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The bile salt export pump (BSEP, ABCB11) is the major transporter protein for the excretion of bile salts into bile. Here we describe a spectrum of BSEP-dependent effects on the course of liver diseases, and present two mutations that differentially affect total bile acid output and the biliary bile acid profile. According to the clinical course of affected(More)
Neurally mediated syncope (NMS) patients suffer from sudden loss of consciousness, which is associated with a high rate of falls and hospitalization. NMS negatively impacts a subject's quality of life and is a growing cost issue in our aging society, as its incidence increases with age. In this paper, we present a solution for prediction of NMS, which is(More)
Syncope is a common disorder with a lifetime prevalence of about 40%. Implantable cardiac electronic devices, including implantable loop recorders (ILR) and implantable cardioverter-defibrillators (ICD), are well established in syncope management. However, despite the successful use of ILR and ICD, diagnosis and therapy still remain challenging in many(More)
Neurally mediated syncope (NMS) is a disorder of the autonomic regulation of postural tone, which is characterized by hypotension and/or bradycardia, resulting in cerebral hypo-perfusion and finally in a sudden loss of consciousness. Prediction of an impending NMS requires detection of pulse presence to derive heart rate (HR) as well as to assess the pulse(More)
Monitoring of cardiovascular function on a beat-to-beat basis is fundamental for protecting patients in different settings including emergency medicine and interventional cardiology, but still faces technical challenges and several limitations. In the present study, we propose a new method for the extraction of cardiovascular performance surrogates from(More)
Urea cycle defects belong to the most common metabolic disorders with a cumulative incidence of 1:8000. A common trait of urea cycle defects is a disturbed detoxification of ammonia leading to hyperammonemia in the event of a high nitrogen load. Most patients develop symptoms in the neonatal period or in infancy, e. g. vomiting, seizures and disturbed(More)
The use of a novel irrigated multipolar ablation and mapping catheter for pulmonary vein isolation in patients with atrial fibrillation (AF) has demonstrated reasonable acute success rates and short procedure times, however, long-term outcome data are limited. The aim of this study was to analyze the long-term efficacy of this novel ablation system(More)
Pulmonary vein isolation (PVI) is a cornerstone therapy in patients with atrial fibrillation (AF). With increasing numbers of PVI procedures, demand arises to reduce the cumulative fluoroscopic radiation exposure for both the physician and the patient. New technologies are emerging to address this issue. Here, we report our first experiences with a new(More)
Neurally medicated syncope (NMS) patients suffer from sudden loss of consciousness, which is associated with a high rate of falls and hospitalization. NMS negatively impacts a subject's quality of life and is a growing cost issue for the healthcare systems in particular since mainly elderly are at risk of NMS in our aging societies. In the present paper we(More)
Two methodologies for neurally mediated syncope (NMS) prediction, based on the joint analysis of the electrocardiogram (ECG) and photoplethysmogram (PPG), are compared. Several features that characterize the variations in the inotropic, chronotropic, vascular tone and blood pressure surrogates were extracted and fed into two prediction models. The first(More)
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