Body composition, muscle strength, functional capacity, and physical disability risk in liver transplanted familial amyloidotic polyneuropathy patients

Abstract: Background: Familial amyloidotic polyneuropathy (FAP) is a neurodegenerative disease leading to sensory and motor polyneuropathies, and functional limitations. Liver transplantation is the only treatment for FAP, requiring medication that negatively affects bone and muscle metabolism. The aim of this study was to compare body composition, levels of specific strength, level of physical disability risk, and functional capacity of transplanted FAP patients (FAPTx) with a group of healthy individuals (CON). Methods: A group of patients with 48 FAPTx (28 men, 20 women) was compared with 24 CON individuals (14 men, 10 women). Body composition was assessed by dual-energy X-ray absorptiometry, and total skeletal muscle mass (TBSMM) and skeletal muscle index (SMI) were calculated. Handgrip strength was measured for both hands as was isometric strength of quadriceps. Muscle quality (MQ) was ascertained by the ratio of strength to muscle mass. Functional capacity was assessed by the six-minute walk test. Results: Patients with FAPTx had significantly lower functional capacity, weight, body mass index, total fat mass, TBSMM, SMI, lean mass, muscle strength, MQ, and bone mineral density. Conclusion: Patients with FAPTx appear to be at particularly high risk of functional disability, suggesting an important role for an early and appropriately designed rehabilitation program.

Effects of an exercise training program in physical condition after liver transplantation in familial amyloidotic polyneuropathy: a case report

Introduction: Familial amyloidotic polyneuropathy (FAP) is a neurodegenerative disease that leads to sensory and motor polyneuropathies as well as functional limitations. So far, liver transplantation is the only treatment for FAP because the mutated protein causing the disease is mainly produced in the liver. With the increasing survival of transplant recipients, functional and cardiovascular problems as consequences of immunosuppressant side effects are increasing associated with sedentary lifestyles and/or retransplantation status. We sought to analyze the impact of exercise training programs on 1 FAP patient’s course long-term after liver transplantation. Methodology. A FAP patient (female; 49 years of age; body mass index 18.8 kg/m2) underwent a liver transplantation 133 months before assessment. She was assessed for body composition, isometric quadriceps muscle strength, functional capacity, fatigue, and levels of physical activity before and after a 6-month period of combined exercise training. Results: After the exercise training program, almost all variables were improved, namely, total body skeletal muscle mass, proximal femoral bone mineral density, quadriceps strength, maximal oxygen consumption on 6 minutes walk test (6mwt) or VO2peak, total ventilation on 6mwt, and fatigue. The improvement in distance on 6mwt (69.2 m) was clinically significant. Preintervention the levels of physical activity were below international recommendations for health; after the program they achieved the recommendations. Conclusion: The results showed an improvement in functional capacity with a decrease in future disability risk associated with a better lifestyle with respect to physical activity levels in 1 patient.

Development of a penetration test for timber impregnation products for use in old buildings

This paper studies the application of commercial biocides to old maritime pine timber structures (Pinus pinaster Ait.) that have previously been impregnated with other products. A method was developed in the laboratory to be used in situ to determine the impregnation depth achieved by a new generation biocide product applied to timber from an old building. This timber had once been treated with an unknown product difficult to characterize without extensive analysis. The test was initially developed in laboratory conditions and later tested on elements of the roof structure of an 18th century building. In both cases the results were promising and mutually consistent with penetration depths for some treatments reaching 2.0 cm. The application in situ proved the tests viability and simplicity of execution giving a clear indication on the feasibility of possible re-treatments.

Paleomagnetic study of the Great Foum Zguid dyke (southern Morocco): A positive contact test related to metasomatic processes

When a paleomagnetic pole is sought for in an igneous body, the host rocks should be subjected to a contact test to assure that the determined paleopole has the age of the intrusion. If the contact test is positive, it precludes the possibility that the measured magnetization is a later effect. Therefore, we investigated the variations of the remanent magnetization along cross-sections of rocks hosting the Foum Zguid dyke (southern Morocco) and the dyke itself. A positive contact test was obtained, but it is mainly related with Chemical/Crystalline Remanent Magnetization due to metasomatic processes in the host-rocks during magma intrusion and cooling, and not only with Thermo-Remanent Magnetization as ordinarily assumed in standard studies. Paleomagnetic data obtained within the dyke then reflect the Earth magnetic field during emplacement of this well-dated (196.9 +/- 1.8 Ma) intrusion.

Development of an in situ penetration test for the uptake of preservatives in applied wood

This paper concerns the study of biocides application in old timber structures of maritime pine (Pinus pinaster Ail.), previously impregnated with other products. A method was developed in laboratory to determine in situ the penetration depth of a product applied superficially. As initial treatment, three traditional products for sawn timber for buildings were used and, for new treatments, two newer, more environmentally benign products were used. Their ability to penetrate the pre-treated surfaces was evaluated after 1, 2 and 3 applications at 24 hours intervals and the results obtained are presented. Finally, the applicability of the developed test to the in-situ evaluation of timber structures is also discussed.

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).

Perimetria de hiperacuidade preferencial no estudo do edema macular diabético

Introdução – A diabetes é uma das maiores epidemias do último século. Mais de 250 milhões de pessoas, em todo o mundo, sofrem de diabetes. Das complicações derivadas da diabetes são as principais causas de cegueira, de insuficiência renal e de amputação de membros inferiores, derivando estes, predominantemente, da disfunção vascular. Quando surge perda de pericitos na parede vascular ocorrem uma série de alterações da microcirculação que levam ao aparecimento de microaneurismas e outras alterações vasculares que possibilitam a passagem de componentes sanguíneos para o tecido retiniano adjacente que, em situação de normalidade, não ocorreriam, sendo esta uma das causas do edema macular exsudativo diabético. A perimetria de hiperacuidade preferencial (PHP) é um teste psicofísico que pretende detetar metamorfopsias na Degenerescência Macular ligada à Idade (DMI). Uma vez que o edema macular diabético (EMD) se destaca como uma das principais causas de deficiência visual e baixa visão, pretende-se verificar a eficácia do PHP no estudo do edema macular diabético, respondendo à seguinte questão: “Qual a capacidade do perímetro de hiperacuidade preferencial em detetar metamorfopsias em pacientes com edema macular diabético?“ Metodologia – Estudo quantitativo, do tipo descritivo e correlacional. Selecionou-se uma amostra de 33 pacientes, onde se analisou um total de 60 olhos. Resultados – A sensibilidade do PHP na deteção de metamorfopsias associadas ao EMD na tomografia de coerência ótica (OCT) foi de 70,6%, a especificidade foi de 11,5% e a eficiência global do teste de 45%. Comparando os resultados encontrados no PHP e no OCT, constatou-se a existência de uma correlação inversa fraca (Phi = -0,215). Conclusões – Este novo método de diagnóstico revela-se sensível, contudo pouco específico e eficaz na deteção de metamorfopsias consequentes da existência de EMD. - ABSTRACT - Introduction – Preferential hyperacuity perimeter (PHP) is a new psychophysical test, which principle is based on the detection of metamorphopsia in age-related macular degeneration (AMD). It is intended to verify its effectiveness in the study of diabetic macular edema (DME). When there is loss of pericytes in the vascular wall occur a number of microcirculatory changes that lead to the appearance of microaneurysms and other vascular changes that allow the passage of blood components to the surrounding retinal tissue than in normal situation does not occur, this being one of the causes exudative diabetic macular edema. Methodology – It was performed a quantitative study, using descriptive and correlational analysis. A sample of 33 patients was selected, and 60 eyes were analyzed. Results – The sensitivity of PHP on the detection of metamorphopsia associated to EMD was 70.6%, the specificity was 11.5% and the global efficiency of the test was 45%. It was found a weak negative correlation (Phi= -0.215) between the PHP and optical coherence tomography (OCT). Conclusions – This new method of diagnosis was sensitive, but not very specific and effective on the detection of metamorphopsia, due to the DME.

Dispositional optimism is unidimensional or bidimensional? The Portuguese Revised Life Orientation Test

The aim of the study is to adapt and then discuss the appropriateness of the Life Orientation Test as a one or two dimension scale. The research includes two studies; one is composed of a sequential sample of 280 people with multiple sclerosis, 71% female, and another includes a convenience sample of 615 individuals from the community, 51.1% female. Because the construct is built upon a theoretical assumption that has one dimension, we examine the hypothesis of one or two factor solutions through confirmatory factor analysis, and the two-dimension solution premise demonstrates better adjustment for both samples. The other psychometric properties explored show appropriate results for the Portuguese sample, and similar to the original ones; the Test therefore seems appropriate for use in cross cultural studies. Based on our results, we discuss whether the questionnaire is a one or two dimension instrument, concluding that it appears appropriate to accept the recommendations of the original authors to use it as a one-dimensional tool and, when necessary, to use both dimensions. - RESUMO: El objetivo del estudio es adaptar y discutir la adecuación de la prueba de Orientación de la Vida en una o dos escalas de dimensión. La investigación engloba dos estudios, uno constituido por una muestra secuencial de 280 personas con esclerosis múltiple, 71% mujeres y otro con una muestra de conveniencia de la comunidad de 615 individuos, 51,1% del sexo femenino. Como el constructo se asienta sobre la presunción teórica de que tiene una dimensión, inspeccionamos la hipótesis de una o dos soluciones de factor a través del análisis factorial confirmatorio y la hipótesis de dos dimensiones manifiesta un mejor ajuste para ambas muestras. Las otras propiedades psicométricas exploradas muestran los resultados apropiados para la muestra portuguesa, y semejantes a los originales. Parece apropiado para los estudios culturales transversales. Basándonos en nuestros resultados, discutimos si el cuestionario es un instrumento de una o dos dimensiones, concluyéndose que parece conveniente seguir las recomendaciones de los autores originales, para utilizarlo como un instrumento unidimensional y, si fuera necesario necesario, utilizar cada una de las dimensiones.

Design of a new test chamber for evaluation of the toxicity of rubber infill

A test chamber was projected and built (according to ISO 16000-9 Standard) to simulate atmospheric conditions experienced by rubber infill (when applied in synthetic turf pitches) and measure accurately the airborne emissions of pollutants such as dusts and volatile organic compounds (VOC), as well as pollutants present in leachates. It should be pointed out that standard ISO 16000-9 is only concerned with the determination of the emission of VOC from building products and furnishing (not specific of synthetic turf materials), whereas other standards are concerned with the emission of leachates only. This procedure is to be considered as a technical option to the lysimeter "global turf system evaluation" when the rubber infill alone is to be evaluated. The advantage of the proposed option considering this "test chamber" is its simplicity and economy. This test chamber is actually installed and being used for tests in LAIST.

Imunorreatividade em material histológico arquivado durante um, quatro e sete anos

Quando aplicada no âmbito da Anatomia Patológica, a imuno-histoquímica tem constituído um poderoso meio de identificação/caracterização de várias estruturas histológicas, permitindo delinear prognóstico e terapêutica para várias patologias. Tendo em conta que as amostras histológicas analisadas podem ser conservadas ao longo de vários anos, interessa avaliar a manutenção da antigenicidade ao longo do tempo, de forma a garantir a qualidade final da técnica quando aplicada em material de arquivo. Assim, o principal objetivo deste trabalho foi comparar a imunorreatividade do material histológico arquivado durante um, quatro e sete anos. Foi utilizado material histológico de próstata, pulmão e mama, no qual se procedeu à imunomarcação de citoqueratinas (Clones AE1/AE3), CD34 e proteína p63, por método de multímero/HRP no sistema Ventana BenchMark Ultra®. Foi realizado um ensaio com recuperação antigênica por alta temperatura (RAAT) e outro sem esta etapa. As imunomarcações (n=162) foram classificadas por três avaliadores independentes num escore quantitativo final (escala 0-100). O par média/desvio-padrão do escore final para os casos com sete anos foi de 69,06/19,05, para os casos com quatro anos foi de 66,47/20,73 e para os casos com um ano foi de 69,08/19,35, não se tendo encontrado diferenças estatisticamente significativas. Os casos sem RAAT obtiveram um par média/desvio-padrão de 54,90/17,00, enquanto os casos com RAAT obtiveram 81,50/11,60, o que revelou diferenças estatisticamente significativas (p=0,000). Para os casos em estudo conclui-se que o fator “tempo de arquivo” não está associado a alterações da imunorreatividade. A importância da RAAT na obtenção de imunomarcação de qualidade sai fortemente realçada. ABSTRACT - When applied within the framework of Pathology, immunohistochemistry has been a powerful means of identification/characterization of various histological structures, allowing to outline prognosis and therapy for various diseases. Given that the analyzed histological samples can be preserved for several years, it is interesting to assess the retention of antigenicity over time in order to ensure the quality of the final technique, when applied to stored material. Thus, the main objective of this study was to compare the immunoreactivity of the histological material archived for one, four and seven years. It was used histological material from prostate, lung and breast, in which it was performed the immunostaining of cytokeratins (clones AE1/AE3), CD34 and p63 protein by the method of multimer/HRP system on a Ventana BenchMark Ultra®. It was conducted a test with heat induced epitope retrieval (HIER) and another one without this step. The stained slides (n=162) were classified by three independent assessors using a quantitative score (scale 0-100). The pair mean/standard deviation of the score for cases with seven years was 69,06/19,05, for cases with four years was 66,47/20,73 and for cases with one year was 69,08/19,35, which did not revealed any statistically significant differences. The cases without HIER had a couple mean/standard deviation of 54.90/17.00 while the cases with HIER obtained 81.50/11.60, which revealed statistically significant differences (p=0.000). For this case study it was concluded that the factor archive period is not associated with changes in immunoreactivity. The importance of HIER in obtaining high quality immunostaining comes out strongly highlighted.

O desenvolvimento da leitura em função de diferentes métodos

Dissertação apresentada à Escola Superior de Educação de Lisboa no âmbito do Mestrado em Ensino Especial

Predicted VO2 on 6 minutes walk test for FAP liver transplanted patients

Physical function is limited post-liver transplantation and exercise training can improve exercise capacity following transplantation but previously assessment of functional capacity is necessary. The 6 minute walk test (6MWT) is routinely used for studying patient’s exercise capacity and is less expensive and time consuming than a Cardiopulmonary Exercise Test (CPXT). There have been some studies looking at the relationship between 6MWT and peak VO2 in patients but few presented an adequate equation for predicted peak VO2 from 6MWT to transplanted patients and none for Familial Amyloidotic Polyneuropathy (FAP) liver transplanted patients (FAPTx). The aim of this study was to compare the validity of 6 equations as predictors of VO2 from the 6MWT and compare it with directly measured VO2 in patients FAPTx.

Lesões da substância branca por ressonância magnética: uma avaliação entre as ponderações DWI e T2 FLAIR

RESUMO: Objetivos – Determinar a sensibilidade e especificidade das ponderações Difusão (DWI) e T2 Fluid-Attenuated Inversion Recovery (FLAIR) na avaliação de lesões da substância branca (SB) e verificar em que medida se complementam, por forma a criar um conjunto de boas práticas na RM cranioencefálica de rotina. Metodologia – Recorrendo-se a uma metodologia quantitativa, efetuou-se uma análise retrospetiva da qual foram selecionados 30 pacientes, 10 sem patologia e 20 com patologia (2 com EM, 7 com Leucoencefalopatia, 6 com doença microangiopática e 5 com patologia da substância branca indefinida). Obteve-se uma amostra de 60 imagens, nomeadamente: 30 imagens ponderadas em DWI e 30 em T2 FLAIR. Recorrendo ao programa Viewdex®, três observadores avaliaram um conjunto de imagens segundo sete critérios: visibilidade, deteção, homogeneidade, localização, margens e dimensões da lesão e capacidade de diagnóstico. Com os resultados obtidos recorreu-se ao cálculo de sensibilidade e especificidade pelas Curvas ROC, bem como à análise estatística, nomeadamente, Teste-T, Índice de Concordância Kappa e coeficiente de correlação de Pearson entre as variáveis em estudo. Resultados – Os resultados de sensibilidade e de especificidade obtidos para a ponderação T2 FLAIR foram superiores (0,915 e 0,038, respetivamente) aos da ponderação DWI (0,08 e 0,100, respetivamente). Não se verificaram variâncias populacionais significativas. Obteve-se uma elevada correlação linear entre as variáveis com um valor r situado entre 0,8 e 0,99. Verificou-se também uma variabilidade considerável entre os observadores. Conclusões – Dados os baixos valores de sensibilidade e especificidade obtidos para a DWI, sugere-se que esta deva ser incluída no protocolo de rotina de crânio como auxiliar no diagnóstico diferencial com outras patologias.

Qualidade do processamento sensorial e vinculação infantil

Estudou-se a relação entre a segurança da vinculação e a qualidade do processamento sensorial na primeira infância. Para o efeito, seleccionou-se uma amostra normativa de 40 díades mãe-bebé, com crianças entre os 11 e os 18 meses. Avaliou-se a qualidade da vinculação, observando a díade no procedimento Situação Estranha. Classificaram-se 17 (42,5%) das crianças no grupo de vinculação segura, sendo que as restantes 23 (57,5%) revelaram uma vinculação não segura. A qualidade do processamento sensorial avaliou-se através do Teste de Funções Sensoriais. Constatou-se que a segurança da vinculação infantil associava-se a um score agregando quatro factores ambientais (nível sócio-económico dos pais; existência de internamentos hospitalares; coeficiente de número de irmãos; local onde a criança passa o dia). O Teste de Funções Sensoriais não apresentou valor prognóstico relativamente ao tipo de vinculação. Porém, a boa qualidade no processamento da informação sensorial parece constituir um factor de resiliência no desenvolvimento da vinculação.

An application of structural equation modeling of test dispositional optimism as mediator or moderator in quality of life in patients with chronic disease

The aim of the present study was to test a hypothetical model to examine if dispositional optimism exerts a moderating or a mediating effect between personality traits and quality of life, in Portuguese patients with chronic diseases. A sample of 540 patients was recruited from central hospitals in various districts of Portugal. All patients completed self-reported questionnaires assessing socio-demographic and clinical variables, personality, dispositional optimism, and quality of life. Structural equation modeling (SEM) was used to analyze the moderating and mediating effects. Results suggest that dispositional optimism exerts a mediator rather than a moderator role between personality traits and quality of life, suggesting that “the expectation that good things will happen” contributes to a better general well-being and better mental functioning.