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lung function tests in cystic fibrosis

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https://www.readbyqxmd.com/read/29351449/personalized-medicine-in-cf-from-modulator-development-to-therapy-for-cystic-fibrosis-patients-with-rare-cftr-mutations
#1
Misak Harutyunyan, Yunjie Huang, Kyu-Shik Mun, Fanmuyi Yang, Kavisha Arora, Anjaparavanda P Naren
Cystic fibrosis (CF) is the most common life-shortening genetic disease affecting approximately 1 in 3500 of the Caucasian population. CF is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. To date, over 2000 CFTR mutations have been identified and produce a wide range of phenotypes. The CFTR protein, a chloride channel, is normally expressed on epithelial cells lining the lung, gut, and exocrine glands. Mutations in CFTR have led to pleiotropic effects in CF patients and have resulted in early morbidity and mortality...
December 14, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29349537/timing-of-spirometry-may-impact-hospital-length-of-stay-for-cystic-fibrosis-pulmonary-exacerbation
#2
Katelyn Krivchenia, Dmitry Tumin, Christopher J Nemastil, Joseph D Tobias, Don Hayes
PURPOSE: The optimal timing of spirometry during hospitalization for acute pulmonary exacerbation (PEx) in patients with cystic fibrosis (CF) is unclear. We retrospectively evaluated whether measuring spirometry earlier during hospitalization was associated with a shorter length of stay (LOS). METHODS: In this retrospective study, we analyzed data from the electronic medical record of CF patients 6 years of age and older admitted to a single center for acute PEx requiring IV antibiotic therapy between 2009 and 2016...
January 18, 2018: Lung
https://www.readbyqxmd.com/read/29330966/static-hyperinflation-is-associated-with-ventilatory-limitation-and-exercise-tolerance-in-adult-cystic-fibrosis
#3
Daniel Stevens
INTRODUCTION: Lung hyperinflation is a potential mechanism limiting exercise tolerance. However, available data on the impact of static hyperinflation on exercise performance in adult cystic fibrosis is lacking. Furthermore, the relative contribution of both static and dynamic hyperinflation to exercise performance is unknown. OBJECTIVES: To determine the impact of static hyperinflation on exercise tolerance and lung dynamics in adult cystic fibrosis. METHODS: Clinical data of 107 adult patients with cystic fibrosis, including pulmonary function, lung volumes, and cardiopulmonary exercise from the Toronto Cystic Fibrosis database were collected and analyzed...
January 13, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29323929/variation-in-cilia-protein-genes-and-progression-of-lung-disease-in-cystic-fibrosis
#4
Elizabeth Blue, Tin L Louie, Jessica X Chong, Scott J Hebbring, Kathleen C Barnes, Nicholas M Rafaels, Michael R Knowles, Ronald L Gibson, Michael J Bamshad, Mary J Emond
RATIONALE: Cystic fibrosis, like primary ciliary dyskinesia, is an autosomal recessive disorder characterized by abnormal mucociliary clearance and obstructive lung disease. We hypothesized that genes underlying the development or function of cilia may modify lung disease severity in persons with cystic fibrosis. OBJECTIVES: To test this hypothesis, we compared variants in 93 candidate genes in both upper and lower tertiles of lung function in a large cohort of children and adults with cystic fibrosis to a population control data set...
January 11, 2018: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29221318/lung-densitometry-why-how-and-when
#5
REVIEW
Mario Mascalchi, Gianna Camiciottoli, Stefano Diciotti
Lung densitometry assesses with computed tomography (CT) the X-ray attenuation of the pulmonary tissue which reflects both the degree of inflation and the structural lung abnormalities implying decreased attenuation, as in emphysema and cystic diseases, or increased attenuation, as in fibrosis. Five reasons justify replacement with lung densitometry of semi-quantitative visual scales used to measure extent and severity of diffuse lung diseases: (I) improved reproducibility; (II) complete vs. discrete assessment of the lung tissue; (III) shorter computation times; (IV) better correlation with pathology quantification of pulmonary emphysema; (V) better or equal correlation with pulmonary function tests (PFT)...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29221293/distance-saturation-product-of-the-6-minute-walk-test-predicts-mortality-of-patients-with-non-cystic-fibrosis-bronchiectasis
#6
Meng-Heng Hsieh, Yueh-Fu Fang, Fu-Tsai Chung, Chung-Shu Lee, Yu-Chen Chang, Yuan-Zhang Liu, Cheng-Hsien Wu, Horng-Chyuan Lin
Background: Previous surveillance methods to monitor the prognoses of patients with bronchiectasis are too complex for use in daily practice. The 6-minute walk test (6MWT) is a simple exercise test to predict the prognosis of chronic obstructive airway disease and numerous chronic lung diseases, including idiopathic pulmonary fibrosis. No studies have investigated exercise-induced oxygen desaturation (EID) and distance-saturation product (DSP) of 6MWT to predict the prognoses of patients with bronchiectasis...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29217601/novel-magnetic-resonance-technique-for-functional-imaging-of-cystic-fibrosis-lung-disease
#7
Sylvia Nyilas, Grzegorz Bauman, Gregor Sommer, Enno Stranzinger, Orso Pusterla, Urs Frey, Insa Korten, Florian Singer, Carmen Casaulta, Oliver Bieri, Philipp Latzin
Lung function tests are commonly used to monitor lung disease in cystic fibrosis (CF). While practical, they cannot locate the exact origin of functional impairment. Contemporary magnetic resonance imaging (MRI) techniques provide information on the location of disease but the need for contrast agents constrains their repeated application. We examined the correlation between functional MRI, performed without administration of contrast agent, and lung clearance index (LCI) from nitrogen multiple-breath washout (N2-MBW)...
December 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29216201/functional-lung-mri-for-regional-monitoring-of-patients-with-cystic-fibrosis
#8
Till F Kaireit, Sajoscha A Sorrentino, Julius Renne, Christian Schoenfeld, Andreas Voskrebenzev, Marcel Gutberlet, Angela Schulz, Peter M Jakob, Gesine Hansen, Frank Wacker, Tobias Welte, Burkhard Tümmler, Jens Vogel-Claussen
PURPOSE: To test quantitative functional lung MRI techniques in young adults with cystic fibrosis (CF) compared to healthy volunteers and to monitor immediate treatment effects of a single inhalation of hypertonic saline in comparison to clinical routine pulmonary function tests. MATERIALS AND METHODS: Sixteen clinically stable CF patients and 12 healthy volunteers prospectively underwent two functional lung MRI scans and pulmonary function tests before and 2h after a single treatment of inhaled hypertonic saline or without any treatment...
2017: PloS One
https://www.readbyqxmd.com/read/29208759/the-role-of-multidetector-computed-tomography-and-the-forced-oscillation-technique-in-assessing-lung-damage-in-adults-with-cystic-fibrosis
#9
Letícia S Lacerda, Agnaldo J Lopes, Alysson R S Carvalho, Alan R M Guimarães, Mônica C Firmida, Marcos C S Castro, Roberto Mogami, Pedro L Melo
BACKGROUND: With increased survival rates and the consequent emergence of an adult population with cystic fibrosis (CF), developing novel tools for periodic evaluations of these patients has become a new challenge. Thus, we sought to determine the contribution of lung-volume quantification using multidetector computed tomography (CT) in adults with CF and to investigate the association between structural changes and functional abnormalities. METHODS: This was a cross-sectional study in which 21 adults with CF and 22 control subjects underwent lung-volume quantification using multidetector CT...
December 5, 2017: Respiratory Care
https://www.readbyqxmd.com/read/29182491/improved-lung-function-after-sinus-surgery-in-cystic-fibrosis-patients-with-moderate-obstruction
#10
Sabrina Khalfoun, Dmitry Tumin, Maroun Ghossein, Meredith Lind, Don Hayes, And Stephen Kirkby
Objectives Cystic fibrosis (CF) is characterized by infection and inflammation of the sinorespiratory tract. Functional endoscopic sinus surgery (FESS) is an option for patients with severe sinusitis. We sought to evaluate pulmonary function testing after FESS in pediatric and adult patients with CF. Study Design Retrospective chart review using data from all patients with CF who underwent FESS from January 2009 to July 2014. Setting Patients were from a single institution. Subjects and Methods Data were extracted for 181 patients and 320 surgeries...
November 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29140739/cftr-genotype-and-maximal-exercise-capacity-in-cystic-fibrosis-a-cross-sectional-study
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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