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lung function tests, cystic fibrosis, children

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https://www.readbyqxmd.com/read/29742145/effect-of-backpack-carrying-on-forced-vital-capacity-in-cystic-fibrosis-a-randomized-crossover-controlled-trial
#1
Yann Combret, Clement Medrinal, Guillaume Prieur, Aurora Robledo Quesada, Pascal Le Roux, Grégory Reychler
BACKGROUND: Backpack carrying impacts lung function in healthy children but the effect in children with cystic fibrosis (CF) is unknown. METHODS: Three backpack positions were tested: no backpack (NB), a 12.5% body-weight backpack carried bilaterally (BB) or unilaterally (UB), at rest and during a 10 minute walk. Primary outcome was forced vital capacity (FVC). Secondary outcomes included comparison of cardio-respiratory variables within and between groups. RESULTS: Nine children with CF (13...
2018: PloS One
https://www.readbyqxmd.com/read/29725972/congruence-between-pulmonary-function-and-computed-tomography-imaging-assessment-of-cystic-fibrosis-severity
#2
Anna Rybacka, Joanna Goździk-Spychalska, Adam Rybacki, Tomasz Piorunek, Halina Batura-Gabryel, Katarzyna Karmelita-Katulska
In cystic fibrosis, pulmonary function tests (PFTs) and computed tomography are used to assess lung function and structure, respectively. Although both techniques of assessment are congruent there are lingering doubts about which PFTs variables show the best congruence with computed tomography scoring. In this study we addressed the issue by reinvestigating the association between PFTs variables and the score of changes seen in computed tomography scans in patients with cystic fibrosis with and without pulmonary exacerbation...
May 4, 2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29720914/vancomycin-versus-vancomycin-plus-rifampin-for-the-treatment-of-acute-pulmonary-exacerbations-of-cystic-fibrosis
#3
Nicholas M Fusco, Calvin J Meaney, Corey Wells, Carla A Frederick, William A Prescott
OBJECTIVES: This study aimed to compare the change in pulmonary function in children and adolescents with cystic fibrosis (CF) who were infected with methicillin-resistant Staphylococcus aureus (MRSA) treated with either vancomycin (VAN) alone or vancomycin plus rifampin (VAN-RIF). METHODS: Included patients were ages 6 to 20 years; hospitalized for an acute pulmonary exacerbation (APE) of CF from May 1, 2012, to April 30, 2014; had a respiratory tract culture positive for MRSA within 1 month of index hospital admission; received at least 48 consecutive hours of VAN or VAN-RIF; and had admission and discharge pulmonary function tests...
March 2018: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29605218/six-minute-walk-test-as-a-determinant-of-the-functional-capacity-of-children-and-adolescents-with-cystic-fibrosis-a-systematic-review
#4
REVIEW
Cibelle Andrade Lima, Armèle Dornelas de Andrade, Shirley Lima Campos, Daniella Cunha Brandão, Ianny Pereira Mourato, Murilo Carlos Amorim de Britto
Cystic Fibrosis (CF) is a multisystem disorder. The involvement of the respiratory system is frequent and culminates in dyspnea and exercise intolerance. Functional capacity is an important diagnostic tool, because it reflects the cardiorespiratory status, quality of life and prognosis. This systematic review aims to assess the reproducibility and validity of the six minute walk test (6MWT) to reflect the functional capacity of children and adolescents with cystic fibrosis, and also the correlation between 6MWT and lung function...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29605210/a-review-of-the-etiology-and-clinical-presentation-of-non-cystic-fibrosis-bronchiectasis-a-tertiary-care-experience
#5
Ozlem Satırer, Ayse Mete Yesil, Nagehan Emiralioglu, Gökcen Dilsa Tugcu, Ebru Yalcın, Deniz Dogru, Nural Kiper, Ugur Ozcelik
INTRODUCTION: Non-cystic fibrosis(CF) bronchiectasis has been recognized in children for the past 200 years. Early childhood pneumonia and underlying conditions such as immunodeficiency, primary ciliary dyskinesia(PCD), and congenital lung pathology should be considered in the etiology. The aim of our study was to describe the clinical characteristics, laboratory, and radiological findings of a large population of patients with non-CF bronchiectasis at a tertiary center. METHODS: We analyzed the clinical findings of 187 patients diagnosed with non-CF bronchiectasis over a period of 10 years (January 2005-December 2015) at the Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29323929/variation-in-cilia-protein-genes-and-progression-of-lung-disease-in-cystic-fibrosis
#6
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 with those of a population control dataset...
April 2018: Annals of the American Thoracic Society
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/29140739/cftr-genotype-and-maximal-exercise-capacity-in-cystic-fibrosis-a-cross-sectional-study
#8
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 cystic fibrosis may be an important determinant of maximal exercise capacity in cystic fibrosis. Previous studies on the relationship between CFTR genotype and maximal exercise capacity are scarce and contradictory. OBJECTIVES: This study was designed to explore factors influencing maximal exercise capacity, expressed as peak oxygen uptake (V...
February 2018: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29136345/regional-lung-function-testing-in-children-using-electrical-impedance-tomography
#9
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...
March 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28981972/standard-versus-biofilm-antimicrobial-susceptibility-testing-to-guide-antibiotic-therapy-in-cystic-fibrosis
#10
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
#11
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/28871575/inert-gas-washout-background-and-application-in-various-lung-diseases
#12
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/28709466/effects-of-inhaled-hypertonic-7-saline-on-lung-function-test-in-preschool-children-with-cystic-fibrosis-results-of-a-crossover-randomized-clinical-trial
#13
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/28682006/air-trapping-in-early-cystic-fibrosis-lung-disease-does-ct-tell-the-full-story
#14
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/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#15
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
#16
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/28599957/pulmonary-surfactant-dysfunction-in-pediatric-cystic-fibrosis-mechanisms-and-reversal-with-a-lipid-sequestering-drug
#17
Lasantha Gunasekara, Mustafa Al-Saiedy, Francis Green, Ryan Pratt, Candice Bjornson, Ailian Yang, W Michael Schoel, Ian Mitchell, Mary Brindle, Mark Montgomery, Elizabeth Keys, John Dennis, Grishma Shrestha, Matthias Amrein
BACKGROUND: Airway surfactant is impaired in cystic fibrosis (CF) and associated with declines in pulmonary function. We hypothesized that surfactant dysfunction in CF is due to an excess of cholesterol with an interaction with oxidation. METHODS: Surfactant was extracted from bronchial lavage fluid from children with CF and surface tension, and lipid content, inflammatory cells and microbial flora were determined. Dysfunctional surfactant samples were re-tested with a lipid-sequestering agent, methyl-β-cyclodextrin (MβCD)...
September 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28586522/is-sweat-chloride-predictive-of-severity-of-cystic-fibrosis-lung-disease-assessed-by-chest-computed-tomography
#18
Daan Caudri, David Zitter, Inez Bronsveld, Harm Tiddens
BACKGROUND: Cystic Fibrosis (CF) lung disease is characterized by a marked heterogeneity. Sweat chloride-level is a functional marker of the CF Transmembrane Regulator (CFTR) protein and could be an important predictor of later disease severity. METHODS: In this retrospective analysis children from the Rotterdam CF clinic with available sweat chloride level at diagnosis and at least one routine spirometry-controlled volumetric chest CT scan in follow-up were included...
September 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28508087/the-vitamin-d-for-enhancing-the-immune-system-in-cystic-fibrosis-disc-trial-rationale-and-design-of-a-multi-center-double-blind-placebo-controlled-trial-of-high-dose-bolus-administration-of-vitamin-d3-during-acute-pulmonary-exacerbation-of-cystic-fibrosis
#19
Vin Tangpricha, Ellen M Smith, Jose Binongo, Suzanne E Judd, Thomas R Ziegler, Seth Walker, Rabindra Tirouvanziam, Susu M Zughaier, Moon Jeong Lee, Supavit Chesdachai, Wendy A Hermes, James F Chmiel, Amit Gaggar, Ruth E Grossmann, Patricia M Joseph, Jessica A Alvarez
Vitamin D deficiency is highly prevalent in children and adults with cystic fibrosis (CF). Recent studies have found an association between vitamin D status and risk of pulmonary exacerbations in children and adults with CF. The ongoing Vitamin D for enhancing the Immune System in Cystic fibrosis (DISC) study is a multi-center, double-blind, randomized, placebo-controlled trial that will test the hypothesis of whether high dose vitamin D given as a single oral bolus of 250,000 IU to adults with CF during a pulmonary exacerbation followed by a maintenance dose of vitamin D will improve time to next pulmonary exacerbation and re-hospitalization, improve survival and lung function compared to placebo and reduce the rates of pulmonary exacerbation,...
June 2017: Contemporary Clinical Trials Communications
https://www.readbyqxmd.com/read/28417451/prophylactic-anti-staphylococcal-antibiotics-for-cystic-fibrosis
#20
REVIEW
Alan R Smyth, Margaret Rosenfeld
BACKGROUND: Staphylococcus aureus causes pulmonary infection in young children with cystic fibrosis. Prophylactic antibiotics are prescribed hoping to prevent such infection and lung damage. Antibiotics have adverse effects and long-term use might lead to infection with Pseudomonas aeruginosa. This is an update of a previously published review. OBJECTIVES: To assess continuous oral antibiotic prophylaxis to prevent the acquisition of Staphylococcus aureus versus no prophylaxis in people with cystic fibrosis, we tested these hypotheses...
April 18, 2017: Cochrane Database of Systematic Reviews
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