Browsing by Author "Barroso, Eduardo"
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- A 5-year follow-up of the benefits of an exercise training program in liver recipients transplanted due to familial amyloidotic polyneuropathyPublication . Tomás, Maria Teresa; Melo, Xavier; Mateus, Élia; Gonçalves, Mafalda; Barroso, Eduardo; Santa-Clara, HelenaBackground: Supervised (SE) and home-based exercise (HBE) training regimes are effective on reconditioning patients with familial amyloidotic polyneuropathy (FAP) after liver transplantation, but research of the long-term retention of the benefits attained in patients with FAP has not yet been conducted. Purpose: In this 5-year follow-up study, we aimed to determine whether the exercise training gains in body composition, physical activity, and function promoted by a 24-week SE or HBE training regimes are retained in patients with FAP who resume normal activity. Methodology: Sixteen liver-transplanted patients with FAP were reassessed for body composition (dual X-ray absorptiometry), physical activity (questionnaire), and function (handgrip strength and 6-minute walk test). Results: Total body fat increased with both exercise regimes during follow-up ( P < .05; η2 = 0.432-0.625) as well as femoral neck bone density ( P = .048; η2 = 0.119). However, gains in upper limbs muscle quality during follow-up ( P < .001; η2 = 0.597) were only found in the SE group ( P = .042; η2 = 0.245). Both exercise regimes showed retaining aptitudes in walking capacity ( P < .05; η2 = 0.329-0.460) and muscle mass ( P = .05; η2 = 0.245). Still, none could retain the physical activity levels. Conclusion: Long-term resumption of normal activity following a 24-week SE or HBE regime in patients with FAP resulted in a loss of exercise-induced increases in physical activity but counterweighted postoperative losses in bone mineral density and substantially retained the benefits in walking capacity, muscle mass, and quality, in particular, in the SE group.
- Alterações da força de preensão em portadores de polineuropatia amiloidótica familiarPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Carolino, Elisabete; Freire, António; Barroso, EduardoIntrodução: A polineuropatia amiloidótica familiar (PAF) é uma doença autossómica dominante neurodegenerativa relacionada com a deposição sistémica de fibras de amiloide essencialmente a nível do sistema nervoso periférico. Clinicamente, caracteriza-se por uma neuropatia sensitivo-motora iniciando-se quase sempre nos membros inferiores e comprometendo subsequentemente as mãos. Até agora, o único tratamento conhecido com efeitos positivos no atrasar da progressão da doença é o transplante hepático com medicação com efeitos negativos para o metabolismo muscular e consequentemente para a capacidade de produção de força. Do nosso conhecimento, não existem caracterizações quantitativas dos níveis de força nestes indivíduos nem comparações com a população saudável. Este conhecimento seria extremamente importante para verificar a evolução clínica e funcional desta doença e para a eventual prescrição adequada de um programa de reabilitação. Objectivo: O objectivo deste estudo foi descrever e comparar os níveis de força de preensão (peak force) entre doentes PAF com ou sem transplante de fígado (PAFTx e PAFNTx, respectivamente) com um grupo de indivíduos saudáveis (GC). Material e métodos: A amostra total foi constituída por 206 indivíduos, divididos em três grupos: 59 indivíduos PAFNTx (23 homens, 36 mulheres; idade 35 ± 8 anos); 85 indivíduos PAFTx (52 homens, 33 mulheres; idade 34 ± 8 anos) e 62 GC (30 homens, 32 mulheres; idade 33 ± 9 anos). A força de preensão foi avaliada com um dinamómetro de preensão portátil E-Link (Biometrics Ltd, UK). Tanto as posições de medição como as ordens fornecidas foram estandardizadas. O valor de força máxima considerado foi classificado de acordo com as normas do American College of Sports Medicine (ACSM) para a força de preensão. Resultados: Os três grupos são diferentes (p < 0,05) no peso, no IMC e na força de preensão em ambas as mãos, bem como na resistência da mão esquerda. Foram encontradas correlações negativas entre a força e a idade, para os grupos PAFNTx e PAFTx, mas não para o grupo GC. Conclusões: De acordo com os nossos resultados, os indivíduos portadores de PAF apresentaram valores mais baixos para a força de preensão em ambas as mãos do que os indivíduos aparentemente saudáveis e consequentemente uma pior classificação nas normas do ACSM. A maioria dos doentes apresenta valores de força de preensão abaixo da média ou mesmo precária. Estes resultados poderão mostrar as implicações negativas na funcionalidade destes indivíduos e indicam também a necessidade de um programa de reabilitação com especificidade ao nível da motricidade da mão.
- Body composition, muscle strength, functional capacity, and physical disability risk in liver transplanted familial amyloidotic polyneuropathy patientsPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Baynard, Tracy; Carnero, Elvis; Bruno, Paula Marta; Barroso, Eduardo; Sardinha, Luís; Fernhall, BoAbstract: 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.
- Comparison of body fat content and distribution of familial amyloidotic polyneuropathy patients versus healthy subjectsPublication . Pimenta, Nuno; Santa-Clara, Helena; Tomás, Maria Teresa; Monteiro, Estela; Cabri, Jan; Freire, António; Barroso, Eduardo; Sardinha, Luís; Fernhall, BoThe 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 subjectsPublication . Pimenta, Nuno; Santa-Clara, Helena; Tomás, Maria Teresa; Monteiro, Estela; Cabri, Jan; Freire, António; Barroso, Eduardo; Sardinha, Luís; Fernhall, BoThe 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.
- Composição corporal, capacidade funcional e tempo em lista activa em indivíduos submetidos a transplante hepático por polineuropatia amiloidótica familiarPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Freire, António; Pimenta, Nuno; Sardinha, Luís; Barroso, EduardoPAF–Doença neurodegenerativa relacionada com a produção hepática de Transtirretina met30 e respectiva deposição sistémica principalmente no sistema nervoso periférico, que se traduz clinicamente por uma neuropatia sensitivo-motora de início periférico e com graves limitações funcionais em alguns casos. Actualmente, o único tratamento conhecido para a PAF é o transplante hepático com medicação agressiva para o metabolismo muscular e ósseo e para a produção de força. Não existe actualmente qualquer caracterização quantitativa da composição corporal ou capacidade funcional para esta população. Objectivos do estudo: comparar a composição corporal e capacidade funcional entre doentes PAF transplantados (PAFT) e um grupo de indivíduos aparentemente saudáveis (GC); analisar possíveis relações entre composição corporal e capacidade funcional e tempo de espera para a cirurgia.
- Do fatigue, physical activity, handgrip strength and quality of life change between pre and post liver transplant?Publication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Barroso, Eduardo; Sardinha, Luís B.Analisar: níveis de fadiga, força de preensão, HRQoL, níveis de actividade física. Será que se alteram em doentes PAF após o transplante de fígado? Dado que os níveis de actividade física se encontram abaixo dos valores mínimos recomendados deveria ser encontrada uma estratégia de aumento do tempo dispendido na actividade física leve a moderada idealmente no PRÉ TRANSPLANTE.
- Efeitos de um programa de exercício físico na aptidão física pós-transplante hepático por polineuropatia amiloidótica familiar: estudo de casoPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Barroso, Eduardo; Sardinha, LuísPolineuropatia Amiloidotica Familiar (PAF) – Amiloidose sistémica heredo-degenerativa e autossómica dominante, cuja manifestação predominante é a polineuropatia mista sensitiva motora e autonómica. Mutação no cromossoma 18: substituição da Valina por Metionina TTR mutada e respectiva deposição sistémica. Objectivos do estudo: verificar o impacto de um programa de exercício combinado na composição corporal, na força muscular, na capacidade funcional, nos níveis de fadiga e nos níveis de actividade física.
- Effects of a randomized trial of exercise on body composition of liver transplanted patientsPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Barroso, Eduardo; Sardinha, Luís; Fernhall, BoLiver 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.
- Effects of an exercise training program in physical condition after liver transplantation in familial amyloidotic polyneuropathy: a case reportPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Barroso, Eduardo; Sardinha, LuísIntroduction: 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.