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.

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).

Association between grip strength, anthropometric data and functional capacity

The hand is one of the most important instruments of the human body, mainly due to the possibility of grip movements. Grip strength has been described as an important predictor of functional capacity. There are several factors that may influence it, such as gender, age and anthropometric characteristics. Functional capacity refers to the ability to perform daily activities which allow the individual to self-care and to live with autonomy. Composite Physical Function (CPF) scale is an evaluation tool for functional capacity that includes daily activities, self-care, sports activities, upper limb function and gait capacity. In 2011, Portugal had 15% of young population (0-14years) and 19% of elderly population (over 65 years). Considering the double-ageing phenomen, it is important to understand the effect of the grip strength in elderly individuals, considering their characteristics, as the need to maintainin dependency as long as possible.

Does 6-minutes walk test predict functional capacity in elderly people?

Portugal has an accentuated aging tendency, presenting an elderly population (individuals with more than 65 years old) of 19.2%. The average life expectancy is 79.2 years. Thus, it’s important to maintain autonomy and independency as long as possible. Functional ability concept rises from the need to evaluate the capacity to conduct daily activities in an independent way. It can be estimated with the 6-minute walk test (6MWT) and other validated test. This test is simple, reliable, valid and consists in a daily activity (walk). The goals of this study was to verify associations between functional capacity measured with two different instruments (6MWT and Composite Physical Function (CPF) scale) and between those results and characterization variables.

Does the 6-minute walk test predicts functional capacity in a sample of elderly women? A pilot study

Introduction: Functional capacity is the capacity to conduct daily activities in an independent way. It can be estimated with the 6-minutes’ walk test (6MWT) and other validated functional tests. Objectives: Verify associations between functional capacity measured with two different instruments (6MWT and Composite Physical Function (CPF) scale) and levels of physical activity and between those and characterization variables. Methods: This sample consisted of 30 apparently healthy elderly women from Loures municipality. Essentially they should be independent and community-dwelling. Characterization data were collected, containing characterization of physical activity levels and anthropometric data. Functional capacity was assessed with CPF scale and distance walked by the 6MWT. Results were analysed using a SPSS v21.0 through correlation tests. Results: The walked distance in 6MWT was positively associated with height (r = 0.406; p = 0.026), physical activity level (r = 0.594; p = 0.001) and functional capacity (r = 0.682; p = 0.000). For each point more obtained in CPF, the distance walked increases on average by 7.5 meters. Relatively to sedentary participants, being insufficiently active increases, on average, the distance walked in 85.8 meters; and being active increases, on average, the distance walked in 108.8 meters. No other associations were observed in our sample. Conclusion: Based on the collected sample, walked distance in 6MWT has a high correlation with results in CPF scale, so this test can be used to predict functional capacity. More attention should be taken to promote strategies to increase walking in older adults.

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.

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.

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.

Predicted VO2 on 6 minutes walk test for FAP liver transplanted patients

Physical function is limited post-liver transplantation and exercise training can improve exercise capacity following transplantation but previously assessment of functional capacity is necessary. The 6 minute walk test (6MWT) is routinely used for studying patient’s exercise capacity and is less expensive and time consuming than a Cardiopulmonary Exercise Test (CPXT). There have been some studies looking at the relationship between 6MWT and peak VO2 in patients but few presented an adequate equation for predicted peak VO2 from 6MWT to transplanted patients and none for Familial Amyloidotic Polyneuropathy (FAP) liver transplanted patients (FAPTx). The aim of this study was to compare the validity of 6 equations as predictors of VO2 from the 6MWT and compare it with directly measured VO2 in patients FAPTx.

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

Mestrado em Fisioterapia

Efeitos de um programa de fisioterapia na dor e incapacidade funcional em indivíduos institucionalizados com osteoartrose do joelho

Mestrado em Fisioterapia

Influência de um programa de exercício na aptidão física e equilíbrio em idosos institucionalizados: projeto de intervenção

Mestrado em Fisioterapia

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.

Força de preensão – Análise de concordância entre dois dinamómetros: JAMAR vs E‑Link

Introdução – Avaliar a força de preensão mostrou ser de primordial importância pela sua relação com a capacidade funcional dos indivíduos, permitindo determinar níveis de risco para incapacidade futura e, assim, estabelecer estratégias de prevenção. Grande parte dos estudos utiliza o dinamómetro hidráulico JAMAR que fornece o valor da força isométrica obtida durante a execução do movimento de preensão palmar. Contudo, existem outros dinamómetros disponíveis, como é o caso do dinamómetro portátil computorizado E‑Link (Biometrics) que fornece o valor da força máxima (peak force), para além de outras variáveis, como a taxa de fadiga. Não existem, contudo, estudos que nos permitam aceitar e comparar ou não os valores obtidos com os dois equipamentos e porventura utilizá‑los indistintamente. Objetivos – Avaliar a concordância entre as medições da força de preensão (força máxima ou peak force em Kg) obtida a partir de dois equipamentos diferentes (dinamómetros portáteis): um computorizado (E‑Link, Biometrics) e outro hidráulico (JAMAR). Metodologia – Foram avaliados 29 indivíduos (13H; 16M; 22±7 anos; 23,2±3,3 kg/m2) em 2 dias consecutivos, na mesma altura do dia. A posição de teste escolhida foi a recomendada pela Associação Americana de Terapeutas Ocupacionais e foi escolhido o melhor resultado de entre 3 tentativas para a mão dominante. Realizou‑se uma análise correlacional entre os valores obtidos na variável analisada em cada equipamento (coeficiente de Spearman) e uma análise de Bland & Altman para verificar a concordância entre as duas medições. Resultados – O coeficiente de correlação entre as duas medições foi elevado (rS= 0,956; p<0,001) e, pela análise de Bland & Altman, os valores obtidos encontram‑se todos dentro do intervalo da média±2SD. Conclusões – As duas medições mostraram ser concordantes, revelando que os dinamómetros testados podem ser comparáveis ou utilizados indistintamente em diferentes estudos e populações. ABSTRACT: Introduction – Assess grip strength has proved to be of vital importance because of its relationship with functional capacity of individuals, in order to determine levels of risk for future disability and thereby establish prevention strategies. Most studies use the JAMAR Hydraulic dynamometer that provides the value of isometric force obtained during the performance of grip movement. However, there are other dynamometers available, such as portable computerized dynamometer E‑Link (Biometrics), which provides the value of maximum force (peak force) in addition to other variables as the rate of fatigue. There are no studies that allow us to accept or not and compare values obtained with both devices and perhaps use them interchangeably. Purpose – To evaluate the agreement between the measurements of grip strength (peak force or maximum force in kg) obtained from two different devices (portable dynamometers): a computerized (E‑Link, Biometrics) and a hydraulic (JAMAR). Methodology – 29 subjects (13H, 16M, 22 ± 7 years, 23.2 ± 3.3 kg/m2) were assessed on two consecutive days at the same time of day. The test position chosen was recommended by the American Association of Occupational Therapists and was considered the best result from three attempts for the dominant hand. A correlation was studied between values obtained in the variable analyzed in each equipment (Spearman coefficient) and Bland‑Altman analysis to assess the agreement between the two measurements. Results – The correlation coefficient between the two measurements was high (rs = 0,956, p <0,001) and Bland & Altman analysis of the values obtained are all within the range of mean±2SD. Conclusions – The two measurements were shown to be concordant, revealing that the tested dynamometers can be comparable or used interchangeably in different studies and populations.