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lung function tests in cystic fibrosis

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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
#1
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...
September 11, 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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
https://www.readbyqxmd.com/read/28709466/effects-of-inhaled-hypertonic-7-saline-on-lung-function-test-in-preschool-children-with-cystic-fibrosis-results-of-a-crossover-randomized-clinical-trial
#9
Raffaella Nenna, Fabio Midulla, Caterina Lambiase, Giovanna De Castro, Anna Maria Zicari, Luciana Indinnimeo, Giuseppe Cimino, Patrizia Troiani, Serena Quattrucci, Giancarlo Tancredi
BACKGROUND: This crossover, randomized, double-blind study (conducted over a 32-week period) was performed to determine, in clinically stable Cystic fibrosis (CF) preschool children: the effects of 7% inhaled hypertonic saline on spirometry and interrupter resistance technique (Rint), and the possible side effects. METHODS: Twelve CF children (6M, mean age ± SD: 5.7 ± 0.8 yrs) were enrolled and randomly assigned to receive hypertonic saline (HS-4 ml 7% sodium chloride), or normal saline (NS-0...
July 15, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28708814/chirp-evoked-otoacoustic-emissions-and-middle-ear-absorbance-for-monitoring-ototoxicity-in-cystic-fibrosis-patients
#10
Angela C Garinis, Douglas H Keefe, Lisa L Hunter, Denis F Fitzpatrick, Daniel B Putterman, Garnett P McMillan, Jeffrey A Gold, M Patrick Feeney
OBJECTIVES: The goal of this study was to investigate the use of transient-evoked otoacoustic emissions (TEOAEs) and middle ear absorbance measurements to monitor auditory function in patients with cystic fibrosis (CF) receiving ototoxic medications. TEOAEs were elicited with a chirp stimulus using an extended bandwidth (0.71 to 8 kHz) to measure cochlear function at higher frequencies than traditional TEOAEs. Absorbance over a wide bandwidth (0.25 to 8 kHz) provides information on middle ear function...
July 13, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28705217/monitoring-clinical-and-microbiological-evolution-of-a-cystic-fibrosis-patient-over-26%C3%A2-years-experience-of-a-brazilian-cf-centre
#11
Cassiana da Costa Ferreira Leite, Tania Wrobel Folescu, Mônica de Cássia Firmida, Renata Wrobel Folescu Cohen, Robson Souza Leão, Flávia Alvim Dutra de Freitas, Rodolpho Mattos Albano, Claudia Henrique da Costa, Elizabeth Andrade Marques
BACKGROUND: Burkholderia cepacia complex is a group of opportunistic pathogens in cystic fibrosis (CF) patients believed to be associated with poor prognosis and patient-to-patient transmissibility. Little is known about clinical outcomes after B. vietnamiensis chronic colonization/infection. CASE PRESENTATION: A 33 yo male patient had diagnosis of CF by 7 yo, after recurrent pneumonia during infancy and lobectomy (left upper lobe) at 6 yo. Burkholderia cepacia complex (Bcc) was first isolated by 13 yo, and the patient fulfilled the criteria for chronic colonization by 15 yo...
July 14, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28695805/clinical-analysis-of-77-patients-with-allergic-bronchopulmonary-aspergillosis-in-peking-union-medical-college-hospital
#12
Mingqiang Zhang, Jinming Gao
Objective To summarize the clinical features of allergic bronchopulmonary aspergillosis(ABPA)to facilitate its early diagnosis and treatment. Methods We retrospectively analyzed the clinical data of 77 patients who had been admitted to Peking Union Medical College Hospital from January 1996 to July 2015 with ABPA. Results The average age of these 77 patients(38 men and 39 women)was(41.8±18.3)years. The co-morbidities included bronchial asthma(n=74,96%)and pulmonary cystic fibrosis(n=3,4%). The main symptoms and signs of ABPA were chronic cough(100%),sputum production(97%),wheeze(86%),sputum plugs(25%),blood-stained sputum(18%),hemoptysis(9%),chest pain(9%),fever(47%),weight loss(30%),and night sweat(12%)...
June 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28682006/air-trapping-in-early-cystic-fibrosis-lung-disease-does-ct-tell-the-full-story
#13
Tim Rosenow, Kathryn Ramsey, Lidija Turkovic, Conor P Murray, L Clara Mok, Graham L Hall, Stephen M Stick
INTRODUCTION: Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping". It is presumed to be localized hyperinflation due to small airway obstruction. In order to test this assumption, we compared air trapping extent to lung volumes measured on CT in young children with CF. MATERIALS AND METHODS: Children aged below 7 years undergoing inspiratory/expiratory CT were recruited from the Australian Respiratory Early Surveillance Team for Cystic Fibrosis cohort...
September 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28664245/a-semiquantitative-mri-score-can-predict-loss-of-lung-function-in-patients-with-cystic-fibrosis-preliminary-results
#14
Juergen F Schaefer, Andreas Hector, Katharina Schmidt, Matthias Teufel, Sabrina Fleischer, Ute Graepler-Mainka, Joachim Riethmueller, Sergios Gatidis, Susanne Schaefer, Konstantin Nikolaou, Dominik Hartl, Ilias Tsiflikas
OBJECTIVES: To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S) as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease. METHODS: This observational study of a single-centre CF cohort included a group of 61 patients (mean age 12.9 ± 4.7 years) receiving morphological and functional pulmonary MRI, pulmonary function testing (PFT) and follow-up of 2 years. MRI was analysed by three raters using MR-CF-S. The inter-rater agreement, correlation of score categories with forced expiratory volume in 1 s (FEV1) at baseline, and the predictive value of clinical parameters, and score categories was assessed for the whole cohort and a subgroup of 40 patients with moderately impaired lung function...
June 29, 2017: European Radiology
https://www.readbyqxmd.com/read/28662114/novel-glycopolymer-sensitizes-burkholderia-cepacia-complex-isolates-from-cystic-fibrosis-patients-to-tobramycin-and-meropenem
#15
Vidya P Narayanaswamy, Scott Giatpaiboon, Shenda M Baker, William P Wiesmann, John J LiPuma, Stacy M Townsend
Burkholderia cepacia complex (Bcc) infection, associated with cystic fibrosis (CF) is intrinsically multidrug resistant to antibiotic treatment making eradication from the CF lung virtually impossible. Infection with Bcc leads to a rapid decline in lung function and is often a contraindication for lung transplant, significantly influencing morbidity and mortality associated with CF disease. Standard treatment frequently involves antibiotic combination therapy. However, no formal strategy has been adopted in clinical practice to guide successful eradication...
2017: PloS One
https://www.readbyqxmd.com/read/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#16
REVIEW
Virginia Mirra, Claudius Werner, Francesca Santamaria
Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28649308/association-between-spirometry-controlled-chest-ct-scores-using-computer-animated-biofeedback-and-clinical-markers-of-lung-disease-in-children-with-cystic-fibrosis
#17
Thomas Kongstad, Kent Green, Frederik Buchvald, Marianne Skov, Tania Pressler, Kim Gjerum Nielsen
Background: Computed tomography (CT) of the lungs is the gold standard for assessing the extent of structural changes in the lungs. Spirometry-controlled chest CT (SCCCT) has improved the usefulness of CT by standardising inspiratory and expiratory lung volumes during imaging. This was a single-centre cross-sectional study in children with cystic fibrosis (CF). Using SCCCT we wished to investigate the association between the quantity and extent of structural lung changes and pulmonary function outcomes, and prevalence of known CF lung pathogens...
2017: European Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28634241/psl-produced-by-mucoid-pseudomonas-aeruginosa-contributes-to-the-establishment-of-biofilms-and-immune-evasion
#18
Christopher J Jones, Daniel J Wozniak
Despite years of research and clinical advances, chronic pulmonary infections with mucoid Pseudomonas aeruginosa remain the primary concern for cystic fibrosis patients. Much of the research on these strains has focused on the contributions of the polysaccharide alginate; however, it is becoming evident that the neutral polysaccharide Psl also contributes to biofilm formation and the maintenance of chronic infections. Here, we demonstrate that Psl produced by mucoid strains has significant roles in biofilm structure and evasion of immune effectors...
June 20, 2017: MBio
https://www.readbyqxmd.com/read/28611235/improvement-in-exercise-duration-lung-function-and-well-being-in-g551d-cystic-fibrosis-patients-a-double-blind-placebo-controlled-randomized-cross-over-study-with-ivacaftor-treatment
#19
Deirdre Edgeworth, Dominic Keating, Matthew Ellis, Brenda Button, Elyssa Williams, Denise Clark, Audrey Tierney, Stephane Heritier, Tom Kotsimbos, John Wilson
G551D, a mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, results in impaired chloride channel function in cystic fibrosis (CF) with multiple end-organ manifestations. The effect of ivacaftor, a CFTR-potentiator, on exercise capacity in CF is unknown. Twenty G551D-CF patients were recruited to a single-centre, double-blind, placebo-controlled, 28-day crossover study of ivacaftor. Variables measured included percentage change from baseline (%Δ) of VO2max (maximal oxygen consumption, primary outcome) during cardiopulmonary exercise testing (CPET), relevant other CPET physiological variables, lung function, body mass index (BMI), sweat chloride and disease-specific health related quality of life (QOL) measures (CFQ-R and Alfred Wellness (AWEscore))...
August 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28606620/efficacy-and-safety-of-lumacaftor-and-ivacaftor-in-patients-aged-6-11-years-with-cystic-fibrosis-homozygous-for-f508del-cftr-a-randomised-placebo-controlled-phase-3-trial
#20
Felix Ratjen, Christopher Hug, Gautham Marigowda, Simon Tian, Xiaohong Huang, Sanja Stanojevic, Carlos E Milla, Paul D Robinson, David Waltz, Jane C Davies
BACKGROUND: Lumacaftor and ivacaftor combination treatment showed efficacy in patients aged 12 years or older with cystic fibrosis homozygous for F508del-cystic fibrosis transmembrane conductance regulator (CFTR) in placebo-controlled studies and patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR in an open-label study. We report efficacy and safety of lumacaftor and ivacaftor in patients with cystic fibrosis aged 6-11 years homozygous for F508del-CFTR. METHODS: In this phase 3, randomised, double-blind, placebo-controlled, multicentre study, patients were enrolled at 54 hospitals and medical centres in nine countries (the USA, Australia, Belgium, Canada, Denmark, France, Germany, Sweden, and the UK)...
July 2017: Lancet Respiratory Medicine
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