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.

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.

Alterações do equilíbrio em indivíduos com ou sem síndrome de Down em repouso e durante a execução de uma tarefa

O equilíbrio é fundamental para o desempenho de Actividades de Vida Diária e de Vida Diária Instrumentais que permitem aos indivíduos com Síndrome de Down manter a sua autonomia. O objectivo principal deste estudo foi verificar as características do estado de equilíbrio em indivíduos com e sem Síndrome de Down, em repouso e durante a execução de uma tarefa, de modo a compreender as implicações da alteração do estado de equilíbrio no desempenho de actividades. Neste estudo, foram seleccionadas duas amostras, de forma probabilística por conveniência, com indivíduos de ambos os sexos. A amostra do grupo experimental foi constituída por oito indivíduos com diagnóstico de Síndrome de Down, com idades compreendidas entre os 17 e os 39 anos de idade. A amostra do grupo de controlo foi constituída por doze indivíduos sem patologia, com idades compreendidas entre os 21 e os 37 anos. O equilíbrio foi avaliado com recurso a uma Plataforma de Forças (Bertec Corporation FP4060-10) para medir a deslocação do centro de pressão (CP) dos indivíduos. A avaliação do equilíbrio decorreu em dois momentos, na posição de pé: em repouso e no desempenho da tarefa segurar um saco de compras. Os resultados diferem entre os dois grupos em repouso e durante a execução da tarefa (segurar no saco). Os valores das variáveis área e distância de deslocação do CP e oscilações corporais, antero-posterior e médio-lateralmente apresentam resultados significativos, o que sugere diferenças do estado de equilíbrio entre as amostras.

Iniciação da marcha em indivíduos com AVE. Sequência de ativação muscular do tibial anterior e solear

Introdução: A iniciação da marcha, enquanto tarefa motora complexa que consiste na transição de uma postura mantida pelo apoio simultâneo dos dois membros inferiores para um equilíbrio dinâmico, permitindo a progressão anterior do corpo, constitui um exemplo que implica uma correta sequência de ativação muscular. Objetivos: Verificar a modificação da fase de iniciação da marcha face à aplicação de um programa de recuperação funcional, analisando a sequência de ativação dos músculos tibial anterior e solear. Registar as repercussões funcionais na participação nas diferentes atividades da vida diária, em contexto padronizado e social. Metodologia: Nos dois participantes em estudo foi realizada uma avaliação antes e após um programa de intervenção, segundo a abordagem do Conceito de Bobath, através da Classificação Internacional de Funcionalidade, Incapacidade e Saúde, da Fugl Meyer Assessment of Sensorymotor Recovery After Stroke, electromiografia e plataforma de forças. Resultados: Após a aplicação do programa de recuperação funcional, verificaram-se alterações na sequência de ativação do tibial anterior e solear. O músculo tibial anterior passou a ser o primeiro a ser recrutado nesta sequência. Conclusão: Foi possível verificar modificações durante a fase de iniciação da marcha, face a um programa de recuperação funcional, em que a sequência de ativação do tibial anterior e solear, tendencialmente, se assemelharam ao comportamento em indivíduos saudáveis, repercutindo-se numa melhoria funcional na participação nas atividades da vida diária.

Estudo dos indicadores de qualidade e de risco na interaça o mãe-filho(a)

O presente estudo tem como principal objetivo identificar na interação mãe- bebés de 3 meses, indicadores de risco e de qualidade e, consequentemente, criar um guião que ajude os profissionais a identificar esses mesmos indicadores. Para o efeito observámos 30 díades mãe-filho(a) em jogo livre distribuídas em dois grupos: 1) 12 díades sem condições assinaláveis de risco e 2) 18 díades em risco socioeconómico. O comportamento interativo dos bebés e das mães foi avaliado através do Child-Adult Relashionship Experimental, designado pela autora de CARE-Index (Crittenden, 2003). Os comportamentos maternos e infantis foram cotados de acordo com os 7 aspetos do comportamento diádico: Expressão Facial, Expressão Verbal, Posição e Contato Corporal, Afetividade, Reciprocidade, Diretividade e Escolha da Atividade. Os resultados do nosso estudo revelam que as díades mãe-filho(a) sem condições assinaláveis de risco apresentam interações mais positivas, recíprocas e ajustadas à idade das crianças do que as díades sujeitas a condições de risco e que o comportamento materno sensível e responsivo surge associado ao comportamento cooperativo infantil e negativamente correlacionado com a dificuldade infantil, enquanto que uma postura controladora, diretiva ou punitiva da mãe surge positivamente correlacionada com a submissão e evitamento infantil. Por fim, encontramos associados a comportamentos maternos sensíveis uma variabilidade de comportamentos infantis desde satisfação, coerção, procura de proximidade ou evitamento. Na discussão dos resultados apresentamos um script dos indicadores de qualidade e de risco do comportamento materno. - Abstract This study main goal was to study mother-infant quality of interaction in dyads with 3 months babies. Moreover, our goal was to explore maternal and infant key behaviors that shape the quality of the interaction in order to organize a script that helps professionals to identify these critical interactive behaviors. To this end we observed 30 mother-child dyads (a) in free play interaction divided into two groups: 1) 12 dyads without any known risk condition and 2) 18 dyads at socioeconomic risk. The interactive behavior of infants and mothers was assessed using the Child-Adult Relationship Experimental, designated by the author of CARE-Index (Crittenden, 2003). The maternal and infant behaviors were rated according to seven aspects of dyadic behavior: Facial Expression, Verbal Expression, Position and Contact Body, Affection, Reciprocity, Directivity and Choice of Activity. Findings indicate that mother-child dyads without know risk conditions are more likely to present positive interactions, reciprocal and age-adjusted than dyads at risk. The sensitive and responsive maternal behavior emerges associated with children's cooperative behavior and negatively correlated with infant difficultness, while a controlling or punitive maternal behavior is positively correlated with infant compliance and avoidance. Finally, sensitive maternal behaviors were associated with a variability of child behaviors, from satisfaction, coercion, avoidance or proximity search. In the results discussion we present a script of the quality and risks indicators of maternal behavior.

Ressonância magnética de corpo inteiro/whole-body MRI

A introdução de Ressonância Magnética de Corpo Inteiro (RM-CI) de alta resolução é baseada no desenvolvimento rápido e intensivo dos equipamentos de RM. Estes avanços associados ao aparecimento de novos métodos de aquisição de imagem, como as técnicas de Multistation ou imagem paralela impulsionam a RM-CI. Associado ao desenvolvimento tecnológico, a RM-CI apresenta vantagens clínicas essencialmente para patologias oncológicas como é o caso das metástases ósseas, e para patologias do foro cardiovascular. Muitas destas situações ainda se encontram em investigação mas os primeiros resultados têm superado todas as expectativas nomeadamente a RM-CI com a aplicação da técnica de Difusão.

Body dissatisfaction in Brazilian schoolchildren: prevalence and associated factors

OBJECTIVE: To examine the prevalence of body dissatisfaction and associated factors in 8- to 11-year-old schoolchildren. METHODS: A cross-sectional study including children aged 8- to 11-years enrolled in public and private schools in Porto Alegre, Southern Brazil, was carried out from August to December, 2001. A total of 901 subjects were selected through cluster sampling. Participants answered a questionnaire aimed at measuring body dissatisfaction and self-esteem and questions about family and social pressures on weight change. Height and weight were measured. The relationship between body dissatisfaction and the variables studied was measured by logistic regression. RESULTS: The prevalence of body dissatisfaction was 82%. Fifty-five percent of the girls wanted a thinner body size, and 28% desired a larger one; the estimates for the boys were 43% and 38%, respectively. Children with the lowest self-esteem (OR=1.80; 95% CI: 1.13-2.89) and who thought their parents (OR=6.10; 95% CI: 2.95-12.60) and friends (OR=1.81; 95% CI: 1.02-3.20) expected them to be thinner showed a higher chance of presenting body dissatisfaction. CONCLUSIONS: Body dissatisfaction was highly prevalent among the evaluated schoolchildren, especially in those with lower self-esteem and who thought their parents and friends expected them to be thinner.

Show me your body and I tell you how hold you are: a non-invasive methodto define 6 life history-classes in Risso's dolphins (Grampus griseus), using an identified trial population in the Atlantic

European Cetacean Society Conference Workshop, Galway, Ireland, 25th March 2012.

Alterações da curvatura da coluna vertebral: influência da fisioterapia, a nível neuromuscular

Mestrado em Fisioterapia

NEE: inclusão e relação escola-família

Dissertação apresentada à Escola Superior de Educação de Lisboa para obtenção do grau de Mestre em Educação Especial - Especialidade Problemas de Cognição e Multideficiência

The adjusted effect of maternal body mass index, energy and macronutrient intakes during pregnancy, and gestational weight gain on body composition of full-term neonates

Objective - To evaluate the effect of prepregnancy body mass index (BMI), energy and macronutrient intakes during pregnancy, and gestational weight gain (GWG) on the body composition of full-term appropriate-for-gestational age neonates. Study Design - This is a cross-sectional study of a systematically recruited convenience sample of mother-infant pairs. Food intake during pregnancy was assessed by food frequency questionnaire and its nutritional value by the Food Processor Plus (ESHA Research Inc, Salem, OR). Neonatal body composition was assessed both by anthropometry and air displacement plethysmography. Explanatory models for neonatal body composition were tested by multiple linear regression analysis. Results - A total of 100 mother-infant pairs were included. Prepregnancy overweight was positively associated with offspring weight, weight/length, BMI, and fat-free mass in the whole sample; in males, it was also positively associated with midarm circumference, ponderal index, and fat mass. Higher energy intake from carbohydrate was positively associated with midarm circumference and weight/length in the whole sample. Higher GWG was positively associated with weight, length, and midarm circumference in females. Conclusion - Positive adjusted associations were found between both prepregnancy BMI and energy intake from carbohydrate and offspring body size in the whole sample. Positive adjusted associations were also found between prepregnancy overweight and adiposity in males, and between GWG and body size in females.

Magnesium, insulin resistance and body composition in healthy postmenopausal women

Objective: This study was conducted to determine the association between magnesium (Mg), body composition and insulin resistance in 136 sedentary postmenopausal women, 50 to 77 years of age. Methods: Diabetics, hypertensives and women on hormonal replacement therapy were excluded and the remaining 74 were divided according to BMI≥25 (obese: OG) and BMI<25 kg/m2 (non-obese: NOG). Nutritional data disclosed that intakes were high for protein and saturated fat, low for carbohydrates, polyunsaturated fat and Mg and normal for the other nutrients, according to recommended dietary allowances (RDA). Mg values in red blood cells (RBC-Mg) and plasma (P-Mg), were determined, as were fasting glucose, and insulin levels, Homeostasis Model Assessment (HOMA), body mass index (BMI), body fat percent (BF %), abdominal fat (AF) and free fat mass (FFM). Results: RBC-Mg values were low in both groups when compared with normal values. There were significant differences in body composition parameters, HOMA and insulin levels, with higher basal insulin levels in OG. RBC-Mg was directly correlated with insulin, HOMA and FFM in both groups, according to Pearson correlations. HOMA in OG was also directly correlated with BMI, FFM and AF. In NOG, HOMA was only correlated with FFM. The low RBC-Mg levels observed were probably due to low Mg intake and to deregulation of factors that control Mg homeostasis during menopause. Conclusions: Both Mg deficit and obesity may independently lead to a higher risk for insulin resistance and cardiovascular disease.

Familial amyloid polyneuropathy (FAP): clinical features, pathophysiology and treatment

In the literature, concepts of “polyneuropathy”, “peripheral neuropathy” and “neuropathy” are often mistakenly used as synonyms. Polyneuropathy is a specific term that refers to a relatively homogenous process that affects multiple peripheral nerves. Most of these tend to present as symmetric polyneuropathies that first manifest in the distal portions of the affected nerves. Many of these distal symmetric polyneuropathies are due to toxic-metabolic causes such as alcohol abuse and diabetes mellitus. Other distal symmetric polyneuropathies may result from an overproduction of substances that result in nerve pathology such as is observed in anti-MAG neuropathy and monoclonal gammopathy of undetermined significance. Other “overproduction” disorders are hereditary such as noted in the Portuguese type of familial amyloid polyneuropathy (FAP). FAP is a manifestation of a group of hereditary amyloidoses; an autosomal dominant, multisystemic disorder wherein the mutant amyloid precursor, transthyretin, is produced in excess primarily by the liver. The liver accounts for approximately 98% of all transthyretin production. FAP is confirmed by detecting a transthyretin variant with a methionine for valine substitution at position 30 [TTR (Met30)]. Familial Amyloidotic Polyneuropathy (FAP) – Portuguese type was first described by a Portuguese neurologist, Corino de Andrade in 1939 and published in 1951. Most persons with this disorder are descended from Portuguese sailors who sired offspring in various locations, primarily in Sweden, Japan and Mallorca. Their descendants emigrated worldwide such that this disorder has been reported in other countries as well. More than 2000 symptomatic cases have been reported in Portugal. FAP progresses rapidly with an average time course from symptom onset to multi-organ involvement and death between ten and twenty years. Treatments directed at removing this aberrant protein such as plasmapheresis and immunoadsorption proved to be unsuccessful. Liver transplantation has been the only effective solution as evidenced by almost 2000 liver transplants performed worldwide. A therapy for FAP with a novel agent, “Tafamidis” has shown some promise in ongoing phase III clinical trials. It is well recognized that regular physical activity of moderate intensity has a positive effect on physical fitness as gauged by body composition, aerobic capacity, muscular strength and endurance and flexibility. Physical fitness has been reported to result in the reduction of symptoms and lesser impairment when performing activities of daily living. Exercise has been advocated as part of a comprehensive approach to the treatment of chronic diseases. Therefore, this chapter concludes with a discussion of the role of exercise training on FAP.

Serial experiments lain's body: o corpo enquanto fronteira entre o físico e o virtual

Dissertação apresentada à Escola Superior de Comunicação Social como parte dos requisitos para obtenção de grau de mestre em Audiovisual e Multimédia.