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.

Knee muscle strength, balance and functional independence in persons with stroke

Purpose: to investigate the relationship between knee muscle strength, balance and functional independence within the first month after stroke. Relevance: cerebral vascular disease is one of the main causes of morbidity, disability and mortality in developed countries. Problems with movement control are frequent after stroke. Lower limb weakness and impaired balance are common problems that are related with the risk of falls and are likely to interfere with the ability to perform daily life activities. Physiotherapy intervention usually starts early after stroke and addresses impairments related to movement and posture in order to improve motor recovery and restore function.

Muscle strength, balance and functional independence in persons with stroke

Cerebral vascular disease is one of the main causes of morbidity, disability and mortality in developed countries. Problems with movement control are frequent after stroke. Lower limb weakness and impaired balance are common problems that are related with the risk of falls and are likely to interfere with the ability to perform daily life activities. Physiotherapy intervention usually starts early after stroke and addresses impairments related to movement and posture in order to improve motor recovery and restore function. Purpose: to investigate the relationship between knee muscle strength, balance and functional independence within the first month after stroke.

Handgrip strength

The basic function of the human hand is the manipulation and grasping of various objects in all daily activities, including work activities. This is greatly influenced by strength and manual dexterity. However age, gender and other contexts such as work or leisure activities could influence strength. Handgrip strength, a measure of maximum voluntary force of the hand, has proved to be reliable and valid as an objective parameter to evaluate the functional integrity of the hand as part of the musculoskeletal system. It correlates highly with strength in other muscular groups and is therefore considered as a good indicator of overall muscular strength and functional stress and could be used as a predictor of physical disability. Handgrip strength assessment is simple and reliable and used commonly by several investigators and health professionals, in different contexts (medical, nutritional, rehabilitation, professional settings, engineering, etc.) and with different purposes (research, diagnostic, assessment, etc.). In clinical and rehabilitation settings is of vital importance in the determination of effectiveness of several interventions and for monitoring evolution of diseases. Various ways (methods, techniques and equipments) of collecting information on grip strength have been reported. This chapter will review basic concepts on handgrip function, methodologies of assessment, contexts of application and correlates, such as physical activity, health or nutritional status. Several populations and reference values as also the relationships between handgrip and clinical status, aging, risk of disability and diseases, will be discussed.

Effects of an exercise training program in physical condition after liver transplantation in familial amyloidotic polyneuropathy: a case report

Introduction: 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.

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.

Idosos ativos, idosos saudáveis

Mestrado em Fisioterapia

Ventilação não invasiva durante o exercício em pacientes com DPOC: revisão sistemática com meta-análise

Mestrado em Fisioterapia

Non invasive ventilation during exercise in COPD patients: a systematic review with meta-analysis

COPD is a major cause of morbidity and mortality worldwide, representing a major public health problem due to the high health and economic resource consumption. Pulmonary rehabilitation is a standard care recommendation for these patients, in order to control the symptoms and optimize the functional capacity, reducing health care costs associated with exacerbations and activity limitations and participation. However, in patients with severe COPD exercise performance can be difficult, due to extreme dyspnea, decreased muscle strength and fatigue. In addition, hypoxemia and dyspnea during efforts and daily activities may occur, limiting their quality of life. Thus, NIV have been used as adjunct to exercise, in order to improve exercise capacity in these patients. However, there is no consensus for this technique recommendation. Our objective was to verify whether the use of NIV during exercise is effective than exercise without NIV in dyspnea, walked distance, blood gases and health status in COPD patients, through a systematic review and meta-analysis.

Treino de músculos inspiratórios em indivíduos saudáveis: estudo randomizado controlado

Introdução – O treino dos músculos inspiratórios (TMI) surge como uma intervenção importante na população com doença pulmonar obstrutiva crónica (DPOC), mas com interesse crescente na população saudável. No entanto, não existem estudos suficientes que comprovem se o treino dos músculos inspiratórios se traduz também numa melhoria da capacidade aeróbia objetivada no consumo de oxigénio (VO2). Assim, a relação entre o TMI e os seus resultados no indivíduo saudável carece de estudo que comprove os efeitos reais do treino. Considerou-se, pelo anteriormente exposto, pertinente a realização de um estudo de investigação na população saudável que permitisse avaliar em que medida um programa de TMI induz alterações na força muscular inspiratória e na capacidade aeróbia. Métodos e análise – A amostra foi constituída por indivíduos saudáveis (n=19) com idades compreendidas entre os 18 e 21 anos que realizam exercício físico regularmente (≥3 vezes por semana ou ≥4h por semana). A capacidade aeróbia foi estimada através do Teste de Ebbeling e a força dos músculos inspiratórios foi medida pela pressão inspiratória máxima (PIM) obtida num dinamómetro específico (MicroRPM®), em dois momentos distintos (pré e pós-treino). A referida amostra foi dividida aleatoriamente em dois grupos (n=9 no grupo experimental e n=10 no grupo de controlo). O grupo experimental (GE) foi submetido a um TMI de alta intensidade (≥50% Pi,máx), enquanto o grupo de controlo (GC) não foi sujeito a qualquer intervenção. O TMI foi realizado através do PowerBreathe Classic® Level 1 e Level 2, que fornece uma pressão consistente e específica para a força muscular inspiratória, independentemente do fluxo inspiratório do indivíduo. Conclusões – Após o treino verificou-se um aumento de 37% na PIM do GE, enquanto o GC apresentou uma melhoria de 7%. Na comparação intragrupos, ambos os grupos aumentaram significativamente tanto a PIM como o VO2 (p<0,05). Já na comparação intergrupos, a diferença foi significativa para a PIM (p=0,000), mas não para o VO2. Serão necessários mais estudos no sentido de concluir e avaliar em que condições o TMI produz alterações na capacidade aeróbia.

Strength, muscle quality and functional capacity in liver transplanted familial amyloidotic polineuropathy patients

Liver 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.

Strength, muscle quality and functional capacity in liver transplanted familial amyloidotic polineuropathy patients

Liver 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).