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https://www.readbyqxmd.com/read/29340169/autoimmune-retinopathy-a-review
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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, 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. These 91 "definite AOSD" patients were further divided into active, remission, and relapse groups...
December 26, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29275557/-forensic-analysis-of-63-cases-of-non-violent-death-occurred-in-custody
#7
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
#8
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
https://www.readbyqxmd.com/read/29247988/interleukin-18-biological-properties-and-role-in-disease-pathogenesis
#9
REVIEW
Gilles Kaplanski
Initially described as an interferon (IFN)γ-inducing factor, interleukin (IL)-18 is indeed involved in Th1 and NK cell activation, but also in Th2, IL-17-producing γδ T cells and macrophage activation. IL-18, a member of the IL-1 family, is similar to IL-1β for being processed by caspase 1 to an 18 kDa-biologically active mature form. IL-18 binds to its specific receptor (IL-18Rα, also known as IL-1R7) forming a low affinity ligand chain. This is followed by recruitment of the IL-18Rβ chain. IL-18 then uses the same signaling pathway as IL-1 to activate NF-kB and induce inflammatory mediators such as adhesion molecules, chemokines and Fas ligand...
January 2018: Immunological Reviews
https://www.readbyqxmd.com/read/29246252/fever-with-lymphadenopathy-kikuchi-fujimoto-disease-a-great-masquerader-a-case-report
#10
Chamara Dalugama, Indika Bandara Gawarammana
BACKGROUND: Kikuchi Fujimoto disease is an uncommon benign condition of necrotizing histiocytic lymphadenitis commonly seen in East Asian and Japanese populations. It commonly presents with fever, cervical lymphadenopathy, and elevated inflammatory markers. Diagnosis of Kikuchi Fujimoto disease is based on histopathological studies of the involved lymph nodes. The presentation of Kikuchi Fujimoto disease can mimic many sinister conditions including lymphoma. Treatment is mainly supportive provided that accurate diagnosis is made and sinister conditions like lymphoma ruled out...
December 16, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29241877/adult-onset-still-s-disease
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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