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.

Ressonância magnética de corpo inteiro/whole-body MRI

A introdução de Ressonância Magnética de Corpo Inteiro (RM-CI) de alta resolução é baseada no desenvolvimento rápido e intensivo dos equipamentos de RM. Estes avanços associados ao aparecimento de novos métodos de aquisição de imagem, como as técnicas de Multistation ou imagem paralela impulsionam a RM-CI. Associado ao desenvolvimento tecnológico, a RM-CI apresenta vantagens clínicas essencialmente para patologias oncológicas como é o caso das metástases ósseas, e para patologias do foro cardiovascular. Muitas destas situações ainda se encontram em investigação mas os primeiros resultados têm superado todas as expectativas nomeadamente a RM-CI com a aplicação da técnica de Difusão.

Body dissatisfaction in Brazilian schoolchildren: prevalence and associated factors

OBJECTIVE: To examine the prevalence of body dissatisfaction and associated factors in 8- to 11-year-old schoolchildren. METHODS: A cross-sectional study including children aged 8- to 11-years enrolled in public and private schools in Porto Alegre, Southern Brazil, was carried out from August to December, 2001. A total of 901 subjects were selected through cluster sampling. Participants answered a questionnaire aimed at measuring body dissatisfaction and self-esteem and questions about family and social pressures on weight change. Height and weight were measured. The relationship between body dissatisfaction and the variables studied was measured by logistic regression. RESULTS: The prevalence of body dissatisfaction was 82%. Fifty-five percent of the girls wanted a thinner body size, and 28% desired a larger one; the estimates for the boys were 43% and 38%, respectively. Children with the lowest self-esteem (OR=1.80; 95% CI: 1.13-2.89) and who thought their parents (OR=6.10; 95% CI: 2.95-12.60) and friends (OR=1.81; 95% CI: 1.02-3.20) expected them to be thinner showed a higher chance of presenting body dissatisfaction. CONCLUSIONS: Body dissatisfaction was highly prevalent among the evaluated schoolchildren, especially in those with lower self-esteem and who thought their parents and friends expected them to be thinner.

The adjusted effect of maternal body mass index, energy and macronutrient intakes during pregnancy, and gestational weight gain on body composition of full-term neonates

Objective - To evaluate the effect of prepregnancy body mass index (BMI), energy and macronutrient intakes during pregnancy, and gestational weight gain (GWG) on the body composition of full-term appropriate-for-gestational age neonates. Study Design - This is a cross-sectional study of a systematically recruited convenience sample of mother-infant pairs. Food intake during pregnancy was assessed by food frequency questionnaire and its nutritional value by the Food Processor Plus (ESHA Research Inc, Salem, OR). Neonatal body composition was assessed both by anthropometry and air displacement plethysmography. Explanatory models for neonatal body composition were tested by multiple linear regression analysis. Results - A total of 100 mother-infant pairs were included. Prepregnancy overweight was positively associated with offspring weight, weight/length, BMI, and fat-free mass in the whole sample; in males, it was also positively associated with midarm circumference, ponderal index, and fat mass. Higher energy intake from carbohydrate was positively associated with midarm circumference and weight/length in the whole sample. Higher GWG was positively associated with weight, length, and midarm circumference in females. Conclusion - Positive adjusted associations were found between both prepregnancy BMI and energy intake from carbohydrate and offspring body size in the whole sample. Positive adjusted associations were also found between prepregnancy overweight and adiposity in males, and between GWG and body size in females.

Criatividade em Dança : conceções, métodos e processos de Composição Coreográfica no ensino da Dança

Preprint. Título do artigo editado:"Criatividade em Dança : conceções, métodos e processos de Composição Coreográfica no ensino da Dança". Publicação na Revista Portuguesa de Educação Artística, 2013 (3), pp. 47-59.

Magnesium, insulin resistance and body composition in healthy postmenopausal women

Objective: This study was conducted to determine the association between magnesium (Mg), body composition and insulin resistance in 136 sedentary postmenopausal women, 50 to 77 years of age. Methods: Diabetics, hypertensives and women on hormonal replacement therapy were excluded and the remaining 74 were divided according to BMI≥25 (obese: OG) and BMI<25 kg/m2 (non-obese: NOG). Nutritional data disclosed that intakes were high for protein and saturated fat, low for carbohydrates, polyunsaturated fat and Mg and normal for the other nutrients, according to recommended dietary allowances (RDA). Mg values in red blood cells (RBC-Mg) and plasma (P-Mg), were determined, as were fasting glucose, and insulin levels, Homeostasis Model Assessment (HOMA), body mass index (BMI), body fat percent (BF %), abdominal fat (AF) and free fat mass (FFM). Results: RBC-Mg values were low in both groups when compared with normal values. There were significant differences in body composition parameters, HOMA and insulin levels, with higher basal insulin levels in OG. RBC-Mg was directly correlated with insulin, HOMA and FFM in both groups, according to Pearson correlations. HOMA in OG was also directly correlated with BMI, FFM and AF. In NOG, HOMA was only correlated with FFM. The low RBC-Mg levels observed were probably due to low Mg intake and to deregulation of factors that control Mg homeostasis during menopause. Conclusions: Both Mg deficit and obesity may independently lead to a higher risk for insulin resistance and cardiovascular disease.

Familial amyloid polyneuropathy (FAP): clinical features, pathophysiology and treatment

In the literature, concepts of “polyneuropathy”, “peripheral neuropathy” and “neuropathy” are often mistakenly used as synonyms. Polyneuropathy is a specific term that refers to a relatively homogenous process that affects multiple peripheral nerves. Most of these tend to present as symmetric polyneuropathies that first manifest in the distal portions of the affected nerves. Many of these distal symmetric polyneuropathies are due to toxic-metabolic causes such as alcohol abuse and diabetes mellitus. Other distal symmetric polyneuropathies may result from an overproduction of substances that result in nerve pathology such as is observed in anti-MAG neuropathy and monoclonal gammopathy of undetermined significance. Other “overproduction” disorders are hereditary such as noted in the Portuguese type of familial amyloid polyneuropathy (FAP). FAP is a manifestation of a group of hereditary amyloidoses; an autosomal dominant, multisystemic disorder wherein the mutant amyloid precursor, transthyretin, is produced in excess primarily by the liver. The liver accounts for approximately 98% of all transthyretin production. FAP is confirmed by detecting a transthyretin variant with a methionine for valine substitution at position 30 [TTR (Met30)]. Familial Amyloidotic Polyneuropathy (FAP) – Portuguese type was first described by a Portuguese neurologist, Corino de Andrade in 1939 and published in 1951. Most persons with this disorder are descended from Portuguese sailors who sired offspring in various locations, primarily in Sweden, Japan and Mallorca. Their descendants emigrated worldwide such that this disorder has been reported in other countries as well. More than 2000 symptomatic cases have been reported in Portugal. FAP progresses rapidly with an average time course from symptom onset to multi-organ involvement and death between ten and twenty years. Treatments directed at removing this aberrant protein such as plasmapheresis and immunoadsorption proved to be unsuccessful. Liver transplantation has been the only effective solution as evidenced by almost 2000 liver transplants performed worldwide. A therapy for FAP with a novel agent, “Tafamidis” has shown some promise in ongoing phase III clinical trials. It is well recognized that regular physical activity of moderate intensity has a positive effect on physical fitness as gauged by body composition, aerobic capacity, muscular strength and endurance and flexibility. Physical fitness has been reported to result in the reduction of symptoms and lesser impairment when performing activities of daily living. Exercise has been advocated as part of a comprehensive approach to the treatment of chronic diseases. Therefore, this chapter concludes with a discussion of the role of exercise training on FAP.

Serial experiments lain's body: o corpo enquanto fronteira entre o físico e o virtual

Dissertação apresentada à Escola Superior de Comunicação Social como parte dos requisitos para obtenção de grau de mestre em Audiovisual e Multimédia.

Body mass index for predicting hyperglycemia and serum lipid changes in Brazilian adolescents

OBJECTIVE: To determine the best cut-offs of body mass index for identifying alterations of blood lipids and glucose in adolescents. METHODS: A probabilistic sample including 577 adolescent students aged 12-19 years in 2003 (210 males and 367 females) from state public schools in the city of Niterói, Southeastern Brazil, was studied. The Receiver Operating Characteristic curve was used to identify the best age-adjusted BMI cut-off for predicting high levels of serum total cholesterol (>150mg/dL), LDL-C (>100mg/dL), serum triglycerides (>100mg/dL), plasma glucose (>100mg/dL) and low levels of HDL-C (< 45mg/dL). Four references were used to calculate sensitivity and specificity of BMI cut-offs: one Brazilian, one international and two American. RESULTS: The most prevalent metabolic alterations (>50%) were: high total cholesterol and low HDL-C. BMI predicted high levels of triglycerides in males, high LDL-C in females, and high total cholesterol and the occurrence of three or more metabolic alterations in both males and females (areas under the curve range: 0.59 to 0.67), with low sensitivity (57%-66%) and low specificity (58%-66%). The best BMI cut-offs for this sample (20.3 kg/m² to 21.0 kg/m²) were lower than those proposed in the references studied. CONCLUSIONS: Although BMI values lower than the International cut-offs were better predictor of some metabolic abnormalities in Brazilian adolescents, overall BMI is not a good predictor of these abnormalities in this population.

Does the intake of LC-PUFAS during pregnancy affect the body composition of term neonates? Preliminary results

Background: The effect of the intake of polynsaturated long chain fatty acids (LCPUFAs) during pregnancy on fetal body composition has been assessed by studies using mostly neonatal anthropometry. Their results have been inconsistent, probably because neonatal anthropometry has several validity limitations. Air displacement plethismography (ADP) is a recently validated non-invasive method for assessing body composition in neonates. Objective: To determine the effect of the intake of LCPUFAs during pregnancy on the body composition of term neonates, measured by ADP. Methods: Cross-sectional study of a convenience sample of healthy full-term neonates and their mothers. The diet during pregnancy was assessed using a validated semi-quantitative food frequency questionnaire; Food Processor Plus® was used to convert food intake into nutritional values. Body composition was estimated by anthropometry and measured by ADP using Pea Pod™ Life Measurements Inc (fat mass - FM, fat-free mass and %FM) within the first 72h after birth. Univariate and multivariate analysis (linear regression model) were performed. Results: 54 mother-neonate pairs were included. Multivariate analysis adjusted to the maternal body mass index shows positive association between LCPUFAs intake and neonatal mid-arm circumference (= 0,610, p = 0,019) and negative association between n-6:n-3 ratio intake and neonatal %FM (= -2,744, p=0,066). Conclusion: To the best of our knowledge, this is the first study on this subject using ADP and showing a negative association between LCPUFAs n-6:n-3 ratio intake in pregnancy and neonatal %FM. This preliminary finding requires confirmation increasing the study power with a greater sample and performing interventional studies.

Clay body wrap with microcurrent: effects in central adiposity

Introduction Increased fat mass is becoming more prevalent in women and its accumulation in the abdominal region can lead to numerous health risks such as diabetes mellitus. The clay body wrap using compounds such as green clay, green tea and magnesium sulfate, in addition to microcurrent, may reduce abdominal fat mass and minimize or prevent numerous health problems. Objective This study aims at measuring the influence of the clay body wrap with microcurrent and aerobic exercise on abdominal fat. Methods Nineteen female patients, randomized into intervention (n = 10) and control (n = 9) groups, were evaluated using ultrasound for visceral and subcutaneous abdominal fat, calipers and abdominal region perimeter for subcutaneous fat and bioimpedance for weight, fat mass percentage and muscular mass. During 10 sessions (5 weeks, twice a week) both groups performed aerobic exercise in a cycloergometer and a clay body wrap with microcurrent was applied to the intervention group. Results When comparing both groups after 5 weeks of protocol, there was a significant decrease in the subcutaneous fat around left anterior superior iliac spine in the intervention group (ρ = 0.026 for a confidence interval 95%). When comparing initial and final abdominal fat in the intervention group, measured by ultrasound (subcutaneous and visceral fat) and by skinfold (subcutaneous fat), we detected a significant abdominal fat reduction. Conclusion This study demonstrated that the clay body wrap used with microcurrent and aerobic exercise can have a positive effect on central fat reduction.