An environment for processing compound media streams

With today's widespread availability of networked multimedia potentials embedded in an infrastructure of qualitative superior kind the distribution of professionally styled multimedia streams has fallen in the realm of possibility. This paper presents a prototypic environment - both model and runtime system - for processing composite media streams variably composed of multimedia data objects. The system consists of an intelligent media database, a Web-authoring tool, a time directed presentation stream and is based on a hypermedia data model of reusable object components. The plug-in free runtime system is designed as a pure JAVA implementation. Further educational applications of our architecture are presented, as well

An environment for processing compound media streams : work in progress paper V 2.0

With today s widespread availability of networked multimedia potentials embedded in an infrastructure of qualitative superior kind the distribution of professionally styled multimedia streams has fallen in the realm of possibility. This paper presents a prototypic environment - both model and runtime system - for processing composite media streams variably composed of multimedia data objects. The system consists of an intelligent media database, a Web-authoring tool, a time directed presentation stream and is based on a hypermedia data model of reusable object components. The plug-in free runtime system is designed as a pure JAVA implementation. Further educational applications of our architecture are presented, as well

Continuous cardiac troponin I release in Fabry disease.

Fabry disease (FD) is a rare lysosomal storage disorder also affecting the heart. The aims of this study were to determine the frequency of cardiac troponin I (cTNI) elevation, a sensitive parameter reflecting myocardial damage, in a smaller cohort of FD-patients, and to analyze whether persistent cTNI can be a suitable biomarker to assess cardiac dysfunction in FD.cTNI values were determined at least twice per year in 14 FD-patients (6 males and 8 females) regularly followed-up in our centre. The data were related to other parameters of heart function including cardiac magnetic resonance imaging (cMRI).Three patients (21%) without specific vascular risk factors other than FD had persistent cTNI-elevations (range 0.05-0.71 ng/ml, normal: <0.01). cMRI disclosed late gadolinium enhancement (LGE) in all three individuals with cTNI values ≥0.01, while none of the 11 patients with cTNI <0.01 showed a pathological enhancement (p<0.01). Two subjects with increased cTNI-values underwent coronary angiography, excluding relevant stenoses. A myocardial biopsy performed in one during this procedure demonstrated substantial accumulation of globotriaosylceramide (Gb3) in cardiomyocytes.Continuous cTNI elevation seems to occur in a substantial proportion of patients with FD. The high accordance with LGE, reflecting cardiac dysfunction, suggests that cTNI-elevation can be a useful laboratory parameter for assessing myocardial damage in FD

An Environment for Processing Compound Media Streams - Work in progress paper V 2.0 -

With today s widespread availability of networked multimedia potentials embedded in an infrastructure of qualitative superior kind the distribution of professionally styled multimedia streams has fallen in the realm of possibility. This paper presents a prototypic environment - both model and runtime system - for processing composite media streams variably composed of multimedia data objects. The system consists of an intelligent media database, a Web-authoring tool, a time directed presentation stream and is based on a hypermedia data model of reusable object components. The plug-in free runtime system is designed as a pure JAVA implementation. Further educational applications of our architecture are presented, as well

Persistent increase in cardiac troponin I in Fabry disease: a case report

Abstract Background Hypertrophic cardiomyopathy is a frequent manifestation in Fabry disease (FD) - an X-linked lysosomal storage disorder caused by reduced activity of the enzyme α-galactosidase A. In FD an elevation of specific cardiac biomarkers, such as cardiac troponin I (cTNI) has been reported in case of clinical manifestation suggestive of myocardial ischemia. In diagnosing acute myocardial infarction cTNI is considered the most reliable parameter. Case Presentation In the referred case we present a 59 years old female patient with the diagnosis of FD presenting with persistently increased cTNI level (lowest value 0.46 ng/ml, highest value 0.69 ng/ml; normal range Conclusions Our case report demonstrates a persistent cTNI release in FD with cardiac involvement. Proving the persistence in a symptom free interval, it might be related to a direct damage of myocytes. In FD cTNI could serve as a beneficial long term parameter providing new perspectives for screening strategies.</p

Cardiac work up in patient 2.

<p>A+B: Coronary angiography (A: right coronary artery; B: left coronary artery) demonstrating no relevant pathology. C+D: Cardiac MRI showing increase in myocardial wall thickness (C) and pathological late gadolinium enhancement (D, arrow). E: Myocardial biopsy revealing strong accumulation of Gb_3, as indicated by numerous vacuoles within the cardiomyocytes (arrow).</p

Baseline data, medical history, biomarkers and cardiac work up in patients with FD in relation to cardiac troponin I elevation and normal values.

<p>*a small fibre dysfunction was proved by quantitative sensory testing or by skin biopsy.</p>†<p>measurement end-diastolic in the posterior wall of the left ventricle.</p>$<p>Arrhythmia was considered if one of the following conditions was detected: persistent or intermittent atrial fibrillation of flatter, sustained tachycardia (heart rate ≥100/minute for more than 30 seconds), non-sustained tachycardia (heart rate ≥100/minute for less than 30 seconds in at least 3 subsequent hear cycles), incomplete bundle branch block (QRS-duration: 100–119 ms) or complete bundle branch block (QRS-duration ≥120 ms).</p>§<p>lower level of quantification.</p