Recent Developments in Osteogenesis Imperfecta

Osteogenesis imperfecta (OI) is an uncommon genetic bone disease associated with brittle bones and fractures in children and adults. Although OI is most commonly associated with mutations of the genes for type I collagen, many other genes (some associated with type I collagen processing) have now been identified. The genetics of OI and advances in our understanding of the biomechanical properties of OI bone are reviewed in this article. Treatment includes physiotherapy, fall prevention, and sometimes orthopedic procedures. In this brief review, we will also discuss current understanding of pharmacologic therapies for treatment of OI

The Use of Populism as a Pragmatist Approach in Indonesia

Populism usually appears when there is dissatisfaction towards the system, and creates two different camps seen as polarization, “the elite” versus “the people.” Those camps are a result of the appeal of anti-elite. Indonesian populist leader also uses the strategy to appeal a large support of citizens, but the strategy must be understood as a part of pragmatist leadership. This paper argues that the utilization of populism in Indonesia is more motivated by pragmatic reasons rather than fundamental reasons. This pragmatic reason can be seen through inconsistent statement in the leadership which, at first, supports populist claim but tends to be different in reality. Political leaders in Indonesia try to strive for a progress as well as to seek a compromise and a support from various groups, including those labeled as elite by the populists themselves. Through an observation of secondary documents from journals and news articles during the latest presidential campaign between Jokowi and Prabowo in 2019, it is concluded that all characteristics of populist strategy have been used pragmatically by both leaders. It is applied to attract more supports in order to respect the political constraints and to work with the system

Silage von früh genutzter Luzerne (Medicago sativa) als Eiweiß- und Raufuttermittel in der ökologischen Broilermast

In der Gruppe der kleinkörnigen Leguminosen weisen Luzerne und Weißklee die höchsten Protein- und Aminosäurengehalte (Lysin, Methionin) sowie die höchsten Erträge pro ha auf. Luzerne, die in einem sehr frühen Vegetationsstadium geworben wurde, kann einen Proteingehalt von 300 g/kg TS, einen Lysingehalt von 18 g/kg TS und einen Methioningehalt von 5g/kg TS erreichen. Ein solches Material kann verlustarm konserviert werden, wenn die Prinzipien des Silierens konsequent angewendet werden. Ein zusätzliches Extrudieren des angewelkten Materials verbessert den Siliererfolg nicht. Bezogen auf die täglich Gesamttrockenmasseaufnahme nehmen Broiler große Mengen Luzernesilage auf: 10-20 % in der Aufzuchtphase und 30 % in der Mastphase. Um den niedrigen ME-Gehalt der Silage dabei auszugleichen, ist es wichtig ergänzend ein darauf ausgerichtetes Kraftfutter anzubieten

The Impact of Early Childhood Rainfall Shocks on the Evolution of Cognitive and Non-cognitive Skills

This paper is the first to estimate the extent to which early childhood climatic shocks affect both cognitive and non-cognitive skills as measured at multiple points in childhood and adolescence. We assess the impact of rainfall observed in utero and during the first two years of life by analyzing a rich longitudinal study of rural youth in a poor province in China. Our empirical strategy entails estimating the impact of rainfall on various measures of cognitive and non-cognitive skills utilizing a reduced form strategy, conditional on county and year-of-birth fixed effects. The results indicate that there is a significant impact of early shocks, particularly shocks in utero and in the first year of life, on cognitive skills, but that this impact may be declining over time. There is little evidence of any impact on non-cognitive skills. We also present evidence that the declining salience of early shocks is consistent with compensatory strategies employed by parents

Biomechanics of Osteogenesis Imperfecta: Current Concepts and Emerging Horizons

Osteogenesis inperfecta (OI) is associated with bone fragility. Long bone fractures are a common occurrence in individuals with OI. Although there have been significant advances in understanding the genetic defects associated with OI, the mechanisms behind bone fragility in this patient population are not yet well understood. This fragility is believed to stem in part from characteristic bone mass deficiencies. Research further suggests that the material properties of the bone are also compromised in individuals with this disorder. There is currently on quantitative method available to assess bone fracture risk in individuals with OI. This chapter examines several critical elements needed to assess bone fracture risk through a unified biomechanical modeling approach. Finite element modeling (FEM) lies at the core of this approach with reliance upon material property and load data. The former stems from micro- and macrostructural scale characterization of bone material properties while the latter derives from assessment of mobility and other activities. As improved tools are developed for fracture risk assessment, clinicians will be afforded more effective methods to examine interventional effects and rehabilitative strategies in the short-and long-term with an ultimate goal of fracture reduction

Ab initio Calculation of Binding Energies of Interstellar Sulphur-Containing Species on Crystalline Water Ice Models

There are different environments in the interstellar medium (ISM), depending on the density, temperature and chemical composition. Among them, molecular clouds, often referred to as the cradle of stars, are paradigmatic environments relative to the chemical diversity and complexity in space. Indeed, there, radio to far-infrared observations revealed the presence of several molecules in the gas phase, while near-infrared spectroscopy detected the existence of submicron sized dust grains covered by H2O-dominated ice mantles. The interaction between gas-phase species and the surfaces of water ices is measured by the binding energy (BE), a crucial parameter in astrochemical modelling. In this work, the BEs of a set of sulphur-containing species on water ice mantles have been computed by adopting a periodic ab initio approach using a crystalline surface model. The Density Functional Theory (DFT)-based B3LYP-D3(BJ) functional was used for the prediction of the structures and energetics. DFT BEs were refined by adopting an ONIOM-like procedure to estimate them at CCSD(T) level toward complete basis set extrapolation, in which a very good correlation between values has been found. Moreover, we show that geometry optimization with the computationally cheaper HF-3c method followed by single point energy calculations at DFT to compute the BEs is a suitable cost-effective recipe to arrive at BE values of the same quality as those computed at full DFT level. Finally, computed data were compared with the available literature data