Juvenile systemic sclerosis (JSSc) is a rare disease of childhood and currently no international consensus exists with regard to its assessment and treatment. This SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) initiative, based on expert opinion informed by the best available evidence, provides recommendations for the assessment and treatment of patients with JSSc with a view to improving their outcome. Experts focused attention not only on the skin assessment but also on the early signs of internal organ involvement whose proper treatment can significantly affect the long-term outcome. A score for disease severity is proposed in order to perform a structured assessment of outcome over time but a validation in a wider patient population is recommended. Finally, a stepwise treatment approach is proposed in order to unify the standard of care throughout Europe with the aim to reduce morbidity and mortality in this disease.

Quality of measurement results and their accuracy in not neccessary to be high in conventional systems, approximate results are enough to know exactly what happens in the system. However, with increased penetration of renewable sources in the grid, need for high quality and precise measurements has risen. In order to have precise information about power quality, good knowledge of the behaviour of power quality analyzers is needed. Good knowledge can only be obtained by calibration of those meters. This paper describes start of development of a fully traceable power quality reference setup for the calibration of power quality analyzers based on digital sampling of voltage/current signals, and shown preliminary results.

Dedifferentiation and acquisition of chromosomal instability in renal cell carcinoma portends dismal prognosis and aggressive clinical behavior. However, the absence of reliable experimental models dramatically impacts the understanding of mechanisms underlying malignant progression. Here we established an in vivo genetic platform to rapidly generate somatic mosaic genetically engineerd immune-competent mouse models of renal tumors, recapitulating the genomic and phenotypic features of these malignancies. Leveraging somatic chromosomal engineering, we demonstrated that ablation of the murine locus syntenic to human 9p21 drives the rapid expansion of aggressive mesenchymal clones with prominent metastatic behavior, characterized by early emergence of chromosomal instability, whole-genome duplication, and conserved patterns of aneuploidy. This model of punctuated equilibrium provides a remarkable example of cross-species convergent evolution. Significance To better understand the role of 9p21 in malignant progression, we generated a somatic mosaic GEMM of renal cancer, capturing the histological, genomic and evolutionary features of human disease. With this technology we demonstrated a critica role of 9p21 loss in metastatic evolution of RCC and provide a unique tool for testing new therapeutic treatments.

This paper presents a laboratory setup for testing and experimental verification of fault detection methods based on contactless measurement of power line magnetic field. The setup consists of a physical power line model scaled to laboratory conditions with two orthogonal measuring coils installed on one support pole underneath phase conductors. Voltages induced in the coils are amplified and filtered out by using analog electrical circuits and are fed to a digital data acquisition board. The sampled voltages are digitally processed by employing a generalized fault detection method previously developed by the authors. In this paper the main steps for designing the measuring coils, analog amplifying and filtering circuits are described in detail. The developed laboratory setup is verified by experiments for different shunt and series faults.

Optimal sizing of standalone hybrid renewable power supply for mobile telephony base stations is considered in this paper. This task is very complex due to stochastic nature of input variables such as wind speed, solar irradiance, ambient temperature and electric load. Further concern is modeling of the power consumed by cooling devices which is also uncertain variable dependent both on ambient conditions and power dissipation of telecommunication equipment. The realistic behavior of the station both in electrical and thermal terms can be analyzed only by a comprehensive probabilistic simulator. The basic idea of this paper is to systematically vary the main input parameters (rated powers of wind turbine and photovoltaic system along with battery capacity) and to run a Monte Carlo simulation for each considered variant observing the technical and economic indicators. Based on the minimum annual costs, it is possible to choose the best available investment solution. Simple optimization can be extended to multi-objective by adding some additional goals, such as minimizing the operating time of a diesel backup generator. The optimization method is tested on an illustrative example of a base station located in the Mediterranean area.