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adult onset stills disease

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https://www.readbyqxmd.com/read/27903264/adult-onset-still-s-disease-evaluation-of-prognostic-tools-and-validation-of-the-systemic-score-by-analysis-of-100-cases-from-three-centers
#1
Piero Ruscitti, Paola Cipriani, Francesco Masedu, Daniela Iacono, Francesco Ciccia, Vasiliki Liakouli, Giuliana Guggino, Francesco Carubbi, Onorina Berardicurti, Paola Di Benedetto, Marco Valenti, Giovanni Triolo, Gabriele Valentini, Roberto Giacomelli
BACKGROUND: Adult-onset Still's disease (AOSD) is rare inflammatory disease of unknown etiology that usually affects young adults. The more common clinical manifestations are spiking fevers, arthritis, evanescent rash, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis. The multi-visceral involvement of the disease and the different complications, such as macrophage activation syndrome, may strongly decrease the life expectancy of AOSD patients. METHODS: This study aimed to identify the positive and negative features correlated with the outcome of patients...
December 1, 2016: BMC Medicine
https://www.readbyqxmd.com/read/27898441/epidemiology-of-myopia
#2
Pei-Chang Wu, Hsiu-Mei Huang, Hun-Ju Yu, Po-Chiung Fang, Chueh-Tan Chen
Myopia is not a simple refractive error, but an eyesight-threatening disease. There is a high prevalence of myopia, 80% to 90%, in young adults in East Asia; myopia has become the leading cause of blindness in this area. As the myopic population increases globally, the severity of its impact is predicted. Approximately one fifth of the myopic population has high myopia (≥-6 diopters), which results in irreversible vision loss such as retinal detachment, choroidal neovascularization, cataracts, glaucoma, and macular atrophy...
November 2016: Asia-Pacific Journal of Ophthalmology
https://www.readbyqxmd.com/read/27888598/adult-onset-still-s-disease-with-different-antibodies-a-case-report-and-review-of-literature
#3
Maryam Mobini, Roya Ghasemian, Fatemeh Zameni
 Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. There is not currently any specific serological markers for AOSD , and  diagnosis still relying on the exclusion of other likely diagnoses. Yamaguchi's criteria are used as a diagnostic criterion which contains negative serologic markers for other collagen vascular diseases including systemic lupus erythematosus and rheumatoid arthritis. Here we report a 28-year-old woman with arthralgia, fever, rash, leukocytosis, lymphadenopathy, sore throat, abnormal liver function and negative rheumatoid factor and ANA but  seropositive for anti-CCP, anti-dsDNA, and C-ANCA...
October 2016: Acta Medica Iranica
https://www.readbyqxmd.com/read/27886796/adult-onset-still-s-disease-advances-in-the-treatment
#4
REVIEW
Santos Castañeda, Ricardo Blanco, Miguel A González-Gay
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder mainly characterized by persistent high spiking fevers, evanescent rash, and joint involvement. The pathogenesis of AOSD is only partially known, but pro-inflammatory cytokines such as tumor necrosis factor (TNF)-α, interleukin (IL)-1, IL-6, IL-18, and IFN-γ seem to play a major role in this disorder. AOSD is at the crossroad of auto-inflammatory syndromes and autoimmune diseases. It is diagnosed by exclusion to determine the presence of high serum ferritin levels, which is usually >1000 μg/L...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27865305/persistent-pruritic-lesions-in-adult-onset-still-s-disease
#5
Jianjun Qiao, Juan Bai, Hong Fang
No abstract text is available yet for this article.
November 2016: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/27852413/-clinical-analysis-of-acute-disseminated-encephalomyelitis-in-44-cases
#6
X N Zhong, B J Zhang, Y G Wang, Y L Huang, Y Q Shu, Z Z Lu, X Q Hu, W Qiu
Objective: To investigate the clinical features in 44 patients with acute disseminated encephalomyelitis (ADEM). Methods: Consecutive ADEM patients admitted to Neurology Department of the Third Affiliated Hospital of Sun yat-sen University during August 2009 to July 2014 were enrolled.Clinical and laboratory data of the patients were reviewed and analyzed. Results: Forty-four patients with ADEM based on the 2012 criteria were recruited, including 23 male and 21 female; 9 children, 11 teenagers and 24 adults...
October 25, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27847518/adult-onset-still-s-disease-associated-with-mycoplasma-pneumoniae-infection-and-hemophagocytic-lymphohistiocytosis
#7
Abhishek Agnihotri, Allison Ruff, Lauren Gotterer, Addie Walker, Amy H McKenney, Andrei Brateanu
Adult Onset Still's Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated by Mycoplasma pneumonia infection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27846765/adult-onset-still-s-disease-like-manifestation-accompanied-by-the-cancer-recurrence-after-long-term-resting-state
#8
Kazuhito Fukuoka, Ayako Miyamoto, Yuko Ozawa, Noriko Ikegaya, Tomohiro Maesono, Yoshinori Komagata, Shinya Kaname, Yoshihiro Arimura
A 72-year-old woman presented nine months ago with skin rash on her bilateral forearms, which was followed by intermittent high fever, and stiffness and swelling of her bilateral fingers. She was diagnosed with seronegative rheumatoid arthritis (RA). She had a past history of breast cancer and had undergone breast preservation surgery 13 years previously. During admission in our hospital, she developed high fever and leukocytosis with a relapsing skin rash, sore throat, polyarthralgia and increased levels of serum ALT/AST and ferritin, all of which fulfilled Yamaguchi's criteria for adult-onset Still's disease (AOSD)...
November 16, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27843372/wissler-fanconi-syndrome-and-related-diagnoses-a-case-report
#9
Mustafa Q Albustani, Robert F Howard
INTRODUCTION: Wissler-Fanconi syndrome is a rare rheumatic syndrome that was first described during the 1940s in Europe. Since then, many papers have been written that cover all aspects of this syndrome, most of which are in French and German language, with only a very few in English (none of them recent). We report here a case that fulfils the criteria for Wissler-Fanconi syndrome. Under the more general descriptive umbrella of Wissler-Fanconi syndrome, our patient also fulfils the Modified Jones criteria, and the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for rheumatoid arthritis, and was interpreted by other internists and another rheumatologist as fulfilling the Yamaguchi criteria for adult onset Still's disease...
2016: Open Access Rheumatol
https://www.readbyqxmd.com/read/27843366/adult-onset-still-s-disease-current-challenges-and-future-prospects
#10
REVIEW
Mariam Siddiqui, Michael S Putman, Anisha B Dua
Adult-onset Still's disease (AOSD) - a multi-systemic inflammatory condition characterized by high fevers, polyarthritis, an evanescent rash, and pharyngitis - has been a challenging condition to diagnose expediently and treat effectively. Questions remain regarding the underlying pathophysiology and etiology of AOSD. Pathognomonic diagnostic tests and reliable biomarkers remain undiscovered. Over the past decade, important progress has been made. Diagnostic criteria employing glycosylated ferritin have improved specificity...
2016: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/27826176/limited-diagnostic-value-of-procalcitonin-in-early-diagnosis-of-adult-onset-still-s-disease
#11
Ewelina Gowin, Jacek Wysocki
A 17-year-old female patient was referred to the Infectious Diseases Ward because of fever lasting for 14 days. On admission to the hospital the patient was in a generally good state, without any abnormalities on physical examination. Laboratory investigation revealed elevated inflammatory markers. Diagnostic imaging comprising chest X-ray, abdominal ultrasonography, and echocardiography showed no abnormalities. During the hospitalization, there occurred episodes of fever with skin rash and musculoskeletal pain of the lower limbs...
2016: Reumatologia
https://www.readbyqxmd.com/read/27825329/intravitreal-dexamethasone-implants-for-the-treatment-of-refractory-scleritis-combined-with-uveitis-in-adult-onset-still-s-disease-a-case-report
#12
Seong Joon Ahn, Sun Jin Hwang, Byung Ro Lee
BACKGROUND: Adult-onset Still's disease is a systemic inflammatory disease which presents with uveitis and scleritis in the eye. Intravitreal dexamethasone implants are used for the treatment of refractory uveitis. CASE PRESENTATION: A 19-year-old woman diagnosed to have adult-onset Still's disease for fevers, joint pain, and a salmon-colored bumpy rash presented with scleritis and uveitis in the left eye. Topical and systemic steroids with oral methotrexate failed to control the inflammation...
November 8, 2016: BMC Ophthalmology
https://www.readbyqxmd.com/read/27818826/macrophage-activation-syndrome-associated-with-adult-onset-still-s-disease-successfully-treated-with-anakinra
#13
Aswini Kumar, Hiroshi Kato
Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still's disease (Still's disease). Whereas an increasing body of evidence supports interleukin-1 (IL-1) blockade as a promising treatment for Still's disease, whether it is therapeutic for MAS associated with Still's disease remains unclear. We report a 34-year-old Caucasian man with one-decade history of TNF-blockade-responsive seronegative arthritis who presented with abrupt onset of fever, serositis, bicytopenia, splenomegaly, hepatitis, and disseminated intravascular coagulation...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27803155/bridging-the-gap-metabolic-and-endocrine-care-of-patients-during-transition
#14
REVIEW
Anita Hokken-Koelega, Aart-Jan van der Lely, Berthold Hauffa, Gabriele Häusler, Gudmundur Johannsson, Mohamad Maghnie, Jesús Argente, Jean DeSchepper, Helena Gleeson, John W Gregory, Charlotte Höybye, Fahrettin Keleştimur, Anton Luger, Hermann L Müller, Sebastian Neggers, Vera Popovic-Brkic, Eleonora Porcu, Lars Sävendahl, Stephen Shalet, Bessie Spiliotis, Maithé Tauber
OBJECTIVE: Seamless transition of endocrine patients from the paediatric to adult setting is still suboptimal, especially in patients with complex disorders, i.e., small for gestational age, Turner or Prader-Willi syndromes; Childhood Cancer Survivors, and those with childhood-onset growth hormone deficiency. METHODS: An expert panel meeting comprised of European paediatric and adult endocrinologists was convened to explore the current gaps in managing the healthcare of patients with endocrine diseases during transition from paediatric to adult care settings...
November 2016: Endocrine Connections
https://www.readbyqxmd.com/read/27787513/the-cyclic-vomiting-syndrome-threshold-a-framework-for-understanding-pathogenesis-and-predicting-successful-treatments
#15
David J Levinthal
Cyclic vomiting syndrome (CVS) is an uncommon, idiopathic disorder defined by recurrent, sudden-onset attacks of repetitive retching and vomiting that are separated by symptom-free intervals. CVS was long regarded as a disorder primarily experienced by children but is now known to present de novo in adulthood. Adult CVS has garnered more research attention over the past 20 years, and these efforts have identified some acute and prophylactic treatments for this disorder. However, CVS still lacks a unifying disease model, and this has hindered the development of new therapies...
October 27, 2016: Clinical and Translational Gastroenterology
https://www.readbyqxmd.com/read/27778518/macrophage-activation-syndrome-associated-with-tocilizumab-treatment-in-adult-onset-still-s-disease
#16
Yumi Tsuchida, Shuji Sumitomo, Hirofumi Shoda, Kanae Kubo, Keishi Fujio, Kazuhiko Yamamoto
No abstract text is available yet for this article.
October 25, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27761568/adult-onset-still-s-disease-with-dermatopathic-lymphadenopathy
#17
Ahmad Z Qureshi, Mohammad AlSheef, Waqas T Qureshi, Waseem Amjad
Adult onset Still's disease (AOSD) is a chronic inflammatory disorder involving multiple systems. The symptoms mimic those of lymphomas, therefore, the diagnosis of lymphoma needs to be excluded prior to establishing the diagnosis of AOSD. Another similar condition is dermatopathic lymphadenopathy (DL). In DL, the histopathological appearance of lymph node biopsy may also mimic AOSD. The DL is associated with several systemic pathologies, such as malignant lymphomas, and rarely AOSD. We present a case of a 43-year-old male presented with 3 months history of fatigue, fever, and lymphadenopathy...
November 2016: Saudi Medical Journal
https://www.readbyqxmd.com/read/27752356/juvenile-idiopathic-arthritis-in-adulthood-fulfilment-of-classification-criteria-for-adult-rheumatic-diseases-long-term-outcomes-and-predictors-of-inactive-disease-functional-status-and-damage
#18
Filipa Oliveira-Ramos, Mónica Eusébio, Fernando M Martins, Ana Filipa Mourão, Carolina Furtado, Raquel Campanilho-Marques, Inês Cordeiro, Joana Ferreira, Marcos Cerqueira, Ricardo Figueira, Iva Brito, Helena Canhão, Maria José Santos, José A Melo-Gomes, João Eurico Fonseca
OBJECTIVES: To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. METHODS: Patients with JIA registered on the Rheumatic Diseases Portuguese Register (Reuma.pt) older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index-articular (JADI-A) and Juvenile Arthritis Damage Index-extra-articular (JADI-E) damage index and disease activity were analysed...
2016: RMD Open
https://www.readbyqxmd.com/read/27751898/still-febrile-after-all-these-weeks
#19
Erica Lin, Isabel A Hujoel, Thomas G Mason
No abstract text is available yet for this article.
October 15, 2016: American Journal of Medicine
https://www.readbyqxmd.com/read/27728682/adult-onset-still-s-disease-a-rare-presentation
#20
Bikkina Ghana Syam, K Jayachandran, Tolstoy
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
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