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lung function tests in cystic fibrosis

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https://www.readbyqxmd.com/read/28737262/overnight-delivery-of-hypertonic-saline-by-nasal-cannula-aerosol-for-cystic-fibrosis
#1
Timothy E Corcoran, Joseph E Godovchik, Karl H Donn, David R Busick, Jennifer Goralski, Landon W Locke, Matthew R Markovetz, Michael M Myerburg, Ashok Muthukrishnan, Lawrence Weber, Ryan T Lacy, Joseph M Pilewski
AIM: Inhaled hypertonic saline increases mucociliary clearance, improves pulmonary function, and decreases exacerbations in cystic fibrosis (CF) but contributes to the already significant treatment burden of CF. Overnight delivery of inhaled medications via a specially designed nasal cannula-aerosol device (Trans-nasal Pulmonary Aerosol Delivery [tPAD]) is an alternative approach. Here, we test whether overnight inhalation of hypertonic saline via tPAD improves mucociliary clearance and assess the tolerability of the device...
July 24, 2017: Pediatric Pulmonology
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/28708814/chirp-evoked-otoacoustic-emissions-and-middle-ear-absorbance-for-monitoring-ototoxicity-in-cystic-fibrosis-patients
#3
Angela C Garinis, Douglas H Keefe, Lisa L Hunter, Denis F Fitzpatrick, Daniel B Putterman, Garnett P McMillan, Jeffrey A Gold, M Patrick Feeney
OBJECTIVES: The goal of this study was to investigate the use of transient-evoked otoacoustic emissions (TEOAEs) and middle ear absorbance measurements to monitor auditory function in patients with cystic fibrosis (CF) receiving ototoxic medications. TEOAEs were elicited with a chirp stimulus using an extended bandwidth (0.71 to 8 kHz) to measure cochlear function at higher frequencies than traditional TEOAEs. Absorbance over a wide bandwidth (0.25 to 8 kHz) provides information on middle ear function...
July 13, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28705217/monitoring-clinical-and-microbiological-evolution-of-a-cystic-fibrosis-patient-over-26%C3%A2-years-experience-of-a-brazilian-cf-centre
#4
Cassiana da Costa Ferreira Leite, Tania Wrobel Folescu, Mônica de Cássia Firmida, Renata Wrobel Folescu Cohen, Robson Souza Leão, Flávia Alvim Dutra de Freitas, Rodolpho Mattos Albano, Claudia Henrique da Costa, Elizabeth Andrade Marques
BACKGROUND: Burkholderia cepacia complex is a group of opportunistic pathogens in cystic fibrosis (CF) patients believed to be associated with poor prognosis and patient-to-patient transmissibility. Little is known about clinical outcomes after B. vietnamiensis chronic colonization/infection. CASE PRESENTATION: A 33 yo male patient had diagnosis of CF by 7 yo, after recurrent pneumonia during infancy and lobectomy (left upper lobe) at 6 yo. Burkholderia cepacia complex (Bcc) was first isolated by 13 yo, and the patient fulfilled the criteria for chronic colonization by 15 yo...
July 14, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28695805/clinical-analysis-of-77-patients-with-allergic-bronchopulmonary-aspergillosis-in-peking-union-medical-college-hospital
#5
Mingqiang Zhang, Jinming Gao
Objective To summarize the clinical features of allergic bronchopulmonary aspergillosis(ABPA)to facilitate its early diagnosis and treatment. Methods We retrospectively analyzed the clinical data of 77 patients who had been admitted to Peking Union Medical College Hospital from January 1996 to July 2015 with ABPA. Results The average age of these 77 patients(38 men and 39 women)was(41.8±18.3)years. The co-morbidities included bronchial asthma(n=74,96%)and pulmonary cystic fibrosis(n=3,4%). The main symptoms and signs of ABPA were chronic cough(100%),sputum production(97%),wheeze(86%),sputum plugs(25%),blood-stained sputum(18%),hemoptysis(9%),chest pain(9%),fever(47%),weight loss(30%),and night sweat(12%)...
June 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28682006/air-trapping-in-early-cystic-fibrosis-lung-disease-does-ct-tell-the-full-story
#6
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...
July 6, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28664245/a-semiquantitative-mri-score-can-predict-loss-of-lung-function-in-patients-with-cystic-fibrosis-preliminary-results
#7
Juergen F Schaefer, Andreas Hector, Katharina Schmidt, Matthias Teufel, Sabrina Fleischer, Ute Graepler-Mainka, Joachim Riethmueller, Sergios Gatidis, Susanne Schaefer, Konstantin Nikolaou, Dominik Hartl, Ilias Tsiflikas
OBJECTIVES: To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S) as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease. METHODS: This observational study of a single-centre CF cohort included a group of 61 patients (mean age 12.9 ± 4.7 years) receiving morphological and functional pulmonary MRI, pulmonary function testing (PFT) and follow-up of 2 years. MRI was analysed by three raters using MR-CF-S. The inter-rater agreement, correlation of score categories with forced expiratory volume in 1 s (FEV1) at baseline, and the predictive value of clinical parameters, and score categories was assessed for the whole cohort and a subgroup of 40 patients with moderately impaired lung function...
June 29, 2017: European Radiology
https://www.readbyqxmd.com/read/28662114/novel-glycopolymer-sensitizes-burkholderia-cepacia-complex-isolates-from-cystic-fibrosis-patients-to-tobramycin-and-meropenem
#8
Vidya P Narayanaswamy, Scott Giatpaiboon, Shenda M Baker, William P Wiesmann, John J LiPuma, Stacy M Townsend
Burkholderia cepacia complex (Bcc) infection, associated with cystic fibrosis (CF) is intrinsically multidrug resistant to antibiotic treatment making eradication from the CF lung virtually impossible. Infection with Bcc leads to a rapid decline in lung function and is often a contraindication for lung transplant, significantly influencing morbidity and mortality associated with CF disease. Standard treatment frequently involves antibiotic combination therapy. However, no formal strategy has been adopted in clinical practice to guide successful eradication...
2017: PloS One
https://www.readbyqxmd.com/read/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#9
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
#10
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/28634241/psl-produced-by-mucoid-pseudomonas-aeruginosa-contributes-to-the-establishment-of-biofilms-and-immune-evasion
#11
Christopher J Jones, Daniel J Wozniak
Despite years of research and clinical advances, chronic pulmonary infections with mucoid Pseudomonas aeruginosa remain the primary concern for cystic fibrosis patients. Much of the research on these strains has focused on the contributions of the polysaccharide alginate; however, it is becoming evident that the neutral polysaccharide Psl also contributes to biofilm formation and the maintenance of chronic infections. Here, we demonstrate that Psl produced by mucoid strains has significant roles in biofilm structure and evasion of immune effectors...
June 20, 2017: MBio
https://www.readbyqxmd.com/read/28611235/improvement-in-exercise-duration-lung-function-and-well-being-in-g551d-cystic-fibrosis-patients-a-double-blind-placebo-controlled-randomised-cross-over-study-with-ivacaftor
#12
Dominic T Keating, Deirdre Edgeworth, Matthew Ellis, Brenda Button, Elyssa Williams, Denise Clark, Audrey Tierney, Stephane Heritier, Anastase T Kotsimbos, John Wilson
G551D , a mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, results in impaired chloride channel function in cystic fibrosis (CF) with multiple end-organ manifestations. The effect of ivacaftor, a CFTR-potentiator, on exercise capacity in cystic fibrosis (CF) is unknown. Twenty G551D-CF patients were recruited to a single-centre, double-blind, placebo-controlled, 28-day crossover study of ivacaftor. Variables measured included percentage change from baseline (%Δ) of VO2max (maximal oxygen consumption, primary outcome) during cardiopulmonary exercise testing (CPET), relevant other CPET physiological variables, lung function, BMI, sweat chloride, and disease specific health related quality of life (QOL) measures (CFQ-R and Alfred Wellness (AWEscore))...
June 13, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28606620/efficacy-and-safety-of-lumacaftor-and-ivacaftor-in-patients-aged-6-11-years-with-cystic-fibrosis-homozygous-for-f508del-cftr-a-randomised-placebo-controlled-phase-3-trial
#13
Felix Ratjen, Christopher Hug, Gautham Marigowda, Simon Tian, Xiaohong Huang, Sanja Stanojevic, Carlos E Milla, Paul D Robinson, David Waltz, Jane C Davies
BACKGROUND: Lumacaftor and ivacaftor combination treatment showed efficacy in patients aged 12 years or older with cystic fibrosis homozygous for F508del-cystic fibrosis transmembrane conductance regulator (CFTR) in placebo-controlled studies and patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR in an open-label study. We report efficacy and safety of lumacaftor and ivacaftor in patients with cystic fibrosis aged 6-11 years homozygous for F508del-CFTR. METHODS: In this phase 3, randomised, double-blind, placebo-controlled, multicentre study, patients were enrolled at 54 hospitals and medical centres in nine countries (the USA, Australia, Belgium, Canada, Denmark, France, Germany, Sweden, and the UK)...
June 8, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28599957/pulmonary-surfactant-dysfunction-in-pediatric-cystic-fibrosis-mechanisms-and-reversal-with-a-lipid-sequestering-drug
#14
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
#15
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/28534947/research-and-progress-on-clc%C3%A2-2-review
#16
Hongwei Wang, Minghui Xu, Qingjie Kong, Peng Sun, Fengyun Yan, Wenying Tian, Xin Wang
Chloride channel 2 (ClC-2) is one of the nine mammalian members of the ClC family. The present review discusses the molecular properties of ClC‑2, including CLCN2, ClC‑2 promoter and the structural properties of ClC‑2 protein; physiological properties; functional properties, including the regulation of cell volume. The effects of ClC‑2 on the digestive, respiratory, circulatory, nervous and optical systems are also discussed, in addition to the mechanisms involved in the regulation of ClC‑2. The review then discusses the diseases associated with ClC‑2, including degeneration of the retina, Sjögren's syndrome, age‑related cataracts, degeneration of the testes, azoospermia, lung cancer, constipation, repair of impaired intestinal mucosa barrier, leukemia, cystic fibrosis, leukoencephalopathy, epilepsy and diabetes mellitus...
July 2017: Molecular Medicine Reports
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
#17
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/28476560/is-there-an-association-between-back-pain-and-stress-incontinence-in-adults-with-cystic-fibrosis-a-retrospective-cross-sectional-study
#18
Jane E Ashbrook, Carol Shacklady, Sue Johnson, Gillian Yeowell, Peter Charles Goodwin
BACKGROUND: Back pain and stress urinary incontinence (SUI) are common in adults with cystic fibrosis (CF). This study aimed to establish whether there is an association between back pain, lung function and stress urinary incontinence and its relative risk. METHOD: This was a cross-sectional, retrospective analysis of the Manchester Musculoskeletal Screening Tool (MMST) data. It includes pain, (Short Form McGill Pain Questionnaire (SF-MPQ and VAS)) and International Consultation on Incontinence Short Form (ICIQ-UI-SF) measures...
May 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28417451/prophylactic-anti-staphylococcal-antibiotics-for-cystic-fibrosis
#19
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/28393935/how-are-the-ancient-cystic-fibrosis-patients-cystic-fibrosis-diagnosed-over-60-years-old
#20
C Prados, M Lerín, J J Cabanillas, L Gómez-Carrera, R Álvarez-Sala
BACKGROUND AND AIMS: To specify the prevalence of patients diagnosed with CF at age of ≥60 year-old and to analyze their characteristics. PATIENTS AND METHODS: Observational study of CF patients which were diagnosed at age ≥60 year-old. The analyzed variables were: age, sex, nationality, lung function parameters, conditions present at diagnosis, microbiological characteristics and genetic findings. RESULTS: eight patients were included...
2017: Respiratory Medicine Case Reports
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