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https://www.readbyqxmd.com/read/28432469/renal-findings-in-patients-with-mulibrey-nanism
#1
Johanna Sivunen, Susann Karlberg, Jouko Lohi, Niklas Karlberg, Marita Lipsanen-Nyman, Hannu Jalanko
BACKGROUND: Mulibrey nanism (MUL) is a rare inherited disease caused by genetic defects affecting peroxisomal TRIM37 protein. MUL affects multiple organs, leading to growth retardation and early onset type 2 diabetes. We aimed to characterize the structure and function of kidneys and the urinary tract in a large cohort of Finnish MUL patients. METHODS: Ultrasound, magnetic resonance imaging (MRI), and autopsy findings of the kidneys and urinary tract from 101 MUL patients were retrospectively analyzed...
April 22, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28432094/the-use-of-synthetic-hybrid-strains-to-determine-the-role-of-replicon-3-in-virulence-of-the-burkholderia-cepacia-complex
#2
Kirsty Agnoli, Roman Freitag, Margarida C Gomes, Christian Jenul, Angela Suppiger, Olga Mannweiler, Carmen Frauenknecht, Daniel Janser, Annette C Vergunst, Leo Eberl
The Burkholderia cepacia complex (Bcc) displays a wealth of metabolic diversity with great biotechnological potential, but the utilisation of these bacteria is limited by their opportunistic pathogenicity to humans. The third replicon of the Bcc, megaplasmid pC3 (0.5 to 1.4 Mb, previously chromosome 3), is important for various phenotypes, including virulence, antifungal and proteolytic activities, and the utilization of certain substrates. Approximately half of plasmid pC3 is well-conserved throughout sequenced Bcc members, while the other half is not...
April 21, 2017: Applied and Environmental Microbiology
https://www.readbyqxmd.com/read/28431569/co-colonisation-with-aspergillus-fumigatus-and-pseudomonas-aeruginosa-is-associated-with-poorer-health-in-cystic-fibrosis-patients-an-irish-registry-analysis
#3
Emma Reece, Ricardo Segurado, Abaigeal Jackson, Siobhán McClean, Julie Renwick, Peter Greally
BACKGROUND: Pulmonary infection is the main cause of death in cystic fibrosis (CF). Aspergillus fumigatus (AF) and Pseudomonas aeruginosa (PA) are the most prevalent fungal and bacterial pathogens isolated from the CF airway, respectively. Our aim was to determine the effect of different colonisation profiles of AF and PA on the clinical status of patients with CF. METHODS: A retrospective analysis of data from the Cystic Fibrosis Registry of Ireland from 2013 was performed to determine the effect of intermittent and persistent colonisation with AF or PA or co-colonisation with both microorganisms on clinical outcome measures in patients with CF...
April 21, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28430818/comparative-genomics-of-burkholderia-multivorans-a-ubiquitous-pathogen-with-a-highly-conserved-genomic-structure
#4
Charlotte Peeters, Vaughn S Cooper, Philip J Hatcher, Bart Verheyde, Aurélien Carlier, Peter Vandamme
The natural environment serves as a reservoir of opportunistic pathogens. A well-established method for studying the epidemiology of such opportunists is multilocus sequence typing, which in many cases has defined strains predisposed to causing infection. Burkholderia multivorans is an important pathogen in people with cystic fibrosis (CF) and its epidemiology suggests that strains are acquired from non-human sources such as the natural environment. This raises the central question of whether the isolation source (CF or environment) or the multilocus sequence type (ST) of B...
2017: PloS One
https://www.readbyqxmd.com/read/28428948/understanding-the-entanglement-neutrophil-extracellular-traps-nets-in-cystic-fibrosis
#5
REVIEW
Saira R Martínez-Alemán, Lizbeth Campos-García, José P Palma-Nicolas, Romel Hernández-Bello, Gloria M González, Alejandro Sánchez-González
Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the gene that codes for the CF trans-membrane conductance regulator. These mutations result in abnormal secretions viscous airways of the lungs, favoring pulmonary infection and inflammation in the middle of neutrophil recruitment. Recently it was described that neutrophils can contribute with disease pathology by extruding large amounts of nuclear material through a mechanism of cell death known as Neutrophil Extracellular Traps (NETs) into the airways of patients with CF...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28428011/cftr-dependent-defect-in-alternatively-activated-macrophages-in-cystic-fibrosis
#6
Abdullah A Tarique, Peter D Sly, Patrick G Holt, Anthony Bosco, Robert S Ware, Jayden Logan, Scott C Bell, Claire E Wainwright, Emmanuelle Fantino
BACKGROUND: The role of the macrophages in cystic fibrosis (CF) lung disease has been poorly studied. We hypothesized that alternatively activated M2 macrophages are abnormal in CF lung disease. METHODS: Blood samples were collected from adults (n=13) children (n=27) with CF on admission for acute pulmonary exacerbation and when clinically stable. Monocytes were differentiated into macrophages and polarized into classical (M1) and alternatively-activated (M2) phenotypes, function determined ex-vivo and compared with healthy controls...
April 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28427956/curcumin-poly-2-methyl-2-oxazoline-b-tetrahydrofuran-b-2-methyl-2-oxazoline-formulation-an-improved-penetration-and-biological-effect-of-curcumin-in-f508del-cftr-cell-lines
#7
Cristine Gonçalves, Jean-Pierre Gomez, William Même, Bazoly Rasolonjatovo, David Gosset, Steven Nedellec, Philippe Hulin, Cécile Huin, Tony Le Gall, Tristan Montier, Pierre Lehn, Chantal Pichon, Philippe Guégan, Hervé Cheradame, Patrick Midoux
Neutral amphiphilic triblock ABA copolymers are of great interest to solubilize hydrophobic drugs. We reported that a triblock ABA copolymer consisting of methyl-2-oxazoline (MeOx) and tetrahydrofuran (THF) (MeOx6-THF19-MeOx6) (TBCP2) can solubilize curcumin (Cur) a very hydrophobic molecule exhibiting multiple therapeutic effects but whose insolubility and low stability in water is a major drawback for clinical applications. Here, we provide evidences by flow cytometry and confocal microscopy that Cur penetration in normal and ΔF508-CFTR human airway epithelial cell lines is facilitated by TBCP2...
April 17, 2017: European Journal of Pharmaceutics and Biopharmaceutics
https://www.readbyqxmd.com/read/28427548/autosomal-dominant-gain-of-function-stat1-mutation-and-severe-bronchiectasis
#8
Oded Breuer, Hagit Daum, Malena Cohen-Cymberknoh, Susanne Unger, David Shoseyov, Polina Stepensky, Baerbel Keller, Klaus Warnatz, Eitan Kerem
BACKGROUND: In a substantial number of patients with non-cystic fibrosis (CF) bronchiectasis an etiology cannot be found. Various complex immunodeficiency syndromes account for a significant portion of these patients but the mechanism elucidating the predisposition for suppurative lung disease often remains unknown. OBJECTIVE: To investigate the cause and mechanism predisposing a patient to severe bronchiectasis. METHODS: A patient presenting with severe non-CF bronchiectasis was investigated...
May 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28427052/glucosylceramide-critically-contributes-to-the-host-defense-of-cystic-fibrosis-lungs
#9
Barbara Kovacic, Carolin Sehl, Barbara Wilker, Markus Kamler, Katrin Anne Becker, Erich Gulbins
BACKGROUND: Cystic fibrosis (CF) is the most common autosomal-recessive disorder in western countries. Previous studies have demonstrated an important role of sphingolipids in the pathophysiology of cystic fibrosis. It has been shown that ceramide has a central role in various pulmonary infections, including those with Pseudomonas aeruginosa (P. aeruginosa). Ceramide is accumulated in the airways of CF mice and patients. However, little is known about a potential role of glucosylceramide in cystic fibrosis...
March 6, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28426833/correction-modulation-of-behaviour-and-virulence-of-a-high-alginate-expressing-pseudomonas-aeruginosa-strain-from-cystic-fibrosis-by-oral-commensal-bacterium-streptococcus-anginosus
#10
Richard D Waite, Muhammad R Qureshi, Robert A Whiley
[This corrects the article DOI: 10.1371/journal.pone.0173741.].
2017: PloS One
https://www.readbyqxmd.com/read/28426137/hydroxyurea-hydroxycarbamide-for-sickle-cell-disease
#11
REVIEW
Sarah J Nevitt, Ashley P Jones, Jo Howard
BACKGROUND: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea (hydroxycarbamide), an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising fetal haemoglobin. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the effects of hydroxyurea therapy in people with SCD (all genotypes), of any age, regardless of setting...
April 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28424688/antimicrobial-activity-of-mesenchymal-stem-cells-current-status-and-new-perspectives-of-antimicrobial-peptide-based-therapies
#12
REVIEW
Francisca Alcayaga-Miranda, Jimena Cuenca, Maroun Khoury
While mesenchymal stem cells (MSCs)-based therapy appears to be promising, there are concerns regarding possible side effects related to the unwanted suppression of antimicrobial immunity leading to an increased risk of infection. Conversely, recent data show that MSCs exert strong antimicrobial effects through indirect and direct mechanisms, partially mediated by the secretion of antimicrobial peptides and proteins (AMPs). In fact, MSCs have been reported to increase bacterial clearance in preclinical models of sepsis, acute respiratory distress syndrome, and cystic fibrosis-related infections...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28424226/objective-measurement-of-adherence-to-out-patient-airway-clearance-therapy-by-high-frequency-chest-wall-compression-in-cystic-fibrosis
#13
Christina L Mikesell, Robert R Kempainen, Theresa A Laguna, Jeremiah S Menk, Andrew R Wey, Philippe R Gaillard, Warren E Regelmann
BACKGROUND: Objective measures of adherence to high-frequency chest wall compression (HFCWC, also known as high frequency chest wall oscillation, HFCWO), a form of airway clearance therapy for patients with cystic fibrosis, are lacking. We used a novel electronic monitoring device integrated into an HFCWC vest to measure adherence compared with self-reported adherence. We determined factors that influenced adherence and how adherence correlated with baseline pulmonary function and pulmonary exacerbations...
April 19, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28423192/systematic-review-of-the-safety-and-efficacy-of-palivizumab-among-infants-and-young-children-with-cystic-fibrosis
#14
Kok Pim Kua, Shaun Wen Huey Lee
BACKGROUND: Respiratory syncytial virus (RSV) is a common pathogen in infants with cystic fibrosis (CF). The use of palivizumab prophylaxis for RSV infection as the standard of care for infants with CF remains controversial. OBJECTIVE: To evaluate the efficacy of palivizumab in reducing the incidence of RSV hospitalization in children with CF who are younger than 2 years of age. METHODS: Four electronic databases (PubMed, Embase, CINAHL, and CENTRAL) were searched from inception until January 31, 2017 for clinical studies investigating the use of palivizumab in infants with CF aged less than 2 years...
April 19, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28422310/adult-onset-cystic-fibrosis-liver-disease-diagnosis-and-characterization-of-an-underappreciated-entity
#15
Christopher Koh, Sasan Sakiani, Pallavi Surana, Xiongce Zhao, Jason Eccleston, David E Kleiner, David Herion, T Jake Liang, Jay H Hoofnagle, Milica Chernick, Theo Heller
BACKGROUND & AIMS: Cystic fibrosis liver disease (CFLD), a leading cause of death in cystic fibrosis (CF), is mostly described in pediatric populations. Adult-onset CFLD lacks sufficient characterization and diagnostic tools. METHODS: A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with a newly proposed CFLD criteria...
April 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28421452/susceptibility-of-candida-albicans-from-cystic-fibrosis-patients
#16
Raquel Sabino, Elisabete Carolino, Richard B Moss, Niaz Banaei, Cristina Verissimo, David A Stevens
Candida albicans is a common microbe, colonizer and potential pathogen found in respiratory cultures of cystic fibrosis (CF) patients. Because of possible development of resistance in patient isolates resulting from residence in the abnormal milieu of CF patient airways, or from exposure to antifungals, and considering the possibility of patient-to-patient spread of microbes and reports of elevated resistance to other fungal pathogens, it was important to assay the susceptibility of isolates of Candida and compare that profile to isolates from the community...
April 18, 2017: Mycopathologia
https://www.readbyqxmd.com/read/28421166/in-vivo-and-in-vitro-interactions-between-pseudomonas-aeruginosa-and-staphylococcus-spp
#17
REVIEW
An Hotterbeekx, Samir Kumar-Singh, Herman Goossens, Surbhi Malhotra-Kumar
The significance of polymicrobial infections is increasingly being recognized especially in a biofilm context wherein multiple bacterial species-including both potential pathogens and members of the commensal flora-communicate, cooperate, and compete with each other. Two important bacterial pathogens that have developed a complex network of evasion, counter-inhibition, and subjugation in their battle for space and nutrients are Pseudomonas aeruginosa and Staphylococcus aureus. Their strain- and environment-specific interactions, for instance in the cystic fibrosis lung or in wound infections, show severe competition that is generally linked to worse patient outcomes...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28421165/the-diverse-cellular-and-animal-models-to-decipher-the-physiopathological-traits-of-mycobacterium-abscessus-infection
#18
REVIEW
Audrey Bernut, Jean-Louis Herrmann, Diane Ordway, Laurent Kremer
Mycobacterium abscessus represents an important respiratory pathogen among the rapidly-growing non-tuberculous mycobacteria. Infections caused by M. abscessus are increasingly found in cystic fibrosis (CF) patients and are often refractory to antibiotic therapy. The underlying immunopathological mechanisms of pathogenesis remain largely unknown. A major reason for the poor advances in M. abscessus research has been a lack of adequate models to study the acute and chronic stages of the disease leading to delayed progress of evaluation of therapeutic efficacy of potentially active antibiotics...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28420758/the-foxm1-inhibitor-rcm-1-suppresses-goblet-cell-metaplasia-and-prevents-il-13-and-stat6-signaling-in-allergen-exposed-mice
#19
Lifeng Sun, Xiaomeng Ren, I-Ching Wang, Arun Pradhan, Yufang Zhang, Hannah M Flood, Bo Han, Jeffrey A Whitsett, Tanya V Kalin, Vladimir V Kalinichenko
Goblet cell metaplasia and excessive mucus secretion associated with asthma, cystic fibrosis, and chronic obstructive pulmonary disease contribute to morbidity and mortality worldwide. We performed a high-throughput screen to identify small molecules targeting a transcriptional network critical for the differentiation of goblet cells in response to allergens. We identified RCM-1, a nontoxic small molecule that inhibited goblet cell metaplasia and excessive mucus production in mice after exposure to allergens...
April 18, 2017: Science Signaling
https://www.readbyqxmd.com/read/28420732/modulation-of-tmem16a-channel-activity-by-the-von-willebrand-factor-type-a-vwa-domain-of-the-calcium-activated-chloride-channel-regulator-1-clca1
#20
Monica Sala-Rabanal, Zeynep Yurtsever, Kayla N Berry, Colin G Nichols, Tom J Brett
Calcium-activated chloride channels (CaCCs) are key players in transepithelial ion transport and fluid secretion, smooth muscle constriction, neuronal excitability, and cell proliferation. The CaCC regulator 1 (CLCA1) modulates the activity of the CaCC TMEM16A/Anoctamin 1 (ANO1) by directly engaging the channel at the cell surface, but the exact mechanism is unknown. Here, we demonstrate that the von Willebrand factor type A (VWA) domain within the cleaved CLCA1 N-terminal fragment is necessary and sufficient for this interaction...
April 18, 2017: Journal of Biological Chemistry
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