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https://www.readbyqxmd.com/read/30554331/efficacy-and-safety-of-cftr-corrector-and-potentiator-combination-therapy-in-patients-with-cystic-fibrosis-for-the-f508del-cftr-homozygous-mutation-a-systematic-review-and-meta-analysis
#1
Hong-Xia Wu, Min Zhu, Xiao-Feng Xiong, Jia Wei, Kai-Quan Zhuo, De-Yun Cheng
INTRODUCTION: Cystic fibrosis (CF) is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. The combination of a cystic fibrosis transmembrane conductance regulator (CFTR) corrector and potentiator has provided a benefit by decreasing sweat chloride concentration in CF for the F508del-CFTR homozygous mutation, but it remains controversial in lung function, nutritional status, clinical score and safety. METHODS: The authors performed a systematic review and meta-analysis of randomized controlled trials (RCTs) to evaluate the efficacy and safety of combination therapy on lung function, nutritional status, clinical score and safety in CF for the F508del-CFTR homozygous mutation...
December 15, 2018: Advances in Therapy
https://www.readbyqxmd.com/read/30553644/-pulmonary-glial-heterotopia-unique-lesion-in-an-infant-with-anencephalic-twin
#2
Louis Tournier, Dominique Berrebi, Michel Peuchmaur, Arnaud Bonnard, Nadia Belarbi, Olivier Sibony, Guillaume Morcrette
Pulmonary glial heterotopia is an extremely rare entity whose pathophysiology remains unclear. We report for the first time one case of pulmonary glial heterotopia occured in a one-month baby free from any malformation. She has the particularity of being born from monozygotic monochorionic twin pregnancy where her anencephalic exencephalic sister suffered a medical termination of pregnancy. She presented neonatal respiratory distress, which recurred one month later. Chest X-ray revealed bilateral cystic pulmonary lesions mainly located in the right lung...
December 12, 2018: Annales de Pathologie
https://www.readbyqxmd.com/read/30553640/-autochthonous-hydatid-cyst-of-the-lung
#3
Morgan Tourne, Clairelyne Dupin, Pierre Mordant, Mathilde Neuville, Camille Taillé, Claire Danel
Cystic echinococcosis, or hydatidosis, also known as hydatid cyst, is a cosmopolitan parasitosis mainly present in breeding areas. This anthropozoonosis is related to the tissue development of an hydatid of an echinococcus tænia, Echinococcus granulosus, found in the digestive tract of canids, at the adult state. In France, this larval cestosis is essentially an import disease developed by patients from endemic areas such as East and North Africa, South America or Asia. However, autochtonous forms, although rare, still persist...
December 12, 2018: Annales de Pathologie
https://www.readbyqxmd.com/read/30550797/modulation-of-the-unfolded-protein-response-pathway-as-an-antiviral-approach-in-airway-epithelial-cells
#4
Aline Schögler, Oliver Caliaro, Melanie Brügger, Blandina I Oliveira Esteves, Izabela Nita, Amiq Gazdhar, Thomas Geiser, Marco P Alves
INTRODUCTION: Rhinovirus (RV) infection is a major cause of cystic fibrosis (CF) lung morbidity with limited therapeutic options. Various diseases involving chronic inflammatory response and infection are associated with endoplasmic reticulum (ER) stress and subsequent activation of the unfolded protein response (UPR), an adaptive response to maintain cellular homeostasis. Recent evidence suggests impaired ER stress response in CF airway epithelial cells, this might be a reason for recurrent viral infection in CF...
December 11, 2018: Antiviral Research
https://www.readbyqxmd.com/read/30548546/a-case-of-pan-resistant-burkholderia-cepacia-complex-bacteraemic-pneumonia-after-lung-transplantation-treated-with-a-targeted-combination-therapy
#5
Mª Luisa Cantón-Bulnes, Ángela Hurtado Martínez, Lorena López-Cerero, Ángel Arenzana Seisdedos, Vicente Merino-Bohorquez, Servicio de Farmacia, José Garnacho-Montero
We describe a case of one patient with cystic fibrosis who developed a pan-resistant Burkholderia Cepacia Complex rapidly progressive bacteraemic pneunonia, following bilateral lung transplantation. Patient was treated with a targeted combination antibiotic therapy (meropenem plus ceftazidime/avibactam plus high doses of nebulized colistimethate sodium). The disease evolution was complicated by multiple organ system dysfunction. Finally, clinical improvement and microbiological cure was achieved. This article is protected by copyright...
December 12, 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/30546859/mechanistic-insights-from-global-metabolomics-studies-into-synergistic-bactericidal-effect-of-a-polymyxin-b-combination-with-tamoxifen-against-cystic-fibrosis-mdr-pseudomonas-aeruginosa
#6
Maytham Hussein, Mei-Ling Han, Yan Zhu, Elena K Schneider-Futschik, Xiaohan Hu, Qi Tony Zhou, Yu-Wei Lin, Dovile Anderson, Darren J Creek, Daniel Hoyer, Jian Li, Tony Velkov
Polymyxins are amongst the most important antibiotics in modern medicine, in recent times their clinical utility has been overshadowed by nosocomial outbreaks of polymyxin resistant MDR Gram-negative 'superbugs'. An effective strategy to surmount polymyxin resistance is combination therapy with FDA-approved non-antibiotic drugs. Herein we used untargeted metabolomics to investigate the mechanism(s) of synergy between polymyxin B and the selective estrogen receptor modulator (SERM) tamoxifen against a polymyxin-resistant MDR cystic fibrosis (CF) Pseudomonas aeruginosa FADDI-PA006 isolate (polymyxin B MIC=8 mg/L , it is an MDR polymyxin resistant P...
2018: Computational and Structural Biotechnology Journal
https://www.readbyqxmd.com/read/30546305/use-of-a-primary-epithelial-cell-screening-tool-to-investigate-phage-therapy-in-cystic-fibrosis
#7
Stephanie Trend, Barbara J Chang, Mark O'Dea, Stephen M Stick, Anthony Kicic
Antimicrobial-resistant microbes are an increasing threat to human health. In cystic fibrosis (CF), airway infections with Pseudomonas aeruginosa remain a key driver of lung damage. With few new antibiotics on the development horizon, alternative therapeutic approaches are needed against antimicrobial-resistant pathogens. Phage therapy, or the use of viruses that infect bacteria, is one proposed novel therapy to treat bacterial infections. However, the airways are complex microenvironments with unique characteristics that may affect the success of novel therapies...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/30546149/clinico-pathological-features-of-the-intradiaphragmatic-bronchogenic-cysts-report-of-a-case-and-review-of-the-literature
#8
S Simonetti, E Canalís, L Macías, M A Carrasco
Bronchogenic cysts represent congenital malformations deriving from an abnormal development of the primitive foregut during embryogenesis. These lesions are rarely found and they are most frequently localized in the mediastinum, or in lung parenchyma. Intramuscular localization is extremely rare, especially within the diaphragm. We report a case of a 54 year old man showing a large lobulated cystic lesion in the left hemidiaphragm. Complete surgery was performed and histological diagnosis of intradiaphragmatic bronchogenic cyst was made during surgery and confirmed after a total section analysis...
September 2018: Pathologica
https://www.readbyqxmd.com/read/30546045/publisher-correction-early-detection-of-pulmonary-exacerbations-in-children-with-cystic-fibrosis-by-electronic-home-monitoring-of-symptoms-and-lung-function
#9
Marieke van Horck, Bjorn Winkens, Geertjan Wesseling, Dillys van Vliet, Kim van de Kant, Sanne Vaassen, Karin de Winter-de Groot, Ilja de Vreede, Quirijn Jöbsis, Edward Dompeling
A correction to this article has been published and is linked from the HTML and PDF versions of this paper. The error has been fixed in the paper.
December 13, 2018: Scientific Reports
https://www.readbyqxmd.com/read/30545681/influence-of-inspiratory-expiratory-ct-registration-on-quantitative-air-trapping
#10
Oliver Weinheimer, Benjamin A Hoff, Aleksa B Fortuna, Antonio Fernández-Baldera, Philip Konietzke, Mark O Wielpütz, Terry E Robinson, Craig J Galbán
RATIONALE AND OBJECTIVES: The aim of this study was to assess variability in quantitative air trapping (QAT) measurements derived from spatially aligned expiration CT scans. MATERIALS AND METHODS: Sixty-four paired CT examinations, from 16 school-age cystic fibrosis subjects examined at four separate time intervals, were used in this study. For each pair, visually inspected lobe segmentation maps were generated and expiration CT data were registered to the inspiration CT frame...
December 10, 2018: Academic Radiology
https://www.readbyqxmd.com/read/30543305/regulators-of-a20-tnfaip3-new-drug-able-targets-in-inflammation
#11
George Momtazi, Bart Lambrecht, Jose R Naranjo, Bettina C Schock
Persistent activation of the transcription factor Nuclear Factor-kappaB (NF-κB) is central to the pathogenesis of many inflammatory disorders, including those of the lung such as cystic fibrosis (CF), asthma, and chronic obstructive pulmonary disease (COPD). Despite recent advances in treatment, management of the inflammatory component of these diseases still remains suboptimal. A20 is an endogenous negative regulator of NF-κB signaling, which has been widely described in several autoimmune and inflammatory disorders and more recently in terms of chronic lung disorders...
December 13, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/30541464/multi-systemic-melioidosis-a-clinical-neurological-and-radiological-case-study-from-hainan-province-china
#12
Wei-Yuan Huang, Gang Wu, Feng Chen, Meng-Meng Li, Jian-Jun Li
BACKGROUND: Melioidosis is a tropical disease caused by Burkholderia pseudomallei (B. pseudomallei). It can infect any organ system and lead to multiple abscesses. A few studies reported that central nervous system (CNS) is also involved. We present a diabetic patient with multi-systemic melioidosis that affected the CNS, thorax, and spleen. The aim was to study the clinical and radiological features of melioidosis and enhance understanding of the disease. CASE PRESENTATION: A 38-year-old male presented with cough and expectoration mixed with blood for several days...
December 12, 2018: BMC Infectious Diseases
https://www.readbyqxmd.com/read/30540818/corticosteroid-use-and-increased-cxcr2-levels-on-leukocytes-are-associated-with-lumacaftor-ivacaftor-discontinuation-in-cystic-fibrosis-patients-homozygous-for-the-f508del-cftr-mutation
#13
Kerstin Pohl, David P Nichols, Jennifer L Taylor-Cousar, Milene T Saavedra, Matthew J Strand, Jerry A Nick, Preston E Bratcher
Cystic fibrosis (CF) is the most common life-shortening genetic disease and is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Several current therapies aim at improving availability and/or function of the mutant CFTR proteins. The combination therapeutic lumacaftor/ivacaftor (Orkambi, luma/iva) partially corrects folding and potentiates CFTR function impaired by the F508del mutation. Despite the potential for clinical benefit, a substantial number of patients discontinue treatment due to intolerable adverse effects...
2018: PloS One
https://www.readbyqxmd.com/read/30540547/identification-of-molecular-signatures-of-cystic-fibrosis-disease-status-using-plasma-based-functional-genomics
#14
Hara Levy, Shuang Jia, Amy Pan, Xi Zhang, Mary L Kaldunski, Melodee L Nugent, Melissa Reske, Rachel A Feliciano, Diana Quintero, Michael M Renda, Katherine J Woods, Kathy Murkowski, Keven Johnson, James Verbsky, Trivikram Dasu, Justin Eze Ideozu, Susanna McColley, Michael W Quasney, Mary K Dahmer, Ellis D Avner, Philip M Farrell, Carolyn L Cannon, Howard Jacob, Pippa M Simpson, Martin J Hessner
Although cystic fibrosis (CF) is attributed to dysfunction of a single gene, the relationships between the abnormal gene product and development of inflammation and progression of lung disease are not fully understood, limiting our ability to predict an individual patient's clinical course and treatment response. To better understand CF progression, we characterized the molecular signatures of CF disease status using plasma-based functional genomics. Peripheral blood mononuclear cells (PBMCs) from healthy donors were cultured with plasma samples from CF patients (n=103) and unrelated, healthy controls (n=31)...
December 12, 2018: Physiological Genomics
https://www.readbyqxmd.com/read/30538635/gene-therapy-for-cystic-fibrosis-lung-disease-overcoming-the-barriers-to-translation-to-the-clinic
#15
Martin Donnelley, David W Parsons
Cystic fibrosis (CF) is a progressive, chronic and debilitating genetic disease caused by mutations in the CF Transmembrane-Conductance Regulator ( CFTR ) gene. Unrelenting airway disease begins in infancy and produces a steady deterioration in quality of life, ultimately leading to premature death. While life expectancy has improved, current treatments for CF are neither preventive nor curative. Since the discovery of CFTR the vision of correcting the underlying genetic defect - not just treating the symptoms - has been developed to where it is poised to become a transformative technology...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/30538347/epidemiological-study-of-echinococcus-granulosus-in-sheep-in-the-gharb-plain-north-west-of-morocco
#16
Kamal Brik, Taoufik Hassouni, Sanaa Youssir, Samir Baroud, Khadija Elkharrim, Driss Belghyti
Hydatidosis is considered endemic in Morocco and caused serious health problems in ruminants. The aim of this research is to present an epidemiological study on echinococcosis in sheep. The study was carried out between April 2016 and May 2017. A total number of 1600 sheeps was examined via autopsy for Echinococcus granulosus in various slaughterhouses in the Gharb plain of Morocco. The Results of the study showed that the prevalence of cystic echinococcosis was 7.63%. The infection was not significant among sheep which age is < 1 year (2...
December 2018: Journal of Parasitic Diseases: Official Organ of the Indian Society for Parasitology
https://www.readbyqxmd.com/read/30534549/evaluation-of-food-insecurity-in-adults-and-children-with-cystic-fibrosis-community-case-study
#17
Perry S Brown, Dixie Durham, Rick D Tivis, Shannon Stamper, Cleary Waldren, Sarah E Toevs, Barbara Gordon, Tiffany A Robb
Advances in the care and treatment of cystic fibrosis (CF) have led to improved mortality rates; therefore, considerably more individuals with CF are living into adulthood. With an increased number of CF patients advancing into adulthood, there is the need for more research that surrounds the aging adult CF patient. It is important to conduct research and collect results on the aging CF population to help better prepare the CF patient, who is dealing with the heavy treatment and financial burden of their disease, build autonomy and increase their quality of life...
2018: Frontiers in Public Health
https://www.readbyqxmd.com/read/30533232/birt-hogg-dub%C3%A3-syndrome-caused-by-a-novel-mutation-in-the-flcl-gene
#18
Charles Volk, Gregory Matwiyoff
Background: Birt-Hogg-Dubé syndrome is a genetic disorder characterized by skin fibrofolliculomas, cystic lung disease, and bilateral renal tumors. It has also been implicated in the formation of tumors in other organs, particularly thyroid and colon. This case presents a young female presenting with only cystic lung disease and kidney tumors, identified as having a never before identified heterozygous mutation in the folliculin ( FLCN ) gene which is the likely cause of her syndrome...
2018: Case Reports in Genetics
https://www.readbyqxmd.com/read/30532741/role-of-the-multidrug-resistance-efflux-pump-mexcd-oprj-in-the-pseudomonas-aeruginosa-quorum-sensing-response
#19
Manuel Alcalde-Rico, Jorge Olivares-Pacheco, Carolina Alvarez-Ortega, Miguel Cámara, José Luis Martínez
Multidrug efflux pumps constitute a category of antibiotic resistance determinants that are a part of the core bacterial genomes. Given their conservation, it is conceivable that they present functions beyond the extrusion of antibiotics currently used for therapy. Pseudomonas aeruginosa stands as a relevant respiratory pathogen, with a high prevalence at hospitals and in cystic fibrosis patients. Part of its success relies on its low susceptibility to antibiotics and on the production of virulence factors, whose expression is regulated in several cases by quorum sensing (QS)...
2018: Frontiers in Microbiology
https://www.readbyqxmd.com/read/30532201/a-genome-wide-association-analysis-reveals-a-potential-role-for-recombination-in-the-evolution-of-antimicrobial-resistance-in-burkholderia-multivorans
#20
Julio Diaz Caballero, Shawn T Clark, Pauline W Wang, Sylva L Donaldson, Bryan Coburn, D Elizabeth Tullis, Yvonne C W Yau, Valerie J Waters, David M Hwang, David S Guttman
Cystic fibrosis (CF) lung infections caused by members of the Burkholderia cepacia complex, such as Burkholderia multivorans, are associated with high rates of mortality and morbidity. We performed a population genomics study of 111 B. multivorans sputum isolates from one CF patient through three stages of infection including an early incident isolate, deep sampling of a one-year period of chronic infection occurring weeks before a lung transplant, and deep sampling of a post-transplant infection. We reconstructed the evolutionary history of the population and used a lineage-controlled genome-wide association study (GWAS) approach to identify genetic variants associated with antibiotic resistance...
December 7, 2018: PLoS Pathogens
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