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https://www.readbyqxmd.com/read/28097632/acquisition-and-adaptation-of-the-airway-microbiota-in-the-early-life-of-cystic-fibrosis-patients
#1
REVIEW
Sébastien Boutin, Alexander H Dalpke
Cystic fibrosis (CF) is a genetic disease in which bacterial infections of the airways play a major role in the long-term clinical outcome. In recent years, a number of next-generation sequencing (NGS)-based studies aimed at deciphering the structure and composition of the airways' microbiota. It was shown that the nasal cavity of CF patients displays dysbiosis early in life indicating a failure in the first establishment of a healthy microbiota. In contrast, within the conducting and lower airways, the establishment occurs normally first, but is sensitive to future dysbiosis including chronic infections with classical pathogens in later life...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28096997/bronchial-tree-shaped-mucous-plug-in-cystic-fibrosis-imaging-guided-management
#2
Ignazio Salamone, Baldassare Mondello, Maria Cristina Lucanto, Simona Cristadoro, Mariangela Lombardo, Mario Barone
We report the case of a 17-year-old boy with cystic fibrosis (CF) who presented with persistent cough; after starting intravenous antibiotics for Pseudomonas aeruginosa he underwent a computed tomography (CT) scan of the chest. CT revealed extensive consolidation in the right lower lobe with relative bronchus obstruction; the cause of bronchial obstruction was detected in the mediastinal window, corresponding to a bronchial tree-shaped, thick, tenacious mucous plug. This was extracted 48 h after unresponsive bronchial washing and endobronchial instillation of rhDNAse, using foreign-body forceps, with subsequent resolution of cough...
March 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28096734/chest-ct-features-of-cystic-fibrosis-in-korea-comparison-with-non-cystic-fibrosis-diseases
#3
So Yeon Yang, Kyung Soo Lee, Min Jae Cha, Tae Jung Kim, Tae Sung Kim, Hyun Jung Yoon
OBJECTIVE: Cystic fibrosis (CF) is a rare congenital disease in Korea, and its clinical and imaging findings are unclear. The objective of our study was to describe the clinical and CT features of CF in Korea and compare its features with those of other diseases mimicking CF. MATERIALS AND METHODS: From November 1994 to December 2014, a presumptive diagnosis of CF was made in 23 patients based on clinical or radiological examination. After the exclusion of 10 patients without diagnostic confirmation, 13 patients were included in the study...
January 2017: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
https://www.readbyqxmd.com/read/28096165/in-vitro-activity-of-ceftolozane-tazobactam-against-multidrug-resistant-non-fermenting-gram-negative-bacilli-isolated-from-patients-with-cystic-fibrosis
#4
Patrick Grohs, Gary Taieb, Philippe Morand, Iheb Kaibi, Isabelle Podglajen, Marie Lavollay, Jean-Luc Mainardi, Fabrice Compain
Ceftolozane-tazobactam was tested against 58 multidrug-resistant non-fermenting Gram-negative bacilli (35 Pseudomonas aeruginosa, 11 Achromobacter xylosoxydans and 12 Stenotrophomonas maltophilia) isolated from cystic fibrosis patients and compared to ceftolozane alone, ceftazidime, meropenem and piperacillin-tazobactam. Ceftolozane-tazobactam was the most active agent against P. aeruginosa but was inactive against A. xylosoxydans and S. maltophilia In time-kill experiments ceftolozane-tazobactam had complete bactericidal activity against 2/6 (33%) clinical isolates...
January 17, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28096157/resistance-to-thiacetazone-derivatives-active-against-mycobacterium-abscessus-involves-mutations-in-the-mmpl5-transcriptional-repressor-mab_4384
#5
Iman Halloum, Albertus Viljoen, Varun Khanna, Derek Craig, Christiane Bouchier, Roland Brosch, Geoffrey Coxon, Laurent Kremer
Available chemotherapeutic options are very limited against Mycobacterium abscessus, which imparts a particular challenge in the treatment of cystic fibrosis (CF) patients infected with this rapid-growing mycobacterium. New drugs are urgently needed against this emerging pathogen, but the discovery of active chemotypes has not been performed intensively. Interestingly, however, the repurposing of thiacetazone (TAC), a drug once used to treat tuberculosis, has increased following the deciphering of its mechanism of action and the detection of significantly more potent analogues...
January 17, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#6
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28094839/improved-fluorescence-assays-to-measure-the-defects-associated-with-f508del-cftr-allow-identification-of-new-active-compounds
#7
Emily Langron, Michela I Simone, Clémence M S Delalande, Jean-Louis Reymond, David L Selwood, Paola Vergani
BACKGROUND AND PURPOSE: Cystic fibrosis (CF) is a debilitating disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which codes for a Cl-/HCO3 - channel. F508del, the most common CF-associated mutation, causes both gating and biogenesis defects in the CFTR protein. This paper describes the optimisation of two fluorescence assays, capable of measuring CFTR function and cellular localisation, and their use in a pilot drug screen. EXPERIMENTAL APPROACH: HEK293 cells expressing YFP-F508del-CFTR, in which halide sensitive YFP is tagged to the N-terminal of CFTR, were used to screen a small library of compounds based on the VX-770 scaffold...
January 17, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28094327/rapid-adaptation-drives-invasion-of-airway-donor-microbiota-by-pseudomonas-after-lung-transplantation
#8
M Beaume, T Köhler, G Greub, O Manuel, J-D Aubert, L Baerlocher, L Farinelli, A Buckling, C van Delden
In cystic fibrosis (CF) patients, chronic airway infection by Pseudomonas leads to progressive lung destruction ultimately requiring lung transplantation (LT). Following LT, CF-adapted Pseudomonas strains, potentially originating from the sinuses, may seed the allograft leading to infections and reduced allograft survival. We investigated whether CF-adapted Pseudomonas populations invade the donor microbiota and adapt to the non-CF allograft. We collected sequential Pseudomonas isolates and airway samples from a CF-lung transplant recipient during two years, and followed the dynamics of the microbiota and Pseudomonas populations...
January 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28093527/human-cystic-fibrosis-macrophages-have-defective-calcium-dependent-protein-kinase-c-activation-of-the-nadph-oxidase-an-effect-augmented-by-burkholderia-cenocepacia
#9
Kaivon Assani, Chandra L Shrestha, Frank Robledo-Avila, Murugesan V Rajaram, Santiago Partida-Sanchez, Larry S Schlesinger, Benjamin T Kopp
Macrophage intracellular pathogen killing is defective in cystic fibrosis (CF), despite abundant production of reactive oxygen species (ROS) in lung tissue. Burkholderia species can cause serious infection in CF and themselves affect key oxidase components in murine non-CF cells. However, it is unknown whether human CF macrophages have an independent defect in the oxidative burst and whether Burkholderia contributes to this defect in terms of assembly of the NADPH oxidase complex and subsequent ROS production...
January 16, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28093464/socioeconomic-status-smoke-exposure-and-health-outcomes-in-young-children-with-cystic-fibrosis
#10
Thida Ong, Michael Schechter, Jing Yang, Limin Peng, Julia Emerson, Ronald L Gibson, Wayne Morgan, Margaret Rosenfeld
BACKGROUND: Lower socioeconomic status (SES) and environmental tobacco smoke (ETS) exposure are both associated with poorer disease outcomes in cystic fibrosis (CF), and children with low SES are disproportionately exposed to ETS. We analyzed a large cohort of young children with CF to distinguish the impact of SES and ETS on clinical outcomes. METHODS: The Early Pseudomonas Infection Control Observational study enrolled Pseudomonas-negative young children with CF <13 years of age...
January 16, 2017: Pediatrics
https://www.readbyqxmd.com/read/28091521/mechanisms-of-intrinsic-resistance-and-acquired-susceptibility-of-pseudomonas-aeruginosa-isolated-from-cystic-fibrosis-patients-to-temocillin-a-revived-antibiotic
#11
Hussein Chalhoub, Daniel Pletzer, Helge Weingart, Yvonne Braun, Michael M Tunney, J Stuart Elborn, Hector Rodriguez-Villalobos, Patrick Plésiat, Barbara C Kahl, Olivier Denis, Mathias Winterhalter, Paul M Tulkens, Françoise Van Bambeke
The β-lactam antibiotic temocillin (6-α-methoxy-ticarcillin) shows stability to most extended spectrum β-lactamases, but is considered inactive against Pseudomonas aeruginosa. Mutations in the MexAB-OprM efflux system, naturally occurring in cystic fibrosis (CF) isolates, have been previously shown to reverse this intrinsic resistance. In the present study, we measured temocillin activity in a large collection (n = 333) of P. aeruginosa CF isolates. 29% of the isolates had MICs ≤ 16 mg/L (proposed clinical breakpoint for temocillin)...
January 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28090087/a-mast-cell-ilc2-th9-pathway-promotes-lung-inflammation-in-cystic-fibrosis
#12
Silvia Moretti, Giorgia Renga, Vasilis Oikonomou, Claudia Galosi, Marilena Pariano, Rossana G Iannitti, Monica Borghi, Matteo Puccetti, Marco De Zuani, Carlo E Pucillo, Giuseppe Paolicelli, Teresa Zelante, Jean-Christophe Renauld, Oxana Bereshchenko, Paolo Sportoletti, Vincenzina Lucidi, Maria Chiara Russo, Carla Colombo, Ersilia Fiscarelli, Cornelia Lass-Flörl, Fabio Majo, Gabriella Ricciotti, Helmut Ellemunter, Luigi Ratclif, Vincenzo Nicola Talesa, Valerio Napolioni, Luigina Romani
T helper 9 (Th9) cells contribute to lung inflammation and allergy as sources of interleukin-9 (IL-9). However, the mechanisms by which IL-9/Th9 mediate immunopathology in the lung are unknown. Here we report an IL-9-driven positive feedback loop that reinforces allergic inflammation. We show that IL-9 increases IL-2 production by mast cells, which leads to expansion of CD25(+) type 2 innate lymphoid cells (ILC2) and subsequent activation of Th9 cells. Blocking IL-9 or inhibiting CD117 (c-Kit) signalling counteracts the pathogenic effect of the described IL-9-mast cell-IL-2 signalling axis...
January 16, 2017: Nature Communications
https://www.readbyqxmd.com/read/28088585/nocardiosis-in-south-of-france-during-the-last-ten-years
#13
Delphine Haussaire, Pierre-Edouard Fournier, Karamoko Djiguiba, Valerie Moal, Tristan Legris, Rajsingh Purgus, Jeremy Bismuth, Xavier Elharrar, Martine Reynaud-Gaubert, Henri Vacher-Coponat
BACKGROUND: Nocardiosis is a rare disease with polymorphic presentations. Epidemiology and clinical presentation could change with the increasing number of immunocompromised patients. METHODS: Medical records and microbiologic data for patients affected by nocardiosis and treated in the university hospital of Marseille between 2004 and 2014 were retrospectively analyzed. RESULTS: We analyzed 34 patients infected by Nocardia spp during this period...
January 11, 2017: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/28088327/cftr-impairment-upregulates-c-src-activity-through-il-1%C3%AE-autocrine-signaling
#14
María Macarena Massip-Copiz, Mariángeles Clauzure, Ángel Gabriel Valdivieso, Tomás Antonio Santa-Coloma
Cystic Fibrosis (CF) is a disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Previously, we found several genes showing a differential expression in CFDE cells (epithelial cells derived from a CF patient). One corresponded to c-Src; its expression and activity was found increased in CFDE cells, acting as a signaling molecule between the CFTR activity and MUC1 overexpression. Here we report that bronchial IB3-1 cells (CF cells) also showed increased c-Src activity compared to 'CFTR-corrected' S9 cells...
January 11, 2017: Archives of Biochemistry and Biophysics
https://www.readbyqxmd.com/read/28088130/vaccine-candidates-against-nontypeable-haemophilus-influenzae-a-review
#15
REVIEW
Ava Behrouzi, Farzam Vaziri, Fatemeh Rahimi-Jamnani, Parviz Afrough, Mohammad Rahbar, Fereshteh Satarian, Seyed Davar Siadat
Nonencapsulated, nontypeable Hemophilus influenzae (NTHi) remains an important cause of acute otitis and respiratory diseases in children and adults. NTHi bacteria are one of the major causes of respiratory tract infections, including acute otitis media, cystic fibrosis, and community-acquired pneumonia among children, especially in developing countries. The bacteria can also cause chronic diseases such as chronic bronchitis and chronic obstructive pulmonary disease in the lower respiratory tract of adults...
2017: Iranian Biomedical Journal
https://www.readbyqxmd.com/read/28087700/two-small-molecules-restore-stability-to-a-sub-population-of-the-cystic-fibrosis-transmembrane-conductance-regulator-with-the-predominant-disease-causing-mutation
#16
Xin Meng, Yiting Wang, Xiaomeng Wang, Joe A Wrennall, Tracy L Rimington, Hongyu Li, Zhiwei Cai, Robert C Ford, David N Sheppard
Cystic fibrosis (CF) is caused by mutations that disrupt the plasma membrane expression, stability, and function of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel. Two small molecules, the CFTR corrector lumacaftor and the potentiator ivacaftor, are now used clinically to treat CF, although some studies suggest that they have counteracting effects on CFTR stability. Here, we investigated the impact of these compounds on the instability of F508del-CFTR, the most common CF mutation...
January 13, 2017: Journal of Biological Chemistry
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
#17
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...
January 10, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28086849/successful-outcome-following-pneumonectomy-in-a-teenage-boy-with-cystic-fibrosis-a-case-report
#18
Zheyi Liew, Santosh Mallikarjuna, Asif Hasan, F Kate Gould, Su Bunn, Matthew F Thomas, Jim L Lordan, Christopher O'Brien, Malcolm Brodlie
BACKGROUND: Cystic fibrosis lung disease is generally a diffuse process however rarely one lung may become particularly damaged through chronic collapse and consolidation resulting in end-stage bronchiectasis with relative sparing of the contralateral lung. This clinical situation is sometimes referred to as "destroyed lung". Lung resection surgery is seldom indicated in cystic fibrosis and the associated medical literature is relatively sparse. CASE PRESENTATION: A 14 year old boy was referred to our centre for lung transplantation assessment...
January 13, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28079883/cysteamine-re-establishes-the-clearance-of-pseudomonas-aeruginosa-by-macrophages-bearing-the-cystic-fibrosis-relevant-f508del-cftr-mutation
#19
Eleonora Ferrari, Romina Monzani, Valeria R Villella, Speranza Esposito, Francesca Saluzzo, Federica Rossin, Manuela D'Eletto, Antonella Tosco, Fabiola De Gregorio, Valentina Izzo, Maria C Maiuri, Guido Kroemer, Valeria Raia, Luigi Maiuri
Cystic fibrosis (CF), the most common lethal monogenic disease in Caucasians, is characterized by recurrent bacterial infections and colonization, mainly by Pseudomonas aeruginosa, resulting in unresolved airway inflammation. CF is caused by mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which functions as a chloride channel in epithelial cells, macrophages, and other cell types. Impaired bacterial handling by macrophages is a feature of CF airways, although it is still debated how defective CFTR impairs bacterial killing...
January 12, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28078950/magnetic-nanoparticles-based-drug-and-gene-delivery-systems-for-the-treatment-of-pulmonary-diseases
#20
Ibrahim M El-Sherbiny, Nancy M Elbaz, Mohammed Sedki, Abdulaziz Elgammal, Magdi H Yacoub
Magnetic nanoparticles (MNPs) have gained much attention due to their unique properties such as biocompatibility and biodegradability as well as magnetic and heat-medicated characteristics. Due to these inherent properties, MNPs have been widely used in various biomedical applications including targeted drug delivery and hyperthermia-based therapy. Hyperthermia is a promising approach for the thermal activation therapy of several diseases, including pulmonary diseases. Additionally, due to their large loading capacity and controlled release ability, several MNP-based drug delivery systems have been emerged for treatment of cystic fibrosis and lung cancer...
January 12, 2017: Nanomedicine
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