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Systemic sclerosis

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https://www.readbyqxmd.com/read/29916558/increased-right-atrial-volume-measured-with-cardiac-magnetic-resonance-is-associated-with-worse-clinical-outcome-in-patients-with-pre-capillary-pulmonary-hypertension
#1
Anna Bredfelt, Göran Rådegran, Roger Hesselstrand, Håkan Arheden, Ellen Ostenfeld
AIMS: Pre-capillary pulmonary hypertension (PHpre-cap ) has a poor prognosis, especially when caused by pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-PAH). Whether cardiac magnetic resonance (CMR)-based quantification of atrial volumes in PHpre-cap is beneficial in risk assessment is unknown. The aims were to investigate if (i) atrial volumes using CMR are associated with death or lung transplantation in PHpre-cap , (ii) atrial volumes differ among four unmatched major PHpre-cap subgroups, and (iii) atrial volumes differ between SSc-PAH and idiopathic/familial PAH (IPAH/FPAH) when matched for pulmonary vascular resistance (PVR)...
June 19, 2018: ESC Heart Failure
https://www.readbyqxmd.com/read/29916014/an-epigenetic-spin-to-als-and-ftd
#2
Mark T W Ebbert, Rebecca J Lank, Veronique V Belzil
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two devastating and lethal neurodegenerative diseases seen comorbidly in up to 15% of patients. Despite several decades of research, no effective treatment or disease-modifying strategies have been developed. We now understand more than before about the genetics and biology behind ALS and FTD, but the genetic etiology for the majority of patients is still unknown and the phenotypic variability observed across patients, even those carrying the same mutation, is enigmatic...
2018: Advances in Neurobiology
https://www.readbyqxmd.com/read/29915595/dysregulated-microrna-involvement-in-multiple-sclerosis-by-induction-of-t-helper-17-cell-differentiation
#3
REVIEW
Chen Chen, Yifan Zhou, Jingqi Wang, Yaping Yan, Lisheng Peng, Wei Qiu
Multiple sclerosis (MS) is an immune-mediated demyelinating disease of the central nervous system. Growing evidence has proven that T helper 17 (Th17) cells are one of the regulators of neuroinflammation mechanisms in MS disease. Researchers have demonstrated that some microRNAs (miRNAs) are associated with disease activity and duration, even with different MS patterns. miRNAs regulate CD4+ T cells to differentiate toward various T cell subtypes including Th17 cells. In this review, we discuss the possible mechanisms of miRNAs in MS pathophysiology by regulating CD4+ T cell differentiation into Th17 cells, and potential miRNA targets for current disease-modifying treatments...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29915590/profiling-of-canonical-and-non-traditional-cytokine-levels-in-interferon-%C3%AE-treated-relapsing-remitting-multiple-sclerosis-patients
#4
Chiara D'Angelo, Marcella Reale, Erica Costantini, Marta Di Nicola, Italo Porfilio, Clara de Andrés, Lidia Fernández-Paredes, Silvia Sánchez-Ramón, Livia Pasquali
Background: Multiple sclerosis (MS) is a chronic, progressive autoimmune disease of the central nervous system in which inflammation plays a key role in the induction, development, and progression. Most of the MS patients present with relapsing-remitting (RR) form, characterized by flare-ups followed by periods of recovery. Many inflammatory and anti-inflammatory cytokines have been proposed as backers in MS pathogenesis, and the balance between these differing cytokines can regulate MS severity...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29914773/transcranial-direct-current-stimulation-a-glimmer-of-hope-for-multiple-sclerosis-fatigue
#5
Samar S Ayache, Moussa A Chalah
Multiple sclerosis (MS) is a neurological disease of the central nervous system characterized by inflammation, demyelination and neurodegeneration. Throughout the disease process, patients may complain of a panel of sensory, motor, cognitive and behavioral symptoms. Fatigue is a debilitating manifestation of central nervous system diseases with physical, cognitive and psychosocial dimensions. In MS, fatigue could be very frequent concerning up to 90% of patients and may have a drastic impact on their quality of life...
June 15, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29914402/environmental-exposures-and-the-risk-of-multiple-sclerosis-in-saudi-arabia
#6
Osama Al Wutayd, Ashri Gad Mohamed, Jameelah Saeedi, Hessa Al Otaibi, Mohammed Al Jumah
BACKGROUND: Multiple sclerosis (MS) is the most common non-traumatic condition that leads to disability among young individuals. It is associated with demyelination, inflammation, and neurodegeneration within the central nervous system. Information on risk factors of multiple sclerosis is crucial for the prevention and control of the disease. The aim of this study was to determine risk factors of MS among adults in Saudi Arabia. METHODS: A matched multicenter case-control study, including 307 MS patients and 307 healthy controls, was conducted in MS clinics and wards in 3 main cities of Saudi Arabia...
June 19, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29913923/national-guidelines-for-evaluating-pain-patients-legal-right-to-prioritised-health-care-at-multidisciplinary-pain-clinics-in-norway-implemented-2009
#7
Karen Walseth Hara, Petter Borchgrevink
Background All nations are posed with the challenge of deciding how to allocate limited health care resources. A Patients' Rights Law from 1999 gives patients in Norway with a serious health condition, for which there is efficacious and cost-effective treatment, a legal right to receive health care from the National Health Care system. Methods Recently national guidelines have been produced for implementing these legal rights within 32 fields of specialist health care. One of these fields deals with serious chronic pain conditions...
December 29, 2017: Scandinavian Journal of Pain
https://www.readbyqxmd.com/read/29912771/systemic-sclerosis-in-the-intensive-care-unit-a-case-series-and-literature-review
#8
Juan Naranjo-Escobar, Andrés A Hormaza, Iván Posso-Osorio, Jessica Pino-Escobar, Juan F Betancur, Gabriel J Tobón
INTRODUCTION: Systemic sclerosis (SSc) is a multisystemic autoimmune disease. Few studies have focused on the outcomes of SSC patients who require intensive care unit (ICU) admission, largely due to the absence of protocols for the optimal management of this disease during an ICU stay. OBJECTIVES: This study aimed to describe the outcomes of a series of SSc patients admitted to the ICU at a single center in Cali, Colombia. METHODS: Case series of SSc patients admitted to the ICU were retrospectively reviewed...
June 15, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29911471/successful-implementation-of-an-automated-electronic-support-system-for-patient-safety-monitoring-the-alemtuzumab-in-multiple-sclerosis-safety-systems-ams3-study
#9
Stephen W Reddel, Michael H Barnett, Sean Riminton, Tej Dugal, Katherine Buzzard, Chenu Tim Wang, Fiona Fitzgerald, Heidi N Beadnall, Diane Erickson, David Gahan, Daniel Wang, Toby Ackland, Richard Thompson
BACKGROUND: Alemtuzumab is a highly effective treatment for relapsing-remitting multiple sclerosis (MS) but requires ongoing pathology monitoring for autoimmune adverse effects. The Alemtuzumab in MS Safety Systems (AMS3) study evaluated the implementation of an automated pathology-monitoring system. OBJECTIVES: To develop an efficient automated clinical decision support system (CDSS) to electronically prompt and track pathology collection and to provide prescribers and patients with customised alerts of abnormal results for identified risks...
June 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/29911021/-mycobacterium-avium-subspecies-paratuberculosis-a-possible-causative-agent-in-human-morbidity-and-risk-to-public-health-safety
#10
REVIEW
Mary Garvey
Mycobacterium avium subspecies paratuberculosis is a bacterial parasite and the causative agent of paratuberculosis, a disease predominately found in cattle and sheep. Infection with this microorganism results in substantial farming economic losses and animal morbidity. The link between infection with this pathogen and human disease has been theorised for many years with Crohn's disease being one of many suspected resultant conditions. Mycobacterium avium may be spread from animal to human hosts by water and foodborne transmission routes, where the foodborne route of exposure represents a significant risk for susceptible populations, namely children and the immune-compromised...
2018: Open Veterinary Journal
https://www.readbyqxmd.com/read/29910814/the-role-of-natural-killer-group-2-member-d-in-chronic-inflammation-and-autoimmunity
#11
REVIEW
Marina Babic, Chiara Romagnani
Current medicine and medical science puts great effort into elucidating the basis of chronicity and finding appropriate treatments for inflammatory diseases; however, the mechanisms driving aberrant immune responses are mostly unknown and deserve further study. Of particular interest is the identification of checkpoints that regulate the function and differentiation of pro-inflammatory cells during pathogenesis, along with means of their modulation for therapeutic purposes. Natural killer group 2, member D (NKG2D) is a potent activator of the immune system, known as a sensor for "induced-self" ligands, i...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29909388/lymphedema-secondary-to-limited-cutaneous-systemic-sclerosis
#12
Sónia Moreira, Jorge Crespo, Lèlita Santos
Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterised by vascular abnormalities, immune system activation and fibrosis. Lymphatic involvement in SSc was described more recently and starts in early stages. This report describes a 46-year-old patient who developed over the last 2 years asymmetric lymphedema in lower extremities. Compromise in lymphatic drainage was confirmed by lymphoscintigraphy. She also presented Raynaud's phenomenon, a scleroderma pattern in nailfold capillaroscopy, cutaneous thickening and anticentromere antibodies, which together resulted in a new diagnosis of limited cutaneous SSc...
June 15, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29907667/symptoms-of-autonomic-dysfunction-in-systemic-sclerosis-assessed-by-the-compass-31-questionnaire
#13
Brittany L Adler, James W Russell, Laura K Hummers, Zsuzsanna H McMahan
OBJECTIVE: Autonomic dysfunction is a known complication of systemic sclerosis (SSc) that can affect vascular tone, gastrointestinal (GI) motility, heart rate, and blood pressure control. We sought to quantify autonomic symptom burden in SSc, and to define the characteristics of patients with SSc and autonomic dysfunction. METHODS: Patients with SSc were consecutively recruited during routine clinical visits at the Johns Hopkins Scleroderma Center and asked to complete the Composite Autonomic Symptom Score (COMPASS)-31 questionnaire, a validated tool to assess symptoms of autonomic dysfunction...
June 15, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29905933/the-classification-of-pediatric-and-young-adult-renal-cell-carcinomas-registered-on-the-children-s-oncology-group-cog-protocol-aren03b2-after-focused-genetic-testing
#14
Mariana M Cajaiba, Lisa M Dyer, James I Geller, Lawrence J Jennings, David George, Dawn Kirschmann, Stephen M Rohan, Nicholas G Cost, Geetika Khanna, Elizabeth A Mullen, Jeffrey S Dome, Conrad V Fernandez, Elizabeth J Perlman
BACKGROUND: Renal cell carcinomas (RCCs) are rare in young patients. Knowledge of their pathologic and molecular spectrum remains limited, and no prospective studies have been performed to date in this population. This study analyzes patients diagnosed with RCC who were prospectively enrolled in the AREN03B2 Children's Oncology Group (COG). The objective was to classify these tumors with the aid of focused genetic testing and to characterize their features. METHODS: All tumors registered as RCC by central review were retrospectively re-reviewed and underwent additional ancillary studies...
June 15, 2018: Cancer
https://www.readbyqxmd.com/read/29905552/-modern-approaches-to-the-treatment-of-multiple-sclerosis-review-and-clinical-case
#15
A Khamidulla, G Kabdrakhmanova, A Utepkaliyeva, D Darin, Zh Urasheva
Multiple sclerosis is a chronic dysimmune neurodegenerative disease of the central nervous system, that affects people of working age and inevitably leads to disability. Treatment of the disease is one of the urgent problems of modern clinical neurology, which is explained by the variety of clinical variants of the flow, the lack of an effective method of treatment. This article is devoted to a review of modern approaches to the issues of etiology, pathogenesis and treatment of multiple sclerosis. Also, the classification of multiple sclerosis depending on the course of the disease, data on modern approaches to the treatment of multiple sclerosis, the criteria for the appointment of differentiated therapy depending on the course of the disease with the use of Disease-Modifying Drugs (DMDs) are given...
May 2018: Georgian Medical News
https://www.readbyqxmd.com/read/29905407/pathophysiological-implications-of-actin-free-gc-globulin-concentration-changes-in-blood-plasma-and-cerebrospinal-fluid-collected-from-patients-with-alzheimer-s-disease-and-other-neurological-disorders
#16
Alina Kułakowska, Joanna Tarasiuk, Katarzyna Kapica-Topczewska, Monika Chorąży, Robert Pogorzelski, Agnieszka Kulczyńska-Przybik, Barbara Mroczko, Robert Bucki
BACKGROUND: The extracellular actin scavenging system (EASS) is composed of plasma Gc-globulin and gelsolin, and is responsible for the elimination of toxic actin from the bloodstream. OBJECTIVES: In this study, we assessed the actin-free Gc-globulin concentrations in blood plasma and cerebrospinal fluid (CSF) obtained from subjects with neurodegenerative and inflammatory diseases of the central nervous system (CNS) as well as in a control group. MATERIAL AND METHODS: Using an enzyme-linked immunosorbent assay (ELISA), we measured the actinfree Gc-globulin concentrations in blood plasma and CSF obtained from subjects diagnosed with Alzheimer's disease (AD) (n = 20), amyotrophic lateral sclerosis (ALS) (n = 12), multiple sclerosis (MS) (n = 42), tick-borne encephalitis (TBE) (n = 12), and from a control group (n = 20)...
June 15, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/29904379/tolerogenic-dendritic-cells-as-a-promising-antigen-specific-therapy-in-the-treatment-of-multiple-sclerosis-and-neuromyelitis-optica-from-preclinical-to-clinical-trials
#17
REVIEW
Georgina Flórez-Grau, Irati Zubizarreta, Raquel Cabezón, Pablo Villoslada, Daniel Benitez-Ribas
The identification of activated T-lymphocytes restricted to myelin-derived immunogenic peptides in multiple sclerosis (MS) and aquaporin-4 water channel in neuromyelitis optica (NMO) in the blood of patients opened the possibility for developing highly selective and disease-specific therapeutic approaches. Antigen presenting cells and in particular dendritic cells (DCs) represent a strategy to inhibit pro-inflammatory T helper cells. DCs are located in peripheral and lymphoid tissues and are essential for homeostasis of T cell-dependent immune responses...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29903394/analysis-of-the-diagnostic-pathway-and-delay-in-patients-with-amyotrophic-lateral-sclerosis-in-the-valencian-community
#18
J F Vázquez-Costa, M Martínez-Molina, M Fernández-Polo, V Fornés-Ferrer, M Frasquet-Carrera, T Sevilla-Mantecón
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is an insidious, clinically heterogeneous neurodegenerative disease associated with a diagnostic delay of approximately 12 months. No study conducted to date has analysed the diagnostic pathway in Spain. METHODS: We gathered data on variables related to the diagnostic pathway and delay for patients diagnosed with ALS between October 2013 and July 2017. RESULTS: The study included 143 patients with ALS (57% men; 68% spinal onset)...
June 11, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/29903027/laquinimod-protects-the-optic-nerve-and-retina-in-an-experimental-autoimmune-encephalomyelitis-model
#19
Anna T Wilmes, Sabrina Reinehr, Sandra Kühn, Xiomara Pedreiturria, Laura Petrikowski, Simon Faissner, Ilya Ayzenberg, Gesa Stute, Ralf Gold, H Burkhard Dick, Ingo Kleiter, Stephanie C Joachim
BACKGROUND: The oral immunomodulatory agent laquinimod is currently evaluated for multiple sclerosis (MS) treatment. Phase II and III studies demonstrated a reduction of degenerative processes. In addition to anti-inflammatory effects, laquinimod might have neuroprotective properties, but its impact on the visual system, which is often affected by MS, is unknown. The aim of our study was to investigate potential protective effects of laquinimod on the optic nerve and retina in an experimental autoimmune encephalomyelitis (EAE) model...
June 14, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29902695/therapeutic-strategies-for-targeting-neurodegenerative-protein-misfolding-disorders
#20
REVIEW
Robert H Scannevin
Neurodegenerative diseases can arise from a multitude of different pathological drivers, however protein misfolding appears to be a common molecular feature central to several disorders. Protein folding, and attainment of correct secondary and tertiary structure, is essential for proper protein function. Protein misfolding gives rise to structural perturbations that can result in loss of protein function or a gain of toxic function, such as through aggregation, either of which can initiate and propagate biological responses that are deleterious to cells...
June 11, 2018: Current Opinion in Chemical Biology
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