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lung function tests in cystic fibrosis

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https://www.readbyqxmd.com/read/29742145/effect-of-backpack-carrying-on-forced-vital-capacity-in-cystic-fibrosis-a-randomized-crossover-controlled-trial
#1
Yann Combret, Clement Medrinal, Guillaume Prieur, Aurora Robledo Quesada, Pascal Le Roux, Grégory Reychler
BACKGROUND: Backpack carrying impacts lung function in healthy children but the effect in children with cystic fibrosis (CF) is unknown. METHODS: Three backpack positions were tested: no backpack (NB), a 12.5% body-weight backpack carried bilaterally (BB) or unilaterally (UB), at rest and during a 10 minute walk. Primary outcome was forced vital capacity (FVC). Secondary outcomes included comparison of cardio-respiratory variables within and between groups. RESULTS: Nine children with CF (13...
2018: PloS One
https://www.readbyqxmd.com/read/29741792/frontline-science-pathological-conditioning-of-human-neutrophils-recruited-to-the-airway-milieu-in-cystic-fibrosis
#2
Osric A Forrest, Sarah A Ingersoll, Marcela K Preininger, Julie Laval, Dominique H Limoli, Milton R Brown, Frances E Lee, Brahmchetna Bedi, Ruxana T Sadikot, Joanna B Goldberg, Vin Tangpricha, Amit Gaggar, Rabindra Tirouvanziam
RATIONALE: Recruitment of neutrophils to the airways, and their pathological conditioning therein, drive tissue damage and coincide with the loss of lung function in patients with cystic fibrosis (CF). So far, these key processes have not been adequately recapitulated in models, hampering drug development. Here, we hypothesized that the migration of naïve blood neutrophils into CF airway fluid in vitro would induce similar functional adaptation to that observed in vivo, and provide a model to identify new therapies...
May 9, 2018: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29725972/congruence-between-pulmonary-function-and-computed-tomography-imaging-assessment-of-cystic-fibrosis-severity
#3
Anna Rybacka, Joanna Goździk-Spychalska, Adam Rybacki, Tomasz Piorunek, Halina Batura-Gabryel, Katarzyna Karmelita-Katulska
In cystic fibrosis, pulmonary function tests (PFTs) and computed tomography are used to assess lung function and structure, respectively. Although both techniques of assessment are congruent there are lingering doubts about which PFTs variables show the best congruence with computed tomography scoring. In this study we addressed the issue by reinvestigating the association between PFTs variables and the score of changes seen in computed tomography scans in patients with cystic fibrosis with and without pulmonary exacerbation...
May 4, 2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29720914/vancomycin-versus-vancomycin-plus-rifampin-for-the-treatment-of-acute-pulmonary-exacerbations-of-cystic-fibrosis
#4
Nicholas M Fusco, Calvin J Meaney, Corey Wells, Carla A Frederick, William A Prescott
OBJECTIVES: This study aimed to compare the change in pulmonary function in children and adolescents with cystic fibrosis (CF) who were infected with methicillin-resistant Staphylococcus aureus (MRSA) treated with either vancomycin (VAN) alone or vancomycin plus rifampin (VAN-RIF). METHODS: Included patients were ages 6 to 20 years; hospitalized for an acute pulmonary exacerbation (APE) of CF from May 1, 2012, to April 30, 2014; had a respiratory tract culture positive for MRSA within 1 month of index hospital admission; received at least 48 consecutive hours of VAN or VAN-RIF; and had admission and discharge pulmonary function tests...
March 2018: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29702751/airway-persistence-by-the-emerging-multi-azole-resistant-rasamsonia-argillacea-complex-in-cystic-fibrosis
#5
Alireza Abdolrasouli, Amelia C Bercusson, Johanna L Rhodes, Ferry Hagen, Jochem B Buil, Alison Y Y Tang, Leonard L de Boer, Anand Shah, Andrew J Milburn, J Stuart Elborn, Andrew L Jones, Jacques F Meis, Matthew C Fisher, Silke Schelenz, Nicholas J Simmonds, Darius Armstrong-James
Infections caused by Rasamsonia argillacea complex have been reported in various clinical settings. Cystic fibrosis (CF) is one of the main underlying conditions. An observational cohort study of CF patients with Rasamsonia in respiratory samples was conducted. Eight isolates from six patients were identified as R. argillacea complex and tested for antifungal susceptibility. All isolates had high MICs to voriconazole and posaconazole and low MECs to echinocandins. Four patients experienced lung function decline in the year preceding first Rasamsonia isolation...
April 27, 2018: Mycoses
https://www.readbyqxmd.com/read/29605218/six-minute-walk-test-as-a-determinant-of-the-functional-capacity-of-children-and-adolescents-with-cystic-fibrosis-a-systematic-review
#6
REVIEW
Cibelle Andrade Lima, Armèle Dornelas de Andrade, Shirley Lima Campos, Daniella Cunha Brandão, Ianny Pereira Mourato, Murilo Carlos Amorim de Britto
Cystic Fibrosis (CF) is a multisystem disorder. The involvement of the respiratory system is frequent and culminates in dyspnea and exercise intolerance. Functional capacity is an important diagnostic tool, because it reflects the cardiorespiratory status, quality of life and prognosis. This systematic review aims to assess the reproducibility and validity of the six minute walk test (6MWT) to reflect the functional capacity of children and adolescents with cystic fibrosis, and also the correlation between 6MWT and lung function...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29605210/a-review-of-the-etiology-and-clinical-presentation-of-non-cystic-fibrosis-bronchiectasis-a-tertiary-care-experience
#7
Ozlem Satırer, Ayse Mete Yesil, Nagehan Emiralioglu, Gökcen Dilsa Tugcu, Ebru Yalcın, Deniz Dogru, Nural Kiper, Ugur Ozcelik
INTRODUCTION: Non-cystic fibrosis(CF) bronchiectasis has been recognized in children for the past 200 years. Early childhood pneumonia and underlying conditions such as immunodeficiency, primary ciliary dyskinesia(PCD), and congenital lung pathology should be considered in the etiology. The aim of our study was to describe the clinical characteristics, laboratory, and radiological findings of a large population of patients with non-CF bronchiectasis at a tertiary center. METHODS: We analyzed the clinical findings of 187 patients diagnosed with non-CF bronchiectasis over a period of 10 years (January 2005-December 2015) at the Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29506377/-italian-cystic-fibrosis-registry-report-2011-2014
#8
Barbara Giordani, Annalisa Amato, Fabio Majo, Gianluca Ferrari, Serena Quattrucci, Laura Minicucci, Rita Padoan, Giovanna Floridia, Gianna Puppo Fornaro, Domenica Taruscio, Marco Salvatore
INTRODUCTION: The Italian Cystic Fibrosis Registry (ICFR) is based on a new agreement about the data flow towards the Registry signed on October, 4th 2016 by the Centre for Rare Diseases of the Italian National Institute of Health (NIH), the clinicians of the Italian National Referral and Support Centres for Cystic Fibrosis, the Paediatric Hospital "Bambino Gesù" (Rome), the Italian Cystic Fibrosis Society, and the Italian League for Cystic Fibrosis. OBJECTIVES: The aim of the present Report is to improve the knowledge on cystic fibrosis (CF) through the epidemiological description of Italian patients...
January 2018: Epidemiologia e Prevenzione
https://www.readbyqxmd.com/read/29501453/staphylococcus-aureus-in-the-airways-of-cystic-fibrosis-patients-a-retrospective-long-term-study
#9
Mathias Schwerdt, Claudia Neumann, Bianca Schwartbeck, Stefanie Kampmeier, Susann Herzog, Dennis Görlich, Angelika Dübbers, Jörg Große-Onnebrink, Christina Kessler, Peter Küster, Holger Schültingkemper, Janina Treffon, Georg Peters, Barbara C Kahl
BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disease associated with chronic airway infections by Staphylococcus aureus as one of the earliest and most prevalent pathogens. We conducted a retrospective study to determine the S. aureus infection status of CF patients treated since 1994 at two certified CF-centres in Münster, Germany, to get insights into the dynamics of S. aureus airway infection and the clinical impact on lung function on a long-term perspective. MATERIALS AND METHODS: We used data from our microbiological database collected between 1994 and 2016 for patients treated at two centres in Münster, Germany, respectively, to determine the infection status for S...
February 24, 2018: International Journal of Medical Microbiology: IJMM
https://www.readbyqxmd.com/read/29424930/inhaled-mannitol-for-cystic-fibrosis
#10
REVIEW
Sarah J Nevitt, Judith Thornton, Clare S Murray, Tiffany Dwyer
BACKGROUND: Several agents are used to clear secretions from the airways of people with cystic fibrosis. Mannitol increases mucociliary clearance, but its exact mechanism of action is unknown. The dry powder formulation of mannitol may be more convenient and easier to use compared with established agents which require delivery via a nebuliser. Phase III trials of inhaled dry powder mannitol for the treatment of cystic fibrosis have been completed and it is now available in Australia and some countries in Europe...
February 9, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29418075/infant-multiple-breath-washout-using-a-new-commercially-available-device-ready-to-replace-the-previous-setup
#11
Anne-Christianne Kentgens, Marisa Guidi, Insa Korten, Lena Kohler, Severin Binggeli, Florian Singer, Philipp Latzin, Pinelopi Anagnostopoulou
INTRODUCTION: Multiple breath washout (MBW) is a sensitive test to measure lung volumes and ventilation inhomogeneity from infancy on. The commonly used setup for infant MBW, based on ultrasonic flowmeter, requires extensive signal processing, which may reduce robustness. A new setup may overcome some previous limitations but formal validation is lacking. AIM: We assessed the feasibility of infant MBW testing with the new setup and compared functional residual capacity (FRC) values of the old and the new setup in vivo and in vitro...
May 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29378680/continuous-glucose-monitoring-to-evaluate-glycaemic-abnormalities-in-cystic-fibrosis
#12
Mariana Zorrón Mei Hsia Pu, Aline Cristina Gonçalves, Walter José Minnicucci, André Moreno Morcillo, José Dirceu Ribeiro, Antonio Fernando Ribeiro
OBJECTIVE: This study aimed to determine the glycaemic profile of patients with cystic fibrosis using a continuous glucose monitoring system (CGMS), and to evaluate the associations of glycaemic abnormalities with sex, age, pubertal stage, CFTR gene mutations, nutritional status, lung function, oral glucose tolerance test, glycated haemoglobin concentrations, fasting insulin concentrations, C peptide concentrations and exocrine pancreatic function. STUDY DESIGN: This observational study evaluated CGMS data from 39 patients with cystic fibrosis who were treated at a referral centre...
January 29, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29356436/health-insurance-and-use-of-recommended-routine-care-in-adults-with-cystic-fibrosis
#13
Susan S Li, Don Hayes, Joseph D Tobias, Wayne J Morgan, Dmitry Tumin
BACKGROUND: Low socioeconomic status is correlated with worse outcomes in patients with cystic fibrosis (CF). Whether insurance status impacts adherence to care in this population is unknown. METHODS: Patients ≥18 years old in the CF Foundation Patient Registry (2005-2013) were grouped based on reported annual insurance as private, public (Medicaid, Medicare or state medical assistance program), others or no insurance. Random effects logistic regression evaluated association between change in insurance status and annual use of recommended routine care...
January 22, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29351449/personalized-medicine-in-cf-from-modulator-development-to-therapy-for-cystic-fibrosis-patients-with-rare-cftr-mutations
#14
Misak Harutyunyan, Yunjie Huang, Kyu-Shik Mun, Fanmuyi Yang, Kavisha Arora, Anjaparavanda P Naren
Cystic fibrosis (CF) is the most common life-shortening genetic disease affecting ~1 in 3,500 of the Caucasian population. CF is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. To date, more than 2,000 CFTR mutations have been identified, which produce a wide range of phenotypes. The CFTR protein, a chloride channel, is normally expressed on epithelial cells lining the lung, gut, and exocrine glands. Mutations in CFTR have led to pleiotropic effects in CF patients and have resulted in early morbidity and mortality...
April 1, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29349537/timing-of-spirometry-may-impact-hospital-length-of-stay-for-cystic-fibrosis-pulmonary-exacerbation
#15
Katelyn Krivchenia, Dmitry Tumin, Christopher J Nemastil, Joseph D Tobias, Don Hayes
PURPOSE: The optimal timing of spirometry during hospitalization for acute pulmonary exacerbation (PEx) in patients with cystic fibrosis (CF) is unclear. We retrospectively evaluated whether measuring spirometry earlier during hospitalization was associated with a shorter length of stay (LOS). METHODS: In this retrospective study, we analyzed data from the electronic medical record of CF patients 6 years of age and older admitted to a single center for acute PEx requiring IV antibiotic therapy between 2009 and 2016...
April 2018: Lung
https://www.readbyqxmd.com/read/29330966/static-hyperinflation-is-associated-with-ventilatory-limitation-and-exercise-tolerance-in-adult-cystic-fibrosis
#16
Daniel Stevens
INTRODUCTION: Lung hyperinflation is a potential mechanism limiting exercise tolerance. However, available data on the impact of static hyperinflation on exercise performance in adult cystic fibrosis are lacking. Furthermore, the relative contribution of both static and dynamic hyperinflation to exercise performance is unknown. OBJECTIVES: The aim of this study was to determine the impact of static hyperinflation on exercise tolerance and lung dynamics in adult cystic fibrosis...
January 13, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29323929/variation-in-cilia-protein-genes-and-progression-of-lung-disease-in-cystic-fibrosis
#17
Elizabeth Blue, Tin L Louie, Jessica X Chong, Scott J Hebbring, Kathleen C Barnes, Nicholas M Rafaels, Michael R Knowles, Ronald L Gibson, Michael J Bamshad, Mary J Emond
RATIONALE: Cystic fibrosis, like primary ciliary dyskinesia, is an autosomal recessive disorder characterized by abnormal mucociliary clearance and obstructive lung disease. We hypothesized that genes underlying the development or function of cilia may modify lung disease severity in persons with cystic fibrosis. OBJECTIVES: To test this hypothesis, we compared variants in 93 candidate genes in both upper and lower tertiles of lung function in a large cohort of children and adults with cystic fibrosis with those of a population control dataset...
April 2018: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29221318/lung-densitometry-why-how-and-when
#18
REVIEW
Mario Mascalchi, Gianna Camiciottoli, Stefano Diciotti
Lung densitometry assesses with computed tomography (CT) the X-ray attenuation of the pulmonary tissue which reflects both the degree of inflation and the structural lung abnormalities implying decreased attenuation, as in emphysema and cystic diseases, or increased attenuation, as in fibrosis. Five reasons justify replacement with lung densitometry of semi-quantitative visual scales used to measure extent and severity of diffuse lung diseases: (I) improved reproducibility; (II) complete vs. discrete assessment of the lung tissue; (III) shorter computation times; (IV) better correlation with pathology quantification of pulmonary emphysema; (V) better or equal correlation with pulmonary function tests (PFT)...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29221293/distance-saturation-product-of-the-6-minute-walk-test-predicts-mortality-of-patients-with-non-cystic-fibrosis-bronchiectasis
#19
Meng-Heng Hsieh, Yueh-Fu Fang, Fu-Tsai Chung, Chung-Shu Lee, Yu-Chen Chang, Yuan-Zhang Liu, Cheng-Hsien Wu, Horng-Chyuan Lin
Background: Previous surveillance methods to monitor the prognoses of patients with bronchiectasis are too complex for use in daily practice. The 6-minute walk test (6MWT) is a simple exercise test to predict the prognosis of chronic obstructive airway disease and numerous chronic lung diseases, including idiopathic pulmonary fibrosis. No studies have investigated exercise-induced oxygen desaturation (EID) and distance-saturation product (DSP) of 6MWT to predict the prognoses of patients with bronchiectasis...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29217601/novel-magnetic-resonance-technique-for-functional-imaging-of-cystic-fibrosis-lung-disease
#20
Sylvia Nyilas, Grzegorz Bauman, Gregor Sommer, Enno Stranzinger, Orso Pusterla, Urs Frey, Insa Korten, Florian Singer, Carmen Casaulta, Oliver Bieri, Philipp Latzin
Lung function tests are commonly used to monitor lung disease in cystic fibrosis (CF). While practical, they cannot locate the exact origin of functional impairment. Contemporary magnetic resonance imaging (MRI) techniques provide information on the location of disease but the need for contrast agents constrains their repeated application. We examined the correlation between functional MRI, performed without administration of contrast agent, and lung clearance index (LCI) from nitrogen multiple-breath washout (N2 -MBW)...
December 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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