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adult onset still disease

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https://www.readbyqxmd.com/read/28614216/refractory-adult-onset-still-disease-complicated-by-macrophage-activation-syndrome-and-acute-myocarditis-a-case-report-treated-with-high-doses-8%C3%A2-mg-kg-d-of-anakinra
#1
Federico Parisi, Annamaria Paglionico, Valentina Varriano, Gianfranco Ferraccioli, Elisa Gremese
RATIONALE: Myocarditis is a rare but potentially fatal complication of Still's disease (about 7% of total cases). PATIENT CONCERNS: A 42-year-old woman was admitted to our ward with high-grade fever, rash and polyarthralgia, lasting since 4 weeks and rapidly complicated by MAS and acute heart failure. DIAGNOSES: Adult Onset Still's Disease rapidly developping macrophage activation syndrome and disseminated intravascular coagulopathy, further complicated by iperacute myocarditis with cardiac arrest...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28603586/sleep-health-behaviors-and-behavioral-interventions-reducing-the-risk-of-cardiovascular-disease-in-adults
#2
REVIEW
Jill L Kaar, Christina M Luberto, Kirsti A Campbell, Jeff C Huffman
Numerous health behaviors, including physical activity, diet, smoking, and sleep, play a major role in preventing the development and progression of cardiovascular disease (CVD). Among these behaviors, sleep may play a pivotal role, yet it has been studied somewhat less than other behaviors and there have been few well-designed sleep intervention studies targeting CVD. Furthermore, despite the fact that these behaviors are often interrelated, interventions tend to focus on changing one health behavior rather than concurrently intervening on multiple behaviors...
May 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28601443/a-cohort-study-of-the-effect-of-winter-dysentery-on-herd-level-milk-production
#3
Ingrid Toftaker, Ingrid Holmøy, Ane Nødtvedt, Olav Østerås, Maria Stokstad
Winter dysentery (WD) is a contagious disease caused by bovine coronavirus. It is characterized by acute onset of diarrhea, fever, depression, and reduced milk yield in adult cattle. Although production loss is a well-known consequence of WD, large-scale studies estimating the effect on milk production are lacking. The objective of this study was to estimate the effect of farmer-reported WD on herd-level milk production and milk composition. A cohort study was performed based on reports of herd outbreaks of winter dysentery during a regional epidemic in Norway during the winter of 2011-2012...
June 7, 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/28598057/macrophage-activation-syndrome
#4
P Babu Raj, B L Harikrishnan, Roshan Mampilly, R Anand
Macrophage Activation Syndrome (MAS) occurs as a severe life-threatening complication of several chronic rheumatic diseases. It is more frequent with systemic onset juvenile arthritis and adult onset Still's disease.1 It can be primary, infection related, malignancy associated or autoimmune3. We report a case of Macrophage Activation Syndrome presenting as pyrexia of unknown origin (PUO) and pancytopenia in the absence of any known triggering factor.
May 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28589388/-efficacy-and-safety-analysis-of-off-label-treatment-with-biologics-in-autoinflammatory-diseases-experiences-from-a%C3%A2-german-registry%C3%A2-graid2
#5
F Proft, M Fleck, C Fiehn, H Schulze-Koops, M Witt, T Dörner, J C Henes
OBJECTIVE: To evaluate the safety and efficacy of therapy with biologics in patients with autoinflammatory diseases (AIF) or macrophage activating syndrome (MAS) in a real-life setting in Germany. METHODS: The German Register of Autoimmune Diseases 2 (GRAID2) is a retrospective, non-interventional, multicenter registry collecting data from all patients with inflammatory rheumatic diseases refractory to conventional therapy and treated with initial off-label biologics between August 2006 and December 2013...
June 6, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28588495/a-brief-history-of-il-1-and-il-1-ra-in-rheumatology
#6
Jean-Michel Dayer, Francesca Oliviero, Leonardo Punzi
The history of what, in 1979, was called interleukin-1 (IL-1), orchestrator of leukocyte inter-communication, began many years before then, initially by the observation of fever induction via the endogenous pyrogen (EP) (1974) and then in rheumatology on the role in tissue destruction in rheumatoid diseases via the induction of collagenase and PGE2 in human synovial cells by a mononuclear cell factor (MCF) (1977). Since then, the family has exploded to presently 11 members as well as many membrane-bound and soluble receptor forms...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28575651/mutations-in-nkx6-2-cause-progressive-spastic-ataxia-and-hypomyelination
#7
Viorica Chelban, Nisha Patel, Jana Vandrovcova, M Natalia Zanetti, David S Lynch, Mina Ryten, Juan A Botía, Oscar Bello, Eloise Tribollet, Stephanie Efthymiou, Indran Davagnanam, Fahad A Bashiri, Nicholas W Wood, James E Rothman, Fowzan S Alkuraya, Henry Houlden
Progressive limb spasticity and cerebellar ataxia are frequently found together in clinical practice and form a heterogeneous group of degenerative disorders that are classified either as pure spastic ataxia or as complex spastic ataxia with additional neurological signs. Inheritance is either autosomal dominant or autosomal recessive. Hypomyelinating features on MRI are sometimes seen with spastic ataxia, but this is usually mild in adults and severe and life limiting in children. We report seven individuals with an early-onset spastic-ataxia phenotype...
June 1, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28572051/late-onset-rasmussen-encephalitis-a-literature-appraisal
#8
REVIEW
Sophie Dupont, Ana Gales, Serge Sammey, Marie Vidailhet, Virginie Lambrecq
Rasmussen Encephalitis (RE) is classically described as a childhood encephalopathy due to a unilateral inflammation of the cerebral cortex with a presumed immune-mediated pathophysiological basis. Unusual variant forms, including adolescent and adult-onset RE have been described but there is still a doubt whether these atypical cases correspond to classical RE patients. To review evidence, a systematic PubMed search was conducted to retrieve papers addressing late onset RE to assess (i) the positivity rate of classical childhood-onset diagnostic criteria for RE in late-onset RE, (ii) the specific clinical and radiological features that could help earlier diagnosis and therapeutic interventions, (iii) the arguments for an autoimmune pathophysiology including (iiia) the association with autoimmune markers or diseases and (iiib) the effects of immunomodulatory or immunosuppressive treatments...
May 29, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28540751/treatment-of-adult-onset-still-s-disease-up-to-date
#9
Dae Hyun Yoo
Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology, and approximately 60-70% of patients may develop a chronic polyphasic form of the disease or a chronic polyarthritis. Due to rarity of disease, treatment of AOSD is not based on controlled study, but on case based experiences. Areas covered: Recently, the application of anti-cytokine therapy based on pathophysiology has resulted in significant progress in the treatment of AOSD. Here, we review current knowledge of the pathogenesis, disease progression, currently available biomarkers of disease activity, standard therapeutic agents, utility of biologic agents, future perspectives for treatment and treatment of macrophage activation syndrome...
June 5, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28540590/pediatric-psoriasis
#10
REVIEW
Maria Relvas, Tiago Torres
Psoriasis is a chronic inflammatory systemic disease associated with an important physical and physiological burden. It primarily affects the skin, but it is associated with several serious medical co-morbidities. One third of total psoriatic cases have their onset during the pediatric age, although some of them may not be diagnosed until the patient reaches adulthood. Additionally, in the pediatric age, there is an association with several medical co-morbidities; thus, an early recognition of the disease and a subsequent appropriate approach may delay or even prevent considerable co-morbidities...
May 24, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28532741/coexistence-of-sarcoidosis-and-adult-onset-still-disease
#11
Huseyin Semiz, Senol Kobak
Sarcoidosis is a chronic, inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It can be presented with bilateral hilar lymphadenopathy, skin lesions, eye involvement and locomotor system findings. Adult onset Still disease (AOSD) is a chronic inflammatory disease which presents with fever, arthritis and typical skin rashes. The disease is rare and can be misdiagnosed due to the absence of typical clinical and laboratory findings. The association of sarcoidosis and AOSD has not been previously reported in the literature...
May 19, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28531906/tocilizumab-induced-drug-reaction-with-eosinophilia-and-systemic-symptoms-syndrome-in-adult-onset-still-disease-a-case-report
#12
Elina Zuelgaray, Fanny Domont, Nathan Peiffer-Smadja, David Saadoun, Patrice Cacoub
No abstract text is available yet for this article.
May 23, 2017: Annals of Internal Medicine
https://www.readbyqxmd.com/read/28529117/new-markers-for-adult-onset-still-s-disease
#13
Stéphane Mitrovic, Bruno Fautrel
Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disorder (SAID). Although the pathogenesis of the disease is complex and far from being fully understood, recent progresses in pathophysiological knowledge have paved the way to new diagnostic approaches. Indeed, AOSD diagnosis can be a real challenge, owing to its infrequency, and to the lack of specificity of the principal clinical features (high fever, arthralgia or arthritis, skin rash) and laboratory findings (elevated acute phase reactants, hyperleukocytosis ≥ 10,000 cells/mm(3) with neutrophils ≥80%)...
May 18, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28507179/elevated-expression-of-the-nlrp3-inflammasome-and-its-correlation-with-disease-activity-in-adult-onset-still-disease
#14
Chia-Wei Hsieh, Yi-Ming Chen, Chi-Chen Lin, Kuo-Tung Tang, Hsin-Hua Chen, Wei-Ting Hung, Kuo-Lung Lai, Der-Yuan Chen
OBJECTIVE: The dysregulation of the NLRP3 (NLR containing a pyrin domain) inflammasome is involved in autoinflammatory diseases. Adult-onset Still disease (AOSD) is regarded as an autoinflammatory disease. However, the pathogenic involvement of NLRP3 inflammasome in AOSD remains unclear and NLRP3 activators in AOSD are currently unknown. METHODS: The mRNA expression of NLRP3 inflammasome signaling in peripheral blood mononuclear cells (PBMC) from 34 patients with AOSD and 14 healthy subjects was determined using quantitative-PCR (qPCR)...
May 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28506441/association-of-traumatic-brain-injury-and-alzheimer-disease-onset-a-systematic-review
#15
REVIEW
J Julien, S Joubert, M-C Ferland, L C Frenette, M M Boudreau-Duhaime, L Malo-Véronneau, E de Guise
BACKGROUND: Inconsistencies regarding the risk of developing Alzheimer disease after traumatic brain injury (TBI) remain in the literature. Indeed, why AD develops in certain TBI patients while others are unaffected is still unclear. OBJECTIVE: The aim of this study was to performed a systematic review to investigate whether certain variables related to TBI, such as TBI severity, loss of consciousness (LOC) and post-traumatic amnesia (PTA), are predictors of risk of AD in adults...
May 11, 2017: Annals of Physical and Rehabilitation Medicine
https://www.readbyqxmd.com/read/28503110/pediatric-mania-the-controversy-between-euphoria-and-irritability
#16
Giulia Serra, Mai Uchida, Claudia Battaglia, Maria Pia Casini, Lavinia De Chiara, Joseph Biederman, Stefano Vicari, Janet Wozniak
Pediatric Bipolar Disorder (BD) is a highly morbid pediatric psychiatric disease, consistently associated with family psychiatric history of mood disorders and associated with high levels of morbidity and disability and with a great risk of suicide. While there is a general consensus on the symptomatology of depression in childhood, the phenomenology of pediatric mania is still highly debated and the course and long-term outcome of pediatric BD still need to be clarified. We reviewed the available studies on the phenomenology of pediatric mania with the aim of summarizing the prevalence, demographics, clinical correlates and course of these two types of pediatric mania...
April 2017: Current Neuropharmacology
https://www.readbyqxmd.com/read/28497222/interleukin-1-blockade-an-update-on-emerging-indications
#17
REVIEW
Anoop Mistry, Sinisa Savic, Jeroen C H van der Hilst
Interleukin (IL)-1 is a pro-inflammatory cytokine that induces local and systemic inflammation aimed to eliminate microorganisms and tissue damage. However, an increasing number of clinical conditions have been identified in which IL-1 production is considered inappropriate and IL-1 is part of the disease etiology. In autoinflammatory diseases, gout, Schnitzler's syndrome, and adult-onset Still's disease, high levels of inappropriate IL-1 production have been shown to be a key process in the etiology of the disease...
May 11, 2017: BioDrugs: Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy
https://www.readbyqxmd.com/read/28479525/lhermitte-duclos-disease-dysplastic-gangliocytoma-of-the-cerebellum-and-cowden-syndrome-clinical-experience-from-a-single-institution-with-long-term-follow-up
#18
Tao Jiang, Junmei Wang, Jiang Du, Shiqi Luo, Raynald Liu, Jian Xie, Ying Wang, Chunde Li
BACKGROUND: Adult-onset Lhermitte-Duclos disease (LDD) and Cowden syndrome (CS) are considered to be a single phakomatosis which belongs to PTEN Hamartoma Tumor Syndrome (PHTS) now. There is still controversy about the diagnosis and treatment. The authors describe the clinical features of LDD and CS with long term follow up. METHODS: From January 2001 to January 2017, 18 patients were admitted to the neurosurgery department of Beijing Tiantan Hospital. The authors analyzed the medical records of each patient and followed up every case...
May 4, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28470759/phosphorylated-signal-transducer-and-activator-of-transcription-3-in-the-epidermis-in-adult-onset-still-s-disease
#19
Takuya Takeichi, Naoki Watanabe, Yoshinao Muro, Shiho Teshigawara, Motoki Sato, Nobutaro Ban, Masashi Akiyama
Adult-onset Still's disease (AOSD) is characterized by multiple systemic inflammation of unknown etiology. Although the typical eruption of AOSD is salmon-pink rheumatoid rash on the trunk and extremities, persistent pruritic papules and plaques have also been reported. Correlations between serum cytokines, including interleukin-6 and -18, and disease activity in AOSD have been reported. Activated signal transducer and activator of transcription 3 (STAT3) is transported into the nucleus, where it functions as a transcription factor that regulates genes involved in cell survival and inflammation...
May 4, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28451698/-still-s-disease-in-children-and-adults
#20
C M Hedrich, C Günther, M Aringer
Systemic juvenile idiopathic arthritis (sJIA) is characterized by fever, arthritis, and other signs of systemic inflammation. Historically, sJIA was named Still's disease after George Frederic Still, who first reported patients. Individuals who manifest after the 16(th) birthday are diagnosed with adult onset Still's disease (AOSD). The pathophysiology of sJIA and AOSD are incompletely understood. Increased activation of inflammasomes and the expression of proinflammatory cytokines play a central role. S100 proteins, which can activate Toll-like receptors, thus, maintaining positive feedback loops, have also been detected at increased levels in sera from sJIA patients...
June 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
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