Infantile Cystinosis

Infantile cystinosis is a rare disorder which leftuntreated results in end -stage renal disease early in life. Together with dehydration and electrolyte imbalance due to renal tubular Fanconi syndrome, endstage renal disease used to be the leading cause of death in children with cystinosis. Specific therapy with cysteamine (cystine -depleting agent) has changed the course of this disease. Instead of being fatal in childhood, it can nowadays be considered a multisystemic adult disorder. The authors report a case of a child diagnosed with Fanconi syndrome at 14 months of age and infantile cystinosis at 19 months of age in whom oral cysteamine treatment led to a good outcome during childhood

The Clifford torus as a self-shrinker for the Lagrangian mean curvature flow

We provide several rigidity results for the Clifford torus in the class of compact self-shrinkers for Lagrangian mean curvature flow.Comment: 10 page

Revelations of U.S. spying against Brazil have put relations between the two at arm’s length once again

Last October, evidence of spying by the U.S. National Security Agency against the Brazilian government was uncovered, which led to the cancellation of President Dilma Rousseff’s visit to the U.S. João Augusto de Castro Neves looks at the implications of the spying row, writing that while it has affected cooperation in areas such as defense and energy, the continuing links and interests between American and Brazilian companies operating in both countries mean that a full-blown trade war is unlikely

Spectroscopic Analysis in the Virtual Observatory Environment with SPLAT-VO

SPLAT-VO is a powerful graphical tool for displaying, comparing, modifying and analyzing astronomical spectra, as well as searching and retrieving spectra from services around the world using Virtual Observatory (VO) protocols and services. The development of SPLAT-VO started in 1999, as part of the Starlink StarJava initiative, sometime before that of the VO, so initial support for the VO was necessarily added once VO standards and services became available. Further developments were supported by the Joint Astronomy Centre, Hawaii until 2009. Since end of 2011 development of SPLAT-VO has been continued by the German Astrophysical Virtual Observatory, and the Astronomical Institute of the Academy of Sciences of the Czech Republic. From this time several new features have been added, including support for the latest VO protocols, along with new visualization and spectra storing capabilities. This paper presents the history of SPLAT-VO, it's capabilities, recent additions and future plans, as well as a discussion on the motivations and lessons learned up to now.Comment: 15 pages, 6 figures, accepted for publication in Astronomy & Computin

Predictors Of The Effectiveness Of Insulin Pumps In Patiens With Type 1 Diabetes Mellitus

Objetivo: O tratamento com bombas de insulina tem se vindo a tornar o tratamento preferencial da Diabetes Tipo 1 (DM1), uma vez que mimetiza a secreção fisiológica de insulina de uma melhor forma que as múltiplas injeções diárias. No entanto, nem todos os doentes melhoram com o tratamento com bombas de insulina. Este estudo tem como objetivo determinar quais os preditores da eficácia das bombas de insulina em doentes com DM1. Métodos: Foi realizado um estudo observacional retrospetivo de doentes que iniciaram o tratamento com bombas de insulina. Foram colhidos dados de quatro momentos do tempo diferentes (antes, aos 6, 12 e 36 meses depois do tratamento) e avaliados para outcomes de controlo glicémico e de segurança. A associação dos preditores iniciais com os outcomes foi analisada através de modelos de regressão linear e logística. Resultados: Foram avaliados 136 doentes (57,4% mulheres, idade 36 ± 12 anos, tempo de DM1 14 ± 9 anos). Durante o follow-up, registou-se uma diminuição média da HbA1c de 0,9 ± 1,2%. A melhoria da HbA1c foi independente do sexo, idade e duração do DM1. Valores de HbA1c basais mais altos, história familiar de diabetes e não estar medicado com estatinas foram preditores de melhoria da HbA1c. Não estar medicado com estatinas e valores de HbA1c basais mais altos mostraram melhorar a HbA1c sem piorar a hipoglicemia. Ter história de hipoglicemia verificou-se como um preditor de hipoglicemia grave. História familiar de diabetes, valores de HbA1c basais mais altos e distúrbios psicológicos/ psiquiátricos foram preditores de cetoacidose diabética. Conclusão: Os benefícios do tratamento com bombas de insulina foram independentes do sexo, idade e duração da DM1. Valores de HbA1c basais, história familiar de diabetes, tratamento com estatinas, história de hipoglicemia e distúrbios psicológicos/ psiquiátricos foram preditores de outcomes e podem permitir a identificação dos doentes que mais beneficiam do tratamento com bombas de insulina ou que estão em risco aumentado de complicações.Purpose: Insulin pump therapy has become the preferential treatment for type 1 diabetes (T1D) as it mimics the physiological secretion of insulin better than multiple daily injections. However, not all patients improve with insulin pump therapy. This study aims to determine the predictors of the effectiveness of insulin pumps in T1D. Methods: We conducted a retrospective observational study of patients who started insulin pumps. Data from four timepoints (before, at 6, 12 and 36 months) were evaluated for outcomes of glycemic control and safety. The association of baseline predictors with outcomes was analyzed using linear and logistic regression models. Results: We evaluated 136 patients (57.4% females, age 36±12 years, duration of T1D 14±9 years). During the follow-up, there was a mean decrease of HbA1c of 0.9±1.2%. The improvement in HbA1c was independent of sex, age and duration of T1D. Higher baseline HbA1c, family history of diabetes and not being treated with statins were predictors of improvement in HbA1c. Not being treated with statins and higher baseline HbA1c predicted improvement in HbA1c without worsening hypoglycemia. History of hypoglycemia was a predictor of severe hypoglycemia. Family history, higher baseline HbA1c and psychological/psychiatric disorders were predictors of ketoacidosis. Conclusion: Benefits of insulin pump were independent of sex, age, and duration of T1D. Baseline HbA1c, family history of diabetes, treatment with statins, history of hypoglycemia and psychological/psychiatric disorders were predictors of outcomes and may allow the identification of patients who benefit most from insulin pump therapy or who are at increased risk of complications

Valor prognóstico da monitorização ambulatória da pressão arterial numa população portuguesa com evento cardiovascular prévio

Ambulatory blood pressure monitoring (ABPM) is a predictor of cardiovascular outcomes in patients without previous cardiovascular events. However, the same predictive value for secondary events is not yet established in the medical community. In this work, we investigate the predictive value of ABPM for patients with prior cardiovascular events, considering the following variables: 24-hour blood pressure (BP), daytime and night-time BP, BP dipping, daytime and night-time pulse pressure and heart rate. We conducted an observational study on a population of adults with previous cardiovascular events, referenced for ABPM between 1996 and 2017 at Centro Hospitalar do Baixo Vouga. A total of 391 ABPM records from 295 hypertensive patients, from which 72.6% were men and 37.6% were diabetic, were evaluated. Previous cardiovascular events included 235 coronary events, 140 ischemic strokes, 11 hemorrhagic strokes and five carotid and peripheral artery surgeries. During a mean follow-up of 4.5 ± 5.2 years, 93 recurrent cardiovascular events were observed, namely 42 ischemic strokes, 38 coronary events, seven hemorrhagic strokes, five peripheral artery surgeries and one carotid endarterectomy. The present work allowed us to conclude that (1) ABPM has predictive value for recurrent cardiovascular events in patients with previous cardiovascular events; (2) the predictive value of systolic and diastolic blood pressure values obtained from ABPM were superior to the 24 hour, and that night-time has no predictive value for recurrent events; (3) ABPM thresholds that predict the second event are lower than those used for predicting first events; (4) ABPM has a stronger prediction value for recurrent events in patients < 65 years when comparing to patients ≥ 65 years old; in patients with < 65 years old, the predictive value of the systolic blood pressure values (24h, daytime and night-time) was superior to the respective diastolic values; (5) ABPM values from before the event and after the event decrease overall.A Monitorização Ambulatória da Pressão Arterial (MAPA) tem valor preditor de eventos cardiovasculares em doentes sem evento prévio. No entanto, o valor preditor para eventos secundários não foi ainda estabelecido na comunidade médica. Neste trabalho, investigamos o valor preditivo da MAPA em doentes com evento cardiovascular prévio, considerando as variáveis seguintes: pressão arterial (PA) de 24 horas, diurna e noturna, queda noturna, pressão de pulso diurno e noturno e frequência cardíaca. Realizámos um estudo observacional numa população de adultos com evento cardiovascular prévio, referenciado para MAPA entre 1996 e 2017 no Centro Hospitalar do Baixo Vouga. Foram avaliados um total de 391 registos de MAPA de 295 doentes hipertensos, dos quais 72.6% eram homens, 37.6% eram diabéticos e 19.2% eram fumadores. Os eventos cardiovasculares prévios incluíram: 235 eventos coronários, 140 acidentes vasculares cerebrais isquémicos, 11 acidentes vasculares cerebrais hemorrágicos e cinco cirurgias vasculares carotídeas e periféricas. Durante um follow-up de 4.5 ± 5.2 anos, foram observados 93 eventos recorrentes, dos quais 42 acidentes vasculares cerebrais isquémicos, 38 eventos coronários, sete acidentes vasculares hemorrágicos, cinco cirurgias arteriais periféricas e uma endarterectomia carotídea. O estudo permitiu concluir que: (1) a MAPA teve valor preditivo para eventos cardiovasculares recorrentes em doentes com evento cardiovascular prévio; (2) de entre os valores da MAPA, o valor preditivo dos valores sistólicos e diastólicos diurnos foram superiores aos de 24 horas, e que os valores noturnos não têm valor preditivo para eventos cardiovasculares recorrentes; (3) os limites da MAPA para predição de eventos recorrentes foram mais baixos que os usados para predição primária (4) ; a MAPA teve um valor preditivo de eventos recorrentes superior nos doentes < 65 anos relativamente aos doentes ≥ 65 anos e em doentes com < 65 anos, o valor preditivo dos valores sistólicos (24 horas, diurnos e noturnos) foi superior ao dos respetivos valores diastólicos; (5) os valores da MAPA antes do evento para depois do evento diminuem na sua globalidade.Programa Doutoral em Ciências e Tecnologias da Saúd

Banality and intersubjectivity in art

This article aims to reflect on the banality of art in its quotidianity. It attempts to question by what social dynamics the common, the banal and the quotidian can come to have artistic value. The answer we seek to build for the question, observes this sensation in its dynamics of sociation, that is, as a bond, as a structure of the collective and experiential character of social life. By understanding the phenomenon as a total social fact, we can say that it is engendered and simultaneously engenders the societal bond in an intersubjective procedure that produces the shared sense