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This study describes the cross-cultural adaptation to Brazilian Portuguese of the Adaptive Behavior Assessment System (ABAS-3), which assesses 11 skills areas within three major adaptive domains: the conceptual, social, and practical. The translation was performed by two independent translators, which was followed by the synthesis of the translations by two experts and then an analysis of the synthesis by 33 specialists from the areas of health and education (three per subscale) who were experts in the domains evaluated by ABAS-3. Of the 1121 items of the five ABAS-3 forms, 82 (7.31%) needed revision. There was good agreement between the specialists in respect of most items, with only minor adjustments being required. There was good evidence of content validity and the adequacy of the adaptation in respect of the conceptual, idiomatic, and semantic aspects of the items of the instrument. This initial process of cross-cultural adaptation was necessary because adaptive behavior is strongly influenced by environmental factors such as socioeconomic status and culture.

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This study focuses on the cross-cultural adaptation of the Emotional Outburst Questionnaire (EOQ) to Brazilian Portuguese and preliminarily assesses its predictive validity. The EOQ evaluates aspects of emotional outbursts (EO), including frequency, duration, intensity, types, associated behaviours, recovery time, triggers, and effectiveness of calming strategies. Two independent translators performed the translation, with subsequent synthesis and analysis revealing that only 33 items (24.81%) required revision. Among these, one item needed partial modification, and two needed total modification. The study demonstrated strong content validity and adaptation in terms of conceptual, idiomatic, and semantic aspects. The EOQ's predictive validity was assessed by analysing the interruption of mental health services in Brazil due to Covid-19 (T1) compared to when services resumed after social distancing measures were lifted (T2). Parents of 25 individuals with developmental disabilities (ASD, DS and ID), with a mean of 11 y/o, mostly male (76%), completed the EOQ. Service interruption during T1 led to increased frequency and duration of severe emotional outbursts reported by caregivers compared to T2 (frequency: p < .001; duration: p = 0.05). This suggests that the EOQ exhibits predictive validity and sensitivity to changes influenced by individual contexts. These findings highlight the EOQ's potential as an outcome measure for intervention development.

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During COVID-19 pandemic an increase electronic media (e-media) usage time was reported correlated to behavioral problems. This study verified the associations between e-media and behavioral/emotional problems in children in the context of the pandemic. Sample: Parents of 277 children aged 7-11 years old. Instruments: socioeconomic questionnaire; MAF-P and CBCL/6-18. Spearman's correlation analyses showed positive associations between the MAF-P and CBCL/6-18 scales. Adult games were correlated with 12 scales; websites for information/entertainment were associated with 6 scales, gambling online with one scale; electronic games and watching YouTube videos were associated with all 17 scales. In conclusion, overall, the results indicate the need of supervising children's e-media access considering the number and frequency of use and the elevated number of emotional and behavioral problems found among the evaluated group

Durante a pandemia da COVID-19 verificou-se aumento no tempo de uso de mídias eletrônicas (e-media) relacionado a problemas comportamentais. Este trabalho avaliou associações entre tempo de uso de e-media e problemas comportamentais/emocionais em crianças no contexto da pandemia. Contou com uma amostra de 277 responsáveis por crianças de 7-11 anos. Os instrumentos utilizados foram questionário socioeconômico, MAF-P e CBCL/6-18. As análises de correlação de Spearman apontaram associações positivas entre as e-media na MAF-P e escalas do CBCL/6-18. Os jogos adultos correlacionaram-se a 12 escalas; sites para informação/diversão foram associados a seis; apostar na internet a um; e jogos eletrônicos e ver vídeos no YouTube associaram-se a 17 escalas. Conclui-se, de modo geral, que os resultados indicam a necessidade de supervisão para acesso a e-media por crianças, considerando o número, a frequência de uso e o elevado número de problemas emocionais e comportamentais verificados no grupo estudado

Durante la pandemia de COVID-19, hubo un aumento en el tiempo dedicado al uso de medios electrónicos (e-media) relacionados con problemas de comportamiento. Este trabajo evaluó las asociaciones entre el tiempo de uso de los medios electrónicos y los problemas conductuales/emocionales en niños en el contexto de la pandemia. Muestra: 277 tutores de niños de 7 a 11 años. Instrumentos: socioeconómico, MAF-P y CBCL/6-18. Los análisis de correlación de Spearman mostraron asociaciones positivas entre los medios electrónicos en las escalas MAF-P y CBCL/6-18. Juegos para adultos correlacionados con 12 escalas; sitios de información/diversión asociados a 6; apostar en internet a las 1; los juegos electrónicos y ver videos en YouTube se asociaron con 17 escalas. Se concluye, en general, que los resultados indican la necesidad de supervisión para el acceso a e-media por parte de los niños, considerando el número y la frecuencia de uso y el elevado número de problemas emocionales y conductuales verificados en el grupo estudiado

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BACKGROUND: The affective expression of sexual behaviour in individuals with Williams syndrome can lead to risky behaviours, especially if parents do not have information on how to provide sex education or support from specialised professionals. METHOD: The Attitudes to Sexuality Questionnaire for Individuals with Intellectual Disabilities was used to identify parental beliefs, attitudes and concerns about the sexuality and sex education of individuals with intellectual disabilities. The sample comprised 35 parents of individuals with Williams syndrome (mean age 12.8 years (SD = 4.5), 57.1% male). RESULTS: Parents believe in the possibility of marriage and sexual relationships for individuals with intellectual disability when they are older and agree with sexual reproduction in adulthood. Parents consider that sex education, in addition to parental guidance, should be provided by professionals. CONCLUSIONS: This data highlights the need for parents to have clear guidelines on interventions in respect of the sexuality of individuals with intellectual disability.

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The causal relationship between emotional outbursts and emotion dysregulation is proposed to be heterogeneous, but cultural influences have not been considered despite established cultural differences in emotional processes (e.g., increased motivation to suppress emotions in interdependent cultures). Responses to the Brazilian Portuguese version of the Emotional Outburst Questionnaire were collected from 327 caregivers of young people (6-25 years) with autism spectrum disorder, Down's syndrome, or intellectual disability. Responses were compared to a previous sample of 268 responses from the English version of the questionnaire. The latent factor structure of the contextual items was measurement invariant across both versions. The Brazilian responses were classified into three distinct clusters (Sensory Sensitivity; Perceived Safety; Perceived Unsafety) which considerably overlapped with the English clusters.

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O estudo investigou a linguagem escrita de crianças do 2º e 4º anos com e sem sinais de transtorno específico de aprendizagem. Objetivou comparar as escritas entre dois os grupos de alunos, de modo a verificar se a diferença entre eles seria menor nas séries iniciais e tenderia a aumentar nas séries finais, como previsto teoricamente com base no efeito Mateus. Participaram do estudo 51 professores e 1085 alunos, sendo 49 indicados com sinais compatíveis com o transtorno específico de aprendizagem. O instrumento utilizado para analisar a escrita dos alunos foi o Teste de Desempenho Escolar (TDE-II). A partir das análises realizadas, observou-se que o total de palavras certas no ditado do TDE-II foi maior no 4º ano do que no 2º ano para a amostra total, como esperado, e que a diferença entre os alunos com e sem queixas de dificuldades foi maior no 4º ano do que no 2º ano. Análise de post-hoc revelou que não houve diferença estatisticamente significativa entre os alunos com queixas do 2º ano e do 4º ano, ou seja, a diferença entre eles foi tão pequena que não se mostrou significativa. Tal resultado corrobora o efeito Mateus, sugerindo que crianças com dificuldades ao início da escolarização tendem a apresentar menores ganhos e se distanciar cada vez mais dos colegas com habilidades mais desenvolvidas. Logo, estratégias e intervenções precisam ser desenvolvidas para facilitar o desenvolvimento da linguagem escrita das crianças e promover avanços logo ao início da escolarização.

The study investigated writing of 2nd and 4th grade children with and without signs of the specific learning disorder, and aimed to compare the difference in writing between these two groups of students, to analyze whether the difference between them would be smaller in the early grades and would tend to increase in the final grades, as theoretically predicted by Matthew effect. 51 teachers and 1085 students participated in this study, among which 49 had signs compatible with the specific learning disorder. The instrument used to analyze the students writing was the School Performance Test (TDE-II). It was observed that, in general, the total number of correct words in the TDE-II dictation was greater in the 4th grade, compared to the 2nd grade, as expected, and the difference between students with and without complaints of difficulties was greater among the students in 4th grade than among 2nd grade students. Post-hoc tests showed that there was no statistically significant difference between 2nd grade students with complaints and 4th grade students of with complaints, that is, the difference between them was so small that it was not significant. This result corroborates the "Matthew effect": children with difficulties at the beginning of schooling tend to present lower gains and, increasingly, distance themselves from colleagues with more developed skills. Thus, strategies and interventions must be designed to facilitate children's literacy and promote advances at beginning of schooling.

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Prader-Willi syndrome (PWS) is a complex genetic disorder requiring interdisciplinary team monitoring and intensive care by parents. So far there is little information on people with PWS in Brazil. Our aim was to describe health problems and treatments used by people with PWS in Brazil and their relationship to their parents' quality of life. Parents answered questionnaires about their child's medical and exercise history, behavior problems, sociodemographic characteristics, and their own quality of life. Results: The responses of the participants showed similar health problems as in other countries. Anxiety and tantrums were the behavioral problems most commonly cited by parents. Parents of people with PWS had lower scores in respect of quality of life than the Brazilian population. Behavioral problems in individuals with PWS were negatively associated with their parents' quality of life. Behavioral and medical conditions in the children were associated with reduced quality of life in the parents. We conclude that heath care should not only be directed toward those with PWS, but also their parents.

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BACKGROUND: Williams-Beuren syndrome is a multisystemic disorder caused by a microdeletion of the 7q11.23 region. Although familial cases with autosomal dominant inheritance have been reported, the vast majority are sporadic. OBJECTIVE: To investigate the main complaints and clinical findings of patients with Williams-Beuren syndrome. METHODS: A total of 757 parents of patients registered in the Brazilian Association of Williams-Beuren Syndrome (ABSW) received a questionnaire via WhatsApp from March to July 2017. RESULTS: In total, 229 parents answered the survey. Age of diagnosis ranged from 2 days to 34 years (median: 3 years). The main clinical findings reported by the parents were abdominal colic (83.3%), failure to thrive (71.5%), feeding difficulty in the first year (68.9%), otitis (56.6%), urinary tract infections (31.9%), precocious puberty (27.1%) and scoliosis (15.9%). Cardiac defects were present in 66% of patients, and the most frequent defect was supravalvular aortic stenosis (36%). Arterial hypertension was reported in 23%. Hypercalcemia was reported in 10.5% of patients, mainly during the first year of life. Hyperacusis and hypersociability were common complaints (both present in 89%). Other behavioral and neuropsychiatric symptoms reported by the parents included attention deficit (89%), anger crises (83%), excessive fear (66%), depression (64%), anxiety (67%) and hypersexuality (33%). The most common complaints were hypersensitivity to sounds, talkative personality, emotional dependence and learning difficulties. In 98.3%, the parents denied family history. CONCLUSIONS: Williams-Beuren syndrome requires close follow-up with different medical specialties due to their variable clinical comorbidities, including language and school learning difficulties, behavioral and psychiatric problems.

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ABSTRACT Background: Williams-Beuren syndrome is a multisystemic disorder caused by a microdeletion of the 7q11.23 region. Although familial cases with autosomal dominant inheritance have been reported, the vast majority are sporadic. Objective: To investigate the main complaints and clinical findings of patients with Williams-Beuren syndrome. Methods: A total of 757 parents of patients registered in the Brazilian Association of Williams-Beuren Syndrome (ABSW) received a questionnaire via WhatsApp from March to July 2017. Results: In total, 229 parents answered the survey. Age of diagnosis ranged from 2 days to 34 years (median: 3 years). The main clinical findings reported by the parents were abdominal colic (83.3%), failure to thrive (71.5%), feeding difficulty in the first year (68.9%), otitis (56.6%), urinary tract infections (31.9%), precocious puberty (27.1%) and scoliosis (15.9%). Cardiac defects were present in 66% of patients, and the most frequent defect was supravalvular aortic stenosis (36%). Arterial hypertension was reported in 23%. Hypercalcemia was reported in 10.5% of patients, mainly during the first year of life. Hyperacusis and hypersociability were common complaints (both present in 89%). Other behavioral and neuropsychiatric symptoms reported by the parents included attention deficit (89%), anger crises (83%), excessive fear (66%), depression (64%), anxiety (67%) and hypersexuality (33%). The most common complaints were hypersensitivity to sounds, talkative personality, emotional dependence and learning difficulties. In 98.3%, the parents denied family history. Conclusions: Williams-Beuren syndrome requires close follow-up with different medical specialties due to their variable clinical comorbidities, including language and school learning difficulties, behavioral and psychiatric problems.

RESUMO Antecedentes: A síndrome de Williams-Beauren é doença de acometimento multisistêmico causado pela microdeleção da região 7q11.23. Apesar de haver casos familiares com herança autossômica dominante, a grande maioria dos casos é esporádica. Objetivo: Investigar as principais queixas e achados clínicos da síndrome. Métodos: 757 pais de pacientes inscritos na Associação Brasileira de Síndrome de Williams-Beuren (ABSW) receberam um questionário pelo WhatsApp, entre março e julho de 2017. Resultados: 229 pais de pacientes responderam à pesquisa. A idade de diagnóstico variou de 2 dias até 34 anos (mediana: 3 anos). Os principais achados reportados pelos pais: cólicas abdominais (83,3%), deficiência ponderoestatural (71,5%), dificuldade de alimentação no primeiro ano (68,9%), otite (56,6%), infecções do trato urinário (31,9%), puberdade precoce (27,1%) e escoliose (15,9%). Cardiopatias estavam presentes em 66%, sendo que a mais frequente era a estenose pulmonar supravalvar (36%). Hipertensão arterial foi reportada em 23%. Hipercalcemia foi reportada em 10,5%, principalmente no primeiro ano de vida. Hiperacusia e hiperssociabilidade foram achados comuns (89%). Os principais achados comportamentais e psiquiátricos reportados pelos pais foram: déficit de atenção (89%), crises de raiva (83%), medo excessivo (66%), depressão (64%), ansiedade (67%) e hiperssexualidade (33%). As queixas principais referidas foram hipersensibilidade a sons, personalidade excessivamente amigável, dependência emocional e dificuldades escolares. Em 98,3% dos casos os pais negaram história familial. Conclusões: A síndrome de Williams-Beuren é requer um seguimento e manejo estritos, com diferentes especialidades médicas devido às comorbidades clínicas variadas, que incluem dificuldades de linguagem e aprendizagem escolar, além de dificuldades comportamentais e psiquiátricas.

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ABSTRACT Objective: Voluntary and automatic orienting of attention enable proper processing of environmental information. Few studies have assessed how this process varies during development in children with attention deficit hyperactivity disorder (ADHD). Methods: This study analyzed voluntary and automatic orienting in 30 children with ADHD and 30 age and sex matched controls (Control group-CG). Two experiments assessed voluntary and automatic orienting by recording reaction times (RT) to conditions in relation to temporal interval, spatial position, cue validity and age. The RT medians calculated for each condition and participant were analyzed using ANOVA to compare ADHD and CG. Results: Children with ADHD exhibited globally higher RT than the CG group. They also showed prejudices during the reorienting process and demonstrated adequate voluntary orienting for shorter intervals. In automatic task, there was no group interaction, expressing early facilitation, but not inhibition of return. Conclusion: These results identify correlations of ADHD and the children's age in relation to voluntary and automatic orienting of attention.