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.

Relação existente entre a percepção de estigma e optimismo, em indivíduos com esclerose múltipla

O objectivo deste estudo é verificar a existência de uma relação entre, a perceção de estigma e otimismo na esclerose múltipla. Foram avaliados 101 indivíduos com esclerose múltipla. Os resultados indicam que existe uma relação evidente entre o optimismo disposicional e a percepção de estigma em pessoas com esclerose múltipla. O optimismo é um factor facilitador relativamente à percepção de estigma social, nomeadamente à capacidade destes indivíduos lidarem com a deficiência e a incapacidade funcional.

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

Mestrado em Fisioterapia

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.

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.

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.

Relationship of balance to function independence in stroke survivors

Cerebral vascular disease is the primary cause of permanent disability in Portugal. Impaired stability is considered an important feature after stroke as it is related with higher risk of falls and functional dependence. Physiotherapy intervention usually starts early after stroke in order to direct motor recovery and help patients to improve their ability to perform activities of daily living (ADL). Purpose: to investigate the relationship of balance to functionality in acute stroke patients. Methods: 16 subjects (8 women and 8 men), mean age 63,62 ± 2,16y, with unilateral ischemic stroke in the middle cerebral artery territory, who were admitted to physiotherapy department of Fernando Fonseca Hospital in Portugal, within the first month after stroke were recruited to participate in this study. All subjects have no cognitive impairment according to Mini Mental State, no history of lower extremity orthopedic problems and no other disease that could interfere with treatments. All patients gave their inform consent to participate in this study. Subjects were assessed with the Modified Barthel Index (MBI) and the Berg Balance Scale (BBS).

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).

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.

Effects of lower limb strengthening on balance and function in persons who suffered a stroke

Introduction - Cerebrovascular diseases, and among them, cerebral vascular accidents, are one of the main causes of morbidity and disability at European Union countries. Clinical framework resulting from these diseases include important limitations in functional ability of the these patients Postural control dysfunctions are one of the most common and devastating consequences of a stroke interfering with function and autonomy and affecting different aspects of people’s life and contributing to decrease quality of life. Neurological physiotherapy plays a central role in the recovery of movement and posture, however it is necessary to study the efficacy of techniques that physiotherapists use to treat these problems. Objectives - The aim of this study was to investigate the effects of a physiotherapy intervention program, based on oriented tasks and strengthening of the affected lower limb, on balance and functionality of individuals who have suffered a stroke. In addition our study aimed to investigate the effect of strength training of the affected lower limb on muscle tone.

O currículo funcional no desenvolvimento da autonomia e da comunicação oral e escrita

Os alunos com necessidades educativas especiais, mais concretamente os alunos que apresentam deficiência mental, com limitações acentuadas no comportamento adaptativo, necessitam um ensino mais individualizado, com uma vertente mais funcional. Os currículos funcionais, planeados de acordo com os contextos de vida atuais e futuros em que cada aluno se insere e se irá inserir, permitem desenvolver competências com significado e úteis para a formação pessoal, social e laboral, possibilitando uma vida adulta com mais qualidade e com mais autonomia. Este estudo incide no desenvolvimento de atividades funcionais para a promoção da autonomia e da comunicação oral e escrita em duas crianças, com 11 anos de idade, com défice cognitivo. Delinearam-se três objetivos para o estudo: i) caraterizar o nível de desenvolvimento e aprendizagem de duas alunas com défice cognitivo, nomeadamente no que respeita à autonomia e ao desenvolvimento da linguagem; ii) desenvolver conteúdos, estratégias e atividades funcionais que facilitem o progressivo aumento da participação das alunas em contextos reais de atividades de vida diária; iii) contribuir para o desenvolvimento de competências de autonomia e comunicação oral e escrita de duas alunas com défice cognitivo. Organizou-se um projeto de intervenção, desenvolvido na lógica da investigação ação. De modo a se elaborar o plano de intervenção, avaliaram-se as competências das alunas ao nível da comunicação oral e escrita, autonomia e funcionalidade, recorrendo à análise documental, a entrevistas às encarregadas de educação e à docente de Educação Especial, à observação direta e à elaboração de diários de aula. O plano de intervenção foi planeado no quadro dos currículos funcionais e centrou-se em atividades de vida diária, selecionadas a partir da análise das necessidades atuais e futuras das alunas. Após a implementação da intervenção, concluiu-se que a aplicação de atividades de um currículo funcional melhorou as competências de autonomia e de comunicação oral e escrita das alunas.- Abstract: Students with special educational needs, more specifically students who have mental disabilities, with significant behavior limits to adapt, need a more individual and functional education. The functional curriculum, planned according to the current and future life contexts in which every student is and will be, allow the development of meaningful skills, useful to their personal education, social life and labour, enabling them with an adulthood life with more quality and more autonomy. This study focuses on the development of functional activities for the promotion of autonomy and of oral and written communication in two children with 11 years old with mental disability. Three goals were outlined for the study: i) to characterize the level of development and learning of two students with cognitive dysfunction, regarding particularly the autonomy and the language development, ii) to develop contents, strategies and functional activities that increase the participation of the students in real daily life activities, iii) to contribute to the development of autonomy and oral and written communication of two students with mental disability. We organized an intervention project, developed in the logic of action research. In order to develop the intervention plan, we evaluated the level of oral and written communication, autonomy and functionality, through document analysis, interviews to the guardians and to the Special Educational teacher, as well as through direct observation and preparation of diaries of lessons. The intervention plan was outlined within the framework of functional curriculum and focused on activities of daily living, selected trough the analysis of current and future needs of the students. After the implementation of the intervention plan, we observed that the application of the activities of a functional curriculum improved the skills of autonomy and oral and written communication of the students

Grip strength: are dinamometers equivalent? JAMAR vs E-Link

Levels of risk for future disability can be assessed with grip strength. This assessment is of fundamental importance for establishing prevention strategies. It also allows verifying relationships with functional capacity of individuals. Most studies on grip strength use the JAMAR Hydraulic dynamometer that provides the value of isometric force obtained during the performance of grip movement and is considered the “gold standard” for measurement of grip strength. However, there are different dynamometers available commercially, 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 for hand strength, among others. Of our knowledge, there are no studies that allow us to accept or not and compare values obtained with both devices and perhaps use them interchangeably. The aim of this study was to evaluate the absolute 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 one hydraulic (JAMAR).

Study of the influence of count’s number in myocardium in the determination of reproducible functional parameters in Gated-SPECT studies simulated with GATE

Myocardial Perfusion Gated Single Photon Emission Tomography (Gated-SPET) imaging is used for the combined evaluation of myocardial perfusion and left ventricular (LV) function. But standard protocols of the Gated-SPECT studies require long acquisition times for each study. It is therefore important to reduce as much as possible the total duration of image acquisition. However, it is known that this reduction leads to decrease on counts statistics per projection and raises doubts about the validity of the functional parameters determined by Gated-SPECT. Considering that, it’s difficult to carry out this analysis in real patients. For ethical, logistical and economical matters, simulated studies could be required for this analysis. Objective: Evaluate the influence of the total number of counts acquired from myocardium, in the calculation of myocardial functional parameters (LVEF – left ventricular ejection fraction, EDV – end-diastolic volume, ESV – end-sistolic volume) using routine software procedures.

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

Mestrado em Fisioterapia.