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Cystic fibrosis

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https://www.readbyqxmd.com/read/29145426/buserelin-alleviates-chloride-transport-defect-in-human-cystic-fibrosis-nasal-epithelial-cells
#1
Marie-Laure Calvez, Nathalie Benz, Florentin Huguet, Aude Saint-Pierre, Elise Rouillé, Christelle Coraux, Claude Férec, Mathieu Kerbiriou, Pascal Trouvé
Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians caused by mutations in the gene encoding the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) chloride (Cl-) channel regulated by protein kinases, phosphatases, divalent cations and by protein-protein interactions. Among protein-protein interactions, we previously showed that Annexin A5 (AnxA5) binds to CFTR and is involved in the channel localization within membranes and in its Cl- channel function. The deletion of phenylalanine at position 508 (F508del) is the most common mutation in CF which leads to an altered protein (F508del-CFTR) folding with a nascent protein retained within the ER and is quickly degraded...
2017: PloS One
https://www.readbyqxmd.com/read/29143574/investigating-the-preventive-effects-of-baicalin-and-gallocatechin-aginst-glyoxal-induced-cystatin-aggregation
#2
Aamir Sohail, Waseem Feeroze Bhat, Mohammad Furkan, Aaliya Shah, Bilqees Bano
Several mammalian proteins form pathological deposits under nonphysiological conditions that are associated with many degenerative diseases. Protein aggregation is associated with aging, as well as a variety of diseases, including cystic fibrosis, amyotrophic lateral sclerosis (ALS), and hypertrophic cardiomyopathy. There is a lack of any potential anti-amyloidogenic agents and therapeutics till date. Polyphenols have been accredited with myriad biological effects. An analysis of the effects of natural agents like baicalin (BC) and gallocatechin (GC) on aggregation process can open new avenues for the treatment of protein misfolding diseases...
November 16, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/29143505/-inhaled-antibiotics-for-the-management-of-non-cystic-fibrosis-bronchiectasis
#3
Gregory Berra, Émilie Chappuis-Gisin, Paola M Soccal, Jérôme Plojoux
Bronchiectasis is irreversible bronchial dilatation associated with chronic respiratory symptoms. Management is aimed at reducing symptoms and slowing the progression of the disease by interrupting the vicious circle: bronchial infection, inflammation, altered mucociliary clearance, lung destruction. Unlike the literature on inhaled antibiotics in cystic fibrosis, literature data are limited and of low quality for bronchiectasis of other causes. However, new recommendations from the European Respiratory Society propose the conditional use of inhaled antibiotics to prevent repeated infectious exacerbations and to eradicate Pseudomonas aeruginosa colonization...
November 15, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29143340/k2p-task-2-and-kcnq1-kcne3-k-channels-are-major-players-contributing-to-intestinal-anion-and-fluid-secretion
#4
Francisca Julio-Kalajzić, Sandra Villanueva, Johanna Burgos, Margarita Ojeda, L Pablo Cid, Thomas J Jentsch, Francisco V Sepúlveda
Anion and fluid secretion across the intestinal epithelium, a process altered in cystic fibrosis and secretory diarrhoea, is mediated by cAMP-activated CFTR Cl(-) channels and requires the simultaneous activity of basolateral K(+) channels to maintain cellular ionic homeostasis and membrane potential. This function is fulfilled by the cAMP-activated K(+) channel formed by the association of pore-forming KCNQ1 with its obligatory KCNE3 β-subunit. Studies using mice show sizable cAMP-activated intestinal anion secretion in the absence of either KCNQ1 or KCNE3 suggesting that an alternative K(+) conductance must compensate for the loss of KCNQ1/KCNE3 activity...
November 16, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/29143200/structural-and-perfusion-magnetic-resonance-imaging-of-the-lung-in-cystic-fibrosis
#5
Christina Amaxopoulou, Ralph Gnannt, Kai Higashigaito, Andreas Jung, Christian J Kellenberger
BACKGROUND: Because of its absence of ionising radiation and possibility for obtaining functional information, MRI is promising for assessing lung disease in children who require repetitive imaging for long-term follow-up. OBJECTIVE: To describe MRI findings in children with cystic fibrosis and evaluate semi-quantitative dynamic contrast-enhanced lung perfusion. MATERIALS AND METHODS: We retrospectively compared lung MRI in 25 children and young adults with cystic fibrosis (median age 3...
November 15, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/29140739/cftr-genotype-and-maximal-exercise-capacity-in-cystic-fibrosis-a-cross-sectional-study
#6
Thomas Radtke, Helge Hebestreit, Sabina Gallati, Jane E Schneiderman, Julia Braun, Daniel Stevens, Erik Hj Hulzebos, Tim Takken, Steven R Boas, Don S Urquhart, Larry C Lands, Sergio Tejero, Aleksandar Sovtic, Tiffany Dwyer, Milos Petrovic, Ryan A Harris, Chantal Karila, Daniela Savi, Jakob Usemann, Meir Mei-Zahav, Elpis Hatziagorou, Felix Ratjen, Susi Kriemler
RATIONALE: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with CF may present an important determinant of aerobic exercise capacity in CF. Previous studies on the relationship between CFTR genotype and aerobic exercise capacity are scarce and contradictory. OBJECTIVES: This study was designed to explore factors influencing aerobic exercise capacity, expressed as peak oxygen consumption (VO2peak) with a specific focus on CFTR genotype in children and adults with CF...
November 15, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29140726/treatment-setting-and-outcomes-of-cystic-fibrosis-pulmonary-exacerbations
#7
Michael S Schechter, Donald R VanDevanter, David J Pasta, Sarah A Short, Wayne J Morgan, Michael W Konstan
RATIONALE: There are important gaps in our knowledge of the optimal treatment of cystic fibrosis (CF) pulmonary exacerbations. Previous observational studies comparing inpatient to outpatient treatment have suffered from methodological weaknesses, especially indication bias. OBJECTIVES: We analyzed data from the Epidemiologic Study of CF using techniques to control for indication bias to determine whether there is an advantage to inpatient treatment of CF pulmonary exacerbations...
November 15, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29138462/the-antibacterial-and-anti-inflammatory-activity-of-chicken-cathelicidin-2-combined-with-exogenous-surfactant-for-the-treatment-of-cystic-fibrosis-associated-pathogens
#8
Brandon J H Banaschewski, Brandon Baer, Christina Arsenault, Teah Jazey, Edwin J A Veldhuizen, Johan Delport, Tracey Gooyers, James F Lewis, Henk P Haagsman, Ruud A W Veldhuizen, Cory Yamashita
Cystic fibrosis (CF) is characterized by recurrent airway infections with antibiotic-resistant bacteria and chronic inflammation. Chicken cathelicin-2 (CATH-2) has been shown to exhibit antimicrobial activity against antibiotic-resistant bacteria and to reduce inflammation. In addition, exogenous pulmonary surfactant has been suggested to enhance pulmonary drug delivery. It was hypothesized that CATH-2 when combined with an exogenous surfactant delivery vehicle, bovine lipid extract surfactant (BLES), would exhibit antimicrobial activity against CF-derived bacteria and downregulate inflammation...
November 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29138047/antibacterial-effect-and-dna-delivery-using-a-combination-of-an-arsonium-containing-lipophosphoramide-with-an-n-heterocyclic-carbene-silver-complex-potential-benefits-for-cystic-fibrosis-lung-gene-therapy
#9
Angélique Mottais, Mathieu Berchel, Yann Sibiril, Véronique Laurent, Deborah Gill, Stephen Hyde, Paul-Alain Jaffrès, Tristan Montier, Tony Le Gall
Cystic Fibrosis (CF), the most common chronic genetic disorder among the Caucasian population, is a life-threatening disease mainly due to respiratory failures resulting from chronic infections and inflammation. Although research in the pharmacological field has recently made significant progress, gene therapy still remains a promising strategy to cure CF, especially because it should be applicable to any patient whatever the mutation profile. Until now, little attention has been paid to bacterial lung infections with regard to gene delivery to the airways; yet, this could greatly impact on the success of gene therapy...
November 11, 2017: International Journal of Pharmaceutics
https://www.readbyqxmd.com/read/29137964/oral-glucose-tolerance-test-and-continuous-glucose-monitoring-to-assess-diabetes-development-in-cystic-fibrosis-patients
#10
María Clemente León, Laura Bilbao Gassó, Antonio Moreno-Galdó, Ariadna Campos Martorrell, Silvia Gartner Tizzano, Diego Yeste Fernández, Antonio Carrascosa Lezcano
INTRODUCTION: Patients with cystic fibrosis (CF) undergo a slow and progressive process toward diabetes. Oral glucose tolerance test (OGTT) is recommended to diagnose impaired glucose levels in these patients. Continuous glucose monitoring (CGM) measures glucose profiles under real-life conditions. OBJECTIVE: To compare OGTT and CGM results in CF patients. METHODS: Paired OGTT and 6-day CGM profiles (146.2±9.1h/patient) were performed in 30 CF patients aged 10-18 years...
November 11, 2017: Endocrinología, diabetes y nutrición
https://www.readbyqxmd.com/read/29136345/regional-lung-function-testing-in-children-using-electrical-impedance-tomography
#11
Barbara Vogt, Sarah Löhr, Zhanqi Zhao, Christian Falkenberg, Tobias Ankermann, Norbert Weiler, Inéz Frerichs
OBJECTIVE: To evaluate regional lung function in lung-healthy children before and after exercise challenge using electrical impedance tomography (EIT). METHODS: Regional lung function was examined using EIT in 100 lung-healthy children (three age subgroups: 74-121, 122-155, 156-195 months) at baseline and 10 min after exercise. Global lung function was assessed by spirometry using Z-Scores of FEV1 , FVC, FEV1 /FVC, and FEF75 . The same lung function measures were determined in 912 EIT image pixels to enable the spatial and temporal ventilation distribution analysis...
November 14, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29136085/tgf-beta1-from-endometriomas-promotes-fibrosis-in-surrounding-ovarian-tissues-via-smad2-3-signaling
#12
Li Bing Shi, Feng Zhou, Hai Yan Zhu, Dong Huang, Xiao Ying Jin, Chao Li, Yongdong Dai, Yi Bin Pan, Song Ying Zhang
To elucidate whether the endometriotic cells of endometriomas synthesize transforming growth factor beta1 (TGF-beta1) and understand how it affects surrounding ovarian tissue. We collected biopsies of the cystic walls from 42 endometriomas and 29 mature teratomas and compared mRNA and protein expression of fibrosis-related factors between the cystic walls. Then we detected TGFB1 mRNA synthesis in endometriomas, and tested TGF-beta1 fibrotic effect in vitro. Moreover, we verified the expression of Smad2/3 Signaling components in the endometriotic cystic wall in order to understand whether TGF-beta1/Smad signaling is involved in fibrosis formation of the tissue surrounding endometriomas...
November 10, 2017: Biology of Reproduction
https://www.readbyqxmd.com/read/29135920/environmental-burkholderia-cenocepacia-strain-enhances-fitness-by-serial-passages-during-long-term-chronic-airways-infection-in-mice
#13
Alessandra Bragonzi, Moira Paroni, Luisa Pirone, Ivan Coladarci, Fiorentina Ascenzioni, Annamaria Bevivino
Burkholderia cenocepacia is an important opportunistic pathogen in cystic fibrosis (CF) patients, and has also been isolated from natural environments. In previous work, we explored the virulence and pathogenic potential of environmental B. cenocepacia strains and demonstrated that they do not differ from clinical strains in some pathogenic traits. Here, we investigated the ability of the environmental B. cenocepacia Mex1 strain, isolated from the maize rhizosphere, to persist and increase its virulence after serial passages in a mouse model of chronic infection...
November 14, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29134570/evaluation-of-tobramycin-exposure-predictions-in-three-bayesian-forecasting-programmes-compared-with-current-clinical-practice-in-children-and-adults-with-cystic-fibrosis
#14
Marc Burgard, Indy Sandaradura, Sebastiaan J van Hal, Sonya Stacey, Stefanie Hennig
BACKGROUND AND OBJECTIVES: Bayesian forecasting (BF) methods for tobramycin dose individualisation has not seen widespread clinical adoption, despite being endorsed by clinical practice guidelines. Several freeware and commercial programmes using BF methods are available to support personalised dosing. This study evaluated exposure estimates, dose recommendations, and predictive performance compared with current clinical practice. METHODS: Data from 105 patients (50 adults and 55 children) with cystic fibrosis who received intravenous tobramycin treatment and had paired concentration-time measurements were analysed using (1) log-linear regression analysis, and (2) three BF programmes: TDMx, InsightRX, and DoseMe...
November 13, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/29134356/secretin-stimulated-ultrasound-estimation-of-pancreatic-secretion-in-cystic-fibrosis-validated-by-magnetic-resonance-imaging
#15
Trond Engjom, Erling Tjora, Gaute Wathle, Friedemann Erchinger, Birger N Lærum, Odd H Gilja, Ingfrid Salvesen Haldorsen, Georg Dimcevski
OBJECTIVES: Secretin-stimulated magnetic resonance imaging (s-MRI) is the best validated radiological modality assessing pancreatic secretion. The purpose of this study was to compare volume output measures from secretin-stimulated transabdominal ultrasonography (s-US) to s-MRI for the diagnosis of exocrine pancreatic failure in cystic fibrosis (CF). METHODS: We performed transabdominal ultrasonography and MRI before and at timed intervals during 15 minutes after secretin stimulation in 21 CF patients and 13 healthy controls...
November 13, 2017: European Radiology
https://www.readbyqxmd.com/read/29134263/diminished-susceptibility-of-african-americans-to-non-tuberculous-mycobacterial-disease
#16
Jerome M Reich, Jong S Kim
The incidence of three granulomatous response diseases-sarcoidosis, tuberculosis, and non-tuberculous mycobacterial pulmonary disease-differ markedly in African-Americans versus Caucasians. In reviewing a large compendium of non-cystic-fibrosis bronchiectasis, we noted that complicating infection with non-tuberculous mycobacteria was relatively infrequent among individuals of African-American descent, confirming previous observations of their inherent resistance. Disease-specific variance among African-Americans in the efficacy of their granulomatous response suggests a nexus, a mediating, immunological mechanism...
November 13, 2017: Lung
https://www.readbyqxmd.com/read/29133559/mutations-in-gene-fusa1-as-a-novel-mechanism-of-aminoglycoside-resistance-in-clinical-strains-of-pseudomonas-aeruginosa
#17
Arnaud Bolard, Patrick Plesiat, Katy Jeannot
Resistance of clinical strains of Pseudomonas aeruginosa to aminoglycosides can result from production of transferable aminoglycoside-modifying enzymes, of 16S rRNA methylases, and/or mutational derepression of intrinsic multidrug efflux pump MexXY(OprM). The present study reports on characterization of a new type of mutants 4- to 8-fold more resistant to 2-deoxystreptamine derivatives (e.g., gentamicin, amikacin, tobramycin), as compared with wild-type strain PAO1. The genetic alterations of three in vitro mutants were mapped on fusA1, and found to result in single amino acid substitutions in domains II, III and V of elongation factor G (EF-G1A), a key component of translational machinery...
November 13, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/29132736/clinical-effects-of-probiotics-in-cystic-fibrosis-patients-a-systematic-review
#18
Stephanie Van Biervliet, Dimitri Declercq, Shawn Somerset
Cystic fibrosis (CF) is characterised by a build-up of thick, intransient mucus linings of the digestive and respiratory mucosa, which disrupts digestive system functioning and microbiota composition. In view of the potential for probiotics to enhance microbiota composition in other contexts, this study investigated the current evidence for probiotics as an adjunct to usual therapy for CF. Electronic clinical databases were interrogated for human randomised, controlled, intervention trials (1985-2015) testing the effects of probiotics on clinical endpoints in CF were reviewed...
April 2017: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/29132121/the-therapeutic-potential-of-cftr-modulators-for-copd-and-other-airway-diseases
#19
REVIEW
George M Solomon, Lianwu Fu, Steven M Rowe, James F Collawn
Airways diseases, especially chronic obstructive pulmonary disease (COPD) and asthma, are common causes of morbidity and mortality worldwide. There is an ongoing unmet need for novel and effective therapies. There is an established pathophysiological link and phenotypic similarity between the chronic bronchitis phenotype of COPD and cystic fibrosis (CF). New evidence suggests that CFTR dysfunction may play a role in other common airways diseases such as COPD, non-atopic asthma and non-CF bronchiectasis. Newly approved and investigational drugs that target both mutant and wildtype CFTR channels have provided a new treatment opportunity addressing the mucus defect in pulmonary diseases that share the same pathophysiology with CF...
November 9, 2017: Current Opinion in Pharmacology
https://www.readbyqxmd.com/read/29131862/optimized-approach-for-the-identification-of-highly-efficient-correctors-of-nonsense-mutations-in-human-diseases
#20
Hana Benhabiles, Sara Gonzalez-Hilarion, Séverine Amand, Christine Bailly, Anne Prévotat, Philippe Reix, Dominique Hubert, Eric Adriaenssens, Sylvie Rebuffat, David Tulasne, Fabrice Lejeune
About 10% of patients with a genetic disease carry a nonsense mutation causing their pathology. A strategy for correcting nonsense mutations is premature termination codon (PTC) readthrough, i.e. incorporation of an amino acid at the PTC position during translation. PTC-readthrough-activating molecules appear as promising therapeutic tools for these patients. Unfortunately, the molecules shown to induce PTC readthrough show low efficacy, probably because the mRNAs carrying a nonsense mutation are scarce, as they are also substrates of the quality control mechanism called nonsense-mediated mRNA decay (NMD)...
2017: PloS One
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