Clinical and biochemical response to neridronate treatment in a patient with osteoporosis-pseudoglioma syndrome (OPPG)

Osteoporosis-pseudoglioma syndrome (OPPG) is a rare autosomal recessive syndrome characterized by juvenile-onset osteoporosis and ocular abnormalities due to a low-density lipoprotein receptor-related protein 5 (LRP5) gene mutation. Treatment with bisphosphonates, particularly with pamidronate and risedronate, has been reported to be of some efficacy in this condition. We report on a patient with OPPG due to an LRP5 gene mutation, who showed an encouraging response after a 36-month period of neridronate therapy. We report a case of a patient treated with bisphosphonates. Bisphosphonates should be administered in OPPG patients as a first-line therapy during early childhood

Analysis of Round Off Errors with Reversibility Test as a Dynamical Indicator

We compare the divergence of orbits and the reversibility error for discrete time dynamical systems. These two quantities are used to explore the behavior of the global error induced by round off in the computation of orbits. The similarity of results found for any system we have analysed suggests the use of the reversibility error, whose computation is straightforward since it does not require the knowledge of the exact orbit, as a dynamical indicator. The statistics of fluctuations induced by round off for an ensemble of initial conditions has been compared with the results obtained in the case of random perturbations. Significant differences are observed in the case of regular orbits due to the correlations of round off error, whereas the results obtained for the chaotic case are nearly the same. Both the reversibility error and the orbit divergence computed for the same number of iterations on the whole phase space provide an insight on the local dynamical properties with a detail comparable with other dynamical indicators based on variational methods such as the finite time maximum Lyapunov characteristic exponent, the mean exponential growth factor of nearby orbits and the smaller alignment index. For 2D symplectic maps the differentiation between regular and chaotic regions is well full-filled. For 4D symplectic maps the structure of the resonance web as well as the nearby weakly chaotic regions are accurately described.Comment: International Journal of Bifurcation and Chaos, 201

3D solutions of the Poisson-Vlasov equations for a charged plasma and particle-core model in a line of FODO cells

We consider a charged plasma of positive ions in a periodic focusing channel of quadrupolar magnets in the presence of RF cavities. The ions are bunched into charged triaxial ellipsoids and their description requires the solution of a fully 3D Poisson-Vlasov equation. We also analyze the trajectories of test particles in the exterior of the ion bunches in order to estimate their diffusion rate. This rate is relevant for a high intensity linac (TRASCO project). A numerical PIC scheme to integrate the Poisson-Vlasov equations in a periodic focusing system in 2 and 3 space dimensions is presented. The scheme consists of a single particle symplectic integrator and a Poisson solver based on FFT plus tri-diagonal matrix inversion. In the 2D version arbitrary boundary conditions can be chosen. Since no analytical self-consistent 3D solution is known, we chose an initial Neuffer-KV distribution in phase space, whose electric field is close to the one generated by a uniformly filled ellipsoid. For a matched (periodic) beam the orbits of test particles moving in the field of an ellipsoidal bunch, whose semi-axis satisfy the envelope equations, is similar to the orbits generated by the self-consistent charge distribition obtained from the PIC simulation, even though it relaxes to a Fermi-Dirac-like distribution. After a transient the RMS radii and emittances have small amplitude oscillations. The PIC simulations for a mismatched (quasiperiodic) beam are no longer comparable with the ellipsoidal bunch model even though the qualitative behavior is the same, namely a stronger diffusion due to the increase of resonances

A discussion of possible approaches to the integration of thermochemical storage systems in concentrating solar power plants

One of the most interesting perspectives for the development of concentrated solar power (CSP) is the storage of solar energy on a seasonal basis, intending to exploit the summer solar radiation in excess and use it in the winter months, thus stabilizing the yearly production and increasing the capacity factor of the plant. By using materials subject to reversible chemical reactions, and thus storing the thermal energy in the form of chemical energy, thermochemical storage systems can potentially serve to this purpose. The present work focuses on the identification of possible integration solutions between CSP plants and thermochemical systems for long-term energy storage, particularly for high-temperature systems such as central receiver plants. The analysis is restricted to storage systems potentially compatible with temperatures ranging from 700 to 1000 ◦C and using gases as heat transfer fluids. On the basis of the solar plant specifications, suitable reactive systems are identified and the process interfaces for the integration of solar plant/storage system/power block are discussed. The main operating conditions of the storage unit are defined for each considered case through process simulation

Calcium Looping for Thermochemical Storage: Assessment of Intrinsic Reaction Rate and Estimate of Kinetic/Transport Parameters for Synthetic CaO/Mayenite Particles from TGA Data

Mayenite-supported CaO represents an affordable and safetycompliant candidate material for thermochemical storage processes. We here analyze the thermogravimetric analysis (TGA) performance of synthetic CaO/mayenite micrometric powder under carbonatation/calcination looping and develop a model to interpret and analyze the experimental results. In the experimental campaign, calcination is run at 900 degrees C, while the carbonatation temperature is varied between 600 and 800 degrees C. For the carbonatation reaction, a generalized shrinking core model assuming a thermodynamically consistent first-order kinetic and a conversion-dependent diffusivity of CO2 inside the porous CaCO3 layer is validated through TGA carbonatation tests conducted with CO2/N-2 mixtures at different compositions. Interestingly, the kinetic constant of this reaction is found to be relatively insensitive to the temperature in the interval considered. In contrast, diffusion-limited regimes are never found for the calcination reaction so that this phase of the cycle can be predicted based on a single kinetic constant of the heterogeneous reaction. This constant is found to follow the typical Arrhenius-type dependence on temperature. Sizably different kinetic and transport parameters are obtained in the first carbonation performed on virgin CaO/mayenite particles with respect to those associated with subsequent cycles. When different parameters are afforded for the first and following cycles, the shrinking core model proposed closely predicts the TGA data over five CaO/CaCO3 cycles. The results found constitute an essential preliminary piece of information for designing equipment geometry and operating conditions of industrial-scale reactors. In this respect, knowledge of the parameters defining the intrinsic reaction rates and diffusive transport is essential in defining the optimal conversion of the material associated with minimal looping time