Browsing by Issue Date, starting with "2018-04"
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- Legionella: Novas Abordagens, Novas NecessidadesPublication . Costa Santiago, R; Ramos, S; Corte-Real, R; Manzano, MJ; Infante, JINTRODUÇÃO: O CHLC desenvolve, desde há vários anos, procedimentos contra o crescimento e proliferação da Legionella, monitorizando e auditando as estruturas e as práticas. De acordo com as recentes orientações e normas emanadas pela DGS, surge a necessidade de criar novos instrumentos para a avaliação do risco e dar resposta às necessidades do Centro Hospitalar. OBJETIVO Identificar os fatores de risco que determinam os pontos críticos para a monitorização da Legionella. MATERIAIS E MÉTODOS: Foram construídas listas e grelhas de observação, com os fatores de risco que determinam os pontos críticos, a partir das quais se observaram as estruturas e práticas do CHLC. Os instrumentos utilizados tiveram como base os documentos publicados pela DGS, IPQ, ECDC, o procedimento multissectorial do CHLC - AMB.102 e os relatórios das auditorias. RESULTADOS: Para a avaliação das condições microbiológicas, os pontos críticos deverão ser aqueles, mais suscetíveis da ocorrência da proliferação da Legionella. Destes, a literatura salienta: Pontos distais das redes prediais Zonas de estagnação de água Idade e complexidade das redes e sistemas Sistemas e equipamentos geradores de aerossóis Após a análise dos pontos críticos foram determinados os fatores de risco que estão associados, nomeadamente: Presença de nutrientes e de biofilmes Zonas suscetíveis a fenómenos de corrosão e incrustação Origem do abastecimento de água Zonas de estagnação de água da rede predial e da água dos sistemas e equipamentos geradores de aerossóis Ausência de biocida na rede predial Tipos de materiais utilizados nas redes de canalização CONCLUSÕES A avaliação do risco permitiu reorganizar os pontos críticos definidos anteriormente e atualizar o conhecimento das condições favoráveis ao crescimento da Legionella na nossa instituição, promovendo a melhoria e atualização do programa do CHLC. Assim, torna-se evidente de que a existência de um cadastro completo e atualizado das infraestruturas, redes, sistemas e equipamentos, incluindo peças desenhadas e memórias descritivas das redes de água fria e quente, das redes dos circuitos de água de aquecimento e arrefecimento e uso terapêutico, é essencial para que a avaliação do risco seja mais proficiente.
- Standardization of Laboratory Lipid Profile Assessment: A Call for Action with a Special Focus on the 2016 ESC/EAS Dyslipidemia Guidelines - Executive Summary: A Consensus Endorsed by the Cardiovascular Risk and Prevention Group of the Portuguese Internal Medicine Society, the Portuguese Atherosclerosis Society, the Portuguese Society of Cardiology, the Portuguese Society of Laboratory Medicine, and the Portuguese Association of Clinical ChemistryPublication . Marques da Silva, P; Sequeira Duarte, J; von Hafe, P; Gil, V; Nunes de Oliveira, J; de Sousa, GEven with improvements in lifestyle interventions, better control of cardiovascular (CV) risk factors, and improvements in CV outcomes, cardiovascular disease (CVD) remains the leading cause of morbidity and mortality in Portugal and Europe. Atherogenic dyslipidemias, particularly hypercholesterolemia, have a crucial causal role in the development of atherosclerotic CVD. The clinical approach to a patient with dyslipidemia requires an accurate diagnosis, based on harmonized and standardized lipid and lipoprotein laboratory assessments. Results and reports of these tests, together with assessment of total CV risk and the respective therapeutic targets, will help ensure that clinical guidelines and good clinical practices are followed, increasing the reliability of screening for lipid disorders, producing more accurate diagnoses and CV risk stratification, and improving CV prevention. To this end, this consensus aims to provide clinicians with practical guidance for the harmonization and standardization of laboratory lipid tests, focusing on the most recent dyslipidemia management guidelines.
- Missense Mutation of TTC7A Mimicking Tricho-Hepato-Enteric (SD/THE) Syndrome in a Patient with Very-Early Onset Inflammatory Bowel DiseasePublication . Farela Neves, J; Afonso, I; Borrego, L; Martins, C; Cordeiro, AI; Neves, C; Lacoste, C; Badens, C; Fabre, ATricho-hepato-enteric syndrome (SD/THE) and Multiple intestinal atresia with combined immune deficiency (MIA-CID) are autosomal recessive disorders that present immunological and gastrointestinal features. There are two different phenotypes of patients with TTC7A mutations: the severe form, caused by null mutations and leading to the classical MIA-CID; and the mild form, caused by missense mutations and leading to predominant features of VEO-IBD, less severe immunological involvement and hair abnormalities. We expand the knowledge about TTC7A deficiency, describing a patient with the mild phenotype of TTC7A deficiency but presenting overlapping features of SD/THE and MIA-CID: intestinal atresia and inflammatory bowel disease evocative of MIA-CID, but also dental abnormalities, huge forehead, liver abnormalities, autoimmune thyroiditis and hypogammaglobulinemia, evocative of SD/THE.
- Orbital Metastasis from an Occult Breast Carcinoma (T0, N1, M1)Publication . Pinto Proença, R; Fernandes, J; Burnier, M; Proença, RThe authors report a case of an orbital metastasis from an occult breast carcinoma. A 66-year-old woman presented with a growing left orbital tumour. Orbital CT scan was consistent with lymphoma. However, ocular pathology revealed small neoplastic cells showing an 'indian file pattern' suggestive of metastatic carcinoma and immunohistochemistry was positive for CK7, CK CAM5.2 and oestrogen receptor. A systemic evaluation was then performed with mammogram, breast ultrasound and MRI considered normal. An exhaustive systemic evaluation revealed multiple bone lesions, a right axillary lymph node lesion, which presented the same pattern on pathology and immunohistochemistry, with no evidence of a primary tumour. A diagnosis of a metastatic lobular carcinoma of the breast (T0, N1, M1) was made and the patient was started on chemotherapy and adjuvant hormonal therapy.
- Tinea Faciei in a Central Portuguese Hospital: A 9-Year SurveyPublication . Borges, A; Brasileiro, A; Galhardas, C; Apetato, MTinea faciei is a relatively uncommon dermatophytosis that affects the glabrous skin of the face. The aim of this study was to analyse the epidemiologic, clinical and mycological features of tinea faciei cases diagnosed at the Dermatology and Venereology Department of Hospital Santo António dos Capuchos (Lisbon, Portugal). Consecutive cases diagnosed between 2008 and 2016 were studied retrospectively. A total of 72 tinea faciei cases have been diagnosed, involving 37 male and 35 female, aged between 8 months and 86 years. The majority were observed in patients younger than 12 years of age (59.72%). Anthropophilic isolates (mainly Microsporum audouinii, Trichophyton soudanense and Trichophyton rubrum) accounted for 75.7% of the identified dermatophytes. One quarter of the patients were also affected by dermatophytosis in other areas, such as the scalp. Only 10 cases were previously treated with topical steroids due to misdiagnosis. Most patients were treated with topical and systemic antifungal therapy with total resolution of skin lesions, without relapse or side effects. In contrast to other European studies, anthropophilic dermatophytes were the main causative agents of tinea faciei. As previously described to tinea capitis, this result is probably due to changes in the epidemiology of dermatophytes worldwide.
- Response and Outcomes of Cardiac Resynchronization Therapy in Patients with Renal DysfunctionPublication . Ilhão Moreira, R; Cunha, PS; Rio, P; Nogueira da Silva, M; Branco, LM; Galrinho, A; Feliciano, J; Soares, R; Cruz Ferreira, R; Oliveira, MMPURPOSE: Renal dysfunction is often associated with chronic heart failure, leading to increased morbi-mortality. However, data regarding these patients after cardiac resynchronization therapy (CRT) is sparse. We sought to evaluate response and long-term mortality in patients with heart failure and renal dysfunction and assess renal improvement after CRT. METHODS: We analyzed 178 consecutive patients who underwent successful CRT device implantation (age 64 ± 11 years; 69% male; 92% in New York Heart Association (NYHA) functional class ≥ III; 34% with ischemic cardiomyopathy). Echocardiographic response was defined as ≥ 15% reduction in left ventricular end-systolic diameter and clinical response as a sustained improvement of at least one NYHA functional class. Renal dysfunction was defined as an estimated glomerular filtration rate (eGFR) below 60 mL/min/1.73 m2. RESULTS: Renal dysfunction was present in 34.7%. Renal dysfunction was not an independent predictor of echocardiographic response (OR 1.109, 95% CI 0.713-1.725, p 0.646) nor clinical response (OR 1.003; 95% CI 0.997-1.010; p 0.324). During follow-up (mean 55.2 ± 32 months), patients with eGFR < 60mL/min/1.73 m2 had higher overall mortality (HR 4.902, 95% CI 1.118-21.482, p 0.035). However, clinical response in patients with renal dysfunction was independently associated with better long-term survival (HR 0.236, 95% CI 0.073-0.767, p 0.016). Renal function was significantly improved in patients who respond to CRT (ΔeGFR + 5.5 mL/min/1.73 m2 at baseline vs. follow-up, p 0.049), while this was not evident in nonresponders. Improvements in eGFR of at least 10 mL/min/1.73 m2 were associated with improved survival in renal dysfunction patients (log-rank p 0.036). CONCLUSION: Renal dysfunction was associated with higher long-term mortality in CRT patients, though, it did not influence echocardiographic nor functional response. Despite worse overall prognosis, renal dysfunction patients who are responders showed long-term survival benefit and improvement in renal function following CRT.
- Complete Atrioventricular Block in Acute Coronary Syndrome: Prevalence, Characterisation and Implication on OutcomePublication . Aguiar Rosa, S; Timóteo, AT; Ferreira, L; Carvalho, R; Oliveira, MM; Cunha, PS; Viveiros Monteiro, A; Portugal, G; Almeida Morais, L; Daniel, P; Cruz Ferreira, RPURPOSE: The aim was to characterise acute coronary syndrome patients with complete atrioventricular block and to assess the effect on outcome. METHODS: Patients admitted with acute coronary syndrome were divided according to the presence of complete atrioventricular block: group 1, with complete atrioventricular block; group 2, without complete atrioventricular block. Clinical, electrocardiographic and echocardiographic characteristics and prognosis during one year follow-up were compared between the groups. RESULTS: Among 4799 acute coronary syndrome patients admitted during the study period, 91 (1.9%) presented with complete atrioventricular block. At presentation, group 1 patients presented with lower systolic blood pressure, higher Killip class and incidence of syncope. In group 1, 86.8% presented with ST-segment elevation myocardial infarction (STEMI), and inferior STEMI was verified in 79.1% of patients in group 1 compared with 21.9% in group 2 ( P<0.001). Right ventricular myocardial infarction was more frequent in group 1 (3.3% vs. 0.2%; P<0.001). Among patients who underwent fibrinolysis complete atrioventricular block was observed in 7.3% in contrast to 2.5% in patients submitted to primary percutaneous coronary intervention ( P<0.001). During hospitalisation group 1 had worse outcomes, with a higher incidence of cardiogenic shock (33.0% vs. 4.5%; P<0.001), ventricular arrhythmias (17.6% vs. 3.6%; P<0.001) and the need for invasive mechanical ventilation (25.3% vs. 5.1%; P<0.001). After a propensity score analysis, in a multivariate regression model, complete atrioventricular block was an independent predictor of hospital mortality (odds ratio 3.671; P=0.045). There was no significant difference in mortality at one-year follow-up between the study groups. CONCLUSION: Complete atrioventricular block conferred a worse outcome during hospitalisation, including a higher incidence of cardiogenic shock, ventricular arrhythmias and death.
- Should We Continue to Measure Endometrial Thickness in Modern-Day Medicine? The Effect on Live Birth Rates and Birth WeightPublication . Costa Ribeiro, V; Santos-Ribeiro, S; De Munck, N; Drakopoulos, P; Polyzos, N; Schutyser, V; Verheyen, G; Tournaye, H; Blockeel, CThe evaluation of endometrial thickness (EMT) is still part of standard cycle monitoring during IVF, despite the lack of robust evidence of any value of this measurement to predict little revalidation in contemporary medical practice; other tools, however, such as endocrine profile monitoring, have become increasingly popular. The aim of this study was to reassess whether EMT affects the outcome of a fresh embryo transfer in modern-day medicine, using a retrospective, single-centre cohort of 3350 IVF cycles (2827 women) carried out between 2010 and 2014. In the multivariate regression analysis, EMT was non-linearly associated with live birth, with live birth rates being the lowest with an EMT less than 7.0 mm (21.6%; P < 0.001) and then between 7.0 mm and 9.0 mm (30.2%; P = 0.008). An EMT less than 7.0 mm was also associated with a decrease in neonatal birthweight z-scores (-0.40; 95% CI -0.69 to -0.12). In conclusion, these results reaffirm the use of EMT as a potential prognostic tool for live birth rates and neonatal birthweight in contemporary IVF, namely when considered together with other ovarian stimulation monitoring methods, such as the late-follicular endocrine profile.
- Pediatric Patient Blood Management Program in Scoliosis Surgery: Net Clinical BenefitsPublication . Pardal, R; Rincon, F; Gouveia, S; Ferro, B; Bargado, F; Campos, MM; Ramos, SH; Espírito Santo, D
- Clinical, Laboratorial and Immunological Aspects of Severe Malaria in Children from Guinea-BissauPublication . Domingos, J; Casimiro, A; Portugal-Calisto, D; Varandas, L; Nogueira, F; Silva, M SMalaria is a parasitic disease of which Plasmodium falciparum causes the most severe form of the disease. The immune response against Plasmodium spp. is complex and remains unclear. The present report aimed to better understand the humoral immune response in severe malaria and analyse new immunodominant antigen candidates as possible serological marker in severe malaria in children. This study included children aged 0-16 years from Guinea-Bissau with clinical signs of severe malaria. Serological and immunochemical characterisation of different anti-P. falciparum antibodies were made by ELISA and immunoblotting using a crude protein extract of P. falciparum. Sera from 12 children with severe malaria were analysed. Nine samples were positive for total anti-P. falciparum antibodies, seven for IgM and eight for total IgG anti-P. falciparum. There was a predominance of IgG1 response, suggesting a cytophilic action in severe malaria and a major role of IgG1 over other immunoglobulins. The antigenic profile of P. falciparum showed a consistent immunoblotting pattern of approximately 180 kDa, 100 kDa and around 50-40 kDa. The serological reactivity found in protein bands makes them as immunodominant antigens and promising candidates for serological markers in the context of severe malaria.