Hospital Prof. Dr. Fernando Fonseca

Unidade Local de Saúde Amadora / Sintra
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    2224 research outputs found

    Modelos Teóricos da Ética Médica

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    Registo oncológico

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    Prescrição Inapropriada em Idosos numa Enfermaria de Medicina Interna

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    INTRODUCTION: Polypharmacy is often observed in elderly patients and is associated with an increased risk of adverse drug reactions, side effects and interactions. Clinicians should be alert to inappropriate drug prescribing and reduce polypharmacy. MATERIAL AND METHODS: Observational, longitudinal, retrospective and descriptive study in an internal medicine ward in a Portuguese hospital. Polypharmacy was defined as the use of five or more different medicines. The purpose of this study was to describe the prevalence of polypharmacy and inappropriate prescribing at admission and discharge in an internal medicine ward, according to deprescribing.org guidelines/algorithms. A total of 838 consecutive patients were admitted between January and July 2017. All patients were aged under 65 years old, and those who died before discharge were excluded. Patients' medications were reviewed from a medical database at hospital admission and discharge. We examined whether patients were taking anticoagulants, proton pump inhibitors, benzodiazepines, antipsychotics and/or antihyperglycemic medication. RESULTS: A total of 483 patients were included, mean age was 79.2 ± 8.0 years, and 42% of patients were male. Median number of medications at admission and discharge was six. Polypharmacy was present in more than 70% of admitted patients. Proton pump inhibitors were the most common inappropriate prescription at discharge (17.2%). DISCUSSION: This study demonstrated a low use of inappropriate medicine (11.2% - 17.2%) in older people discharged from hospital, when compared to other studies. CONCLUSION: Our study shows that polypharmacy is present in more than 70% of elderly admitted patients. Nevertheless, the drug inappropriateness rate was not significantly affected by polypharmacy at both admission and discharge, being overall lower than published data.Introdução: A polimedicação é observada nos doentes idosos e está associada a um maior risco de reações adversas, efeitos secundários e interações. Os clínicos devem atentos à prescrição inapropriada e à redução da polimedicação. Material e Métodos: Estudo observacional, longitudinal, retrospetivo e descritivo, realizado numa enfermaria de medicina interna num hospital português. Definimos a polimedicação como o uso de cinco ou mais medicamentos. O objetivo foi descrever a prevalência da polimedicação e a prescrição inapropriada, na admissão e alta, de acordo com as guidelines/algoritmos definidos em deprescribing. org. Admitimos 838 doentes entre janeiro e julho de 2017. Excluímos todos aqueles com idade inferior a 65 anos e óbitos. A medicação dos doentes foi revista a partir da base de dados hospitalar, à admissão e à data de alta. Examinámos se os doentes estavam a tomar anticoagulantes, inibidores da bomba de protões, benzodiazepinas, antipsicóticos e/ou anti hiperglicémicos. Resultados: Incluímos 483 doentes, com média de idade de 79,2 ± 8,0 anos, e 42% dos quais eram homens. A mediana da medicação à admissão e à alta foi seis. A polimedicação estava presente em mais de 70% dos doentes admitidos. Os inibidores da bomba de protões foram a classe mais inapropriadamente prescrita à data de alta (17,2%). Discussão: Demonstrámos um uso reduzido de fármacos inapropriados (11,2% - 17,2%) nos idosos, à alta hospitalar, quando comparado com outros estudos. Conclusão: Demonstrámos que a polimedicação estava presente em mais de 70% dos idosos admitidos. Contudo, a taxa de prescrição inapropriada não afetou significativamente a polimedicação na admissão e na alta, sendo inferior aos dados publicados.info:eu-repo/semantics/publishedVersio

    Mutational mechanism for DAB1 (ATTTC)n insertion in SCA37: ATTTT repeat lengthening and nucleotide substitution

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    Dynamic mutations by microsatellite instability are the molecular basis of a growing number of neuromuscular and neurodegenerative diseases. Repetitive stretches in the human genome may drive pathogenicity, either by expansion above a given threshold, or by insertion of abnormal tracts in nonpathogenic polymorphic repetitive regions, as is the case in spinocerebellar ataxia type 37 (SCA37). We have recently established that this neurodegenerative disease is caused by an (ATTTC)n insertion within an (ATTTT)n in a noncoding region of DAB1. We now investigated the mutational mechanism that originated the (ATTTC)n insertion within an ancestral (ATTTT)n . Approximately 3% of nonpathogenic (ATTTT)n alleles are interspersed by AT-rich motifs, contrarily to mutant alleles that are composed of pure (ATTTT)n and (ATTTC)n stretches. Haplotype studies in unaffected chromosomes suggested that the primary mutational mechanism, leading to the (ATTTC)n insertion, was likely one or more T>C substitutions in an (ATTTT)n pure allele of approximately 200 repeats. Then, the (ATTTC)n expanded in size, originating a deleterious allele in DAB1 that leads to SCA37. This is likely the mutational mechanism in three similar (TTTCA)n insertions responsible for familial myoclonic epilepsy. Because (ATTTT)n tracts are frequent in the human genome, many loci could be at risk for this mutational process.info:eu-repo/semantics/publishedVersio

    Transient and persistent acute kidney injury in acute liver failure

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    BACKGROUND: Acute Kidney Injury (AKI) is a very frequent complication in the Acute Liver Failure (ALF) population associated with negative outcomes. We aim to evaluate the impact of AKI duration on the outcomes of an ALF population. METHODS: A 20-year retrospective analysis of ALF patients admitted to an Intensive Care Unit (ICU) was performed. Chronic liver failure, chronic kidney disease on renal replacement therapy, dialysis requirement within the week prior or an ICU stay of less than 48 h after AKI diagnosis, were exclusion criteria. AKI was defined according to the KDIGO criteria and classified into transient (< 48 h duration) or persistent (48 h duration). RESULTS: A total of 51 patients were included in the analysis and most had AKI (66.7%). Persistent AKI patients (70.6%) presented more frequently with AKI at admission and a higher SOFA score than transient AKI and no AKI, p < 0.05. More severe AKI, sepsis, vasopressor support and mechanical ventilation were also more common (p < 0.05). Nineteen (55.9%) were classified as persistent AKI exclusively by serum creatinine and 15 (44.1%) by both serum creatinine and urine output criteria. Mean survival time at 30 days was 11.3 days for persistent AKI, 25.3 days for transient AKI and 27.0 days for no AKI, p = 0.01. Adjusted multivariate cox regression analysis showed that persistent AKI predicted in-hospital mortality but it lost significance when AKI severity was introduced in the model. CONCLUSION: Persistent AKI was common in ALF patients and associated with more severe AKI, worst systemic complications and a higher 30-day mortality, compared to transient and no AKI patients.info:eu-repo/semantics/publishedVersio

    O Internato Médico do HFF em 2019: Continuidade e novos desafios

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    Vascular White Matter Lesions in Young Adults: A Neurology Outpatient Clinic Registry

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    Introduction: Although frequently assumed to be age-related changes, vascular white matter lesions (WML) are sometimes found in young adults. Etiology is usually attributed to sporadic small vessel disease; nevertheless, genetic disorders may also be implicated. We aimed to characterize the population of young adults with vascular WML in Neurology outpatient clinics. Methods: Neurologists from 12 Portuguese hospitals were invited to include patients aged 18-55 years evaluated in consultation, with vascular WML on MRI, scoring II or III in the Fazekas scale. Central imaging validation was performed by 2 independent, blinded, Neuroradiologists. Demographic and clinical data were collected as well as results of investigations performed. Results: During 2 years, 77 patients were included (mean age 47.7 years). Vascular risk factors were present in 88.3% patients (hypertension in 53.2%) and previous history of stroke in 36.4%. Patients without history of stroke were younger (46.6 ± 7.2 vs. 49.6 ± 3.9 years, p = 0.045) and had fewer vascular risk factors (p < 0.001). They were more frequently females (87.8 vs. 46.4%, p < 0.001), and headache (30.6 vs. 3.6%, p = 0.007), contrary to focal symptoms (16.3 vs. 53.6%, p = 0.001), was the most frequent reason of referral. Etiological investigations performed differed between Neurologists. A genetic disorder was identified in 6 out of 58 patients (CADASIL n = 5; COL4A1 n = 1). Conclusion: Young adults with vascular WML evaluated in Neurology outpatient clinics concentrate in the oldest age groups. Vascular risk factors should be screened carefully in this population. Among patients without history of stroke, females largely outweigh males. Diagnostic investigations performed do not follow a standardized protocol.info:eu-repo/semantics/publishedVersio

    A Rare Presentation of Ogilvie's Syndrome

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    Ogilvie's syndrome or acute colonic pseudo-obstruction is characterized by massive colon dilation in the absence of mechanical obstruction or toxic megacolon. The phenotype associated with secretory diarrhoea is rare and is related to increased potassium channel activity in the colon, inducing excessive potassium loss, with increased sensitivity to normal serum aldosterone levels. The recommended therapy is potassium-sparing agents. We present the case of an 85-year-old patient who was admitted at the emergency department with prostration, abdominal distension and diarrhoea, corresponding to functional colonic dilation precipitated by severe hypokalaemia. Resolution of the condition only occurred after spironolactone was administered for suspected primary hyperaldosteronism, which was not proved as the patient showed normal aldosterone serum levels. The pathophysiological mechanism of abnormal potassium secretion in this scenario corresponds to 'relative hyperaldosteronism' caused by increased sensitivity of colonocytes to aldosterone. LEARNING POINTS: Colonic pseudo-obstruction is not usually associated with secretory diarrhoea and severe hypokalaemia.Although serum aldosterone levels are normal, the treatment of choice is spironolactone due to its effect on the potassium channels in colonocytes.It is essential to recognize this specific phenotype so that the correct clinical approach, diagnosis and management can be established.info:eu-repo/semantics/publishedVersio

    A multicenter, non-interventional study to evaluate the disease activity in Multiple Sclerosis after withdrawal of Natalizumab in Portugal.

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    Abstract OBJECTIVES: Natalizumab (NTZ) is very effective for treatment of relapsing-remitting multiple sclerosis (RRMS), its use is mainly limited by safety issues. Discontinuation of NTZ is associated with recurrence of disease activity (reactivation and rebound). The best strategy for subsequent therapy and the predictive factors for recurrence in such patients are areas of active research. We aimed to evaluate predictors of reactivation in a multicentric study. PATIENTS AND METHODS: Multicentric retrospective observational study in five portuguese MS referral centers. Demographic, clinical and imagiological data were collected in the year prior, during and in the year following NTZ discontinuation. Predictors of reactivation and rebound after NTZ suspension were studied using a multivariate Cox model. RESULTS: Sixty-nine patients were included. They were mainly non-naïve patients (97%), with a mean age of 29.1 ± 8.3 years at diagnosis, and a mean age of 37.2 ± 10.3 years at NTZ initiation. The mean annualized relapse rate (ARR) previous, during and after NTZ was 1.6 ± 1.2, 0.2 ± 0.5 and 0.6 ± 1.0, respectively. The median EDSS before, during and after NTZ was 3.5 (IQR 3.3), 3.5 (IQR 3.5) and 4.0 (IQR 3.8), respectively. The median number of infusions was 26.0 (IQR 12.5) and the main reason to NTZ discontinuation was progressive multifocal leukoencephalopathy (PML) risk (70%). After NTZ suspension, reactivation was observed in 25 (36%) patients after a median time of 20.0 (IQR 29.0) weeks. Reactivation predictors in our sample included NTZ suspension for reasons other than PML (adjusted HR = 0.228, 95% CI [0.084- 0.616], p = 0.004), ARR before NTZ (adjusted HR = 1.914 95% [CI 1.330-2.754], p < 0.001) and a longer disease duration at time of NTZ initiation (adjusted HR = 1.154, 95% CI [1.020-1.306], p = 0.023). Rebound occurred in 5 (7%) patients after a median time of 20 (IQR 34.5) weeks. CONCLUSION: Significant predictors of disease reactivation in our cohort were discontinuation of NTZ for reasons other than PML risk, higher disease activity before NTZ treatment, and longer disease duration. Our study provides valuable data of portuguese patients after NTZ withdrawal.info:eu-repo/semantics/publishedVersio

    Recurrent Gastrointestinal Bleeding from Dieulafoy's Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association

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    Von Willebrand disease (vWD) is the most prevalent hereditary bleeding disorder, affecting 0.6-1.3% of the population. While gastrointestinal bleeding from angiodysplasia is a well-known complication of vWD, the same is not true for Dieulafoy's lesions (DLs). We report the case of a 21-year-old black male with type 1 vWD and 2 previous hospital admissions for severe anemia with no visible blood loss. In both episodes, DLs were identified and treated endoscopically, one in the stomach and another in the duodenum. The patient presented to the emergency department in September 2016 with dizziness, fatigue, and again no visible blood loss. He was hemodynamically stable, and laboratory workup showed a hemoglobin level of 3.4 g/dL. After transfusion of packed red blood cells, intravenous iron, and von Willebrand factor/factor VIII concentrate infusions, the patient underwent upper endoscopy and colonoscopy, which were normal. Small-bowel capsule endoscopy showed dark blood and a fresh clot in the proximal jejunum. At this site, push enteroscopy identified a pulsatile vessel with an overlying minimal mucosal defect, consistent with a DL, type 2b of the Yano-Yamamoto classification, which was successfully treated with adrenaline and 2 hemoclips. The patient remains stable after 18 months of follow-up, with a hemoglobin level of 13.2 g/dL. This is a case of recurrent severe occult gastrointestinal bleeding from multiple DL in a young patient with vWD who is otherwise healthy. Three other cases of DL bleeding in the setting of vWD have been reported in the literature, suggesting a possible association between these 2 entities.info:eu-repo/semantics/publishedVersio

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    Unidade Local de Saúde Amadora / Sintra
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