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https://www.readbyqxmd.com/read/28342743/measurement-of-ion-fluxes-across-epithelia
#1
REVIEW
Miroslaw Zajac, Krzysztof Dolowy
Epithelial tissues line all wet surfaces of vertebrate bodies. Their major function is directional transport of ions and water. Cells forming an epithelial layer are bound together by a tight junction that forms a barrier to ion flux. Ions and water are transported via specialized molecules. The presence of a defect in a single ion channel molecule leads to cystic fibrosis - the most common, fatal, human genetic disease. The paper describes ion transport data obtained by means of different experimental techniques...
March 22, 2017: Progress in Biophysics and Molecular Biology
https://www.readbyqxmd.com/read/28340353/molecular-structure-of-the-human-cftr-ion-channel
#2
Fangyu Liu, Zhe Zhang, László Csanády, David C Gadsby, Jue Chen
The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-binding cassette (ABC) transporter that uniquely functions as an ion channel. Here, we present a 3.9 Å structure of dephosphorylated human CFTR without nucleotides, determined by electron cryomicroscopy (cryo-EM). Close resemblance of this human CFTR structure to zebrafish CFTR under identical conditions reinforces its relevance for understanding CFTR function. The human CFTR structure reveals a previously unresolved helix belonging to the R domain docked inside the intracellular vestibule, precluding channel opening...
March 23, 2017: Cell
https://www.readbyqxmd.com/read/28340310/viruses-in-cystic-fibrosis-patients-airways
#3
Lisa Billard, Rozenn Le Berre, Léa Pilorgé, Christopher Payan, Geneviève Héry-Arnaud, Sophie Vallet
Although bacteria have historically been considered to play a major role in cystic fibrosis (CF) airway damage, a strong impact of respiratory viral infections (RVI) is also now recognized. Emerging evidence confirms that respiratory viruses are associated with deterioration of pulmonary function and exacerbation and facilitation of bacterial colonization in CF patients. The aim of this review is to provide an overview of the current knowledge on respiratory viruses in CF airways, to discuss the resulting inflammation and RVI response, to determine how to detect the viruses, and to assess their clinical consequences, prevalence, and interactions with bacteria...
March 24, 2017: Critical Reviews in Microbiology
https://www.readbyqxmd.com/read/28339466/in-silico-search-for-modifier-genes-associated-with-pancreatic-and-liver-disease-in-cystic-fibrosis
#4
Pascal Trouvé, Emmanuelle Génin, Claude Férec
Cystic Fibrosis is the most common lethal autosomal recessive disorder in the white population, affecting among other organs, the lung, the pancreas and the liver. Whereas Cystic Fibrosis is a monogenic disease, many studies reveal a very complex relationship between genotype and clinical phenotype. Indeed, the broad phenotypic spectrum observed in Cystic Fibrosis is far from being explained by obvious genotype-phenotype correlations and it is admitted that Cystic Fibrosis disease is the result of multiple factors, including effects of the environment as well as modifier genes...
2017: PloS One
https://www.readbyqxmd.com/read/28338676/dirhamnolipids-secreted-from-pseudomonas-aeruginosa-modify-anjpegungal-susceptibility-of-aspergillus-fumigatus-by-inhibiting-%C3%AE-1-3-glucan-synthase-activity
#5
Benoit Briard, Vero Rasoldier, Perrine Bomme, Noureddine ElAouad, Catherine Guerreiro, Pierre Chassagne, Laetitia Muszkieta, Jean-Paul Latgé, Laurence Mulard, Anne Beauvais
Pseudomonas aeruginosa and Aspergillus fumigatus are the two microorganisms responsible for most of the chronic infections in cystic fibrosis patients. P. aeruginosa is known to produce quorum-sensing controlled rhamnolipids during chronic infections. Here we show that the dirhamnolipids secreted from P. aeruginosa (i) induce A. fumigatus to produce an extracellular matrix, rich in galactosaminogalactan, 1,8-dihydroxynaphthalene (DHN)- and pyo-melanin, surrounding their hyphae, which facilitates P. aeruginosa binding and (ii) inhibit A...
March 24, 2017: ISME Journal
https://www.readbyqxmd.com/read/28338025/preventing-perioperative-bleeding-in-patients-with-inherited-bleeding-disorders
#6
Colin Watterson, Nicholas Beacher
Data sourcesCochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register, a regularly updated database informed by trials identified within electronic databases including MEDLINE. Further defined searches were undertaken in PubMed, Embase, The Cochrane Library, ClinicalTrials.gov and WHO International Clinical Trials Registry Platform. Additional hand searching of relevant journals and books of conference proceedings was undertaken.Study selectionRandomised and quasi-randomised controlled trials in people of all ages with haemophilia or VWD undergoing oral or dental procedures using antifibrinolytic agents (tranexamic acid (TXA) or epsilon aminocaproic acid (EACA)) to prevent perioperative bleeding compared to no intervention with or without placebo...
March 2017: Evidence-based Dentistry
https://www.readbyqxmd.com/read/28337719/an-early-health-economic-analysis-of-the-potential-cost-effectiveness-of-an-adherence-intervention-to-improve-outcomes-for-patients-with-cystic-fibrosis
#7
Paul Tappenden, Susannah Sadler, Martin Wildman
BACKGROUND: Cystic fibrosis (CF) negatively impacts upon health-related quality of life and survival. Adherence to nebulised treatments is low; improving adherence is hypothesised to reduce rates of exacerbation requiring intravenous antibiotics and lung function decline. OBJECTIVE: A state transition model was developed to assess the cost effectiveness of an intervention aimed at increasing patient adherence to nebulised and inhaled antibiotics compared with current CF care, in advance of the forthcoming CFHealthHub randomised controlled trial (RCT)...
March 23, 2017: PharmacoEconomics
https://www.readbyqxmd.com/read/28337699/lung-ultrasound-is-comparable-with-chest-roentgenogram-for-diagnosis-of-community-acquired-pneumonia-in-hospitalised-children
#8
Krishna Kumar Yadav, Shally Awasthi, Anit Parihar
OBJECTIVE: To evaluate the accuracy of lung ultrasound (LUS) in comparison to chest roentgenogram (CXR) in hospitalised children with community-acquired pneumonia (CAP). METHODS: This study was a hospital based prospective observational study, conducted between January 2014 and December 2014. Hospitalised children aged 2 to 59 mo with community-acquired pneumonia were included in the study. The informed written consent was taken from parents (or legal guardian) before recruitment...
March 24, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28337235/the-concentration-of-calprotectin-in-the-stools-of-children-with-diagnosed-cystic-fibrosis
#9
Sabina Więcek, Halina Woś, Bożena Kordys-Darmolińska, Magda Sankiewicz-Szkółka, Urszula Grzybowska-Chlebowczyk
INTRODUCTION: Calprotectin is a protein that plays a regulatory role in inflammatory reactions as an antibacterial and antiproliferative factor. AIM: To assess the concentration of calprotectin in the stools of patients with diagnosed cystic fibrosis. MATERIAL AND METHODS: Forty-one patients were included in the study, 24 boys and 17 girls, aged from 7 weeks to 18 years. The concentration of calprotectin in stools was assessed with the ELISA method...
2017: Przegla̜d Gastroenterologiczny
https://www.readbyqxmd.com/read/28337084/therapeutic-drug-monitoring-of-continuous-infusion-doripenem-in-a-pediatric-patient-on-continuous-renal-replacement-therapy
#10
Jeffrey J Cies, Wayne S Moore, Susan B Conley, Paul Shea, Adela Enache, Arun Chopra
An 11-year-old African American male with severe combined immunodeficiency variant, non-cystic fibrosis bronchiectasis, pancreatic insufficiency, chronic mycobacterium avium-intracellulare infection, chronic sinusitis, and malnutrition presented with a 1-week history of fevers. He subsequently developed respiratory decompensation and cefepime was discontinued and doripenem was initiated. Doripenem was the carbapenem used due to a national shortage of meropenem. By day 7 the patient (24.7 kg) had a positive fluid balance of 6925 mL (28% FO), and on days 7 into 8 developed acute kidney injury evidenced by an elevated serum creatinine of 0...
January 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28337076/nebulized-gentamicin-as-an-alternative-to-nebulized-tobramycin-for-tracheitis-in-pediatric-patients
#11
Justin K Chen, Brittany L Martin-McNew, Lisa M Lubsch
OBJECTIVES: Tracheitis is an infection of the lower respiratory tract and is defined by the US Centers for Disease Control and Prevention (CDC) based on signs and symptoms with no radiographic evidence of pneumonia. One method of treatment involves the use of tobramycin given by nebulizer. The purpose of this study was to compare the safety and efficacy of nebulized gentamicin with nebulized tobramycin. METHODS: This study was conducted in patients under 21 years of age who received greater than or equal to 1 day of gentamicin, 80 mg, or tobramycin, 300 mg, given twice a day by nebulization within the 14-month study period...
January 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28336132/complete-genome-sequence-of-sequential-pandoraea-apista-isolates-from-the-same-cystic-fibrosis-patient-supports-a-model-of-chronic-colonization-with-in-vivo-strain-evolution-over-time
#12
Alexander L Greninger, Jessica Streithorst, Jeffrey A Golden, Charles Y Chiu, Steve Miller
Pandoraea apista in the family Burkholderiaceae is an emerging opportunistic pathogen in cystic fibrosis patients. Here, we describe a case from which 3 separate isolates of P. apista were recovered over a 1-year period. Using a combination of first-, second-, and third-generation sequencing technologies, we sequenced and de novo assembled the complete genomes of these 3 P. apista isolates. The genome of P. apista TF81F4 sequenced in this study was 5.58 Mb with a GC% of 62.3%, differed in sequence from other Pandoraea species by >20%, and included a number of previously undescribed loci...
January 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/28335779/activity-of-amp2041-against-human-and-animal-multidrug-resistant-pseudomonas-aeruginosa-clinical-isolates
#13
Clotilde Silvia Cabassi, Andrea Sala, Davide Santospirito, Giovanni Loris Alborali, Edoardo Carretto, Giovanni Ghibaudo, Simone Taddei
BACKGROUND: Antimicrobial resistance is a growing threat to public health. Pseudomonas aeruginosa is a relevant pathogen causing human and animal infections, frequently displaying high levels of resistance to commonly used antimicrobials. The increasing difficulty to develop new effective antibiotics have discouraged investment in this area and only a few new antibiotics are currently under development. An approach to overcome antibiotic resistance could be based on antimicrobial peptides since they offer advantages over currently used microbicides...
March 23, 2017: Annals of Clinical Microbiology and Antimicrobials
https://www.readbyqxmd.com/read/28334845/infantile-tremor-syndrome-and-subdural-hemorrhage-in-an-infant-with-cystic-fibrosis
#14
Anirban Mandal, Mayank Priyadarshi, Kanaram Jat, Sushil Kumar Kabra
Cystic fibrosis (CF), an autosomal recessive disease with multi-system involvement, may present with bleeding in infancy owing to vitamin K malabsorption. Infantile tremor syndrome (ITS) is an obscure condition associated with vitamin B12 and other micronutrient deficiencies, described predominantly in Indian subcontinent. We describe an infant presenting with ITS and chronic subdural hemorrhage. He was subsequently diagnosed to have CF. The ITS and subdural hemorrhage is rarely reported in children with CF...
February 23, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28333826/complex-relation-between-diet-and-phospholipid-fatty-acids-in-children-with-cystic-fibrosis
#15
Sara Moukarzel, Roger A Dyer, Sheila M Innis
OBJECTIVES: Altered total plasma n-6 and n-3 fatty acids are common in cystic fibrosis (CF). Whether alterations extend to plasma phosphatidylcholine (PC) and phosphatidylethanolamine (PE) and are explained by diet is unclear. The present study was to describe the dietary intake of a large group of children with CF and to determine whether dietary fat composition explains differences in plasma PC and PE fatty acids between children with and without CF. METHODS: Dietary intake was assessed using a food frequency questionnaire...
April 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28332032/qualitative-assessment-of-the-symptoms-and-impact-of-pancreatic-exocrine-insufficiency-pei-to-inform-the-development-of-a-patient-reported-outcome-pro-instrument
#16
Colin D Johnson, Rob Arbuckle, Nicola Bonner, Gary Connett, Enrique Dominguez-Munoz, Philippe Levy, Doris Staab, Nicola Williamson, Markus M Lerch
BACKGROUND: Pancreatic exocrine insufficiency (PEI) affects patients with chronic pancreatitis (CP) and cystic fibrosis (CF) who produce insufficient digestive pancreatic enzymes. Common symptoms include steatorrhoea, diarrhea, and abdominal pain. OBJECTIVE: The objective of the study was to develop and test the content validity of a patient-reported outcome (PRO) instrument assessing PEI symptoms and their impact on health-related quality of life. METHODS: Instrument development was supported by a literature review, expert physician interviews (n = 10: Germany 4, UK 3, France 3), and exploratory, qualitative, concept-elicitation interviews with patients with CF and CP with PEI (n = 61: UK 29, Germany 18, France 14) and expert physicians (n = 10)...
March 22, 2017: Patient
https://www.readbyqxmd.com/read/28330773/a-3-year-prognostic-score-for-adults-with-cystic-fibrosis
#17
L Nkam, J Lambert, A Latouche, G Bellis, P R Burgel, M N Hocine
BACKGROUND: Therapeutic progress in patients with cystic fibrosis (CF) has resulted in improved prognosis over the past decades. We aim to reevaluate prognostic factors of CF and provide a prognostic score to predict the risk of death or lung transplantation (LT) within a 3-year period in adult patients. METHODS: We developed a logistic model using data from the French CF Registry and combined the coefficients into a prognostic score. The discriminative abilities of the model and the prognostic score were assessed by c-statistic...
March 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28329612/aquagenic-wrinkling-of-the-palms-a-case-report-and-literature-review
#18
Matteo Megna, Mariateresa Cantelli, Donata Martellotta, Gabriella Calabrò, Anna Balato, Fabio Ayala
Aquagenic wrinkling of the palms (AWP) is an unusual and rare dermatological condition characterized by excessive palmar wrinkling, occurring within a few minutes of water exposure. Cystic fibrosis (CF) or CF carrier state associated forms, drug induced cases, and idiopathic forms have been described. We report the case of a 27-year-old woman with a 7-year history of transient excessive wrinkling of her palms after brief exposure to water. We present also a comprehensive review of the literature. We believe that AWP has been underdiagnosed thus far and we would like to encourage investigations such as sweat chloride test or genetic studies in these patients because of the association with CF or CF carrier state, particularly when AWP appears in younger ages...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28329534/acquired-aquagenic-papulotranslucent-acrokeratoderma
#19
Sarah E Yagerman, Marie Lager, Nicholas A Soter
Aquagenic papulotranslucent acrokeratoderma isa rare condition with the development of white-totransluscentpapules and plaques after exposureto water. While the first report was described asan autosomal dominant hereditary condition,there have since been acquired cases reported inassociation with cystic fibrosis, with prior exposureto a drug, or as an idiopathic condition. We presenta 24-year-old man with acquired aquagenicpapulotranslucent acrokeratoderma that has beenpresent since infancy, without a family history,without prior drug exposure, and without anypersonal or family history of cystic fibrosis...
December 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28329304/cystic-fibrosis-pulmonary-exacerbations-attributable-to-respiratory-syncytial-virus-and-influenza-a-population-based-study
#20
Ranjani Somayaji, Christopher H Goss, Umer Khan, Moni Neradilek, Kathleen M Neuzil, Justin R Ortiz
Background: Characterization of the role of respiratory viral pathogens on cystic fibrosis (CF) pulmonary disease is needed. We aimed to determine the association of influenza and respiratory syncytial virus (RSV) activity with risk of pulmonary exacerbation (PEx) in persons with CF in the United States. Methods: We conducted a cohort study from January 2003 - March 2009 using the CF Foundation Patient Registry merged with CDC respiratory virus surveillance data...
March 9, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
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