-Background: Rheumatic chorea (RC) has recently been linked to an antibody-mediated immune mechanism. Objective/Method: To verify if this knowledge reflected in management changes we conceived a descriptive study. Results: The medical charts of 20 children (13 females) aged 6 to 12 years (mean 8 years), diagnosed as RC from June 1996 to June 1999, were reviewed. All patients received some medical treatment. Haloperidol was the most prescribed medication (15 patients -75 %). Sulpiride, diazepam and valproate were also used as symptomatic treatment. Imune-modulating therapy with prednisone was prescribed for seven children. The shortest course of chorea (16 days) occurred in a patient treated with prednisone. Conclusion: Prednisone has been prescribed for rheumatic chorea besides the traditional symptomatic approach. A great variety of antichoreic drugs are being employed.KEY WORDS: chorea, rheumatic fever, therapy, prognosis. Conduta na coréia reumática: um estudo observacional RESUMO -Fundamentos:A patogenia da coréia reumática relaciona-se a um mecanismo imunomediado. Objetivos/Métodos: A fim de verificar se este conhecimento resultou em mudanças na terapêutica desta doença, elaborou-se um estudo descritivo. Resultados: Foram revistos os prontuários de 20 crianças (13 meninas) entre 6 e 12 anos de idade (média 8 anos), com diagnóstico de coréia reumática realizado entre Junho de 1996 e Junho de 1999. Todos pacientes utilizaram algum tipo de medicação. Haloperidol foi a substância mais prescrita (15 pacientes -75 %). Sulpiride, diazepam e ácido valpróico foram também utilizados.

Early dietary treatment of phenylketonuria (PKU), an inborn error of phenylalanine (Phe) metabolism, results in normal cognitive development. Although health-related quality of life (HRQoL) of PKU patients has been reported as unaffected in high-income countries, there are scarce data concerning HRQoL and adherence to treatment of PKU children and adolescents from Brazil. The present study compared HRQoL scores in core dimensions of Brazilian early-treated PKU pediatric patients with those of a reference population, and explored possible relationships between adherence to treatment and HRQoL. Early-treated PKU pediatric patient HRQoL was evaluated by self- and parent-proxy reports of the Pediatric Quality of Life Inventory (PedsQL) core scales. Adherence to treatment was evaluated by median Phe levels and percentage of results within the therapeutic target range in two periods. Means for total and core scales scores of PedsQL self- and parent proxy-reports of PKU patients were significantly lower than their respective means for controls. Adequacy of median Phe concentrations and the mean percentage of values in the target range fell substantially from the first year of life to the last year of this study. There was no significant difference in mean total and core scale scores for self- and parent proxy-reports between patients with adequate and those with inadequate median Phe concentrations. The harmful consequences for intellectual capacity caused by poor adherence to dietary treatment could explain the observed decrease in all HRQoL scales, especially in school functioning. Healthcare system and financial difficulties may also have influenced negatively all HRQoL dimensions.

Epilepsy is one of the most prevalent chronic disorders of childhood which can threaten child development and mental health. Among cognitive disorders, dyscalculia is one of the most important. In this study, 39 children and adolescents with idiopathic epilepsy underwent clinical and neuropsychological assessment to determine the intellectual level, math skills, reading and writing performance and neuropsychological profile. It was observed that the mathematical ability was below schooling expectations in a higher frequency than expected. There were no significant differences in mathematical performance among groups divided by number of antiepileptic drugs used, duration of disease and types and frequency of seizures. There was a positive correlation with intelligence quotient and attentional and reading level. These results suggest the existence not only of dyscalculia, but the concurrence of attentional and reading problems for the poor mathematical performance in this population.Keywords: mathematical ability, dyscalculia, epilepsy, children, adolescents. RESUMOA epilepsia é uma das doenças crônicas mais comuns da infância que pode ameaçar o desenvolvimento infantil e saúde mental. Dos distúrbios cognitivos, a discalculia é um dos mais importantes. Neste estudo, 39 crianças e adolescentes com epilepsia idiopática, foram submetidos à avaliação clínica e neuropsicológica para determinar o nível intelectual, habilidade em matemática, leitura e escrita e perfil neuropsicológico. Observou-se que a habilidade em matemática ficou abaixo das expectativas de escolarização em uma frequência maior do que a esperada. Não houve diferenças significativas no desempenho matemático em relação ao número de drogas antiepilépticas utilizadas, duração da doença, tipo e frequência das crises. Houve uma correlação positiva com o quociente de inteligência e nível de atenção e leitura. Estes resultados sugerem a existência não só de discalculia, mas também a existência de problemas atencionais e de leitura que fatores causais para a dificuldade matemática apresentada nesta amostra.Palavras-chave: habilidade em matemática, discalculia, epilepsia, crianças, adolescentes.Epilepsy is a chronic neurologic disorder characterized by unprovoked recurrent seizures 1 . Estimation shows that around 5 and 10 million children worldwide have active epilepsy, representing about 25% of the global epilepsy population 2 . Forty percent of the people who develop epilepsy per year are younger than 15 years and more than 80% live in developing countries 2 . Population-based studies on childhood-onset epilepsy suggest an annual incidence of 61-124/ 100,000 in developing countries and 41-50/100,000 in developed countries 2 .Epilepsy is also related to behavioral, cognitive and psychiatric disorders, including autism and intellectual disability in 30% of patients 3,4 . There is a higher incidence of several cognitive disorders, in spite of normal intelligence,