Browsing by Author "Ferreira, S."
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- Alergia às proteínas do leite de vaca com manifestações gastrointestinaisPublication . Ferreira, S.; Pinto, M.; Carvalho, P.; Gonçalves, J.; Lima, R.; Pereira, F.A alergia as proteínas do leite de vaca (APLV) constituiu a alergia alimentar mais frequente em crianças com idade inferior a tres anos, podendo as suas manifestações gastrointestinais ocorrer em qualquer idade. Na suspeita clínica de APLV baseada na anamnese e exame físico, deve instituir-se dieta de eliminação durante um período variável consoante o quadro clínico, seguida de uma prova de provocação oral (PPO) que ira confirmar ou excluir o diagnostico. Exames complementares como testes cutâneos e IgEs séricas especificas poderão ser necessários. O tratamento baseia-se na dieta de eliminação e pressupõe aconselhamento nutricional e vigilância do crescimento. Habitualmente repete-se a PPO após tres a 12 meses de dieta de eliminação, verificando-se aquisição de tolerância em mais de 80% das crianças aos três anos de idade. O objetivo deste artigo e fazer uma breve revisão e atualizacao sobre as manifestações gastrointestinais da APLV em idade pediátrica, apresentando uma proposta de abordagem fundamentada em recomendações internacionais recentes.
- Baixa estatura e haploinsuficiência do gene SHOXPublication . Costa, M.; Magalhães, J.; Ferreira, S.; Rocha, M.; Ribeiro, L.; Oliveira, M.; Cardoso, H.; Borges, T.Introdução: A haploinsuficiência do gene SHOX (short stature homeobox gene) é uma das causas genéticas mais frequentes de baixa estatura isolada ou familiar, cuja gravidade clínica pode ser muito variável. Caso Clinico: Os autores descrevem uma adolescente de 14 anos, com membros curtos e baixa estatura. A radiografia do punho demonstrou deformidade de Madelung. O estudo molecular revelou a deleção do gene SHOX (FISH del (X) (Xp22.3Xp22.3) (SHOX). Um exame físico cuidadoso assume extrema importância na avaliação clínica de baixa estatura. A deformidade de Madelung pode surgir apenas na adolescência. O tratamento com Hormona de Crescimento pode aumentar a estatura final. Discussão/conclusões: A prevalência de baixa estatura por mutações do gene SHOX parece ser semelhante ao défice de hormona de crescimento e Síndroma de Turner. O objetivo dos autores foi chamar a atenção sobre esta entidade clínica não muito conhecida, que pode ter implicações terapêuticas com um diagnóstico atempado.
- Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profilePublication . Martín-Martín, L.; López, A.; Vidriales, B.; Caballero, M.; Rodrigues, A.; Ferreira, S.; Lima, M.; Almeida, S.; Valverde, B.; Martínez, P.; Ferrer, A.; Candeias, J.; Ruíz-Cabello, F.; Buadesa, J.; Sempere, A.; Villamor, N.; Orfao, A.; Almeida, J.Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of leukemia/lymphoma, whose diagnosis can be difficult to achieve due to its clinical and biological heterogeneity, as well as its overlapping features with other hematologic malignancies. In this study we investigated whether the association between the maturational stage of tumor cells and the clinico-biological and prognostic features of the disease, based on the analysis of 46 BPDCN cases classified into three maturation-associated subgroups on immunophenotypic grounds. Our results show that blasts from cases with an immature plasmacytoid dendritic cell (pDC) phenotype exhibit an uncommon CD56- phenotype, coexisting with CD34+ non-pDC tumor cells, typically in the absence of extramedullary (e.g. skin) disease at presentation. Conversely, patients with a more mature blast cell phenotype more frequently displayed skin/extramedullary involvement and spread into secondary lymphoid tissues. Despite the dismal outcome, acute lymphoblastic leukemia-type therapy (with central nervous system prophylaxis) and/or allogeneic stem cell transplantation appeared to be the only effective therapies. Overall, our findings indicate that the maturational profile of pDC blasts in BPDCN is highly heterogeneous and translates into a wide clinical spectrum -from acute leukemia to mature lymphoma-like behavior-, which may also lead to variable diagnosis and treatment.
- Economic Impact of Prosthetic Joint Infection - an Evaluation Within the Portuguese National Health SystemPublication . Sousa, A.; Carvalho, A.; Pereira, C.; Reis, E.; Santos, A.; Abreu, M.; Soares, D.; Fragoso, R.; Ferreira, S.; Reis, M.; Sousa, R.Introduction: Prosthetic infection is a devastating complication of arthroplasty and carries significant economic burden. The objective of this study was to analyze the economic impact of prosthetic hip and knee infection in Portuguese National Health System. Material and Methods: Case-control study carried out from January 2014 to December 2015. The mean costs of primary arthroplasties and prosthetic revision surgeries for non-infectious reasons were compared with the costs of prosthetic infections treated with debridement and preservation of the prosthesis or with two-stage exchange arthroplasty.The reimbursement for these cases was also evaluated and compared with its real costs. Results: A total of 715 primary arthroplasties, 35 aseptic revisions, 16 surgical debridements and 15 revisions for infectious reasons were evaluated. The cost of primary arthroplasties was 3,230€ in the hips and 3,618€ in the knees. The cost of aseptic revision was 6,089€ in the hips and 7,985€ in the knees. In the cases treated with debridement and implant retention the cost was 5,528€ in the hips and 4,009€ in the knees. In cases of infections treated with a two-stage revision the cost was 11,415€ and 13,793€ for hips and knees, respectively. Conclusion: As far as we know this is the first study that analyzes the economic impact of prosthetic infection in the Portuguese context. Although direct compensation for treating infected cases is much lower than calculated costs, infected cases push the overall hospital case-mix-index upwards thus increasing financial compensation for the entire cohort of treated patients. This knowledge will allow for more informed decisions about health policies in the future.
- Health-related quality of life and utilities in gastric premalignant conditions and malignant lesions: a multicentre study in a high prevalence countryPublication . Areia, M.; Alves, S.; Brito, D.; Cadime, A.; Carvalho, R.; Saraiva, S.; Ferreira, S.; Moleiro, J.; Pereira, A.; Carrasquinho, J.; Lopes, L.; Ramada, J.; Marcos-Pinto, R.; Pedroto, I.; Contente, L.; Eliseu, L.; Vieira, A.; Sampaio, M.; Sousa, H.; Almeida, N.; Gregório, C.; Portela, F.; Sofia, C.; Braga, V.; Baginha, E.; Bana e Costa, T.; Chagas, C.; Mendes, L.; Magalhães-Costa, P.; Matos, L.; Gonçalves, F.; Dinis-Ribeiro, M.BACKGROUND AND AIMS: A recent review of economic studies relating to gastric cancer revealed that authors use different tests to estimate utilities in patients with and without gastric cancer. Our aim was to determine the utilities of gastric premalignant conditions and adenocarcinoma with a single standardized health measure instrument. METHODS: Cross-sectional nationwide study of patients undergoing upper endoscopy (n=1,434) using the EQ-5D-5L quality of life (QoL) questionnaire. RESULTS: According to EQ-5D-5L, utilities in individuals without gastric lesions were 0.78 (95% confidence interval: 0.76-0.80), with gastric premalignant conditions 0.79 (0.77-0.81), previously treated for gastric cancer 0.77 (0.73-0.81) and with present cancer 0.68 (0.55-0.81). Self-reported QoL according to the visual analogue scale (VAS) for the same groups were 0.67 (0.66-0.69), 0.67 (0.66-0.69), 0.62 (0.59-0.65) and 0.62 (0.54-0.70) respectively. Utilities were consistently lower in women versus men (no lesions 0.71 vs. 0.78; premalignant conditions 0.70 vs. 0.82; treated for cancer 0.72 vs. 0.78 and present cancer 0.66 vs. 0.70). CONCLUSION: The health-related QoL utilities of patients with premalignant conditions are similar to those without gastric diseases whereas patients with present cancer show decreased utilities. Moreover, women had consistently lower utilities than men. These results confirm that the use of a single standardized instrument such as the EQ-5D-5L for all stages of the gastric carcinogenesis cascade is feasible and that it captures differences between conditions and gender dissimilarities, being relevant information for authors pretending to conduct further cost-utility analysis.
- INFLUÊNCIA DA INICIATIVA HOSPITAL AMIGO DOS BEBÉS NA TAXA DE SUCESSO DE ALEITAMENTO MATERNOPublication . Freitas, I.; Mendes, J.; Monteiro, P.; Chaves, S.; Moreira, S.; Ferreira, S.
- LINFOPENIA T CD4 NO LUPUS ERITEMATOSO SISTÉMICOPublication . Ferreira, S.; Vasconcelos, J.; Marinho, A.; Farinha, F.; Almeida, I.; Correia, J.; Barbosa, P.; Mendonça, T.; Vasconcelos, C.Abstract: Background: Systemic Lupus Erythematosus (SLE) is an inflammatory chronic disease characterized by the presence of autoantibodies, immunocomplex production and organ injury. Several alterations of the immune system have been described, namely of CD4 T cells, with particular focus on regulatory subgroup. Objective: Quantify peripheral CD4 T cells in a population of patients with SLE and correlate it with lupus activity, affected organs, therapeutics and infections. Methods: Retrospective study involving all SLE patients seen in the clinical immunology outpatient clinic of the Hospital Geral Santo António, Porto that has done some peripheral blood flow cytometry study. Results: Twenty-nine patients have been evaluated, 16 were taking glucocorticoids and six immunossupressors. The mean SLEDAI at the study time was nine and the ECLAM was three. Thirty-one percent of the patients had leukopenia, 76% lymphocytopenia and the same number CD4 depletion. Fifty-five percent of the patients had CD4 levels lower than 500/mm3, 31% lower than 200/mm3. All patients with SLEDAI ?20 and ECLAM ?4 had CD4 counts inferior to 500/mm3 and all patients with inactive disease had CD4 superior to 500/mm3. There have been three opportunistic infections: cryptococcal meningitis, pulmonary aspergilosis, Pneumocystis jirovecii pneumonia, all in patients with CD4 counts lower than 500/mm3. Conclusion: Decreased CD4 T cells counts have been very common in this study population. There is an inverse relation between CD4 cells counts and disease activity. Opportunistic infections occurred in patients with severe CD4 depletion. Keywords: Systemic Lupus Erythematosus; CD4 T Lymphocytes; Lymphocytopenia; SLE Activity; Opportunistic infections
- Papuloerythroderma of Ofuji: First Case Report in PortugalPublication . Raposo, I.; Sanches, M.; Ferreira, S.; Selores, M.
- Paraplegia espástica familiar tipo 4 – antecipação ou variabilidade fenotípica?Publication . Rodrigues, N.; Ferreira, S.; Rodrigues, L.; Castro, A.; Barbosa, C.; Gomes, R.Introdução: A Paraplegia Espástica Familiar (PEF) é um grupo heterogéneo de doenças neurodegenerativas hereditárias, com uma prevalência de 2/100000 indivíduos na população portuguesa. Caracteriza-se sobretudo por espasticidade progressiva e insidiosa dos membros inferiores, por degeneração do feixe corticoespinhal. Caso Clínico: Apresentamos uma criança com espasticidade e hiperreflexia progressivas dos membros inferiores, com vários familiares da linha paterna com sintomatologia semelhante, sugerindo ao diagnóstico de paraplegia espástica familiar. Foi identificada no probando uma mutação causal de paraplegia espástica familiar tipo 4. Conclusão: Nesta família, a idade de início variou entre os cinco e os 50 anos, e diminuiu em média 22,5 anos ao longo de três gerações e, por outro lado, a apresentação e evolução da doença foram aparentemente mais graves em gerações sucessivas, sugerindo a existência de fenómeno de antecipação. ABSTRACT Introduction: Hereditary spastic paraplegia is a heterogeneous group of inherited neurodegenerative diseases, with a prevalence of 2/100000 in the Portuguese population. It is mainly characterized by progressive and insidious spasticity of the lower limbs due to degeneration of corticospinal tracts. Case Report: We present a child with progressive spasticity and hyperreflexia of lower limbs, with several relatives of the paternal line with similar symptoms, suggesting the diagnosis of hereditary spastic paraplegia. A causing mutation of hereditary spastic paraplegia type 4 was identified in the proband. Conclusion: In this family, the age of onset varies from five to 50 years, and decreased in average 22,5 years over three generations. The clinical presentation and progression apparently tended to be more severe in successive generations, witch these suggests the phenomenon of anticipation.
- Psoríase e Doença Cardiovascular: Impacto da Terapêutica com Inibidores do TNF-alfaPublication . Andrade, F.; Ferreira, S.; Torres, T.A psoríase é uma doença inflamatória crónica que atinge 2 a 3% da população. Vários estudos têm demonstrado que a psoríase se associa a um aumento de risco de doença cardiovascular devido ao aumento da prevalência de fatores de risco cardiovasculares tradicionais, e à inflamação sistémica crónica que promove o desenvolvimento de disfunção endotelial e aterosclerose prematura. Esta revisão da literatura tem como objetivo avaliar o impacto dos inibidores do fator de necrose tumoral alfa na função endotelial e no risco de doença cardiovascular nos doentes com psoríase moderada a grave. Os estudos realizados até ao momento sugerem que o tratamento da psoríase com estes fármacos se associa a melhoria dos marcadores de disfunção endotelial e aterosclerose subclínica e a diminuição do risco de doença cardiovascular.