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Raynaud disease

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https://www.readbyqxmd.com/read/28429690/-our-experience-in-the-diagnosis-and-treatment-of-postural-orthostatic-tachycardia-syndrome-vasovagal-syncope-and-inappropriate-sinus-tachycardia-in-children
#1
Sezen Ugan Atik, Reyhan Dedeoğlu, Aida Koka, Funda Öztunç
OBJECTIVES: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded...
April 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28420068/pentraxin-3-levels-are-associated-with-vasculitis-and-disease-activity-in-childhood-onset-systemic-lupus-erythematosus
#2
S Sahin, A Adrovic, K Barut, S Durmus, R Gelisgen, H Uzun, O Kasapcopur
Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28420047/organ-damage-accrual-and-distribution-in-systemic-lupus-erythematosus-patients-followed-up-for-more-than-10-years
#3
M Taraborelli, I Cavazzana, N Martinazzi, M Grazia Lazzaroni, M Fredi, L Andreoli, F Franceschini, A Tincani
Objective The aim of this study was to determine the prevalence, predictors and progression of organ damage in a monocentric cohort of systemic lupus erythematosus patients with a long follow-up. Organ damage was assessed by the Systemic Lupus International Collaborating Clinics Damage Index one year after diagnosis and every five years. Disease activity was measured by the systemic lupus erythematosus disease activity index (SLEDAI)-2K at the beginning of the follow-up. Univariate and multivariable analyses were used to detect items associated with damage...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28417469/oesophageal-involvement-in-26-consecutive-patients-with-mucous-membrane-pemphigoid
#4
O Zehou, J-J Raynaud, C Le Roux-Villet, M Alexandre, G Airinei, F Pascal, M Heller, N Lièvre, L Laroche, F Caux, R Benamouzig, C Prost-Squarcioni
BACKGROUND: Oesophageal involvement of mucous membrane pemphigoid (MMP) has not yet been thoroughly described. OBJECTIVES: This study was undertaken to systematically characterise the endoscopic lesions of a series of patients with oesophageal symptoms seen in a referral centre for autoimmune bullous diseases. METHODS: Clinical, endoscopic and immunological findings of consecutively referred MMP patients with oesophageal involvement, systemic and endoscopic treatments and follow-up, are described...
April 18, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28417151/a-review-of-the-role-and%C3%A2-clinical-utility-of-anti-ro52-trim21-in-systemic-autoimmunity
#5
REVIEW
Adrian Y S Lee
Anti-Ro52/tripartite motif-containing 21 (TRIM21) is a ubiquitous antibody found in a number of systemic autoimmune conditions including Sjögren's syndrome, systemic lupus erythematosus and systemic sclerosis, appearing in about half of these patients. Once coupled with its closely related antibody, anti-Ro60 as the anti-SSA antibody, anti-Ro52 is emerging as a unique antibody with direct pathogenic disease involvement and distinct clinical properties. As a result, recent attention has turned to this antibody and its clinical associations and utility...
April 17, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28414153/inflammatory-myopathy-associated-with-myasthenia-gravis-with-and-without-thymic-pathology-report-of-four-cases-and-literature-review
#6
REVIEW
Ernestina Santos, Ester Coutinho, Ana Martins da Silva, António Marinho, Carlos Vasconcelos, Ricardo Taipa, Manuel Melo Pires, Guilherme Gonçalves, Carlos Lopes, Maria Isabel Leite
INTRODUCTION: the association of myasthenia gravis (MG) and inflammatory myopathy is rare and often only one of the diseases is diagnosed. Thymus pathology may be in the origin of such disease association. METHODS: we described four patients with both MG and inflammatory myopathy. RESULTS: these cases correspond to 2.3% of our MG cohort. Case 1: MG, polymyositis and thymolipoma; case 2: MG and necrotizing myopathy without thymic pathology on a background of scleroderma, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST); case 3: MG and dermatomyositis without thymic pathology; case 4: MG and dermatomyositis with type C thymoma...
April 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28391171/a-two-scale-modeling-and-computational-framework-for-vibration-induced-raynaud-syndrome
#7
Yue Hua, Pierre Lemerle, Jean-François Ganghoffer
Hand-Arm Vibration syndrome (HAVS), usually caused by long-term use of hand-held power tools, can in certain manifestations alter the peripheral blood circulation in the hand-arm region. HAVS typically occurs after exposure to cold, causing an abnormally strong vasoconstriction of blood vessels. A pathoanatomical mechanism suggests that a reduction of the lumen of the blood vessels in VWF (Vibration White Finger) subjects, due to either hypertrophy or thickening of the vessel wall, may be at the origin of the disease...
March 28, 2017: Journal of the Mechanical Behavior of Biomedical Materials
https://www.readbyqxmd.com/read/28387421/apn-cd13-is-over-expressed-by-psoriatic-fibroblasts-and-is-modulated-by-cgrp-and-il-4-but-not-by-retinoic-acid-treatment
#8
Pascale Gerbaud, Jean Guibourdenche, Rafika Jarray, Marc Conti, Patricia Palmic, Stéphanie Leclerc-Mercier, Julie Bruneau, Olivier Hermine, Yves Lepelletier, Françoise Raynaud
Psoriasis vulgaris is a common skin inflammatory disease characterized by recurrent flare episodes associated with scaly well-demarcated skin plaques. Skin biopsies from psoriatic patients with high PASI score (22.67 ± 8.67) and from HD were used to study APN/CD13. APN/CD13 is over-expressed in LP and nLP compare to HD skins and fibroblasts. This over-expression is positively correlated with specific enzymatic activity enhancement. However, discrepancies between APN/CD13 expression in LP and nLP prompt us to focus our study on APN/CD13 modulation...
April 7, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28386141/infrared-thermal-imaging-in-connective-tissue-diseases
#9
REVIEW
Marek Chojnowski
Infrared thermal imaging (IRT) is a non-invasive, non-contact technique which allows one to measure and visualize infrared radiation. In medicine, thermal imaging has been used for more than 50 years in various clinical settings, including Raynaud's phenomenon and systemic sclerosis. Imaging and quantification of surface body temperature provides an indirect measure of the microcirculation's overall performance. As such, IRT is capable of confirming the diagnosis of Raynaud's phenomenon, and, with additional cold or heat challenge, of differentiating between the primary and secondary condition...
2017: Reumatologia
https://www.readbyqxmd.com/read/28382775/dermoscopic-features-of-periungual-papules-in-multicentic-reticulohistiocytosis-dermoscopy-in-multicentic-reticulohistiocytosis
#10
M Sobjanek, M Sławińska, A Romaszkiewicz, M Sokołowska-Wojdyło, E Jasiel-Walikowska, R Nowicki
A 66-year-old woman was diagnosed with MRH based on clinical and histopathological findings. The disease had started 6 months before with arthralgia of the right shoulder joint and symmetric xanthelasma on the palpebrae. One month later symmetric, bilateral arthralgia affecting most of the proximal and distal intraphalangeal joints of both hands have occurred. The multiple, asymptomatic, 2-3 mm in size, reddish -NDASH- brown papules adjacent to the proximal nail fold and two larger asymptomatic flesh-coloured nodules located at the extensor surface of the right antebrachium and the dorsal surface of the third right finger have appeared at the same time...
April 6, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28350093/inhibitory-effect-of-angelica%C3%A2-gigas-on-cold%C3%A2-induced-rhoa-activation-in-vascular-cells
#11
Kangwook Lee, Min Soo Chae, Sung-Gook Cho, Ho Yeon Go, Seung-Ho Sun, Junbock Jang, Ki-Yong Jung, You-Kyung Choi, Yun-Kyung Song, Sung Yong Sim, Hye Lim Lee, Mi Suk Kang, Chan-Yong Jeon, Seong Gyu Ko
The herbal extract Angelica gigas (AG) has been applied as a vasodilating agent for patients suffering from vascular diseases for many years; however, the underlying mechanism has not been fully elucidated. The present study hypothesized that the anti‑vasoconstrictive effect of AG may be effective in the treatment of abnormal cold‑mediated vasospasms that occur in Raynaud's phenomenon (RP). The effect of AG on the activity of ras homolog gene family member A (RhoA) was investigated in cold‑exposed vascular cells...
March 28, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28344932/mutation-in-tnxb-gene-causes-moderate-to-severe-ehlers-danlos-syndrome
#12
Carolyn S Kaufman, Merlin G Butler
We report a 28-year-old female who presented with severe joint pain, chronic muscle weakness, Raynaud's phenomenon, and hypermobility. She was found to have a 6074A > T nucleotide transition in the TNXB gene causing an amino acid protein change at Asp2025Val classified as likely pathogenic. We add this clinical report to the literature and classical human disease gene catalogs to identify this specific mutation as disease-causing. This gene variant was reported previously in a different 36-year-old patient who shared our patient's symptoms of joint hypermobility, skeletal and joint pain, skin elasticity and musculoskeletal problems, thereby causing a more severe presentation than seen in the hypermobility type of Ehlers-Danlos syndrome (EDS)...
May 27, 2016: World Journal of Medical Genetics
https://www.readbyqxmd.com/read/28343718/-comparative-evaluation-of-dermoscopy-and-capillaroscopy-in-raynaud-s-phenomenon
#13
J Moreau, A-S Dupond, N Dan, T Untereiner, C Vidal, F Aubin
OBJECTIVE: Raynaud's phenomenon (RP) is a common cause for consultation. Capillaroscopy is a well-established technique to detect capillary abnormalities suggestive of a connective tissue disease, but it is sometimes unavailable. The aim of this study was to compare dermoscopy and capillaroscopy in the assessment of RP. METHODS: This was a prospective single-centre observational study in adult patients consulting for RP at the Hôpital Nord Franche-Comté between January 2014 and June 2015...
March 23, 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28329526/chronic-granulomatous-disease-as-a-risk-factor-for-cutaneous-lupus-in-childhood
#14
Sandrina Carvalho, Susana Machado, Rita Sampaio, Margarida Guedes, Júlia Vasconcelos, Diogo Semedo, Manuela Selores
Chronic granulomatous disease (CGD) is a primaryimmunodeficiency disorder that affects the phagocyticcells of the innate immune system. It is characterizedby recurrent or persistent infections with granulomaformation. Lupus-like lesions have been reported incarriers of CGD and less frequently, in patients withCGD. Immunological study in these patients areusually negative. We describe the case of an 8-yearoldboy with CGD who developed chronic and acutecutaneous lupus erythematous with angular cheilitis,oral ulcers, Raynaud phenomenon, and positiveserologies for antinuclear, anticentromere, and anti-Saccharomyces cerevisiae antibodies...
March 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28303481/morphea-and-eosinophilic-fasciitis-an-update
#15
REVIEW
Jorre S Mertens, Marieke M B Seyger, Rogier M Thurlings, Timothy R D J Radstake, Elke M G J de Jong
Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated...
March 16, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28292796/hand-arm-vibration-syndrome-what-family-physicians-should-know
#16
REVIEW
Shixin Cindy Shen, Ronald A House
OBJECTIVE: To provide family physicians with an understanding of the epidemiology, pathogenesis, symptoms, diagnosis, and management of hand-arm vibration syndrome (HAVS), an important and common occupational disease in Canada. SOURCES OF INFORMATION: A MEDLINE search was conducted for research and review articles on HAVS. A Google search was conducted to obtain gray literature relevant to the Canadian context. Additional references were obtained from the articles identified...
March 2017: Canadian Family Physician Médecin de Famille Canadien
https://www.readbyqxmd.com/read/28286685/acute-vision-loss-following-endoscopic-sinus-surgery
#17
Serena Byrd, Adnan S Hussaini, Jastin Antisdel
A 41-year-old female with a history of uterine cancer and Celiac and Raynaud's Disease presented to our institution with frequent migraines and nasal congestion. She underwent functional endoscopic sinus surgery (FESS) and experienced acute unilateral vision loss postoperatively. Rapid recognition of the etiology and effective treatment are paramount given the permanent and irreversible vision loss that can result. Arterial vasospasm following FESS is rare. Patients with autoimmune diseases have perhaps an increased risk for vasospasm secondary to an increased vasoreactive profile...
2017: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/28265301/assessing-histone-demethylase-inhibitors-in-cells-lessons-learned
#18
Stephanie B Hatch, Clarence Yapp, Raquel C Montenegro, Pavel Savitsky, Vicki Gamble, Anthony Tumber, Gian Filippo Ruda, Vassilios Bavetsias, Oleg Fedorov, Butrus Atrash, Florence Raynaud, Rachel Lanigan, LeAnne Carmichael, Kathy Tomlin, Rosemary Burke, Susan M Westaway, Jack A Brown, Rab K Prinjha, Elisabeth D Martinez, Udo Oppermann, Christopher J Schofield, Chas Bountra, Akane Kawamura, Julian Blagg, Paul E Brennan, Olivia Rossanese, Susanne Müller
BACKGROUND: Histone lysine demethylases (KDMs) are of interest as drug targets due to their regulatory roles in chromatin organization and their tight associations with diseases including cancer and mental disorders. The first KDM inhibitors for KDM1 have entered clinical trials, and efforts are ongoing to develop potent, selective and cell-active 'probe' molecules for this target class. Robust cellular assays to assess the specific engagement of KDM inhibitors in cells as well as their cellular selectivity are a prerequisite for the development of high-quality inhibitors...
2017: Epigenetics & Chromatin
https://www.readbyqxmd.com/read/28256352/post-traumatic-raynaud-s-phenomenon-a-case-report
#19
Liang Jie Cheok, Chee Kheong Ooi
BACKGROUND: Raynaud's phenomenon has multiple etiologies, ranging from occupational causes to systemic disease. Most occupational causes of Raynaud's phenomenon usually present with vascular compromise. CASE REPORT: A 41-year-old Chinese woman presented to the emergency department with progressive pain and bluish discoloration over her right index finger after minor trauma. The clinical examination revealed discoloration over multiple fingertips on both hands. She was diagnosed with Raynaud's phenomenon with possible underlying systemic disease...
February 27, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28240590/a-meta-analysis-of-avascular-necrosis-in-systemic-lupus-erythematosus-prevalence-and-risk-factors
#20
Tatiana Nevskaya, Maeve P Gamble, Janet E Pope
OBJECTIVES: To determine the prevalence of and risk factors for avascular necrosis (AVN) in systemic lupus erythematosus (SLE). METHODS: MEDLINE, CINAHL, Web of Science, EMBASE and Cochrane Library were searched from inception to July, 2015 and a random effects model was used to combine frequencies; study quality was assessed using STROBE. RESULTS: 2,041 citations identified 62 articles. Many results had high heterogeneity. The prevalence of symptomatic AVN was 9% (range 0...
February 24, 2017: Clinical and Experimental Rheumatology
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