Abstract Background Sepsis is an uncontrolled inflammatory response against a systemic infection that results in elevated mortality, mainly induced by bacterial products known as endotoxins, producing endotoxemia. Disseminated intravascular coagulation (DIC) is frequently observed in septic patients and is associated with organ failure and death. Sepsis activates endothelial cells (ECs), promoting a prothrombotic phenotype contributing to DIC. Ion channel mediated calcium permeability participates in coagulation. The transient reception potential melastatin 7 (TRPM7) non-selective divalent cation channel that also contains an α-kinase domain, which is permeable to divalent cations including Ca2+, regulates endotoxin-stimulated calcium permeability in ECs and is associated with increased mortality in septic patients. However, whether endothelial TRPM7 mediates endotoxemia-induced coagulation is not known. Therefore, our aim was to examine if TRPM7 mediates coagulation during endotoxemia. Results The results showed that TRPM7 regulated endotoxin-induced platelet and neutrophil adhesion to ECs, dependent on the TRPM7 ion channel activity and by the α-kinase function. Endotoxic animals showed that TRPM7 mediated neutrophil rolling on blood vessels and intravascular coagulation. TRPM7 mediated the increased expression of the adhesion proteins, von Willebrand factor (vWF), intercellular adhesion molecule 1 (ICAM-1), and P-selectin, which were also mediated by the TRPM7 α-kinase function. Notably, endotoxin-induced expression of vWF, ICAM-1 and P-selectin were required for endotoxin-induced platelet and neutrophil adhesion to ECs. Endotoxemic rats showed increased endothelial TRPM7 expression associated with a procoagulant phenotype, liver and kidney dysfunction, increased death events and an increased relative risk of death. Interestingly, circulating ECs (CECs) from septic shock patients (SSPs) showed increased TRPM7 expression associated with increased DIC scores and decreased survival times. Additionally, SSPs with a high expression of TRPM7 in CECs showed increased mortality and relative risk of death. Notably, CECs from SSPs showed significant results from the AUROC analyses for predicting mortality in SSPs that were better than the Acute Physiology and Chronic Health Evaluation II (APACHE II) and the Sequential Organ Failure Assessment (SOFA) scores. Conclusions Our study demonstrates that sepsis-induced DIC is mediated by TRPM7 in ECs. TRPM7 ion channel activity and α-kinase function are required by DIC-mediated sepsis-induced organ dysfunction and its expression are associated with increased mortality during sepsis. TRPM7 appears as a new prognostic biomarker to predict mortality associated to DIC in SSPs, and as a novel target for drug development against DIC during infectious inflammatory diseases.

Abstract Background Skeletal muscle is sensitive to bile acids (BA) because it expresses the TGR5 receptor for BA. Cholic (CA) and deoxycholic (DCA) acids induce a sarcopenia-like phenotype through TGR5-dependent mechanisms. Besides, a mouse model of cholestasis-induced sarcopenia was characterised by increased levels of serum BA and muscle weakness, alterations that are dependent on TGR5 expression. Mitochondrial alterations, such as decreased mitochondrial potential and oxygen consumption rate (OCR), increased mitochondrial reactive oxygen species (mtROS) and unbalanced biogenesis and mitophagy, have not been studied in BA-induced sarcopenia. Methods We evaluated the effects of DCA and CA on mitochondrial alterations in C2C12 myotubes and a mouse model of cholestasis-induced sarcopenia. We measured mitochondrial mass by TOM20 levels and mitochondrial DNA; ultrastructural alterations by transmission electronic microscopy; mitochondrial biogenesis by PGC-1α plasmid reporter activity and protein levels by western blot analysis; mitophagy by the co-localisation of the MitoTracker and LysoTracker fluorescent probes; mitochondrial potential by detecting the TMRE probe signal; protein levels of OXPHOS complexes and LC3B by western blot analysis; OCR by Seahorse measures; and mtROS by MitoSOX probe signals. Results DCA and CA caused a reduction in mitochondrial mass and decreased mitochondrial biogenesis. Interestingly, DCA and CA increased LC3II/LC3I ratio and decreased autophagic flux concordant with raised mitophagosome-like structures. In addition, DCA and CA decreased mitochondrial potential and reduced protein levels in OXPHOS complexes I and II. The results also demonstrated that DCA and CA decreased basal, ATP-linked, FCCP-induced maximal respiration and spare OCR. DCA and CA also reduced the number of cristae. In addition, DCA and CA increased the mtROS. In mice with cholestasis-induced sarcopenia, TOM20, OXPHOS complexes I, II and III, and OCR were diminished. Interestingly, the OCR and OXPHOS complexes were correlated with muscle strength and bile acid levels. Conclusion Our results showed that DCA and CA decreased mitochondrial mass, possibly by reducing mitochondrial biogenesis, which affects mitochondrial function, thereby altering potential OCR and mtROS generation. Some mitochondrial alterations were also observed in a mouse model of cholestasis-induced sarcopenia characterised by increased levels of BA, such as DCA and CA.

Abstract Background Skeletal muscle generates force and movements and maintains posture. Under pathological conditions, muscle fibers suffer an imbalance in protein synthesis/degradation. This event causes muscle mass loss and decreased strength and muscle function, a syndrome known as sarcopenia. Recently, our laboratory described secondary sarcopenia in a chronic cholestatic liver disease (CCLD) mouse model. Interestingly, the administration of ursodeoxycholic acid (UDCA), a hydrophilic bile acid, is an effective therapy for cholestatic hepatic alterations. However, the effect of UDCA on skeletal muscle mass and functionality has never been evaluated, nor the possible involved mechanisms. Methods We assessed the ability of UDCA to generate sarcopenia in C57BL6 mice and develop a sarcopenic-like phenotype in C2C12 myotubes and isolated muscle fibers. In mice, we measured muscle strength by a grip strength test, muscle mass by bioimpedance and mass for specific muscles, and physical function by a treadmill test. We also detected the fiber's diameter and content of sarcomeric proteins. In C2C12 myotubes and/or isolated muscle fibers, we determined the diameter and troponin I level to validate the cellular effect. Moreover, to evaluate possible mechanisms, we detected puromycin incorporation, p70S6K, and 4EBP1 to evaluate protein synthesis and ULK1, LC3 I, and II protein levels to determine autophagic flux. The mitophagosome-like structures were detected by transmission electron microscopy. Results UDCA induced sarcopenia in healthy mice, evidenced by decreased strength, muscle mass, and physical function, with a decline in the fiber's diameter and the troponin I protein levels. In the C2C12 myotubes, we observed that UDCA caused a reduction in the diameter and content of MHC, troponin I, puromycin incorporation, and phosphorylated forms of p70S6K and 4EBP1. Further, we detected increased levels of phosphorylated ULK1, the LC3II/LC3I ratio, and the number of mitophagosome-like structures. These data suggest that UDCA induces a sarcopenic-like phenotype with decreased protein synthesis and autophagic flux. Conclusions Our results indicate that UDCA induces sarcopenia in mice and sarcopenic-like features in C2C12 myotubes and/or isolated muscle fibers concomitantly with decreased protein synthesis and alterations in autophagic flux.

Abstract Background Satellite cells are tissue-specific stem cells primarily responsible for the regenerative capacity of skeletal muscle. Satellite cell function and maintenance are regulated by extrinsic and intrinsic mechanisms, including the ubiquitin–proteasome system, which is key for maintaining protein homeostasis. In this context, it has been shown that ubiquitin-ligase NEDD4-1 targets the transcription factor PAX7 for proteasome-dependent degradation, promoting muscle differentiation in vitro. Nonetheless, whether NEDD4-1 is required for satellite cell function in regenerating muscle remains to be determined. Results Using conditional gene ablation, we show that NEDD4-1 loss, specifically in the satellite cell population, impairs muscle regeneration resulting in a significant reduction of whole-muscle size. At the cellular level, NEDD4-1-null muscle progenitors exhibit a significant decrease in the ability to proliferate and differentiate, contributing to the formation of myofibers with reduced diameter. Conclusions These results indicate that NEDD4-1 expression is critical for proper muscle regeneration in vivo and suggest that it may control satellite cell function at multiple levels.

Abstract The article aims to analyze the perception of intergenerational social mobility of Mapuche university students, based on the construc tion of a typology of future projections in relation to the variables of social origin and parents' education. Sample was composed by 200 students between 18 - 28 years (M = 21.32; SD = 2.75), of which 63.5% were women and 36.5% were men. Two- step cluster analyses was the method of the study, which is recommended for categorical data. The analyses suggested to hold back a five cluster model with a good (.7) cluster quality, based on Kaufman and Rousseeuw works (1990). The sample was analysed according to the obtained clusters, for describe sociodemographic characteristics. Results indicated that the origin social class, parents' education and rurality are determinant factors in the social mobility perception. It is concluded that the access to higher education, it is not a determinant factor in the social mobility perception of mapuche university students, and in the case of students who perceive it, is an intra-class mobility.

Resumen Este artículo tiene como objetivo analizar la percepción de movilidad social intergeneracional de estudiantes universitarios mapuche, a partir de la construcción de una tipología de proyecciones futuras con relación a las variables origen social y educacional de sus padres. La muestra estuvo conformada por 200 estudiantes, entre 18 y 28 años (M= 21.32 años; DT = 2.75), de los cuales el 63.5% fueron mujeres y el 36.5% hombres. El método utilizado fue el análisis de clúster bietápico, el cual es recomendado para variables categóricas. El análisis sugirió retener un modelo de cinco conglomerados de calidad buena (.7), según el criterio establecido por Kaufman y Rousseeuw (1990). La muestra fue analizada a partir de los conglomerados obtenidos, para describir sus características según variables sociodemográficas de interés. Los resultados señalan que las variables clase social de origen, educación de los padres y ruralidad, son factores determinantes en la percepción de movilidad social. A partir de la segmentación por conglomerados, se concluyó que el acceso a la educación superior no es un factor determinante en la proyección de movilidad social futura para los estudiantes universitarios mapuche y, cuando así lo perciben, se trata de una movilidad intraclase.

Resumen (analítico) Los estudios evidencian que la identidad étnica es un factor protector para sociedades con relaciones interétnicas e interculturales desiguales. Sin embargo, los instrumentos para su medición presentan escasas validaciones psicométricas en contextos indígenas chilenos y latinoamericanos. El estudio evaluó las propiedades psicométricas de la Escala de Identidad Étnica en una muestra de 501 adolescentes indígenas mapuche con una edad promedio de 15.63 años. Los análisis factoriales exploratorios y confirmatorios evidenciaron una estructura factorial coincidente con la propuesta teórica de tres factores (exploración, resolución y afirmación), la cual explicó el 76 % de la varianza. El análisis de fiabilidad indicó un alfa ordinal de .90 para la escala total y el análisis correlacional demostró validez externa. Se concluyó que la escala presenta evidencia psicométrica para su uso en población adolescente mapuche.

Abstract (analytical) Recent studies evidence that ethnic identity is a protective factor for interethnic and intercultural societies with unequal relations. The tools that measure ethnic identity have limited psychometric validations for indigenous Chilean and Latin American populations. This study evaluated the psychometric properties of the ethnic identity scale with a sample of Mapuche indigenous adolescents consisting of 501 students with an average age of 15.63, through which exploratory and confirmatory factorial analyses were performed. The factorial assessment results showed strong internal reliability. The suitability of the three factors included in the theoretical proposal (exploration, resolution and affirmation) explained 76% of the variance. The reliability measures indicated a .90 value for the ordinal alpha and the correlations demonstrated external validity. It is concluded that the EIS presents sufficient psychometric evidence for use in the Mapuche adolescent population.

Resumo (analítico) Estudos mostram que a identidade étnica é um fator de proteção para sociedades com relações interétnicas e interculturais desiguais. No entanto, os instrumentos para a sua medição apresentam pouca validação psicométrica nos contextos indígenas chilenos e latino-americanos. O estudo avaliou as propriedades psicométricas da escala de identidade étnica numa amostra de 501 adolescentes Mapuche indígenas com uma idade média de 15.63 anos. As análises exploratórias e confirmatórias dos fatores mostraram uma estrutura de fatorial que coincidia com a proposta teórica de três fatores (exploração, resolução e afirmação), o que explicava 76% da variância. A análise de fiabilidade indicou um alfa ordinal de .90 para a escala total e a análise correlacional demonstrou a validade externa. Concluiu-se que a EIE apresenta provas psicométricas para uso na população adolescente Mapuche.

SUMMARY: Extensive periods of hypoxic cause adaptive changes that make it possible to respond to the demands imposed by the environment. However, there are cases where this exposure is intermittent, as is the case of workers in andean areas. The objective of this communication was to verify the morphological and mechanical effects on diaphragm and lungs of rats subjected to intermittent hypoxic. Four 6-monthold Sprague Dawley rats were used. Two rats were subjected to 10 cycles of intermittent hypobaric hypoxic (IHH) of 96 h (~428 torr, PO2 90 mm Hg), followed by 96 h of normobaric normoxia, for 80 days. Uniaxial traction and staining tests were performed with Hematoxylin-Eosin and Picrosirius red de Junqueira. When comparing the curves of the diaphragms, those subjected to hypoxic slightly reduce their effort with respect to the condition of normoxia, in the lung tissue the hypoxic negatively affects its resistance, these show a lower slope with respect to the normoxics. In the histological analysis, the pulmonary parenchyma had a lower number of blood vessels and cellularity, such as a greater area fraction of alveolar spaces and amount of total collagen in IHH group. In the diaphragm, IHH group had a lower number of irregularly distributed myocytes and a lower amount of total collagen. In conclusion, the main findings indicate that the diaphragm and lung tissue subjected to IHH undergo structural changes, which result in a decrease in tensile strength.

RESUMEN: Periodos extensos de hipoxia provocan cambios adaptativos que permiten responder a las demandas impuestas por el ambiente. Sin embargo, existen casos donde esta exposición es intermitente, como es el caso de los trabajadores en zonas andinas. El objetivo de esta comunicación fue comprobar los efectos morfológicos y mecánicos en diafragma y pulmones de ratas sometidas a la hipoxia intermitente. Se utilizaron 4 ratas Sprague Dawley de 6 meses de edad. Dos ratas fueron sometidas a 10 ciclos de hipoxia hipobárica intermitente (HHI) de 96 h (~428 torr; PO2 90 mm Hg), seguidos de 96 h de normoxia normobárica, durante 80 días. Se realizaron pruebas tracción uniaxial y de tinción con HematoxilinaEosina y Picrosirius red de Junqueira. Al comparar las curvas de los diafragmas, los sometidos a hipoxia reducen levemente su esfuerzo respecto a la condición de normoxia, en el tejido pulmonar la hipoxia afecta negativamente su resistencia, estas muestran una pendiente menor respecto a las normóxicas. En el análisis histológico, el parénquima pulmonar presentó menor cantidad de vasos sanguíneos y celularidad, como una mayor fracción de área de los espacios alveolares y cantidad de colágeno total en el grupo HHI. En el diafragma, el grupo HHI presentó menor cantidad de miocitos distribuidos irregularmente y de colágeno total. En conclusión, los principales hallazgos indican que el diafragma y el tejido pulmonar sometido a HHI sufren cambios estructurales, que se traducen en una disminución en su capacidad de resistencia tensil.

The emergence and dissemination of antimicrobial-resistant bacteria (ARB) is currently seen as one of the major threats to human and animal public health. Veterinary use of antimicrobials in both developing and developed countries is many-fold greater than their use in human medicine and is an important determinant in selection of ARB. In light of the recently outlined National Plan Against Antimicrobial Resistance in Chile, our findings on antimicrobial use in salmon aquaculture and their impact on the environment and human health are highly relevant. Ninety-five percent of tetracyclines, phenicols and quinolones imported into Chile between 1998 and 2015 were for veterinary use, mostly in salmon aquaculture. Excessive use of antimicrobials at aquaculture sites was associated with antimicrobial residues in marine sediments 8 km distant and the presence of resistant marine bacteria harboring easily transmissible resistance genes, in mobile genetic elements, to these same antimicrobials. Moreover, quinolone and integron resistance genes in human pathogens isolated from patients in coastal regions adjacent to aquaculture sites were identical to genes isolated from regional marine bacteria, consistent with genetic communication between bacteria in these different environments. Passage of antimicrobials into the marine environment can potentially diminish environmental diversity, contaminate wild fish for human consumption, and facilitate the appearance of harmful algal blooms and resistant zoonotic and human pathogens. Our findings suggest that changes in aquaculture in Chile that prevent fish infections and decrease antimicrobial usage will prove a determining factor in preventing human and animal infections with multiply-resistant ARB in accord with the modern paradigm of One Health.

El aumento de la resistencia bacteriana múltiple a antimicrobianos es considerado una gran amenaza para la salud pública mundial y como generador de una importante crisis en el funcionamiento de los sistemas de salud. Esta crisis es discutida diariamente por los gobiernos y los parlamentos, las instituciones globales de salud, fundaciones benéficas y de científicos y de profesionales de la salud y también de consumidores de productos animales. En todos los países del orbe se ha identificado al uso de antimicrobianos en la crianza industrial de animales como un importante determinante en la selección de esta resistencia. Aprovechando la oportunidad que se ha planteado en Chile con el diseño del Plan Nacional Contra la Resistencia a los Antimicrobianos, hemos creído importante revisitar y actualizar sumariamente nuestros estudios sobre el uso de antimicrobianos en la acuicultura del salmón y de su potencial impacto en el ambiente y la salud humana y animal. Estos estudios indican que 95% de tres grupos de antimicrobianos importados al país, que incluyen tetraciclinas, fenicoles y quinolonas, son usados en medicina veterinaria y mayormente en la acuicultura del salmón. Nuestros estudios indican que el excesivo uso de estos antimicrobianos genera la presencia de residuos de antimicrobianos en el ambiente marino hasta 8 km de los sitios de acuicultura, los que seleccionan a bacterias con resistencia múltiple en dicho ambiente, ya que ellas contienen variados genes de resistencia a estos antimicrobianos. Estos genes de resistencia están contenidos en elementos genéticos móviles incluyendo plásmidos e integrones, los que son trasmitidos a otras bacterias permitiendo su potencial diseminación epidémica entre poblaciones bacterianas. Bacterias del ambiente marino contienen genes idénticos a los genes de resistencia a quinolonas e integrones similares a los de patógenos humanos, sugiriendo comunicación genética entre estas bacterias de diversos ambientes. Alrededor de los recintos de acuicultura, este uso exagerado de antimicrobianos contamina con ellos también a peces silvestres para consumo humano y potencialmente selecciona BRA en su carne y en los productos de acuicultura. El consumo de estos productos selecciona bacterias resistentes en el microbioma humano y facilita también el intercambio genético entre bacterias del ambiente acuático y la microbiota comensal y patógena humana. El pasaje de antimicrobianos al ambiente marino disminuye la diversidad en él, y potencialmente podría facilitar la aparición de florecimientos de algas nocivas, la infección de peces por patógenos piscícolas resistentes los antimicrobianos y la aparición de patógenos zoonóticos resistentes, incluyendo a Vibrio parahaemolyticus. Estos hallazgos sugieren que la prevención de infecciones en peces y la disminución del uso de antimicrobianos en su crianza, será en Chile un factor determinante en la prevención de infecciones humanas y animales con resistencia múltiple a los antimicrobianos, de acuerdo con el paradigma moderno e integral de Una Salud.

The main point of this study is to determine the dimensions of the thorax and its relation to the ventilatory function of a group of swimmers members of the swimming team at Universidad Católica del Maule. 42 male subjects, whose age vary between 18 and 26 years old, were evaluated. 18 subjects were on the swimming team and 24 were sedentary students, all of them study in Universidad Católica del Maule. They were performed a basic bodily anthropometry assessment (weight and body size) and they were also performed a specific anthropometric assessment of the thorax, as well as one of the anteroposterior diameter (ATD) and the transverse diameter (TDT), thorax and the mesosternal perimeter (MP). Finally, they were performed an evaluation of the ventilatory function trough body plethysmography. For the statistical analysis the normality of the data was determined, then came the use of t of student or U of Mann-Whitney and Pearson or Spearman, depending of the case, whether it was to determine the statistical significance or correlation, respectably. A level of statistical significance of p> 0,05 was considered. A significant increase of the body mass index (BMI) was observed, bigger thorax dimensions, PDA to full pulmonary capacity (FPC) and PME to FPC and residual volume. In the ventilatory function there was a significant increase of maximal expiratory pressure (MEP), maximal inspiratory pressure (MIP), inspiratory capacity (IC) and FPC. There were also correlations that vary from good to excellent between the diameter and perimeter of the thorax and IC and FMP, among the swimmers. Thus, it is concluded that relations exist between the anthropometrical of the thorax and the ventilatory function in swimmers of the Universidad Católica del Maule, Talca, Chile.

El objetivo del siguiente estudio fue determinar las dimensiones del tórax y su relación con la función ventilatoria en un grupo de nadadores correspondientes a la selección de natación de la Universidad Católica del Maule. Se evaluaron 42 sujetos de sexo masculino entre 18 y 26 años, 18 seleccionados de natación y 24 estudiantes sedentarios, todos de la Universidad Católica del Maule. Se les realizó antropometría corporal básica (peso y talla corporal) y específica de tórax, diámetro antero-posterior (DAT) y transverso (DTT) de tórax y perímetro mesoesternal (PME). Posterior a esto, se evaluó la función ventilatoria a través de pletismografía corporal. Para el análisis estadístico se determinó la normalidad de los datos posteriormente se utilizó t de student o U de Mann-Whitney y Pearson o Spearman según correspondiera, para establecer significancia estadística o correlación, respectivamente. Se consideró un nivel de significancia estadística de p<0,05. Se observó un aumento significativo del índice de masa corporal (IMC), mayores dimensiones de tórax en nadadores, DAT a capacidad pulmonar total (CPT) y PME a CPT y volumen residual (VR). En función ventilatoria existió un aumento significativo de presión espiratoria máxima (PEM), presión inspiratoria máxima (PIM), capacidad inspiratoria (CI) y CPT. También se observaron correlaciones de buenas a excelentes entre diámetro y perímetro de tórax con CI y CPT en los nadadores. Por tanto, se concluye que existió una relación entre la antropometría de tórax y la función ventilatoria en nadadores de la Universidad Católica del Maule.

O Projeto Minas-Rio é o maior projeto da Anglo American no mundo e considera um circuito de remoagem com Vertimill para adequar a distribuição de tamanho de partículas para alimentar o mineroduto que irá transportar o minério da cidade de Conceição do Mato Dentro, no Estado de Minas Gerais, ao Porto do Açu, no Rio de Janeiro, Brasil. Uma campanha de testes em escala-piloto com Vertimill foi realizada nas instalações da Metso localizadas na cidade de York, Estado da Pensilvânia, EUA, para fornecer informações para o dimensionamento do circuito de moagem industrial. O principal objetivo desse trabalho é propor uma forma de simular o circuito industrial com Vertimill, utilizando o modelo de balanço populacional, normalmente usado para simular moinhos de bolas. As simulações foram baseadas na função seleção e na função quebra determinadas a partir dos testes de laboratório utilizando um moinho de bolas de batelada. As simulações foram realizadas utilizando um modelo de Vertimill implementado no simulador de processos minerais ModsimTM. Os resultados das simulações mostraram que é possível simular os testes-piloto, com boa precisão, através de testes simplificados em escala de laboratório, com pequenas quantidades de amostras.

The Minas-Rio Project is the biggest project from Anglo American in the world and considers Vertimill in the regrinding circuit to adequate the particle size distribution to feed slurry pipeline that will pump the ore from Conceição do Mato Dentro in Minas Gerais State to Acu Port in Rio de Janeiro State, Brazil. A Vertimill pilot test campaign was carried out at Metso's pilot plant facility located in York city, Pennsylvania State, USA, to provided information to sizing the industrial grinding circuit. The main objective of this work is proposing a way to simulate the industrial Vertimill using the population balance model, normally used to simulate ball mills. The simulations were based on the selection and breakage functions determined from the laboratory tests using a batch ball mill. The simulations were performed using a Vertimill model implemented in the ModsimTM plant-wide simulator. The results of simulations shows that was possible to simulate the pilot tests, with good accuracy, considering simple laboratory tests with small quantities of samples.

Fabry's disease is an X-linked recessive inborn error of metabolism of glycosphingolipids, caused by the deficiency of the lisosomal enzyme alpha-galactosidase. It is a rare disease with an estimated incidence rate of approximately 1:80.000 to 1:117,000 births in the general population. Recently, the growing knowledge about this disease has permitted the development of enzyme replacement therapy, which has modified the prognosis and quality of life of these patients. In Chile, the real incidence is unknown, but the increase in the number of patients diagnosed during the last five years, mainly in the north of the country. This guide was prepared with the intention of establishing a consensus for the diagnosis, treatment and monitoring of the patients with Fabry disease based on the present available scientific evidence.

La enfermedad de Fabry es un error innato del catabolismo de los glucoesfingolipidos, de herencia recesiva ligada al cromosoma X, causado por la deficiencia de la enzima lisosomal alfa-galactosidasa A (α-gal A). Es un defecto poco frecuente, con una incidencia estimada de 1:80.000 a 1:117.000, entre la población general. Recientemente, el creciente conocimiento acerca de esta enfermedad, ha permitido el desarrollo de la terapia de reemplazo enzimático, la cual ha modificado el pronóstico y calidad de vida de los pacientes. En Chile, se desconoce la incidencia real, pero el aumento del número de pacientes diagnosticados durante los últimos cinco años, principalmente en la zona norte del país, ha generado un mayor interés por esta enfermedad. Esta guía fue elaborada con la intención de establecer un consenso para el diagnóstico, tratamiento y seguimiento de los pacientes con enfermedad de Fabry, basado en la evidencia científica, actualmente disponible.

Objetivou-se analisar energeticamente a produção de etanol a partir do milho (Zea mays L.). As pesquisas de campo foram realizadas na região paulista do Médio Paranapanema, São Paulo (SP), no período de janeiro a dezembro de 2007. Avaliou-se o consumo energético referente às fases de produção e processamento industrial do grão. O custo energético total da produção agrícola correspondeu a 15.633,7MJ ha-1, e o item mais oneroso foi o de "insumos" (77,5%). Nas etapas industriais, o consumo energético foi equivalente a 3.882,2MJ t-1. As operações de "hidrólise, sacarificação e tratamento do caldo" representaram 50,2% do dispêndio energético total. O custo energético do milho foi de 7,9MJ L-1 nas operações agronômicas de produção e 11,8MJ L-1 nas etapas de processamento industrial. O balanço energético do cultivo e da industrialização foi de 1,2MJ.

This research analyzed the energetic consumption of etanol from the corn crops (Zea mays L.). The field surveys were carried out in the Midle Paranapanema River Region, São Paulo state, Brazil, in the period from January to December 2007. The energy consumption on stage of production and industrial processing of grain were evaluated. It was verified that the total energetic cost of the crop production corresponded to 15,633.7MJ ha-1, and the most onerous item was the "inputs" (77.5%). In the industrial step, the energetic consumption was equivalent to 3,882.2MJ t-1. The operations of "hydrolysis, saccharification and treatment of the broth" represented 50.2% of the total energetic expenditure. It was also observed an energetic cost of 7.9MJ L-1 in relation to the etanol produced in the main crop production operations, and 11.8MJ L-1 in the industrial processing. The energy balance of crop production and industrialization was of 1.2MJ.

Buscou-se, com este trabalho, analisar energeticamente o sistema de produção de etanol utilizando como fonte de carboidratos, a mandioca. As pesquisas de campo foram realizadas na região do Médio Paranapanema, Estado de São Paulo, no período de janeiro a dezembro de 2007. Avaliou-se o consumo energético referente às fases de produção e processamento industrial e se verificou que o custo energético total da produção agrícola correspondeu a 9.528,33 MJ ha-1, sendo que o item mais oneroso foi o de insumos (35,72%). Nas etapas industriais o consumo energético foi equivalente a 2.208,28 MJ t-1. As operações de hidrólise/sacarificação/tratamento do caldo representaram 56,72% do dispêndio energético total. A mandioca apresentou um custo energético de 1,54 MJ L-1 em relação ao etanol produzido nas principais operações agronômicas de produção e 11,76 MJ L-1 nas etapas de processamento industrial. A eficiência energética observada no cultivo e industrialização da mandioca foi de 1,76.

This work aimed to analyze the energetic consumption of the etanol production system, using the cassava as carbohidrates source. The researches were carried out from the field in the mid region of Paranapanema river, Sao Paulo state, during the period January to December, 2007. The energy consumption referring to the phases of crop production and industrial processing were appraised. It was verified that the total energetic cost of the crop production corresponded to 9,528.33 MJ ha-1, and the most onerous item was the inputs (35.72%). In the industrial step, the energetic consumption was equivalent to 2,208.28 MJ t-1. The operations of hydrolysis/saccharification/ treatment of the juice represented 56.72% of the total energetic expenditure. The cassava crop presented an energetic cost of 1.54 MJ L-1 in relation to the etanol produced in the main agronomic operations crop production, and 11.76 MJ L-1 in the industrial processing. The energy efficiency observed in the cultivation and industrialization of the cassava was of 1.76.

Analisou-se energeticamente a produção de etanol originária da cana-de-açúcar. As pesquisas de campo foram realizadas na região paulista do Médio Paranapanema, no período de janeiro a dezembro de 2007, avaliando-se o consumo energético referente às fases de produção e processamento industrial. Verificou-se que o custo energético total da produção agrícola correspondeu a 14.370,9MJ ha-1, e o item mais oneroso foi o de "insumos" (50,4%). Nas etapas industriais, o consumo energético foi equivalente a 1.641,6MJ t-1. As operações de "hidrólise, sacarificação e tratamento do caldo" representaram 71,7% do dispêndio energético total. Na cana-de-açúcar, observou-se um custo energético de 2,0MJ L-1 em relação ao etanol produzido nas principais operações agronômicas de produção e 19,4MJ L-1 nas etapas de processamento industrial. A eficiência energética no cultivo e na industrialização foi de 1,1.

This research analyzed the energetic consumption of the etanol, using as raw-material sugar cane. The searches were carried out from the field in the Midle Paranapanema River Region, Sao Paulo state, in the period from January to December 2007. The referring energy consumption from the crop production phase and industrial processing were evaluated. It was verified that the total energetic cost of the crop production corresponded to 14370.9MJ ha-1, and the most onerous item was the "inputs" (50.4%). In the industrial step, the energetic consumption was equivalent to 1,641.56MJ t-1. The 'hydrolysis, saccharification and broth treatment operations' represented 71.72% of the total energetic expenditure. It was also observed an energetic cost of 2.0MJ L-1 in relation to the etanol produced in the main crop production operations, and 19.4MJ L-1 in the industrial processing. The energy efficiency observed in the crop production and industrialization were of 1.1.