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Cystic fibrosys

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https://www.readbyqxmd.com/read/29351449/personalized-medicine-in-cf-from-modulator-development-to-therapy-for-cystic-fibrosis-patients-with-rare-cftr-mutations
#1
Misak Harutyunyan, Yunjie Huang, Kyu-Shik Mun, Fanmuyi Yang, Kavisha Arora, Anjaparavanda P Naren
Cystic fibrosis (CF) is the most common life-shortening genetic disease affecting approximately 1 in 3500 of the Caucasian population. CF is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. To date, over 2000 CFTR mutations have been identified and produce a wide range of phenotypes. The CFTR protein, a chloride channel, is normally expressed on epithelial cells lining the lung, gut, and exocrine glands. Mutations in CFTR have led to pleiotropic effects in CF patients and have resulted in early morbidity and mortality...
December 14, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29351448/extracellular-oxidation-in-cystic-fibrosis-airway-epithelium-causes-enhanced-egfr-adam17-activity
#2
Marta Stolarczyk, Guido Veit, Andrea Schnur, Mieke Veltman, Gergely L Lukacs, Bob J Scholte
The EGFR/ADAM17 signaling pathway mediates the shedding of growth factors and secretion of cytokines, and is involved in chronic inflammation and tissue remodeling. Since these are hallmarks of cystic fibrosis (CF) lung disease, we hypothesized that CFTR deficiency enhances EGFR/ADAM17 activity in human bronchial epithelial cells. In CFBE41o- cells lacking functional CFTR (iCFTR-) cultured at air-liquid interface (ALI) we found enhanced ADAM17-mediated shedding of the EGFR ligand amphiregulin (AREG) compared to genetically identical cells with induced CFTR expression (iCFTR+)...
December 14, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29351441/vx-809-vx-770-treatment-reduces-inflammatory-response-to-pseudomonas-aeruginosa-in-primary-differentiated-cystic-fibrosis-bronchial-epithelial-cells
#3
Manon Ruffin, Lucie Roussel, Émilie Maillé, Simon Rousseau, Emmanuelle Brochiero
Cystic fibrosis patients exhibit chronic P. aeruginosa respiratory infections and sustained pro-inflammatory state favoring lung tissue damage and remodeling, ultimately leading to respiratory failure. Loss of CFTR function is associated with MAPK hyper-activation and increased cytokines expression, such as the interleukin 8 (CXCL8). Recently, new therapeutic strategies directly targeting the basic CFTR defect have been developed and ORKAMBI (Vx-809/Vx-770 combination) is the only FDA-approved treatment for CF patients homozygous for the F508del mutation...
December 20, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29351436/therapeutic-attenuation-of-the-epithelial-sodium-channel-with-a-splunc1-derived-peptide-in-airway-diseases
#4
James F Collawn, Rafal Bartoszewski, Ahmad Lazrak, Sadis Matalon
The airway surface liquid (ASL) coats the lining of the airways and allows for efficient cilial movement and therefore effective mucociliary transport. Cell surface channels regulate the hydration status of the ASL and the two critical channels in the process are the cystic fibrosis transmembrane conductance regulator (CFTR) and the amiloride-sensitive epithelial sodium channel (ENaC). In cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) there is loss of CFTR function or compromised CFTR function in chronic bronchitis, respectively...
January 4, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29349726/fungal-respiratory-infections-in-cystic-fibrosis-cf-recent-progress-and-future-research-agenda
#5
EDITORIAL
Jean-Philippe Bouchara, Françoise Symoens, Carsten Schwarz, Vishnu Chaturvedi
No abstract text is available yet for this article.
February 2018: Mycopathologia
https://www.readbyqxmd.com/read/29349537/timing-of-spirometry-may-impact-hospital-length-of-stay-for-cystic-fibrosis-pulmonary-exacerbation
#6
Katelyn Krivchenia, Dmitry Tumin, Christopher J Nemastil, Joseph D Tobias, Don Hayes
PURPOSE: The optimal timing of spirometry during hospitalization for acute pulmonary exacerbation (PEx) in patients with cystic fibrosis (CF) is unclear. We retrospectively evaluated whether measuring spirometry earlier during hospitalization was associated with a shorter length of stay (LOS). METHODS: In this retrospective study, we analyzed data from the electronic medical record of CF patients 6 years of age and older admitted to a single center for acute PEx requiring IV antibiotic therapy between 2009 and 2016...
January 18, 2018: Lung
https://www.readbyqxmd.com/read/29346439/interspecies-diversity-of-chloride-channel-regulators-calcium-activated-3-genes
#7
Lars Mundhenk, Nancy A Erickson, Nikolai Klymiuk, Achim D Gruber
Members of the chloride channel regulators, calcium-activated (CLCA) family, have been implicated in diverse biomedical conditions, including chronic inflammatory airway diseases such as asthma, chronic obstructive pulmonary disease, and cystic fibrosis, the activation of macrophages, and the growth and metastatic spread of tumor cells. Several observations, however, could not be repeated across species boundaries and increasing evidence suggests that select CLCA genes are particularly prone to dynamic species-specific evolvements...
2018: PloS One
https://www.readbyqxmd.com/read/29346420/initial-acquisition-and-succession-of-the-cystic-fibrosis-lung-microbiome-is-associated-with-disease-progression-in-infants-and-preschool-children
#8
Marianne S Muhlebach, Bryan T Zorn, Charles R Esther, Joseph E Hatch, Conor P Murray, Lidija Turkovic, Sarath C Ranganathan, Richard C Boucher, Stephen M Stick, Matthew C Wolfgang
The cystic fibrosis (CF) lung microbiome has been studied in children and adults; however, little is known about its relationship to early disease progression. To better understand the relationship between the lung microbiome and early respiratory disease, we characterized the lower airways microbiome using bronchoalveolar lavage (BAL) samples obtained from clinically stable CF infants and preschoolers who underwent bronchoscopy and chest computed tomography (CT). Cross-sectional samples suggested a progression of the lower airways microbiome with age, beginning with relatively sterile airways in infancy...
January 2018: PLoS Pathogens
https://www.readbyqxmd.com/read/29346379/a-putative-lateral-flagella-of-the-cystic-fibrosis-pathogen-burkholderia-dolosa-regulates-swimming-motility-and-host-cytokine-production
#9
Damien Roux, Matthew Schaefers, Bradley S Clark, Molly Weatherholt, Diane Renaud, David Scott, John J LiPuma, Gregory Priebe, Craig Gerard, Deborah R Yoder-Himes
Burkholderia dolosa caused an outbreak in the cystic fibrosis clinic at Boston Children's Hospital and was associated with high mortality in these patients. This species is part of a larger complex of opportunistic pathogens known as the Burkholderia cepacia complex (Bcc). Compared to other species in the Bcc, B. dolosa is highly transmissible; thus understanding its virulence mechanisms is important for preventing future outbreaks. The genome of one of the outbreak strains, AU0158, revealed a homolog of the lafA gene encoding a putative lateral flagellin, which, in other non-Bcc species, is used for movement on solid surfaces, attachment to host cells, or movement inside host cells...
2018: PloS One
https://www.readbyqxmd.com/read/29345970/the-prevalence-and-significance-of-staphylococcus-aureus-in-patients-with-non-cystic-fibrosis-bronchiectasis
#10
Mark L Metersky, Timothy R Aksamit, Alan Barker, Radmila Choate, Charles L Daley, Leigh A Daniels, Angela DiMango, Edward Eden, David Griffith, Margaret Johnson, Michael Knowles, Anne E O'Donnell, Kenneth Olivier, Matthias Salathe, Byron Thomashow, Gregory Tino, Gerard Turino, Kevin L Winthrop, David Mannino
RATIONALE: Staphylococcus aureus is commonly cultured from the sputum of patients with bronchiectasis, however little is known about the prevalence of the organism in these patients, the characteristics of patients who have grown the organism or its implications. OBJECTIVES: Determine the relationship between S. aureus and pulmonary function, frequency of exacerbations and frequency of hospitalization in patients with bronchiectasis Methods: The Bronchiectasis Research Registry is a database of adults with non-cystic fibrosis bronchiectasis identified from 13 sites within the United States...
January 18, 2018: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29345411/ethnicity-socioeconomic-status-and-the-severity-and-course-of-non-cystic-fibrosis-bronchiectasis
#11
Sally de Boer, Christopher A Lewis, Wendy Fergusson, Kevin Ellyett, Margaret L Wilsher
Background and aims This study evaluated whether there are ethnic factors which affect the severity and progression of bronchiectasis in our adult multi-ethnic population in Auckland, New Zealand. METHODS: Clinical records were reviewed from patients attending the outpatient facilities of our institution between 2007 and 2010. Data collected included demographics, clinical features, smoking status, self-reported ethnicity, socioeconomic status (NZDep), pulmonary function and sputum microbiology...
January 18, 2018: Internal Medicine Journal
https://www.readbyqxmd.com/read/29344711/adult-picc-device-may-be-used-as-a-tunnelled-central-venous-catheter-in-children
#12
Brooke T Lawson, Ian A Zealley
PURPOSE: Central venous access in children, in particular small children and infants, is challenging. We have developed a technique employing adult peripherally inserted central venous catheters (PICCs) as tunnelled central venous catheters (TCVCs) in children. The principal advantage of this novel technique is that the removal technique is less complex than that of conventional cuffed TCVCs. The catheter can be removed simply by being pulled out and does not require general anaesthesia...
January 17, 2018: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/29343421/frequency-of-small-colony-variants-and-antimicrobial-susceptibility-of-methicillin-resistant-staphylococcus-aureus-in-cystic-fibrosis-patients
#13
Nuntra Suwantarat, Mayer Rubin, Latetia Bryan, Tsigereda Tekle, Michael P Boyle, Karen C Carroll, Mark T Jennings
BACKGROUND: Small-colony variants (SCVs) are a distinct phenotype of Staphylococcus aureus, known for their role in chronic, difficult to treat infections, including cystic fibrosis (CF) lung disease. The goal of this study was to characterize SCV MRSA infection in an adult and pediatric CF population and to identify antibiotic susceptibility patterns unique to SCV MRSA. METHODS: We recovered methicillin-resistant S. aureus (MRSA) from respiratory culture samples from CF patients at the Johns Hopkins Hospital during a 6month study period...
December 2, 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/29342838/biopolymer-based-nanoparticles-for-cystic-fibrosis-lung-gene-therapy-studies
#14
Elena Fernández Fernández, Beatriz Santos-Carballal, Chiara de Santi, Joanne M Ramsey, Ronan MacLoughlin, Sally-Ann Cryan, Catherine M Greene
Lung gene therapy for cystic fibrosis disease has not been successful due to several challenges such as the absence of an appropriate vector. Therefore, optimal delivery of emerging therapeutics to airway epithelial cells demands suitable non-viral systems. In this work, we describe the formulation and the physicochemical investigation of biocompatible and biodegradable polymeric nanoparticles (NPs), including PLGA and chitosan (animal and non-animal), as novel methods for the safe and efficient delivery of CFTR-specific locked nucleic acids (LNAs)...
January 13, 2018: Materials
https://www.readbyqxmd.com/read/29342367/cystic-fibrosis-pulmonary-guidelines-use-of-cftr-modulator-therapy-in-patients-with-cystic-fibrosis
#15
Clement L Ren, Rebecca L Morgan, Christopher Oermann, Helaine E Resnick, Cynthia Brady, Annette Campbell, Richard DeNagel, Margaret Guill, Jeffrey Hoag, Andrew Lipton, Thomas Newton, Stacy Peters, Donna Beth Willey-Courand, Edward T Naureckas
BACKGROUND: CFTR modulators are a new class of medications that target the underlying defect in in cystic fibrosis (CF). Ivacaftor (IVA) and IVA combined with lumacaftor (IVA/LUM) have been approved by the FDA for use in CF patients. However, the FDA label for these medications encompasses patient groups that were not studied as part of the drug approval process. CF clinicians, patients, and their families have recognized a need for recommendations to guide the use of these medications...
January 17, 2018: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29339749/volatile-molecules-from-bronchoalveolar-lavage-fluid-can-rule-in-pseudomonas-aeruginosa-and-rule-out-staphylococcus-aureus-infections-in-cystic-fibrosis-patients
#16
Mavra Nasir, Heather D Bean, Agnieszka Smolinska, Christiaan A Rees, Edith T Zemanick, Jane E Hill
Respiratory infections caused by Pseudomonas aeruginosa and Staphylococcus aureus are the leading cause of morbidity and mortality in cystic fibrosis (CF) patients. The authors aimed to identify volatile biomarkers from bronchoalveolar lavage (BAL) samples that can guide breath biomarker development for pathogen identification. BAL samples (n = 154) from CF patients were analyzed using two-dimensional gas chromatography time-of-flight mass spectrometry. Random Forest was used to select suites of volatiles for identifying P...
January 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29339019/immuno-modulatory-functions-of-the-type-3-secretion-system-and-impacts-on-the-pulmonary-host-defense-a-role-for-exos-of-pseudomonas-aeruginosa-in-cystic-fibrosis
#17
REVIEW
Nawal Belmadi, Yongzheng Wu, Lhousseine Touqui
Number of previous reviews had described the structures and the various functions of the exotoxins produced by the type-3 secretion system of Pseudomonas aeruginosa and their roles in the interactions of this bacterium with host cells. In this review, we summarize some relevant data of literature on ExoS, an exotoxin from the type-3 secretion system of P. aeruginosa, with a particular focus on the role of this toxin in the airways innate response of the host to infection by this bacterium, and its implication in the elimination of Staphylococcus aureus from the airways of patients with cystic fibrosis...
January 12, 2018: Toxicon: Official Journal of the International Society on Toxinology
https://www.readbyqxmd.com/read/29338740/is-sweat-testing-for-cystic-fibrosis-feasible-in-patients-with-down-syndrome
#18
Katharina Ruf, Antonia Demerath, Helge Hebestreit, Steffen Kunzmann
BACKGROUND: Recurrent airway infections are common in patients with Down's syndrome (DS). Hence, ruling out Cystic Fibrosis (CF) in these patients is often required. In the past, the value of sweat testing - the gold standard to diagnose CF - has been questioned in DS as false positive results have been reported. However, these reports are based on measurements of sweat osmolality or sodium concentrations, not chloride concentrations. This study analyses sweat secretion rate and chloride concentration in sweat samples of patients with DS in comparison to healthy controls...
January 16, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29338445/investigational-inhaled-therapies-for-non-cf-bronchiectasis
#19
Sabina Antonela Antoniu
Bronchiectasis not related to cystic fibrosis (non-CF bronchiectasis) are associated with a high unmet therapeutic need due to the lack of specifically authorized medications, especially via the inhalation route. In non-CF bronchiectasis chronic infection with Pseudomonas aeruginosa is common and favored by the persistent local inflammation and viscid sputum production. Therefore inhaled antibiotics, mucolytics or anti-inflammatory agents could represent appropriate therapeutic interventions in this setting Areas covered: This review herein discusses the inhaled therapies currently under investigation for non-CF bronchiectasis and their potential therapeutic positioning in exacerbation versus stable state...
January 17, 2018: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/29336943/one-time-quantitative-pcr-detection-of-pseudomonas-aeruginosa-to-discriminate-intermittent-from-chronic-infection-in-cystic-fibrosis
#20
Sébastien Boutin, Michael Weitnauer, Selina Hassel, Simon Y Graeber, Mirjam Stahl, A Susanne Dittrich, Marcus A Mall, Alexander H Dalpke
BACKGROUND: Chronic airway infection with Pseudomonas aeruginosa is a major risk factor of progression of lung disease in patients with cystic fibrosis (CF). Chronic P. aeruginosa infection evolves from intermittent infection that is amenable to antibiotic eradication, whereas chronically adapted P. aeruginosa becomes resistant to antibiotic therapy. Discrimination of intermittent versus chronic infection is therefore of high therapeutic relevance, yet the available diagnostic methods are only partly satisfactory...
January 11, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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