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Adult onset stills

Katherine A Lyseng-Williamson
The EU indication for anakinra has been extended to include Still's disease, a serious rare inflammatory disorder of unknown aetiology that comprises adult-onset Still's disease (AOSD) and systemic juvenile idiopathic arthritis (SJIA). As activated interleukin-1 pathways are associated with the systemic manifestations of these disorders, targeted treatment with anakinra, an interleukin-1 inhibitor, has been investigated. Across clinical and real-world studies in patients with AOSD and SJIA, treatment with anakinra achieved clinical remission/response, provided rapid and sustained improvements in systemic and laboratory manifestations, and allowed the use of corticosteroid- and disease-modifying anti-rheumatic drugs (DMARD) to be reduced or discontinued...
2018: Drugs & Therapy Perspectives: for Rational Drug Selection and Use
Makiko Yashiro, Hiroshi Furukawa, Tomoyuki Asano, Shuzo Sato, Hiroko Kobayashi, Hiroshi Watanabe, Eiji Suzuki, Tadashi Nakamura, Tomohiro Koga, Toshimasa Shimizu, Masataka Umeda, Fumiaki Nonaka, Yukitaka Ueki, Katsumi Eguchi, Atsushi Kawakami, Kiyoshi Migita
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder in which inflammasome activation plays a pathophysiological role. In view of the inflammatory nature of AOSD, we investigated whether serum amyloid A (SAA) gene polymorphisms affect the susceptibility of patients with AOSD.Eighty-seven Japanese patients with AOSD and 200 healthy Japanese subjects were recruited in this study. The genotypes of the -13C/T SNP in the 5'-flanking region of the SAA1 gene (rs12218) and two SNPs within exon 3 of SAA1 (2995C/T and 3010C/T polymorphisms) were determined using polymerase chain reaction fragment length polymorphism (PCR-RFLP) assay in all subjects...
December 2018: Medicine (Baltimore)
Anna Portale, Agata Fiumara, Luisa Scalora, Filippo Greco, Pierluigi Smilari, Valeria Venti, Elena Pustorino, Piero Pavone
INTRODUCTION: Stroke is the clinical designation for a rapidly developing loss of brain function due to an interruption in the blood supply to all or part of the brain. It is the third cause of death in adults and one of the top 10 causes in pediatric age. The perinatal period of onset is the second only to adult age group in the incidence of stroke. Arterial ischemic stroke during childhood occurs most frequently in the perinatal period with an incidence of 1 out 2300-5000 live infant births...
December 12, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Dilek Karadoğan, Özgür Önal, Yalçın Kanbay
BACKGROUND AND AIM: Smoking is still a public health concern in many countries, especially among young adults. Consequently, we determined what factors affect university students' smoking behavior in Turkey. METHODS: This cross-sectional study was conducted between March and June 2017 using a simple random sampling method. A self-administered questionnaire was used to collect information on participants' (N = 2,505; mean age = 20.9 ± 2.5 years; 58.9% women) sociodemographic characteristics, cigarette smoking status, and related risk factors...
2018: PloS One
Zhu-Man Lv, Rong-Jian Zhao, Xiao-Song Zhi, Ying Huang, Jia-Yin Chen, Ning-Ning Song, Chang-Jun Su, Yu-Qiang Ding
Adult neurogenesis is present in the dentate gyrus and the subventricular zone in mammalian brain under physiological conditions. Recently, adult neurogenesis has also been reported in other brain regions after brain injury. In this study, we established a focal striatal ischemic model in adult mice via photothrombosis (PT) and investigated how focal ischemia elicits neurogenesis in the striatum. We found that astrocytes and microglia increased in early post-ischemic stage, followed by a 1-week late-onset of doublecortin (DCX) expression in the striatum...
2018: Frontiers in Cellular Neuroscience
Andreas Traschütz, Judith van Gaalen, Mayke Oosterloo, Maaike Vreeburg, Erik-Jan Kamsteeg, Natalie Deininger, Olaf Rieß, Matthias Reimold, Tobias Haack, Ludger Schöls, Bart P van de Warrenburg, Matthis Synofzik
Spinocerebellar ataxia type 21 (SCA21/ATX-TMEM240) was recently found to be caused by mutations in TMEM240, with still limited knowledge on the phenotypic spectrum and disease course. Here we present five subjects from three novel SCA21 families from different parts of the world (including a novel c.196G > A, p.G66R TMEM240 variant from Colombia), demonstrating that, in addition to cerebellar ataxia, not only hypokinetic features (hypomimia, bradykinesia), but also hyperkinetic movement disorders (poly-mini-myoclonus, proximal myoclonus) are a recurrent part of the phenotypic spectrum of SCA21...
November 29, 2018: Parkinsonism & related Disorders
Vijay Kumar
Sepsis is a major health problem all over the world. Despite its existence since the time of Hippocrates (470 BC), sepsis is still a serious medical problem for physicians working in both pediatric and adult intensive care units. The most current US FDA-approved drug called recombinant human activated protein C or Drotrecogin-α is also failed in clinical trials and showed similar effects as placebo. The epidemiological data and studies have indicated sepsis as a major socioeconomic burden all over the world...
December 1, 2018: Inflammation
L Brussino, E Heffler, C Bucca, S Nicola, G Rolla
Asthma is a chronic and heterogeneous disease, which is defined as severe disease whenever it requires treatment with a high dose of inhaled corticosteroids plus a second controller and/or systemic corticosteroids to prevent it from becoming ''uncontrolled" or if it remains ''uncontrolled" despite this therapy. Severe asthma is a heterogeneous condition consisting of phenotypes such as eosinophilic asthma, which is characterized by sputum eosinophilia, associated with mild to moderate increase in blood eosinophil count, frequently adult-onset, and associated with chronic rhinosinusitis with nasal polyps in half of the cases...
2018: BioMed Research International
E Barbour-Tuck, M C Erlandson, W Johnson, N Muhajarine, H Foulds, A D G Baxter-Jones
BACKGROUND: The prevalence of overweight and obesity doubles between adolescence and young adulthood. However, the exact age, and appropriate metric to use, to identify when overweight develops is still debated. AIM: To examine the age of onset of overweight by sex and four metrics: body mass index (BMI), fat mass (%FM), waist circumference (WC) and waist-to-height ratio (WHtR). SUBJECTS AND METHODS: Between 1991 and 2017, serial measures of body composition, were taken on 237 (108 males) individuals (aged 8 to 40 years of age)...
November 29, 2018: Annals of Human Biology
Lisette H Koens, Marina A J Tijssen, Fiete Lange, Bruce H R Wolffenbuttel, Alessandra Rufa, David S Zee, Tom J de Koning
Inborn errors of metabolism in adults are still largely unexplored. Despite the fact that adult-onset phenotypes have been known for many years, little attention is given to these disorders in neurological practice. The adult-onset presentation differs from childhood-onset phenotypes, often leading to considerable diagnostic delay. The identification of these patients at the earliest stage of disease is important, given that early treatment may prevent or lessen further brain damage. Neurological and psychiatric symptoms occur more frequently in adult forms...
November 28, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
Julia C Wilmanns, Raghav Pandey, Olivia Hon, Anjana Chandran, Jan M Schilling, Elvira Forte, Qizhu Wu, Gael Cagnone, Preeti Bais, Vivek Philip, David Coleman, Heidi Kocalis, Stuart K Archer, James T Pearson, Mirana Ramialison, Joerg Heineke, Hemal H Patel, Nadia A Rosenthal, Milena B Furtado, Mauro W Costa
OBJECTIVE: Congenital heart disease (CHD) is the most frequent birth defect worldwide. The number of adult patients with CHD, now referred to as ACHD, is increasing with improved surgical and treatment interventions. However the mechanisms whereby ACHD predisposes patients to heart dysfunction are still unclear. ACHD is strongly associated with metabolic syndrome, but how ACHD interacts with poor modern lifestyle choices and other comorbidities, such as hypertension, obesity, and diabetes, is mostly unknown...
November 15, 2018: Molecular Metabolism
Francesca Maradonna, Oliana Carnevali
Exposure to potential Endocrine Disrupting Chemicals (EDCs) pose a documented risk to both wildlife and human health. Many studies so far described declining sperm counts, genital malformations, early puberty onset, highlighting the negative impact on reproduction caused by the exposure to many anthropogenic chemicals. In the last years, increasing evidence suggested that these compounds, other than altering reproduction, affect metabolism and induce the onset of obesity and metabolic disorders. According to the "environmental obesogens" hypothesis, evidence exists that exposure to potential EDCs during critical periods when adipocytes are differentiating, and organs are developing, can induce diseases that manifest later in the life...
2018: Frontiers in Endocrinology
Mario Inacio, Rob Creath, Mark W Rogers
Aging brings about challenges in the ability to recover balance through protective stepping, especially in the lateral direction. Previous work has suggested that lateral protective stepping during weight transfer may be affected by impaired muscle composition and performance of the hip abductors (AB) in older adults. Hence, this study investigated the influence of hip abductor-adductor (AB-AD) neuromuscular performance on the weight transfer phase of lateral protective stepping in younger and older adults...
November 9, 2018: Journal of Biomechanics
Christina I Günter, Hans-Günther Machens, Felicitas P Ilg, Alexander Hapfelmeier, Wolfgang Jelkmann, Silvia Egert-Schwender, Shibashish Giri, Augustinus Bader
In adult's burn injuries belong to the top 15 causes of injury. Annually more than a million patients receive specialized treatment. Improving burned patients' outcomes is still a challenge. Effects of erythropoietin (EPO) are reported to be pro-angiogenic, pro-regenerative, anti-inflammatory, immunomodulatory and hypoxia/ischemia protective. Study objectives were to demonstrate cytoprotective and regenerative effects of EPO in burned patients in terms of improved wound healing, reduced morbidity and mortality...
2018: Frontiers in Pharmacology
Takashi Shimizu, Akie Kikuchi-Taura, Soichiro Tsuji, Masato Matsushita, Shiro Ohshima, Yukihiko Saeki
OBJECTIVES: In this study, we investigated whether monocyte CD64 (mCD64) expression is correlated with disease activity in patients with adult-onset Still disease (AOSD) and whether it could be used to distinguish between active and inactive disease states. METHODS: We reviewed a series of 10 patients with a definite diagnosis of AOSD, recruited from January 2013 to December 2016. We used flow cytometry to quantitatively measure mCD64 expression levels in patients presenting with active and inactive disease states and statistically analyzed the corresponding changes...
November 7, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Khalid Mahmood, Samar Mahmood, Shayan Marsia
Adult onset Still's disease (AOSD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, erythematous rash, and other systemic presentations. We report a case of a 21-year-old male who presented with high spiking fever, dry cough, generalized body ache, arthralgia, and an erythematous rash. He was eventually diagnosed to have AOSD based on the Yamaguchi criteria, after a month of visiting three different healthcare facilities and receiving two misdiagnoses and treatment regimes not specific to his diagnosis...
September 4, 2018: Curēus
Jianjun Qiao, Sha Zhou, Sheng Li, Ruzeng Xue, Yunlei Pan, Shunli Tang, Qingmiao Sun, Yanyan Wu, Su Wang, Yinhua Wu, Juan Bai, Tingting Qu, Xiuming Zhang, Chuanyin Sun, Hong Fang
AIMS: Persistent pruritic eruptions (PPEs), presenting with dyskeratotic keratinocytes histologically, are common in patients with adult-onset Still's disease (AOSD). The lesions may be histologically similar to other entities that present with dyskeratosis. METHODS AND RESULTS: To investigate whether histopathological findings can be used to discriminate among PPEs and other entities presenting with dyskeratotic keratinocytes, cutaneous histopathological changes of PPEs associated with AOSD (n=26) were compared with those of systemic lupus erythematosus (SLE) (n=16), dermatomyositis (n=19), and drug eruption (n=16)...
November 8, 2018: Histopathology
Young Ho Lee
No abstract text is available yet for this article.
November 2, 2018: Annals of the Rheumatic Diseases
Daan R van Renswoude, Linda van den Berg, Maartje E J Raijmakers, Ingmar Visser
This study examines how salience and a center bias drive infants' first fixation while looking at complex scenes. Adults are known to have a strong center bias, their first point of gaze is nearly always in the center of the scene. The center bias is likely to be a strategic bias, as looking towards the center minimizes the distance to other parts of the scene and important objects are often located at the center. In an experimental design varying salience regions of scenes and start positions we examined infants' (N = 48, Age = 5-20-month-olds) first fixation after scene onset...
November 12, 2018: Vision Research
Cinzia Magagnotti, Gianpaolo Zerbini, Isabella Fermo, Rose Mary Carletti, Riccardo Bonfanti, Fabiana Vallone, Annapaola Andolfo
Despite research progresses, the chance to accurately predict the risk for diabetic nephropathy (DN) is still poor. So far, the first evidence of DN is micro-albuminuria, which is detected only 10-20 years after the onset of diabetes. Our goal is to develop new predictive tools of nephropathy starting from urine, which can be easily obtained using noninvasive procedures and it is directly related to kidney. Since it is reasonable to suppose that, in predisposed patients, the mechanisms leading to nephropathy start acting since the diabetes onset, urine from children with recent diagnosis of type 1 diabetes was subjected to proteomic analysis in comparison to age-matched controls...
October 24, 2018: Journal of Proteomics
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