Browsing by Author "Soares, M."
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- Craniossinostoses - importância clínica e implicações funcionaisPublication . Cristóvão, C.; Emílio, A.; Soares, M.; Dias, A.; Matos, M.; Távora, L.RESUMO Introdução: A craniossinostose é uma condição patológica que resulta do encerramento precoce de uma ou várias suturas cranianas, podendo, para além da questão estética, levar ao aparecimento de complicações neurológicas, mais frequente quando existe envolvimento de mais de uma sutura. Descrição dos Casos: Os autores apresentam dois casos clínicos de craniossinostose, com diferentes tempos de seguimento, ilustrando a problemática da intervenção cirúrgica e a necessidade de observação precoce e seguimento por Neurocirurgia. Discussão: Sendo uma patologia relativamente frequente na população pediátrica geral deverá constituir um motivo de atenção especial por parte do Pediatra/Médico Assistente na avaliação periódica em Consulta de Rotina, referenciando à Consulta de Neurocirurgia o mais precocemente possível.
- Inquéritos de satisfação: Triagem de Manchester no Hospital de Santo AntónioPublication . Almeida, P.; Campelo, J.; Figueiredo, A.; Gonçalves, L.; Guedes, A.; Guimarães, N.; Laranjo, P.; Luís, D.; Mendes, M.; Monjardino, M.; Neto, J.; Pimenta, J.; Rebocho, M.; Soares, M.
- Natural history and survival in stage 1 Val30Met transthyretin familial amyloid polyneuropathyPublication . Coelho, T.; Inês, M.; Conceição, I.; Soares, M.; de Carvalho, M.; Costa, J.Objective: To assess the natural history and treatment effect on survival among patients with transthyretin-associated familial amyloid polyneuropathy (TTR-FAP) stage 1 Val30Met. Methods: Multi-institutional, hospital-based study of patients with TTR-FAP Val30Met prospectively followed up until December 2016, grouped into untreated (n = 1,771), liver transplant (LTx)-treated (n = 957), or tafamidis-treated (n = 432) cohorts. Standardized mortality ratios, Kaplan-Meier, and Cox methods were used to estimate excess mortality, survival, and adjusted hazard ratios (HRs) for all-cause mortality. Results: Disease-modifying treatments decreased TTR-FAP excess mortality from 10 to 4 (standardized mortality ratio 3.92, 95% confidence interval [CI] 2.64-5.59). Median overall survival of untreated and LTx-treated cohorts was 11.61 (95% CI 11.14-11.87) and 24.73 years (95% CI 22.90-27.09), respectively, and was not reached in the tafamidis-treated cohort (maximum follow-up, 10 years). Both disease-modifying treatments improved survival. Among early-onset patients (younger than 50 years of age), tafamidis reduced the mortality risk compared with untreated patients by 91% (HR 0.09, 95% CI 0.03-0.25, p < 0.001) and with LTx-treated patients by 63% (HR 0.37, 95% CI 0.14-1.00, p = 0.050). Previous tafamidis treatment did not affect mortality risk after LTx (HR 0.83, 95% CI 0.25-2.78, p = 0.763). Among late-onset patients (50 years and older), tafamidis reduced mortality risk by 82% compared with untreated patients (HR 0.18, 95% CI 0.06-0.49, p = 0.001). Conclusion: LTx and tafamidis convey substantial survival benefits, but TTR-FAP mortality remains higher than in the general population. These results strongly reinforce the importance of timely diagnosis and earlier treatment, boosting the pursuit for an increased life expectancy. Classification of evidence: This study provides Class III evidence that for patients with stage 1 Val30Met TTR-FAP, LTx and tafamidis increase survival.
- Perfil do grande queimado em cuidados intensivos pediátricos - Experiência de doze anosPublication . Soares, M.; Valente, P.; Vale, M.C.; Estrada, J.; Ventura, L.; Barata, D.; Vasconcelos, C.Introdução: Nos países industrializados cerca de 1% da população requer anualmente tratamento médico por queimadura e 5% destes obrigam a internamento hospitalar. Objectivo: Caracterizar o perfil nosodemográfico do grande queimado pediátrico internado na Unidade de Cuidados Intensivos Pediátricos (UCIP) do Hospital de Dona Estefânia (HDE). População e Métodos: Efectuou-se estudo retrospectivo através da consulta dos processos de todas as crianças internadas por queimadura na UCIP do HDE no período compreendido entre Abril de 1991 e Outubro de 2004. Resultados: Neste período ocorreu um total de 90 internamentos, correspondendo a 80 crianças, sendo 10 referentes a reinternamentos. Os agentes causais foram o fogo em 26 casos (32,5%) e o líquido quente e a corrente eléctrica em 23 (28,8%) casos cada. O maior número de queimaduras ocorreu no grupo etário dos 1 aos 6 anos (n=46). A média da área de superfície corporal queimada foi de 29,6% ± 25,6. Trinta e oito porcento das crianças foram submetidas a suporte ventilatório. Os doentes com queimaduras provocadas pelo fogo apresentaram uma maior área de superfície corporal queimada (45,7 ± 24,7%), uma maior demora de internamento na UCIP (412,5 ± 658,4 horas), um maior índice de intervenção terapêutica (TISS 35,7±9,7) e a uma maior mortalidade. As queimaduras provocadas por corrente eléctrica tiveram menor gravidade. Observaram-se 6 óbitos, todos causados por sépsis. Conclusão: O agente causal mais frequente de queimadura foi o fogo. Mais de metade das crianças internadas por queimadura pertencia ao grupo etário dos 1 aos 6 anos. A principal causa de morte foi a infecção. ABSTRAT Background: In industrialised countries, up to 1% of a population requires medical treatment each year due to burn injury, and 5% of these patients require hospitalization. Objective: To evaluate the characteristics of the paediatric severe burned patients admitted to the Paediatric Intensive Care Unit (PICU) of Dona Estefânia Hospital (HDE). Patients and Methods: Data were collected by the retrospective review of medical record of burn children who were hospitalized in the PICU of HDE, between April 1991 and October 2004. Results: During this period 90 patients were admitted, 10 of then being readmissions. The leading agents were fire in 26 patients (32,5%) followed by hot liquids and electricity in 23 patients (28,8%) each. The highest incidence of paediatric burn were in children aged 1-6 years (n=46). The mean total body surface area burned was 29,6% ± 25,6. Thirty-eight percent of the children were submitted to mechanical ventilation. Fire burn patients presented with a higher total body surface area burned (45,7 ± 24,7%), a higher average durations of PICU length of stay (412,5 ± 658,4 hours), a higher Therapeutic Intervention Scoring System (TISS 35,7±9,7) and mortality. The electrical burns were less severe. There were 6 deaths all caused by sepsis. Conclusions: Fire was the most frequent burn agent. More than half of the children admitted were aged 1-6 years. Infection was the leading cause of mortality.
- Real-world data from the Portuguese Nivolumab Expanded Access Program (EAP) in previously treated Non Small Cell Lung Cancer (NSCLC)Publication . Figueiredo, A.; Almeida, M.A.; Almodovar, M.T.; Alves, P.; A, Araujo; Araújo, D.; Barata, F.; Barradas, L.; Barroso, A.; Brito, U.; Camacho, E.; Canário, D.; Cardoso, T.; Chaves, A.; Costa, L.; Cunha, J.; Duarte, J.; Estevinho, F.; Felizardo, M.; Fernandes, J.P.; Ferreira, L.; Ferreira, L.; Fidalgo, Paula; Freitas, C.; Garrido, P.; Gil, N.; Hasmucrai, D.; Jesus, E.; Lopes, J.A.; de Macedo, J.E.; Meleiro, A.; Neveda, R.; Nogueira, F.; Pantorotto, M.; Parente, B.; Pego, A.; Rocha, M.; Roque, J.; Santos, C.; Saraiva, J.; Silva, E.; Silva, S.; Simões, S.; Soares, M.; Teixeira, E.; Timóteo, T.; Hespanhol, V.Objective: The main aim of the study was to evaluate the efficacy and safety profile of Nivolumab, an immune-checkpoint-inhibitor antibody, in advanced, previously treated, Non-Small Cell Lung Cancer (NSCLC) patients, in a real world setting. Methods: We performed a retrospective, multicentre data analysis of patients who were included in the Portuguese Nivolumab Expanded Access Program (EAP). Eligibility criteria included histologically or citologically confirmed NSCLC, stage IIIB and IV, evaluable disease, sufficient organ function and at least one prior line of chemotherapy. The endpoints included Overall Response Rate (ORR), Disease Control Rate (DCR), Progression Free Survival (PFS) and Overall Survival (OS). Safety analysis was performed with the National Cancer Institute Common Terminology Criteria for Adverse Events (CTCAE), version 4.0, and immune-related Adverse Events (irAEs) were treated according to protocol treatment guidelines. Tumour response was assessed using the Response Evaluation Criteria in Solid Tumours (RECIST) version 1.1. Data was analysed using SPSS, version 21.0 (IBM Statistics). Results: From June 2015 to December 2016, a total of 229 patients with advanced NSCLC were enrolled at 30 Portuguese centres. Clinical data were collected up to the end of July 2018. The baseline median age was 64 years (range 37-83) and the majority of patients were males (70.3%) and former/current smokers (69.4%). Patients with non-squamous histology predominated (88.1%), and 67.6% of the patients had received 2 or more prior lines of chemotherapy. Out of 229 patients, data was available for 219 patients (3 patients did not start treatment, while data was unavailable in 7 patients); of the 219 patients, 15.5% were not evaluated for radiological tumour assessment, 1.4% had complete response (CR), 21% partial response (PR), 31% stable disease (SD) and 31.1% progressive disease (PD). Thus, the ORR was 22.4% and DCR was 53.4% in this population. At the time of survival analysis the median PFS was 4.91 months (95% CI, 3.89-6.11) and median OS was 13.21 months (95% CI, 9.89-16.53). The safety profile was in line with clinical trial data. Conclusions: Efficacy and safety results observed in this retrospective analysis were consistent with observations reported in clinical trials and from other centres.