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ADULT ONSET STILL

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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/28527721/indicators-and-outcomes-of-transfer-to-tertiary-pediatric-hospitals-for-patients-with-testicular-torsion
#6
Janae Preece, Christina Ching, Katelyn Yackey, Venkata Jayanthi, Daryl McLeod, Seth Alpert, Daniel DaJusta
INTRODUCTION: Testicular torsion threatens testicular viability with increased risk of loss with delayed management. Still, healthy adolescents continue to be transferred from community hospitals to tertiary hospitals for surgical management for torsion even though adult urologists may be available. We sought to determine reasons behind patient transfer and to evaluate whether transfer to tertiary centers for testicular torsion leads to increased rates of testicular loss. MATERIALS AND METHODS: A retrospective chart review was performed for patients presenting to our free-standing pediatric tertiary care facility with surgically confirmed testicular torsion during the 5-year period between January 2011 and January 2016...
April 27, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28526006/cardiometabolic-profile-of-obese-children-in-a-sub-saharan-african-setting-a-cross-sectional-study
#7
Eunice Chedjou-Nono, Suzanne Sap, Simeon-Pierre Choukem, Issa Ngosso Tetanye, Daniel Nebongo, Olivier Koki Ndombo
BACKGROUND: Cardiovascular and metabolic consequences of obesity in children, unlike adults, are still not well understood nor have they been subject to extensive research in Africa. We aimed to identify the cardio-metabolic complications associated with childhood obesity at the early phase of the management of obese children in a reference center in Cameroon. METHODS: In this cross-sectional study conducted from November 2013 to September 2014 and based on World Health Organization (WHO) classification of Obesity (BMI > 3SD under 5 years and BMI > 2SD from 5 and above), we included children aged 3 to 17 years who were being followed up for obesity at the pediatric endocrinology unit of the Mother and Child Center of the Chantal BIYA Foundation in Yaounde, Cameroon...
May 19, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28510288/the-relationship-of-physiopsychosocial-factors-and-spiritual-well-being-in-elderly-residents-implications-for-evidence-based-practice
#8
Yi-Heng Chen, Li-Chan Lin, Li-Lan Chuang, Mei-Li Chen
BACKGROUND: Older adults in residential settings frequently suffer from functional decline, mental illness, and social isolation, which make them more vulnerable to spiritual distress. However, empirical evidence of the interrelationships between physiopsychosocial variables and spiritual well-being are still lacking, limiting the application of the biopsychosocial-spiritual model in institutional healthcare practice. AIMS: To explain the mechanisms by which these variables are linked, this cross-sectional study tested a causal model of predictors of spiritual well-being among 377 institutionalized older adults with disability using a structural equation modeling approach...
May 16, 2017: Worldviews on Evidence-based Nursing
https://www.readbyqxmd.com/read/28507179/elevated-expression-of-the-nlrp3-inflammasome-and-its-correlation-with-disease-activity-in-adult-onset-still-disease
#9
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
#10
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
#11
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
#12
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/28492421/description-of-malignancy-rates-in-childhood-and-adult-onset-systemic-lupus-erythematous-by-proportional-meta-analysis
#13
Priscila Rodrigues da Silva Aoki, Regina El Dib, Clovis Artur Almeida Silva, Claudia Saad Magalhaes
OBJECTIVE: Describe malignancy rates in childhood onset and adult onset systemic lupus erythematous (SLE) by proportional meta-analysis. METHODS: Two reviewers screened data from PubMed (1966-2015), EMBASE (1980-2015), and LILACS (1982-2015) for SLE-associated malignancy. Proportional meta-analysis with a random-effects model and 95% confidence intervals (CIs) were calculated according to SLE onset age and mean follow-up time. Statistical difference was defined by 95% CI overlap...
June 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
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
#14
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/28474982/posttraumatic-epilepsy-long-term-follow-up-of-children-with-mild-traumatic-brain-injury
#15
Amit Keret, Odeya Bennett-Back, Guy Rosenthal, Tal Gilboa, Moatasim Shweiki, Yigal Shoshan, Mony Benifla
OBJECTIVE Posttraumatic epilepsy (PTE) is a known complication of traumatic brain injury (TBI). The true incidence of PTE in children is still uncertain, because most research has been based primarily on adults. This study aimed to determine the true incidence of PTE in a pediatric population with mild TBI (MTBI) and to identify risk factors for the development of epileptic events. METHODS Data were collected from electronic medical records of children 0-17 years of age, who were admitted to a single medical center between 2007 and 2009 with a diagnosis of MTBI...
May 5, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28470759/phosphorylated-signal-transducer-and-activator-of-transcription-3-in-the-epidermis-in-adult-onset-still-s-disease
#16
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/28464258/long-term-outcome-in-adolescent-onset-generalized-genetic-epilepsies
#17
Bernd J Vorderwülbecke, Alexander B Kowski, Andrea Kirschbaum, Hannah Merkle, Philine Senf, Dieter Janz, Martin Holtkamp
OBJECTIVE: Until now, it has been unclear if the three subsyndromes of adolescent-onset generalized genetic epilepsy (GGE) differ in long-term prognosis. Therefore, this study aimed to compare long-term seizure outcome in juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and epilepsy with generalized tonic-clonic seizures alone (EGTCS). METHODS: This retrospective study is based on the archive of an institutional tertiary care outpatient clinic for adult patients with epilepsy...
May 2, 2017: Epilepsia
https://www.readbyqxmd.com/read/28461699/cortical-abnormalities-in-bipolar-disorder-an-mri-analysis-of-6503-individuals-from-the-enigma-bipolar-disorder-working-group
#18
D P Hibar, L T Westlye, N T Doan, N Jahanshad, J W Cheung, C R K Ching, A Versace, A C Bilderbeck, A Uhlmann, B Mwangi, B Krämer, B Overs, C B Hartberg, C Abé, D Dima, D Grotegerd, E Sprooten, E Bøen, E Jimenez, F M Howells, G Delvecchio, H Temmingh, J Starke, J R C Almeida, J M Goikolea, J Houenou, L M Beard, L Rauer, L Abramovic, M Bonnin, M F Ponteduro, M Keil, M M Rive, N Yao, N Yalin, P Najt, P G Rosa, R Redlich, S Trost, S Hagenaars, S C Fears, S Alonso-Lana, T G M van Erp, T Nickson, T M Chaim-Avancini, T B Meier, T Elvsåshagen, U K Haukvik, W H Lee, A H Schene, A J Lloyd, A H Young, A Nugent, A M Dale, A Pfennig, A M McIntosh, B Lafer, B T Baune, C J Ekman, C A Zarate, C E Bearden, C Henry, C Simhandl, C McDonald, C Bourne, D J Stein, D H Wolf, D M Cannon, D C Glahn, D J Veltman, E Pomarol-Clotet, E Vieta, E J Canales-Rodriguez, F G Nery, F L S Duran, G F Busatto, G Roberts, G D Pearlson, G M Goodwin, H Kugel, H C Whalley, H G Ruhe, J C Soares, J M Fullerton, J K Rybakowski, J Savitz, K T Chaim, M Fatjó-Vilas, M G Soeiro-de-Souza, M P Boks, M V Zanetti, M C G Otaduy, M S Schaufelberger, M Alda, M Ingvar, M L Phillips, M J Kempton, M Bauer, M Landén, N S Lawrence, N E M van Haren, N R Horn, N B Freimer, O Gruber, P R Schofield, P B Mitchell, R S Kahn, R Lenroot, R Machado-Vieira, R A Ophoff, S Sarró, S Frangou, T D Satterthwaite, T Hajek, U Dannlowski, U F Malt, V Arolt, W F Gattaz, W C Drevets, X Caseras, I Agartz, P M Thompson, O A Andreassen
Despite decades of research, the pathophysiology of bipolar disorder (BD) is still not well understood. Structural brain differences have been associated with BD, but results from neuroimaging studies have been inconsistent. To address this, we performed the largest study to date of cortical gray matter thickness and surface area measures from brain magnetic resonance imaging scans of 6503 individuals including 1837 unrelated adults with BD and 2582 unrelated healthy controls for group differences while also examining the effects of commonly prescribed medications, age of illness onset, history of psychosis, mood state, age and sex differences on cortical regions...
May 2, 2017: Molecular Psychiatry
https://www.readbyqxmd.com/read/28451698/-still-s-disease-in-children-and-adults
#19
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/28450379/posner-schlossman-syndrome-in-wenzhou-china-a-retrospective-review-study
#20
Jun Hong Jiang, Shao Dan Zhang, Ma Li Dai, Juan Yuan Yang, Yan Qian Xie, Cheng Hu, Guang Yun Mao, Fan Lu, Yuan Bo Liang
AIM: To describe the incidence of Posner-Schlossman syndrome (PSS) in Lucheng District, Wenzhou, China, over a 10-year period. METHODS: We reviewed retrospectively the medical records of all inpatient and outpatient patients diagnosed with PSS during the years 2005-2014 in the Eye Hospital of Wenzhou Medical University. The keywords of 'glaucomatocyclitic crisis', 'Posner-Schlossman syndrome' and 'PSS' were used for the retrieval. Only patients with registered residing address in Lucheng District where the hospital located were finally selected...
April 27, 2017: British Journal of Ophthalmology
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