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.

Efeitos de um programa baseado em tarefas orientadas na competência de marcha em indivíduos com acidente vascular cerebral

Mestrado em Fisioterapia.

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

Mestrado em Fisioterapia

Comparação de dois protocolos de reabilitação após reconstrução cirúrgica do ligamento cruzado anterior

Mestrado em Fisioterapia

Análise descritiva das práticas e contextos em fisioterapia respiratória nos hospitais centrais públicos de Luanda

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.

The importance of the program for the promotion of physical activity in perceived fatigue in people with multiple sclerosis

The aims of the study is to examine for intervention program of physical activity in the perception of fatigue, in patients with multiple sclerosis.

Implication of an intervention program for the promotion of physical activity in the quality of life of people with multiple sclerosis

The aims of this study is to examine the interest for quality of life of an implementation of program physical activity, with patients of multiple sclerosis.

Psychological well-being and perception of disease severity in people with multiple sclerosis, who underwent a program of self-regulation to promote physical activity

Rehabilitation is very important for in the results of treatment in individuals with multiple sclerosis. Rehabilitation processes occur through gradual changes. These changes integrate intrinsic and extrinsic mechanisms of the individual, promoting adaptations to the needs and activities of daily living according to individual goals. Recommendations for exercise in multiple sclerosis: these recommendations apply only to patients with EDSS less than 7; moderate intensity aerobic exercise for a total of 20 to 30 minutes, twice or three times for week; the resistance training with low or moderate intensity is well tolerated by patients with MS; associated with these exercises were recommended flexibility exercises of moderate intensity, as well as strengthening exercises. The aim of this study is to examine the implications of the program of self-regulation in the perception of illness and mental health (psychological well-being domain) in multiple sclerosis patients.

Importance of program physical activity in quality of life in multiple sclerosis patients, six months after the intervention

Background: Multiple Sclerosis (MS) is a chronic disease of the central nervous system that affects more often young adults in the prime of his career and personal development, with no cure and unknown causes. The most common signs and symptoms are fatigue, muscle weakness, changes in sensation, ataxia, changes in balance, gait difficulties, memory difficulties, cognitive impairment and difficulties in problem solving MS is a relatively common neurological disorder in which various impairments and disabilities impact strongly on function and daily life activities. Purpose: The aim of this study is to examine the implications of an Intervention Program of Physical Activity (IPPA) in quality of life in MS patients, six months after the intervention.

Evidence based for exercise program in physical therapy in patients with multiple sclerosis

Background: Multiple sclerosis is a disease of the central nervous system that affects more frequently young women. It is a progressive and unpredictable disease, resulting in some cases of disabilities and limitations to physical, psychological and social level. Purpose: To review the literature for evidence based of the effectiveness of physiotherapy intervention in multiple sclerosis.

A supervised versus home-based program effects on liver transplanted familial amyloidotic polyneuropathy patients: walking, fatigue and quality of life

Purpose: To evaluate the effects of a six months exercise training program on walking capacity, fatigue and health related quality of life (HRQL). Relevance: Familial amyloidotic polyneuropathy disease (FAP) is an autossomic neurodegenerative disease, related with systemic deposition of amyloidal fibre mainly on peripheral nervous system and mainly produced in the liver. FAP often results in severe functional limitations. Liver transplantation is used as the only therapy so far, that stop the progression of some aspects of this disease. Transplantation requires aggressive medication which impairs muscle metabolism and associated to surgery process and previous possible functional impairments, could lead to serious deconditioning. Reports of fatigue are common feature in transplanted patients. The effect of supervised or home-based exercise training programs in FAP patients after a liver transplant (FAPTX) is currently unknown.

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.

Intermittent claudication and physical exercise: effectiveness of a home-based program

Peripheral arterial disease (PAD) as a high incidence in general population and 12% to 20% of population with more than 60 years has already clinical symptoms, such as intermittent claudication (IC), pain, loss of strength and functional incapacity. There are already some studies who refer the possible positive effects of physical exercise in functional consequences of PAD.

A supervised versus home-based program effects on liver transplanted familial amyloidotic polyneuropathy patients: walking, fatigue and quality of life

Familial 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.