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To evaluate the ethnic diversity of children with a systemic autoinflammatory disease (SAID) in a multi-ethnic Canadian province.Self-reported ethnicity of 149 children and adolescents with a SAID in British Columbia, Canada, was analysed for ethnic representation among individual patients, across the cohort, within particular SAIDs, and compared to provincial census data on ethnic diversity.Half of reported cases had a diagnosis of either PFAPA (23.5%) or an unclassifiable autoinflammatory syndrome (31.5%), with a monogenic SAID diagnosed in only 12.8% of cases. The majority of participants (73.1%) were mixed ethnicity with European and Asian heritage reported most frequently (57.0% and 23.0% of all responses, respectively). Ethnic diversity reflected regional diversity except for West Asian, Arabic, Jewish, and Eastern European heritage, which were over-represented in SAID patients, and Chinese descent, which was under-represented in our cohort compared to the general population of British Columbia.Results from this study show extensive multi-ethnic diversity in individual patients and across the various SAIDs inclusive of monogenic SAIDs that are frequently associated with particular ethnicities. Although not disproportionately represented, this is the first report of systemic autoinflammatory disease in Canadian children of Indigenous heritage.

The popularity of internet-distributed TV entertainment services, such as Netflix, has transformed TV consumption behavior. Currently, the level of control viewers have over their TV experiences, along with the release of complete seasons at once, are some of the factors that stimulate the so-called binge-watching phenomenon (the consumption of several episodes of a program in a single sitting). Most of binge-watching studies have focused on viewers’ habits and health effects. This paper presents a study that relates to viewers’ behaviors and moods. It was carried out with 13 young participants at their home, watching online content, collecting physiological, inertial, and self-reported data. We identify and compare binge-watching with non-binge-watching behaviors. Our results suggest that while viewers recur to online serial entertainment in pursuit of leisure related needs, such as relaxation, relief from boredom and escapism, the act of binge-watching tends to make them feel rather unsatisfied with no change in Arousal. Nevertheless, in binge-watching the Positive Affect increases while the Negative decreases. Moreover, watching a single episode only, tends to result in increased arousal and but not necessarily in increased satisfaction. This preliminary finding can be the starting point of fruitful future investigations on unpacking further motives and nuances from this outcome.

Diatoms, along with brown seaweeds, are the main producers of fucoxanthin, an oxycarotenoid with important biological functions such as antioxidant, anti-obesity, antitumor, antidiabetic, and anti-inflammatory activities. In this scenario, the marine diatom Phaeodactylum tricornutum is considered an important source of this carotenoid. Fucoxanthin production by this diatom has been studied through different approaches, e.g., in vitro and in vivo investigation of the effects of environmental factors and strains, as well as through metabolic engineering analysis. The optimization of environmental factors such as light exposure, pH, temperature and aeration has been reported to modulate fucoxanthin production by P. tricornutum cell cultures. In parallel, the identification and selection of strains with an elevated fucoxanthin biosynthesis and accumulation have also contributed to improve the biotechnological performance of that microalga. Indeed, much can be done from the screening of the genomic sequence of P. tricornutum, which has been completed in 2008. Candidate genes for specific reactions in carotenoid biosynthesis pathways can be cloned, expressed, and identified by combining genetic and analytical strategies. Furthermore, metabolic reactions can be used to perform in vitro simulations, mitigating laboratory costs. This chapter aims to present an overview of fucoxanthin biosynthesis in P. tricornutum as well as the different ways to enhance its production., CNPq -Conselho Nacional de Desenvolvimento Científico e Tecnológico(307099/2015-6), info:eu-repo/semantics/publishedVersion

PubMedID: 28864648 Objective. To arrive at consensus for candidate outcomes for disease activity assessment in largevessel vasculitis (LVV) in clinical trials. Methods.A Delphi survey including 99 items was circulated among international experts for 3 rounds. Results. Fifty-seven items were accepted for both giant cell arteritis and Takayasu arteritis. Sixty-seven percent of experts voted to have a common approach for both diseases with additional disease-specific items such as weight loss, scalp tenderness/necrosis, morning stiffness, dizziness, visual symptoms, and imaging. Conclusion. This study highlights similarities and differences in experts' perspectives for assessing clinical activity in LVV and may guide a consensus-driven core set of validated outcomes. Copyright © 2017. All rights reserved. National Institute of Arthritis and Musculoskeletal and Skin Diseases: U01 AR51874 04, U54 AR057319 National Center for Research Resources: U54 RR019497 Sponsored by the Vasculitis Clinical Research Consortium, which has received support from the US National Institute of Arthritis and Musculoskeletal and Skin Diseases (U54 AR057319 and U01 AR51874 04), the National Center for Research Resources (U54 RR019497), and the Office of Rare Diseases Research. Additional support for the work of the OMERACT Vasculitis Working Group was received through a Patient-Centered Outcomes Research Institute Pilot Project Grant. S.Z. Aydin, MD, Associate Professor, Division of Rheumatology, The Ottawa Hospital Research Institute, University of Ottawa; H. Direskeneli, MD, Professor of Rheumatology, Division of Rheumatology, Marmara University Faculty of Medicine; P.A. Merkel, MD, MPH, Professor of Medicine and Epidemiology, Division of Rheumatology, and Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania. Address correspondence to Dr. P.A. Merkel, Section of Rheumatology, University of Pennsylvania, White Building, 5th Floor Penn, 3400 Spruce St., Philadelphia, Pennsylvania 19104, USA. E-mail: pmerkel@upenn.edu Accepted for publication June 3, 2017.

Objective To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Methods Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener’s), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauciimmune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of Results In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9–15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4–6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0–6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. Conclusion This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.

RESUMEN Antecedentes: La acalasia es el desorden motor primario del esófago, en el cual hay una relajación ineficiente del esfínter esofágico inferior con pérdida progresiva de la perístasis esofágica, como consecuencia de ambos mecanismos se produce dilatación gradual del esófago. La acalasia puede ser primaria (idiopática) y secundaria. La causa más común de acalasia secundaria es la enfermedad de Chagas. Objetivo: Analizar nuestra experiencia con los datos de pacientes diagnosticados y tratados de acalasia en el Servicio de Cirugía General del Hospital Nacional de Itaugua. Diseño: Observacional. Material y Método: Pacientes diagnosticados de Acalasia en el Servicio de Cirugía General del Hospital Nacional de Itaugua entre enero de 2008 y diciembre de 2013. Todos los casos de acalasia diagnosticados de forma consecutiva en el período e institución antes señalados que pudieron ser intervenidos quirurgicamente. Se excluyeron los casos sin intervención quirurgica. Se consideró la edad, género; también nos interesó la presentación de los pacientes con anemia y el motivo de consulta con el cual se presentaron. Conclusiones: La miotomía asociada a una funduplicatura parcial por laparoscopía, es una técnica segura y efectiva para el alivio de la disfagia en los pacientes con acalasia. En los dolicomegaesofagos de no existir contraindicación la esofagectomía debe ser evaluada. SUMMARY Background: Achalasia is a primary Motor disorder of the esophagus in which there is an inefficient lower esophageal sphincter relaxation with progressive loss of esophageal peristalsis, as a result of two mechanisms gradual dilation of the esophagus occurs. Achalasia can be primary (idiopathic) and secondary. The most common cause of secondary achalasia is Chagas disease. To analyze our experience with data from patients diagnosed and treated for achalasia in the Department of Surgery of the National Hospital of Itaugua. Design: Observational. Material And Methods: Patients diagnosed with achalasia in General Surgery Itaugua National Hospital between January 2008 and December 2013 All cases of achalasia diagnosed consecutively in the mentioned period and institution that could be surgically operated. Cases without surgical intervention were excluded. Age, gender was considered; also interested us Patients presenting with anemia and the reason for consultation which were presented. Conclusions: myotomy associated with a partial laparoscopic fundoplication is safe and effective for the relief of dysphagia in patients with achalasia art. In the absence of contraindications dolicomegaesofagos esophagectomy should be evaluated.