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Cystic fibrosis

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https://www.readbyqxmd.com/read/28812224/risk-factors-for-totally-implantable-venous-access-device-associated-complications-in-cystic-fibrosis
#1
C McCarthy, O O'Carroll, M E O'Brien, T McEnery, A Franciosi, C Gunaratnam, N G McElvaney
BACKGROUND: Candidaemia is an important nosocomial infection, seen frequently in immunocompromised and critically ill patients and increasingly recognised in cystic fibrosis (CF) patients with totally implantable venous access devices (TIVADs). This study aims to investigate the incidence and risk factors for the development of TIVAD-associated candidaemia and to assess the rate of TIVAD-related complications in CF patients. METHODS: A 10-year retrospective study was carried out on adult CF patients attending a single centre...
August 15, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/28811770/clinical-and-laboratory-profile-of-children-with-cystic-fibrosis-experience-of-a-tertiary-care-center-in-pakistan
#2
Danish Abdul Aziz, Abdul Gaffar Billoo, Ahad Qureshi, Misha Khalid, Salman Kirmani
OBJECTIVE: To determine the clinical presentation, diagnostic investigations and laboratory workup done in admitted children with cystic fibrosis at Aga Khan University Hospital Karachi, Pakistan. METHODS: This is athree years retrospective study from January 2013 to December 2015 conducted at The Aga Khan University Hospital Karachi Pakistan, enrolling admitted patient from birth to 15 years of either gender, diagnosed with CF on the basis of clinical features and positive sweat chloride test...
May 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28811674/regenerative-medicine-and-cell-based-approaches-to-restore-pancreatic-function
#3
REVIEW
Cara Ellis, Adam Ramzy, Timothy J Kieffer
The pancreas is a complex organ with exocrine and endocrine components. Many pathologies impair exocrine function, including chronic pancreatitis, cystic fibrosis and pancreatic ductal adenocarcinoma. Conversely, when the endocrine pancreas fails to secrete sufficient insulin, patients develop diabetes mellitus. Pathology in either the endocrine or exocrine pancreas results in devastating economic and personal consequences. The current standard therapy for treating patients with type 1 diabetes mellitus is daily exogenous insulin injections, but cell sources of insulin provide superior glycaemic regulation and research is now focused on the goal of regenerating or replacing β cells...
August 16, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28811149/three-center-feasibility-of-lung-clearance-index-in-infants-and-preschool-children-with-cystic-fibrosis-and-other-lung-diseases
#4
Mirjam Stahl, Simon Y Graeber, Cornelia Joachim, Sandra Barth, Isabell Ricklefs, Gesa Diekmann, Matthias V Kopp, Lutz Naehrlich, Marcus A Mall
BACKGROUND: Lung clearance index (LCI) detects early ventilation inhomogeneity and has been suggested as sensitive endpoint in multicenter intervention trials in infants and preschoolers with cystic fibrosis (CF). However, the feasibility of multicenter LCI in this age group has not been determined. We, therefore, investigated the feasibility of LCI in infants and preschoolers with and without CF in a three-center setting. METHODS: Following central training, standardized SF6-MBW measurements were performed in 73 sedated children (10 controls, 49 with CF and 14 with other lung diseases), mean age 2...
August 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28809446/deferasirox-for-managing-iron-overload-in-people-with-thalassaemia
#5
REVIEW
Claudia Bollig, Lisa K Schell, Gerta Rücker, Roman Allert, Edith Motschall, Charlotte M Niemeyer, Dirk Bassler, Joerg J Meerpohl
BACKGROUND: Thalassaemia is a hereditary anaemia due to ineffective erythropoiesis. In particular, people with thalassaemia major develop secondary iron overload resulting from regular red blood cell transfusions. Iron chelation therapy is needed to prevent long-term complications.Both deferoxamine and deferiprone are effective; however, a review of the effectiveness and safety of the newer oral chelator deferasirox in people with thalassaemia is needed. OBJECTIVES: To assess the effectiveness and safety of oral deferasirox in people with thalassaemia and iron overload...
August 15, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28808008/mathematical-model-reveals-role-of-nucleotide-signaling-in-airway-surface-liquid-homeostasis-and-its-dysregulation-in-cystic-fibrosis
#6
Conner I Sandefur, Richard C Boucher, Timothy C Elston
Mucociliary clearance is composed of three components (i.e., mucin secretion, airway surface hydration, and ciliary-activity) which function coordinately to clear inhaled microbes and other foreign particles from airway surfaces. Airway surface hydration is maintained by water fluxes driven predominantly by active chloride and sodium ion transport. The ion channels that mediate electrogenic ion transport are regulated by extracellular purinergic signals that signal through G protein-coupled receptors. These purinoreceptors and the signaling pathways they activate have been identified as possible therapeutic targets for treating lung disease...
August 14, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28807917/bedaquiline-inhibits-the-atp-synthase-in-mycobacterium-abscessus-and-is-effective-in-infected-zebrafish
#7
Christian Dupont, Albertus Viljoen, Sangeeta Thomas, Françoise Roquet-Banères, Jean-Louis Herrmann, Kevin Pethe, Laurent Kremer
Pulmonary infections caused by Mycobacterium abscessus are emerging as a global threat, especially in cystic fibrosis patients. Further intensifying the concern of M. abscessus infection is the recent evidence of human-to-human transmission of the infection. M. abscessus is a naturally multidrug resistant, fast-growing pathogen for which pharmacological options are limited. Repurposing antitubercular drugs represents an attractive option for the development of chemotherapeutic alternatives against M. abscessus infections...
August 14, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28806187/pain-is-an-underestimated-symptom-in-cystic-fibrosis
#8
Alexandra Masson, Maya Kirszembaum, Isabelle Sermet-Gaudelus
PURPOSE OF REVIEW: Life expectancy is increasing in cystic fibrosis and new aspects of the disease have to be taken into account in cystic fibrosis care. RECENT FINDINGS: Pain is encountered among 70% of adult and pediatric patients with cystic fibrosis. This symptom is underestimated by the multidisciplinary team. It has been reported as impacting quality of life and adherence to treatments. The location of pain is inconstant among the different studies but the major symptoms are headaches, gastrointestinal, and chest pain...
August 12, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28805948/pathogenic-role-of-adgrg2-in-cbavd-patients-replicated-in-chinese-population
#9
B Yang, J Wang, W Zhang, H Pan, T Li, B Liu, H Li, B Wang
Congenital bilateral absence of the vas deferens (CBAVD) is an important cause of obstructive azoospermia and male infertility worldwide. Cystic fibrosis transmembrane conductance regulator (CFTR) mutations are the main pathogenic cause, although a proportion of cases are still unexplained. Recently, adhesion G protein-coupled receptor G2 (ADGRG2) gene, a novel pathogenic gene for CBAVD was identified. We did a single population replication study in Chinese CBAVD patients to replicate its role in CBAVD developing...
August 14, 2017: Andrology
https://www.readbyqxmd.com/read/28805732/the-mechanistic-links-between-insulin-and-cystic-fibrosis-transmembrane-conductance-regulator-cftr-cl-channel
#10
REVIEW
Yoshinori Marunaka
The cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel belongs to the ATP-binding cassette (ABC) transporter superfamily and regulates Cl(-) secretion in epithelial cells for water secretion. Loss-of-function mutations to the CFTR gene cause dehydrated mucus on the apical side of epithelial cells and increase the susceptibility of bacterial infection, especially in the airway and pulmonary tissues. Therefore, research on the molecular properties of CFTR, such as its gating mechanism and subcellular trafficking, have been intensively pursued...
August 14, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28804700/abridged-version-of-the-awmf-guideline-for-the-medical-clinical-diagnostics-of-indoor-mould-exposure-s2k-guideline-of-the-german-society-of-hygiene-environmental-medicine-and-preventive-medicine-ghup-in-collaboration-with-the-german-association-of-allergists
#11
Gerhard A Wiesmüller, Birger Heinzow, Ute Aurbach, Karl-Christian Bergmann, Albrecht Bufe, Walter Buzina, Oliver A Cornely, Steffen Engelhart, Guido Fischer, Thomas Gabrio, Werner Heinz, Caroline E W Herr, Jörg Kleine-Tebbe, Ludger Klimek, Martin Köberle, Herbert Lichtnecker, Thomas Lob-Corzilius, Rolf Merget, Norbert Mülleneisen, Dennis Nowak, Uta Rabe, Monika Raulf, Hans Peter Seidl, Jens-Oliver Steiß, Regine Szewszyk, Peter Thomas, Kerttu Valtanen, Julia Hurraß
This article is an abridged version of the AWMF mould guideline "Medical clinical diagnostics of indoor mould exposure" presented in April 2016 by the German Society of Hygiene, Environmental Medicine and Preventive Medicine (Gesellschaft für Hygiene, Umweltmedizin und Präventivmedizin, GHUP), in collaboration with the above-mentioned scientific medical societies, German and Austrian societies, medical associations and experts. Indoor mould growth is a potential health risk, even if a quantitative and/or causal relationship between the occurrence of individual mould species and health problems has yet to be established...
2017: Allergo Journal International
https://www.readbyqxmd.com/read/28803050/the-arest-cf-experience-in-biobanking-more-than-just-tissues-tubes-and-time
#12
Luke W Garratt, Anthony Kicic, Colin Robertson, Sarath Ranganathan, Peter D Sly, Stephen M Stick
Research to further improve outcomes for people with CF is dependent upon well characterised, archived and accessible clinical specimens. The recent article by Beekman et al. published in Journal of Cystic Fibrosis summarised a scientific meeting at the 13th ECFS Basic Science Conference. This meeting discussed how well-annotated, clinical biobanks for CF could be established in Europe to meet the needs of therapeutic development. The Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) has conducted biobanking of CF research and clinical specimens since the late 1990s and is custodian of the most comprehensive paediatric CF biobank in the world that focuses on the first years of life...
August 9, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28802361/functional-endoscopic-sinus-surgery-improves-the-quality-of-life-in-children-suffering-from-chronic-rhinosinusitis-with-nasal-polyps
#13
Melina Fetta, Nikolaos S Tsilis, John V Segas, Thomas P Nikolopoulos, Petros V Vlastarakos
OBJECTIVE: To evaluate the impact of FESS in children with chronic rhinosinusitis with nasal polyps, regarding their overall postoperative quality-of-life (QoL) and constituent QoL domains. Potential differences between cystic fibrosis (CF) sufferers and non-sufferers, or cases with recurrent sinonasal polyposis versus single-operations were also explored. METHODS: 39 children were studied. The mean patient age was 10.9 years; four children suffered from cystic fibrosis...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28802277/microbiome-in-lung-explants-of-idiopathic-pulmonary-fibrosis-a-case-control-study-in-patients-with-end-stage-fibrosis
#14
Georgios D Kitsios, Mauricio Rojas, Daniel J Kass, Adam Fitch, John C Sembrat, Shulin Qin, Kristen L Veraldi, Kevin F Gibson, Kathleen Lindell, Joseph M Pilewski, Barbara Methe, Kelvin Li, John McDyer, Bryan J McVerry, Alison Morris
The microbiome has been proposed to play a role in the progression of idiopathic pulmonary fibrosis (IPF) based on bronchoalveolar lavage analyses, but the microbiome of lung tissue in IPF has not been explored. In a case-control study of lung explants analysed by 16S rRNA gene sequencing, we could not reliably detect bacterial DNA in basilar tissue samples from patients with either chronic or acute exacerbations of IPF, in contrast to control candidate-donor lungs or cystic fibrosis explants. Thus, our data do not indicate microbiome alterations in regions of IPF lung with advanced fibrosis...
August 11, 2017: Thorax
https://www.readbyqxmd.com/read/28800360/prerequisites-for-a-dry-powder-inhaler-for-children-with-cystic-fibrosis
#15
Anne J Lexmond, Paul Hagedoorn, Henderik W Frijlink, Bart L Rottier, Anne H de Boer
Correct inhalation technique is essential for effective use of dry powder inhalers (DPIs), as their effectiveness largely depends on the patient's inhalation manoeuvre. Children are an especially challenging target population for DPI development due to the large variability in understanding and inspiratory capacities. We previously performed a study in which we determined the prerequisites for a paediatric DPI in a mostly healthy paediatric population, for which we used an empty test inhaler with variable internal airflow resistance and mouthpiece...
2017: PloS One
https://www.readbyqxmd.com/read/28800247/reply-from-the-authors-of-comparison-of-lung-clearance-index-and-magnetic-resonance-imaging-for-assessment-of-lung-disease-in-children-with-cystic-fibrosis
#16
Mirjam Stahl, Mark O Wielpütz, Hans-Ulrich Kauczor, Marcus A Mall
No abstract text is available yet for this article.
August 11, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28800245/the-respective-roles-of-lci-and-mri-in-the-clinical-management-of-cystic-fibrosis-patients
#17
Sylvia Verbanck, Eef Vanderhelst
No abstract text is available yet for this article.
August 11, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28800122/increased-expression-of-plasma-induced-abcc1-mrna-in-cystic-fibrosis
#18
Justin E Ideozu, Xi Zhang, Amy Pan, Zainub Ashrafi, Katherine J Woods, Martin J Hessner, Pippa Simpson, Hara Levy
The ABCC1 gene is structurally and functionally related to the cystic fibrosis transmembrane conductance regulator gene (CFTR). Upregulation of ABCC1 is thought to improve lung function in patients with cystic fibrosis (CF); the mechanism underlying this effect is unknown. We analyzed the ABCC1 promoter single nucleotide polymorphism (SNP rs504348), plasma-induced ABCC1 mRNA expression levels, and ABCC1 methylation status and their correlation with clinical variables among CF subjects with differing CFTR mutations...
August 11, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28799841/high-usability-of-a-smartphone-application-for-reporting-symptoms-in-adults-with-cystic-fibrosis
#19
Jamie Wood, Sue Jenkins, David Putrino, Siobhain Mulrennan, Sue Morey, Nola Cecins, Kylie Hill
Introduction In cystic fibrosis, exacerbations impair lung function and health-related quality of life, increase healthcare costs and reduce survival. Delayed reporting of worsening symptoms can result in more severe exacerbations and worse clinical outcomes; therefore there is a need for a novel approach to facilitate the early identification and treatment of exacerbations in this population. This study investigated the usability of a smartphone application to report symptoms in adults with cystic fibrosis, and the observer agreement in clinical decision-making between senior clinicians interpreting smartphone application responses...
January 1, 2017: Journal of Telemedicine and Telecare
https://www.readbyqxmd.com/read/28797493/allergic-and-noninvasive-infectious-pulmonary-aspergillosis-syndromes
#20
REVIEW
Eavan G Muldoon, Mary E Strek, Karen C Patterson
Aspergillus spp are ubiquitous in the environment, and inhalation of Aspergillus spores is unavoidable. An intact immune system, with normal airway function, protects most people from disease. Globally, however, the toll from aspergillosis is high. The literature has largely focused on invasive aspergillosis, yet the burden in terms of chronicity and prevalence is higher for noninvasive Aspergillus conditions. This article discusses allergic aspergilloses and provides an update on the diagnosis and management of allergic bronchopulmonary aspergillosis, including in patients with cystic fibrosis, and an update on severe asthma with fungal sensitization...
September 2017: Clinics in Chest Medicine
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