A supervised versus home-based program effects on liver transplanted familial amyloidotic polyneuropathy patients: walking, fatigue and quality of life

Purpose: To evaluate the effects of a six months exercise training program on walking capacity, fatigue and health related quality of life (HRQL). Relevance: Familial amyloidotic polyneuropathy disease (FAP) is an autossomic neurodegenerative disease, related with systemic deposition of amyloidal fibre mainly on peripheral nervous system and mainly produced in the liver. FAP often results in severe functional limitations. Liver transplantation is used as the only therapy so far, that stop the progression of some aspects of this disease. Transplantation requires aggressive medication which impairs muscle metabolism and associated to surgery process and previous possible functional impairments, could lead to serious deconditioning. Reports of fatigue are common feature in transplanted patients. The effect of supervised or home-based exercise training programs in FAP patients after a liver transplant (FAPTX) is currently unknown.

Comparison of body fat content and distribution of familial amyloidotic polyneuropathy patients versus healthy subjects

The deposition of amyloid fibers at the peripheral nervous system can induce motor neuropathy in Familial Amiloidotic Polyneuropethy (FAP) patients. This produces progressive reductions in functional capacity. The only treatment for FAP is a liver transplant, followed by aggressive medication that can affect patients' metabolism. To our knowledge, there are no data on body fat distribution or comparison between healthy and FAP subjects, which may be important for clinical assessment and management of this disease. PURPOSE: To analyze body fat content and distribution between FAP patients and healthy subjects. METHODS: Body fat content and distribution were measured through Double Energy X-ray Densitometry (DXA) in two groups. Group 1 consisted of 43 Familial Amyloidotic Polyneuropathy patients (19 males, 32 + 8 Yrs, and 24 females, 37 + 5 yrs), who had liver transplant less than 2 months before. Group 2 consisted of 18 healthy subjects of similar age (8 males, 36 + 7 yrs, and 10 females, 39 + 5 yrs). RESULTS: Healthy subjects showed higher values than FAP patients for: BMI (24,2+2,3kg/m2 vs 22,3+3,8 kg/m2 respectively, p<0,05), % trunk BF (26,21+8,34kg vs 20,78+9,05kg respectively, p<0,05), % visceral BF (24,43+7,97% vs 19,21+9,30% respectively, p<0,05), % abdominal BF (26,63+8,51% vs 20,63+10,35% respectively, p<0,05) abdominal subcutaneous BF (0,533+0,421kg vs 0,353+0,257kg respectively, p=0,05), abdominal BF/BF ratio (0,09+0,02 vs 0,08+0,02 respectively, p<0,05) and abdominal BF/trunk BF ratio (0,19+0,03 vs 0,17+0,03 respectively, p<0,05). CONCLUSIONS: These results showed that FAP patients soon after liver transplantation exhibited a healthier body fat profile compared to controls. However, fat content and distribution varied widely in FAP subjects, suggesting an individualized approach for assessment and intervention rather than general guidelines. Future research is needed to investigate the long term consequences on body fat following liver transplant in this population.

Comparison of body fat content and distribution of familial amyloidotic polyneuropathy patients versus healthy subjects

The deposition of amyloid fibers at the peripheral nervous system can induce motor neuropathy in Familial Amiloidotic Polyneuropethy (FAP) patients. This produces progressive reductions in functional capacity. The only treatment for FAP is a liver transplant, followed by aggressive medication that can affect patients' metabolism. To our knowledge, there are no data on body fat distribution or comparison between healthy and FAP subjects, which may be important for clinical assessment and management of this disease.

Strength, muscle quality and functional capacity in liver transplanted familial amyloidotic polineuropathy patients

Liver transplantation is the unique treatment for several end-stage diseases. Familial Amiloidotic Polineuropathy (FAP) is a neurodegenerative disease related with systemic deposition of amyloidal fiber mainly on peripheral nervous system, clinically translated by an autonomous sensitive-motor neuropathy with severe functional limitations in some cases. The unique treatment for FAP disease is a liver transplant with a very aggressive medication to muscle metabolism and force production. To our knowledge there are no quantitative characterizations of body composition, strength or functional capacity in this population.

Effects of a randomized trial of exercise on body composition of liver transplanted patients

Liver transplantation is used as a only therapy so far, that stop the progression of some aspects of familial amyloidotic polyneuropathy disease (FAP) an autossomic neurodegenerative disease. FAP often results in severe functional limitations. Transplantation requires aggressive medication which impairs bone and muscle metabolism. Malnutrition plus weight loss is already one feature of FAP patients. All this may produce negative consequences on body composition. The effect of exercise training in FAP patients after a liver transplant (FAPTX) is currently unknown. The purpose of this study is to evaluate the effects of a six months exercise training program on body composition in FAPTX patients.

The portuguese insurance industry: basis for strategic process decision

Nowadays, the Portuguese insurance industry operates in a market with a much more aggressive structure than a few decades ago. Markets and the economy have become globalised since the last decade of the 20th century. Market forces have gradually shifted – power is now mainly on the demand side. In order to meet the new requirements, the insurance industry must develop a strong strategic ability to respond to constant changes of the new international economic order.One of the basic aspects of this strategic development will focus on the ability to predict the future. We introduce the subject by briefly describing the sector, its organisational structure in the Portuguese market, and challenges arising from the development of the European Union. We then analyse the economic and financial structure of the sector. From this point of view, we aim at the possibility of designing models that could explain the demand for insurance, claims and technical reserves evolution. Such models, (even if based on the past), would resolve, at least partly, one of the greatest difficulties experienced by insurance companies when estimating the budget. Thus, we examine the existence of variables that explain the previous points, which are capable of forming a basis for designing models that are simple but efficient, and can be used for strategic planning.

Electromagnetic transients analysis of lightning overvoltages on wind power plants

As wind power generation undergoes rapid growth, lightning and overvoltage incidents involving wind power plants have come to be regarded as a serious problem. Firstly, lightning location systems are discussed, as well as important parameters regarding lightning protection. Also, this paper presents a case study, based on a wind turbine with an interconnecting transformer, for the study of adequate lightning and overvoltage protection measures. The electromagnetic transients circuit under study is described, and computational results are presented.

Strength, muscle quality and functional capacity in liver transplanted familial amyloidotic polineuropathy patients

Liver transplantation is the unique treatment for several end stage diseases. Familial Amiloidotic Polineuropathy (FAP) is a neurodegenerative disease related with systemic deposition of amyloidal fibre mainly on peripheral nervous system, clinically translated by an autonomous sensitive-motor neuropathy with severe functional limitations in some cases. The unique treatment for FAP disease is a liver transplant with a very aggressive medication to muscle metabolism and force production. To our knowledge there are no quantitative characterizations of body composition, strength or functional capacity in this population. The purpose of this study was to compare levels of specific strength (isometric strength adjusted by lean mass or muscle quality) and functional capacity (meters in 6 minutes walk test) between FAP patients after a liver transplant (4.1±2 months after transplant surgery) (FAPT) and a healthy group (HG).

O fim de um ciclo? A coconstrução da articulação curricular entre o 1.º e o 2.º Ciclos do Ensino Básico

Dissertação Apresentada à Escola Superior de Educação de Lisboa para obtenção de grau de mestre em Ciências da Educação - Especialidade Supervisão em Educação

Avaliação ecocardiográfica da função sistólica ventricular direita em doentes com disfunção ventricular esquerda: parâmetros convencionais versus strain bidimensional

Mestrado em Tecnologia de Diagnóstico e Intervenção Cardiovascular.

Historical Heritage: A Study to Conservation

Considering ancient monuments and historical buildings, it seems that these mortars have proved to be durable and reliable materials. The restoration and maintenance of old renders is one of the key aspects of correct rehabilitation practice. The ideal course of action is to replace the damaged material by a material with compatible characteristics.The study in development presents the chemical, physical and morphologic analysis performed for ancient air lime mortars belonging to historical monuments: Santa Marta Fortress in the coast line Lisbon-Cascais dated from XVII century and Defense Wall of Lisbon dated from XI century, which layout could be associated to roman period. It is important to underline that the studied samples of ancient portuguese air lime mortars, have been submitted during centuries to very severe maritime environment that includes daily cycles of wet/dry, wind, friction and the constant presence of salts, generally aggressive. However, they show very good performance and conservation state, unlike most of the new air lime mortars, which are generally considered weak, not very durable, materials. This work is included in a study intending to determine key factors to the durability of these ancient materials in presence of water. Visible reaction rims around some aggregates suggests the occurrence of pozzolanic reactions between aggregates and the lime binder that creates neoformation products, such as calcium-silico-aluminates, which seems be, besides the pores filling, the responsible for the resistance and cohesion of these ancient mortars submitted to aggressive humid environments.

As duas margens do rio: contrastes urbanos e regulação da violência na escola

o objeto deste artigo é analisar as respostas da escola ao fenômeno da violência em meio escolar e qual a influência das características dos contextos urbanos nesse processo. A identificação de escolas situadas em contextos urbanos fortemente degradados e com elevada conflitualidade e desigualdade social, mas que apresentavam níveis reduzidos de violência no seu interior e, inversamente, escolas com uma frequência elevada de ocorrências que se situavam em contextos urbanos mais estruturados e maioritariamente de classe média, levou- nos a questionar as concepções largamente aceitas e difundidas sobre o aparente contágio de conflitualidade em escolas situadas em meios sociais desfavorecidos. As evidências encontradas na análise dessa relação entre as escolas e o contexto social local permitiram- nos refutar o determinismo das características do meio social sobre as escolas, bem como a consequente incapacidade dessas na regulação e prevenção da violência. o centramento da pesquisa na dimensão organizacional, em concreto nas estratégias definidas e desenvolvidas pelas escolas no combate ao problema da violência, permitiu-nos compreender e demonstrar que as orientações, as práticas e os processos organizacionais têm um papel decisivo na pacificação dos quotidianos escolares.

Indisciplina na sala de aula: a perspetiva de professores do 3º Ciclo do Ensino Básico e do Ensino Secundário

Dissertação apresentada à Escola Superior de Educação de Lisboa para obtenção de grau de mestre em Ciências da Educação

Bullying escolar – Intervenientes, ações e sentimentos associados: um estudo com alunos do 2º e 3º ciclos da região do Algarve

Introdução – O bullying pode ser definido como atitudes agressivas, intencionais e repetidas durante um período de tempo. Diversos estudos verificaram a prevalência de bullying entre estudantes de vários países e demonstraram que este é um problema internacional e que pelo menos um em cada dez estudantes está envolvido numa situação de bullying. Objetivos – Caracterizar as situações de bullying no ambiente escolar, o papel do estudante, os sentimentos associados a essas ações e identificar as características do agressor. Métodos – A amostra foi constituída por 519 alunos matriculados em duas escolas da região sul de Portugal que preencheram um questionário anónimo sobre uma situação de bullying que vivenciaram, descrevendo o papel do aluno e o que sentiram nesta situação. Resultados – Os resultados revelaram que em 61,7% dos casos se tratou de agressão física e 29,7% de agressão verbal. Desempenharam o papel de agressores 12,7% dos alunos, 21,8% foram vítimas e 63,6% foram testemunhas desta situação. 10,6% dos alunos relataram sentir bem, 11% mostraram indiferença e 78,4% dos alunos sentiram‑se mal com a situação de bullying. Verificou‑se que, com o avanço da idade, o estudante aumentava em 1,5 vezes a probabilidade de desempenhar o papel de agressor e os rapazes apresentavam 5,2 vezes mais probabilidades de vir a ser agressor numa situação de bullying. Conclusão – O presente estudo verificou que a maioria dos alunos participou de uma situação de bullying escolar como testemunha, sendo os casos mais comuns de agressão física. A maioria dos alunos sentiu‑se mal com essa situação. Os rapazes e os alunos com mais idade tiveram mais probabilidade de vir a desempenhar o papel de agressor numa situação de bullying.

Synthesis and characterization of rabies virus glycoprotein-tagged amphiphilic cyclodextrins for siRNA delivery in human glioblastoma cells: in vitro analysis

In man brain cancer is an aggressive, malignant form of tumour, it is highly infiltrative in nature, is associated with cellular heterogeneity and affects cerebral hemispheres of the brain. Current drug therapies are inadequate and an unmet clinical need exists to develop new improved therapeutics. The ability to silence genes associated with disease progression by using short interfering RNA (siRNA) presents the potential to develop safe and effective therapies. In this work, in order to protect the siRNA from degradation, promote cell specific uptake and enhance gene silencing efficiency, a PEGylated cyclodextrin (CD)-based nanoparticle, tagged with a CNS-targeting peptide derived from the rabies virus glycoprotein (RVG) was formulated and characterized. The modified cyclodextrin derivatives were synthesized and co-formulated to form nanoparticles containing siRNA which were analysed for size, surface charge, stability, cellular uptake and gene-knockdown in brain cancer cells. The results identified an optimised co-formulation prototype at a molar ratio of 1:1.5:0.5 (cationic cyclodextrin:PEGylated cyclodextrin:RVG-tagged PEGylated cyclodextrin) with a size of 281±39.72nm, a surface charge of 26.73±3mV, with efficient cellular uptake and a 27% gene-knockdown ability. This CD-based formulation represents a potential nanocomplex for systemic delivery of siRNA targeting brain cancer.