated-with-airway-microbiota-diversity-and-reduced-exacerbation-frequency-in-copd
#5
Alison J Dicker, Megan L Crichton, Andrew J Cassidy, Gill Brady, Adrian Hapca, Roger Tavendale, Gisli G Einarsson, Elizabeth Furrie, J Stuart Elborn, Stuart Schembri, Sara E Marshall, Colin N A Palmer, James D Chalmers
BACKGROUND: In cystic fibrosis and bronchiectasis, genetic mannose binding lectin (MBL) deficiency is associated with increased exacerbations and earlier mortality; associations in COPD are less clear. Preclinical data suggest MBL interferes with phagocytosis of Haemophilus influenzae, a key COPD pathogen. We investigated whether MBL deficiency impacted on clinical outcomes or microbiota composition in COPD. METHODS: Patients with COPD (n=1796) underwent MBL genotyping; linkage to health records identified exacerbations, lung function decline and mortality...
November 3, 2017: Thorax
https://www.readbyqxmd.com/read/29099608/mucin-production-and-hydration-responses-to-mucopurulent-materials-in-normal-vs-cf-airway-epithelia
#6
Lubna H Abdullah, Raymond Coakley, Megan J Webster, Yunxiang Zhu, Robert Tarran, Giorgia Radicioni, Mehmet Kesimer, Richard C Boucher, C William Davis, Carla M P Ribeiro
RATIONALE: Cystic fibrosis (CF) airways disease produces a muco-obstructive lung phenotype characterized by airways mucus plugging, epithelial mucous cell metaplasia/hyperplasia, chronic infection and inflammation. Simultaneous biochemical and functional in vivo studies of mucin synthesis and secretion from CF airways are not available. In vitro translational models may quantitate differential CF vs. normal mucin and fluid secretory responses to infectious/inflammatory stimuli. OBJECTIVES: We tested the hypothesis that CF airways exhibit defective epithelial fluid, but not mucin, secretory responses to bacterial/inflammatory host products...
November 3, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29042177/antibiotic-exposure-and-interpersonal-variance-mask-the-effect-of-ivacaftor-on-respiratory-microbiota-composition
#7
Anton Y Peleg, Jocelyn M Choo, Katherine M Langan, Deirdre Edgeworth, Dominic Keating, John Wilson, Geraint B Rogers, Tom Kotsimbos
BACKGROUND: G551D is a class III mutation of the cystic fibrosis transmembrane regulator (CFTR) that results in impaired chloride channel function in cystic fibrosis (CF). Ivacaftor, a CFTR-potentiating agent improves sweat chloride, weight, lung function, and pulmonary exacerbation rate in CF patients with G551D mutations, but its effect on the airway microbiome remains poorly characterised. METHODS: Twenty CF patients with at least one G551D mutation from a single centre were recruited to a 4month double-blind, placebo-controlled, crossover study of ivacaftor with 28days of active treatment...
October 15, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28981972/standard-versus-biofilm-antimicrobial-susceptibility-testing-to-guide-antibiotic-therapy-in-cystic-fibrosis
#8
REVIEW
Valerie Waters, Felix Ratjen
BACKGROUND: The antibiotics used to treat pulmonary infections in people with cystic fibrosis are typically chosen based on the results of antimicrobial susceptibility testing performed on bacteria traditionally grown in a planktonic mode (grown in a liquid). However, there is considerable evidence to suggest that Pseudomonas aeruginosa actually grows in a biofilm (or slime layer) in the airways of people with cystic fibrosis with chronic pulmonary infections. Therefore, choosing antibiotics based on biofilm rather than conventional antimicrobial susceptibility testing could potentially improve response to treatment of Pseudomonas aeruginosa in people with cystic fibrosis...
October 5, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28965267/exercise-performance-and-quality-of-life-in-children-with-cystic-fibrosis-and-mildly-impaired-lung-function-relation-with-antibiotic-treatments-and-hospitalization
#9
Kristof Vandekerckhove, Michiel Keyzer, Jasper Cornette, Ilse Coomans, Filip Pyl, Frans De Baets, Petra Schelstraete, Filomeen Haerynck, Daniel De Wolf, Sabine Van Daele, Jan Boone
This study evaluates the impact of antibiotic treatments and hospitalization on exercise performance and health-related quality of life (QOL) in children with mild cystic fibrosis (CF) lung disease. Forty-seven children between 7 and 17 years with mild CF underwent a maximal exercise test including spiro-ergometry and filled out a QOL-questionnaire (PedsQL™). Amount of antibiotic treatments (AB) and hospitalization days in the last 3 years were reviewed. FEV1% was mildly decreased (91.7 ± 17.9 L/min, p = 0...
December 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28965239/burkholderia-cepacia-complex-in-cystic-fibrosis-in-a-brazilian-reference-center
#10
Priscila Dentini, Fernando Augusto Lima Marson, Luciana Cardoso Bonadia, Carmen Sílvia Bertuzzo, Antônio Fernando Ribeiro, Carlos Emílio Levy, José Dirceu Ribeiro
The Burkholderia cepacia complex (BCC) can cause a severe decline in lung function in cystic fibrosis (CF). Our objective was to determine the BCC prevalence and to evaluate its clinical impact on CF. Clinical and laboratory variables were determined for CF patients with BCC (Group-A = 50 patients) and without BCC (Group-B = 134 patients). The microorganisms were identified by biochemical tests, the Vitek2(®)Compact test, recA-PCR and recA-nested-PCR with species-specific primers and DNA sequencing. The patients were evaluated by the Shwachman-Kulczycki score (SKCS), Bhalla score (BS), spirometry and body mass index (BMI)...
December 2017: Medical Microbiology and Immunology
https://www.readbyqxmd.com/read/28955034/microrna-9-downregulates-the-ano1-chloride-channel-and-contributes-to-cystic-fibrosis-lung-pathology
#11
Florence Sonneville, Manon Ruffin, Christelle Coraux, Nathalie Rousselet, Philippe Le Rouzic, Sabine Blouquit-Laye, Harriet Corvol, Olivier Tabary
Cystic fibrosis results from reduced cystic fibrosis transmembrane conductance regulator protein activity leading to defective epithelial ion transport. Ca(2+)-activated Cl(-) channels mediate physiological functions independently of cystic fibrosis transmembrane conductance regulator. Anoctamin 1 (ANO1/TMEM16A) was identified as the major Ca(2+)-activated Cl(-) channel in airway epithelial cells, and we recently demonstrated that downregulation of the anoctamin 1 channel in cystic fibrosis patients contributes to disease severity via an unknown mechanism...
September 27, 2017: Nature Communications
https://www.readbyqxmd.com/read/28948140/sepsis-and-acute-respiratory-distress-syndrome-requiring-extracorporeal-life-support-in-an-adolescent-with-mild-cystic-fibrosis
#12
Lauren Elizabeth Faricy, Gwynne Church
Outcomes for invasive mechanical ventilation and extracorporeal membrane oxygenation (ECMO) to treat acute respiratory failure in patients with mild cystic fibrosis (CF) lung disease are not known. We present a case of the successful use of ECMO to treat acute respiratory failure secondary to staphylococcal sepsis in an adolescent CF patient with previously normal lung function. Her post-ECMO course was notable for severe airflow obstruction, hypoxemia, deconditioning, and growth failure. She had significantly improved at six months follow-up, though she continued to have moderate airflow obstruction on pulmonary function testing...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28919081/body-weight-and-body-mass-index-in-patients-with-end-stage-cystic-fibrosis-stabilize-after-the-start-of-enteral-tube-feeding
#13
Francis M Hollander, Nicole M de Roos, Gerdien Belle van Meerkerk, Ferdinand Teding van Berkhout, Harry G M Heijerman, Ed A van de Graaf
BACKGROUND: Enteral tube feeding (ETF) is widely used in patients with cystic fibrosis (CF) and end-stage lung disease, but previous studies have been limited to investigating whether ETF improves outcomes in patients with moderately or mildly impaired pulmonary function. OBJECTIVE: This study investigated body weight, body mass index (BMI; calculated as kg/m(2)), pulmonary function, and the presence of CF-related diabetes before and after the start of ETF. DESIGN: This was a retrospective observational study...
November 2017: Journal of the Academy of Nutrition and Dietetics
https://www.readbyqxmd.com/read/28871575/inert-gas-washout-background-and-application-in-various-lung-diseases
#14
Jakob Usemann, Sophie Yammine, Florian Singer, Philipp Latzin
Multiple breath inert gas washout (MBW) is a lung function technique to measure ventilation inhomogeneity. The technique was developed more than 60 years ago, but not much used for many decades. Technical improvements, easy protocols and higher sensitivity compared with standard lung function tests in some disease groups have led to a recent renaissance of MBW. The lung clearance index (LCI) is a common measure derived from MBW tests, and offers information on lung pathology complementary to that from conventional lung function tests such as spirometry...
September 5, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28862396/association-between-lung-function-physical-activity-level-and-postural-evaluation-variables-in-adult-patients-with-cystic-fibrosis
#15
Inaê Angélica Cherobin, Paulo de Tarso Roth Dalcin, Bruna Ziegler
INTRODUCTION: With the evolution of the cystic fibrosis (CF) disease, the decline of lung function associated with metabolic disorders and malnutrition, causes alterations in respiratory mechanics, musculoskeletal disorders and thoracic deformities, bringing injury to the individual's quality of life. OBJECTIVE: To verify the association between lung function, physical activity level and postural evaluation variables in adults with CF. METHODS: All patients underwent clinical evaluation and spirometry...
September 1, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28833401/a-novel-approach-based-on-low-field-nmr-for-the-detection-of-the-pathological-components-of-sputum-in-cystic-fibrosis-patients
#16
Michela Abrami, Fiorentina Ascenzioni, Enea Gino Di Domenico, Massimo Maschio, Alessandro Ventura, Marco Confalonieri, Sante Di Gioia, Massimo Conese, Barbara Dapas, Gabriele Grassi, Mario Grassi
PURPOSE: Development of a reliable, simple method to monitor lung condition in cystic fibrosis (CF) patients. Lung functionality assessment in CF patients is relevant, as most of them still die of respiratory failure. In lung mucus (sputum) of CF patients, components such as proteins, biopolymers, DNA, bacteria, and mucin are pathologically increased. As lung functionality is related to the amount of the pathological components in the sputum, their determination can help clinicians in monitoring lung condition and planning therapy...
August 22, 2017: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
https://www.readbyqxmd.com/read/28799841/high-usability-of-a-smartphone-application-for-reporting-symptoms-in-adults-with-cystic-fibrosis
#17
Jamie Wood, Sue Jenkins, David Putrino, Siobhain Mulrennan, Sue Morey, Nola Cecins, Kylie Hill
Introduction In cystic fibrosis, exacerbations impair lung function and health-related quality of life, increase healthcare costs and reduce survival. Delayed reporting of worsening symptoms can result in more severe exacerbations and worse clinical outcomes; therefore there is a need for a novel approach to facilitate the early identification and treatment of exacerbations in this population. This study investigated the usability of a smartphone application to report symptoms in adults with cystic fibrosis, and the observer agreement in clinical decision-making between senior clinicians interpreting smartphone application responses...
January 1, 2017: Journal of Telemedicine and Telecare
https://www.readbyqxmd.com/read/28767774/factors-associated-with-malnutrition-in-adolescent-and-adult-patients-with-cystic-fibrosis
#18
Gabriela Cristofoli Barni, Gabriele Carra Forte, Luis Felipe Forgiarini, Claudine Lacerda de Oliveira Abrahão, Paulo de Tarso Roth Dalcin
Objective: To determine the prevalence of malnutrition in patients attending an adult cystic fibrosis (CF) program and to investigate the associations of malnutrition with the clinical characteristics of those patients. Methods: This was a cross-sectional study involving patients with clinically stable CF patients (16 years of age or older). The patients underwent clinical assessment, nutritional assessments, pulmonary function tests, and pancreatic function assessment...
July 31, 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/28754426/relevance-of-multidrug-resistant-pseudomonas-aeruginosa-infections-in-cystic-fibrosis
#19
REVIEW
S Stefani, S Campana, L Cariani, V Carnovale, C Colombo, M M Lleo, V D Iula, L Minicucci, P Morelli, G Pizzamiglio, G Taccetti
Multidrug-resistant (MDR) Pseudomonas aeruginosa is an important issue for physicians who take care of patients with cystic fibrosis (CF). Here, we review the latest research on how P. aeruginosa infection causes lung function to decline and how several factors contribute to the emergence of antibiotic resistance in P. aeruginosa strains and influence the course of the infection course. However, many aspects of the practical management of patients with CF infected with MDR P. aeruginosa are still to be established...
September 2017: International Journal of Medical Microbiology: IJMM
https://www.readbyqxmd.com/read/28737262/overnight-delivery-of-hypertonic-saline-by-nasal-cannula-aerosol-for-cystic-fibrosis
#20
Timothy E Corcoran, Joseph E Godovchik, Karl H Donn, David R Busick, Jennifer Goralski, Landon W Locke, Matthew R Markovetz, Michael M Myerburg, Ashok Muthukrishnan, Lawrence Weber, Ryan T Lacy, Joseph M Pilewski
AIM: Inhaled hypertonic saline increases mucociliary clearance, improves pulmonary function, and decreases exacerbations in cystic fibrosis (CF) but contributes to the already significant treatment burden of CF. Overnight delivery of inhaled medications via a specially designed nasal cannula-aerosol device (Trans-nasal Pulmonary Aerosol Delivery [tPAD]) is an alternative approach. Here, we test whether overnight inhalation of hypertonic saline via tPAD improves mucociliary clearance and assess the tolerability of the device...
September 2017: Pediatric Pulmonology
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