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[pt] Este trabalho apresenta um método geral e prático, baseado em técnicas de convolução discreta, para avaliar o valor esperado e a variância dos índices de perda de carga associados a um sistema de geração. O método proposto trata as indisponibilidades das unidades geradoras e os valores estimados para os picos de carga como variáveis aleatórias, especificadas por médias e variâncias, e modela, sem qualquer restrição, geradores com estados de capacidade intermediários. Também é mostrado como as técnicas de Transformada Rápida de Fourier podem ser aplicadas na solução deste problema. O algoritmo proposto é aplicado a vários sistemas de geração, incluindo o sistema teste de confiabilidade do IEEE, e os resultados são discutidos. / [en] This work presents a pratical and general method based on discrete convolution techniques for evaluating the expectation and variance of the loss of load índices, for a single-area system, by treating forced outage rates and forecast peak loads as random variables with specified means and variances. The proposed method easily accounts for partial forced outage rates associated with unit derated states, without any restrictions. Also, it is shown how fast Fourier transtorm techniques can be used to carry out the discrete convolution. The proposed algorithm is applied to typical generating systems including the IEEE Reliability Test System, and the results are discussed.

[pt] Este trabalho descreve um método de cálculo da confiabilidade da capacidade de geração de sistemas de potência, para fins de operação do mesmo. O objetivo desejado foi o de permitir uma discussão matemático- estatistica, no que diz respeito a determinação da reserva girante a ser mantida pelos sistemas de potência. Foram abordados conceitos básicos de confiabilidade e de processos de Markov. O trabalho é concluído com a apresentação de exemplos numéricos. / [en] This work describes a method of calculating the reliability of the generating capacity of power systems necessary in order to operate them. The purpose is to open a mathematical-statistical discussion about the definition of the spinning reserve to be maintained by the power systems. This work also touches on basic concepts of reliability and Marlov processes. It concludes with the presentation of numerical examples.

Mitochondrial disorders, with incidence 1:5000 live births children, are one of the most common metabolic diseases. Clinically, it is heterogeneous group of disorders caused by mutations in more than 250 genes. Diagnostic of patients with suspected mitochondrial disorder relies on broad spectrum of biochemical analysis. One of them is a measurement of Mitochondrial Energy Generating Capacity (MEGC). The principle of MEGC analysis is measuring oxidations rate of 14 C - labeled substrates in 10 different incubations. These incubations contain [1-14 C]pyruvate, [U-14 C]malate or [1,4-14 C]succinate, donors and acceptors of Acetyl-CoA and inhibitors of TCA cycle. The results of MEGC analysis provide a variety of information about mitochondrial energy metabolism (MEM) of individual in particular tissue. In diagnostic of patients with suspected mitochondrial disorder is MEGC routinely determined in skeletal muscle. The aim of this study is to optimize MEGC analysis for its use in cultures skin fibroblasts. In sum, MEGC analysis was performed in 23 patients with primary deficiency of oxidative phosphorylation (OXPHOS), in 7 patients with secondary deficiency of OXPHOS and in 15 controls cell lines. The results of MEGC in cultured skin fibroblasts were then compared with results of spectrophotometric...