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Abstract Background Down syndrome is the most commonly genetic cause of developmental delay and intellectual disability, affecting 1:700 live births. It is associated with heart disease and recurrent infections, among other complications that greatly impair the patient's quality of life. Objective To evaluate the major factors associated with quality of life in a cohort of patients with Down syndrome. Methods We assessed 1,187 patients with Down syndrome, older than 4 years old, with an adaptation of the Personal Outcomes Scale validated for Portuguese language, interviewing patients, parents, and caregivers. Results A bad quality of life was reported in 56.4% of the sample. The main factors associated with better quality of life were female sex, first medical visit before 4 months old, higher parental education, a professionally active mother, and prenatal care. The main factors associated with worse quality of life were family history of alcohol abuse and psychiatric disorders and comorbidity with autism and epilepsy. Conclusion Clinical comorbidities such as autism and epilepsy carry a heavy burden among patients with Down syndrome, while factors related to family support, such as employment status and educational background of the parents, enhance quality of life. The factors associated with quality of life among patients with Down syndrome should be adequately evaluated in medical consultation and targeted in public health policies.
Resumo Antecedentes A síndrome de Down é a mais comum causa identificável de atraso de desenvolvimento e deficiência intelectual, afetando 1 a cada 700 nascidos vivos. Está associada a cardiopatias, infecções recorrentes e outras complicações que impactam significativamente a qualidade de vida dos pacientes. Objetivo Avaliar os principais fatores associados a qualidade de vida em uma coorte de pacientes com Síndrome de Down. Métodos Avaliamos 1.187 pacientes com síndrome de Down com mais de 4 anos de idade utilizando uma adaptação da versão validada para o português da Escala Pessoal de Resultados, entrevistando pacientes, pais e cuidadores. Resultados Uma má qualidade de vida foi encontrada em 56.4% da amostra. Os principais fatores associados à melhor qualidade de vida foram sexo feminino, primeira consulta médica antes dos 4 meses de idade, maior nível educacional dos pais, mãe profissionalmente ativa e atenção pré-natal. Os principais fatores associados à pior qualidade de vida foram o histórico familiar de abuso de álcool e distúrbios psiquiátricos, além de comorbidade com autismo e epilepsia. Conclusão As comorbidades clínicas como autismo e epilepsia levam a um maior impacto entre os pacientes com síndrome de Down, enquanto fatores relacionados ao apoio familiar, como situação profissional e formação educacional dos pais, estão associados à melhor qualidade de vida. Os fatores associados à qualidade de vida de pacientes com síndrome de Down devem ser adequadamente avaliados em consulta médica e alvo de políticas públicas de saúde.
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Abstract Objective: The aim of this current report was to present a critical review of the use of cannabidiol (CBD) in the treatment of refractory epilepsies in the pediatric population. Data source: Literature review was carried out in the Medline (PubMed), Cochrane, and Scientific Electronic Library Online (SciELO) databases with the descriptors "Cannabidiol" and "Epilepsy." The search was not limited by the date of publication, language, or study design. A total of 69 articles were included in the review. Data synthesis: The efficacy of CBD in treating epileptic seizures has been confirmed by randomized controlled trials for Lennox-Gastaut syndrome, Dravet syndrome, and tuberous sclerosis complex. The incidence of side effects reported in subjects of the studies is high. However, most studies indicate a good safety profile and tolerance to the drug, with most of the adverse effects being mild to moderate and transient. Conclusions: There is no consensus on the release of CBD as a therapeutic tool by the drug regulatory agencies worldwide. However, the use of CBD is promising since it has presented satisfactory results in crisis control in well-designed studies. In addition, this drug has a good safety and tolerance profile. However, further studies with a long follow-up period are needed to confirm its usefulness and the long-term safety in pediatric patients.
RESUMO Objetivo: Apresentar uma revisão crítica do uso de canabidiol no tratamento de epilepsias refratárias na população pediátrica. Fontes de dados: Revisão da literatura realizada nas bases de dados Medical Literature Analysis and Retrieval System Online (Medline/PubMed), Cochrane e Scientific Electronic Library Online (SciELO), com os descritores "cannabidiol" e "epilepsy". A busca não foi limitada por data de publicação, idioma ou desenho do estudo. Sessenta e nove artigos foram incluídos na revisão. Síntese dos dados: A eficácia do canabidiol no tratamento de crises epilépticas foi confirmada por ensaios clínicos randomizados para síndrome de Lennox-Gastaut, síndrome de Dravet e complexo de esclerose tuberosa. A incidência de efeitos colaterais relatados nos indivíduos dos estudos é alta. No entanto, a maioria dos estudos indica um bom perfil de segurança e tolerância ao medicamento, com efeitos adversos leves a moderados e transitórios. Conclusões: Não há consenso sobre a liberação do canabidiol como ferramenta terapêutica nas diversas agências reguladoras de medicamentos em todo o mundo. Entretanto, seu uso é promissor, uma vez que apresentou resultados satisfatórios no controle de crises em estudos bem delineados. Além disso, esse medicamento apresenta um bom perfil de segurança e tolerância. No entanto, mais estudos com longo período de acompanhamento são necessários para confirmar sua utilidade e sua segurança em longo prazo em pacientes pediátricos.
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Abstract Introduction Nowadays, there is no consensus on whether central auditory processing disorder is a primary or a secondary deficit to other cognitive deficits. A better understanding of the association between cognitive functions and central auditory skills may help elucidate this dilemma. Objective To investigate possible associations between auditory abilities and cognitive functions in schoolchildren. Methods Fifty-eight schoolchildren, aged between 8 years and 0 months old and 11 years and 11 months old, who underwent the following tests: masking level difference, gaps in noise, pitch pattern sequence test, dichotic digits test, sustained auditory attention ability test, Wechsler intelligence scale for children - IV, junior Hayling test, five digits test, and behavior rating inventory of executive function. Results Significant correlations were found between the hearing ability of temporal resolution and executive functions, temporal ordering/sequencing, binaural integration and separation, and sustained auditory attention, operational memory, inhibitory control, and cognitive flexibility; binaural integration was also associated with intelligence. The statistically significant positive correlation found between the ability of binaural interaction and the components of emotional control and behavior regulation of the behavior rating inventory of executive function was unexpected. Conclusion The associations identified reinforce the complexity of the tasks involved in the evaluation of central auditory processing and the need for multidisciplinary evaluation for the differential diagnosis of auditory processing disorder. Confirmation of the presence or absence of comorbidities between different disorders allows directing the therapeutic behaviors and reducing the impact of possible auditory and/or cognitive deficits in the different daily life situations of children.
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Observa-se um aumento progressivo da evasão escolar e do fracasso acadêmico. A demanda por avaliação e diagnóstico sobre questões de aprendizagem está crescendo nos serviços públicos de saúde. Para direcionar os métodos de intervenção e minimizar as dificuldades de aprendizagem, é necessário identificar os fatores que levam ao fracasso escolar e diferenciar as dificuldades escolares. O presente estudo tem como objetivo descrever o modelo de avaliação interdisciplinar realizado no Ambulatório de Transtornos de Aprendizagem (ATA) do Centro de Neuropediatria do Hospital das Clínicas da Universidade Federal do Paraná e relatar três casos avaliados. Os dados apresentados neste estudo baseiam-se em 56 avaliações realizadas em crianças e adolescentes com queixas de dificuldades de aprendizagem, a fim de caracterizar a população atendida, o modelo de avaliação aplicado e as conclusões da equipe interdisciplinar. Nesta amostra, a maior frequência foi do sexo masculino (78,6%), com idade média de 10 anos e 5 meses. As dificuldades escolares foram percebidas em 44,6% dos casos, o transtorno de aprendizagem em 7,1% e outros transtornos em 17,9%. Esses resultados coincidem com outros estudos sobre dificuldades de aprendizagem e demonstram que o modelo utilizado pelo ATA é eficaz para concluir sobre a proposta diagnóstica e de intervenção.
There is a progressive increase of student evasion as well as of academic failure. The demand for evaluation and diagnosis about learning issues is growing up in the public health service care. In order to direct the intervention methods and minimize the learning difficulties is necessary identify the factors that lead to academic failure and differentiate the learning disabilities. The present study aims to describe the interdisciplinary model for evaluation performed at the Learning Disorder Clinic (ATA) of the Neuropediatrics Center at Hospital das Clínicas from Universidade Federal do Paraná and report three evaluated cases. The data presented in this study are based on 56 evaluations performed in children and adolescents with complaints of learning difficulties in order to characterize the population served, the evaluation model applied and the conclusions from the interdisciplinary team. On this sample, the highest frequency was from males (78.6%) aged on average 10 years and 5 months. The learning difficulties was noticed in 44.6% of the cases, the leaning disorder in 7.1% and other disorders in 17.9%. These results are in agreement with other studies about learning disabilities and demonstrates that the model used by ATA is effective to conclude about diagnostic and intervention proposal.
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RESUMO: Objetivo: A participação em atividades esportivas e recreativas promove a inclusão e a qualidade de vida (QV) de crianças/adolescentes com deficiência. Este estudo visa avaliar e descrever o efeito do esporte adaptado (EA) na QV e o perfil biopsicossocial de crianças/adolescentes com paralisia cerebral (PC). Métodos: Foram avaliados e encaminhados ao EA (futebol e natação) 47 crianças e adolescentes com PC. A QV foi avaliada pelo Instrumento para Avaliação de Resultados de Reabilitação em Pediatria (IARPP) e o perfil biopsicossocial pela Lista de Verificação Comportamental para Crianças/Adolescentes (CBCL). Foram verificadas as influências de sexo, idade, raça, renda, escolaridade e topografia da espasticidade. Resultados: Dezessete crianças/adolescentes praticaram o EA e foram reavaliados após um ano. Foi observada melhora significativa nas dimensões transferências e mobilidade (p=0,009), função e extremidade superior (p=0,021) e função global (p=0,004) do IARRP. Houve melhora significativa considerando as síndromes problemas de atenção (p=0,026) e problemas de déficit de atenção e hiperatividade (p=0,008) na análise do Manual Diagnóstico e Estatístico de Transtornos Mentais (DSM) Orientado (CBCL). Crianças com diplegia obtiveram mais benefício que aquelas com hemiplegia em relação às dimensões dor e conforto (p=0,02) e dimensão global (p=0,027) (IARPP). Os meninos apresentaram maiores escores em total de competência (p=0,048); o grupo extremamente pobre obteve maiores índices na síndrome quebrar regras (p=0,008). Conclusão: O EA apresentou efeito positivo na QV e no perfil biopsicossocial das crianças e dos adolescentes com PC dessa amostra, especialmente considerando: função global e de extremidades superiores, capacidade para transferências e mobilidade, e benefícios nos problemas relacionados às dificuldades na atenção.
ABSTRACT Objective: The participation in sports and recreational activities promotes inclusion and the quality of life (QOL) for people with some type of disability. This study aims to evaluate and describe the effect of adapted sports (AS) on the QOL and biopsychosocial profile of children/adolescents with cerebral palsy (CP). Methods: Forty-seven children/adolescents with CP were evaluated and referred to AS (soccer and swimming). The QOL was evaluated by the Pediatric Outcome Data Collection Instrument (PODCI) and the biopsychosocial profile by the Behavior Checklist for Children/Adolescents (CBCL). These instruments considered the influence of gender, age, race, social income, education and topography of spasticity. Results: Seventeen children/adolescents who practiced AS were re-evaluated after one year. There was significant improvement in the dimensions of transfers and mobility (p=0.009), upper extremity function (p=0.021) and global function (p=0.004) of IARRP. There was significant improvement considering the attention disorder syndrome (p=0.026), and the attention deficit hyperactivity disorders (p=0.008) in the Diagnostic and Statistical Manual of Mental Disorders (DSM)-oriented analysis (CBCL). Children/adolescents with diplegia obtained greater benefit than those with hemiplegia in relation to the comfort and pain (p=0.02) and global dimension (p=0.027) (PODCI). The boys had higher scores in total competence (p=0.048); the extremely poor group obtained higher levels in the breaking rules syndrome (p=0.008). Conclusions: The AS had a positive effect on the QOL and biopsychosocial profile of children/adolescents with CP in this sample, especially considering the global and upper extremity function, capacity for transfers and mobility, and benefits in the problems related to difficulties in attention.
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Humans , Male , Female , Child , Adolescent , Quality of Life , Sports , Cerebral Palsy/psychology , Prospective StudiesABSTRACT
With this article we intend to demonstrate the importance of evaluation and follow up of children with learning disabilities, through a multidisciplinary team. As well as to establish the need of intervention. We evaluate 69 children, from Aline Picheth Public School, in Curitiba, attending first or second grade of elementary school, through general and evolutionary neurological examination, pediatric checklist symptoms, and social, linguistic and psychological (WISC-III, Bender Infantile and WPPSI-figures) evaluation. The incidence was higher in boys (84,1 percent), familiar history of learning disabilities was found in 42 percent, and writing abnormalities in 56,5 percent. The most frequent diagnosis was attention deficit and hyperactivity disorder, in 39,1 percent. With this program, we aimed to reduce the retention taxes and stress the importance of this evaluation, and, if necessary, multidisciplinar intervention in the cases of learning disabilities