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ADULT ONSET STILL

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https://www.readbyqxmd.com/read/28646209/upregulation-of-circulating-microrna-134-in-adult-onset-still-s-disease-and-its-use-as-potential-biomarker
#1
Tsai-Ling Liao, Yi-Ming Chen, Chia-Wei Hsieh, Hsin-Hua Chen, Hsiu-Chin Lee, Wei-Ting Hung, Kuo-Tung Tang, Der-Yuan Chen
Adult-onset Still's disease (AOSD) is a multi-systemic inflammatory disorder of unknown etiology. To date, no single diagnostic test is available for AOSD. Herein, we investigated the pathogenic role of microRNAs in AOSD. MicroRNA profiles in plasma from AOSD patients and healthy controls were analyzed by microarray analysis, followed by quantitative reverse transcription PCR validation. The biological functions of microRNAs were evaluated using in vitro cell-based assay. Among the differentially expressed microRNAs, microRNA-134 (miR-134) expression was positively correlated with AOSD activity scores and significantly decreased after effective treatment...
June 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28640110/case-report-of-a-modified-meso-rex-bypass-as-a-treatment-technique-for-late-onset-portal-vein-cavernous-transformation-with-portal-hypertension-after-adult-deceased-donor-liver-transplantation
#2
Dongdong Han, Rui Tang, Liang Wang, Ang Li, Xin Huang, Shan Shen, Jiahong Dong
RATIONALE: Portal vein thrombosis is a complication after liver transplantation and cavernous transformation of the portal vein (CTPV) is a result of portal vein thrombosis, with symptoms of portal hypertension revealed by an enhanced CT scan. Meso-Rex bypass is an artificial shunt connecting the left portal vein to the superior mesenteric vein and is mainly used for idiopathic cavernomas. This technique is also used for post-transplant portal vein thrombosis in pediatric patients thereby bypassing obstructed sites of the extrahepatic portal vein...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28639937/a-multicentre-randomised-controlled-trial-of-intravenous-immunoglobulin-compared-with-standard-therapy-for-the-treatment-of-transverse-myelitis-in-adults-and-children-strive
#3
Michael Absoud, Peter Brex, Olga Ciccarelli, Onyinye Diribe, Gavin Giovannoni, Jennifer Hellier, Rosemary Howe, Rachel Holland, Joanna Kelly, Paul McCrone, Caroline Murphy, Jackie Palace, Andrew Pickles, Michael Pike, Neil Robertson, Anu Jacob, Ming Lim
BACKGROUND: Transverse myelitis (TM) is an immune-mediated disorder of the spinal cord that affects adults and children and that causes motor, sensory and autonomic dysfunction. There is a prolonged recovery phase, which may continue for many years. Neuromyelitis optica (NMO) is an uncommon relapsing inflammatory central nervous system condition in which TM can be the first presenting symptom. As TM and NMO affect many patients in the prime of their working life, the disorder can impose a significant demand on health resources...
May 2017: Health Technology Assessment: HTA
https://www.readbyqxmd.com/read/28614216/refractory-adult-onset-still-disease-complicated-by-macrophage-activation-syndrome-and-acute-myocarditis-a-case-report-treated-with-high-doses-8%C3%A2-mg-kg-d-of-anakinra
#4
Federico Parisi, Annamaria Paglionico, Valentina Varriano, Gianfranco Ferraccioli, Elisa Gremese
RATIONALE: Myocarditis is a rare but potentially fatal complication of Still's disease (about 7% of total cases). PATIENT CONCERNS: A 42-year-old woman was admitted to our ward with high-grade fever, rash and polyarthralgia, lasting since 4 weeks and rapidly complicated by MAS and acute heart failure. DIAGNOSES: Adult Onset Still's Disease rapidly developping macrophage activation syndrome and disseminated intravascular coagulopathy, further complicated by iperacute myocarditis with cardiac arrest...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28612321/the-changing-shape-of-the-iscev-standard-pattern-onset-vep
#5
Dorothy A Thompson, Dennis M Fritsch, Sharon E Hardy
PURPOSE: Pattern onset VEPs do not always show distinct C1-C2-C3 peaks and troughs. Our purpose was to study changes in pattern onset VEP with age to determine when the illustrated ISCEV standard onset VEP waveform can be reliably recorded. METHODS: We recorded pattern onset VEPs from an Oz electrode referred to mid-frontal electrode according to ISCEV standards by presenting checks of 60' and 15' side length in a 15° field. Twenty-four adults aged 20-63 years participated...
June 13, 2017: Documenta Ophthalmologica. Advances in Ophthalmology
https://www.readbyqxmd.com/read/28603586/sleep-health-behaviors-and-behavioral-interventions-reducing-the-risk-of-cardiovascular-disease-in-adults
#6
REVIEW
Jill L Kaar, Christina M Luberto, Kirsti A Campbell, Jeff C Huffman
Numerous health behaviors, including physical activity, diet, smoking, and sleep, play a major role in preventing the development and progression of cardiovascular disease (CVD). Among these behaviors, sleep may play a pivotal role, yet it has been studied somewhat less than other behaviors and there have been few well-designed sleep intervention studies targeting CVD. Furthermore, despite the fact that these behaviors are often interrelated, interventions tend to focus on changing one health behavior rather than concurrently intervening on multiple behaviors...
May 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28603070/clinical-utility-of-quantitative-pcr-for-chimerism-and-engraftment-monitoring-after-allogeneic-stem-cell-transplantation-for-hematologic-malignancies
#7
Müberra Ahci, Karin Stempelmann, Ulrike Buttkereit, Pietro Crivello, Mirko Trilling, Andreas Heinold, Nina Kristin Steckel, Michael Koldehoff, Peter A Horn, Dietrich W Beelen, Katharina Fleischhauer
Although quantitative PCR (qPCR) has been explored for chimerism monitoring after allogeneic stem cell transplantation (SCT), evidence regarding its clinical utility compared to standard short tandem repeat (STR) is still limited. We retrospectively studied commercial qPCR and STR chimerism with respective positivity thresholds of 0.1% and 1%, in 359 peripheral blood (PB) and 95 bone marrow (BM) samples from 30 adult patients after first HLA-matched SCT for myeloid malignancies or acute lymphatic leukemia. Concordance between the two methods was 79...
June 8, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28601443/a-cohort-study-of-the-effect-of-winter-dysentery-on-herd-level-milk-production
#8
Ingrid Toftaker, Ingrid Holmøy, Ane Nødtvedt, Olav Østerås, Maria Stokstad
Winter dysentery (WD) is a contagious disease caused by bovine coronavirus. It is characterized by acute onset of diarrhea, fever, depression, and reduced milk yield in adult cattle. Although production loss is a well-known consequence of WD, large-scale studies estimating the effect on milk production are lacking. The objective of this study was to estimate the effect of farmer-reported WD on herd-level milk production and milk composition. A cohort study was performed based on reports of herd outbreaks of winter dysentery during a regional epidemic in Norway during the winter of 2011-2012...
June 7, 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/28598057/macrophage-activation-syndrome
#9
P Babu Raj, B L Harikrishnan, Roshan Mampilly, R Anand
Macrophage Activation Syndrome (MAS) occurs as a severe life-threatening complication of several chronic rheumatic diseases. It is more frequent with systemic onset juvenile arthritis and adult onset Still's disease.1 It can be primary, infection related, malignancy associated or autoimmune3. We report a case of Macrophage Activation Syndrome presenting as pyrexia of unknown origin (PUO) and pancytopenia in the absence of any known triggering factor.
May 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28589388/-efficacy-and-safety-analysis-of-off-label-treatment-with-biologics-in-autoinflammatory-diseases-experiences-from-a%C3%A2-german-registry%C3%A2-graid2
#10
F Proft, M Fleck, C Fiehn, H Schulze-Koops, M Witt, T Dörner, J C Henes
OBJECTIVE: To evaluate the safety and efficacy of therapy with biologics in patients with autoinflammatory diseases (AIF) or macrophage activating syndrome (MAS) in a real-life setting in Germany. METHODS: The German Register of Autoimmune Diseases 2 (GRAID2) is a retrospective, non-interventional, multicenter registry collecting data from all patients with inflammatory rheumatic diseases refractory to conventional therapy and treated with initial off-label biologics between August 2006 and December 2013...
June 6, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28588495/a-brief-history-of-il-1-and-il-1-ra-in-rheumatology
#11
Jean-Michel Dayer, Francesca Oliviero, Leonardo Punzi
The history of what, in 1979, was called interleukin-1 (IL-1), orchestrator of leukocyte inter-communication, began many years before then, initially by the observation of fever induction via the endogenous pyrogen (EP) (1974) and then in rheumatology on the role in tissue destruction in rheumatoid diseases via the induction of collagenase and PGE2 in human synovial cells by a mononuclear cell factor (MCF) (1977). Since then, the family has exploded to presently 11 members as well as many membrane-bound and soluble receptor forms...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28575651/mutations-in-nkx6-2-cause-progressive-spastic-ataxia-and-hypomyelination
#12
Viorica Chelban, Nisha Patel, Jana Vandrovcova, M Natalia Zanetti, David S Lynch, Mina Ryten, Juan A Botía, Oscar Bello, Eloise Tribollet, Stephanie Efthymiou, Indran Davagnanam, Fahad A Bashiri, Nicholas W Wood, James E Rothman, Fowzan S Alkuraya, Henry Houlden
Progressive limb spasticity and cerebellar ataxia are frequently found together in clinical practice and form a heterogeneous group of degenerative disorders that are classified either as pure spastic ataxia or as complex spastic ataxia with additional neurological signs. Inheritance is either autosomal dominant or autosomal recessive. Hypomyelinating features on MRI are sometimes seen with spastic ataxia, but this is usually mild in adults and severe and life limiting in children. We report seven individuals with an early-onset spastic-ataxia phenotype...
June 1, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28572051/late-onset-rasmussen-encephalitis-a-literature-appraisal
#13
REVIEW
Sophie Dupont, Ana Gales, Serge Sammey, Marie Vidailhet, Virginie Lambrecq
Rasmussen Encephalitis (RE) is classically described as a childhood encephalopathy due to a unilateral inflammation of the cerebral cortex with a presumed immune-mediated pathophysiological basis. Unusual variant forms, including adolescent and adult-onset RE have been described but there is still a doubt whether these atypical cases correspond to classical RE patients. To review evidence, a systematic PubMed search was conducted to retrieve papers addressing late onset RE to assess (i) the positivity rate of classical childhood-onset diagnostic criteria for RE in late-onset RE, (ii) the specific clinical and radiological features that could help earlier diagnosis and therapeutic interventions, (iii) the arguments for an autoimmune pathophysiology including (iiia) the association with autoimmune markers or diseases and (iiib) the effects of immunomodulatory or immunosuppressive treatments...
May 29, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28540751/treatment-of-adult-onset-still-s-disease-up-to-date
#14
Dae Hyun Yoo
Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology, and approximately 60-70% of patients may develop a chronic polyphasic form of the disease or a chronic polyarthritis. Due to rarity of disease, treatment of AOSD is not based on controlled study, but on case based experiences. Areas covered: Recently, the application of anti-cytokine therapy based on pathophysiology has resulted in significant progress in the treatment of AOSD. Here, we review current knowledge of the pathogenesis, disease progression, currently available biomarkers of disease activity, standard therapeutic agents, utility of biologic agents, future perspectives for treatment and treatment of macrophage activation syndrome...
June 5, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28540590/pediatric-psoriasis
#15
REVIEW
Maria Relvas, Tiago Torres
Psoriasis is a chronic inflammatory systemic disease associated with an important physical and physiological burden. It primarily affects the skin, but it is associated with several serious medical co-morbidities. One third of total psoriatic cases have their onset during the pediatric age, although some of them may not be diagnosed until the patient reaches adulthood. Additionally, in the pediatric age, there is an association with several medical co-morbidities; thus, an early recognition of the disease and a subsequent appropriate approach may delay or even prevent considerable co-morbidities...
May 24, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28532741/coexistence-of-sarcoidosis-and-adult-onset-still-disease
#16
Huseyin Semiz, Senol Kobak
Sarcoidosis is a chronic, inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It can be presented with bilateral hilar lymphadenopathy, skin lesions, eye involvement and locomotor system findings. Adult onset Still disease (AOSD) is a chronic inflammatory disease which presents with fever, arthritis and typical skin rashes. The disease is rare and can be misdiagnosed due to the absence of typical clinical and laboratory findings. The association of sarcoidosis and AOSD has not been previously reported in the literature...
May 19, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28531906/tocilizumab-induced-drug-reaction-with-eosinophilia-and-systemic-symptoms-syndrome-in-adult-onset-still-disease-a-case-report
#17
Elina Zuelgaray, Fanny Domont, Nathan Peiffer-Smadja, David Saadoun, Patrice Cacoub
No abstract text is available yet for this article.
May 23, 2017: Annals of Internal Medicine
https://www.readbyqxmd.com/read/28529117/new-markers-for-adult-onset-still-s-disease
#18
Stéphane Mitrovic, Bruno Fautrel
Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disorder (SAID). Although the pathogenesis of the disease is complex and far from being fully understood, recent progresses in pathophysiological knowledge have paved the way to new diagnostic approaches. Indeed, AOSD diagnosis can be a real challenge, owing to its infrequency, and to the lack of specificity of the principal clinical features (high fever, arthralgia or arthritis, skin rash) and laboratory findings (elevated acute phase reactants, hyperleukocytosis ≥ 10,000 cells/mm(3) with neutrophils ≥80%)...
May 18, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28527721/indicators-and-outcomes-of-transfer-to-tertiary-pediatric-hospitals-for-patients-with-testicular-torsion
#19
Janae Preece, Christina Ching, Katelyn Yackey, Venkata Jayanthi, Daryl McLeod, Seth Alpert, Daniel DaJusta
INTRODUCTION: Testicular torsion threatens testicular viability with increased risk of loss with delayed management. Still, healthy adolescents continue to be transferred from community hospitals to tertiary hospitals for surgical management for torsion even though adult urologists may be available. We sought to determine reasons behind patient transfer and to evaluate whether transfer to tertiary centers for testicular torsion leads to increased rates of testicular loss. MATERIALS AND METHODS: A retrospective chart review was performed for patients presenting to our free-standing pediatric tertiary care facility with surgically confirmed testicular torsion during the 5-year period between January 2011 and January 2016...
April 27, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28526006/cardiometabolic-profile-of-obese-children-in-a-sub-saharan-african-setting-a-cross-sectional-study
#20
Eunice Chedjou-Nono, Suzanne Sap, Simeon-Pierre Choukem, Issa Ngosso Tetanye, Daniel Nebongo, Olivier Koki Ndombo
BACKGROUND: Cardiovascular and metabolic consequences of obesity in children, unlike adults, are still not well understood nor have they been subject to extensive research in Africa. We aimed to identify the cardio-metabolic complications associated with childhood obesity at the early phase of the management of obese children in a reference center in Cameroon. METHODS: In this cross-sectional study conducted from November 2013 to September 2014 and based on World Health Organization (WHO) classification of Obesity (BMI > 3SD under 5 years and BMI > 2SD from 5 and above), we included children aged 3 to 17 years who were being followed up for obesity at the pediatric endocrinology unit of the Mother and Child Center of the Chantal BIYA Foundation in Yaounde, Cameroon...
May 19, 2017: BMC Pediatrics
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