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To present the genetic heterogeneity of a sample of the Brazilian population with transthyretin (TTR) mutations. This cohort study was descriptive and retrospective, and enrolled patients with peripheral neuropathy of unknown cause that were found to have a mutation in the TTR gene during the process of etiological investigation, between July 1997 to January 2016. Over the study period, 129 point mutations were identified in 448 tested patients, of whom 128 were of Brazilian origin. The TTR Val30Met mutation was identified in 116 patients (90.6%); while 7 (4.7%) patients had a pathogenic non-TTR mutation and 7 (4.7%) carried non-pathogenic mutations (4.7%). The four non-TTRMet30 pathogenic mutations were TTR Aps38Tyr; TTR Ile107Val; TTR Val71Ala; and TTR Val122Ile. In the non-pathogenic group, we only found two mutations, including TTR Gly6Ser and TTR Thr119Thr. Our study depicts a scenario of greater genetic heterogeneity among Brazilian hereditary transthyretin amyloidosis (hATTR) patients with familial amyloidotic polyneuropathy (FAP). We expect that this number will grow fast over a short period of time, due to increasing availability of genetic tests, increasing knowledge of the disease and the multivariate origin of our population.

Electrical installations in hazardous locations (classified areas) need careful attention because non-conformities can bring considerable risks not only to the site, but also for other industries and residential areas nearby. To assure safe operation of an industrial facility it is necessary not only that all electrical and electronic equipment to be used in hazardous locations is correctly specified and purchased (known as “Ex equipment”), but also properly installed and maintained. The discussion in this paper is largely based upon International Electrotechnical Commission (IEC) standards; however, conceptually the same philosophy applies to American based standards and installations as well.

This paper evaluates the protection system of an offshore gas production unit and proposes utilization of devices dedicated to identify the arc flash incidents inside the panels. A quantitative analysis has been performed by comparing the incident energy levels of the original design with the levels provided by the new design. The new design includes numerical relays equipped with dual sensing of arc flash: optical detection of the light and high-speed overcurrent detection. The analysis indicates that a drastic reduction of incident energy can be achieved. The reduction is based on very fast detection and elimination of the arc, lowering the arcing time.

The petroleum and chemical industries are facing pressure to become more energy efficient and sustainable. The use of green energy is a feasible option to address that issue. This paper discusses the application of various source technologies available to offshore petroleum and gas production platforms. The pros and cons of each source is considered, as applied to critical systems, including safety systems. A hybrid system using photovoltaic cells and wind generation, installed on an offshore platform in Brazil is reviewed.