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adult onset still disease

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https://www.readbyqxmd.com/read/28540751/treatment-of-adult-onset-still-s-disease-up-to-date
#1
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. 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...
May 25, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28540590/pediatric-psoriasis
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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...
April 27, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28438546/clinical-predictors-of-remission-and-persistence-of-adult-onset-asthma
#13
Guus A Westerhof, Hanneke Coumou, Selma B de Nijs, ElsJ Weersink, Elizabeth H Bel
BACKGROUND: Adult-onset asthma is an important but relatively understudied asthma phenotype and little is known about its natural course and prognosis. The remission rate is believed to be low, and it is still obscure which factors predict remission or persistence of the disease. OBJECTIVE: To determine the remission rate, and identify predictors of persistence and remission of adult-onset asthma. METHODS: Two hundred adult patients with recently diagnosed (<1 year) asthma were recruited from secondary and tertiary pulmonary clinics and prospectively followed for 5 years...
April 21, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28436448/elevated-circulating-levels-of-the-interferon-%C3%AE-induced-chemokines-are-associated-with-disease-activity-and-cutaneous-manifestations-in-adult-onset-still-s-disease
#14
Jae Ho Han, Chang-Hee Suh, Ju-Yang Jung, Mi-Hyun Ahn, Mi Hwa Han, Ji Eun Kwon, Hyunee Yim, Hyoun-Ah Kim
C-X-C motif chemokine 9 (CXCL9), CXCL10, and CXCL11 are produced in response to interferon-γ (IFN-γ) and trigger inflammation with the accumulation of activated lymphocytes. It appears that these chemokines could play a role in the pathogenesis of adult-onset Still's disease (AOSD). Therefore, we investigated the associations between the levels of these chemokine and clinical manifestations in patients with active AOSD. Serum levels of IFN-γ, CXCL9, CXCL10 and CXCL11 were determined using enzyme-linked immunosorbent assays...
April 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28424565/invasive-pneumococcal-disease-still-lots-to-learn-and-a-need-for-standardized-data-collection-instruments
#15
T J Marrie, G J Tyrrell, Sumit R Majumdar, Dean T Eurich
Background. Large studies of invasive pneumococcal disease (IPD) are frequently lacking detailed clinical information. Methods. A population-based 15-year study of IPD in Northern Alberta. Results. 2435 patients with a mean age of 54.2 years formed the study group. Males outnumbered females and Aboriginal and homeless persons were overrepresented. High rates of smoking, excessive alcohol use, and illicit drug use were seen. Almost all (87%) had a major comorbidity and 15% had functional limitations prior to admission...
2017: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/28421535/microrna-metabolism-and-dysregulation-in-amyotrophic-lateral-sclerosis
#16
REVIEW
Paola Rinchetti, Mafalda Rizzuti, Irene Faravelli, Stefania Corti
MicroRNAs (miRNAs) are a subset of endogenous, small, non-coding RNA molecules involved in the post-transcriptional regulation of eukaryotic gene expression. Dysregulation in miRNA-related pathways in the central nervous system (CNS) is associated with severe neuronal injury and cell death, which can lead to the development of neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS). ALS is a fatal adult onset disease characterized by the selective loss of upper and lower motor neurons. While the pathogenesis of ALS is still largely unknown, familial ALS forms linked to TAR DNA-binding protein 43 (TDP-43) and fused in sarcoma (FUS) gene mutations, as well as sporadic forms, display changes in several steps of RNA metabolism, including miRNA processing...
April 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28421028/adult-onset-niemann-pick-disease-type-c-rapid-treatment-initiation-advised-but-early-diagnosis-remains-difficult
#17
Tobias Piroth, Kai Boelmans, Florian Amtage, Michel Rijntjes, Anna Wierciochin, Thomas Musacchio, Cornelius Weiller, Jens Volkmann, Stephan Klebe
Niemann-Pick type C disease (NP-C) presents with heterogeneous neurological and psychiatric symptoms. Adult onset is rare and possibly underdiagnosed due to frequent lack of specific and obvious key symptoms. For both early and adolescent/adult onset, the available data from studies and case reports describe a positive effect of Miglustat (symptom relief or stabilization). However, due to the low frequency of NP-C, experience with this therapy is still limited. We describe two adult-onset cases of NP-C. In both cases, vertical supranuclear gaze palsy was not recognized at symptom onset...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28412707/application-of-the-2016-eular-acr-printo-classification-criteria-for-macrophage-activation-syndrome-in-patients-with-adult-onset-still-disease
#18
Sung Soo Ahn, Byung-Woo Yoo, Seung Min Jung, Sang-Won Lee, Yong-Beom Park, Jason Jungsik Song
OBJECTIVE: To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with adult-onset Still disease (AOSD). METHODS: We performed a retrospective analysis of patients with AOSD with fever who were admitted to Severance Hospital between 2005 and 2016...
April 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28411118/regulation-of-motor-proteins-axonal-transport-deficits-and-adult-onset-neurodegenerative-diseases
#19
REVIEW
Scott T Brady, Gerardo A Morfini
Neurons affected in a wide variety of unrelated adult-onset neurodegenerative diseases (AONDs) typically exhibit a "dying back" pattern of degeneration, which is characterized by early deficits in synaptic function and neuritic pathology long before neuronal cell death. Consistent with this observation, multiple unrelated AONDs including Alzheimer's disease, Parkinson's disease, Huntington's disease, and several motor neuron diseases feature early alterations in kinase-based signaling pathways associated with deficits in axonal transport (AT), a complex cellular process involving multiple intracellular trafficking events powered by microtubule-based motor proteins...
April 11, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28397955/unusual-manifestations-and-unusual-mimics-of-adult-onset-still-s-disease
#20
Marwan H Adwan, Faris G Bakri, Ahmad Z Qureshi
[No Abstract Available].
April 2017: Saudi Medical Journal
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