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https://www.readbyqxmd.com/read/28920529/surgical-interventions-for-organ-and-limb-ischemia-associated-with-primary-and-secondary-antiphospholipid-antibody-syndrome-with-arterial-involvement
#1
Carlos A Hinojosa, Javier E Anaya-Ayala, Karla Bermudez-Serrato, Ramón García-Alva, Hugo Laparra-Escareno, Adriana Torres-Machorro, Rene Lizola
OBJECTIVE: The association of antiphospholipid antibody syndrome (APS) and hypercoagulability is well known. Arterial compromise leading to ischemia of organs and/or limbs in patients with APS is uncommon, frequently unrecognized, and rarely described. We evaluated our institutional experience. METHODS: Retrospective review was conducted. From August 2007 to September 2016, 807 patients with diagnosis of APS were managed in our Institution. Patients with primary and secondary APS who required interventions were examined...
January 1, 2017: Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#2
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
September 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913687/non-atherosclerotic-vascular-disease-in-women
#3
REVIEW
Lee Joseph, Esther S H Kim
Takayasu arteritis, fibromuscular dysplasia (FMD), spontaneous arterial dissection, Raynaud's phenomenon, and chilblains are vascular conditions that are associated with an increased predisposition in women and are often underdiagnosed. Takayasu arteritis has an incidence rate of 2.6 cases per million individuals per year in the USA and predominantly affects women of childbearing age. HLA-B5 genetic locus is linked with Takayasu arteritis susceptibility. Methods to determine active disease are limiting; currently utilized clinical and imaging findings and laboratory tests are of limited value for this purpose...
September 14, 2017: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28906413/differences-in-clinical-features-observed-between-childhood-onset-versus-adult-onset-systemic-lupus-erythematosus-a-systematic-review-and-meta-analysis
#4
Pravesh Kumar Bundhun, Alka Kumari, Feng Huang
BACKGROUND: Systemic lupus erythematosus (SLE) affects people in childhood (childhood onset) or in adulthood (adult onset). Observational studies that have previously compared childhood-onset versus adult-onset SLE were often restricted to 1 ethnic group, or to a particular area, with a small sample size of patients. We aimed to systematically compare childhood-onset versus adult-onset SLE through a meta-analysis. METHODS: Electronic databases were searched for relevant publications comparing childhood-onset with adult-onset SLE...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28905453/sexual-steroids-in-serum-and-prostatic-tissue-of-human-non-cancerous-prostate-sterproser-trial
#5
Yann Neuzillet, Jean-Pierre Raynaud, Camélia Radulescu, Jean Fiet, Franck Giton, Jean-François Dreyfus, Tarek P Ghoneim, Thierry Lebret, Henry Botto
BACKGROUND: The specific involvement of the sex steroids in the growth of the prostatic tissue remains unclear. Sex steroid concentrations in plasma and in fresh surgical samples of benign central prostate were correlated to prostate volume. METHODS: Monocentric prospective study performed between September 2014 and January 2017. Age, obesity parameters, and both serum and intraprostatic concentrations of sex steroids were collected complying with the latest Endocrine Society guidelines and the steroids assessed by GC/MS...
September 14, 2017: Prostate
https://www.readbyqxmd.com/read/28904690/-mixed-connective-tissue-disease-prevalence-and-clinical-characteristics-in-african-black-study-of-7-cases-in-gabon-and-review-of-the-literature
#6
Landry Missounga, Josaphat Iba Ba, Ingrid Rosalie Nseng Nseng Ondo, Maria Ines Carine Nziengui Madjinou, Doris Malekou, Emeline Gracia Mouendou Mouloungui, Emmanuel Ecke Nzengue, Jean Bruno Boguikouma, Moussavou Kombila
The literature reports that mixed connective tissue disease seems more frequent in the black population and among Asians. This study aims to determine the prevalence of mixed connective tissue disease (MCTD) among connective tissue disorders and all rheumatologic pathologies in a hospital population in Gabon as well as to describe the clinical features of this disease. We conducted a retrospective study by reviewing the medical records of patients treated for mixed connective tissue disease (Kasukawa criteria) and other entities of connective tissue disorders (ACR criteria) in the Division of Rheumatology at the University Hospital in Libreville between January 2010 and December 2015...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28901690/hypothenar-hammer-syndrome-in-an-office-worker
#7
Fan Zhang, Yuranga Weerakkody, Patrik Tosenovsky
Hypothenar hammer syndrome (HHS) is an uncommon cause of unilateral Raynaud's phenomenon, splinter haemorrhages and hypothenar weakness. The typical patient is a male blue-collar worker who uses their hypothenar eminence to hammer objects as part of their work. The distal ulnar artery beyond Guyon's canal is superficial and vulnerable to blunt trauma. CTA and MRA are common initial investigations and can suggest the diagnosis. DSA is the gold standard imaging modality and offers therapeutic opportunities. Management is controversial, but unless there is critical digital ischaemia, conservative treatment is first line...
September 13, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28895508/esvm-guidelines-the-diagnosis-and-management-of-raynaud-s-phenomenon
#8
Jill Belch, Anita Carlizza, Patrick H Carpentier, Joel Constans, Faisel Khan, Jean-Claude Wautrecht, Adriana Visona, Christian Heiss, Marianne Brodeman, Zsolt Pécsvárady, Karel Roztocil, Mary-Paula Colgan, Dragan Vasic, Anders Gottsäter, Beatrice Amann-Vesti, Ali Chraim, Pavel Poredoš, Dan-Mircea Olinic, Juraj Madaric, Sigrid Nikol, Ariane L Herrick, Muriel Sprynger, Peter Klein-Weigel, Franz Hafner, Daniel Staub, Zan Zeman
Regarding the clinical diagnosis of Raynaud's phenomenon and its associated conditions, investigations and treatment are substantial, and yet no international consensus has been published regarding the medical management of patients presenting with this condition. Most knowledge on this topic derives from epidemiological surveys and observational studies; few randomized studies are available, almost all relating to drug treatment, and thus these guidelines were developed as an expert consensus document to aid in the diagnosis and management of Raynaud's phenomenon...
September 12, 2017: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/28894946/an-international-survey-on-non-invasive-techniques-to-assess-the-microcirculation-in-patients-with-raynaud-s-phenomenon-sunshine-survey
#9
Francesca Ingegnoli, Nicola Ughi, Graham Dinsdale, Annalisa Orenti, Patrizia Boracchi, Yannick Allanore, Ivan Foeldvari, Alberto Sulli, Maurizio Cutolo, Vanessa Smith, Ariane L Herrick
To canvas opinion concerning the role of non-invasive techniques in the assessment of patients with Raynaud's phenomenon (Rp) in clinical and research settings: four nailfold capillaroscopy methods [videocapillaroscopy (NVC), dermoscopy, stereomicroscopy, digital USB microscopy], four laser Doppler methods (laser Doppler flowmetry, imaging, anemometry/velocimetry, laser speckle contrast analysis), thermographic imaging, and upper limb arterial Doppler ultrasound. Emails with a link to the survey were sent to physicians from the European Scleroderma Trials and Research group (EUSTAR), the EULAR Study Group on Microcirculation in Rheumatic Diseases (SG_MC/RD) and members of the pediatric rheumatology Email board...
September 11, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28869417/efficacy-of-intensive-hand-physical-therapy-in-patients-with-systemic-sclerosis
#10
József Horváth, Zsófia Bálint, Erika Szép, Aletta Deiszinger, Tünde Minier, Nelli Farkas, Enikő Török, Éva Horváthné Papp, Dalma Komjáti, Zsuzsanna Mándó, László Czirják, Cecilia Varjú
OBJECTIVES: To evaluate the efficacy of a three-week period of complex and intensive hand physical therapy on hand function in patients with systemic sclerosis (SSc). METHODS: Thirty-one patients with SSc were treated. Hand stretching exercises, ergotherapy supplemented with thermal and mud baths, whirlpool therapy and soft tissue massage were daily used during a three-week period. The control SSc group (n=22) received similar management for their large joints leaving out their hands...
August 30, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28869415/a-novel-iloprost-administration-method-with-portable-syringe-pump-for-the-treatment-of-acral-ulcers-and-raynaud-s-phenomenon-in-systemic-sclerosis-patients-a-pilot-study-iloporta
#11
Paolo Fraticelli, Giuseppe Pio Martino, Marta Murri, Massimo Mattioli, Armando Gabrielli
OBJECTIVES: The objective of the study is to evaluate the feasibility, safety and efficacy of home infusion of iloprost with the new portable syringe pump Infonde®, for the treatment of scleroderma-related Raynaud's phenomenon and digital ulcers. METHODS: 12 scleroderma patients were treated with iloprost at home, using the pump, with infusion cycles of 2 days per month (24 hours a day), for 6 months. RESULTS: The home treatment proved feasible since ten patients (83%) completed the entire infusion cycle, thus satisfying the feasibility target imposed by the protocol (75%)...
August 31, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28864092/facts-and-controversies-in-mixed-connective-tissue-disease
#12
REVIEW
Julia Martínez-Barrio, Lara Valor, F Javier López-Longo
Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterised by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titers of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility. Although widely discussed, most authors today accept MCTD as an independent entity...
August 29, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28859256/clinical-trials-in-raynaud-phenomenon-a-spoonful-of-sugar-pill-makes-the-medicine-go-down-in-flames
#13
EDITORIAL
James R Seibold, Fredrick M Wigley
The peripheral vascular disease in systemic sclerosis (scleroderma) is a challenging problem because it presents with both vasospasm and an underlying structural vascular disease that leads not only to severe Raynaud phenomenon but often critical digital ischemia with progressive tissue damage or acute ulceration. While current therapy with a calcium channel blocker alone or combined with another vasodilating agent provides some modest benefit, it does not provide adequate control of Raynaud's events in many patients with scleroderma...
August 31, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28840223/erratum-to-investigation-of-metabolites-for-estimating-blood-deposition-time
#14
Karolina Lech, Fan Liu, Sarah K Davies, Katrin Ackermann, Joo Ern Ang, Benita Middleton, Victoria L Revell, Florence I Raynaud, Igor Hoveijn, Roelof A Hut, Debra J Skene, Manfred Kayser
No abstract text is available yet for this article.
August 24, 2017: International Journal of Legal Medicine
https://www.readbyqxmd.com/read/28831927/-necrotic-toes-in-a-female-patient-using-interferon
#15
T J R M Auping, P Maat, K van der Waal, R H H Bemelmans
BACKGROUND: Interferon has an important role in treatment of viral hepatitis, multiple sclerosis and solid and non-solid tumours. Ischaemia and necrosis of the extremities are relatively little-known adverse effects of treatment with interferon. CASE DESCRIPTION: A 44-year-old woman was treated with interferon-beta for relapsing-remitting multiple sclerosis. She developed ischaemia and necrosis of the right lower extremity. Extensive laboratory and imaging investigations offered no clear diagnosis, leading to suspicion of a connexion with interferon-beta treatment...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28831595/juvenile-systemic-sclerosis-experience-from-a-tertiary-care-center-from-india
#16
Narendra Kumar Bagri, Dinesh Raj, Jasmeet Kaur, Harish Punia, Isha Saini, Rakesh Lodha, S K Kabra
Juvenile systemic sclerosis (JSSc) is a rare disorder with paucity of information on its treatment and longterm  outcome. Herein, we are sharing our experience with this rare entity. Case records of children, diagnosed to have systemic sclerosis attending Pediatric Rheumatology Clinic at All India Institute of Medical Sciences, New Delhi from January 1998 to June 2016 were reviewed. The demographic, clinical, laboratory, treatment and outcome details were recorded. Disease outcome was classified arbitrarily as controlled, partly controlled or non-responsive/progressive based on: (A) ability to perform activities of daily life (ADL) and (B) presence or absence of musculoskeletal symptoms, skin changes (ulceration/progressive digital pitting/gangrene), and visceral organ involvement (dyspahgia, cardiopulmonary symptoms)...
August 22, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28819792/validation-of-the-acr-eular-classification-criteria-for-systemic-sclerosis-in-patients-with-early-scleroderma
#17
Farley Carvalho Araújo, Cíntia Zumstein Camargo, Cristiane Kayser
The aim of this study was to validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients with SSc, including patients with early SSc. Fifty-six consecutive patients with early SSc (2001 LeRoy and Medsger criteria), 122 patients with established SSc (1980 ACR classification criteria), and 141 patients with SSc-like disorders were included in this cross-sectional study. The diagnostic performance of the 2013 ACR/EULAR criteria was compared with the 1980 ACR criteria in several subsets of patients...
August 17, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28811188/familial-1p36-3-microduplication-resulting-from-a-1p-9q-non-reciprocal-translocation
#18
Valentine Marquet, Sylvie Bourthoumieu, Amelia Dobrescu, Cécile Laroche-Raynaud, Catherine Yardin
Unlike the 1p36 microdeletion syndrome, which has been extensively described, 1p36 microduplications have rarely been reported. We describe a three years old boy presenting with a severe global developmental delay and a few dysmorphic features. Cytogenetic analyses revealed a maternally inherited 3.35 Mb microduplication of chromosomal band 1p36.3. The maternal grandfather is also carrier of the same chromosomal rearrangement. Interestingly, the duplicated 1p36.3 segment was found to be localized at the telomeric end of the long arms of a chromosome 9, probably deriving from a 1p36...
August 12, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28807722/coronary-microvascular-dysfunction-is-not-associated-with-a-history-of-reproductive-risk-factors-in-women-with-angina-pectoris-an-ipower-substudy
#19
Hannah Elena Suhrs, Anna Meta Kristensen, Anna Bay Rask, Marie Mide Michelsen, Daria Frestad, Naja Dam Mygind, Kira Bové, Eva Prescott
BACKGROUND: Reproductive risk factors such as preeclampsia and recurrent miscarriages have been associated with adverse cardiovascular (CV) events. Underlying coronary microvascular dysfunction (CMD) may be a common denominator. PURPOSE: We investigated whether a history of reproductive risk factors was associated with CMD in women with angina pectoris and no obstructive coronary artery disease (CAD). METHODS: Participants from the iPOWER study, including women with angina pectoris and no obstructive CAD (<50% stenosis), were invited to complete an electronic survey regarding reproductive risk factors: recurrent miscarriages, gestational diabetes, preeclampsia, rhesus immunity, polycystic ovary syndrome and menopausal status as well as migraine and Raynaud phenomenon...
August 11, 2017: Maturitas
https://www.readbyqxmd.com/read/28803485/raynaud-s-phenomenon-and-its-impact-on-activities-in-daily-life-during-one-year-of-follow-up-in-early-systemic-sclerosis
#20
G Sandqvist, P Wollmer, A Scheja, M Wildt, R Hesselstrand
OBJECTIVE: To investigate Raynaud's phenomenon (RP) and its impact on daily life activities during 1 year of follow-up in early systemic sclerosis (SSc). METHOD: Fourteen SSc patients with a median disease duration of 2 years were enrolled in the study. Every 7 weeks the patients completed a 7 day diary documenting the frequency and duration of RP attacks, the activity causing the attack, and how they handled the attack. The patients also recorded in the diary daily self-assessments of the difficulties with RP, using a 0-10 ordinal scale according to the Raynaud's Condition Score...
August 13, 2017: Scandinavian Journal of Rheumatology
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