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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.
- 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.
- 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).
- 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).
- A supervised versus home-based program effects on liver transplanted familial amyloidotic polyneuropathy patients: walking, fatigue and quality of lifePublication . Tomás, Maria Teresa; Santa-Clara, Helena; Monteiro, Estela; Gil, João; Bruno, Paula Marta; Barroso, Eduardo; Sardinha, LuísFamilial amyloidotic polyneuropathy is a systemic deposition of amyloidal fibre mainly on peripheral nervous system (but also in other systems like heart, gastrointestinal tract, kidneys, etc) and mainly produced in the liver. Purpose of this study: to evaluate the effects of a six months exercise training program(supervised or home-based) on walking capacity, fatigue and health related quality of life (HRQL) on Familial Amyloidotic Polyneuropathy patients submitted to a liver transplant.
- 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 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.
- 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.