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https://www.readbyqxmd.com/read/28231023/increasing-nocardia-incidence-associated-with-bronchiectasis-at-a-tertiary-care-center
#1
Michael H Woodworth, Jennifer L Saullo, Paul M Lantos, Gary M Cox, Jason E Stout
RATIONALE: Nocardia is a genus of pathogens that most commonly afflicts immunocompromised hosts, but may be an emerging infection among persons with bronchiectasis. OBJECTIVES: To examine the epidemiology and clinical presentation of adult patients with Nocardia and bronchiectasis relative to other patient groups. METHODS: We examined a retrospectively-assembled cohort of adults at Duke University Hospital in Durham, North Carolina with at least one positive culture from a bodily fluid or tissue specimen for Nocardia between January 1996 and December 2013...
February 23, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28230981/high-potency-phenylquinoxalinone-cystic-fibrosis-transmembrane-conductance-regulator-cftr-activators
#2
Jung-Ho Son, Jie S Zhu, Puay-Wah Phuan, Onur Cil, Andrew P Teuthorn, Colton K Ku, Sujin Lee, Alan S Verkman, Mark J Kurth
We previously identified phenylquinoxalinone CFTRact-J027 (4) as a cystic fibrosis transmembrane conductance regulator (CFTR) activator with an EC50 of ~200 nM, and demonstrated its therapeutic efficacy in mouse models of constipation. Here, structure-activity studies were done on 36 synthesized phenylquinoxalinone analogs to identify compounds with improved potency and altered metabolic stability. Synthesis of the phenylquinoxalinone core was generally accomplished by condensation of 1,2-phenylenediamines with substituted phenyloxoacetates...
February 23, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28230293/influence-of-complex-childhood-diseases-on-variation-in-growth-and-skeletal-development
#3
REVIEW
Babette S Zemel
The study of human growth and skeletal development by human biologists is framed by the larger theoretical concerns regarding the underpinnings of population variation and human evolution. This unique perspective is directly relevant to the assessment of child health and well-being at the individual and group level, as well as the construction of growth charts. Environmental, behavioral (nutrition and physical activity), and disease-related factors can prevent attainment of full genetic potential for growth...
February 23, 2017: American Journal of Human Biology: the Official Journal of the Human Biology Council
https://www.readbyqxmd.com/read/28230279/folate-protects-hepatocytes-of-hyperhomocysteinemia-mice-from-apoptosis-via-cystic-fibrosis-transmembrane-conductance-regulator-cftr-activated-endoplasmic-reticulum-stress
#4
Anning Yang, Yue Sun, Caiyan Mao, Songhao Yang, Min Huang, Mei Deng, Ning Ding, Xiaoling Yang, Minghao Zhang, Shaoju Jin, Yideng Jiang, Ying Huang
Folate deficiency is a known risk factor for liver injury; however, the underlying mechanism remains unclear. In this study, we employed a high homocysteine-induced liver injury model of Apolipoprotein E-deficient (ApoE(-/-) ) mice fed high-methionine diet and found that high homocysteine induced endoplasmic reticulum (ER) stress and liver cell apoptosis by downregulation of cystic fibrosis transmembrane conductance regulator (CFTR) expression; observations that were attenuated with supplementation of dietary folate...
February 23, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28229102/the-use-of-home-spirometry-in-pediatric-cystic-fibrosis-patients-results-of-a-feasibility-study
#5
Aarti Shakkottai, Samya Z Nasr
Medication adherence is poor among pediatric cystic fibrosis (CF) patients, with adolescents having one of the lowest adherence rates. We wanted to identify an adherence intervention that would be acceptable to CF adolescents and assess its feasibility. We surveyed 40 adolescents with CF and asked about barriers to and motivators for their own adherence and to generate ideas for potential adherence interventions. Since most of the respondents chose frequent spirometry at home and medication reminders for interventions, we selected 5 subjects, 10 to 14 years of age, with CF to test the feasibility of home spirometry and medication reminders in pediatric CF patients...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28229097/pediatric-hypovitaminosis-d-molecular-perspectives-and-clinical-implications
#6
Rafiu Ariganjoye
Vitamin D, a secosteroid, is essential for the development and maintenance of healthy bone in both the adult and pediatric populations. Low level of 25-hydroxy vitamin D (25-(OH)-D) is highly prevalent in children worldwide and has been linked to various adverse health outcomes including rickets, osteomalacia, osteomalacic myopathy, sarcopenia, and weakness, growth retardation, hypocalcemia, seizure and tetany, autism, cardiovascular diseases, diabetes mellitus, cancers (prostate, colon, breast), infectious diseases (viral, tuberculosis), and autoimmune diseases, such as multiple sclerosis and Hashimoto's thyroiditis...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28228494/an-evaluation-of-rgm-medium-for-isolation-of-nontuberculous-mycobacteria-from-respiratory-samples-from-patients-with-cystic-fibrosis-in-the-united-states
#7
Rongpong Plongla, Clair L Preece, John D Perry, Peter H Gilligan
A novel selective agar (RGM medium) has been advocated for the isolation of rapidly growing mycobacteria from the sputum of cystic fibrosis (CF) patients. The aim of this study was to compare RGM medium with Burkholderia cepacia selective agar (BCSA) and a standard AFB culture method for the isolation of nontuberculous mycobacteria (NTM) from patients with CF. The applicability of matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) for identification of NTM isolated on RGM was also assessed...
February 22, 2017: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/28225751/lower-cystic-fibrosis-transmembrane-conductance-regulator-cftr-promotes-the-proliferation-and-migration-of-endometrial-carcinoma
#8
Xian Xia, Jie Wang, Yuan Liu, Ming Yue
BACKGROUND The incidence and death rates of endometrial cancer are alarmingly increasing. The diagnosis and treatment of endometrial cancer is crucial to decreasing mortality. Cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the adenosine triphosphate (ATP)-binding cassette transporter family and plays an essential role in anion regulation and tissue homeostasis of various epithelia. This study explored the expression of CFTR in endometrial carcinoma and the role of CFTR in proliferation and migration of endometrial carcinoma cells...
February 22, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28223466/short-term-effect-of-autogenic-drainage-on-ventilation-inhomogeneity-in-adult-subjects-with-stable-non-cystic-fibrosis-bronchiectasis
#9
William Poncin, Grégory Reychler, Noémie Leeuwerck, Nathalie Bauwens, Anne-Sophie Aubriot, Candice Nader, Giuseppe Liistro, Sophie Gohy
BACKGROUND: Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from a multiple-breath washout test, is a promising tool for assessing airway function in patients with non-cystic fibrosis bronchiectasis. However, it is unknown whether ventilation inhomogeneity could improve after successful elimination of excessive secretions within bronchiectasis. The objective of this work was to assess the short-term effects of lung secretion clearance using the autogenic drainage technique on standard lung function tests and LCI in subjects with non-cystic fibrosis bronchiectasis...
February 21, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28223377/exopolysaccharide-repressing-small-molecules-with-antibiofilm-and-antivirulence-activity-against-pseudomonas-aeruginosa
#10
Erik van Tilburg Bernardes, Laetitia Charron-Mazenod, David J Reading, Shauna L Reckseidler-Zenteno, Shawn Lewenza
Biofilm formation is a universal virulence strategy in which bacteria grow in dense microbial communities enmeshed within a polymeric extracellular matrix that protects them from antibiotic exposure and the immune system. Pseudomonas aeruginosa is an archetypal biofilm-forming organism that utilizes a biofilm growth strategy to cause chronic lung infections in Cystic Fibrosis (CF) patients. The extracellular matrix of P. aeruginosa biofilms is comprised mainly of exopolysaccharides (EPS) and DNA. Both mucoid and non-mucoid isolates of P...
February 21, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28223376/bactericidal-and-fungicidal-activity-of-n-chlorotaurine-is-enhanced-in-cystic-fibrosis-sputum-medium
#11
Martina Gruber, Ivan Moser, Markus Nagl, Michaela Lackner
Lung infections with multiresistant pathogens are a major problem of patients suffering from cystic fibrosis (CF). N-chlorotaurine (NCT), a microbicidal active chlorine compound with no resistance development, is well tolerated upon inhalation. The aim of this study was to investigate NCT on its bactericidal and fungicidal activity in vitro in artificial sputum medium (ASM) mimicking the composition of cystic fibrosis mucus.The medium was inoculated with bacteria (Staphylococcus aureus including some MRSA strains, Pseudomonas aeruginosa, Escherichia coli) or spores of fungi (Aspergillus fumigatus, Aspergillus terreus, Candida albicans, Scedosporium apiospermum, Scedosporium boydii, Lomentospora prolificans, Scedosporium aurantiacum, Scedosporium minutisporum, Exophiala dermatitidis, Geotrichum sp...
February 21, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28222887/rare-er-protein-misfolding-mistrafficking-disorders-therapeutic-developments
#12
REVIEW
Ramanath Narayana Hegde, Advait Subramanian, Prathyush Pothukuchi, Seetharaman Parashuraman, Alberto Luini
The presence of a functional protein at the appropriate location in the cell is the result of the processes of transcription, translation, folding and trafficking to the correct destination. There are numerous diseases that are caused by protein misfolding, mainly due to mutations in the respective gene. The consequences of this misfolding may be that proteins effectively lose their function, either by being removed by the cellular quality control machinery or by accumulating at the incorrect intracellular or extracellular location...
February 9, 2017: Tissue & Cell
https://www.readbyqxmd.com/read/28222269/restoring-cftr-function-reduces-airway-bacteria-and-inflammation-in-people-with-cystic-fibrosis-and-chronic-lung-infections
#13
Katherine B Hisert, Sonya L Heltshe, Christopher Pope, Peter Jorth, Xia Wu, Rachael M Edwards, Matthew Radey, Frank J Accurso, Daniel J Wolter, Gordon Cooke, Ryan J Adam, Suzanne Carter, Brenda Grogan, Jan L Launspach, Seamas C Donnelly, Charles Gallagher, James E Bruce, David Stoltz, Michael J Welsh, Lucas R Hoffman, Edward F McKone, Pradeep K Singh
RATIONALE: Previous work indicates that ivacaftor improves CFTR activity and lung function in people with cystic fibrosis (CF) and G551D-CFTR mutations, but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently of CFTR activity once CF lung disease is established. OBJECTIVES: To better understand the relationship between CFTR activity, airway microbiology and inflammation, and lung function in subjects with CF and chronic airway infections...
February 21, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28222031/a-retrospective-analysis-of-longitudinal-changes-in-bone-mineral-content-in-cystic-fibrosis
#14
Adela Chirita-Emandi, Sheila Shepherd, Andreas Kyriakou, Jane D McNeilly, Carol Dryden, Donna Corrigan, Anne Devenny, Syed Faisal Ahmed
BACKGROUND: We aimed to describe the longitudinal changes in bone mineral content and influencing factors, in children with cystic fibrosis (CF). METHODS: One hundred children (50 females) had dual X-ray absorptiometry (DXA) performed. Of these, 48 and 24 children had two to three scans, respectively over 10 years of follow-up. DXA data were expressed as lumbar spine bone mineral content standard deviation score (LSBMCSDS) adjusted for age, gender, ethnicity and bone area...
February 21, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28221736/individual-pharmacokinetic-variation-leads-to-underdosing-of-ciprofloxacin-in-some-cystic-fibrosis-patients
#15
A N Ø Schultz, N Høiby, X C Nielsen, T Pressler, K Dalhoff, M Duno, A Buchard, H K Johansen, H Wang, C S Dalbøge
Ciprofloxacin (CIP) is frequently used when treating cystic fibrose (CF) patients with intermittent Pseudomonas aeruginosa (P. aeruginosa) lung colonization. However, approximately 20% of the patients progress to chronic infection despite early intervention. The aim of this study, was to investigate the pharmacokinetics of CIP, to evaluate if CYP3A4-related metabolism is involved and to find the optimal dose needed to eradicate intermittently colonizing bacteria in the lungs of CF patients. Methods An open-label, prospective pharmacokinetic study was performed...
March 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28220034/exhaled-and-nasal-nitric-oxide-in-relation-to-lung-function-blood-cell-counts-and-disease-characteristics-in-cystic-fibrosis
#16
Christina Krantz, Christer Janson, Annika Hollsing, Kjell Alving, Andrei Malinovschi
BACKGROUND: Patients with CF have similar or lower exhaled nitric oxide (FeNO) and lower nasal nitric oxide (nNO) levels than controls. There are divergent results on alveolar NO (CalvNO) concentrations in relation to CF. There are inconsistent results on correlation between different nitric oxide parameters and lung function and inflammation in CF. AIM: To compare FeNO, CalvNO and nNO levels between subjects with CF, asthma and healthy controls and to study whether these parameters are related to lung function, blood cell counts or clinical characteristics in CF patients...
February 21, 2017: Journal of Breath Research
https://www.readbyqxmd.com/read/28219060/steady-state-therapy-with-azithromycin-or-low-dose-prednisolone-in-paediatric-cystic-fibrosis-patients-inflammatory-markers-and-disease-progression
#17
Galina Shmarina, Alexander Pukhalsky, Lucine Avakian, Sergey Semykin, Daria Pukhalskaya, Vladimir Alioshkin
BACKGROUND: Anti-inflammatory therapy is a logical approach to slowing the inevitable lung function deterioration in cystic fibrosis (CF) patients. This study's aim was to evaluate inflammatory markers and disease progression in paediatric CF patients chronically treated with azithromycin or low-dose prednisolone. METHODS: The study included 204 patients with CF and 100 healthy controls; 102 CF patients were treated with basic therapy only (without anti-inflammatory treatment; WAT), and 102 individuals received basic therapy along with azithromycin (n = 59) or low-dose prednisolone (n = 43)...
February 21, 2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28218802/non-invasive-ventilation-for-cystic-fibrosis
#18
REVIEW
Fidelma Moran, Judy M Bradley, Amanda J Piper
BACKGROUND: Non-invasive ventilation may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis by providing ventilatory support and avoiding tracheal intubation. Using non-invasive ventilation, in the appropriate situation or individuals, can improve lung mechanics through increasing airflow and gas exchange and decreasing the work of breathing. Non-invasive ventilation thus acts as an external respiratory muscle. This is an update of a previously published review...
February 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28218655/taste-receptors-mediate-sinonasal-immunity-and-respiratory-disease
#19
REVIEW
Jennifer E Douglas, Noam A Cohen
The bitter taste receptor T2R38 has been shown to play a role in the pathogenesis of chronic rhinosinusitis (CRS), where the receptor functions to enhance upper respiratory innate immunity through a triad of beneficial immune responses. Individuals with a functional version of T2R38 are tasters for the bitter compound phenylthiocarbamide (PTC) and exhibit an anti-microbial response in the upper airway to certain invading pathogens, while those individuals with a non-functional version of the receptor are PTC non-tasters and lack this beneficial response...
February 17, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28218486/determinants-and-outcomes-of-upfront-surgery-versus-medical-therapy-for-chronic-rhinosinusitis-in-cystic-fibrosis
#20
Noel Ayoub, Andrew Thamboo, Al-Rahim Habib, Jayakar V Nayak, Peter H Hwang
BACKGROUND: The indications for surgical management of chronic rhinosinusitis (CRS) in patients with cystic fibrosis (CF) are poorly defined. In this study we compare outcomes of medical versus surgical treatment and examine trends associated with the transition from medical to surgical therapy in CF patients. METHODS: One hundred thirty-six patients with CF referred to a tertiary rhinology practice were retrospectively divided into 3 cohorts: Medical, Upfront Surgery, or Crossover, if they converted from medical to surgical management...
February 20, 2017: International Forum of Allergy & Rhinology
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