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Still onset adult disease

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https://www.readbyqxmd.com/read/29023182/potential-clinical-utility-of-multiple-system-atrophy-biomarkers
#1
Kurt A Jellinger
Multiple system atrophy (MSA), an adult-onset, fatal disorder of uncertain aetiology, characterized by parkinsonism, cerebellar, autonomic and motor dysfunctions, is an α-synucleinopathy with glioneuronal degeneration involving multiple parts of the nervous system. The clinical variants correlate with the morphological phenotypes of striatonigral degeneration (MSA-P), olivoponto-cerebellar atrophy (MSA-C), and mixed type MSA. Neuropathological hallmark is the deposition of aberrant α-synuclein in glia and neurons forming cytoplasmic inclusions that cause cell dysfunction/demise...
October 12, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/29020327/cognitive-changes-in-prevalent-and-incident-cardiovascular-disease-a-12-year-follow-up-in-the-maastricht-aging-study-maas
#2
Syenna H J Schievink, Martin P J van Boxtel, Kay Deckers, Robert J van Oostenbrugge, Frans R J Verhey, Sebastian Köhler
Aims: Cardiovascular disease (CVD) has been suggested to accelerate cognitive decline and to be a risk factor for dementia, but still little is known about the cognitive course after a first cardiovascular event. Therefore, the present study aims to investigate the cognitive trajectories in both prevalent and incident CVD over a 12-year time period in the general population. Methods and results: Cognitively healthy participants (age 24-82 years, n = 1823) of a prospective cohort study were serially assessed at baseline, 6 and 12 years...
July 22, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28984582/multiple-system-atrophy-an%C3%A2-oligodendroglioneural-synucleinopathy
#3
Kurt A Jellinger
Multiple system atrophy (MSA) is an orphan, fatal, adult-onset neurodegenerative disorder of uncertain etiology that is clinically characterized by various combinations of parkinsonism, cerebellar, autonomic, and motor dysfunction. MSA is an α-synucleinopathy with specific glioneuronal degeneration involving striatonigral, olivopontocerebellar, and autonomic nervous systems but also other parts of the central and peripheral nervous systems. The major clinical variants correlate with the morphologic phenotypes of striatonigral degeneration (MSA-P) and olivopontocerebellar atrophy (MSA-C)...
September 26, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28928271/the-value-of-18-f-fdg-pet-ct-in-identifying-the-cause-of-fever-of-unknown-origin-fuo-and-inflammation-of-unknown-origin-iuo-data-from-a-prospective-study
#4
Verena Schönau, Kristin Vogel, Matthias Englbrecht, Jochen Wacker, Daniela Schmidt, Bernhard Manger, Torsten Kuwert, Georg Schett
BACKGROUND: Fever of unknown origin (FUO) and inflammation of unknown origin (IUO) are diagnostically challenging conditions. Diagnosis of underlying disease may be improved by (18)F-fluorodesoxyglucose positron emission tomography ((18)F-FDG-PET). METHODS: Prospective study to test diagnostic utility of (18)F-FDG-PET/CT in a large cohort of patients with FUO or IUO and to define parameters that increase the likelihood of diagnostic (18)F-FDG-PET/CT. Patients with FUO or IUO received (18)F-FDG-PET/CT scanning in addition to standard diagnostic work-up...
September 19, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28926333/pneumonitis-in-adult-onset-still-s-disease-uncommon-or-under-diagnosed
#5
Silvia Fernandes, Margarida Almeida, José Alberto Pereira da Silva, José Carlos Romeu
The adult onset Still's Disease is an uncommon entity characterized by multiple clinical manifestations. Pneumonitis, less often considered, deserves particular emphasis given the need for differential diagnosis and because it can progress to severe respiratory failure. With the aim to highlight the pulmonary parenchyma involvement in patients with adult onset Still's Disease, we present a case report which progresses with pneumonitis.
August 31, 2017: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/28899403/effects-of-hla-drb1-alleles-on-susceptibility-and-clinical-manifestations-in-japanese-patients-with-adult-onset-still-s-disease
#6
Tomoyuki Asano, Hiroshi Furukawa, Shuzo Sato, Makiko Yashiro, Hiroko Kobayashi, Hiroshi Watanabe, Eiji Suzuki, Tomoyuki Ito, Yoshifumi Ubara, Daisuke Kobayashi, Nozomi Iwanaga, Yasumori Izumi, Keita Fujikawa, Satoshi Yamasaki, Tadashi Nakamura, Tomohiro Koga, Toshimasa Shimizu, Masataka Umeda, Fumiaki Nonaka, Michio Yasunami, Yukitaka Ueki, Katsumi Eguchi, Naoyuki Tsuchiya, Shigeto Tohma, Koh-Ichiro Yoshiura, Hiromasa Ohira, Atsushi Kawakami, Kiyoshi Migita
BACKGROUND: HLA-DRB1 alleles are major determinants of genetic predisposition to rheumatic diseases. We assessed whether DRB1 alleles are associated with susceptibility to particular clinical features of adult onset Still's disease (AOSD) in a Japanese population by determining the DRB1 allele distributions. METHODS: DRB1 genotyping of 96 patients with AOSD and 1,026 healthy controls was performed. Genomic DNA samples from the AOSD patients were also genotyped for MEFV exons 1, 2, 3, and 10 by direct sequencing...
September 12, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28870100/biologic-drugs-in-adult-onset-still-s-disease-a-systematic-review-and-meta-analysis-of-observational-studies
#7
Piero Ruscitti, Francesco Ursini, Paola Cipriani, Giovambattista De Sarro, Roberto Giacomelli
BACKGROUND: Biological drugs, mainly interleukin (IL)-1 and IL-6 antagonists, but also tumor necrosis factor (TNF) inhibitors, have been used in the treatment of adult onset Still's disease patients (AOSD). METHODS: We summarised the available evidence for the effectiveness of biologic drugs in AOSD. A systematic review of the literature was performed in order to identify all the available data concerning the effectiveness of biologic drugs in AOSD. The proportion of patients achieving complete remission or any clinical response was calculated...
November 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28867213/transgenerational-pancreatic-impairment-with-igf2-h19-epigenetic-alteration-induced-by-p-p-dde-exposure-in-early-life
#8
Yang Song, Lei Yang
The hypothesis of fetal origins indicates that exposures in early development could induce epigenetic modifications in the male germ-line, affecting the susceptibility of adult-onset disease for generations. p,p'-DDE, the primary metabolite of persistent organochlorine pesticide DDT, is highly correlated with impaired glucose tolerance (IGT) and a strong contributing factor to type 2 diabetes. In our previous study, ancestral p,p'-DDE exposure could induce transgenerational impaired male fertility with sperm Igf2 hypomethylation...
October 5, 2017: Toxicology Letters
https://www.readbyqxmd.com/read/28838228/red-blood-cell-distribution-width-is-useful-in-discriminating-adult-onset-still-s-disease-and-sepsis-within-24-hours-after-hospitalization
#9
Hee-Jin Park, Jungsik Song, Yong-Beom Park, Soo-Kon Lee, Sang-Won Lee
Background/Aims: Red blood cell distribution width (RDW) is a value representing the heterogeneity in the size of red blood cell, and it is usually used in distinguishing types of anaemia. Recently, it was reported that it could reflect the burden of inflammation in diverse diseases and their prognosis. Hence, in this study, we investigated whether RDW may contribute to discriminating adult onset Still's disease (AOSD) from sepsis in serious febrile patients within 24 hours after hospitalization...
August 25, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28809934/long-term-exposure-to-air-pollution-and-the-incidence-of-parkinson-s-disease-a-nested-case-control-study
#10
Chiu-Ying Chen, Hui-Jung Hung, Kuang-Hsi Chang, Chung Y Hsu, Chih-Hsin Muo, Chon-Haw Tsai, Trong-Neng Wu
BACKGROUND: Previous studies revealed that chronic exposure to air pollution can significantly increase the risk of the development of Parkinson's disease (PD), but this relationship is inconclusive as large-scale prospective studies are limited and the results are inconsistent. Therefore, the purpose of this study was to ascertain the adverse health effects of air pollution exposure in a nationwide population using a longitudinal approach. MATERIALS AND METHODS: We conducted a nested case-control study using the National Health Insurance Research Dataset (NHIRD), which consisted of 1,000,000 beneficiaries in the National Health Insurance Program (NHI) in the year 2000 and their medical records from 1995 to 2013 and using public data on air pollution concentrations from monitoring stations across Taiwan released from the Environmental Protection Administration to identify people with ages ≥ 40 years living in areas with monitoring stations during 1995-1999 as study subjects...
2017: PloS One
https://www.readbyqxmd.com/read/28782197/early-life-adversity-epigenetics-and-visceral-hypersensitivity
#11
REVIEW
S Liu, S I Hagiwara, A Bhargava
Abdominal pain is associated with many gastrointestinal dysfunctions, such as irritable bowel syndrome (IBS), functional dyspepsia, and inflammatory bowel disease (IBD). Visceral hypersensitivity is a key reason for development of abdominal pain that presents in these gastrointestinal disorders/diseases. The pathogenesis of visceral hypersensitivity is complex and still far from being fully understood. In animal studies, visceral hypersensitivity has been linked to several early-life adverse (ELA) events. In humans, IBD, functional dyspepsia, and IBS can have adult onset, though the adverse events that lead to visceral hypersensitivity are largely uncharacterized...
September 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/28781105/adult-s-onset-still-disease-occurring-during-pregnancy-case-report-and-literature-review
#12
Léo Plaçais, Arsène Mekinian, Marie Bornes, Armelle Poujol-Robert, Naiké Bigé, Eric Maury, Olivier Fain
INTRODUCTION: Adult onset Still's disease is a rare affection classified among non-hereditary autoinflammatory diseases. We here report a case of AOSD revealed during pregnancy with a life-threatening presentation along with a review of 19 cases from literature. CASE: A 38-years old woman was treated in our department for diffuse systemic sclerosis and associated Sjögren syndrome. She was pregnant and presented with acute fever and arthralgias. Laboratory data revealed mild liver cytolysis but a large screening for infectious and auto-immune diseases was negative and hepato-biliar imaging was normal...
July 10, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28772271/remission-phase-in-paediatric-type-1-diabetes-new-understanding-and-emerging-biomarkers
#13
Mireia Fonolleda, Marta Murillo, Federico Vázquez, Joan Bel, Marta Vives-Pi
Type 1 diabetes (T1D) is a metabolic disease of unknown aetiology that results from the autoimmune destruction of the β-cells. Clinical onset with classic hyperglycaemic symptoms occurs much more frequently in children and young adults, when less than 30% of β-cells remain. Exogenous insulin administration is the only treatment for patients. However, due to glucose dysregulation, severe complications develop gradually. Recently, an increase in T1D incidence has been reported worldwide, especially in children...
August 3, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28765926/imaging-characteristics-of-adult-onset-still-s-disease-demonstrated-with-18f-fdg-pet-ct
#14
Lei Jiang, Yan Xiu, Taoying Gu, Caihong Dong, Bing Wu, Hongcheng Shi
The diagnosis of adult onset Still's disease (AOSD) is non‑specific, and requires the exclusion of other diseases including infectious, inflammatory and malignant diseases. The current study aimed to summarize the imaging characteristics of fluorodeoxyglucose (18F‑FDG) positron emission tomography (PET)/computerized tomography (CT) in patients with AOSD. The 18F‑FDG PET/CT characteristic observations of 32 patients with definite AOSD were retrospectively reviewed based on visual interpretation and the semi‑quantitative index of standard uptake value of maximum (SUVmax)...
September 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28760536/validation-of-the-fautrel-classification-criteria-for-adult-onset-still-s-disease
#15
Delphine Lebrun, Stéphanie Mestrallet, Monique Dehoux, Jean Louis Golmard, Benjamin Granger, Sophie Georgin-Lavialle, Laurent Arnaud, Gilles Grateau, Jacques Pouchot, Bruno Fautrel
OBJECTIVES: To validate the Fautrel classification criteria for adult-onset Still's disease (AOSD) and to compare the discriminative performance to that of the Yamaguchi criteria. METHODS: We retrospectively reviewed the medical charts of 426 patients who had serum ferritin level and percentage glycosylated ferritin assayed at the biochemistry laboratory of Bichat Hospital. Medical data were extracted by use of a standardized form. All clinical, biological, and imaging features were collected, as well, evidence favoring an alternative diagnosis, specifically symptoms suggestive of other immune-mediated inflammatory diseases (IMID) or active infections...
July 12, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28758587/atypical-form-of-adult-onset-still-s-disease-with-distal-interphalangeal-joints-involvement
#16
Safaa Belghali, Nejla El Amri, Khadija Baccouche, Sadok Laataoui, Monia Bouzaoueche, Hela Zeglaoui, Elyes Bouajina
Distal interphalangeal (DIP) joints involvement in the adult-onset Still's disease (ASD) has been described in some publications but is rarely severe. We report severe DIP joints damages in a young patient with ASD. A 22 years old patient presented to our department complaining of inflammatory joints pain associated with prolonged fever and cutaneous rash. Physical examination identified polyarthritis and hepatosplenomegaly but no lymphadenopathies. After an extensive screening for neoplastic, infectious or hematologic diseases, the patient was finally diagnosed with ASD...
July 28, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28757235/adult-onset-still-s-disease-the-evidence-that-anti-interleukin-1-treatment-is-effective-and-well-tolerated-a-comprehensive-literature-review
#17
REVIEW
Guido Junge, June Mason, Eugen Feist
The literature contains many reports of the use of commercially available anti-IL-1 agents (anakinra/Kineret(®), canakinumab/Ilaris(®), or rilonacept/Arcalyst(®)) in treatment-resistant adult-onset Still's disease (AOSD). These have been widely summarized in many review articles, but a full account of all reports with each of the agents used is not available. This literature review includes all reports of treatment outcomes in patients treated for AOSD with any commercially available anti-IL-1 agent (excluding cases of unconfirmed or atypical AOSD or treatments only for rare AOSD complications)...
June 23, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28755070/-still-s-disease-in-children-and-adults
#18
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...
July 28, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28725546/pulmonary-involvement-in-adult-still-s-disease-case-report-and-brief-review-of-literature
#19
Aldo Guerrieri, Giulia Angeletti, Massimiliano Mazzolini, Ilaria Bassi, Stefano Nava
BACKGROUND: Adult onset Still disease (AOSD) is a rare condition characterized by elevated fever along with arthritic symptoms, elevated polymorphonuclear neutrophil count, evanescent rash, and hyperferritinemia. Diagnosis can be made only after have ruled out more frequent conditions, and Yamagouchi or Fautrel criteria should be applied. Parenchimal lung involvement (PLI) is present in less than 5% of AOSD cases and ranges from aspecific reticular interstitial opacities to life threatening conditions, such as acute respiratory distress syndrome (ARDS)...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28724438/synergy-between-dinotefuran-and-fipronil-against-the-cat-flea-ctenocephalides-felis-improved-onset-of-action-and-residual-speed-of-kill-in-adult-cats
#20
Romain Delcombel, Hamadi Karembe, Bakela Nare, Audrey Burton, Julian Liebenberg, Josephus Fourie, Marie Varloud
BACKGROUND: The cat flea, Ctenocephalides felis felis (C. felis), is a cosmopolitan hematophagous ectoparasite, and is considered to be the most prevalent flea species in both Europe and the USA. Clinical signs frequently associated with flea bites include pruritus, dermatitis and in severe cases even pyodermatitis and alopecia. Ctenocephalides felis is also a vector for several pathogens and is an intermediate host for the cestode Dipylidium caninum. Treatment of cats with a fast-acting pulicide, that is persistently effective in protecting the animal against re-infestation, is therefore imperative to their health...
July 19, 2017: Parasites & Vectors
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