rogersmcclure190992
Familial chylomicronemia is a disease in which a genetic mutation affects the ability of the organism to metabolize triglycerides bound to lipoproteins, causing extremely high plasma triglycerides and associated consequences. The most frequent complication is acute pancreatitis, which may lead to multiorganic failure or pancreatic insufficiency. Familial chylomicronemia also exerts a profound negative impact on quality of life, social relationships and professional development. The gene most frequently affected is lipoprotein lipase-1 gene (LPL), the enzyme in charge of hydrolyzing circulating triglycerides for tissue uptake. Mutations in other genes regulating maturation, transport or polymerization (eg. APOC2, APOAV, LMF-1, GPIHBP-1) of lipoprotein lipase-1, may also be involved. However, in about 30% of patients the causal variant is not identified. https://www.selleckchem.com/products/sorafenib.html Familial chylomicronemia should be suspected in patients with severe hypertriglyceridemia with poor response to conventional treatment, or accompanied by eruptive xanthomas, lipemia retinalis or abdominal pain. The availability of risk scores and genetic tests should facilitate its opportune detection and management. Nutritional therapy is based on a very-low-fat diet with adequate supply of lipid-soluble vitamins and essential fatty acids, plus avoidance of alcohol consumption. Current pharmacological treatment may include fibrates and omega-3 fatty acids but prioritizes biotechnological agents targeting the molecular disturbances of the disease. These include an antisense oligonucleotide against apoC-III (volanesorsen), a monoclonal antibody against angiopoietin-like protein-3 (evinacumab), and other agents currently in development.The six-minute walk test has been generally applied in people with pathologies and some studies have proposed models to predict maximum oxygen consumption. Our objective was to elaborate on an equation to predict the maximum oxygen consumption in the six-minute walking test for university students. A hundred and forty people participated in this study. The six-minute walking test was applied and after on a gradual exercise test was performed to determine the maximum oxygen consumption. A multivariate equation was developed and the analysis was done using the SPSS v.22 program (p less then 0.05). The predictive model include gender, age, body mass index, distance performed and heart rate recovery (r = 0.83; p less then 0.001). The equation fulfilled the assumptions of independence (p = 0.13), normality (p = 0.49) and homoscedasticity (p = 0.64). The Bland-Altman diagram indicated that there were no significant differences between the equation and the measurement of the maximum oxygen consumption (p = 0.89), with a confidence interval of 0.054 ml·kg·min-1 (95% CI [-0.72; 0.83]). The equation predicts the maximum oxygen consumption. It is suggested to evaluate university students considering biological and environmental differences between countries.Bloodstream infections (BI) are an important cause of morbidity and mortality in cancer patients. A retrospective study was performed in two hospitals aimed to evaluate characteristics of BI episodes occurred in adult patients with hematologic (HN) and solid (SN) neoplasia other than non-melanoma skin cancers in the period 2009-2016. A total of 467 episodes of bacteremia and 16 of fungemia were identified. A total of 200 (41.4%) bacteremias occurred in patients with HN and 283 (58.6%) in patients with SN. The most frequent SN and HN were colon cancer (18.7%) and non-Hodgkin lymphoma (27%), respectively. The main risk factors for BI were a previous surgical procedure in SN and chemotherapy in the previous 30 days and use of central venous catheter in HN. Infections were mainly acquired in the hospital environment and the most frequent presentation was bacteremia without focus, mostly in HN (38% vs. 20.8%, p less then 0.001). Gram negative bacilli (GNB) were isolated in 336 (69.5%) episodes and predominated over Gram positive cocci (GPC) in both groups. Escherichia coli was the most frequent GNB isolated in both SN (24.7%) and HN patients (20.5%). The most frequent GPC was Staphylococcus aureus. Multidrug-resistance was found in 15% of the isolates in SN and 18% in HN. The overall mortality was 40.5% in patients with HN and 37.5% in patients with SN, with the majority of deaths occurring in the first 30 days.Neurological complications in orthotopic heart transplantation represent a major cause of morbidity and mortality despite successful transplantation. The aim of our study was to evaluate neurological complications on the outcome of patients with heart transplantation. We retrospectively studied 193 adult patients (aged = 18 years) who underwent heart transplant at Hospital Italiano in Buenos Aires from November 2014 to August 2018. We evaluated demographic and clinical vari ables and outcome of patients with and without neurological complications. We included 193 patients with a mean age of 51 ± 12 years of which 74% (n = 143) were men. The two most frequent causes that led to heart transplantation were idiopathic cardiomyopathy in 34% (n = 65) and ischemic cardiomyopathy in 29% (n = 56). Hemodynamic instability was present at the moment of transplant in 92% (n = 176) of the cases. Central neurological complications in the first week post-transplant occurred in 12% (n = 23). The most frequent were encephalopathy (5%), subdural hematoma (2%), subarachnoid hemorrhage (2%), seizures (2%) and ischemic stroke (1%). Peripheral neuropathy was observed in 4% of cases. Hospital mortality was 11% (n = 22) and 88% (n = 170) was discharged at home. Those who presented central neurological complications had higher in-hospital mortality compared to those who did not (32% vs. 9%, p = 0.002).Polysomnography without real-time technical supervision (sleep test level II) h as been described with adequate quality of neurological and respiratory signals. We compare the efficiency and quality of sleep in hospitalized patients and in the sleep laboratory. The study was retrospective, in a consecutive sample of systematic collection based on PSG level II. We include 486 patients 156 hospitalized and 330 outpatients; 94 men (60.2%) vs. 181 (55%); age 67.5 ± 12.8 vs. 59.3 ± 14.7 years, p 3 hours, the efficiency (%) was lower in hospitalized 73.3 (60.8-82.1) vs. 78.5 (67.1-86.2), p less then 0.01. In hospitalized patients the quality and efficiency of sleep were lower with a small proportion of patients sleeping four hours during a polysomnography test.