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.

Aspectos mensurables relevantes para el aprendizaje organizativo en acuerdos de cooperación interempresarial

Esta ponencia presenta un estudio de las principales líneas teóricas existentes sobre la gestión, la teoría de los costes de transacción, la de los recursos y capacidades y la teoría de la KM. Se desarrollan las aportaciones más importantes sobre el aprendizaje en las organizaciones realizadas por la literatura especializada. Seguidamente y a partir de la bibliografía analizada se establecen las relaciones existentes entre la cooperación interempresarial y la creación de conocimiento organizacional en el seno de las empresas, para determinar que se debe medir en el aprendizaje organizativo en acuerdos de cooperación interempresarial.

Elementos para entender el comportamiento de las cajas de ahorros andaluzas entre 1990 y 2000

En este trabajo analizamos el proceso de fusiones llevados a cabo en Andalucía entre sus cajas de ahorros que no han obedecido a modelos asépticos, sino que por el contrario han sido inducidos desde el gobierno autonómico. Al final el modelo resultante ha padecido esa intervención y se han producido cambios en las estrategias. La consecuencia ha sido que aquellas entidades con una mayor presión política están siendo las más débiles. También los mejores resultados lo han conseguido aquellas cajas que han mantenido a sus equipos directivos. Al mismo tiempo la especialización geográfica también ha sido y está siendo determinante en la solvencia, rentabilidad y expansión-consolidación. No ha habido un modelo de fusión y la cooperación, como alternativa, ha fracasado.

La perspectiva entonada de la ejecución musical con el movimiento : desajustes en la tríada : acompañante-bailarín-profesor de danza

En este estudio se presenta un modelo de trabajo dentro de una clase de danza, basado en la interacción entre el Acompañante Musical de Danza (AMD), el Bailarín (en su condición de estudiante) y el Profesor de danza. El trabajo tiene un carácter introspectivo y se basa en el análisis crítico de la perspectiva del autor, como sujeto implicado en el proceso de la enseñanza del movimiento, desde la posición de acompañante musical en las clases de técnica de danza. El estudio tiene por marco el trabajo realizado en la Escuela Superior de Danza de Lisboa (ESD). Durante este ensayo nos referiremos al AMD, como aquél que construye, ejecuta y ajusta su performance musical, buscando una correspondencia acentual y de frase, con el movimiento que observa. El problema que abordaremos es el desajuste o ruido en la comunicación que sucede durante las diferentes fases del proceso de aprendizaje y ejecución de la frase de movimiento, entre el AMD, el Bailarín y el Profesor. Será analizada la cuestión de porque los 3 sujetos de este modelo no siempre compatibilizan de manera más adecuada, especulándose que se deba a una serie de problemas o causas en el nivel de la información sensorial y la perspectiva de las interacciones entre ellos. Son propuestas algunas estrategias que (i) optimizan el trabajo del AMD; (ii) hacen posible una mejor comunicación con el alumno; y (iii) permiten ampliar el campo pedagógico al aprendizaje musical del movimiento.

El texto en el aprendizaje de la lecto-escritura

La investigación didáctica viene señalando la dificultad en la transposición hacia la práctica lectiva de los resultados de la investigación en los dominios que convergen en el acto de enseñar y aprender. Esta dificultad se constata diariamente: si, por un lado, hay maestros que tienen una actitud innovadora (una minoría), por otro, hay profesores con los cuales se perpetúan formas de enseñar a leer y escribir cercanas a las presentadas en los manuales portugueses de finales del siglo XIX, comienzos del siguiente. La necesidad de cambiar prácticas se impone cuando la evaluación de las competencias de lectura y escritura de los alumnos revela niveles demasiado bajos de desempeño (cf. resultados de los exámenes nacionales (2000 a 2008) y en los estudios internacionales en los cuales Portugal estuvo implicado (Reading Literacy – IEA 1992, Pisa 2000,2003, 2006). Con el objetivo de mejorar las enseñanzas/aprendizajes de la lengua, fue creado el programa de formación de profesores (despacho 546-2007). La cuestión central es comprender cómo cambiar las prácticas en clase de manera que se tenga en cuenta los resultados de la investigación, logrando un aprendizaje más efectivo. A través de una práctica de enseñanza de lectura y de escritura basada en textos, se enfoca el cambio de prácticas demasiado centradas en la enseñanza del código: letras, sílabas y palabras. El recorrido didáctico presentado sugiere el texto oral y escrito como base (Teberosky & Sepúlveda 2009), valoriza el descubrimiento (Jolibert 1991, Rieben 1995), las verbalizaciones del alumnado sobre las estrategias de abordaje de los escritos y la sistematización.

Nutritional status influences generic and disease-specific quality of life measures in haemodialysis patients

Background: Poor nutritional status and worse health-related quality of life (QoL) have been reported in haemodialysis (HD) patients. The utilization of generic and disease specific QoL questionnaires in the same population may provide a better understanding of the significance of nutrition in QoL dimensions. Objective: To assess nutritional status by easy to use parameters and to evaluate the potential relationship with QoL measured by generic and disease specific questionnaires. Methods: Nutritional status was assessed by subjective global assessment adapted to renal patients (SGA), body mass index (BMI), nutritional intake and appetite. QoL was assessed by the generic EuroQoL and disease specific Kidney Disease Quality of Life-Short Form (KDQoL-SF) questionnaires. Results: The study comprised 130 patients of both genders, mean age 62.7 ± 14.7 years. The prevalence of undernutrition ranged from 3.1% by BMI ≤ 18.5 kg/m2 to 75.4% for patients below energy and protein intake recommendations. With the exception of BMI classification, undernourished patients had worse scores in nearly all QoL dimensions (EuroQoL and KDQoL-SF), a pattern which was dominantly maintained when adjusted for demographics and disease-related variables. Overweight/obese patients (BMI ≥ 25) also had worse scores in some QoL dimensions, but after adjustment the pattern was maintained only in the symptoms and problems dimension of KDQoL-SF (p = 0.011). Conclusion: Our study reveals that even in mildly undernourished HD patients, nutritional status has a significant impact in several QoL dimensions. The questionnaires used provided different, almost complementary perspectives, yet for daily practice EuroQoL is simpler. Assuring a good nutritional status, may positively influence QoL.

Performance of coastal sea-defense infrastructure at El Jadida (Morocco) against tsunami threat: lessons learned from the Japanese 11 March 2011 tsunami

This paper seeks to investigate the effectiveness of sea-defense structures in preventing/reducing the tsunami overtopping as well as evaluating the resulting tsunami impact at El Jadida, Morocco. Different tsunami wave conditions are generated by considering various earthquake scenarios of magnitudes ranging from M-w = 8.0 to M-w = 8.6. These scenarios represent the main active earthquake faults in the SW Iberia margin and are consistent with two past events that generated tsunamis along the Atlantic coast of Morocco. The behavior of incident tsunami waves when interacting with coastal infrastructures is analyzed on the basis of numerical simulations of near-shore tsunami waves' propagation. Tsunami impact at the affected site is assessed through computing inundation and current velocity using a high-resolution digital terrain model that incorporates bathymetric, topographic and coastal structures data. Results, in terms of near-shore tsunami propagation snapshots, waves' interaction with coastal barriers, and spatial distributions of flow depths and speeds, are presented and discussed in light of what was observed during the 2011 Tohoku-oki tsunami. Predicted results show different levels of impact that different tsunami wave conditions could generate in the region. Existing coastal barriers around the El Jadida harbour succeeded in reflecting relatively small waves generated by some scenarios, but failed in preventing the overtopping caused by waves from others. Considering the scenario highly impacting the El Jadida coast, significant inundations are computed at the sandy beach and unprotected areas. The modeled dramatic tsunami impact in the region shows the need for additional tsunami standards not only for sea-defense structures but also for the coastal dwellings and houses to provide potential in-place evacuation.

Nutritional status and overhydration: can bioimpedance spectroscopy be useful in haemodialysis patients?

Background: Protein-energy wasting (PEW), associated with inflammation and overhydration, is common in haemodialysis (HD) patients and is associated with high morbidity and mortality. Objective: Assess the relationship between nutritional status, markers of inflammation and body composition through bioimpedance spectroscopy (BIS) in HD patients. Methods: This observational, cross-sectional, single centre study, carried out in an HD centre in Forte da Casa (Portugal), involved 75 patients on an HD programme. In all participating patients, the following laboratory tests were conducted: haemoglobin, albumin, C-reactive protein (CRP) and 25-hydroxyvitamin D3 [25(OH)D3]. The body mass index of all patients was calculated and a modified version of subjective global assessment (SGA) was produced for patients on dialysis. Intracellular water (ICW) and extracellular water (ECW) were measured by BIS (Body Composition Monitor®, Fresenius Medical Care®) after the HD session. In statistical analysis, Spearman’s correlation was used for the univariate analysis and linear regression for the multivariate analysis (SPSS 14.0). A P value of <.05 was considered statistically significant. Results: PEW, inversely assessed through the ICW/body weight (BW) ratio, was positively related to age (P<.001), presence of diabetes (P=.004), BMI (P=.01) and CRP (P=.008) and negatively related to albumin (p=.006) and 25(OH)D3 (P=.007). Overhydration, assessed directly through the ECW/BW ratio, was positively related with CRP (P=.009) and SGA (P=.03), and negatively with 25(OH)D3 (P=.006) and BMI (P=.01). In multivariate analysis, PEW was associated with older age (P<.001), the presence of diabetes (P=.003), lower 25(OH)D3 (P=.008), higher CRP (P=.001) and lower albumin levels (P=.004). Over-hydration was associated with higher CRP (P=.001) and lower levels of 25(OH)D3 (P=.003). Conclusions: Taking these results into account, the ICW/BW and ECW/BW ratios, assessed with BIS, have proven to be good markers of the nutritional and inflammatory status of HD patients. BIS may be a useful tool for regularly assessing the nutritional and hydration status in these patients and may allow nutritional advice to be improved and adjusted.

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.

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.

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.

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.