Consensus-based care recommendations for adults with myotonic dystrophy type 1.

PURPOSE OF REVIEW: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects between 1 in 3,000 and 8,000 individuals globally. No evidence-based guideline exists to inform the care of these patients, and most do not have access to multidisciplinary care centers staffed by experienced professionals, creating a clinical care deficit. RECENT FINDINGS: The Myotonic Dystrophy Foundation (MDF) recruited 66 international clinicians experienced in DM1 patient care to develop consensus-based care recommendations. MDF created a 2-step methodology for the project using elements of the Single Text Procedure and the Nominal Group Technique. The process generated a 4-page Quick Reference Guide and a comprehensive, 55-page document that provides clinical care recommendations for 19 discrete body systems and/or care considerations. SUMMARY: The resulting recommendations are intended to help standardize and elevate care for this patient population and reduce variability in clinical trial and study environments.

How many polysomnograms must sleep fellows score before becoming proficient at scoring sleep?

OBJECTIVES: In the field of sleep medicine, there is a paucity of evidence-based curriculum development strategies in the literature. We chose to determine the number of polysomnograms (PSG) necessary to be scored by sleep fellows in order to reasonably approximate sleep scoring by a Diplomate of the American Board of Sleep Medicine (DABSM). DESIGN: The fifth PSG scored by two sleep fellows during the 12 consecutive months of training was chosen for analysis. A DABSM not involved in the training of fellows scored sleep on each of the selected PSG with replication of montage and filter settings. Epoch by epoch comparison of sleep stage scoring is described as the frequency of concordance between fellow and DABSM (f correct). MEASUREMENTS AND RESULTS: The mean (SD) f correct for all PSG for each fellow was 0.83 (0.06) and 0.83 (0.08) (p = 0.93). Concordance between sleep fellow and DABSM approached 0.8 after scoring between 20-30 PSG. This milestone was reached after the fourth month of training. F correct was highest for stage 2 sleep and REM sleep and most variable for slow wave sleep and stage 1 sleep. The variability in f correct for these stages was in part related to the relative paucity of these sleep stages. CONCLUSIONS: Scoring of sleep becomes reasonably proficient after scoring approximately 20-30 PSG and/or four months of dedicated sleep disorders training. A standard measure of concordance that corrects for epoch sample size may be helpful for use in future similar investigations.

Hipotermia y consumo de oxigeno en el post-operatorio de revascularizacion miocardica / Hypothermia and oxigen comsumtion in the postoperative of miocardic

Para evaluar la relación entre la temperatura (T) y el consumo de oxígeno (VO2), se diseño la siguiente investigación de tipo prospectivo descriptivo. Se determinó la temperatura y el VO2 por intermedio del Catéter se Swan-Ganz, agrupándose los valores en 3 fases. La relación entre las medias de la T y del VO2 en las 3 fases mostró una curva bifásica, con una fase inicial ascendente donde el VO2 aumentó a medida que aumentó la temperatura y otra fase descendente donde la relación fue inversa, el VO2 disminuyó al aumentar la T

Radicales libres y lesión por reperfusión / Free-oxygen radicals and injuries by reperfusion

Los radicales libres son especies químicas reactivas que debido a su composición son capaces de producir daño a los componentes celulares. Los sistemas biológicos está continuamente expuestos a los radicales libres de oxígeno por intermedio de reacciones de óxido-reducción. Sin embargo, los organismos aeróbicos poseen sustancias que ayudan a prevenir la lesión mediada por radicales libres. Se entiende por lesión por reperfusión al daño tisular producido luego de un proceso isquémico, producido de la incapacidad del tejido de barrer los radicales libres una vez reestablecido el flujo sanguíneo, ello conduce a introducir el concepto de Oxígeno Paradójico