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"Cystic fibrosis"

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https://www.readbyqxmd.com/read/28917611/end-of-life-practice-patterns-at-u-s-adult-cystic-fibrosis-care-centers-a-national-retrospective-chart-review
#1
Elaine Chen, Karen Homa, Jessica Goggin, Kathryn A Sabadosa, Sarah Hempstead, Bruce C Marshall, Albert Faro, Elisabeth P Dellon
BACKGROUND: There are many challenges to providing end-of-life care (EOLC) to people with cystic fibrosis (CF). METHODS: Chart abstraction was used to examine EOLC in adults with CF who died between 2011 and 2013. RESULTS: We reviewed 248 deaths from 71 CF care centers. Median age at death was 29years (range 18-73). While median FEV1 was in the severe lung disease category (FEV1<40%), 38% had mild or moderate lung disease in the year preceding death...
September 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28916704/delayed-neutrophil-apoptosis-enhances-net-formation-in-cystic-fibrosis
#2
Robert D Gray, Gareth Hardisty, Kate H Regan, Maeve Smith, Calum T Robb, Rodger Duffin, Annie Mackellar, Jennifer M Felton, Lily Paemka, Brian N McCullagh, Christopher D Lucas, David A Dorward, Edward F McKone, Gordon Cooke, Seamas C Donnelly, Pradeep K Singh, David A Stoltz, Christopher Haslett, Paul B McCray, Moira K B Whyte, Adriano G Rossi, Donald J Davidson
BACKGROUND: Cystic fibrosis (CF) lung disease is defined by large numbers of neutrophils and associated damaging products in the airway. Delayed neutrophil apoptosis is described in CF although it is unclear whether this is a primary neutrophil defect or a response to chronic inflammation. Increased levels of neutrophil extracellular traps (NETs) have been measured in CF and we aimed to investigate the causal relationship between these phenomena and their potential to serve as a driver of inflammation...
September 15, 2017: Thorax
https://www.readbyqxmd.com/read/28916430/therapeutic-approaches-to-cftr-dysfunction-from-discovery-to-drug-development
#3
Hongyu Li, Emanuela Pesce, David N Sheppard, Ashvani K Singh, Nicoletta Pedemonte
Cystic fibrosis (CF) mutations have complex effects on the cystic fibrosis transmembrane conductance regulator (CFTR) protein. They disrupt its processing to and stability at the plasma membrane and function as an ATP-gated Cl(-) channel. Here, we review therapeutic strategies to overcome defective CFTR processing and stability. Because CF mutations have multiple impacts on the assembly of CFTR protein, combination therapy with several pharmacological chaperones is likely to be required to rescue mutant CFTR expression at the plasma membrane...
September 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28916014/integrated-behavioral-health-care-in-pediatric-subspecialty-clinics
#4
REVIEW
Chase Samsel, Monique Ribeiro, Patricia Ibeziako, David R DeMaso
Comorbid behavioral and physical health conditions are accompanied by troubling symptom burden, functional impairment, and treatment complexity. Pediatric subspecialty care clinics offer an opportunity for the implementation of integrated behavioral health (BH) care models that promote resiliency. This article reviews integrated BH care in oncology, palliative care, pain, neuropsychiatry, cystic fibrosis, and transplantation. Examples include integrated care mandates, standards of care, research, and quality improvement by child and adolescent psychiatrists (CAPs) and allied BH clinicians...
October 2017: Child and Adolescent Psychiatric Clinics of North America
https://www.readbyqxmd.com/read/28914424/new-perspectives-in-nanotherapeutics-for-chronic-respiratory-diseases
#5
Adriana Lopes da Silva, Fernanda Ferreira Cruz, Patricia Rieken Macedo Roccco, Marcelo Marcos Morales
According to the World Health Organization (WHO), hundreds of millions of people of all ages and in all countries suffer from chronic respiratory diseases, with particular negative consequences such as poor health-related quality of life, impaired work productivity, and limitations in the activities of daily living. Chronic obstructive pulmonary disease, asthma, occupational lung diseases (such as silicosis), cystic fibrosis, and pulmonary arterial hypertension are the most common of these diseases, and none of them are curable with current therapies...
September 15, 2017: Biophysical Reviews
https://www.readbyqxmd.com/read/28914348/immunogenicity-and-antimicrobial-effectiveness-of-pseudomonas-aeruginosa-specific-bacteriophage-in-a-human-lung-in-vitro-model
#6
Joseph R Shiley, Kristen K Comfort, Jayne B Robinson
The rise of antibiotic resistant bacteria is posing a serious threat to human health. For example, resistant strains of Pseudomonas aeruginosa have resulted in untreatable and potentially lethal infections in both cystic fibrosis and immunocompromised patients. Due to the growing need for alternative treatment options, bacteriophage, or phage, therapy is gaining considerable attention. While previous studies have demonstrated the effectiveness of phage in combating persistent bacterial infections, there is currently a lack of knowledge regarding the host immunological response following phage exposure...
September 15, 2017: Applied Microbiology and Biotechnology
https://www.readbyqxmd.com/read/28912311/draft-genome-sequence-of-the-human-pathogenic-fungus-scedosporium-boydii
#7
Ludovic Duvaux, Jason Shiller, Patrick Vandeputte, Thomas Dugé de Bernonville, Christopher Thornton, Nicolas Papon, Bruno Le Cam, Jean-Philippe Bouchara, Amandine Gastebois
The opportunistic fungal pathogen Scedosporium boydii is the most common Scedosporium species in French patients with cystic fibrosis. Here we present the first genome report for S. boydii, providing a resource which may enable the elucidation of the pathogenic mechanisms in this species.
September 14, 2017: Genome Announcements
https://www.readbyqxmd.com/read/28911954/erratum-of-hearing-thresholds-at-high-frequency-in-patients-with-cystic-fibrosis-a-systematic-review
#8
Debora T M Caumo, Lúcia B Geyer, Adriane R Teixeira, Sérgio S M Barreto
No abstract text is available yet for this article.
September 2017: Brazilian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/28910514/pharmacokinetics-of-single-dose-ceftaroline-fosamil-in-children-with-cystic-fibrosis
#9
Jennifer Le, John S Bradley, Sara Hingtgen, Shannon Skochko, Nanette Black, Ronald N Jones, Meerana Lim, Edmund V Capparelli
BACKGROUND: Single-dose pharmacokinetics (PK) and safety of ceftaroline fosamil with population pharmacokinetic/pharmacodynamic (PK/PD) modeling for staphylococcal pneumonia was performed in children with CF. METHODS: Subjects between 6 and 18 years old were evaluated in this phase 1, open-label, single-dose, prospective study using 10 mg/kg (up to 600 mg). Non-compartmental analysis and population-based PK analyses with Monte Carlo simulation (for doses 8-20 mg/kg every 8 h, infused over 1-4 h) were conducted...
September 14, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28910260/shining-new-light-on-newborn-screening-of-cystic-fibrosis-in-the-province-of-quebec
#10
Léticia Khendek
Newborn screening of cystic fibrosis, a severe genetic disease with high treatment burden, is offered in all of North America with the exception of the province of Quebec. This condition, when diagnosed on symptomatic presentation, is marked by chronic infections and progressive lung function decline leading to eventual respiratory failure. Patients continue to have a median age of survival notably below the Canadian average. Despite prevalence rates of cystic fibrosis almost three times the national average in certain regions of Quebec, the province still does not offer screening to its newborns...
September 14, 2017: Canadian Journal of Public Health. Revue Canadienne de Santé Publique
https://www.readbyqxmd.com/read/28906321/variceal-hemorrhage-and-adverse-liver-outcomes-in-patients-with-cystic-fibrosis-cirrhosis
#11
Wen Ye, Michael R Narkewicz, Daniel H Leung, Wikrom Karnsakul, Karen F Murray, Estella M Alonso, John C Magee, Sarah Jane Schwarzenberg, Alexander Weymann, Jean P Molleston
OBJECTIVES: Cirrhosis occurs in 5-10% of CF (cystic fibrosis) patients, often accompanied by portal hypertension. We analyzed three adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver death (LD), and risk factors for these in CF Foundation Patient Registry (CFFPR) subjects with reported cirrhosis. METHODS: We determined 10-year incidence rates for VB, LT, LD, and all-cause mortality (ACM), and examined risk factors using competing risk models and Cox-proportional hazard regression...
September 12, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28905459/rescue-therapy-within-the-uk-cystic-fibrosis-registry-an-exploration-of-predictors-of-intravenous-antibiotic-use-amongst-adults-with-cf
#12
Zhe Hui Hoo, Martin J Wildman, Rachael Curley, Stephen J Walters, Michael J Campbell
BACKGROUND AND OBJECTIVE: Intravenous (i.v.) antibiotics are needed for rescue when preventative therapy fails to achieve stability among adults with cystic fibrosis (CF). Understanding the distribution of i.v. days can provide insight into the care that adults with CF need. We aim to determine the baseline characteristics that are associated with higher i.v. use, in particular to test the hypothesis that prior-year i.v. use is associated with future-year i.v. use. METHODS: This is a cross-sectional analysis of the 2013-2014 UK CF registry data...
September 14, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28904353/toll-like-receptor%C3%A2-2-induced-cytotoxic-t-lymphocyte-associated-protein%C3%A2-4-regulates-aspergillus-induced-regulatory-t-cells-with-pro-inflammatory-characteristics
#13
Ruud P H Raijmakers, Evelien G G Sprenkeler, Floor E Aleva, Cor W M Jacobs, Thirumala-Devi Kanneganti, Leo A B Joosten, Frank L van de Veerdonk, Mark S Gresnigt
Patients with cystic fibrosis, chronic obstructive pulmonary disease, severe asthma, pre-existing pulmonary lesions, and severely immunocompromised patients are susceptible to develop infections with the opportunistic pathogenic fungus Aspergillus fumigatus, called aspergillosis. Infections in these patients are associated with persistent pro-inflammatory T-helper (TH)2 and TH17 responses. Regulatory T-cells, natural suppressor cells of the immune system, control pro-inflammatory T-cell responses, but can also contribute to disease by shifting to a pro-inflammatory TH17-like phenotype...
September 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28903517/association-of-vancomycin-trough-concentration-with-response-to-treatment-for-acute-pulmonary-exacerbation-of-cystic-fibrosis
#14
Nicholas M Fusco, Richard Francisconi, Calvin J Meaney, Desiree Duman, Carla A Frederick, William A Prescott
Background: Our goal was to determine the relationship between serum vancomycin trough concentrations (VTCs) and changes in pulmonary function among individuals with an acute pulmonary exacerbation (APE) of cystic fibrosis (CF). Methods: We included subjects who were ≥6 years of age, were hospitalized for an APE of CF between May 1, 2012, and April 30, 2014, were administered vancomycin for ≥48 hours, and had a history of airway infection with methicillin-resistant Staphylococcus aureus...
September 1, 2017: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/28902888/dendrimer-based-selective-autophagy-induction-rescues-%C3%AE-f508-cftr-and-inhibits-pseudomonas-aeruginosa-infection-in-cystic-fibrosis
#15
Scott Mackenzie Brockman, Manish Bodas, David Silverberg, Ajit Sharma, Neeraj Vij
BACKGROUND: Cystic Fibrosis (CF) is a genetic disorder caused by mutation(s) in the CF-transmembrane conductance regulator (Cftr) gene. The most common mutation, ΔF508, leads to accumulation of defective-CFTR protein in aggresome-bodies. Additionally, Pseudomonas aeruginosa (Pa), a common CF pathogen, exacerbates obstructive CF lung pathology. In the present study, we aimed to develop and test a novel strategy to improve the bioavailability and potentially achieve targeted drug delivery of cysteamine, a potent autophagy-inducing drug with anti-bacterial properties, by developing a dendrimer (PAMAM-DEN)-based cysteamine analogue...
2017: PloS One
https://www.readbyqxmd.com/read/28900798/phenotypic-plasticity-in-gene-expression-and-physiological-response-in-red-drum-sciaenops-ocellatus-exposed-to-a-long-term-freshwater-environment
#16
Mariel Gullian Klanian, Omar Zapata Pérez, Miguel Angel Vela-Magaña
Red drum (Sciaenops ocellatus) is a euryhaline fish commonly found in the Gulf of Mexico and along the Atlantic coast of North America. Because of high commercial demand and its euryhaline characteristics, aquaculture of this species has diversified from marine to low-salinity aquaculture systems. In recent years, interest in the feasibility of producing red drum in inland freshwater systems has grown and this prompted us to investigate its osmoregulatory capacity after rearing for 8 months in a freshwater aquaculture system...
September 12, 2017: Fish Physiology and Biochemistry
https://www.readbyqxmd.com/read/28898585/use-of-a-high-throughput-phenotypic-screening-strategy-to-identify-amplifiers-a-novel-pharmacological-class-of-small-molecules-that-exhibit-functional-synergy-with-potentiators-and-correctors
#17
Kenneth A Giuliano, Shinichiro Wachi, Lawrence Drew, Danijela Dukovski, Olivia Green, Cecilia Bastos, Matthew D Cullen, Sheila Hauck, Bradley D Tait, Benito Munoz, Po-Shun Lee, John Preston Miller
Cystic fibrosis (CF) is a lethal genetic disorder caused by mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Despite recent groundbreaking approval of genotype-specific small-molecule drugs, a significant portion of CF patients still lack effective therapeutic options that address the underlying cause of the disease. Through a phenotypic high-throughput screen of approximately 54,000 small molecules, we identified a novel class of CFTR modulators called amplifiers. The identified compound, the characteristics of which are represented here by PTI-CH, selectively increases the expression of immature CFTR protein across different CFTR mutations, including F508del-CFTR, by targeting the inefficiencies of early CFTR biosynthesis...
September 1, 2017: SLAS Discovery
https://www.readbyqxmd.com/read/28894479/the-european-multicentre-bronchiectasis-audit-and-research-collaboration-embarc-experiences-from-a-successful-ers-clinical-research-collaboration
#18
REVIEW
James D Chalmers, Megan Crichton, Pieter C Goeminne, Michael R Loebinger, Charles Haworth, Marta Almagro, Montse Vendrell, Anthony De Soyza, Raja Dhar, Lucy Morgan, Francesco Blasi, Stefano Aliberti, Jeanette Boyd, Eva Polverino
In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare diseases such as cystic fibrosis, there has been little research and few clinical trials in bronchiectasis. Guidelines are primarily based on expert opinion and treatment is challenging because of the heterogeneous nature of the disease. In an effort to address decades of underinvestment in bronchiectasis research, education and clinical care, the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) was established in 2012 as a collaborative pan-European network to bring together bronchiectasis researchers...
September 2017: Breathe
https://www.readbyqxmd.com/read/28894437/evolution-of-stenotrophomonas-maltophilia-in-cystic-fibrosis-lung-over-chronic-infection-a-genomic-and-phenotypic-population-study
#19
Alfonso Esposito, Arianna Pompilio, Clotilde Bettua, Valentina Crocetta, Elisabetta Giacobazzi, Ersilia Fiscarelli, Olivier Jousson, Giovanni Di Bonaventura
Stenotrophomonas maltophilia has been recognized as an emerging multi-drug resistant opportunistic pathogen in cystic fibrosis (CF) patients. We report a comparative genomic and phenotypic analysis of 91 S. maltophilia strains from 10 CF patients over a 12-year period. Draft genome analyses included in silico Multi-Locus Sequence Typing (MLST), Single-Nucleotide Polymorphisms (SNPs), and pangenome characterization. Growth rate, biofilm formation, motility, mutation frequency, in vivo virulence, and in vitro antibiotic susceptibility were determined and compared with population structure over time...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28891754/mounier-kuhn-syndrome-presenting-with-recurrent-atelectasis
#20
Christine Quentin, Nicolas Lefevre, Eddy Bodart, Laurence Hanssens
Objective and importance Mounier Kuhn syndrome is usually diagnosed in adulthood, and only a few cases have been described in childhood. Clinical presentation We present the case of a seven-year-old boy suffering from recurrent pneumonia and atelectasis. Intervention Previously performed chest X-rays showed bilateral hyperinflation and tracheobronchomegaly. Chest computed tomography (CT) confirmed the presence of distal enlargement of trachea and bronchi. Tracheobronchomegaly associated with recurrent respiratory tract infections is consistent with Mounier Kuhn syndrome...
September 11, 2017: Acta Clinica Belgica
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