Markers of Hemophagocytic Lymphohistiocytosis Are Associated with Mortality in Critically Ill Patients.

Critically ill patients often display systemic immune dysregulation and increased inflammatory activity. Hemophagocytic lymphohistiocytosis (HLH) represents a rare syndrome defined by the inappropriate survival of cytotoxic T cells and the occurrence of cytokine storms. Although HLH is characterized by relatively high mortality rates, little is known about the predictive value of its diagnostic criteria.

NT-proBNP Reflects Left Ventricular Hypertrophy Rather than Left Ventricular Dilatation or Systolic Dysfunction in Patients with Fabry Disease.

: The diagnosis and follow-up of cardiac involvement in Fabry disease constitutes an important challenge for clinicians caring for affected patients. Combining cardiac imaging with laboratory biomarkers appears most appropriate for longitudinal monitoring. Therefore, we examined the use of NT-proBNP and its association with imaging findings in patients with Fabry disease.

Pregnancy outcomes of Fabry disease in Austria (PROFABIA)-a retrospective cohort-study.

Background: Pregnancy and delivery outcomes in women with Fabry disease are not well described.

Methods: Retrospective cohort-study of women with Fabry disease in Austria using a specific questionnaire and the Austrian Mother-Child Health Passport.

Results: Out of a total of 44 enrolled women (median age at study entry 44 years, p25: 30, p75: 51), 86.

Phenotyping of a novel and novel variant in a patient presenting with microhematuria and mildly impaired kidney function: a case report.

We describe the case of a 44-year-old male patient with a longstanding history of microhematuria and mildly impaired kidney function (CKD G2A1). The family history disclosed three females who also had microhematuria. Genetic testing by whole exome sequencing revealed two novel variants in (NM_000092.

Long-Term Monitoring of Cardiac Involvement under Migalastat Treatment Using Magnetic Resonance Tomography in Fabry Disease.

Fabry cardiomyopathy is characterized by left ventricular hypertrophy, myocardial fibrosis, arrhythmia, and premature death. Treatment with migalastat, an oral pharmacological chaperone, was associated with a stabilization of cardiac biomarkers and a reduction in left ventricular mass index, as measured by echocardiography. A recent study, using cardiac magnetic resonance (CMR) as the gold standard, found a stable course of myocardial involvement after 18 months of treatment with migalastat.

Differences in Automated External Defibrillator Types in Out-of-Hospital Cardiac Arrest Treated by Police First Responders.

Police first responder systems also including automated external defibrillation (AED) has in the past shown considerable impact on favourable outcomes after out-of-hospital cardiac arrest (OHCA). While short hands-off times in chest compressions are known to be beneficial, various AED models use different algorithms, inducing longer or shorter durations of crucial timeframes along basic life support (BLS). Yet, data on details of these differences, and also of their potential impact on clinical outcomes are scarce.

Risk stratification and long-term outcome of patients receiving in-hospital medical emergency team critical care: experience from Austria's largest medical center.

Background: We aimed to investigate predictors for long-term survival of in-hospital patients with medical emergency team (MET) consultation with or without in-hospital cardiac arrest (IHCA) in Austria's largest medical center.

Methods: Data of patients, who needed an intervention of a MET between 01/2014 and 03/2020 were reviewed for this retrospective analysis.

Results: In total, 708 MET calls were analyzed.

RANTES and CD40L under Conditions of Long-Term Physical Exercise: A Potential Link to Adaptive Immunity.

Regular physical exercise was found to be associated with an improved immune response in previous studies. RANTES and CD40L play a pivotal role in host defense, and individuals lacking adequate expression are prone to virus and opportunistic infections. A total of 98 participants were enrolled in this study.

Cardiac remodeling in ambitious endurance-trained amateur athletes older than 50 years-an observational study.

Background: Data on cardiac remodeling in veteran athletes are conflicting but of clinical importance.

Methods: Sixty-nine clinically stable and healthy individuals >50 years were identified (median 55 (IQR 52-64), 26% female). Echocardiographic features were identified in individuals, who have performed endurance sports at 70% of their maximum heart rate for at least 1 hour 3 times/ week over the previous 5 years.

Identification of Subclinical Microvascular Biomarkers in Coronary Heart Disease in Retinal Imaging.

Purpose: Cardiovascular disease and foremost coronary heart disease (CHD) are the worldwide leading causes of death. The aim of this study was to use non-invasive, multimodel retinal imaging to define microvascular features in patients with and without coronary angiography (CA)-confirmed CHD.

Methods: In this prospective, cross-sectional pilot study we included adult patients who presented to a tertiary referral center for elective CA due to suspected CHD.

MiRNA Let-7a and Let-7d Are Induced by Globotriaosylceramide via NF-kB Activation in Fabry Disease.

Background: Fabry disease is a hereditary genetic defect resulting in reduced activity of the enzyme α-galactosidase-A and the accumulation of globotriaosylceramide (Gb3) in body fluids and cells. Gb3 accumulation was especially reported for the vascular endothelium in several organs.

Methods: Three Fabry disease patients were screened using a micro-RNA screen.

Long-term physical activity modulates adipsin and ANGPTL4 serum levels, a potential link to exercise-induced metabolic changes.

Background: Within the presented prospective study, we aimed to illuminate the effect of long-term physical exercise on serum levels of adipsin (complement factor D) and angiopoietin-like 4 (ANGPTL4). Although past studies already outlined the effects of acute exercise, our trial design aimed to depict the development under long-term physical activity conditions.

Methods: Ninety-eight participants were included in the study and were asked to perform eight months of moderate physical activity for at least 150 minutes/week and/or vigorous-intensity exercise for at least 75 minutes/week.

Safety and image quality of cardiovascular magnetic resonance imaging in patients with retained epicardial pacing wires after heart transplantation.

Background: Temporary epicardial pacing wires, implemented in patients during heart transplantation, are routinely removed before discharge. However, in some cases, these wires may remain in situ and are often considered as a contraindication for cardiovascular magnetic resonance (CMR) imaging in the future. Therefore, we aimed to provide data about safety and image quality of CMR in these patients.

Adaptive development of concomitant secondary mitral and tricuspid regurgitation after transcatheter aortic valve replacement.

Aims: Concomitant secondary atrioventricular regurgitation is frequent in patients with severe aortic stenosis scheduled for transcatheter aortic valve replacement (TAVR). The future implications of leaving associated valve lesions untreated after TAVR remain unknown. Aim of the present study was to characterize the evolution of concomitant secondary atrioventricular regurgitations and to evaluate their impact on long-term prognosis.