Anemia de células falciformes na idade pediátrica : contribuição para o estudo das suas principais complicações e indicadores de prognóstico

RESMO: Introdução: A anemia de células falciformes doença hereditária, com repercussão multi-orgânica, tem grande variabilidade na sua expressão clínica. Daí o interesse do estudo de indicadores de prognóstico. A investigação realizada foi precedida de um resumo histórico incidindo sobre a compreensão de aspectos fundamentais da doença ao longo dos tempos. Na primeira parte do estudo e após revisão bibliográfica, foram referidos dados da fisiopatologia como base para os estudos que integram a presente dissertação. Abordou-se o estado da arte relativamente às complicações, aos indicadores de prognóstico e à terapêutica utilizada. Objectivos: Constituíram objectivos deste estudo realizado numa amostra populacional representativa: identificar as lesões a nível dos sistemas cardio-respiratório e nervoso central, avaliando-se as respectivas repercussões; avaliar a presença de indicadores de prognóstico entre as variáveis seleccionadas; estudar a eficácia e toxicidade da HU nos doentes com as formas graves da ACF. Para a prossecução destes objectivos foram delineados para além do estudo global três estudos específicos: Estudo 1- repercussão no sistema cardio-respiratório; Estudo 2- repercussão no sistema nervoso central; Estudo 3- terapêutica com hidroxiureia. Doentes e métodos: Procedeu-se a um estudo prospectivo e multi-institucional durante um período de três anos tendo-se seleccionado para a amostra, e de acordo com critérios pré-definidos, 30 doentes com ACF na fase estável da doença, com idades compreendidas entre os sete e os 18 anos, todos de origem africana à excepção de um caucasiano. O diagnóstico baseou-se em técnicas de electroforese e estudo molecular que definiu o genotipo da doença e a presença da delecção da -talassémia assim como os haplotipos da amostra populacional. Foram utilizadas diferentes metodologias para avaliar a existência de lesão pulmonar e cerebral. Através do estudo estatístico foram seleccionadas diversas variáveis como hipotéticos indicadores de prognóstico. Estudo 1. Para determinar a existência de lesão a nível pulmonar usaram-se duas metodologias diferentes, a avaliação da função pulmonar com estudo da saturação da Hb em O2 no sangue arterial e a tomografia computadorizada de alta resolução. Estudou-se também a possível disfunção cardíaca como repercussão da lesão pulmonar, através do ecocardiograma, e os indicadores de prognóstico com significado estatístico para a lesão encontrada. Estudo 2. O desenho deste estudo foi sobreponível ao anterior, mas com metodologia adequada para o SNC. Procedeu-se ao estudo das lesões cerebrais por meio de exames imagiológicos, (RMN-CE e DTC) e de testes psicológicos. Correlacionaram-se as três metodologias utilizadas e a importância de cada uma para a decisão de atitudes terapêuticas preventivas. Estudo 3. Consistiu num estudo aberto prospectivo não controlado com nove crianças e adolescentes com formas graves de ACF, com o objectivo de avaliar a eficácia da terapêutica com hidroxiureia, durante um período de 24 meses. Todos os doentes completaram no mínimo 15 meses de terapêutica, com uma dose final média de 194 mg/K/dia. Resultados globais: Durante o período anterior à investigação caracterizou-se a amostra populacional estudada quanto ao fenotipo genético, clínico e hematológico de acordo com os critérios utilizados por outros investigadores. Verificou-se: predomínio do haplotipo Bantu na forma homozigótica em 53% dos doentes; número total de EVO ≥3/ano em 87,5% dos doentes; crises de sequestração em 18,75%; dactilites no primeiro ano de vida em 31,2%; quadro de sépsis grave apenas num doente; crises de hiper-hemólise em 50%; e STA em 59,38% dos doentes. Quanto ao fenotipo hematológico evidenciaram-se como factores de risco reticulocitose (13,1x103/l) e hiperbilirrubinémia (2,5 mg/dl) e como factores de bom prognóstico a presença de delecção de um gene da -talassémia em 46,9% dos doentes e valor médio de Hb 8,1 g/dl. Resultados dos estudos parcelares: Estudo 1. Deste estudo infere-se que a DPR ligeira foi diagnosticada em 70% dos doentes, uma vez que as alterações da difusão não foram estatisticamente significativas, o estudo dos gases no sangue não evidenciaram resultados anormais e a TCAR evidenciou alterações em 43,3% dos doentes. Apenas num doente se verificou doença pulmonar obstrutiva relacionada com maior número da STA.O estudo da disfunção cardíaca encontrada em 86,7% dos doentes não reflecte a repercussão da DPR a nível cardíaco, podendo estar associada às alterações fisiopatológicas da própria anemia crónica. Encontraram-se indicadores de prognóstico hematológicos e clínicos. Entre os primeiros, valores de Hb ≥8,5 g/dl e de HbF ≥13% foram considerados indicadores de bom prognóstico para a lesão pulmonar. Em relação aos parâmetros clínicos, as STA não foram consideradas indicadoras de prognóstico para a DPR ao contrário do que se verificou com o número de EVO. Pela análise dos parâmetros genéticos e socio-económicos provou-se a ausência de relação estatisticamente significativa com lesão pulmonar. Estudo 2. Pela RMN-CE foram diagnosticados ES em 33,3% com uma localização preferencial na substância branca profunda em 26,6% dos doentes. Relativamente aos parâmetros hematológicos seleccionados, o valor médio da HbF 8,6% constituíu um indicador de bom prognóstico para o aparecimento de ES, enquanto o valor médio de leucócitos 12.39x103/μl foi considerado um indicador de mau prognóstico. No estudo do DTC apenas um doente apresentou aumento da velocidade do fluxo cerebral na ACM igual a 196 cm/segundos, associado a vasculopatia grave. Os testes psicológicos alterados em 80% dos doentes mostraram ser o método mais sensível para detectar alterações do neurodesenvolvimento, mas sem correlação com os ES em 10% dos doentes. Realça-se a baixa percentagem de DTC patológicos encontrados neste estudo em relação ao número elevado de ES e de testes psicológicos alterados, não se verificando concordância entre os três exames. Dos indicadores de prognóstico estudados a -talassémia foi considerada um factor de protecção para o coeficiente de inteligência da escala de Wechsler. Em relação a parâmetros clínicos estudados os doentes com maior número de EVO, tem em média valores inferiores nos testes psicológicos. Estudo 3. Neste estudo verificou-se que o valor médio da HbF aumentou significativamente de 7,0±4% para 13,7±5,3% (p=0,028) ao fim de 15 meses de terapêutica com hidroxiureia. Clinicamente todos os doentes responderam significativamente com uma redução de 80% no número de EVO, 69% no número de internamentos, 76% no número de dias de hospitalização e 67% no número de transfusões. Deste modo comprovou-se não só a eficácia desta terapêutica neste grupo pediátrico como também a falta de efeitos secundários significativos. Considera-se a necessidade de estudos mais prolongados e em grande séries, para com segurança se usar a HU antes que a lesão orgânica se estabeleça, portanto logo nos primeiros anos de vida. Conclusão: Na amostra populacional estudada foram evidenciadas lesões pulmonares e cerebrais na grande maioria dos doentes que condicionaram a sua qualidade de vida. Foram identificados indicadores de prognóstico que poderão eventualmente ditar medidas terapêuticas precoces com o objectivo de diminuir a morbilidade e a mortalidade neste grupo etário. Demonstrou-se que a terapêutica com a HU foi eficaz e bem tolerada----------ABSTRACT: Background: Sickle cell anemia (SCA), a hereditary disease characterized by pain and lifetime multi-organic lesion, is a challenge for all that work with carriers of this disease. The clinical expression variability of SCA is a constant reality and a problem to be solved in the current world of investigation, for which the knowledge of prognostic indicators responsible for the different aspects of clinical evolution diversity wiil be an added value. The study is preceded by a historical summary of the most important factors in the evolution of SCA, which are in themselves, an incentive for future research. In the first part of the study, after an extensive bibliographical revision, physiopathology data is referred to in general and specifically regarding the target organs, that constituted the base for the studies presented in the dissertation. The state of the art for the complications to be studied, the choice of prognostic indicators and the therapeutics application, were approached for the renewed interest in the theme. Aims: In regard to the investigation, the objective was to study the lesions in the most affected organs of a chosen pediatric group, to investigate prognostic indicators for lung and cerebral lesions and to evaluate the protective effect of hydroxyurea in children with severe outcomes. Patients and methods: A prospective and multi-institutional study was carried out during a three-year period, February 1998 to March 2001, with children and adolescents followed up at a Immunohematology Outpatient Clinic of Dona Estefânia's Hospital, Lisbon. Based in predefined criteria, 30 children with SCA were selected in a stable phase of the disease, aged from seven to 18 years old, all of whom were of African origin with exception of one who was Caucasian. The diagnosis was based on electrophoresis techniques and molecular study that allowed to define the genotype, the presence of deletional alpha-thalassemia as well as haplotypes in the population. Different methodologies were used to evaluate the existence of lung and cerebral lesion. Statistical study of the different variables selected the prognostic indicators. In Study 1, to determine the existence of lung lesion two different methodologies were used: pulmonar function study with arterial blood gases determination; and high resolution computerized tomography. Heart dysfunction as a repercussion of lung lesion was also studied through echocardiography, and prognostic indicators were statistically significant for lesions found. The design of Study 2 was similar to Study 1, but with the appropriate methodology for CNS. After neurological examination, which was normal in all patients (control group), cerebral lesions were studied with imagiologic exams (MRN-CE and TCD) and psychological tests. These three methodologies were correlated and the importance of each one in the decision of the therapeutic profilactic attitudes. Study 3 consisted of a controlled prospective open study in children with severe forms of SCA, with the aim of the evaluating therapeutic effectiveness of hydroxyurea, during a period of 24 months. Results: In the global overall study preceding the Studies 1,2 and 3, there were a prevalence of haplotype Bantu (53%) and other risk factors, namely the number of VOC (87,5%), sequestration crisis (18,75%), dactilytis in first year of life(31,2%), hyperhemolysis crisis (50%) and ATC in more than half of the patients (59,38%). This group of bad prognostic indicators, associated with the population of the lower class according to the Graffar scale, demonstrates the importance of primary health care services, information provided to the children and their relatives, as well as the interest in prophylactic therapeutics, specific screening and prenatal diagnosis. Study 1. It was evident from this study that slight RPD was diagnosed in 70% of the patients, because alterations of the diffusion had no statistical significance and arterial blood gases determinations were normal. Only one patient had restrictive lung disease related with numerous ACS. However ACS was not considered a prognostic indicator for RPD, contrary to the number of EVO. HRTC revealed discreet fibrotic lines that could be related with slight RPD, but the lack of correlation of these two exams (33%) supports the value of lung function tests for precocious diagnosis of RPD. Heart dysfunction was found in 86,7% of patients, does not reflect the repercussion of RPD, but with the physiopathology of chronic anemia. Hematologic and clinical prognostic indicators were found. Good prognostic indicators for the non-evolution of RPD with average Hb values of ≥ 8,5 g/dl and average HbF values of ≥13%, respectively. The genetic and social-economic factors had no statistical significance; nevertheless, they were more prevalent among Bantu haplotype (53,3%) in patients with RPD. Study 2. RMN-CE detected SI in 33,3% of the patients, with preferential location in deep white substance in 26,6% and in front lobe in 20%. This distribution can be related to structural aspects of the brain and with the high sensibility of this organ to hypoxia. From the hematological parameters selected, average HbF value 8,6% and average leucocyte count 12.39x103/μl were prognostic indicators with different meaning to SI. The increase in the total bilirubin related to hyperhemolysis clinically explains the genesis of SI In the TCD study, only one patient had increased cerebral flow speed >196 cm/sec in CMA, which corresponded to serious vasculopathy in AngioMR. This patient never present previously neurological symptoms and had several hyperhemolysis crisis and VOC as risk factors. Low percentage of pathological TCD in this study, in relation to the high number of SI and altered tests, although without correlation among the three exams, is probably attributed to factors related to the methodology, aspects of cerebral physiopathology or perhaps a sign of good prognostic if the duration of study had not been so short. TCD should be used as a screening method in the age groups with higher risk of AVC and should never be considered separately in prophylactic therapeutics indication. Psychological tests were the most sensitive method to detect neurodevelopment impairment; in 80% of patients the neuropsychologics tests were altered, but without correlation with SI (10%). Since SI can become evident during the first two years of life and develop with time, the first psychological tests should be carried out between 3 and 5 years of age to timely be referred to special education and stimulation programs. Prognostic indicators to psychological tests were also found: alpha-thalassemia was found to be a protection factor of the IQ, just as other hematologic factors (hematocrit, MGCV and erythrocytes count). In relation to clinical parameters, although without statistical significance, patients with larger number of VOC had average lower scores versus the average in tests, except in TP. Results from different studies were conclusive as to the type of lesion found and the importance of prognostic indicators. Study 3. All the patients completed a minimum of 15 months therapeutic treatment with the final average daily dose of 19±4 mg/kg/day. The average value of the fetal hemoglobin increased significantly from 7,0±3,9% to 13,7±5,3% (p=0.028). The HbF average values increased from 6% to 15% after 15 months of therapeutic treatment. Clinically there was a reduction of 80% in the number of VOE , 69% in the number of hospitalization, 76% in the number of days of hospitalization and 67% in the number of transfusions. Once again the effectiveness of this treatment in this pediatric group, as well as the lack of any significant secondary effects, was evident. The study confirms the need for further detailed research in order to safely effect the appropriate treatment prior to the development of organic lesions, which ideally should be in the first year of life. Conclusions: These results allow us to clarify the importance of either pulmonary lesions or either nervous central system impairment among patients, children and adolescents, with sickle cell anemia. These lesions were demonstrated in most of the patients studied compromising their quality of life and the mortality. The treatment with HU is proved to be effective and having low toxicity.

Consumers Performance Evaluation of the Participation in Demand Response Programs Using Baseline Methods

Demand response has gain increasing importance in the context of competitive electricity markets environment. The use of demand resources is also advantageous in the context of smart grid operation. In addition to the need of new business models for integrating demand response, adequate methods are necessary for an accurate determination of the consumers’ performance evaluation after the participation in a demand response event. The present paper makes a comparison between some of the existing baseline methods related to the consumers’ performance evaluation, comparing the results obtained with these methods and also with a method proposed by the authors of the paper. A case study demonstrates the application of the referred methods to real consumption data belonging to a consumer connected to a distribution network.

Determining the Adjustment Baseline Parameters to Define an Accurate Customer Baseline Load

Demand response is an energy resource that has gained increasing importance in the context of competitive electricity markets and of smart grids. New business models and methods designed to integrate demand response in electricity markets and of smart grids have been published, reporting the need of additional work in this field. In order to adequately remunerate the participation of the consumers in demand response programs, improved consumers’ performance evaluation methods are needed. The methodology proposed in the present paper determines the characterization of the baseline approach that better fits the consumer historic consumption, in order to determine the expected consumption in absent of participation in a demand response event and then determine the actual consumption reduction. The defined baseline can then be used to better determine the remuneration of the consumer. The paper includes a case study with real data to illustrate the application of the proposed methodology.

Real Time Pricing Approaches to Deal With Unexpected Wind Power Variations

The use of renewables have been increased I several countries around the world, namely in Europe. The wind power is generally the larger renewable resource with very specific characteristics in what concerns its variability and the inherent impacts in the power systems and electricity markets operation. This paper focuses on the Portuguese context of renewables use, including wind power. The work here presented includes the use of a real time pricing methodology developed by the authors aiming the reduction of electricity consumption in the moments of unexpected low wind power. A more specific example of application of real time pricing is demonstrated for the minimization of the operation costs in a distribution network. When facing lower wind power generation than expected from day ahead forecast, demand response is used in order to minimize the impacts of such wind availability change. In this way, consumers actively participate in regulation up and spinning reserve ancillary services through demand response programs.

Incentive-based demand response programs designed by asset-light retailers for day-ahead market

Following the deregulation experience of retail electricity markets in most countries, the majority of the new entrants of the liberalized retail market were pure REP (retail electricity providers). These entities were subject to financial risks because of the unexpected price variations, price spikes, volatile loads and the potential for market power exertion by GENCO (generation companies). A REP can manage the market risks by employing the DR (demand response) programs and using its' generation and storage assets at the distribution network to serve the customers. The proposed model suggests how a REP with light physical assets, such as DG (distributed generation) units and ESS (energy storage systems), can survive in a competitive retail market. The paper discusses the effective risk management strategies for the REPs to deal with the uncertainties of the DAM (day-ahead market) and how to hedge the financial losses in the market. A two-stage stochastic programming problem is formulated. It aims to establish the financial incentive-based DR programs and the optimal dispatch of the DG units and ESSs. The uncertainty of the forecasted day-ahead load demand and electricity price is also taken into account with a scenario-based approach. The principal advantage of this model for REPs is reducing the risk of financial losses in DAMs, and the main benefit for the whole system is market power mitigation by virtually increasing the price elasticity of demand and reducing the peak demand.

Demand Response Programs Definition Using Demand Price Elasticity to Define Consumers Aggregation for an Improved Remuneration Structure

The positioning of the consumers in the power systems operation has been changed in the recent years, namely due to the implementation of competitive electricity markets. Demand response is an opportunity for the consumers’ participation in electricity markets. Smart grids can give an important support for the integration of demand response. The methodology proposed in the present paper aims to create an improved demand response program definition and remuneration scheme for aggregated resources. The consumers are aggregated in a certain number of clusters, each one corresponding to a distinct demand response program, according to the economic impact of the resulting remuneration tariff. The knowledge about the consumers is obtained from its demand price elasticity values. The illustrative case study included in the paper is based on a 218 consumers’ scenario.

Intelligent decision support systems for collaboration in industrial plants

Dissertação apresentada para obtenção do Grau de Doutor em Sistemas de Informação Industriais, Engenharia Electrotécnica, pela Universidade Nova de Lisboa, Faculdade de Ciências e Tecnologia

Optimization of Generation and Aggregated Consumption Shifting for Demand Response Programs Definition

The concept of demand response has drawing attention to the active participation in the economic operation of power systems, namely in the context of recent electricity markets and smart grid models and implementations. In these competitive contexts, aggregators are necessary in order to make possible the participation of small size consumers and generation units. The methodology proposed in the present paper aims to address the demand shifting between periods, considering multi-period demand response events. The focus is given to the impact in the subsequent periods. A Virtual Power Player operates the network, aggregating the available resources, and minimizing the operation costs. The illustrative case study included is based on a scenario of 218 consumers including generation sources.

Clay body wrap with microcurrent: Effects in central adiposity

Introduction: Increased fat mass is becoming more prevalent in women and its accumulation in the abdominal region can lead to numerous health risks such as diabetes mellitus. The clay body wrap using compounds such as green clay, green tea and magnesium sulfate, in addition to microcurrent, may reduce abdominal fat mass and minimize or prevent numerous health problems. Objective: This study aims at measuring the influence of the clay body wrap with microcurrent and aerobic exercise on abdominal fat. Methods: Nineteen female patients, randomized into intervention (n = 10) and control (n = 9) groups, were evaluated using ultrasound for visceral and subcutaneous abdominal fat, calipers and abdominal region perimeter for subcutaneous fat and bioimpedance for weight, fat mass percentage and muscular mass. During 10 sessions (5 weeks, twice a week) both groups performed aerobic exercise in a cycloergometer and a clay body wrap with microcurrent was applied to the intervention group. Results: When comparing both groups after 5 weeks of protocol, there was a significant decrease in the subcutane- ous fat around left anterior superior iliac spine in the intervention group (ρ = 0.026 for a confidence interval 95%). When comparing initial and final abdominal fat in the intervention group, measured by ultrasound (subcutaneous and visceral fat) and by skinfold (subcutaneous fat), we detected a significant abdominal fat reduction. Conclusion: This study demonstrated that the clay body wrap used with microcurrent and aerobic exercise can have a positive effect on central fat reduction.

Eficiência energética na iluminação pública

O presente trabalho aborda a temática da eficiência energética em sistemas de iluminação pública. A principal motivação prende-se com o peso significativo que a parcela energética destes sistemas ocupa na economia mundial. O uso eficiente de energia é uma crescente preocupação devido à diminuição de recursos, às consequências climáticas cada vez mais marcadas e ao elevado custo da energia, representando ainda um papel fundamental ao nível económico e de competitividade. A Iluminação Pública (IP) representa um peso importante nas despesas correntes dos municípios. É assim importante encontrar uma solução que permita manter níveis de segurança e conforto necessários às populações e que proporcione uma redução substancial do peso da IP nas despesas municipais. Neste sentido, este trabalho propõe-se estudar esta problemática, apresentando uma sistematização de soluções eficientes, quer a nível de lâmpadas e luminárias como também ao nível de tecnologias que auxiliem e complementem a eficiência de uma instalação de iluminação pública. A dissertação está dividida em duas partes. A primeira parte sistematiza os consumos verificados em Portugal, a vários níveis (consumo de energia elétrica, evolução do consumo energético de iluminação pública, etc.) abordando as políticas de eficiência energética, e são descritos alguns procedimentos que possibilitam a poupança energética na iluminação pública, aliada a instalações eficientes. A segunda parte da dissertação contempla o estudo de um caso prático cujo objetivo é propor soluções técnicas que permitam melhorar a eficiência energética na iluminação pública de Esposende, face à situação atual do concelho. Serão propostas várias soluções, tais como luminárias LED, balastros electrónicos reguláveis, lâmpadas de menor consumo e até mesmo o uso da telegestão.

COMPARISON OF A COMMERCIAL ENZYME IMMUNOASSAY WITH PLAQUE REDUCTION NEUTRALIZATION FOR MATERNAL AND INFANT MEASLES ANTIBODY MEASUREMENT

The most practicable assay for measurement of measles IgG (mIgG) in large numbers of sera is an enzyme immunoassay (EIA). To assess how EIA results would agree with those by the gold standard method of plaque reduction neutralization (PRN) we compared the results from the two methods in 43 pairs of maternal and umbilical cord sera, and sera from the corresponding infants when aged 11 - 14 months. In maternal-cord sera, the differences between mean antibody levels by EIA or PRN were not statistically significant, though in individual sera, differences could be large. However, agreement was less good for infants sera, in which levels of mIgG were very low. The conclusions of a study of transplacental transport of mIgG would not be affected by the use of either technique. When studying waning immunity in infants, PRN should be the method of choice, while results from studies using EIA should be interpreted with caution.

Paralisia cerebral na Região de Lisboa e Vale do Tejo : factores de risco : caracterização multidimensional : inclusão escolar

RESUMO - A Paralisia Cerebral (PC) deve ser olhada como uma patologia do neurodesenvolvimento: a infância é um período de actividade exploratória por essência, a restrição motora condiciona as várias áreas do desenvolvimento. Contextos, apoios, oportunidades e experiências de vida serão determinantes no desenvolvimento de todo o seu potencial. Objectivos/finalidade: Identificar, descrever, comparar e analisar factores de risco associados à PC, sua caracterização multidimensional e integração escolar aos 5 e 10 anos. Procurou‐se contribuir para a sua prevenção primária e secundária, e obter dados para planeamento e implementação dos programas de apoio. Métodos: Adoptou‐se a abordagem do Programa Nacional de Vigilância da Paralisia Cerebral (PNVPC) e da Surveillance of Cerebral Palsy in Europe (SCPE). Analisaram‐se factores de risco, competências funcionais, défices associados, severidade e integração escolar de duas populações de Lisboa e Vale do Tejo, (nascimento 1996/1997‐2001/2002 e prevalência aos 5 e 10 anos). Descreveram‐se os dados, efectuaram‐se correlações, aplicaram‐se testes de independência e compararam‐se com dados dos nadovivos, dados nacionais e europeus. Analisaram‐se os factores que influenciaram a integração escolar através de métodos de regressão logística. Resultados/Conclusões/Recomendações: 1,65‰ e 1,57‰ dos nadovivos desenvolveram PC; a prevalência aos 5 anos foi de 1,7‰ e de 1,48‰; 5,9% e 7,9% faleceram antes dos 5 anos. Em 2001/2002 verificou‐se aumento de: PC espástica bilateral‐2/3membros, prematuridade, causa pos‐neonatal, níveis funcionais ligeiros e graves; percentil estaturo‐ponderal <3 (5‐anos). Diminuição de: disquinésia, anóxia e alguns défices associados. Destacaram‐se as associação: prematuridade e PC espástica bilateral‐ 2/3membros; nascer de termo e anóxia, disquinésia, primíparas, défices associados e severidade; infecção pré‐natal e QI<50, epilepsia e severidade; causa pos‐neonatal e PC espástica bilateral‐4membros e múltiplos défices. Aos 5 anos, as variáveis explicativas para a não inclusão escolar foram: QI<50 e epilepsia; uma elevada percentagem de crianças com PC moderada/grave encontrava‐se integrada; 75% das que se encontravam desintegradas mantiveram‐se nesta situação aos 10. Nesta idade, as variáveis explicativas para a não inclusão escolar foram: QI<50 e motricidade fina; 35,1% encontrava‐se fora do ensino regular; 4,5%, embora em idade de escolaridade obrigatória, não frequentavam qualquer estabelecimento escolar. Informação sistematizada, abrangente, objectiva, simples e acessível, sobre novos casos de PC, factores de risco, prevalência em idades‐chave e caracterização multidimensional constitui uma ferramenta clínica e epidemiológica, que deve sustentar as políticas de saúde, educacionais e sociais, contribuindo para a permanência destas crianças no ensino regular, trazendo às crianças e famílias o suporte que as encorajem e sustentem nestes processos. ABSTRACT ------- Cerebral Palsy (CP) must be recognized as a neurodevelopmental disorder: childhood is, on its nature, a period for exploring the environment and therefore motor deficit interferes with all developmental areas. The context, support, opportunities and life experiences are determinants for the development of his full potential. Objective/Aim: To identify, describe, compare and analyze CP risk factors the multidimensional characterization and school integration levels at the age of 5 and 10 years. We aim to contribute to CP primary and secondary prevention and provide information for service planning and implementation of support programs. Methods: The approach of National Cerebral Palsy Surveillance Programme (NCPSP) and Surveillance of Cerebral Palsy in Europe (SCPE) were used. For two groups of children from Lisboa e Vale do Tejo region, birth data 1996/1997‐2001/2002 and prevalence at 5 and 10 years, were analyzed: CP risk factors, functional ability, associated impairments, severity and school integration settings. Data collected was described, analyzed using correlations, applied tests of independence and compared with new born data, national data and european data. To analyze the factors related to school inclusive settings, logistic regression was appealed. Results/Conclusions/Recommendations: 1,65% and 1,57‰ of the new‐born alive developed CP. The prevalence at 5 years was 1,7‰ and 1,48‰ 5,9% and 7,9% died before their 5th birthday. Bilateral spastic CP 2/3limb, preterm birth, cases of post‐neonatal origin, mild and sever functional impairment; weight and height percentile <3 at 5 years old Increased in 2001. Decreased dyskinetic CP, anoxia and some additional imparments. Were identified among other the association between prematurity and spastic bilateral CP‐2/3 members; born at term and anoxia, dyskinetic CP, first child, associated impairments and severity; prenatal infection and IQ<50, epilepsy and severity; post‐neonatal cause and spastic bilateral CP‐4 members and associated impairments. At 5‐years‐old the more explanatory variables for not be in a school inclusive settings were IQ<50 and epilepsy, a high percentage of children with moderate/severe CP was attending regular school, but most children who were out of inclusive settings at 5 years continue on this situations at 10‐years‐old. At this age the more explanatory variables for not be in a school inclusive settings were: IQ<50 and upper limb function; 35,1% were out of regular school; 4,5%, even in compulsory school age, are out of school. Standardized comprehensive, objective, simple and accessible information about CP new cases, risk factors, prevalence in the key‐age and children multidimensional characterization constitutes a clinical and epidemiological tool that should sustain health, educational and social policy. This would support the continuity of these children in regular schools, encouraging g them and their families in these processes.

Eficiência Energética no Hospital Pedro Hispano

Os crescentes custos ligados ao consumo elétrico, não apenas de cariz financeiro mas também ambiental, despertam cada vez mais para a importância da definição de estratégias de melhor utilização de recursos e eficiência energética. Esta importância tem sido reforçada pela definição de decretos-lei que vêm colocar metas e limites relativamente às despesas energéticas. Estes diplomas são também acompanhados por programas de incentivo para um setor ligado à eficiência energética. Em Portugal as medidas ligadas ao setor tem vindo a ser redirecionadas para o consumo final de energia, com a definição de metas para as instalações de maior consumo. As instalações hospitalares são grandes centros de consumo energético devido não só ao elevado número de utentes que recebem mas também pelos diversos tipos de equipamentos elétricos usados para a prestação dos serviços médicos. Como consequência disso, os investimentos e os custos operacionais são elevados, o que reforça a necessidade de gerir os gastos e consumos energéticos com a procura constante de melhoria na recolha de informação sobre todo o sistema e na adequação de intervenções com vista a uma maior eficiência energética. O Hospital Pedro Hispano vem desde algum tempo a investir no sentido de conhecer mais e melhor toda a instalação bem como os consumos energéticos a ela associados. Algumas medidas foram tomadas nesse sentido nomeadamente a instalação de analisadores de energia, de modo a obter um retrato mais fiel e fidedigno dos principais vetores de consumo. Neste momento a gestão técnica do hospital tem em análise uma grande parte da instalação recolhendo dados do consumo elétrico real do hospital. Nesta dissertação procurou-se fazer uma análise e enquadramento dos programas e metas ligados ao setor energético com ênfase nos diplomas que visão e abrangem as instalações hospitalares. Dos vários programas de incentivo à adoção de políticas de maior eficiência energética é dado especial destaque ao programa ECO.AP que visa a celebração de contratos para implementação de medidas de poupança energética ao setor público. Em colaboração com o HPH, iniciaram-se os trabalhos pelo estudo e identificação das principais fases e ferramentas utilizadas na gestão energética do edifício tendo como objetivo a reavaliação dos vetores energéticos já identificados no HPH e a criação e contabilização de novos grupos de consumo. Através de várias medições do consumo elétrico, num total superior a 650 horas de funcionamento, foi possível a criação do mapa de desagregação de consumos para o ano de 2013. A desagregação realizada conta com 3 novos vetores energéticos e com a reavaliação do peso relativo de mais 5 grupos de consumo. Das medições efetuadas destaca-se a reavaliação do consumo da central de bombagem onde a parcela considerada até à data estava 3 vezes acima do valor real medido. Com base na desagregação feita foram apontadas e estudadas medidas de implementação com o objetivo de reduzir os consumos energético em todo o hospital, destacando-se a solução apresentada para a central de bombagem. Esta medida traria um grande impacto em toda a fatura energética, não só pela sua viabilidade, mas também porque atuaria num grande centro de consumo onde até ao momento nenhuma ação do género foi implementada.

Strategic Drivers for Implementing Sustainability Programs in Portuguese Organizations– Let’s Listen to Aristotle: From Triple to Quadruple Bottom Line

This article describes a study that investigated the main strategic drivers that influence the implementation of sustainability/social responsibility programs. An online survey was administered to managers of Portuguese organizations with certified management systems. The findings suggest that the implementation of such programs is mainly correlated to: 1.) the approach to understanding and working toward the satisfaction of the community’s needs (in the broad sense of social responsibility); 2.) how systematically sustainability within the organization is identified and managed (e.g., pollution prevention, improved environmental performance, and compliance with the applicable environmental laws); and 3.) the degree to which the organization tries to understand the needs of the employees and works toward satisfying them. In addition to the survey, five interviews with top managers of the surveyed organizations provided some useful insights. There was no consensus on the meaning of sustainability and social responsibility: some described it as an instrumental approach for obtaining better organizational results, while others regarded it as the right thing to do (i.e., it is values driven). In all cases, however, the managers supported a kind of umbrella construct under which different size corporations use different models (for example, the Dow Jones Sustainability Index (DJSI), Global Reporting Initiative (GRI), ISO 14001 environmental management systems), although some managers reported that they simply do not know what to do. All of those surveyed agreed that the lack of a systematic approach could represent a major threat to their organization, making them willing to pay more attention and take more action on the issue of sustainability. An additional suggestion made by managers was to change from a triple bottom line (economic dimension, environmental dimension, social equity dimension) to a quadruple bottom line by adding another dimension: personal and family happiness. This fourth dimension was recognized by the Greek philosopher/thinker Aristotle (384-322 BCE) who thought of happiness as the highest good (virtue) and ultimate goal and purpose of life, achieved through living well, in harmony. Such harmony suggests a balance and a lack of excess—in other words a sustainable existence.

Advantages of the rapid HIV-1 test in occupational accidents with potentially contaminated material among health workers

In occupational accidents involving health professionals handling potentially contaminated material, the decision to start or to continue prophylactic medication against infection by Human Immunodeficiency Virus (HIV) has been based on the ELISA test applied to a blood sample from the source patient. In order to rationalize the prophylactic use of antiretroviral agents, a rapid serologic diagnostic test of HIV infection was tested by the enzymatic immunoabsorption method (SUDS HIV 1+2, MUREX®) and compared to conventional ELISA (Abbott HIV-1/ HIV-2 3rd Generation plus EIA®). A total of 592 cases of occupational accidents were recorded at the University Hospital of Ribeirão Preto from July 1998 to April 1999. Of these, 109 were simultaneously evaluated by the rapid test and by ELISA HIV. The rapid test was positive in three cases and was confirmed by ELISA and in one the result was inconclusive and later found to be negative by ELISA. In the 106 accidents in which the rapid test was negative no prophylactic medication was instituted, with an estimated reduction in costs of US$ 2,889.35. In addition to this advantage, the good correlation of the rapid test with ELISA, the shorter duration of stress and the absence of exposure of the health worker to the adverse effects of antiretroviral agents suggest the adoption of this test in Programs of Attention to Accidents with Potentially Contaminated Material.