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https://www.readbyqxmd.com/read/29161417/bolus-weekly-vitamin-d3-supplementation-impacts-gut-and-airway-microbiota-in-adults-with-cystic-fibrosis-a-double-blind-randomized-placebo-controlled-clinical-trial
#1
Mansi Kanhere, Jiabei He, Benoit Chassaing, Thomas R Ziegler, Jessica A Alvarez, Elizabeth A Ivie, Li Hao, John Hanfelt, Andrew T Gewirtz, Vin Tangpricha
Background: Disruption of gut microbiota may exacerbate severity of cystic fibrosis (CF). Vitamin D deficiency is a common co-morbidity in patients with CF that may influence composition of the gut microbiota. Objectives: Compare microbiota of vitamin D sufficient and insufficient CF patients, and assess impact of a weekly high-dose vitamin D3 bolus regimen on gut and airway microbiome in adults with CF and vitamin D insufficiency (25(OH)D <30 ng/mL). Design: Forty-one subjects with CF were classified into two groups - vitamin D insufficient (n=23) and vitamin D sufficient (n=18)...
November 16, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29160746/nucleic-acid-therapies-for-cystic-fibrosis
#2
Shruti Sasaki, Shuling Guo
Nucleic acid therapeutics are an established class of drugs that enable specific targeting of a gene of interest. This diverse family of drugs includes antisense oligonucleotides, siRNAs, and mRNA replacement therapies, which can elicit both gene repression and activation, primarily at the RNA level. Recent advances in medicinal chemistry have increased drug potency and enhanced delivery and distribution to a broad array of tissue and cell types. A key advantage of nucleic acid therapeutics is in their application to monogenic diseases...
November 21, 2017: Nucleic Acid Therapeutics
https://www.readbyqxmd.com/read/29159032/use-of-ceftolozane-tazobactam-in-a-cystic-fibrosis-patient-with-multidrug-resistant-pseudomonas-infection-and-renal-insufficiency
#3
Katie Stokem, Jonathan B Zuckerman, David P Nicolau, Minkey Wungwattana, Edmund H Sears
We report the successful use of ceftolozane/tazobactam (C/T) to treat a pulmonary exacerbation in a 35 year old female, post lung transplant, with cystic fibrosis (CF), malnutrition, chronic kidney disease, and multi-drug resistant Pseudomonas aeruginosa infection (MDR PSA). Given the complexity of the clinical profile, we measured drug levels of C/T during treatment of her current exacerbation to determine pharmacokinetics. The patient achieved an estimated ceftolozane peak of 174.1 μg/mL and trough of 9...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29158263/high-throughput-screening-identifies-fau-protein-as-a-regulator-of-mutant-cystic-fibrosis-transmembrane-conductance-regulator-channel
#4
Valeria Tomati, Emanuela Pesce, Emanuela Caci, Elvira Sondo, Paolo Scudieri, Monica Marini, Felice Amato, Giuseppe Castaldo, Roberto Ravazzolo, Luis Jv Galietta, Nicoletta Pedemonte
In cystic fibrosis (CF), deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel causes misfolding and premature degradation. One possible approach to reducing CF's detrimental health effects could be the identification of proteins whose suppression rescues F508del-CFTR function in bronchial epithelial cells. However, searches for these potential targets have not yet been conducted, particularly in a relevant airway background using a functional readout...
November 20, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29157922/development-and-electronic-validation-of-the-revised-cystic-fibrosis-questionnaire-cfq-r-teen-adult-new-tool-for-monitoring-psychosocial-health-in-cf
#5
Amparo Solé, Casilda Olveira, Ines Pérez, David Hervás, Vincent Valentine, Anick N Baca Yepez, Gabriel Olveira, Alexandra L Quittner
BACKGROUND: The Cystic Fibrosis Questionnaire-Revised (CFQ-R+14) is a disease-specific, health-related quality of life instrument for cystic fibrosis (CF) patients ≥14years. We have developed a Spanish electronic version of the CFQ-R (e-CFQ-R+14 Spain). Our aim was to compare the paper and electronic versions and to validate the electronic version. METHODS: Fifty CF patients completed the study. All answered the paper and electronic versions on day 1 and repeated the e-CFQR version 15days later...
November 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29157921/pooling-of-bronchoalveolar-lavage-in-children-with-cystic-fibrosis-does-not-adversely-affect-the-microbiological-yield-or-sensitivity-in-detecting-pulmonary-inflammation
#6
Paul McNally, Jennifer O'Rourke, Emmanuelle Fantino, Archana Chacko, Rishi Pabary, Andrew Turnbull, Tim Grant, Niamh O'Sullivan, Claire Wainwright, Barry Linnane, Jane C Davies, Peter D Sly
BACKGROUND: Bronchoalveolar lavage (BAL) is a potentially useful outcome measure for clinical trials in children with CF but its use is limited by variations in approach internationally. We sought to determine if pooling adversely affected the diagnostic properties of BAL. METHODS: Children undergoing bronchoscopy for clinical reasons were included. A multi-step study protocol ensured BAL was collected and analysed both separately and as a pooled fluid. RESULTS: Eighty-five children (53 CF, 32 control) were recruited...
November 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29157900/a-practical-molecular-identification-of-nonfermenting-gram-negative-bacteria-from-cystic-fibrosis
#7
Carolina Paulino da Costa Capizzani, Natália Candido Caçador, Elizabeth Andrade Marques, Carlos Emílio Levy, Ludmilla Tonani, Lidia Alice Gomes Monteiro Marin Torres, Ana Lúcia da Costa Darini
Identification of nonfermenting Gram-negative bacteria (NFGNB) of cystic fibrosis patients is hard and misidentification could affect clinical outcome. This study aimed to propose a scheme using polymerase chain reaction to identify NFGNB. This scheme leads to reliable identification within 3 days in an economically viable manner when compared to other methods.
November 4, 2017: Brazilian Journal of Microbiology: [publication of the Brazilian Society for Microbiology]
https://www.readbyqxmd.com/read/29155707/optimized-lc-ms-ms-method-for-the-high-throughput-analysis-of-clinical-samples-of-ivacaftor-its-major-metabolites-and-lumacaftor-in-biological-fluids-of-cystic-fibrosis-patients
#8
Elena K Schneider, Felisa Reyes-Ortega, Jian Li, Tony Velkov
Defects in the cystic fibrosis trans-membrane conductance regulator (CFTR) are the cause of cystic fibrosis (CF), a disease with life-threatening pulmonary manifestations. Ivacaftor (IVA) and ivacaftor-lumacaftor (LUMA) combination are two new breakthrough CF drugs that directly modulate the activity and trafficking of the defective CFTR-protein. However, there is still a dearth of understanding on pharmacokinetic/pharmacodynamic parameters and the pharmacology of ivacaftor and lumacaftor. The HPLC-MS technique for the simultaneous analysis of the concentrations of ivacaftor, hydroxymethyl-ivacaftor, ivacaftor-carboxylate, and lumacaftor in biological fluids in patients receiving standard ivacaftor or ivacaftor-lumacaftor combination therapy has previously been developed by our group and partially validated to FDA standards...
October 15, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29155436/enzyme-replacement-therapy-for-infantile-onset-pompe-disease
#9
REVIEW
Min Chen, Lingli Zhang, Shuyan Quan
BACKGROUND: Infantile-onset Pompe disease is a rare and progressive autosomal-recessive disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). Current treatment involves enzyme replacement therapy (with recombinant human alglucosidase alfa) and symptomatic therapies (e.g. to control secretions). Children who are cross-reactive immunological material (CRIM)-negative require immunomodulation prior to commencing enzyme replacement therapy.Enzyme replacement therapy was developed as the most promising therapeutic approach for Pompe disease; however, the evidence is lacking, especially regarding the optimal dose and dose frequency...
November 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29155399/green-synthesis-of-anisotropic-gold-nanoparticles-using-hordenine-and-their-antibiofilm-efficacy-against-pseudomonas-aeruginosa
#10
Jobina Rajkumari, Himani Meena, Muralitharan Gangatharan, Siddhardha Busi
Pseudomonas aeruginosa is a notorious pathogen that causes biofilm aided infections in patients with cystic fibrosis and burn wounds, resulting in significant mortality in immunocompromised individuals. This study reports a novel one-step biosynthesis of gold nanoparticles using phytocompound, hordenine (HD), as a reducing and capping agent. The synthesis of the anisotropic hordenine-fabricated gold nanoparticles (HD-AuNPs) with an average particle size of 136.87 nm was achieved within 12 h of incubation at room temperature...
December 2017: IET Nanobiotechnology
https://www.readbyqxmd.com/read/29155014/resveratrol-dimer-trans-%C3%AE%C2%B5-viniferin-prevents-rotaviral-diarrhea-in-mice-by-inhibition-of-the-intestinal-calcium-activated-chloride-channel
#11
Bo Yu, Yu Jiang, Bo Zhang, Hong Yang, Tonghui Ma
We previously identified, by a natural-product screen, resveratrol oligomers as inhibitors of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel. Here, we report the resveratrol dimer trans-ε-viniferin (TV) and tetramer r-2-viniferin (RV) as inhibitors of the intestinal calcium-activated chloride channel (CaCC) and demonstrate their antisecretory efficacy in a neonatal mouse model of rotaviral diarrhea. Short-circuit measurements show inhibition of CaCC current in the human colonic cell line HT-29 by TV and RV with IC50∼1 and 20μM, respectively...
November 14, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/29154969/adults-with-cystic-fibrosis-have-deficits-in-bone-structure-and-strength-at-the-distal-tibia-despite-similar-size-and-measuring-standard-and-relative-sites
#12
Kyle K Nishiyama, Sanchita Agarwal, Anna Kepley, Fernando Rosete, Yizhong Hu, X Edward Guo, Claire L Keating, Emily A DiMango, Elizabeth Shane
Individuals with cystic fibrosis (CF) have lower bone mineral density (BMD) by DXA and are at higher risk of fracture than healthy controls. However, the 2-dimensional measurement of areal BMD (aBMD) provided by DXA is influenced by bone size and the true extent of the bone deficit is unclear. Our objective was to use high-resolution peripheral quantitative computed tomography (HR-pQCT) and individual trabecula segmentation (ITS) analysis to compare volumetric BMD (vBMD), microarchitecture and estimated strength at the distal radius and tibia in 26 young adults with CF and 26 controls matched for age, gender, and race...
November 14, 2017: Bone
https://www.readbyqxmd.com/read/29154949/a-novel-microscopy-based-assay-identifies-extended-synaptotagmin-1-esyt1-as-a-positive-regulator-of-anoctamin-1-traffic
#13
Joana R Lérias, Madalena C Pinto, Hugo M Botelho, Nikhil T Awatade, Margarida C Quaresma, Iris A L Silva, Podchanart Wanitchakool, Rainer Schreiber, Rainer Pepperkok, Karl Kunzelmann, Margarida D Amaral
An attractive possibility to treat Cystic Fibrosis (CF), a severe condition caused by dysfunctional CFTR, an epithelial anion channel, is through the activation of alternative (non-CFTR) anion channels. Anoctamin 1 (ANO1) was demonstrated to be a Ca(2+)-activated chloride channel (CaCC) and thus of high potential to replace CFTR. Despite that ANO1 is expressed in human lung CF tissue, it is present at the cell surface at very low levels. In addition, little is known about regulation of ANO1 traffic, namely which factors promote its plasma membrane (PM) localization...
November 15, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29154907/an-integrated-mathematical-epithelial-cell-model-for-airway-surface-liquid-regulation-by-mechanical-forces
#14
Dan Wu, Richard C Boucher, Brian Button, Timothy Elston, Ching-Long Lin
A robust method based on reverse engineering was utilized to construct the ion-channel conductance functions for airway epithelial sodium channels (ENaC), the cystic fibrosis transmembrane conductance regulator (CFTR), and calcium-activated chloride channels (CaCC). The ion-channel conductance models for both normal (NL) and cystic fibrosis (CF) airway epithelia were developed and then coupled to an adenosine triphosphate (ATP) metabolism model and a fluid transport model (collectively called the integrated cell model) to investigate airway surface liquid (ASL) volume regulation and hence mucus concentration, by mechanical forces in NL and CF human airways...
November 15, 2017: Journal of Theoretical Biology
https://www.readbyqxmd.com/read/29154891/acute-pain-perception-during-different-sampling-methods-for-respiratory-culture-in-cystic-fibrosis-patients
#15
Hanneke Eyns, Elke De Wachter, Anne Malfroot, Peter Vaes
BACKGROUND: Reliable identification of lower respiratory tract pathogens is crucial in the management of cystic fibrosis (CF). As part of another study (NCT02363764) investigating the bacterial yield of three sampling methods: nasal swabs (NS), cough swabs (CS) and (induced) sputum samples ((I)SS) in both expectorating (EPs) and non-expectorating (NEPs) patients with CF, the present study aimed to explore the prevalence of respiratory culture sampling-related pain as assessed by self-report within a cohort of children and adults...
November 14, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/29151314/effect-of-inhaled-steroids-on-clinical-and-inflammatoryparameters-in-children-with-cystic-fibrosis
#16
Zeynep Seda Uyan, Göksenin Ünlügüzel Üstün, Goncagül Haklar, Erkan Çakır, Sedat Oktem, Refika Ersu, Bülent Taner Karadağ, Fazilet Karakoç, Elif Dağlı
Background/aim: The effectiveness of inhaled corticosteroids (ICSs) in cystic fibrosis (CF) is controversial. The aim of this study was to investigate the effect of an ICS on bronchial hyperreactivity (BHR), oxidative status, and clinical and inflammatory parameters in CF patients. Materials and methods: CF patients were randomized to receive either 2 mg/day nebulized budesonide or 0.9% normal saline as placebo for 8 weeks. Results: Twenty-nine CF patients (mean age: 10.5 ± 2.9 years) were enrolled in the study...
November 13, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29150449/claudin-10-isoform-expression-and-cation-selectivity-change-with-salinity-in-salt-secreting-epithelia-of-f-heteroclitus
#17
William S Marshall, Jason P Breves, Ellen M Doohan, Christian K Tipsmark, Scott P Kelly, George N Robertson, Patricia M Schulte
To provide insight into claudin (Cldn) tight junction (TJ) protein contributions to branchial salt secretion in marine teleost fishes, this study examined cldn-10 TJ protein isoforms of a euryhaline teleost (mummichog; Fundulus heteroclitus) in association with salinity change and measurements of transepithelial cation selectivity. Mummichogs were transferred from fresh water (FW) to seawater (SW, 35 ‰) and from SW to hypersaline SW (2SW, 60 ‰) in a time course with transfer control groups (FW to FW and SW to SW)...
November 17, 2017: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29150357/caregiver-burden-of-parents-of-young-children-with-cystic-fibrosis
#18
C Fitzgerald, S George, R Somerville, B Linnane, P Fitzpatrick
BACKGROUND: There is a paucity of research examining the impact of informal caregiving on parents of young children with cystic fibrosis (CF). The aim of this study was to examine caregiver burden and identify risk factors associated with high caregiver burden in mothers and fathers of young children with CF. METHODS: This was a cross-sectional study of parents of young children with CF. A total of 213 families were invited to complete the CarerQoL questionnaire, a validated tool composed of two parts: (i) the CarerQol-7D which describes the care situation in terms of the negative and positive effects of caregiving and (ii) the visual analogue scale (VAS) which measures happiness on a scale from 0 to 10 (0=completely unhappy and 10=completely happy)...
November 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29150178/-relationships-between-copd-and-nontuberculous-mycobacteria-pulmonary-infections
#19
REVIEW
C Balavoine, C Andréjak, S Marchand-Adam, F X Blanc
Nontuberculous mycobacteria (NTM) pulmonary infections are increasingly frequent in pneumological practice. The diagnosis is based on an association of clinical, microbiological and radiological criteria. Some risk factors for NTM disease have been described, including chronic respiratory diseases, but without specific focus on chronic obstructive pulmonary disease (COPD). COPD patients have more risk to be infected by NTM than patients with the main other chronic respiratory diseases (except cystic fibrosis) (odds ratio [OR] at 15...
November 14, 2017: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/29150009/new-comorbidities-in-the-changing-face-of-cystic-fibrosis-care
#20
EDITORIAL
Michael Bonk, Michael M Rey, Denis Hadjiliadis
No abstract text is available yet for this article.
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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