Incidence, disease onset and short-term outcome in urea cycle disorders – cross-border surveillance in Germany, Austria and Switzerland

Background: Urea cycle disorders (UCDs) are a group of rare inherited metabolic disorders. Affected individuals often present with hyperammonemic encephalopathy (HE) and have an increased risk of severe neurologic disease and early death. The study aims to provide epidemiologic data and to describe the disease manifestation and short-term outcome. Method: Cross-border surveillance of newly diagnosed patients with UCDs - below 16 years of age - was performed from July 2012 to June 2015 in Germany and Austria and from January 2012 to December 2015 in Switzerland. Inquiries were sent monthly to all Pediatric Departments in Germany and Switzerland, and quarterly to the Austrian Metabolic Group. In addition, data were collected via a second source (metabolic laboratories) in all three countries. Results: Between July 2012 and June 2015, fifty patients (Germany: 39, Austria: 7, Switzerland: 4) with newly diagnosed UCDs were reported and later confirmed resulting in an estimated cumulative incidence of 1 in 51,946 live births. At diagnosis, thirty-nine patients were symptomatic and 11 asymptomatic [10 identified by newborn screening (NBS), 1 by high-risk-family screening (HRF)]. The majority of symptomatic patients (30 of 39 patients) developed HE with (n = 25) or without coma (n = 5), 28 of them with neonatal onset. Despite emergency treatment 15 of 30 patients with HE already died during the newborn period. Noteworthy, 10 of 11 patients diagnosed by NBS or HRF remained asymptomatic. Comparison with the European registry and network for intoxication type metabolic diseases (E-IMD) demonstrated that cross-national surveillance identified a higher number of clinically severe UCD patients characterized by earlier onset of symptoms, higher peak ammonium concentrations in plasma and higher mortality. Conclusion: Cross-border surveillance is a powerful tool to identify patients with UCDs demonstrating that (1) the cumulative incidence of UCDs is lower than originally suggested, (2) the mortality rate is still high in patients with neonatal onset of symptoms, and (3) onset type and peak plasma ammonium concentration predict mortality

BZ-BattExt – DMFC as Battery-Extender in solar-boat application

For special applications Direct Methanol Fuel Cells (DMFC) are close to commercial application or already commercialized today. However for the step from laboratory to a broader market of fuel cells, a significant cost reduction, as well as an improvement in life time and power density of the systems is needed. These items were the focus of the project BZ-BattExt, to be reached by new knowledge in alternative materials, operation strategies as also the realisation of enhanced sub systems. This project is funded by the German Federal Ministry of Education and Research in the program of Micro fuel cells. In the project the feasibility of a micro-DMFC system is evaluated which enables a minimised system periphery due to an improved system architecture. For this, alternative materials and functional components were developed and investigated. New membranes with reduced water and methanol permeation allow operation at low air stoichiometry and favourable system efficiency. Gas diffusion layers of various compositions were tested and optimised material was selected. New sealing materials with good methanol stability and optimized processibility in commercial production process were developed. MEA preparation was adapted to the new materials. The use of a simple, cost-effective way of stack production was demonstrated for DMFC use. The investigation and construction of enhanced subsystems and operation strategies, which enable and optimise the use of new components and materials, as also the realisation of the micro-DMFC system is a focus of the project. The technical feasibility of the results is investigated in the application, which means it is tested as battery extender of a solar boat. The DMFC fuel cell system serves as a basis for an efficient, compact and cost effective alternative for battery- or battery-extender systems and can fulfil a broad variety of applications

DMFC as Battery-Extender in solar-boat application

For special applications Direct Methanol Fuel Cells (DMFC) are close to commercial application or already commercialized today. However for the step from laboratory to a broader market of fuel cells, a significant cost reduction, as well as a lifetime and power density improvement of the systems is needed. The Goals of the BZ-BattExt Project should be reached by applying new knowledge in alternative materials, improved operation strategies and enhanced sub systems. In the project a 100 W DMFC compact system as battery extender was successfully developed and operated. The reduction of the number of components and the simplification of the system led to a high reduction in price, weight and volume. The feasibility of a micro-DMFC system was evaluated which enables a minimised system periphery due to an improved System Architecture. For this, alternative materials and functional components were developed and investigated leading to new membranes with reduced water and methanol permeation allowing a low air stoich operation and higher system efficiency. Gas diffusion layers of various compositions were tested and optimised materials were selected. New sealing materials with good methanol stability and optimized processibility in commercial production Processes were developed and the MEA preparation was adapted to the new materials. The use of a simple, cost-effective way of stack production was demonstrated for DMFC use. Using this new components and materials, coupled with the enhanced subsystem architectures and enhanced operation strategies, the build up and start-up of an improved micro DMFC System was achieved. The technical feasibility of the Results was investigated in the real application. The micro DMFC System was used as a battery range extender in a 6m solar boat. The DMFC fuel cell system serves as a basis for an efficient, compact and cost effective alternative for battery- or battery-extender systems and can fulfil a broad variety of applications

Generation of Negative Air Ions by Use of Piezoelectric Cold Plasma Generator

The negative air ions (NAI) are used for the removal of particles or droplets from the air. In this study, three types of piezoelectric cold plasma generators (PCPG), in combination with cylindrical electrostatic ion filters, are applied for NAI production. The high voltage on the filter cylinder is induced by the electric field from the piezoelectric transformer of the PCPG. To achieve the dc bias, the cylinder of the electrostatic filter is connected to the ground over ultrafast switching diodes. The ion concentrations are measured for different airflows, PCPG powers, and electrostatic filter geometries. The NAI concentration in the order of magnitude of 107 cm−3, and a negative-to-positive ion concentration ratio of over 200 is reached. The production of ozone is evaluated and the PCPG configuration with a minimum ozone production rate is proposed. The ozone concentration below 60 ppb is reached in the airflow of 90 m3/h

Piezoelectric Direct Discharge: Devices and Applications

The piezoelectric direct discharge (PDD) is a comparatively new type of atmospheric pressure gaseous discharge for production of cold plasma. The generation of such discharge is possible using the piezoelectric cold plasma generator (PCPG) which comprises the resonant piezoelectric transformer (RPT) with voltage transformation ratio of more than 1000, allowing for reaching the output voltage >10 kV at low input voltage, typically below 25 V. As ionization gas for the PDD, either air or various gas mixtures are used. Despite some similarities with corona discharge and dielectric barrier discharge, the ignition of micro-discharges directly at the ceramic surface makes PDD unique in its physics and application potential. The PDD is used directly, in open discharge structures, mainly for treatment of electrically nonconducting surfaces. It is also applied as a plasma bridge to bias different excitation electrodes, applicable for a broad range of substrate materials. In this review, the most important architectures of the PDD based discharges are presented. The operation principle, the main operational characteristics and the example applications, exploiting the specific properties of the discharge configurations, are discussed. Due to the moderate power achievable by PCPG, of typically less than 10 W, the focus of this review is on applications involving thermally sensitive materials, including food, organic tissues, and liquids

Visualization of Activated Area on Polymers for Evaluation of Atmospheric Pressure Plasma Jets

The treatment of a polymer surface using an atmospheric pressure plasma jet (APPJ) causes a local increase of the surface free energy (SFE). The plasma-treated zone can be visualized with the use of a test ink and quantitatively evaluated. However, the inked area is shrinking with time. The shrinkage characteristics are collected using activation image recording (AIR). The recording is conducted by a digital camera. The physical mechanisms of activation area shrinkage are discussed. The error sources are analyzed and methods of error reduction are proposed. The standard deviation of the activation area is less than 3%. Three polymers, acrylonitrile butadiene styrene (ABS), high-density polyethylene (HDPE), and polyoxymethylene (POM), are examined as a test substrate material. Due to a wide variation range of SFE and a small hydrophobic recovery, HDPE is chosen. Since the chemical mixtures tend to temporal changes of the stoichiometry, the pure formamide test ink with 58 mN/m is selected. The method is tested for the characterization of five different types of discharge: (i) pulsed arc APPJ with the power of about 700 W; (ii) piezoelectric direct discharge APPJ; (iii) piezoelectric driven needle corona in ambient air; (iv) piezoelectric driven plasma needle in argon; and (v) piezoelectric driven dielectric barrier discharge (DBD). For piezoelectrically driven discharges, the power was either 4.5 W or 8 W. It is shown how the AIR method can be used to solve different engineering problems

Fuel Cell System for Solar Boat Battery-Extender - BZ-BattExt -

For special applications Direct Methanol Fuel Cells (DMFC) are close to commercial application or already commercialized today. However for the step from laboratory to a broader market of fuel cells, significant cost reduction, as also an improvement in life time and power density of the systems is needed. These items were the focus of the project BZ-BattExt, to be reached by new knowledge in alternative materials, operation strategies as also the realisation of enhanced sub systems. This project is funded by the German Federal Ministry of Education and Research in the program of Micro fuel cells. In the project the feasibility of a micro-DMFC-system is evaluated which enables a minimised system periphery due to an improved system architecture. For this, alternative materials and functional components were developed and investigated. New membranes with reduced water and methanol permeation allow operation at low air stoichiometry, favourable for system efficiency. Gas diffusion layers of various compositions were tested and optimised material selected. New sealing materials with good methanol stability and optimized processibility in commercial production process were developed. MEA preparation was adapted to the new materials. The use of a simple, cost-effective way of stack production was demonstrated for DMFC use. The investigation and construction of enhanced subsystems and operation strategies, which enable and optimise the use of new components and materials, as also the realisation of the micro-DMFC-system is a focus of the project. The technical feasibility of the results is investigated application oriented, which means it is tested as battery extender of a solar boat. The fuel cell system serves as a basis for an efficient, compact and cost effective micro-DMFC-System as alternative for battery- or battery-extender systems which covers a broad variety of applications