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"Cystic fibrosis"

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https://www.readbyqxmd.com/read/28646623/pregnancy-outcome-in-women-with-cystic-fibrosis-related-diabetes
#1
Quitterie Reynaud, Stéphanie Poupon-Bourdy, Muriel Rabilloud, Lina Al Mufti, Christine Rousset Jablonski, Lydie Lemonnier, Raphaële Nove-Josserand, Sandrine Touzet, Isabelle Durieu
With increasing life expectancy more women with cystic fibrosis and diabetes mellitus become pregnant. We investigated how pre-gestational diabetes (cystic fibrosis related diabetes) influenced pregnancy outcome and the clinical status of these women MATERIAL AND METHODS: We analyzed all pregnancies reported to the French cystic fibrosis registry between 2001 and 2012, and compared forced expiratory volume (FEV1 ) and body mass index before and after pregnancy in women with and without pre-gestational diabetes having a first delivery RESULTS: A total 249 women delivered 314 infants...
June 24, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28646244/increased-soluble-vcam-1-and-normal-p-selectin-in-cystic-fibrosis-a-cross-sectional-study
#2
Jan K Nowak, Irena Wojsyk-Banaszak, Edyta Mądry, Andrzej Wykrętowicz, Patrycja Krzyżanowska, Sławomira Drzymała-Czyż, Agata Nowicka, Andrzej Pogorzelski, Ewa Sapiejka, Wojciech Skorupa, Mariusz Szczepanik, Aleksandra Lisowska, Jaroslaw Walkowiak
PURPOSE: As life expectancy in cystic fibrosis (CF) increases, questions regarding its potential impact on cardiovascular health arise. Soluble vascular cell adhesion molecule 1 (sVCAM-1), P-selectin (sP-selectin) are proposed as biomarkers of cardiovascular disease. We aimed to: compare their concentrations in clinically stable CF patients and healthy subjects (HS) and verify whether they independently correlate with CF characteristics. METHODS: Serum sVCAM-1 and sP-selectin levels were measured using ELISA...
June 23, 2017: Lung
https://www.readbyqxmd.com/read/28645157/secretory-iga-response-against-pseudomonas-aeruginosa-in-the-upper-airways-and-the-link-with-chronic-lung-infection-in-cystic-fibrosis
#3
Renan Mauch, Claudio Rossi, Talita Aiello, José Ribeiro, Antonio Ribeiro, Niels Høiby, Carlos Levy
We assessed the diagnostic ability of an ELISA test for measurement of specific secretory IgA (sIgA) in saliva to identify Cystic Fibrosis (CF) patients with P. aeruginosa chronic lung infection and intermittent lung colonization. A total of 102 Brazilian CF patients and 53 healthy controls were included. Specific serum IgG response was used as a surrogate to distinguish CF patients according to their P. aeruginosa colonization/infection status. The rate of sIgA positivity was 87.1% in CF chronically infected patients (Median value = 181...
June 22, 2017: Pathogens and Disease
https://www.readbyqxmd.com/read/28643178/economic-evaluation-of-tobramycin-inhalation-powder-for-the-treatment-of-chronic-pulmonary-pseudomonas-aeruginosa-infection-in-patients-with-cystic-fibrosis
#4
Srilekha Panguluri, Praveen Gunda, Laurie Debonnett, Kamal Hamed
BACKGROUND: Chronic lung infection with Pseudomonas aeruginosa occurs in approximately 50% of patients with cystic fibrosis (CF). This infection further compromises lung function, and significantly contributes to the increased healthcare costs. OBJECTIVES: Inhaled tobramycin, used to manage P. aeruginosa infection in CF patients, is available as powder (tobramycin inhalation powder, TIP) and solution (tobramycin inhalation solution, TIS). Evidence suggests increased adherence with the use of TIP over TIS...
June 22, 2017: Clinical Drug Investigation
https://www.readbyqxmd.com/read/28642745/structure-of-o-antigen-and-hybrid-biosynthetic-locus-in-burkholderia-cenocepacia-clonal-variants-recovered-from-a-cystic-fibrosis-patient
#5
A A Hassan, Rita F Maldonado, Sandra C Dos Santos, Flaviana Di Lorenzo, Alba Silipo, Carla P Coutinho, Vaughn S Cooper, Antonio Molinaro, Miguel A Valvano, Isabel Sá-Correia
Burkholderia cenocepacia is an opportunistic pathogen associated with chronic lung infections and increased risk of death in patients with cystic fibrosis (CF). In this work, we investigated the lipopolysaccharide (LPS) of clinical variants of B. cenocepacia that were collected from a CF patient over a period of 3.5 years, from the onset of infection until death by necrotizing pneumonia (cepacia syndrome). We report the chemical structure of the LPS molecule of various sequential isolates and the identification of a novel hybrid O-antigen (OAg) biosynthetic cluster...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28642338/cellular-distribution-and-function-of-ion-channels-involved-in-transport-processes-in-rat-tracheal-epithelium
#6
Anne Hahn, Johannes Faulhaber, Lalita Srisawang, Andreas Stortz, Johanna J Salomon, Marcus A Mall, Stephan Frings, Frank Möhrlen
Transport of water and electrolytes in airway epithelia involves chloride-selective ion channels, which are controlled either by cytosolic Ca(2+) or by cAMP The contributions of the two pathways to chloride transport differ among vertebrate species. Because rats are becoming more important as animal model for cystic fibrosis, we have examined how Ca(2+)- dependent and cAMP- dependent Cl(-) secretion is organized in the rat tracheal epithelium. We examined the expression of the Ca(2+)-gated Cl(-) channel anoctamin 1 (ANO1), the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel, the epithelial Na(+) channel ENaC, and the water channel aquaporin 5 (AQP5) in rat tracheal epithelium...
June 2017: Physiological Reports
https://www.readbyqxmd.com/read/28640808/transmembrane-helical-interactions-in-the-cftr-channel-pore
#7
Jhuma Das, Andrei A Aleksandrov, Liying Cui, Lihua He, John R Riordan, Nikolay V Dokholyan
Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene affect CFTR protein biogenesis or its function as a chloride channel, resulting in dysregulation of epithelial fluid transport in the lung, pancreas and other organs in cystic fibrosis (CF). Development of pharmaceutical strategies to treat CF requires understanding of the mechanisms underlying channel function. However, incomplete 3D structural information on the unique ABC ion channel, CFTR, hinders elucidation of its functional mechanism and correction of cystic fibrosis causing mutants...
June 22, 2017: PLoS Computational Biology
https://www.readbyqxmd.com/read/28639921/multisystem-imaging-findings-of-cystic-fibrosis-in-adults-recognizing-typical-and-atypical-patterns-of-disease
#8
Sarah Averill, Meghan G Lubner, Christine O Menias, Sanjeev Bhalla, Vincent M Mellnick, Tabassum A Kennedy, Perry J Pickhardt
OBJECTIVE: There is an expanding and increasingly heterogeneous population of adult patients with cystic fibrosis (CF). Although CF is usually diagnosed in children with progressive multisystem involvement, up to 7% of CF cases are currently diagnosed de novo in adults with subtle manifestations distinct from the typical features of classic CF. The purpose of this article is to present the wide spectrum of CF in adults, including both classic and nonclassic variants, with an emphasis on the nonclassic imaging findings...
July 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28639066/enzymatic-mechanisms-involved-in-evasion-of-fungi-to-the-oxidative-stress-focus-on-scedosporium-apiospermum
#9
C Staerck, P Vandeputte, A Gastebois, A Calenda, S Giraud, N Papon, J P Bouchara, M J J Fleury
The airways of patients with cystic fibrosis (CF) are frequently colonized by various filamentous fungi, mainly Aspergillus fumigatus and Scedosporium species. To establish within the respiratory tract and cause an infection, these opportunistic fungi express pathogenic factors allowing adherence to the host tissues, uptake of extracellular iron, or evasion to the host immune response. During the colonization process, inhaled conidia and the subsequent hyphae are exposed to reactive oxygen species (ROS) and reactive nitrogen species (RNS) released by phagocytic cells, which cause in the fungal cells an oxidative stress and a nitrosative stress, respectively...
June 21, 2017: Mycopathologia
https://www.readbyqxmd.com/read/28638254/inflammation-and-oxidation-biomarkers-in-patients-with-cystic-fibrosis-the-influence-of-azithromycin
#10
Casilda Olveira, Alicia Padilla, Antonio Dorado, Victoria Contreras, Eduardo Garcia-Fuentes, Elehazara Rubio-Martin, Nuria Porras, Esperanza Doña, Ana Carmona, Gabriel Olveira
OBJECTIVE: In addition to their antibiotic effect, macrolides appear to modulate the inflammatory response in cystic fibrosis (CF) and could influence oxidative stress. The objective of this study was to assess oxidation biomarkers and levels of inflammation and to determine whether there is an association between these parameters and the intake of macrolides. MATERIALS AND METHODS: The subjects included in this cross-sectional study were, on the one hand, clinically stable patients with CF and, on the other, healthy controls...
June 2017: Eurasian Journal of Medicine
https://www.readbyqxmd.com/read/28635619/on-the-importance-of-accurate-quantification-of-individual-volatile-metabolites-in-exhaled-breath
#11
David Smith, Patrik Spanel
It is argued that shortcomings of certain approaches to breath analysis research based on superficial interpretation of non-quantitative data are inadvertently inhibiting the progression of non-invasive breath analysis into clinical practice. The objective of this perspective is to suggest more clinically profitable approaches to breath research. Thus, following a discourse on the challenges and expectations in breath research, a brief indication is given of the analytical techniques currently used for the analysis of very humid exhaled breath...
June 21, 2017: Journal of Breath Research
https://www.readbyqxmd.com/read/28635544/heparin-new-life-for-an-old-drug
#12
Carlos Raúl Aláez-Versón, Elena Lantero, Xavier Fernàndez-Busquets
Heparin is one of the oldest drugs, which nevertheless remains in widespread clinical use as an inhibitor of blood coagulation. The history of its identification a century ago unfolded amid one of the most fascinating scientific controversies turning around the distribution of credit for its discovery. The composition, purification and structure-function relationship of this naturally occurring glycosaminoglycan regarding its classical role as anticoagulant will be dealt with before proceeding to discuss its therapeutic potential in, among other, inflammatory and infectious disease, cancer treatment, cystic fibrosis and Alzheimer's disease...
June 21, 2017: Nanomedicine
https://www.readbyqxmd.com/read/28634241/psl-produced-by-mucoid-pseudomonas-aeruginosa-contributes-to-the-establishment-of-biofilms-and-immune-evasion
#13
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/28634173/a-comparison-of-2-respiratory-devices-for-sputum-clearance-in-adults-with-non-cystic-fibrosis-bronchiectasis
#14
Yasmin R Silva, Tracy A Greer, Lucy C Morgan, Frank Li, Claude S Farah
BACKGROUND: Airway clearance techniques are a vital part of routine care for patients with bronchiectasis. There is no clear superior modality. The Flutter combines oscillations (6-20Hz) and positive expiratory pressure; the Lung Flute combines positive expiratory pressure and low frequency acoustic waves (18-22Hz), to augment clearance. This project aimed to compare these devices. METHODS: This was a randomized crossover study of adult subjects with stable non-cystic fibrosis bronchiectasis (expectorating >25 mL/d)...
June 20, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28634121/population-pharmacokinetics-of-tobramycin-inhalation-solution-in-pediatric-patients-with-cystic-fibrosis
#15
Xinting Wang, Stephan Koehne-Voss, SivaNaga S Anumolu, Jing Yu
Tobramycin inhalation solution (TOBI) given as a twice-daily inhalation of nebulized aerosols of 300 mg is approved for the treatment of Pseudomonas aeruginosa infection in cystic fibrosis (CF) patients over 6 years of age. To investigate tobramycin pharmacokinetics (PK) after inhalation of TOBI in pediatric CF patients below 7 years, a population PK approach was used to evaluate tobramycin PK data in patients 6 months to 44 years of age from four clinical studies. The final model used a two-compartmental, first-order absorption model with effect of body mass index on the apparent central volume of distribution...
June 17, 2017: Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28633068/multi-electrode-system-for-measurement-of-transmembrane-ion-fluxes-through-living-epithelial-cells
#16
Mirosław Zając, Andrzej Lewenstam, Krzysztof Dolowy
Cystic Fibrosis (CF) is the most common fatal human genetic disease. It is caused by the defect in a single anion channel protein which affects ion and water transport across the epithelial tissue. A flat multi-electrode platform of diameter 12mm, allowing for measurement of four ions: sodium, potassium, hydrogen and chloride by exchangeable/replaceable ion-selective electrodes is described. The measurement is possible owing to the architecture of the platform which accommodates all the electrodes and inlets/outlets...
June 13, 2017: Bioelectrochemistry
https://www.readbyqxmd.com/read/28632196/drug-hypersensitivity-and-desensitizations-mechanisms-and-new-approaches
#17
REVIEW
Leticia de Las Vecillas Sánchez, Leila A Alenazy, Marlene Garcia-Neuer, Mariana C Castells
Drug hypersensitivity reactions (HSRs) are increasing in the 21st Century with the ever expanding availability of new therapeutic agents. Patients with cancer, chronic inflammatory diseases, cystic fibrosis, or diabetes can become allergic to their first line therapy after repeated exposures or through cross reactivity with environmental allergens. Avoidance of the offending allergenic drug may impact disease management, quality of life, and life expectancy. Precision medicine provides new tools for the understanding and management of hypersensitivity reactions (HSRs), as well as a personalized treatment approach for IgE (Immunoglobuline E) and non-IgE mediated HSRs with drug desensitization (DS)...
June 20, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28630204/a-low-molecular-weight-alginate-oligosaccharide-disrupts-pseudomonal-microcolony-formation-and-enhances-antibiotic-effectiveness
#18
Manon F Pritchard, Lydia C Powell, Alison A Jack, Kate Powell, Konrad Beck, Hannah Florance, Julian Forton, Philip D Rye, Arne Dessen, Katja E Hill, David W Thomas
In chronic respiratory disease the formation of dense, 3-dimensional 'micro colonies' by Pseudomonas aeruginosa within the airway plays an important role in contributing to resistance to treatment. An in vitro biofilm model of pseudomonal microcolony formation using artificial sputum (AS) medium was established to study the effects of low molecular weight alginate oligomers (OligoG CF-5/20) on pseudomonal growth, microcolony formation and the efficacy of colistin. The studies employed clinical cystic fibrosis (CF) isolates (n=3) and reference non-mucoid and mucoid multi-drug resistant (MDR) CF isolates (n=7)...
June 19, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28630072/pseudomonas-quinolone-signal-pqs-induces-oxidative-stress-and-inhibits-heme-oxygenase-1-expression-in-lung-epithelial-cells
#19
Maher Y Abdalla, Traci Hoke, Javier Seravalli, Barbara L Switzer, Melissa Bavitz, Jill D Fliege, Peter J Murphy, Bradley E Britigan
Pseudomonas aeruginosa (PA) causes lung infection in patients with cystic fibrosis (CF). Pseudomonas Quinolone Signal (PQS) is a secreted PA virulence factor that contributes to the pathogenicity of PA. We were able to detect PQS in sputum samples from CF patients infected with PA, but not in samples from uninfected patients. We then tested the hypothesis that PQS induces oxidative stress in host cells by determining the ability of PQS to induce production of reactive oxygen species (ROS) in lung epithelial cells (A549 and primary normal human bronchial epithelial (NHBE)) and macrophages (J774A...
June 19, 2017: Infection and Immunity
https://www.readbyqxmd.com/read/28628280/combination-antimicrobial-susceptibility-testing-for-acute-exacerbations-in-chronic-infection-of-pseudomonas-aeruginosa-in-cystic-fibrosis
#20
REVIEW
Valerie Waters, Felix Ratjen
BACKGROUND: Antibiotic therapy for acute pulmonary exacerbations in people with cystic fibrosis is usually chosen based on the results of antimicrobial susceptibility testing of individual drugs. Combination antimicrobial susceptibility testing assesses the efficacy of drug combinations including two or three antibiotics in vitro and can often demonstrate antimicrobial efficacy against bacterial isolates even when individual antibiotics have little or no effect. Therefore, choosing antibiotics based on combination antimicrobial susceptibility testing could potentially improve response to treatment in people with cystic fibrosis with acute exacerbations...
June 19, 2017: Cochrane Database of Systematic Reviews
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