Aborto recorrente das causas na mulher ��s consequ��ncias para o casal

RESUMO: O aborto recorrente (AR) �� um evento extremamente traum��tico com grande impacto na vida dos casais. Apesar de avan��os significativos verificados na investiga����o m��dica, cerca de 50% dos casos continua sem uma causa identificada. Alguns aspectos como a caracteriza����o inadequada das doentes e das perdas gestacionais, assim como diferentes metodologias utilizadas no seu estudo, t��m influenciado a preval��ncia de alguns dos factores causais e dificultado a compreens��o do AR. Da mesma forma, pouco se sabe sobre as diferen��as de g��nero na viv��ncia psicol��gica do aborto recorrente e das suas eventuais repercuss��es para o relacionamento do casal, centrando-se os poucos estudos existentes preferencialmente na mulher. Por esta raz��o, o objectivo desta tese foi a caracteriza����o dos factores m��dicos associados ao AR e das consequ��ncias psicol��gicas desta entidade, contribuindo para promover estrat��gias cl��nicas baseadas na evid��ncia espec��fica. Na primeira parte desta tese (cap��tulos 1 e 2), ap��s uma breve introdu����o geral e atrav��s de uma revis��o da literatura, efectua-se uma reflex��o sobre o tema, abordando a epidemiologia do aborto recorrente, os factores m��dicos e os aspectos psicol��gicos associados. Nos cap��tulos 3 e 4 descrevemos tr��s estudos efectuados em mulheres portuguesas com aborto recorrente. O primeiro estudo teve por objectivo caracterizar os factores m��dicos e determinar o padr��o da perda recorrente de gravidez, numa coorte de mulheres submetidas a um protocolo de diagn��stico definido. As participantes foram agrupadas de acordo com a paridade (AR prim��rio ou secund��rio) e a idade gestacional das perdas (embrion��rias ou fetais). As anomalias da cavidade uterina, a SAAF e as transloca����es equilibradas parentais foram os factores mais prevalentes. 15,6% das participantes eram obesas. Em 55,5% dos casos n��o foi identificado nenhum factor. A hist��ria obst��trica materna influenciou significativamente os resultados encontrados: os factores anat��micos e a SAAF foram mais prevalentes em nul��paras e as perdas inexplicadas foram mais frequentes em mulheres com AR secund��rio. Assim, os nossos dados refor��am os resultados de pesquisas anteriores sobre a import��ncia da obesidade, da s��ndrome de anticorpos antifosfol��pidos e das anomalias uterinas estruturais como factores associados ao AR e mostram que os a paridade �� um moderador da import��ncia desses factores. Cap��tulo 6 94 A aus��ncia de resultados consensuais na literatura sobre a etiologia do AR condiciona a pesquisa sistem��tica de alguns factores, envolvendo exames dispendiosos, muitas vezes sem que exista evid��ncia que suporte a sua associa����o com esta entidade. A trombofilia heredit��ria �� uma das condi����es frequentemente investigadas nestas doentes. O nosso segundo estudo pretende contribuir para clarificar o papel de duas muta����es (factor V Leiden e protrombina G20210A) na perda recorrente de gravidez e esclarecer a necessidade do seu rastreio nestas situa����es. Foi efectuada a pesquisa destes polimorfismos em 100 mulheres com AR inexplicado e num grupo de controlo de mult��paras sem hist��ria de perdas de gravidez. Na nossa amostra n��o se verificou uma associa����o entre perdas embrion��rias recorrentes e estas muta����es. Nas mulheres com este tipo de perdas, a preval��ncia do FLV foi inclusive menor do que a verificada nos controlos. Pelo contr��rio, nas participantes com perdas fetais a preval��ncia destes polimorfismos foi muito superior �� verificada nos controlos, sugerindo uma poss��vel associa����o entre estas duas entidades. A pequena dimens��o deste ��ltimo subgrupo de mulheres, n��o nos permitiu contudo tirar conclus��es. Uma investiga����o prospectiva multic��ntrica �� necess��ria antes de recomendar a pesquisa da trombofilia heredit��ria na investiga����o do AR. Procur��mos incluir tamb��m nesta tese uma dimens��o psicol��gica e contribuir assim para o conhecimento dos processos relacionais originados pelo AR. No terceiro estudo foram investigadas as diferen��as de g��nero na viv��ncia do AR e o seu impacto no relacionamento e sexualidade do casal. Participaram neste estudo 30 casais sem filhos, com pelo menos 3 abortos espont��neos consecutivos. Cada membro do casal respondeu a um conjunto de question��rios (Impact of Events Scale, Perinatal Grief Scale, Partnership Questionnaire e Intimate Relationship Scale). Os resultados mostram que as mulheres sofrem mais intensamente do que os homens com o AR, relacionando-se a intensidade do seu sofrimento com a qualidade do relacionamento conjugal. A sexualidade do casal �� tamb��m afectada pelo stress e pelo sofrimento associados ao AR. Uma avalia����o e acompanhamento deste tipo de problemas s��o imprescind��veis para ajudar estes casais a manterem a qualidade afectiva e sexual da sua rela����o. Finalmente, no cap��tulo 5 sumari��mos as conclus��es de toda a contribui����o pessoal para a investiga����o sobre os factores associados e repercuss��es para o casal da perda recorrente de gravidez.-------------------ABSTRACT: Recurrent miscarriage (RM), a rare condition, has been described as a traumatic event for couples. Parental chromosomal anomalies, maternal thrombophilic disorders and structural uterine anomalies have been directly associated with RM. However, despite significant advances in medical research, the vast majority of cases remain unexplained. Aspects as the ethnic diversity of the population with different expression of genes, the inappropriate characterization of patients and of pregnancy losses, as well as different methodologies used in their study, have influenced the prevalence of etiological factors and have hampered the understanding of this problem. Similarly, little is known about gender differences in psychological experience of RM and its implications for the relationship of the couple. The first objective of this thesis is the characterization of the medical factors and of the psychological consequences related with RM, in the Portuguese population, helping to promote specific evidence-based clinical strategies. In the first part of this thesis, and after a brief general introduction (Chapter 1), a critical review of literature on the definition, the epidemiology and the dimensions involved, with a special emphasis on associated medical and psychological aspects of recurrent miscarriage, is presented (Chapter 2). In Chapters 3 and 4 we describe three studies carried out in Portuguese couples with RM. The first study aimed to investigate the etiological factors and the pattern of pregnancy loss in a cohort of women with RM. Subjects were divided in groups according to their parity (primary or secondary RM) and time of pregnancy loss (embryonic or fetal). Parental chromosome anomalies, uterine anomalies and antiphospholipid syndrome were the most prevalent medical factors. 15.6% of the women were obese. In the majority of cases (55.5%) no identifiable cause was detected. Parity influenced significantly our results. There was a higher prevalence of anatomic factors and antiphospholipid syndrome in primary RM. On the other hand, unexplained losses were more frequent in secondary RM. Except for the parental chromosomal abnormalities; the frequency of risk factors was similar among women with fetal or embryonic losses. Our data emphasizes the results of previous research on the importance of obesity, antiphospholipid syndrome and structural uterine abnormalities as known risk factors for RM, and shows that parity is an important moderator of the weight of those risk factors. Our second study aims to clarify the role of two mutations (factor V Leiden and prothrombin G20210A) and elucidate the need for their screening in Portuguese women with RM. FVL and PT G20210A analysis was carried out in 100 women with three or more consecutive miscarriages and a control group of 100 parous controls with no history of pregnancy losses. Secondary analysis was made regarding gestational age at miscarriage (embryonic and fetal loss). Overall the prevalence of FVL and PT G20210A was similar in RM women compared with controls. In the RM embryonic subgroup of women, FVL prevalence was inclusively lower than that of controls. Conversely in women with fetal losses both polymorphisms were much more frequent, although statistical significance was not reached due to the small size of this subgroup of patients. These data indicate that inherited maternal thrombophilia is not associated with RM prior to 10 weeks of gestation. Therefore, its screening is not indicated as an initial approach in Portuguese women with RM and a negative personal history of thromboembolic.96 Our second study aims to clarify the role of two mutations (factor V Leiden and prothrombin G20210A) and elucidate the need for their screening in Portuguese women with RM. FVL and PT G20210A analysis was carried out in 100 women with three or more consecutive miscarriages and a control group of 100 parous controls with no history of pregnancy losses. Secondary analysis was made regarding gestational age at miscarriage (embryonic and fetal loss). Overall the prevalence of FVL and PT G20210A was similar in RM women compared with controls. In the RM embryonic subgroup of women, FVL prevalence was inclusively lower than that of controls. Conversely in women with fetal losses both polymorphisms were much more frequent, although statistical significance was not reached due to the small size of this subgroup of patients. These data indicate that inherited maternal thrombophilia is not associated with RM prior to 10 weeks of gestation. Therefore, its screening is not indicated as an initial approach in Portuguese women with RM and a negative personal history of thromboembolic. In our third study, we investigate gender differences in RM experience and its impact on the couple's relationship and sexuality. Each member of 30 couples with RM answered a set of questionnaires, including the Impact of Events Scale (Horowitz et al., 1979), the Perinatal Grief Scale (Toedter et al., 1988), the Partnership Questionnaire (Hahlweg, 1979) and the Intimate Relationship Scale (Hetherington e Soeken, 1990). Results showed that men do grieve, but less intensely than women. Although the quality of the couple���s relationship seemed not to be adversely affected by RM, both partners described sexual changes after those events. Grief was related to the quality of communication in the couple for women, and to the quality of sex life for men. An understanding of such issues is critical in helping these couples to maintain sexual and affective quality of their relationship. Finally, in Chapter 5, conclusions and clinical implications of all personal contribution to the investigation on associated factors and relational consequences of recurrent miscarriage are presented.

Integrating early aspects with goal-oriented requirements engineering

Disserta����o apresentada na Faculdade de Ci��ncias e Tecnologia da Universidade Nova de Lisboa para obten����o do grau de Mestre em Engenharia Inform��tica

Le retable majeur de la S�� velha de Coimbra et la polychromie dans le dioc��se de Coimbra �� l�����poque baroque: aspects techniques et esth��tiques

Doutoramento em Conserva����o e Restauro, especialidade Teoria, Hist��ria e T��cnicas

Estudo sobre o insight em pessoas com psicose

Para al��m das vari��veis cl��nicas e sociodemogr��ficas existem concerteza importantes componentes individuais que desempenham um contributo importante no n��vel de insight apresentado por cada pessoa doente, por exemplo, o n��vel de intelig��ncia, personalidade, cultura, experi��ncias passadas, mem��ria, etc. A natureza cl��nica, emocional e/ou intelectual do termo ajuda-nos a compreender a complexidade da dificuldade que existe na sua tradu����o e, inclusive, na sua compreens��o. Da�� que as defini����es atribu��das ao conceito sejam muito distintas e variem consoante a forma����o te��rica do autor/investigador. Pretende-se, a partir dessa identifica����o/compreens��o, promover a qualidade de vida destas pessoas atrav��s do desenvolvimento de novas aprendizagens que possibilitem uma coopera����o activa. �� igualmente fundamental ir ao encontro das capacidade intactas de maneira a possibilitar a aquisi����o de novos(s) comportamento(s) que tenham um impacte positivo nas queixas, sinais, sintomas, incapacidade e disfuncionalidade apresentados pelo/a utente. Uma vez que a pr��pria conceptualiza����o do termo traduzir�� aquilo que se pretende avaliar,ser�� efectuada uma reflex��o detalhada acerca dos instrumentos e defini����es que t��m sido mais utilizadas para explorar o insight nas psicoses.Procurei, no meu trabalho de investiga����o, real��ar e promover a import��ncia que cada sujeito, alvo de interven����o, desempenha ao longo do seu processo de recupera����o e na preven����o de reca��das. No seguimento dos objectivos acima descritos, para al��m da revis��o te��rica efectuada ao fen��meno em termos de conceptualiza����o e estudos desenvolvidos na ��rea de investiga����o, foi,neste estudo, realizada a contribui����o para a valida����o do instrumento ���Assessment of Insight in Psychosis: a re-standartization of a New Scale��� de Markov�� & Berrios (2003).O fen��meno de insight escolhido pela Insight Scale, relata menos as mudan��as vividas em rela����o �� doen��a mental, e mais a actual consci��ncia e articula����o de tais mudan��as. Tendo como base uma abordagem psicopedag��gica, o fen��meno do insight aqui explorado assentou numa perspectiva reabilitativa, actual e multidimensional, que fosse para al��m das dimens��es cl��nicas tradicionais. Neste sentido �� apresentada uma escala original, intitulada ���Escala de Avalia����o do Insight e Identifica����o das Necessidades em Pessoas com Psicose���, bem como um modelo de interven����o psicopedag��gico breve, assente nos pressupostos descritos ao longo do trabalho.-----------------------------------------ABSTRACT: The importance of insight in people with mental illnesses was first studied in psychiatry, in the first decades of the 20th century, by people as important as Lewis (1934) and Jaspers (1959). However, this field of investigation was left unexplored for many years. Only in the last decade has this phenomenon become the object of numerous scientific investigations, having been given special attention by its investigators. For this reason a significant number of instruments for evalauting insight in psychotic disorders were developed. Since then many papers have been published, which has allowed for a more in depth knowledge on the subject. Therefore, in recent years, the concept of insight has been developed in an attempt to clarify its compexity. A once dichotomic phenomenon, described in terms of presence or absence, became considered multidimensional, which made the identification of different levels of insight and different dimensions possible. Current concepts categorize insight into five dimensions: the awareness of the patient in relation to his/her mental illness, the awareness of the patient in relation to the social consequences of his/her illness, the awareness of the need for treatment, the awareness of the symptoms and the explanation of those symptoms in relation to the illness. The lack of insight in psychiatry, in general terms, and as this phenomenon has been described, the lack of awareness of having a mental illness, represents one of the most common symptoms of schizophrenia and affects a big part of the population that suffer from this illness. It is estimated that bewteen 50 and 80 per cent of patients with schizophrenia do not believe that they are ill, which, consequently has a big impact in the process of adherence to treatment. It is still not possible, however, to identify all the factors that determine the lack of insight in schizophrenics. There are psychological, social and cultural influences that almost certainly play their role in the lack of insight registered in this pathology.Since the impact of scizophrenia is felt in many aspects of the individual���s life, its effective treatment should be directed at various levels, including the improvement of insight. One of the objectives of this study is to explore the relationship between the level of insight in psychosis and the clinical and sociodemographic variables, the psychopathology and its global functioning. As well as the clinical and sociodemographic variables, there are of course important individual components that contribute to the level of insight seen in each patient, for example, their level of inteligence, personality, culture, past experiences, memory, etc. The clinical, emotional and/or intelectual nature of the term helps us understand the difficulty that lies in its interpretation as well as in its comprehension. Therefore, the definitions attributed to the term are very different and vary according to the theoretical training of the investigator. It is intended, from this identification/understanding, to promote the quality of life of these people through the development of new findings that might enable an active cooperation. It is equally fundamental to observe their unimpaired capacities in order to enable the acquisition of new behaviour(s) that have a positive impact on the complaints, signs, symptoms, incapacity and disfunctioning seen in the patient.As the actual comprehension of the term explains what we intend to evaluate, a detailed reflection is made on the instruments and definitions that have been used the most to explore insight in psychosis.In this investigation I tried to underline and promote the importance that each subject, undergoing medical intervention, plays during his/her process of recovery and prevention of relapses. Considering the above mentioned objectives, as well as a theoretical review of the phenomenon in terms of conceptualization and investigative studies developed, this study contributed to the validation of the instrument.The insight phenomenon chosen by the ���Insight Scale���, records less changes experienced in relation to the mental illness and more actual awareness and articulation of these changes. Based on a psychopedagogical approach, the insight phenomenon explored here settled on a rehabilitation, current and multidimensional perspective that would go beyond the traditional clinical dimensions. For this reason an original scale entitled ���Insight Evaluation Scale and Need Identification in Psychosis Patients��� is presented, as well as a psychopedagogical intervention model soon to be used with admitted patients based on the presuppositions described in this study.

Anemia de c��lulas falciformes na idade pedi��trica : contribui����o para o estudo das suas principais complica����es e indicadores de progn��stico

RESMO: Introdu����o: A anemia de c��lulas falciformes doen��a heredit��ria, com repercuss��o multi-org��nica, tem grande variabilidade na sua express��o cl��nica. Da�� o interesse do estudo de indicadores de progn��stico. A investiga����o realizada foi precedida de um resumo hist��rico incidindo sobre a compreens��o de aspectos fundamentais da doen��a ao longo dos tempos. Na primeira parte do estudo e ap��s revis��o bibliogr��fica, foram referidos dados da fisiopatologia como base para os estudos que integram a presente disserta����o. Abordou-se o estado da arte relativamente ��s complica����es, aos indicadores de progn��stico e �� terap��utica utilizada. Objectivos: Constitu��ram objectivos deste estudo realizado numa amostra populacional representativa: identificar as les��es a n��vel dos sistemas cardio-respirat��rio e nervoso central, avaliando-se as respectivas repercuss��es; avaliar a presen��a de indicadores de progn��stico entre as vari��veis seleccionadas; estudar a efic��cia e toxicidade da HU nos doentes com as formas graves da ACF. Para a prossecu����o destes objectivos foram delineados para al��m do estudo global tr��s estudos espec��ficos: Estudo 1- repercuss��o no sistema cardio-respirat��rio; Estudo 2- repercuss��o no sistema nervoso central; Estudo 3- terap��utica com hidroxiureia. Doentes e m��todos: Procedeu-se a um estudo prospectivo e multi-institucional durante um per��odo de tr��s anos tendo-se seleccionado para a amostra, e de acordo com crit��rios pr��-definidos, 30 doentes com ACF na fase est��vel da doen��a, com idades compreendidas entre os sete e os 18 anos, todos de origem africana �� excep����o de um caucasiano. O diagn��stico baseou-se em t��cnicas de electroforese e estudo molecular que definiu o genotipo da doen��a e a presen��a da delec����o da ���-talass��mia assim como os haplotipos da amostra populacional. Foram utilizadas diferentes metodologias para avaliar a exist��ncia de les��o pulmonar e cerebral. Atrav��s do estudo estat��stico foram seleccionadas diversas vari��veis como hipot��ticos indicadores de progn��stico. Estudo 1. Para determinar a exist��ncia de les��o a n��vel pulmonar usaram-se duas metodologias diferentes, a avalia����o da fun����o pulmonar com estudo da satura����o da Hb em O2 no sangue arterial e a tomografia computadorizada de alta resolu����o. Estudou-se tamb��m a poss��vel disfun����o card��aca como repercuss��o da les��o pulmonar, atrav��s do ecocardiograma, e os indicadores de progn��stico com significado estat��stico para a les��o encontrada. Estudo 2. O desenho deste estudo foi sobrepon��vel ao anterior, mas com metodologia adequada para o SNC. Procedeu-se ao estudo das les��es cerebrais por meio de exames imagiol��gicos, (RMN-CE e DTC) e de testes psicol��gicos. Correlacionaram-se as tr��s metodologias utilizadas e a import��ncia de cada uma para a decis��o de atitudes terap��uticas preventivas. Estudo 3. Consistiu num estudo aberto prospectivo n��o controlado com nove crian��as e adolescentes com formas graves de ACF, com o objectivo de avaliar a efic��cia da terap��utica com hidroxiureia, durante um per��odo de 24 meses. Todos os doentes completaram no m��nimo 15 meses de terap��utica, com uma dose final m��dia de 19���4 mg/K/dia. Resultados globais: Durante o per��odo anterior �� investiga����o caracterizou-se a amostra populacional estudada quanto ao fenotipo gen��tico, cl��nico e hematol��gico de acordo com os crit��rios utilizados por outros investigadores. Verificou-se: predom��nio do haplotipo Bantu na forma homozig��tica em 53% dos doentes; n��mero total de EVO ���3/ano em 87,5% dos doentes; crises de sequestra����o em 18,75%; dactilites no primeiro ano de vida em 31,2%; quadro de s��psis grave apenas num doente; crises de hiper-hem��lise em 50%; e STA em 59,38% dos doentes. Quanto ao fenotipo hematol��gico evidenciaram-se como factores de risco reticulocitose (13,1x103/���l) e hiperbilirrubin��mia (2,5 mg/dl) e como factores de bom progn��stico a presen��a de delec����o de um gene da ���-talass��mia em 46,9% dos doentes e valor m��dio 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 altera����es da difus��o n��o foram estatisticamente significativas, o estudo dos gases no sangue n��o evidenciaram resultados anormais e a TCAR evidenciou altera����es em 43,3% dos doentes. Apenas num doente se verificou doen��a pulmonar obstrutiva relacionada com maior n��mero da STA.O estudo da disfun����o card��aca encontrada em 86,7% dos doentes n��o reflecte a repercuss��o da DPR a n��vel card��aco, podendo estar associada ��s altera����es fisiopatol��gicas da pr��pria anemia cr��nica. Encontraram-se indicadores de progn��stico hematol��gicos e cl��nicos. Entre os primeiros, valores de Hb ���8,5 g/dl e de HbF ���13% foram considerados indicadores de bom progn��stico para a les��o pulmonar. Em rela����o aos par��metros cl��nicos, as STA n��o foram consideradas indicadoras de progn��stico para a DPR ao contr��rio do que se verificou com o n��mero de EVO. Pela an��lise dos par��metros gen��ticos e socio-econ��micos provou-se a aus��ncia de rela����o estatisticamente significativa com les��o pulmonar. Estudo 2. Pela RMN-CE foram diagnosticados ES em 33,3% com uma localiza����o preferencial na subst��ncia branca profunda em 26,6% dos doentes. Relativamente aos par��metros hematol��gicos seleccionados, o valor m��dio da HbF 8,6% constitu��u um indicador de bom progn��stico para o aparecimento de ES, enquanto o valor m��dio de leuc��citos 12.39x103/��l foi considerado um indicador de mau progn��stico. 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 psicol��gicos alterados em 80% dos doentes mostraram ser o m��todo mais sens��vel para detectar altera����es do neurodesenvolvimento, mas sem correla����o com os ES em 10% dos doentes. Real��a-se a baixa percentagem de DTC patol��gicos encontrados neste estudo em rela����o ao n��mero elevado de ES e de testes psicol��gicos alterados, n��o se verificando concord��ncia entre os tr��s exames. Dos indicadores de progn��stico estudados a ���-talass��mia foi considerada um factor de protec����o para o coeficiente de intelig��ncia da escala de Wechsler. Em rela����o a par��metros cl��nicos estudados os doentes com maior n��mero de EVO, tem em m��dia valores inferiores nos testes psicol��gicos. Estudo 3. Neste estudo verificou-se que o valor m��dio da HbF aumentou significativamente de 7,0��4% para 13,7��5,3% (p=0,028) ao fim de 15 meses de terap��utica com hidroxiureia. Clinicamente todos os doentes responderam significativamente com uma redu����o de 80% no n��mero de EVO, 69% no n��mero de internamentos, 76% no n��mero de dias de hospitaliza����o e 67% no n��mero de transfus��es. Deste modo comprovou-se n��o s�� a efic��cia desta terap��utica neste grupo pedi��trico como tamb��m a falta de efeitos secund��rios significativos. Considera-se a necessidade de estudos mais prolongados e em grande s��ries, para com seguran��a se usar a HU antes que a les��o org��nica se estabele��a, portanto logo nos primeiros anos de vida. Conclus��o: Na amostra populacional estudada foram evidenciadas les��es pulmonares e cerebrais na grande maioria dos doentes que condicionaram a sua qualidade de vida. Foram identificados indicadores de progn��stico que poder��o eventualmente ditar medidas terap��uticas precoces com o objectivo de diminuir a morbilidade e a mortalidade neste grupo et��rio. Demonstrou-se que a terap��utica 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 Estef��nia'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/��l 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.

Quelques Aspects de la Reforme Carolingienne du rituel eucharistique. Les cas exemplaires de la "Fractio" et l"'Immixtio"

pp. 359-365

Caracteriza����o e Impacte das quedas de doentes, como indicador de qualidade, num hospital E.P.E.

RESUMO - Introdu����o: As quedas dos doentes s��o um dos principais eventos adversos que podem ocorrer em contexto hospitalar, levando por um lado, ao aumento de encargos econ��micos e sociais, e por outro a consequ��ncias f��sicas e psicol��gicas nos doentes e respectiva fam��lia. Para a sua preven����o, �� necess��rio investir na seguran��a do doente atrav��s de uma avalia����o e consequente gest��o do risco de quedas. Este trabalho insere-se na estrat��gia de melhorar a seguran��a do doente e a qualidade dos cuidados prestados. Objectivos: Avaliar em que medida o formul��rio existente responde ��s actuais necessidades de gest��o do risco de quedas e suas consequ��ncias. Por outro lado, com base numa extensa revis��o da literatura nacional e internacional, e tendo em conta as din��micas de qualidade e seguran��a do doente que t��m sido criadas no hospital em estudo, propor um formul��rio mais eficaz que permita responder aos desafios que se colocam nos dias de hoje. Metodologia: Tratou-se de um estudo descritivo explorat��rio assente no paradigma quantitativo. A popula����o do estudo foi constitu��da por 98 doentes que sofreram quedas durante o internamento no ano de 2010, num hospital E.P.E. (Entidade P��blica Empresarial) Conclus��o: As quedas ocorreram mais frequentemente em indiv��duos do sexo masculino com doen��as c��rebro-vasculares e infecciosas, sendo que na maioria das vezes n��o resultaram em consequ��ncias graves. O formul��rio que aqui se recomenda tem a vantagem de ser informatizado e caracterizar aspectos relacionados com os custos. ---------------- ABSTRACT - Introduction: Falls of patients are one of the main adverse events that may occur in a hospital context, leading on the one hand, to an increase of economic and social burden, and, on the other hand, physical and psychological consequences for patients and their families. For its prevention, we need to invest in patient safety through an assessment and consequent management of the fall risk. This work is inserted in the strategy to improve the Patient safety and the quality of health care. Objectives: To assess the extent to which the form responds to current needs of managing the fall risk and their consequences. On the other hand, on the basis of an extensive review of national and international literature, and taking into account the dynamics of quality and safety of the patient that are created at the hospital in study, to propose a form more efficient in order to respond to the challenges that we face nowadays. Methodology: It was an exploratory descriptive study based on the quantitative paradigm. The population study was constituted by 98 patients who had suffered falls during hospitalization in the year 2010, in a hospital E.P.E. Conclusion: Falling occurred more frequently in male individuals with cerebral-vascular and infectious diseases, and in most of the time, falls have not resulted in serious consequences. The form recommended here has the advantage of being computerized and characterize aspects related with costs.

An assessment of the social and economic cohesion aspects of the development of the information society in Europe

Neste trabalho pretende consolidar-se a contribui����o portuguesa para o estudo comunit��rio ���Na Assessment of the Social and Economic Cohesion Aspects of the Development of the Information Society in Europe��� elaborado por um cons��rcio europeu liderado pela Nexus Europe e em que interv��m o ISEGI- Instituto Superior de Estat��stica e Gest��o de Informa����o, da Universidade Nova de Lisboa, como parceiro nacional portugu��s.

Embracing transience and subjectivity in the conservation of complex contemporary artworks: contributions from ethnographic and psychological paradigms

Disserta����o para obten����o do Grau de Mestre em Conserva����o e Restauro, Perfil Ci��ncias da Conserva����o Especializa����o em Arte Contempor��nea

The human side of change: Qualitative research on a public sector hospital merger

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA ��� School of Business and Economics

A way out of the crises for expatriates: The mediating role of psychological capital

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA ��� School of Business and Economics

Durability aspects related to rubble stone masonry walls strengthened with reinforced micro-concrete layers

International Seminar on Seismic Risk and Rehabilitation of Stone Masonry Housing, Azores, Portugal, 1998

Psychological contract in non-governmental organizations

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA ��� School of Business and Economics

Suicide study : a psychological autopsy

RESUMO: O'suic��dio'��'atualmente'um'problema'de'sa��de'p��blica.'Estimarse'que'um'milh��o'de'pessoas' morra'anualmente'devido'ao'suic��dio.'De'acordo'com'diversas'ag��ncias'e'organiza����es'estimar 'que'ocorram'entre'20'a'40'tentativas'de'suic��dio'por'cada'suic��dio'consumado.'Os'custos'associados'ao'suic��dio,'quer'humanos'quer'econ��micos's��o'enormes'e'estendemrse'��'fam��lia,'emprego,'economia'e'finan��as.'Os'n��meros'oficiais'do'suic��dio'em'Portugal'indicam'uma'taxa ligeiramente'acima'dos'10'por' cada'100'000'habitantes'mas' comportando' regi��es'onde' s��o'observadas' taxas' muito' elevadas,' como' no' Sudoeste' de' Portugal' onde' se' observam particularmente taxas 3'vezes'acima'da'm��dia'nacional.' Investig��mos' o' fen��meno' com' os' objetivos' de' validar' a' t��cnica' de' aut��psia' psicol��gica' em' contexto' comunit��rio' nunca' realizado' em' Portugal,' e' dar' um' contributo' para' uma' compreens��o'mais'alargada'deste'problema'de'sa��de'p��blica,'especialmente'nesta'regi��o'de' Odemira.' A' aut��psia' psicol��gica' consiste' em' reunir' detalhadamente' informa����o' sobre' a'personalidade' e' vida' de' algu��m' que' morreu' em' circunst��ncias' equ��vocas' (por' suic��dio,' homic��dio' ou' acidente)'recorrendo'ainda'a'registos'e'documenta����o,'bem'como'procedendo'a entrevistas' com' familiares,' colegas' ou' amigos.' Foi' feita' uma' adapta����o' de' uma' entrevista' semirestruturada' para' o' efeito.' Foram' recrutados' 2' grupos' de' participantes:' um' grupo' de familiares de pessoas que cometeram'suic��dio'(n=30)'e'um'grupo'de'familiares'de'pessoas'que morreram de causas' naturais' (n=24).' O' estudo' decorreu' em' 3' momentos' com' sess��es' de' apresenta����o' (i),' sess��es' de' prepara����o' (ii)' e' entrevistas' para' recolha' de' informa����o' e'monitoriza����o.'' Os' principais' resultados'mostram' que' existiram' dificuldades' na' obten����o' da' informa����o' a'partir' dos' entrevistados,' a'maioria' dos' suicidas' eram'homens' acima' dos' 40' anos' de' idade,' afetados' por' les��es' graves' ou' doen��as' graves' ' no' per��odo' adulto' embora' apresentassem'condi����es' v��lidas' para' trabalhar,' reformados,' vivendo' em' fam��lia,' com' tra��os' de'personalidade' amargurados,' tristes' ou' pessimistas,' com' escassas' atividades' nos' tempos' de' lazer,' sem' problemas' som��ticos' significativos' ou' perturba����es'mentais' que' comunicaram' a inten����o de morrer previamente'e'provenientes'de'fam��lias'sem'dificuldades'econ��micas'ou'rela����es'familiares'desadequadas.' Tendo'em'conta'a'literatura,'algumas'das'caracter��sticas'parecem'ser'muito'particulares'desta'popula����o.'Aparentemente'o'suic��dio'poder��'ter'implica����es'gen��ticas'que'deveriam'ser'tidas'em'conta'em'futuras'investiga����es.' A'integra����o'da'sa��de'mental'nos'cuidados'de'sa��de'prim��rios'afigurarse'urgente'tendo'em' conta' que' a' escassez' de' profissionais' de' sa��de'mental' ��' enorme' numa' parte' do' pa��s' onde'ocorre' o'maior'n��mero'de' suic��dios.'Mudar'de'modelo'e'reorganizar'os' servi��os'de' forma'a' poder' dar' uma' resposta' ao' d��fice' de' tratamento' de' sa��de' mental' e' ter' em' conta' os' determinantes'sociais'da'sa��de'para'fazer'face'ao'isolamento'��'fundamental.' ---------------ABSTRACT: Suicide' is,'nowadays,' a'public'health'problem.'A'million'dies' annually'by' suicide'worldwide.' According'to'several'agencies'and'organizations'an'estimation'of'20'to'40'suicide'attempts'is advanced for each complete' suicide. Associated'costs'to'suicide,'both'human'and'economic,'are' huge' and' spread' on' family,' jobs,' economy,' and' finances.' Official' available' figures' for' suicide' in' Portugal' indicate' a' rate' slightly' over' 10' for' each' 100' 000' inhabitants,' but' with'regions'where'one' can' actually' find'extremely'high' rates,' like' for' instance' in' the'Southwest' part' of' the' country,' where' are' regularly' found' rates' 3' times' higher,' when' compared' to'national'average.'' We' have' investigated' the' phenomenon' aiming' to' validate' the' technique' of' psychological'autopsy' in' a' community' context' never' explored' before' in' Portugal' and' to' contribute' for' a' wider' understanding' of' this' public' health' problem,' especially' in' this' region' of' Odemira.' Psychological'autopsy'consists'into'a'detailed'gathering'of'information'about'the'personality'and' life' of' someone' died' in' equivocal' circumstances' (suicide,' homicide,' accident),' registries and other'documentation'as'well'as'interviews'with'family'members,'corworkers'and'friends.' An' adaptation' of' a' semirstructured' interview' was' made.' Two' groups' of' participants' were'recruited: a'group of'relatives'of'people'committing'suicide'(n=30),'and'a'group'of'relatives'of'people' whose' death' was' after' natural' causes' (n=24).' Study' was'made' in' 3'moments' with'presentation'sessions;'(i)'preparation'sessions;'(ii)'interviewing'data'collection'and'monitoring' iii).'' Main'results'showed'difficulties'to'obtain'information'from'interviewees,'most'suicides'were' from males over 40 years old, affected'by'serious'illness'or'severe'injury'in'adulthood'but'valid'to'work,'retired,'living'in'family,'with'bitterness,'sad'or'pessimistic'as'personality'traits,'with'few' leisure' activities,' without' significant' health' somatic' problems' or' mental' disorders,'communicating'intention'to'die'previously,'coming'from'families'with'no'indication'of'financial'distress'or'inadequate'family'relationship.' Taking'into'account'the'literature'it'looks'like'some'features'seem'to'be'quiet'particular'from'this'population.'Apparently'one'might'say'that'suicide'could'have'genetic'implications'which'further'research'should'account'in'the'future.' Integration' of'mental' health' into' primary' care' is' urgent' once' the' scarcity' of'mental' health' professionals' is' enormous' in' a' part' of' the' country' where' most' suicides' occur.' Scaling' up services must go in order'to'address'to'treatment'gap'and'social'determinants'of'health'should'be'taken'into'account'to'face'isolation.' Results' show' that' the' profile' of' the' suicidal' in' the' region' of' Odemira' is' particular' and'implications' of' genetics' should' be' taken' into' account.' Moreover' much' can' be' done' in'organization' of' services' in' the' region' where' we' performed' the' present' study' in' order' to'address'to'treatment'gap'for'mental'disorders'and'to'the'social'determinants'of'health.