keyword
MENU ▼
Read by QxMD icon Read
search

Cystic fibrosis

keyword
https://www.readbyqxmd.com/read/29779042/combining-theoretical-and-experimental-data-to-decipher-cftr-3d-structures-and-functions
#1
Brice Hoffmann, Ahmad Elbahnsi, Pierre Lehn, Jean-Luc Décout, Fabio Pietrucci, Jean-Paul Mornon, Isabelle Callebaut
Cryo-electron microscopy (cryo-EM) has recently provided invaluable experimental data about the full-length cystic fibrosis transmembrane conductance regulator (CFTR) 3D structure. However, this experimental information deals with inactive states of the channel, either in an apo, quiescent conformation, in which nucleotide-binding domains (NBDs) are widely separated or in an ATP-bound, yet closed conformation. Here, we show that 3D structure models of the open and closed forms of the channel, now further supported by metadynamics simulations and by comparison with the cryo-EM data, could be used to gain some insights into critical features of the conformational transition toward active CFTR forms...
May 19, 2018: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/29779024/a-randomized-clinical-trial-of-metformin-to-treat-autosomal-dominant-polycystic-kidney-disease
#2
Stephen L Seliger, Kaleab Z Abebe, Kenneth R Hallows, Dana C Miskulin, Ronald D Perrone, Terry Watnick, Kyongtae Tae Bae
BACKGROUND: Metformin inhibits cyclic AMP generation and activates AMP-activated protein kinase (AMPK), which inhibits the cystic fibrosis transmembrane conductance regulator and Mammalian Target of Rapamycin pathways. Together these effects may reduce cyst growth in autosomal dominant polycystic kidney disease (ADPKD). METHODS: A phase II, double-blinded randomized placebo-controlled trial of 26 months duration. Participants will include nondiabetic adults (n = 96) aged 18-60 years, with an estimated glomerular filtration rate (eGFR) ≥50 mL/min/1...
May 18, 2018: American Journal of Nephrology
https://www.readbyqxmd.com/read/29778403/the-cf-sputum-induction-trial-cf-spit-to-assess-lower-airway-bacterial-sampling-in-young-children-with-cystic-fibrosis-a-prospective-internally-controlled-interventional-trial
#3
Katherine Ronchetti, Jo-Dee Tame, Christopher Paisey, Lena P Thia, Iolo Doull, Robin Howe, Eshwar Mahenthiralingam, Julian T Forton
BACKGROUND: Pathogen surveillance is challenging but crucial in children with cystic fibrosis-who are often non-productive of sputum even if actively coughing-because infection and lung disease begin early in life. The role of sputum induction as a diagnostic tool for infection has not previously been systematically addressed in young children with cystic fibrosis. We aimed to assess the pathogen yield from sputum induction compared with that from cough swab and single-lobe, two-lobe, and six-lobe bronchoalveolar lavage...
May 16, 2018: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/29778402/cough-swabs-less-useful-but-induced-sputum-very-useful-in-symptomatic-older-children-with-cystic-fibrosis
#4
André Schultz, Daan Caudri
No abstract text is available yet for this article.
May 16, 2018: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/29778213/nitric-oxide-an-old-molecule-with-noble-functions-in-pseudomonas-aeruginosa-biology
#5
Masanori Toyofuku, Sang-Sun Yoon
Pseudomonas aeruginosa, a Gram-negative bacterium, is characterized by its versatility that enables persistent survival under adverse conditions. It can grow on diverse energy sources and readily acquire resistance to antimicrobial agents. As an opportunistic human pathogen, it also causes chronic infections inside the anaerobic mucus airways of cystic fibrosis patients. As a strict respirer, P. aeruginosa can grow by anaerobic nitrate ( [Formula: see text] ) respiration. Nitric oxide (NO) produced as an intermediate during anaerobic respiration exerts many important effects on the biological characteristics of P...
2018: Advances in Microbial Physiology
https://www.readbyqxmd.com/read/29777836/phage-therapy-against-achromobacter-xylosoxidans-lung-infection-in-a-patient-with-cystic-fibrosis-a-case-report
#6
N Hoyle, P Zhvaniya, N Balarjishvili, D Bolkvadze, L Nadareishvili, D Nizharadze, J Wittmann, C Rohde, M Kutateladze
Respiratory infections can lead to serious complications in CF patients, especially when infected with antibiotic resistant bacteria. Alternative treatments for these infections are being sought out to help address this problem. We present a clinical case of a cystic fibrosis (CF) patient, with multi-drug resistant (MDR) Achromobacter xylosoxidans chronic lung infection who was successfully managed with bacteriophage therapy.
May 16, 2018: Research in Microbiology
https://www.readbyqxmd.com/read/29777470/prevalence-of-depression-among-caregivers-of-indian-children-with-cystic-fibrosis
#7
Javeed Iqbal Bhat, Wasim Ahmad Wani, Bashir A Charoo, Syed Wajid Ali, Qazi Iqbal Ahmad, Ambreen Ali Ahangar
OBJECTIVE: To study the prevalence of depression among caregivers of children with cystic fibrosis and its impact on the health and well being of these children. METHODS: This cross-sectional study was conducted in a tertiary care hospital from September 2015 through August 2016. Forty one parents of children receiving treatment at the Cystic fibrosis (CF) clinic were approached to be part of the study. Six families declined the request resulting in 85% recruitment rate...
May 19, 2018: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/29775685/phenotypic-and-genetic-resistance-traits-in-pseudomonas-aeruginosa-strains-infecting-cystic-fibrosis-patients-a-french-cohort-study
#8
Nathalie Courtois, Yvan Caspar, Max Maurin
Pseudomonas aeruginosa is responsible for chronic respiratory tract colonisation and acute exacerbations in cystic fibrosis (CF) patients. This Gram-negative bacterium often develops multidrug resistance, which represents a therapeutic challenge. Our objective was to characterize the phenotypic and genetic β-lactam resistance traits of P. aeruginosa strains isolated from CF patients followed at Grenoble Alpes university hospital. The susceptibility to β-lactam compounds of 123 P. aeruginosa strains collected from the lower respiratory tract of 45 CF patients, between 2010 and 2014, was evaluated...
May 15, 2018: International Journal of Antimicrobial Agents
https://www.readbyqxmd.com/read/29773689/risk-factors-for-lung-disease-progression-in-children-with-cystic-fibrosis
#9
Marieke van Horck, Kim van de Kant, Bjorn Winkens, Geertjan Wesseling, Vincent Gulmans, Han Hendriks, Chris van der Grinten, Quirijn Jӧbsis, Edward Dompeling
To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009-2014). Lung disease progression was expressed as a decline in lung function (FEV1 %pred) and the pulmonary exacerbation rate (PEx). Potential risk factors at baseline included: sex, age, best FEV1%pred, best forced vital capacity (FVC)%pred, genotype, BMI z-score, pancreatic insufficiency, medication use (proton pump inhibitors [PPI], prophylactic antibiotics, and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis, and colonisation with Pseudomonas aeruginosa The data of 545 children were analysed...
May 17, 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29773609/cured-bronchi-extending-the-use-of-nebulised-hypertonic-saline-outside-of-cystic-fibrosis
#10
EDITORIAL
Clémence Martin, Lucile Regard, Pierre-Régis Burgel
No abstract text is available yet for this article.
May 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29773589/ivacaftor-for-cystic-fibrosis
#11
Peter J Barry, Anna L Donaldson, Andrew M Jones
No abstract text is available yet for this article.
May 17, 2018: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/29773197/efficient-zinc-uptake-is-critical-for-the-ability-of-pseudomonas-aeruginosa-to-express-virulence-traits-and-colonize-the-human-lung
#12
Maria Chiara Mastropasqua, Iain Lamont, Lois W Martin, David W Reid, Melania D'Orazio, Andrea Battistoni
We have recently shown that Pseudomonas aeruginosa, an opportunistic pathogen that chronically infects the lungs of patients with cystic fibrosis (CF) and other forms of lung disease, is extremely efficient in recruiting zinc from the environment and that this capability is required for its ability to cause acute lung infections in mice. To verify that P. aeruginosa faces zinc shortage when colonizing the lungs of human patients, we analyzed the expression of three genes that are highly induced under conditions of zinc deficiency (zrmA, dksA2 and rpmE2), in bacteria in the sputum of patients with inflammatory lung disease...
July 2018: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/29772761/high-concentrations-of-sodium-chloride-improve-microbicidal-activity-of-ibuprofen-against-common-cystic-fibrosis-pathogens
#13
Adrián J Muñoz, Roxana V Alasino, Ariel G Garro, Valeria Heredia, Néstor H García, David C Cremonezzi, Dante M Beltramo
Ibuprofen (IBU-H), a widely used anti-inflammatory, also shows a marked antimicrobial effect against several bacterial species, including those involved in cystic fibrosis such as Pseudomona aeruginosa , methicillin resistant Staphylococcus aureus and Burkholderia cepacia complex. Additionally, our results show significant synergy between water soluble Na-ibuprofen (IBU-Na) and ionic strength. Salt concentrations above 0.5 M modify the zeta potential promoting the action of Na-IBU; thus, with 1 M sodium chloride, IBU-Na is ten times more efficient than in the absence of ionic strength, and the minimum effective contact time is reduced from hours to minutes...
May 17, 2018: Pharmaceuticals
https://www.readbyqxmd.com/read/29772528/peptide-inhibitors-of-chloride-channels-for-treating-secretory-diarrhea
#14
Jitu Ma, Xueqin Ding, Yulong Yin, Pengfei Huang
Morbidity and mortality associated with diarrheal diseases remain significant burdens on global health. In the developing world, the major sources of secretory diarrhea are infectious, including those caused by bacteria such as enterotoxic Escherichia coli , and viruses such as rotavirus. In many cases of secretory diarrhea, activation of pathways for cyclic nucleotides and/or Ca2+ signaling in the apical membrane of enterocytes increases the conductance of Cl- channels at the enterocyte lumen-facing membrane...
June 1, 2018: Frontiers in Bioscience (Landmark Edition)
https://www.readbyqxmd.com/read/29771095/-a-progress-of-macrolides-therapy-for-chronic-rhinosinusitis
#15
REVIEW
S Shen, C S Wang
Macrolides are a type of antibiotics with macrocyclic lactone ring, which have been commonly used in the treatment of diffuse panbronchiolitis, chronic obstructive pulmonary disease, bronchial asthma, cystic fibrosis and other diseases. Macrolides not only have effect on antibacterial, but also effect on anti-inflammatory and immunoregulation. Chronic rhinosinusitis (CRS) is a common disease entity of infectious and inflammatory diseases that involved in nasal cavity and nasal sinuses, with various clinical phenotypes...
May 2018: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/29770214/case-report-haemolytic-anaemia-with-ceftazidime-use-in-a-patient-with-cystic-fibrosis
#16
Jun Yong, Freddy Frost, Dilip Nazareth, Martin Walshaw
Drug-induced Immune Haemolytic Anaemia (DIIHA) is a rare but serious complication of cephalosporin use. Ceftazidime is recognized to be a rare cause of DIIHA. We report and discuss a case of DIIHA in a person with cystic fibrosis who developed severe haemolytic anaemia following use of ceftazidime in the management of an acute pseudomonal pulmonary exacerbation.
2018: F1000Research
https://www.readbyqxmd.com/read/29769279/microbial-interactions-in-the-cystic-fibrosis-airway
#17
Ann M Granchelli, Frederick R Adler, Ruth H Keogh, Christiana Kartsonaki, David R Cox, Theodore G Liou
Interactions in the airway ecology of cystic fibrosis may alter organism persistence and clinical outcomes. Better understanding of such interactions could guide clinical decisions. We fitted logistic regression models using generalized estimating equations to longitudinal two-year patient cohorts in the Cystic Fibrosis Foundation Patient Registry, 2003-2011 to study associations between airway organisms present in each calendar year and their presence in the subsequent year. Models were adjusted for clinical characteristics and multiple observations per patient and tested for sensitivity to cystic fibrosis-specific treatments...
May 16, 2018: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/29769187/intraoperative-intraluminal-injection-of-n-acetylcysteine-allowing-treatment-of-distal-intestinal-obstruction-syndrome-without-the-need-for-enterotomy
#18
Nicholas Js Chilvers, James Wheeler
We describe a case of an 18-year-old man who suffers from cystic fibrosis and developed distal intestinal obstruction syndrome while being treated as an inpatient. Following failed medical management, we proceeded to laparotomy where the small bowel was decompressed with retrograde milking into the stomach, leaving a section of impacted stool in the distal ileum. N-acetylcysteine was injected into the bowel lumen proximal to the obstruction. This resulted in dissolution of the stool without the need for enterotomy and is, to our knowledge, the first successful example of this technique in the literature...
May 16, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29769033/case-report-on-pathogenetic-link-between-gluten-and-iga-nephropathy
#19
Stefano Costa, Giovanni Currò, Salvatore Pellegrino, Maria Cristina Lucanto, Giovanni Tuccari, Antonio Ieni, Giuseppina Visalli, Giuseppe Magazzù, Domenico Santoro
BACKGROUND: A relationship between IgA nephropathy (IgAN) and celiac disease (CD) has been reported. We show the pathogenetic link for the first time. CASE PRESENTATION: A 39-year-old man with cystic fibrosis (CF) and CF-related diabetes started to present gross hematuria, back pain and headache. At admission, laboratory analysis showed increase in serum creatinine of 1.5 mg/dl, together with hematuria and mild proteinuria (1 g/24 h). He underwent a renal biopsy to investigate the cause of hematuria and renal failure...
May 16, 2018: BMC Gastroenterology
https://www.readbyqxmd.com/read/29768684/practical-approach-to-the-gastrointestinal-manifestations-of-cystic-fibrosis
#20
REVIEW
Rishi Bolia, Chee Y Ooi, Peter Lewindon, Jonathan Bishop, Sarath Ranganathan, Jo Harrison, Kristyn Ford, Natalie van der Haak, Mark R Oliver
Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner...
May 16, 2018: Journal of Paediatrics and Child Health
keyword
keyword
109435
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"