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"Cystic fibrosis"

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https://www.readbyqxmd.com/read/28738346/synergistic-activity-of-berberine-with-azithromycin-against-pseudomonas-aeruginosa-isolated-from-patients-with-cystic-fibrosis-of-lung-in-vitro-and-in-vivo
#1
YongTao Li, JianRong Huang, LanJuan Li, LinSheng Liu
BACKGROUND/AIMS: Pseudomonas aeruginosa (PA) is one of the major opportunistic pathogens which can cause chronic lung infection of cystic fibrosis (CF). The formation of PA biofilm promotes CF development and restricts the antimicrobial efficacies of current antibiotics. METHODS: The antimicrobial effects of azithromycin (AZM) and berberine (BER) alone and in combination were evaluated using microdilution method, checkerboard assay, time-kill test, qRT-PCR analysis and absorption method...
July 24, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28738321/serum-phospholipid-fatty-acid-composition-in-cystic-fibrosis-patients-with-and-without-liver-cirrhosis
#2
Sławomira Drzymała-Czyż, Mariusz Szczepanik, Patrycja Krzyżanowska, Monika Duś-Żuchowska, Andrzej Pogorzelski, Ewa Sapiejka, Paweł Juszczak, Aleksandra Lisowska, Berthold Koletzko, Jarosław Walkowiak
BACKGROUND/AIMS: Cystic fibrosis (CF) liver disease is the third most frequent cause of death in CF patients. Although it alters fatty acid (FA) metabolism, data concerning the profile of FA in CF patients with liver cirrhosis is lacking. This study aimed to assess the FA composition of serum phospholipids in CF patients with and without liver cirrhosis. METHODS: The study comprised 25 CF patients with liver cirrhosis and 25 without it. We assessed Z-scores for body height and weight, lung function, exocrine pancreatic sufficiency and colonization with Pseudomonas aeruginosa...
July 22, 2017: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/28737283/prevalence-of-hearing-and-vestibular-loss-in-cystic-fibrosis-patients-exposed-to-aminoglycosides
#3
Jaynee A Handelsman, Samya Z Nasr, Crystal Pitts, William M King
AIM: Cystic Fibrosis (CF) patients frequently use aminoglycosides (AGS) to treat CF exacerbation due to colonization with Pseudomonas aeruginosa. Although AGS can cause vestibular and auditory sensory losses that can negatively impact quality of life, little is known about the prevalence of vestibular loss in this population. The aim of this study was to determine the prevalence of hearing loss and/or vestibular dysfunction in CF patients treated with AGS. METHODS: The relationship between hearing status and vestibular status was also investigated...
July 24, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28737262/overnight-delivery-of-hypertonic-saline-by-nasal-cannula-aerosol-for-cystic-fibrosis
#4
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/28737140/bone-mineral-density-of-indian-children-and-adolescents-with-cystic-fibrosis
#5
Sumita Gupta, Aparna Mukherjee, Rajesh Khadgawat, Madhulika Kabra, Rakesh Lodha, Sushil K Kabra
OBJECTIVE: To document bone mineral density of children and adolescents with cystic fibrosis. DESIGN: Cross-sectional study. SETTING: Tertiary-care center of Northern India, July 2012 to August 2015. PARTICIPANTS: 52 children aged 6-18 years with cystic fibrosis and 62 healthy controls of similar age and sex. METHODS: Both patients and controls were stratified into two groups, as pre-pubertal and peri-/post-pubertal, and compared for whole body bone mineral density, measured using dual energy X-ray absorptiometry...
July 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28737138/optimizing-bone-health-in-cystic-fibrosis-success-is-the-sum-of-small-efforts
#6
Sonal Kansra, Noreen West
No abstract text is available yet for this article.
July 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28737008/design-and-synthesis-of-new-4-alkylidene-beta-lactams-benzyl-and-phenethyl-carbamate-as-key-fragments-to-switch-on-the-antibacterial-activity
#7
Daria Giacomini, Giulia Martelli, Miriam Piccichè, Enrico Calaresu, Clementina Elvezia Anna Cocuzza, Rosario Musumeci
The emergence of multidrug resistant bacterial strains is particularly important in some chronic pathologies such as cystic fibrosis (CF), where persistent colonization and selection of resistant strains being favoured by the frequent and repeated use of antibacterial agents. Staphylococcus aureus is a common pathogen in CF patients with an associated increased multidrug resistance. In a previous research we demonstrated that the presence of a 4-alkylidene side chain directly linked to a beta-lactam appeared to strengthen the potency against S...
July 23, 2017: ChemMedChem
https://www.readbyqxmd.com/read/28736296/a-new-targeted-cftr-mutation-panel-based-on-next-generation-sequencing-technology
#8
Marco Lucarelli, Luigi Porcaro, Alice Biffignandi, Lucy Costantino, Valentina Giannone, Luisella Alberti, Sabina Maria Bruno, Carlo Corbetta, Erminio Torresani, Carla Colombo, Manuela Seia
Searching for mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR) is a key step in the diagnosis of and neonatal and carrier screening for cystic fibrosis (CF), and it has implications for prognosis and personalized therapy. The large number of mutations and genetic and phenotypic variability make this search a complex task. Herein, we tested the clinical and laboratory validity of an extended search for mutations in CFTR using a next-generation sequencing-based method, with a panel of 188 CFTR mutations customized for the Italian population...
July 19, 2017: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/28735882/antibiotic-resistance-and-population-structure-of-cystic-fibrosis-pseudomonas-aeruginosa-isolates-from-a-spanish-multi-centre-study
#9
Carla López-Causapé, Juan de Dios-Caballero, Marta Cobo, Amparo Escribano, Óscar Asensio, Antonio Oliver, Rosa Del Campo, Rafael Cantón
The first Spanish multi-centre study on the microbiology of cystic fibrosis (CF) was conducted from 2013 to 2014. The study involved 24 CF units from 17 hospitals, and recruited 341 patients. The aim of this study was to characterise Pseudomonas aeruginosa isolates, 79 of which were recovered from 75 (22%) patients. The study determined the population structure, antibiotic susceptibility profile and genetic background of the strains. Fifty-five percent of the isolates were multi-drug-resistant, and 16% were extensively-drug-resistant...
July 20, 2017: International Journal of Antimicrobial Agents
https://www.readbyqxmd.com/read/28735752/conformational-changes-of-cftr-upon-phosphorylation-and-atp-binding
#10
Zhe Zhang, Fangyu Liu, Jue Chen
The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel evolved from an ATP-binding cassette transporter. CFTR channel gating is strictly coupled to phosphorylation and ATP hydrolysis. Previously, we reported essentially identical structures of zebrafish and human CFTR in the dephosphorylated, ATP-free form. Here, we present the structure of zebrafish CFTR in the phosphorylated, ATP-bound conformation, determined by cryoelectron microscopy to 3.4 Å resolution. Comparison of the two conformations shows major structural rearrangements leading to channel opening...
July 18, 2017: Cell
https://www.readbyqxmd.com/read/28733286/the-regulator-ldhr-and-the-d-lactate-dehydrogenase-ldha-of-burkholderia-multivorans-play-a-role-in-carbon-overflow-and-in-planktonic-cellular-aggregates-formation
#11
Inês N Silva, Marcelo J Ramires, Lisa A Azevedo, Ana R Guerreiro, Andreia C Tavares, Jörg D Becker, Leonilde M Moreira
LysR-type transcriptional regulators (LTTR) are the most commonly found regulators in Burkholderia cepacia complex, comprising opportunistic pathogens causing chronic respiratory infections in cystic fibrosis (CF) patients. Despite LTTRs being global regulators of pathogenicity in several bacteria, few have been characterized in Burkholderia Here, we showed that gene ldhR of B. multivorans encoding a LTTR is co-transcribed with ldhA encoding a d-lactate dehydrogenase, and evaluate their implication in virulence traits like exopolysaccharide (EPS) synthesis and biofilm formation...
July 21, 2017: Applied and Environmental Microbiology
https://www.readbyqxmd.com/read/28732801/bicarbonate-in-cystic-fibrosis
#12
REVIEW
Karl Kunzelmann, Rainer Schreiber, Hans Beat Hadorn
BACKGROUND: Cystic fibrosis (CF, mucoviscidosis) is caused by mutations in the gene encoding CF transmembrane conductance regulator (CFTR), which is a chloride and bicarbonate channel necessary for fluid secretion and extracellular alkalization. For a long time, research concentrated on abnormal Cl(-) and Na(+) transport, but neglected bicarbonate as a crucial factor in CF. METHODS: The present short review reports early findings as well as recent insights into the role of CFTR for bicarbonate transport and its defects in CF...
July 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28730885/ataluren-in-cystic-fibrosis-development-clinical-studies-and-where-are-we-now
#13
Noreen Zainal Abidin, Iram J Haq, Aaron I Gardner, Malcolm Brodlie
Cystic fibrosis (CF) is one of the most common genetically-acquired life-limiting conditions worldwide. The underlying defect is dysfunction of the cystic fibrosis transmembrane-conductance regulator (CFTR) which leads to progressive lung disease and other multi-system effects. Around 10% of people with CF have a class I nonsense mutation that leads to production of shortened CFTR due to a premature termination codon (PTC). Areas covered: We discuss the discovery of the small-molecule drug ataluren, which in vitro has been shown to allow read-through of PTCs and facilitate synthesis of full-length protein...
July 21, 2017: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/28729470/detection-and-monitoring-of-lung-inflammation-in-cystic-fibrosis-during-respiratory-tract-exacerbation-using-diffusion-weighted-magnetic-resonance-imaging
#14
Pierluigi Ciet, Silvia Bertolo, Mirco Ros, Eleni Rosalina Andrinopoulou, Valentina Tavano, Francesca Lucca, Thorsten Feiweier, Gabriel P Krestin, Harm A W M Tiddens, Giovanni Morana
The aim was to investigate whether diffusion-weighted magnetic resonance imaging (DWI) detects and monitors inflammatory and lung function changes during respiratory tract exacerbations (RTE) treatment in patients with cystic fibrosis (CF).29 patients with RTE underwent DWI pre- and post-antibiotic treatment. A control group of 27 stable patients, matched for age and sex, underwent DWI with the same time gap as those undergoing RTE treatment. Clinical status and lung function were assessed at each DWI time point...
July 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28728770/don-t-push-your-luck-educational-family-board-not-bored-game-for-school-age-children-living-with-chronic-conditions
#15
Andrea Kennedy, Lisa Semple, Kerri Alderson, Vanessa Bouskill, Janice Karasevich, Brenda Riske, Sheri van Gunst
PURPOSE: Children who are living with chronic conditions may be supported in self-care through enjoyable active learning and family social processes. This research focused on development and evaluation of "Don't Push Your Luck!", an educational board game designed to inspire family discussion about chronic conditions, and help affected children learn about self-care choices and consequences. DESIGN AND METHODS: Mixed-method research was conducted with families from one outpatient Cystic Fibrosis Clinic and four Hemophilia Treatment Centres in Canada and United States (N=72)...
July 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28728531/disclosures-of-cystic-fibrosis-related-information-to-romantic-partners
#16
Katie Broekema, Kirsten M Weber
In this article, we offer insights into how individuals with cystic fibrosis (CF) share information about their disease with a romantic partner. Using communication privacy management as a sensitizing theoretical construct, four themes emerged following 13 qualitative interviews with persons with CF. Themes about sharing CF-related information with a romantic partner include weighing the risks and benefits of sharing information, the role of health-related triggered rules, the motivations for disclosures, and the reactions from outsiders...
August 2017: Qualitative Health Research
https://www.readbyqxmd.com/read/28727474/a-primer-on-exocrine-pancreatic-insufficiency-fat-malabsorption-and-fatty-acid-abnormalities
#17
Samer Alkaade, Ashley A Vareedayah
Exocrine pancreatic insufficiency (EPI) is characterized by a deficiency of exocrine pancreatic enzymes, resulting in deficits in digestion of all macronutrients, with deficiencies in digestion of fats being the most clinically relevant. The leading cause of EPI is chronic pancreatitis. However, many other causes and conditions may be implicated, including cystic fibrosis, pancreatic duct obstruction, gastric and pancreatic surgery, diabetes mellitus and other conditions. Physical and biochemical causes of EPI include decreased production and secretion of lipase, increased lipase destruction, pancreatic duct obstruction, decreased lipase stimulation and degradation, as well as gastrointestinal motility disorders...
July 2017: American Journal of Managed Care
https://www.readbyqxmd.com/read/28727186/telephone-monitoring-and-home-visits-significantly-improved-the-quality-of-life-treatment-adherence-and-lung-function-in-children-with-cystic-fibrosis
#18
Elisavet-Anna Chrysochoou, Elpis Hatziagorou, Fotis Kirvassilis, John Tsanakas
Cystic Fibrosis (CF) is a chronic and systemic disease with a progressive course. As survival rates continue to improve, there is a growing demand for new therapeutic options that improve treatment adherence, disease management and quality of life (QoL) (1). The aim of this study was to evaluate the safety and effectiveness of a home care programme for children with CF and to assess the value of regular telephone contact with the CF team based at Hippokration Hospital, Thessaloniki, Greece. This article is protected by copyright...
July 20, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28726737/respiratory-tract-infections-and-the-role-of-biologically-active-polysaccharides-in-their-management-and-prevention
#19
REVIEW
Milos Jesenak, Ingrid Urbancikova, Peter Banovcin
Respiratory tract infections (RTIs) are the most common form of infections in every age category. Recurrent respiratory tract infections (RRTIs), a specific form of RTIs, represent a typical and common problem associated with early childhood, causing high indirect and direct costs on the healthcare system. They are usually the consequence of immature immunity in children and high exposure to various respiratory pathogens. Their rational management should aim at excluding other severe chronic diseases associated with increased morbidity (e...
July 20, 2017: Nutrients
https://www.readbyqxmd.com/read/28726588/reconstituting-mouse-lungs-with-conditionally-reprogrammed-human-bronchial-epithelial-cells
#20
Ryan LaRanger, Jennifer R Peters-Hall, Melissa Coquelin, Busola R Alabi, Chris Chen, Woodring E Wright, Jerry Shay
We developed methods for conditionally reprogramming (CR) primary human bronchial epithelial cells (HBECs) to extend their functional life span and permit their differentiation into both upper and lower airway lung epithelium. We also developed a bioreactor to support vascular perfusion and rhythmic breathing of decellularized mouse lungs reconstituted with CR HBECs isolated from patients with and without cystic fibrosis (CF). While conditionally reprogrammed cells only differentiate into an upper airway epithelium after 35 days at the air liquid interface, in reconstituted lungs these cells differentiate into upper airway bronchial epithelium and lower airway alveolar structures after 12 days...
July 20, 2017: Tissue Engineering. Part A
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