Skipping of exon 30 in C5 gene results in complete human C5 deficiency and demonstrates the importance of C5d and CUB domains for stability

The deficiency of complement C5 is rare and frequently associated with severe and recurrent infections, especially caused by Neisseria spp. We observed the absence of component C5 in the serum of 3 siblings from a Brazilian family with history of consanguinity. The patients had suffered from recurrent episodes of meningitis and other less severe infections. Sera from these patients were unable to mediate hemolytic activity either by the classical or alternative pathways and presented extremely low levels of C5 protein (13, 0.9 and 1.0 mu g/ml-normal range: 45-190 mu g/ml). Hemolytic activity could be restored by the addition of purified C5 to deficient serum. Sequencing of sibling C5 cDNA revealed a homozygous 153 bp deletion that corresponds precisely to exon 30. The parents carried the same deletion but only in one allele. Sequencing of the corresponding region in the genomic DNA revealed a C to C substitution within intron 30 and, most significantly, the substitution of GAG(4028) for GAA(4028) at the 3` end of exon 30 which is most likely responsible for skipping of exon 30. The resulting in-frame deletion in the C5 mRNA codes for a mutant C5 protein lacking residues 1289-1339. These residues map to the CUB and C5d domains of the C5 alpha chain. This deletion is expected to produce a non-functional and unstable C5 protein which is more susceptible to degradation. (C) 2009 Published by Elsevier Ltd.

Does the neuromotor abnormality type affect the salivary parameters in individuals with cerebral palsy?

Background: Previous studies reported alterations in salivary flow rate and biochemical parameters of saliva in cerebral palsy (CP) individuals; however, none of these considered the type of neuromotor abnormality among CP individuals, thus it remains unclear whether the different anatomical and extended regions of the brain lesions responsible for the neurological damage in CP might include disruption of the regulatory mechanism of saliva secretion as part of the encephalopathy. The aim of this study was to evaluate salivary flow rate, pH and buffer capacity in saliva of individuals with CP, aged 3-16 years, with spastic neuromotor abnormality type and clinical patterns of involvement. Methods: Sixty-seven individuals with CP spasticity movement disorder, were divided in two groups according to age (3-8- and 9-16-years-old) and compared with 35 sibling volunteers with no neurological damage, divided in two groups according to age (3-8- and 9-16-years-old). Whole saliva was collected under slight suction and pH and buffer capacity were determined using a digital pHmeter. Buffer capacity was measured by titration using 0.01N HCL, and flow rate was calculated in ml/min. Results: In both age groups studied, whole saliva flow rate, pH and buffer capacity were significantly lower in the spastic CP group (P < 0.05). The clinical patterns of involvement did not influence the studied parameters. Conclusion: These findings show that individuals with spastic cerebral palsy present lower salivary flow rate, pH and buffer capacity that can increase the risk of oral disease in this population.

The world of the Sumerian mother goddess : an interpretation of her myths

The present study is an interpretation of the two myths copied in the Old Babylonian period in which the Sumerian mother goddess is one of the main actors. The first myth is commonly called “Enki and Ninḫursaĝa”, and the second “Enki and Ninmaḫ”. The theoretical point of departure is that myths have society as their referents, i.e. they are “talking about” society, and that this is done in an ideological way. This study aims at investigating on the one hand which contexts in the Mesopotamian society each section of the myths refers to, and on the other hand which ideological aspects that the myths express in terms of power relations. The myths are contextualized in relation to their historical and social setting. If the myth for example deals with working men, male work in the area during the relevant period is discussed. The same method of contextualization is used regarding marriage, geographical points of reference and so on. Also constellations of mythical ideas are contextualized, through comparison with similar constellations in other Mesopotamian myths. Besides the method of contextualization, the power relations in the myths are investigated. According to this latter method, the categories at issue, their ranking, as well as their changed ranking, are noted. The topics of the myths are issues important for the kingship and the country, such as irrigation, trade, health and healing, birth, collective work, artisanry and rivalry. All these aspects are used in order to express what the power relations between the goddess Ninḫursaĝa/Ninmaḫ and the god Enki look like. The relations are negotiated and recalibrated, which leads to the goddess getting a lowered status. Part of the negotiations and recalibrations is gender behavior, which is related to historical developments in society. The present work points to the function of these myths as tools of recalibrating not only deities, but also men and women in society.

Familjemedlem till person med psykossjukdom : bemötande och utanförskap i psykiatrisk vård

This thesis focused on the situation of family members of persons with psychotic illness, particular on their experience of the approach of the healthcare professionals and of their feelings of alienation regarding the professional care of their family member. A further aim was to explore how siblings who have participated in a support group for siblings experienced their situation. A questionnaire was developed that enabled the aims of this thesis to be investigated (I). Seventy family members from various parts of Sweden participated, and data were collected via the questionnaire developed in study I (II-III). Thirteen siblings who previously had participated in a support group participated in follow-up focus groups interviews (IV). The questionnaire developed was shown to be reliable and valid in these studies (I). In many cases, the participants had experienced an approach from professionals that indicated that they did not experience openness, confirmation and cooperation, and that they felt powerless and socially isolated in relation to the care. There was also found to be a certain degree of association between how the participants experienced the approach and whether they felt alienation (II). The majority of the participants considered openness, confirmation, and cooperation to be important aspects of professional’s approach. The result also identified a low level of agreement between the participants’ experience and what they considered to be significant in the professional’s approach (III). The findings revealed the complexity of being a sibling of an individual with psychotic illness. Participating in a support group for siblings can be of importance in gaining knowledge and minimizing feelings of being alone (IV). Although the psychiatric care services in Sweden have been aware of the importance of cooperating with family members, the results indicated that there is a need for further research in this area.

O ECR na relação fornecedor x atacadista

Este relatório intitulado “Pesquisa sobre o ECR na Relação Fornecedor X Atacadista no Brasil”, foi desenvolvido pelo Professor Manoel de Andrade e Silva Reis, para o Núcleo de Pesquisas e Projetos (NPP) da Fundação Getúlio Vargas de São Paulo. Inicialmente apresenta alguns conceitos importantes para o entendimento do texto e faz considerações gerais sobre o ECR no Brasil e seu histórico. A principal parte do trabalho é a pesquisa realizada com 10 FORNECEDORES e 20 ATACADISTAS, sendo que 60% dos FORNECEDORES e 40% dos ATACADISTAS responderam aos questionários. As principais conclusões da pesquisa são: 1) Existem sérios obstáculos a transpor para a implantação abrangente do ECR na relação FORNECEDOR X ATACADISTA no Brasil, como: a) O setor ATACADISTA tem um forte preconceito com relação ao ECR, pelo receio de perda de mercado com a implantação de uma nova forma de fazer negócio. Isso evita que seus membros aprofundem o conhecimento das suas estratégias e ferramentas; b) Empresas ATACADISTAS tendem a ser muito fechadas, dificilmente abrindo suas informações para fornecedores, um dos requisitos básicos para a implantação bem sucedida do ECR. Têm também grande rivalidade com seus pares ATACADISTAS. c) O setor ATACADISTA desconfia das verdadeiras intenções dos FORNECEDORES. Há ATACADISTAS que chegaram a implantar uma ferramenta do ECR para teste e desistiram, por sentirem que os FORNECEDORES fogem aos acordos estabelecidos, visando, em especial, preencher as cotas de venda. 2) Na opinião das empresas ATACADISTAS, a sobrevivência setor depende de fatores como: a) Necessidade de uma radical mudança cultural das empresas, de forma a adotar os conceitos de compra e venda necessários no ECR; b) Necessidade de especialização em canais escolhidos e nos produtos característicos dos mesmos, visando tornar-se especialista na sua comercialização; c) Gestão mais adequada dos níveis de estoque; d) Maior agilidade no acesso ao pequeno varejo. 3) Fatores que podem aumentar a atratividade do setor ATACADISTA para os FORNECEDORES: a) Redução do número de transações; b) Redução dos custos pelos elevados volumes; c) Prestação de serviços ao pequeno varejo (devoluções, trocas, promoções, treinamento e incentivos); d) Pulverização das vendas.

A dinâmica da organização interna da mesa diretoria e comissões da assembléia do estado do Rio de Janeiro

A globalização do capitalismo joga indivíduo em um mundo de controvérsias, quase de crise existencial, já que oscila entre sonho de satisfazer todos os seus desejos realidade de não poder realizá-los. globalização que interessa são as ressacas emocionais do indivíduo, que distancia da razão, do equilíbrio entre poder querer torna um compulsivo do consumo. processo de massificação homogeneização cultural, que não apenas gera vícios consumistas nos indivíduos, como também os impede de participarem politicamente. Politicamente, uma primeira fase da globalização caracterizada pela égide das monarquias absolutistas que concentram enorme poder mobilizam os recursos econômicos, militares burocráticos, para manterem expandirem seus impérios coloniais. Os principais desafios que enfrentam advinham das rivalidades entre elas, seja pelas disputas dinástico-territoriais ou pela posse de novas colônias no além do mar. Politicamente globalização recente caracteriza-se pela crescente adoção de regimes em procura de uma democracia mais ampla participativa participação política, portanto, exigência básica para que indivíduo supere as barreiras impostas pela globalização consiga desenvolver ações de cidadania dentro da própria sociedade global. Nesse sentido, necessário encurtar distanciamento entre as formas institucionais existentes, sejam jurídicas ou políticas, real possibilidade de reconhecer nas leis, nas instituições, as suas próprias leis seu próprio poder.

Competição e alavancagem na indústria de aços planos

Pesquisas recentes nos campos das Finanças e de Economia Industrial vêem mostrando que as decisões de financiamento corporativo são interdependentes de decisões relativas ao mercado de produtos. As teorias apresentadas apóiam-se na responsabilidade limitada dos detentores do capital próprio para construir modelos nos quais a dívida é utilizada estrategicamente. Altos níveis de endividamento tendem a favorecer o acirramento da concorrência e a produzir resultados piores do que numa situação de conluio, provocando a adoção de mecanismos de limitação da alavancagem e da concorrência pelo oligopólio. Evidências indicam que firmas alavancadas suavizam a concorrência enquanto firmas menos ou não alavancadas tornam a concorrência mais acirrada ante a presença de um rival mais alavancado. Dados de corte seccional das firmas participantes do mercado brasileiro de aços planos ao carbono foram tratados pelo método econométrico para verificar o comportamento da variável de endividamento tendo como variáveis explicativas de interesse os preços praticados e as quantidades vendidas pelas firmas e, como variáveis de controle, algumas dentre aquelas sugeridas pela teoria da estrutura de capital. Os resultados mostram evidências de que a variável de concorrência "preço" influencia as decisões de estrutura de capital das firmas siderúrgicas produtoras de aços planos e que a correlação com a alavancagem é positiva. No entanto, nenhuma conclusão pôde ser obtida sobre a variável quantidade. Cálculos adicionais produziram resultados que são indicativos de iniciativas de aumento de concorrência pelas firmas mais alavancadas da indústria, como redução de preço, aumento de lucratividade e maior participação de mercado, sugerindo que as circunstâncias de expansão de demanda favorecem a ruptura do conluio.

Centralized allocation in multiple markets

We study the problem of centralized allocation of indivisible objects in multiple markets. We show that the set of allocation rules that are group strategy-proof and Pareto-efficient are sequential dictatorships. Therefore, the solution of the joint al-location in multiple markets is significantly narrower than in the single-market case. Our result also applies to dynamic allocation problems. Finally, we provide conditions under which the solution of the single-market allocation coincides with the multiple-market case, and we apply this result to the study of the school choice problem with sibling priorities.

Relações interétnicas em conflito entre makuxi e wapixana na terra indígena Raposa Serra do Sol

This dissertation present an analysis of the interethnic conflict between Makuxi and Wapixana at the current moment in the Maloca of the Adobe, Aboriginal Land Fox-Mountain range of the Sun, in the State of Roraima. The theoretical field was boarded in the Ethnology, pursuing situations in local history, with edges in ethno-history. The research elapsed of the deepening necessity on the social relations and aboriginal politics, for the intercultural professional exercise of educator, appealing the bibliographical survey and participant comment as method; not directive interviews, photographs, filmings and daily register in of field, as techniques carried through in the period of 2006 to 2007. Although to inhabit in the same area and to establish marriages between itself, individuals and groups express tensions, aggravated with the landmark and legal recognition of the area, which generated inter dispute and intraetnias, mainly with the intrusion of farmers, rizicultores and the form of governmental influence. A relation of rivalry, individual and collective was evidenced, suggesting the strengthenig and not it fractionly, of the fights external politics, interns and in way to the cultural diversity and social adversity

Agregação familiar e resultados maternos e perinatais da pré-eclâmpsia severa em população do Rio Grande do Norte

To determine whether there is familiar aggregation of severe preeclampsia in a Brazilian population from Rio Grande do Norte and to characterize the maternal and perinatal outcomes in the studied population. Methods: A case control study was performed with 412 participants who were admitted at Maternidade Escola Januário Cicco (MEJC) for medical care. Of these, 264 subjects presented normal blood pressure and 148 were cases. Cases were composed of eclampsia (n=47), HELLP Syndrome (n=85) and Eclampsia associated with HELLP syndrome (n=16). The diagnosis of these illness were based on the citeria developed by National High Blood Pressure Education Program Working (2000). An interview was performed with each subject and questions related to personal and familiar history of hypertension, preeclampsia, HELLP syndrome and eclampsia. Statistical analysis was performed and comparison of median and mean between cases and controls were performed, with the level of significance of 5%. The Odds-Ratio was determined to estimate the risk of preeclampsia within the families. Results: There were no difference in the demographic data between cases and controls. Previous history of chronic hypertension and preeclampsia was more frequent in the case group. Headaches were more frequent in eclampsia and epigastric pain in the HELLP syndrome cases. Bleeding and oliguria were more frequently found in the eclampsia associated with HELLP syndrome cases. Acute Renal insufficiency was a common complication in the case group, but these cases did not evolve to chronic renal insufficiency. The maternal mortality was 0.4% and the perinatal mortality was high, 223 per 1,000 live births. The 111 risk of a woman to develop preeclampsia whose mother has hypertension or had preeclampsia was respectively 2.5 and 3.5. This risk was increased 5 times, when a sibling has hypertension and 6 times when both sibling and mother had previous history of preeclampsia. Conclusions: This study confirms that there is familiar aggregation of preeclampsia in this Brazilian population. The potential for cardiovascular complications due to development of chronic hypertension indicates the need of closely follow up of women who develop preeclampsia

A produção da subjetividade no contexto institucional de um seminário católico

Esta pesquisa visa estudar a produção da subjetividade no contexto institucional de um Seminário Católico. Este estabelecimento funciona em regime de internato no qual atualmente 70 seminaristas estudam Filosofia durante 3 anos, numa etapa preparatória para o sacerdócio. Pesquisamos o funcionamento da vida institucional através da observação participante e utilizamos entrevistas semi-estruturadas para entender como o seminarista experiencia sua vida. Os resultados preliminares indicam que a vida no contexto institucional do Seminário produz diversas modalizações da subjetividade nos seminaristas internados no estabelecimento: há uma perda considerável de autonomia pessoal, da liberdade de ir e vir, agir e decidir, originando comportamentos de dependência excessiva, de resistência à mudança, de conversão ao papel proposto, de rivalidade fraterna. A contradição detectada entre o aparelho repressivo e o discurso participativo parece produzir nos indivíduos características marcadas pela clivagem e pela recusa.

O internato escolar O Ateneu: produção de subjetividade na instituição total

Dans cet article on cherche à identifier les effets de l'institutionnalisation dans la production de la subjectivité dans un établissement scolaire total. La recherche est basée sur le roman «O Ateneu » de Raul Pompeia, analysé à partir de la théorie de Goffman (1961-1987) relative aux institutions totales. on décrit l itinéraire moral que le personnage Sergio développe, à son entrée à l'internat, évoquant les vicissitudes par lesquelles il passe dans ce contexte institutionnel : période d'adaptation, crises évolutives, initiations sexuelles, problèmes de rivalités, etc. les établissements totaux semblent s'organiser d'une façon caractéristique et fonctionner de manière autonome. on pourra comprendre les problèmes sociaux et les effets sur la subjectivité produite par les institutions totales par l étude des relations de pouvoir subjacentes à ces types d'établissements. Le temps pendant lequel un individu vit comme interné peut laisser des marques profondes dans sa subjectivité et se configure comme un thème personnel approprié.

Root canal treatment of three-rooted maxillary second premolars: Report of four cases

The aim of this report is to contribute to a better understanding of the radiographic, clinical and anatomic findings in maxillary second premolars. This paper reports the endodontic treatment of two cases of three-rooted three-canal maxillary second premolars in different patients, and two sound maxillary second premolars also with three canals and three independent roots in a sibling of one of the patients. Although the presence of maxillary second premolars with one or two canals and one root is much more common, other anatomic conditions can be found. A correct clinical and radiographic diagnosis based on knowledge of root canal anatomy and critical interpretation of radiographs is necessary for a safer and successful endodontic treatment of these teeth.

The taxonomic status of Leptodactylus geminus Barrio, 1973

The advertisement calls of Leptodactylus geminus, Leptodactylus gracilis, Leptodactylus plaumanni, and Leptodactylus fuscus are analyzed and compared with published reports. Based on calling data and examination of type material, Leptodactylus geminus is synonymized with L, plaumanni. To differentiate between the sibling species L. gracilis and L. plaumanni, fieldwork is needed, including recording of advertisement calls. Reliable identification of red specimens is not possible based on morphology and coloration alone.