INICIACIÓN A LA PRÓTESIS BUCODENTAL

GUÍA PEDAGÓGIC

Haemoglobinopathies in Europe: health & migration policy perspectives

BACKGROUND: Major haemoglobinopathies (MH), such as thalassaemia syndromes (Thal) and sickle cell disorders (SCD), are genetic defects associated with chronic anaemia and other complications. In Europe, MH are rare diseases (RD) but their prevalence is significantly growing in many countries due to mobility and migration flows. This creates a growing health problem in the EU that has not yet been effectively addressed by Member States (MS) authorities. The present study has been conducted with the aim of: (i) providing an overview of policies for MH in 10 EU member states (MS) (ii) analysing the challenges linked to these RD due to growing requirements imposed by population, mobility and migration trends and (iii) identifying gaps, proposing improvements on existing policies, or developing new ones to fit the identified needs. METHODS: The study has been undertaken by a group of members of the European Network for Rare and Congenital Anaemias (ENERCA) and the Thalassaemia International Federation (TIF), in collaboration with the public affairs firm Burson-Marsteller Brussels. Data from 10 EU countries have been gathered using targeted desk research and one-to-one interviews with local stakeholders, including healthcare professionals, patients and public health officers/providers. RESULTS: 1. MH are the most common RD in all the 10 countries, 2. Data on prevalence, overall burden, trends, and clinical follow up costs are lacking in most countries. 3. Neonatal screening practices show a wide variation across and within countries. 4. Awareness on MH and their related complications is very low, exception made of Italy, Greece, Cyprus and UK, 5. No disaggregated data is available to understand the impact of mobility and migration on the prevalence of haemoglobinopathies, and how healthcare delivery systems should adapt to respond to this situation. 6. Targeted policy measures and/or actions are generally lacking and/or delayed. CONCLUSIONS: Ten policy recommendations have been drawn from this study, building on 2006 WHO recommendations for MH to include haemoglobinopathies in National Plans of Actions for Rare Diseases

Inactive matriptase-2 mutants found in IRIDA patients still repress hepcidin in a transfection assay despite having lost their serine protease activity.

L'article final de l'éditeur contient 9 pages. Le manuscrit accepté contient 32 pages.International audienceMutations of the TMPRSS6 gene, which encodes Matriptase-2, are responsible for iron-refractory iron-deficiency anemia. Matriptase-2 is a transmembrane protease that downregulates hepcidin expression. We report one frameshift (p.Ala605ProfsX8) and four novel missense mutations (p.Glu114Lys, p.Leu235Pro, p.Tyr418Cys, p.Pro765Ala) found in IRIDA patients. These mutations lead to changes in both the catalytic and noncatalytic domains of Matriptase-2. Analyses of the mutant proteins revealed a reduction of autoactivating cleavage and the loss of N-Boc-Gln-Ala-Arg-p-nitroanilide hydrolysis. This resulted either from a direct modification of the active site or from the lack of the autocatalytic cleavage that transforms the zymogen into an active protease. In a previously described transfection assay measuring the ability of Matriptase-2 to repress the hepcidin gene (HAMP) promoter, all mutants retained some, if not all, of their transcriptional repression activity. This suggests that caution is called for in interpreting the repression assay in assessing the functional relevance of Matriptase-2 substitutions. We propose that Matriptase-2 activity should be measured directly in the cell medium of transfected cells using the chromogenic substrate. This simple test can be used to determine whether a sequence variation leading to an amino acid substitution is functionally relevant or not

La imagen y la narrativa como herramientas para el abordaje psicosocial en escenarios de violencia. Municipios de Bogotá D.C. Dabeiba, Dosquebradas y Pereira.

La imagen y la narrativa como herramientas para el abordaje psicosocial en escenarios de violencia. Municipios de Bogotá D.C. Dabeiba, Dosquebradas y Pereira.Mediante el acompañamiento psicosocial en escenarios de violencia, a través del abordaje de contextos desde un enfoque narrativo (herramienta metodológica), busca potenciar las habilidades del individuo de manera que genere nuevas posibilidades de cambio tanto de en sí mismo como de su entorno. Esta herramienta permite transformar sentimientos, pensamientos de vivencias traumáticas en historias de superación y mejora, en donde la victima adquiere un rol importante; pues se convierte en protagonista de una historia subjetiva, donde narra hechos que han podido afectar su integridad física y mental. Por tal razón, mediante una orientación adecuada y dirigida por un facilitador, permitiría que los eventos violentos traumáticos puedan llegar a ser “Transformados en una historia de victimización por una historia de supervivencia, para que pueda reconstruir su vida” Teniendo en cuenta las herramientas adquiridas, se toma para la socialización el relato del Señor Edison quien es uno de las muchas voces registradas en el” (Banco Mundial 2009 p. 37) donde refleja el impacto que le ha generado ser integrante de las FARC; además de la importancia de ser aceptado en la sociedad. Además de este caso, se tiene como referencia el caso de Panduri, donde se logran establecer emergentes psicosociales, impactos que generan estigmatización y el aporte de estrategias que impulsan la potenciación de recursos de afrontamiento. De acuerdo a lo anterior se busca fortalecer las capacidades y competencias de cada estudiante mediante una perspectiva objetiva y reflexiva frente a los diferentes entornos de violencia que se pueden encontrar en Colombia.By means of psychosocial accompaniment in scenarios of violence, through the approach of contexts from a narrative approach (methodological tool), seeks to enhance the skills of the individual in a way that generates new possibilities for change both in itself and its environment. This tool allows to transform feelings, thoughts of traumatic experiences in stories of improvement and betterment, where the victim acquires an important role because it becomes the protagonist of a subjective story, where the victim narrates facts that have been able to affect their physical and mental integrity; for this reason, by means of an adequate orientation directed by a facilitator, it allows traumatic violent events to become "A history of victimization to a history of survival, so that the person can rebuild his life" Taking into account the tools acquired, is taken for socialization the story of Mr. Edison who is one of the many voices recorded in the (World Bank 2009 page 37) where he reflects the impact that has been generated for him by being a member of the FARC, and the importance of being accepted in society; In addition to this case, the case of Panduri is taken as reference, where psychosocial arisings are established, impacts that generate stigmatization and the contribution of strategies that impulse the strengthening of coping resources. In accordance with the text above, the aim is to strengthen the abilities and competencies of each student through an objective and reflective perspective regarding the different environments of violence that can be found in Colombia

Safety and immediate humoral response of COVID-19 vaccines in chronic kidney disease patients:the SENCOVAC study

BACKGROUND: Chronic kidney disease (CKD) patients are at high-risk for severe Covid-19. The multicentric, observational and prospective SENCOVAC study aims to describe the humoral response and safety of SARS-CoV-2 vaccines in CKD patients. Safety and immediate humoral response results are reported here. METHODS: Four cohorts of patients were included: kidney transplant (KT) recipients, haemodialysis (HD), peritoneal dialysis (PD) and non-dialysis CKD patients from 50 Spanish centres. Adverse events after vaccine doses were recorded. At baseline and on day 28 after the last vaccine dose, anti-Spike antibodies were measured and compared between cohorts. Factors associated with development of anti-Spike antibodies were analyzed. RESULTS: 1746 participants were recruited: 1116 HD, 171 PD, 176 non-dialysis CKD patients and 283 KT recipients. Most patients (98%) received mRNA vaccines. At least one vaccine reaction developed after the first dose in 763 (53.5%) and after the second dose in 741 (54.5%) of patients. Anti-Spike antibodies were measured in the first 301 patients. At 28 days, 95% of patients had developed antibodies: 79% of KT, 98% of HD, 99% of PD and 100% of non-dialysis CKD patients (p<0.001). In a multivariate adjusted analysis, absence of an antibody response was independently associated to KT (OR 20.56, p = 0.001) and to BNT162b2 vaccine (OR 6.03, p = 0.023). CONCLUSION: The rate of anti-Spike antibody development after vaccination in KT patients was low but in other CKD patients it approached 100%; suggesting that KT patients require persistent isolation measures and booster doses of a Covid-19 vaccine. Potential differences between Covid-19 vaccines should be explored in prospective controlled studies

Resistencia de Fasciola hepatica al Triclabendazol: primer reporte en ovinos de la provincia de Santa Cruz, Patagonia Argentina

En el presente informe técnico se describen los principales resultados de los estudios de eficacia fasciolicida de antiparasitarios realizados en un establecimiento cercano a Los Antiguos, Santa Cruz, a partir de los cuales se confirmó la existencia de una cepa de F. hepatica resistente a triclabendazol. Los trabajos descriptos fueron realizados por el Grupo de Salud Animal de la EEA INTA Bariloche, las Agencia de Extensión Rural INTA San Julián y Los Antiguos, la EEA INTA Esquel, y el Laboratorio de Farmacología del Centro de Investigación Veterinaria de Tandil (CIVETAN). El trabajo completo se encuentra publicado en la revista Veterinary Parasitology: Regional Studies and Reports. 45. 100927.https://www.sciencedirect.com/science/article/abs/pii/S2405939023000977EEA Santa CruzFil: Larroza, Marcela Patricia. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Bariloche. Área Producción Animal. Grupo Sanidad Animal; Argentina.Fil: Aguilar, Marcelo Javier. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Santa Cruz. Agencia de Extensión Rural San Julián; Argentina.Fil: Soler, Paula. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Bariloche. Área Producción Animal. Grupo Sanidad Animal; Argentina.Fil: Mora, Julio Cesar. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Santa Cruz. Agencia de Extensión Rural Los Antiguos; Argentina.Fil: Roa, Martin Angel. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Santa Cruz. Agencia de Extensión Rural Los Antiguos; Argentina.Fil: Cabrera, Francisco Raúl. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Bariloche. Área de producción Animal. Grupo de Sanidad Animal; Argentina.Fil: Martinez Stanziola, Juan Pablo. Instituto Nacional de Tecnología Agropecuaria (INTA). Centro Regional Patagonia Sur. Estación Experimental Agroforestal Esquel (EEAf Esquel). Campo Anexo Trevelin; Argentina.Fil: Ceballos, Laura. Universidad Nacional del Centro de la Provincia de Buenos Aires. Facultad de Ciencias Veterinarias; Argentina.Fil: Ceballos, Laura. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Centro de Investigación Veterinaria de Tandil; Argentina.Fil: Alvarez, Luís. Universidad Nacional del Centro de la Provincia de Buenos Aires. Facultad de Ciencias Veterinarias; Argentina.Fil: Alvarez, Luís. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Centro de Investigación Veterinaria de Tandil; Argentina

Recommendations for Pregnancy in Rare Inherited Anemias

Rare inherited anemias are a subset of anemias caused by a genetic defect along one of the several stages of erythropoiesis or in different cellular components that affect red blood cell integrity, and thus its lifespan. Due to their low prevalence, several complications on growth and development, and multi-organ system damage are not yet well defined. Moreover, during the last decade there has been a lack of proper understanding of the impact of rare anemias on maternal and fetal outcomes. In addition, there are no clear-cut guidelines outlining the pathophysiological trends and management options unique to this special population. Here, we present on behalf of the European Hematology Association, evidence- and consensus-based guidelines, established by an international group of experts in different fields, including hematologists, gynecologists, general practitioners, medical geneticists, and experts in rare inherited anemias from various European countries for standardized and appropriate choice of therapeutic interventions for the management of pregnancy in rare inherited anemias, including Diamond-Blackfan Anemia, Congenital Dyserythropoietic Anemias, Thalassemia, Sickle Cell Disease, Enzyme deficiency and Red cell membrane disorders

The climate-smart village approach: Framework of an integrative strategy for scaling up adaptation options in agriculture

Increasing weather risks threaten agricultural production systems and food security across the world. Maintaining agricultural growth while minimizing climate shocks is crucial to building a resilient food production system and meeting developmental goals in vulnerable countries. Experts have proposed several technological, institutional, and policy interventions to help farmers adapt to current and future weather variability and to mitigate greenhouse gas (GHG) emissions. This paper presents the climate-smart village (CSV) approach as a means of performing agricultural research for development that robustly tests technological and institutional options for dealing with climatic variability and climate change in agriculture using participatory methods. It aims to scale up and scale out the appropriate options and draw out lessons for policy makers from local to global levels. The approach incorporates evaluation of climate-smart technologies, practices, services, and processes relevant to local climatic risk management and identifies opportunities for maximizing adaptation gains from synergies across different interventions and recognizing potential maladaptation and trade-offs. It ensures that these are aligned with local knowledge and link into development plans. This paper describes early results in Asia, Africa, and Latin America to illustrate different examples of the CSV approach in diverse agroecological settings. Results from initial studies indicate that the CSV approach has a high potential for scaling out promising climate-smart agricultural technologies, practices, and services. Climate analog studies indicate that the lessons learned at the CSV sites would be relevant to adaptation planning in a large part of global agricultural land even under scenarios of climate change. Key barriers and opportunities for further work are also discussed

DNA Vaccine-Generated Duck Polyclonal Antibodies as a Postexposure Prophylactic to Prevent Hantavirus Pulmonary Syndrome (HPS)

Andes virus (ANDV) is the predominant cause of hantavirus pulmonary syndrome (HPS) in South America and the only hantavirus known to be transmitted person-to-person. There are no vaccines, prophylactics, or therapeutics to prevent or treat this highly pathogenic disease (case-fatality 35–40%). Infection of Syrian hamsters with ANDV results in a disease that closely mimics human HPS in incubation time, symptoms of respiratory distress, and disease pathology. Here, we evaluated the feasibility of two postexposure prophylaxis strategies in the ANDV/hamster lethal disease model. First, we evaluated a natural product, human polyclonal antibody, obtained as fresh frozen plasma (FFP) from a HPS survivor. Second, we used DNA vaccine technology to manufacture a polyclonal immunoglobulin-based product that could be purified from the eggs of vaccinated ducks (Anas platyrhynchos). The natural “despeciation" of the duck IgY (i.e., Fc removed) results in an immunoglobulin predicted to be minimally reactogenic in humans. Administration of ≥5,000 neutralizing antibody units (NAU)/kg of FFP-protected hamsters from lethal disease when given up to 8 days after intranasal ANDV challenge. IgY/IgYΔFc antibodies purified from the eggs of DNA-vaccinated ducks effectively neutralized ANDV in vitro as measured by plaque reduction neutralization tests (PRNT). Administration of 12,000 NAU/kg of duck egg-derived IgY/IgYΔFc protected hamsters when administered up to 8 days after intranasal challenge and 5 days after intramuscular challenge. These experiments demonstrate that convalescent FFP shows promise as a postexposure HPS prophylactic. Moreover, these data demonstrate the feasibility of using DNA vaccine technology coupled with the duck/egg system to manufacture a product that could supplement or replace FFP. The DNA vaccine-duck/egg system can be scaled as needed and obviates the necessity of using limited blood products obtained from a small number of HPS survivors. This is the first report demonstrating the in vivo efficacy of any antiviral product produced using DNA vaccine-duck/egg system