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lung function tests, cystic fibrosis, children

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https://www.readbyqxmd.com/read/28871575/inert-gas-washout-background-and-application-in-various-lung-diseases
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/28188011/effect-of-posture-on-lung-ventilation-distribution-and-associations-with-structure-in-children-with-cystic-fibrosis
#10
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/28126911/characterization-of-inpatient-cystic-fibrosis-pulmonary-exacerbations
#11
Jonathan D Cogen, Assaf P Oron, Ronald L Gibson, Lucas R Hoffman, Matthew P Kronman, Thida Ong, Margaret Rosenfeld
BACKGROUND AND OBJECTIVES: Pulmonary exacerbations lead to significant morbidity and mortality in patients with cystic fibrosis (CF). National consensus guidelines exist, but few studies report current practice in the treatment and monitoring of pulmonary exacerbations. The goal of this study was to characterize consistency and variability in the inpatient management of CF-related pulmonary exacerbations. We focused on the use of guideline-recommended maintenance therapies, antibiotic selection and treatment regimens, use of systemic corticosteroids, and frequency of lung function testing...
February 2017: Pediatrics
https://www.readbyqxmd.com/read/28087053/six-minute-walk-test-results-predict-risk-of-hospitalization-for-youths-with-cystic-fibrosis-a-5-year-follow-up-study
#12
Márcio V F Donadio, João P Heinzmann-Filho, Fernanda M Vendrusculo, Patrícia X H Frasson, Paulo J C Marostica
OBJECTIVE: To evaluate the association of 6-minute walk test (6MWT) and other variables (anthropometry, chronic Pseudomonas aeroginosa colonization, pulmonary function, and respiratory muscle strength) with the risk of hospitalization for pulmonary exacerbation in children and adolescents with cystic fibrosis (CF). STUDY DESIGN: Cohort study that included patients with CF aged 6-18 years. All participants underwent spirometry, manovacuometry, and 6MWT during the 5-year follow-up...
March 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/27943680/progression-of-lung-disease-in-preschool-patients-with-cystic-fibrosis
#13
MULTICENTER STUDY
Sanja Stanojevic, Stephanie D Davis, George Retsch-Bogart, Hailey Webster, Miriam Davis, Robin C Johnson, Renee Jensen, Maria Ester Pizarro, Mica Kane, Charles C Clem, Leah Schornick, Padmaja Subbarao, Felix A Ratjen
RATIONALE: Implementation of intervention strategies to prevent lung damage in early cystic fibrosis (CF) requires objective outcome measures that capture and track lung disease. OBJECTIVES: To define the utility of the Lung Clearance Index (LCI), measured by multiple breath washout, as a means to track disease progression in preschool children with CF. METHODS: Children with CF between the ages of 2.5 and 6 years with a confirmed diagnosis of CF and age-matched healthy control subjects were enrolled at three North American CF centers...
May 1, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27873431/hypoglycemia-is-common-in-children-with-cystic-fibrosis-and-seen-predominantly-in-females
#14
Belma Haliloglu, Yasemin Gokdemir, Zeynep Atay, Saygin Abali, Tulay Guran, Fazilet Karakoc, Refika Ersu, Bulent Karadag, Serap Turan, Abdullah Bereket
OBJECTIVE: To determine the prevalence of hypoglycemia in children and adolescents with cystic fibrosis (CF) in 2-hour oral glucose tolerance test (OGTT) and continuous glucose monitoring (CGM) under free-living conditions. RESEARCH DESIGN AND METHODS: Height, weight, body mass index (BMI), hemoglobin A1c (HbA1c), and Forced expiratory volume (FEV1%) were measured in children with CF (aged 5-18 years). Following OGTT, CGM was installed for 3 days. The total hypoglycemic and hyperglycemic time (%) during 3 days was measured...
November 22, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27718754/special-considerations-for-the-treatment-of-pulmonary-exacerbations-in-children-with-cystic-fibrosis
#15
Valerie Waters, Sanja Stanojevic, Felix Ratjen
Introduction Cystic fibrosis (CF) is a disease characterized by recurrent flares of respiratory symptoms, known as pulmonary exacerbations (PExs), which have a cumulative, detrimental effect on lung function decline and overall mortality. Although much research has been done on the effects of PExs in adults with CF, considerably less is known about these events in young children with CF. Areas covered This review describes the typical presentation of PExs in children and their impact on long-term clinical outcomes...
October 8, 2016: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/27461961/quantification-of-heterogeneity-in-lung-disease-with-image-based-pulmonary-function-testing
#16
Charlene S Stahr, Chaminda R Samarage, Martin Donnelley, Nigel Farrow, Kaye S Morgan, Graeme Zosky, Richard C Boucher, Karen K W Siu, Marcus A Mall, David W Parsons, Stephen Dubsky, Andreas Fouras
Computed tomography (CT) and spirometry are the mainstays of clinical pulmonary assessment. Spirometry is effort dependent and only provides a single global measure that is insensitive for regional disease, and as such, poor for capturing the early onset of lung disease, especially patchy disease such as cystic fibrosis lung disease. CT sensitively measures change in structure associated with advanced lung disease. However, obstructions in the peripheral airways and early onset of lung stiffening are often difficult to detect...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27447840/abnormal-glucose-tolerance-in-infants-and-young-children-with-cystic-fibrosis
#17
MULTICENTER STUDY
Yaling Yi, Andrew W Norris, Kai Wang, Xingshen Sun, Aliye Uc, Antoinette Moran, John F Engelhardt, Katie Larson Ode
RATIONALE: In cystic fibrosis, abnormal glucose tolerance is associated with decreased lung function and worsened outcomes. Translational evidence indicates that abnormal glucose tolerance may begin in early life. OBJECTIVES: To determine whether very young children with cystic fibrosis have increased abnormal glucose tolerance prevalence compared with control subjects. The secondary objective was to compare area under the curve for glucose and insulin in children with cystic fibrosis with control subjects...
October 15, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27104277/evaluation-of-children-with-cystic-fibrosis-by-impulse-oscillometry-when-stable-and-at-exacerbation
#18
Ayfer Sakarya, Zeynep S Uyan, Canan Baydemir, Yonca Anık, Ela Erdem, Yasemin Gokdemir, Bulent Karadag, Fazilet Karakoc, Refika Ersu
BACKGROUND: Pulmonary function tests are important in the diagnosis and follow-up of airway disease in cystic fibrosis (CF). Conventional spirometry for which repeated forced expiration maneuver are needed is considered as the main method. Impulse oscillometry (IOS) is a non-invasive method, which needs minimal cooperation. We performed a prospective cross-sectional study to determine the pulmonary function in CF children with IOS, and evaluate the IOS measurements during acute exacerbation...
November 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/26861259/children-with-recurrent-pneumonia-and-non-cystic-fibrosis-bronchiectasis
#19
Maria Francesca Patria, Benedetta Longhi, Mara Lelii, Claudia Tagliabue, Marinella Lavelli, Carlotta Galeone, Nicola Principi, Susanna Esposito
BACKGROUND: Recurrent pneumonia (RP) is one of the most frequent causes of pediatric non-cystic fibrosis (CF) bronchiectasis (BE) and a consequent accelerated decline in lung function. The aim of this study was to analyse the clinical records of children with RP in attempt to identify factors that may lead to an early suspicion of non-CF BE. METHODS: We recorded the demographic and clinical data, and lung function test results of children without CF attending our outpatient RP clinic between January 2009 to December 2013 who had undergone chest high-resolution computed tomography ≥ 8 weeks after an acute pneumonia episode and ≤ 6 months before enrolment...
February 9, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/26808981/efficacy-and-long-term-outcomes-of-palivizumab-prophylaxis-to-prevent-respiratory-syncytial-virus-infection-in-infants-with-cystic-fibrosis-in-northern-ireland
#20
H E Groves, L Jenkins, M Macfarlane, A Reid, F Lynn, M D Shields
BACKGROUND: RSV causes considerable morbidity and mortality in children. In cystic fibrosis (CF) viral infections are associated with worsening respiratory symptoms and bacterial colonization. Palivizumab is effective in reducing RSV hospitalization in high risk patient groups. Evidence regarding its effectiveness and safety in CF is inconclusive. CF screening in N. Ireland enabled timely palivizumab prophylaxis, becoming routine in 2002. OBJECTIVES: To determine the effect of palivizumab on RSV-related hospitalization and compare lung function and bacterial colonization at age 6 years for those born pre- and post-introduction of palivizumab prophylaxis...
April 2016: Pediatric Pulmonology
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