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https://www.readbyqxmd.com/read/29214548/first-clinical-symptom-as-a-prognostic-factor-in-systemic-sclerosis-results-of-a-retrospective-nationwide-cohort-study
#1
Manuel Rubio-Rivas, Xavier Corbella, Melany Pestaña-Fernández, Carles Tolosa-Vilella, Alfredo Guillen-Del Castillo, Dolores Colunga-Argüelles, Luis Trapiella-Martínez, Nerea Iniesta-Arandia, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, Jose Antonio Vargas-Hitos, Juan José Ríos-Blanco, Jose Antonio Todolí-Parra, Mónica Rodríguez-Carballeira, Adela Marín-Ballvé, Pablo Segovia-Alonso, Xavier Pla-Salas, Ana Belén Madroñero-Vuelta, Manuel Ruiz-Muñoz, Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar, E Callejas Moraga, E Calvo, C Carbonell, M J Castillo, A J Chamorro, D Colunga, X Corbella, M V Egurbide, G Espinosa, V Fonollosa, M Freire, F J García Hernández, R González León, A Guillén Del Castillo, N Iniesta, R Lorenzo, A B Madroñero, B Marí, A Marín, N Ortego-Centeno, M Pérez Conesa, M Pestaña, X Pla, J J Ríos Blanco, M Rodríguez Carballeira, M Rubio Rivas, M Ruiz Muñoz, L Sáez Comet, P Segovia, C P Simeón, A Soto, E Tarí, J A Todolí, C Tolosa, L Trapiella, J A Vargas Hitos, G Verdejo
The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited...
December 7, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29212687/comparative-study-on-the-effectiveness-of-a-corticosteroid-injection-for-carpal-tunnel-syndrome-in-patients-with-and-without-raynaud-s-phenomenon
#2
Y H Roh, J H Noh, H S Gong, G H Baek
AIMS: The aim of this study was to compare the efficacy of a corticosteroid injection for the treatment of carpal tunnel syndrome (CTS) in patients with and without Raynaud's phenomenon. PATIENTS AND METHODS: In a prospective study, 139 patients with CTS were treated with a corticosteroid injection (10 mg triamcinolone acetonide); 34 had Raynaud's phenomenon and 105 did not (control group). Grip strength, perception of touch with a Semmes-Weinstein monofilament and the Boston Carpal Tunnel Questionnaires (BCTQ) were assessed at baseline and at six, 12 and 24 weeks after the injection...
December 2017: Bone & Joint Journal
https://www.readbyqxmd.com/read/29211693/the-t-12-21-p13-q22-in-pediatric-b-acute-lymphoblastic-leukemia-an-update
#3
Maximilian Becker, Kristie Liu, Carlos A Tirado
Erratum: Figure 1 on the last edition The Journal of the Association of Genetic Technologists. 2017;43(3): 113-127 does not contain the derivative 21. We are replacing this figure with the present one. In the section Secondary genetic aberrations we would like to add that: Deletions of 11q23 are observed in 5-6% of cases (Raynaud et al., 1999; Attarbaschi et al., 2004; Alvarez et al., 2005; Forestier et al., 2007).
2017: Journal of the Association of Genetic Technologists
https://www.readbyqxmd.com/read/29209686/neuromuscular-taping-enhances-hand-function-in-patients-with-systemic-sclerosis-a-pilot-study
#4
S Parisi, C Celletti, M Scarati, M Priora, A Laganà, C L Peroni, F Camerota, G La Torre, D Blow, E Fusaro
Hand functioning is often impaired in patients with Systemic sclerosis. Neuromuscular Taping is a novel application of tape able to improve functioning. The aim of this study was to evaluate the possible role of this application in the hand functionality of patients with Systemic sclerosis. Women with a diagnosis of SSc has been recruited and evaluated using different scales before and immediately after NMT application and after one, three and six months. Fifty-three women has been evaluated and Cochin Hand Functional Disability scale, Hand Mobility in Sclerodermia, Modified Rodnan Skin Score and Dreiser Algo - Functional Index scores showed statistical significant differences during all the period; moreover a reduction of pain and Raynaud Phenomenon's and an improvement of finger flexion has been observed...
November 2017: La Clinica Terapeutica
https://www.readbyqxmd.com/read/29201156/evidence-based-management-of-raynaud-s-phenomenon
#5
REVIEW
Ariane L Herrick
Raynaud's phenomenon (RP) is relevant to the rheumatologist because it may signify an underlying connective tissue disease and also because it can be very challenging to treat, especially when it has progressed to digital ulceration or critical ischaemia. This review article discusses diagnosis (does this patient have an underlying connective tissue disease?), including the role for nailfold capillaroscopy, and treatment. Management of 'uncomplicated' RP is first described and then treatment of RP complicated by progression to digital ulceration or critical ischaemia, highlighting recent advances (including phosphodiesterase type 5 inhibition, and endothelin 1 receptor antagonism) and the evidence base underpinning these...
December 2017: Therapeutic Advances in Musculoskeletal Disease
https://www.readbyqxmd.com/read/29198478/the-evaluation-of-a-home-based-program-for-hands-in-patients-with-systemic-sclerosis
#6
Sibila Floriano Landim, Manoel Barros Bertolo, Marcos Felipe Marcatto de Abreu, Ana Paula Del Rio, Cecilia Carmen Mazon, João Francisco Marques-Neto, Janet L Poole, Eduardo de Paiva Magalhães
STUDY DESIGN: This study used a quasi-experimental design where patients were evaluated before and after participation in the self-management program. INTRODUCTION: Hands are commonly affected in systemic sclerosis (SSc). Strategies to maintain or improve hand function are indicated upon diagnosis and throughout the course of the disease. PURPOSE OF THE STUDY: The purpose of this study was to develop and evaluate a home-based program for hands in patients with SSc...
November 29, 2017: Journal of Hand Therapy: Official Journal of the American Society of Hand Therapists
https://www.readbyqxmd.com/read/29193819/efficacy-and-safety-of-selexipag-in-adults-with-raynaud-s-phenomenon-secondary-to-systemic-sclerosis-a-randomized-placebo-controlled-phase-ii-study
#7
RANDOMIZED CONTROLLED TRIAL
Christopher P Denton, Éric Hachulla, Gabriela Riemekasten, Andreas Schwarting, Jean-Marie Frenoux, Aline Frey, Franck-Olivier Le Brun, Ariane L Herrick
OBJECTIVE: To determine the effect of selexipag, an oral, selective IP prostacyclin receptor agonist, on the frequency of attacks of Raynaud's phenomenon (RP) in patients with systemic sclerosis (SSc). METHODS: Patients with SSc-related RP were randomized 1:1 to placebo (n = 38) or selexipag (n = 36) in individualized doses (maximum of 1,600 μg twice daily) during a 3-week titration period. The primary end point was the weekly average number of RP attacks during the study maintenance period, analyzed using a Bayesian approach with a negative binomial model adjusted for baseline number of RP attacks...
December 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29173190/migraine-in-systemic-autoimmune-diseases
#8
Cavestro Cinzia, Ferrero Marcella
BACKGROUND AND OBJECTIVE: Migraine and systemic autoimmune diseases are 2-3-fold more common in women and various studies have reported an association between the two pathologies. METHODS: This review takes into account epidemiological studies involving migraine and systemic lupus erythematosus, antiphospholipid syndrome, Sjogren's syndrome, and other diffuse connective tissue diseases. This scientific literature analysis consists of the main articles found in Medline with a search up to April 2017...
November 24, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/29173135/a-64-year-old-woman-with-interstitial-lung-disease-and-positive-antibodies-against-aminoacyl-transfer-rna-synthetases-in-the-absence-of-myositis-presentation-of-an-anti-pl-12-positive-antisynthetase-syndrome
#9
Katrien Ghysen, Mathias Leys
Introduction The antisynthetase syndrome is a rare autoimmune disease described by the presence of inflammatory myositis, interstitial lung disease and antibodies against aminoacyl-transfer RNA synthetases. Interstitial lung disease can be the only manifestation in the absence of an inflammatory myositis. Other clinical signs are Raynaud phenomenon, hyperkeratotic skin lesions, fever and inflammatory polyarthritis. Case presentation We report the case of a 64-year old woman who complained of a dry cough, progressive dyspnea and arthralgia since 2 years, with no other systemic symptoms...
November 27, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29164658/successful-treatment-of-raynaud-s-phenomenon-and-digital-ulcers-in-systemic-sclerosis-patients-with-botulinum-toxin-b-injection-assessment-of-peripheral-vascular-disorder-by-angiography-and-dermoscopic-image-of-nail-fold-capillary
#10
Sei-Ichiro Motegi, Akiko Sekiguchi, Shintaro Saito, Hirohisa Ishibuchi, Chikako Kishi, Masahito Yasuda, Osamu Ishikawa
We recently identified the efficacy and safety of a botulinum toxin (BTX)-A/B in Raynaud's phenomenon (RP) and digital ulcers (DU) in Japanese patients with systemic sclerosis (SSc). Detailed assessments of peripheral vascular disorder using angiography and dermoscopic images of nail fold capillaries have not been performed previously. This study aimed to evaluate the effect of BTX-B on SSc-associated peripheral vascular disorder. Two SSc patients who suffered with RP and DU were treated with a BTX-B injection, and thereafter the symptoms of RP were improved and DU healed in both patients...
November 22, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/29151520/dropped-head-syndrome-and-the-presence-of-rimmed-vacuoles-in-a-muscle-biopsy-in-scleroderma-polymyositis-overlap-syndrome-associated-with-anti-ku-antibody
#11
Yoshida Takeshi, Yoshida Mai, Mitsuyo Kinjo, Jonosono Manabu, Higuchi Itsuro
A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29145709/defining-a-unified-vascular-phenotype-in-systemic-sclerosis
#12
REVIEW
Yannick Allanore, Oliver Distler, Marco Matucci-Cerinic, Christopher P Denton
Microcirculation impairment and related vasculopathy is a hallmark of systemic sclerosis. Digital ulcers are second only to Raynaud's phenomenon as a vascular complication occurring in scleroderma patients. Digital ulcers are painful and generate disability. Furthermore, patients may develop recurrent digital ulcers and one may ask whether the outcomes of such patients might be different to those of unaffected ones. Recently several registries have provided relevant information about digital ulcers. Male sex and severe skin disease seem to be the main associated factors observed in several registries...
November 16, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29123575/systemic-sclerosis-and-the-gastrointestinal-tract
#13
REVIEW
Irena Walecka
Systemic sclerosis (SSc) is an autoimmunological disease of unknown origin with complex pathogenesis and multiple organ involvement. It is characterised by vascular and immunological abnormalities leading to fibrosis of the skin and internal organs. It is a rather rare disease with a prevalence of around 20 per 100,000. The disease results in heterogeneous clinical findings and different courses. Systemic sclerosis usually begins with the onset of Raynaud's phenomenon (RP), followed by skin sclerosis and internal organ involvement, although it may appear synchronously with RP...
2017: Przegla̜d Gastroenterologiczny
https://www.readbyqxmd.com/read/29118999/associates-and-predictors-of-pleurisy-or-pericarditis-in-sle
#14
Seungwon Ryu, Wei Fu, Michelle A Petri
Background/Purpose: Serositis is one of both ACR and SLICC classification criteria for systemic lupus erythematosus (SLE) and a common type of extra renal flare. However, little is known about clinical or immunological associations of pleurisy or pericarditis. The aim of this study is to analyze associates and predictors of pleurisy versus pericarditis in Caucasians and African Americans with SLE. Methods: 2,390 SLE patients in the Hopkins Lupus Cohort were analyzed for demographic, clinical and serologic associates of pleurisy or pericarditis, defined using the SELENA revision of the SLE Disease Activity Index (SLEDAI)...
2017: Lupus Science & Medicine
https://www.readbyqxmd.com/read/29114398/multi-system-manifestations-of-mycoplasma-pneumoniae-infection-in-a-young-patient
#15
Ibrahim Al Busaidi, Mohammed Al-Amin, Shadin Ibrahim, Abdullah Balkhair, Zied Gaifer
Introduction.Mycoplasma pneumoniae is a small cell-wall-lacking bacterium that belongs to the mycoplasma (Mollicutes) prokaryote micro-organisms. It is a common cause of both upper and lower respiratory tract infections in all age groups. Respiratory illness is the most common manifestation of M. pneumoniae infection; however, extrapulmonary involvement may be present or predominant. The skin, mucus membranes, central nervous system, cardiovascular system, haematopoietic system, kidneys and musculoskeletal system are the most commonly involved extrapulmonary sites...
September 2017: JMM Case Reports
https://www.readbyqxmd.com/read/29111348/nrpa-308-a-new-neuropilin-1-antagonist-exerts-in%C3%A2-vitro-anti-angiogenic-and-anti-proliferative-effects-and-in%C3%A2-vivo-anti-cancer%C3%A2-effects-in-a-mouse-xenograft-model
#16
Wang-Qing Liu, Yves Lepelletier, Matthieu Montès, Lucia Borriello, Rafika Jarray, Renaud Grépin, Bertrand Leforban, Ali Loukaci, Rachid Benhida, Olivier Hermine, Sylvie Dufour, Gilles Pagès, Christiane Garbay, Françoise Raynaud, Reda Hadj-Slimane, Luc Demange
Neuropilin-1 (NRP-1) is an extra-cellular receptor for the main Vascular Endothelial Growth Factor over-expressed in tumour tissues, VEGF-A165. Consequently, NRP-1 is involved in angiogenesis and in tumour growth, and its over-expression is related to a clinical poor prognosis. NRP-1 appears as a major target in oncology, which remains poorly exploited. Herein, we report a new series of 18 small-sized fully organic VEGF-A165/NRP-1 antagonists (NRPas). These compounds share an original scaffold, including two linkers (sulphonamide and amide) and three aromatic cores...
October 28, 2017: Cancer Letters
https://www.readbyqxmd.com/read/29104809/mycobacterium-intracellulare-infection-mimicking-progression-of-scleroderma
#17
Simon Krabbe, Merete Engelhart, Sören Thybo, Søren Jacobsen
This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal nail fold capillaries, interstitial lung disease, Raynaud's phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed attempts of immunosuppressive treatments were found to be caused by Mycobacterium intracellulare...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29087124/-antiphospholipid-syndrome-in-pediatrics-a-case-report
#18
Eugenia Micheletti, Luciana Blanco, Ana Cechinni, Marcelo Acerenza, Paula Canda
The antiphospholipid syndrome is a multisystem autoimmune disease in which autoantibodies against a variety of phospholipids and phospholipid binding proteins are produced. It occurs in 1.8% of the population and only 2% of the cases are pediatric. The spectrum of clinical manifestations is wide from asymptomatic patients to a life-threatening disease like the catastrophic antiphospholipid syndrome. Any organ can be affected. The most frequent manifestations in pediatrics correspond to venous thrombosis in 60% of patients, arterial thrombosis in 32%, hematological disease in 38% (thrombocytopenia, leucopenia), skin alterations in 18% (livedo reticularis, Raynaud's phenomenon) and neurological signs in 16%...
December 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29086923/the-non-neuronal-and-non-muscular-effects-of-botulinum-toxin-a-graceful-opportunity-for-a-deadly-molecule-to-treat-a-human-disease-in-the-skin-and-beyond
#19
REVIEW
S A Grando, C B Zachary
There is growing evidence that botulinum neurotoxins (BoNTs) exhibit biological effects on various human cell types with a host of associated clinical implications. The BoNT receptors and intracellular targets are not unique for neurotransmission. They have been found in both neuronal and non-neuronal cells, but there are differences in the way BoNT binds to, and acts on neuronal vs. non-neuronal cells. The non-neuronal cells expressing one or more BoNT/A-binding proteins and/or cleavage target SNAP-25 include epidermal keratinocytes, mesenchymal stem cells from subcutaneous adipose, nasal mucosal cells, urothelial cells, intestinal epithelial cells, neutrophils, macrophages, and prostate, breast and alveolar epithelial cells, BoNT/A can also elicit specific biological effects in dermal fibroblasts, sebocytes and vascular endothelial cells...
October 31, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/29083916/molecular-and-silica-supported-molybdenum-alkyne-metathesis-catalysts-influence-of-electronics-and-dynamics-on-activity-revealed-by-kinetics-solid-state-nmr-and-chemical-shift-analysis
#20
Deven P Estes, Christopher P Gordon, Alexey Fedorov, Wei-Chih Liao, Henrike Ehrhorn, Celine Bittner, Manuel Luca Zier, Dirk Bockfeld, Ka Wing Chan, Odile Eisenstein, Christophe Raynaud, Matthias Tamm, Christophe Copéret
Molybdenum-based molecular alkylidyne complexes of the type [MesCMo{OC(CH3)3-x(CF3)x}3] (MoF0, x = 0; MoF3, x = 1; MoF6, x = 2; MoF9, x = 3; Mes = 2,4,6-trimethylphenyl) and their silica-supported analogues are prepared, characterized at the molecular level, in particular by solid-state NMR, and their alkyne metathesis catalytic activity is evaluated. The 13C NMR chemical shift of the alkylidyne carbon increases with increasing number of fluorine atoms on the alkoxide ligands for both molecular and supported catalysts, however, with more shielded values for the supported complexes...
October 30, 2017: Journal of the American Chemical Society
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