Switching to Glycerol Phenylbutyrate in 48 Patients with Urea Cycle Disorders: Clinical Experience in Spain

Background and objectives: Glycerol phenylbutyrate (GPB) has demonstrated safety and efficacy in patients with urea cycle disorders (UCDs) by means of its clinical trial program, but there are limited data in clinical practice. In order to analyze the efficacy and safety of GPB in clinical practice, here we present a national Spanish experience after direct switching from another nitrogen scavenger to GPB. Methods: This observational, retrospective, multicenter study was performed in 48 UCD patients (age 11.7 ± 8.2 years) switching to GPB in 13 centers from nine Spanish regions. Clinical, biochemical, and nutritional data were collected at three different times: prior to GPB introduction, at first follow-up assessment, and after one year of GPB treatment. Number of related adverse effects and hyperammonemic crisis 12 months before and after GPB introduction were recorded. Results: GPB was administered at a 247.8 ± 102.1 mg/kg/day dose, compared to 262.6 ± 126.1 mg/kg/day of previous scavenger (46/48 Na-phenylbutyrate). At first follow-up (79 ± 59 days), a statistically significant reduction in ammonia (from 40.2 ± 17.3 to 32.6 ± 13.9 μmol/L, p < 0.001) and glutamine levels (from 791.4 ± 289.8 to 648.6 ± 247.41 μmol/L, p < 0.001) was observed. After one year of GPB treatment (411 ± 92 days), we observed an improved metabolic control (maintenance of ammonia and glutamine reduction, with improved branched chain amino acids profile), and a reduction in hyperammonemic crisis rate (from 0.3 ± 0.7 to less than 0.1 ± 0.3 crisis/patients/year, p = 0.02) and related adverse effects (RAE, from 0.5 to less than 0.1 RAEs/patients/year p < 0.001). Conclusions: This study demonstrates the safety of direct switching from other nitrogen scavengers to GPB in clinical practice, which improves efficacy, metabolic control, and RAE compared to previous treatments.This study was funded by AECOM (Spanish Association for the Study of Inborn Errors of Metabolism). Immedica Pharma Spain funded medical writing support and article processing charges

Desenvolvimento de uma Escala Curta Para Medir Envolvimento com Produtos Ecológicos

This study develops a short, general scale to measure sustainable product involvement. This is done in a Costa Rican context, via a relatively large sample, demographically similar to the national population. The study also evaluates the viability of the C-OAR-SE scaling technique for this purpose. A five-item instrument is developed, its reliability and validity psychometrically confirmed. The scale addresses the levels and types of involvement that consumers might have. It suits not only academic researchers, but also practitioners in different areas. We conclude that C-OAR-SE is a viable technique. It complements traditional psychometric methods well so as to be considered by researchers in the different fields of business.Este estudio desarrolla una escala corta y general para medir el involucramiento con productos ecológicos. Se realiza en un contexto costarricense, mediante una muestra relativamente amplia y demográficamente similar a la población nacional. El estudio también evalúa la viabilidad de la técnica escalar C-OAR-SE para este fin. Una medida de cinco ítems es desarrollada, su fiabilidad y validez psicométricamente confirmadas. La escala mide los niveles y tipos de involucramiento que puedan tener los consumidores. No sólo le sirve a investigadores académicos, sino que también a profesionales en diferentes áreas. Concluimos que C-OAR-SE es una técnica viable. Complementa bien los métodos psicométricos tradicionales para ser considerada por investigadores en los diferentes campos de la administración.Cette étude développe une petite échelle générale pour mesurer l’implication dans des produits écologiques. Elle est réalisée dans un contexte costaricien, sur base d’un échantillon relativement large et démographiquement similaire à la population&nbsp; nationale.&nbsp; L’étude évalue également la viabilité de la technique d’échelle C-OAR-SE à cette fin. Une mesure de cinq éléments est développée, et sa fiabilité et validité confirmées de manière psychométrique. L’échelle mesure les niveaux et les types d’implication possibles des consommateurs. C’est utile non seulement pour les chercheurs académiques mais aussi pour les professionnels dans différents domaines. Nous concluons que C-OAR-SE est une technique viable. Elle complémente bien les techniques psychométriques traditionnelles et peut être prise en considération par les chercheurs dans les différents domaines de l’administration.&nbsp;Este estudo desenvolve uma escala curta e geral para medir o envolvimento com produtos ecológicos. É realizado em um contexto costarriquenho, por meio de uma amostra relativamente ampla e demograficamente semelhante à população nacional. O estudo também avalia a viabilidade da técnica escalar C-OAR-SE para esse fim. Uma medida de cinco itens é desenvolvida, sua fiabilidade e validade confirmadas psicometricamente. A escala mede os níveis e tipos de envolvimento que os consumidores possam ter. Não é útil apenas para pesquisadores acadêmicos, mas também para profissionais de diferentes áreas. Concluímos que C-OAR-SE é uma técnica viável. Complementa bem as técnicas psicométricas tradicionais a ser considerada pelos pesquisadores nas diferentes áreas da administraçã

Taller sobre autogestión y fondos rotatorios (proyecto coordinación de servicios sociales en Centroamérica)

Tabla de contenido: 1. Recomendaciones y conclusiones sobre el taller de autogestión / por Lucy Conejo – 2. Boleta de seguimiento población activa en programa fondo rotatorio / por Lucy Conejo – 3. Seminario-taller desarrollo de habilidades emprendedoras / por Lucy Conejo – 4. El poder de la determinación / por Lucy Conejo — 5. Examen de los hábitos de escucha: prueba de comprensión / por Lucy Conejo – 6. Árbol de problemas: diagrama de causas y efectos / por Aníbal Robles – 7. Programa fondo rotatorio de prestarnos / por Aníbal Robles – 8. Programa Fondo rotatorio de préstamos / por Aníbal RoblesUT Librarie