Hospital de Santa Maria

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    2133 research outputs found

    Frontotemporal dementia: neuroanatomical correlates of an atypical presentation

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    Frontotemporal dementia (FTD) is a heterogeneous group of disorders characterised by frontal and temporal lobes atrophy. Three different clinical subtypes are recognised: behavioural variant, progressive non-fluent aphasia and semantic dementia. Neuroanatomical associations in a diffuse neurodegenerative disease such as FTD should be interpreted carefully; however, each FTD subtype has provided a clinical model that has contributed immensely to our understanding of clinical/neuroanatomical relationships. This case report and recent studies suggest that neuroanatomical findings concerning face-processing mechanisms in FTD can identify the brain regions that are critical for face processing. As seen in this case, right fusiform gyrus atrophy seems to be implied in the aetiology of prosopagnosia

    Infecção periprotética da anca e joelho: algumas recomendações

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    A infecção periprotética representa uma das complicações mais graves da cirurgia reconstrutiva ortopédica. Estima-se que a incidência da infecção periprotética seja de 1% em artroplastias primárias da anca, de 2% em artroplastias primárias do joelho e de 5% nas artroplastias de revisão da anca e joelho. O objectivo central deste trabalho foi apresentar algumas das recomendações mais importantes no âmbito da prevenção, diagnóstico e tratamento da infecção periprotética de acordo com uma revisão bibliográfica actual da literatura ortopédica internacional. Na falta de evidência científica inequívoca as recomendações aqui descritas não são, por isso, vinculativas. Todavia, são de importância capital na condição de linha orientadora para a abordagem da infecção periprotética, sendo certo que são necessários mais estudos com um maior tempo de recuo para se chegar a conclusões mais consistentes

    Why should we screen for perinatal depression? Ten reasons to do it

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    In this paper we review some of the best available evidence to argue that screening for perinatal depression should be systematically conducted since pregnancy. Our view is organized in ten topics: (1) perinatal depression high prevalence; (2) its potential negative consequences, including maternal, conjugal, foetal, infantile, and child effects; (3) its under-detection and treatment; (4) its stigma; (5) the professionals and women misconceptions related to perinatal depression; (6) the availability of valid and short self-report screening instruments for perinatal depression and (7) their acceptability; (8) the increase in recognition, diagnosis, and treatment rates in comparison with routine practice; (9) the opportunity, given the large number of contacts that women have with health professionals in the perinatal period; and (10) perinatal depression screening potential cost-effectiveness

    Left ventricular false aneurysm characterized by cardiovascular magnetic resonance and late enhancement technique

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    Left ventricular pseudoaneurysm is a rare complication of myocardial infarction. Differentiation between true and false aneurysms, although difficult, is crucial, because of the different treatment options they entail. We present a rare case of a giant pseudoaneurysm of the left ventricle related with a previous myocardial infarction. The present case highlights the pivotal role of magnetic resonance imaging for differential diagnosis between these entities

    Left Ventricular Diastolic Function in Type 2 Diabetes Mellitus and the Association With Coronary Artery Calcium Score: A Cardiac MRI Study

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    OBJECTIVE: The purpose of this study was to compare cardiac MRI-derived parameters of left ventricular (LV) diastolic function between uncomplicated type 2 diabetes mellitus (DM2) and normoglycemic control subjects and to evaluate whether these parameters of LV diastolic function are related to coronary atherosclerosis. SUBJECTS AND METHODS: We prospectively studied 41 subjects with DM2 and 21 normoglycemic control subjects (30 women and 32 men; mean age, 57.2 ± 7.1 [SD] years) with no evidence of overt cardiovascular disease. We used cardiac MRI to measure LV volumes, LV peak filling rate (PFR), and transmitral flow and CT to determine coronary artery calcium scores. RESULTS: Absolute values of the peak filling rate (PFR) were significantly lower in DM2 patients than in control subjects (mean ± SD, 293.2 ± 51.7 vs 375.7 ± 102.8 mL/s, respectively; p < 0.001). Mitral peak E velocities (mean ± SD, 42.8 ± 10.7 vs 48.8 ± 10.4 cm/s; p = 0.040) and peak E velocity-to-peak A velocity ratios (0.88 ± 0.3 vs 1.1 ± 0.3; p = 0.002) were also lower in DM2 patients compared with control subjects. DM2 patients with coronary artery calcification showed a lower PFR normalized to stroke volume (SV) (mean ± SD, 4.4 ± 1.0 vs 5.3 ± 1.4, respectively; p = 0.038) and lower mitral peak E velocities (40.1 ± 11.3 vs 48.0 ± 7.3 cm/s; p = 0.024) than DM2 patients without coronary calcification. PFR normalized to SV was independently associated with the presence of coronary artery calcification (β = -1.5, p = 0.005). CONCLUSION: DM2 decreases cardiovascular MRI-derived parameters of LV diastolic function. Patients with DM2 and coronary atherosclerosis show a more impaired LV diastolic function than patients without coronary atherosclerosis

    A rare manifestation of neonatal alloimmune thrombocytopaenia

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    Neonatal alloimmune thrombocytopaenia (NAIT) results from a fetomaternal incompatibility with maternal sensitisation against a fetal human platelet antigen (HPA) and antibodies transfer to the fetal circulation, leading to platelet destruction. The clinical presentation is variable and isolated intraocular haemorrhage is rare. We present the case of a male newborn, with intrauterine growth restriction, born at 29 weeks due to pre-eclampsia. He presented proptosis of the left eye, hyphaema and elevated intraocular pressure, with no other signs of haemorrhage. Severe thrombocytopaenia was found (27×10(9)/L). Perinatal infection and maternal thrombocytopaenia were excluded. Positive anti-HPA-1a and antihuman leucocyte antigen class I alloantibodies were found in the mother. Platelet crossmatch between the father's platelets and mother's plasma was positive. Platelet transfusions and intravenous immunoglobulin were given with favourable response. This case highlights an unusual presentation of NAIT, which should be suspected in the presence of severe thrombocytopaenia in the first 24-72 h of life

    MÓDULO 9 - 2º Curso de Formação para Internos 2013 - 2014: Hematologia e Oncologia -

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    NOD2 gene mutations in ulcerative colitis: useless or misunderstood?

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    PURPOSE: NOD2 mutations have been linked to an increased risk of Crohn's disease and to some of its phenotypes. The association between NOD2 mutations and susceptibility to ulcerative colitis (UC) remains somewhat controversial and potential correlations between these mutations and UC phenotype have not been studied. AIM: To assess whether NOD2 mutations are a risk factor for UC in Portugal and if there are any genotype-phenotype correlations in these patients. METHODS: The three main NOD2 mutations were searched in 200 patients with UC and in 202 healthy controls. RESULTS: NOD2 mutations were present in 28 patients with UC (14.0 %) and in 27 controls (13.4 %) (p = 0.853). Mutation carriers were more likely to receive steroids during the first year of disease than non-carriers (54.2 % vs. 29.6 %, p = 0.018) and among these patients the need for intravenous administration was more frequent in those with the R702W polymorphism (90.0 % vs. 45.5 %, p = 0.014). In patients with severe colitis admitted for intravenous steroids, a greater proportion of mutation carriers was considered intravenous-steroid refractory and required salvage therapy (90.0 % vs. 38.1 %, p = 0.004). Patients with NOD2 mutation were submitted to colectomy more frequently than non-carriers (17.9 % vs. 4.1 %. p = 0.015). No correlation with the need for immunosuppressants/immunomodulators was found. CONCLUSIONS: In the Portuguese population, NOD2 mutations do not increase the risk of UC but are associated with a more aggressive course including greater need of steroids in the first year, increased incidence of intravenous-steroid refractoriness and a higher colectomy rate

    Brain activation of the defensive and appetitive survival systems in obsessive compulsive disorder

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    Several studies have shown that basic emotions are responsible for a significant enhancement of early visual processes and increased activation in visual processing brain regions. It may be possible that the cognitive uncertainty and repeated behavioral checking evident in Obsessive Compulsive Disorder (OCD) is due to the existence of abnormalities in basic survival circuits, particularly those associated with the visual processing of the physical characteristics of emotional-laden stimuli. The objective of the present study was to test if patients with OCD show evidence of altered basic survival circuits, particularly those associated with the visual processing of the physical characteristics of emotional stimuli. Fifteen patients with OCD and 12 healthy controls underwent functional magnetic resonance imaging acquisition while being exposed to emotional pictures, with different levels of arousal, intended to trigger the defensive and appetitive basic survival circuits. Overall, the present results seem to indicate dissociation in the activity of the defense and appetitive survival systems in OCD. Results suggest that the clinical group reacts to basic threat with a strong activation of the defensive system mobilizing widespread brain networks (i.e., frontal, temporal, occipital-parietal, and subcortical nucleus) and blocking the activation of the appetitive system when facing positive emotional triggers from the initial stages of visual processing (i.e., superior occipital gyrus)

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