Hospital Prof. Dr. Fernando Fonseca

Unidade Local de Saúde Amadora / Sintra
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    2224 research outputs found

    Demencia rápidamente progresiva en adulto joven como forma de presentación de panencefalitis esclerosante subaguda

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    Cuidados intermédios de proximidade

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    Caffeine Consumption and Mortality in Diabetes: An Analysis of NHANES 1999-2010.

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    Aim: An inverse relationship between coffee consumption and mortality has been reported in the general population. However, the effect of coffee consumption in diabetes remains unclear. We aimed to evaluate the association of caffeine consumption and caffeine source with mortality among patients with diabetes. Methods: We examined the association of caffeine consumption with mortality among 1974 women and 1974 men with diabetes, using the National Health and Nutrition Examination Survey (NHANES) 1999-2010. Caffeine consumption was assessed at baseline using 24 h dietary recalls. Cox proportional hazard models were fitted to estimate hazard ratios (HR) for all-cause, cardiovascular, and cancer-related mortality according to caffeine consumption and its source, adjusting for potential confounders. Results: A dose-dependent inverse association between caffeine and all-cause mortality was observed in women with diabetes. Adjusted HR for death among women who consumed caffeine, as compared with non-consumers, were: 0.57 (95% CI, 0.40-0.82) for <100 mg of caffeine/day, 0.50 (95% CI, 0.32-0.78) for 100 to <200 mg of caffeine/day, and 0.39 (95% CI, 0.23-0.64) for ≥200 mg of caffeine/day (p = 0.005 for trend). This association was not observed in men. There was a significant interaction between sex and caffeine consumption (p = 0.015). No significant association between total caffeine consumption and cardiovascular or cancer mortality was observed. Women who consumed more caffeine from coffee had reduced risk of all-cause mortality (p = 0.004 for trend). Conclusion: Our study showed a dose-dependent protective effect of caffeine consumption on mortality among women with diabetes.info:eu-repo/semantics/publishedVersio

    An exceptional cause of drug-induced colitis: cholestyramine.

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    Utilização de Concentrado de Complexo Protrombínico na reversão de hipocoagulação oral

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    Introdução: Os doentes sob hipocoagulação oral podem necessitar de reversão em caso de hemorragia ativa ou procedimentos emergentes. Perante hemorragias graves, os doentes sob anticoagulantes orais diretos (DOAC’s) parecem cursar mais favoravelmente do que doentes sob antivitamínicos K. Existe maior incidência de hemorragia digestiva no primeiro grupo, no entanto esta não parece alterar a mortalidade. Na ausência de antídotos específicos, é recomendada a utilização de concentrado de complexo protrombínico (CCP). Objetivos: Avaliar a utilização de CCP para reversão de hipocoagulação oral durante o ano de 2017, no nosso hospital. Métodos: Consulta dos registos do nosso serviço relativamente à utilização de CCP em 2017. Pesquisa dos processos clínicos dos doentes e recolha dos dados clínico-laboratoriais relevantes. Resultados: Foram avaliados 59 doentes, 62,71% sob varfarina e 37,29% sob DOAC’s. Em ambos os grupos, a maioria era do género masculino e com idade superior a 70 anos (65% vs. 55% e 62% vs. 77%, respetivamente). O principal motivo para anticoagulação era fibrilhação auricular não valvular (68% vs. 88%). Todos os doentes apresentavam hemorragia ativa grave. Mais de metade apresentava função renal alterada (57% vs. 59%). Cerca de 1/3 necessitaram de outros componentes sanguíneos /hemoderivados (32,4% vs. 36%). A mortalidade foi semelhante nos dois grupos (27% vs. 23%). Conclusão: Na nossa experiência a reversão de anticoagulação com complexo protrombínico (CCP) demonstrou resultados equivalentes, tanto em doentes sob varfarina como DOAC’s. A mortalidade e o consumo de componentes sanguíneos/hemoderivados foram semelhantes em ambos os grupos. Contudo, a elevada prevalência de disfunção renal nesta amostra poderá enviesar os resultados. A prescrição adequada e acompanhamento dos doentes hipocoagulados é fundamental.N/

    COPD and Cardiovascular Disease.

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    COPD is one of the major public health problems in people aged 40 years or above. It is currently the 4th leading cause of death in the world and projected to be the 3rd leading cause of death by 2020. COPD and cardiac comorbidities are frequently associated. They share common risk factors, pathophysiological processes, signs and symptoms, and act synergistically as negative prognostic factors. Cardiac disease includes a broad spectrum of entities with distinct pathophysiology, treatment and prognosis. From an epidemiological point of view, patients with COPD are particularly vulnerable to cardiac disease. Indeed, mortality due to cardiac disease in patients with moderate COPD is higher than mortality related to respiratory failure. Guidelines reinforce that the control of comorbidities in COPD has a clear benefit over the potential risk associated with the majority of the drugs utilized. On the other hand, the true survival benefits of aggressive treatment of cardiac disease and COPD in patients with both conditions have still not been clarified. Given their relevance in terms of prevalence and prognosis, we will focus in this paper on the management of COPD patients with ischemic coronary disease, heart failure and dysrhythmia.info:eu-repo/semantics/publishedVersio

    BP Reduction, Kidney Function Decline, and Cardiovascular Events in Patients without CKD.

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    BACKGROUND AND OBJECTIVES: In the Systolic Blood Pressure Intervention Trial (SPRINT), intensive systolic BP treatment (target <120 mm Hg) was associated with fewer cardiovascular events and higher incidence of kidney function decline compared with standard treatment (target <140 mm Hg). We evaluated the association between mean arterial pressure reduction, kidney function decline, and cardiovascular events in patients without CKD. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We categorized patients in the intensive treatment group of the SPRINT according to mean arterial pressure reduction throughout follow-up: <20, 20 to <40, and ≥40 mm Hg. We defined the primary outcome as kidney function decline (≥30% reduction in eGFR to <60 ml/min per 1.73 m2 on two consecutive determinations at 3-month intervals), and we defined the secondary outcome as cardiovascular events. In a propensity score analysis, patients in each mean arterial pressure reduction category from the intensive treatment group were matched with patients from the standard treatment group to calculate the number needed to treat regarding cardiovascular events and the number needed to harm regarding kidney function decline. RESULTS: In the intensive treatment group, 1138 (34%) patients attained mean arterial pressure reduction <20 mm Hg, 1857 (56%) attained 20 to <40 mm Hg, and 309 (9%) attained ≥40 mm Hg. Adjusted hazard ratios for kidney function decline were 2.10 (95% confidence interval, 1.22 to 3.59) for mean arterial pressure reduction between 20 and 40 mm Hg and 6.22 (95% confidence interval, 2.75 to 14.08) for mean arterial pressure reduction ≥40 mm Hg. In propensity score analysis, mean arterial pressure reduction <20 mm Hg presented a number needed to treat of 44 and a number needed to harm of 65, reduction between 20 and <40 mm Hg presented a number needed to treat of 42 and a number needed to harm of 35, and reduction ≥40 mm Hg presented a number needed to treat of 95 and a number needed to harm of 16. CONCLUSIONS: In the intensive treatment group of SPRINT, larger declines in mean arterial pressure were associated with higher incidence of kidney function decline. Intensive treatment seemed to be less favorable when a larger reduction in mean arterial pressure was needed to attain the BP target.info:eu-repo/semantics/publishedVersio

    Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.

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    Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms that may show overlapping morphology and immunohistochemistry with uterine smooth muscle tumors. In this study, we evaluated the morphologic, immunohistochemical, and molecular features of 32 PEComas, including 11 with aggressive behavior. Two distinct morphologies were observed: classic (n=30) and those with a lymphangioleiomyomatosis appearance (n=2). In the former, patients ranged from 32 to 77 (mean: 51) years and 13% had tuberous sclerosis. Tumors ranged from 0.2 to 17 (mean: 5.5) cm with 77% arising in the corpus. Epithelioid cells were present in 100% and a spindled component was seen in 37%. Nuclear atypia was low (53%), intermediate (17%), or high (30%). Mitoses ranged from 0 to 36 (mean: 6) and 0 to 133 (mean: 19) per 10 and 50 high-power fields, with atypical mitoses present in 30%. Thin and delicate vessels were noted in 100%, clear/eosinophilic and granular cytoplasm in 93%, stromal hyalinization in 73%, necrosis in 30%, and lymphovascular invasion in 10%. All tumors were positive for HMB-45, cathepsin K, and at least one muscle marker, with most expressing melan-A (77%) and/or MiTF (79%). A PSF-TFE3 fusion was identified in one while another showed a RAD51B-OPHN1 fusion. Follow-up ranged from 2 to 175 (mean: 41) months, with 63% of patients alive and well, 20% dead of disease, 13% alive with disease, and 3% dead from other causes. In the latter group (n=2), patients were 39 and 49 years old, one had tuberous sclerosis, while the other had pulmonary lymphangioleiomyomatosis. Both tumors expressed HMB-45, cathepsin K, and muscle markers, but lacked TFE3 and RAD51B rearrangements. The 2 patients are currently alive and well. Application of gynecologic-specific criteria (≥4 features required for malignancy: size ≥5 cm, high-grade atypia, mitoses >1/50 high-power fields, necrosis, and lymphovascular invasion) for predicting outcome misclassified 36% (4/11) of aggressive tumors; thus, a modified algorithm with a threshold of 3 of these features is recommended to classify a PEComa as malignant.info:eu-repo/semantics/publishedVersio

    A Complex Case of Cholestasis in a Patient with ABCB4 and ABCB11 Mutations

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    The low-phospholipid-associated cholelithiasis (LPAC) syndrome is a form of symptomatic cholelithiasis occurring in young adults, characterized by recurrence of symptoms after cholecystectomy and presence of hepatolithiasis. The case refers to a healthy 39-year-old Caucasian male who presented with abdominal pain and jaundice. His blood tests showed conjugated hyperbilirubinemia and elevated liver enzymes (total bilirubin 6.65 mg/dL, γ-glutamyltransferase 699 IU/L) and abdominal computed tomography revealed dilation of common bile duct and left intrahepatic ducts. Magnetic resonance cholangiopancreatography identified choledocholithiasis, retrieved by endoscopic retrograde cholangiopancreatography, after which there was a worsening of jaundice (total bilirubin 23 mg/dL), which persisted for several weeks, possibly due to ciprofloxacin toxicity. After an extensive workup including liver biopsy, the identification of two foci of hepatolithiasis on reevaluation abdominal ultrasound raised the hypothesis of LPAC syndrome and the patient was started on ursodeoxycholic acid, with remarkable improvement. Genetic testing identified the mutation c.1954A>G (p.Arg652Gly) in ABCB4 gene (homozygous) and c.1331T>C (p.Val444Ala) in ABCB11 gene (heterozygous). In conclusion, we describe the unique case of an adult male with choledocholithiasis, hepatolithiasis, and persistent conjugated hyperbilirubinemia after retrieval of stones, fulfilling the criteria for LPAC syndrome and with possible superimposed drug-induced liver injury, in whom ABCB4 and ABCB11 mutations were found, both of which had not been previously described in association with LPAC.info:eu-repo/semantics/publishedVersio

    A Rare Cause of Diarrhea in the Occident: A Case of Colonic Schistosomiasis

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    Unidade Local de Saúde Amadora / Sintra
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