Differential diagnosis of perinatal hypophosphatasia: radiologic perspectives

Perinatal hypophosphatasia (HPP) is a rare, potentially life-threatening, inherited, systemic metabolic bone disease that can be difficult to recognize in utero and postnatally. Diagnosis is challenging because of the large number of skeletal dysplasias with overlapping clinical features. This review focuses on the role of fetal and neonatal imaging modalities in the differential diagnosis of perinatal HPP from other skeletal dysplasias (e.g., osteogenesis imperfecta, campomelic dysplasia, achondrogenesis subtypes, hypochondrogenesis, cleidocranial dysplasia). Perinatal HPP is associated with a broad spectrum of imaging findings that are characteristic of but do not occur in all cases of HPP and are not unique to HPP, such as shortening, bowing and angulation of the long bones, and slender, poorly ossified ribs and metaphyseal lucencies. Conversely, absent ossification of whole bones is characteristic of severe lethal HPP and is associated with very few other conditions. Certain features may help distinguish HPP from other skeletal dysplasias, such as sites of angulation of long bones, patterns of hypomineralization, and metaphyseal characteristics. In utero recognition of HPP allows for the assembly and preparation of a multidisciplinary care team before delivery and provides additional time to devise treatment strategies

Simulation of DC/DC Boost Converter by using Three-Phase Indirect Matrix Converter

In this paper, a new circuit topology is presented, which is composed of an indirect matrix converter (IMC) and a dc/dc boost converter that connects to the neutral point of a motor. An indirect matrix converter (IMC) connected with two input power sources: a gasoline generator as the main ac power supply and batteries as the secondary power source. The IMC is small in size because of having a dc-link part without an electrolytic capacitor. The dc-link part is utilized by connection with a boost-up chopper with batteries as a secondary input power source. The proposed technique successfully further reduce the size of the converter by removing the boos

Effect of combined addition of Si and Sb on the microstructure and creep properties of AZ91 magnesium alloy

Addition of silicon (0.5 wt.%) together with small amount of antimony (0.2 wt.%) to AZ91 alloy has been carried out to study its effect on the microstructure and creep properties at 150 and 200 °C. The results show that the combined addition (Si and Sb) introduces fine polygonal shaped Mg2Si precipitates at the grain boundaries and in the grains. Besides, the addition suppresses the precipitation of discontinuous Mg17Al12 phase during ageing treatment. These lead to an improvement in creep behavior of AZ91 at 150 and 200 °C temperatures. Moreover, extended steady state creep, reduced minimum creep rate and total creep strain are also observed with the combined addition. Presence of fine and stable Mg2Si precipitates and suppression of discontinuous precipitation are the possible reasons for the improvement in creep properties

Assessment of regional transmission connection capacity based on two novel indices

In deregulated market environments, transmission system expansion planning becomes a troublesome task as transmission system operators lose their direct control over plant investments. Moreover, the increase in the share of renewable sources in electricity generation creates an uncertainty about future generation investments in terms of location and size. Assessment of transmission connection capacities, which determines the boundaries of new generation amount that can safely be connected from a region, is a mechanism that facilitates economic and reliable transmission expansion. In this study, use of two novel indices, namely region-based evaluation index (REI) and line-based evaluation index (LEI), is proposed for this purpose. REI aims to indicate the suitability of a region for new generation investments from a holistic point of view. Its calculation is based on the loadings of the lines under N-1 conditions, and their overloading probabilities. LEI, on the other hand, is usable to assess each variation of the considered line's loading. By tracking LEI of a line, one can follow if a line's loading exceeds limits during contingencies, and observe if the loading benefits from additional generation. It allows identifying the weak points of the analysed system. The proposed methodology was demonstrated on projected Turkish Transmission Network model of 2026