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Cystic Fibrosis

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https://www.readbyqxmd.com/read/29245055/high-proportion-of-abnormal-pap-smear-tests-and-cervical-dysplasia-in-women-with-cystic-fibrosis
#1
Christine Rousset-Jablonski, Quitterie Reynaud, Raphaëlle Nove-Josserand, Isabelle Ray-Coquard, Yahia Mekki, François Golfier, Isabelle Durieu
OBJECTIVES: Insufficient gynecological follow-up and cervical screening has been reported in women with cystic fibrosis (CF). Some of these patients will require a pulmonary transplantation, known to be associated with a higher risk of cervical dysplasia. The aim of this study was to explore the results of cervical screening in adult women with CF, and to report the prevalence of abnormal pap smear tests in this population. STUDY DESIGN: We retrospectively analyzed medical records of sexually active women with CF who attended a gynecological consultation in Lyon University CF referral center between June 2014 and December 2015...
December 9, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/29243949/twenty-facts-about-cystic-fibrosis
#2
(no author information available yet)
No abstract text is available yet for this article.
December 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29242245/more-new-treatments-for-cystic-fibrosis
#3
Robert Scott-Jupp
No abstract text is available yet for this article.
December 14, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29241892/forecasting-the-long-term-clinical-and-economic-outcomes-of-lumacaftor-ivacaftor-in-cystic-fibrosis-patients-with-homozygous-phe508del-mutation
#4
Piyameth Dilokthornsakul, Mausam Patidar, Jonathan D Campbell
OBJECTIVES: To forecast lifetime outcomes and cost of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis (CF) with homozygous phe508del mutation from the US payer perspective. METHODS: A lifetime Markov model was developed from a US payer perspective. The model included five health states: 1) mild lung disease (percent predicted forced expiratory volume in 1 second [FEV1] >70%), 2) moderate lung disease (40% ≤ FEV1 ≤ 70%), 3) severe lung disease (FEV1 < 40%), 4) lung transplantation, and 5) death...
December 2017: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
https://www.readbyqxmd.com/read/29241629/the-expression-of-mirc1-mir17-92-cluster-in-sputum-samples-correlates-with-pulmonary-exacerbations-in-cystic-fibrosis-patients
#5
Kathrin Krause, Benjamin T Kopp, Mia F Tazi, Kyle Caution, Kaitlin Hamilton, Asmaa Badr, Chandra Shrestha, Dmitry Tumin, Don Hayes, Frank Robledo, Luanne Hall-Stoodley, Brett G Klamer, Xiaoli Zhang, Santiago Partida-Sanchez, Narasimham L Parinandi, Stephen E Kirkby, Duaa Dakhlallah, Karen S McCoy, Estelle Cormet-Boyaka, Amal O Amer
INTRODUCTION: Cystic fibrosis (CF) is a multi-organ disorder characterized by chronic sino-pulmonary infections and inflammation. Many patients with CF suffer from repeated pulmonary exacerbations that are predictors of worsened long-term morbidity and mortality. There are no reliable markers that associate with the onset or progression of an exacerbation or pulmonary deterioration. Previously, we found that the Mirc1/Mir17-92a cluster which is comprised of 6 microRNAs (Mirs) is highly expressed in CF mice and negatively regulates autophagy which in turn improves CF transmembrane conductance regulator (CFTR) function...
December 11, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29240582/the-morphologic-spectrum-of-sertoliform-cystadenoma-of-the-rete-testis-a-series-of-15-cases
#6
Swetha Paluru, Thomas M Ulbright, Mahul Amin, Rodolfo Montironi, Jonathan I Epstein
Sertoliform cystadenoma of the rete testis (SCRT) is rare with only 9 cases reported to date in the literature, none with follow-up. Four large genitourinary pathology consult services were searched. We identified 15 cases of SCRT. Men were 21 to 84 years old (mean, 46 y) and had testicular discomfort or mass. Other findings were seminoma (n=1), spermatocele (n=2), hydrocele (n=1), varicocele (n=1), and scrotal hematoma (n=1). Eight had preoperative serum tumor markers, which were normal. Tumors ranged from 0...
December 7, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29240403/multivalent-glycomimetics-with-affinity-and-selecivity-towards-fucose-binding-receptors-from-emerging-pathogens
#7
Cristina Nativi, David Goyard, Veronica Baldoneschi, Annabelle Varrot, Michele Fiore, Anne Imberty, Barbara Richichi, Olivier Renaudet
Bacterial and fungal pathogens involved in lung infection in cystic fibrosis patients utilize a particular family of glycan-binding proteins, characterized by the presentation of six fucose-binding sites on a ring shape scaffold. These lectins are attractive targets for anti-infectious compounds that could interfere in the recognition of host tissues by pathogens. The design of a cyclopeptide-based hexavalent structure allowed for the presentation of six fucose residues. The synthetic hexavalent compound displays appropriate geometry resulting in high avidity binding by lectins from Aspergillus fumigatus and Burkholderia ambifaria...
December 14, 2017: Bioconjugate Chemistry
https://www.readbyqxmd.com/read/29240302/correlation-between-sinus-and-lung-cultures-in-lung-transplant-patients-with-cystic-fibrosis
#8
Kevin J Choi, Tracy Z Cheng, Adam L Honeybrook, Alice L Gray, Laurie D Snyder, Scott M Palmer, Ralph Abi Hachem, David W Jang
BACKGROUND: Lung transplantation has revolutionized the treatment of end-stage pulmonary disease due to cystic fibrosis. However, infection of the transplanted lungs can lead to serious complications, including graft failure and death. Although many of these patients have concurrent sinusitis, it is unclear whether bacteria from the sinuses can infect the allograft. METHODS: This is a single-institution retrospective study of all patients who underwent lung transplantation for cystic fibrosis from 2005 to 2015 at Duke University Hospital...
December 14, 2017: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/29239766/fam13a-is-a-modifier-gene-of-cystic-fibrosis-lung-phenotype-regulating-rhoa-activity-actin-cytoskeleton-dynamics-and-epithelial-mesenchymal-transition
#9
Harriet Corvol, Nathalie Rousselet, Kristin E Thompson, Laura Berdah, Guillaume Cottin, Tobias Foussigniere, Elisabeth Longchampt, Laurence Fiette, Edouard Sage, Céline Prunier, Mitchell Drumm, Craig A Hodges, Pierre-Yves Boëlle, Loic Guillot
BACKGROUND: Cystic fibrosis (CF) lung disease severity is highly variable and dependent on several factors including genetic modifiers. Family with sequence similarity 13 member A (FAM13A) has been previously associated with lung function in the general population as well as in several chronic lung diseases, such as chronic obstructive pulmonary disease (COPD), we examined whether FAM13A is a modifier gene of CF lung phenotype. We also studied how FAM13A may contribute to the physiopathological mechanisms associated with CF...
November 24, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29236544/gene-therapy-for-cystic-fibrosis-improved-delivery-techniques-and-conditioning-with-lysophosphatidylcholine-enhance-lentiviral-gene-transfer-in-mouse-lung-airways
#10
Patricia Cmielewski, Nigel Farrow, Sharnna Devereux, David Parsons, Martin Donnelley
Purpose/Aim: Cystic fibrosis (CF) is the most common, fatal recessive genetic disease among the Caucasian population. Gene therapy has the potential to treat CF long term, however physiological barriers can prevent VSV-G pseudotyped lentiviral (LV) vectors from efficiently accessing the relevant receptors on the basolateral membrane of airway epithelial cells. The aims of this experiment were to use our new dose delivery techniques to determine whether conditioning the mouse lung conducting airways with lysophosphatidylcholine (LPC) improves the level of airway gene expression...
December 13, 2017: Experimental Lung Research
https://www.readbyqxmd.com/read/29235532/investigation-of-the-effects-of-the-cftr-potentiator-ivacaftor-on-human-p-glycoprotein-abcb1
#11
Swathi Lingam, Nopnithi Thonghin, Robert C Ford
Ivacaftor is a potentiator of the CFTR chloride channel and is in worldwide clinical use for the chronic treatment of cystic fibrosis in patients. There is evidence that the bioavailability of ivacaftor in the body may be influenced by the multi-drug exporter P-glycoprotein. Here we have employed purified and reconstituted P-glycoprotein to study its interaction with ivacaftor as well as the ability of the drug to compete with a known transported substrate of the protein. We find that ivacaftor stimulates the ATPase activity of the purified protein and can compete with the transport of the fluorescent substrate Hoechst 33342...
December 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29235345/mucus-and-cell-penetrating-nanoparticles-embedded-in-nano-into-micro-formulations-for-pulmonary-delivery-of-ivacaftor-in-cystic-fibrosis
#12
Barbara Porsio, Emanuela Fabiola Craparo, Nicolò Mauro, Gaetano Giammona, Gennara Cavallaro
Here, mucus-penetrating nanoparticles for pulmonary administration of Ivacaftor in patients with cystic fibrosis (CF) were produced with the dual aim to enhance Ivacaftor delivery to the airway epithelial cells, by a rapid diffusion through the mucus barrier and, at the same time, promote Ivacaftor lung cellular uptake. Pegylated and Tat-decorated Fluorescent Nanoparticles (FNPs) were produced by nanoprecipitation, starting by two synthetic copolymers and showed nanometric sizes (~70 nm), slightly negative ζ potential and high cytocompatibility towards human bronchial epithelium cells...
December 13, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/29234895/intestinal-stem-cells-to-advance-drug-development-precision-and-regenerative-medicine-a-paradigm-shift-in-translational-research
#13
Jonathan P Mochel, Albert E Jergens, Dawn Kingsbury, Hyun Jung Kim, Martín G Martín, Karin Allenspach
Recent advances in our understanding of the intestinal stem cell niche and the role of key signaling pathways on cell growth and maintenance have allowed the development of fully differentiated epithelial cells in 3D organoids. Stem cell-derived organoids carry significant levels of proteins that are natively expressed in the gut and have important roles in drug transport and metabolism. They are, therefore, particularly relevant to study the gastrointestinal (GI) absorption of oral medications. In addition, organoids have the potential to serve as a robust preclinical model for demonstrating the effectiveness of new drugs more rapidly, with more certainty, and at lower costs compared with live animal studies...
December 12, 2017: AAPS Journal
https://www.readbyqxmd.com/read/29234058/relation-of-ultrasound-findings-and-abdominal-symptoms-obtained-with-the-cfabd-score-in-cystic-fibrosis-patients
#14
Harold Tabori, Anke Jaudszus, Christin Arnold, Hans-Joachim Mentzel, Michael Lorenz, Ruth K Michl, Thomas Lehmann, Diane M Renz, Jochen G Mainz
Abdominal symptoms are a hallmark of Cystic fibrosis (CF). Yet, their association with morphological abnormalities of different abdominal organs is still poorly understood. Aim was therefore to relate these symptoms, assessed with a questionnaire, to findings in abdominal ultrasound (US). In 114 CF patients of all ages, findings in US considering seventeen specific parameters were related to abdominal symptoms compiled with our novel CF-specific 26-modal symptom score (CFAbd-Score). US abnormalities were detected in 95% of the patients...
December 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29233472/drugs-during-pregnancy-and-breast-feeding-in-women-diagnosed-with-cystic-fibrosis-an-update
#15
M A G M Kroon, A M Akkerman-Nijland, B L Rottier, G H Koppelman, O W Akkerman, D J Touw
No abstract text is available yet for this article.
December 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29233471/resolvin-d1-regulates-epithelial-ion-transport-and-inflammation-in-cystic-fibrosis-airways
#16
Fiona C Ringholz, Gerard Higgins, Aurélie Hatton, Ali Sassi, Ahmad Moukachar, Coral Fustero-Torre, Monika Hollenhorst, Isabelle Sermet-Gaudelus, Brian J Harvey, Paul McNally, Valerie Urbach
BACKGROUND: Cystic Fibrosis (CF) lung disease is characterised by dysregulated ion transport that promotes chronic bacterial infection and inflammation. The impact of the specialised pro-resolution mediator resolvin D1 (RvD1) on airway surface liquid (ASL) dynamics and innate defence had not yet been investigated in CF airways. METHODS: Ex vivo studies were performed on primary cultures of alveolar macrophages and bronchial epithelial cells from children with CF and in human bronchial epithelial cell lines; in vivo studies were performed in homozygous F508del-CFTR mice treated with vehicle control or RvD1 (1-100nM)...
December 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29232848/a-metagenomic-and-in-silico-functional-prediction-of-gut-microbiota-profiles-may-concur-in-discovering-new-cystic-fibrosis-patient-targeted-probiotics
#17
Pamela Vernocchi, Federica Del Chierico, Andrea Quagliariello, Danilo Ercolini, Vincenzina Lucidi, Lorenza Putignani
Cystic fibrosis (CF) is a life-limiting hereditary disorder that results in aberrant mucosa in the lungs and digestive tract, chronic respiratory infections, chronic inflammation, and the need for repeated antibiotic treatments. Probiotics have been demonstrated to improve the quality of life of CF patients. We investigated the distribution of gut microbiota (GM) bacteria to identify new potential probiotics for CF patients on the basis of GM patterns. Fecal samples of 28 CF patients and 31 healthy controls (HC) were collected and analyzed by 16S rRNA-based pyrosequencing analysis of GM, to produce CF-HC paired maps of the distribution of operational taxonomic units (OTUs), and by Phylogenetic Investigation of Communities by Reconstruction of Unobserved States (PICRUSt) for Kyoto Encyclopedia of Genes and Genomes (KEGG) biomarker prediction...
December 9, 2017: Nutrients
https://www.readbyqxmd.com/read/29232734/impact-of-surgery-on-growth-pulmonary-functions-and-acute-pulmonary-exacerbations-in-children-with-non-cystic-fibrosis-bronchiectasis
#18
Nagehan Emiralioglu, Deniz Dogru, Songul Yalcin, Gokcen Dılsa Tugcu, Ebru Yalcin, Ugur Ozcelik, Saniye Ekinci, Nural Kiper
No abstract text is available yet for this article.
December 12, 2017: Thoracic and Cardiovascular Surgeon
https://www.readbyqxmd.com/read/29232160/microrna-145-antagonism-reverses-tgf-%C3%AE-inhibition-of-f508del-cftr-correction-in-airway-epithelia
#19
Farruk Lutful Kabir, Namasivayam Ambalavanan, Gang Liu, Peng Li, George M Solomon, Charitharth V Lal, Marina Mazur, Brian Halloran, Tomasz Szul, William T Gerthoffer, Steven M Rowe, William T Harris
INTRODUCTION: microRNAs (miRNAs) destabilize mRNA transcripts and inhibit protein translation. miR-145 is of particular interest in cystic fibrosis (CF) as it has a direct binding site in the 3'-untranslated region of CFTR and is upregulated by the CF genetic modifier TGF-β. HYPOTHESIS: miR-145 mediates TGF-β inhibition of CFTR synthesis and function in airway epithelia. METHODS: Primary human CF (F508del homozygous) and non-CF airway epithelial cells (AECs) were grown to terminal differentiation at air-liquid interface on permeable supports...
December 12, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29231866/the-consequences-of-being-in-an-infectious-biofilm-microenvironmental-conditions-governing-antibiotic-tolerance
#20
REVIEW
Majken Sønderholm, Thomas Bjarnsholt, Maria Alhede, Mette Kolpen, Peter Ø Jensen, Michael Kühl, Kasper N Kragh
The main driver behind biofilm research is the desire to understand the mechanisms governing the antibiotic tolerance of biofilm-growing bacteria found in chronic bacterial infections. Rather than genetic traits, several physical and chemical traits of the biofilm have been shown to be attributable to antibiotic tolerance. During infection, bacteria in biofilms exhibit slow growth and a low metabolic state due to O₂ limitation imposed by intense O₂ consumption of polymorphonuclear leukocytes or metabolically active bacteria in the biofilm periphery...
December 12, 2017: International Journal of Molecular Sciences
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