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lung function tests in cystic fibrosis

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https://www.readbyqxmd.com/read/28534947/research-and-progress-on-clc%C3%A2-2-review
#1
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...
May 18, 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
#2
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
#3
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
#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/28393935/how-are-the-ancient-cystic-fibrosis-patients-cystic-fibrosis-diagnosed-over-60-years-old
#5
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
https://www.readbyqxmd.com/read/28381428/doxycycline-improves-clinical-outcomes-during-cystic-fibrosis-exacerbations
#6
Xin Xu, Tarek Abdalla, Preston E Bratcher, Patricia L Jackson, Gina Sabbatini, J Michael Wells, Xiang-Yang Lou, Rebecca Quinn, J Edwin Blalock, J P Clancy, Amit Gaggar
Matrix metalloprotease-9 (MMP-9) plays a role in progression of cystic fibrosis, and doxycycline can reduce MMP-9 in vitro Here, we explore the effect of doxycycline during cystic fibrosis exacerbation treatment on MMP-9 related readouts and clinical end-points.This randomised, double-blind, placebo-controlled study enrolled hospitalised patients with cystic fibrosis undergoing exacerbation. In total, 20 participants were given doxycycline and 19 participants were given placebo over an 8-day period during hospitalisation...
April 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28325850/phenotypic-diversity-and-genotypic-flexibility-of-burkholderia-cenocepacia-during-long-term-chronic-infection-of-cystic-fibrosis-lungs
#7
Amy Huei-Yi Lee, Stephane Flibotte, Sunita Sinha, Adrianna Paiero, Rachel L Ehrlich, Sergey Balashov, Garth D Ehrlich, James E A Zlosnik, Joshua Chang Mell, Corey Nislow
Chronic bacterial infections of the lung are the leading cause of morbidity and mortality in cystic fibrosis patients. Tracking bacterial evolution during chronic infections can provide insights into how host selection pressures-including immune responses and therapeutic interventions-shape bacterial genomes. We carried out genomic and phenotypic analyses of 215 serially collected Burkholderia cenocepacia isolates from 16 cystic fibrosis patients, spanning a period of 2-20 yr and a broad range of epidemic lineages...
April 2017: Genome Research
https://www.readbyqxmd.com/read/28284526/association-between-glucose-intolerance-and-bacterial-colonisation-in-an-adult-population-with-cystic-fibrosis-emergence-of-stenotrophomonas-maltophilia
#8
C Lehoux Dubois, V Boudreau, F Tremblay, A Lavoie, Y Berthiaume, R Rabasa-Lhoret, A Coriati
BACKGROUND: Diabetes is common in cystic fibrosis (CF). Glucose can be detected in the airway when the blood glucose is elevated, which favours bacterial growth. We investigated the relationship between dysglycemia and lung pathogens in CF. METHODS: Cross-sectional and prospective analysis of CF patients (N=260) who underwent a 2h-oral glucose tolerance test. Clinical data was collected. RESULTS: Stenotrophomonas maltophilia (S. maltophilia) was the sole bacteria increased in dysglycemic (AGT: 20...
May 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28238229/suspecting-non-cystic-fibrosis-bronchiectasis-what-the-busy-primary-care-clinician-needs-to-know
#9
REVIEW
Diego J Maselli, Bravein Amalakuhan, Holly Keyt, Alejandro A Diaz
AIMS: Non-cystic fibrosis bronchiectasis (NCFB) is a chronic, progressive respiratory disorder characterised by irreversibly and abnormally dilated airways, persistent cough, excessive sputum production and recurrent pulmonary infections. In the last several decades, its prevalence has increased, making it likely to be encountered in the primary care setting. The aim was to review the clinical presentation and diagnosis of NCFB, with an emphasis on the role of computed tomography (CT)...
February 2017: International Journal of Clinical Practice
https://www.readbyqxmd.com/read/28223466/short-term-effect-of-autogenic-drainage-on-ventilation-inhomogeneity-in-adult-subjects-with-stable-non-cystic-fibrosis-bronchiectasis
#10
William Poncin, Grégory Reychler, Noémie Leeuwerck, Nathalie Bauwens, Anne-Sophie Aubriot, Candice Nader, Giuseppe Liistro, Sophie Gohy
BACKGROUND: Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from a multiple-breath washout test, is a promising tool for assessing airway function in patients with non-cystic fibrosis bronchiectasis. However, it is unknown whether ventilation inhomogeneity could improve after successful elimination of excessive secretions within bronchiectasis. The objective of this work was to assess the short-term effects of lung secretion clearance using the autogenic drainage technique on standard lung function tests and LCI in subjects with non-cystic fibrosis bronchiectasis...
February 21, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28218802/non-invasive-ventilation-for-cystic-fibrosis
#11
REVIEW
Fidelma Moran, Judy M Bradley, Amanda J Piper
BACKGROUND: Non-invasive ventilation may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis by providing ventilatory support and avoiding tracheal intubation. Using non-invasive ventilation, in the appropriate situation or individuals, can improve lung mechanics through increasing airflow and gas exchange and decreasing the work of breathing. Non-invasive ventilation thus acts as an external respiratory muscle. This is an update of a previously published review...
February 20, 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
#12
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/28187782/sputum-dna-sequencing-in-cystic-fibrosis-non-invasive-access-to-the-lung-microbiome-and-to-pathogen-details
#13
Rounak Feigelman, Christian R Kahlert, Florent Baty, Frank Rassouli, Rebekka L Kleiner, Philipp Kohler, Martin H Brutsche, Christian von Mering
BACKGROUND: Cystic fibrosis (CF) is a life-threatening genetic disorder, characterized by chronic microbial lung infections due to abnormally viscous mucus secretions within airways. The clinical management of CF typically involves regular respiratory-tract cultures in order to identify pathogens and to guide treatment. However, culture-based methods can miss atypical or slow-growing microbes. Furthermore, the isolated microbes are often not classified at the strain level due to limited taxonomic resolution...
February 10, 2017: Microbiome
https://www.readbyqxmd.com/read/28182336/f508del-genotype-in-endoscopic-sinus-surgery-do-differences-in-outcomes-exist-between-genotypic-subgroups
#14
Ashleigh A Halderman, Natalie West, James Benke, Christopher R Roxbury, Sandra Y Lin
BACKGROUND: The impact of endoscopic sinus surgery (ESS) on pulmonary function in cystic fibrosis (CF) patients with chronic rhinosinusitis remains unclear, as studies have demonstrated conflicting results. To date, no study has looked specifically at the impact of CF genotype on lung function after ESS. In this study, we reviewed changes in pulmonary function test (PFT) results after ESS in F508del homozygotes and heterozygotes. METHODS: The charts of 25 patients with CF without prior lung transplant, who underwent ESS performed by the same surgeon between the period of July 2005 to July 2015, were retrospectively reviewed...
May 2017: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/28126911/characterization-of-inpatient-cystic-fibrosis-pulmonary-exacerbations
#15
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/28120553/what-makes-a-good-pediatric-transplant-lung-insights-from-in-vivo-lung-morphometry-with-hyperpolarized-3-he-magnetic-resonance-imaging
#16
Emily F Fishman, James D Quirk, Stuart C Sweet, Jason C Woods, David S Gierada, Mark S Conradi, Marilyn J Siegel, Dmitriy A Yablonskiy
Obtaining information on transplanted lung microstructure is an important part of the current care for monitoring transplant recipients. However, until now this information was only available from invasive lung biopsy. The objective of this study was to evaluate the use of an innovative non-invasive technique, in vivo lung morphometry with hyperpolarized ³He MRI-to characterize lung microstructure in the pediatric lung transplant population. This technique yields quantitative measurements of acinar airways' (alveolar ducts and sacs) parameters, such as acinar airway radii and alveolar depth...
May 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28118936/etiology-of-bronchiectasis-in-a-cohort-of-2047-patients-an-analysis-of-the-spanish-historical-bronchiectasis-registry
#17
Casilda Olveira, Alicia Padilla, Miguel-Ángel Martínez-García, David de la Rosa, Rosa-María Girón, Montserrat Vendrell, Luis Máiz, Luis Borderías, Eva Polverino, Eva Martínez-Moragón, Olga Rajas, Francisco Casas, Rosa Cordovilla, Javier de Gracia
INTRODUCTION: Bronchiectasis is caused by many diseases. Establishing its etiology is important for clinical and prognostic reasons. The aim of this study was to evaluate the etiology of bronchiectasis in a large patient sample and its possible relationship with demographic, clinical or severity factors, and to analyze differences between idiopathic disease, post-infectious disease, and disease caused by other factors. METHODS: Multicenter, cross-sectional study of the SEPAR Spanish Historical Registry (RHEBQ-SEPAR)...
January 21, 2017: Archivos de Bronconeumología
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
#18
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/28025037/impact-of-azithromycin-on-the-clinical-and-antimicrobial-effectiveness-of-tobramycin-in-the-treatment-of-cystic-fibrosis
#19
Dave P Nichols, Carrie L Happoldt, Preston E Bratcher, Silvia M Caceres, James F Chmiel, Kenneth C Malcolm, Milene T Saavedra, Lisa Saiman, Jennifer L Taylor-Cousar, Jerry A Nick
BACKGROUND: Concomitant use of oral azithromycin and inhaled tobramycin occurs in approximately half of US cystic fibrosis (CF) patients. Recent data suggest that this combination may be antagonistic. METHODS: Test the hypothesis that azithromycin reduces the clinical benefits of tobramycin by analyses of clinical trial data, in vitro modeling of P. aeruginosa antibiotic killing, and regulation of the MexXY efflux pump. RESULTS: Ongoing administration of azithromycin associates with reduced ability of inhaled tobramycin, as compared with aztreonam, to improve lung function and quality of life in a completed clinical trial...
May 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27999822/glucose-200%C3%A2-mg-dl-during-continuous-glucose-monitoring-identifies-adult-patients-at-risk-for-development-of-cystic-fibrosis-related-diabetes
#20
J L Taylor-Cousar, J S Janssen, A Wilson, C G St Clair, K M Pickard, M C Jones, S J Brayshaw, C S Chacon, C M Barboa, M K Sontag, F J Accurso, D P Nichols, M T Saavedra, J A Nick
Rationale. Cystic fibrosis related diabetes (CFRD) is the most common comorbidity in patients with CF. In spite of increased screening, diagnosis, and treatment of CFRD, the mortality rate in patients with CFRD still far exceeds the mortality rate in those without CFRD. Guidelines suggest that screening for CFRD be performed annually using the 2-hour 75-gram oral glucose tolerance test (OGTT). Adherence to recommended screening has been poor, with only approximately one-quarter of adults with CF undergoing OGTT in 2014...
2016: Journal of Diabetes Research
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