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adult onset still disease

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https://www.readbyqxmd.com/read/29447637/crohn-disease-associated-neutrophilic-urticarial-dermatosis-report-and-literature-review-of-neutrophilic-urticarial-dermatosis
#1
Zhe Hou, Brian R Hinds, Philip R Cohen
Neutrophilic urticarial dermatosis (NUD) is a useful diagnostic term for urticarial lesions that are less pruritic and more painful than conventional urticaria. The histopathologic features include neutrophilic infiltrates in the interstitial dermis with a higher density than idiopathic urticaria. NUD has been associated with several systemic conditions, which are predominantly autoimmune and autoinflammatory in nature. A woman with Crohn disease who developed NUD is described. Literature reports of other conditions in which neutrophilic urticarial dermatosis have been observed are also reviewed and summarized...
November 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29441182/pediatric-cardiac-anamnesis-prevention-of-additional-diagnostic-tests
#2
REVIEW
Izet Masic, Zijo Begic, Nabil Naser, Edin Begic
Pediatrics is defined as the science of a healthy and sick child from birth to end of adolescence. Diseases of the cardiovascular system are the leading causes of mortality in adults, with frequent onset in childhood. The cardiologic examination starts with anamnesis in a pleasant atmosphere, refined space, enough time and patience, detailed measurements, and preferably a noncrying child. Anamnesis, regardless of the development of diagnostic procedures, still constitutes the basis of every clinical examination...
2018: International Journal of Preventive Medicine
https://www.readbyqxmd.com/read/29435558/association-between-amyloid-and-tau-accumulation-in-young-adults-with-autosomal-dominant-alzheimer-disease
#3
Yakeel T Quiroz, Reisa A Sperling, Daniel J Norton, Ana Baena, Joseph F Arboleda-Velasquez, Danielle Cosio, Aaron Schultz, Molly Lapoint, Edmarie Guzman-Velez, John B Miller, Leo A Kim, Kewei Chen, Pierre N Tariot, Francisco Lopera, Eric M Reiman, Keith A Johnson
Importance: It is critically important to improve our ability to diagnose and track Alzheimer disease (AD) as early as possible. Individuals with autosomal dominant forms of AD can provide clues as to which and when biological changes are reliably present prior to the onset of clinical symptoms. Objective: To characterize the associations between amyloid and tau deposits in the brains of cognitively unimpaired and impaired carriers of presenilin 1 (PSEN1) E280A mutation...
February 12, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29420734/convergent-pathways-of-the-hyperferritinemic-syndromes
#4
Grant S Schulert, Scott W Canna
Hyperferritinemia and pronounced hemophagocytosis help distinguish a subset of patients with a particularly inflammatory and deadly systemic inflammatory response syndrome. Two clinically similar disorders typify these hyperferritinemic syndromes: hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS). HLH is canonically associated with a complete disturbance of perforin/granzyme-mediated cytotoxicity, whereas MAS occurs in the context of the related rheumatic diseases systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still's disease (AOSD), with associated IL-1 family cytokine activation...
February 6, 2018: International Immunology
https://www.readbyqxmd.com/read/29416600/impaired-autophagic-flux-and-its-related-inflammation-in-patients-with-adult-onset-still-s-disease
#5
Chia-Wei Hsieh, Chun-Yu Chang, Yi-Ming Chen, Hsin-Hua Chen, Wei-Ting Hung, Ning-Rong Gung, Shiow-Jiuan Wey, Der-Yuan Chen
The pathogenic role of autophagic immune regulation in adult-onset Still's disease (AOSD) is unclear. We investigated the relative levels of autophagy in AOSD patients and healthy controls, its association with disease activity or course, and the change in autophagy after 6 months of therapy. Autophagosome levels were determined from the mean fluorescence intensity of autophagosomotropic dye incorporated into circulating immune cells. The fluorescent signal from lymphocytes, monocytes, and granulocytes from AOSD patients was greater than from controls...
January 2, 2018: Oncotarget
https://www.readbyqxmd.com/read/29416343/biological-therapy-of-traditional-therapy-resistant-adult-onset-still-s-disease-an-evidence-based-review
#6
REVIEW
Sha Zhou, Jianjun Qiao, Juan Bai, Yinhua Wu, Hong Fang
Background: Biotherapy is becoming increasingly important in the treatment of adult-onset Still's disease (AOSD). The aim of our study was to evaluate the efficacy and safety of biological therapy for AOSD resistant to traditional therapy. Patients and methods: Database of Library of Congress, the PubMed, and Web of Science Core Collection were used to retrieve relevant articles published in English language until March 2017. Only studies published in English language were included, and the additional references quoted in these articles were also checked...
2018: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/29390286/purulent-constrictive-pericarditis-caused-by-salmonella-enteritidis-in-a-patient-with-adult-onset-still-s-disease-a-case-report
#7
Chia-Cheng Kuo, Wen-Liang Yu, Chen-Hui Lee, Nan-Chun Wu
RATIONALE: Purulent pericarditis is a rare and usually fatal disease. Immunodeficiency state and preexisting pericardial effusion can predispose patients to infections. However, we are not aware of similar cases in patients with adult-onset Still's disease (AOSD). In addition, it is seldom caused by Salmonella bacteria. PATIENT CONCERNS: We report a 30-year-old woman with dyspnea on exertion and epigastric fullness. She was newly diagnosed with AOSD 4 months previously and medicated with prednisolone...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29389688/adult-onset-still-disease-masquerading-as-multiple-organ-failure-neither-benign-nor-so-rare
#8
Arun Agarwal, Mudit Agarwal, Vipul Khandelwal, Priya Mathur
No abstract text is available yet for this article.
February 1, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29384877/acute-appendicitis-complicated-with-necrotizing-fasciitis-in-a-patient-with-adult-onset-still-s-disease-a-case-report
#9
Zheng-Hao Huang, Yu-Chen Chiu, Li-Lu Ho, Hsiu-Lung Fan, Chun-Chi Lu
RATIONALE: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, spiking fever, arthralgia/ arthritis, and lymphadenopathy. AOSD sometimes was fatal when it is complicated by macrophage activation syndrome (MAS) or hemophagocytic lymphohistiocytosis (HLH). Nonetheless, the literature provides no recommendations for treatment of AOSD patients with severe sepsis. PATIENT CONCERNS: A previously healthy 65-year-old man with history of AOSD was referred to our hospital for persistent right lower quadrant abdominal pain for 2 days...
February 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29374869/-social-impact-bonds-for-health-promotion-and-preventive-medicine
#10
Roni S Moran, Daniel S Moran, Gil Fire
The World Health Organization (WHO) has declared obesity a global epidemic. WHO sheds much light on this matter in its publications on health promotion and preventative medicine. Lack of physical activity, an unbalanced diet and an unhealthy lifestyle are the leading causes of developing obesity and chronic diseases. In Israel, the growing rate of obesity is a reason for concern. About 500,000 diabetics, mainly as a result of obesity, live in Israel today and by 2030 the number is expected to rise to 2,000,000...
January 2018: Harefuah
https://www.readbyqxmd.com/read/29370863/application-of-the-international-league-against-rheumatism-classification-criteria-for-systemic-juvenile-idiopathic-arthritis-as-a-prognostic-factor-in-patients-with-adults-onset-still-s-disease
#11
Ji Won Yang, Eunyoung Lee, Ji-Yeon Seo, Ju-Yang Jung, Chang-Hee Suh, Hyoun-Ah Kim
BACKGROUND: Adult-onset Still's disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification. This study evaluated the concordance between the International League Against Rheumatism (ILAR) criteria for systemic JIA and the Yamaguchi criteria and then compared their possible prognostic value in patients with AOSD. METHODS: In a retrospective review of 169 adults with suspected AOSD, patients were classified according to the Yamaguchi or ILAR criteria...
January 25, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29362813/two-severe-cases-of-adult-onset-still-s-disease-with-persistent-pruritic-eruptions
#12
Yang Liu, Tetsuya Ikawa, Yayoi Tada, Takamitsu Tanaka, Takamitsu Ohnishi, Shinichi Watanabe, Daisuke Tsukui, Kurumi Asako, Hajime Kono
No abstract text is available yet for this article.
January 24, 2018: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29340169/autoimmune-retinopathy-a-review
#13
REVIEW
Aristófanes Mendonça Canamary, Walter Yukihiko Takahashi, Juliana Maria Ferraz Sallum
Autoimmune retinopathy (AIR) is a rare and still poorly understood immune-mediated disease that may cause inflammation from circulating autoantibodies against the retina. It may be related to history of autoimmune disease in the patient or in a family member or the presence of neoplastic disease in the individual. The disease may be subdivided into paraneoplastic and non-paraneoplastic AIR. When related to melanoma, it is referred to as MAR, and when related to other cancers, it is called CAR. The exact prevalence of AIR is unknown...
2018: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/29334750/-remission-of-steroid-resistant-stills-disease-treated-with-anakinra-evidenced-by-fdg-pet-ct-examination-case-report
#14
Zdeněk Adam, Jana Skřičková, Milan Krtička, Zdeněk Řehák, Renata Koukalová, Andrea Šprláková, Marta Krejčí, Luděk Pour, Zdenka Adamová, Eva Pourová, Zdeněk Král
After elimination of infectious causes, neoplastic causes and the systemic autoimmune disease of connective tissue, a patient with high fevers over 39 °C was diagnosed with Stills disease. High doses of prednisone led to resolution of symptoms, however after reducing the doses of prednisone to 15 mg, high fevers over 39 °C returned, as well as joint pains. The high doses of prednisone led to decompensation of diabetes mellitus even with 4 daily insulin dosages. Therefore it was proceeded to regular subcutaneous administration of anakinra once a day...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29322632/approaches-to-sample-size-calculation-for-clinical-trials-in-rare-diseases
#15
Frank Miller, Sarah Zohar, Nigel Stallard, Jason Madan, Martin Posch, Siew Wan Hee, Michael Pearce, Mårten Vågerö, Simon Day
We discuss 3 alternative approaches to sample size calculation: traditional sample size calculation based on power to show a statistically significant effect, sample size calculation based on assurance, and sample size based on a decision-theoretic approach. These approaches are compared head-to-head for clinical trial situations in rare diseases. Specifically, we consider 3 case studies of rare diseases (Lyell disease, adult-onset Still disease, and cystic fibrosis) with the aim to plan the sample size for an upcoming clinical trial...
January 10, 2018: Pharmaceutical Statistics
https://www.readbyqxmd.com/read/29313056/generalized-purpura-as-an-atypical-skin-manifestation-of-adult-onset-still-s-disease-in-a-patient-with-beh%C3%A3-et-s-disease
#16
Chika Omigawa, Takashi Hashimoto, Takaaki Hanafusa, Takeshi Namiki, Ken Igawa, Hiroo Yokozeki
No abstract text is available yet for this article.
January 9, 2018: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29282446/refractory-adult-onset-still-s-disease
#17
Wahinuddin Sulaiman, Aris Chandran Abdullah, Jerome Tan Tsen Chuen, Shaffie Baba, Norain Karim
It is often a challenge and a dilemma for clinicians encountering patients with pyrexia of unknown origin. Numerous tests performed to determine the underlying cause often give inconclusive results. We present a 52-year-old man with undulating fever for more than 10 months with persistent hyperferritinaemia, and negative immunological and serological markers. Despite corticosteroids, disease modifying anti-rheumatic agents and immune-modulator therapy, he succumbed to the illness. A diagnosis of refractory Adult onset Still's disease complicated by haemophagocytic lymphohistiocytosis was made...
October 25, 2017: Curēus
https://www.readbyqxmd.com/read/29278009/beneficial-use-of-serum-ferritin-and-heme-oxygenase-1-as-biomarkers-in-adult-onset-still-s-disease-a-multicenter-retrospective-study
#18
Yohei Kirino, Yasushi Kawaguchi, Yoshifumi Tada, Hiroshi Tsukamoto, Toshiyuki Ota, Masahiro Iwamoto, Hiroki Takahashi, Kohei Nagasawa, Shuji Takei, Takahiko Horiuchi, Hisae Ichida, Seiji Minota, Atsuhisa Ueda, Akihide Ohta, Yoshiaki Ishigatsubo
BACKGROUND: Heme oxygenase (HO)-1 is a heme-degrading enzyme highly expressed in monocyte/macrophage, serum levels of which may be promising biomarker for adult-onset Still's disease (AOSD). We here report data on the use of serum ferritin and HO-1 levels in AOSD. METHODS: Under the Hypercytokinemia Study Group collaboration, we collected sera from a total of 145 AOSD patients. Three independent experts judged whether the patients were definite AOSD depending on the clinical information...
January 11, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29275557/-forensic-analysis-of-63-cases-of-non-violent-death-occurred-in-custody
#19
S M Yang, Y B Cheng
OBJECTIVES: To collect cases of non-violent death occurred in custody for analyzing the forensic characteristics and related influencing factors. METHODS: Sixty-three cases of non-violent death among detainees in custody that handled by a forensic science center from 2000 to 2015 were collected. The type, onset season, medical history, clinical manifestation, treatment and duration of related fatal diseases were analyzed retrospectively. RESULTS: The deaths due to diseases in custody were common in male, and with a high incidence in middle aged adults...
October 2017: Fa Yi Xue za Zhi
https://www.readbyqxmd.com/read/29251027/efficacy-and-safety-of-tocilizumab-with-inhibition-of-interleukin-6-in-adult-onset-still-s-disease-a-meta-analysis
#20
Yubo Ma, Meng Wu, Xu Zhang, Qing Xia, Jiajia Yang, Shengqian Xu, Faming Pan
OBJECTIVE: Tocilizumab (TCZ) is the only available biologic inhibiting interliukine-6 presently, and emerging evidences have figured that elevated serum level of interleukine-6 is a crucial link of the pathogenesis of adult-onset Still's disease (AOSD). However, evidence about the efficacy and safety of TCZ in AOSD with strong power is still scarce. Thus, this meta-analysis was conducted to synthetically assess the efficacy and safety of TCZ on AOSD. METHODS: PubMed, Web of Science, Cochrane Library, Chinese National Knowledge Infrastructure and Chinese Biological Medical Literature database were comprehensively searched up to 31 July, 2017 for the pertinent studies...
December 18, 2017: Modern Rheumatology
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