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Still onset adult disease

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https://www.readbyqxmd.com/read/29241877/adult-onset-still-s-disease
#1
REVIEW
Javier Narváez
Adult onset Still's disease is a rare systemic condition at the crossroads between auto-inflammatory syndromes and autoimmune diseases, with considerable heterogeneity in terms of clinical presentation, evolution and severity. This article reviews the main advances and lesser known aspects of this entity related to its clinical spectrum (atypical cutaneous lesions, unusual manifestations, macrophage activation syndrome, disease phenotypes), the emerging controversy around its association with delayed malignancy, the search for new biomarkers for its diagnosis, evaluation of prognosis (clinical factors, prognostic indexes and biomarkers to identify patients at risk of severe organ failure or life-threatening complications), and the determinants in the choice of biological treatment...
December 11, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/29239928/aspergillus-pneumonia-in-a-patient-with-adult-onset-still-disease-successfully-treated-with-anakinra
#2
Emre Bilgin, Abdulsamet Erden, Levent Kilic, Alper Sari, Berkan Armagan, Umut Kalyoncu, Ömer Karadag
No abstract text is available yet for this article.
December 12, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29239927/an-adult-patient-with-a-novel-mutation-in-nlrp3-gene-associated-with-cryopyrin-associated-periodic-syndrome-mimicking-adult-onset-still-disease
#3
Vanessa Ocampo, Maria-Clara Ortiz-Sierra, Andrés F Echeverri, Iván Posso-Osorio, Juan-Pablo Suso, Gabriel J Tobón
No abstract text is available yet for this article.
December 12, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29230477/interleukin-1-inhibitors-and-dacryoadenitis-in-adult-onset-still-disease
#4
Paul Breillat, Maylis Tourte, Pierre Romero, Gilles Hayem, Ilaria Padovano, Félicie Costantino, Maxime Breban
No abstract text is available yet for this article.
December 12, 2017: Annals of Internal Medicine
https://www.readbyqxmd.com/read/29225867/visceral-leishmaniasis-triggering-an-adult-onset-still-s-disease-a-unique-case
#5
Silvia Spoto, Sebastiano Costantino, Emanuele Valeriani, Marta Fogolari, Eleonora Cella, Giordano Dicuonzo, Massimo Ciccozzi, Silvia Angeletti
Adult-onset Still's disease (AOSD) due to visceral leishmaniasis (VL) has not been previously reported. This case report analyzes a single episode of AOSD probably due to a visceral leishmaniasis successfully treated with pentamidine isethionate and prednisone.
December 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29224679/a-review-on-sle-and-malignancy
#6
REVIEW
May Y Choi, Kelsey Flood, Sasha Bernatsky, Rosalind Ramsey-Goldman, Ann E Clarke
Systemic lupus erythematosus (SLE) is a chronic, systemic autoimmune disease characterized by autoantibody production, complement activation, and immune complex deposition. It predominantly affects young and middle-aged women. While improvements in the diagnosis and treatment of SLE have altered prognosis, morbidity and mortality rates remain higher than the general population. In addition to renal injury, cardiovascular disease, and infection, malignancy is known to be a significant cause of death in this population...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29221343/adult-onset-langerhans-cell-histiocytosis-of-the-sternum
#7
Yi-Jhih Huang, Kuan-Hsun Lin, Tai-Kuang Chao, Tsai-Wang Huang, Hsu-Kai Huang
Langerhans cell histiocytosis (LCH) is a rare disease with uncertain etiology that is more prevalent in children. LCH typically invades skeletal systems, but in rare cases, it has been reported in the ribs or sternum. Optimal treatment choices for single-site, skeletal LCH are still undefined. We report a case of adult-onset LCH of the sternum. The range of surrounding soft tissue invasion was confirmed by three-dimensional fusion and reconstruction of chest computed tomography and magnetic resonance images...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29211757/distinct-fecal-and-oral-microbiota-composition-in-human-type-1-diabetes-an-observational-study
#8
Pieter F de Groot, Clara Belzer, Ömrüm Aydin, Evgeni Levin, Johannes H Levels, Steven Aalvink, Fransje Boot, Frits Holleman, Daniël H van Raalte, Torsten P Scheithauer, Suat Simsek, Frank G Schaap, Steven W M Olde Damink, Bart O Roep, Joost B Hoekstra, Willem M de Vos, Max Nieuwdorp
OBJECTIVE: Environmental factors driving the development of type 1 diabetes (T1D) are still largely unknown. Both animal and human studies have shown an association between altered fecal microbiota composition, impaired production of short-chain fatty acids (SCFA) and T1D onset. However, observational evidence on SCFA and fecal and oral microbiota in adults with longstanding T1D vs healthy controls (HC) is lacking. RESEARCH DESIGN AND METHODS: We included 53 T1D patients without complications or medication and 50 HC matched for age, sex and BMI...
2017: PloS One
https://www.readbyqxmd.com/read/29208852/extremely-high-serum-ferritin-an-instrumental-marker-of-masquerading-adult-onset-still-s-disease-with-hemophagocytic-syndrome
#9
Shun Yamashita, Naoko E Furukawa, Tomoyo Matsunaga, Yuka Hirakawa, Masaki Tago, Shu-Ichi Yamashita
BACKGROUND Adult-onset Still's disease (AOSD) is a rare multi-systemic inflammatory disorder of unknown etiology characterized by spiking fever, characteristic rash, and arthritis. It often associates with high serum ferritin levels. CASE REPORT An 88-year-old woman had fever of over 39°C without response to extended-spectrum antibiotics for 6 days. She had non-specific erythema with infiltration on her trunk. She had leukocytosis with neutrophilia of 80%, mild hepatic dysfunction, normal level of rheumatoid factor and antinuclear antibody, thrombocytopenia, elevated d-dimer and soluble interleukin2 receptor, extremely high serum ferritin (78 662 ng/mL), and splenomegaly...
December 6, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29188860/a-keto-mediet-approach-with-coconut-substitution-and-exercise-may-delay-the-onset-of-alzheimer-s-disease-among-middle-aged
#10
B C Perng, M Chen, J C Perng, P Jambazian
BACKGROUND: Coconut oil has been widely used to improve health because there is much information available by word of mouth, in books, and on the internet. However, researchers still continue to search for the best diets to improve the quality of life, especially for people with cognitive decline. OBJECTIVES: The aim of this review is to develop a novel dietary approach, the Keto-Mediet, which may help prevent the onset of Alzheimer's disease. METHODS: Evidence gained through literature review from 1982 to 2015 on gene-by-diet interaction and lipid and glucose metabolism in the brains of Alzheimer's patients is converted into the new Keto-Mediet approach...
2017: Journal of Prevention of Alzheimer's Disease
https://www.readbyqxmd.com/read/29176023/the-role-of-enterovirus-infections-in-type-1-diabetes-in-tunisia
#11
Imen Boussaid, Asma Boumiza, Ramzi Zemni, Elyes Chabchoub, Latifa Gueddah, Ines Slim, Foued Ben Hadj Slama
BACKGROUND: Enteroviral infections have long been suspected in having a role in β cell destruction and therefore leading to the onset of clinical type 1 diabetes (T1D). The frequency of enterovirus (EV)-related T1D in North Africa is still unknown. The aim of the present study was to investigate the relationship between infection with EV and T1D in Tunisia. METHODS: A total of 95 T1D patients (41 children and 54 adults) and 141 healthy control subjects (57 children and 84 adults) were tested for the presence of EV-RNA by a highly sensitive nested reverse transcription-polymerase chain reaction (RT-PCR) method...
November 25, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29168664/pulmonary-arterial-hypertension-and-acute-respiratory-distress-syndrome-in-a-patient-with-adult-onset-stills-disease
#12
Grant H Lowther, Jason Chertoff, Jessica Cope, Hassan Alnuaimat, Ali Ataya
Adult-onset Still's disease (AOSD) is an inflammatory disorder characterized by recurrent fevers, arthralgia, leukocytosis, and a salmon-colored rash. Diagnosis is made based on the Yamaguchi criteria. Various cardiac and pulmonary manifestations have been described in association with AOSD, including acute respiratory distress syndrome (ARDS) and pulmonary arterial hypertension (PAH). We describe the first case of both PAH and ARDS in a patient with AOSD who, despite aggressive therapy, declined rapidly and ultimately died...
October 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/29164007/adult-onset-still-s-disease-is-this-truly-a-diagnosis-of-exclusion
#13
Caleb W Anderson, Phalgoon A Shah, Jefferson R Roberts
Adult-onset Still's Disease is a rare, idiopathic, inflammatory disorder characterized by arthralgia, evanescent, salmon-colored rash, and daily fevers as well as lymphadenopathy, pharyngitis, splenomegaly, myalgias, and serositis. The inciting etiology of this syndrome is unknown, though it has been hypothesized that infection triggers an autoimmune response. The Yamaguchi Criteria, the most sensitive and widely used diagnostic criteria, requires both a minimum set of criteria to be met as well other potential etiologies to be excluded...
November 2017: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
https://www.readbyqxmd.com/read/29163345/fluid-distribution-pattern-in-adult-onset-congenital-idiopathic-and-secondary-normal-pressure-hydrocephalus-implications-for-clinical-care
#14
Shigeki Yamada, Masatsune Ishikawa, Kazuo Yamamoto
Objective: In spite of growing evidence of idiopathic normal-pressure hydrocephalus (NPH), a viewpoint about clinical care for idiopathic NPH is still controversial. A continuous divergence of viewpoints might be due to confusing classifications of idiopathic and adult-onset congenital NPH. To elucidate the classification of NPH, we propose that adult-onset congenital NPH should be explicitly distinguished from idiopathic and secondary NPH. Methods: On the basis of conventional CT scan or MRI, idiopathic NPH was defined as narrow sulci at the high convexity in concurrent with enlargement of the ventricles, basal cistern and Sylvian fissure, whereas adult-onset congenital NPH was defined as huge ventricles without high-convexity tightness...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29159471/evidence-for-genetic-overlap-between-adult-onset-still-s-disease-and-hereditary-periodic-fever-syndromes
#15
R Sighart, J Rech, A Hueber, N Blank, S Löhr, A Reis, H Sticht, U Hüffmeier
OBJECTIVE: Adult onset Still's disease (AOSD) is a severe, autoimmune disease that can be challenging to treat with conventional therapeutics and biologicals in a considerable number of cases. Therefore, there is a high need to understand its pathogenesis better. As major clinical symptoms overlap between AOSD and hereditary periodic fever syndromes (HPFS), we analysed four known HPFS genes in AOSD. METHODS: We performed Sanger sequencing and quantitative analysis of all coding regions of MEFV, TNFRSF1A, MVK and NLRP3 in 40 AOSD patients...
November 20, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29153996/clinical-presentation-of-anti-n-methyl-d-aspartate-receptor-and-anti-voltage-gated-potassium-channel-complex-antibodies-in-children-a-series-of-24-cases
#16
Bahadir Konuskan, Mirac Yildirim, Haluk Topaloglu, Ilknur Erol, Ulkuhan Oztoprak, Huseyin Tan, Rahsan Gocmen, Banu Anlar
OBJECTIVE: The symptomatology and paraclinical findings of antibody-mediated encephalitis, a relatively novel disorder, are still being characterized in adults and children. A high index of suspicion is needed in order to identify these cases among children presenting with various neurological symptoms. The aim of this study is to examine the clinical, demographic and laboratory findings and outcome of children with anti-NMDAR and anti-VGKC encephalitis for any typical or distinctive features...
November 7, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29151803/cognitive-consequences-of-trilingualism
#17
Scott R Schroeder, Viorica Marian
Aims and Objectives: The objectives of the present research were to examine the cognitive consequences of trilingualism and explain them relative to the cognitive consequences of bilingualism. Approach: A comparison of cognitive abilities in trilinguals and bilinguals was conducted. In addition, we proposed a cognitive plasticity framework to account for cognitive differences and similarities between trilinguals and bilinguals. Data and Analysis: Three aspects of cognition were analyzed: (1) cognitive reserve in older adults, as measured by age of onset of Alzheimer's disease and mild cognitive impairment; (2) inhibitory control in children and younger adults, as measured by response times on behavioral Simon and flanker tasks; and (3) memory generalization in infants and toddlers, as measured by accuracy on behavioral deferred imitation tasks...
2017: International Journal of Bilingualism: Cross-disciplinary, Cross-linguistic Studies of Language Behavior
https://www.readbyqxmd.com/read/29148405/autophagy-inhibition-in-adult-onset-still-s-disease-still-more-space-for-hydroxychloroquine
#18
Charalampos Papagoras, Akrivi Chrysanthopoulou, Alexandros Mitsios, Athanasios Arampatzioglou, Konstantinos Ritis, Panagiotis Skendros
No abstract text is available yet for this article.
November 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29147588/diagnostic-challenge-a-report-of-two-adult-onset-still-s-disease-cases
#19
Sakunee Niranvichaiya, Daranporn Triwongwaranat
This study reports two adult-onset Still's disease (AOSD) cases that met both Yamaguchi's and Fautrel's criteria and that presented with notable clinical manifestations. One case presented with atypical dermographism-like rash with an extremely high ferritin level. The other case presented with typical salmon-pink maculopapular rash but had atypical positive rheumatoid factor. This suggests that although negative rheumatoid factor is one of the criteria used for the diagnosis of AOSD, a positive rheumatoid factor result does not exclude AOSD...
2017: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/29132389/advances-challenges-and-future-directions-for-stem-cell-therapy-in-amyotrophic-lateral-sclerosis
#20
REVIEW
Yuri Ciervo, Ke Ning, Xu Jun, Pamela J Shaw, Richard J Mead
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative condition where loss of motor neurons within the brain and spinal cord leads to muscle atrophy, weakness, paralysis and ultimately death within 3-5 years from onset of symptoms. The specific molecular mechanisms underlying the disease pathology are not fully understood and neuroprotective treatment options are minimally effective. In recent years, stem cell transplantation as a new therapy for ALS patients has been extensively investigated, becoming an intense and debated field of study...
November 13, 2017: Molecular Neurodegeneration
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