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Progressive systemic sclerosis

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https://www.readbyqxmd.com/read/28079847/relationship-between-abnormalities-on-high-resolution-computerized-tomography-pulmonary-function-and-bronchoalveolar-lavage-in-progressive-systemic-sclerosis
#1
Gulfidan Cakmak, Tuba Selcuk Can, Sule Gundogdu, Canan Akman, Hande Ikitimur, Benan Musellim, Gul Ongen
:  Introduction and aim: Progressive systemic sclerosis (pSS) is a multisystemic connective tissue disease characterized by fibrosis of the skin and internal organs including lung. The mechanisms that leads to progressive lung fibrosis in scleroderma remain obscure. In this study, we aimed to investigate the correlation between HRCT findings and patients' clinical and functional status and the degree of alveolitis based on the BAL resultsMaterials and methods: 65 patients with pSS were evaluated...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28070599/organophosphate-neurotoxicity-to-the-voluntary-motor-system-on-the-trail-of-environment-caused-amyotrophic-lateral-sclerosis-the-known-the-misknown-and-the-unknown
#2
Samantha J Merwin, Teresa Obis, Yanelli Nunez, Diane B Re
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset paralytic disorder. It is characterized by progressive degeneration of the motor neurons controlling voluntary movement. The underlying mechanisms remain elusive, a fact that has precluded development of effective treatments. ALS presents as a sporadic condition 90-95% of the time, i.e., without familial history or obvious genetic mutation. This suggests that ALS has a strong environmental component. Organophosphates (OPs) are prime candidate neurotoxicants in the etiology of ALS, as exposure to OPs was linked to higher ALS incidence among farmers, soccer players, and Gulf War veterans...
January 9, 2017: Archives of Toxicology
https://www.readbyqxmd.com/read/28069782/delivering-patient-centred-care-in-rural-family-practice-using-the-patient-s-concept-of-health-to-guide-treatment
#3
Jennifer M Charlesworth, Evelyn McManus
Through an examination of the life of an 83-year-old patient diagnosed clinically with secondary progressive multiple sclerosis (MS), general practice specialists, consultants and junior doctors will see the importance of assessing their patient's concept of health and how to use this understanding to target healthcare options within their healthcare system. This article highlights, in a resource limited context of rural family practice, the utility of a strong physician-patient relationship, recalls the definition of patient-centred care, and the role of judicious inaction in certain contexts...
January 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28069095/deficits-in-social-cognition-an-unveiled-signature-of-multiple-sclerosis
#4
Moussa A Chalah, Samar S Ayache
BACKGROUND AND OBJECTIVES: Multiple sclerosis (MS) is a chronic progressive inflammatory disease of the central nervous system, representing the primary cause of non-traumatic disability in young adults. Cognitive dysfunction can affect patients at any time during the disease process and might alter the six core functional domains. Social cognition is a multi-component construct that includes the theory of mind, empathy and social perception of emotions from facial, bodily and vocal cues...
January 10, 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/28069073/quantification-of-postural-stability-in-minimally-disabled-multiple-sclerosis-patients-by-means-of-dynamic-posturography-an-observational-study
#5
Lucia Grassi, Stefano Rossi, Valeria Studer, Gessica Vasco, Caterina Motta, Fabrizio Patanè, Enrico Castelli, Silvia Rossi, Paolo Cappa
BACKGROUND: Multiple Sclerosis (MS) is a widespread progressive neurologic disease with consequent impairments in daily activities. Disorders of balance are frequent and equilibrium tests are potentially useful to quantify disability and to verify treatment effectiveness. The fair sensitivity of the widely used not-perturbed tests to detect balance disturbances in MS patients have prompted the development of mechatronic systems capable to impose known equilibrium perturbations, in order to challenge the balance control and, consequently, to better assess the level of impairment...
January 10, 2017: Journal of Neuroengineering and Rehabilitation
https://www.readbyqxmd.com/read/28067667/excessive-expression-of-mir-27-impairs-treg-mediated-immunological-tolerance
#6
Leilani O Cruz, Somaye Sadat Hashemifar, Cheng-Jang Wu, Sunglim Cho, Duc T Nguyen, Ling-Li Lin, Aly Azeem Khan, Li-Fan Lu
MicroRNAs (miRs) are tightly regulated in the immune system, and aberrant expression of miRs often results in hematopoietic malignancies and autoimmune diseases. Previously, it was suggested that elevated levels of miR-27 in T cells isolated from patients with multiple sclerosis facilitate disease progression by inhibiting Th2 immunity and promoting pathogenic Th1 responses. Here we have demonstrated that, although mice with T cell-specific overexpression of miR-27 harbor dysregulated Th1 responses and develop autoimmune pathology, these disease phenotypes are not driven by miR-27 in effector T cells in a cell-autonomous manner...
January 9, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28067393/longitudinal-assessment-of-metal-concentrations-and-copper-isotope-ratios-in-the-g93a-sod1-mouse-model-of-amyotrophic-lateral-sclerosis
#7
T Gabriel Enge, Heath Ecroyd, Dianne F Jolley, Justin J Yerbury, Anthony Dosseto
Amyotrophic lateral sclerosis (ALS) is a motor neuron disease, which involves progressive motor neuron degeneration in the central nervous system (CNS). The G93A SOD1 mouse model simulates one of the most common causes of familial ALS through the overexpression of a mutated form of the human gene encoding copper/zinc superoxide dismutase (SOD1). Transition metals, particularly Cu and Zn, have been shown to behave abnormally in the disease context and have been hypothesized to contribute to and potentially trigger the disease...
January 9, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28065580/fatigue-favors-in-vitro-th1-and-th17-like-cell-expansion-and-reduces-corticoid-sensitivity-in-ms-patients
#8
Hélcio Alvarenga-Filho, Marisa Salles, Joana Hygino, Thais B Ferreira, Priscila M Sacramento, Clarice Monteiro, Claudia Cristina F Vasconcelos, Regina Maria Papais Alvarenga, Cleonice A M Bento
Fatigue is a common "ghost" symptom in patients with multiple sclerosis (MS), an autoimmune disease mediated by T cells that target myelin antigens of the central nervous system. As fatigue has been associated with inflammatory states, its occurrence may negatively impact MS progression. The aim of this study was to evaluate the impact of fatigue on the cytokine profile of patients with relapsing-remitting (RR) MS. For our study, blood were collected from MS patients in clinical remission phase with (n=15) and without (n=15) fatigue...
December 30, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28061886/circadian-course-of-the-p300-erp-in-patients-with-amyotrophic-lateral-sclerosis-implications-for-brain-computer-interfaces-bci
#9
Helena Erlbeck, Ursula Mochty, Andrea Kübler, Ruben G L Real
BACKGROUND: Accidents or neurodegenerative diseases like amyotrophic lateral sclerosis (ALS) can lead to progressing, extensive, and complete paralysis leaving patients aware but unable to communicate (locked-in state). Brain-computer interfaces (BCI) based on electroencephalography represent an important approach to establish communication with these patients. The most common BCI for communication rely on the P300, a positive deflection arising in response to rare events. To foster broader application of BCIs for restoring lost function, also for end-users with impaired vision, we explored whether there were specific time windows during the day in which a P300 driven BCI should be preferably applied...
January 7, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28053878/microparticle-subpopulations-are-potential-markers-of-disease-progression-and-vascular-dysfunction-across-a-spectrum-of-connective-tissue-disease
#10
E M McCarthy, D Moreno-Martinez, F L Wilkinson, N J McHugh, I N Bruce, J D Pauling, M Y Alexander, B Parker
OBJECTIVE: Microparticles (MPs) are membrane-bound vesicles derived from vascular and intravascular cells such as endothelial cells (EMPs) and platelets (PMPs). We investigated EMP and PMP numbers across a spectrum of autoimmune rheumatic diseases (AIRDs) with the aim of comparing the levels of, and relationship between, EMPs and PMPs. METHODS: Patients with Systemic Lupus Erythematosus (SLE) (n = 24), Systemic Sclerosis (SSc) (n = 24), Primary Raynauds Phenomenon (RP) (n = 17) and "other CTD" (n = 15) (Primary Sjogrens Syndrome, UCTD or MCTD) as well as 15 healthy controls were recruited...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/28049398/infectious-agents-and-neurodegenerative-diseases-exploring-the-links
#11
Mohammad Zubair Alam, Qamre Alam, Mohammad Amjad Kamal, Asif Ahmad Jiman-Fatani, Esam I Azhar, Mohammad Azhar Khan, Absarul Haque
Recent studies have shown that bacterial and viral infections are risk factors for various neurodegenerative diseases such as Amyotrophic lateral sclerosis (ALS), Multiple Sclerosis (MS), Alzheimer's disease (AD), and Lyme disease (LD). However, it is still controversial how the infections play a role in neurological diseases progression. Infections in central nervous system may lead multiple damages in infected and neighboring cells. The infection leads to the activation of inflammatory processes and host immune responses, which acts as defense mechanism and also causes damage to the host neuronal functions and viability...
January 3, 2017: Current Topics in Medicinal Chemistry
https://www.readbyqxmd.com/read/28039187/initial-combination-therapy-with-ambrisentan-and-tadalafil-in-connective-tissue-disease-associated-pulmonary-arterial-hypertension-ctd-pah-subgroup-analysis-from-the-ambition-trial
#12
John Gerry Coghlan, Nazzareno Galiè, Joan Albert Barberà, Adaani E Frost, Hossein-Ardeschir Ghofrani, Marius M Hoeper, Masataka Kuwana, Vallerie V McLaughlin, Andrew J Peacock, Gérald Simonneau, Jean-Luc Vachiéry, Christiana Blair, Hunter Gillies, Karen L Miller, Julia H N Harris, Jonathan Langley, Lewis J Rubin
BACKGROUND: Patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), in particular systemic sclerosis (SSc), had an attenuated response compared with idiopathic PAH in most trials. Thus, there is uncertainty regarding the benefit of PAH-targeted therapy in some forms of CTD-PAH. OBJECTIVE: To explore the safety and efficacy of initial combination therapy with ambrisentan and tadalafil versus ambrisentan or tadalafil monotherapy in patients with CTD-PAH and SSc-PAH enrolled in the AMBITION trial...
December 30, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28038794/prognostic-role-of-exhaled-breath-condensate-ph-and-fraction-exhaled-nitric-oxide-in-systemic-sclerosis-related-interstitial-lung-disease
#13
Alfredo Guillen-Del Castillo, Sara Sánchez-Vidaurre, Carmen P Simeón-Aznar, María J Cruz, Vicente Fonollosa-Pla, Xavier Muñoz
INTRODUCTION: Interstitial lung disease (ILD) is one of the major causes of death in systemic sclerosis (SSc). This study investigated exhaled breath (EB) and exhaled breath condensate (EBC) biomarkers in patients with SSc and analyzed their role as a prognostic tool in SSc-related ILD. METHODS: Fraction exhaled nitric oxide (FeNO) and exhaled carbon monoxide (eCO) measured in EB, together with pH, nitrite, nitrate and interleukin-6 levels measured in EBC were prospectively analyzed in 35 patients with SSc...
December 27, 2016: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28038680/changes-in-plasma-cxcl4-levels-are-associated-with-improvements-in-lung-function-in-patients-receiving-immunosuppressive-therapy-for-systemic-sclerosis-related-interstitial-lung-disease
#14
Elizabeth R Volkmann, Donald P Tashkin, Michael D Roth, Philip J Clements, Dinesh Khanna, Daniel E Furst, Maureen Mayes, Julio Charles, Chi-Hong Tseng, Robert M Elashoff, Shervin Assassi
BACKGROUND: Increased circulatory levels of the chemokine CXCL4 have been associated with the presence of interstitial lung disease (ILD) in an observational study of patients with systemic sclerosis (SSc). The purpose of the present study was to evaluate the relationship between baseline CXCL4 level and extent of ILD in the context of a randomized controlled trial and to determine whether changes in CXCL4 levels in response to immunosuppression are associated with future progression of SSc-ILD...
December 30, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28035300/review-of-common-conditions-associated-with-periodontal-ligament-widening
#15
REVIEW
Hamed Mortazavi, Maryam Baharvand
PURPOSE: The aim of this article is to review a group of lesions associated with periodontal ligament (PDL) widening. MATERIALS AND METHODS: An electronic search was performed using specialized databases such as Google Scholar, PubMed, PubMed Central, Science Direct, and Scopus to find relevant studies by using keywords such as "periodontium", "periodontal ligament", "periodontal ligament space", "widened periodontal ligament", and "periodontal ligament widening"...
December 2016: Imaging Science in Dentistry
https://www.readbyqxmd.com/read/28030334/trichuris-suis-secrete-products-that-reduce-disease-severity-in-a-multiple-sclerosis-model
#16
Christine Søholm Hansen, Henrik Hasseldam, Idahella Hyldgaard Bacher, Stig Milan Thamsborg, Flemming Fryd Johansen, Helene Kringel
Multiple sclerosis is a chronic inflammatory central nervous system (CNS) disease, which affects about 1 in 1000 individuals in the western world. It has been suggested that this relatively high prevalence is linked to a high level of hygiene, i.e. a reduced exposure to various microorganisms, including parasites. Parasites are known to employ different immunomodulatory and antiinflammatory strategies, which enable them to evade destruction by the immune system. We have investigated the immunomodulation by the swine whipworm, Trichuris suis, by measuring the impact of oral administration of T...
March 1, 2017: Acta Parasitologica
https://www.readbyqxmd.com/read/28017237/serum-antibodies-targeting-neurons-of-the-monoaminergic-systems-in-guillain-barr%C3%A3-syndrome
#17
Claudia Rink, Angelika Görtzen, Rüdiger W Veh, Harald Prüss
Guillain-Barré syndrome (GBS) is an autoimmune disease with progressive flaccid paralysis of the extremities. Several auto-antibodies have been identified, binding to myelin, gangliosides, astrocytes or proteins at the nodes of Ranvier. Some epitopes are not confined to the peripheral nerve, suggesting that auto-antibodies may also contribute to symptoms of the central nervous system, which are common in GBS and include anxiety, depression, hallucinations, oneiroid psychosis or fatigue. This notion is supported by treating patients with plasma exchange, resulting in improvement of both central and peripheral symptoms...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28017129/kynurenine-system-and-multiple-sclerosis-pathomechanism-and-drug-targets-with-an-emphasis-on-laquinimod
#18
Zsófia Majláth, Ádám Annus, László Vécsei
Multiple sclerosis is a common chronic, disabling autoimmune neurological disease affecting mainly young adults. In its pathomechanism, neurodegenerative and acute inflammatory characteristics are both involved. Disease-modifying therapies aim to reduce relapse-rate and slow down the deterioration in neurological functions. The currently available therapies fail to exert neuroprotective effects and most of them are associated with potentially toxic side-effects, therefore, ongoing research aims to develop novel drug candidates to cover these therapeutic gaps...
December 23, 2016: Current Drug Targets
https://www.readbyqxmd.com/read/28013199/na-deposition-in-the-fibrotic-skin-of-systemic-sclerosis-patients-detected-by-23na-magnetic-resonance-imaging
#19
Christoph Kopp, Christian Beyer, Peter Linz, Anke Dahlmann, Matthias Hammon, Jonathan Jantsch, Patrick Neubert, Daniela Rosenhauer, Dominik N Müller, Alexander Cavallaro, Kai-Uwe Eckardt, Georg Schett, Friedrich C Luft, Michael Uder, Jörg H W Distler, Jens Titze
OBJECTIVE: Skin fibrosis is the predominant feature of SSc and arises from excessive extracellular matrix deposition. Glycosaminoglycans are macromolecules of the extracellular matrix, which facilitate Na(+) accumulation in the skin. We used (23)Na-MRI to quantify Na(+) in skin. We hypothesized that skin Na(+) might accumulate in SSc and might be a biomarker for skin fibrosis. METHODS: In this observational case-control study, skin Na(+) was determined by (23)Na-MRI using a Na(+) volume coil in 12 patients with diffuse cutaneous SSc and in 21 control subjects...
December 24, 2016: Rheumatology
https://www.readbyqxmd.com/read/28011392/selective-induction-of-p-glycoprotein-at-the-cns-barriers-during-symptomatic-stage-of-an-als-animal-model
#20
Gary N Y Chan, Rebecca A Evans, David B Banks, Emily V Mesev, David S Miller, Ronald E Cannon
P-glycoprotein (P-gp), Breast cancer resistance protein (BCRP) and Multidrug resistance-associated protein 2 (MRP2) residing at the blood-brain barrier (BBB) and the blood-spinal cord barrier (BSCB) are major obstacles for drug delivery to the Central Nervous System (CNS). Disease-induced changes of these xenobiotic transporters at the CNS barriers have been previously documented. Changes in the functional expression of these transporters at the CNS barriers would limit the clinical efficacy of therapeutic agents targeting the CNS...
December 21, 2016: Neuroscience Letters
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