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lung function tests in cystic fibrosis

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https://www.readbyqxmd.com/read/27930727/airway-microbiota-in-bronchoalveolar-lavage-fluid-from-clinically-well-infants-with-cystic-fibrosis
#1
Theresa A Laguna, Brandie D Wagner, Cynthia B Williams, Mark J Stevens, Charles E Robertson, Cole W Welchlin, Catherine E Moen, Edith T Zemanick, Jonathan K Harris
BACKGROUND: Upper airway cultures guide the identification and treatment of lung pathogens in infants with cystic fibrosis (CF); however, this may not fully reflect the spectrum of bacteria present in the lower airway. Our objectives were to characterize the airway microbiota using bronchoalveolar lavage fluid (BALF) from asymptomatic CF infants during the first year of life and to investigate the relationship between BALF microbiota, standard culture and clinical characteristics. METHODS: BALF, nasopharyngeal (NP) culture and infant pulmonary function testing data were collected at 6 months and one year of age during periods of clinical stability from infants diagnosed with CF by newborn screening...
2016: PloS One
https://www.readbyqxmd.com/read/27925430/pulmonary-hypertension-as-estimated-by-doppler-echocardiography-in-adolescent-and-adult-patients-with-cystic-fibrosis-and-their-relationship-with-clinical-lung-function-and-sleep-findings
#2
Bruna Ziegler, Christiano Perin, Fernanda Cano Casarotto, Simone Chaves Fagondes, Sérgio Saldanha Menna-Barreto, Paulo de Tarso Roth Dalcin
BACKGROUND: In cystic fibrosis (CF) patients, end stage of pulmonary disease is characterized by pulmonary hypertension (PH), hypoxemia, decrease in exercise tolerance and sleep quality. OBJECTIVE: To evaluate the association between clinical, lung function, sleep quality and polysomnographic variables with PH in CF patients aged 16 years or older. METHODS: In a cross-sectional study, 51 clinically stable CF patients underwent a clinical evaluation, an overnight polysomnography and answered sleep questionnaires (Pittsburgh Sleep Quality Index - PSQI and Epworth sleepiness scale - ESS)...
December 7, 2016: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/27920537/factors-related-to-depression-and-anxiety-in-adults-with-bronchiectasis
#3
Elif Yelda Özgün Niksarlioglu, Gülcihan Özkan, Gülşah Günlüoğlu, Mehmet Atilla Uysal, Sule Gül, Lütfiye Kilic, Ayse Yeter, Güngör Çamsarı
INTRODUCTION AND BACKGROUND: Patients with chronic lung diseases frequently have depressive and anxiety symptoms, but there are very few studies looking at this in patients with bronchiectasis. AIM: This study aimed to investigate depression and anxiety and related factors among patients with non-cystic fibrosis bronchiectasis. PATIENTS AND METHODS: This was a prospective study of 133 patients with bronchiectasis. Patients with confirmed diagnosis of bronchiectasis with high-resolution computed tomography were enrolled in the study...
2016: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/27917292/deep-resequencing-of-cftr-in-762-f508del-homozygotes-reveals-clusters-of-non-coding-variants-associated-with-cystic-fibrosis-disease-traits
#4
Briana Vecchio-Pagán, Scott M Blackman, Melissa Lee, Melis Atalar, Matthew J Pellicore, Rhonda G Pace, Arianna L Franca, Karen S Raraigh, Neeraj Sharma, Michael R Knowles, Garry R Cutting
Extensive phenotypic variability is commonly observed in individuals with Mendelian disorders, even among those with identical genotypes in the disease-causing gene. To determine whether variants within and surrounding CFTR contribute to phenotypic variability in cystic fibrosis (CF), we performed deep sequencing of CFTR in 762 patients homozygous for the common CF-causing variant, F508del. In phase 1, ~200 kb encompassing CFTR and extending 10 kb 5' and 5 kb 3' of the gene was sequenced in 486 F508del homozygotes selected from the extremes of sweat chloride concentration...
2016: Human Genome Variation
https://www.readbyqxmd.com/read/27895643/azithromycin-attenuates-pseudomonas-induced-lung-inflammation-by-targeting-bacterial-proteins-secreted-in-the-cultured-medium
#5
Teresinha Leal, Gabriella Bergamini, François Huaux, Nadtha Panin, Sabrina Noel, Barbara Dhooghe, Jeremy B Haaf, Pierluigi Mauri, Sara Motta, Dario Di Silvestre, Paola Melotti, Claudio Sorio
BACKGROUND: Pseudomonas aeruginosa airway infections are a major cause of morbidity and mortality in patients with cystic fibrosis (CF). Azithromycin improves the related clinical outcomes, but its mechanisms of action remain poorly understood. We tested the hypothesis that azithromycin downregulates P. aeruginosa-induced pro-inflammatory responses by modifying release of bacterial proteins. METHODS: We monitored inflammatory markers in lungs of CF mutant mice and their littermate controls in response to conditioned media (CM) collected from the reference P...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27882640/physiological-markers-of-exercise-capacity-and-lung-disease-severity-in-cystic-fibrosis
#6
Laurie Smith, Charles C Reilly, Victoria MacBean, Caroline J Jolley, Caroline Elston, John Moxham, Gerrard F Rafferty
BACKGROUND AND OBJECTIVE: Peak aerobic capacity (VO2 peak) is an important outcome measure in cystic fibrosis (CF), but measurement is not widely available and can be influenced by patient motivation, pain and fatigue. Alternative markers of disease severity would be helpful. Neural respiratory drive, measured using parasternal intercostal muscle electromyography (EMGpara), reflects the load to capacity balance of the respiratory system and provides a composite measure of pulmonary function impairment in CF...
November 24, 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/27879399/bone-subtraction-radiography-in-adult-patients-with-cystic-fibrosis
#7
Verena C Obmann, Andreas Christe, Lukas Ebner, Zsolt Szucs-Farkas, Sebastian R Ott, Sai Yarram, Enno Stranzinger
BACKGROUND: Bone subtraction radiography allows reading pulmonary changes of chest radiographs more accurately without superimposition of bones. PURPOSE: To evaluate the value of bone subtraction chest radiography using dual energy (DE) bone subtracted lung images compared to conventional radiographs (CR) in adult patients with cystic fibrosis (CF). MATERIAL AND METHODS: Forty-nine DE radiographs of 24 patients (16 men) with CF (mean age, 32 years; age range, 18-71 years) were included...
November 22, 2016: Acta Radiologica
https://www.readbyqxmd.com/read/27873431/hypoglycemia-is-common-in-children-with-cystic-fibrosis-and-seen-predominantly-in-females
#8
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/27820955/antifungal-therapies-for-allergic-bronchopulmonary-aspergillosis-in-people-with-cystic-fibrosis
#9
REVIEW
Heather E Elphick, Kevin W Southern
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is an allergic reaction to colonisation of the lungs with the fungus Aspergillus fumigatus and affects around 10% of people with cystic fibrosis. ABPA is associated with an accelerated decline in lung function. High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, their many side effects are well-documented. A group of compounds, the azoles, have activity against Aspergillus fumigatus and have been proposed as an alternative treatment for ABPA...
November 8, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27796267/exercise-capacity-following-a-percutaneous-endoscopic-gastrostomy-in-a-young-female-with-cystic-fibrosis-a-case-report
#10
Owen W Tomlinson, Alan R Barker, Patrick J Oades, Craig A Williams
Cystic fibrosis (CF) is a genetic condition affecting the respiratory and gastrointestinal systems, with patients experiencing problems maintaining weight, especially during rapid growth periods such as puberty. The aim of this case report was to monitor the effect of gastrostomy insertion and implementation of overnight supplemental feeding upon clinical outcomes, including body mass index (BMI), lung function (FEV1), and exercise-related variables (maximal oxygen uptake [VO2max] and ventilatory efficiency [VE/VO2]) in an 11-year-old female with CF Combined incremental and supramaximal exercise testing to exhaustion was performed at four time points: 3 months prior to the procedure (T1), 2 days prior to (T2), 4 months (T3), and 1 year following the procedure (T4)...
August 2016: Physiological Reports
https://www.readbyqxmd.com/read/27737759/screening-for-cystic-fibrosis-related-diabetes-matching-pathophysiology-and-addressing-current-challenges
#11
Valérie Boudreau, Quitterie Reynaud, Catherine Lehoux Dubois, Adèle Coriati, Katherine Desjardins, Isabelle Durieu, Rémi Rabasa-Lhoret
Nearly 50% of adult patients with cystic fibrosis (CF) have diabetes. The occurrence of CF-related diabetes (CFRD) is preceded and is associated with deterioration of lung function and nutritional status. Microvascular complications can occur, but the main cause of death is respiratory failure rather than cardiovascular causes as in type 1 or type 2 diabetes. Because other methods such as glycated hemoglobin (A1C) levels are less sensitive in patients with CF, the recommended screening test is the oral glucose tolerance test (OGTT) with a 75 g glucose dose...
October 2016: Canadian Journal of Diabetes
https://www.readbyqxmd.com/read/27736037/ventilatory-limitation-and-dynamic-hyperinflation-during-exercise-testing-in-cystic-fibrosis
#12
Solon Karapanagiotis, Simone Gambazza, Anna Brivio, Francesco D'Abrosca, Carla Colombo
OBJECTIVE: To investigate the presence of dynamic hyperinflation after the Modified Shuttle Test (MST) and its relationship with lung function, exercise tolerance, and clinical symptoms in Cystic Fibrosis (CF). METHODS: Retrospective observational study. Subjects in clinically stable condition with a CF diagnosis based on a positive sweat test (chloride >60 mEq/L) and/or presence of two disease causing mutations, with available data on MST, spirometry, maximal voluntary ventilation, and inspiratory capacity manoeuvres were considered for the analysis...
October 13, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27730186/key-paediatric-messages-from-amsterdam
#13
Jonathan Grigg, Jürg Barben, Kajsa Bohlin, Mark L Everard, Graham Hall, Mariëlle Pijnenburg, Kostas N Priftis, Franca Rusconi, Fabio Midulla
The Paediatric Assembly of the European Respiratory Society (ERS) maintained its high profile at the 2015 ERS International Congress in Amsterdam. There were symposia on preschool wheeze, respiratory sounds and cystic fibrosis; an educational skills workshop on paediatric respiratory resuscitation; a hot topic session on risk factors and early origins of respiratory diseases; a meet the expert session on paediatric lung function test reference values; and the annual paediatric grand round. In this report the Chairs of the Paediatric Assembly's Groups highlight the key messages from the abstracts presented at the Congress...
April 2016: ERJ Open Research
https://www.readbyqxmd.com/read/27718754/special-considerations-for-the-treatment-of-pulmonary-exacerbations-in-children-with-cystic-fibrosis
#14
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/27715657/self-gated-non-contrast-enhanced-functional-lung-mr-imaging-for-quantitative-ventilation-assessment-in-patients-with-cystic-fibrosis
#15
Simon Veldhoen, Andreas M Weng, Janine Knapp, Andreas S Kunz, Daniel Stäb, Clemens Wirth, Florian Segerer, Helge Hebestreit, Uwe Malzahn, Herbert Köstler, Thorsten A Bley
Purpose To assess the clinical feasibility of self-gated non-contrast-enhanced functional lung (SENCEFUL) magnetic resonance (MR) imaging for quantitative ventilation (QV) imaging in patients with cystic fibrosis (CF). Materials and Methods Twenty patients with CF and 20 matched healthy volunteers underwent functional 1.5-T lung MR imaging with the SENCEFUL imaging approach, in which a two-dimensional fast low-angle shot sequence is used with quasi-random sampling. The lungs were manually segmented on the ventilation-weighted images to obtain QV measurements, which were compared between groups...
October 6, 2016: Radiology
https://www.readbyqxmd.com/read/27646604/the-mucus-barrier-to-inhaled-gene-therapy
#16
Gregg A Duncan, James Jung, Justin Hanes, Jung Soo Suk
Recent evidence suggests that the airway mucus gel layer may be impermeable to the viral and synthetic gene vectors used in past inhaled gene therapy clinical trials for diseases like cystic fibrosis. These findings support the logic that inhaled gene vectors that are incapable of penetrating the mucus barrier are unlikely to provide meaningful benefit to patients. In this review, we discuss the biochemical and biophysical features of mucus that contribute its barrier function, and how these barrier properties may be reinforced in patients with lung disease...
September 20, 2016: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/27592691/minimal-clinically-important-difference-for-change-in-6-minute-walk-test-distance-of-adults-with-pathology-a-systematic-review
#17
Richard W Bohannon, Rebecca Crouch
RATIONALE, AIMS, AND OBJECTIVES: The 6-minute walk test (6MWT) is widely used as a test of functional exercise capacity. Several studies have reported the minimal clinically important difference (MCID) for the 6MWT; however, the findings of the studies have not been examined in the context of one another. In this review, we aimed to summarize available information on the MCID for the 6MWT performed by patients with pathology. METHODS: Relevant literature was identified by searches of 3 electronic databases (PubMed, Scopus, and Cumulative Index of Nursing and Allied Health), examination of article reference lists, and consultation with an expert...
September 4, 2016: Journal of Evaluation in Clinical Practice
https://www.readbyqxmd.com/read/27576234/respiratory-conditions-update-cystic-fibrosis
#18
Lyle L Pritchard
Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction...
September 2016: FP Essentials
https://www.readbyqxmd.com/read/27556254/risk-stratification-model-to-detect-early-pulmonary-disease-in-infants-with-cystic-fibrosis-diagnosed-by-newborn-screening
#19
Lacrecia J Britton, Gabriela R Oates, Robert A Oster, Staci T Self, Robert B Troxler, Wynton C Hoover, Hector H Gutierrez, William T Harris
OBJECTIVE: The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes. METHODS: This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4-year period (2008-2012)...
November 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27531053/compensation-for-large-thorax-excursions-in-eit-imaging
#20
B Schullcke, S Krueger-Ziolek, B Gong, U Mueller-Lisse, K Moeller
Besides the application of EIT in the intensive care unit it has recently also been used in spontaneously breathing patients suffering from asthma bronchiole, cystic fibrosis (CF) or chronic obstructive pulmonary disease (COPD). In these cases large thorax excursions during deep inspiration, e.g. during lung function testing, lead to artifacts in the reconstructed images. In this paper we introduce a new approach to compensate for image artifacts resulting from excursion induced changes in boundary voltages...
September 2016: Physiological Measurement
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