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Cystic Fibrosis

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https://www.readbyqxmd.com/read/27933179/associations-between-genetics-medical-status-physical-exercise-and-psychological-well-being-in-adults-with-cystic-fibrosis
#1
Lena Backström-Eriksson, Agneta Bergsten-Brucefors, Lena Hjelte, Bo Melin, Kimmo Sorjonen
BACKGROUND: Cystic fibrosis (CF) is the most common autosomal recessive, life-shortening disease among people of European origin. Type of genetic mutation and regular physical exercise has an impact on clinical outcome. This cross-sectional study explores the associations between genetics, medical status, physical exercise and psychological well-being in adult patients with CF. METHODS: Adult patients with CF (N=68; mean age: 32.2; range 18-67 years; 46% women) completed the Cystic Fibrosis Questionnaire-Revised and Hospital Anxiety Depression Scale...
2016: BMJ Open Respiratory Research
https://www.readbyqxmd.com/read/27932986/role-of-quercetin-in-modulating-chloride-transport-in-the-intestine
#2
Bo Yu, Yu Jiang, Lingling Jin, Tonghui Ma, Hong Yang
Epithelial chloride channels provide the pathways for fluid secretion in the intestine. Cystic fibrosis transmembrane conductance regulator (CFTR) and calcium-activated chloride channels (CaCCs) are the main chloride channels in the luminal membrane of enterocytes. These transmembrane proteins play important roles in many physiological processes. In this study, we have identified a flavonoid quercetin as a modulator of CaCC chloride channel activity. Fluorescence quenching assay showed that quercetin activated Cl(-) transport in a dose-dependent manner, with EC50 ~37 μM...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27931123/developments-in-multibreath-washout-testing-in-children-with-cystic-fibrosis
#3
Clare Saunders, Katie Bayfield, Samantha Irving, Christopher Short, Andrew Bush, Jane C Davies
Lung Clearance Index (LCI) is becoming recognized as an important addition in the monitoring of pediatric cystic fibrosis (CF). The non-invasive technique is easy to perform in all ages, reproducible and increasingly being used in clinical trials. There is interest to utilize it within the clinic setting but its current use is mostly as a research tool. The procedure is highly dependent on skilled operators and a relaxed testing environment is key to obtaining good quality measurements. Standardization of LCI is part of an ongoing collaborative, multicenter process...
December 9, 2016: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/27931057/-quantification-of-ventilation-inflammation-perfusion-and-structure-vips
#4
J Renne, J Vogel-Claussen
Technical innovation in pulmonary imaging during the recent years has led to a shift from morphological description of pulmonary pathologies to regional quantification of pulmonary function. This article summarizes current clinical standards in pulmonary imaging and introduces the reader to new innovative techniques of functional lung imaging. In the context of actual clinical studies for COPD, asthma, pulmonary hypertension and cystic fibrosis the application of quantitative imaging methods using computed tomography and magnetic resonance imaging is demonstrated and possible future applications are discussed...
December 2016: Pneumologie
https://www.readbyqxmd.com/read/27930727/airway-microbiota-in-bronchoalveolar-lavage-fluid-from-clinically-well-infants-with-cystic-fibrosis
#5
Theresa A Laguna, Brandie D Wagner, Cynthia B Williams, Mark J Stevens, Charles E Robertson, Cole W Welchlin, Catherine E Moen, Edith T Zemanick, Jonathan K Harris
BACKGROUND: Upper airway cultures guide the identification and treatment of lung pathogens in infants with cystic fibrosis (CF); however, this may not fully reflect the spectrum of bacteria present in the lower airway. Our objectives were to characterize the airway microbiota using bronchoalveolar lavage fluid (BALF) from asymptomatic CF infants during the first year of life and to investigate the relationship between BALF microbiota, standard culture and clinical characteristics. METHODS: BALF, nasopharyngeal (NP) culture and infant pulmonary function testing data were collected at 6 months and one year of age during periods of clinical stability from infants diagnosed with CF by newborn screening...
2016: PloS One
https://www.readbyqxmd.com/read/27927212/change-in-pseudomonas-aeruginosa-prevalence-in-cystic-fibrosis-adults-over-time
#6
Mathew R Crull, Kathleen J Ramos, Ellen Caldwell, Nicole Mayer-Hamblett, Moira L Aitken, Christopher H Goss
BACKGROUND: Little is known about risk factors for chronic and mucoid Pseudomonas aeruginosa (Pa) infection in cystic fibrosis (CF) adults, and whether the prevalence is changing. METHODS: We employed a retrospective cohort to analyze data from a single adult CF center (2002 to 2012). Regression models were used to assess independent predictors and change in prevalence of chronic and mucoid Pa infection over time. RESULTS: The odds ratio of mucoid Pa infection was significantly less in individuals with better baseline lung function (OR 0...
December 7, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27927050/the-use-of-multiple-breath-washout-for-assessing-cystic-fibrosis-in-infants
#7
Gwyneth Davies, Paul Aurora
Lung Clearance Index, measured using the multiple breath washout (MBW) technique, may be a useful test in infants with Cystic Fibrosis (CF). However, the requirement for specialised equipment and a number of important technical and methodological considerations relevant to testing in infants have complicated matters, and to date prevented its widespread translatability in this age group. Areas covered: We review the current status of infant MBW testing in CF, focusing on Lung Clearance Index. This includes a review of recent developments in the field relevant to testing methodology in the infant population, use in evaluating lung disease in CF in infancy, and the associated challenges which remain...
December 7, 2016: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/27927014/genetic-modification-of-the-lung-directed-toward-treatment-of-human-disease
#8
Dolan Sondhi, Katie Stiles, Bishnu P De, Ronald G Crystal
Genetic modification therapy is a promising therapeutic strategy for many diseases of the lung intractable to other treatments. Lung gene therapy has been the subject of numerous preclinical animal experiments and human clinical trials, for targets including genetic diseases such as cystic fibrosis and α1-antitrypsin deficiency, complex disorders such as asthma, allergy and lung cancer, infections such as respiratory syncytial virus and Pseudomonas, as well as pulmonary arterial hypertension, transplant rejection and lung injury...
December 7, 2016: Human Gene Therapy
https://www.readbyqxmd.com/read/27925791/reemergence-of-lower-airway-microbiota-in-lung-transplant-patients-with-cystic-fibrosis
#9
Saad A Syed, Fiona J Whelan, Barbara Waddell, Harvey R Rabin, Michael D Parkins, Michael G Surette
RATIONALE: Chronic lung infections are a hallmark of cystic fibrosis; they are responsible for progressive airway destruction and ultimately lead to respiratory death or the requirement for life-saving bilateral lung transplant. Furthermore, recurrent isolation of airway pathogens such as Pseudomonas aeruginosa in the allograft after transplant is associated with adverse outcomes, including bronchiolitis obliterans syndrome and acute infections. Little information exists on the impact of bilateral lung transplant on the lower-airway microbiota...
December 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27925783/constrictive-bronchiolitis-a-distinct-phenotype-of-cystic-fibrosis-lung-disease
#10
Jonathan H Rayment, Felix Ratjen
No abstract text is available yet for this article.
December 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27925782/changes-in-the-cystic-fibrosis-airway-microbiome-after-lung-transplant-the-more-things-change-the-more-they-stay-the-same
#11
Shawn D Aaron
No abstract text is available yet for this article.
December 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27925453/chest-computed-tomography-scores-in-patients-with-cystic-fibrosis-colonized-with-methicillin-resistant-staphylococcus-aureus-mrsa
#12
Michal Gur, Elena Spinelli, Glorida Tridello, Susanna Baltieri, Lucia Pinali, Stefania Montemezzi, Lea Bentur, Baroukh Maurice Assael
BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) is an important pathogen in Cystic Fibrosis (CF), with increasing incidence in recent years. We examined the association between bacterial colonization in the sputum (MRSA with or without pseudomonas (PA)) and computed tomography (CT) scores in CF patients. METHODS: MRSA patients were divided according to PA status based on at least three consecutive sputum cultures; controls were patients without MRSA (with or without PA), matched for gender and age at CT...
December 7, 2016: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/27925430/pulmonary-hypertension-as-estimated-by-doppler-echocardiography-in-adolescent-and-adult-patients-with-cystic-fibrosis-and-their-relationship-with-clinical-lung-function-and-sleep-findings
#13
Bruna Ziegler, Christiano Perin, Fernanda Cano Casarotto, Simone Chaves Fagondes, Sérgio Saldanha Menna-Barreto, Paulo de Tarso Roth Dalcin
BACKGROUND: In cystic fibrosis (CF) patients, end stage of pulmonary disease is characterized by pulmonary hypertension (PH), hypoxemia, decrease in exercise tolerance and sleep quality. OBJECTIVE: To evaluate the association between clinical, lung function, sleep quality and polysomnographic variables with PH in CF patients aged 16 years or older. METHODS: In a cross-sectional study, 51 clinically stable CF patients underwent a clinical evaluation, an overnight polysomnography and answered sleep questionnaires (Pittsburgh Sleep Quality Index - PSQI and Epworth sleepiness scale - ESS)...
December 7, 2016: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/27924468/whether-a-novel-drug-delivery-system-can-overcome-the-problem-of-biofilms-in-respiratory-diseases
#14
REVIEW
Kamal Dua, Shakti D Shukla, Rakesh K Tekade, Philip M Hansbro
Biofilm comprises a community of microorganisms which form on medical devices and can lead to various threatening infections. It is a major concern in various respiratory diseases like cystic fibrosis, chronic obstructive pulmonary disease, etc. The treatment strategies for such infections are difficult due to the resistance of the microflora existing in the biofilms against various antimicrobial agents, thus posing threats to the patient population. The present era witnesses the beginning of research to understand the biofilm physiology and the associated microfloral diversity by applying -omics approaches...
December 6, 2016: Drug Delivery and Translational Research
https://www.readbyqxmd.com/read/27922234/long-term-azithromycin-therapy-in-patients-with-cystic-fibrosis
#15
Nagehan Emiralioğlu, Zeynelabidin Öztürk, Ebru Yalçın, Deniz Doğru, Uğur Özçelik, Nural Kiper
Inflammation is a central contributor to the pathogenesis of cystic fibrosis (CF) pulmonary disease; so limiting the excessive production of inflammatory mediators represents a major therapeutic strategy for slowing the decline in lung function and improving survival. The macrolide antibiotic azithromycin (AZM) has anti-inflammatory properties and immunomodulatory effects that may be beneficial in CF. The aim of this study was to document the long term use of AZM effect on pulmonary function, nutritional status and number of pulmonary exacerbations in patients with CF...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27922233/diagnosis-of-cystic-fibrosis-with-chloride-meter-sherwood-m926s-chloride-analyzer%C3%A2-and-sweat-test-analysis-system-cf%C3%AE-collection-system%C3%A2-compared-to-the-gibson-cooke-method
#16
Nagehan Emiralioğlu, Uğur Özçelik, Ebru Yalçın, Deniz Doğru, Nural Kiper
Sweat test with Gibson Cooke (GC) method is the diagnostic gold standard for cystic fibrosis (CF). Recently, alternative methods have been introduced to simplify both the collection and analysis of sweat samples. Our aim was to compare sweat chloride values obtained by GC method with other sweat test methods in patients diagnosed with CF and whose CF diagnosis had been ruled out. We wanted to determine if the other sweat test methods could reliably identify patients with CF and differentiate them from healthy subjects...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27922232/changing-epidemiology-of-non-cystic-fibrosis-bronchiectasis
#17
Semiha Bahçeci, Sait Karaman, Hikmet Tekin Nacaroğlu, Selçuk Yazıcı, Saniye Girit, Şule Ünsal-Karkıner, Demet Can
Non-cystic fibrosis bronchiectasis again becomes a major health problem due to inappropriate antibiotic use and increasing frequency of protracted bacterial bronchitis. The aim was to determine the changes in etiology of bronchiectasis. Patients who admitted to Behçet Uz Children Hospital between 2005 and 2015 (n=110) were retrospectively examined. The etiology of bronchiectasis was detected as; primary ciliary dyskinesia 26.4%, protracted bacterial bronchitis 22.8%, primary immune deficiency 11.8%, bronchiolitis obliterans 8...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27921442/nebulized-antibiotics-in-mechanically-ventilated-patients-roadmap-and-challenges
#18
G Poulakou, G Siakallis, S Tsiodras, A Arfaras Melainis, G Dimopoulos
Nebulized antibiotics use has become common practice in the therapeutics of pneumonia in cystic fibrosis patients. There is an increasing interest in their use for respiratory infections in mechanically ventilated (MV) patients in order to a) overcome pharmacokinetic issues in the lung compartment with traditional systemic antibiotic use and b) prevent the emergence of multi-drug-resistant (MDR) pathogens. Areas covered: The beneficial effects of antibiotic nebulization in MV patients e.g. increasing efficacy, reduced toxicity and prevention of resistance are described...
December 6, 2016: Expert Review of Anti-infective Therapy
https://www.readbyqxmd.com/read/27921040/new-insights-into-the-molecular-mechanisms-targeting-tubular-channels-transporters-in-pkd-development
#19
REVIEW
Ming Wu, Shengqiang Yu
BACKGROUND: Autosomal dominant polycystic kidney disease (PKD) or autosomal recessive PKD is caused by a mutation in the PKD1, PKD2 or PKHD1 gene, which encodes polycystin-1, polycystin-2 or fibrocystin, respectively. Embryonic and postnatal mutation studies show that transport or channel function is dysregulated before the initiation of cystogenesis, suggesting that the abnormality of transport or channel function plays a critical role in the pathology of PKD. SUMMARY: Polycystin-2 by itself is a calcium-permeable cation channel, and its channel function can be regulated by polycystin-1 or fibrocystin...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27920537/factors-related-to-depression-and-anxiety-in-adults-with-bronchiectasis
#20
Elif Yelda Özgün Niksarlioglu, Gülcihan Özkan, Gülşah Günlüoğlu, Mehmet Atilla Uysal, Sule Gül, Lütfiye Kilic, Ayse Yeter, Güngör Çamsarı
INTRODUCTION AND BACKGROUND: Patients with chronic lung diseases frequently have depressive and anxiety symptoms, but there are very few studies looking at this in patients with bronchiectasis. AIM: This study aimed to investigate depression and anxiety and related factors among patients with non-cystic fibrosis bronchiectasis. PATIENTS AND METHODS: This was a prospective study of 133 patients with bronchiectasis. Patients with confirmed diagnosis of bronchiectasis with high-resolution computed tomography were enrolled in the study...
2016: Neuropsychiatric Disease and Treatment
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