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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/27933058/skewed-lung-ccr4-to-ccr6-cd4-t-cell-ratio-in-idiopathic-pulmonary-fibrosis-is-associated-with-pulmonary-function
#2
Ayodeji Adegunsoye, Cara L Hrusch, Catherine A Bonham, Mohammad R Jaffery, Kelly M Blaine, Meghan Sullivan, Matthew M Churpek, Mary E Strek, Imre Noth, Anne I Sperling
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal lung disease. While it has been suggested that T cells may contribute to IPF pathogenesis, these studies have focused primarily on T cells outside of the pulmonary interstitium. Thus, the role of T cells in the diseased lung tissue remains unclear. OBJECTIVE: To identify whether specific CD4(+) T cell subsets are differentially represented in lung tissue from patients with IPF. METHODS: CD4(+) T cell subsets were measured in lung tissue obtained from patients with IPF at the time of lung transplantation, and from age- and gender-matched organ donors with no known lung disease...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27932986/role-of-quercetin-in-modulating-chloride-transport-in-the-intestine
#3
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/27932335/multicentre-randomised-placebo-controlled-trial-of-oral-anticoagulation-with-apixaban-in-systemic-sclerosis-related-pulmonary-arterial-hypertension-the-sphinx-study-protocol
#4
Alicia Calderone, Wendy Stevens, David Prior, Harshal Nandurkar, Eli Gabbay, Susanna M Proudman, Trevor Williams, David Celermajer, Joanne Sahhar, Peter K K Wong, Vivek Thakkar, Nathan Dwyer, Jeremy Wrobel, Weng Chin, Danny Liew, Margaret Staples, Rachelle Buchbinder, Mandana Nikpour
INTRODUCTION: Systemic sclerosis (SSc) is a severe and costly multiorgan autoimmune connective tissue disease characterised by vasculopathy and fibrosis. One of the major causes of SSc-related death is pulmonary arterial hypertension (PAH), which develops in 12-15% of patients with SSc and accounts for 30-40% of deaths. In situ thrombosis in the small calibre peripheral pulmonary vessels resulting from endothelial dysfunction and an imbalance of anticoagulant and prothrombotic mediators has been implicated in the complex pathophysiology of SSc-related PAH (SSc-PAH), with international clinical guidelines recommending the use of anticoagulants for some types of PAH, such as idiopathic PAH...
December 8, 2016: BMJ Open
https://www.readbyqxmd.com/read/27932299/provisional-matrix-a-role-for-versican-and-hyaluronan
#5
REVIEW
Thomas N Wight
Hyaluronan and versican are extracellular matrix (ECM) components that are enriched in the provisional matrices that form during the early stages of development and disease. These two molecules interact to create pericellular "coats" and "open space" that facilitate cell sorting, proliferation, migration, and survival. Such complexes also impact the recruitment of leukocytes during development and in the early stages of disease. Once thought to be inert components of the ECM that help hold cells together, it is now quite clear that they play important roles in controlling cell phenotype, shaping tissue response to injury and maintaining tissue homeostasis...
December 5, 2016: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/27932290/the-diagnosis-of-idiopathic-pulmonary-fibrosis-current-and-future-approaches
#6
REVIEW
Fernando J Martinez, Alison Chisholm, Harold R Collard, Kevin R Flaherty, Jeffrey Myers, Ganesh Raghu, Simon L F Walsh, Eric S White, Luca Richeldi
With the recent development of two effective treatments for patients with idiopathic pulmonary fibrosis, an accurate diagnosis is crucial. The traditional approach to diagnosis emphasises the importance of thorough clinical and laboratory evaluations to exclude secondary causes of disease. High-resolution CT is a critical initial diagnostic test and acts as a tool to identify patients who should undergo surgical lung biopsy to secure a definitive histological diagnosis of usual interstitial pneumonia pattern...
December 5, 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27932263/long-term-disease-and-economic-outcomes-of-prior-authorization-criteria-for-hepatitis-c-treatment-in-pennsylvania-medicaid
#7
Mina Kabiri, Jagpreet Chhatwal, Julie M Donohue, Mark S Roberts, A Everette James, Michael A Dunn, Walid F Gellad
BACKGROUND: Several highly effective but costly therapies for hepatitis C virus (HCV) are available. As a consequence of their high price, 36 state Medicaid programs limited treatment coverage to patients with more advanced HCV stages. States have only limited information available to predict the long-term impact of these decisions. METHODS: We adapted a validated hepatitis C microsimulation model to the Pennsylvania Medicaid population to estimate the existing HCV prevalence in Pennsylvania Medicaid and estimate the impact of various HCV drug coverage policies on disease outcomes and costs...
December 5, 2016: Healthcare
https://www.readbyqxmd.com/read/27932191/iga-nephropathy-clinicopathologic-study-following-the-oxford-classification-progression-peculiarities-and-gender-related-differences
#8
Živile Riispere, Arvydas Laurinavičius, Anne Kuudeberg, Elviira Seppet, Kristin Sepp, Madis Ilmoja, Merike Luman, Külli Kõlvald, Asta Auerbach, Mai Ots-Rosenberg
BACKGROUND AND AIM: Immunoglobulin A nephropathy (IgAN) is the most frequent glomerular disease worldwide and one of the main causes of chronic kidney disease. We aimed to investigate clinicopathological correlations in IgAN patients by gender. MATERIALS AND METHODS: The study was based on a retrospective analysis of renal biopsy data and clinical manifestations of the disease. Consecutive 73 biopsy-proven IgAN cases of male (62%) and female (38%) patients were investigated...
November 22, 2016: Medicina
https://www.readbyqxmd.com/read/27932134/efficacy-and-safety-of-therapy-with-simeprevir-and-sofosbuvir-in-liver-transplant-recipients-infected-by-hepatitis-c-virus-genotype-4-cohort-spanish-society-of-liver-transplantation-cohort
#9
G Sanchez Antolin, M Testillano, J M Pascasio, I Narvaez Rodriguez, M Prieto, A Otero, J I Herrero, M Londoño, I Fernandez Vazquez, L Castells
BACKGROUND: Patients with hepatitis C virus (HCV) genotype 4 infection are poorly represented in clinical trials of 2nd-generation direct-acting antivirals (DAAs), and more data are needed to help guide treatment decisions. We still have even fewer data concerning liver transplant patients. Simeprevir (SIM) and sofosbuvir (SOF) combination is useful to treat this genotype. The aim of this study was to know the efficacy and safety of the combination SIM + SOF ± ribavirin (RBV) in a group of liver transplant patients with HCV genotype 4 infection in Spain in real life...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27932059/classical-transient-receptor-potential-trpc6-channels-support-myofibroblast-differentiation-and-development-of-experimental-pulmonary-fibrosis
#10
Katharina Hofmann, Susanne Fiedler, Sarah Vierkotten, Jonas Weber, Stephan Klee, Jie Jia, Wolfgang Zwickenpflug, Veit Flockerzi, Ursula Storch, Ali Önder Yildirim, Thomas Gudermann, Melanie Königshoff, Alexander Dietrich
Pulmonary fibrosis (PF) is a chronic progressive lung disease without effective medical treatment options leading to respiratory failure and death within 3-5 years of diagnosis. The pathological process of PF is driven by aberrant wound-healing involving fibroblasts and myofibroblasts differentiated by secreted profibrotic transforming growth factor β (TGF-β1). Classical transient receptor potential 6 (TRPC6), a Na(+)- and Ca(2+)-permeable cation channel, is able to promote myofibroblast conversion of primary rat cardiac and human dermal fibroblasts and TRPC6-deficiency impaired wound healing after injury...
December 5, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27931613/later-onset-fabry-disease-cardiac-damage-progress-in-silence-experience-with-a-highly-prevalent-mutation
#11
Ting-Rong Hsu, Sheng-Che Hung, Fu-Pang Chang, Wen-Chung Yu, Shih-Hsien Sung, Chia-Lin Hsu, Ivan Dzhagalov, Chia-Feng Yang, Tzu-Hung Chu, Han-Jui Lee, Yung-Hsiu Lu, Sheng-Kai Chang, Hsuan-Chieh Liao, Hsiang-Yu Lin, Tsan-Chieh Liao, Pi-Chang Lee, Hsing-Yuan Li, An-Hang Yang, Hui-Chen Ho, Chuan-Chi Chiang, Ching-Yuang Lin, Robert J Desnick, Dau-Ming Niu
BACKGROUND: Recently, several studies revealed a much higher prevalence of later onset Fabry disease (FD) than previously expected. It suggested that later onset FD might present as an important hidden health issue in certain ethnic or demographic populations in the world. However, the natural history of its phenotype has not been systemically investigated, especially the cardiac involvement. OBJECTIVES: The study analyzed a large-scale newborn screening program for FD to understand the natural course of later onset FD...
December 13, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27931223/pattern-and-prognostic-value-of-cardiac-involvement-in-patients-with-late-onset-pompe-disease-a-comprehensive-cardiovascular-magnetic-resonance-approach
#12
Matthias Boentert, Anca Florian, Bianca Dräger, Peter Young, Ali Yilmaz
BACKGROUND: Pompe disease is an autosomal recessive disorder caused by deficiency of the lysosomal α-1,4-glucosidase leading to accumulation of glycogen in target tissues with progressive organ failure. While the early infantile-onset form is characterized by early severe hypertrophic cardiomyopathy with cardiac and respiratory failure, clinically relevant cardiomyopathy seems to be uncommon in patients with late-onset Pompe disease, and the prevalence and nature of myocardial abnormalities are still to be clarified...
December 7, 2016: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/27931086/liver-involvement-in-patients-with-pizz-emphysema-candidates-for-lung-transplantation
#13
Lise Morer, Laurence Choudat, Gaelle Dauriat, Francois Durand, Dominique Cazals-Hatem, Gabriel Thabut, Olivier Brugière, Yves Castier, Hervé Mal
Information about the prevalence and nature of liver disorders in adults with alpha1-antitrypsin deficiency is scarce. At our center, systematic liver biopsy screening is part of the evaluation before lung transplantation (LT) in the emphysema patients with the PiZZ phenotype. Our aim was to report our experience with this prospective screening. Clinical, liver function and imaging parameters as well as liver histology data were analyzed for 23 consecutive adult patients with PiZZ severe emphysema referred to our center for consideration of LT from 2006 to 2014...
December 8, 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/27930988/potential-involvement-of-ppar-%C3%AE-activation-in-diminishing-the-hepatoprotective-effect-of-fenofibrate-in-nafld-accuracy-of-non-invasive-panel-in-determining-the-stage-of-liver-fibrosis-in-rats
#14
Asmaa M Hamed, Omnyah A El-Kharashi, Suzi S A Boctor, Lobna F Abd-Elaziz
BACKGROUND: Although Fenofibrate (FF) is a hypolipedmic drug and one of the PPARα agonists which is a drug target for non alcoholic liver disease (NAFLD), no studies had investigated its potential hepatic effects in such cases. AIM: To compare between the effect of FF and Gemfibrozil (GF) on the prognosis of NAFLD in rats. METHODS: Sixty four rats were used and classified into two main groups. Group I (treated for 6 weeks): naïve, FF, GF groups and Group II (treated for 14 weeks and drugs were added at the last 6 weeks): Control, high fat diet (HFD) untreated, HFD+FF, HFD+FF+folic acid (FA) and HFD+GF groups...
December 5, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27929753/generation-and-characterization-of-abbv642-a-dual-variable-domain-immunoglobulin-molecule-dvd-ig-that-potently-neutralizes-vegf-and-pdgf-bb-and-is-designed-for-the-treatment-of-exudative-age-related-macular-degeneration
#15
Kun Ding, Lucia Eaton, Diana Bowley, Matthew Rieser, Qing Chang, Maria C Harris, Anca Clabbers, Feng Dong, Jikui Shen, Sean F Hackett, Debra S Touw, Jacqueline Bixby, Suju Zhong, Lorenzo Benatuil, Sahana Bose, Christine Grinnell, Gregory M Preston, Ramesh Iyer, Ramkrishna Sadhukhan, Susan Marchie, Gary Overmeyer, Tariq Ghayur, Deborah A van Riet, Shibo Tang, Peter A Campochario, Jijie Gu
Exudative age-related macular degeneration (AMD) is the most common cause of moderate and severe vision loss in developed countries. Intraocular injections of vascular endothelial growth factor (VEGF or VEGF-A)-neutralizing proteins provide substantial benefit, but frequent, long-term injections are needed. In addition, many patients experience initial visual gains that are ultimately lost due to subretinal fibrosis. Preclinical studies and early phase clinical trials suggest that combined suppression of VEGF and platelet-derived growth factor-BB (PDGF-BB) provides better outcomes than suppression of VEGF alone, due to more frequent regression of neovascularization (NV) and suppression of subretinal fibrosis...
December 8, 2016: MAbs
https://www.readbyqxmd.com/read/27928683/specific-autoantibodies-in-dermatomyositis-a-helpful-tool-to-classify-different-clinical-subsets
#16
Giulia Merlo, Andrea Clapasson, Emanuele Cozzani, Luigi Sanna, Giampaola Pesce, Marcello Bagnasco, Martina Burlando, Aurora Parodi
Autoantibodies are important in the diagnosis of dermatomyositis. They can be divided in two different groups: myositis-associated autoantibodies (MAA) prevailing in overlap syndromes, and myositis-specific autoantibodies (MSA), with diagnostic specificity exceeding 90%. Our purpose was to detect retrospectively the prevalence of the most common MSAs in a group of 19 adult DM patients (13 women, 6 men). A severe DM (SDM), with extensive cutaneous and muscular manifestations, dysphagia, and sometimes pneumopathy, was detected in ten cases...
December 7, 2016: Archives of Dermatological Research
https://www.readbyqxmd.com/read/27928635/valproic-acid-attenuates-renal-fibrosis-through-the-induction-of-autophagy
#17
Koichiro Kawaoka, Shigehiro Doi, Ayumu Nakashima, Kyoko Yamada, Toshinori Ueno, Toshiki Doi, Takao Masaki
BACKGROUND: Renal fibrosis is a common pathological feature of the progression of chronic kidney disease. Although valproic acid (VPA) has been recently shown to induce autophagy, the effect of VPA-induced autophagy on renal fibrosis remains unknown. We, therefore, investigated whether VPA-induced autophagy suppresses renal fibrosis in a mouse model of unilateral ureteral obstruction (UUO). METHODS: Male C57BL/6 mice were divided into five groups (n = 8 per group): (1) sham group; (2) vehicle group; (3) VPA-treated group; (4) 3-methyladenine (3-MA; autophagy inhibitor)-treated group; and (5) VPA plus 3-MA-treated group...
December 7, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27927713/high-fibre-diet-and-acetate-supplementation-change-the-gut-microbiota-and-prevent-the-development-of-hypertension-and-heart-failure-in-doca-salt-hypertensive-mice
#18
Francine Z Marques, Erin M Nelson, Po-Yin Chu, Duncan Horlock, April Fiedler, Mark Ziemann, Jian K Tan, Sanjaya Kuruppu, Niwanthi W Rajapakse, Assam El-Osta, Charles R Mackay, David M Kaye
BACKGROUND: -Dietary intake of fruit and vegetables is associated with lower incidence of hypertension, but the mechanisms involved have not been elucidated. Here we evaluated the effect of a high fibre diet and supplementation with the short-chain fatty acid (SFCA) acetate on the gut microbiota and the prevention of cardiovascular disease. METHODS: -Gut microbiome, cardiorenal structure/function and blood pressure were examined in sham and mineralocorticoid-excess treated mice with a control diet, high fibre diet or acetate supplementation...
December 7, 2016: Circulation
https://www.readbyqxmd.com/read/27927050/the-use-of-multiple-breath-washout-for-assessing-cystic-fibrosis-in-infants
#19
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
#20
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
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