GORLIN-GOLTZ SYNDROME: A CASE REPORT

Introdução: O Síndrome de Gorlin-Goltz é uma condição hereditária autossómica dominante rara caracterizada por: carcinomas basocelulares, queratoquistos odontogénicos, depressões palmo-plantares, calcificações da foice cerebral e malformações esqueléticas. Associa-se também a meduloblastoma e outras neoplasias. Caso Clínico: Adolescente de 11 anos, com antecedentes de macrocefalia. Referenciada à consulta de estomatologia por quistos mandibulares recidivantes. O exame anátomo-patológico revelou tratarem-se de queratoquistos odontogénicos, pelo que é encaminhada para a consulta de pediatria. Objetivamente, apresentava macrocefalia e facies “gros seiro” com bossas frontais e hipertelorismo. Efetuou estudo genético, que detetou mutação do gene PTCH1, confirmando a suspeita clínica. Discussão: Os queratoquistos odontogénicos são o achado mais representativo do síndrome de Gorlin-Goltz nas duas primeiras décadas de vida, sendo de extrema importância um elevado índice de suspeição por parte do pediatra, com especial relevância para as alterações minor. Após o estabelecimento do diagnóstico, é necessária uma equipa multidisciplinar para um adequado seguimento e tratamento atempado.Introduction: The Gorlin-Goltz syndrome is a rare autosomal dominant inherited condition characterized by: basal cell carcinomas, odontogenic keratocysts, palmar or plantar pits, calcification of the falx cerebri and skeletal malformations. Medulloblastoma and other tumors are also associated with it. Case report: An 11-year-old female, with a history of macrocephaly, was referred to the Stomatology Department for recurrent mandibular cyst. Histopathological exam revealed odontogenic keratocysts, so the patient was referred to the Pediatric Department. Physical examination revealed macrocephaly and coarse face, with frontal bossing and hy pertelorism. The genetic study performed detected a mutation in PTCH1 gene, confirming the clinical suspicion. Discussion: Odontogenic keratocysts are the most representative finding in Gorlin-Goltz Syndrome in the first two decades of life, therefore a high suspicion level is mandatory among pediatricians concerning detection of minor changes. After the diagnosis, a multidisciplinary team is required for adequate follow-up and timely treatment

Fractional electrical dynamics in fruits and vegetables

Fractional calculus (FC) was originally developed in a pure mathematical viewpoint. However, nowadays FC is applied in many emerging fields of physics and engineering. This paper studies the fractional electrical impedance of vegetables and fruits having FC modelling in mind. In this line of thought, are developed several experiments for measuring the impedance of botanical elements, based in the Bode and polar diagrams. An electrical circuit that models these systems is presented and conclusions are drawn.N/

Application of Genetic Algorithms to the Implementation of Fractional Electromagnetic Potentials

A genetic algorithm (GA) is a search technique based on the natural selection process. The GAs provide further flexibility and robustness that are unique for signal process. Recently, a closer look of some phenomena present in electrical systems, such as motors, transformers and lines, and the motivation towards the development of comprehensive models, seem to point out the requirement for a fractional calculus approach. Bearing these ideas in mind, we address the analysis and the synthesis of fractional-order multipoles, based in a GA optimization scheme.N/

Fractional dynamics and control of heat diffusion systems

In this paper we study a heat diffusion system on a fractional calculus perspective. Bearing theses ideas in mind, several fractional PID tuning methodologies are investigated and compared. The simulations demonstrate the good performance of the proposed fractional-order algorithm.N/

Fractional order electrical impedance of fruits and vegetables

The idea of fractional calculus is not new. Fractional derivatives are almost as old as integer-order definition. In 1695 Leibniz discussed this problem with L’Hospital, but many other contributions are due to investigators such as Liouville, Abel, Heaviside and Riemann, that formalized the theory of the non-integer order systems. The area of fractional calculus has primarily been the domain of mathematicians, and only had the theoretical foundation. Nowadays, this concept is employed in physics, engineering, biology, economy and other scientific fields. In our work, we apply the concepts of fractional calculus and the theory of electrical impedance to botanical elements. The fractional order behaviour of these type of systems are studied and the relation with the electrical impedance is formulated.N/

Fractional Dynamics and Control of Distributed Parameter Systems

Fractional Calculus (FC) goes back to the beginning of the theory of differential calculus. Nevertheless, the application of FC just emerged in the last two decades, due to the progress in the area of chaos that revealed subtle relationships with the FC concepts. In the field of dynamical systems theory some work has been carried out but the proposed models and algorithms are still in a preliminary stage of establishment. Having these ideas in mind, the paper discusses a FC perspective in the study of the dynamics and control of some distributed parameter systems

Perfil tecnológico e qualitativo das unidades produtoras de polpa do Estado de Alagoas.

Com o objetivo de conhecer a situacao atual da producao de polpa do Estado de Alagoas, foi realizado, em agosto de 1997, um diagnostico com os produtores de polpas de frutas congeladas.bitstream/item/33833/1/Pa-045.pd

Prevention of nasal trauma due to CPAP in a preterm newborn: case report

The administration of continuous positive airway pressure through nasal CPAP has been used in the treatment of neonatal respiratory diseases as a means of providing ventilatory support to preterm infants, producing adequate gas exchange, decreasing the complications induced by invasive mechanical ventilation and reducing bronchopulmonary dysplasia. The nasal prong is the most common device used to connect to the airways with the purpose of providing mechanical ventilation. Nasal prongs are available in different sizes and made of lightweight and flexible material. Despite its advantages, this device can cause trauma to the nose causing nasal discomfort and disfigurement. We report the case of an extremely low birth weight preterm who used a system of nasal CPAP developed by the nurses of the Neonatal Intensive Care Unit of the HCPA to protect the skin, mucosa, and nasal septum. Protection was successful

Metodologia de desenvolvimento do software Sispaf.

Introdução; Ferramentas de desenvolvimento; Apache Web Server; O que é um servidor Web; O servidor apache; Instalação; Personal home Page (PHP); O que é PHP; Instalação do PHP; Configuração dos arquivos apache e PHP; Apache; PHP; Testando a instalação do apache e PHP; Testando a instalação; MYSQL; O que é MYSQL; Instalando o MySQL; MySQL-Front; Considerações finais.bitstream/item/37205/1/Doc147.pd

Melting of polymer blends in single-screw extrusion : an experimental study

Melting is a major step in plasticating single screw extrusion, but most of the existing phenomenological know how was gathered by performing Maddock-type experiments with homopolymers. Given the current widespread industrial use of polymer blends, it is worth determining whether the same mechanisms and mathematical models apply, or whether different sequences develop. This work reports the results of Maddock-type experiments using a PA6/PP blend, both in its immiscible and compatibilized varieties. A melting mechanism combining the features of the classical Tadmor mechanism and of the dispersed melting mechanism, also previously reported in the literature, was observed.The authors are grateful to Portuguese Fundacao para a Ciencia e Tecnologia for supporting this work under grant SFRH/BD/19997/2004 and to DSM, the Netherlands, for supplying PA6