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lung function tests, cystic fibrosis, children

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https://www.readbyqxmd.com/read/28599957/pulmonary-surfactant-dysfunction-in-pediatric-cystic-fibrosis-mechanisms-and-reversal-with-a-lipid-sequestering-drug
#1
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)...
June 6, 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
#2
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...
June 6, 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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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...
April 22, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/26861259/children-with-recurrent-pneumonia-and-non-cystic-fibrosis-bronchiectasis
#14
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
#15
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
https://www.readbyqxmd.com/read/26782645/chronic-aspergillus-fumigatus-colonization-of-the-pediatric-cystic-fibrosis-airway-is-common-and-may-be-associated-with-a-more-rapid-decline-in-lung-function
#16
Rosalind V Saunders, Deborah E Modha, Alison Claydon, Erol A Gaillard
Filamentous fungi are commonly isolated from the respiratory tract of CF patients, but their clinical significance is uncertain and the reported incidence variable. We report on the degree of Aspergillus fumigatus airway colonization in a tertiary pediatric CF cohort, evaluate the sensitivity of routine clinical sampling at detecting A. fumigatus, and compare lung function of A. fumigatus-colonized and non-colonized children.We carried out an 8-year retrospective cohort analysis using local databases, examining 1024 respiratory microbiological specimens from 45 children...
July 1, 2016: Medical Mycology: Official Publication of the International Society for Human and Animal Mycology
https://www.readbyqxmd.com/read/26405283/impact-of-lung-disease-on-respiratory-impedance-in-young-children-with-cystic-fibrosis
#17
Kathryn A Ramsey, Sarath C Ranganathan, Catherine L Gangell, Lidija Turkovic, Judy Park, Billy Skoric, Stephen M Stick, Peter D Sly, Graham L Hall
This study aimed to evaluate the ability of the forced oscillation technique (FOT) to detect underlying lung disease in preschool children with cystic fibrosis (CF) diagnosed following newborn screening.184 children (aged 3-6 years) with CF underwent lung function testing on 422 occasions using the FOT to assess respiratory resistance and reactance at the time of their annual bronchoalveolar lavage collection and chest computed tomography scan. We examined associations between FOT outcomes and the presence and progression of respiratory inflammation, infection and structural lung disease...
December 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/26383784/changes-in-multiple-breath-washout-measures-after-raised-volume-rapid-thoracoabdominal-compression-maneuvers-in-infants
#18
Padmaja Subbarao, Zihang Lu, Krzysztof Kowalik, Meghan Brown, Susan Balkovec, Per Gustafsson, Wendy Lou, Felix Ratjen
Multiple breath inert gas washout (MBW) measurements in infants are performed supine and often obtained under sedation and thus are combined with other lung function tests such as raised volume rapid thoracoabdominal compression (RVRTC). In this study, we sought to determine the effects of RVRTC maneuvers on MBW measures. Compared with tests performed prior to RVRTC, MBW measured after RVRTC was associated with a small reduction in functional residual capacity and a more pronounced decrease in cumulative expired volume in both healthy children and children with obstructive lung disease (cystic fibrosis or recurrent wheeze) indicating a more efficient washout after the raised volume maneuvers...
February 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/26381521/progressive-ventilation-inhomogeneity-in-infants-with-cystic-fibrosis-after-pulmonary-infection
#19
Shannon J Simpson, Sarath Ranganathan, Judy Park, Lidija Turkovic, Roy M Robins-Browne, Billy Skoric, Kathryn A Ramsey, Tim Rosenow, Georgia L Banton, Luke Berry, Stephen M Stick, Graham L Hall
Measures of ventilation distribution are promising for monitoring early lung disease in cystic fibrosis (CF). This study describes the cross-sectional and longitudinal impacts of pulmonary inflammation and infection on ventilation homogeneity in infants with CF.Infants diagnosed with CF underwent multiple breath washout (MBW) testing and bronchoalveolar lavage at three time points during the first 2 years of life.Measures were obtained for 108 infants on 156 occasions. Infants with a significant pulmonary infection at the time of MBW showed increases in lung clearance index (LCI) of 0...
December 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/26285162/false-normal-lung-clearance-index-in-infants-with-cystic-fibrosis-due-to-software-algorithms
#20
Pinelopi Anagnostopoulou, Sophie Yammine, Anne Schmidt, Insa Korten, Elisabeth Kieninger, Ines Mack, Daniel Trachsel, Gaudenz Hafen, Alexander Moeller, Carmen Casaulta, Philipp Latzin
BACKGROUND: Lung clearance index (LCI), a marker of ventilation inhomogeneity, is elevated early in children with cystic fibrosis (CF). However, in infants with CF, LCI values are found to be normal, although structural lung abnormalities are often detectable. We hypothesized that this discrepancy is due to inadequate algorithms of the available software package. AIM: Our aim was to challenge the validity of these software algorithms. METHODS: We compared multiple breath washout (MBW) results of current software algorithms (automatic modus) to refined algorithms (manual modus) in 17 asymptomatic infants with CF, and 24 matched healthy term-born infants...
October 2015: Pediatric Pulmonology
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