The natural history of hypertrophic cardiomyopathy

In the early years of the disease recognition, hypertrophic cardiomyopathy (HCM) was viewed as an ominous disease with unfavourable prognosis and with an annual mortality between 4% and 6%. At that time, 73% of the patients reported in the literature came from only two referral centres. With the introduction of echocardiography, our understanding of HCM has improved and non-selected patient populations were assembled in several centres. A more benign prognostic profile was documented with an annual mortality rate of 1.5% or less. In the 2000s, important therapeutic interventions further improved the prognosis of patients with HCM: implantable-cardioverter defibrillator for prevention of sudden death, heart transplantation for treatment of severe refractory heart failure, and an extensive treatment with myectomy for relief of left ventricular outflow tract gradient. The natural history of HCM has changed substantially with contemporary treatment achieving an annual mortality rate less than 1% with extended longevity and a greatly improved quality of life

Imprimitive groups highly transitive on blocks

We classify imprimitive groups acting highly transitively on blocks and satisfying conditions common in geometry. They can be realized as suitable subgroups of twisted wreath products

Identification of DC thermal steady-state differential inductance of ferrite power inductors

In this paper, we propose a method for the identification of the differential inductance of saturable ferrite inductors adopted in DC–DC converters, considering the influence of the operating temperature. The inductor temperature rise is caused mainly by its losses, neglecting the heating contribution by the other components forming the converter layout. When the ohmic losses caused by the average current represent the principal portion of the inductor power losses, the steady-state temperature of the component can be related to the average current value. Under this assumption, usual for saturable inductors in DC–DC converters, the presented experimental setup and characterization method allow identifying a DC thermal steady-state differential inductance profile of a ferrite inductor. The curve is obtained from experimental measurements of the inductor voltage and current waveforms, at different average current values, that lead the component to operate from the linear region of the magnetization curve up to the saturation. The obtained inductance profile can be adopted to simulate the current waveform of a saturable inductor in a DC–DC converter, providing accurate results under a wide range of switching frequency, input voltage, duty cycle, and out-put current values

Gravity “steps” at Mt. Etna volcano (Italy). Instrumental effects or evidences of earthquake-triggered magma density changes?

On two occasions, sudden gravity changes occurred simultaneously at two summit Etna’s stations, during local low-magnitude earthquakes. A systematic coupling between earthquakes inducing comparable maximum acceleration and displacement at the observation points and gravity steps is missing, implying (a) the non-instrumental nature of the steps and (b) the need for particular underlying conditions for the triggering mechanism(s) to activate. We review some of the volcanological processes that could induce fast underground mass redistributions, resulting in gravity changes at the surface. These processes involve bubbles and crystals present in the magma and require particular conditions in order to be effective as mass-redistributing processes. The gravity steps could be a geophysical evidence of the dynamical stress transfer between tectonic and magmatic systems at a local scale. Given the implications that these transfers may have on the volcanic activity, routine volcano monitoring should include the observation of fast gravity changes

Geomorphological and geochemical characterization of the 11 August 2008 mud volcano eruption at S. Barbara village (Sicily, Italy) and its possible relationship with seismic activity

On 11 August 2008 a paroxysmal eruption occurred at Santa Barbara mud volcano (MV), located close to Caltanissetta, one of the most densely populated cities of Sicily (Italy). An associated minor event took place on August 2009. Both the events caused severe damage to civil infrastructures located within a range of about 2 km from the eruptive vent. Geomorphological, geochemical, and seismological investigations were carried out for framing the events in the appropriate geodynamic context. Geomorphological surveys recognized, in the immediate surrounding of the main emission point, two different families of processes and landforms: (i) ground deformations and (ii) changes in morphology and number of the fluid emitting vents. These processes were associated to a wider network of fractures, seemingly generated by the shock wave produced by the gas blast that occurred at the main paroxysm. Geochemical characterization allowed an estimation of the source of the fluids, or at least their last standing, at about 3 km depth. Finally, the close time relationships observed between anomalous increments of seismic activity and the two main paroxysmal events accounted for a possible common trigger for both the phenomena, even with different timing due to the very different initial conditions and characteristics of the two processes, i.e. seismogenesis and gas overloading

A Joint Inversion of Ground Deformation and Focal Mechanisms Data for Magmatic Source Modelling

The paucity of geodetic data acquired on active volcanoes can make the understanding of modelling magmatic systems quite difficult. In this study, we propose a novel approach, which allows improving the parameter estimation of analytical models of magmatic sources (e.g., shape, depth, dimensions, volume change, etc.) by means of a joint inversion of surface ground deformation data and P-axes of focal plane solutions. The methodology is first verified against a synthetic dataset of surface deformation and strain within the medium, and then applied to real data from an unrest episode occurred before the May 13 2008 eruption at Mt. Etna (Italy). The main results clearly indicate the joint inversion improves the accuracy of the estimated source parameters by about 70 %. The statistical tests indicate that the source depth is the parameter with the highest increment of accuracy. In addition, a sensitivity analysis confirms that displacements data are more useful to constrain the pressure and the horizontal location of the source than its depth, while the P-axes better constrain the depth estimation

Disentangling the Structure-Activity Relationships of Naphthalene Diimides as Anticancer G-Quadruplex-Targeting Drugs

In the context of developing efficient anticancer therapies aimed at eradicating any sort of tumors, G-quadruplexes represent excellent targets. Small molecules able to interact with G-quadruplexes can interfere with cell pathways specific of tumors and common to all cancers. Naphthalene diimides (NDIs) are among the most promising, putative anticancer G-quadruplextargeting drugs, due to their ability to simultaneously target multiple G-quadruplexes and their strong, selective in vitro and in vivo anticancer activity. Here, all the available biophysical, biological, and structural data concerning NDIs targeting Gquadruplexes were systematically analyzed. Structure−activity correlations were obtained by analyzing biophysical data of their interactions with G-quadruplex targets and control duplex structures, in parallel to biological data concerning the antiproliferative activity of NDIs on cancer and normal cells. In addition, NDI binding modes to G-quadruplexes were discussed in consideration of the structures and properties of NDIs by in-depth analysis of the available structural models of G-quadruplex/NDI complexes

Foreland segmentation along an active convergent margin: New constraints in southeastern Sicily (Italy) from seismic and geodetic observations

We performed an in-depth analysis of the ongoing tectonics of a large sector of southern Sicily, including the Hyblean Foreland and the front of the Maghrebian Chain, as well as the Ionian Sea offshore, through the integration of seismic and GPS observations collected in the nearly two decades. In particular, a dataset consisting of more than 1100 small-to moderate-magnitude earthquakes (1.0 <= M-L <= 4.6) has been used for local earthquake tomography in order to trace the characteristics of the faulting systems, and for focal mechanisms computation to resolve the current local stress field and to characterise the faulting regime of the investigated area. In addition, GPS measurements, carried out on both episodic and continuous stations, allowed us to infer the main features of the current crustal deformation pattern. Main results evidence that the Hyblean Plateau is subject to a general strike-slip faulting regime, with a maximum horizontal stress axis NW-SE to NNW-SSE oriented, in agreement with the Eurasia-Nubia direction of convergence. The Plateau is separated into two different tectonic crustal blocks by the left-lateral strike-slip Scicli-Ragusa Fault System. The western block moves in agreement with central Sicily while the eastern one accommodates part of the contraction arising from the main Eurasia-Nubia convergence. Furthermore, we provided evidences leading to consider the Hyblean-Maltese Escarpment Fault System as an active boundary characterised by a left-lateral strike-slip motion, separating the eastern block of the Plateau from the Ionian basin. All these evidences lend credit to a crustal segmentation of the southeastern Sicily. (C) 2014 Elsevier B.V. All rights reserved

Random Wave Run-up with a Physically-based Lagrangian Shoreline Model

AbstractIn the present paper the run-up of random waves was calculated by means of a numerical method. In situ measurements based on a video imaging technique have been used for the validation of the present numerical model. The on-site run-up measurements have been carried out at Lido Signorino beach, near Marsala, Italy,along a transect, normal to the shore. A video camera and a linear array of rods have been used to obtain field data. Numerical simulations with a 1DH Boussinesq-type of model for breaking waves which takes into account the wave run-up by means of a Lagrangian shoreline model have been carried out. In such simulations random waves of given spectrum have been propagated in a numerical flume having the same beach slope of the measured transect. The comparison between registered and estimated run-up underlined an acceptable agreement. Indeed, the numerical model tends to underestimate the actual R2%, with the maximum underestimate being less than 24%, which is a reasonable error in many cases of engineering interest