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Cystic Fibrosis

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https://www.readbyqxmd.com/read/28727474/a-primer-on-exocrine-pancreatic-insufficiency-fat-malabsorption-and-fatty-acid-abnormalities
#1
Samer Alkaade, Ashley A Vareedayah
Exocrine pancreatic insufficiency (EPI) is characterized by a deficiency of exocrine pancreatic enzymes, resulting in deficits in digestion of all macronutrients, with deficiencies in digestion of fats being the most clinically relevant. The leading cause of EPI is chronic pancreatitis. However, many other causes and conditions may be implicated, including cystic fibrosis, pancreatic duct obstruction, gastric and pancreatic surgery, diabetes mellitus and other conditions. Physical and biochemical causes of EPI include decreased production and secretion of lipase, increased lipase destruction, pancreatic duct obstruction, decreased lipase stimulation and degradation, as well as gastrointestinal motility disorders...
July 2017: American Journal of Managed Care
https://www.readbyqxmd.com/read/28727186/telephone-monitoring-and-home-visits-significantly-improved-the-quality-of-life-treatment-adherence-and-lung-function-in-children-with-cystic-fibrosis
#2
Elisavet-Anna Chrysochoou, Elpis Hatziagorou, Fotis Kirvassilis, John Tsanakas
Cystic Fibrosis (CF) is a chronic and systemic disease with a progressive course. As survival rates continue to improve, there is a growing demand for new therapeutic options that improve treatment adherence, disease management and quality of life (QoL) (1). The aim of this study was to evaluate the safety and effectiveness of a home care programme for children with CF and to assess the value of regular telephone contact with the CF team based at Hippokration Hospital, Thessaloniki, Greece. This article is protected by copyright...
July 20, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28726737/respiratory-tract-infections-and-the-role-of-biologically-active-polysaccharides-in-their-management-and-prevention
#3
REVIEW
Milos Jesenak, Ingrid Urbancikova, Peter Banovcin
Respiratory tract infections (RTIs) are the most common form of infections in every age category. Recurrent respiratory tract infections (RRTIs), a specific form of RTIs, represent a typical and common problem associated with early childhood, causing high indirect and direct costs on the healthcare system. They are usually the consequence of immature immunity in children and high exposure to various respiratory pathogens. Their rational management should aim at excluding other severe chronic diseases associated with increased morbidity (e...
July 20, 2017: Nutrients
https://www.readbyqxmd.com/read/28726588/reconstituting-mouse-lungs-with-conditionally-reprogrammed-human-bronchial-epithelial-cells
#4
Ryan LaRanger, Jennifer R Peters-Hall, Melissa Coquelin, Busola R Alabi, Chris Chen, Woodring E Wright, Jerry Shay
We developed methods for conditionally reprogramming (CR) primary human bronchial epithelial cells (HBECs) to extend their functional life span and permit their differentiation into both upper and lower airway lung epithelium. We also developed a bioreactor to support vascular perfusion and rhythmic breathing of decellularized mouse lungs reconstituted with CR HBECs isolated from patients with and without cystic fibrosis (CF). While conditionally reprogrammed cells only differentiate into an upper airway epithelium after 35 days at the air liquid interface, in reconstituted lungs these cells differentiate into upper airway bronchial epithelium and lower airway alveolar structures after 12 days...
July 20, 2017: Tissue Engineering. Part A
https://www.readbyqxmd.com/read/28726496/a-preclinical-study-in-rhesus-macaques-for-cystic-fibrosis-to-assess-gene-transfer-and-transduction-by-aav1-and-aav5-with-a-dual-luciferase-reporter-system
#5
William Guggino, Janet Benson, JeanClare Seagrave, Ziying Yan, John F Engelhardt, Guangping Gao, Thomas J Conlon, Liudmila Cebotaru
Cystic fibrosis (CF) is an autosomal recessive disease that is potentially treatable by gene therapy. Since the identification of the gene encoding CFTR, a number of preclinical and clinical trials have been conducted using the first generation of adeno-associated virus, AAV2. All these studies showed that AAV gene therapy for CF is safe, but clinical benefit was not clearly demonstrated. Thus, a new generation of AAV Vectors based on other serotypes is needed to move the field forward. Here we have tested two AAV serotypes (AAV1 and AAV5), using a dual-luciferase reporter system with firefly and Renilla luciferase genes packaged into AAV1 or AAV5, respectively...
July 20, 2017: Human Gene Therapy. Clinical Development
https://www.readbyqxmd.com/read/28721579/survival-of-patients-with-cystic-fibrosis-depending-on-mutation-type-and-nutritional-status
#6
A Szwed, A John, J Goździk-Spychalska, W Czaiński, W Czerniak, J Ratajczak, H Batura-Gabryel
The purpose of the study was to evaluate the influence of nutrition and of the severity of mutation type on survival rate in cystic fibrosis (CF) patients. Data were longitudinally collected from 60 hospitalized adult CF patients, aged 18-50. The variables consisted of body mass index (BMI) ratio, Cole's BMI cut-off points, severity of mutation type, and survival rate of CF patients. We found that the mean BMI was strongly associated with the severity of mutation type and was significantly lower in patients with severe mutations of grade I and II...
July 19, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28720732/pseudomonas-aeruginosa-alters-staphylococcus-aureus-sensitivity-to-vancomycin-in-a-biofilm-model-of-cystic-fibrosis-infection
#7
Giulia Orazi, George A O'Toole
The airways of cystic fibrosis (CF) patients have thick mucus, which fosters chronic, polymicrobial infections. Pseudomonas aeruginosa and Staphylococcus aureus are two of the most prevalent respiratory pathogens in CF patients. In this study, we tested whether P. aeruginosa influences the susceptibility of S. aureus to frontline antibiotics used to treat CF lung infections. Using our in vitro coculture model, we observed that addition of P. aeruginosa supernatants to S. aureus biofilms grown either on epithelial cells or on plastic significantly decreased the susceptibility of S...
July 18, 2017: MBio
https://www.readbyqxmd.com/read/28720345/the-potential-of-phage-therapy-in-cystic-fibrosis-essential-human-bacterial-phage-interactions-and-delivery-considerations-for-use-in-pseudomonas-aeruginosa-infected-airways
#8
REVIEW
Stephanie Trend, Angela M Fonceca, William G Ditcham, Anthony Kicic, Arest Cf
As antimicrobial-resistant microbes become increasingly common and a significant global issue, novel approaches to treating these infections particularly in those at high risk are required. This is evident in people with cystic fibrosis (CF), who suffer from chronic airway infection caused by antibiotic resistant bacteria, typically Pseudomonas aeruginosa. One option is bacteriophage (phage) therapy, which utilises the natural predation of phage viruses upon their host bacteria. This review summarises the essential and unique aspects of the phage-microbe-human lung interactions in CF that must be addressed to successfully develop and deliver phage to CF airways...
July 15, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28719800/variants-in-the-il-8-gene-and-the-response-to-inhaled-bronchodilators-in-cystic-fibrosis
#9
Larissa Lazzarini Furlan, José Dirceu Ribeiro, Carmen Sílvia Bertuzzo, João Batista Salomão Junior, Dorotéia Rossi Silva Souza, Fernando Augusto Lima Marson
OBJECTIVE: IL-8 (interleukin 8) protein promotes inflammatory responses, even in airways. The presence of IL-8 gene variants causes altered inflammatory responses and possibly varied responses to inhaled bronchodilators (BD). Thus, this study analyzed the IL-8 variants (rs4073, rs2227306, and rs2227307) and their association with the response to BD in cystic fibrosis (CF) patients. METHODS: Analysis of IL-8 gene variants was performed by RFLP-PCR. The association between spirometry markers and the response to BD was evaluated by Mann-Whitney and Kruskal-Wallis tests...
July 15, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28718995/a-systematic-review-of-the-prevalence-and-impact-of-urinary-incontinence-in-cystic-fibrosis
#10
REVIEW
Katherine B Frayman, Traci M Kazmerski, Susan M Sawyer
This systematic review synthesizes published articles investigating the prevalence, severity and impact of urinary incontinence (UI), a condition associated with cystic fibrosis (CF). References were identified through searching Medline, Embase and PubMed using the medical subject headings 'cystic fibrosis' AND 'urinary incontinence'. Articles were included if UI prevalence was investigated as an outcome. Twelve studies met selection criteria. The prevalence of UI ranged from 5% to 76%. Age and gender contributed to this variability...
July 18, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28717172/evolution-of-the-pseudomonas-aeruginosa-mutational-resistome-in-an-international-cystic-fibrosis-clone
#11
Carla López-Causapé, Lea Mette Sommer, Gabriel Cabot, Rosa Rubio, Alain A Ocampo-Sosa, Helle Krogh Johansen, Joan Figuerola, Rafael Cantón, Timothy J Kidd, Soeren Molin, Antonio Oliver
Emergence of epidemic clones and antibiotic resistance development compromises the management of Pseudomonas aeruginosa cystic fibrosis (CF) chronic respiratory infections. Whole genome sequencing (WGS) was used to decipher the phylogeny, interpatient dissemination, WGS mutator genotypes (mutome) and resistome of a widespread clone (CC274), in isolates from two highly-distant countries, Australia and Spain, covering an 18-year period. The coexistence of two divergent CC274 clonal lineages was revealed, but without evident geographical barrier; phylogenetic reconstructions and mutational resistome demonstrated the interpatient transmission of mutators...
July 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28716479/lower-exhaled-nitric-oxide-in-infants-with-cystic-fibrosis-compared-to-healthy-controls
#12
Insa Korten, Margot Liechti, Florian Singer, Gaudenz Hafen, Isabelle Rochat, Pinelopi Anagnostopoulou, Dominik Müller-Suter, Jakob Usemann, Alexander Moeller, Urs Frey, Philipp Latzin, Carmen Casaulta
Exhaled nitric oxide (FENO) is a well-known, non-invasive airway biomarker. In patients with Cystic Fibrosis (CF) FENO is decreased. To understand if reduced FENO is primary related to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) dysfunction or an epiphenomenon of chronic inflammation, we measured FENO in 34 infants with CF prior to clinical symptoms and in 68 healthy controls. FENO was lower in CF compared to controls (p=0.0006) and the effect was more pronounced in CF infants without residual CFTR function (p<0...
July 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28716451/multidrug-resistant-pseudomonas-aeruginosa-lower-respiratory-tract-infections-in-the-intensive-care-unit-prevalence-and-risk-factors
#13
Trang D Trinh, Evan J Zasowski, Kimberly C Claeys, Abdalhamid M Lagnf, Shravya Kidambi, Susan L Davis, Michael J Rybak
Intensive care unit (ICU) admission is a risk for multidrug-resistant (MDR) Pseudomonas aeruginosa, but factors specific to critically ill pneumonia patients are not fully characterized. Objective was to determine risk factors associated with MDR P. aeruginosa pneumonia among ICU patients. This was a retrospective case-control study of P. aeruginosa pneumonia in the ICU; cystic fibrosis and colonizers were excluded. Risk factors included comorbid conditions and prior healthcare exposure (anti-pseudomonal antibiotics, hospitalizations, nursing home, P...
June 19, 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/28714611/mapping-targetable-inflammation-and-outcomes-with-cystic-fibrosis-biomarkers
#14
Olivia Giddings, Charles R Esther
Cystic fibrosis is characterized by an overly exuberant neutrophilic inflammatory response to pathogens and other stimuli that starts very early in disease. The overwhelming nature of this response is a primary cause of remodeling and destruction of the airways, suggesting that anti-inflammatory therapies could be beneficial in CF. However, finding therapies that can effectively reduce the inflammatory response without compromising host defenses remains elusive. New approaches towards mapping inflammatory targets promise to aid in developing novel therapeutic strategies and improve outcomes in individuals with CF...
July 17, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28714601/cf-related-diabetes-containing-the-metabolic-miscreant-of-cystic-fibrosis
#15
Amir Moheet, Antoinette Moran
Cystic fibrosis-related diabetes (CFRD) is associated with both an increase in morbidity and mortality in people with cystic fibrosis (CF). With increased screening and improved life expectancy of people with CF, the prevalence of CFRD is expected to rise further. The underlying pathophysiological mechanisms causing glucose intolerance and diabetes in patients with CF are not well understood but both functional and structural abnormalities in islet cells are likely to have key roles. Insulin therapy improves health outcomes in patients with CF...
July 17, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28713772/harnessing-neutrophil-survival-mechanisms-during-chronic-infection-by-pseudomonas-aeruginosa-novel-therapeutic-targets-to-dampen-inflammation-in-cystic-fibrosis
#16
REVIEW
Benoît S Marteyn, Pierre-Régis Burgel, Laurent Meijer, Véronique Witko-Sarsat
More than two decades after cloning the cystic fibrosis transmembrane regulator (CFTR) gene, the defective gene in cystic fibrosis (CF), we still do not understand how dysfunction of this ion channel causes lung disease and the tremendous neutrophil burden which persists within the airways; nor why chronic colonization by Pseudomonas aeruginosa develops in CF patients who are thought to be immunocompetent. It appears that the microenvironment within the lung of CF patients provides favorable conditions for both P...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28713271/luminally-acting-agents-for-constipation-treatment-a-review-based-on-literatures-and-patents
#17
REVIEW
Hong Yang, Tonghui Ma
Constipation is one of the most frequently reported gastrointestinal (GI) disorders that negatively impacts quality of life and is associated with a significant economic burden to the patients and society. Traditional treatments including lifestyle modification and laxatives are often ineffective in the more severe forms of constipation and over the long term. New medications targeting at intestinal chloride channels and colonic serotonin receptors have been demonstrated effective in recent years. Emerging agents focusing on improving intestinal secretion and/or colonic motility have been shown effective in animal models and even in clinical trials...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28712885/detection-of-cftr-function-and-modulation-in-primary-human-nasal-cell-spheroids
#18
John J Brewington, Erin T Filbrandt, F J LaRosa, Alicia J Ostmann, Lauren M Strecker, Rhonda D Szczesniak, John P Clancy
BACKGROUND: Expansion of CFTR modulators to patients with rare/undescribed mutations will be facilitated by patient-derived models quantifying CFTR function and restoration. We aimed to generate a personalized model system of CFTR function and modulation using non-surgically obtained nasal epithelial cells (NECs). METHODS: NECs obtained by curettage from healthy volunteers and CF patients were expanded and grown in 3-dimensional culture as spheroids, characterized, and stimulated with cAMP-inducing agents to activate CFTR...
July 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28712576/comparing-effectiveness-and-outcomes-in-asthma-and-cystic-fibrosis
#19
REVIEW
Michael S Schechter
As technology yields new treatments, pediatric pulmonologists need determine how best to use them and how to decide which ones are best for any specific group or individual patient. Physicians have always customized therapies based upon patient response, but the new concept of "Personalized (or precision) medicine" focuses attention to a greater degree on the individual needs of patients based on their genetic, biomarker, phenotypic, or psychosocial characteristics. The newly developed biologics for treatment of asthma and CFTR modulators for treatment of cystic fibrosis (CF) highlight this newer approach...
June 12, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28712180/causes-of-chronic-non-infectious-diarrhoea-in-infants-less-than-6-months-of-age-rarely-recognized-entities
#20
Iqra Mushtaq, Huma Ashraf Cheema, Hassan Suleman Malik, Nadia Waheed, Muhammad Almas Hashmi, Hassan Suleman Malik
BACKGROUND: Non-infectious causes of chronic diarrhoea are important and easily missed. The study was done with the objectives to identify different causes of chronic non-infectious diarrhoea in infants less than 6 months of age. METHODS: All patients less than 6 months of age presenting for the first time to a Paediatric Gastroenterology tertiary care centre with a history of chronic diarrhoea and negative stool cultures were enrolled over a period of 8 months...
January 2017: Journal of Ayub Medical College, Abbottabad: JAMC
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