Serum creatine kinase isoenzymes in children with osteogenesis imperfecta

This study evaluates serum creatine kinase isoenzyme activity in children with osteogenesis imperfecta to determine its usefulness as a biochemical marker during treatment with bisphosphonate. The changes of creatine kinase (CK) isoenzyme activity during and after discontinuation therapy were observed. These results could be useful in addressing over-treatment risk prevention. Introduction The brain isoenzyme of creatine kinase (CKbb) is highly expressed in mature osteoclasts during osteoclastogenesis, thus plays an important role in bone resorption. We previously identified high serum CKbb levels in 18 children with osteogenesis imperfect (OI) type 1 treated for 1 year with bisphosphonate (neridronate). In the present study, serum CK isoenzymes were evaluated in the same children with continuous versus discontinued neridronate treatment over a further 2-year follow-up period. Methods This study included 18 children with OI type 1, 12 with continued (group A) and 6 with ceased (group B) neridronate treatment. Auxological data, serum biochemical markers of bone metabolism, bone mineral density z-score, and serum total CK and isoenzyme activities were determined in both groups. Results Serum CKbb was progressively and significantly increased in group A (p < 0.004) but rapidly decreased to undetectable levels in group B. In both groups, the cardiac muscle creatine kinase isoenzyme (CKmb) showed a marked decrease, while serum C-terminal telopeptide (CTx) levels were almost unchanged. Conclusions This study provides evidence of the cumulative effect of neridronate administration in increasing serum CKbb levels and the reversible effect after its discontinuation. This approach could be employed for verifying the usefulness of serum CKbb as a biochemical marker in patients receiving prolonged bisphosphonate treatment. Moreover, the decreased serum CKmb levels suggest a systemic effect of these drugs

Possible mechanisms of electronic phase separation in oxide interfaces

LaAlO3/SrTiO3 ad LaTiO3/SrTiO3 interfaces are known to host a strongly inhomogeneous (nearly) two-dimensional electron gas (2DEG). In this work we present three unconventional electronic mechanisms of electronic phase separation (EPS) in a 2DEG as a possible source of inhomogeneity in oxide interfaces. Common to all three mechanisms is the dependence of some (interaction) potential on the 2DEG's density. We first consider a mechanism resulting from a sizable density-dependent Rashba spin-orbit coupling. Next, we point out that an EPS may also occur in the case of a density-dependent superconducting pairing interaction. Finally, we show that the confinement of the 2DEG to the interface by a density-dependent, self-consistent electrostatic potential can by itself cause an EPS.Comment: 4 pages and 4 figures, Proceedings of the International Conference "Superstripes 2014", 25-31 July 2015, Erice, Ital

Method for size optimisation of large wind\u2013hydrogen systems with high penetration on power grids

Wind power generation is growing rapidly in many locations around the world. Power systems are able to absorb large amounts of wind capacity, but operational problems arise when the wind power penetration becomes high. Such factors as voltage dips, frequency variations, low power system stability, low reactive power and power flow imbalances reduce the economic value and represent a barrier to the unlimited development of wind energy. Hydrogen production from wind power that is not matched with hourly electricity demand appears to be an attractive storage option capable of providing a balancing service to the electricity generators and suppliers for mitigation of the negative impacts due to the random nature of wind. Because of its multi-functionality, hydrogen can be used directly as a fuel, mixed with methane, or transmitted through pipelines to the users. The aim of this paper is to produce useful suggestions for the planning, development and sizing of wind\u2013hydrogen systems by taking into account the local and regional resources, demands, constraints and opportunities. This study considers both the economic and technological variables and describes an optimisation method (OM) for analysing power systems in which part of the electricity generated by a grid-connected wind plant is used to produce hydrogen by electrolysis. An example application of this OM has been developed for a specific geographical area located in central Sicily. Our results identify the potential and the limitations connected to cases that use excess wind power to produce hydrogen for civil applications

CONFISCA DI PREVENZIONE E CIVIL FORFEITURE. VERSO UN NUOVO MODELLO DI &apos;CONFISCA CIVILE&apos; DELLA RICCHEZZA ILLECITA

Il presente lavoro si propone l\u2019obiettivo di studiare l\u2019istituto della confisca misura di prevenzione di cui al d.lgs. n. 159/2011, offrendone una qualificazione in termini civilistici che conduca a una rivisitazione della disciplina ad oggi vigente. L\u2019elaborato prende le mosse da un\u2019indagine sulla genesi e sull\u2019evoluzione storica dell\u2019istituto, a cui segue un\u2019analisi dell\u2019attuale assetto normativo e dei suoi principali problemi applicativi e interpretativi. Il lavoro si focalizza quindi sul problema della qualificazione della natura giuridica di questa confisca, ripercorrendo e sistematizzando le tesi assunte dalla dottrina e dalla giurisprudenza italiane, nonch\ue9 esaminando il diritto vivente europeo, dell\u2019Unione europea e della CEDU. Con un simile approccio viene anche studiata la civil forfeiture dell\u2019ordinamento degli Stati Uniti d\u2019America, individuando nella relativa giurisprudenza della Corte Suprema spunti preziosi per l\u2019individuazione dei criteri distintivi della natura della confisca italiana. Tale natura, in definitiva, non \ue8 parsa essere n\ue9 propriamente \u201cpreventiva\u201d, n\ue9 tantomeno \u201cpunitiva\u201d, entrambe le definizioni risultando \u2013 per varie ragioni \u2013 insoddisfacenti e incoerenti rispetto all\u2019effettiva finalit\ue0 della confisca in esame, che \ue8 quella di neutralizzare, cio\ue8 azzerare, l\u2019incremento patrimoniale generatosi in modo illecito. La confisca dovrebbe cio\ue8 ricondurre il patrimonio della persona nelle condizioni in cui si sarebbe trovato qualora la condotta illecita, e il lucro da essa discendente, non si fosse verificato. La natura dell\u2019istituto risulta dunque \u201ccivile\u201d, in quanto rispondente a logiche essenzialmente privatistiche, quali i modi di acquisto della propriet\ue0 e il divieto generale di ingiustificato arricchimento. Di tale nuovo modello di confisca \u2013 in un\u2019ottica de iure condendo \u2013 vengono infine delineate le caratteristiche essenziali della disciplina applicativa (quale azione dello Stato-attore nei confronti del privato-convenuto) e del relativo statuto costituzionale e convenzionale (quello in materia di tutela della propriet\ue0: in particolare, artt. 41, 42 Cost. e 1 Prot. add. Cedu), sottolineandone le potenziali ricadute positive non solo in termini di efficienza nel contrasto della criminalit\ue0, ma anche di rispetto dei diritti fondamentali della persona.The work aims at studying a particular kind of confiscation, called \u201cconfisca di prevenzione\u201d, ruled by the Legislative Decree n. 159/2011, proposing a qualification of it as a civil measure in order to revise the current legislation. The dissertation starts from the analysis of the origins and evolution of this confiscation, then examines the current legal framework and the main issues related to its application and interpretation. The work thus focuses on the question of the qualification of the nature of the measure, going through the theories developed by Italian scholars and the Italian case law, also studying the European Union Law and the European Convention of Human Rights. By such a method the dissertation deals also with the civil forfeiture of the United States and the related case-law by the American Supreme Court, that offers important hints in order to detect the criteria identifying the nature of the Italian confisca di prevenzione. Indeed its nature, ultimately, does not seems to be \u201cpreventive\u201d or \u201cpunitive\u201d, since both these qualifications do not fit with the real goal of this confiscation, i.e. the neutralization, the resetting, of the illicit store of wealth. The measure should lead back the asset of the person to the situation in which he/she would have been if the unlawful conduct were not committed. The nature of the confiscation is therefore \u201ccivil\u201d, since it is governed by private law rules, including the ways to legally acquire property and the general prohibition of unlawful enrichment. Finally, using a de iure condendo approach, the work drafts the main features ruling the application of this civil confiscation (the action of the State against the owner) and the guarantees, set by the Italian Constitution and the European Convention (articles 41, 42 Const. and article 1 of Protocol n.1 of ECHR), pointing out its potential benefits both on the efficiency of this tool and on the protection of the fundamental human rights