RESUMEN
Supravalvular aortic stenosis (SVAS) is a congenital malformation associated with Williams-Beuren syndrome. It is characterized by severe stenosis of the sinotubular junction. The smooth muscle layer is thickened due to an alteration of the Elastin gene. The treatment is surgical and there are several techniques to relieve obstruction which range from the single-patch technique to the replacement of all three aortic sinuses with three patches. However, the optimal technique is still unclear. The objective of this article is to carry out a bibliographic review of surgical techniques for SVAS and the description of the application of the Doty technique for a 4-year-old infant at the Roberto Gilbert Elizalde Hospital.
La estenosis aórtica supravalvular es una malformación congénita asociada al síndrome de Williams-Beuren. Se caracteriza por una severa estenosis en la unión sinotubular. La capa de músculo liso se engrosa debido a una alteración en el gen Elastina. El tratamiento es quirúrgico y existen varias técnicas para aliviar la obstrucción, que van desde la técnica de un solo parche hasta el reemplazo de los tres senos aórticos con tres parches. Sin embargo, la técnica óptima aún no está clara. El objetivo de este artículo es realizar una revisión bibliográfica de las técnicas quirúrgicas para estenosis valvular aórtica supravalvular y describir la aplicación de la técnica de Doty en una niña de 4 años en el Hospital Roberto Gilbert Elizalde.
RESUMEN
Las vacunas previenen millones de muertes cada año y su eficacia y seguridad han sido ampliamente establecidas. En términos económicos, la vacunación es una de las intervenciones sanitarias más costo efectivas, generando un importante ahorro y crecimiento económico que supone a largo plazo. Se ha demostrado que la vacunación de adultos disminuye la morbilidad y la mortalidad asociadas a enfermedades infecciosas prevenibles, reduciendo las complicaciones y las hospitalizaciones, incluidos los ingresos a las unidades de cuidados intensivos. Hemos elaborado este documento de consenso con el objeto de diseñar un esquema de vacunación pragmático, accesible y estandarizado del adulto, según categoría de riesgo y edad, sobre la base de la evidencia disponible de vacunas accesibles y nuevas vacunas habiendo utilizado el Tercer Consenso de la Sociedad Paraguaya de Infectología del 2019 como base para las recomendaciones finales.
SUMMARY Vaccines prevent millions of deaths each year, and their efficacy and safety have been widely established. In economic terms, vaccination is one of the most cost-effective health interventions, generating significant savings and long-term economic growth. Adult vaccination has been shown to decrease morbidity and mortality associated with preventable Infectious diseases, reducing complications and hospitalizations, including admissions to intensive care units. We have prepared this consensus document in order to design a pragmatic, accessible and standardized vaccination scheme for adults, according to risk category and age, based on the available evidence of available vaccines and new vaccines, having used the third consensus of the Paraguayan Infectious Diseases Society of 2019 as a basis for the final recommendations.
RESUMEN
Cell therapy and the use of mAbs that interfere with T cell effector functions constitute promising approaches for the control of allograft rejection. In the current study, we investigated a novel approach combining administration of autologous tolerogenic dendritic cells with short-term treatment with CD3-specific Abs. Permanent acceptance of pancreatic islet allografts was achieved in mice treated with the combination therapy the day before transplantation but not in recipients treated with either therapy alone. The combination treatment induced a marked decrease in T cells infiltrating the allografts and a sustained reduction of antidonor responses. Importantly, CD4(+)Foxp3(+) regulatory T cells appeared to play a crucial role in the long-term graft acceptance. Their frequency increased significantly in the spleen, draining lymph nodes, and transplanted islets and remained elevated over the long term; they exhibited increased donor-specific suppressive functions; and their removal at the time of transplantation abrogated the therapeutic effect of the combined therapy. These results support the therapeutic potential of protocols combining autologous dendritic cells and low-dose CD3 Abs, both currently in clinical development, and that act in synergy to control allogeneic immune responses and favor graft survival in a full-mismatch situation.