Browsing by Author "Rodrigues, J"
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- Alpha-1 Antitrypsin Deficiency: Principles of CarePublication . Rodrigues, J; Mineiro, A; Reis, A; Ventura, D; Fernandez-Llimos, F; Costa, F; Gomes, J; Silva, JM; Lopes, P; Robalo Cordeiro, CAlpha-1 antitrypsin deficiency is an autosomal co-dominant inherited disorder that results in decreased circulating levels of alpha-1 antitrypsin (also known as alpha-1 proteinase inhibitor) and predisposes affected individuals to early onset lung and liver disease. There is currently no cure for alpha-1 antitrypsin deficiency. However, appropriate treatment and a high standard of clinical care can prevent patients from being seriously affected and having to undergo major medical interventions, such as organ transplantation. Beyond managing the symptoms associated with alpha-1 antitrypsin deficiency, alpha-1 proteinase inhibitor therapy is the only treatment for the condition's underlying cause. Early diagnosis is important to ensure efficient therapeutic strategies and to minimize further deterioration of lung function. alpha-1 antitrypsin deficiency is under diagnosed globally, partly because the disease has no unique presenting symptoms. This document was prepared by a Portuguese multidisciplinary group and it aims to set out comprehensive principles of care for Alpha-1 antitrypsin deficiency. These include the importance of registries, the need for clinical research, the need for consistent recommendations (regarding diagnosis, treatment and monitoring), the role of reference centres, the requirement for sustained access to treatment, diagnostic and support services, and the role of patient organizations.
- Avaliação do Tempo Atribuído à Travessia de Peões: Contributo para uma Lisboa Mais InclusivaPublication . Boaventura, S; Rodrigues, J; Plancha, T; Martins, M; Silva, M; Da Silva, VN; Horta, L; Soares Branco, PIntroduction: With progressive ageing of the Portuguese population, it is paramount that the conditions of outdoor accessibility and safety are adapted to this age group. The aim of this study was to assess whether the time allocated to pedestrian crossing in the crosswalks with pedestrian crossing lights between Curry Cabral Hospital and local public transport is enough to allow safe passage of the elderly. Material and methods: We evaluated 100 ambulatory care patients from the Physical Medicine and Rehabilitation department. All of them answered a questionnaire, the Activities-Specific Balance Confidence Scale (Portuguese version) and performed a 10-meter walk test. All crosswalks with pedestrian crossing lights between the hospital and local public transport were analyzed, in a total of 26, and the gait speed required to perform a safe crossing was calculated. Results: Mean age of patients was 75 years and the majority (73%) were female. The study showed that all patients could safely cross 17 (65%) crosswalks. The nine remaining crosswalks (35%) represented an obstacle to our sample. Discussion: If the required gait speed as currently set in legislation for the disabled was implemented, 99% of the patients would have been able to cross the crosswalks safely. Conclusion: It is essential to apply the gait speed set in legislation, since non-compliance endangers elderly patients in Curry Cabral Hospital, increasing the likelihood of accidents and the feeling of insecurity on the streets.
- Avaliação do Tempo Atribuído à Travessia de Peões: Contributo Para uma Lisboa Mais InclusivaPublication . Boaventura, S; Rodrigues, J; Plancha, T; Martins, M; Silva, M; Brás da Silva, V; Horta, L; Soares Branco, PIntrodução: Com o envelhecimento progressivo da população portuguesa, é fundamental que as condições de acessibilidade e segurança na via pública estejam adaptadas a este grupo etário. O objectivo deste estudo foi avaliar se o tempo atribuído à travessia de peões nas passadeiras semaforizadas entre o Hospital de Curry Cabral e os transportes públicos locais é suficiente para permitir a travessia dos idosos em segurança. Material e Métodos: Foram avaliados 100 doentes seguidos em consulta externa no Serviço de Medicina Física e de Reabilitação. Todos responderam a um questionário, à escala de confiança no equilíbrio específica da atividade e executaram o teste de marcha de 10 metros. Foram analisadas todas as passadeiras semaforizadas dos percursos de marcha entre o hospital e os transportes públicos locais, num total de 26 e calculada a velocidade de marcha necessária para realizar a travessia das passadeiras em segurança. Resultados: A média de idade dos doentes foi de 75 anos, sendo a maioria do género feminino (73%). Concluímos que todos os doentes conseguem atravessar em segurança 17 (65%) passadeiras, representando as restantes nove (35%) um obstáculo para a nossa amostra. Discussão: Se o valor de velocidade de marcha nas passadeiras estipulado na legislação para os cidadãos com mobilidade condicionada fosse devidamente aplicado, 99% da amostra teria conseguido atravessar as passadeiras em segurança. Conclusão: É fundamental que o valor de velocidade de marcha nas passadeiras estipulado na legislação seja aplicado, pois o seu incumprimento coloca em risco os utentes idosos do Hospital de Curry Cabral, aumentando a probabilidade de acidentes e o sentimento de insegurança na via pública.
- Comparison of Nasopharyngeal Samples for SARS‐CoV ‐2 Detection in a Paediatric CohortPublication . Rodrigues, J; Gouveia, C; Santos, MA; Costa, O; Côrte‐Real, R; Brito, MJAim: The diagnosis of coronavirus disease 2019 (COVID-19) depends on accurate and rapid testing. Choosing an appropriate sample may impact diagnosis. Naso-oropharyngeal swabs (NOS) are most frequently used, despite several limitations. Since studies suggest nasopharyngeal aspirate (NPA) as a superior alternative in children, we hypothesised collecting both nasopharyngeal swab and aspirate would improve our diagnostic accuracy. Methods: Observational, longitudinal, prospective study from 7 March to 7 May in a tertiary paediatric hospital in Lisbon. The objective was to compare the rate of detection of SARS-CoV-2 between NOS and NPA samples collected simultaneously. Results: A total of 438 samples collected from 85 patients with confirmed COVID-19. There were 47.7% overall positive specimens - 32% (70/219) positive NOS and 63.5% (139/219) positive NPA. The tests were 67.6% concordant (k = 0.45). 50.3% had positive NPA with negative NOS, while 1.3% had positive NOS with negative NPA. NPA proved to be more sensitive (98.6% with 95% confidence interval 91.2-99.9% vs. 49.6% with 95% confidence interval 41.1-58.2%, P < 0.001). Additionally, the difference between NPA and NOS positive samples was statistically significant across all population groups (age, health condition, clinical presentation, contact with COVID-19 patients or need for hospitalisation), meaning NPA is more sensitive overall. Conclusions: Nasopharyngeal aspirates had greater sensitivity than naso-oropharyngeal swabs in detecting SARS-CoV-2. Our results suggest paediatric patients would benefit from collecting nasopharyngeal aspirates in hospital settings, whenever feasible, to improve diagnosis of COVID-19.
- Dexamethasone Intravitreal Implant (Ozurdex) in Diabetic Macular Edema: Real-World Data Versus Clinical Trials OutcomesPublication . Neves, P; Matias, I; Rodrigues, J; Santos, M; Dutra-Medeiros, M; Martins, DAim: To investigate the safety and efficacy of intravitreal dexamethasone implants (Ozurdex®/DEX) in patients with diabetic macular edema (DME) either naïve or non-naïve to anti-VEGF therapies who switched to DEX implant independent of response to anti-vascular endothelial growth factors (anti-VEGFs). Methods: This was an audit retrospective review of medical records of patients with DME who switched to the DEX intravitreal implant. Patients were divided into 2 groups: patients naïve to antiangiogenic therapy and patients who were previously treated with anti-VEGFs. Data regarding demographics, changes in mean best-corrected visual acuity (BCVA), central macular thickness (CMT), and intraocular pressure (IOP) was collected over 6mo. The demographic data mean changes in BCVA, CMT, and IOP were compared. Six-month follow-up data of 47 patients (57 eyes), who either switched to DEX implant irrespective of response to previous treatments or were treatment naïve before receiving DEX implant, was collected. Results: Improvement in mean BCVA was observed from 1-4mo after injection with a decreased effect at month 6 as expected, with better outcomes in naïve compared to non-naïve patients. A statistically relevant decrease in mean CMT was observed during the follow-up period. An increase in mean IOP was observed in the first 2mo after DEX therapy. The mean number of injections of the overall population during the 6mo was 1.3. A subgroup analysis showed no relevant difference between phakic versus pseudophakic patients relative to measured outcomes. There was no cataract progression during the follow-up period and no adverse events reported. Conclusion: This real-life setting study shows that intravitreal DEX implant is effective and safe. The timings of greater therapeutic impact are concordant with previous studies and suggest that earlier treatment with corticosteroids may have an additional benefit in naïve patients.
- Diagnostic Challenge in a Sickle Cell Disease Patient with COVID-19Publication . Rodrigues, J; Silva, TM; Garcia, AM; Soares, E; Brito, MJAcute chest syndrome is a life-threatening complication in sickle cell disease. Infections are frequently implied, and like other viruses, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may be a trigger. In addition, due to their inflammatory status, they may present a higher risk for severe coronavirus disease 2019 (COVID-19). Pneumonia and acute chest syndrome share clinical, laboratory, and radiological features and may overlap, which makes their differential diagnosis especially challenging. We describe a case of an adolescent with homozygous sickle cell disease that developed acute chest syndrome in the context of COVID-19. With it, we intend to bring awareness to the potential role of imaging in the differential diagnosis and in establishing the best approach for the patient. Chest computed tomography findings were suggestive of an alternative diagnosis to COVID-19 pneumonia and red cell transfusion, fluid management, analgesics, and antibiotics were administered with favorable outcome.
- Endobronchial Amphotericin B to Treat Hemoptysis in an Inoperable Patient with AspergillosisPublication . Pinto, M; Rodrigues, J; Silva, M; Maia, D; Miguel, AA 37-year-old man presented with chronic cavitary pulmonary aspergillosis and hemoptysis refractory to systemic antifungal therapy with voriconazole and bronchial artery embolization. Surgical excision was unfeasible due to the patient's refusal of blood transfusions. Ten sessions of intracavitary instillation of amphotericin B via flexible bronchoscopy were then performed. Hemoptysis cessation and aspergilloma resolution were achieved, with no toxicity or side effects, and the clinical benefits were sustained at six months of follow-up.
- Fístula Pleuro-Cutânea 52 Anos após OleotóraxPublication . Pires, G; Goes, MJ; Geraldes, V; Rodrigues, J; Rolo, J; Castro, AO oleotórax foi largamente utilizado entre 1930 e 1950 como tratamento da tuberculose pulmonar. Embora tenha sido abandonado a partir da década de 50, pelo sucesso terapêutico dos antibacilares e pela evolução da cirurgia torácica, continuaram a surgir, muitos anos mais tarde, complicações. Os autores apresentam o caso clínico de um doente com 80 anos de idade, com antecedentes de tuberculose pulmonar, tratada há 52 anos com oleotórax, o qual foi internado para esclarecimento de um tumor na região infra-clavicular direita, tendo a tomografia axial computorizada revelado a existência de uma fístula pleuro-cutânea e a punção mostrado que o conteúdo era oleoso.
- Gastroenterite Aguda em Crianças Internadas na Área de LisboaPublication . Escobar, C; Silva, T; Costa, B; Oliveira, M; Correia, P; Cordeiro Ferreira, G; Costa, I; Júlio, C; Rodrigues, J; Machado, J; Marques, A; Simões, MJ; Oleastro, M; Brito, MJIntrodução: A Gastroenterite Aguda (GEA) é uma patologia com importante morbilidade sendo a segunda causa de internamento na idade pediátrica. Objetivo: Caracterizar a GEA, em crianças internadas em dois hospitais da área de Lisboa com diferentes características demográficas. Métodos: Estudo prospetivo de maio 2011 a junho 2012. Pesquisados potenciais agentes etiológicos por técnicas convencionais e de biologia molecular em amostras de fezes e analisados dados epidemiológicos e clínicos. Resultados: Total de 140 amostras de crianças com GEA com identificação do agente em 83,6%: 64,3% vírus, 27,9% parasitas e 21,4% bactérias. Os agentes mais frequentes foram rotavírus (26,4%), norovírus II (13,6%), enterovírus (12,1%), Microsporidia (11,4%), Escherichia coli (9,3%), Campylobacter jejuni (7,9%), Giardia sp. (5,7%), Cryptosporidium sp. (5%) e Salmonella sp. (4,3%). Coinfecções (2 ou mais agentes) em 40 doentes (28,6%). Mediana de idade de 1,4 anos (min-5 dias; max-17 anos) sendo a etiologia viral mais frequente abaixo dos 5 anos (p<0.01), com o rotavírus identificado em crianças mais jovens (média=1,7 anos). Dois picos sazonais: o rotavírus entre Janeiro e Março e norovírus entre Agosto e Outubro. Apenas 10 (7,1%) doentes estavam vacinados para rotavírus, mas nenhum com o esquema completo. A presença de sangue nas fezes (p=0,02) e a febre (p=0,039) foram mais frequentes na infeção bacteriana, os vómitos (p<0.01) e os sintomas respiratórios (p=0,046) na infeção por rotavírus. Registaram-se complicações clínicas em 50 doentes (35,7%): desidratação (47), invaginação íleo-cecal (1), adenite mesentérica (1) e apendicite fleimonosa (1). Conclusão: Os vírus são os agentes mais frequentes de GEA sobretudo na criança pequena (idade <5 anos), sendo o rotavírus e norovírus os principais agentes. O número de coinfecções foi significativo mas não se associou a maior morbilidade. A ausência de identificação de agente em alguns casos pode refletir a necessidade de outros meios diagnósticos ou a existência de agentes ainda desconhecidos.
- Liver Depurative Techniques: A Single Liver Transplantation Center ExperiencePublication . Rodrigues, J; Castro, S; Moya, B; Fortuna, P; Martins, A; Pereira, JP; Bento, L; Perdigoto, R; Barroso, E; Marcelino, PIn a liver transplant (LT) center, treatments with Prometheus were evaluated. The main outcome considered was 1 and 6 months survival. Methods. During the study period, 74 patients underwent treatment with Prometheus; 64 were enrolled,with a mean age of 51 13 years; 47men underwent 212 treatments (mean, 3.02 per patient). The parameters evaluated were age, sex, laboratorial (liver enzymes, ammonia) and clinical (model for end-stage liver disease and Child-Turcotte-Pugh score) data. Results. Death was verified in 23 patients (35.9%) during the hospitalization period, 20 patients (31.3%) were submitted to liver transplantation, and 21 were discharged. LT was performed in 4 patients with acute liver failure (ALF, 23.7%), in 7 patients with acute on chronic liver failure (AoCLF, 43.7%), and in 6 patients with liver disease after LT (30%). Seven patients who underwent LT died (35%). In the multivariate analysis, older age (P ¼ .015), higher international normalized ratio (INR) (P ¼ .019), and acute liver failure (P ¼ .039) were independently associated with an adverse 1-month clinical outcome. On the other hand, older age (P ¼ .011) and acute kidney injury (P ¼ .031) at presentation were both related to worse 6-month outcome. For patients with ALF and AoCLF we did not observe the same differences. Conclusions. In this cohort, older age was the most important parameter defining 1- and 6-month survival, although higher INR and presence of ALF were important for 1-month survival and AKI for 6-month survival. No difference was observed between patients who underwent LT or did not have LT.