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https://www.readbyqxmd.com/read/29346439/interspecies-diversity-of-chloride-channel-regulators-calcium-activated-3-genes
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/29336853/updated-guidelines-on-infection-prevention-in-pediatric-ambulatory-settings
#15
Deborah L McBride
The American Academy of Pediatrics (AAP) recently released updated guidelines on infection control and prevention in pediatric outpatient settings. Key recommendations include mandatory influenza vaccination for all healthcare workers offered free to all staff, expanded guidance on infection control practices for children with cystic fibrosis, guidance on additional prevention procedures during community outbreaks of infectious diseases and recommendations on improving communication between healthcare facilities when referring potentially contagious patients...
January 11, 2018: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/29336141/label-free-optical-biodetection-of-pathogen-virulence-factors-in-complex-media-using-microtoroids-with-multifunctional-surface-functionality
#16
Pelin Toren, Erol Ozgur, Mehmet Bayindir
Early detection of pathogens or their virulence factors in complex media has a key role in early diagnosis and treatment of many diseases. Nano-molar and selective detection of Exotoxin A, which is a virulence factor secreted from Pseudomonas aeruginosa in the sputum of Cystic Fibrosis (CF) patients, can pave the way for early diagnosis of P. aeruginosa infections. In this study, we conducted a preliminary study to demonstrate the feasibility of optical biodetection of P. aeruginosa Exotoxin A in a diluted artificial sputum mimicking the CF respiratory environment...
January 16, 2018: ACS Sensors
https://www.readbyqxmd.com/read/29333815/prevalence-of-vitamin-k-deficiency-and-associated-factors-in-non-supplemented-cystic-fibrosis-patients
#17
Patrycja Krzyżanowska, Sławomira Drzymala-Czyż, Nataliya Rohovyk, Lyudmyla Bober, Jerzy Moczkco, Marta Rachel, Jarosław Walkowiak
INTRODUCTION: Vitamin K deficiency is highly prevalent in cystic fibrosis (CF) patients despite supplementation. Moreover, no reliable risk factors for its occurrence are known. The aim was to assess the prevalence of vitamin K deficiency and associated factors in non-supplemented CF patients. METHODS: Prothrombin concentration induced by vitamin K absence (PIVKA-II) and the undercarboxylated osteocalcin percentage (u-OC) were determined. In all patients clinical status was assessed and its relation to vitamin K deficiency determined...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29333432/cytomegalovirus-associated-pulmonary-exacerbation-in-patients-with-cystic-fibrosis
#18
Akhil Sawant, Giulia Spoletini, Paul Whitaker, Christine Etherington, Ian Clifton, Daniel Peckham
CMV is an unusual cause of pulmonary exacerbation in immunocompetent individuals with CF http://ow.ly/Rdds30hlnjV.
January 2018: ERJ Open Research
https://www.readbyqxmd.com/read/29332976/australasian-guideline-2nd-edition-an-annex-to-the-clsi-and-uk-guidelines-for-the-performance-of-the-sweat-test-for-the-diagnosis-of-cystic-fibrosis
#19
John Massie, Ronda Greaves, Michael Metz, Veronica Wiley, Peter Graham, Samantha Shepherd, Richard Mackay
No abstract text is available yet for this article.
November 2017: Clinical Biochemist. Reviews
https://www.readbyqxmd.com/read/29332379/correlation-among-atherosclerosis-cardiac-and-respiratory-function-in-subjects-with-cystic-fibrosis-a-pilot-study
#20
Marco M Ciccone, Francesca Cortese, Michele Gesualdo, Pietro Scicchitano, Gabriella Ricci, Santa Carbonara, Michele Milo, Rosa Carbonara, Flavia Urbano, Anna M Cortese, Antonio Vergori, Antonio Manca, Luigi Mappa
No abstract text is available yet for this article.
January 11, 2018: Minerva Medica
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