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.

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.

Checklist cirúrgica: contributo para uma intervenção na área da segurança do doente

Mestrado em Intervenção Sócio-Organizacional na Saúde - Área de especuialização: Políticas de administração e gestão de serviços de saúde.

The impact of exercise training on liver transplanted familial amyloidotic polyneuropathy (FAP) patients

Liver transplantation is nowadays the only effective answer to adjourn the outcome of functional limitations associated with familial amyloidotic polyneuropathy (FAP), a neurodegenerative disease characterized by sensory and motor polyneuropathies. Nevertheless, there is a detrimental impact associated with the after-surgery period on the fragile physical condition of these patients. Exercise training has been proven to be effective on reconditioning patients after transplantation. However, the effects of exercise training in liver transplanted FAP patients have not been scrutinized yet.

Betão auto-compactável com agregados reciclados provenientes da pré-fabricação

A crescente necessidade de tornar a construção mais sustentável e o intuito de contribuir com soluções de reaproveitamento e de escoamento dos desperdícios produzidos na indústria da construção foram determinantes para o financiamento atribuído a um projeto de investigação, com o objetivo de avaliar a viabilidade da utilização de agregados reciclados de betão provenientes da indústria de pré-fabricação para a produção de betões autocompactáveis (BAC). Desta forma, não só se pode contribuir positivamente para a melhoria da qualidade final das peças produzidas pela indústria de pré-fabricação, dispensando a utilização de métodos de vibração falíveis, mas também se contribui para a sustentabilidade do meio ambiente, minimizando o consumo de recursos naturais e a deposição de aterros de resíduos. O projeto é subdividido em diversas fases de otimização e de análise. Numa fase inicial, serão produzidos e selecionados os betões de origem (BO), onde se incluem os materiais produzidos em laboratório e os materiais provenientes de estruturas pré-fabricadas, os quais darão origem aos agregados a analisar. Esses agregados reciclados (AR) sofrerão um processo de trituração orientado (trituração primária e trituração primária e secundária) que pode influenciar as características do próprio betão. Dos resultados obtidos, seleciona-se o método de trituração a adotar para as fases subsequentes. Estas consistirão na produção de betões convencionais e autocompactáveis, que reproduzam aproximadamente a mesma classe de resistência dos betões de origem, nomeadamente 20, 45, 65 e 80 MPa, variando a sua composição (rácio a/c, adições e superplastificantes) e as taxas de agregados reciclados finos (AFR) e grossos (AGR). As percentagens de substituição AFR/AGR a considerar serão: 0/25, 25/0, 25/25, 0/100, 100/0 e 100/100%. Posteriormente, será efetuada a otimização desses betões para classes de resistência mais elevadas, utilizando, para o efeito, agregados reciclados com resistências mecânicas correspondentes a uma classe inferior. Serão incluídos neste estudo os betões de elevado desempenho autocompactáveis, cuja resistência se pretende que seja igual ou superior a 80 MPa. Em todas as fases, serão executados ensaios de avaliação às características mecânicas e de durabilidade dos betões, incluindo a análise microestrutural para a observação da evolução da estrutura porosa da pasta e dos agregados reciclados e da interface pasta/agregados. Pretende-se obter como resultado final um betão com agregados reciclados com agregados reciclados, cujas propriedades sejam compatíveis ou superiores às de um betão auto-compactável com agregados naturais.