11 resultados para defeito microtubular

em Universidade Federal do Rio Grande do Norte(UFRN)


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Beta thalassemia arises as a consequence of the reduction (β+, β++, βsilent) or absence (β0) of beta globin chain synthesis and results from a number of mechanisms that lead to genetic defects. The inheritance of beta thalassemia is characterized by the existence of heterozygous individuals, compound heterozygotes, homozygotes and those with coinheritance of beta thalassemia allele and other thalassemias and/or hemoglobin variants. The aim of this study was to perform molecular and laboratory characterization of beta thalassemia in heterozygous and homozygous individuals and in those with coinheritance of S beta thalassemia. A total of 48 individuals were included (35 heterozygotes, 4 homozygotes and 9 S beta thalessemia carriers) referred to the Integrated Laboratory of Clinical Analyses of the Federal University of Rio Grande do Norte (UFRN) and the Hematology Ambulatory Facility of the Dalton Barbosa Cunha Hemocenter (Hemonorte Natal, Brazil). Peripheral blood samples form each patient underwent the following laboratory examinations: erythrogram, hemoglobin electrophoresis at alkaline pH, measurements of Hb A2, Fetal Hb and serum ferritin. DNA was extracted using the illustra blood genomicPrep Mini Spin Kit and molecular characterization was performed by the PCR/RFLP technique, which involves digestion with specific restriction enzymes for IVS-1 nt 1 (G®A), IVS-1 nt 6 (T®C) and codon 39 (CAG®TAG) mutations. Of the 35 heterozygotes, 37.1% showed IVS-1 nt 6 mutation, 42.9% IVS-1 nt 1 and 20% were carriers of other mutations not identified by the technique used. The four homozygous patients presented with the IVS-1 nt 6 mutation, while 66.7% of the individuals with S beta thalassemia had the IVS-1 nt 1 mutation. Codon 39 was not detected in any of the patients investigated. Of the thallasemic alleles found, 40.4% were IVS- 1 nt 1, 40.4% IVS-1 nt 6 and 19.2% were not identified. Laboratory data showed that the heterozygotes exhibited microcytosis and hypochromia, evidenced by MCV ranging from 57 to 75fL and MCH from 15.9 to 23.6 pg. Hemoglobin A2 varied between 3.7 and 7.2%. The homogygotes also showed reduced MCV and MCH and elevated HbA2.. Comparison of laboratory data between heterozygous individuals with IVS-1 nt 1 and IVS-1 nt 6 mutations showed that heterozygotes for the IVS1-1 mutation had significantly lower mean MCV and MCH (p = 0.023 and 0.007, respectively) and significantly higher hemoglobin A2 (p < 0.001) when compared to heterozygotes for the IVS-1 nt 6 mutation. PCR/RFLP was useful in identifying the presence or absence of IVS-1 nt 6, IVS-1 nt 1 and codon 39 mutations in most of the patients investigated here. This is the first study conducted in the state of Rio Grande do Norte, Brazil aimed at identifying beta thalassemia mutations and represents an important contribution to the knowledge regarding the molecular profile of beta thalassemia in our country

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The reconfiguration of a distribution network is a change in its topology, aiming to provide specific operation conditions of the network, by changing the status of its switches. It can be performed regardless of any system anomaly. The service restoration is a particular case of reconfiguration and should be performed whenever there is a network failure or whenever one or more sections of a feeder have been taken out of service for maintenance. In such cases, loads that are supplied through lines sections that are downstream of portions removed for maintenance may be supplied by the closing of switches to the others feeders. By classical methods of reconfiguration, several switches may be required beyond those used to perform the restoration service. This includes switching feeders in the same substation or for substations that do not have any direct connection to the faulted feeder. These operations can cause discomfort, losses and dissatisfaction among consumers, as well as a negative reputation for the energy company. The purpose of this thesis is to develop a heuristic for reconfiguration of a distribution network, upon the occurrence of a failure in this network, making the switching only for feeders directly involved in this specific failed segment, considering that the switching applied is related exclusively to the isolation of failed sections and bars, as well as to supply electricity to the islands generated by the condition, with significant reduction in the number of applications of load flows, due to the use of sensitivity parameters for determining voltages and currents estimated on bars and lines of the feeders directly involved with that failed segment. A comparison between this process and classical methods is performed for different test networks from the literature about networks reconfiguration

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The idea of considering imprecision in probabilities is old, beginning with the Booles George work, who in 1854 wanted to reconcile the classical logic, which allows the modeling of complete ignorance, with probabilities. In 1921, John Maynard Keynes in his book made explicit use of intervals to represent the imprecision in probabilities. But only from the work ofWalley in 1991 that were established principles that should be respected by a probability theory that deals with inaccuracies. With the emergence of the theory of fuzzy sets by Lotfi Zadeh in 1965, there is another way of dealing with uncertainty and imprecision of concepts. Quickly, they began to propose several ways to consider the ideas of Zadeh in probabilities, to deal with inaccuracies, either in the events associated with the probabilities or in the values of probabilities. In particular, James Buckley, from 2003 begins to develop a probability theory in which the fuzzy values of the probabilities are fuzzy numbers. This fuzzy probability, follows analogous principles to Walley imprecise probabilities. On the other hand, the uses of real numbers between 0 and 1 as truth degrees, as originally proposed by Zadeh, has the drawback to use very precise values for dealing with uncertainties (as one can distinguish a fairly element satisfies a property with a 0.423 level of something that meets with grade 0.424?). This motivated the development of several extensions of fuzzy set theory which includes some kind of inaccuracy. This work consider the Krassimir Atanassov extension proposed in 1983, which add an extra degree of uncertainty to model the moment of hesitation to assign the membership degree, and therefore a value indicate the degree to which the object belongs to the set while the other, the degree to which it not belongs to the set. In the Zadeh fuzzy set theory, this non membership degree is, by default, the complement of the membership degree. Thus, in this approach the non-membership degree is somehow independent of the membership degree, and this difference between the non-membership degree and the complement of the membership degree reveals the hesitation at the moment to assign a membership degree. This new extension today is called of Atanassov s intuitionistic fuzzy sets theory. It is worth noting that the term intuitionistic here has no relation to the term intuitionistic as known in the context of intuitionistic logic. In this work, will be developed two proposals for interval probability: the restricted interval probability and the unrestricted interval probability, are also introduced two notions of fuzzy probability: the constrained fuzzy probability and the unconstrained fuzzy probability and will eventually be introduced two notions of intuitionistic fuzzy probability: the restricted intuitionistic fuzzy probability and the unrestricted intuitionistic fuzzy probability

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The electric energy is essential to the development of modern society and its increasing demand in recent years, effect from population and economic growth, becomes the companies more interested in the quality and continuity of supply, factors regulated by ANEEL (Agência Nacional de Energia Elétrica). These factors must be attended when a permanent fault occurs in the system, where the defect location that caused the power interruption should be identified quickly, which is not a simple assignment because the current systems complexity. An example of this occurs in multiple terminals transmission lines, which interconnect existing circuits to feed the demand. These transmission lines have been adopted as a feasible solution to suply loads of magnitudes that do not justify economically the construction of new substations. This paper presents a fault location algorithm for multiple terminals transmission lines - two and three terminals. The location method is based on the use of voltage and current fundamental phasors, as well as the representation of the line through its series impedance. The wavelet transform is an effective mathematical tool in signals analysis with discontinuities and, therefore, is used to synchronize voltage and current data. The Fourier transform is another tool used in this work for extract voltage and current fundamental phasors. Tests to validate the location algorithm applicability used data from faulty signals simulated in ATP (Alternative Transients Program) as well as real data obtained from oscillographic recorders installed on CHESF s lines.

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In general, the designs of equipment takes into account the effects and processes of deterioration it will undergo and arrives at an approximate useful life. However, changes in operational processes and parameters, the action of external agents, the kind of maintenance conducted, the means of monitoring, and natural and accidental occurrences completely modify the desired performance of the equipment. The discontinuities that occur in anisotropic materials often and due to different factors evolve from being subcritical to critical acquiring the status of defect and compromising the physical integrity of the equipment. Increasingly sophisticated technological means of detection, monitoring and assessment of these discontinuities are required to respond ever more rapidly to the requirements of industry. This paper therefore presents a VPS (Virtual Pipe System) computational tool which uses the results of ultrasonic tests on equipment, plotting the discontinuities found in models created in the CAD and CAE systems, and then simulates the behavior of these defects in the structure to give an instantaneous view of the final behavior. This paper also presents an alternative method of conventional ultrasonic testing which correlates the integrity of an overlay (carbon steel and stainless steel attached by welding) and the reflection of ultrasonic waves coming from the interface between the two metals, thus making it possible to identify cracks in the casing and a shift of the overlay

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This work presents contributions in the detection and identication of faults in multilevel inverters through the study of the converters behavior under these operation conditions. Basically, the approached fault consists of an open-circuit in any switch of a three-level clamped diode inverter. The converter operation is characterized in the pre and post-fault states. A wave form behavior analysis of the pole voltage, phase current and dc-bus current is also done, which highlights characteristics that allow the detection of failure and, even, under favorable conditions, the identication of the faulty device. A compensation strategy of the approached fault (open-switch) is also investigated with the purpose of maintaining the driving system operational when a failure occurs. The proposed topology uses SCRs in parallel with the internal switches of the inverter, which allows, in some occasions, the full utilization of the dc-bus

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Coordenação de Aperfeiçoamento de Pessoal de Nível Superior

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High dependability, availability and fault-tolerance are open problems in Service-Oriented Architecture (SOA). The possibility of generating software applications by integrating services from heterogeneous domains, in a reliable way, makes worthwhile to face the challenges inherent to this paradigm. In order to ensure quality in service compositions, some research efforts propose the adoption of verification techniques to identify and correct errors. In this context, exception handling is a powerful mechanism to increase SOA quality. Several research works are concerned with mechanisms for exception propagation on web services, implemented in many languages and frameworks. However, to the extent of our knowledge, no works found evaluates these mechanisms in SOA with regard to the .NET framework. The main contribution of this paper is to evaluate and to propose exception propagation mechanisms in SOA to applications developed within the .NET framework. In this direction, this work: (i)extends a previous study, showing the need to propose a solution to the exception propagation in SOA to applications developed in .NET, and (ii) show a solution, based in model obtained from the results found in (i) and that will be applied in real cases through of faults injections and AOP techniques.

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Surface defects on steel parts borne costs of smelting industries due to the need of rework. Sand molds are frequently used in foundry industries and largely responsible for providing surface defects. This study aims to optimize the levels of the molding process variables to minimize the occurrence of surface defects in steel castings in silica sand molds chemically linked by cold cure process. The methodology used the experimental design with split plot, being considered in the study the resin percentage factors in the mold formulation, addition of iron oxide, type of paint, the paint application method, amount of ink layers, use of hot air along the lines and waiting time of the mold before casting. They were analyzed as response variables erosion defects, sand inclusion, penetration, porosity and surface finish. Tensile strength tests were performed to evaluate the influence of factors on mechanical parameters and the microstructural parameters were carried out the analysis of X-ray diffraction, scanning electron microscopy (SEM) and thermal analysis (TG / DSC / dilatometry). The results elucidate that for the faulty erosion, the only significant factor with a 95% confidence level was the type of ink and the ink alumina-based superior results obtained. For the sand inclusion of defect, there were three significant factors, with best results obtained with alumina-based paint and spray applied using hot air in the mold before casting the metal. For the defect penetration, there were four significant factors, the best results being achieved with 0.8% of resin and addition of iron oxide in the molding formulation, the paint being applied by brush and standby time of 24 hours before leak. For the defect porosity with a 95% confidence level, no significant factors. For the defect surface finish, the best results were achieved with the 0.8% formulation of the resin in the mold and application of the paint brush. To obtain the levels of the factors that optimize all defects simultaneously, we performed a weighted average of the results of each type of fault, concluding that the best levels of the factors were: 0.8% resin and addition of iron oxide in the formulation of the template, application of two coats of paint applied with a brush or spray, using hot air in the mold before casting and 24 hours of waiting ready the mold before casting. These levels of the optimized factors were used in an experiment to confirm that ratified the results, helping to reduce rework and consequently reducing costs of cast steel parts.

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Objective: To evaluate the planning and quality of plaster models for fabricati on of removable parti al dentures received from three commercial prosthodonti c laboratories located in the city of João Pessoa, PB, Brazil, which perform the casting procedures in their facilities. Methods: Forty 40 plaster models were photographed per laboratory, totalizing 120 models. The evaluation was performed using two questi onnaires, one designed for the dental prosthesis technicians, and another applied by the investigator for the visual evaluati on of the models. Data were analyzed using the SPSS soft ware version 13.0. Results: Ninety-two (76.7%) models did not present planning. In addition, no model presented references of insertion plane or guide pins. Calculati on of the mouth preparati on index (MPI) to evaluate the distribution of the oclusal and cingulum abutments or rests showed that 86 (71.7%) models were classifi ed as poor, 23 (19.2%) models as good and only 11 (9,.2%) models as acceptable. Defects were found in 102 (85%) models. Conclusion: The prosthodontists are not preparing the mouth of their pati ents, neglecti ng the planning of removable partial dentures, and passing this responsibility to the dental prosthesis technicians. In addition, the quality of the models sent to the laboratories was unsatisfactory.

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Objective: To evaluate the planning and quality of plaster models for fabricati on of removable parti al dentures received from three commercial prosthodonti c laboratories located in the city of João Pessoa, PB, Brazil, which perform the casting procedures in their facilities. Methods: Forty 40 plaster models were photographed per laboratory, totalizing 120 models. The evaluation was performed using two questi onnaires, one designed for the dental prosthesis technicians, and another applied by the investigator for the visual evaluati on of the models. Data were analyzed using the SPSS soft ware version 13.0. Results: Ninety-two (76.7%) models did not present planning. In addition, no model presented references of insertion plane or guide pins. Calculati on of the mouth preparati on index (MPI) to evaluate the distribution of the oclusal and cingulum abutments or rests showed that 86 (71.7%) models were classifi ed as poor, 23 (19.2%) models as good and only 11 (9,.2%) models as acceptable. Defects were found in 102 (85%) models. Conclusion: The prosthodontists are not preparing the mouth of their pati ents, neglecti ng the planning of removable partial dentures, and passing this responsibility to the dental prosthesis technicians. In addition, the quality of the models sent to the laboratories was unsatisfactory.