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Cystic fibrosys

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https://www.readbyqxmd.com/read/28538847/-frequency-of-the-most-common-mutations-of-the-cftr-gene-in-peruvian-patients-with-cystic-fibrosis-using-the-arms-pcr-technique
#1
Ruth Aquino, Ana Protzel, Juan Rivera, Hugo Abarca, Milagros Dueñas, Cecilia Nestarez, Nestor Purizaga, Benoit Diringer
Objectives.: To determine the frequency of the ten most common mutations of the CFTR gene reported in Latin Americausing amplification-refractory mutation system-polymerase chain reaction (ARMS-PCR) in patients with cystic fibrosis (CF) in two referral hospitals in Peru during the year 2014. Materials and Methods.: The frequency of the ten most common mutations of the CFTR gene was assessed in patients of the Hospital Nacional Edgardo Rebagliati Martins and the Instituto Nacional de Salud del Niño, both located in Lima, Peru...
January 2017: Revista Peruana de Medicina Experimental y Salud Pública
https://www.readbyqxmd.com/read/28538779/sweat-test-and-cystic-fibrosis-overview-of-test-performance-at-public-and-private-centers-in-the-state-of-s%C3%A3-o-paulo-brazil
#2
Maria Fátima Servidoni, Carla Cristina Souza Gomez, Fernando Augusto Lima Marson, Adyléia Aparecida Dalbo Contrera Toro, Maria Ângela Gonçalves de Oliveira Ribeiro, José Dirceu Ribeiro, Antônio Fernando Ribeiro
Objective: The sweat test (ST) measures chloride levels in sweat and is considered the gold standard for the diagnosis of cystic fibrosis (CF). However, the reliability of a ST depends on their being performed by experienced technicians and in accordance with strict guidelines. Our aim was to evaluate how sweat stimulation, sweat collection, and chloride measurement are performed at 14 centers (9 public centers and 5 private centers) that routinely perform STs in the state of São Paulo, which has the highest frequency of CF in Brazil...
March 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/28534947/research-and-progress-on-clc%C3%A2-2-review
#3
Hongwei Wang, Minghui Xu, Qingjie Kong, Peng Sun, Fengyun Yan, Wenying Tian, Xin Wang
Chloride channel 2 (ClC-2) is one of the nine mammalian members of the ClC family. The present review discusses the molecular properties of ClC‑2, including CLCN2, ClC‑2 promoter and the structural properties of ClC‑2 protein; physiological properties; functional properties, including the regulation of cell volume. The effects of ClC‑2 on the digestive, respiratory, circulatory, nervous and optical systems are also discussed, in addition to the mechanisms involved in the regulation of ClC‑2. The review then discusses the diseases associated with ClC‑2, including degeneration of the retina, Sjögren's syndrome, age‑related cataracts, degeneration of the testes, azoospermia, lung cancer, constipation, repair of impaired intestinal mucosa barrier, leukemia, cystic fibrosis, leukoencephalopathy, epilepsy and diabetes mellitus...
May 18, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28534862/differences-in-gene-expression-profiles-between-early-and-late-isolates-in-monospecies-achromobacter-biofilm
#4
Signe M Nielsen, Rikke L Meyer, Niels Nørskov-Lauritsen
Bacteria of genus Achromobacter are emerging pathogens in cystic fibrosis (CF) capable of biofilm formation and development of antimicrobial resistance. Evolutionary adaptions in the transition from primary to chronic infection were assessed by transcriptomic analysis of successive isolates of Achromobacter xylosoxidans from a single CF patient. Several efflux pump systems targeting antimicrobial agents were upregulated during the course of the disease, whereas all genes related to motility were downregulated...
May 19, 2017: Pathogens
https://www.readbyqxmd.com/read/28533270/classical-activation-of-macrophages-and-vardenafil
#5
Richmond Muimo
Inhibitors of phosphodiesterase 5 (PDE5) - sildenafil citrate (Viagra; Pfizer) and vardenafil hydrochloride (Levitra; Bayer/GlaxoSmithKline) - approved for the treatment of erectile dysfunction and pulmonary arterial hypertension also rescue the loss of cystic fibrosis (CF) chloride channel function and the mislocalization of F508del-CFTR in affected tissues in CF. Can PDE5 inhibitors provide a therapeutic strategy which combines ability to correct the basic ion transport defect and to control de-regulated lung inflammation in CF?...
June 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28530849/allergic-bronchopulmonary-aspergillosis-in-cystic-fibrosis-mr-imaging-of-airway-mucus-contrasts-as-a-tool-for-diagnosis
#6
Gaël Dournes, Patrick Berger, John Refait, Julie Macey, Stephanie Bui, Laurence Delhaes, Michel Montaudon, Olivier Corneloup, Jean-François Chateil, Roger Marthan, Michaël Fayon, François Laurent
Purpose To assess the diagnostic accuracy of mucus contrast characterization by using magnetic resonance (MR) imaging to discriminate allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF). Materials and Methods The study was approved by the local Ethics Committee, and all patients or their parents gave written informed consent. One hundred ten consecutive patients with CF were screened between January 2014 and July 2015. All patients underwent a non-contrast material-enhanced MR protocol that included routine T1-weighted and T2-weighted sequences...
May 22, 2017: Radiology
https://www.readbyqxmd.com/read/28530203/eit-based-pulsatile-impedance-monitoring-during-spontaneous-breathing-in-cystic-fibrosis
#7
Sabine Krueger-Ziolek, Benjamin Schullcke, Bo Gong, Ullrich Müller-Lisse, Knut Moeller
OBJECTIVE: Evaluating the lung function in patients with obstructive lung disease by electrical impedance tomography (EIT) usually requires breathing maneuvers containing deep inspirations and forced expirations. Since these maneuvers strongly depend on the patient's co-operation and health status, normal tidal breathing was investigated in an attempt to develop continuous maneuver-free measurements. APPROACH: Ventilation related and pulsatile impedance changes were systematically analyzed during normal tidal breathing in 12 cystic fibrosis (CF) patients and 12 lung-healthy controls (HL)...
June 2017: Physiological Measurement
https://www.readbyqxmd.com/read/28530085/-long-term-treatment-with-macrolides-in-chronic-lung-diseases
#8
REVIEW
Michal Shteinberg, Sonia Schneer, Ophir Lavon, Yochai Adir
Macrolide agents have both antibacterial properties as well as various effects on the inflammatory system. In recent years there is growing evidence regarding the favourable effects of macrolides in a range of chronic respiratory conditions. Historically, erythromycin and clarithromycin were found to stabilize pulmonary deterioration in diffuse panbronchiolitis. In cystic fibrosis patients colonized with pseudomonas aeruginosa, long term treatment with azithromycin reduces exacerbations and presents improved lung function...
September 2016: Harefuah
https://www.readbyqxmd.com/read/28529637/role-of-the-cxcl8-cxcr1-2-axis-in-cancer-and-inflammatory-diseases
#9
REVIEW
Helen Ha, Bikash Debnath, Nouri Neamati
The chemokine receptors CXCR1/2 and their ligand CXCL8 are essential for the activation and trafficking of inflammatory mediators as well as tumor progression and metastasis. The CXCL8-CXCR1/2 signaling axis is involved in the pathogenesis of several diseases including chronic obstructive pulmonary diseases (COPD), asthma, cystic fibrosis and cancer. Interaction between CXCL8 secreted by select cancer cells and CXCR1/2 in the tumor microenvironment is critical for cancer progression and metastasis. The CXCL8-CXCR1/2 axis may play an important role in tumor progression and metastasis by regulating cancer stem cell (CSC) proliferation and self-renewal...
2017: Theranostics
https://www.readbyqxmd.com/read/28529073/real-life-acute-lung-function-changes-after-lumacaftor-ivacaftor-first-administration-in-pediatric-patients-with-cystic-fibrosis
#10
Aurélie Labaste, Camille Ohlmann, Catherine Mainguy, Virginie Jubin, Marie Perceval, Laurianne Coutier, Philippe Reix
The combination of lumacaftor and ivacaftor (LUM/IVA) has been reported to induce a mean acute absolute drop of -4.1% predicted forced expiratory volume in 1s (FEV1) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV1 changes after the first dose of LUM/IVA in CF patients. A total of 32 pediatric patients were included. Respiratory manifestations occurred in only 3 patients (9.4%), but FEV1 consistently decreased (-10.4±4.6%, range: -1.5; -21.8%). FEV1 only partially resumed after salbutamol inhalation...
May 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28528545/discovery-and-biosynthesis-of-gladiolin-a-burkholderia-gladioli-antibiotic-with-promising-activity-against-mycobacterium-tuberculosis
#11
Lijiang Song, Matthew Jenner, Joleen Masschelein, Cerith Jones, Matthew J Bull, Simon R Harris, Ruben C Hartkoorn, Anthony Vocat, Isolda Romero-Canelón, Paul Coupland, Gordon Webster, Matthew Dunn, Rebecca Weiser, Christopher Paisey, Stewart T Cole, Julian Parkhill, Eshwar Mahenthiralingam, Gregory L Challis
An antimicrobial activity screen of Burkholderia gladioli BCC0238, a clinical isolate from a cystic fibrosis patient, led to the discovery of gladiolin, a novel macrolide antibiotic with potent activity against Mycobacterium tuberculosis H37Rv. Gladiolin is structurally-related to etnangien, a highly unstable antibiotic from Sorangium cellulosum that is also active against Mycobacteria. Like etnangien, gladiolin was found to inhibit RNA polymerase, a validated drug target in M. tuberculosis. However, gladiolin lacks the highly labile hexaene moiety of etnangien and was thus found to possess significantly increased chemical stability...
May 22, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28526799/acquired-resistance-to-macrolides-in-pseudomonas-aeruginosa-from-cystic-fibrosis-patients
#12
Muhammad-Hariri Mustafa, Shaunak Khandekar, Michael M Tunney, J Stuart Elborn, Barbara C Kahl, Olivier Denis, Patrick Plésiat, Hamidou Traore, Paul M Tulkens, Francis Vanderbist, Françoise Van Bambeke
Cystic fibrosis (CF) patients receive chronic treatment with macrolides for their antivirulence and anti-inflammatory properties. We, however, previously showed that Pseudomonas aeruginosa, considered as naturally resistant to macrolides, becomes susceptible when tested in a eukaryotic medium rather than a conventional broth.We therefore looked for specific macrolide resistance determinants in 333 CF isolates from four European CF centres in comparison with 48 isolates from patients suffering from hospital-acquired pneumonia (HAP)...
May 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28526275/increasing-total-serum-ige-allergic-bronchopulmonary-aspergillosis-and-lung-function-in-cystic-fibrosis
#13
Florian Gothe, Matthias Kappler, Matthias Griese
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity disorder contributing to lung disease in cystic fibrosis (CF) and challenging to diagnose. OBJECTIVE: This study analyzed the predictive value of increasing total IgE (t-IgE) levels in a CF cohort alongside with clinical and serologic data. METHODS: A total of 387 children and young adults were followed from 2000 to 2006 and retrospectively classified into 6 groups...
May 16, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28525415/effects-of-collagenase-digestion-and-stromal-vascular-fraction-supplementation-on-volume-retention-of-fat-grafts
#14
Jonathan B Olenczak, Scott A Seaman, Kant Y Lin, Angela Pineros-Fernandez, Catherine E Davis, Lisa S Salopek, Shayn M Peirce, Patrick S Cottler
OBJECTIVE: The use of autologous fat as a soft tissue filler has increased over the past decade in both reconstructive and aesthetic surgeries. Enhancement of autologous fat grafts with the addition of the stromal vascular fraction (SVF) has been reported to improve long-term volume retention. Stromal vascular fraction is most commonly isolated using enzymatic digestion, but it is unknown what effect the digestion process has on the adipocytes and SVF cells that comprise the graft. Some clinicians have reported use of enzymatically digested fat grafts to alter the physical properties of the tissue in specialized applications...
June 2017: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/28523577/ffa3-activation-stimulates-duodenal-bicarbonate-secretion-and-prevents-nsaid-induced-enteropathy-via-the-glp-2-pathway-in-rats
#15
Hyder Said, Yasutada Akiba, Kazuyuki Narimatsu, Koji Maruta, Ayaka Kuri, Ken-Ichi Iwamoto, Atsukazu Kuwahara, Jonathan D Kaunitz
BACKGROUND: Therapy with nonsteroidal anti-inflammatory drugs (NSAIDs) is associated with enteropathy in humans and experimental animals, a cause of considerable morbidity. Unlike foregut NSAID-associated mucosal lesions, most treatments for this condition are of little efficacy. We propose that the endogenously released intestinotrophic hormone glucagon-like peptide-2 (GLP-2) prevents the development of NSAID-induced enteropathy. Since the short-chain fatty acid receptor FFA3 is expressed on enteroendocrine L cells and on enteric nerves in the gastrointestinal tract, we further hypothesized that activation of FFA3 on L cells protects the mucosa from injury via GLP-2 release with enhanced duodenal HCO3(-) secretion...
May 18, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28523349/diagnosis-of-bronchiectasis-and-airway-wall-thickening-in-children-with-cystic-fibrosis-objective-airway-artery-quantification
#16
Wieying Kuo, Marleen de Bruijne, Jens Petersen, Kazem Nasserinejad, Hadiye Ozturk, Yong Chen, Adria Perez-Rovira, Harm A W M Tiddens
OBJECTIVES: To quantify airway and artery (AA)-dimensions in cystic fibrosis (CF) and control patients for objective CT diagnosis of bronchiectasis and airway wall thickness (AWT). METHODS: Spirometer-guided inspiratory and expiratory CTs of 11 CF and 12 control patients were collected retrospectively. Airway pathways were annotated semi-automatically to reconstruct three-dimensional bronchial trees. All visible AA-pairs were measured perpendicular to the airway axis...
May 18, 2017: European Radiology
https://www.readbyqxmd.com/read/28523001/the-role-of-transient-receptor-potential-vanilloid-4-in-pulmonary-inflammatory-diseases
#17
REVIEW
Rachel G Scheraga, Brian D Southern, Lisa M Grove, Mitchell A Olman
Ion channels/pumps are essential regulators of organ homeostasis and disease. In the present review, we discuss the role of the mechanosensitive cation channel, transient receptor potential vanilloid 4 (TRPV4), in cytokine secretion and pulmonary inflammatory diseases such as asthma, cystic fibrosis (CF), and acute lung injury/acute respiratory distress syndrome (ARDS). TRPV4 has been shown to play a role in lung diseases associated with lung parenchymal stretch or stiffness. TRPV4 indirectly mediates hypotonicity-induced smooth muscle contraction and airway remodeling in asthma...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28522687/integrin-linked-kinase-signaling-promotes-cyst-growth-and-fibrosis-in-polycystic-kidney-disease
#18
Archana Raman, Gail A Reif, Yuqiao Dai, Aditi Khanna, Xiaogang Li, Lindsay Astleford, Stephen C Parnell, James P Calvet, Darren P Wallace
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by innumerous fluid-filled cysts and progressive deterioration of renal function. Previously, we showed that periostin, a matricellular protein involved in tissue repair, is markedly overexpressed by cyst epithelial cells. Periostin promotes cell proliferation, cyst growth, interstitial fibrosis, and the decline in renal function in PKD mice. Here, we investigated the regulation of these processes by the integrin-linked kinase (ILK), a scaffold protein that links the extracellular matrix to the actin cytoskeleton and is stimulated by periostin...
May 18, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28522431/wnk1-and-p38-mapk-distribution-in-ionocytes-and-accessory-cells-of-euryhaline-teleost-fish-implies-ionoregulatory-function
#19
W S Marshall, R R F Cozzi, M Spieker
Ionocytes of euryhaline teleost fish secrete NaCl, under regulation by serine and threonine kinases, including with-no-lysine kinase (WNK1) and p38 mitogen-activated protein kinase (MAPK). Mummichogs (Fundulus heteroclitus L.) were acclimated to freshwater (FW), full strength seawater (SW) and hypersaline conditions (2SW). Immunocytochemistry of ionocytes in opercular epithelia of fish acclimated to SW and 2SW revealed that WNK1-anti-pT58 phosphoantibody localized strongly to accessory cells and was present in the cytosol of ionocytes close to, cystic fibrosis transmembrane conductance regulator (CFTR) in the apical membrane and sodium, potassium, 2 chloride cotransporter (NKCC) in basolateral membrane...
May 18, 2017: Biology Open
https://www.readbyqxmd.com/read/28521806/high-density-lipoprotein-promoting-proliferation-and-migration-of-type-ii-alveolar-epithelial-cells-during-inflammation-state
#20
Zhongji Yu, Jingru Jin, Yuhui Wang, Jian Sun
BACKGROUND: To investigate the effect and mechanism of high density lipoprotein (HDL) on type II alveolar epithelial cells during inflammation state. METHODS: The original generation of type II alveolar epithelial cells were separated in rats and treated with PBS/LPS/HDL/HDL + LPS. To observe the proliferation and migration of type II alveolar epithelial cells with bromodeoxyuridine(BrdU) assay, transwell assay and wound healing experiments. In addition, western blot detected the expression of TP-binding cassette transporter A1 (ABCA1), cystic fibrosis transmembrane conductance regulator (CFTR) and the phosphorylation of AKT/extracellular signal-regulated kinase(ERK)/mitogen-activated protein kinase(MAPK)...
May 18, 2017: Lipids in Health and Disease
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