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.

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.

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).

Pt/Carbon materials as Bi-Functional catalysts for N-decane hydroisomerization

The activity and selectivity of bi-functional carbon-supported platinum catalysts for the hydroisomerization of n-alkanes have been studied. The influence of the properties of the carbon support on the performance of the catalysts were investigated by incorporating the metallic function on a series of carbons with varied porosity (microporous: GL-50 from Norit, and mesoporous: CMK-3) and surface chemistry (modified by wet oxidation). The characterization results achieved with H-2 chemisorption and TEM showed differences in surface metal concentrations and metal-support interactions depending on the support composition. The highest metal dispersion was achieved after oxidation of the carbon matrix in concentrated nitric acid, suggesting that the presence of surface functional sites distributed in inner and outer surface favors a homogeneous metal distribution. On the other hand, the higher hydrogenating activity of the catalysts prepared with the mesoporous carbon pointed out that a fast molecular traffic inside the pores plays an important role in the catalysts performance. For n-decane hydroisomerization of long chain n-alkanes, higher activities were obtained for the catalysts with an optimized acidity and metal dispersion along with adequate porosity, pointing out the importance of the support properties in the performance of the catalysts.

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.

Knee muscle strength, balance and functional independence in persons with stroke

Purpose: to investigate the relationship between knee muscle strength, balance and functional independence within the first month after stroke. Relevance: cerebral vascular disease is one of the main causes of morbidity, disability and mortality in developed countries. Problems with movement control are frequent after stroke. Lower limb weakness and impaired balance are common problems that are related with the risk of falls and are likely to interfere with the ability to perform daily life activities. Physiotherapy intervention usually starts early after stroke and addresses impairments related to movement and posture in order to improve motor recovery and restore function.

Muscle strength, balance and functional independence in persons with stroke

Cerebral vascular disease is one of the main causes of morbidity, disability and mortality in developed countries. Problems with movement control are frequent after stroke. Lower limb weakness and impaired balance are common problems that are related with the risk of falls and are likely to interfere with the ability to perform daily life activities. Physiotherapy intervention usually starts early after stroke and addresses impairments related to movement and posture in order to improve motor recovery and restore function. Purpose: to investigate the relationship between knee muscle strength, balance and functional independence within the first month after stroke.

Composition and antioxidant activity of thymus vulgaris volatiles: comparison between supercritical fluid extraction and hydrodistillation

Supercritical fluid extraction (SEE) of the volatile oil from Thymus vulgaris L. aerial flowering parts was performed under different conditions of pressure, temperature, mean particle size and CO2 flow rate and the correspondent yield and composition were compared with those of the essential oil isolated by hydrodistillation (HD). Both the oils were analyzed by GC and GC-MS and 52 components were identified. The main volatile components obtained were p-cymene (10.0-42.6% for SFE and 28.9-34.8% for HD), gamma-terpinene (0.8-6.9% for SFE and 5.1-7.0% for HD), linalool (2.3-5.3% for SFE and 2.8-3.1% for HD), thymol (19.5-40.8% for SFE and 35.4-41.6% for HD), and carvacrol (1.4-3.1% for SFE and 2.6-3.1% for HD). The main difference was found to be the relative percentage of thymoquinone (not found in the essential oil) and carvacryl methyl ether (1.0-1.2% for HD versus t-0.4 for SFE) which can explain the higher antioxidant activity, assessed by Rancimat test, of the SFE volatiles when compared with HD. Thymoquinone is considered a strong antioxidant compound.

Essential oils of Origanum vulgare L. subsp glandulosum (Desf.) letswaart from Tunisia: chemical composition and antioxidant activity

BACKGROUND: Characterisation of the essential oils from O. glandulosum collected in three locations of Tunisia, chemical composition and the evaluation of their antioxidant activities were carried out. RESULTS: The essential oils from Origanum vulgare L. subsp. glandulosum (Desf.) letswaart collected from three localities of north Tunisia - Krib, Bargou and Nefza - were obtained in yields of 2.5, 3.0 and 4.6% (v/w), respectively. The essential oils were analysed by GC and GC/MS and assayed for their total phenolics content, by the Folin-Ciocalteu method, and antioxidant effectiveness, using the 2,2-diphenyl-1-picrylhydrazil (DPPH) radical scavenging assay. The main components of these essential oils, from Nefza, Bargou and Krib, were p-cymene (36%, 40% and 46%), thymol (32%, 39% and 18%), gamma-terpinene (24%, 12% and 16%) and carvacrol (2%, 2% and 15%), respectively). The ability to scavenge the DPPH radicals, expressed by IC50, ranged from 59 to 80 mg L-1. The total phenolic content, expressed in gallic acid equivalent (GAE) g kg(-1) dry weight, varied from 9.37 to 17.70 g kg(-1) dw. CONCLUSIONS: A correlation was identified between the total phenolic content of the essential oils and DPPH radical scavenger capacity. The occurrence of a p-cymene chemotype of O. glandulosum in the northern region of Tunisia is demonstrated.

Influence of LPG composition on straw applied one componente PU winter foam

O principal objectivo desta tese é obter uma relação directa entre a composição dos gases liquefeitos de petróleo (GLP), propano, n-butano e isobutano, usados como aerossóis propulsores numa lata de poliuretano de um componente, com as propriedades das espumas produzidas por spray. As espumas obtidas, terão de ter como requisito principal, um bom desempenho a temperaturas baixas, -10ºC, sendo por isso designadas por espumas de Inverno. Uma espuma é considerada como tendo um bom desempenho se não apresentar a -10/-10ºC (temperatura lata/ spray) glass bubbles, base holes e cell collapse. As espumas deverão ainda ter densidades do spray no molde a +23/+23ºC abaixo dos 30 g/L, um rendimento superior a 30 L, boa estabilidade dimensional e um caudal de espuma a +5/+5ºC superior a 5 g/s. Os ensaios experimentais foram realizados a +23/+23ºC, +5/+5ºC e a -10/-10ºC. A cada temperatura, as espumas desenvolvidas, foram submetidas a testes que permitiram determinar a sua qualidade. Testes esses que incluem os designados por Quick Tests (QT): o spray no papel e no molde das espumas nas referidas temperaturas. As amostras do papel e no molde são especialmente analisadas, quanto, às glass bubbles, cell collapse, base holes, cell structur e, cutting shrinkage, para além de outras propriedades. Os QT também incluem a análise da densidade no molde (ODM) e o estudo do caudal de espumas. Além dos QT foram realizados os testes da estabilidade dimensional das espumas, testes físicos de compressão e adesão, testes de expansão das espumas após spray e do rendimento por lata de espuma. Em todos os ensaios foi utilizado um tubo adaptador colocado na válvula da lata como método de spray e ainda mantida constante a proporção das matérias-primas (excepto os gases, em estudo). As experiências iniciaram-se com o estudo de GLPs presentes no mercado de aerossóis. Estes resultaram que o GLP: propano/ n-butano/ isobutano: (30/ 0/ 70 w/w%), produz as melhores espumas de inverno a -10/-10ºC, reduzindo desta forma as glass bubbles, base holes e o cell collapse produzido pelos restantes GLP usados como aerossóis nas latas de poliuretano. Testes posteriores tiveram como objectivo estudar a influência directa de cada gás, propano, n-butano e isobutano nas espumas. Para tal, foram usadas duas referências do estudo com GLP comercializáveis, 7396 (30 /0 /70 w/w %) e 7442 (0/ 0/ 100 w/w %). Com estes resultados concluí-se que o n-butano produz más propriedades nas espumas a -10/- 10ºC, formando grandes quantidades de glass bubbles, base holes e cell collapse. Contudo, o uso de propano reduz essas glass bubbles, mas em contrapartida, forma cell collapse.Isobutano, porém diminui o cell collapse mas não as glass bubbles. Dos resultados experimentais podemos constatar que o caudal a +5/+5ºC e densidade das espumas a +23/+23ºC, são influenciados pela composição do GLP. O propano e n-butano aumentam o caudal de espuma das latas e a sua densidade, ao contrário com o que acontece com o isobutano. Todavia, pelos resultados obtidos, o isobutano proporciona os melhores rendimentos de espumas por lata. Podemos concluir que os GLPs que contivessem cerca de 30 w/w % de propano (bons caudais a +5/+5ºC e menos glass bubbles a -10/-10ºC), e cerca 70 w/w % de isobutano (bons rendimentos de espumas, bem como menos cell collapse a -10/-10ºC) produziam as melhores espumas. Também foram desenvolvidos testes sobre a influência da quantidade de gás GLP presente numa lata. A análise do volume de GLP usado, foi realizada com base na melhor espuma obtida nos estudos anteriores, 7396, com um GLP (30 / 0/ 70 w/w%), e foram feitas alterações ao seu volume gás GLP presente no pré-polímero. O estudo concluiu, que o aumento do volume pode diminuir a densidade das espumas, e o seu decréscimo, um aumento da densidade. Também indico u que um mau ajuste do volume poderá causar más propriedades nas espumas. A análise económica, concluiu que o custo das espumas com mais GLP nas suas formulações, reduz-se em cerca de 3%, a quando de um aumento do volume de GLP no pré-polímero de cerca de 8 %. Esta diminuição de custos deveu-se ao facto, de um aumento de volume de gás, implicar uma diminuição na quantidade das restantes matérias-primas, com custos superiores, já que o volume útil total da lata terá de ser sempre mantido nos 750 mL. Com o objectivo de melhorar a qualidade da espuma 7396 (30/0/70 w/w %) obtida nos ensaios anteriores adicionou-se à formulação 7396 o HFC-152a (1,1-di fluoroetano). Os resultados demonstram que se formam espumas com más propriedades, especialmente a -10/-10ºC, contudo proporcionou excelentes shaking rate da lata. Através de uma pequena análise de custos não é aconselhável o seu uso pelos resultados obtidos, não proporcionando um balanço custo/benefício favorável. As três melhores espumas obtidas de todos os estudos foram comparadas com uma espuma de inverno presente no mercado. 7396 e 7638 com um volume de 27 % no prépolímero e uma composição de GLP (30/ 0 / 70 w/w%) e (13,7/ 0/ 86,3 w/w%), respectivamente, e 7690, com 37 % de volume no pré-polímero e GLP (30/ 0 / 70 w/w%), apresentaram em geral melhores resultados, comparando com a espuma benchmark . Contudo, os seus shaking rate a -10/-10ºC, de cada espuma, apresentaram valores bastante inferiores à composição benchmarking.

Study of the influence of count’s number in myocardium in the determination of reproducible functional parameters in Gated-SPECT studies simulated with GATE

Myocardial Perfusion Gated Single Photon Emission Tomography (Gated-SPET) imaging is used for the combined evaluation of myocardial perfusion and left ventricular (LV) function. But standard protocols of the Gated-SPECT studies require long acquisition times for each study. It is therefore important to reduce as much as possible the total duration of image acquisition. However, it is known that this reduction leads to decrease on counts statistics per projection and raises doubts about the validity of the functional parameters determined by Gated-SPECT. Considering that, it’s difficult to carry out this analysis in real patients. For ethical, logistical and economical matters, simulated studies could be required for this analysis. Objective: Evaluate the influence of the total number of counts acquired from myocardium, in the calculation of myocardial functional parameters (LVEF – left ventricular ejection fraction, EDV – end-diastolic volume, ESV – end-sistolic volume) using routine software procedures.

Estudo das propriedades dos adjuvantes na compatibilidade/robustez cimento/adjuvante

A qualidade do betão pode ser controlada pelo comportamento da fluidez da pasta de cimento, o qual está relacionado com a dispersão das partículas de cimento. Um dos maiores avanços na tecnologia do betão tem sido o desenvolvimento de aditivos. Um destes tipos de aditivos, os Superplastificantes (SP), fornecem a possibilidade de se obter uma melhor dispersão das partículas de cimento, produzindo pastas com elevada fluidez. Com o desenvolvimento de betões de alta resistência e elevado desempenho, os superplastificantes tornaram-se indispensáveis. Os superplastificantes são adsorvidos nas partículas de cimento e esta adsorção depende da composição do clínquer do cimento e do tipo de SP utilizado. Com a difusão do emprego dos aditivos redutores de água, têm surgido vários problemas de compatibilidade cimento/adjuvante. Esta investigação dedicada aos superplastificantes, fortes redutores de água, visou estudar quais as propriedades que poderiam influenciar a sua compatibilidade/robustez com o cimento. Também se procurou ganhar experiência com as técnicas analíticas de caracterização de adjuvantes. Assim, utilizou-se um tipo de cimento e dois tipos de superplastificantes (poli(étercarboxilatos) e poli(naftalenossulfonatos)) disponíveis no mercado português. Mantendo a mesma razão água/cimento (A/C), pretendeu-se determinar a natureza química, grau de funcionalização, teor e tipo de contra-ião, teor de sulfatos/sulfonatos do adjuvante e o comportamento dos superplastificantes nas pastas cimentícias, de forma a poder determinar indicadores de compatibilidade entre cimentos e superplastificantes. Constatou-se que a natureza química, o grau de funcionalização e a quantidade consumida dos superplastificantes têm influência nas pastas. Os indicadores de compatibilidade por parte dos superplastificantes parecem estar relacionados com o comprimento da cadeia lateral de éter e com o rácio CO2R/CO2 -. A alteração do momento da adição do adjuvante tem influência na compatibilidade cimento/adjuvante, sendo benéfico para os poli(étercarboxilatos) e prejudicial para o poli(naftalenossulfonato).

Avaliação da aptidão física funcional em idosos hipertensos da Cidade da Praia - Cabo Verde

Mestrado em Fisioterapia.

Size-segregated chemical composition of aerosol emissions in an urban road tunnel in Portugal

An atmospheric aerosol study was performed in 2008 inside an urban road tunnel, in Lisbon, Portugal. Using a high volume impactor, the aerosol was collected into four size fractions (PM0.5, PM0.5-1, PM1-2.5 and PM2.5-10) and analysed for particle mass (PM), organic and elemental carbon (OC and EC), polycyclic aromatic hydrocarbons (PAH), soluble inorganic ions and elemental composition. Three main groups of compounds were discriminated in the tunnel aerosol: carbonaceous, soil component and vehicle mechanical wear. Measurements indicate that Cu can be a good tracer for wear emissions of road traffic. Cu levels correlate strongly with Fe, Mn, Sn and Cr, showing a highly linear constant ratio in all size ranges, suggesting a unique origin through sizes. Ratios of Cu with other elements can be used to source apportion the trace elements present in urban atmospheres, mainly on what concerns coarse aerosol particles. (C) 2013 Elsevier Ltd. All rights reserved.