RESUMO Objetivo O objetivo deste estudo é apresentar achados de linguagem, comportamento e neurodesenvolvimento de uma menina com diagnóstico da Síndrome de Angelman, avaliada aos três e aos oito anos. Método Os instrumentos de avaliação foram Observação do Comportamento Comunicativo, Early Language Milestone Scale (ELM) e Teste de Screening de Desenvolvimento DENVER-II (TSDD-II). Resultados No caso apresentado, verifica-se a presença dos sinais fenotípicos da SA, tais como boca larga, dentes espaçados, língua protuberante, estrabismo, fissuras palpebrais ascendentes e sialorreia. Na avaliação de linguagem, foram verificados déficits expressivos e receptivos, com ausência de oralidade e prejuízos na compreensão. O TSDD-II e a ELMS indicaram grave comprometimento de todas as habilidades avaliadas aos três e aos oito anos. O desempenho encontrado, nas duas avaliações, foi muito semelhante em todas as áreas do desenvolvimento infantil. Ao longo dos anos, verificou-se pouca evolução, apesar do grande investimento terapêutico e educacional. Conclusão A presença de um quadro complexo como a SA demanda necessidades clínicas de alta complexidade, situação agravada frente à escassez de recursos terapêuticos que possam minimizar os impactos deletérios da síndrome, culminando em comprometimento da qualidade de vida da população com a SA, bem como de suas famílias.

Infant's patterns of regulatory behavior contribute to infant socioemotional development and attachment. These behavioral patterns affect and are affected by the quality of mother-infant interaction. In most studies with full-term infants, the Social-Positive Oriented pattern (i.e., the infant's ability to soothe his/her emotions in the context of reciprocal and positive interactions) is the most prevalent pattern, followed by the Distressed-Inconsolable and by the Self-Comfort Oriented patterns. However, these patterns are understudied in other populations beyond the US and European countries. The current research addresses this gap by studying the regulatory behavior patterns and their association with mother-infant interactions in Brazilian dyads and evaluating the association of these regulatory patterns with demographics. Analyses were based on data collected for 40 infants (20 boys, 20 girls) and their mothers. Infants' regulatory behavior patterns were evaluated in the Face-to-Face Still-Face paradigm and mother-infant interaction was evaluated during free play at 3 months age. Notably, our findings indicate that Distressed-Inconsolable was the most prevalent pattern in this sample; followed by the Social-Positive Oriented and the Self-Comfort Oriented patterns. Furthermore, we found that maternal sensitivity and family SES (social-economic status) predicted infant patterns of regulatory behavior.

Three infant regulatory behavior patterns have been identified during the Face-to-Face Still-Face paradigm (FFSF) in prior research samples: a Social-Positive Oriented pattern (i.e., infants exhibit predominantly positive social engagement), a Distressed-Inconsolable pattern (i.e., infants display conspicuous negative affect that persists or increases across FFSF episodes), and a Self-Comfort Oriented pattern (e.g., infants primarily engage in self-comforting behaviors such as thumb-sucking). However, few studies have examined these patterns outside US and European countries or evaluated potential cross-country differences in these patterns. In this study, we compared the regulatory behavior patterns of 74 Brazilian and 124 Portuguese infants in the FFSF at 3 months of age, and evaluated their links to demographic and birth variables. The prevalence of the three regulatory patterns varied by country. The most frequent pattern in the Portuguese sample was the Social-Positive Oriented, followed by the Distressed-Inconsolable and the Self-Comfort Oriented. However, in the Brazilian sample, the Distressed-Inconsolable pattern was the most prevalent, followed by the Social-Positive Oriented and the Self-Comfort Oriented. Moreover, in the Brazilian sample, familial SES was higher among infants with a Social-Positive pattern whereas 1st-minute Apgar scores were lower among Portuguese infants with a Distressed-Inconsolable Oriented pattern of regulatory behavior. In each sample, Social Positive pattern of regulatory behavior was associated with maternal sensitivity, Self-Comfort Oriented pattern of regulatory behavior with maternal control, and Distressed-Inconsolable pattern with maternal unresponsivity.

Science and technology are increasingly present on contemporary societies and the ideological requirement of informed democratic citizenship implies the need that individuals learn and understand science. It is both a cultural object and product that can be received and worked at different levels and within several approaches by the individuals and the communities. Part of this work can be addressed by the professionals of sociocultural community development. A set of fields of action is available for the intervention of these workers, therefore giving to these professionals an important part in conquering, with and within the communities, a democratic space where more informed citizens can improve democratic participation. In this text, the options taken by students when designing science related activities within the context of an optional course of a 1st cycle of a portuguese program over the past four years are analysed and discussed. Entertaining activities seem to be a quite common choice among these students.