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Connective tissue diseases

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https://www.readbyqxmd.com/read/27933210/educational-needs-of-health-professionals-working-in-rheumatology-in-europe
#1
Theodora P M Vliet Vlieland, Cornelia H M van den Ende, Francoise Alliot-Launois, Catherine Beauvais, Milena Gobbo, Annamaria Iagnocco, Ingrid E Lundberg, Pedro V Munuera-Martínez, Christina H Opava, Yeliz Prior, Anthony Redmond, Hana Smucrova, Dieter Wiek
OBJECTIVE: To explore the availability of postgraduate education for health professionals (HPs) working in rheumatology in Europe, and their perceived educational needs and barriers for participation in current educational offerings. METHODS: Structured interviews were conducted with national representatives of rheumatology HPs' organisations and an online survey among individual HPs was disseminated through existing European League Against Rheumatism (EULAR) networks (10 languages including English)...
2016: RMD Open
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
#2
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/27931995/descriptive-epidemiology-of-deployment-related-medical-conditions-and-shipboard-training-related-injuries-in-a-chinese-navy-population
#3
R-R Qi, J-Q Wang, L-L Pan, W Zhou, J-L Liu, J-T Ju, Y-L Cai
OBJECTIVES: To investigate the deployment-related medical conditions and shipboard tactical training-related injuries in a Chinese Navy population. STUDY DESIGN: A retrospective study with the Chinese Navy was conducted. METHODS: The medical records of 1543 Navy crewmembers from 2011 to 2015 were collected. The distribution and incidence rate (IR) of different types of medical conditions were provided and compared between the Aden Gulf deployment and nondeployment periods...
December 2016: Public Health
https://www.readbyqxmd.com/read/27931961/imaging-of-connective-tissue-diseases-beyond-visceral-organ-imaging
#4
REVIEW
Maurizio Cutolo, Nemanja Damjanov, Barbara Ruaro, Ana Zekovic, Vanessa Smith
Connective tissues diseases (CTDs) can also be diagnosed early by "external" and safe imaging methods beyond the visceral organ analysis. This study aims to explore various imaging techniques used in diagnosing CTDs. Skin impairment in systemic sclerosis (SSc) may be recognized and studied by the modified Rodnan skin score (mRSS), which has some drawbacks, whereas high-frequency ultrasound (US) seems advantageous for the early identification of skin involvement. Salivary gland involvement in Sjögren syndrome (SS) can be assessed using standard tests such as unstimulated salivary flow test, salivary gland scintigraphy or contrast sialography...
August 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27931023/increased-need-for-gastrointestinal-surgery-and-increased-risk-of-surgery-related-complications-in-patients-with-ehlers-danlos-syndrome-a-systematic-review
#5
Marie-Louise Kulas Søborg, Julie Leganger, Jacob Rosenberg, Jakob Burcharth
BACKGROUND/AIMS: Ehlers-Danlos syndromes (EDSs) constitute a rare group of inherited connective tissue diseases, characterized by multisystemic manifestations and general tissue fragility. Most severe complications include vascular and gastrointestinal (GI) emergencies requiring acute surgery. The purpose of this systematic review was to assess the causes of GI-related surgery and related mortality and morbidity in patients with EDSs. METHODS: A systematic search was conducted in PubMed, Embase, and Scopus to identify relevant studies...
December 9, 2016: Digestive Surgery
https://www.readbyqxmd.com/read/27923814/mir-483-targeting-of-ctgf-suppresses-endothelial-to-mesenchymal-transition-therapeutic-implications-in-kawasaki-disease
#6
Ming He, Zhen Chen, Marcy Martin, Jin Zhang, Panjamaporn Sangwung, Brian Woo, Adriana Tremoulet, Chisato Shimizu, Mukesh K Jain, Jane C Burns, John Shyy
RATIONALE: Endothelial-to-mesenchymal transition (EndoMT) is implicated in myofibroblast-like cell-mediated damage to the coronary arterial wall in acute Kawasaki disease (KD) patients, as evidenced by positive staining for connective tissue growth factor (CTGF) and EndoMT markers in KD autopsy tissues. However, little is known about the molecular basis of EndoMT involved in KD. OBJECTIVE: We investigated the microRNA (miRNA) regulation of CTGF and the consequent EndoMT in KD pathogenesis...
December 6, 2016: Circulation Research
https://www.readbyqxmd.com/read/27922338/pathophysiology-of-aortic-aneurysm-insights-from-human-genetics-and-mouse-models
#7
Nicole K Wilson, Russell A Gould, Elena Gallo MacFarlane, Mibava Leducq Consortium
Aneurysms are local dilations of an artery that predispose the vessel to sudden rupture. They are often asymptomatic and undiagnosed, resulting in a high mortality rate. The predisposition to develop thoracic aortic aneurysms is often genetically inherited and associated with syndromes affecting connective tissue homeostasis. This review discusses how elucidation of the genetic causes of syndromic forms of thoracic aortic aneurysm has helped identify pathways that contribute to disease progression, including those activated by TGF-β, angiotensin II and Notch ligands...
December 6, 2016: Pharmacogenomics
https://www.readbyqxmd.com/read/27921043/metabolic-vascular-syndrome-new-insights-into-a-multidimensional-network-of-risk-factors-and-diseases
#8
REVIEW
Gerhard H Scholz, Markolf Hanefeld
BACKGROUND: Since 1981, we have used the term metabolic syndrome to describe an association of a dysregulation in lipid metabolism (high triglycerides, low high-density lipoprotein cholesterol, disturbed glucose homeostasis (enhanced fasting and/or prandial glucose), gout, and hypertension), with android obesity being based on a common soil (overnutrition, reduced physical activity, sociocultural factors, and genetic predisposition). We hypothesized that main traits of the syndrome occur early and are tightly connected with hyperinsulinemia/insulin resistance, procoagulation, and cardiovascular diseases...
October 2016: Visceral Medicine
https://www.readbyqxmd.com/read/27921041/signaling-crosstalk-between-tubular-epithelial-cells-and-interstitial-fibroblasts-after-kidney-injury
#9
REVIEW
Roderick J Tan, Dong Zhou, Youhua Liu
BACKGROUND: A wide variety of kidney diseases ultimately lead to tubulointerstitial damage. The initial site of injury is usually the renal tubules, with activation of fibroblasts occurring later. Self-limited disease is characterized by transient cellular activation with timed deactivation and ultimately a return to normal functioning, whereas sustained responses characterize chronic disease and the development of irreversible fibrosis. The underlying molecular and cellular mechanisms of this cascade of events remain an area of active research...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27920729/transcriptional-network-architecture-of-breast-cancer-molecular-subtypes
#10
Guillermo de Anda-Jáuregui, Tadeo E Velázquez-Caldelas, Jesús Espinal-Enríquez, Enrique Hernández-Lemus
Breast cancer heterogeneity is evident at the clinical, histological and molecular level. High throughput technologies allowed the identification of intrinsic subtypes that capture transcriptional differences among tumors. A remaining question is whether said differences are associated to a particular transcriptional program which involves different connections between the same molecules. In other words, whether particular transcriptional network architectures can be linked to specific phenotypes. In this work we infer, construct and analyze transcriptional networks from whole-genome gene expression microarrays, by using an information theory approach...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27919567/splicing-factor-proline-glutamine-rich-is-a-novel-autoantigen-of-dermatomyositis-and-associated-with-anti-melanoma-differentiation-associated-gene-5-antibody
#11
Yuji Hosono, Ran Nakashima, Satoshi Serada, Kosaku Murakami, Yoshitaka Imura, Hajime Yoshifuji, Koichiro Ohmura, Tetsuji Naka, Tsuneyo Mimori
OBJECTIVE: Anti-MDA5 antibody positive dermatomyositis (DM) and clinically amyopathic DM (CADM) often develop into rapidly progressive interstitial lung disease, but their pathogenesis remains unclear. We observed that sera from DM/CADM patients immunoprecipitated a common 110 kDa polypeptide. We investigated this autoantigen and its clinical significance. METHODS: Autoantibodies were screened in 333 patients with various connective tissue diseases (CTDs) and 20 healthy controls (HCs) by immunoprecipitation with [(35)S]methionine-labeled HeLa cells...
December 2, 2016: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27913855/what-can-the-microstructure-of-stones-tell-us
#12
REVIEW
James C Williams, Elaine Worcester, James E Lingeman
How stones are retained within the kidney while small in size is still not fully understood. In this paper, we show two examples of how stones are retained during early growth: one is growth on Randall's (interstitial) plaque, and the other is growth on mineral that has formed as a luminal plug in a terminal collecting duct. These two mechanisms of stone retention during early growth have distinctive morphologic features that can be seen by methods that show the microscopic structure of the stones. Stones growing on Randall's plaque display an apatite region that is typically not large in size (<0...
December 2, 2016: Urolithiasis
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#13
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27913607/autoimmune-comorbidities-in-hashimoto-s-thyroiditis-different-patterns-of-association-in-adulthood-and-childhood-adolescence
#14
R M Ruggeri, F Trimarchi, G Giuffrida, R Certo, E Cama, A Campennì, A Alibrandi, F De Luca, M Wasniewska
OBJECTIVE: Hashimoto's thyroiditis (HT), the most common autoimmune thyroid disease at any age, is often associated with other autoimmune diseases. The present study was aimed to describe the type and frequency of non-thyroidal autoimmune diseases (NTADs) in HT patients and to delineate the clinical pattern of diseases clustering in pediatric/adolescent and adult age. DESIGN: Cross-sectional study. METHODS: 1053 newly diagnosed HT patients (500 adults (467 F, mean age 40...
February 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27913222/human-liver-regeneration-in-advanced-cirrhosis-is-organized-by-the-portal-tree
#15
Katalin Dezső, András Rókusz, Edina Bugyik, Armanda Szücs, András Szuák, Bence Dorogi, Mátyás Kiss, Ágnes Nemeskéri, Péter Nagy, Sándor Paku
BACKGROUND&AIMS: In advanced cirrhosis new hepatocytic nodules are generated by budding of ductules in areas of parenchymal extinction. However, the vascular alterations in the areas of parenchymal extinction, the blood supply and the structure of the new hepatocytic nodules have not been analyzed in detail. METHODS: Explanted human cirrhotic livers of three different etiologies and two experimental models of cirrhosis in rats were examined thoroughly. 3D reconstruction of the immunohistochemically stained serial sections and casting of human and experimental cirrhotic livers have been used to reveal the structural organization of the regenerative buds...
November 29, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27912147/inhibition-of-endocan-attenuates-monocrotaline-induced-connective-tissue-disease-related-pulmonary-arterial-hypertension
#16
Haiyan Zhao, Yunxin Xue, Yun Guo, Yue Sun, Dongmei Liu, Xiaofei Wang
Connective tissue disease related pulmonary arterial hypertension (CTD-PAH) is characterized by vascular remodeling, endothelial dysfunction and inflammation. Endocan is a novel endothelial dysfunction marker. The aim of the present study was to investigate the role of endocan in CTD-PAH. Monocrotaline (MCT)-induced PAH rats were used as the CTD-PAH model. Short hairpin RNA packed in a lentiviral vector used to inhibit endocan expression was intratracheally instilled in rats prior to the MCT injection. Endocan was found to be increased in the serum and lung of MCT-induced PAH rats...
November 29, 2016: International Immunopharmacology
https://www.readbyqxmd.com/read/27911810/in-vivo-bioluminescence-imaging-reveals-copper-deficiency-in-a-murine-model-of-nonalcoholic-fatty-liver-disease
#17
Marie C Heffern, Hyo Min Park, Ho Yu Au-Yeung, Genevieve C Van de Bittner, Cheri M Ackerman, Andreas Stahl, Christopher J Chang
Copper is a required metal nutrient for life, but global or local alterations in its homeostasis are linked to diseases spanning genetic and metabolic disorders to cancer and neurodegeneration. Technologies that enable longitudinal in vivo monitoring of dynamic copper pools can help meet the need to study the complex interplay between copper status, health, and disease in the same living organism over time. Here, we present the synthesis, characterization, and in vivo imaging applications of Copper-Caged Luciferin-1 (CCL-1), a bioluminescent reporter for tissue-specific copper visualization in living animals...
November 29, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27910296/the-year-since-the-guidelines-a-concise-update-on-recent-advances-in-pulmonary-hypertension
#18
Abhishek Mishra, Maninder Singh, Edo Kaluski
Since the updated pulmonary hypertension (PH) guidelines published in 2015, two major landmark trials have provided additional insight regarding therapeutic algorithms of PH. In this review, we concisely summarized the key findings of peer‑reviewed studies published in the last one year in the field of PH. These studies have enhanced our therapeutic abilities by introducing a new potent agent, selexipag, and by demonstrating the advantage of upfront combination therapy (endothelin receptor antagonist and phosphodiesterase‑5 inhibitor) versus single agent therapy in group 1 PH...
February 2017: Minerva Cardioangiologica
https://www.readbyqxmd.com/read/27907186/inter-tissue-gene-co-expression-networks-between-metabolically-healthy-and-unhealthy-obese-individuals
#19
Lisette J A Kogelman, Jingyuan Fu, Lude Franke, Jan Willem Greve, Marten Hofker, Sander S Rensen, Haja N Kadarmideen
BACKGROUND: Obesity is associated with severe co-morbidities such as type 2 diabetes and nonalcoholic steatohepatitis. However, studies have shown that 10-25 percent of the severely obese individuals are metabolically healthy. To date, the identification of genetic factors underlying the metabolically healthy obese (MHO) state is limited. Systems genetics approaches have led to the identification of genes and pathways in complex diseases. Here, we have used such approaches across tissues to detect genes and pathways involved in obesity-induced disease development...
2016: PloS One
https://www.readbyqxmd.com/read/27906873/polyautoimmunity-a%C3%A2-significant-issue-in%C3%A2-connective-tissue-diseases
#20
Marzena Olesińska, Katarzyna Romanowska-Próchnicka
No abstract text is available yet for this article.
November 28, 2016: Polskie Archiwum Medycyny Wewnętrznej
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