Hydrocephalus and moderate mental retardation in a boy with Van der Woude phenotype and IRF6 gene mutation

In this report, we present a boy with lower lip pits, distinct craniofacial dysmorphism with cleft lip and palate, central nervous system malformation, and severe mental retardation. Similar but less pronounced facial findings were present in his mentally normal mother and maternal grandfather, both presenting with lower lip pits. Cleft lip was present in patient's father. Analysis of the VWS1 and VWS2 regions were performed to elucidate the molecular basis of the phenotype of the propositus. Screening or mutations at the IRF6 gene detected a pathogenic mutation (c.960G > C) in the propositus and in his mother; and a single nucleotide polymorphism (c.175-5C > G) in the propositus and in his father. Clinical and genetic aspects of this case are discussed.

EFFICIENT FAULT LOCATION IN UNDERGROUND DISTRIBUTION SYSTEMS THROUGH OPTIMUM-PATH FOREST

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Dexamethasone treatment in vivo counteracts the functional pancreatic islet alterations caused by malnourishment in rats

The effects of dexamethasone (Dex) on the metabolic parameters, peripheral insulin, and glucose sensitivity in vivo as well as on islet function ex vivo of rats submitted to low-protein diet were analyzed. Dexamethasone (1.0 mg/kg body weight) was administered intraperitoneally daily to adult Wistar rats fed on a normal-protein diet or low-protein diet (LPD) for 5 days, whereas control rats fed on a normal-protein diet or low-protein diet (LP) received saline alone. At the end of the experimental period, LP rats showed a significant reduction in serum insulin, total serum protein, and serum albumin levels compared with rats fed on a normal-protein diet (P < .05). All these parameters tended to be normalized in LPD rats (P < .05); furthermore, these rats exhibited increased serum glucose and nonesterified fatty acid levels compared with LP rats (P < .05). Rats submitted to the low-protein diet demonstrated normal peripheral glucose sensitivity and improved peripheral insulin sensitivity, which was reversed by Dex treatment. A reduced area of islets from LP rats was partially recovered in LPD rats (P < .05). At 16.7 mmol/L glucose, insulin secretion from LPD islets was also partially recovered and was significantly higher than that from LP islets (P < .05). In conclusion, induction of insulin resistance by Dex treatment reverses most of the metabolic alterations in rats submitted to a low-protein diet. In addition, several islet functions were also improved by Dex, confirming the plasticity of pancreatic islets in adverse conditions. (C) 2008 Elsevier B.V. All rights reserved.

Functional alterations in endocrine pancreas of rats with different degrees of dexamethasone-induced insulin resistance

Objectives: We have analyzed the peripheral insulin and glucose sensitivity in vivo, and islet function ex vivo in rats with different degrees of insulin resistance induced by dexamethasone (DEX).Methods: Dexamethasone, in the concentrations of 0.1 (DEX 0.1), 0.5 (DEX 0.5), and 1.0 mg/kg body weight (DEX 1.0) was administered daily, intraperitoneally, to adult Wistar rats for 5 days, whereas controls received saline.Results: Dexamethasone treatment induced peripheral insulin resistance in a dose-dependent manner. At the end of the treatment, only DEX 1.0 rats showed significant increase of postabsorptive blood glucose and serum triglycerides, and nonesterified fatty acids levels. Incubation of pancreatic islets in increasing glucose concentrations (2.8-22 mM) led to an augmented insulin secretion in all DEX-treated rats. Leucine, carbachol, and high KCl concentrations induced the insulin release in DEX 0.5 and DEX 1.0, whereas arginine augmented secretion in all DEX-treated groups.Conclusions: We demonstrate that in DEX 0.5 and, especially in DEX 0.1 groups, but not in DEX 1.0, the adaptations that occurred in the endocrine pancreas are able to counteract metabolic disorders (glucose intolerance and dyslipidemia). These animal models seem to be interesting approaches for the study of degrees of subjacent effects that may mediate type 2 diabetes (DEX 1.0) and islet function alterations, without collateral effects (DEX 0.1 and DEX 0.5).

On 3-Parameter Families of Piecewise Smooth Vector Fields in the Plane

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Design of a simple detection cell with extended optical path length for capillary electrophoresis: Application to multiresidue pesticide analysis

Absorbance detection in capillary electrophoresis (CE), offers an excellent mass sensitivity, but poor concentration detection limits owing to very small injection volumes (normally I to 10 nL). This aspect can be a limiting factor in the applicability of CE/UV to detect species at trace levels, particularly pesticide residues. In the present work, the optical path length of an on-column detection cell was increased through a proper connection of the column (75 mu m i.d.) to a capillary detection cell of 180 mu m optical path length in order to improve detectability. It is shown that the cell with an extended optical path length results in a significant gain in terms of signal to noise ratio. The effect of the increase in the optical path length has been evaluated for six pesticides, namely, carbendazim, thiabendazole, imazalil, procymidone triadimefon, and prochloraz. The resulting optical enhancement of the detection cell provided detection limits of ca. 0.3 mu g/mL for the studied compounds, thus enabling the residue analysis by CE/UV.

Environmental management in ethanol and sugarcane plants in Brazil

There has been much discussion on the importance of Brazilian ethanol in promoting a more sustainable society. However, there is a lack of analysis of whether sugarcane plants/factories that produce this ethanol are environmentally suitable. Thus, the objective of this study was to analyse stages of environmental management at four Brazilian ethanol-producing plants, examining the management practices adopted and the factors behind this adoption. The results indicate that (1) only one of the four plants is in the environmentally proactive stage; (2) all plants are adopting operational and organisational environmental management practices; (3) all plants have problems in communicating environmental management practices; and (4) the plant with the most advanced environmental management makes intense use of communication practices and is strongly oriented towards a more environmentally aware international market. This paper is an attempt to explain the complex relationship between the evolution of environmental management, environmental practices and motivation using a framework. The implications for society, plant directors and scholars are described, as well as the study's limitations.

Neural network based estimation of torque in induction motors for real-time applications

Induction motors are largely used in several industry sectors. The selection of an induction motor has still been inaccurate because in most of the cases the load behavior in its shaft is completely unknown. The proposal of this article is to use artificial neural networks for torque estimation with the purpose of best selecting the induction motors rather than conventional methods, which use classical identification techniques and mechanical load modeling. Since proposed approach estimates the torque behavior from the transient to the steady state, one of its main contributions is the potential to also be implemented in control schemes for real-time applications. Simulation results are also presented to validate the proposed approach.

GREEN MACHINING ORIENTED TO DIMINISH DENSITY GRADIENT FOR MINIMIZATION of DISTORTION IN ADVANCED CERAMICS

After sintering advanced ceramics, there are invariably distortions, caused in large part by the heterogeneous distribution of density gradients along the compacted piece. To correct distortions, machining is generally used to manufacture pieces within dimensional and geometric tolerances. Hence, narrow material removal limit conditions are applied, which minimize the generation of damage. Another alternative is machining the compacted piece before sintering, called the green ceramic stage, which allows machining without damage to mechanical strength. Since the greatest concentration of density gradients is located in the outer-most layers of the compacted piece, this study investigated the removal of different allowance values by means of green machining. The output variables are distortion after sintering, tool wear, cutting force, and the surface roughness of the green ceramics and the sintered ones. The following results have been noted: less distortion is verified in the sintered piece after 1mm allowance removal; and the higher the tool wear the worse the surface roughness of both green and sintered pieces.

Analysis of the accumulated energy by thermal stratification of liquid inside storage tanks with porous layers

A mathematical model was developed in order to study the behavior of thermal stratification of liquid in a typical storage tank with porous medium. The model employs a transient stream function-vorticity formulation to predict the development of stream function and temperature fields in a charging process. Parameters analyzed include Biot, Darcy, Reynolds and Richardson numbers, position, and the thickness of the porous medium. The results show the influence of these physical parameters that should be considered for a good design of storage tanks with thermal stratification.

A new chart for monitoring the covariance matrix of bivariate processes

In this article we consider a control chart based on the sample variances of two quality characteristics. The points plotted on the chart correspond to the maximum value of these two statistics. The main reason to consider the proposed chart instead of the generalized variance |S| chart is its better diagnostic feature, that is, with the new chart it is easier to relate an out-of-control signal to the variables whose parameters have moved away from their in-control values. We study the control chart efficiency considering different shifts in the covariance matrix. In this way, we obtain the average run length (ARL) that measures the effectiveness of a control chart in detecting process shifts. The proposed chart always detects process disturbances faster than the generalized variance |S| chart. The same is observed when the size of the samples is variable, except in a few cases in which the size of the samples switches between small size and very large size.

Proposal of a Transmission Line Model Based on Lumped Elements: An Analytic Solution

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Analysis of the Electrical Characteristics of an Alternative Solution for the Brazilian-Amazon Transmission System

This article analyzes the electrical parameters of a 3-phase transmission line using a 280-m-high steel tower that has been proposed for the Amazon transmission system in Brazil. The height of the line conductors and the distance between them are intrinsically related to the longitudinal and transverse parameters of the line. Hence, an accurate study is carried out in order to show the electrical variations between a transmission line using the new technology and a conventional 3-phase 440-kV line, considering a wide range of frequencies and variable soil resistivity. First, a brief review of the fundamental theory of line parameters is presented. In addition, by using a digital line model, simulations are carried out in the time domain to analyze possible and critical over-voltage transients on the proposed line representation.