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https://www.readbyqxmd.com/read/28934665/venous-thromboembolism-in-children-with-cystic-fibrosis-retrospective-incidence-and-intrapopulation-risk-factors
#1
Jessica Knight-Perry, Brian R Branchford, Dianne Thornhill, Stacey L Martiniano, Scott D Sagel, Michael Wang
INTRODUCTION: Pediatric venous thromboembolism (VTE) is a rare but serious medical condition. Cystic fibrosis (CF) is a risk for recurrent pediatric VTE and has potential thrombophilic tendency. However, much remains unknown, including incidence and intrapopulation risk factors. METHODS: A retrospective cohort of pediatric CF patients followed at Children's Hospital Colorado from January 1st 2003 through May 20th 2016 was examined. Cases were identified by informatics and validated manually...
September 5, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28934361/restriction-modification-mediated-barriers-to-exogenous-dna-uptake-and-incorporation-employed-by-prevotella-intermedia
#2
Christopher D Johnston, Chelsey A Skeete, Alexey Fomenkov, Richard J Roberts, Susan R Rittling
Prevotella intermedia, a major periodontal pathogen, is increasingly implicated in human respiratory tract and cystic fibrosis lung infections. Nevertheless, the specific mechanisms employed by this pathogen remain only partially characterized and poorly understood, largely due to its total lack of genetic accessibility. Here, using Single Molecule, Real-Time (SMRT) genome and methylome sequencing, bisulfite sequencing, in addition to cloning and restriction analysis, we define the specific genetic barriers to exogenous DNA present in two of the most widespread laboratory strains, P...
2017: PloS One
https://www.readbyqxmd.com/read/28933340/renal-histology-and-mri-findings-in-a-%C3%A2-37-year-old-japanese-patient-with-autosomal-recessive-polycystic-kidney-disease%C3%A2
#3
Yusuke Ito, Akinari Sekine, Daisuke Takada, Junko Yabuuchi, Yuta Kogure, Toshiharu Ueno, Keiichi Sumida, Masayuki Yamanouchi, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Keiichi Kinowaki, Takeshi Fujii, Kenichi Ohashi, Hiroaki Kikuchi, Shintaro Mandai, Motoko Chiga, Takayasu Mori, Eisei Sohara, Shinichi Uchida, Yoshifumi Ubara
A 37-year-old Japanese man with a serum creatinine level of 2.5 mg/dL and hepatomegaly was admitted to our hospital for investigation of renal failure. Magnetic resonance imaging (MRI) showed hepatomegaly with small cystic lesions that had high signal intensity on T2-weighted images. There was no splenomegaly, and the kidneys were nearly normal in size with a few small cystic lesions. Renal biopsy revealed that interstitial fibrosis and tubular atrophy affected 60% of the cortex. There was cystic tubular dilation, mainly affecting the distal loop of Henle and distal tubules, since immunohistochemical staining of the dilated tubules was positive for cytokeratin 7 and Tamm-Horsfall protein but was negative for aquaporin 3 and CD10...
September 21, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28932575/pediatric-lung-transplantation
#4
REVIEW
Christian Benden
Pediatric lung transplantation has been undertaken since the 1980s, and it is today considered an accepted therapy option in carefully selected children with end-stage pulmonary diseases, providing carefully selected children a net survival benefit and improved health-related quality of life. Nowadays, >100 pediatric lung transplants are done worldwide every year. Here, specific pediatric aspects of lung transplantation are reviewed such as the surgical challenge, effects of immunosuppression on the developing pediatric immune system, and typical infections of childhood, as it is vital to comprehend that children undergoing lung transplants present a real challenge as children are not 'just small adults'...
August 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28930641/face-masks-and-cough-etiquette-reduce-the-cough-aerosol-concentration-of-pseudomonas-aeruginosa-in-people-with-cystic-fibrosis
#5
Michelle E Wood, Rebecca E Stockwell, Graham R Johnson, Kay A Ramsay, Laura J Sherrard, Nassib Jabbour, Emma Ballard, Peter O'Rourke, Timothy J Kidd, Claire E Wainwright, Luke D Knibbs, Peter D Sly, Lidia Morawska, Scott C Bell
RATIONALE: People with cystic fibrosis (CF) generate Pseudomonas aeruginosa in droplet nuclei during coughing. The use of surgical masks has been recommended in healthcare settings to minimise pathogen transmission between CF patients. OBJECTIVE: To determine if face masks and cough etiquette reduce viable P. aeruginosa aerosolised during cough. METHODS: Twenty-five adults with CF and chronic P. aeruginosa infection were recruited. Participants performed six talking and coughing maneuvers, with or without face masks (surgical and N95) and hand covering the mouth when coughing (cough etiquette) in an aerosol-sampling device...
September 20, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28930490/tezacaftor-ivacaftor-in-subjects-with-cystic-fibrosis-and-f508del-f508del-cftr-or-f508del-g551d-cftr
#6
Scott H Donaldson, Joseph M Pilewski, Matthias Griese, Jon Cooke, Lakshmi Viswanathan, Elizabeth Tullis, Jane C Davies, Julie A Lekstrom-Himes, Linda T Wang
RATIONALE: Tezacaftor (formerly VX-661) is an investigational small molecule that improves processing and trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) in vitro, and improves CFTR function alone and in combination with ivacaftor. OBJECTIVES: To evaluate safety and efficacy of tezacaftor monotherapy and tezacaftor/ivacaftor combination therapy in subjects with CF homozygous for F508del or compound heterozygous for F508del and G551D...
September 20, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28929280/ecology-of-scedosporium-species-present-knowledge-and-future-research
#7
A Rougeron, S Giraud, A Alastruey-Izquierdo, J Cano-Lira, J Rainer, A Mouhajir, S Le Gal, G Nevez, W Meyer, J P Bouchara
The genus Scedosporium, which comprises at least five clinically relevant species, i.e. Scedosporium apiospermum, Scedosporium boydii, Scedosporium aurantiacum, Scedosporium dehoogii and Scedosporium minutisporum, ranks the second among the filamentous fungi colonizing the airways of patients with cystic fibrosis (CF). This colonization of the airways is thought to contribute to the inflammatory reaction leading to a progressive deterioration of the lung function. Additionally, these colonizing fungi may lead to severe disseminated infections in case of lung transplantation...
September 19, 2017: Mycopathologia
https://www.readbyqxmd.com/read/28928729/high-throughput-genetic-screen-reveals-that-early-attachment-and-biofilm-formation-are-necessary-for-full-pyoverdine-production-by-pseudomonas-aeruginosa
#8
Donghoon Kang, Natalia V Kirienko
Pseudomonas aeruginosa is a re-emerging, multidrug-resistant, opportunistic pathogen that threatens the lives of immunocompromised patients, patients with cystic fibrosis, and those in critical care units. One of the most important virulence factors in this pathogen is the siderophore pyoverdine. Pyoverdine serves several critical roles during infection. Due to its extremely high affinity for ferric iron, pyoverdine gives the pathogen a significant advantage over the host in their competition for iron. In addition, pyoverdine can regulate the production of multiple bacterial virulence factors and perturb host mitochondrial homeostasis...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28926942/the-semi-synthetic-peptide-lin-sb056-1-in-combination-with-edta-exerts-strong-antimicrobial-and-antibiofilm-activity-against-pseudomonas-aeruginosa-in-conditions-mimicking-cystic-fibrosis-sputum
#9
Giuseppantonio Maisetta, Lucia Grassi, Semih Esin, Ilaria Serra, Mariano A Scorciapino, Andrea C Rinaldi, Giovanna Batoni
Pseudomonas aeruginosa is a major cause of chronic lung infections in cystic fibrosis (CF) patients. The ability of the bacterium to form biofilms and the presence of a thick and stagnant mucus in the airways of CF patients largely contribute to antibiotic therapy failure and demand for new antimicrobial agents able to act in the CF environment. The present study investigated the anti-P. aeruginosa activity of lin-SB056-1, a recently described semi-synthetic antimicrobial peptide, used alone and in combination with the cation chelator ethylenediaminetetraacetic acid (EDTA)...
September 16, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28926088/treatments-for-priapism-in-boys-and-men-with-sickle-cell-disease
#10
REVIEW
Francis I Chinegwundoh, Sherie Smith, Kofi A Anie
BACKGROUND: Sickle cell disease comprises a group of genetic haemoglobin disorders. The predominant symptom associated with sickle cell disease is pain resulting from the occlusion of small blood vessels by abnormally 'sickle-shaped' red blood cells. There are other complications, including chronic organ damage and prolonged painful erection of the penis, known as priapism. Severity of sickle cell disease is variable, and treatment is usually symptomatic. Priapism affects up to half of all men with sickle cell disease, however, there is no consistency in treatment...
September 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28925369/generation-of-a-gene-corrected-isogenic-control-ipsc-line-from-cystic-fibrosis-patient-specific-ipscs-homozygous-for-p-phe508del-mutation-mediated-by-talens-and-ssodn
#11
Sylvia Merkert, Christien Bednarski, Gudrun Göhring, Toni Cathomen, Ulrich Martin
Cystic fibrosis (CF) is a monogenetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which affects multiple organs. Human induced pluripotent stem cells (iPSCs) derived from CF patients and the generation of isogeneic gene-corrected control cell lines enable disease modelling, drug discovery or toxicological studies and therefore the development of CF patient-specific therapies. We have previously generated a hiPSC line from a CF patient homozygous for the p...
August 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28923877/interplay-among-resistance-profiles-high-risk-clones-and-virulence-in-the-caenorhabditis-elegans-pseudomonas-aeruginosa-infection-model
#12
Irina Sánchez-Diener, Laura Zamorano, Carla López-Causapé, Gabriel Cabot, Xavier Mulet, Carmen Peña, Rosa Del Campo, Rafael Cantón, Antonio Doménech-Sánchez, Luis Martínez-Martínez, Susana C Arcos, Alfonso Navas, Antonio Oliver
The increasing prevalence of nosocomial infections produced by multidrug-resistant (MDR) or extensively drug-resistant (XDR) Pseudomonas aeruginosa is frequently linked to widespread international strains designated as high-risk clones. In this work we attempted to decipher the interplay between resistance profiles, high-risk clones and virulence, testing a large (n=140) collection of well characterized P. aeruginosa isolates from different sources (bloodstream infections, nosocomial outbreaks, cystic fibrosis and environment) in a Caenorhabditis elegans infection model...
September 18, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28923873/an-anti-persister-strategy-for-the-treatment-of-chronic-pseudomonas-aeruginosa-infections
#13
Martina Koeva, Alina D Gutu, Wesley Hebert, Jeffrey D Wager, Lael M Yonker, George A O'Toole, Frederick M Ausubel, Samuel M Moskowitz, Diane Joseph-McCarthy
Bacterial persisters are a quasi-dormant sub-population of cells that are tolerant to antibiotic treatment. The combination of the aminoglycoside tobramycin with fumarate as an antibacterial potentiator utilizes an anti-persister strategy that is aimed at reducing recurrent Pseudomonas aeruginosa infections by enhancing the killing of P. aeruginosa persisters. Stationary phase cultures of P. aeruginosa were used to generate persister cells. A range of tobramycin concentrations was tested with a range of metabolite concentrations to determine the potentiation effect of the metabolite under a variety of conditions, including a range of pH values and in the presence of azithromycin or cystic fibrosis (CF) patient sputum...
September 18, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28923457/blood-flow-regulation-and-oxidative-stress-during-submaximal-cycling-exercise-in-patients-with-cystic-fibrosis
#14
Matthew A Tucker, Breana Berry, Nichole Seigler, Gareth W Davison, John C Quindry, Dabney Eidson, Kathleen T McKie, Ryan A Harris
BACKGROUND: The impact of blood flow regulation and oxidative stress during exercise in cystic fibrosis (CF) has yet to be investigated. METHODS: A maximal graded exercise test was conducted to determine exercise capacity (VO2 peak) and peak workload in 14 pediatric patients with mild CF (age 14±3y, FEV1 93±16 % predicted) and 14 demographically-matched controls. On a separate visit, participants performed submaximal cycling up to 60% of peak workload where brachial artery blood velocity was determined using Doppler ultrasound...
September 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28923373/outbreak-of-burkholderia-cepacia-bacteraemia-in-a-tertiary-care-centre-due-to-contaminated-ultrasound-probe-gel
#15
Rehab Abdelfattah, Suliman Aljumaah, Ahmed Alqahtani, Sahar Althawadi, Irene Barron, Saleh Almofada
BACKGROUND: Burkholderia cepacia is a significant opportunistic organism in hospitalized and immunocompromised patients, particularly in cystic fibrosis [1] AIMS: We describe the epidemiological investigation of an outbreak of B. cepacia bacteraemia. METHODS: The study examined 14 patients during their admission to three ICUs (Intensive Care Units) in a tertiary care hospital between January-June 2016. The outbreak involved 9 (57%) female and 6 (43%) male patients...
September 15, 2017: Journal of Hospital Infection
https://www.readbyqxmd.com/read/28923132/characterization-of-environmental-pseudomonas-aeruginosa-using-multilocus-sequence-typing-scheme
#16
Júlia Radó, Edit Kaszab, Tünde Petrovics, Judit Pászti, Balázs Kriszt, Sándor Szoboszlay
PURPOSE: The objectives of this study were to examine environmental (hydrocarbon degrading) Pseudomonas aeruginosa isolates with Multilocus Sequence Typing (MLST) and to determine their relevant features, such as serotype, virulence genes, biofilm forming ability and hydrocarbon degrading capacity. METHODOLOGY: The diversity of environmental isolates was assessed with an MLST scheme. Investigation of virulence determinants included serotyping, hemolytic activity test and the detection of virulence genes exoS, exoY, exoT, exoU, exoA...
September 19, 2017: Journal of Medical Microbiology
https://www.readbyqxmd.com/read/28923049/roflumilast-reverses-cftr-mediated-ion-transport-dysfunction-in-cigarette-smoke-exposed-mice
#17
S Vamsee Raju, Lawrence Rasmussen, Peter A Sloane, Li Ping Tang, Emily Falk Libby, Steven M Rowe
BACKGROUND: Dysfunction in cystic fibrosis transmembrane conductance regulator (CFTR) can be elicited by cigarette smoke and is observed in patients with chronic bronchitis. We have previously demonstrated in human airway epithelial cell monolayers that roflumilast, a clinically approved phosphodiesterase 4 inhibitor that reduces the risk of exacerbations in chronic obstructive pulmonary disease patients with chronic bronchitis and a history of exacerbations, activates CFTR-dependent chloride secretion via a cAMP-mediated pathway, partially restores the detrimental effects of cigarette smoke on CFTR-mediated ion transport, and increases CFTR-dependent gastrointestinal fluid secretion in isolated murine intestine segments...
September 18, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28922813/meta-analyses-and-the-evidence-base-for-microbial-outcomes-in-the-treatment-of-pulmonary-mycobacterium-avium-intracellulare-complex-disease
#18
Jotam G Pasipanodya, Deborah Ogbonna, Devyani Deshpande, Shashikant Srivastava, Tawanda Gumbo
Objectives: To perform a systematic review and meta-analysis of the level of funding support and the sputum culture conversion rates in pulmonary Mycobacterium avium-intracellulare complex (P-MAC) disease in adult patients without cystic fibrosis or HIV infection, treated with recommended antibiotic regimens. Methods: We performed a literature search to identify clinical trials, prospective studies and registries that reported outcomes in P-MAC patients. Studies that reported P-MAC diagnosis and treatments based on established guidelines met the inclusion criteria and were examined for bias and quality...
September 1, 2017: Journal of Antimicrobial Chemotherapy
https://www.readbyqxmd.com/read/28922614/mexxy-efflux-pump-overexpression-and-aminoglycoside-resistance-in-cystic-fibrosis-isolates-of-i-pseudomonas-aeruginosa-i-from-chronic-infections
#19
Manu Singh, Yvonne Yau, Shirley Wang, Valerie Waters, Ayush Kumar
In this study, we analyzed 15 multidrug resistant cystic fibrosis isolates of Pseudomonas aeruginosa from chronic lung infections for expression of four different multidrug efflux systems, MexAB-OprM, MexCD-OprJ, MexEF-OprN and MexXY, using quantitative reverse transcriptase-PCR. Overexpression of MexXY pump was observed in all of the isolates tested. Analysis of regulatory genes that control the expression of these four efflux pumps revealed a number of previously uncharacterized mutations. Our work shows that MexXY pump overexpression is common in cystic fibrosis isolates and could be contributing to their reduced aminoglycoside susceptibility...
September 18, 2017: Canadian Journal of Microbiology
https://www.readbyqxmd.com/read/28922375/elevated-igg4-serum-levels-in-patients-with-cystic-fibrosis
#20
Axelle Clerc, Quitterie Reynaud, Stéphane Durupt, Colette Chapuis-Cellier, Raphaële Nové-Josserand, Isabelle Durieu, Jean Christophe Lega
OBJECTIVE: Serum immunoglobulin (Ig) G4 elevation has been associated with several pathological conditions other than IgG4-related disease (IgG4-RD). In cystic fibrosis (CF), an elevation of specific IgG4 has been associated with colonization and infection by Pseudomonas aeruginosa. IgG4 elevation may be a marker of chronic infection or inflammatory stimulation. The aim of this study was to explore the prevalence of elevated IgG4 levels in CF and its correlation with the major clinical and microbiological features found in CF patients...
2017: PloS One
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