João Bexiga Martins Pisco (1941-2019)

Conhecido pela maioria do meio Médico Nacional como Professor Pisco, marcou a Medicina Portuguesa e mais concretamente a Radiologia Nacional e Internacional com o seu trabalho, deixando um extenso legado. Diferenciou-se na área da Radiologia de Intervenção, à qual dedicou grande parte da sua vida profissional e pessoal. Fez o Internato da Especialidade de Radiologia nos Hospitais Civis de Lisboa de 1972 a 1975. Em seguida fez um Fellowship em Radiologia Cardíaca no National Heart Hospital de Londres de 1975 a 1976. Posteriormente perseguiu uma carreira internacional, ocupando os cargos de Fellow, Instrutor e Assistente de Radiologia na Universidade de Louisiana nos EUA entre 1977 e 1980. Esta ligação aos EUA marcou toda a sua carreira, na forma de abordar a Medicina, o Ensino e a Investigação. Quem conheceu e trabalhou com o Professor Pisco sabe que o seu lema era muito americano: “see one, do one, teach one”. Regressou a Portugal na década de 1980 e, desde então, foi um dos pioneiros na implementação da Radiologia de Intervenção a nível nacional. Foram, quase 40 anos a formar Radiologistas de Intervenção em Portugal, sempre disposto a partilhar os seus conhecimentos e competências técnicas em angiografia. Os primeiros 20 anos da carreira nacional foram passados como Director do Serviço de Radiologia do Hospital de Santa Marta, entre 1980 a 1998. Em seguida foi Director do Serviço de Radiologia do Hospital de Pulido Valente, de Outubro de 1998 a Setembro de 2005, quando deixou o Sistema Nacional de Saúde. Assumiu o cargo de Director do Serviço de Angiografia Diagnostica e Terapêutica do Hospital Saint Louis, desde Setembro de 1998 até aos últimos dias de vida, sempre com enorme energia e entusiasmo

Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathways

The risk of developing neurodegenerative diseases increases with age. Although many of the molecular pathways regulating proteotoxic stress and longevity are well characterized, their contribution to disease susceptibility remains unclear. In this study, we describe a new Caenorhabditis elegans model of Machado–Joseph disease pathogenesis. Pan-neuronal expression of mutant ATXN3 leads to a polyQ-length dependent, neuron subtype-specific aggregation and neuronal dysfunction. Analysis of different neurons revealed a pattern of dorsal nerve cord and sensory neuron susceptibility to mutant ataxin-3 that was distinct from the aggregation and toxicity profiles of polyQ-alone proteins. This reveals that the sequences flanking the polyQ-stretch in ATXN3 have a dominant influence on cell-intrinsic neuronal factors that modulate polyQ-mediated athogenesis. Aging influences the ATXN3 phenotypes which can be suppressed by the nregulation of the insulin/insulin growth factor-1-like signaling pathway and activation of heat shock factor-1.This work was supported by grants from Fundacão Ciência eTecnologia (FCT) to P.M. (PTDC/SAU-GMG/64076/2006, PTDC/SAU-GMG/112617/2009, SFRH/BD/27258/2006 to A.T.C., UMINHO/BI/052/2010 to A.J. and SFRH/BD/51059/2010 to A.N.C.), from the National Ataxia Foundation to PM and from the National Institutes of Health (NIGMS, NIA and NINDS) to R.M. This work was also granted by the Hospital San Rafael (Coruna) with the Rafael Hervada prize on Biomedical Research (2010)

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