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Cystic Fibrosis

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https://www.readbyqxmd.com/read/28549610/combined-lung-liver-pancreas-transplantation-in-a-recipient-with-cystic-fibrosis
#1
A S Barbas, M J Dib, D P Al-Adra, N Goldaracena, G Sapisochin, T K Waddell, S Keshavjee, N Selzner, C Chaparro, M S Cattral
Cystic fibrosis (CF) affects multiple organs including the lung, liver, and pancreas. Lung transplant, liver transplant, and combined lung-liver transplant have become well-established therapies for CF patients with end-stage organ failure. Thus far, however, there has been limited experience with pancreas transplantation in CF. In this report, we detail the clinical history, transplant procedure, and post-operative recovery of a patient who underwent combined lung-liver-pancreas transplant for advanced CF.
May 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28549609/development-and-validation-of-cf-medication-beliefs-questionnaire-a-mixed-methods-approach
#2
Michelle N Eakin, Shang-En Chung, Jessica Hoehn, Belinda Borrelli, Devin Rand-Giovannetti, Kristin A Riekert
BACKGROUND: Beliefs about medication have been associated with adherence in other diseases but there are no existing disease-specific medication beliefs questionnaires for CF. This mixed-methods validated the Cystic Fibrosis Medication Belief Questionnaire (CF-MBQ), based on social cognitive theory. METHODS: Based on previous research, items were developed for five domains: motivation, self-efficacy, perceived importance, and decisional balance to take or miss medications...
May 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28549169/regulatory-dynamics-of-11p13-suggest-a-role-for-ehf-in-modifying-cf-lung-disease-severity
#3
Lindsay R Stolzenburg, Rui Yang, Jenny L Kerschner, Sara Fossum, Matthew Xu, Andrew Hoffmann, Kay-Marie Lamar, Sujana Ghosh, Sarah Wachtel, Shih-Hsing Leir, Ann Harris
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF), but are not good predictors of lung phenotype. Genome-wide association studies (GWAS) previously identified additional genomic sites associated with CF lung disease severity. One of these, at chromosome 11p13, is an intergenic region between Ets homologous factor (EHF) and Apaf-1 interacting protein (APIP). Our goal was to determine the functional significance of this region, which being intergenic is probably regulatory...
May 26, 2017: Nucleic Acids Research
https://www.readbyqxmd.com/read/28548990/outbreaks-of-nontuberculous-mycobacteria
#4
Geeta Sood, Nikki Parrish
PURPOSE OF REVIEW: The purpose of this review is to summarize the emerging literature on nontuberculous mycobacteria outbreaks in healthcare settings. As our ability to identify mycobacterial species develops, we are better able to recognize epidemiologic connections and better understand the prevalence and importance of these outbreaks and pseudo-outbreaks in healthcare settings. RECENT FINDINGS: The number of outbreaks related to nontuberculous outbreaks is increasing because of heightened awareness and better diagnostic tests for species level identification of mycobacteria...
May 25, 2017: Current Opinion in Infectious Diseases
https://www.readbyqxmd.com/read/28548661/co-evolution-with-staphylococcus-aureus-leads-to-lipopolysaccharide-alterations-in-pseudomonas-aeruginosa
#5
Mikael Tognon, Thilo Köhler, Bartosz G Gdaniec, Youai Hao, Joseph S Lam, Marie Beaume, Alexandre Luscher, Angus Buckling, Christian van Delden
Detrimental and beneficial interactions between co-colonizing bacteria may influence the course of infections. In cystic fibrosis (CF) airways, Staphylococcus aureus prevails in childhood, whereas Pseudomonas aeruginosa progressively predominates thereafter. While a range of interactions has been identified, it is unclear if these represent specific adaptations or correlated responses to other aspects of the environment. Here, we investigate how P. aeruginosa adapts to S. aureus by evolving P. aeruginosa in the presence and absence of S...
May 26, 2017: ISME Journal
https://www.readbyqxmd.com/read/28547678/immunosuppression-drug-therapy-in-lung-transplantation-for-cystic-fibrosis
#6
REVIEW
Pamela Burcham, Lisa Sarzynski, Sabrina Khalfoun, Kimberly J Novak, Julie C Miller, Dmitry Tumin, Don Hayes
Cystic fibrosis (CF) is a common indication for lung transplantation (LTx) in children and adults with severe and irreversible lung disease. In the setting of LTx in the CF population, immunosuppressive medications are used to prevent allograft rejection despite the majority of these patients being chronically infected with numerous, and often antibiotic-resistant, pathogens. There is limited evidence for the optimal post-LTx immunosuppression regimen in patients with CF, particularly in children. This article provides a review of immunosuppression regimens in the pediatric and adult CF post-LTx population, investigating drug dosing and monitoring, and medication combinations...
May 25, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28546993/nationwide-genetic-analysis-for-molecularly-unresolved-cystic-fibrosis-patients-in-a-multiethnic-society-implications-for-preconception-carrier-screening
#7
Doron M Behar, Ori Inbar, Michal Shteinberg, Michal Gur, Huda Mussaffi, David Shoseyov, Moshe Ashkenazi, Soliman Alkrinawi, Concetta Bormans, Fahed Hakim, Meir Mei-Zahav, Malena Cohen-Cymberknoh, Adi Dagan, Dario Prais, Ifat Sarouk, Patrick Stafler, Bat El Bar Aluma, Gidon Akler, Elie Picard, Micha Aviram, Ori Efrati, Galit Livnat, Joseph Rivlin, Lea Bentur, Hannah Blau, Eitan Kerem, Amihood Singer
BACKGROUND: Preconception carrier screening for cystic fibrosis (CF) is usually performed using ethnically targeted panels of selected mutations. This has been recently challenged by the use of expanded, ethnically indifferent, pan-population panels. Israel is characterized by genetically heterogeneous populations carrying a wide range of CFTR mutations. To assess the potential of expanding the current Israeli preconception screening program, we sought the subset of molecularly unresolved CF patients listed in the Israeli CF data registry comprising ~650 patients...
May 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/28546419/direct-binding-of-the-corrector-vx-809-to-human-cftr-nbd1-evidence-of-an-allosteric-coupling-between-the-binding-site-and-the-nbd1-cl4-interface
#8
Rhea P Hudson, Jennifer E Dawson, P Andrew Chong, Zhengrong Yang, Linda Millen, Philip J Thomas, Christie G Brouillette, Julie D Forman-Kay
Understanding the mechanism of action of modulator compounds for the cystic fibrosis transmembrane conductance regulator (CFTR) is key for optimization of therapeutics as well as obtaining insights into the molecular mechanisms of CFTR function. We demonstrate direct binding of VX-809 to the first nucleotide-binding domain (NBD1) of human CFTR. Disruption of the interaction between C-terminal helices and the NBD1 core upon VX-809 binding is observed from chemical shift changes in the NMR spectra of residues in the helices and on the surface of β-strands S3, S9 and S10...
May 25, 2017: Molecular Pharmacology
https://www.readbyqxmd.com/read/28545684/mri-of-cystic-fibrosis-lung-manifestations-sequence-evaluation-and-clinical-outcome-analysis
#9
O Scholz, T Denecke, J Böttcher, C Schwarz, H-J Mentzel, F Streitparth, M H Maurer, A Pfeil, A Huppertz, A Mehl, D Staab, B Hamm, D M Renz
AIM: To evaluate different magnetic resonance imaging (MRI) sequences for diagnosis of pulmonary manifestations of cystic fibrosis (CF) in comparison to chest computed tomography (CT), including an extended outcome analysis. MATERIALS AND METHODS: Twenty-eight patients with CF (15 male, 13 female, mean age 30.5±9.4 years) underwent CT and MRI of the lung. MRI (1.5 T) included different T2- and T1-weighted sequences: breath-hold HASTE (half Fourier acquisition single shot turbo spin echo) and VIBE (volumetric interpolated breath-hold examination, before and after contrast medium administration) sequences and respiratory-triggered PROPELLER (periodically rotated overlapping parallel lines with enhanced reconstruction) sequences with and without fat signal suppression, and perfusion imaging...
May 22, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28545452/a-simple-fast-and-inexpensive-method-for-mutation-scanning-of-cftr-gene
#10
Juan Emilio Figueredo Lago, Anny Armas Cayarga, Yaimé Josefina González González, Teresa Collazo Mesa
BACKGROUND: Mutation scanning methods in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene may not distinguish between a Cystic Fibrosis (CF) causing mutation and a benign variant. We have developed a simple and fast method for scanning 14 selected CF-causing mutations which have high frequency in Latin America. METHODS: In a group of 35 samples coming from CF patients previously characterized and using two allele-specific real-time multiplex PCRs targeting wild-type and mutant alleles respectively, we detect the presence of mutations by analyzing the Ct variation...
May 25, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28544683/comprehensive-genetic-study-of-cystic-fibrosis-in-slovak-patients-in-25-years-of-genetic-diagnostics
#11
Andrea Soltysova, Eva Tothova Tarova, Andrej Ficek, Marian Baldovic, Helena Polakova, Hana Kayserova, Ludevit Kadasi
INTRODUCTION: Cystic fibrosis (CF) has one of the longest histories in hereditary disease molecular diagnostics. However, identification of causative mutations in the CFTR gene is complicated by over two thousand currently identified mutations; with more still being discovered. Knowledge of mutation spectrum may improve effective routine diagnostics and is obligatory in mutation-specific treatment. OBJECTIVES: This study presents comprehensive mutation screening of the CFTR gene; with 275 unrelated, clinically confirmed and treated cystic fibrosis (CF) patients diagnosed in 25 years genetic testing in Slovakia...
May 20, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28544189/year-in-review-2016-interstitial-lung-disease-pulmonary-vascular-disease-pulmonary-function-paediatric-lung-disease-cystic-fibrosis-and-sleep
#12
REVIEW
Adelle S Jee, Tamera J Corte, Stephen J Wort, Neil D Eves, Claire E Wainwright, Amanda Piper
No abstract text is available yet for this article.
May 25, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28543609/understanding-the-school-experiences-of-children-and-adolescents-with-serious-chronic-illness-a-systematic-meta-review
#13
REVIEW
A Lum, C E Wakefield, B Donnan, M A Burns, J E Fardell, G M Marshall
BACKGROUND: Serious chronic illness can have a detrimental effect on school attendance, participation and engagement, leaving affected students at risk of failing to meet their developmental potential. An improved understanding of factors that help to explain or mitigate this risk can help educators and health professionals deliver the most effective support. This meta-review critiqued the available evidence examining the link between six chronic illnesses (asthma, cancer, chronic kidney diseases, heart diseases, cystic fibrosis and gastrointestinal diseases) and children's and adolescents' school experiences and outcomes, as well as investigating the medical, school, psychosocial and sociodemographic factors that are linked to poorer or better school outcomes...
May 23, 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/28542554/sodium-tanshinone-iia-sulfonate-stimulated-cl-secretion-in-mouse-trachea
#14
Peng-Xiao Chen, Yi-Lin Zhang, Jia-Wen Xu, Ming-Hao Yu, Jie-Hong Huang, Lei Zhao, Wen-Liang Zhou
Sodium tanshinone IIA sulfonate (STS) is a derivate of tanshinone IIA, a lipophilic compound in Salvia miltiorrhiza. This study aimed to investigate the effect of STS on ion transport in mouse tracheal epithelium and the mechanisms underlying it. Short-circuit current (Isc) was measured to evaluate the effect of STS on transepithelial ion transport. Intracellular Ca2+ imaging was performed to observe intracellular Ca2+ concentration ([Ca2+]i) changes induced by STS in primary cultured mouse tracheal epithelial cells...
2017: PloS One
https://www.readbyqxmd.com/read/28542286/clinical-impact-of-chronic-obstructive-pulmonary-disease-on-non-cystic-fibrosis-bronchiectasis-a-study-on-1-790-patients-from-the-spanish-bronchiectasis-historical-registry
#15
David De la Rosa, Miguel-Angel Martínez-Garcia, Rosa Maria Giron, Montserrat Vendrell, Casilda Olveira, Luis Borderias, Luis Maiz, Antoni Torres, Eva Martinez-Moragon, Olga Rajas, Francisco Casas, Rosa Cordovilla, Javier de Gracia
BACKGROUND: Few studies have evaluated the coexistence of bronchiectasis (BE) and chronic obstructive pulmonary disease (COPD) in series of patients diagnosed primarily with BE. The aim of this study was to analyse the characteristics of patients with BE associated with COPD included in the Spanish Bronchiectasis Historical Registry and compare them to the remaining patients with non-cystic fibrosis BE. METHODS: We conducted a multicentre observational study of historical cohorts, analysing the characteristics of 1,790 patients who had been included in the registry between 2002 and 2011...
2017: PloS One
https://www.readbyqxmd.com/read/28541746/the-evolving-cystic-fibrosis-microbiome-a-comparative-cohort-study-spanning-sixteen-years
#16
Nicole Acosta, Fiona J Whelan, Ranjani Somayaji, Ali Poonja, Michael G Surette, Harvey R Rabin, Michael D Parkins
RATIONALE: The cystic fibrosis (CF) airways are infected with a diverse polymicrobial community. OBJECTIVES: Understanding how changes in the CF microbiome have occurred over time, similar to the observed changes in the prevalence of cultured pathogens, is key in understanding the microbiome's role in disease. METHODS: Drawing from a prospectively collected and maintained sputum biobank, we identified 45 patients with sputum samples collected between the ages of 18-21 years in three successive cohorts of adults transitioning to our CF clinic: A(1997-2000), B(2004-2007), and C(2010-2013)...
May 25, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28540765/an-update-on-pediatric-bronchiectasis
#17
Danielle F Wurzel, Anne B Chang
The prevalence and awareness of bronchiectasis not related to cystic fibrosis (CF) is increasing and it is now recognized as a major cause of respiratory morbidity, mortality and healthcare utilization worldwide. The need to elucidate the early origins of bronchiectasis is increasingly appreciated and has been identified as an important research priority. Current treatments for pediatric bronchiectasis are limited to antimicrobials, airway clearance techniques and vaccination. Several new drugs targeting airway inflammation are currently in development...
May 25, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28540046/evaluation-of-a-novel-technique-in-airway-clearance-therapy-specific-cough-technique-sct-in-cystic-fibrosis-a-pilot-study-of-a-series-of-n-of-1-randomised-controlled-trials
#18
Sandra Gursli, Leiv Sandvik, Egil Bakkeheim, Bjørn Skrede, Britt Stuge
OBJECTIVES: The aim of this pilot study was to evaluate the efficacy, safety and participants' perception of a novel technique in airway clearance therapy - specific cough technique in cystic fibrosis. METHODS: We conducted randomised controlled individual trials (N-of-1 randomised controlled trials) in six adults. Each trial included 8 weeks of treatment with two interventions each week, one with specific cough technique and one with forced expiration technique...
2017: SAGE Open Medicine
https://www.readbyqxmd.com/read/28539697/physical-activity-levels-in-individuals-with-cystic-fibrosis-related-diabetes
#19
Sarah Currie, Krista Greenwood, Lara Weber, Hussein Khakee, Michelle Legasto, Elizabeth Tullis, Kenneth Wu, Anna Tsang, Erika Szego, Sunita Mathur
Purpose: The literature on physical activity (PA) in adults with cystic fibrosis, particularly in those with cystic fibrosis-related diabetes (CFRD), is limited. PA may be an important part of blood glucose management in CFRD. The purpose of this study was to describe PA levels in adults with CFRD and determine their adherence to the Canadian Diabetes Association (CDA) aerobic exercise training guidelines. Methods: Adults with CFRD were recruited from a hospital-based CF clinic. PA was measured using the Seven-Day Physical Activity Recall (telephone interview), adherence to CFRD management with the Self-Care Inventory-Revised (questionnaire), and blood glucose control from glycated hemoglobin levels documented in participants' medical chart within 3 months...
2017: Physiotherapy Canada. Physiothérapie Canada
https://www.readbyqxmd.com/read/28539256/anorexia-nervosa-in-cystic-fibrosis
#20
REVIEW
Lynette Linkson, Patricia Macedo, Felicity M R Perrin, Caroline M Elston
This article explores the challenges associated with diagnosing and managing eating disorders such as anorexia nervosa amongst adolescents and adults with cystic fibrosis. It reviews the known risk factors, generic verses disease specific eating disorder risk screening tools and considers the ethical dilemmas associated with critically low body mass indices. A case review is included to illustrate the complexities of managing both conditions in the context of declining respiratory function.
March 23, 2017: Paediatric Respiratory Reviews
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