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Cystic fibrosys

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https://www.readbyqxmd.com/read/28213960/association-of-rhinovirus-with-exacerbations-in-young-children-affected-by-cystic-fibrosis-preliminary-data
#1
Sacha Stelzer-Braid, Nancy Liu, Michael Doumit, Russell D'Cunha, Yvonne Belessis, Adam Jaffe, William D Rawlinson
Rhinovirus (RV) is a common respiratory viral infection linked to worsening of chronic respiratory diseases including cystic fibrosis (CF) and asthma. RV was tested by RT-PCR in samples (n = 465) collected from the upper (nasal swab, oropharyngeal suction and sputum) and lower (bronchoalveolar washings) respiratory tract of 110 children with CF. Air samples (n = 52) collected from the operating theatres and outpatient clinics were tested for RV. RV was found in 43% of children <5 years suffering an exacerbation, and 12% of older children (5-17 years)...
February 18, 2017: Journal of Medical Virology
https://www.readbyqxmd.com/read/28213469/insulin-signaling-via-the-pi3k-akt-pathway-regulates-airway-glucose-uptake-and-barrier-function-in-a-cftr-dependent-manner
#2
Samuel A Molina, Hannah K Moriarty, Danny T Infield, Barry R Imhoff, Rachel J Vance, Agnes H Kim, Jason M Hansen, William R Hunt, Michael Koval, Nael A McCarty
Cystic fibrosis-related diabetes (CFRD) is the most common co-morbidity associated with cystic fibrosis (CF) and correlates with increased rates of lung function decline. Since glucose is a nutrient present in the airways of patients with bacterial airway infections and since insulin controls glucose metabolism, the effect of insulin on CF airway epithelia was investigated to determine the role of insulin receptors and glucose transport in regulating glucose availability in the airway. The response to insulin by human airway epithelial cells was characterized by qPCR, immunoblot, immunofluorescence, and glucose uptake assays...
February 17, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28211475/biofilm-formation-of-the-black-yeast-like-fungus-exophiala-dermatitidis-and-its-susceptibility-to-antiinfective-agents
#3
Lisa Kirchhoff, Maike Olsowski, Katrin Zilmans, Silke Dittmer, Gerhard Haase, Ludwig Sedlacek, Eike Steinmann, Jan Buer, Peter-Michael Rath, Joerg Steinmann
Various fungi have the ability to colonize surfaces and to form biofilms. Fungal biofilm-associated infections are frequently refractory to targeted treatment because of resistance to antifungal drugs. One fungus that frequently colonises the respiratory tract of cystic fibrosis (CF) patients is the opportunistic black yeast-like fungus Exophiala dermatitidis. We investigated the biofilm-forming ability of E. dermatitidis and its susceptibility to various antiinfective agents and natural compounds. We tested 58 E...
February 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28210295/global-impact-of-bronchiectasis-and-cystic-fibrosis
#4
REVIEW
Margarida Redondo, Holly Keyt, Raja Dhar, James D Chalmers
EDUCATIONAL AIMS: To recognise the clinical and radiological presentation of the spectrum of diseases associated with bronchiectasis.To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems. : Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections...
September 2016: Breathe
https://www.readbyqxmd.com/read/28209466/pharmacokinetics-and-safety-of-cavosonstat-n91115-in-healthy-and-cystic-fibrosis-adults-homozygous-for-f508del-cftr
#5
Scott H Donaldson, George M Solomon, Pamela L Zeitlin, Patrick A Flume, Alicia Casey, Karen McCoy, Edith T Zemanick, Arun Mandagere, Janice M Troha, Steven A Shoemaker, James F Chmiel, Jennifer L Taylor-Cousar
BACKGROUND: Cavosonstat (N91115), an orally bioavailable inhibitor of S-nitrosoglutathione reductase, promotes cystic fibrosis transmembrane conductance regulator (CFTR) maturation and plasma membrane stability, with a mechanism of action complementary to CFTR correctors and potentiators. METHODS: A Phase I program evaluated pharmacokinetics, drug-drug interactions and safety of cavosonstat in healthy and cystic fibrosis (CF) subjects homozygous for F508del-CFTR...
February 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28209107/gastro-intestinal-manifestations-in-cystic-fibrosis-patients
#6
REVIEW
S Van Biervliet, C de Clercq, D Declercq, E Van Braeckel, S Van Daele, F De Baets, D De Looze
Cystic fibrosis (CF) is a life-limiting disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). This defective chloride channel, present in different organ systems such as respiratory system, gastrointestinal tract, reproductive system and sweat glands, disturbs the ion and water transport over the membranes leading to the well known CF symptoms. CF has outgrown paediatric care, as half of CF patients are currently adults. The CF gastrointestinal tract has its own particularities...
September 2016: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/28208841/assessment-of-correlation-between-sweat-chloride-levels-and-clinical-features-of-cystic-fibrosis-patients
#7
Manzoor A Raina, Mosin S Khan, Showkat A Malik, Ab Hameed Raina, Mudassir J Makhdoomi, Javed I Bhat, Syed Mudassar
INTRODUCTION: Cystic Fibrosis (CF) is an autosomal recessive disorder and the incidence of this disease is undermined in Northern India. The distinguishable salty character of the sweat belonging to individuals suffering from CF makes sweat chloride estimation essential for diagnosis of CF disease. AIM: The aim of this prospective study was to elucidate the relationship of sweat chloride levels with clinical features and pattern of CF. MATERIALS AND METHODS: A total of 182 patients, with clinical features of CF were included in this study for quantitative measurement of sweat chloride...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28208047/targeted-therapy-for-chronic-respiratory-disease-a-new-paradigm
#8
Peter G Gibson, Matthew J Peters, Claire E Wainwright
Targeted therapy has emerged as a highly effective treatment approach for chronic respiratory diseases. Many of these conditions have dismal outcomes; however, targeted therapy shows great results for the subgroup who respond. This represents a new way to approach these conditions and offers great promise as a future treatment direction. In severe eosinophilic asthma, therapy that targets the interleukin-5 pathway with monoclonal antibodies leads to a 50% reduction in asthma exacerbations in previously refractory disease...
February 20, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28207476/faecal-calprotectin-in-cystic-fibrosis-and-it-s-relation-to-disease-parameters-a-longitudinal-analysis-over-12-years
#9
Helmut Ellemunter, Alexander Engelhardt, Katharina Schüller, Gratiana Steinkamp
OBJECTIVES: Faecal calprotectin (FC) is a marker of inflammation in the intestinal tract. We assessed FC levels longitudinally in patients with cystic fibrosis (CF) and evaluated the relation between FC results and relevant markers of disease. METHODS: Calprotectin was measured in faecal samples starting in 2003 and values were stored in the centre's patient database. In this retrospective analysis we searched for associations of FC concentrations with disease severity and progression...
February 15, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28204942/molecular-modeling-in-the-age-of-clinical-genomics-the-enterprise-of-the-next-generation
#10
Jeremy W Prokop, Jozef Lazar, Gabrielle Crapitto, D Casey Smith, Elizabeth A Worthey, Howard J Jacob
Protein modeling and molecular dynamics hold a unique toolset to aide in the characterization of clinical variants that may result in disease. Not only do these techniques offer the ability to study under characterized proteins, but they do this with the speed that is needed for time-sensitive clinical cases. In this paper we retrospectively study a clinical variant in the XIAP protein, C203Y, while addressing additional variants seen in patients with similar gastrointestinal phenotypes as the C203Y mutation...
March 2017: Journal of Molecular Modeling
https://www.readbyqxmd.com/read/28202251/high-incidence-of-non-tuberculous-mycobacteria-positive-cultures-among-adolescent-with-cystic-fibrosis
#11
Zoé Cavalli, Quitterie Reynaud, Romain Bricca, Raphaële Nove-Josserand, Stéphane Durupt, Philippe Reix, Marie Perceval, Michèle Pérouse de Montclos, Gérard Lina, Isabelle Durieu
BACKGROUND: We evaluated the prevalence of non-tuberculous mycobacteria (NTM)-positive cultures among our cystic fibrosis (CF) center patients, reviewed risk factors for NTM positivity, and determined its impact on lung function evolution. METHODS: From 2009 to 2014, CF adults and children attending the CF center of Lyon (France) and having at least one positive NTM isolate were included. Each case was matched by age and gender with two CF patients with no NTM isolate (controls)...
February 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28198020/beyond-cystic-fibrosis-transmembrane-conductance-regulator-cftr-single-channel-kinetics-implications-for-therapeutic-intervention
#12
Carmel M McNicholas
No abstract text is available yet for this article.
February 15, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28196856/vardenafil-reduces-macrophage-pro-inflammatory-overresponses-in-cystic-fibrosis-through-pde5-and-cftr-dependent-mechanisms
#13
Sabrina Noel, Nadtha Panin, Mathilde Beka, Barbara Dhooghe, Francois Huaux, Teresinha Leal
Chronic inflammation that progressively disrupts the lung tissue is a hallmark of Cystic Fibrosis (CF). In mice, vardenafil, an inhibitor of phosphodiesterase type 5 (PDE5), restores transepithelial ion transport and corrects mislocalization of the most common CF mutation, F508del-CFTR. It also reduces lung pro-inflammatory responses in mice and in patients with CF. To test the hypothesis that macrophages are target effector cells of the immunomodulatory effect of vardenafil, we isolated lung macrophages from mice homozygous for the F508del mutation or invalidated for the cftr gene and from their corresponding wild-type littermates...
February 14, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28196530/r248g-cystic-fibrosis-transmembrane-conductance-regulator-mutation-in-three-siblings-presenting-with-recurrent-acute-pancreatitis-and-reproductive-issues-a-case-series
#14
Seiichi Villalona, Guillermo Glover-López, Juan Antonio Ortega-García, Rosa Moya-Quiles, Pedro Mondejar-López, Maria C Martínez-Romero, Mariano Rigabert-Montiel, María D Pastor-Vivero, Manuel Sánchez-Solís
BACKGROUND: Mutational combinations of the cystic fibrosis transmembrane conductance regulator, CFTR, gene have different phenotypic manifestations at the molecular level with varying clinical consequences for individuals possessing such mutations. Reporting cystic fibrosis transmembrane conductance regulator mutations is important in understanding the genotype-phenotype correlations and associated clinical presentations in patients with cystic fibrosis. Understanding the effects of mutations is critical in developing appropriate treatments for individuals affected with cystic fibrosis, non-classic cystic fibrosis, or cystic fibrosis transmembrane conductance regulator-related disorders...
February 15, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28196020/the-prevalence-and-clinicopathological-characteristics-of-high-grade-pancreatic-intraepithelial-neoplasia-autopsy-study-evaluating-the-entire-pancreatic-parenchyma
#15
Yoko Matsuda, Toru Furukawa, Shinichi Yachida, Makoto Nishimura, Atsuko Seki, Keisuke Nonaka, Junko Aida, Kaiyo Takubo, Toshiyuki Ishiwata, Wataru Kimura, Tomio Arai, Mari Mino-Kenudson
OBJECTIVE: We sought to identify clinicopathological characteristics of high-grade pancreatic intraepithelial neoplasia (PanIN)/carcinoma in situ to facilitate screening for pancreatic ductal adenocarcinoma. METHODS: We evaluated PanIN lesions in 173 consecutive autopsy cases with no evidence of pancreatic ductal adenocarcinoma and/or intraductal papillary mucinous neoplasm (mean age, 80.5 years) by submitting the entire pancreas for microscopic examination. RESULTS: PanIN-3 was found in 4% of examined cases, whereas PanIN-1 and PanIN-2 were present in 77% and 28%, respectively...
February 14, 2017: Pancreas
https://www.readbyqxmd.com/read/28195023/cheer-national-study-of-chronic-rhinosinusitis-practice-patterns
#16
Nikita Chapurin, Melissa A Pynnonen, Rhonda Roberts, Kristine Schulz, Jennifer J Shin, David L Witsell, Kourosh Parham, Alan Langman, David Carpenter, Andrea Vambutas, Anh Nguyen-Huynh, Anne Wolfley, Walter T Lee
Objectives (1) Describe national patterns of chronic rhinosinusitis (CRS) care across academic and community practices. (2) Determine the prevalence of comorbid disorders in CRS patients, including nasal polyposis, allergic rhinitis, asthma, and cystic fibrosis. (3) Identify demographic, clinical, and practice type factors associated with endoscopic sinus surgery (ESS). Study Design Multisite cross-sectional study. Setting Otolaryngology's national research network CHEER (Creating Healthcare Excellence through Education and Research)...
February 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28194750/differential-responses-of-human-dendritic-cells-to-metabolites-from-the-oral-airway-microbiome
#17
Katrine Whiteson, Sudhanshu Agrawal, Anshu Agrawal
Small molecule metabolites that are produced or altered by host-associated microbial communities are emerging as significant immune response modifiers. However, there is a key gap in our knowledge of how oral microbial metabolites affect the immune response. Here, we examined the effects of metabolites from five bacterial strains commonly found in the oral/airway microbial communities of humans. The five strains, each isolated from cystic fibrosis patient sputum, were Pseudomonas aeruginosa FLR01 non-mucoid (P1) and FLR02 mucoid (P2) forms, Streptococcus pneumoniae (Sp), Streptococcus salivarius (Ss), and Rothia mucilaginosa (Rm)...
February 14, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28194692/role-of-cftr-mutation-analysis-in-the-diagnostic-algorithm-for-cystic-fibrosis
#18
Michelle Ratkiewicz, Matthew Pastore, Karen Sharrock McCoy, Rohan Thompson, Don Hayes, Shahid Ijaz Sheikh
BACKGROUND: The cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation identification is being used with increased frequency to aid in the diagnosis of cystic fibrosis (CF) in those suspected with CF. Aim of this study was to identify diagnostic outcomes when CFTR mutational analysis was used in CF diagnosis. CFTR mutational analysis results were also compared with sweat chloride results. METHODS: This study was done on all patients at our institution who had CFTR mutation analysis over a sevenyear period since August 2006...
February 14, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28193033/wearable-potentiometric-chloride-sweat-sensor-the-critical-role-of-the-salt-bridge
#19
Dong-Hoon Choi, Jin Seob Kim, Garry R Cutting, Peter C Searson
The components of sweat provide an array of potential biomarkers for health and disease. Sweat chloride is of interest as a biomarker for cystic fibrosis, electrolyte metabolism disorders, electrolyte balance, and electrolyte loss during exercise. Developing wearable sensors for biomarkers in sweat is a major technological challenge. Potentiometric sensors provide a relatively simple technology for on-body sweat chloride measurement, however, equilibration between reference and test solutions has limited the time over which accurate measurements can be made...
December 20, 2016: Analytical Chemistry
https://www.readbyqxmd.com/read/28190780/pregnancy-among-cystic-fibrosis-women-in-the-era-of-cftr-modulators
#20
Sonya L Heltshe, Emily M Godfrey, Tatiana Josephy, Moira L Aitken, Jennifer L Taylor-Cousar
BACKGROUND: Little is known about how new therapies that partially correct the basic cystic fibrosis (CF) defect (ivacaftor and lumacaftor) might alter hormonal contraceptive effectiveness, impact pregnancy outcomes, or affect pregnancy timing. Examination of pregnancy rates among CF women during periods of CFTR modulator therapy initiation will provide foundation for further research in this area. METHODS: The Cystic Fibrosis Foundation Patient Registry was used to examine pregnancy rates and outcomes by genotype class before, during, and after the introduction of CFTR modulator therapies between 2005 and 2014...
February 9, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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