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Cystic Fibrosis

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https://www.readbyqxmd.com/read/29457700/impact-of-home-spirometry-on-medication-adherence-among-adolescents-with-cystic-fibrosis
#1
Aarti Shakkottai, Niko Kaciroti, Lauren Kasmikha, Samya Z Nasr
OBJECTIVE: Medication adherence among adolescents with cystic fibrosis (CF) is often suboptimal and this has significant impact on their health and quality of life. The purpose of the study was to evaluate the impact of frequent home pulmonary function (PFT) monitoring on medication adherence among adolescents with CF. HYPOTHESIS: We hypothesized that weekly home PFT monitoring will improve adherence while not significantly adding to the treatment burden. METHODS: Individuals aged 12-21 years with CF were provided a spirometer to measure PFTs weekly for 1 year...
February 19, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29457316/assessment-of-pulmonary-structure-function-relationships-in-young-children-and-adolescents-with-cystic-fibrosis-by-multivolume-proton-mri-and-ct
#2
Francesca Pennati, David J Roach, John P Clancy, Alan S Brody, Robert J Fleck, Andrea Aliverti, Jason C Woods
BACKGROUND: Lung disease is the most frequent cause of morbidity and mortality in patients with cystic fibrosis (CF), and there is a shortage of sensitive biomarkers able to regionally monitor disease progression and to assess early responses to therapy. PURPOSE: To determine the feasibility of noncontrast-enhanced multivolume MRI, which assesses intensity changes between expiratory and inspiratory breath-hold images, to detect and quantify regional ventilation abnormalities in CF lung disease, with a focus on the structure-function relationship...
February 19, 2018: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29456196/improvement-in-exophiala-dermatitidis-airway-persistence-and-respiratory-decline-in-response-to-interferon-gamma-therapy-in-a-patient-with-cystic-fibrosis
#3
Christopher P Eades, Darius P H Armstrong-James, Jimstan Periselneris, Andrew Jones, Nicholas Simmonds, Peter Kelleher, Anand Shah
No abstract text is available yet for this article.
February 15, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29456195/mast-cell-tryptase-changes-with-aspergillus-fumigatus-host-crosstalk-in-cystic-fibrosis-patients
#4
Carine Gomez, Ania Carsin, Marion Gouitaa, Martine Reynaud-Gaubert, Jean-Christophe Dubus, Jean-Louis Mège, Stéphane Ranque, Joana Vitte
Pulmonary and systemic antifungal immunity influences quality of life and survival of people with cystic fibrosis. Aspergillus fumigatus (Af) induces specific IgG and IgE. Mast cells respond to IgE, IgG and direct interactions with Af. Mast cells are the source of the protease tryptase. We aimed at evaluating serum baseline tryptase as a potential biomarker of the Af-host interaction in cystic fibrosis patients. Serum baseline tryptase, IgE and IgG directed to Af extract and Af molecular allergens were measured in 76 cystic fibrosis patients...
February 15, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29454860/velocity-landscape-correlation-resolves-multiple-flowing-protein-populations-from-fluorescence-image-time-series
#5
Elvis Pandžić, Asmahan Abu-Arish, Renee W Whan, John W Hanrahan, Paul W Wiseman
Molecular, vesicular and organellar flows are of fundamental importance for the delivery of nutrients and essential components used in cellular functions such as motility and division. With recent advances in fluorescence/super-resolution microscopy modalities we can resolve the movements of these objects at higher spatio-temporal resolutions and with better sensitivity. Previously, spatio-temporal image correlation spectroscopy has been applied to map molecular flows by correlation analysis of fluorescence fluctuations in image series...
February 15, 2018: Methods: a Companion to Methods in Enzymology
https://www.readbyqxmd.com/read/29451946/first-experience-in-switzerland-in-phe508del-homozygous-cystic-fibrosis-patients-with-end-stage-pulmonary-disease-enrolled-in-a-lumacaftor-ivacaftor-therapy-trial-preliminary-results
#6
Christian Murer, Lars Christian Huber, Thomas Kurowski, Astrid Hirt, Cécile A Robinson, Urs Bürgi, Christian Benden
AIMS OF THE STUDY: Cystic fibrosis is the most common genetic disorder in Caucasians. The combination of the cystic fibrosis transmembrane conductance regulator (CFTR) corrector lumacaftor / potentiator ivacaftor (LUM/IVA) has been shown to increase forced expiratory volume in 1 second (FEV1) moderately, but predominantly reduce acute exacerbation rate (AER) in Phe508del homozygous cystic fibrosis patients; however, patients with FEV1 <40% predicted were excluded from studies. We used LUM/IVA on a "compassionate use" basis in cystic fibrosis patients with end-stage pulmonary disease...
February 16, 2018: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29449653/cftr-mutation-enhances-dishevelled-degradation-and-results-in-impairment-of-wnt-dependent-hematopoiesis
#7
Huaqin Sun, Yan Wang, Jieting Zhang, Yan Chen, Yanyan Liu, Ziyuan Lin, Mingfeng Liu, Kai Sheng, Huijuan Liao, Kam Sze Tsang, Xiaohu Zhang, Xiaohua Jiang, Wenming Xu, Meng Mao, Hsiao Chang Chan
Mutations of cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis (CF) with a multitude of clinical manifestations. Some CF patients develop clinically significant anemia, suggesting that CFTR may regulate hematopoiesis. Here, we report that cftr mutant zebrafish model exhibits primitive and definitive hematopoietic defects with impaired Wnt signaling. Cftr is found to interact, via its PDZ-binding domain (PDZBD), with Dishevelled (Dvl), a key component of Wnt signaling required for hematopoietic progenitor specification, thus protecting Dvl from Dapper1 (Dpr1)-induced lysosomal degradation...
February 15, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29449440/lung-clearance-index-to-monitor-treatment-response-in-pulmonary-exacerbations-in-preschool-children-with-cystic-fibrosis
#8
Jonathan H Rayment, Sanja Stanojevic, Stephanie D Davis, George Retsch-Bogart, Felix Ratjen
BACKGROUND: Antibiotic treatment for pulmonary symptoms in preschool children with cystic fibrosis (CF) varies among clinicians. The lung clearance index (LCI) is sensitive to early CF lung disease, but its utility to monitor pulmonary exacerbations in young children has not been assessed. OBJECTIVE: We aim to (1) understand how LCI changes during lower respiratory tract symptoms relative to a recent clinically stable measurement, (2) determine whether LCI can identify antibiotic treatment response and (3) compare LCI changes to changes in spirometric indices...
February 15, 2018: Thorax
https://www.readbyqxmd.com/read/29449399/genome-sequences-of-12-pseudomonas-lundensis-strains-isolated-from-the-lungs-of-humans
#9
Brittan S Scales, John R Erb-Downward, Nicole R Falkowski, John J LiPuma, Gary B Huffnagle
We report here the first complete genome sequence of a human Pseudomonas lundensis isolate, strain AU1044, and the draft genomes of 11 other clinical P. lundensis strains, isolated from the lungs of cystic fibrosis patients. The genome of strain AU1044 is 4.81 Mb and encodes seven 16S rRNAs.
February 15, 2018: Genome Announcements
https://www.readbyqxmd.com/read/29449345/antimicrobial-activity-of-a-novel-bioengineered-honey-against-non-typeable-haemophilus-influenzae-biofilms-an-in-vitro-study
#10
Rachel S Newby, Matthew Dryden, Raymond N Allan, Rami J Salib
The opportunistic pathogen non-typeable Haemophilus influenzae (NTHi) plays an important role in many chronic respiratory diseases including otitis media, chronic rhinosinusitis, cystic fibrosis and chronic obstructive pulmonary disease. Biofilm formation has been implicated in NTHi colonisation, persistence of infection and recalcitrance towards antimicrobials. There is therefore a pressing need for the development of novel treatment strategies that are effective against NTHi biofilm-associated diseases. SurgihoneyRO is a honey-based product that has been bioengineered to enable the slow release of H 2 O 2 , a reactive oxygen species to which H...
February 15, 2018: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29447028/elastase-as-a-potential-biomarker-for-radiation-induced-gut-wall-injury-of-the-distal-bowel-in-an-experimental-mouse-model
#11
Fei Sjöberg, Dilip Kumar Malipatlolla, Piyush Patel, Ulrica Wilderäng, Marie Kalm, Gunnar Steineck, Cecilia Bull
BACKGROUND AND PURPOSE: Traditionally, elastase has been used to study exocrine activity of the pancreas in patients with chronic pancreatitis and cystic fibrosis, and calprotectin as a marker for gut-wall inflammation in patients with inflammatory bowel disease. The aim of the study was to find out whether elastase and calprotectin could be used as inflammatory markers for radiation-induced gut wall injury of the distal bowel. MATERIAL AND METHODS: Adult male mice were exposed to two, three, or four fractions of 6 Gy or 8 Gy irradiation to the sigmoid and rectum of the large bowel, using a linear accelerator...
February 15, 2018: Acta Oncologica
https://www.readbyqxmd.com/read/29446825/phytomedicines-medicines-derived-from-plants-for-sickle-cell-disease
#12
REVIEW
Oluseyi Oniyangi, Damian H Cohall
BACKGROUND: Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. Laboratory research and limited clinical trials have suggested positive effects of phytomedicines both in vivo and in vitro...
February 15, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29446568/making-a-cautious-case-for-expanding-reproductive-genetic-carrier-screens-australian-researchers-report-success-and-caveats-with-a-simultaneous-panel-of-cystic-fibrosis-fragile-x-syndrome-and-spinal-muscular-atrophy
#13
https://www.readbyqxmd.com/read/29444761/chronic-infection-sustained-by-a-pseudomonas-aeruginosa-high-risk-clone-producing-the-vim-1-metallo-%C3%AE-lactamase-in-a-cystic-fibrosis-patient-after-lung-transplantation
#14
Simona Pollini, Claudia Mugnaioli, Daniela Dolce, Silvia Campana, Anna Silvia Neri, Giovanni Taccetti, Gian Maria Rossolini
BACKGROUND: The significance of chronic lung infection by multidrug-resistant (MDR) pathogens in Cystic Fibrosis (CF) transplanted patients remains controversial, and the available information is overall limited. Here we describe the case of a chronic infection, sustained by a metallo-β-lactamase (MBL)-producing P. aeruginosa strain, in a CF patient following lung transplantation. METHODS: Twelve P. aeruginosa isolates collected from a CF patient over a 15-years follow-up period after lung transplantation were analysed for their antibiotic susceptibility profile, MBL production and clonal relatedness...
February 12, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29444760/risk-factors-for-persistent-aspergillus-respiratory-isolation-in-cystic-fibrosis
#15
Gina Hong, Kevin J Psoter, Mark T Jennings, Christian A Merlo, Michael P Boyle, Denis Hadjiliadis, Steven M Kawut, Noah Lechtzin
BACKGROUND: Aspergillus species are increasingly detected in the respiratory tracts of individuals with cystic fibrosis (CF), and chronic Aspergillus fumigatus is associated with more frequent hospitalizations for pulmonary exacerbations. However, patient and clinical factors that may contribute to the acquisition of persistent Aspergillus infection have yet to be identified. The objective of this study was to identify risk factors for development of Aspergillus respiratory isolation in CF...
February 12, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29444656/implementation-of-a-successful-eradication-protocol-for-burkholderia-cepacia-complex-in-cystic-fibrosis-patients
#16
Bryan A Garcia, Jacque L Carden, Dana L Goodwin, Tim A Smith, Amit Gaggar, Kevin Leon, Veena B Antony, Steven M Rowe, George M Solomon
BACKGROUND: Infection with Burkholderia cepacia complex (Bcc) results in a heterogeneous clinical course ranging from asymptomatic colonization of the airways to fulminant respiratory failure in patients with cystic fibrosis (CF). Early eradication of Pseudomonas aeruginosa improves clinical outcomes. The efficacy and clinical outcomes following implementation of an eradication protocol for Bcc are less well understood. METHODS: We developed and implemented a single center Bcc eradication protocol that included an intensive combination of intravenous, inhaled, and oral antibiotic therapies based on in vitro sensitivities...
February 14, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29444099/airway-ciliary-dysfunction-and-respiratory-symptoms-in-patients-with-transposition-of-the-great-arteries
#17
Maliha Zahid, Abha Bais, Xin Tian, William Devine, Dong Ming Lee, Cyrus Yau, Daniel Sonnenberg, Lee Beerman, Omar Khalifa, Cecilia W Lo
BACKGROUND: Our prior work on congenital heart disease (CHD) with heterotaxy, a birth defect involving randomized left-right patterning, has shown an association of a high prevalence of airway ciliary dysfunction (CD; 18/43 or 42%) with increased respiratory symptoms. Furthermore, heterotaxy patients with ciliary dysfunction were shown to have more postsurgical pulmonary morbidities. These findings are likely a reflection of the common role of motile cilia in both airway clearance and left-right patterning...
2018: PloS One
https://www.readbyqxmd.com/read/29443734/prognostic-significance-of-pulmonary-hypertension-in-patients-with-cystic-fibrosis-a-systematic-review-and-meta-analysis
#18
Diandian Li, Bo Wang, Hao Wang, Qun Liu
BACKGROUND AND OBJECTIVE: Pulmonary hypertension (PH) is frequently found in advanced parenchymal lung diseases like cystic fibrosis (CF), but the role played by PH in the clinical outcome of CF patients remains unclear. The aim of this study is to determine the influence of PH on survival in the CF population by meta-analysis. METHODS: Publications addressing the associations between PH and overall survival (OS) or other clinical characteristics in CF patients were selected from electronic databases...
February 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29442501/exploiting-the-s4-s5-specificity-of-human-neutrophil-proteinase-3-to-improve-the-potency-of-peptidyl-di-chlorophenyl-phosphonate-ester-inhibitors-a-kinetic-and-molecular-modeling-analysis
#19
Carla Guarino, Natalia Gruba, Renata Grzywa, Edyta Dyguda-Kazimierowicz, Yveline Hamon, Monika Legowska, Marcin Skoreński, Sandrine Dallet-Choisy, Sylvain Marchand-Adam, Christine Kellenberger, Dieter E Jenne, Marcin Sieńczyk, Adam Lesner, Francis Gauthier, Brice Korkmaz
The neutrophilic serine protease proteinase 3 (PR3) is involved in inflammation and immune response and thus appears as a therapeutic target for a variety of infectious and inflammatory diseases. Here we combined kinetic and molecular docking studies to increase the potency of peptidyl-diphenyl phosphonate PR3 inhibitors. Occupancy of the S1 subsite of PR3 by a nVal residue and of the S4-S5 subsites by a biotinylated Val residue as obtained in biotin-VYDnVP(O-C6H4-4-Cl)2 enhanced the second order inhibition constant kobs/[I] towards PR3 by more than ten times (kobs/[I] = 73...
February 14, 2018: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/29438119/liver-disease-in-patients-with-cystic-fibrosis
#20
Natasha Kamal, Pallavi Surana, Christopher Koh
PURPOSE OF REVIEW: The aim of this study was to provide an overview of the current understanding of the pathophysiology, diagnosis and management of cystic fibrosis-liver disease (CFLD). RECENT FINDINGS: CFLD has a variety of manifestations. Previously, it was thought that patients progressed from mild cholestatic disease to cirrhosis to decompensated cirrhosis with portal hypertension. Newer evidence suggests that some patients may develop cirrhosis while others develop noncirrhotic portal hypertension...
February 12, 2018: Current Opinion in Gastroenterology
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