Assessment of Respiratory Function and Need for Noninvasive Ventilation in a Cohort of Patients with Myotonic Dystrophy Type 1 Followed at One Single Expert Center.

Introduction: Respiratory insufficiency is one of the main causes of death in myotonic dystrophy type 1 (DM1). Although there is general consensus that these patients have a restrictive ventilatory pattern, hypoventilation, chronic hypercapnia, and sleep disturbances, the prevalence of respiratory disease and indication for the effects of noninvasive ventilation (NIV) need to be further explored. Objectives: To describe respiratory function and need for NIV at baseline and over time in a cohort of adult patients with DM1. Methods: A total of 151 adult patients with DM1 were subjected to arterial blood gas analysis, sitting and supine forced vital capacity (FVC), peak cough expiratory flow (PCEF), nocturnal oximetry, and maximal inspiratory pressure and expiratory pressure (MIP/PEP). Results: On first assessment, 84 of 151 had normal respiratory function (median age: 38 years, median BMI: 23.9, and median disease duration: 11 years); 67 received an indication to use NIV (median age: 49 years, median BMI: 25,8, and median disease duration: 14 years). After a median time of 3.85 years, 43 patients were lost to follow-up; 9 of 84 required NIV; only 17 of 67 with the new NIV prescription were adherent. Conclusions: We provide additional data on the natural history of respiratory function decline and treatment adherence in a relatively large cohort of well-characterized patients with DM1. A high proportion (28%) was lost to follow-up. A minority (11%) required NIV, and only 25% were treatment adherent, irrespective of specific demographics and respiratory features. Our results also confirm previous findings, showing that age, disease duration, and higher BMIs are predisposing factors for respiratory impairment.

National Registry of Acute Coronary Syndrome in Paraguay (RENASCA-PY).

OBJECTIVE: To determine the initial management and in-hospital mortality of patients with acute coronary syndrome who attended referral hospitals in Paraguay. METHOD: Observational, multicenter study, in patients over 18 years with a confirmed diagnosis of acute coronary syndrome. RESULTS: 780 patients were included from May 2015 to February 2016; the mean age was 64.1 ± 12.3 years, 64.1% male. The clinical presentation was acute coronary syndrome with ST elevation in 40.1% and without elevation in 59.9%. In patients with ST elevation there is a high percentage of late attendance, more than 12 h of evolution in 49.8%; those with less than 12 h of evolution underwent reperfusion in 52.2% of the cases, received fibrinolytics in 36.3% of the cases, and primary percutaneous coronary intervention 15.9%. In-hospital mortality for acute coronary syndrome was 10.3%, with ST-segment elevation was 12.8%, and without ST-segment elevation was 8.6%. CONCLUSIONS: The management of acute coronary syndrome in Paraguay needs a comprehensive approach, which promotes earlier care, and increases the implementation of reperfusion therapies in the health services network, in order to improve the therapeutic response rates and decrease hospital mortality.

OBJETIVO: Determinar el tratamiento inicial y la mortalidad intrahospitalaria de pacientes con síndrome coronario agudo que acudieron a centros hospitalarios de referencia de Paraguay. MÉTODO: Estudio observacional y multicéntrico en pacientes mayores de 18 años con diagnóstico confirmado de síndrome coronario agudo. RESULTADOS: Se incluyó a 780 pacientes desde mayo de 2015 hasta febrero de 2016; la edad media fue de 64.1 ± 12.3 años y el género masculino representó el 64.1%. La presentación clínica fue la de síndrome coronario agudo con elevación del ST en 40.1% y sin elevación del ST en 59.9%. En pacientes con elevación del ST se observó un alto porcentaje de consultas tardías, mayor de 12 h de evolución en 49.8%; en aquéllos con menos de 12 h de evolución se indicó la reperfusión en 52.2%, el 36.3% recibió fibrinolíticos y 15.9% intervención coronaria percutánea primaria. La mortalidad hospitalaria del síndrome coronario agudo fue de 10.3%, con elevación del segmento ST en 12.8% y sin elevación del segmento ST en 8.6%. CONCLUSIONES: El tratamiento del síndrome coronario agudo en el Paraguay requiere un abordaje integral, que promueva consultas más tempranas y aumente la institución de tratamientos de reperfusión en la red de servicios de salud; el objetivo es mejorar los índices de respuesta terapéutica y disminuir la mortalidad hospitalaria.

The Spinal Muscular Atrophy Health Index: Italian validation of a disease-specific outcome measure.

Patient report outcome measures in Spinal Muscular Atrophy (SMA) represent a potential complement to observer rated scales which can be used to better understand treatment response. We developed, translated and validated an Italian version of the Spinal Muscular Atrophy Health Index (SMAHI), a disease-specific, patient reported outcome measure questionnaire, designed to estimate the patients' perception of disease burden. Test-retest reliability was assessed in 37 patients (16 children aged 12-17 and 21 adults) and was excellent in both cohorts. Internal consistency in an additional 98 patients (24 children, 74 adults) was also excellent (Cronbach's alpha = 0.93 and 0.91 respectively). In children the highest level of disease burden was generated from lower limb dysfunction and fatigue as well as their perception of decreased performance in social situations. Most patients in the adult cohort were sitters and complained of problems with upper limb functions as well as of fatigue. The SMAHI-IT was also able to differentiate between SMA types according to diseases severity. The results of our study demonstrate that the SMAHI can be considered a marker of disease-specific burden in patients with SMA with a high test-retest reliability and internal validity in Italian patients aged 12 and older.

Fisiopatología, perfil epidemiológico y manejo terapéutico en el síndrome coronario agudo / Pathophysiology, epidemiological profile and therapeutic management in acute coronary syndrome

Las enfermedades cardiovasculares siguen siendo ampliamente la primera causa de muerte en el mundo actual. La cardiopatía isquémica conlleva a una importante carga de gastos de Salud Pública, por lo cual es importante conocer la prevalencia, epidemiología, fisiopatología y el manejo diagnóstico y terapéutico adecuado del síndrome coronario agudo (SCA). Los hallazgos recientes indican que los primeros pasos en la aterosclerosis son esencialmente inflamatorios. Una respuesta inflamatoria sistémica a menudo acompaña al SCA, y la documentación de su presencia ha sido ampliamente reconocida como un indicador de eventos coronarios a repetición. La medicina basada en la evidencia sugiere fuertemente la importancia de la etiología inflamatoria en el SCA. Los factores tradicionales de riesgo coronario terminan en un pasaje final común que desarrolla un proceso inflamatorio en la pared arterial. El entendimiento mejorado y la comprensión adecuada de la influencia de los procesos inflamatorios en el SCA pueden llevar no solo a una mejor utilización de la terapéutica actualmente disponible sino también al desarrollo de nuevas herramientas terapéuticas. Sin duda alguna los refinamientos constantes en las diferentes estrategias terapéuticas del SCA, sumados a la combinación del entendimiento científico en el uso adecuado de los marcadores inflamatorios, los nuevos agentes farmacológicos y las nuevas técnicas de intervención coronaria percutánea con los nuevos stents y otros dispositivos intracoronarios van a aclarar nuestras dudas y mejorar nuestro manejo diagnóstico y terapéutico del síndrome coronario agudo basado en la evidencia científica

Cardiovascular diseases are still widely the leading cause of death in the world today. Ischemic heart disease leads to a significant burden of public health expenses, which is why it is important to know the prevalence, epidemiology, pathophysiology and the adequate diagnostic and therapeutic management of acute coronary syndrome (ACS). Recent findings indicate that the first steps in atherosclerosis are essentially inflammatory. A systemic inflammatory response often accompanies ACS, and the documentation of its presence has been widely recognized as an indicator of recurrent coronary events. Evidence based medicine strongly suggests the importance of the inflammatory etiology in ACS. The traditional coronary risk factors end in a common final passage that develops an inflammatory process in the arterial wall. Improved and adequate understanding of the influence of inflammatory processes in ACS can lead not only to a better use of currently available therapeutics but also to the development of new therapeutic tools. Undoubtedly the constant refinements in the different therapeutic strategies of the ACS, combined with the addition of scientific understanding in the proper use of inflammatory markers, new pharmacological agents and new techniques of percutaneous coronary intervention with newer stents and other intracoronary devices will clarify our doubts and improve our diagnostic and therapeutic management of acute coronary syndrome based on scientific evidence

Heavy drinkers of Ilex paraguariensis beverages show lower lipid profiles but higher body weight.

Obesity is a widely recognized risk factor for several diseases, reaching an epidemic magnitude worldwide. Natural polyphenols may improve blood lipids and body weight, but their clinical relevance in the general population remains unclear. Thus, we aimed to analyze the relationship of intake of Ilex paraguariensis (I. paraguariensis) beverages to lipid profiles and body weight in a large patient population. Patients were recruited to participate in an educational program to change habits to a healthy lifestyle. Anamnesis, clinical and laboratory assessments were conducted at study enrollment and during follow­up. I. paraguariensis beverages were defined according to preparation as obtained by repeated cold water extraction (CWE), hot water infusions, or water and sugar decoction. Heavy drinkers were defined as those persons consuming >1 L/day of one or more preparation types. Participants (N = 18,287) aged ≥18 years entered the study. Overall prevalence of I. paraguariensis consumption was 91.2%. All three forms were drunk by 35.7%, whereas CWE + hot water infusion and CWE alone by 28.4% and 14.5% participants, respectively. Heavy CWE drinkers had lower total cholesterol (191.4 ± 49.4 vs. 194.6 ± 48.3 mg/dl, p = .02) and lower low­density lipoprotein cholesterol (118.6 ± 38.9 vs. 121.2 ± 47.1 mg/dl, p = .001), but body weight was higher (81.1 ± 16.8 vs. 77.2 ± 16.4 kg, p < .0001) compared with moderate drinkers. Fasting glucose was lower (104.5 ± 48.7 vs. 107.2 ± 49.5 mg/dl, p < .001), and consumption of carbohydrates was higher (36.3% vs. 28.7%, p < .001). A low­lipids high­body­weight paradox could be observed in a population of heavy drinkers of I. paraguariensis beverages. Induced hypoglycemia and compensatory higher intake of refined carbohydrates may represent a possible cause.

Perfil epidemiológico y retardo en la consulta de pacientes hospitalizados por síndrome coronario agudo / Epidemiologic profile and delay to attend hospital ofpatients with acute coronary syndrome

Las enfermedades cardiovasculares son la primera causa de muerte en el mundo actual. La cardiopatía isquémica conlleva a una importante carga de gastos de Salud Pública, por lo cual es importante conocer la incidencia de pacientes con síndrome coronario agudo (SCA) y su respectivo comportamiento. Este trabajo estudió el tiempo de asistencia al Servicio de Urgencias desde el inicio de la angina de pecho en pacientes con SCA, y evaluó la relación entre factores de riesgo acumulado e incidencia de SCA con elevación del segmento ST (SCACEST). La población de estudio fue de 89 pacientes con SCA, siendo el promedio de edad general de 65 años, el 63% de los pacientes que era del sexo masculino tenía una edad promedio de 61,76 años y el resto del sexo femenino tenía como edad promedio 70,51 años. El 40% de los pacientes presentó SCACEST, el 24% del total de pacientes llegó antes de las 3 horas de iniciados los síntomas lo cual no guardó relación con la procedencia de los mismos. La hipertensión arterial y la diabetes mellitus fueron los factores de riesgo predominantes. Aún permanece alto el porcentaje de pacientes que acuden fuera del periodo terapéutico adecuado (76%), y esta cifra se mantiene sin diferencia significativa si se clasifican de acuerdo a la procedencia. Cada factor de riesgo cardiovascular aumenta la posibilidad de desarrollar un SCACEST a más del 10% en pacientes con SCA.

Cardiovascular diseases are the leading cause of death in the world. Ischemiccardiomyopathy produces an important economic burden to public health. Therefore, it isimportant to know the incidence of patients with acute coronary syndrome (ACS). Thisstudy investigates consultation time at the hospital emergency service from the beginningof symptoms in patients with ACS. In addition, it evaluates the relation betweenaccumulated risk factors and the incidence of ACS with ST segment elevation. The studypopulation comprised 89 patients with ACS with an average of 65 years of age. Sixty threepercent of the patients were male and had an average of 61.7 years of age while femalepopulation had an average of 70.5 years of age. The percentage of patients with ACS withST elevation was 40%. Only 24% of the total number of patients arrived within the first 3hours to the hospital, and it was unrelated to the place where they came from. Thepredominant risk factors were arterial hypertension and diabetes mellitus. The percentageof people who arrive out of the adequate therapeutic window (76%) still remain high.However, the difference is not statistical significant if it is classified by site of origin. Each of the cardiovascular risk factors increases by 10% the chance of developing ACS with STelevation in patients with ACS.

Sorbents with high efficiency for CO2 capture based on amines-supported carbon for biogas upgrading.

Sorbents for CO2 capture have been prepared by wet impregnation of a commercial active carbon (Ketjen-black, Akzo Nobel) with two CO2-philic compounds, polyethylenimine (PEI) and tetraethylenepentamine (TEPA), respectively. The effects of amine amount (from 10 to 70wt.%), CO2 concentration in the feed, sorption temperature and gas hourly space velocity on the CO2 capture performance have been investigated. The sorption capacity has been evaluated using the breakthrough method, with a fixed bed reactor equipped with on line gas chromatograph. The samples have been characterized by N2 adsorption-desorption, scanning electron microscopy and energy dispersive X-ray (SEM/EDX). A promising CO2 sorption capacity of 6.90 mmol/gsorbent has been obtained with 70wt.% of supported TEPA at 70°C under a stream containing 80vol% of CO2. Sorption tests, carried out with simulated biogas compositions (CH4/CO2 mixtures), have revealed an appreciable CO2 separation selectivity; stable performance was maintained for 20 adsorption-desorption cycles.

Viabilidad miocárdica: importancia clínica de su evaluación con estudios de medicina nuclear / Myocardial viability: clinical importante of the evaluation with nuclear medicine studies

El término miocardio viable hace referencia a que el músculo está vivo aunque exista una condición inicial que haga pensar que el mismo está necrótico. Esta condición es el déficit de motilidad ventricular global o regional, por lo tanto al hablar de viabilidad miocárdica aseveramos que exista disfunción ventricular reversible. El miocardio ventricular responde de forma diferente ante la reducción del flujo coronario, por ende se pueden encontrar distintos estados miocárdicos en un espectro que va desde el músculo con flujo normal y función conservada hasta encontrar en el otro extremo al músculo necrótico con ausencia total de flujo, déficit contráctil y daño irreversible en donde la restitución del flujo no mejora la función ventricular. El concepto de que la disfunción ventricular en reposo puede ser revertida luego de revascularización exitosa está actualmente bien establecido. La utilidad clínica de la evaluación de viabilidad está basada en que corrigiendo cada estado fisiopatológico se pueden obtener beneficios pronósticos y terapéuticos. De allí la relevancia de identificar prospectivamente pacientes con disfunción ventricular izquierda potencialmente reversible en quienes el pronóstico puede ser favorablemente alterado con revascularización miocárdica

Prueba de esfuerzo indicaciones más frecuentes en un medio hospitalario / Test of effort more frequent indications in hospitable means

Hace una revisión critica global que incluye, relevamiento de datos del paciente, protocolos utilizados, calidad técnica de los estuidos y el informe final del colega remitente