Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/west syndrome) and immunity

West syndrome is a severe epilepsy, occurring in infancy, that comprises epileptic seizures known as spasms, in clusters, and a unique EEG pattern, hypsarrhythmia, with psychomotor regression. Maturation of the brain is a crucial component. The onset is within the first year of life, before 12 months of age. Patients are classified as cryptogenic (10 to 20%), when there are no known or diagnosed previous cerebral insults, and symptomatic (80 to 90%), when associated with pre-existing cerebral damages. The time interval from a brain insult to infantile spasms onset ranged from 6 weeks to 11 months. West syndrome has a time-limited natural evolutive course, usually disappearing by 3 or 4 years of age. In 62% of patients, there are transitions to another age-related epileptic encephalopathies, the Lennox-Gastaut Syndrome and severe epilepsy with multiple independent foci. Spontaneous remission and remission after viral infections may occur. Therapy with ACTH and corticosteroids are the most effective. Reports about intravenous immunoglobulins action deserve attention. There is also immune dysfunction, characterized mainly by anergy, impaired cell-mediated immunity, presence of immature thymocytes in peripheral blood, functional impairment of T lymphocytes induced by plasma inhibitory factors, and altered levels of immunoglobulins. Changes in B lymphocytes frequencies and increased levels of activated B cells have been reported. Sensitized lymphocytes to brain extract were also described. Infectious diseases are frequent and may, sometimes, cause fatal outcomes. Increase of pro-inflamatory cytokines in serum and cerebrospinal fluid of epileptic patients were reported. Association with specific HLA antigens was described by several authors (HLA-DR7, HLA-A7, HLA-DRw52, and HLA-DR5). Auto-antibodies to brain antigens, of several natures (N-methyl-d-aspartate glutamate receptor, gangliosides, brain tissue extract, synaptic membrane, and others), were described in epileptic patients and in epileptic syndromes. Experimental epilepsy studies with anti-brain antibodies demonstrated that epileptiform discharges can be obtained, producing hyperexcitability leading to epilepsy. We speculate that in genetically prone individuals, previous cerebral lesions may sensitize immune system and trigger an autoimmune disease. Antibody to brain antigens may be responsible for impairment of T cell function, due to plasma inhibitory effect and also cause epilepsy in immature brains. © 2008 Bentham Science Publishers Ltd.

Cardiovascular and autonomic alterations in rats with parkinsonism induced by 6-OHDA and treated with L-DOPA

Evaluate the effects caused by L-DOPA on cardiovascular and autonomic parameters in an animal model of Parkinsonism induced by 6-hydroxydopamine (6-OHDA).Adult male Wistar rats were subjected to bilateral microinfusion of 6-OHDA or saline (sham group) in the substantia nigra, and treated by gavage with L-DOPA or water for 7days after surgery. On the 6th day the rats were subjected to femoral artery catheterization for cardiovascular recording. Mean arterial pressure (MAP) and heart rate (HR) were evaluated at baseline and during head up tilt (HUT) protocol. Spectral analysis of cardiovascular variability was performed using the V2.4 CardioSeries software v2.4. The lesion was quantified by dopamine levels in the striatum.Dopamine levels in the striatum were decreased in 6-OHDA rats (sham: 4.79±0.49ng/mg; 6-OHDA: 1.99±0.68ng/mg) and were not recovered by Prolopa treatment. Baseline values of MAP and HR were not different between groups. HUT induced an increase in MAP and HR (ΔMAP: 17±1mmHg, ΔHR: 39±4bpm) that were attenuated in 6-OHDA and in Prolopa treated animals. At baseline, the systolic arterial pressure (SAP) variance was lower in the 6-OHDA and sham Prolopa groups. Spontaneous baroreflex sensitivity was higher at baseline in the 6-OHDA group as compared to all studied groups.Our data suggest that treatment with Prolopa did not interfere with cardiovascular variables at baseline. However, during HUT, the 6-OHDA and Prolopa control animals presented a lower cardiovascular compensation, suggesting a possible autonomic impairment in Parkinsonism induced by 6-OHDA.

Chemoreflex and baroreflex alterations in Parkinsonism induced by 6-OHDA in unanesthetized rats

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

A ação pedagógica sob a perspectiva de Henri Wallon

This research was supported theory as the theory of Henri Wallon which proposes a reflection on the overall development of the child offering subsidies for a acting that values the child in its multiple dimensions. The objective is understood to seek subsidies in theory that can guide the construction of an education in the Children's activites, focused on the development aspects of cognitive, affective and motor of the child. It adopts as methodology the bibliographical revision, the intervention, observation and data collect, using as pedagogic resource playing activities in an institution of Infantile Education. The research noted the possibility of action with the concepts proposed by the Wallonian theory, furthermore, the Children's Fitness, using the recreational activities such as teaching resources boost child development. Therefore, concluded that physical education should include Child in the process of teaching-learning the full development of the child.

Analisando conhecimentos e práticas de agentes educacionais e professoras relacionados à alimentação infantil

Objetivou-se identificar os conhecimentos e as práticas relacionados à alimentação de crianças entre zero e três anos, a fim de complementar a formação das profissionais que trabalham com crianças, na área da educação nutricional. O procedimento metodológico seguiu as orientações do estudo de caso, com abordagem qualitativa. Os saberes e as práticas das participantes do estudo foram analisados por meio de questões abertas e fechadas e das observações dos tópicos relacionados à nutrição infantil, juntamente com os dados sobre a formação dessas profissionais. Pode-se concluir que tanto as agentes educacionais quanto as professoras de Ensino Infantil são detentoras de um conhecimento sobre Nutrição Infantil, porém a preocupação maior destas foi atender as necessidades nutricionais das crianças por meio de uma boa alimentação, deixando, em segundo plano, as atividades pedagógicas, as quais poderiam ser realizadas em um programa de educação nutricional, construído coletivamente e inserido no projeto político-pedagógico da escola.

Honey consumption in the state of São Paulo: a risk to human health?

Infantile botulism was recognized in 1976 as a paralyzing disease caused by the ingestion of viable spores that would germinate and colonize the intestinal tract of infants, with local production and absorption of Clostridium botulinum toxin. The possible origins of botulinic spores are dust and honey, which has been identified as a dietary risk factor for infantile botulism. The objectives of the present study were to investigate 100 honey samples obtained in the state of São Paulo (Brazil) in terms of incidence of botulinic spores and of microbiologic quality, in agreement with Decree 367/9. All 100 samples analysed were negative for the presence of Salmonella, Shigella, total coliforms. C botulinum spores were present in 3 samples (3%) and molds and yeasts, in 64 samples (64%), but only 25 (25%) exceeded established criteria, with counts ranging from zero to 1.5 x 10(5) CFU/g. The presence of small sporogenic Gram-positive rods was observed in 42 (42%) of the 100 samples tested but these bacteria were not identified. (C) 2003 Elsevier Ltd. All rights reserved.

Formação e atitude dos professores de educação infantil sobre violência familiar contra criança

A violência contra crianças é apontada como uma das principais causas de morbi-mortalidade na infância e deve ser identificada por todos os profissionais que lidam com esse público, inclusive os professores. Objetivou-se avaliar o preparo dos professores de educação infantil, no que se refere à percepção e notificação dos casos de violência contra criança. Foram visitadas todas as instituições de ensino infantil de Araçatuba-SP, e os profissionais que consentiram (n=236), os quais responderam a um questionário sobre o tema. Os dados foram analisados quanti-qualitativamente. 91,1% se consideram responsáveis pela notificação e 86,9% se dizem capazes de identificar agressões. 80,9% declararam ter recebido informações a respeito da violência contra criança, entretanto, 67,7% sentem-se inseguros quanto à identificação dos casos. Conclui-se que, mesmo possuindo formação para proceder ante a violência, a maioria dos pesquisados ainda não se sente preparada, o que pode gerar negligência.

A criança disfônica: diagnóstico, tratamento e evolução clínica

Os nódulos vocais são causas freqüentes de disfonias infantis, porém, através das vídeoendoscopias, outras lesões têm sido diagnosticadas na laringe infantil, como os cistos, sulcos, pontes e micromembranas, denominadas de lesões estruturais mínimas. OBJETIVO: Avaliar, em 71 crianças com disfonia agendadas nos ambulatórios de Foniatria da Faculdade de Medicina de Botucatu (Unesp) nos últimos cinco anos: sexo, idade, diagnósticos, tratamentos e evolução clínica. FORMA DE ESTUDO: Clínico retrospectivo. CASUÍSTICA E MÉTODO: Realizou-se estudo retrospectivo das avaliações otorrinolaringológicas e endoscópicas de 71 crianças com queixas de disfonia e idade entre 3 a 13 anos (45 meninos e 26 meninas). As crianças haviam sido submetidas a exame endoscópico com telescópio rígido ou nasofibroscópio flexível. Foram excluídas as crianças com disfonias funcionais ou com queixas de obstrução respiratória. RESULTADOS: Os principais diagnósticos foram: nódulo vocal (47 casos; 66,2%), cisto epidérmico (7 casos; 9,9%), cisto aberto fistulizado (6 casos; 8,4%), sulco vocal (5 casos; 7,1%), ponte + cisto (2 casos; 2,8%), micromembrana anterior (2 casos; 2,8%) e pólipo vocal (2 casos; 2,8%). As disfonias foram mais freqüentes entre os meninos (63,3%). A fonoterapia isolada foi o tratamento de escolha nos casos de nódulo vocal (evolução favorável), sulco vocal (evolução desfavorável) e micro-membrana (evolução desfavorável). Nas demais lesões (cistos, pontes e pólipos) o tratamento de escolha foi a cirurgia associada à fonoterapia, com resultados satisfatórios, exceto nos casos que recusaram a cirurgia. CONCLUSÃO: Os nódulos vocais são as principais causas de disfonias em crianças, porém as lesões estruturais mínimas mostraram-se também freqüentes, exigindo exame minucioso para o diagnóstico.

Experiences of family members regarding the oral health care of children

Este estudo objetivou verificar a compreensão das experiências dos familiares em relação ao cuidado com a saúde bucal das crianças. É estudo qualitativo, realizado em 2007, em distrito de saúde do município de Ribeirão Preto, SP, com 12 cuidadores. Utilizou-se referencial teórico da vulnerabilidade e a perspectiva hermenêutica. Três categorias empíricas foram elaboradas: os significados do cuidado com a saúde bucal, em busca das causas e da prevenção de agravos bucais e a realidade dos serviços de saúde bucal. Entre outros elementos potencializadores da vulnerabilidade infantil aos agravos bucais, emergiu a supervalorização da causalidade biológica, do atendimento de alta complexidade e da odontologia estética e, entre os protetores, a valorização do saber popular e a integração de ações e conhecimentos profissionais. Aponta-se para a revisão das estratégias de prevenção e promoção de saúde bucal, fornecendo elementos para auxiliar os serviços de saúde a reorganizarem o cuidado com a saúde bucal de crianças.

Kinetic characterization of hypophosphatasia mutations with physiological substrates

We have analyzed 16 missense mutations of the tissue-nonspecific AP (TNAP) gene found in patients with hypophosphatasia. These mutations span the phenotypic spectrum of the disease, from the lethal perinatal/infantile forms to the less severe adult and odontohypophosphatasia. Site-directed mutagenesis was used to introduce a sequence tag into the TNAP cDNA and eliminate the glycosylphosphatidylinositol (GPI)-anchor recognition sequence to produce a secreted epitope-tagged TNAP (setTNAP). The properties of GPI-anchored TNAP (gpiTNAP) and setTNAP were found comparable. After introducing each single hypophosphatasia mutation, the setTNAP and mutant TNAP cDNAs were expressed in COS-1 cells and the recombinant flagged enzymes were affinity purified. We characterized the kinetic behavior, inhibition, and heat stability properties of each mutant using the artificial substrate p-nitrophenylphosphate (pNPP) at pH 9.8. We also determined the ability of the mutants to metabolize two natural substrates of TNAP, that is, pyridoxal-5'-phosphate (PLP) and inorganic pyrophosphate (PPi), at physiological pH. Six of the mutant enzymes were completely devoid of catalytic activity (R54C, R54P, A94T, R206W, G317D, and V365I), and 10 others (A16V, A115V, A160T, A162T, E174K, E174G, D277A, E281K, D361V, and G439R) showed various levels of residual activity. The A160T substitution was found to decrease the catalytic efficiency of the mutant enzyme toward pNPP to retain normal activity toward PPi and to display increased activity toward PLP. The A162T substitution caused a considerable reduction in the pNPPase, PPiase, and PLPase activities of the mutant enzyme. The D277A mutant was found to maintain high catalytic efficiency toward pNPP as substrate but not against PLP or PPi. Three mutations ( E174G, E174K, and E281K) were found to retain normal or slightly subnormal catalytic efficiency toward pNPP and PPi but not against PLP. Because abnormalities in PLP metabolism have been shown to cause epileptic seizures in mice null for the TNAP gene, these kinetic data help explain the variable expressivity of epileptic seizures in hypophosphatasia patients.

Random amplification of polymorphic DNA reveals clonal relationships among enteropathogenic Escherichia coli isolated from non-human primates and humans

Enteropathogenic Escherichia coli ( EPEC) strains are important agents of infantile diarrhea all over the world, gaining even greater importance in developing countries. EPEC have also been isolated from various animal species, but most isolates belong to serotypes that differ from those recovered from humans. However, it has been demonstrated that several isolates from non- human primates belong to the serogroups and/ or serotypes related to those implicated in human disease. The objective of this study was to evaluate the genetic differences between thirteen strains isolated from non- human primates and the same number of strains isolated from human infections. Human isolates belonged to the same serogroup/ serotype as the monkey strains and the evaluation was done by analysis of random amplified polymorphic DNA. Dendrogram analysis showed that there was no clustering between human and monkey strains. Human and non- human isolates of the EPEC serotypes O127:H40 and O128:H2 shared 90 and 87% of their bands, respectively, indicating strong genomic similarity between the strains, leading to the speculation that they may have arisen from the same pathogenic clone. To our knowledge, this study is the first one comparing genomic similarity between human and non- human primate strains and the results provide further evidence that monkey EPEC strains correlate with human EPEC, as suggested in a previous investigation.

Cognitive and olfactory deficits in Machado-Joseph disease: A dopamine transporter study

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Cell-mediated and humoral immunity in West syndrome

The immunological status of five children with West syndrome consequent to previous cerebral lesions was investigated. Three children had West syndrome and two were in transition from West to Lennox-Gastaut syndrome. All of them showed cellular immunological deficiencies in the following tests: sensitization to DNCB, intracutaneous reaction to PHA, inhibition of leukocyte migration, blastic transformation of lymphocytes, T and B lymphocytes in peripheral blood and levels of serum immunoglobulins. These immunological deficiencies, of different degrees of severity, were associated with frequent infections in these children. A possible association between the immunological deficiencies and autoimmunity is discussed.

Nutritional management and weight changes during hospitalization of Brazilian infants with diarrhoea: Primary reliance on oral feeding or continuous nasogastric drip with locally made, modulated minced chicken formula

The nutritional management of infants admitted with diarrhoea to the University Hospital of Botucatu includes a change from bolus feeding of a modulated minced-chicken formula to a continuous nasogastric drip (NGD) feeding, whenever the required calorie intake is not achieved or the diarrhoea does not subside. To evaluate this approach, the clinical course and weight changes of 63 children, aged 1-20 months, were reviewed; most (81 per cent) were below the third percentile for weight at admission and 76 per cent had a total duration of diarrhoea ≥10 days. Associated infections, mainly systemic, were present at or after admission in 70 per cent of them. Twenty-five survivors needed nutritional support (NS), predominantly NGD, for a median duration of 30 per cent of their admission time, and were compared to 31 survivors managed without NS. Those who necessitated NS lost weight for a significantly longer median time (12x4 days, p<0.005), but their total weight loss was similar (5x4 per cent) as well as diarrhoea's duration (8x7 days). There was a tendency for a longer hospitalization (21x16 days 0.05