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- Strength, muscle quality and functional capacity in liver transplanted familial amyloidotic polineuropathy patientsPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Pimenta, Nuno; Cabri, Jan; Freire, António; Barroso, EduardoLiver 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.
- A importância da actividade física em pacientes submetidos a transplante hepático: estudo longitudinalPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Bruno, Paula Marta; Barroso, Eduardo; Sardinha, LuísPolineuropatia Amiloidótica Familiar (PAF) é uma doença neurodegenerativa que conduz a polineuropatia motora e sensitiva e a limitações funcionais. O transplante de fígado é presentemente a única intervenção eficaz no retardar da evolução da PAF. Objectivos do estudo: verificar os efeitos de um período de 6 meses de destreino na composição corporal e capacidade funcional num grupo de pacientes PAF submetidos a transplante hepático e que previamente haviam participado num programa de exercício combinado com a duração de 6 meses.
- Paramiloidose, transplante hepático e força muscular: qual a relação?Publication . Tomás, Maria Teresa; Freire, Amtónio; Morbey, A.; Veloso, J.; Monteiro, Estela; Barroso, EduardoParamiloidose - Doença hereditária autossómica dominante, de evolução crónica e progressiva, envolve predominantemente os membros inferiores, conduzindo à diminuição da força muscular e da sensibilidade e ao compromisso da marcha. Objectivos do estudo: 1. Avaliar os níveis de força máxima do quadricípete em sujeitos submetidos a transplante hepático (TH) por PAF, antes do TH, à data da alta, 3 meses após o TH, 6 meses após o TH, 1 ano após o TH; 2. Comprovar quantitativamente a eventual recuperação da força muscular; 3. Inferir os benefícios de actividade física/estilos de vida mais activos no pós-TH de doentes com PAF.
- 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.
- Sarcopenia in liver transplant due to familial amyloidotic polyneuropathy (FAP): the relevance of muscle massPublication . Tomás, Maria Teresa; Melo, Xavier; Mateus, Élia; Barroso, Eduardo; Santa-Clara, HelenaIntroduction: Loss of muscle mass and function is a common occurrence in liver transplant Familial Amyloidotic Polyneuropathy (FAP) patients. Sarcopenia is associated with morbidity and mortality before and after liver transplantation. However, the ability of skeletal muscle mass to recover after transplant remains questionable and thus the importance of clinical exercise prescription. Methods: Participants were 39 FAP patients aged 23-59 years, who had been submitted to a liver transplant (Tx) (22 men) between 2 and 4 months post-tx. Sarcopenia was defined according to the International Working Group on Sarcopenia and Society of Sarcopenia, Cachexia, and Wasting Disorders. Whole-body dual x-ray absorptiometry was used to measure body fat and lean-soft tissue. Skeletal Muscle Index (SMI) was calculated adjusting the value of appendicular skeletal mass to the squared height. Functional aerobic capacity was assessed using the 6 min walk test (6MWT), and handgrip strength were measured on the dominant hand using a hand dynamometer. Results: The prevalence of sarcopenia using SMI was 45.5% in men and 41.2% in women. A fat mass higher than 16% for men and 26% for women was found in 54.5% of men and 52.9% of women. Also, 27.3% of men and 17.6% of the women could be classified as having sarcopenia with low mobility (distance < 400 m). Discussion and Conclusions: Sarcopenia is common in FAP patients and a liver transplant seems to increase the prevalence, also because an aggressive medication with impact on muscle metabolism should be made longtime. More data are needed on the long-term effects of sarcopenia after transplant, especially in light of the high rate of metabolic syndrome. A clinical exercise prescription seems to be necessary for these patients but more studies are needed (e.g. longitudinal studies).
- 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.
- 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.
- Peak force in familial amyloidotic polyneuropathyPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Cabri, Jan; Freire, António; Barroso, EduardoFamilial Amyloidotic Polyneuropathy FAP)- A neurodegenerative disease related with systemic deposition of amyloid fibers mainly at the level of the peripheral nervous system. Clinically, the disease is characterized by an autonomous sensitive-motor neuropathy, beginning nearly always in foot, and subsequently involving the hands. Purpose: Compare the levels of hand grip strength (peak force) in FAP patients with (FAPT) or without (FAPNT) liver transplant and in a healthy group (HG).
- 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.
- Strength, muscle quality and functional capacity in liver transplanted familial amyloidotic polineuropathy patientsPublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Pimenta, N.; Cabri, Jan; Freire, António; Barroso, EduardoLiver 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).