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lung function tests, cystic fibrosis, children

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
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
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
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
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
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
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
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
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
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
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
Jeong Eun Shin, Jae Won Shim, Deok Soo Kim, Hae Lim Jung, Moon Soo Park, Jung Yeon Shim
Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease due to bronchial colonization of Aspergillus fumigatus that occurs in susceptible patients with asthma or cystic fibrosis. A 10-year-old girl was referred to the Department of Pediatric Pulmonology for persistent consolidations on chest radiography. Pulmonary consolidations were observed in the right upper and left lower lobes and were not resolved with a 4-week prescription of broad-spectrum antibiotics. The patient had a history of atopic dermatitis and allergic rhinitis but no history of asthma...
May 2015: Korean Journal of Pediatrics
Laís Cristina Rizzo, Gilberto Bueno Fischer, Paulo José Cauduro Maróstica, Helena Teresinha Mocelin
OBJECTIVE: to compare the characteristics of cystic fibrosis patients treated in two reference centers in southern Brazil in order to observe trends in the treatment and clinical outcomes that may produce changes in clinical conduct. METHODS: cross-sectional, retrospective study with 83 patients diagnosed with cystic fibrosis, aged one month to eighteen years. The variables analyzed were obtained through review of medical records, including: demographic and clinical characteristics, socioeconomic status, pulmonary function test, bacterial colonization profile, medication and physiotherapy...
March 2015: Revista da Associação Médica Brasileira
Padmaja Subbarao, Carlos Milla, Paul Aurora, Jane C Davies, Stephanie D Davis, Graham L Hall, Sonya Heltshe, Philipp Latzin, Anders Lindblad, Jessica E Pittman, Paul D Robinson, Margaret Rosenfeld, Florian Singer, Tim D Starner, Felix Ratjen, Wayne Morgan
The lung clearance index (LCI) is a lung function parameter derived from the multiple-breath washout (MBW) test. Although first developed 60 years ago, the technique was not widely used for many years. Recent technological advances in equipment design have produced gains in popularity for this test among cystic fibrosis (CF) researchers and clinicians, particularly for testing preschool-aged children. LCI has been shown to be feasible and sensitive to early CF lung disease in patients of all ages from infancy to adulthood...
June 2015: Annals of the American Thoracic Society
Kathryn A Ramsey, André Schultz, Stephen M Stick
Biomarkers in cystic fibrosis are used i. for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis, and ii. to assess aspects of lung disease severity (e.g. inflammation, infection). Effective biomarkers can aid disease monitoring and contribute to the development of new therapies. The tests of cystic fibrosis transmembrane regulator function each have unique strengths and weaknesses, and biomarkers of inflammation, infection and tissue destruction have the potential to enhance the management of cystic fibrosis through the early detection of disease processes...
September 2015: Paediatric Respiratory Reviews
Robert H Cleveland, Gregory S Sawicki, Catherine Stamoulis
BACKGROUND: To assess the severity of lung disease in cystic fibrosis (CF), scoring systems based on chest radiographs (CXRs), CT and MRI have been used extensively, although primarily in research settings rather than for clinical purposes. It has recently been shown that those based on CXRs (primarily the Brasfield and Wisconsin systems) are as sensitive and valid as those based on CT. The reproducibility and correlation of both systems to pulmonary function tests (PFTs) were recently investigated and were found to be statistically identical...
October 2015: Pediatric Radiology
Urszula Borawska-Kowalczyk, Dorota Sands
AIM: 1. Evaluation of health-related quality of life (HRQOL) in adolescents with cystic fibrosis (CF). 2. Evaluation of HRQOL in children with CF from the parents' perspective. 3. Evaluation of the relationship between HRQOL and both medical and psychosocial factors. MATERIAL AND METHODS: Health-related quality of life was measured with the Cystic Fibrosis Questionnaire - Revised. Seventy patients with cystic fibrosis, aged 14-18 years completed the version for adolescents and adults (CFQ-R 14⁺ and 70 parents of children aged 6-13 years filled out the version for parents (CFQ-R 6-13)...
January 2015: Developmental Period Medicine
Vidhu Thaker, Alexandra L Haagensen, Ben Carter, Zbys Fedorowicz, Brian W Houston
BACKGROUND: Cystic fibrosis is an inherited condition causing disease most noticeably in the lungs, digestive tract and pancreas. People with cystic fibrosis often have malnutrition and growth delay. Adequate nutritional supplementation does not improve growth optimally and hence an anabolic agent, recombinant growth hormone, has been proposed as a potential intervention. OBJECTIVES: To evaluate the effectiveness and safety of recombinant human growth hormone therapy in improving lung function, quality of life and clinical status of children and young adults with cystic fibrosis...
2015: Cochrane Database of Systematic Reviews
Paloma Lopes Francisco Parazzi, Fernando Augusto de Lima Marson, Maria Angela Gonçalves de Oliveira Ribeiro, Celize Cruz Bresciani de Almeida, Luiz Cláudio Martins, Ilma Aparecida Paschoal, Adyleia Aparecida Dalbo Contrera Toro, Camila Isabel Santos Schivinski, Jose Dirceu Ribeiro
BACKGROUND: Exercise has been studied as a prognostic marker for patients with cystic fibrosis (CF), as well as a tool for improving their quality of life and analyzing lung disease. In this context, the aim of the present study was to evaluate and compare variables of lung functioning. Our data included: (i) volumetric capnography (VCAP) parameters: expiratory minute volume (VE), volume of exhaled carbon dioxide (VCO2), VE/VCO2, ratio of dead space to tidal volume (VD/VT), and end-tidal carbon dioxide (PetCO2); (ii) spirometry parameters: forced vital capacity (FVC), percent forced expiratory volume in the first second of the FVC (FEV1%), and FEV1/FVC%; and (iii) cardiorespiratory parameters: heart rate (HR), respiratory rate, oxygen saturation (SpO2), and Borg scale rating at rest and during exercise...
2015: BMC Pulmonary Medicine
Cédric Bernarde, Marlène Keravec, Jérôme Mounier, Stéphanie Gouriou, Gilles Rault, Claude Férec, Georges Barbier, Geneviève Héry-Arnaud
BACKGROUND: Airway microbiota composition has been clearly correlated with many pulmonary diseases, and notably with cystic fibrosis (CF), an autosomal genetic disorder caused by mutation in the CF transmembrane conductance regulator (CFTR). Recently, a new molecule, ivacaftor, has been shown to re-establish the functionality of the G551D-mutated CFTR, allowing significant improvement in lung function. OBJECTIVE AND METHODS: The purpose of this study was to follow the evolution of the airway microbiota in CF patients treated with ivacaftor, using quantitative PCR and pyrosequencing of 16S rRNA amplicons, in order to identify quantitative and qualitative changes in bacterial communities...
2015: PloS One
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