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This paper proposes an optimization model, using Mixed-Integer Linear Programming (MILP), to support decisions related to making investments in the design of power grids serving industrial clients that experience interruptions to their energy supply due to disruptive events. In this approach, by considering the probabilities of the occurrence of a set of such disruptive events, the model is used to minimize the overall expected cost by determining an optimal strategy involving pre- and post-event actions. The pre-event actions, which are considered during the design phase, evaluate the resilience capacity (absorption, adaptation and restoration) and are tailored to the context of industrial clients dependent on a power grid. Four cases are analysed to explore the results of different probabilities of the occurrence of disruptions. Moreover, two scenarios, in which the probability of occurrence is lowest but the consequences are most serious, are selected to illustrate the model's applicability. The results indicate that investments in pre-event actions, if implemented, can enhance the resilience of power grids serving industrial clients because the impacts of disruptions either are experienced only for a short time period or are completely avoided.

Background: Floating-Harbor syndrome (FHS) is a rare condition characterized by short stature, delays in expressive language, and a distinctive facial appearance. Recently, heterozygous truncating mutations in SRCAP were determined to be disease-causing. With the availability of a DNA based confirmatory test, we set forth to define the clinical features of this syndrome., Methods and Results: Clinical information on fifty-two individuals with SRCAP mutations was collected using standardized questionnaires. Twenty-four males and twenty-eight females were studied with ages ranging from 2 to 52 years. The facial phenotype and expressive language impairments were defining features within the group. Height measurements were typically between minus two and minus four standard deviations, with occipitofrontal circumferences usually within the average range. Thirty-three of the subjects (63%) had at least one major anomaly requiring medical intervention. We did not observe any specific phenotype-genotype correlations., Conclusions: This large cohort of individuals with molecularly confirmed FHS has allowed us to better delineate the clinical features of this rare but classic genetic syndrome, thereby facilitating the development of management protocols.

Gliomatosis cerebri (GC) is a rare form of CNS neoplasia in which there is diffuse involvement of the nervous tissue with or without the presence of tumor mass. The origin of the tumor is unknown, nor whether it represents a disease with diffuse onset or infiltration from a neoplastic focus. Here we studied the histopathologic characteristics of 6 cases with a diagnosis of GC and performed an immunohistochemical analysis using glial fibrillary acidic protein (GFAP), synaptophysin, nestin and vimentin. Most tumor cells were negative for GFAP, even though there were foci of positivity for this marker in all cases. We detected the presence of many positive cells for nestin and vimentin in all studied samples. The presence of these cells may indicate origin of the tumor from undifferentiated cells with a high degree of mobility.