CANVAS: una nueva etiología de la ataxia del adulto. La asociación con tos orienta al diagnóstico. Comunicación de 2 pacientes / Late-Onset Cerebellar Ataxia with Neuropathy: Uncovering the Role of RFC1 Gene Mutations

Presentamos dos pacientes no relacionados con ataxia cerebelosa de inicio tardío asociada con neuropatía y tos seca de larga data. Un paciente tenía dos hermanos afectados con neuropatía sensorial y tos. Ambos probandos tuvieron investigaciones extensas que incluyó pruebas genéticas negativas para las ataxias más comunes, así como pruebas paraneoplásicas y otras causas inmunológicas. Ambos pacientes mostraron una expansión intrónica anormal en el pentanucleótido AAGGG del gen RFC1. Esta etiología se informa como causa frecuente de ataxia de inicio en adultos; la presencia de tos puede conducir al diagnóstico correcto.

We report two unrelated patients with late-onset cerebellar ataxia associated with neuropathy and a long-standing dry cough. One patient had two siblings affected with sensory neuropathy and cough. Both probands had extensive investigations including genetics testing negative for most common ataxias as well as testing for paraneoplasic and other immunologic causes. Both patients showed an abnormal intronic expansion in the pentanucleotide AAGGG of the gene RFC1. This etiology is being reported as frequent cause of adult-onset ataxia; the presence of cough may lead to the correct diagnosis.

Modelo de avaliação da viabilidade econômico-financeira da implantação de usinas de reciclagem de resíduos da construção civil em municípios brasileiros / Economic and financial viability evaluation model for the implementation of waste recycling plants construction in Brazilian municipalities

RESUMO Este trabalho teve como objetivo geral desenvolver um modelo de avaliação da viabilidade econômico-financeira da implantação de usinas de reciclagem de resíduos da construção civil e demolição (URRCD) em municípios brasileiros. O modelo de avaliação proposto para cada projeto de investimento (PI) em estudo uniu o modelo de negócio Canvas, o plano de negócios, a metodologia multi-índice proposta por Souza e Clemente (2009) e ampliada por Lima et al. (2015) por meio do aplicativo web $AVEPI. Também foi realizada uma análise de sensibilidade por meio de limites de elasticidade e valores-limite, bem como uma análise estocástica utilizando a simulação de Monte Carlo (SMC). Com base na proposição desse modelo, este foi aplicado em um estudo de caso no município de Pato Branco (PR). Os resultados mostram que há viabilidade econômico-financeira na implantação desse PI, uma vez que o valor presente líquido médio é de aproximadamente R$ 583.919,87, e a recuperação do investimento se concretizará em 12 anos (payback). Por meio da SMC, visualizou-se que há alta probabilidade (99,78%) de que o investimento na URRCD de Pato Branco seja rentável. Entretanto, os índices de elasticidade mostraram que é preciso monitorar as receitas esperadas e os custos estimados com mais atenção. Assim, será preciso um trabalho, por parte dos gestores da URRCD, em prol de sempre estarem buscando aumentar a demanda pelos agregados reciclados, como maneira de aumentar a receita da usina, desde que se respeite a sua capacidade de produção.

ABSTRACT This work had as main objective to develop an evaluation model for analyzing the economic and financial feasibility of implementing Construction and Demolition Waste Recycling Plants (URRCD) in Brazilian municipalities. The evaluation model proposed for the Investment Project (IP) under study was performed using the business model Canvas, the business plan, the multi-index method proposed by Souza and Clemente (2009) and expanded by Lima et al. (2015) through the $AVEPI Web app. Also, a sensitivity analysis was performed, through the elasticity limits and limit values as well as a stochastic analysis using the Monte Carlo Simulation (MCS). From the proposition of this model, it was applied in a case study in Pato Branco, Paraná. The results showed that there is economic and financial viability in the implementation of this IP, since the average Net Present Value (NPV) is about R$ 583,919.87 and the investment recovery will materialize in 12 years (Payback). Through MCS, it was observed that there is a high probability (99.78%) that the investment in the URRCD of Pato Branco, Paraná, be profitable. However, the elasticity indices showed that it is necessary to monitor the expected revenues and the estimated costs with more attention. Thus, it will require a work by the managers of the URRCD, in favor of always seeking to increase the demand for recycled aggregates, as a way to increase the revenue of the plant, as long as it does not exceed its production capacity.

Solvent hazard in canvas-rubber shoe factories

1. This investigation has removed definitely our apprehension that gasoline and naphtha, which are potential hazards in the three convas-rubber shoe factories, might be a real danger. The highest concentration of gasoline vapor found was only 460 p.p.m, which is lower than the maximum allowable concentration-500 p.p.m2. The statistical analysis of the blood pressure by age group did not show any significant difference between the exposed and unexposed groups of workers, indicating no appreciable effects of the gasoline and naphtha vapors3. The symptoms found among the workers were also analyzed and were found to be not due to the solvent. There were, however, some individuals who showed more susceptibility or sensitiveness to gasoline vapor; and these individuals should be transferred to other departments of the factories free from such vapor4. The psychrometric findings showed that there is need of rearrangement of electric fans in one factory to promote air circulation, and opening of more windows in the two others to correct deficient temperature. Regarding Kata value (cooling power of air) and wind velocity in the different sections of the factories, the following standards are recommended: wind velocity of 50-100 feet per minute where there is no gasoline vapor; not less than 200 feet per minute where gasoline vapor is present; and Kata cooling power of not less than five where the temperature is more than 85 degrees F5. For preventive measures, it is recommended that exhaoust ventilation systems be provided in the vicinity of the canvas-rubber shoe making tables and in the rubber cement mixers. The factories should also be inspected at least twice a year to determine the concentration of solvent vapor in the different working areas. (Conclusions and recommendations)