Phosphatidylserine Increases IKBKAP Levels in Familial Dysautonomia Cells

Familial Dysautonomia (FD) is an autosomal recessive congenital neuropathy that results from abnormal development and progressive degeneration of the sensory and autonomic nervous system. The mutation observed in almost all FD patients is a point mutation at position 6 of intron 20 of the IKBKAP gene; this gene encodes the IκB kinase complex-associated protein (IKAP). The mutation results in a tissue-specific splicing defect: Exon 20 is skipped, leading to reduced IKAP protein expression. Here we show that phosphatidylserine (PS), an FDA-approved food supplement, increased IKAP mRNA levels in cells derived from FD patients. Long-term treatment with PS led to a significant increase in IKAP protein levels in these cells. A conjugate of PS and an omega-3 fatty acid also increased IKAP mRNA levels. Furthermore, PS treatment released FD cells from cell cycle arrest and up-regulated a significant number of genes involved in cell cycle regulation. Our results suggest that PS has potential for use as a therapeutic agent for FD. Understanding its mechanism of action may reveal the mechanism underlying the FD disease

Outcomes following small bowel obstruction due to malignancy in the national audit of small bowel obstruction

Introduction Patients with cancer who develop small bowel obstruction are at high risk of malnutrition and morbidity following compromise of gastrointestinal tract continuity. This study aimed to characterise current management and outcomes following malignant small bowel obstruction. Methods A prospective, multicentre cohort study of patients with small bowel obstruction who presented to UK hospitals between 16th January and 13th March 2017. Patients who presented with small bowel obstruction due to primary tumours of the intestine (excluding left-sided colonic tumours) or disseminated intra-abdominal malignancy were included. Outcomes included 30-day mortality and in-hospital complications. Cox-proportional hazards models were used to generate adjusted effects estimates, which are presented as hazard ratios (HR) alongside the corresponding 95% confidence interval (95% CI). The threshold for statistical significance was set at the level of P ≤ 0.05 a-priori. Results 205 patients with malignant small bowel obstruction presented to emergency surgery services during the study period. Of these patients, 50 had obstruction due to right sided colon cancer, 143 due to disseminated intraabdominal malignancy, 10 had primary tumours of the small bowel and 2 patients had gastrointestinal stromal tumours. In total 100 out of 205 patients underwent a surgical intervention for obstruction. 30-day in-hospital mortality rate was 11.3% for those with primary tumours and 19.6% for those with disseminated malignancy. Severe risk of malnutrition was an independent predictor for poor mortality in this cohort (adjusted HR 16.18, 95% CI 1.86 to 140.84, p = 0.012). Patients with right-sided colon cancer had high rates of morbidity. Conclusions Mortality rates were high in patients with disseminated malignancy and in those with right sided colon cancer. Further research should identify optimal management strategy to reduce morbidity for these patient groups

Pedagogia: concepções e práticas em transformação Pedagogy: conceptions and practices in transformation

Este artigo apresenta uma análise das concepções educacionais do curso de Pedagogia voltadas para a formação social do Educador ao longo da história. Neste curso, ainda não existe uma clareza em relação a sua identidade. Os documentos oficiais e algumas entidades representativas priorizam a docência como base de formação, outras priorizam o Pedagogo como cientista da educação. Nas últimas décadas no Brasil, surgiu a Educação Popular, precursora da Pedagogia Social, que aborda o trabalho com os grupos sociais historicamente excluídos e valoriza a diversidade cultural nos processos de Educação Não Formal. Este artigo faz parte de uma pesquisa que analisa os documentos e produções bibliográficas a respeito das Diretrizes Curriculares Nacionais para o Curso de Pedagogia, instituída pela Resolução CNE/CP n. 1 de 15/05/2006. Percebe-se nas produções acadêmicas analisadas, que existem avanços no sentido de uma abordagem mais social no aspecto de formação nos cursos de Pedagogia. Mas, ao mesmo tempo, existem incoerências na base de formação dos profissionais, pois a ênfase na docência restringe sua atuação. Esses estudos revelaram que a Pedagogia tem tornado-se, ainda que superficialmente, mais social e a Educação Popular tem tido a necessidade de organizar o seu trabalho de forma mais pedagógica. Ou seja, a Pedagogia Social tem surgido como instrumento da Educação para atender às novas demandas da sociedade e formar o Educador Social como o seu agente transformador.<br>This article presents an analysis of the educational conceptions of the Pedagogy course dimensioned for Educators social formation through history. In this course their identity isn't clear yet. The official documents and some representative entities consider teaching as priority base for formation, others prioritise the Pedagogy professional an education scientist. In the last decades in Brazil, the Popular Education appeared, precedent of the Social Pedagogy, which approaches the work with social groups historically excluded and values the cultural diversity in the Not-Formal Education process. This article makes part of a research that analyses the documents and bibliography productions about the National Curricular Rule for the Pedagogy Course, instituted by the Resolution CNE/CP n. 1 of 05/15th/2006. It is realized on the academic productions analyzed, that exist advances on the meaning of a more social approach in the aspect of the formation in the Pedagogy's courses. But, at the same time, there are incoherences in the base of the vocational training, because the emphasis on the teaching profession restricts its actuation. Those studies revealed that the Education has become itself, even thought superficially, more social and the Popular Education has had the necessity to organize its work in a more educational way. In other words, the Social Pedagogy has appeared as an instrument of the Education to answer the new demands of the society and to form the Social Educator as its transformer agent