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Connective tissue diseases

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https://www.readbyqxmd.com/read/28226694/multi-output-gaussian-processes-for-enhancing-resolution-of-diffusion-tensor-fields
#1
Hernan Dario Vargas Cardona, Alvaro A Orozco, Mauricio A Alvarez, Hernan Dario Vargas Cardona, Alvaro A Orozco, Mauricio A Alvarez, Hernan Dario Vargas Cardona, Mauricio A Alvarez, Alvaro A Orozco
Second order diffusion tensor (DT) fields are widely used in several clinical applications: brain fibers connections, diagnosis of neuro-degenerative diseases, image registration, brain conductivity models, etc. However, due to current acquisition protocols and hardware limitations in MRI machines, the diffusion magnetic resonance imaging (dMRI) data is obtained with low spatial resolution (1 or 2 mm(3) for each voxel). This issue can be significant, because tissue fibers are much smaller than voxel size. Interpolation has become in a successful methodology for enhancing spatial resolution of DT fields...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28226541/combined-reconstruction-of-atrial-morphology-and-paracardial-fat-depositions-to-study-remodeling-in-atrial-fibrillation
#2
N Popovic, P Haemers, R Willems, P Claus, N Popovic, P Haemers, R Willems, P Claus, P Claus, P Haemers, N Popovic, R Willems
Atrial fibrillation (AF) is the most frequent sustained cardiac arrhythmia. AF is shown to be a progressive disease, which is strongly associated with important structural (morphological and histological), electrical and mechanical remodeling of the left atrium (LA). Adipose tissue accumulation has been shown to be a risk factor in AF. We set out to explore the connection between adipose tissue coverage of the LA surface, for both the complete LA and specific regions and the progression of AF. In this study we propose a methodology to study the regional coverage of the left-atrial wall with adipose tissue based on magnetic resonance data...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28225753/complement-drives-glucosylceramide-accumulation-and-tissue-inflammation-in-gaucher-disease
#3
Manoj K Pandey, Thomas A Burrow, Reena Rani, Lisa J Martin, David Witte, Kenneth D Setchell, Mary A Mckay, Albert F Magnusen, Wujuan Zhang, Benjamin Liou, Jörg Köhl, Gregory A Grabowski
Gaucher disease is caused by mutations in GBA1, which encodes the lysosomal enzyme glucocerebrosidase (GCase). GBA1 mutations drive extensive accumulation of glucosylceramide (GC) in multiple innate and adaptive immune cells in the spleen, liver, lung and bone marrow, often leading to chronic inflammation. The mechanisms that connect excess GC to tissue inflammation remain unknown. Here we show that activation of complement C5a and C5a receptor 1 (C5aR1) controls GC accumulation and the inflammatory response in experimental and clinical Gaucher disease...
February 22, 2017: Nature
https://www.readbyqxmd.com/read/28224486/human-herpes-virus-6-encephalitis-causing-severe-anterograde-amnesia-associated-with-rituximab-azathioprine-and-prednisolone-combination-therapy-for-dermatomyositis
#4
Thomas Baumer, Charlie Fry, Sebastian Luppe, Harsha Gunawardena, Kasia Sieradzan
Human herpes virus-6 (HHV-6) reactivation is a well-recognised complication following haematological stem cell transplantation, but it is novel in the context of combination immunomodulatory therapy for autoimmune disease. We report a case of severe anterograde amnesia caused by HHV-6 encephalitis in a young female patient on rituximab, azathioprine and prednisolone for dermatomyositis (DM). The use of targeted biologic treatments for systemic autoimmune connective tissue diseases (CTDs) is increasing, particularly when refractory to conventional management...
February 21, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/28224343/amphotericin-b-increases-transglutaminase-2-expression-associated-with-upregulation-of-endocytotic-activity-in-mouse-microglial-cell-line-bv-2
#5
Kenji Kawabe, Katsura Takano, Mitsuaki Moriyama, Yoichi Nakamura
Amphotericin B (AmB), a polyene antibiotic, is reported to cause the microglial activation to induce nitric oxide (NO) production and proinflammatory cytokines expression, and change neurotrophic factors expression in cultured microglia (Motoyoshi et al. in Neurochem Int 52:1290-1296, 2008). On the other hand, tissue-type transglutaminase (TG2) is involved in connection to phagocytes with apoptotic cells. Engulfment of neurons by activated microglia is thought to cause neurodegenerative diseases but detail is unclear, and involvement of TG2 in phagocytosis has been reported in our previous study using lipopolysaccharide-stimulated BV-2 cells (Kawabe et al...
February 21, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28223177/pneumocystis-pneumonia-in-patients-with-inflammatory-or-autoimmune-diseases-usefulness-of-lymphocyte-subtyping
#6
Yi Li, Marc Ghannoum, Chuntao Deng, Yanxia Gao, Huadong Zhu, Xuezhong Yu, Valery Lavergne
OBJECTIVES: No consensus currently exists on indications for Pneumocystis jirovecii prophylaxis in patients with inflammatory or autoimmune diseases. The main objective was to identify biomarkers associated with Pneumocystis jirovecii pneumonia (PcP) in this population. METHODS: A retrospective study was carried out at Beijing Union Medical College Hospital (2003-2014). All patients with an inflammatory or autoimmune diseases presenting with acute onset of fever and respiratory symptoms were included...
February 18, 2017: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/28220742/calcific-uraemic-arteriolopathy-calciphylaxis-in-patients-on-renal-replacement-therapy
#7
S Sebastian, H F Jordaan, J W Schneider, M R Moosa, M R Davids
BACKGROUND: Calcific uraemic arteriolopathy (calciphylaxis) is an unusual and potentially fatal condition characterised by small-vessel calcification and ischaemic skin necrosis. It mainly affects patients with end-stage renal disease (ESRD) on haemodialysis, but may rarely occur in the absence of ESRD in conditions such as primary hyperparathyroidism, malignancy, alcoholic liver disease and connective tissue disease. METHODS: We reviewed the records of all patients diagnosed with calciphylaxis while on renal replacement therapy at Tygerberg Hospital, Cape Town, South Africa, between 1990 and 2014, to describe its presentation, course and final outcome...
January 30, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28220607/neurological-and-spinal-manifestations-of-the-ehlers-danlos-syndromes
#8
Fraser C Henderson, Claudiu Austin, Edward Benzel, Paolo Bolognese, Richard Ellenbogen, Clair A Francomano, Candace Ireton, Petra Klinge, Myles Koby, Donlin Long, Sunil Patel, Eric L Singman, Nicol C Voermans
The Ehlers-Danlos syndromes (EDS) are a heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin extensibility, and tissue fragility. This communication briefly reports upon the neurological manifestations that arise including the weakness of the ligaments of the craniocervical junction and spine, early disc degeneration, and the weakness of the epineurium and perineurium surrounding peripheral nerves. Entrapment, deformation, and biophysical deformative stresses exerted upon the nervous system may alter gene expression, neuronal function and phenotypic expression...
February 21, 2017: American Journal of Medical Genetics. Part C, Seminars in Medical Genetics
https://www.readbyqxmd.com/read/28217887/gastric-antral-vascular-ectasia-in-systemic-sclerosis-where-do-we-stand
#9
REVIEW
Hala El-Gendy, Kyrillus S Shohdy, Gehad G Maghraby, Kerolos Abadeer, Moustafa Mahmoud
Gastric antral vascular ectasia (GAVE) continues to be a challenge in both diagnosis and treatment. GAVE has a diverse group of associations and presumed causes, including cirrhosis, chronic renal failure and autoimmune connective tissue diseases. However, in most occasions, the management plan of GAVE itself is the same whatever the underlying disease by using Argon plasma coagulation (APC). Herein, we will discuss three cases of systemic sclerosis-associated GAVE presenting with either acute or chronic gastrointestinal bleeding showing variable responses to APC...
February 20, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28217691/%C3%AE-1-antitrypsin-deficiency-a-misfolded-secretory-protein-variant-with-unique-effects-on-the-endoplasmic-reticulum
#10
David H Perlmutter
In the classical form of α1-antitrypsin deficiency (ATD) a point mutation leads to accumulation of a misfolded secretory glycoprotein in the endoplasmic reticulum (ER) of liver cells and so ATD has come to be considered a prototypical ER storage disease. It is associated with two major types of clinical disorders, chronic obstructive pulmonary disease (COPD) by loss-of-function mechanisms and hepatic cirrhosis and carcinogenesis by gain-of-function mechanisms. The lung disease predominantly results from proteolytic damage to the pulmonary connective tissue matrix because of reduced levels of protease inhibitor activity of α1-anitrypsin (AT) in the circulating blood and body fluids...
September 2016: Endoplasmic Reticulum Stress in Diseases
https://www.readbyqxmd.com/read/28217270/neurological-assessment-and-nerve-conduction-study-findings-in-22-patients-with-alkaptonuria-from-jordan
#11
Omar Alrawashdeh, Mohammad Alsbou, Hamed Alzoubi, Hani Al-Shagahin
Alkaptonuria is a rare metabolic disease characterised by accumulative deposition of homogentisic acid in the connective tissue of the body. This results in early degeneration of tendons, cartilages, heart valves, and other tissues. The main objective of the study is to examine the possibility of the nervous system involvement in patients with alkaptonuria The sample consists of two groups; 22 patients with AKU and 20 controls. A neurological assessment has been carried out including detailed medical history, neurological examination, and a nerve conduction study of the nerves of the dominant hand...
November 2, 2016: Neurology International
https://www.readbyqxmd.com/read/28216321/effects-of-dmso-on-a-rabbit-ear-hypertrophic-scar-model-a-controlled-randomized-experimental-study
#12
Elif Sari, Bulent Bakar, Gungor Cagdas Dincel, Fatma Azize Budak Yildiran
Dimethyl sulfoxide (DMSO) is an anti-inflammatory, antibacterial, analgesic drug widely used to treat several diseases as reported in the literature. It has a detractive effect on collagen deposition in the abnormal tissue. This study aimed to investigate the possible therapeutic effects of DMSO on hypertrophic scar formation in rabbits. Twenty-four New Zealand male albino rabbits were randomly divided into four groups: control, sham, DMSO, and TRA (triamcinolone acetonide). Except the control group, punch biopsy defects were created on each animal's right ear...
January 23, 2017: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
https://www.readbyqxmd.com/read/28216193/frequency-of-concomitant-fibromyalgia-in-rheumatic-diseases-monocentric-study-of-691-patients
#13
Angelique Fan, Bruno Pereira, Anne Tournadre, Zuzana Tatar, Sandrine Malochet-Guinamand, Sylvain Mathieu, Marion Couderc, Martin Soubrier, Jean-Jacques Dubost
OBJECTIVE: Fibromyalgia (FM) is a confounding factor for diagnosing and assessing rheumatic disease activity. This study sought to assess the extent of this syndrome in rheumatism patients at a French rheumatology department. METHOD: This monocentric epidemiological study enrolled all patients consulting due to rheumatoid arthritis (RA), spondyloarthritis (SpA), or connective tissue disease (CTD). FM diagnosis was confirmed or excluded according to the rheumatologist opinion and the 1990 American College of Rheumatology (ACR) criteria...
January 18, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28216081/decreased-calcium-pump-expression-in-human-erythrocytes-is-connected-to-a-minor-haplotype-in-the-atp2b4-gene
#14
Boglárka Zámbó, György Várady, Rita Padányi, Edit Szabó, Adrienn Németh, Tamás Langó, Ágnes Enyedi, Balázs Sarkadi
Plasma membrane Ca(2+)-ATPases are key calcium exporter proteins in most tissues, and PMCA4b is the main calcium transporter in the human red blood cells (RBCs). In order to assess the expression level of PMCA4b, we have developed a flow cytometry and specific antibody binding method to quantitatively detect this protein in the erythrocyte membrane. Interestingly, we found several healthy volunteers showing significantly reduced expression of RBC-PMCA4b. Western blot analysis of isolated RBC membranes confirmed this observation, and indicated that there are no compensatory alterations in other PMCA isoforms...
February 3, 2017: Cell Calcium
https://www.readbyqxmd.com/read/28214498/vasculitic-and-autoimmune-wounds
#15
REVIEW
Victoria K Shanmugam, Divya Angra, Hamza Rahimi, Sean McNish
OBJECTIVE: Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions...
March 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28213717/the-minor-collagens-in-articular-cartilage
#16
REVIEW
Yunyun Luo, Dovile Sinkeviciute, Yi He, Morten Karsdal, Yves Henrotin, Ali Mobasheri, Patrik Önnerfjord, Anne Bay-Jensen
Articular cartilage is a connective tissue consisting of a specialized extracellular matrix (ECM) that dominates the bulk of its wet and dry weight. Type II collagen and aggrecan are the main ECM proteins in cartilage. However, little attention has been paid to less abundant molecular components, especially minor collagens, including type IV, VI, IX, X, XI, XII, XIII, and XIV, etc. Although accounting for only a small fraction of the mature matrix, these minor collagens not only play essential structural roles in the mechanical properties, organization, and shape of articular cartilage, but also fulfil specific biological functions...
February 17, 2017: Protein & Cell
https://www.readbyqxmd.com/read/28213592/the-evidence-of-benefits-of-exercise-training-in-interstitial-lung-disease-a-randomised-controlled-trial
#17
Leona M Dowman, Christine F McDonald, Catherine J Hill, Annemarie L Lee, Kathryn Barker, Claire Boote, Ian Glaspole, Nicole S L Goh, Anne M Southcott, Angela T Burge, Rebecca Gillies, Alicia Martin, Anne E Holland
BACKGROUND: Uncertainty exists regarding the clinical relevance of exercise training across the range of interstitial lung diseases (ILDs). OBJECTIVE: To establish the impact of exercise training in patients with ILDs of differing aetiology and severity. METHODS: 142 participants with ILD (61 idiopathic pulmonary fibrosis (IPF), 22 asbestosis, 23 connective tissue disease-related ILD (CTD-ILD) and 36 with other aetiologies) were randomised to either 8 weeks of supervised exercise training or usual care...
February 17, 2017: Thorax
https://www.readbyqxmd.com/read/28213468/microencapsulation-of-lefty-secreting-engineered-cells-for-pulmonary-fibrosis-therapy-in-mice
#18
Hongge Ma, Shupei Qiao, Zeli Wang, Shuai Geng, Yufang Zhao, Xiaolu Hou, Weiming Tian, Xiongbiao Chen, Lifen Yao
Idiopathic pulmonary fibrosis (IPF) is a progressive disease that causes unremitting deposition of extracellular matrix proteins, thus resulting in distortion of the pulmonary architecture and impaired gas exchange. Associated with high morbidity and mortality, IPF is generally refractory to current pharmacological therapies. Lefty A, a potent inhibitor of transforming growth factor (TGF)-β signaling, has been shown to have promising antifibrotic ability in vitro for the treatment of renal fibrosis and other potential organ fibroses...
February 17, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#19
REVIEW
Antonio Alberto Zuppa, Riccardo Riccardi, Simonetta Frezza, Francesca Gallini, Rita Maria Paola Luciano, Giovanni Alighieri, Costantino Romagnoli, Sara De Carolis
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur...
February 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28212278/the-effect-of-the-human-peptide-ghk-on-gene-expression-relevant-to-nervous-system-function-and-cognitive-decline
#20
Loren Pickart, Jessica Michelle Vasquez-Soltero, Anna Margolina
Neurodegeneration, the progressive death of neurons, loss of brain function, and cognitive decline is an increasing problem for senior populations. Its causes are poorly understood and therapies are largely ineffective. Neurons, with high energy and oxygen requirements, are especially vulnerable to detrimental factors, including age-related dysregulation of biochemical pathways caused by altered expression of multiple genes. GHK (glycyl-l-histidyl-l-lysine) is a human copper-binding peptide with biological actions that appear to counter aging-associated diseases and conditions...
February 15, 2017: Brain Sciences
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