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adult onset still disease

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https://www.readbyqxmd.com/read/29909561/molecular-genetic-analysis-for-periodic-fever-syndromes-a-supplemental-role-for-the-diagnosis-of-adult-onset-still-s-disease
#1
Hongbin Li, Irina Abramova, Sandra Chesoni, Qingping Yao
Adult-onset Still's disease (AOSD) represents a systemic autoinflammatory disease (SAID), and its diagnostic criteria are clinical without genetic testing. Given shared manifestations between AOSD and hereditary SAIDs, molecular analysis may help differentiate these diseases. A PubMed literature search was conducted using key words "adult-onset Still's disease," "autoinflammatory disease," and "genetic mutation" between 1970 and February 2018. Articles on genetic mutations in the genes MEFV, TNFRSF1A, mevalonate kinase, or NOD2 for hereditary SAIDs in AOSD/systemic onset juvenile idiopathic arthritis (SJIA) patients were reviewed and analyzed...
June 17, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29906427/adult-onset-still-s-disease-a-classic-presentation-of-a-rare-illness
#2
Amrit K Kamboj, Samantha M Ryan, Mark J Enzler
No abstract text is available yet for this article.
June 12, 2018: American Journal of Medicine
https://www.readbyqxmd.com/read/29903680/-non-infective-endocarditis
#3
A Le Bot, P Jégo, E Donal, E Flécher, M Revest, P Tattevin
Non-infective endocarditis, also referred to as non-bacterial thrombotic endocarditis, represent a wide range of rare pathologies, often severe. This review gathered the data available in the literature, to decipher the major information collected on the pathophysiology, the diagnosis and the treatment of these heterogeneous diseases, often misdiagnosed. Characteristics of non-infective endocarditis are similar to infective endocarditis in terms of valvular lesions (mostly left-sided, with regurgitations and vegetations), and their complications (embolism)...
June 11, 2018: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29901588/clinical-analysis-of-215-consecutive-cases-with-fever-of-unknown-origin-a-cohort-study
#4
Yong-Zhi Zhai, Xin Chen, Xin Liu, Zhi-Qiang Zhang, Hong-Ju Xiao, Gang Liu
Fever of unknown origin (FUO) is a frequently observed phenomenon in clinical practice. The present study was aimed to investigate potential causes of FUO, thereby improving clinical diagnosis of this disorder.In this retrospective study, clinical data were collected from 215 patients who were diagnosed with FUO between January 2009 and December 2010, and an 18 to 36 months follow-up visit was also performed for these patients.Among these FUO cases, the most common causes of the disease were infectious diseases (IDs) (42...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29891069/immune-mediated-ataxias
#5
Bastien Joubert, Kevin Rostásy, Jérôme Honnorat
Immune-mediated cerebellar ataxia (CA) comprises a group of rare diseases that are still incompletely described, and are probably underdiagnosed. Both acute and progressive progressions are possible. Different syndromes have been identified, including CA associated with anti-GAD antibodies, the cerebellar type of Hashimoto encephalopathy, primary autoimmune CA, gluten ataxia, opsoclonus-myoclonus syndrome, and paraneoplastic cerebellar degenerations. Most of these syndromes are associated with autoantibodies targeting neuronal antigens...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29876803/oxidative-stress-in-spinocerebellar-ataxia-type-7-is-associated-with-disease-severity
#6
Y Torres-Ramos, A Montoya-Estrada, B Cisneros, K Tercero-Pérez, G León-Reyes, N Leyva-García, Oscar Hernández-Hernández, Jonathan J Magaña
Spinocerebellar ataxia type 7 is a neurodegenerative inherited disease caused by a CAG expansion in the coding region of the ATXN7 gene, which results in the synthesis of polyglutamine-containing ataxin-7. Expression of mutant ataxin-7 disturbs different cell processes, including transcriptional regulation, protein conformation and clearance, autophagy, and glutamate transport; however, mechanisms underlying neurodegeneration in SCA7 are still unknown. Implication of oxidative stress in the pathogenesis of various neurodegenerative diseases, including polyglutamine disorders, has recently emerged...
June 6, 2018: Cerebellum
https://www.readbyqxmd.com/read/29861211/the-effect-of-rheumatoid-arthritis-on-the-risk-of-cerebrovascular-disease-and-coronary-artery-disease-in-young-adults
#7
Yih-Ru Chen, Fang-I Hsieh, Chi-Ching Chang, Nai-Fang Chi, Hsin-Chiao Wu, Hung-Yi Chiou
BACKGROUND: Only a few studies have investigated the affect of rheumatoid arthritis (RA) on the risk of cerebrovascular disease (CVD)/coronary artery disease (CAD) in young adults. This study, therefore, examined the association between RA and the risk of CVD/CAD in young adults and the interaction effects between cardiovascular risk factors and RA on the risk of CVD/CAD. METHODS: Data regarding 52,840 subjects (10,568 patients with RA and 42,272 age-, sex-, urbanization-, and income-matched non-RA controls) were collected from the National Health Insurance Research Database (NHIRD) in 2006...
May 31, 2018: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/29860231/therapeutic-innovation-in-adult-onset-still-s-disease-and-other-rare-inflammatory-disorders-how-to-secure-evidence-based-medicine
#8
EDITORIAL
Philippe Guilpain, Alain Le Quellec, Alexandre Thibault Jacques Maria
No abstract text is available yet for this article.
June 2, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29794515/adult-onset-still-disease-presenting-with-insterstitial-lung-disease-and-dermatomyositis-like-atypical-persistent-eruption-successfully-treated-with-mycophenolate-mofetil
#9
Eduardo Martín-Nares, Alvaro López-Iñiguez, Paulina Estefania Castro-Gallegos, Heriberto Ontiveros-Mercado, Manuel Soria-Orozco, Lorena Valerdi-Contreras
No abstract text is available yet for this article.
April 19, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29787836/increased-severity-and-epidermal-alterations-in-persistent-versus-evanescent-skin-lesions-in-adult-onset-still-s-disease
#10
Elina Zuelgaray, Maxime Battistella, Camille Sallé de Chou, Marie-Dominique Vignon-Pennamen, Michel Rybojad, Antoine Petit, Florence Cordoliani, François Chasset, Claude Bachmeyer, Laurence Fardet, Bruno Fautrel, Patrice Cacoub, Dan Lipsker, Martine Bagot, Jean-David Bouaziz
No abstract text is available yet for this article.
May 19, 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29784018/adding-colchicine-to-immunosuppressive-treatments-a-potential-option-for-biologics-refractory-adult-onset-still-s-disease
#11
Tomoyuki Asano, Makiko Yashiro Furuya, Shuzo Sato, Hiroko Kobayashi, Hiroshi Watanabe, Eiji Suzuki, Kiyoshi Migita
BACKGROUND: Adult-onset Still's disease (AOSD) is a rare inflammatory disorder characterized by the classical triad of daily spiking fever, arthritis, and typical salmon-colored rash. Resistance to first-line corticosteroids and second-line disease modified anti-rheumatic-drugs defines refractory AOSD, which mostly includes the polycyclic or chronic courses of the disease. Anti-cytokine therapies are recommended in AOSD patients who are refractory to traditional treatments. This is the first report on the efficacy of colchicine in a patient with AOSD which was refractory to immunosuppressive treatments including biologics...
May 21, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29782428/unprovoked-pulmonary-embolism-identified-on-initial-presentation-of-adult-onset-still-disease-in-an-elderly-patient-with-no-malignancy
#12
Manjeet Bhamra, Abhimanyu Amarnani, David Ozeri
No abstract text is available yet for this article.
May 19, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29782055/clinical-and-immunological-effects-of-adsorptive-myeloid-lineage-leukocyte-apheresis-in-patients-with-immune-disorders
#13
REVIEW
Takuro Kanekura
Adsorptive granulocyte and monocyte apheresis (GMA) with the Adacolumn® is an extracorporeal treatment, which uses cellulose acetate (CA) beads as adsorptive leukocytapheresis carriers designed to remove elevated and potentially activated myeloid lineage leukocytes. Reports on the clinical efficacy of GMA in patients with skin lesions have appeared in the published work. Dermatological diseases, which are known to respond to GMA, include pyoderma gangrenosum, skin lesions of Behçet's disease, rheumatoid arthritis, pustular psoriasis, psoriatic arthritis, adult-onset Still's disease, Sweet's syndrome, cutaneous allergic vasculitis and systemic lupus erythematosus rashes...
May 21, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29742054/overview-of-fever-of-unknown-origin-in-adult-and-paediatric-patients
#14
REVIEW
Luciano Attard, Marina Tadolini, Domenico Umberto De Rose, Marco Cattalini
Fever of unknown origin (FUO) can be caused by a wide group of diseases, and can include both benign and serious conditions. Since the first definition of FUO in the early 1960's, several updates to the definition, diagnostic and therapeutic approaches have been proposed. This review outlines a case report of an elderly Italian male patient with high fever and migrating arthralgia who underwent many procedures and treatments before a final diagnosis of Adult-onset Still's disease was achieved. This case report highlights the difficulties in diagnosing certain causes of FUO that requires a very high index of suspicion...
January 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29733534/adult-onset-still-s-disease-initially-thought-to-be-an-odontogenic-infection-a-case-report
#15
Shunsuke Hino, Satoshi Nakamura, Takahiro Kaneko, Norio Horie, Tetsuo Shimoyama
OBJECTIVE: To present a case of Adult-onset Still's disease (AOSD) initially suspected to be odontogenic inflammation. BACKGROUND: Adult-onset Still's disease is a rare, complex autoinflammatory disease and a known cause of fever of unknown origin. MATERIALS AND METHODS: The patient had both a fever and dental pain. Following meticulous examination, the patient was diagnosed with AOSD. CONCLUSION: Clinicians should keep in mind that a patient such as AOSD may visit their clinics...
June 2018: Gerodontology
https://www.readbyqxmd.com/read/29731191/-intra-cardiac-manifestation-during-adult-onset-still-s-disease-s-a-tricuspid-vegetation-as-a-rare-expression-of-systemic-disease
#16
Q Quelven, B Cador, M Poinot, E Fletcher, E Le Mouel, P Jego
INTRODUCTION: Adult-onset Still's Disease is a rare multisystemic inflammatory disease characterized by fever, maculo-papular erythematous rash and arthralgia. Adult-onset Still's disease is a diagnosis of exclusion. CASE REPORT: We report the case of a 33 years old man, hospitalized for fever, arthralgia and throat manifestations, leading to Adult-onset Stills's Disease diagnosis. Cardiac ultrasound revealed tricuspid vegetation. Once infectious causes were ruled out, the vegetation was related to Adult-onset Still's Disease according to Fautrel and Yamaguchi criteria...
May 3, 2018: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29729254/physical-exercise-reserved-amyloid-beta-induced-brain-dysfunctions-by-regulating-hippocampal-neurogenesis-and-inflammatory-response-via-mapk-signaling
#17
Li-Na Sun, Jin-Shun Qi, Rong Gao
Alzheimer's disease (AD) is one of the leading causes of dementia that induced by aggregation of amyloid-beta (Aβ) in brain tissue. With high structural and functional plasticity, hippocampus plays fundamental roles in cognitive regulation. Moreover, impaired hippocampal functions present during early onset of AD. Hence, targeting on improving hippocampal plasticity would be recognized as the effective strategy in AD therapy. Physical exercise is widely encouraged healthy life style. However, whether exercise could reserve the neural dysfunctions in AD model and the possible neurobiological mechanism still need for better understanding...
May 2, 2018: Brain Research
https://www.readbyqxmd.com/read/29723378/oral-mucosa-lesions-as-atypical-manifestation-of-adult-onset-still%C3%A2-s-disease
#18
María Lorena Brance, Eldo Luis Neffen
Adult-onset Still's disease is a systemic inflammatory disorder of unknown etiology, characterized by skin rash, spiking fever, arthralgias or arthritis, and leukocytosis. The typical skin rash is evanescent, salmon-pink, nonpruritic and maculopapular, predominantly on the extremities. It is considered one of the major Yamaguchi's criteria in adult-onset Still's disease. However, atypical skin lesions are also described. Here, a 61-year-old woman with sore throat, spiking fever, polyarthritis and evanescent salmon-pink nonpruritic maculopapular skin rash on the extremities was diagnosed with adult-onset Still's disease...
March 2018: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29722877/rituximab-for-induction-and-maintenance-therapy-of-granulomatosis-with-polyangiitis-a-single-centre-cohort-study-on-114-patients
#19
Xavier Puéchal, Michele Iudici, Ana Luisa Calich, Alexandre Vivot, Benjamin Terrier, Alexis Régent, Pascal Cohen, Claire Le Jeunne, Luc Mouthon, Philippe Ravaud, Loïc Guillevin
Objectives: To assess efficacy and safety of rituximab (RTX) induction and maintenance therapy for granulomatosis with polyangiitis (GPA) in a single-centre cohort study. Methods: All patients with active GPA, not enrolled in trials, who received ⩾1 RTX infusion(s) for induction were included. At remission, protocolized maintenance RTX infusions were given every 6 months for 18 months. Kaplan-Meier curves were used to estimate survival rates. Univariable analyses identified factors associated with remission failure and relapse, and Cox models retained independent predictors of relapse...
May 2, 2018: Rheumatology
https://www.readbyqxmd.com/read/29660063/five-successful-pregnancies-with-antenatal-anakinra-exposure
#20
Chelsey J F Smith, Christina D Chambers
Objectives: Our aim is to add to the limited existing prospective data on IL-1 inhibitor use in pregnancy. Methods: Data were obtained from the Organization of Teratology Information Specialists Autoimmune Disease in Pregnancy Project, a prospective cohort study of pregnancy outcomes in the USA and Canada. Eligible women were enrolled prior to 19 weeks' gestation between 2004 and 2017. Outcomes were obtained by maternal interview and medical record abstraction. Results: Five pregnancies with anakinra exposure were identified, all resulting in full-term singleton live births with no major or long-term complications...
April 12, 2018: Rheumatology
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