Abdominoplasty and Thoraco-Epigastric Flaps for Large Anterior Trunk Defects after Dermatofibrosarcoma Protuberans Wide Resection: Two Illustrative Cases

INTRODUCTION: Excision of large dermatofibrosarcoma protuberans in the anterior aspect of the trunk often results in large surgical defects that frequently dictate the need for microsurgical reconstruction. However, this option is not always available. PRESENTATION OF CASE: The authors describe two patients with very large anterior trunk dermatofibrosarcoma protuberans: one in the epigastric region and the other in the hypogastric region. In the patient with the hypogastric tumor, a classical abdominoplasty flap associated with umbilical transposition was used to cover the skin defect after muscle and fascial plication, and placement of a polypropylene mesh. In the patient with the epigastric tumor, a synthetic mesh was also placed, and the skin and subcutaneous defect was reconstructed with a reverse abdominoplasty flap and two thoraco-epigastric flaps. In both cases, complete closure was possible without immediate or late complications. DISCUSSION: The local options described in this paper present several potential advantages compared to microsurgical reconstruction, namely they are easier and faster to perform and teach; they provide a good skin color and texture match; they are not associated with distant donor site morbidity; follow-up is usually less cumbersome; the post-operative hospital stay tends to be shorter; they are less costly; they are less prone to complete failure. CONCLUSION: The authors believe that these two patients clearly show that local flaps, although frequently neglected, continue to be valid options for reconstructing large anterior trunk defects, even in the current era of microsurgery enthusiasm.

Estenose Aórtica Subvalvular após Correcção Anatómica da Transposição das Grandes Artérias: Caso Clínico

Os autores descrevem o caso clínico de uma criança do sexo masculino, com 12 anos de idade, com diagnóstico pós-natal de transposição das grandes artérias com comunicação interventricular, submetida a switch arterial e encerramento da comunicação interventricular. Durante o seguimento pós-operatório detectou-se um obstáculo entre o ventrículo esquerdo e a aorta ascendente que foi, inicialmente, de grau ligeiro. No último ano começou a referir sintomatologia (cansaço para esforços moderados). Na avaliação efectuada detectou-se uma estenose aórtica subvalvular grave, tendo o doente sido submetido a tratamento cirúrgico, com bom resultado. A estenose aórtica subvalvular é uma complicação rara, que tem sido descrita em estudos de follow-up de switch arterial, sobretudo nas situações em que o defeito primário é a transposição das grandes artérias com comunicação interventricular.

Stenosis of the Branches of the Neopulmonary Artery after the Arterial Switch Operation: a Cardiac Magnetic Resonance Imaging Study

Background : The neonatal arterial switch operation (ASO) is now the standard of care for children born with transposition of the great arteries. Stenosis of the neopulmonary artery on long‑term follow up is a known complication. Methods : We performed a retrospective analysis of eleven patients who underwent a cardiac magnetic resonance imaging (MRI) due to echocardiographic evidence suggestive of stenosis of the neopulmonary artery or its branches (mean estimated Doppler gradient 48 mmHg, min 30 mmHg, max 70 mmHg). A comprehensive evaluation of anatomy and perfusion was done by cardiac MRI. Results : The branches of the neopulmonary artery (neo PA) showed decreased caliber in three patients unilaterally and in two patients, bilaterally. Magnetic resonance (MR) perfusion studies showed concomitant decreased flow, with discrepancy between the two lungs of 35/65% or worse, only in the three patients with unilateral obstruction, by two different MR perfusion methods. Conclusions : Cardiac MR can be used as a comprehensive non‑invasive imaging technique to diagnose stenosis of the branches of the neopulmonary after the ASO, allowing evaluation of anatomy and function of the neoPA, its branches, and the differential perfusion to each lung, thus facilitating clinical decision making.

Decision-making process in radiology: the magnetic resonance example in the TA context

Based on the report for “Project IV” unit of the PhD programme on Technology Assessment (Doctoral Conference) at Universidade Nova de Lisboa (December 2011). This thesis research has the supervision of António Moniz (FCT-UNL and ITAS-KIT) and Michael Decker (Karlsruhe Institute of Technology-ITAS). Other members of the thesis committee are Carlos Alberto da Silva (University of Évora), José Maria de Albuquerque (Institute of Welding and Quality), Lotte Steuten (University of Twente), Mário Forjaz Secca (FCT-UNL) and Nelson Chibeles Martins (FCT-UNL).

Technology governance in radiology: the example of magnetic resonance imaging

Based on the report for the unit “Project III” of the PhD programme on Technology Assessment in 2011. The unit was supervised by Prof. António B. Moniz (FCT-UNL).

A Directiva 2011-24-UE, relativa ao exercício dos direitos dos doentes em matéria de cuidados transfronteiriços : análise : potenciais repercussões no direito de acesso aos cuidados de saúde transfronteiriços em Portugal

RESUMO - 1. INTRODUÇÃO: Ao longo dos tempos, assistiu-se a um aumento da importância da Saúde Pública na Comunidade Europeia, mas só há relativamente pouco tempo teve o merecido lugar de destaque à luz da legislação comunitária. Neste contexto e com a adopção do Programa Europeu de Saúde Pública, surge a necessidade de actualizar o pensamento nesta área. Assim, é identificada uma oportunidade para formular uma estratégia, que seja passível de reduzir desigualdades e que também em compreenda as necessidades de saúde. Com o expandir da questão e com o propósito de reduzir as desigualdades, surge a Directiva 2011/24/UE, que visa regulamentar os direitos dos doentes em matéria de cuidados transfronteiriços. 2. OBJETIVO: Este trabalho apresenta como objetivo primordial analisar a Directiva 2011/24/UE, bem como a Lei n.º 52/2014, de 25 de Agosto, e identificar as principais barreiras, ao exercício do direito de acesso aos cuidados de saúde transfronteiriços, pelos beneficiários do SNS em Portugal, derivadas da aplicação de tais instrumentos legais. 3. METODOLOGIA: Foi utilizada uma abordagem analítica e documental, baseada na metodologia qualitativa. 4. CONCLUSÕES: As principais barreiras ao direito de acesso aos cuidados de saúde transfronteiriços, para os beneficiários do SNS em Portugal, são de ordem financeira, linguística e cultural, informacional, de mobilidade física, de proximidade geográfica, de carácter administrativo e de continuidade dos cuidados. A transposição da Directiva 2011/24/UE para o quadro jurídico português resulta essencialmente em iniquidades no âmbito do acesso aos cuidados de saúde transfronteiriços.

Concomitant involvement of the small intestine and the distal esophagus in an infant with massive necrotizing enterocolitis

Necrotizing enterocolitis is a disease of the newborn that may involve the small intestine and/or the colon, and the stomach. To our knowledge, massive necrosis of the small intestine with concomitant involvement of the esophagus has never been reported. A case of a 6-month-old boy with necrotizing enterocolitis and pan-necrosis of the small intestine, cecum, and the lower third of the esophagus is presented. After 70 days of treatment, intestinal transit was established by an anastomosis between the first centimeter of jejunum and the ascending colon. Finally, esophageal transit was established by a total gastric transposition with cervical esophagogastric anastomosis. The patient was maintained under total parenteral nutrition, and after 19 months he developed fulminant hepatic failure due to parenteral nutrition; he then underwent combined liver and small bowel transplantation. After 2 months, the patient died due to undefined neurologic complications, probably related to infection or immunosuppressive therapy.

Mark Rothko : Da origem mítica à refundação artística do mundo

Fenómeno assinalável no nosso século foi a emergência da chamada “arte global” (Belting), dando conta da crise do “mundo da arte” e a disseminação generalizada das práticas artísticas. Neste contexto a obra de Rothko ganha uma força inesperada. Sendo usualmente inscrito no “modernismo” com os seu valores de pureza e especificidade do meio, neste caso a pintura, a nossa investigação revela que o gesto Rothkoniano excede largamente esta representação, que levaria a distinguir radicalmente entre uma fase mítica e surrealista, uma fase abstracionista dos “colour field” e finalmente uma fase sublime das pinturas da Capela Ecuménica de Rothko. Existe uma continuidade evidente que remete para uma geoestética, onde a terra e a sua habitabilidade desempenham um papel crucial. Daí a necessidade de inscrever a obra de Rothko na geofilosofia contemporânea, tal com foi delineada por Gilles Deleuze e Félix Guattari. Procedeu-se, assim, a uma análise crítica da obra e da estética de Rothko, que profeticamente, mas inconscientemente, parece abrir o caminho para o pensamento de uma arte da terra. Trata-se de uma linha de continuidade que atravessa toda a obra de Rothko, refletindo uma picturação do mundo e a vontade de criar de um mundo pictórico e poético, reduzido a elementos mínimos, pós-figurativos mas onde se reconhece a incidência dos motivos como frame e abertura, linha de horizonte e pórticos e passagens. Num segundo momento, explora-se essa dimensão “inconsciente” num projeto artístico pessoal, que se desdobra em abordagens picturais, de pintura, de instalação e de vídeo, que denominamos por “A Terra como Acontecimento”. Este projeto prolonga o esforço Rothkoniano, ao mesmo tempo que o altera profundamente, nomeadamente pelo uso dos materiais, pela mutação no uso da cor, bem como pela maneira como os elementos figurativos são radicalmente alterados pela mera transposição da perspetiva usada. Se a ressonância rothkoniana está bem presente, não menos presente está a intenção de um confronto dialogante com a Obra de Mark Rothko. Aquilo que neste importante artista, era o inconsciente, marcado pelo mito e teologia, pela delimitação da linha de horizonte, bem clássica, e, acima de tudo, pela sua verticalidade marcadamente teológica, “A Terra como Acontecimento” é a matéria que é profundamente radicalizada, bem como a lógica concetual, a qual é preferentemente circular, sem orientação absoluta, e incompleta, o que implica uma outra visão da “abertura”/”fecho”, tão essencial na obra de Rothko. Desta investigação espera-se um contributo significativo para os debates atuais sobre a arte na contemporaneidade.

A liberalização do mercado energético e o papel das entidades reguladoras

The liberalisation of the energy market goes back to the 1990s, when it was impelled by the European legislator. Since then, three legislative packages, temporarily successive, were approved. Those packages contained the measures to be implemented in order to deepen the internal energy market. Besides the opening up of several national markets to competition, the European legislator aimed the creation of a real internal energy market within the European Union. The unbundling regime was one of the most important steps with respect to the liberalisation process. The introduction of these rules ensured independence to the various market operators. A real and effective right of choice was granted to the consumers so they may choose their electricity and natural gas supplier. Therefore, the activity of comercialisation is subject to competition. However, some activities of the electricity’s and natural gas’ chain of value, namely the activities of transportation and distribution, were kept under regulation rules. Even though it may seem odd, the assignment of important competences and strong powers to a regulatory authority was essential in order to achieve the liberalisation process’ goals. Electricity and natural gas are essential public goods; therefore the market operators are legally bound to public service obligations, such as the security, the universality and the continuity of the supply. The performance of these obligations may become, in some cases, unprofitable for those operators. For such reason, the protection of the consumers’ rights shall only be properly defended if there is a regulatory authority that monitors the behaviour of the operators and sanctions the failure to comply with the public service obligations. Portugal, as a Member State of the European Union, transposed into the national legal order the European directives concerning the liberalisation process. This transposition has caused radical changes to the electricity and natural gas’ national markets. The Entidade Reguladora dos Serviços Energéticos also suffered various mutations in order to keep up with the regulatory demands regarding the liberalisation process.

Os processos criativos na aprendizagem da técnica do violoncelo no ensino vocacional de música

Relatório de estágio de mestrado em Ensino de Música

As agências europeias no Direito Administrativo Europeu

Dissertação de mestrado em Direito Administrativo

A linear algebra approach to OLAP

Inspired by the relational algebra of data processing, this paper addresses the foundations of data analytical processing from a linear algebra perspective. The paper investigates, in particular, how aggregation operations such as cross tabulations and data cubes essential to quantitative analysis of data can be expressed solely in terms of matrix multiplication, transposition and the Khatri–Rao variant of the Kronecker product. The approach offers a basis for deriving an algebraic theory of data consolidation, handling the quantitative as well as qualitative sides of data science in a natural, elegant and typed way. It also shows potential for parallel analytical processing, as the parallelization theory of such matrix operations is well acknowledged.

Redefining the MED13L syndrome

Congenital cardiac and neurodevelopmental deficits have been recently linked to the mediator complex subunit 13-like protein MED13L, a subunit of the CDK8-associated mediator complex that functions in transcriptional regulation through DNA-binding transcription factors and RNA polymerase II. Heterozygous MED13L variants cause transposition of the great arteries and intellectual disability (ID). Here, we report eight patients with predominantly novel MED13L variants who lack such complex congenital heart malformations. Rather, they depict a syndromic form of ID characterized by facial dysmorphism, ID, speech impairment, motor developmental delay with muscular hypotonia and behavioral difficulties. We thereby define a novel syndrome and significantly broaden the clinical spectrum associated with MED13L variants. A prominent feature of the MED13L neurocognitive presentation is profound language impairment, often in combination with articulatory deficits.

Investigations on relational control of time perception

Tese de Doutoramento em Psicologia Básica

Dynamic three-dimensional reconstruction of the heart by transesophageal echocardiography

OBJECTIVE: To evaluate echocardiography accuracy in performing and obtaining images for dynamical three-dimensional (3D) reconstruction. METHODS: Three-dimensional (3D) image reconstruction was obtained in 20 consecutive patients who underwent transesophageal echocardiography. A multiplanar 5 MHz transducer was used for 3D reconstruction. RESULTS: Twenty patients were studied consecutively. The following cardiac diseases were present: valvar prostheses-6 (2 mitral, 2 aortic and 2 mitral and aortic); mitral valve prolapse- 3; mitral and aortic disease - 2; aortic valve disease- 5; congenital heart disease- 3 (2 atrial septal defect- ASD - and 1 transposition of the great arteries -TGA); arteriovenous fistula- 1. In 7 patients, color Doppler was also obtained and used for 3D flow reconstruction. Twenty five cardiac structures were acquired and 60 reconstructions generated (28 of mitral valves, 14 of aortic valves, 4 of mitral prostheses, 7 of aortic prostheses and 7 of the ASD). Fifty five of 60 (91.6%) reconstructions were considered of good quality by 2 independent observers. The 11 reconstructed mitral valves/prostheses and the 2 reconstructed ASDs provided more anatomical information than two dimensional echocardiography (2DE) alone. CONCLUSION: 3D echocardiography using a transesophageal transducer is a feasible technique, which improves detection of anatomical details of cardiac structures, particularly of the mitral valve and atrial septum.