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- Portuguese Version of the Pain Beliefs and Perceptions Inventory: a Multicenter Validation StudyPublication . Azevedo, LF; Sampaio, R; Dias, C; Romão, J; Lemos, L; Agualusa, L; Vaz-Serra, S; Patto, T; Costa-Pereira, A; Castro-Lopes, JMBackground: We aimed to perform the translation, cultural adaptation, and validation of the Pain Beliefs and Perceptions Inventory (PBPI) for the European Portuguese language and chronic pain population. Methods: This is a longitudinal multicenter validation study. A Portuguese version of the PBPI (PBPI-P) was created through a process of translation, back translation, and expert panel evaluation. The PBPI-P was administered to a total of 122 patients from 13 chronic pain clinics in Portugal, at baseline and after 7 days. Internal consistency and test-retest reliability were assessed by Cronbach's alpha (α) and intraclass correlation coefficient (ICC). Construct (convergent and discriminant) validity was assessed based on a set of previously developed theoretical hypotheses about interrelations between the PBPI-P and other measures. Exploratory and confirmatory factor analyses were performed to test the theoretical structure of the PBPI-P. Results: The internal consistency and test-retest reliability coefficients for each respective subscale were α = 0.620 and ICC = 0.801 for mystery; α = 0.744 and ICC = 0.841 for permanence; α = 0.778 and ICC = 0.791 for constancy; and α = 0.764 and ICC = 0.881 for self-blame. Exploratory and confirmatory factor analysis revealed a four-factor structure (performance, constancy, self-blame, and mystery) that explained 63% of the variance. The construct validity of the PBPI-P was shown to be adequate, with more than 90% of the previously defined hypotheses regarding interrelations with other measures confirmed. Conclusion: The PBPI-P has been shown to be adequate and to have excellent reliability, internal consistency, and validity. It may contribute to a better pain assessment and is suitable for research and clinical use.
- Ventilação Não Invasiva: Três Anos de Experiência de Uma Unidade de Cuidados Intermédios PediátricosPublication . Correia, C; Parreira, L; Painho, T; Fernandes, A; Lima, S; Oom, PIntrodução: A ventilação não invasiva tem sido utilizada de forma crescente em crianças com insuficiência respiratória aguda,evitando as complicações associadas à ventilação invasiva. Pretendeu-se descrever a experiência de ventilação não invasiva numa unidade de cuidados intermédios e avaliar a sua eficácia em evitar a transferência das crianças para uma unidade de cuidados intensivos pediátricos. Métodos: Estudo longitudinal retrospetivo de todas as crianças admitidas numa unidade de cuidados intermédios e submetidas a ventilação não invasiva (2012-2015). Foram analisados dados demográficos, ocorrência de complicações, frequência respiratória, frequência cardíaca, pH, pressão parcial de dióxido de carbono e relação entre a saturação periférica de oxigénio e fração inspiratória de oxigénio, antes e duas, quatro, seis, 12 e 24 horas após inicio da ventilação não invasiva. Os doentes foram divididos em dois grupos, sucesso ou insucesso da ventilação não invasiva, de acordo com a necessidade de transferência. Resultados: Foram incluídas 35 crianças com mediana de idade 42 dias. O diagnóstico principal foi bronquiolite em 28 doentes e a indicação para iniciar a ventilação não invasiva foi insuficiência respiratória aguda em 32. Foram eficazmente ventilados 29 (82,9%) e seis necessitaram de ser transferidos. Verificou-se uma melhoria da frequência respiratória, frequência cardíaca, pH e pressão parcial de dióxido de carbono a partir das duas horas de ventilação não invasiva no grupo de sucesso (p < 0,05). Não houve complicações major. Discussão: Confirmou-se a efectividade e segurança da ventilação não invasiva em evitar o agravamento clínico das crianças com insuficiência respiratória aguda numa unidade de cuidados intermédios, evitando a ventilação invasiva e/ou transferência para uma unidade de cuidados intensivos pediátricos.
- Impacto Alimentar Esofágico, um Dilema DiagnósticoPublication . Rúbio, C; Furtado, F; Cunha, F; Queirós, G; Oliveira, L; Cabral, JO impacto alimentar esofágico é uma situação que na maioria dos casos é secundária a patologia esofágica congénita ou adquirida, podendo constituir a primeira manifestação da doença. Descreve-se um rapaz, 11 anos, saudável, com sialorreia e dor retrosternal que surgiram subitamente durante o jantar. Foi submetido a endoscopia digestiva alta, que revelou um fragmento de carne impactado no terço distal do esófago (que foi extraído) e estrias longitudinais na mucosa. A análise histológica das biópsias esofágicas foi compatível com eosinofilia esofágica. Iniciou terapêutica com inibidor da bomba de protões com melhoria clínica e histológica. O diagnóstico diferencial das principais causas de impacto alimentar associadas a eosinofilia esofágica, nomeadamente esofagite eosinofílica, doença do refluxo gastroesofágico e eosinofilia esofágica respondedora a inibidor da bomba de protões, constitui um desafio clínico.
- Cost-Effectiveness of the 13-Valent Pneumococcal Conjugate Vaccine in Children in PortugalPublication . Gouveia, M; Fiorentino, F; Jesus, G; Costa, J; Borges, MPneumococcal infections are the leading cause of vaccine-preventable death in children. In June 2015, the 13-valent pneumococcal conjugate vaccine (PCV13) was introduced in the Portuguese Immunization Program. We evaluated the cost-effectiveness of children vaccinated with PCV13 versus no vaccination for preventing pneumococcal diseases.
- Outcomes of Parenchyma-Preserving Hepatectomy and Right Hepatectomy for Solitary Small Colorectal Liver Metastasis: A LiverMetSurvey StudyPublication . Hosokawa, I; Allard, MA; Mirza, DF; Kaiser, G; Barroso, E; Lapointe, R; Laurent, C; Ferrero, A; Miyazaki, M; Adam, RBACKGROUND: Occasionally, right hepatectomy, rather than parenchyma-preserving hepatectomy, has been performed for solitary small colorectal liver metastasis. The relative oncologic benefits of parenchyma-preserving hepatectomy and right hepatectomy are unclear. This study compared the outcomes of patients with solitary small colorectal liver metastasis in the right liver who underwent parenchyma-preserving hepatectomy and those who underwent right hepatectomy. METHODS: The study population consisted of a multicentric cohort of 21,072 patients operated for colorectal liver metastasis between 2000 and 2015 whose data were collected in the LiverMetSurvey registry. Patients with a pathologically confirmed solitary tumor of less than 30 mm in size in the right liver were included. The short- and long-term outcomes of patients who underwent parenchyma-preserving hepatectomy were compared to those of patients who underwent right hepatectomy. RESULTS: Of the 1,720 patients who were eligible for the study, 1,478 (86%) underwent parenchyma-preserving hepatectomy and 242 (14%) underwent right hepatectomy. The parenchyma-preserving hepatectomy group was associated with lower rates of major complications (3% vs 10%; P < .001) and 90-day mortality (1% vs 3%; P = .008). Liver recurrence occurred similarly in both groups (20% vs 22%; P = .39). The 5-year recurrence-free survival and overall survival rates were similar in both groups. However, in patients with liver-only recurrence, repeat hepatectomy was more frequently performed in the parenchyma-preserving hepatectomy group than in the right hepatectomy group (67% vs 31%; P < .001), and the overall 5-year survival rate was significantly higher in the parenchyma-preserving hepatectomy group than in the right hepatectomy group (55% vs 23%; P < .001). CONCLUSION: Parenchyma-preserving hepatectomy should be considered the standard procedure for solitary small colorectal liver metastasis in the right liver when technically feasible.
- Prevalence of Late-Onset Pompe Disease in Portuguese Patients with Diaphragmatic Paralysis - DIPPER StudyPublication . Guimarães, MJ; Winck, JC; Conde, B; Mineiro, A; Raposo, M; Moita, J; Marinho, A; Silva, JM; Pires, N; André, S; Loureiro, CPompe disease is a rare autosomal recessive neuromuscular disorder caused by acid α-glucosidase enzyme (GAA) deficiency and divided into two distinct variants, infantile- and late-onset. The late-onset variant is characterized by a spectrum of phenotypic variation that may range from asymptomatic, to reduced muscle strength and/or diaphragmatic paralysis. Since muscle strength loss is characteristic of several different conditions, which may also cause diaphragmatic paralysis, a protocol was created to search for the diagnosis of Pompe disease and exclude other possible causes. METHODS: We collected a sample size of 18 patients (10 females, 8 males) with a median age of 60 years and diagnosis of diaphragmatic paralysis of unknown etiology, followed in the Pulmonology outpatient consultation of 9 centers in Portugal, over a 24-month study period. We evaluated data from patient's clinical and demographic characteristics as well as complementary diagnostic tests including blood tests, imaging, neurophysiologic and respiratory function evaluation. All patients were evaluated for GAA activity with DBS (dried blood test) or serum quantification and positive results confirmed by serum quantification and sequencing. RESULTS: Three patients were diagnosed with Pompe's disease and recommended for enzyme replacement therapy. The prevalence of Pompe, a rare disease, in our diaphragmatic paralysis patient sample was 16.8%. CONCLUSION: We conclude that DBS test for GAA activity should be recommended for all patients with diaphragmatic paralysis which, despite looking at all the most common causes, remains of unknown etiology; this would improve both the timing and accuracy of diagnosis for Pompe disease in this patient population. Accurate diagnosis will lead to improved care for this rare, progressively debilitating but treatable neuromuscular disease.
- Multistage ECMO Cannulas: First Holes Get It All?Publication . Fortuna, P; Germano, N; José, C; Martins, A
- Cognitive Impairment and Magnetic Resonance Imaging Correlates in Primary Progressive Multiple SclerosisPublication . Gouveia, A; Dias, S; Santos, T; Rocha, H; Coelho, C; Ruano, L; Galego, O; Diogo, M; Seixas, D; Sá, MJ; Batista, SObjectives: To characterize cognitive impairment in primary progressive multiple sclerosis (PPMS) and to correlate the pattern of cognitive deficits with brain magnetic resonance imaging (MRI) volumetric data. Materials and methods: In a multicenter cross-sectional study, we recruited consecutive patients with PPMS as well as age, sex, and education level-matched healthy controls (HC). All participants underwent neuropsychological (NP) assessment, and brain MRI was performed in patients with PPMS for analysis of lesion load, subcortical GM volumes, and regional cortical volumes. Results: We recruited 55 patients with PPMS and 36 HC. Thirty-six patients were included in the MRI analysis. Patients with PPMS performed significantly worse than HC in all NP tests. Subcortical GM volume was significantly correlated with all NP tests, except for Stroop Test, with the largest effect for the thalamus (r=-.516 [BVMT-R DR, P=.016 FDR-corrected] to r=.664 [SDMT, P<.001 FDR-corrected]). In the stepwise linear regression model, thalamic volume was the only predictor of performance in all NP tests. Conclusion: Cognitive impairment is common in PPMS and affects all evaluated cognitive domains. Subcortical GM volume, particularly of the thalamus, is a strong predictor of cognitive performance, suggesting it has a central role in the pathophysiology of PPMS-related cognitive dysfunction.
- Tumor Miofibroblástico Inflamatório da Bexiga em Doente de 6 Anos de IdadePublication . Nascimento, L; Romagosa, C; Castellote, AO tumor miofibroblástico inflamatório é uma neoplasia rara, proveniente do tecido visceral e partes moles, que pode ocorrer em qualquer parte do corpo. Surge maioritariamente nas duas primeiras décadas de vida. É tendencialmente benigno, mas frequentemente confundido com tumor maligno pelo seu aspecto agressivo em imagem. A maioria destes tumores que ocorrem no sistema genito-urinário envolvem a bexiga. O sintoma de apresentação mais frequente é a hematúria. O principal diagnóstico diferencial em idade pediátrica é o rabdomiossarcoma, o tumor vesical mais frequente, cujo tratamento é cistectomia radical com linfadenectomia pélvica. No caso do tumor miofibroblástico inflamatório a cirurgia é conservadora. Descrevemos o caso de uma doente de 6 anos previamente saudável, que iniciou um quadro de disúria, polaquiúria e hematúria macroscópica, tendo sido identificada por ecografia uma massa vesical, diagnosticada como um tumor miofibroblástico inflamatório da bexiga