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The cutaneous mastocytosis is characterized by infiltration and proliferation of mast cells in the skin with no evidence of extracutaneous involvement. The clinical findings most frequently found are brownish macules or papules slightly elevated, frequently localized in the limbs, chest and abdomen. Mutations in the c-kit receptor (CD117) are frequent and provide the pathophysiology basis of the disease. These mutations allow autophosphorylation of the c-kit receptor and subsequent degranulation of mast cell precipitated with a variety of stimuli, releasing local or systemic cell mediators capable of causing symptoms. Skin lesions occur in both the systemic and the cutaneous form of the disease, though the latter is more frequently seen in children, while most adults with mastocytosis present signs and symptoms of systemic involvement. Although this clinical pattern, we report a rare case of adult-onset cutaneous mastocytosis in a masculine patient of 32 years old. Clinical presentation featured hyperpigmented macules with brownish coloration diffused through the chest and limbs. Skin and bone marrow biopsies and laboratory testing were made and strengthened the diagnosis of cutaneous mastocytosis, a clinical presentation with little similar case reports.

Low-code application development as proposed by the OutSystems Platform enables fast mobile and desktop application development and deployment. It hinges on visual development of the interface and business logic but also on easy integration with data stores and services while delivering robust applications that scale. Data integration increasingly means accessing a variety of NoSQL stores. Unfortunately, the diversity of data and processing models, that make them useful in the first place, is difficult to reconcile with the simplification of abstractions exposed to developers in a low-code platform. Moreover, NoSQL data stores also rely on a variety of general purpose and custom scripting languages as their main interfaces. In this paper we propose a polyglot data access layer for the OutSystems Platform that uses SQL with optional embedded script snippets to bridge the gap between low-code and full access to NoSQL stores. In detail, we characterize the challenges for integrating a variety of NoSQL data stores; we describe the architecture and proof-of-concept implementation; and evaluate it with a sample application., Comment: Extended version of "Building a Polyglot Data Access Layer for a Low-Code Application Development Platform", to appear in conference DAIS'20

Background Studies have demonstrated an association between anti-TNF/immunomodulator agents used in inflammatory bowel disease (IBD) and impaired hepatitis B virus (HBV) vaccine immunogenicity, but little data exist on whether specific medication types affect protective HBsAb titers. Our aim was to analyze this association. Methods This is a retrospective cohort study. Inclusion criteria: age ≥18, diagnosis of Crohn's disease (CD) or ulcerative colitis (UC), previous HBV vaccination series and/or ≥1 positive HBsAb, and record of IBD therapy in 6 months before titer level. Patients were stratified based upon medication exposures: anti-TNF, immunomodulator, combination anti-TNF and immunomodulatory, and a reference arm. Titer levels following vaccination and specific medication types given in the 6 months before titer were recorded. Seroprotection was defined as HBsAb ≥10 IU/l and ≥100 IU/l. Results The study cohort (N = 391) was 70.8% white, 51.4% female and 64.2% had CD and 35.8% had UC. The mean age was 45.8 years. A significantly lower percentage of patients exposed to anti-TNF, immunomodulator or dual therapy had titers ≥10 (P < 0.01). Regarding specific medications, only patients exposed to infliximab (P < 0.01) were less likely to have titer levels ≥10, after controlling for other medication exposures, age at titer level, and interval time between vaccination/titer level. This was not found for patients exposed to adalimumab, methotrexate, 6-mercaptopurine, or azathioprine. Conclusions Patients exposed to infliximab were significantly less likely to have protective HBsAb titer levels following vaccination, a trend not seen in patients on adalimumab. Efforts to vaccinate IBD patients against HBV before use of immunomodulators and anti-TNFs, infliximab specifically, and screen periodically thereafter must be reinforced.

Pediatric management of complicated pleural effusion (CPE) remains controversial. Different approaches include antibiotics and chest tube drainage alone or the use of fibrinolitics, videothorascoscopy (VTC) and surgical decortication through thoracotomy.The aim of the present study was to review, evaluate and update technical approach to CPE. We retrospectively reviewed the clinical files of children admitted to the Pediatric Respiratory Ward between 1992 and 2003 with the diagnosis of CPE. Twenty-five patients were included [15 male (60%)]. Mean (±SD) age was 37,4 (±37,0) months. Bacteria were identified in 17/25 (68%) [S. aureus in 6/17 (35%), St. pneumoniae in 5/17 (29%)], 16/17 (94%)in the pleural fluid. Twenty-five children were treated with antibiotics and thoracocentesis (100%). Chest tube drainage was required in 22/25 (88%) with mean (±DP) duration of 14,2 (±7,8) days. Fibrinolitics were employed in 1 only case and surgical decortication in 11/25 (44%). One patient (4%) was submitted to primary VTC. Median length of stay was 30,4 (±15,1) days and no deaths were recorded. Center skills in CPE management are critical on the choice of the technique and the timing of approach. This seems to influence immediate prognosis. Resumo: A abordagem do derrame pleural parapneumónico complicado, em idade pediátrica, permanece controversa. As opções terapêuticas incluem antibioticoterapia e drenagem pleural contínua, instilação intrapleural de fibrinolíticos, videotoracoscopia e toracotomia com descorticação. O objectivo deste estudo foi rever, avaliar e actualizar a abordagem ao derrame pleural complicado. Procedeu-se à revisão retrospectiva dos processos clínicos das crianças internadas na UPP por derrame pleural complicado entre 1992 e 2003. Foram incluídos 25 doentes, com idade média (±DP): 37,4 (± 37,0) meses, sendo 15/25 (60%) do sexo masculino. A identificação do agente foi possível em 17/25 (68%) casos [S. aureus 6/17 (35%), St. pneumoniae 5/17 (29%)], no líquido pleural em 16/17 (94%) casos. Todos os doentes realizaram toracocentese e antibioticoterapia sistémica. A drenagem pleural contínua foi instituída em 22/25 (88%) casos com duração média (±DP): 14,2 (± 7,8) dias; em 1 caso houve instilação de fibrinolíticos intrapleurais e em 11/25 (44%) realizou-se toracotomia com descorticação. Um doente foi submetido a videotoracoscopia primária. A duração média de internamento (±DP) foi de 30,4 (± 15,1) dias e não ocorreram óbitos. A experiência do centro é determinante na abordagem escolhida e na rapidez de actuação. Provavelmente ambas influenciam o prognóstico imediato. Key-words: Pleural effusion, pleural empyema, child, Palavras-chave: Derrame pleural, empiema pleural, criança

Tese de doutoramento em Ciências e Tecnologias da Informação, apresentada ao Departamento de Engenharia Informática da Faculdade de Ciências e Tecnologia da Universidade de Coimbra Technological devices increasingly become smaller, more mobile, powerful and efficient. However, each time we have to hurdle through unintuitive menus, errors and incompatibilities we become stressed by our technology. As first put forward by the renowned computer scientist Mark Weiser, the ultimate form of computers may be an extension of our subconscious. The ideal computer would be capable of truly understanding people's unconscious actions and desires. Instead of humans adapting to technology and learning how to use it, it would be technology that would adapt to the disposition and uniqueness of each human being. This thesis focuses on the realm of Human-in-the-loop Cyber-Physical Systems (HiTLCPSs). HiTLCPSs infer the users’ intents, psychological states, emotions and actions, using this information to determine the system's behavior. This involves using a large variety of sensors and mobile devices to monitor and evaluate human nature. Therefore, this technology has strong ties with wireless sensor networks, robotics, machine-learning and the Internet of Things. In particular, our work focuses on the usage of smartphones within these systems. It begins by describing a framework to understand the principles and theory of HiTLCPSs. It provides some insights into current research being done on this topic, its challenges, and requirements. Another of the thesis' objectives is to present our innovative taxonomy of human roles, where we attempt to understand how a human may interact with HiTLCPSs and how to best explore this resource. This thesis also describes concrete examples of the practical usage of HiTL paradigms. As such, we included a comprehensive description of our research work and associated prototypes, where the major theoretical concepts behind HiTLCPS were applied and evaluated to specific scenarios. Finally, we discuss our personal view on the future and evolution of these systems. A tecnologia tem vindo a tornar-se cada vez mais pequena, móvel, poderosa e eficiente. No entanto, lidar com menus pouco intuitivos, erros, e incompatibilidades, causa frustração aos seus utilizadores. Segundo o reconhecido cientista Mark Weiser, os computadores do futuro poderão vir a existir como se fossem uma extensão do nosso subconsciente. O computador ideal seria capaz de entender, em toda a sua plenitude, as ações e os desejos inconscientes dos seres humanos. Em vez de serem os humanos a adaptarem-se à tecnologia e a aprender a usá-la, seria a tecnologia a aprender a adaptar-se à disposição e individualidade de cada ser humano. Esta tese foca-se na área dos Human-in-the-loop Cyber-Physical Systems (HiTLCPSs). Os HiTLCPSs inferem as intenções, estados psicológicos, emoções e ações dos seus utilizadores, usando esta informação para determinar o comportamento do sistema ciber-físico. Isto envolve a utilização de uma grande variedade de sensores e dispositivos móveis que monitorizam e avaliam a natureza humana. Assim sendo, esta tecnologia tem fortes ligações com redes de sensores sem fios, robótica, algoritmos de aprendizagem de máquina e a Internet das Coisas. Em particular, o nosso trabalho focou-se na utilização de smartphones dentro destes sistemas. Começamos por descrever uma estrutura para compreender os princípios e teoria associados aos HiTLCPSs. Esta análise permitiu-nos adquirir alguma clareza sobre a investigação a ser feita sobre este tópico, e sobre os seus desafios e requisitos. Outro dos objetivos desta tese é o de apresentar a nossa inovadora taxonomia sobre os papeis do ser humano nos HiTLCPSs, onde tentamos perceber as possíveis interações do ser humano com estes sistemas e as melhores formas de explorar este recurso. Esta tese também descreve exemplos concretos da utilização prática dos paradigmas HiTL. Desta forma, incluímos uma descrição do nosso trabalho experimental e dos protótipos que lhe estão associados, onde os conceitos teóricos dos HiTLCPSs foram aplicados e avaliados em diversos casos de estudo. Por fim, apresentamos a nossa perspetiva pessoal sobre o futuro e evolução destes sistemas. Fundação Luso-Americana para o Desenvolvimento FP7-ICT-2007-2 GINSENG project iCIS project (CENTRO-07-ST24-FEDER-002003) SOCIALITE project (PTDC/EEI-SCR/2072/2014)