Echocardiographic study of paediatric patients with mucopolysaccharidosis

Principle Mucopolysaccharidosis is an inborn error of metabolism causing glucosaminoglycans tissue storage. Cardiovascular involvement is variable but contributes significantly towards the morbidity and mortality of the patients. Objective To characterise the echocardiographic abnormalities in children and adolescents with different types of mucopolysaccharidosis. Method Echocardiograms and medical records of 28 patients aged 2–14 years, seen from 2003 to 2005, were revised. At that time, the enzymatic replacement therapy was still not available in our institution.Results Echocardiographic alterations were detected in 26 patients (93 per cent), whereas 16 (57 per cent) had abnormal auscultation, and only 6 (21 per cent) presented with cardiovascular complaint. Mitral valve thickening with dysfunction (regurgitation, stenosis, or double lesion) was diagnosed in 60.8 per cent, left ventricular hypertrophy in 43 per cent and aortic valve thickening with regurgitation in 35.8 per cent of the patients. There was no systolic dysfunction and mild left diastolic dysfunction was shown in 21.5 per cent of the patients. Pulmonary hypertension was present in 36 per cent of the patients, causing the only two deaths recorded. There was a strong association between the accumulation of dermatan sulphate and the presence of mitral valve dysfunction (p = 0.0003), aortic valve dysfunction (p = 0.006), and pulmonary hypertension (p = 0.006). Among individuals with two or more examinations, 82 per cent had a worsening evolution. Conclusions Echocardiographic alterations in children with Mucopolysaccharidosis are frequent and have a progressive character Left valve lesions, ventricular hypertrophy, and pulmonary hypertension were the most common findings and there was an association between the accumulation of dermatan sulphate and cardiovascular involvement. Unlike in adults, pulmonary hypertension was the main cause of death, not left ventricle systolic dysfunction

Bioeticistas brasileiros e os princípios da universalidade e da integralidade no SUS

OBJETIVO: Compreender os sentidos que bioeticistas brasileiros atribuem aos princípios da universalidade e da integralidade no sistema público de saúde brasileiro. PROCEDIMENTOS METODOLÓGICOS: Estudo exploratório qualitativo, realizado com 20 professores universitários de bioética atuantes no campo das ciências da saúde, com funções de diretores e ex-diretores da Sociedade Brasileira de Bioética e de diretorias regionais, no período de julho de 2007 a julho de 2008. Foram realizadas entrevistas semi-estruturadas, com perguntas abertas, sendo realizada análise de conteúdo. ANÁLISE DE RESULTADOS: Quanto ao princípio da universalidade de acesso dos cidadãos brasileiros a um sistema público, as manifestações dos entrevistados se posicionaram majoritariamente em prol de sua manutenção. Todavia, quanto ao princípio da integralidade, as divergências foram manifestas, ensejando a maioria em restringi-lo. CONCLUSÕES: Os bioeticistas relatam pluralismo de valores morais e dificuldades em decidir moralmente sobre o que seria um sistema de saúde justo

A advocacia em saúde como uma estratégia para a promoção da saúde

O conceito de promoção da saúde vem sendo desenvolvido há aproximadamente trinta anos e já está sedimentado. Atualmente, torna-se muito importante elaborar estratégias para sua implementação. O presente artigo é uma reflexão sobre a prática da advocacia em saúde enquanto estratégia para a implementação dos princípios e diretrizes da promoção da saúde. O ponto de partida é o questionamento sobre como conciliar os aspectos conceituais e metodológicos da promoção da saúde, considerados dentro de nossa realidade. A conclusão principal aponta para uma proposta de desenvolvimento de uma " Advocacia em Promoção da Saúde."

A dança «en-cena» o outro: prerrogativas para uma educação estética através do processo criativo

The dance installs in the body another scene of senses that raises the creative performance and not that can be speculated about it. There, ""who dance"" is the Other one, making the body acting of another manner, beyond the systemic contour of Ego. Without the presence of this forged performance, the body would not pass of one ruled machine in function of something and not in favor of himself, as original movement of himself. This article intends to make a reflection about the corporal perception in the dance, trying to point out the creative performance. So, we opened a way to understand the process creative as field of corporal sensitization where the educative intervention is accomplished in the body.

Vibration-induced extra torque during electrically-evoked contractions of the human calf muscles

Background: High-frequency trains of electrical stimulation applied over the lower limb muscles can generate forces higher than would be expected from a peripheral mechanism (i.e. by direct activation of motor axons). This phenomenon is presumably originated within the central nervous system by synaptic input from Ia afferents to motoneurons and is consistent with the development of plateau potentials. The first objective of this work was to investigate if vibration (sinusoidal or random) applied to the Achilles tendon is also able to generate large magnitude extra torques in the triceps surae muscle group. The second objective was to verify if the extra torques that were found were accompanied by increases in motoneuron excitability. Methods: Subjects (n = 6) were seated on a chair and the right foot was strapped to a pedal attached to a torque meter. The isometric ankle torque was measured in response to different patterns of coupled electrical (20-Hz, rectangular 1-ms pulses) and mechanical stimuli (either 100-Hz sinusoid or gaussian white noise) applied to the triceps surae muscle group. In an additional investigation, M(max) and F-waves were elicited at different times before or after the vibratory stimulation. Results: The vibratory bursts could generate substantial self-sustained extra torques, either with or without the background 20-Hz electrical stimulation applied simultaneously with the vibration. The extra torque generation was accompanied by increased motoneuron excitability, since an increase in the peak-to-peak amplitude of soleus F waves was observed. The delivery of electrical stimulation following the vibration was essential to keep the maintained extra torques and increased F-waves. Conclusions: These results show that vibratory stimuli applied with a background electrical stimulation generate considerable force levels (up to about 50% MVC) due to the spinal recruitment of motoneurons. The association of vibration and electrical stimulation could be beneficial for many therapeutic interventions and vibration-based exercise programs. The command for the vibration-induced extra torques presumably activates spinal motoneurons following the size principle, which is a desirable feature for stimulation paradigms.

Measurement of ethylene production during banana ripening

Introduction. This protocol aims at measuring fruit ethylene production during ripening. It can be used to compare ethylene production between different banana varieties or to compare ethylene production between fruit produced in different pedo-climatic conditions. The principle, key advantages, starting plant material, time required and expected results are presented. Materials and methods. This part describes the required laboratory materials and the three steps necessary for calculating the amount of ethylene produced during banana postharvest ripening. Possible troubleshooting is considered.

Extraction and purification of total RNA from banana tissues (small scale)

Introduction. This protocol aims at preparing total RNA for gene expression analysis by Northern blots, RT-PCR and real-time quantitative PCR; cDNA isolation by RTPCR; and cDNA library construction. The principle, key advantages, starting plant material, time required for obtaining total RNA and expected results are presented. Materials and methods. This part describes the required materials and the 27 steps necessary for preparing RNA from peel and pulp fruit tissue: preparation of plant tissue powder, preparation of the complete RNA extraction buffer and isolation of RNA from ground banana fruit tissue. Results. Extraction of total RNA by the method described makes it possible to achieve electrophoresis under denatured conditions and in vitro reverse transcription. An example for Northern blot analysis is illustrated.

Determination of banana fruit susceptibility to post-harvest diseases: wound anthracnose, quiescent anthracnose and crown rot

Introduction. This protocol aims at ( a) evaluating the resistance to post-harvest diseases within different genotypes of bananas, and ( b) comparing different origins of bananas ( geographic origin, physiological stage, etc.) for their susceptibility to post-harvest diseases. The principle, key advantages, starting plant material, time required and expected results are presented. Materials and methods. Materials required and details of the twelve steps of the protocol ( fruit sampling and inoculum preparation, wound anthracnose resistance study, quiescent anthracnose resistance study and crown-rot resistance study) are described. Results. Typical symptoms of the different diseases are obtained after artificial inoculation.

Biochemical characterization of pulp of banana fruit: measurement of soluble sugars, organic acids, free ACC and in vitro ACC oxidase

Introduction. We present some protocols aiming at partially characterizing banana fruit quality through measurement of some key biochemical parameters. The principle, key advantages, starting plant material, time required and expected results are presented. Materials and methods. This part describes the required laboratory materials and the steps necessary for achieving four protocols making it possible to measure sugar, organic acids and free ACC contents, and in vitro ACC oxidase activity. Results. Standard results obtained by using the protocols described are presented in the figures.

Measurement of banana green life

Introduction. This protocol aims at measuring the storage life potential of banana fruit, and at determining the physiological age of fruit. The principle, key advantages, starting plant material, time required and expected results are presented. Materials and methods. This part describes the required laboratory materials and the five steps necessary for calculating the banana green life duration, which corresponds to the number of days between the fruit harvest and climacteric crisis. Results. The measurement of O-2 and CO2 concentrations allows one to detect the climacteric peak which marks the end of the banana green life.

Method for early quantification of quiescent infections of Colletotrichum musae on bananas

Introduction. This protocol aims at detecting and quantifying quiescent infections of Colletotrichum musae on bananas. The principle, key advantages, starting plant material, time required and expected results are presented. Materials and methods. The materials required and details of the three steps of the protocol (fruit sampling, fruit ripening and anthracnose lesion quantification) are described. Possible troubleshooting is discussed. Results. The protocol results in the quantification of anthracnose lesions on the fruits, which makes it possible to predict postharvest losses due to anthracnose (peel rot), and also to propose a better management of postharvest fungicide applications.

Methodology to forecast the harvest date of banana bunches

Introduction. This method is used to forecast the harvest date of banana bunches from as early as the plant shooting stage. It facilitates the harvest of bunches with the same physiological age. The principle, key advantages, time required and expected results are presented. Materials and methods. Details of the four steps of the method ( installation of the temperature sensor, tagging bunches at the flowering stage, temperature sum calculation and estimation of bunch harvest date) are described. Possible problems are discussed. Results. The application of the method allows drawing a curve of the temperature sum accumulated by the bunches which have to be harvested at exactly 900 degree-days physiological age.